Document:

Prepared by R.R. Donnelley Financial -- EX-4.2

 Exhibit 4.2 

AMENDED AND RESTATED INVESTOR RIGHTS AGREEMENT 

THIS AMENDED AND RESTATED INVESTOR RIGHTS AGREEMENT (the “Agreement”) is made as of the 6th day of December, 2006 and among
XENON PHARMACEUTICALS INC., a corporation (the “Company”) continued under the Canada Business Corporations Act and the investors listed on Exhibit A and Exhibit B hereto, referred to hereinafter as the “Investors” and
each individually as an “Investor” and other shareholders of the Company who have signed an agreement to be bound by this Agreement or the First Investors Rights Agreement. 

RECITALS 
 WHEREAS, the Investors listed
in Exhibit A (the “Series B Investors”) purchased Series B preferred shares (“Series B Shares”) of the Company and entered into an investor rights agreement with the Company (the “First Investor Rights
Agreement”) as of May 19, 2000; 
 WHEREAS, the Investors listed in Exhibit B (the “Series E
Investors”), other than Novartis Pharma AG (“Novartis”), MX Associates LLP (“MXA”), Lipoterx Ltd. (“Lipoterx”), Takeda Research Investment, Inc. (“TRI”) and other subscribers of Series E preferred
shares of the Company (“Series E Shares”) marked with an asterisk in Exhibit B hereto, purchased Series E Shares pursuant to that certain share purchase agreement dated March 26, 2001 and entered into an investor rights
agreement with the Company and the Series B Investors (the “Second Investor Rights Agreement”) as of March 26, 2001, which amended and restated the First Investor Rights Agreement; 

WHEREAS, Novartis purchased Series E Shares pursuant to a share purchase agreement (the “Novartis Purchase Agreement”)
dated September 20, 2004 (the “Novartis Financing”) and entered into an investor rights agreement with the Company, the then Series B Investors and the then Series E Investors (the “Third Investor Rights
Agreement”) as of October 26, 2004 which amended and restated the Second Investor Rights Agreement; 
 WHEREAS, MXA and
Lipoterx (collectively, the “Investor Group”) purchased Series E Shares pursuant to a share purchase agreement (the “Investor Group Purchase Agreement”) dated February 27, 2006 and entered into an investor rights
agreement with the Company, the Series B Investors, and the then Series E Investors (the “Fourth Investor Rights Agreement”) as of April 10, 2006 which amended and restated the Third Investor Rights Agreement; 

WHEREAS, TRI purchased Series E Shares pursuant to a share purchase agreement (the “TRI Purchase Agreement”) dated
May 19, 2006 and entered into an investor rights agreement with the Company, the then Series B Investors, the then Series E Investors, Novartis and the Investor Group (the “Fifth Investor Rights Agreement”) as of
June 30, 2006 which amended and restated the Fourth Investor Rights Agreement; 
 WHEREAS, Takeda purchased Series E Shares
pursuant to a share purchase agreement (the “Takeda Purchase Agreement”) dated September 28, 2006 (the “Takeda Financing”); 

WHEREAS, Roche Finance Ltd. (“Roche”) purchased Series E Shares pursuant to a share purchase agreement (the “Roche
Purchase Agreement”) dated November 1, 2006 (the “Roche Financing”); 

 WHEREAS, in connection with the consummation of the Takeda Financing, the parties desire
to enter into this Agreement as the Restated and Amended Investor Rights Agreement in order to grant registration, information rights and other rights to the Investors as set forth below. 

WHEREAS, in connection with the consummation of the Roche Financing, the parties also desire to enter into this Agreement as the
Restated and Amended Investor Rights Agreement in order to grant registration, information rights and other rights to the Investors as set forth below. 

NOW THEREFORE, in consideration of these premises and for other good and valid consideration, the receipt and sufficiency of which are
hereby acknowledged, the parties hereto agree as follows: 
 ARTICLE 1 

GENERAL 
  

	1.1	Definitions 

 As used in this Agreement the following terms shall have the following respective meanings: 

“Articles of the Company” means the articles of the Company as amended from time to time; 

“Common Shares” means the Company’s common shares, as they may be renamed or redesignated from, time to time. 

“Exchange Act” means the United States Securities Exchange Act of 1934, as amended. 

“Form S-3” or “Form F-3” means such form under the Securities Act as in effect on the date hereof or any successor or similar
registration form under the Securities Act subsequently adopted by the SEC which permits inclusion or incorporation of substantial information by reference to other documents filed by the Company with the SEC. 

“Holder” means any person owning of record Registrable Securities that have not been sold to the public or any assignee of record of such
Registrable Securities in accordance with Section 2.10 hereof. 
 “Initial Offering” means the Company’s first firm commitment
underwritten public offering of its Common Shares registered under the Securities Act. 
 “Register,” “registered,” and
“registration” refer to a registration effected by preparing and filing a registration statement in compliance with the Securities Act, and the declaration or ordering of effectiveness of such registration statement or document. 

“Registrable Securities” means (a) Common Shares of the Company issued or issuable upon conversion of the Shares; and (b) any Common
Shares of the Company issued as (or issuable upon the conversion or exercise of any warrant, right or other security which is issued as) a dividend or other distribution with respect to, or in exchange for or in replacement of, such above-described
securities. Notwithstanding the foregoing, Registrable Securities shall not include any securities sold by a person to the public either pursuant to a registration statement or Rule 144 or sold in a private transaction in which the
transferor’s rights under Article 2 of this Agreement are not assigned. 

  
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 “Registrable Securities then outstanding” shall be the number of shares determined by calculating the
total number of shares of the Company’s Common Shares that are Registrable Securities and either (a) are then issued and outstanding or (b) are issuable pursuant to then exercisable or convertible securities. 

“Registration Expenses” shall mean all expenses incurred by the Company in complying with Sections 2.2, 2.3 and 2.4 hereof, including, without
limitation, all registration and filing fees, printing expenses, fees and disbursements of counsel for the Company, reasonable fees and disbursements not to exceed twenty-five thousand U.S. dollars ($25,000 U.S.) of a single special counsel for the
Holders, blue sky fees and expenses and the expense of any special audits incident to or required by any such registration (but excluding the compensation of regular employees of the Company which shall be paid in any event by the Company). 

“SEC” or “Commission” means the United States Securities and Exchange Commission. 

“Securities Act” shall mean the United States Securities Act of 1933, as amended. 

“Selling Expenses” shall mean all underwriting discounts and selling commissions applicable to the sale. 

“Shares” shall mean the Company’s Series B Shares held by the Series B Investors and their permitted assigns, the Company’s
Series E Shares held by the Series E Investors and their permitted assigns, and the Company’s Series A preferred shares held by the holders on May 19, 2000 of Series A preferred shares of the Company who have agreed to
become parties to and bound by this Agreement as of the date hereof and their permitted assigns. 
 “Special Registration Statement” shall mean a
registration statement relating to any employee benefit plan or with respect to any corporate reorganisation or other transaction under Rule 145 of the Securities Act or other transaction registered in Form S-4 or F-4 (or substantially
similar form under the Securities Act). 
 ARTICLE 2 

REGISTRATION; RESTRICTIONS ON TRANSFER 
  

	2.1	Restrictions on Transfer. 

  

	 	(a)	Each Holder agrees not to make any disposition of all or any portion of the Shares or Registrable Securities unless and until: 

  

	 	(i)	There is then in effect a registration statement under the Securities Act covering such proposed disposition and such disposition is made in accordance with such registration statement; or 

 

	 	(ii)	(A) The transferee has agreed in writing to be bound by the terms of this Agreement (including, in the case of a transfer of Series E Shares held by Novartis, the provisions of Sections 5.1, 5.2 and 5.3
hereof), (B) such Holder shall have notified the Company of the proposed disposition and shall have furnished the Company with a detailed statement of the circumstances surrounding the proposed disposition, and (C) if reasonably requested
by the Company, such Holder shall have furnished the Company with an opinion of counsel, reasonably satisfactory to the Company, that such disposition will not require registration of such shares under the Securities Act. 

  
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	 	(iii)	Notwithstanding the provisions of paragraphs (i) and (ii) above, no such registration statement or opinion of counsel shall be necessary for a transfer by a Holder (A) which is a partnership to its
partners or former partners in accordance with partnership interests, (B) which is a corporation to its shareholders in accordance with their interest in the corporation, (C) which is a limited liability company to its members or former
members in accordance with their interest in the limited liability company, (D) to the Holder’s family member or trust for the benefit of an individual Holder, or (E) to (I) any limited partnership of which the general partner is
under common control with those persons who controlled the Holder or its manager or general partner, as the case may be, as of the date of the transfer; (II) any corporation or other person whose senior officers are, or which is managed by, a
corporate manager whose senior officers are common officers of the Holder or its manager or general partner, as the case may be, as of the date of the transfer; (III) to persons who are bona fide investors (including the general partner or fund
manager, as the case may be, and any of its associates or affiliates) in the Holder who are entitled to participate in a distribution of the assets of the Holder upon winding up, liquidation or dissolution where the Shares are distributed to them on
such occurrence; and (IV) the parent, subsidiary or affiliate of the Holder; provided that in each case the transferee will be subject to the terms of this Agreement to the same extent as if he were an original Holder hereunder; and no such
registration statement or agreement, notice, information or opinion of counsel shall be necessary in respect of a disposition that is not subject to the requirements of the Securities Act or is a disposition that does not require registration of
such shares under the Securities Act. 

  

	 	(b)	Each certificate representing Shares or Registrable Securities, where required by the Company, shall (unless otherwise permitted by the provisions of the Agreement) be stamped or otherwise imprinted with a legend
substantially similar to the following (in addition to any legend required under applicable state securities laws): 

 THE
SECURITIES REPRESENTED HEREBY HAVE NOT BEEN REGISTERED UNDER THE SECURITIES ACT OF 1933 (THE “ACT”) AND - MAY NOT BE OFFERED, SOLD OR OTHERWISE TRANSFERRED, ASSIGNED, PLEDGED OR HYPOTHECATED UNLESS AND UNTIL REGISTERED UNDER THE ACT OR
UNLESS THE COMPANY HAS RECEIVED AN OPINION OF COUNSEL SATISFACTORY TO THE COMPANY AND ITS COUNSEL THAT SUCH REGISTRATION IS NOT REQUIRED. 
  

	 	(c)	The Company shall be obligated to reissue promptly unlegended certificates at the request of any holder thereof if the holder shall have obtained an opinion of counsel (which counsel may be counsel to the Company)
reasonably acceptable to the Company to the effect that the securities proposed to be disposed of may lawfully be so disposed of without registration, qualification or legend. 

 

	 	(d)	Any legend endorsed on an instrument pursuant to applicable state securities laws and the stop-transfer instructions with respect to such securities shall be removed upon receipt by the Company of an order of the
appropriate blue sky authority authorizing such removal. 

  
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	2.2	Demand Registration. 

  

	 	(a)	Subject to the conditions of this Section 2.2, if the Company shall receive a written request from the Holders of a majority in interest of the Registrable Securities then outstanding (the “Initiating
Holders”) that the Company file a registration statement under the Securities Act covering the registration of at least a majority of the Registrable Securities then outstanding (or a lesser percent if the anticipated aggregate offering price,
net of underwriting discounts and commissions, would exceed Cdn. $5,000,000 (a “Qualified Public Offering”)), then the Company shall, within thirty (30) days of the receipt thereof, give written notice of such request to all Holders,
and subject to the limitations of this Section 2.2, effect, as expeditiously as reasonably possible, the registration under the Securities Act of all Registrable Securities that the Holders request to be registered. 

 

	 	(b)	If the Initiating Holders intend to distribute the Registrable Securities covered by their request by means of an underwriting, they shall so advise the Company as a part of their request made pursuant to this
Section 2.2 or any request pursuant to Section 2.4 and the Company shall include such information in the written notice referred to in Section 2.2(a) or Section 2.4(a), as applicable. In such event, the right of any Holder to
include its Registrable Securities in such registration shall be conditioned upon such Holder’s participation in such underwriting and the inclusion of such Holder’s Registrable Securities in the underwriting to the extent provided herein.
All Holders proposing to distribute their securities through such underwriting shall enter into an underwriting agreement in customary form with the underwriter or underwriters selected for such underwriting by a majority in interest of the
Initiating Holders (which underwriter or underwriters shall be reasonably acceptable to the Company). Notwithstanding any other provision of this Section 2.2 or Section 2.4, if the underwriter advises the Company that marketing factors
require a limitation of the number of securities to be underwritten (including Registrable Securities) then the Company shall so advise all Holders of Registrable Securities which would otherwise be underwritten pursuant hereto, and the number of
shares that may be included in the underwriting shall be allocated to the Holders of such Registrable Securities on a pro rata basis based on the number of Registrable Securities held by all such Holders (including the Initiating Holders); provided,
however, that the number of shares of Registrable Securities to be included in such underwriting and registration shall not be reduced unless all other securities of the Company are first entirely excluded from the underwriting and registration. Any
Registrable Securities excluded or withdrawn from such underwriting shall be withdrawn from the registration. 

  

	 	(c)	The Company shall not be required to effect a registration pursuant to this Section 2.2: 

  

	 	(i)	prior to the earlier of (A) the third anniversary of the date of this Agreement or (B) one hundred eighty (180) days following the effective date of the registration statement pertaining to the Initial
Offering; 

  
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	 	(ii)	after the Company has effected two (2) registrations pursuant to this Section 2.2, and such registrations have been declared or ordered effective; 

 

	 	(iii)	during the period starting with the date of filing of, and ending on the date one hundred eighty (180) days following the effective date of the registration statement pertaining to a public offering, other than
pursuant to a Special Registration Statement; provided that the Company makes reasonable good faith efforts to cause such registration statement to become effective; 

 

	 	(iv)	if within thirty (30) days of receipt of a written request from Initiating Holders pursuant to Section 2.2(a), the Company gives notice to the Holders of the Company’s intention to make a public offering,
other than pursuant to a Special Registration Statement, within ninety (90) days; 

  

	 	(v)	if the Company shall furnish to Holders requesting a registration statement pursuant to this Section 2.2, a certificate signed by the Chairman of the Board stating that in the good faith judgment of the Board of
Directors of the Company, it would be seriously detrimental to the Company and its shareholders for such registration statement to be effected at such time, in which event the Company shall have the right to defer such filing for a period of not
more than ninety (90) days after receipt of the request of the Initiating Holders; provided that such right to delay a request shall be exercised by the Company not more than once in any twelve (12) month period; or 

 

	 	(vi)	if the Initiating Holders propose to dispose of shares of Registrable Securities that may be immediately registered on Form S-3 or Form F-3 pursuant to a request made pursuant to Section 2.4 below.

  

	2.3	Piggyback Registrations 

 The Company shall notify all Holders of Registrable Securities in writing at least
twenty (20) days prior to the filing of any registration statement under the Securities Act for purposes of a public offering of securities of the Company (including, but not limited to, registration statements relating to secondary offerings
of securities of the Company, but excluding Special Registration Statements) and will afford each such Holder an opportunity to include in such registration statement all or part of such Registrable Securities held by such Holder. Each Holder
desiring to include in any such registration statement all or any part of the Registrable Securities held by it shall, within fifteen (15) days after the above-described notice from the Company, so notify the Company in writing. Such notice
shall state the intended method of disposition of the Registrable Securities by such Holder. If a Holder decides not to include all of its Registrable Securities in any registration statement thereafter filed by the Company, such Holder shall
nevertheless continue to have the right to include any Registrable Securities in any subsequent registration statement or registration statements as may be filed by the Company with respect to offerings of its securities, all upon the terms and
conditions set forth herein. 
  

	 	(a)	 Underwriting. If the registration statement under which the Company gives notice under this Section 2.3 is for an underwritten offering,
the Company shall so advise the Holders of Registrable Securities. In such event, the right of any such Holder to be included in a registration pursuant to this Section 2.3 shall be conditioned upon such Holder’s participation in such
underwriting and the inclusion of such Holder’s Registrable Securities in the underwriting to the extent provided herein. All Holders 

  
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proposing to distribute their Registrable Securities through such underwriting shall enter into an underwriting agreement in customary form with the underwriter or underwriters selected for such
underwriting by the Company. Notwithstanding any other provision of the Agreement, if the underwriter determines in good faith that marketing factors require a limitation of the number of shares to be underwritten, the number of shares that may be
included in the underwriting shall be allocated, first, to the Company; second, to the Holders on a pro rata basis based on the total number of Registrable Securities held by the Holders; and third, to any shareholder of the Company (other than a
Holder) on a pro rata basis. No such reduction shall (i) reduce the securities being offered by the Company for its own account to be included in the registration and underwriting, or (ii) reduce the amount of securities of the selling
Holders included in the registration below twenty-five percent (25%) of the total amount of securities included in such registration, unless such offering is the Initial Offering and such registration does not include shares of any other
selling shareholders, in which event any or all of the Registrable Securities of the Holders may be excluded in accordance with the immediately preceding sentence. In no event will shares of any other selling shareholder be included in such
registration which would reduce the number of shares which may be included by Holders without the written consent of Holders of not less than sixty-six and two-thirds percent (66 2/3%) of the Registrable Securities proposed to be sold in the
offering. If any Holder disapproves of the terms of any such underwriting, such Holder may elect to withdraw therefrom by written notice to the Company and the underwriter, delivered at least ten (10) business days prior to the effective date
of the registration statement. Any Registrable Securities excluded or withdrawn from such underwriting shall be excluded and withdrawn from the registration. For any Holder which is a partnership or corporation, the partners, retired partners and
shareholders of such Holder, or the estates and family members of any such partners and retired partners and any trusts for the benefit of any of the foregoing person shall be deemed to be a single “Holder,” and any pro rata reduction with
respect to such “Holder” shall be based upon the aggregate amount of shares carrying registration rights owned by all entities and individuals included in such “Holder,” as defined in this sentence. 

 

	 	(b)	Right to Terminate Registration. The Company shall have the right to terminate or withdraw any registration initiated by it under this Section 2.3 prior to the effectiveness of such registration whether or
not any Holder has elected to include securities in such registration. The Registration Expenses of such withdrawn registration shall be borne by the Company in accordance with Section 2.5 hereof. 

 

	2.4	Form S-3 or Form F-3 Registration 

 In case the Company shall receive from any Holder or Holders of
Registrable Securities a written request or requests that the Company effect a registration on Form S-3 or Form F-3 (or any successor form) or any similar short-form registration statement and any related qualification or compliance with
respect to all or a part of the Registrable Securities owned by such Holder or Holders, the Company will: 
  

	 	(a)	promptly give written notice of the proposed registration, and any related qualification or compliance, to all other Holders of Registrable Securities; and 

  
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	 	(b)	as soon as practicable, effect such registration and all such qualifications and compliances as may be so requested and as would permit or facilitate the sale and distribution of all or such portion of such
Holder’s or Holders’ Registrable Securities as are specified in such request, together with all or such portion of the Registrable Securities of any other Holder or Holders joining in such request as are specified in a written request
given within fifteen (15) days after receipt of such written notice from the Company; provided, however, that the Company shall not be obligated to effect any such registration, qualification or compliance pursuant to this Section 2.4:

  

	 	(i)	if either Form S-3 or Form F-3 is not available for such offering by the Holders, or 

  

	 	(ii)	if the Holders, together with the holders of any other securities of the Company entitled to inclusion in such registration, propose to sell Registrable Securities and such other securities (if any) at an aggregate
price to the public of less than five hundred thousand Canadian dollars ($500,000 Cdn.), or 

  

	 	(iii)	during the period starting with the date of filing of, and ending on the date one hundred eighty (180) days following the effective date of, the registration statement pertaining to a public offering, other than
pursuant to a Special Registration Statement; provided that the Company makes reasonable good faith efforts to cause such registration to be effective; or 

  

	 	(iv)	if within thirty (30) days of receipt of a written request from any Holder or Holders pursuant to this Section 2.4, the Company gives notice to such Holder or Holders of the Company’s intention to make a
public offering within ninety (90) days, other than pursuant to a Special Registration Statement, or 

  

	 	(v)	if the Company shall furnish to the Holders a certificate signed by the Chairman of the Board of Directors of the Company stating that in the good faith judgment of the Board of Directors of the Company, it would be
seriously detrimental to the Company and its shareholders for such Form S-3 or Form F-3 registration to be effected at such time, in which event the Company shall have the right to defer the filing of the Form S-3 or Form F-3
registration statement for a period of not more than ninety (90) days after receipt of the request of the Holder or Holders under this Section 2.4 provided, that such right to delay a request shall be exercised by the Company not more than
once in any twelve (12) month period, or 

  

	 	(vi)	if the Company has already effected two (2) registrations within the preceding twelve (12) month period on Form S-3 or Form F-3 for the Holders pursuant to this Section 2.4, or

  

	 	(vii)	in any particular jurisdiction in which the Company would be required to qualify to do business or to execute a general consent to service of process in effecting such registration, qualification or compliance.

  

	 	(c)	Subject to the foregoing, the Company shall file a Form S-3 or Form F-3 registration statement covering the Registrable Securities and other securities so requested to be registered as soon as practicable
after receipt of the request or requests of the Holders. Registrations effected pursuant to this Section 2.4 shall not be counted as demands for registration or registrations effected pursuant to Sections 2.2 or 2.3, respectively.

  
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	2.5	Expenses of Registration 

 Except as specifically provided herein, all Registration Expenses incurred in
connection with any registration, qualification or compliance pursuant to Section 2.2 or any registration under Section 2.3 or Section 2.4 herein shall be borne by the Company. All Selling Expenses incurred in connection with any
registrations hereunder, shall be borne by the holders of the securities so registered pro rata on the basis of the number of shares so registered. The Company shall not, however, be required to pay for expenses of any registration proceeding begun
pursuant to Section 2.2 or 2.4, the request of which has been subsequently withdrawn by the Initiating Holders unless (a) the withdrawal is based upon material adverse information concerning the Company of which the Initiating Holders were
not aware at the time of such request or (b) the Holders of a majority of Registrable Securities agree to forfeit their right to one requested registration pursuant to Section 2.2 or 2.4, as applicable, in which event such right shall be
forfeited by all Holders. If the Holders are required to pay the Registration Expenses, such expenses shall be borne by the holders of securities (including Registrable Securities) requesting such registration in proportion to the number of shares
for which registration was requested. If the Company is required to pay the Registration Expenses of a withdrawn offering pursuant to clause (a) above, then the Holders shall not forfeit their rights pursuant to Section 2.2 or
Section 2.4 to a demand registration. 
  

	2.6	Obligations of the Company 

 Whenever required to effect the registration of any Registrable Securities, the
Company shall, as expeditiously as reasonably possible: 
  

	 	(a)	Prepare and file with the SEC a registration statement with respect to such Registrable Securities and use all reasonable efforts to cause such registration statement to become effective, and, upon the request of the
Holders of a majority of the Registrable Securities registered thereunder, keep such registration statement effective for up to ninety days or, if earlier, until the Holder or Holders have completed the distribution related thereto.

  

	 	(b)	Prepare and file with the SEC such amendments and supplements to such registration statement and the prospectus used in connection with such registration statement as may be necessary to comply with the provisions of
the Securities Act with respect to the disposition of all securities covered by such registration statement for the period set forth in paragraph (a) above. 

  

	 	(c)	Furnish to the Holders such number of copies of a prospectus, including a preliminary prospectus, in conformity with the requirements of the Securities Act, and such other documents as they may reasonably request in
order to facilitate the disposition of Registrable Securities owned by them. 

  

	 	(d)	Use its reasonable efforts to register and qualify the securities covered by such registration statement under such other securities or Blue Sky laws of such jurisdictions as shall be reasonably requested by the
Holders; provided that the Company shall not be required in connection therewith or as a condition thereto to qualify to do business or to file a general consent to service of process in any such states or jurisdictions. 

  
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	 	(e)	In the event of any underwritten public offering, enter into and perform its obligations under an underwriting agreement, in usual and customary form, with the managing underwriter(s) of such offering. Each Holder
participating in such underwriting shall also enter into and perform its obligations under such an agreement. 

  

	 	(f)	Notify each Holder of Registrable Securities covered by such registration statement at any time when a prospectus relating thereto is required to be delivered under the Securities Act of the happening of any event as a
result of which the prospectus included in such registration statement, as then in effect, includes an untrue statement of a material fact or omits to state a material fact required to be stated therein or necessary to make the statements therein
not misleading in the light of the circumstances then existing. The Company will use reasonable efforts to amend or supplement such prospectus in order to cause such prospectus not to include any untrue statement of a material fact or omit to state
a material fact required to be stated therein or necessary to make the statements therein not misleading in the light of the circumstances then existing. Each Holder of Registrable Securities agrees that upon receipt of any notice from the Company
of the happening of any event of the kind described in this paragraph (f) of this Section 2.6, such Holder will discontinue such Holder’s disposition of Registrable Securities pursuant to the registration statement covering such
Registrable Securities until such Holder’s receipt of the copies of the supplemented or amended prospectus contemplated by paragraph (f) of this Section 2.6 and, if so directed by the Company, will deliver to the Company (at the
Company’s expense) all copies, other than permanent file copies, then in such Holder’s possession of the prospectus covering such Registrable Securities that was in effect at the time of receipt of such notice. 

 

	 	(g)	Use its reasonable efforts to furnish, on the date that such Registrable Securities are delivered to the underwriters for sale, if such securities are being sold through underwriters, (i) an opinion, dated as of
such date, of the counsel representing the Company for the purposes of such registration, in form and substance as is customarily given to underwriters in an underwritten public offering, addressed to the underwriters, if any, and (ii) a letter
dated as of such date, from the independent certified public accountants of the Company, in form and substance as is customarily given by independent certified public accountants to underwriters in an underwritten public offering addressed to the
underwriters. 

  

	2.7	Termination of Registration Rights 

 All registration rights granted under this Article 2 shall terminate and be
of no further force and effect four (4) years after the date of the Company’s Initial Offering. In addition, a Holder’s registration rights shall expire if (a) the Company has completed its Initial Offering and is subject to the
provisions of the Exchange Act, (b) such Holder (together with its affiliates, partners and former partners) holds less than 1% of the Company’s outstanding Common Shares (treating all preferred shares of the Company on an as converted
basis) and (c) all Registrable Securities held by and issuable to such Holder (and its affiliates, partners, former partners, members and former members) may be sold under Rule 144 during any ninety (90) day period. 

  
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	2.8	Delay of Registration; Furnishing Information 

  

	 	(a)	No Holder shall have any right to obtain or seek an injunction restraining or otherwise delaying any such registration as the result of any controversy that might arise with respect to the interpretation or
implementation of this Article 2. 

  

	 	(b)	It shall be a condition precedent to the obligations of the Company to take any action pursuant to Section 2.2, 2.3 or 2.4 that the selling Holders shall furnish to the Company such information regarding
themselves, the Registrable Securities held by them and the intended method of disposition of such securities as shall be required to effect the registration of their Registrable Securities. 

 

	 	(c)	The Company shall have no obligation with respect to any registration requested pursuant to Section 2.2 or Section 2.4 if, due to the operation of subsection 2.2(b), the number of shares or the anticipated
aggregate offering price of the Registrable Securities to be included in the registration does not equal or exceed the number of shares or the anticipated aggregate offering price required to originally trigger the Company’s obligation to
initiate such registration as specified in Section 2.2 or Section 2.4, whichever is applicable. 

  

	2.9	Indemnification 

 In the event any Registrable Securities are included in a registration statement under
Sections 2.2, 2.3 or 2.4: 
  

	 	(a)	 To the extent permitted by law, the Company will indemnify and hold harmless each Holder, the partners, officers and directors of each Holder, any
underwriter (as defined in the Securities Act) for such Holder and each person, if any, who controls such Holder or underwriter within the meaning of the Securities Act or the Exchange Act, against any losses, claims, damages, or liabilities (joint
or several) to which they may become subject under the Securities Act, the Exchange Act or other federal or state law, insofar as such losses, claims, damages or liabilities (or actions in respect thereof) arise out of or are based upon any of the
following statements, omissions or violations (collectively a “Violation”) by the Company: (i) any untrue statement or alleged untrue statement of a material fact contained in such registration statement, including any preliminary
prospectus or final prospectus contained therein or any amendments or supplements thereto, (ii) the omission or alleged omission to state therein a material fact required to be stated therein, or necessary to make the statements therein not
misleading, or (iii) any violation or alleged violation by the Company of the Securities Act, the Exchange Act, any state securities law or any rule or regulation promulgated under the Securities Act, the Exchange Act or any state securities
law in connection with the offering covered by such registration statement; and the Company will pay as incurred to each such Holder, partner, officer, director, underwriter or controlling person for any legal or other expenses reasonably incurred
by them in connection with investigating or defending any such loss, claim, damage, liability or action; provided however, that the indemnity agreement contained in this Section 2.9(a) shall not apply to amounts paid in settlement of any such
loss, claim, damage, liability or action if such settlement is effected without the consent of the Company, which consent shall not be unreasonably withheld, nor shall the Company be liable in any such case for any such loss, claim, damage,
liability or action to the 

  
 -11- 

	 	
extent that it arises out of or is based upon a Violation which occurs in reliance upon and in conformity with written information furnished expressly for use in connection with such registration
by such Holder, partner, officer, director, underwriter or controlling person of such Holder. 

  

	 	(b)	To the extent permitted by law, each Holder will, if Registrable Securities held by such Holder are included in the securities as to which such registration qualifications or compliance is being effected, indemnify and
hold harmless the Company, each of its directors, its officers and each person, if any, who controls the Company within the meaning of the Securities Act, any underwriter and any other Holder selling securities under such registration statement or
any of such other Holder’s partners, directors or officers or any person who controls such Holder, against any losses, claims, damages or liabilities (joint or several) to which the Company or any such director, officer, controlling person,
underwriter or other such Holder, or partner, director, officer or controlling person of such other Holder may become subject under the Securities Act, the Exchange Act or other federal or state law, insofar as such losses, claims, damages or
liabilities (or actions in respect thereto) arise out of or are based upon any Violation, in each case to the extent (and only to the extent) that such Violation occurs in reliance upon and in conformity with written information furnished by such
Holder under an instrument duly executed by such Holder and stated to be specifically for use in connection with such registration; and each such Holder will pay as incurred any legal or other expenses reasonably incurred by the Company or any such
director, officer, controlling person, underwriter or other Holder, or partner, officer, director or controlling person of such other Holder in connection with investigating or defending any such loss, claim, damage, liability or action if it is
judicially determined that there was such a violation; provided, however, that the indemnity agreement contained in this Section 2.9(b) shall not apply to amounts paid in settlement of any such loss, claim, damage, liability or action if such
settlement is effected without the consent of the Holder, which consent shall not be unreasonably withheld; provided further, that in no event shall any indemnity under this Section 2.9 exceed the net proceeds from the offering received by such
Holder. 

  

	 	(c)	Promptly after receipt by an indemnified party under this Section 2.9 of notice of the commencement of any action (including any governmental action), such indemnified party will, if a claim in respect thereof is
to be made against any indemnifying party under this Section 2.9, deliver to the indemnifying party a written notice of the commencement thereof and the indemnifying party shall have the right to participate in, and, to the extent the
indemnifying party so desires, jointly with any other indemnifying party similarly noticed, to assume the defense thereof with counsel mutually satisfactory to the parties; provided, however, that an indemnified party shall have the right to retain
its own counsel, with the fees and expenses to be paid by the indemnifying party, if representation of such indemnified party by the counsel retained by the indemnifying party would be inappropriate due to actual or potential differing interests
between such indemnified party and any other party represented by such counsel in such proceeding. The failure to deliver written notice to the indemnifying party within a reasonable time of the commencement of any such action, if materially
prejudicial to its ability to defend such action, shall relieve such indemnifying party of any liability to the indemnified party under this Section 2.9, but the omission so to deliver written notice to the indemnifying party will not relieve
it of any liability that it may have to any indemnified party otherwise than under this Section 2.9. 

  
 -12- 

	 	(d)	If the indemnification provided for in this Section 2.9 is held by a court of competent jurisdiction to be unavailable to an indemnified party with respect to any losses, claims, damages or liabilities referred to
herein, the indemnifying party, in lieu of indemnifying such indemnified party thereunder, shall to the extent permitted by applicable law contribute to the amount paid or payable by such indemnified party as a result of such loss, claim, damage or
liability in such proportion as is appropriate to reflect the relative fault of the indemnifying party on the one hand and of the indemnified party on the other in connection with the Violation(s) that resulted in such loss, claim, damage or
liability, as well as any other relevant equitable considerations. The relative fault of the indemnifying party and of the indemnified party shall be determined by a court of law by reference to, among other things, whether the untrue or alleged
untrue statement of a material fact or the omission to state a material fact relates to information supplied by the indemnifying party or by the indemnified party and the parties’ relative intent, knowledge, access to information and
opportunity to correct or prevent such statement or omission; provided, that in no event shall any contribution by a Holder hereunder exceed the proceeds from the offering received by such Holder. 

 

	 	(e)	The obligations of the Company and Holders under this Section 2.9 shall survive completion of any offering of Registrable Securities in a registration statement and the termination of this agreement. No
Indemnifying Party, in the defense of any such claim or litigation, shall, except with the consent of each Indemnified Party, consent to entry of any judgment or enter into any settlement which does not include as an unconditional term thereof the
giving by the claimant or plaintiff to such Indemnified Party of a release from all liability in respect to such claim or litigation. 

  

	2.10	Assignment of Registration Rights 

 The rights to cause the Company to register Registrable Securities pursuant
to this Article 2 may be assigned by a Holder to a transferee or assignee of Registrable Securities which (a) is a subsidiary, parent, affiliate, general partner, limited partner, retired partner, member or retired member of a Holder,
(b) is a Holder’s family member or trust for the benefit of an individual Holder, (c) acquires at least one hundred thousand (100,000) shares of Registrable Securities (as adjusted for any subdivision or consolidation of shares)
or (d) is (I) a limited partnership of which the general partner is under common control with those persons who controlled the Holder or its manager or general partner, as the case may be, as of the date of the transfer, (II) a corporation
or other person whose senior officers are, or which is managed by a corporate manager whose senior officers are, common officers of the Holder or its manager or general partner, as the case may be, as of the date of the transfer; and (III) a person
who is a bona fide investor (including the general partner or fund manager, as the case maybe, and any of its associates or affiliates) in the Holder who is entitled to participate in a distribution of the assets of the Holder upon winding up,
liquidation or dissolution where the Shares are distributed to them on such occurrence, provided, however, (i) the transferor shall, within ten (10) days after such transfer, furnish to the Company written notice of the name and address of
such transferee or assignee and the securities with respect to which such registration rights are being assigned and (ii) such transferee shall agree in writing to be subject to all restrictions set forth in this Agreement. 

  
 -13- 

	2.11	Amendment of Registration Rights 

 Any provision of this Article 2 may be amended and the observance thereof may
be waived (either generally or in a particular instance and either retroactively or prospectively), only with the written consent of the Company and the consent of both the Holders of at least a majority of the Registrable Securities then
outstanding and the consent of Investors holding at least 75% of the Registrable Securities then held by the Investors. Any amendment or waiver effected in accordance with this Section 2.11 shall be binding upon each Holder and the Company. By
acceptance of any benefits under this Article 2, Holders of Registrable Securities hereby agree to be bound by the provisions hereunder. 
  

	2.12	Limitation on Subsequent Registration Rights 

 Other than as provided in Section 10.11, after the date of
this Agreement, the Company shall not, without the prior written consent of the Holders of at least sixty-six and two-thirds percent (66 2/3%) of the Registrable Securities then outstanding, enter into any agreement with any holder or prospective
holder of any securities of the Company that would grant such holder registration rights pari passu or senior to those granted to the Holders hereunder. Notwithstanding the foregoing or any other provision of this Agreement, the holders of
Series A preferred shares of the Company who have become parties to and bound by the First Investor Rights Agreement except for the rights and obligations under Article 3 and Article 4 of the First Investor Rights Agreement shall be considered
Holders for all purposes under Article 2 of this Agreement. 
  

	2.13	“Market Stand-OM Agreement; Agreement to Furnish Information 

 Each Holder hereby agrees that such Holder
shall not sell, transfer, make any short sale of, grant any option for the purchase of, or enter into any hedging or similar transaction with the same economic effect as a sale, any Common Shares (or other securities) of the Company held by such
Holder (other than those included in the registration) for a period specified by the representative of the underwriters of Common Shares (or other securities) of the Company not to exceed one hundred eighty (180) days following the effective
date of a registration statement of the Company filed under the Securities Act or a prospectus filed in Canada as contemplated in Section 2.15; provided that: 
  

	 	(a)	such agreement shall apply only to the Company’s Initial Offering or an offering contemplated in Section 2.15; and 

  

	 	(b)	all officers and directors of the Company enter into similar agreements. 

 Each Holder agrees to execute and
deliver such other agreements as may be reasonably requested by the Company or the underwriter which are consistent with the foregoing or which are necessary to give further effect thereto. In addition, if requested by the Company or the
representative of the underwriters of Common Shares (or other securities) of the Company, each Holder shall provide, within ten (10) days of such request, such information as may be required by the Company or, such representative in connection
with the completion of any public offering of the Company’s securities pursuant to a registration statement filed under the Securities Act. The obligations described in this Section 2.13 shall not apply to a registration relating solely to
employee benefit plans on Form S-1 or Form S-8 or similar forms that may be promulgated in the future, or a registration relating solely to a Commission Rule 145 transaction on Form S-4 or similar forms that may be promulgated in the future.
The Company may impose stop-transfer instructions with respect to the shares of Common Shares (or other securities) subject to the foregoing restriction until the end of said one hundred eighty (180) day period. Each Holder agrees that any
transferee of any shares of Registrable Securities shall be bound by this Section 2.13. 

  
 -14- 

	2.14	Rule 144 Reporting 

 With a view to making available to the Holders the benefits of certain rules and
regulations of the SEC which may permit the sale of the Registrable Securities to the public without registration, the Company agrees to use its best efforts to: 
  

	 	(a)	Make and keep public information available, as those terms are understood and defined in SEC Rule 144 or any similar or analogous rule promulgated under the Securities Act, at all times after the effective date of
the first registration filed by the Company for an offering of its securities to the general public; 

  

	 	(b)	File with the SEC, in a timely manner, all reports and other documents required of the Company under the Exchange Act; and 

  

	 	(c)	So long as a Holder owns any Registrable Securities, furnish to such Holder forthwith upon request: a written statement by the Company as to its compliance with the reporting requirements of said Rule 144 of the
Securities Act, and of the Exchange Act (at any time after it has become subject to such reporting requirements); a copy of the most recent annual or quarterly report of the Company; and such other reports and documents as a Holder may reasonably
request in availing itself of any rule or regulation of the SEC allowing it to sell any such securities without registration. 

  

	2.15	Filing of Canadian Prospectus 

 If the Company at any time files a prospectus in any province of Canada, the
Company shall file such prospectus, and use all reasonable efforts to obtain a receipt therefor, in Ontario and any other province in which Series E Shares have been issued, and such prospectus shall qualify the issuance of Common Shares
issuable upon the conversion of the Series E Shares such that the underlying Common Shares resulting from the conversion of the Series E Shares will be freely tradable under Canadian securities laws, to the maximum extent permitted by
applicable law. 
 ARTICLE 3 

COVENANTS OF THE COMPANY 
  

	3.1	Basic Financial Information and Reporting 

  

	 	(a)	The Company will maintain true books and records of account in which full and correct entries will be made of all its business transactions pursuant to a system of accounting established and administered in accordance
with Canadian generally accepted accounting principles consistently applied, and will set aside on its books all such proper accruals and reserves as shall be required under Canadian generally accepted accounting principles consistently applied.

  

	 	(b)	 As soon as practicable after the end of each fiscal year of the Company, and in any event within one hundred twenty (120) days thereafter, the
Company will furnish each Investor a balance sheet of the Company, as at the end of such fiscal year, and a statement of income and a statement of cash flows of the Company, for such year, all

  
 -15- 

	 	
prepared in accordance with Canadian generally accepted accounting principles consistently applied and setting forth in each case in comparative form the figures for the previous fiscal year, all
in reasonable detail. Such audited financial statements shall be accompanied by a report and opinion thereon by independent public accountants of national standing selected by the Company’s Board of Directors. 

 

	 	(c)	The Company will furnish each Investor, as soon as practicable after the end of the first, second and third quarterly accounting periods in each fiscal year of the Company, and in any event within forty-five
(45) days thereafter, a balance sheet of the Company as of the end of each such quarterly period, and a statement of income and a statement of cash flows of the Company for such period and for the current fiscal year to date, prepared in
accordance with Canadian generally accepted accounting principles, with the exception that no notes need be attached to such statements and year-end audit adjustments need not have been made. 

 

	 	(d)	So long as a Series B Investor listed on Exhibit C hereto owns not less than 2% of the total outstanding Common Shares (calculated on a fully diluted basis) or securities convertible or exchangeable into not
less than 2% of the outstanding Common Shares (calculated on a fully diluted basis), each such Series 13 Investor shall (subject to the following) be entitled to have an observer attend all board meetings of the Company and receive copies of
all materials sent to directors of the Company in connection with a board meeting of the Company; provided however, that the Company shall be under no obligation to deliver any materials hereunder where the board of directors determines, acting
reasonably, that a matter should be considered at an “in camera” session of the board of directors without any observers present. In the event that Ventures West 7 Limited Partnership ceases to own at least 2% of the total outstanding
Common Shares (calculated on a fully diluted basis) or securities convertible or exchangeable into at least 2% of the outstanding Common Shares (calculated on a fully diluted basis), it will cease to have an observer attending all board meetings of
the Company but as long as it owns not less than 1% of the total outstanding Common Shares (calculated on a fully diluted basis) or securities convertible or exchangeable into at least 1% of the outstanding Common Shares (calculated on a fully
diluted basis), it will continue to have the right to receive copies of all materials sent to directors of the Company in connection with a board meeting of the Company; provided however, that the Company shall be under no obligation to deliver any
materials hereunder where the board of directors determines, acting reasonably, that a matter should be considered at an “in camera” session of the board of directors without any observers present. 

 

	3.2	Confidentiality of Records 

 Each Investor agrees to use, and to use its best efforts to insure that its
authorized representatives use, the same degree of care as such Investor uses to protect its own confidential information to keep confidential any information furnished to it which the Company identifies as being confidential or proprietary (so long
as such information is not in the public domain), except that such Investor may disclose such proprietary or confidential information to any partner, subsidiary or parent of such Investor for the purpose of evaluating its investment in the Company
as long as such partner, subsidiary or parent is advised of the confidentiality provisions of this Section 3.2. 

  
 -16- 

	3.3	Reservation of Common Shares 

 The Company will at all times reserve and keep available, solely for issuance and
delivery upon the conversion of the Series B Shares or Series E Shares, as the case may be, all Common Shares issuable from time to time upon such conversion. 
  

	3.4	Proprietary Information and Inventions Agreement 

 The Company shall require all new employees and consultants
from the date of this Agreement to deliver and execute an agreement relating to the Company’s proprietary information in the form currently executed by all employees of the Company (a copy of which has been provided to the Investors as part of
their due diligence). 
  

	3.5	Directors’ Insurance 

 The Company shall, at all times when any Investor shall have a representative on the
Company’s board of directors, an insurance policy covering acts and omissions of directors having a per claim limit of not less than $2 million (Cdn). 
  

	3.6	Termination of Covenants 

 All covenants of the Company contained in Article 3 of this Agreement shall expire
and terminate as to each Investor upon the earlier of (i) the effective date of the registration statement pertaining to the Initial Offering, which results in the preferred shares of the Company being converted into Common Shares, or
(ii) upon (a) the sale, lease or other disposition of all or substantially all of the assets of the Company or (b) an acquisition of the Company by another corporation or entity by consolidation, merger or other reorganization in
which the holders of the Company’s outstanding voting shares immediately prior to such transaction own, immediately after such transaction, securities representing less than fifty percent (50%) of the voting power of the corporation or
other entity surviving such transaction, provided that this Section 3.6(ii)(b) shall not apply to a merger effected exclusively for the purpose of changing the domicile of the Company (a “Change in Control”). 

ARTICLE 4 
 RIGHTS OF
FIRST REFUSAL 
  

	4.1	Subsequent Offerings 

 Each Investor, for so long as such Investor owns not less than 100,000 Shares (a
“Major Investor”), shall have a right of first refusal to purchase its pro rata share of all Equity Securities, as defined below, that the Company may, from time to time, propose to sell and issue after the date of this Agreement, other
than the issuance of the Series E Shares pursuant to the Investor Group Purchase Agreement, the issuance of an aggregate of 379,594 Series E Shares to Chancellor V-A L.P., Chancellor V, .L.P., Citiventure 2000, L.P. and Alpha Technologies
Limited, the issuance of the Series E Shares to TRI pursuant to the TRI Purchase Agreement, the issuance of the Series E Shares to Takeda pursuant to the Takeda Purchase Agreement, the issuance of the Series E Shares to Roche pursuant
to the Roche Purchase Agreement and the issuance of the Equity Securities excluded by Section 4.6 hereof. Each Major Investor’s pro rata share is equal to the ratio of (a) the number of shares of the Company’s Common Shares
(including all shares of Common Shares issued or issuable upon conversion of the Shares) which such Major Investor is deemed to be a holder immediately prior to the issuance of such Equity Securities to (b) the total number of shares of the
Company’s 

  
 -17- 

 
outstanding Common Shares (including all shares of Common Shares issued or issuable upon conversion of the Shares or upon the exercise of any outstanding warrants or options) immediately prior to
the issuance of the Equity Securities. The term “Equity Securities” shall mean (i) any Common Shares, Series B Shares, Series E Shares or other security of the Company, (ii) any security convertible, with or without
consideration, into any Common Shares, Series B Shares, Series E Shares or other security (including any option to purchase such a convertible security), (iii) any security carrying any warrant or right to subscribe to or purchase any
Common Shares, Series B Shares, Series E Shares or other security or (iv) any such warrant or right. 
  

	4.2	Exercise of Rights 

 If the Company proposes to issue any Equity Securities, it shall give each Major Investor
written notice of its intention, describing the Equity Securities, the price and the terms and conditions upon which the Company proposes to issue the same. Each Major Investor shall have ten business days from the giving of such notice to agree to
purchase its pro rata share of the Equity Securities for the price and upon the terms and conditions specified in the notice by giving written notice to the Company and stating therein the quantity of Equity Securities to be purchased.
Notwithstanding the foregoing, the Company shall not be required to offer or sell such Equity Securities to any Major Investor who would cause the Company to be in violation of applicable Canadian, provincial or U.S. federal securities laws by
virtue of such offer or sale. 
  

	4.3	Issuance of Equity Securities to Other Persons 

 If not all of the Major Investors elect to purchase their pro
rata share of the Equity Securities, then the Company shall promptly notify in writing the Major Investors who do so elect and shall offer such Major Investors the right to acquire such unsubscribed shares. The Major Investors shall have five
(5) days after receipt of such notice to notify the Company of its election to purchase all or a portion thereof of the unsubscribed shares. If the Major Investors fail to exercise in full the rights of first refusal, the Company shall have
ninety (90) days thereafter to sell the Equity Securities in respect of which the Major Investor’s rights were not exercised, at a price and upon general terms and conditions materially no more favorable to the purchasers thereof than
specified in the Company’s notice to the Major Investors pursuant to Section 4.2 hereof. If the Company has not sold such Equity Securities within ninety (90) days of the notice provided pursuant to Section 4.2, the Company shall
not thereafter issue or sell any Equity Securities, without first offering such securities to the Major Investors in the manner provided above. 
  

	4.4	Termination and Waiver of Rights of First Refusal 

 The rights of first refusal established by this Article 4
shall not apply to, and shall terminate upon the earlier of: 
  

	 	(a)	effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering is conducted at a minimum price of $4.42 (U.S.) per Common Share (subject to
adjustment for any share consolidation or subdivision or the grant of any stock dividends subsequent to the date hereof) and for minimum gross proceeds (before underwriting discounts, commission, expenses of issue and fees) of not less than $40
million (U.S.) and the Common Shares are listed or quoted upon a recognized senior stock exchange or public securities quotation system (including, without limitation, the Toronto Stock Exchange or the NASDAQ National Market); or 

 

	 	(b)	a Change in Control. 

  
 -18- 

 The rights of first refusal established by this Article 4 may be amended, or any provision waived with the
written consent of Major Investors holding two thirds in interest of the Registrable Securities held by all Major Investors and in the case of an amendment, with the written consent of the Company, or as permitted by Section 10.6.
Notwithstanding any other provision of this Agreement, the written consent of the Company and Series B Investors who are Major Investors holding two thirds in interest of the Registrable Securities held by Series B Investors who are Major
Investors is required to effect any amendment to subsection 4.4(a) to the extent applicable to the termination of the rights of first refusal with respect to the rights of Series B Investors and the written consent of the Company and
Series E Investors who are Major Investors holding two thirds in interest of the Registrable Securities held by Series E Investors who are Major Investors is required to effect an amendment to subsection 4.4(a) to the extent applicable to
the termination of the rights of first refusal with respect to the rights of Series E Investors. 
  

	4.5	Transfer of Rights of First Refusal 

 The rights of first refusal of each Major Investor under this Article 4
may be transferred to the same parties, subject to the same restrictions as any transfer of registration rights pursuant to Section 2.10. 
  

	4.6	Excluded Securities 

 The rights of first refusal established by this Article 4 shall have no application to any
of the following Equity Securities: 
  

	 	(a)	6,829,477 Common Shares (and/or options, warrants or other Common Shares purchase rights issued pursuant to such options, warrants or other rights) to employees, officers or directors of, or consultants or advisors to
the Company or any subsidiary, pursuant to stock purchase or stock option plans or other arrangements (“Employment Securities”) that are approved by the Board of Directors, plus such additional number of Employment Securities, the issuance
of which are approved by the Board of Directors and the shareholders of the Company; 

  

	 	(b)	Equity Securities issued pursuant to any rights or agreements outstanding as of the date of this Agreement (as listed in the Schedule of Exceptions to the Purchase Agreement dated March 26, 2001 (the
“Purchase Agreement”)); 

  

	 	(c)	Equity Securities issued pursuant to any such rights or agreements granted after the date of this Agreement; provided that the rights of first refusal established by this Article 4 applied (or was waived) with respect
to the initial sale or grant by the Company of such rights or agreements; 

  

	 	(d)	any Equity Securities issued for consideration other than cash pursuant to a merger, consolidation, acquisition or similar business combination approved by the Board of Directors; 

 

	 	(e)	Equity Securities issued in connection with any subdivision of shares, stock dividend or recapitalization by the Company; 

  
 -19- 

	 	(f)	Equity Securities issued upon conversion of the Series A, Series B, Series C, Series D and Series E preferred shares; 

 

	 	(g)	up to 1,000,000 Equity Securities issued pursuant to any equipment leasing or loan arrangement, or debt financing from a bank or similar financial or lending institution approved by the Board of Directors provided that
the per share value attributed to such Equity Securities is greater than or equal to $2.9469 (U.S.); 

  

	 	(h)	any Equity Securities that are issued by the Company pursuant to a registration statement filed under the Securities Act or a prospectus filed in Canada as contemplated in Section 2.15; and 

 

	 	(i)	up to 250,000 Equity Securities issued pursuant to agreements approved by the Board of Directors whereby the Company issues Equity Securities in lieu of paying licensing or similar fees in cash provided that the per
share value attributed to such Equity Securities is greater than or equal to $2.9469 (U.S.). 

  

	4.7	Canadian — U.S. Dollar Exchange Rate 

 For the purpose of converting Canadian dollar amounts to U.S.
dollars, or vice versa, on the issuance of securities contemplated in this Article 4, the exchange rate shall be calculated based on the Bank of Canada noon spot rate for U.S. dollars against Canadian dollars on the date that such securities are
issued. 
 ARTICLE 5 

NOVARTIS RIGHTS AND OBLIGATIONS 
  

	5.1	Agreement to Vote 

 Novartis agrees with the Company and the other Investors that it will vote the Series E
Shares held by it, whether at law, pursuant to the share rights attached to the Series E Shares (the “Series E Share Rights”) or pursuant to any agreement to which the other Series E Investors are a party, and it will grant
all consents, waivers and approvals, make all elections and take any action required by such Series E Share Rights or any such agreement (including this Agreement), in the same proportions as the votes cast (or waivers, consents, approvals,
elections or actions taken) by the other Series E Investors. 
  

	5.2	Exception 

 Notwithstanding Section 5.1, Novartis agrees with the Company and the other Investors that it
shall not be required to vote its Series E Shares in accordance with Section 5.1 with respect to matters to be voted on by the holders of Common Shares where the holders of the Series E Shares are entitled to vote their Series E
Shares as if they were Common Shares, whether at law or otherwise. 
  

	5.3	Adjustment to Conversion Rights 

 Novartis agrees with the Company and the other Investors that all references
to US$6.85 in Section 2.5(b)(iii) of the Series E Share Rights shall be deemed to be US$8.25 for the purpose of determining adjustments to conversion rights of the Series E Shares acquired by Novartis pursuant to the Novartis Purchase
Agreement. 

  
 -20- 

 ARTICLE 6 

TRI RIGHTS AND OBLIGATIONS 
  

	6.1	Agreement to Vote 

 TM agrees with the Company and the other Investors that it will vote the Series E
Shares held by it, whether at law, pursuant to the Series E Share Rights or pursuant to any agreement to which the other Series E Investors are a party, and it will grant all consents, waivers and approvals, make all elections and take any
action required by such Series E Share Rights or any such agreement (including this Agreement), in the same proportions as the votes cast (or waivers, consents, approvals, elections or actions taken) by the other Series E Investors. 

 

	6.2	Exception 

 Notwithstanding Section 6.1, TRI agrees with the Company and the other Investors that it shall
not be required to vote its Series E Shares in accordance with Section 6.1 with respect to matters to be voted on by the holders of Common Shares where the holders of the Series E Shares are entitled to vote their Series E Shares
as if they were Common Shares, whether at law or otherwise. 
  

	6.3	Adjustment to Conversion Rights 

 Notwithstanding section 2.5(d) of the share rights attached to the
Series E Shares under the Articles of the Company, TRI will convert all Series E Shares held by it into Common. Shares of the Company immediately prior to completion of any Change of Control Transaction or an Asset Transfer, as such terms
are defined in the Articles of the Company. 
 ARTICLE 7 

OTHER NEW INVESTORS RIGHTS AND OBLIGATIONS 
  

	7.1	Takeda Rights and Obligations 

  

	 	(a)	Agreement to Vote 

 Takeda agrees with the Company and the other Investors that it will vote the Series E
Shares held by it, whether at law, pursuant to the Series E Share Rights or pursuant to any agreement to which the other Series E Investors are a party, and it will grant all consents, waivers and approvals, make all elections and take any
action required by such Series E Share Rights or any such agreement (including this Agreement), in the same proportions as the votes cast (or waivers, consents, approvals, elections or actions taken) by the other Series E Investors. 

 

	 	(b)	Exception 

 Notwithstanding subsection (a) above, Takeda agrees with the Company and the other Investors
that it shall not be required to vote its Series E Shares in accordance with subsection (a) with respect to matters to be voted on by the holders of Common Shares where the holders of the Series E Shares are entitled to vote their
Series E Shares as if they were Common Shares, whether at law or otherwise. 

  
 -21- 

	 	(c)	Adjustment to Conversion Rights 

 Notwithstanding section 2.5(d) of the share rights attached to the
Series E Shares under the Articles of the Company, Takeda will convert all Series E Shares held by it into Common Shares of the Company immediately prior to completion of any Change of Control Transaction or an Asset Transfer, as such
terms are defined in the Articles of the Company. 
  

	7.2	Roche Rights and Obligations 

  

	 	(a)	Agreement to Vote 

 Roche agrees with the Company and the other Investors that it will vote the Series E
Shares held by it, whether at law, pursuant to the Series E Share Rights or pursuant to any agreement to which the other Series E Investors are a party, and it will grant all consents, waivers and approvals, make all elections and take any
action required by such Series E Share Rights or any such agreement (including this Agreement), in the same proportions as the votes cast (or waivers, consents, approvals, elections or actions taken) by the other Series E Investors. 

 

	 	(b)	Exception 

 Notwithstanding subsection (a) above, Roche agrees with the Company and the other Investors
that it shall not be required to vote its Series E Shares in accordance with subsection (a) above with respect to matters to be voted on by the holders of Common Shares where the holders of the Series E Shares are entitled to vote
their Series E Shares as if they were Common Shares, whether at law or otherwise. 
  

	 	(c)	Adjustment to Conversion Rights 

 Notwithstanding section 2.5(d) of the share rights attached to the
Series E Shares under the Articles of the Company, Roche will convert all Series E Shares held by it into Common Shares of the Company immediately prior to completion of any Change of Control Transaction or an Asset Transfer, as such terms
are defined in the Articles of the Company, and shall receive the appropriate consideration for such Common Shares; provided, however, that (i) if such Change of Control Transaction or Asset Transfer occurs more than twenty-four
(24) months following the Closing of the Roche Purchase Agreement, this provision shall not apply and Roche shall have no obligation to convert its Series E Shares into Common Shares and (ii) if the Company should sell Series E
Shares to a Strategic Investor (a Strategic Investor shall mean an investor which is a pharmaceutical or biotechnology company making an investment in connection with a separate licensing agreement with the Company) and the Strategic Investor is not
obligated to convert its Series E Shares into Common Shares upon any Change of Control Transaction or an Asset Transfer, this Agreement shall be amended to delete this Section 7.2 (c). 

ARTICLE 8 
 CONFIRMATION
OF ADJUSTMENT TO CONVERSION RIGHTS 
 ATTACHED TO SERIES E SHARE RIGHTS 

 

	8.1	Confirmation in respect of Adjustment to Conversion Rights 

 Subject to Section 5.3 above, each of the
Series E Investors agrees and confirms with each other and the Company that Section 2.5(b)(iii) of the Articles of the Company (which sets out the provision relating to the “Adjustment to Conversion Right” applicable to
Series E Shares) as set out in an amendment to Articles approved by the shareholders of the Company at a meeting of the shareholders of the Company on March 31, 2006, shall be valid and binding on each of them if and when such provision
becomes effective as part of the Articles of the Company and each of the Series E Investors shall take all reasonable actions to support and give effect to such provision. 

  
 -22- 

 ARTICLE 9 

CONVERSION OF SERIES B PREFERRED SHARES 

AND SERIES E PREFERRED SHARES 
  

	9.1	Conversion of Series B preferred shares and Series E preferred shares 

 The parties hereby agree that
the Company will be able to cause the Series B preferred shares of the Company and Series E preferred shares of the Company to be converted into Common Shares with the closing of a firmly underwritten initial public offering of the Company
if such offering is conducted at a minimum price of $4.42 (U.S.) per Common Share and the minimum gross proceeds to the Company are not less than $40 (U.S.) million and the Common Shares are listed or quoted upon a recognized senior stock exchange
or public securities quotation system (including, without limitation, the Toronto Stock Exchange or the NASDAQ National Market). 

ARTICLE 10ARTICLE 10 

MISCELLANEOUS 
  

	10.1	Governing Law 

 This Agreement shall be governed by and construed under the laws of the Province of British
Columbia. 
  

	10.2	Survival 

 The representations, warranties, covenants, and agreements made herein shall survive any
investigation made by any Investor or Holder and the closing of the transactions contemplated hereby. All statements as to factual matters contained in any certificate or other instrument delivered by or on behalf of the Company pursuant hereto in
connection with the transactions contemplated hereby shall be deemed to be representations and warranties by the Company hereunder solely as of the date of such certificate or instrument. 

 

	10.3	Successors and Assigns 

 Except as otherwise expressly provided herein, the provisions hereof shall enure to the
benefit of, and be binding upon, the successors, assigns, heirs, executors, and administrators of the parties hereto and shall enure to the benefit of and be enforceable by each person who shall be a holder of Registrable Securities from time to
time; provided, however, that prior to the receipt by the Company of adequate written notice of the transfer of any Registrable Securities specifying the full name and address of the transferee, the Company may deem and treat the person listed as
the holder of such shares in its records as the absolute owner and holder of such shares for all purposes, including the payment of dividends or any redemption price. 
  

	10.4	Entire Agreement 

 This Agreement, the Exhibits and Schedules hereto and the other documents delivered pursuant
thereto constitute the full and entire understanding and agreement between the parties with regard to the subjects hereof and no party shall be liable or bound to any other in any manner by any representations, warranties, covenants and agreements
except as specifically set forth herein and therein. 

  
 -23- 

	10.5	Severability 

 In the event one or more of the provisions of this Agreement should, for any reason, be held to
be invalid, illegal or unenforceable in any respect, such invalidity, illegality, or unenforceability shall not affect any other provisions of this Agreement, and this Agreement shall be construed as if such invalid, illegal or unenforceable
provision had never been contained herein. 
  

	10.6	Amendment and Waiver 

  

	 	(a)	Except as otherwise expressly provided, this Agreement may be amended or modified only upon the written consent of the Company and the consent of both the holders of at least a majority of the Registrable Securities and
the consent of Investors holding at least 75% of the Registrable Securities then held by the Investors. 

  

	 	(b)	Except as otherwise expressly provided, the obligations of the Company and the rights of the Holders under this Agreement may be waived only with the written consent of the holders of at least a majority of the
Registrable Securities. 

  

	 	(c)	Notwithstanding the foregoing, this Agreement may be amended with only the written consent of the Company to include additional purchasers of Shares as “Investors”, “Holders” and parties hereto. Any
amendment or waiver effected in accordance with clauses (a) and (b) of this Section 10.6 shall be binding upon each Investor, its successors and assigns, and the Company. 

 

	 	(d)	For the purposes of determining the number of Holders or Investors entitled to vote or exercise any rights hereunder, the Company shall be entitled to rely solely on the list of record holders of its shares as
maintained by or on behalf of the Company. 

  

	10.7	Delays or Omissions 

 It is agreed that no delay or omission to exercise any right, power, or remedy accruing to
any Holder, upon any breach, default or noncompliance of the Company under this Agreement shall impair any such right, power, or remedy, nor shall it be construed to be a waiver of any such breach, default or noncompliance, or any acquiescence
therein, or of any similar breach, default or noncompliance thereafter occurring. It is further agreed that any waiver, permit, consent or approval of any kind or character on any Holder’s part of any breach, default or noncompliance under the
Agreement or any waiver on such Holder’s part of any provisions or conditions of this Agreement must be in writing and shall be effective only to the extent specifically set forth in such writing. All remedies, either under this Agreement, by
law, or otherwise afforded to Holders, shall be cumulative and not alternative. 
  

	10.8	Notices 

 All notices required or permitted hereunder shall be in writing and shall be deemed effectively given:
(a) upon personal delivery to the party to be notified, (b) when sent by confirmed electronic mail or facsimile if sent during normal business hours of the recipient; if not, then on the next business day, (c) five (5) days after
having been sent by registered or certified mail, return receipt requested, postage prepaid, or (d) one (1) business day after deposit with a nationally recognized overnight courier, 

  
 -24- 

 
specifying next day delivery, with written verification of receipt. All communications shall be sent to the party to be notified at the address as set forth on the signature pages hereof or
Exhibits A and B hereto or at such other address as such party may designate by ten (10) days advance written notice to the other parties hereto. 
  

	10.9	Aggregation of Stock 

 All Shares held or acquired by affiliated entities or persons shall be aggregated
together for the purpose of determining the availability of any rights under this Agreement. 
  

	10.10	Titles and Subtitles 

 The titles of the sections and subsections of this Agreement are for convenience of
reference only and are not to be considered in construing this Agreement. 
  

	10.11	Additional Investors 

 Notwithstanding anything to the contrary contained herein, if the Company shall issue
additional Series E Shares pursuant to the Purchase Agreement, any purchaser of such Series E Shares may become a party to this Agreement by executing and delivering an additional counterpart signature page to this Agreement and shall be
deemed an “Investor” hereunder. 
  

	10.12	Counterparts 

 This Agreement may be executed in any number of counterparts, each of which shall be an original,
but all of which together shall constitute one instrument. 

  
 -25- 

 IN WITNESS WHEREOF, the parties hereto have executed this AMENDED AND RESTATED INVESTOR
RIGHTS AGREEMENT as of the date set forth in the first paragraph hereof. 
  

													
	XENON PHARMACEUTICALS INC.	 		 	INTERWEST INVESTORS VII, L.P. by
		 		 		 	its general partner INTERWEST
		 		 	MANAGEMENT PARTNERS VII, LLC
					
	By:	 	 /s Simon Pimstone
	 		 	By:	 	 /s Vijay Sondhi

		 	Simon Pimstone	 		 		 	Name:	 	Vijay Sondhi
		 	President and CEO	 		 		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.
			
	INTERWEST INVESTORS VII, L.P. by	 		 	GC & H INVESTMENTS
	its general partner INTERWEST	 		 		 		 	
	MANAGEMENT PARTNERS VII, LLC	 		 		 		 	
					
	By:	 	 /s Vijay Sondhi
	 		 	By:	 	 /s Vijay Sondhi

		 	Name:	 	Vijay Sondhi	 		 		 	Name:	 	Vijay Sondhi
		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.	 		 		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.
			
	WORKING OPPORTUNITY FUND	 		 	ROYAL BANK OF CANADA
	(EVCC) LTD. by its manager GROWTH	 		 		 		 	
	WORKS CAPITAL LTD	 		 		 		 	
					
	By:	 	 /s Vijay Sondhi
	 		 	By:	 	 /s Vijay Sondhi

		 	Authorized Signatory	 		 		 	Name:	 	Vijay Sondhi
		 		 		 		 		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.
					
		 		 		 		 	J.P. MORGAN PARTNERS (BHCA), L.P.
					
		 		 		 		 	By: JPMP MASTER FUND
		 		 		 		 	MANAGER, L.P. its general partner
		 		 		 		 	 By: JPMP CAPITAL CORP., its general

Partners

				
	 /s Vijay Sondhi
	 		 	By:	 	 /s Vijay Sondhi

	GEORGE G. MONTGOMERY	 		 		 	Name:	 	Vijay Sondhi
		 		 		 		 		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.

  
 -26- 

													
	VENTURES WEST 7. U.S. LIMITED	 		 	VENTURES WEST 7 LIMITED
	PARTNERSHIP by its manager,	 		 	PARTNERSHIP by its general partner,
	VENTURES WEST 7 MANAGEMENT	 		 	VENTURES WEST 7 MANAGEMENT
	(INTERNATIONAL) INC.	 		 	LTD.
					
	By:	 	 /s Vijay Sondhi
	 		 	By:	 	 /s Vijay Sondhi

		 	Authorized Signatory	 		 		 	Authorized Signatory
			
	FIDELITY SELECT PORTFOLIOS:	 		 	FIDELITY CANADIAN GROWN
	BIOTECHNOLOGY PORTFOLIO	 		 	COMPANY FUND
					
	By:	 	 /s Vijay Sondhi
	 		 	By:	 	 /s Vijay Sondhi

		 	Name:	 	Vijay Sondhi	 		 		 	Name:	 	Vijay Sondhi
		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.	 		 		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.
			
	FIDELITY CANADIAN ASSET	 		 	FIDELITY CANADIAN
	ALLOCATION FUND	 		 	OPPORTUNITIES FUND (FORMERLY
		 		 	FIDELITY CANADIAN AGGRESSIVE (FUND)
					
	By:	 	 /s Vijay Sondhi
	 		 	By:	 	 /s Vijay Sondhi

		 	Name:	 	Vijay Sondhi	 		 		 	Name:	 	Vijay Sondhi
		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.	 		 		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.
			
	NOVO A/S	 		 	WARNER LAMBERT COMPANY
					
	By:	 	 /s Vijay Sondhi
	 		 	By:	 	 /s Vijay Sondhi

		 	Name:	 	Vijay Sondhi	 		 		 	Name:	 	Vijay Sondhi
		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.	 		 		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.
			
	CHANCELLOR V-A L.P	 		 	CHANCELLOR V, L.P
			
	By: IPC Direct Associates V, L.L.C., its	 		 	By: IPC Direct Associates V, L.L.C., its
	general partner	 		 	general partner
	By: INVESCO Private Capital, Inc. its	 		 	By: INVESCO Private Capital, Inc. its
	managing member	 		 	managing member
					
	By:	 	 /s Vijay Sondhi
	 		 	By:	 	 /s Vijay Sondhi

		 	Name:	 	Vijay Sondhi	 		 		 	Name:	 	Vijay Sondhi
		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.	 		 		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.

  
 -27- 

													
	CITIVENTURE 2000, L.P.	 		 	ALPHA TECHNOLOGIES LIMITED
					
	 By: IPC Direct Associates V, L.L.C., its

general partner
	 		 		 		 	
	 By: INVESCO Private Capital, Inc., its

managing member
	 		 		 		 	
					
	By:	 	 /s Vijay Sondhi
	 		 	By:	 	 /s Vijay Sondhi

		 	Name:	 	Vijay Sondhi	 		 		 	Name:	 	Vijay Sondhi
		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.	 		 		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.
			
	NOVARTIS PHARMA AG	 		 	LIPOTERX LTD
			
		 		 	By: Lipoterx LLP, its general partner
					
	By:	 	 /s Vijay Sondhi
	 		 	By:	 	 /s Vijay Sondhi

		 	Name:	 	Vijay Sondhi	 		 		 	Name:	 	Vijay Sondhi
		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.	 		 		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.
			
	MX ASSOCIATES LLP	 		 	TAKEDA RESEARCH INVESTMENT, INC.
					
	By:	 	 /s Vijay Sondhi
	 		 	By:	 	 /s Vijay Sondhi

		 	Name:	 	Vijay Sondhi	 		 		 	Name:	 	Vijay Sondhi
		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.	 		 		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.
			
	TAKEDA PHARMACEUTICAL	 		 	ROCHE FINANCE LTD
	COMPANY LIMITED	 		 		 		 	
					
	By:	 	 /s Vijay Sondhi
	 		 	By:	 	  

		 	Name:	 	Vijay Sondhi	 		 		 	Name:	 	
		 	Title:	 	Chief Financial Officer of Xenon Pharmaceuticals Inc.	 		 		 	Title:	 	

  
 -28- 

											
	MX Associates LLP	 		 	TAKEDA RESEARCH INVESTMENT, INC.
					
	By:	 	 c/s
	 		 	By:	 	 c/s

		 	 Name:
 Title:
	 		 		 	 Name:
 Title:
	 	
			
	TAKEDA PHARMACEUTICAL COMPANY LIMITED	 		 	ROCHE FINANCE LTD
					
	By:	 	 c/s
	 		 	By:	 	 /s Kniertinger Maier

		 	Name:	 		 		 	Name:	 	Kniertinger Maier
		 	Title:	 		 		 	Title:	 	

  
 -29- 

 Exhibit A 

SCHEDULE OF SERIES B INVESTORS 
  

					
	 	  	 Series B Investors
	  	 Address

			
	1.	  	Interwest Partners VII, L.P.	  	 3000 Sand Hill Road,
 Building 3, Suite 255

Menlo Park, CA 94052

			
	2.	  	Interwest Investors VII, L.P.	  	 3000 Sand Hill Road,
 Building 3, Suite 255

Menlo Park, CA 94052

			
	3.	  	GC&H Investments	  	
			
	4.	  	Working Opportunity Fund (EVCC) Ltd.	  	 Suite 2600, Royal Centre,
 1055 West Georgia
Street,
 Vancouver, British
 Columbia V6E 3R5

			
	5.	  	Royal Bank of Canada	  	 Suite 1340, 1111 West
 Georgia Street,

Vancouver, British
 Columbia V6E 4M3

			
	6.	  	Ventures West 7 Limited Partnership	  	 Suite 280, 1285 West
 Pender Street,

Vancouver, British
 Columbia V6E 4B1

  
 -30- 

 Exhibit B 

SCHEDULE OF SERIES E INVESTORS 
  

					
	 	  	 Series E Investors
	  	 Address

			
	1.	  	Fidelity Select Portfolios: Biotechnology Portfolio	  	82 Devonshire Street E20E Boston, MA 02109
			
	2.	  	Fidelity Canadian Opportunities Fund	  	82 Devonshire Street E20E Boston, MA 02109
			
	3.	  	Fidelity Canadian Growth Company Fund	  	82 Devonshire Street E20E Boston, MA 02109
			
	4.	  	Fidelity Canadian Asset Allocation Fund	  	82 Devonshire Street E20E Boston, MA 02109
			
	5.	  	Chancellor V, L.P.	  	 1166 Avenue of the
 Americas 27th Floor
 New York, NY 10036

			
	6.	  	Chancellor V-A, L.P.	  	 1166 Avenue of the
 Americas 27th Floor
 New York, NY 10036

			
	7.	  	Citiventure 2000, L.P.	  	 1166 Avenue of the
 Americas 27th Floor
 New York, NY 10036

			
	8.	  	Alpha Technologies Limited	  	 c/o UBS International Inc.
 633 W. Fifth Street
64th Floor

			
	9.	  	Working Opportunity Fund (EVCC) Ltd.	  	 Suite 2600, Royal Centre,
 1055 West Georgia
Street, Vancouver, British Columbia
 V6E 3R5

			
	10.	  	InterWest Partners VII, L.P.	  	 3000 Sand Hill Road,
 Building 3, Suite 255

Menlo Park, CA 94052

			
	11.	  	InterWest Investors VII, L.P.	  	 3000 Sand Hill Road,
 Building 3, Suite 255

Menlo Park, CA 94052

			
	12.	  	JP Morgan Partners (BHCA), L.P.	  	 1 Bush St, 12th Floor

San Francisco, California
 94104

			
	13.	  	George G. Montgomery	  	 1 Bush St., 12th Floor

San Francisco, California

94104

  
 -31- 

					
	 	  	 Series E Investors
	  	 Address

			
	14.	  	Novo A/S	  	 Krogshoejvej 41, DK-2880
 Bagsvaerd,
Denmark

			
	15.	  	Warner Lambert Company	  	 Eastern Point Rd.
 Groton, CT,
06340

			
	16.	  	Royal Bank of Canada	  	 Suite 1340, 1111 West
 Georgia Street

Vancouver, British Columbia
 V6E 4M3

			
	17.	  	Ventures West 7 Limited Partnership	  	 Suite 280, 1285 West Pender
 Street, Vancouver,
British
 Columbia V6E 4B1

			
	18.	  	Ventures West 7 U.S. Limited Partnership	  	 Suite 280, 1285 West Pender
 Street, Vancouver,
British
 Columbia V6E 4B1

			
	19.	  	Novartis Pharma AG	  	 Lichtstrasse 35
 CH-4002 Basel

Switzerland

			
	20.	  	MX Associates LLP	  	 4620 Wesley Avenue
 Cincinnati, Ohio
45212

			
	21.	  	Lipoterx Ltd.	  	 Suite 102 3131 Harvey
 Avenue

Cincinnati, Ohio 45229

			
	22.	  	Takeda Research Investment, Inc.	  	 Suite 300 435 Tasso Street
 Palo Alto,
California 94301

			
	23.	  	*(a) Takeda Pharmaceutical Company Limited	  	 1-1, Doshomachi 4-chome,
 Chuo-ku

Osaka 540-8645 Japan

			
		  	*(b) Roche Finance Ltd.	  	 Grenzacherstrasse 124 CH-
 4070 Basel

Switzerland

  
 -32- 

 Exhibit C 

SCHEDULE OF CANADIAN SERIES B INVESTORS 
  

					
	 	  	 Series B Investors
	  	 Address

			
	1.	  	Working Opportunity Fund (EVCC) Ltd	  	Suite 2600, Royal Centre, 1055 West Georgia Street, Vancouver, British Columbia V6E 3R5
			
	2.	  	Royal Bank of Canada	  	Suite 1340, 1111 West Georgia Street Vancouver, British Columbia V6E 4M3
			
	3.	  	Ventures West 7 Limited Partnership	  	Suite 280, 1285 West Fender Street, Vancouver, British Columbia V6E 4B1

  
 -33- 

 XENON PHARMACEUTICALS INC. 

(the “Corporation”) 

CONSENT AND AGREEMENT TO VOTE 

Shareholder Information: 
  

			
	 Name:
	  	 Number and Series of Preferred Share held by Shareholder:

	 Interwest Investors VII, L.P.
	  	 Series A Preferred Shares: 0
 Series B
Preferred Shares: 131,334
 Series E Preferred Shares: 33,358

		
	 Interwest Partners VII, L.P.
	  	 Series A Preferred Shares: 0
 Series B
Preferred Shares: 2,742,485
 Series E Preferred Shares: 696,569

 (collectively and individually know as the “Shareholder”) 

(the above together with any other shares of the Corporation acquired or to be acquired by the Shareholders after the date of this consent and agreement to
vote that are entitled to vote at the Corporation’s shareholders’ meeting(s) called to consider the matters (or part thereof) shall together be referred to below, the “Shareholders’ Shares”) 

For good and valuable consideration (which is acknowledged), the Shareholder irrevocably: 

 

	1.	consents to the amendment of the Amended and Restated Investor Rights Agreement dated December 6th, 2006, as amended from time to time (the “IRA”) by
(the “IRA Amendment”): 

  

	 	a.	section 4.4 (a) of the IRA (under the heading “Termination and Waiver of Rights of First Refusal”) be deleted in its entirety and be replaced with the following: 

“effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering
is on terms as approved by the Board of Directors of the Company and will be completed on or before December 31, 2014 and will result in the common shares of the Company to be listed or quoted upon a recognized senior stock exchange or public
securities quotation system (including without limitation, the Toronto Stock Exchange or the NASDAQ National Market) (the “IPO”)”; 
  

	 	b.	inserting new subsections under section 4.6 of the IRA as follows: 

 “(j) up to a maximum
number of common shares of the Company that is equal to US$5 million divided by the issue price per common share of the Company as approved by the Board of Directors of the Company in the IPO by way of private placement in connection with a
collaboration arrangement; 
 (k) all Equity Securities of the Company issued pursuant to or in connection with the IPO.” 

	 	c.	All references to “Canadian generally accepted accounting principles” be changed to “Canadian generally accepted accounting principles or US generally accepted accounting principles” in the IRA; and

  

	 	d.	With respect to the IPO, all of the Shareholder’s rights under section 2.2 to 2.12 (inclusive) and 2.14 of the IRA are waived and no such right shall apply to or be effective in relation to the IPO.

  

	2.	approves that all options (and related Common Shares) issuable under the 2014 Equity Incentive Plan as approved by the Board of Directors and to be confirmed and adopted by the shareholders meeting to be held on or
before June 30, 2014 (the “New Equity Incentive Plan”) be deemed “Excepted Securities”, “Series E Excepted Securities” and “Current Series E Excepted Securities” as such terms are used in the
share rights set out in the Articles (Schedule A to the Addendum) of the Corporation. 

  

	3.	consents to the amendment of the amended and restated shareholders agreement dated December 6th, 2006, as amended from time to time (the “Shareholders
Agreement”) as follows (the “Shareholders Agreement Amendments”): 

  

	 	a.	the whole of article 2 of the Shareholders Agreement be deleted but if by December 31, 2014, the closing of the IPO has not occurred, such Article 2 shall be deemed to be reinstated into the Shareholders Agreement
without further action from the Corporation or any of the shareholders of the Corporation; 

  

	 	b.	section 4.12 of the Shareholders Agreement be deleted in its entirety and by replaced by the following: 

“4.12 Termination — This Agreement may be terminated upon written agreement or consent of Founders holding not less than 75%
of the aggregate number of Shares held by all Founders and by Investors holding not less than 75% of the aggregate number of Shares held by all Investors as at the time of such agreement or consent. Any such written agreement or consent to terminate
shall be binding on all parties to the Agreement.” 
  

	4.	agrees that the Shareholders Agreement shall be terminated upon closing of the IPO (as defined above) (“Shareholders Agreement Termination”). 

 

	5.	agrees to: 

  

	 	a.	vote any and all of the Shareholder’s Shares in the capital of the Corporation owned or beneficially owned by the Shareholder, and all shares in the capital of the Corporation over which the Shareholder has a proxy
to vote, including but not limited to the Shareholder’s Shares, in favour of any resolution that the Corporation may put before shareholders of the Corporation at one or more shareholders meetings to be held on or before June 30, 2014
(i) for approval, ratification or confirmation of the aforementioned New Equity Incentive Plan, IRA Amendment, Shareholders Agreement Amendment, or Shareholders Agreement Termination, and any other approvals related to the foregoing and
(ii) for approval of the consolidation of the common shares of the Corporation prior to the IPO and the adoption of new articles and bylaws for the Corporation in preparation for or in connection with the closing of the IPO (including if the
directors deem appropriate, the creation of a new class of preferred shares issuable in series), and any other approvals related to the foregoing, each as approved by the Board of Directors of the Corporation and in connection with the IPO or in
preparation for the IPO ((i) and (ii) together “Approved Matters”); 

	 	b.	upon written request or direction of the Corporation, execute and not revoke a form of proxy appointing any director of the Corporation as proxy, with full power of substitution, to attend, vote and otherwise act for
and on behalf of the Shareholder in respect of all of the Shareholder’s Shares in respect of all such matters which may come before a meeting of the shareholders of the Corporation relating to the Approved Matters; and 

 

	 	c.	execute all such documents and take all such other action as may in the opinion of the Corporation be necessary or desirable in connection with the foregoing. 

 

	6.	acknowledges and agrees that this consent and agreement to vote constitutes a legal, valid and binding agreement of the Shareholder for which good and valuable consideration has been received. 

All defined terms used in this consent and agreement to vote shall have the meanings set out above and the definitions in the IRA and Shareholders Agreement
shall only apply to any text in quotes relating to suggested amendments to such agreements. 
  

			
	DATED                 , 2014.
	
	         /s/ Gilbert H. Kliman

		
	Name:	 	 Gilbert H. Kliman

		
	Title:	 	 Managing Director

	
	Authorized Signatory for the Shareholder

 XENON PHARMACEUTICALS INC. 

(the “Corporation”) 

CONSENT AND AGREEMENT TO VOTE 

Shareholder Information: 
 Name: California Emerging
Ventures II, LLC (the “Shareholder”) 
 Number and Series of Preferred Shares held by the Shareholder: 

Series A Preferred Shares: 0 

Series B Preferred Shares: 11,826 

Series E Preferred Shares: 7,121 
 (the
above together with any other shares of the Corporation acquired or to be acquired by the Shareholder after the date of this consent and agreement to vote that are entitled to vote at the Corporation’s shareholders’ meeting(s) called to
consider the matters (or part thereof) shall together be referred to below, the “Shareholder’s Shares”) 
 For good and valuable
consideration (which is acknowledged), the Shareholder irrevocably: 
  

	1.	consents to the amendment of the Amended and Restated Investor Rights Agreement dated December 6th, 2006, as amended from time to time (the “IRA”) by
(the “IRA Amendment”): 

  

	 	(a)	section 4.4 (a) of the IRA (under the heading “Termination and Waiver of Rights of First Refusal”) be deleted in its entirety and be replaced with the following: 

“effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering
is on terms as approved by the Board of Directors of the Company and will be completed on or before December 31, 2014 and will result in the common shares of the Company to be listed or quoted upon a recognized senior stock exchange or public
securities quotation system (including without limitation, the Toronto Stock Exchange or the NASDAQ National Market) (the “IPO”)”; 
  

	 	(b)	inserting new subsections under section 4.6 of the IRA as follows: 

 “(j) up to a maximum
number of common shares of the Company that is equal to US$5 million divided by the issue price per common share of the Company as approved by the Board of Directors of the Company in the IPO by way of private placement in connection with a
collaboration arrangement; 
 (k) all Equity Securities of the Company issued pursuant to or in connection with the IPO.” 

 

	 	(c)	All references to “Canadian generally accepted accounting principles” be changed to “Canadian generally accepted accounting principles or US generally accepted accounting principles” in the IRA; and

	 	(d)	With respect to the IPO, all of the Shareholder’s rights under section 2.2 to 2.12 (inclusive) and 2.14 of the IRA are waived and no such right shall apply to or be effective in relation to the IPO.

  

	2.	approves that all options (and related Common Shares) issuable under the 2014 Equity Incentive Plan as approved by the Board of Directors and to be confirmed and adopted by the shareholders meeting to be held on or
before June 30, 2014 (the “New Equity Incentive Plan”) be deemed “Excepted Securities”, “Series E Excepted Securities” and “Current Series E Excepted Securities” as such terms are used in the
share rights set out in the Articles (Schedule A to the Addendum) of the Corporation. 

  

	3.	consents to the amendment of the amended and restated shareholders agreement dated December 6th, 2006, as amended from time to time (the “Shareholders
Agreement”) as follows (the “Shareholders Agreement Amendments”): 

  

	 	(a)	the whole of article 2 of the Shareholders Agreement be deleted but if by December 31, 2014, the closing of the IPO has not occurred, such Article 2 shall be deemed to be reinstated into the Shareholders Agreement
without further action from the Corporation or any of the shareholders of the Corporation; 

  

	 	(b)	section 4.12 of the Shareholders Agreement be deleted in its entirety and by replaced by the following: 

“4.12 Termination — This Agreement may be terminated upon written agreement or consent of Founders holding not less than 75%
of the aggregate number of Shares held by all Founders and by Investors holding not less than 75% of the aggregate number of Shares held by all Investors as at the time of such agreement or consent. Any such written agreement or consent to terminate
shall be binding on all parties to the Agreement.” 
  

	4.	agrees that the Shareholders Agreement shall be terminated upon closing of the IPO (as defined above) (“Shareholders Agreement Termination”). 

 

	5.	agrees to: 

  

	 	(a)	vote any and all of the Shareholder’s Shares in the capital of the Corporation owned or beneficially owned by the Shareholder, and all shares in the capital of the Corporation over which the Shareholder has a proxy
to vote, including but not limited to the Shareholder’s Shares, in favour of any resolution that the Corporation may put before shareholders of the Corporation at one or more shareholders meetings to be held on or before June 30, 2014
(i) for approval, ratification or confirmation of the aforementioned New Equity Incentive Plan, IRA Amendment, Shareholders Agreement Amendment, or Shareholders Agreement Termination, and any other approvals related to the foregoing and
(ii) for approval of the consolidation of the common shares of the Corporation prior to the IPO and the adoption of new articles and bylaws for the Corporation in preparation for or in connection with the closing of the [PO (including if the
directors deem appropriate, the creation of a new class of preferred shares issuable in series), and any other approvals related to the foregoing, each as approved by the Board of Directors of the Corporation and in connection with the IPO or in
preparation for the IPO ((i) and (ii) together “Approved Matters”); 

  

	 	(b)	upon written request or direction of the Corporation, execute and not revoke a form of proxy appointing any director of the Corporation as proxy, with full power of substitution, to attend, vote and otherwise act for
and on behalf of the Shareholder in respect of all of the Shareholder’s Shares in respect of all such matters which may come before a meeting of the shareholders of the Corporation relating to the Approved Matters; and 

	 	(c)	execute all such documents and take all such other action as may in the opinion of the Corporation be necessary or desirable in connection with the foregoing. 

 

	6.	acknowledges and agrees that this consent and agreement to vote constitutes a legal, valid and binding agreement of the Shareholder for which good and valuable consideration has been received. 

All defined terms used in this consent and agreement to vote shall have the meanings set out above and the definitions in the IRA and Shareholders Agreement
shall only apply to any text in quotes relating to suggested amendments to such agreements. 
 DATED     April 9th    , 2014. 
  

			
	California Emerging Ventures II, LLC
	
	By: GSA Partners II, LLC, its Manager
	By: Grove Street Advisors, LLC, its Manager
		
	Name:	 	 Ann St. Germain

	
	 /s/ Ann St. Germain

		
	Title:	 	 Member & CEO

	
	Authorized Signatory for the Shareholder

 XENON PHARMACEUTICALS INC. 

(the “Corporation”) 

CONSENT AND AGREEMENT TO VOTE 

Shareholder Information: 
 Name: Cape 1998 Trust (the
“Shareholder”) 
 Number and Series of Preferred Shares held by the Shareholder: 

Series A Preferred Shares: 0 

Series B Preferred Shares: 237 

Series E Preferred Shares: 142 
 (the above
together with any other shares of the Corporation acquired or to be acquired by the Shareholder after the date of this consent and agreement to vote that are entitled to vote at the Corporation’s shareholders’ meeting(s) called to consider
the matters (or part thereof) shall together be referred to below, the “Shareholders’ Shares”) 
 For good and valuable consideration (which
is acknowledged), the Shareholder irrevocably: 
  

	1.	consents to the amendment of the Amended and Restated Investor Rights Agreement dated December 6th, 2006, as amended from time to time (the “IRA”) by
(the “IRA Amendment”): 

  

	 	(a)	section 4.4 (a) of the IRA (under the heading “Termination and Waiver of Rights of First Refusal”) be deleted in its entirety and be replaced with the following: 

“effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering
is on terms as approved by the Board of Directors of the Company and will be completed on or before December 31, 2014 and will result in the common shares of the Company to be listed or quoted upon a recognized senior stock exchange or public
securities quotation system (including without limitation, the Toronto Stock Exchange or the NASDAQ National Market) (the “IPO”)”; 
  

	 	(b)	inserting new subsections under section 4.6 of the IRA as follows: 

 “(j) up to a maximum
number of common shares of the Company that is equal to US$5 million divided by the issue price per common share of the Company as approved by the Board of Directors of the Company in the IPO by way of private placement in connection with a
collaboration arrangement; 
 (k) all Equity Securities of the Company issued pursuant to or in connection with the IPO.” 

 

	 	(c)	All references to “Canadian generally accepted accounting principles” be changed to “Canadian generally accepted accounting principles or US generally accepted accounting principles” in the IRA; and

	 	(d)	With respect to the IPO, all of the Shareholder’s rights under section 2.2 to 2.12 (inclusive) and 2.14 of the IRA are waived and no such right shall apply to or be effective in relation to the IPO.

  

	2.	approves that all options (and related Common Shares) issuable under the 2014 Equity Incentive Plan as approved by the Board of Directors and to be confirmed and adopted by the shareholders meeting to be held on or
before June 30, 2014 (the “New Equity Incentive Plan”) be deemed “Excepted Securities”, “Series E Excepted Securities” and “Current Series E Excepted Securities” as such terms are used in the
share rights set out in the Articles (Schedule A to the Addendum) of the Corporation. 

  

	3.	consents to the amendment of the amended and restated shareholders agreement dated December 6th, 2006, as amended from time to time (the “Shareholders
Agreement”) as follows (the “Shareholders Agreement Amendments”): 

  

	 	(a)	the whole of article 2 of the Shareholders Agreement be deleted but if by December 31, 2014, the closing of the IPO has not occurred, such Article 2 shall be deemed to be reinstated into the Shareholders Agreement
without further action from the Corporation or any of the shareholders of the Corporation; 

  

	 	(b)	section 4.12 of the Shareholders Agreement be deleted in its entirety and by replaced by the following: 

“4.12 Termination — This Agreement may be terminated upon written agreement or consent of Founders holding not less than 75%
of the aggregate number of Shares held by all Founders and by Investors holding not less than 75% of the aggregate number of Shares held by all Investors as at the time of such agreement or consent. Any such written agreement or consent to terminate
shall be binding on all parties to the Agreement.” 
  

	4.	agrees that the Shareholders Agreement shall be terminated upon closing of the IPO (as defined above) (“Shareholders Agreement Termination”). 

 

	5.	agrees to: 

  

	 	(a)	vote any and all of the Shareholder’s Shares in the capital of the Corporation owned or beneficially owned by the Shareholder, and all shares in the capital of the Corporation over which the Shareholder has a proxy
to vote, including but not limited to the Shareholder’s Shares, in favour of any resolution that the Corporation may put before shareholders of the Corporation at one or more shareholders meetings to be held on or before June 30, 2014
(i) for approval, ratification or confirmation of the aforementioned New Equity Incentive Plan, IRA Amendment, Shareholders Agreement Amendment, or Shareholders Agreement Termination, and any other approvals related to the foregoing and
(ii) for approval of the consolidation of the common shares of the Corporation prior to the IPO and the adoption of new articles and bylaws for the Corporation in preparation for or in connection with the closing of the IPO (including if the
directors deem appropriate, the creation of a new class of preferred shares issuable in series), and any other approvals related to the foregoing, each as approved by the Board of Directors of the Corporation and in connection with the IPO or in
preparation for the IPO ((i) and (ii) together “Approved Matters”); 

  

	 	(b)	upon written request or direction of the Corporation, execute and not revoke a form of proxy appointing any director of the Corporation as proxy, with full power of substitution, to attend, vote and otherwise act for
and on behalf of the Shareholder in respect of all of the Shareholder’s Shares in respect of all such matters which may come before a meeting of the shareholders of the Corporation relating to the Approved Matters; and 

	 	(c)	execute all such documents and take all such other action as may in the opinion of the Corporation be necessary or desirable in connection with the foregoing. 

 

	6.	acknowledges and agrees that this consent and agreement to vote constitutes a legal, valid and binding agreement of the Shareholder for which good and valuable consideration has been received. 

All defined terms used in this consent and agreement to vote shall have the meanings set out above and the definitions in the IRA and Shareholders Agreement
shall only apply to any text in quotes relating to suggested amendments to such agreements. 
 DATED
        April 14        , 2014. 
  

			
	         /s/ Lillian Cape

		
	Name:	 	 Lillian Cape

		
	Title:	 	 Trustee Cape 1998 Trust

	
	Authorized Signatory for the Shareholder

 XENON PHARMACEUTICALS INC. 

(the “Corporation”) 

CONSENT AND AGREEMENT TO VOTE 

Shareholder Information: 
  

			
	 Name:
	  	 Number and Series of Preferred Shares held by the Shareholder:

	Mac & Co. FBO Fidelity Canadian Asset Allocation Fund	  	 Series A Preferred Shares: 0
 Series B
Preferred Shares: 0
 Series E Preferred Shares: 583,942

		
	Mac & Co. FBO Fidelity Canadian Growth Company Fund	  	 Series A Preferred Shares: 0
 Series B
Preferred Shares: 0
 Series E Preferred Shares: 594,202

		
	Mac & Co. FBO Fidelity Canadian Opportunities Fund	  	 Series A Preferred Shares: 0
 Series B
Preferred Shares: 0
 Series E Preferred Shares: 7,424

		
	 MAG & CO. FBO Fidelity Select Portfolios:

Biotechnology Portfolio
	  	 Series A Preferred Shares: 0
 Series B
Preferred Shares: 0
 Series E Preferred Shares: 981,626

 (collectively and individually the “Shareholder”) 

(the above together with any other shares of the Corporation acquired or to be acquired by the Shareholder after the date of this consent and agreement to
vote that are entitled to vote at the Corporation’s shareholders’ meeting(s) called to consider the matters (or part thereof) shall together be referred to below, the “Shareholders’ Shares”) 

For good and valuable consideration (which is acknowledged), the Shareholder irrevocably: 

 

	1.	consents to the amendment of the Amended and Restated Investor Rights Agreement dated December 6th, 2006, as amended from time to time (the “IRA”) by
(the “IRA Amendment”): 

  

	 	(a)	section 4.4 (a) of the IRA (under the heading “Termination and Waiver of Rights of First Refusal”) be deleted in its entirety and be replaced with the following: 

“effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering
is on terms as approved by the Board of Directors of the Company and will be completed on or before December 31, 2014 and will result in the common shares of the Company to be listed or quoted upon a recognized senior stock exchange or public
securities quotation system (including without limitation, the Toronto Stock Exchange or the NASDAQ National Market) (the “IPO”)”; 
  

	 	(b)	inserting new subsections under section 4.6 of the IRA as follows: 

 “(j) up to a maximum number of common shares of the Company that is equal to US$5 million
divided by the issue price per common share of the Company as approved by the Board of Directors of the Company in the IPO by way of private placement in connection with a collaboration arrangement; 

(k) all Equity Securities of the Company issued pursuant to or in connection with the IPO.” 

 

	 	(c)	All references to “Canadian generally accepted accounting principles” be changed to “Canadian generally accepted accounting principles or US generally accepted accounting principles” in the IRA; and

  

	 	(d)	With respect to the IPO, all of the Shareholder’s rights under section 2.2 to 2.12 (inclusive) and 2.14 of the IRA are waived and no such right shall apply to or be effective in relation to the IPO.

  

	2.	approves that all options (and related Common Shares) issuable under the 2014 Equity Incentive Plan as approved by the Board of Directors and to be confirmed and adopted by the shareholders meeting to be held on or
before June 30, 2014 (the “New Equity Incentive Plan”) be deemed “Excepted Securities”, “Series E Excepted Securities” and “Current Series E Excepted Securities” as such terms are used in the
share rights set out in the Articles (Schedule A to the Addendum) of the Corporation. 

  

	3.	consents to the amendment of the amended and restated shareholders agreement dated December 6th, 2006, as amended from time to time (the “Shareholders
Agreement”) as follows (the “Shareholders Agreement Amendments”): 

  

	 	(a)	the whole of article 2 of the Shareholders Agreement be deleted but if by December 31, 2014, the closing of the IPO has not occurred, such Article 2 shall be deemed to be reinstated into the Shareholders Agreement
without further action from the Corporation or any of the shareholders of the Corporation; 

  

	 	(b)	section 4.12 of the Shareholders Agreement be deleted in its entirety and by replaced by the following: 

“4.12 Termination — This Agreement may be terminated upon written agreement or consent of Founders holding not less than 75%
of the aggregate number of Shares held by all Founders and by Investors holding not less than 75% of the aggregate number of Shares held by all Investors as at the time of such agreement or consent. Any such written agreement or consent to terminate
shall be binding on all parties to the Agreement.” 
  

	4.	agrees that the Shareholders Agreement shall be terminated upon closing of the IPO (as defined above) (“Shareholders Agreement Termination”). 

 

	5.	agrees to: 

  

	 	(a)	 vote any and all of the Shareholder’s Shares in the capital of the Corporation owned or beneficially owned by the Shareholder, and all shares in
the capital of the Corporation over which the Shareholder has a proxy to vote, including but not limited to the Shareholder’s Shares, in favour of any resolution that the Corporation may put before shareholders of the Corporation at one or more
shareholders meetings to be held on or before June 30, 2014 (i) for approval, ratification or confirmation of the aforementioned New Equity Incentive Plan, IRA Amendment, Shareholders Agreement Amendment, or Shareholders Agreement
Termination, and any other 

	 	
approvals related to the foregoing and (ii) for approval of the consolidation of the common shares of the Corporation prior to the IPO and the adoption of new articles and bylaws for the
Corporation in preparation for or in connection with the closing of the IPO (including if the directors deem appropriate, the creation of a new class of preferred shares issuable in series), and any other approvals related to the foregoing, each as
approved by the Board of Directors of the Corporation and in connection with the IPO or in preparation for the IPO ((i) and (ii) together “Approved Matters”); 

 

	 	(b)	upon written request or direction of the Corporation, execute and not revoke a form of proxy appointing any director of the Corporation as proxy, with full power of substitution, to attend, vote and otherwise act for
and on behalf of the Shareholder in respect of all of the Shareholder’s Shares in respect of all such matters which may come before a meeting of the shareholders of the Corporation relating to the Approved Matters; and 

 

	 	(c)	execute all such documents and take all such other action as may in the opinion of the Corporation be necessary or desirable in connection with the foregoing. 

 

	6.	acknowledges and agrees that this consent and agreement to vote constitutes a legal, valid and binding agreement of the Shareholder for which good and valuable consideration has been received. 

All defined terms used in this consent and agreement to vote shall have the meanings set out above and the definitions in the IRA and Shareholders Agreement
shall only apply to any text in quotes relating to suggested amendments to such agreements. 
  

			
	DATED         April 15        , 2014.
	
	Fidelity Investments Canada ULC as trustee of Fidelity Canadian Asset Allocation Fund
	
	         /s/ Stacie M. Smith

		
	Name:	 	 Stacie Smith

		
	Title:	 	 Authorized Signatory

	
	Authorized Signatory for the Shareholder

  

			
	DATED         April 15        , 2014.
	
	Fidelity Investments Canada ULC as trustee of Fidelity Canadian Growth Company Fund
	
	         /s/ Stacie M. Smith

		
	Name:	 	 Stacie Smith

		
	Title:	 	 Authorized Signatory

	
	Authorized Signatory for the Shareholder

			
	DATED         April 15        , 2014.
	
	Fidelity Investments Canada ULC as trustee of Fidelity Canadian Opportunities Fund
	
	         /s/ Stacie M. Smith

		
	Name:	 	 Stacie Smith

		
	Title:	 	 Authorized Signatory

	
	Authorized Signatory for the Shareholder

  

			
	DATED         April 15        , 2014.
	
	Fidelity Select Portfolios: Biotechnology Portfolio
	
	         /s/ Stacie M. Smith

		
	Name:	 	 Stacie Smith

		
	Title:	 	 Deputy Treasurer

	
	Authorized Signatory for the Shareholder

 XENON PHARMACEUTICALS INC. 

(the “Corporation”) 

CONSENT AND AGREEMENT TO VOTE 

Shareholder Information: 
  

			
	 Name:
	  	 Number and Series of Preferred Share held by Shareholder:

	Chancellor V, L.P.	  	 Series A Preferred Shares: 0
 Series B
Preferred Shares: 0
 Series E Preferred Shares: 939,645

	Chancellor V-A, L.P.	  	 Series A Preferred Shares: 0
 Series B
Preferred Shares: 0
 Series E Preferred Shares: 492,821

	Citiventure 2000, L.P.	  	 Series A Preferred Shares: 0
 Series B
Preferred Shares: 0
 Series E Preferred Shares: 148,098

 (collectively and individually know as the “Shareholder”) 

(the above together with any other shares of the Corporation acquired or to be acquired by the Shareholders after the date of this consent and agreement to
vote that are entitled to vote at the Corporation’s shareholders’ meeting(s) called to consider the matters (or part thereof) shall together be referred to below, the “Shareholders’ Shares”) 

For good and valuable consideration (which is acknowledged), the Shareholder irrevocably: 

 

	1.	consents to the amendment of the Amended and Restated Investor Rights Agreement dated December 6th, 2006, as amended from time to time (the “IRA”) by
(the “IRA Amendment”): 

  

	 	(a)	section 4.4 (a) of the IRA (under the heading “Termination and Waiver of Rights of First Refusal”) be deleted in its entirety and be replaced with the following: 

“effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering
is on terms as approved by the Board of Directors of the Company and will be completed on or before December 31, 2014 and will result in the common shares of the Company to be listed or quoted upon a recognized senior stock exchange or public
securities quotation system (including without limitation, the Toronto Stock Exchange or the NASDAQ National Market) (the “IPO”)”; 
  

	 	(b)	inserting new subsections under section 4.6 of the IRA as follows: 

 “(j) up to a maximum
number of common shares of the Company that is equal to US$5 million divided by the issue price per common share of the Company as approved by the Board of Directors of the Company in the IPO by way of private placement in connection with a
collaboration arrangement; 

 (k) all Equity Securities of the Company issued pursuant to or in connection with the IPO.”

  

	 	(c)	All references to “Canadian generally accepted accounting principles” be changed to “Canadian generally accepted accounting principles or US generally accepted accounting principles” in the IRA; and

  

	 	(d)	With respect to the IPO, all of the Shareholder’s rights under section 2.2 to 2.12 (inclusive) and 2.14 of the IRA are waived and no such right shall apply to or be effective in relation to the IPO.

  

	2.	approves that all options (and related Common Shares) issuable under the 2014 Equity Incentive Plan as approved by the Board of Directors and to be confirmed and adopted by the shareholders meeting to be held on or
before June 30, 2014 (the “New Equity Incentive Plan”) be deemed “Excepted Securities”, “Series E Excepted Securities” and “Current Series E Excepted Securities” as such terms are used in the
share rights set out in the Articles (Schedule A to the Addendum) of the Corporation. 

  

	3.	consents to the amendment of the amended and restated shareholders agreement dated December 6th, 2006, as amended from time to time (the “Shareholders
Agreement”) as follows (the “Shareholders Agreement Amendments”): 

  

	 	(a)	the whole of article 2 of the Shareholders Agreement be deleted but if by December 31, 2014, the closing of the IPO has not occurred, such Article 2 shall be deemed to be reinstated into the Shareholders Agreement
without further action from the Corporation or any of the shareholders of the Corporation; 

  

	 	(b)	section 4.12 of the Shareholders Agreement be deleted in its entirety and by replaced by the following: 

“4.12 Termination — This Agreement may be terminated upon written agreement or consent of Founders holding not less than 75%
of the aggregate number of Shares held by all Founders and by Investors holding not less than 75% of the aggregate number of Shares held by all Investors as at the time of such agreement or consent. Any such written agreement or consent to terminate
shall be binding on all parties to the Agreement.” 
  

	4.	agrees that the Shareholders Agreement shall be terminated upon closing of the IPO (as defined above) (“Shareholders Agreement Termination”). 

 

	5.	agrees to: 

  

	 	(a)	vote any and all of the Shareholder’s Shares in the capital of the Corporation owned or beneficially owned by the Shareholder, and all shares in the capital of the Corporation over which the Shareholder has a proxy
to vote, including but not limited to the Shareholder’s Shares, in favour of any resolution that the Corporation may put before shareholders of the Corporation at one or more shareholders meetings to be held on or before June 30, 2014
(i) for approval, ratification or confirmation of the aforementioned New Equity Incentive Plan, IRA Amendment, Shareholders Agreement Amendment, or Shareholders Agreement Termination, and any other approvals related to the foregoing and
(ii) for approval of the consolidation of the common shares of the Corporation prior to the IPO and the adoption of new articles and bylaws for the Corporation in preparation for or in connection with the closing of the IPO (including if the
directors deem appropriate, the creation of a new class of preferred shares issuable in series), and any other approvals related to the foregoing, each as approved by the Board of Directors of the Corporation and in connection with the IPO or in
preparation for the IPO ((i) and (ii) together “Approved Matters”); 

	 	(b)	upon written request or direction of the Corporation, execute and not revoke a form of proxy appointing any director of the Corporation as proxy, with full power of substitution, to attend, vote and otherwise act for
and on behalf of the Shareholder in respect of all of the Shareholder’s Shares in respect of all such matters which may come before a meeting of the shareholders of the Corporation relating to the Approved Matters; and 

 

	 	(c)	execute all such documents and take all such other action as may in the opinion of the Corporation be necessary or desirable in connection with the foregoing. 

 

	6.	acknowledges and agrees that this consent and agreement to vote constitutes a legal, valid and binding agreement of the Shareholder for which good and valuable consideration has been received. 

All defined terms used in this consent and agreement to vote shall have the meanings set out above and the definitions in the IRA and Shareholders Agreement
shall only apply to any text in quotes relating to suggested amendments to such agreements. 
 DATED
        April 25        , 2014. 
  

			
	         /s/ Johnston L. Evans

		
	Name:	 	 Johnston L. Evans

		
	Title:	 	  

	
	Authorized Signatory for the Shareholder

 XENON PHARMACEUTICALS INC. 

(the “Corporation”) 

CONSENT AND AGREEMENT TO VOTE 

Shareholder Information: 
 Name: Lipoterx Ltd. (the
“Shareholder”) 
 Number and Series of Preferred Shares held by the Shareholder: 

Series A Preferred Shares: 0 

Series B Preferred Shares: 0 

Series E Preferred Shares: 4,207,811 
 (the above
together with any other shares of the Corporation acquired or to be acquired by the Shareholder after the date of this consent and agreement to vote that are entitled to vote at the Corporation’s shareholders’ meeting(s) called to consider
the matters (or part thereof) shall together be referred to below, the “Shareholders’ Shares”) 
 For good and valuable consideration (which
is acknowledged), the Shareholder irrevocably: 
  

	1.	consents to the amendment of the Amended and Restated Investor Rights Agreement dated December 6th, 2006, as amended from time to time (the “IRA”) by
(the “IRA Amendment”): 

  

	 	(a)	section 4.4 (a) of the IRA (under the heading “Termination and Waiver of Rights of First Refusal”) be deleted in its entirety and be replaced with the following: 

“effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering
is on terms as approved by the Board of Directors of the Company and will be completed on or before December 31, 2014 and will result in the common shares of the Company to be listed or quoted upon a recognized senior stock exchange or public
securities quotation system (including without limitation, the Toronto Stock Exchange or the NASDAQ National Market) (the “IPO”)”; 
  

	 	(b)	inserting new subsections under section 4.6 of the IRA as follows: 

 “(j) up to a maximum
number of common shares of the Company that is equal to US$5 million divided by the issue price per common share of the Company as approved by the Board of Directors of the Company in the IPO by way of private placement in connection with a
collaboration arrangement; 
 (k) all Equity Securities of the Company issued pursuant to or in connection with the IPO.” 

 

	 	(c)	All references to “Canadian generally accepted accounting principles” be changed to “Canadian generally accepted accounting principles or US generally accepted accounting principles” in the IRA; and

	 	(d)	With respect to the IPO, all of the Shareholder’s rights under section 2.2 to 2.12 (inclusive) and 2.14 of the IRA are waived and no such right shall apply to or be effective in relation to the IPO.

  

	2.	approves that all options (and related Common Shares) issuable under the 2014 Equity Incentive Plan as approved by the Board of Directors and to be confirmed and adopted by the shareholders meeting to be held on or
before June 30, 2014 (the “New Equity Incentive Plan”) be deemed “Excepted Securities”, “Series E Excepted Securities” and “Current Series E Excepted Securities” as such terms are used in the
share rights set out in the Articles (Schedule A to the Addendum) of the Corporation. 

  

	3.	consents to the amendment of the amended and restated shareholders agreement dated December 6th, 2006, as amended from time to time (the “Shareholders
Agreement”) as follows (the “Shareholders Agreement Amendments”): 

  

	 	(a)	the whole of article 2 of the Shareholders Agreement be deleted but if by December 31, 2014, the closing of the IPO has not occurred, such Article 2 shall be deemed to be reinstated into the Shareholders Agreement
without further action from the Corporation or any of the shareholders of the Corporation; 

  

	 	(b)	section 4.12 of the Shareholders Agreement be deleted in its entirety and by replaced by the following: 

“4.12 Termination — This Agreement may be terminated upon written agreement or consent of Founders holding not less than 75%
of the aggregate number of Shares held by all Founders and by Investors holding not less than 75% of the aggregate number of Shares held by all Investors as at the time of such agreement or consent. Any such written agreement or consent to terminate
shall be binding on all parties to the Agreement.” 
  

	4.	agrees that the Shareholders Agreement shall be terminated upon closing of the IPO (as defined above) (“Shareholders Agreement Termination”). 

 

	5.	agrees to: 

  

	 	(a)	vote any and all of the Shareholder’s Shares in the capital of the Corporation owned or beneficially owned by the Shareholder, and all shares in the capital of the Corporation over which the Shareholder has a proxy
to vote, including but not limited to the Shareholder’s Shares, in favour of any resolution that the Corporation may put before shareholders of the Corporation at one or more shareholders meetings to be held on or before June 30, 2014
(i) for approval, ratification or confirmation of the aforementioned New Equity Incentive Plan, IRA Amendment, Shareholders Agreement Amendment, or Shareholders Agreement Termination, and any other approvals related to the foregoing and
(ii) for approval of the consolidation of the common shares of the Corporation prior to the IPO and the adoption of new articles and bylaws for the Corporation in preparation for or in connection with the closing of the IPO (including if the
directors deem appropriate, the creation of a new class of preferred shares issuable in series), and any other approvals related to the foregoing, each as approved by the Board of Directors of the Corporation and in connection with the IPO or in
preparation for the IPO ((i) and (ii) together “Approved Matters”); 

  

	 	(b)	upon written request or direction of the Corporation, execute and not revoke a form of proxy appointing any director of the Corporation as proxy, with full power of substitution, to attend, vote and otherwise act for
and on behalf of the Shareholder in respect of all of the Shareholder’s Shares in respect of all such matters which may come before a meeting of the shareholders of the Corporation relating to the Approved Matters; and 

	 	(c)	execute all such documents and take all such other action as may in the opinion of the Corporation be necessary or desirable in connection with the foregoing. 

 

	6.	acknowledges and agrees that this consent and agreement to vote constitutes a legal, valid and binding agreement of the Shareholder for which good and valuable consideration has been received. 

All defined terms used in this consent and agreement to vote shall have the meanings set out above and the definitions in the IRA and Shareholders Agreement
shall only apply to any text in quotes relating to suggested amendments to such agreements. 
 DATED
        April 30        , 2014. 
  

			
	         /s/ Evan A. Stein

		
	Name:	 	 Evan A. Stein MD

		
	Title:	 	 Managing Partner

	
	Authorized Signatory for the Shareholder

 XENON PHARMACEUTICALS INC. 

(the “Corporation”) 

CONSENT AND AGREEMENT TO VOTE 

Shareholder Information: 
 Name: MX Associates, LLP (the
“Shareholder”) 
 Number and Series of Preferred Shares held by the Shareholder: 

Series A Preferred Shares: 0 

Series B Preferred Shares: 0 

Series E Preferred Shares: 5,972,378 
 (the above
together with any other shares of the Corporation acquired or to be acquired by the Shareholder after the date of this consent and agreement to vote that are entitled to vote at the Corporation’s shareholders’ meeting(s) called to consider
the matters (or part thereof) shall together be referred to below, the “Shareholder’s Shares”) 
 For good and valuable consideration (which
is acknowledged), the Shareholder irrevocably: 
  

	1.	consents to the amendment of the Amended and Restated Investor Rights Agreement dated December 6th, 2006, as amended from time to time (the “IRA”) by
(the “IRA Amendment”): 

  

	 	(a)	section 4.4 (a) of the IRA (under the heading “Termination and Waiver of Rights of First Refusal”) be deleted in its entirety and be replaced with the following: 

“effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering
is on terms as approved by the Board of Directors of the Company and will be completed on or before December 31, 2014 and will result in the common shares of the Company to be listed or quoted upon a recognized senior stock exchange or public
securities quotation system (including without limitation, the Toronto Stock Exchange or the NASDAQ National Market) (the “IPO”)”; 
  

	 	(b)	inserting new subsections under section 4.6 of the IRA as follows: 

 “(j) up to a maximum
number of common shares of the Company that is equal to US$5 million divided by the issue price per common share of the Company as approved by the Board of Directors of the Company in the IPO by way of private placement in connection with a
collaboration arrangement; 
 (k) all Equity Securities of the Company issued pursuant to or in connection with the IPO.” 

 

	 	(c)	All references to “Canadian generally accepted accounting principles” be changed to “Canadian generally accepted accounting principles or US generally accepted accounting principles” in the IRA; and

	 	(d)	With respect to the IPO, all of the Shareholder’s rights under section 2.2 to 2.12 (inclusive) and 2.14 of the IRA are waived and no such right shall apply to or be effective in relation to the IPO.

  

	2.	approves that all options (and related Common Shares) issuable under the 2014 Equity Incentive Plan as approved by the Board of Directors and to be confirmed and adopted by the shareholders meeting to be held on or
before June 30, 2014 (the “New Equity Incentive Plan”) be deemed “Excepted Securities”, “Series E Excepted Securities” and “Current Series E Excepted Securities” as such terms are used in the
share rights set out in the Articles (Schedule A to the Addendum) of the Corporation. 

  

	3.	consents to the amendment of the amended and restated shareholders agreement dated December 6th, 2006, as amended from time to time (the “Shareholders
Agreement”) as follows (the “Shareholders Agreement Amendments”): 

  

	 	(a)	the whole of article 2 of the Shareholders Agreement be deleted but if by December 31, 2014, the closing of the IPO has not occurred, such Article 2 shall be deemed to be reinstated into the Shareholders Agreement
without further action from the Corporation or any of the shareholders of the Corporation; 

  

	 	(b)	section 4.12 of the Shareholders Agreement be deleted in its entirety and by replaced by the following: 

“4.12 Termination — This Agreement may be terminated upon written agreement or consent of Founders holding not less than 75%
of the aggregate number of Shares held by all Founders and by Investors holding not less than 75% of the aggregate number of Shares held by all Investors as at the time of such agreement or consent. Any such written agreement or consent to terminate
shall be binding on all parties to the Agreement.” 
  

	4.	agrees that the Shareholders Agreement shall be terminated upon closing of the IPO (as defined above) (“Shareholders Agreement Termination”). 

 

	5.	agrees to: 

  

	 	(a)	vote any and all of the Shareholder’s Shares in the capital of the Corporation owned or beneficially owned by the Shareholder, and all shares in the capital of the Corporation over which the Shareholder has a proxy
to vote, including but not limited to the Shareholder’s Shares, in favour of any resolution that the Corporation may put before shareholders of the Corporation at one or more shareholders meetings to be held on or before June 30, 2014
(i) for approval, ratification or confirmation of the aforementioned New Equity Incentive Plan, IRA Amendment, Shareholders Agreement Amendment, or Shareholders Agreement Termination, and any other approvals related to the foregoing and
(ii) for approval of the consolidation of the common shares of the Corporation prior to the IPO and the adoption of new articles and bylaws for the Corporation in preparation for or in connection with the closing of the IPO (including if the
directors deem appropriate, the creation of a new class of preferred shares issuable in series), and any other approvals related to the foregoing, each as approved by the Board of Directors of the Corporation and in connection with the IPO or in
preparation for the IPO ((i) and (ii) together “Approved Matters”); 

  

	 	(b)	upon written request or direction of the Corporation, execute and not revoke a form of proxy appointing any director of the Corporation as proxy, with full power of substitution, to attend, vote and otherwise act for
and on behalf of the Shareholder in respect of all of the Shareholder’s Shares in respect of all such matters which may come before a meeting of the shareholders of the Corporation relating to the Approved Matters; and 

	 	(c)	execute all such documents and take all such other action as may in the opinion of the Corporation be necessary or desirable in connection with the foregoing. 

 

	6.	acknowledges and agrees that this consent and agreement to vote constitutes a legal, valid and binding agreement of the Shareholder for which good and valuable consideration has been received. 

All defined terms used in this consent and agreement to vote shall have the meanings set out above and the definitions in the IRA and Shareholders Agreement
shall only apply to any text in quotes relating to suggested amendments to such agreements. 
  

			
	DATED          May 2        , 2014.
	
	         /s/ August Truendle

		
	Name:	 	 August Truendle

		
	Title:	 	 Manager

	
	Authorized Signatory for the Shareholder

 XENON PHARMACEUTICALS INC. 

(the “Corporation”) 

CONSENT AND AGREEMENT TO VOTE 

Shareholder Information: 
 Name: Novartis Pharma AG (the
“Shareholder”) 
 Number and Series of Preferred Shares held by the Shareholder: 

Series A Preferred Shares: 0 

Series B Preferred Shares: 0 

Series E Preferred Shares: 1,333,333 
 (the above
together with any other shares of the Corporation acquired or to be acquired by the Shareholder after the date of this consent and agreement to vote that are entitled to vote at the Corporation’s shareholders’ meeting(s) called to consider
the matters (or part thereof) shall together be referred to below, the “Shareholder’s Shares”) 
 For good and valuable consideration (which
is acknowledged), the Shareholder irrevocably: 
  

	1.	consents to the amendment of the Amended and Restated Investor Rights Agreement dated December 6th, 2006, as amended from time to time (the “IRA”) by
(the “IRA Amendment”): 

  

	 	(a)	section 4.4 (a) of the IRA (under the heading “Termination and Waiver of Rights of First Refusal”) be deleted in its entirety and be replaced with the following: 

“effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering
is on terms as approved by the Board of Directors of the Company and will be completed on or before December 31, 2014 and will result in the common shares of the Company to be listed or quoted upon a recognized senior stock exchange or public
securities quotation system (including without limitation, the Toronto Stock Exchange or the NASDAQ National Market) (the “IPO”)”; 
  

	 	(b)	inserting new subsections under section 4.6 of the IRA as follows: 

 “(j) up to a maximum
number of common shares of the Company that is equal to US$5 million divided by the issue price per common share of the Company as approved by the Board of Directors of the Company in the IPO by way of private placement in connection with a
collaboration arrangement; 
 (k) all Equity Securities of the Company issued pursuant to or in connection with the IPO.” 

 

	 	(c)	All references to “Canadian generally accepted accounting principles” be changed to “Canadian generally accepted accounting principles or US generally accepted accounting principles” in the IRA; and

	 	(d)	With respect to the IPO, all of the Shareholder’s rights under section 2.2 to 2.12 (inclusive) and 2.14 of the IRA are waived and no such right shall apply to or be effective in relation to the IPO.

  

	2.	approves that all options (and related Common Shares) issuable under the 2014 Equity Incentive Plan as approved by the Board of Directors and to be confirmed and adopted by the shareholders meeting to be held on or
before June 30, 2014 (the “New Equity Incentive Plan”) be deemed “Excepted Securities”, “Series E Excepted Securities” and “Current Series E Excepted Securities” as such terms are used in the
share rights set out in the Articles (Schedule A to the Addendum) of the Corporation. 

  

	3.	consents to the amendment of the amended and restated shareholders agreement dated December 6th, 2006, as amended from time to time (the “Shareholders
Agreement”) as follows (the “Shareholders Agreement Amendments”): 

  

	 	(a)	the whole of article 2 of the Shareholders Agreement be deleted but if by December 31, 2014, the closing of the IPO has not occurred, such Article 2 shall be deemed to be reinstated into the Shareholders Agreement
without further action from the Corporation or any of the shareholders of the Corporation; 

  

	 	(b)	section 4.12 of the Shareholders Agreement be deleted in its entirety and by replaced by the following: 

“4.12 Termination — This Agreement may be terminated upon written agreement or consent of Founders holding not less than 75%
of the aggregate number of Shares held by all Founders and by Investors holding not less than 75% of the aggregate number of Shares held by all Investors as at the time of such agreement or consent. Any such written agreement or consent to terminate
shall be binding on all parties to the Agreement.” 
  

	4.	agrees that the Shareholders Agreement shall be terminated upon closing of the IPO (as defined above) (“Shareholders Agreement Termination”). 

 

	5.	agrees to: 

  

	 	(a)	vote any and all of the Shareholder’s Shares in the capital of the Corporation owned or beneficially owned by the Shareholder, and all shares in the capital of the Corporation over which the Shareholder has a proxy
to vote, including but not limited to the Shareholder’s Shares, in favour of any resolution that the Corporation may put before shareholders of the Corporation at one or more shareholders meetings to be held on or before June 30, 2014
(i) for approval, ratification or confirmation of the aforementioned New Equity Incentive Plan, IRA Amendment, Shareholders Agreement Amendment, or Shareholders Agreement Termination, and any other approvals related to the foregoing and
(ii) for approval of the consolidation of the common shares of the Corporation prior to the IPO and the adoption of new articles and bylaws for the Corporation in preparation for or in connection with the closing of the IPO (including if the
directors deem appropriate, the creation of a new class of preferred shares issuable in series), and any other approvals related to the foregoing, each as approved by the Board of Directors of the Corporation and in connection with the IPO or in
preparation for the IPO ((i) and (ii) together “Approved Matters”); 

  

	 	(b)	upon written request or direction of the Corporation, execute and not revoke a form of proxy appointing any director of the Corporation as proxy, with full power of substitution, to attend, vote and otherwise act for
and on behalf of the Shareholder in respect of all of the Shareholder’s Shares in respect of all such matters which may come before a meeting of the shareholders of the Corporation relating to the Approved Matters; and 

	 	(c)	execute all such documents and take all such other action as may in the opinion of the Corporation be necessary or desirable in connection with the foregoing. 

 

	6.	acknowledges and agrees that this consent and agreement to vote constitutes a legal, valid and binding agreement of the Shareholder for which good and valuable consideration has been received. 

All defined terms used in this consent and agreement to vote shall have the meanings set out above and the definitions in the IRA and Shareholders Agreement
shall only apply to any text in quotes relating to suggested amendments to such agreements. 
  

									
	DATED     April 16    , 2014.	 		 	DATED     April 16    , 2014.
			
	         /s/ Petra Wittlin
	 		 	 /s/ Adrian Zumbach

					
	Name:	 	 Petra Wittlin
	 		 	Name:	 	 Adrian Zumbach

					
	Title:	 	 Head of Finance NIBR Europe
	 		 	Title:	 	 Legal Counsel NIBR

				
	Authorized Signatory for the Shareholder	 		 		 	

 XENON PHARMACEUTICALS INC. 

(the “Corporation”) 

CONSENT AND AGREEMENT TO VOTE 

Shareholder Information: 
 Name: Novo A/S (the
“Shareholder”) 
 Number and Series of Preferred Shares held by the Shareholder: 

Series A Preferred Shares: 0 

Series B Preferred Shares: 0 

Series E Preferred Shares: 437,956 
 (the above
together with any other shares of the Corporation acquired or to be acquired by the Shareholder after the date of this consent and agreement to vote that are entitled to vote at the Corporation’s shareholders’ meeting(s) called to consider
the matters (or part thereof) shall together be referred to below, the “Shareholder’s Shares”) 
 For good and valuable consideration (which
is acknowledged), the Shareholder irrevocably: 
  

	1.	consents to the amendment of the Amended and Restated Investor Rights Agreement dated December 6th, 2006, as amended from time to time (the “IRA”) by
(the “IRA Amendment”): 

  

	 	(a)	section 4.4 (a) of the IRA (under the heading “Termination and Waiver of Rights of First Refusal”) be deleted in its entirety and be replaced with the following: 

“effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering
is on terms as approved by the Board of Directors of the Company and will be completed on or before December 31, 2014 and will result in the common shares of the Company to be listed or quoted upon a recognized senior stock exchange or public
securities quotation system (including without limitation, the Toronto Stock Exchange or the NASDAQ National Market) (the “IPO”)”; 
  

	 	(b)	inserting new subsections under section 4.6 of the IRA as follows: 

 “(j) up to a maximum
number of common shares of the Company that is equal to US$5 million divided by the issue price per common share of the Company as approved by the Board of Directors of the Company in the IPO by way of private placement in connection with a
collaboration arrangement; 
 (k) all Equity Securities of the Company issued pursuant to or in connection with the IPO.” 

 

	 	(c)	All references to “Canadian generally accepted accounting principles” be changed to “Canadian generally accepted accounting principles or US generally accepted accounting principles” in the IRA; and

	 	(d)	With respect to the IPO, all of the Shareholder’s rights under section 2.2 to 2.12 (inclusive) and 2.14 of the IRA are waived and no such right shall apply to or be effective in relation to the IPO.

  

	2.	approves that all options (and related Common Shares) issuable under the 2014 Equity Incentive Plan as approved by the Board of Directors and to be confirmed and adopted by the shareholders meeting to be held on or
before June 30, 2014 (the “New Equity Incentive Plan”) be deemed “Excepted Securities”, “Series E Excepted Securities” and “Current Series E Excepted Securities” as such terms are used in the
share rights set out in the Articles (Schedule A to the Addendum) of the Corporation. 

  

	3.	consents to the amendment of the amended and restated shareholders agreement dated December 6th, 2006, as amended from time to time (the “Shareholders
Agreement”) as follows (the “Shareholders Agreement Amendments”): 

  

	 	(a)	the whole of article 2 of the Shareholders Agreement be deleted but if by December 31, 2014, the closing of the IPO has not occurred, such Article 2 shall be deemed to be reinstated into the Shareholders Agreement
without further action from the Corporation or any of the shareholders of the Corporation; 

  

	 	(b)	section 4.12 of the Shareholders Agreement be deleted in its entirety and by replaced by the following: 

“4.12 Termination — This Agreement may be terminated upon written agreement or consent of Founders holding not less than 75%
of the aggregate number of Shares held by all Founders and by Investors holding not less than 75% of the aggregate number of Shares held by all Investors as at the time of such agreement or consent. Any such written agreement or consent to terminate
shall be binding on all parties to the Agreement.” 
  

	4.	agrees that the Shareholders Agreement shall be terminated upon closing of the IPO (as defined above) (“Shareholders Agreement Termination”). 

 

	5.	agrees to: 

  

	 	(a)	vote any and all of the Shareholder’s Shares in the capital of the Corporation owned or beneficially owned by the Shareholder, and all shares in the capital of the Corporation over which the Shareholder has a proxy
to vote, including but not limited to the Shareholder’s Shares, in favour of any resolution that the Corporation may put before shareholders of the Corporation at one or more shareholders meetings to be held on or before June 30, 2014
(i) for approval, ratification or confirmation of the aforementioned New Equity Incentive Plan, IRA Amendment, Shareholders Agreement Amendment, or Shareholders Agreement Termination, and any other approvals related to the foregoing and
(ii) for approval of the consolidation of the common shares of the Corporation prior to the IPO and the adoption of new articles and bylaws for the Corporation in preparation for or in connection with the closing of the IPO (including if the
directors deem appropriate, the creation of a new class of preferred shares issuable in series), and any other approvals related to the foregoing, each as approved by the Board of Directors of the Corporation and in connection with the IPO or in
preparation for the IPO ((i) and (ii) together “Approved Matters”); 

  

	 	(b)	upon written request or direction of the Corporation, execute and not revoke a form of proxy appointing any director of the Corporation as proxy, with full power of substitution, to attend, vote and otherwise act for
and on behalf of the Shareholder in respect of all of the Shareholder’s Shares in respect of all such matters which may come before a meeting of the shareholders of the Corporation relating to the Approved Matters; and 

	 	(c)	execute all such documents and take all such other action as may in the opinion of the Corporation be necessary or desirable in connection with the foregoing. 

 

	6.	acknowledges and agrees that this consent and agreement to vote constitutes a legal, valid and binding agreement of the Shareholder for which good and valuable consideration has been received. 

All defined terms used in this consent and agreement to vote shall have the meanings set out above and the definitions in the IRA and Shareholders Agreement
shall only apply to any text in quotes relating to suggested amendments to such agreements. 
  

			
	DATED     April 10    , 2014.
	
	         /s/ Thomas Dyrberg

		
	Name:	 	 Thomas Dyrberg

		
	Title:	 	 Senior Partner

	
	Authorized Signatory for the Shareholder

 XENON PHARMACEUTICALS INC. 

(the “Corporation”) 

CONSENT AND AGREEMENT TO VOTE 

Shareholder Information: 
 Name: Roche Finance Ltd. (the
“Shareholder”) 
 Number and Series of Preferred Shares held by the Shareholder: 

Series A Preferred Shares: 0 

Series B Preferred Shares: 0 

Series E Preferred Shares: 738,615 
 (the above
together with any other shares of the Corporation acquired or to be acquired by the Shareholder after the date of this consent and agreement to vote that are entitled to vote at the Corporation’s shareholders’ meeting(s) called to consider
the matters (or part thereof) shall together be referred to below, the “Shareholder’s Shares”) 
 For good and valuable consideration (which
is acknowledged), the Shareholder irrevocably: 
  

	1.	consents to the amendment of the Amended and Restated Investor Rights Agreement dated December 6th, 2006, as amended from time to time (the “IRA”) by
(the “IRA Amendment”): 

  

	 	a.	section 4.4 (a) of the IRA (under the heading “Termination and Waiver of Rights of First Refusal”) be deleted in its entirety and be replaced with the following: 

“effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering
is on terms as approved by the Board of Directors of the Company and will be completed on or before December 31, 2014 and will result in the common shares of the Company to be listed or quoted upon a recognized senior stock exchange or public
securities quotation system (including without limitation, the Toronto Stock Exchange or the NASDAQ National Market) (the “IPO”)”; 
  

	 	b.	inserting new subsections under section 4.6 of the IRA as follows: 

 “(j) up to a maximum
number of common shares of the Company that is equal to US$5 million divided by the issue price per common share of the Company as approved by the Board of Directors of the Company in the IPO by way of private placement in connection with a
collaboration arrangement; 
 (k) all Equity Securities of the Company issued pursuant to or in connection with the IPO.” 

 

	 	c.	All references to “Canadian generally accepted accounting principles” be changed to “Canadian generally accepted accounting principles or US generally accepted accounting principles” in the IRA; and

	 	d.	With respect to the IPO, all of the Shareholder’s rights under section 2.2 to 2.12 (inclusive) and 2.14 of the IRA are waived and no such right shall apply to or be effective in relation to the IPO.

  

	2.	approves that all options (and related Common Shares) issuable under the 2014 Equity Incentive Plan as approved by the Board of Directors and to be confirmed and adopted by the shareholders meeting to be held on or
before June 30, 2014 (the “New Equity Incentive Plan”) be deemed “Excepted Securities”, “Series E Excepted Securities” and “Current Series E Excepted Securities” as such terms are used in the
share rights set out in the Articles (Schedule A to the Addendum) of the Corporation. 

  

	3.	consents to the amendment of the amended and restated shareholders agreement dated December 6th, 2006, as amended from time to time (the “Shareholders
Agreement”) as follows (the “Shareholders Agreement Amendments”): 

  

	 	a.	the whole of article 2 of the Shareholders Agreement be deleted but if by December 31, 2014, the closing of the IPO has not occurred, such Article 2 shall be deemed to be reinstated into the Shareholders Agreement
without further action from the Corporation or any of the shareholders of the Corporation; 

  

	 	b.	section 4.12 of the Shareholders Agreement be deleted in its entirety and by replaced by the following: 

“4.12 Termination — This Agreement may be terminated upon written agreement or consent of Founders holding not less than 75%
of the aggregate number of Shares held by all Founders and by Investors holding not less than 75% of the aggregate number of Shares held by all Investors as at the time of such agreement or consent. Any such written agreement or consent to terminate
shall be binding on all parties to the Agreement.” 
  

	4.	agrees that the Shareholders Agreement shall be terminated upon closing of the IPO (as defined above) (“Shareholders Agreement Termination”). 

 

	5.	agrees to: 

  

	 	a.	vote any and all of the Shareholder’s Shares in the capital of the Corporation owned or beneficially owned by the Shareholder, and all shares in the capital of the Corporation over which the Shareholder has a proxy
to vote, including but not limited to the Shareholder’s Shares, in favour of any resolution that the Corporation may put before shareholders of the Corporation at one or more shareholders meetings to be held on or before June 30, 2014
(i) for approval, ratification or confirmation of the aforementioned New Equity Incentive Plan, IRA Amendment, Shareholders Agreement Amendment, or Shareholders Agreement Termination, and any other approvals related to the foregoing and
(ii) for approval of the consolidation of the common shares of the Corporation prior to the IPO and the adoption of new articles and bylaws for the Corporation in preparation for or in connection with the closing of the IPO (including if the
directors deem appropriate, the creation of a new class of preferred shares issuable in series), and any other approvals related to the foregoing, each as approved by the Board of Directors of the Corporation and in connection with the IPO or in
preparation for the IPO ((i) and (ii) together “Approved Matters”); 

  

	 	b.	upon written request or direction of the Corporation, execute and not revoke a form of proxy appointing any director of the Corporation as proxy, with full power of substitution, to attend, vote and otherwise act for
and on behalf of the Shareholder in respect of all of the Shareholder’s Shares in respect of all such matters which may come before a meeting of the shareholders of the Corporation relating to the Approved Matters; and 

	 	c.	execute all such documents and take all such other action as may in the opinion of the Corporation be necessary or desirable in connection with the foregoing. 

 

	6.	acknowledges and agrees that this consent and agreement to vote constitutes a legal, valid and binding agreement of the Shareholder for which good and valuable consideration has been received. 

All defined terms used in this consent and agreement to vote shall have the meanings set out above and the definitions in the IRA and Shareholders Agreement
shall only apply to any text in quotes relating to suggested amendments to such agreements. 
  

									
	DATED                 , 2014.	 		 	DATED                 , 2014.
			
	 Roche Finance Ltd
	 		 	 Roche Finance Ltd

					
	Name:	 	 /s/ Carole Nuechterlein
	 		 	Name:	 	 /s/ Andreas Knierzinger

		 	Carole Nuechterlein	 		 		 	Andreas Knierzinger
					
	Title:	 	 Authorized Signatory
	 		 		 	 Authorized Signatory

				
	Authorized Signatory for the Shareholder	 		 		 	

 XENON PHARMACEUTICALS INC. 

(the “Corporation”) 

CONSENT AND AGREEMENT TO VOTE 

Shareholder Information: 
 Name: Takeda Pharmaceutical
Company Limited (the “Shareholder”) 
 Number and Series of Preferred Shares held by the Shareholder: 

Series A Preferred Shares: 0 

Series B Preferred Shares: 0 

Series E Preferred Shares: 524,016 
 (the above
together with any other shares of the Corporation acquired or to be acquired by the Shareholder after the date of this consent and agreement to vote that are entitled to vote at the Corporation’s shareholders’ meeting(s) called to consider
the matters (or part thereof) shall together be referred to below, the “Shareholder’s Shares”) 
 For good and valuable consideration (which
is acknowledged), the Shareholder irrevocably: 
  

	1.	consents to the amendment of the Amended and Restated Investor Rights Agreement dated December 6th, 2006, as amended from time to time (the “IRA”) as
set forth below: 

  

	 	a.	section 4.4 (a) of the IRA (under the heading “Termination and Waiver of Rights of First Refusal”) be deleted in its entirety and be replaced with the following: 

“effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering
is on terms as approved by the Board of Directors of the Company and will be completed on or before December 31, 2014 and will result in the common shares of the Company to be listed or quoted upon a recognized senior stock exchange or public
securities quotation system (including without limitation, the Toronto Stock Exchange or the NASDAQ National Market) (the “IPO”)”; 
  

	 	b.	inserting new subsections under section 4.6 of the IRA as follows: 

 “(j) up to a maximum
number of common shares of the Company that is equal to US$5 million divided by the issue price per common share of the Company as approved by the Board of Directors of the Company in the IPO by way of private placement in connection with a
collaboration arrangement; 
 (k) all Equity Securities of the Company issued pursuant to or in connection with the IPO.” 

 

	 	c.	All references to “Canadian generally accepted accounting principles” be changed to “Canadian generally accepted accounting principles or US generally accepted accounting principles” in the IRA; and

	 	d.	With respect to the IPO, all of the Shareholder’s rights under section 2.2 to 2.12 (inclusive) and 2.14 of the IRA are waived and no such right shall apply to or be effective in relation to the IPO,

 the above which shall hereinafter be referred to as the “IRA Amendment”. 

 

	2.	approves that all options (and related Common Shares) issuable under the 2014 Equity Incentive Plan as approved by the Board of Directors and to be confirmed and adopted by the shareholders meeting to be held on or
before June 30, 2014 (the “New Equity Incentive Plan”) be deemed “Excepted Securities”, “Series E Excepted Securities” and “Current Series E Excepted Securities” as such terms are used in the
share rights set out in the Articles (Schedule A to the Addendum) of the Corporation. 

  

	3.	consents to the amendment of the amended and restated shareholders agreement dated December 6th, 2006, as amended from time to time (the “Shareholders
Agreement”) as follows (the “Shareholders Agreement Amendments”): 

  

	 	a.	the whole of article 2 of the Shareholders Agreement be deleted but if by December 31, 2014, the closing of the IPO has not occurred, such Article 2 shall be deemed to be reinstated into the Shareholders Agreement
without further action from the Corporation or any of the shareholders of the Corporation; 

  

	 	b.	section 4.12 of the Shareholders Agreement be deleted in its entirety and by replaced by the following: 

“4.12 Termination — This Agreement may be terminated upon written agreement or consent of Founders holding not less than 75%
of the aggregate number of Shares held by all Founders and by Investors holding not less than 75% of the aggregate number of Shares held by all Investors as at the time of such agreement or consent. Any such written agreement or consent to terminate
shall be binding on all parties to the Agreement.” 
  

	4.	agrees that the Shareholders Agreement shall be terminated upon closing of the IPO (as defined above) (“Shareholders Agreement Termination”). 

 

	5.	agrees to: 

  

	 	a.	vote any and all of the Shareholder’s Shares in the capital of the Corporation owned or beneficially owned by the Shareholder, and all shares in the capital of the Corporation over which the Shareholder has a proxy
to vote, including but not limited to the Shareholder’s Shares, in favour of any resolution that the Corporation may put before shareholders of the Corporation at one or more shareholders meetings to be held on or before June 30, 2014
(i) for approval, ratification or confirmation of the aforementioned New Equity Incentive Plan, IRA Amendment, Shareholders Agreement Amendment, or Shareholders Agreement Termination, and any other approvals related to the foregoing and
(ii) for approval of the consolidation of the common shares of the Corporation prior to the IPO and the adoption of new articles and bylaws for the Corporation in preparation for or in connection with the closing of the IPO (including if the
directors deem appropriate, the creation of a new class of preferred shares issuable in series), and any other approvals related to the foregoing, each as approved by the Board of Directors of the Corporation and in connection with the IPO or in
preparation for the IPO ((i) and (ii) together “Approved Matters”); 

	 	b.	upon written request or direction of the Corporation, execute and not revoke a form of proxy appointing any director of the Corporation as proxy, with full power of substitution, to attend, vote and otherwise act for
and on behalf of the Shareholder in respect of all of the Shareholder’s Shares in respect of all such matters which may come before a meeting of the shareholders of the Corporation relating to the Approved Matters; and 

 

	 	c.	execute all such documents and take all such other action as may in the opinion of the Corporation be necessary or desirable in connection with the foregoing. 

 

	6.	acknowledges and agrees that this consent and agreement to vote constitutes a legal, valid and binding agreement of the Shareholder for which good and valuable consideration has been received. 

All defined terms used in this consent and agreement to vote shall have the meanings set out above and the definitions in the IRA and Shareholders Agreement
shall only apply to any text in quotes relating to suggested amendments to such agreements. 
  

			
	DATED   April 15        , 2014.
	
	         /s/ for Icen Aralia

		
	Name:	 	 for Icen Aralia

		
	Title:	 	 VP, Global R&D, Takeda Pharmaceutical Company Ltd.

	
	Authorized Signatory for the Shareholder

 XENON PHARMACEUTICALS INC. 

(the “Corporation”) 

CONSENT AND AGREEMENT TO VOTE 

Shareholder Information: 
 Name: Takeda Ventures, Inc.
(previously known as Takeda Research Investment, Inc.) (the “Shareholder”) 
 Number and Series of Preferred Shares held by the Shareholder: 

Series A Preferred Shares: 0 

Series B Preferred Shares: 0 

Series E Preferred Shares: 529,926 
 (the above
together with any other shares of the Corporation acquired or to be acquired by the Shareholder after the date of this consent and agreement to vote that are entitled to vote at the Corporation’s shareholders’ meeting(s) called to consider
the matters (or part thereof) shall together be referred to below, the “Shareholder’s Shares”) 
 For good and valuable consideration (which
is acknowledged), the Shareholder irrevocably: 
  

	1.	consents to the amendment of the Amended and Restated Investor Rights Agreement dated December 6th, 2006, as amended from time to time (the “IRA”) as
set forth below: 

  

	 	a.	section 4.4 (a) of the IRA (under the heading “Termination and Waiver of Rights of First Refusal”) be deleted in its entirety and be replaced with the following: 

“effective date of the registration statement pertaining to a firmly underwritten initial public offering by the Company, if such offering
is on terms as approved by the Board of Directors of the Company and will be completed on or before December 31, 2014 and will result in the common shares of the Company to be listed or quoted upon a recognized senior stock exchange or public
securities quotation system (including without limitation, the Toronto Stock Exchange or the NASDAQ National Market) (the “IPO”)”; 
  

	 	b.	inserting new subsections under section 4.6 of the IRA as follows: 

 “(j) up to a maximum
number of common shares of the Company that is equal to US$5 million divided by the issue price per common share of the Company as approved by the Board of Directors of the Company in the IPO by way of private placement in connection with a
collaboration arrangement; 
 (k) all Equity Securities of the Company issued pursuant to or in connection with the IPO.” 

 

	 	c.	All references to “Canadian generally accepted accounting principles” be changed to “Canadian generally accepted accounting principles or US generally accepted accounting principles” in the IRA; and

	 	d.	With respect to the IPO, all of the Shareholder’s rights under section 2.2 to 2.12 (inclusive) and 2.14 of the IRA are waived and no such right shall apply to or be effective in relation to the IPO,

 the above which shall hereinafter be referred to as the “IRA Amendment”. 

 

	2.	approves that all options (and related Common Shares) issuable under the 2014 Equity Incentive Plan as approved by the Board of Directors and to be confirmed and adopted by the shareholders meeting to be held on or
before June 30, 2014 (the “New Equity Incentive Plan”) be deemed “Excepted Securities”, “Series E Excepted Securities” and “Current Series E Excepted Securities” as such terms are used in the
share rights set out in the Articles (Schedule A to the Addendum) of the Corporation. 

  

	3.	consents to the amendment of the amended and restated shareholders agreement dated December 6th, 2006, as amended from time to time (the “Shareholders
Agreement”) as follows (the “Shareholders Agreement Amendments”): 

  

	 	a.	the whole of article 2 of the Shareholders Agreement be deleted but if by December 31, 2014, the closing of the IPO has not occurred, such Article 2 shall be deemed to be reinstated into the Shareholders Agreement
without further action from the Corporation or any of the shareholders of the Corporation; 

  

	 	b.	section 4.12 of the Shareholders Agreement be deleted in its entirety and by replaced by the following: 

“4.12 Termination — This Agreement may be terminated upon written agreement or consent of Founders holding not less than 75%
of the aggregate number of Shares held by all Founders and by Investors holding not less than 75% of the aggregate number of Shares held by all Investors as at the time of such agreement or consent. Any such written agreement or consent to terminate
shall be binding on all parties to the Agreement.” 
  

	4.	agrees that the Shareholders Agreement shall be terminated upon closing of the IPO (as defined above) (“Shareholders Agreement Termination”). 

 

	5.	agrees to: 

  

	 	a.	vote any and all of the Shareholder’s Shares in the capital of the Corporation owned or beneficially owned by the Shareholder, and all shares in the capital of the Corporation over which the Shareholder has a proxy
to vote, including but not limited to the Shareholder’s Shares, in favour of any resolution that the Corporation may put before shareholders of the Corporation at one or more shareholders meetings to be held on or before June 30, 2014
(i) for approval, ratification or confirmation of the aforementioned New Equity Incentive Plan, IRA Amendment, Shareholders Agreement Amendment, or Shareholders Agreement Termination, and any other approvals related to the foregoing and
(ii) for approval of the consolidation of the common shares of the Corporation prior to the IPO and the adoption of new articles and bylaws for the Corporation in preparation for or in connection with the closing of the IPO (including if the
directors deem appropriate, the creation of a new class of preferred shares issuable in series), and any other approvals related to the foregoing, each as approved by the Board of Directors of the Corporation and in connection with the IPO or in
preparation for the IPO ((i) and (ii) together “Approved Matters”); 

	 	b.	upon written request or direction of the Corporation, execute and not revoke a form of proxy appointing any director of the Corporation as proxy, with full power of substitution, to attend, vote and otherwise act for
and on behalf of the Shareholder in respect of all of the Shareholder’s Shares in respect of all such matters which may come before a meeting of the shareholders of the Corporation relating to the Approved Matters; and 

 

	 	c.	execute all such documents and take all such other action as may in the opinion of the Corporation be necessary or desirable in connection with the foregoing. 

 

	6.	acknowledges and agrees that this consent and agreement to vote constitutes a legal, valid and binding agreement of the Shareholder for which good and valuable consideration has been received. 

All defined terms used in this consent and agreement to vote shall have the meanings set out above and the definitions in the IRA and Shareholders Agreement
shall only apply to any text in quotes relating to suggested amendments to such agreements. 
  

			
	DATED   April 15        , 2014.
	
	 /s/ G.R. Martin

		
	Name:	 	 G. R. Martin

		
	Title:	 	 President & CEO

	
	Authorized Signatory for the Shareholder

 JOINDER TO INVESTOR RIGHTS AGREEMENT 

This Joinder (this “Agreement”) is delivered and effective as of November 7, 2013, by Lexington Private Equity VI,
L.P. (“Lexington”) pursuant to that certain Amended and Restated Investor Rights Agreement of Xenon Pharmaceuticals Inc. (the “Company”), dated as of December 6, 2006 (as amended, the “Investor Rights
Agreement”). Terms defined in the Investor Rights Agreement and not otherwise defined herein shall have the meanings ascribed to them in the Investor Rights Agreement. 

WHEREAS, on the date hereof, Lexington acquired the 435,037 shares of Series E Preferred Stock of the Company (the “Transferred
Shares”) held by J.P. Morgan Partners (BHCA), L.P. (the “Transferor”), and all the rights and obligations with respect thereto (the “Transfer”); and 

NOW, THEREFORE, in connection with the Transfer, Lexington hereby agrees as follows: 

 

	 	1.	Upon execution of this Agreement, Lexington shall become a party to the Investor Rights Agreement and shall be bound by all the terms and provisions thereof. Lexington shall succeed to all rights and be subject to all
the obligations of the Transferor with respect to the Transferred Shares as set forth in the Investor Rights Agreement, including without limitation pursuant to Sections 2.10, 4.5 and 10.3 thereof, and become a Holder, Investor and Major Investor
thereunder. 

  

	 	2.	The Transferee’s execution of a signature page to this Agreement shall constitute the execution by the Transferee of a counterpart signature page to the Investor Rights Agreement with respect to the Transferred
Shares. 

  

	 	3.	The Transferee hereby requests that the Company update Exhibits A and B to the Investor Rights Agreement and its books and records to reflect the Transfer. For purposes of delivering any notices, requests and other
communications under the Investor Rights Agreement, the address of the Lexington is included on its signature page hereto. 

  

	 	4.	This Agreement shall be governed by and construed under the laws of the Province of British Columbia. 

[Signature Page follows] 

 The parties have executed this Agreement as of the date first set forth above. 

 

			
	LEXINGTON PRIVATE EQUITY VI, L.P.
		
	By:	 	Archer Venture Acquisitions 1, LLC,
		 	its general partner
		
	By:	 	Lexington Partners GP Holdings VII LLC,
		 	its sole member
		
	By:	 	Lexington Partners L.P.,
		 	its sole member
		
	By:	 	 /s/Thomas Giannetti

	Name:	 	Thomas Giannetti
	Title:	 	Chief Financial Officer
	
	c/o Lexington Partners L.P.
	660 Madison Avenue, 23rd Floor
	New York, NY 10021-8405
	Attention: Thomas Giannetti
	Telephone: (212) 754-0411
	Fax: (212) 754-1494
	Email: tgiannetti@lexpartners.com
	
	with a required copy to (which shall not constitute notice):
	
	Proskauer Rose LLP
	1 International Place
	Boston, MA 02110-2600
	Attention: Sean Hill and Ori Solomon
	Telephone: (617) 526-9600
	Fax: (617) 526-9899
	Email: lexdeals@proskauer.com

 SIGNATURE PAGE TO JOINDER TO AMENDED AND RESTATED INVESTOR RIGHTS AGREEMENT XENON PHARMACEUTICALS INC.

 JOINDER TO INVESTOR RIGHT AGREEMENT 

(International Business Machines Corporation) 

This Joinder (this “Agreement”) is delivered and effective as of December 4, 2013, by International Business Machines
Corporation (“IBM”) pursuant to that certain Amended and Restated Investor Rights Agreement of Xenon Pharmaceuticals Inc. (the “Company”) dated as of December 6, 2006, as amended, (the “Investor Rights
Agreement”). Terms defined in the Investor Rights Agreement and not otherwise defined herein shall have the meanings ascribed to them in the Investor Rights Agreement. 

WHEREAS, on the date hereof, IBM acquired the 7,121 shares of Series E Preferred Shares and the 11,826 shares of Series B Preferred Shares of
the Company (the “Transferred Shares”) held by Ventures West 7 U.S. Limited Partnership (the “Transferor”), and all the rights and obligations with respect thereto (the “Transfer”); and 

NOW, THEREFORE, in connection with the Transfer, IBM hereby agrees as follows: 

 

	 	1.	Upon execution of this Agreement, IBM shall become a party to the Investor Rights Agreement and shall be bound by all terms and provisions thereof. IBM shall succeed to all rights and be subject to all the obligations
of the Transferor with respect to the Transferred Shares as set forth in the Investor Rights Agreement, and become a Holder and Investor thereunder. 

  

	 	2.	The Transferee’s execution of a signature page to this Agreement shall constitute the execution by the Transferee of a counterpart signature page to the Investor Rights Agreement with respect to the Transferred
Shares. 

  

	 	3.	The Transferee hereby requests that the Company update its books and records to reflect the Transfer. For purposes of delivering any notices, requests and other communications under the Investor Rights Agreement, the
address of IBM is included on its signature page hereto. 

  

	 	4.	This Agreement shall be governed by and construed under the laws of the Province of British Columbia. 

[Signature Page follows] 

  
 Page 1 of 2 

 Duly-authorized signatory of IBM has executed this Agreement below, effective as of the date
first set forth above. 
  

			
	INTERNATIONAL BUSINESS MACHINES CORPORATION
		
	By:	 	 /s/ Robert Pemberton

	Name:	 	 Robert Pemberton

	Title:	 	 Vice President, Corporate Development

 Contact information for International Business Machines Corporation: 

International Business Machines Corporation 

New Orchard Road, MD 250 
 Armonk,
NY 10504 
 Attention: Kevin G. Liddy 

Telephone: 314-252-6030 

Facsimile: 314-252-4336 
 Email:
kgliddy@us.ibm.com 
 SIGNATURE PAGE TO JOINDER TO AMENDED 

AND RESTATED INVESTOR RIGHTS AGREEMENT 

XENON PHARMACEUTICALS INC. 

 JOINDER TO INVESTOR RIGHT AGREEMENT 

(California Emerging Ventures H, LLC) 

This Joinder (this “Agreement”) is delivered and effective as of December 4, 2013, by California Emerging Ventures II,
LLC (“CEV II”) pursuant to that certain Amended and Restated Investor Rights Agreement of Xenon Pharmaceuticals Inc. (the “Company”) dated as of December 6, 2006, as amended, (the “Investor Rights
Agreement”). Terms defined in the Investor Rights Agreement and not otherwise defined herein shall have the meanings ascribed to them in the Investor Rights Agreement. 

WHEREAS, on the date hereof, CEV II acquired the 7,121 shares of Series E Preferred Shares and the 11,826 shares of Series B Preferred Shares
of the Company (the “Transferred Shares”) held by Ventures West 7 U.S. Limited Partnership (the “Transferor”), and all the rights and obligations with respect thereto (the “Transfer”); and 

NOW, THEREFORE, in connection with the Transfer, CEV II hereby agrees as follows: 

 

	 	1.	Upon execution of this Agreement, CEV II shall become a party to the Investor Rights Agreement and shall be bound by all terms and provisions thereof. CEV II shall succeed to all rights and be subject to all the
obligations of the Transferor with respect to the Transferred Shares as set forth in the Investor Rights Agreement, and become a Holder and Investor thereunder. 

  

	 	2.	The Transferee’s execution of a signature page to this Agreement shall constitute the execution by the Transferee of a counterpart signature page to the Investor Rights Agreement with respect to the Transferred
Shares. 

  

	 	3.	The Transferee hereby requests that the Company update its books and records to reflect the Transfer. For purposes of delivering any notices, requests and other communications under the Investor Rights Agreement, the
address of CEV II is included on its signature page hereto. 

  

	 	4.	This Agreement shall be governed by and construed under the laws of the Province of British Columbia. 

[Signature Page follows] 

  
 Page 1 of 2 

 Duly-authorized signatory of CEV II has executed this Agreement below, effective as of the date
first set forth above. 
  

			
	CALIFORNIA EMERGING VENTURES II, LLC
		
	By:	 	GSA Partners II, LLC, its Manager
	by:	 	Grove Street Advisors, LLC, its Manager
		
	By:	 	 /s/ Ann St. Germain

	Name:	 	 ANN ST. GERMAIN

	Title:	 	 MEMBER & CFO

 Contact Information for California Emerging Ventures II, LLC: 

California Emerging Ventures II, LLC 

c/o Merrill Lynch 
 1000 Federal
Street, 17th Floor 
 Boston, MA 02110 

Attention: Andrew Lodoen 

Telephone: 617-846-4185 

Facsimile: 617-830-6026 
 Email:
Andrew_lodoen@ml.com 
 California Emerging Ventures II, LLC 

c/o Grove Street Advisors, LLC 

20 William Street, Sutie 230 

Wellesley, MA 02481 
 Attention:
Ann St. Germain 
 Telephone: 781-263-6140 

Facsimile: 781-263-6101 
 Email:
ams@grovestreet.com 
 SIGNATURE PAGE TO JOINDER TO AMENDED 

AND RESTATED INVESTOR RIGHTS AGREEMENT 

XENON PHARMACEUTICALS INC. 

 JOINDER TO INVESTOR RIGHT AGREEMENT (BancBoston Capital Inc.) 

This Joinder (this “Agreement”) is delivered and effective as of December 4, 2013, by BancBoston Capital Inc.
(“BancBoston”) pursuant to that certain Amended and Restated Investor Rights Agreement of Xenon Pharmaceuticals Inc. (the “Company”) dated as of December 6, 2006, as amended, (the “Investor Rights
Agreement”). Terms defined in the Investor Rights Agreement and not otherwise defined herein shall have the meanings ascribed to them in the Investor Rights Agreement. 

WHEREAS, on the date hereof, BancBoston acquired the 4,795 shares of Series E Preferred Shares and the 7,961 shares of Series B Preferred
Shares of the Company (the “Transferred Shares”) held by Ventures West 7 U.S. Limited Partnership (the “Transferor”), and all the rights and obligations with respect thereto (the “Transfer”); and

 NOW, THEREFORE, in connection with the Transfer, BancBoston hereby agrees as follows: 

 

	 	1.	Upon execution of this Agreement, BancBoston shall become a party to the Investor Rights Agreement and shall be bound by all terms and provisions thereof. BancBoston shall succeed to all rights and be subject to all the
obligations of the Transferor with respect to the Transferred Shares as set forth in the Investor Rights Agreement, and become a Holder and Investor thereunder. 

  

	 	2.	The Transferee’s execution of a signature page to this Agreement shall constitute the execution by the Transferee of a counterpart signature page to the Investor Rights Agreement with respect to the Transferred
Shares. 

  

	 	3.	The Transferee hereby requests that the Company update its books and records to reflect the Transfer. For purposes of delivering any notices, requests and other communications under the Investor Rights Agreement, the
address of BancBoston is included on its signature page hereto. 

  

	 	4.	This Agreement shall be governed by and construed under the laws of the Province of British Columbia. 

[Signature Page follows] 

  
 Page 1 of 2 

 Duly-authorized signatory of BancBoston has executed this Agreement below, effective as of the
date first set forth above. 
  

			
	BANCBOSTON CAPITAL INC.
		
	By:	 	/s/ Andrew T. Golomb
	Name:	 	Andrew T. Golomb
	Title:	 	Director

 Contact Information for BancBoston Capital Inc.: 

BancBoston Capital Inc. 
 c/o
Bank of America 
 101 South Tryon Street 

Charlotte, NC 28255 
 Attention:
Andrew T. Golomb 
 Telephone: 980-387-1966 

Facsimile: 704-208-2782 
 Email:
andrew.t.golomb@baml.com 
 With a copy to: 

BancBoston Capital Inc. 
 c/o
Corrum Capital Management LLC 
 214 North Tryon Street 

Suite 1950 
 Charlotte, NC 28202

 Attention: Andrew Lobas, Associate 

Telephone: 704-330-7314 
 Email:
alobas@corrumcapital.com 
 SIGNATURE PAGE TO JOINDER TO AMENDED 

AND RESTATED INVESTOR RIGHTS AGREEMENT 

XENON PHARMACEUTICALS INC. 

 JOINDER TO INVESTOR RIGHT AGREEMENT 

(Cape 1998 Trust) 
 This
Joinder (this “Agreement”) is delivered and effective as of December 4, 2013, by Cape 1998 Trust pursuant to that certain Amended and Restated Investor Rights Agreement of Xenon Pharmaceuticals Inc. (the
“Company”) dated as of December 6, 2006, as amended, (the “Investor Rights Agreement”). Terms defined in the Investor Rights Agreement and not otherwise defined herein shall have the meanings ascribed to them
in the Investor Rights Agreement. 
 WHEREAS, on the date hereof, Cape 1998 Trust acquired the 142 shares of Series E Preferred Shares and
the 237 shares of Series B Preferred Shares of the Company (the “Transferred Shares”) held by Ventures West 7 U.S. Limited Partnership (the “Transferor”), and all the rights and obligations with respect thereto (the
“Transfer”); and 
 NOW, THEREFORE, in connection with the Transfer, Cape 1998 Trust hereby agrees as follows: 

 

	 	1.	Upon execution of this Agreement, Cape 1998 Trust shall become a party to the Investor Rights Agreement and shall be bound by all terms and provisions thereof. Cape 1998 Trust shall succeed to all rights and be subject
to all the obligations of the Transferor with respect to the Transferred Shares as set forth in the Investor Rights Agreement, and become a Holder and Investor thereunder. 

 

	 	2.	The Transferee’s execution of a signature page to this Agreement shall constitute the execution by the Transferee of a counterpart signature page to the Investor Rights Agreement with respect to the Transferred
Shares. 

  

	 	3.	The Transferee hereby requests that the Company update its books and records to reflect the Transfer. For purposes of delivering any notices, requests and other communications under the Investor Rights Agreement, the
address of Cape 1998 Trust is included on its signature page hereto. 

  

	 	4.	This Agreement shall be governed by and construed under the laws of the Province of British Columbia. 

[Signature Page follows] 

  
 Page 1 of 2 

 Duly-authorized signatory of Cape 1998 Trust has executed this Agreement below, effective as of the date first
set forth above. 
  

			
	CAPE 1998 TRUST
		
	By:	 	/s/ Lillian J. Cape
	Name:	 	Lillian J. Cape
	Title:	 	Trustee

 Contact Information for Cape 1998 Trust: 

Cape 1998 Trust 
 c/o
Howson & Simon LLP 
 101 Ygnacio Valley Road, 

Suite 310 
 Walnut Creek, CA94596

 Attention: Carla S. Lundstrom 

Telephone: 925-977-9060 

Facsimile: 925-977-9064 
 Email:
clundstrom@howson-simon.com 
 with a copy to: 

Cape 1998 Trust 
 c/o Lillian
Cape, Trustee 
 1750 Taylor Street, #2001 

San Francisco, CA 94133 

Telephone: 415-923-9876 
 Email:
libisf@me.com 
 SIGNATURE PAGE TO JOINDER TO AMENDED 

AND RESTATED INVESTOR RIGHTS AGREEMENT 

XENON PHARMACEUTICALS INC.Prepared by R.R. Donnelley Financial -- EX-10.1

 Exhibit 10.1 
  

 
 EXECUTION VERSION 

EXCLUSIVE COLLABORATIVE RESEARCH AND OPTION AGREEMENT 

by and between 
 MERCK
SHARP & DOHME RESEARCH LTD. 
 and 

XENON PHARMACEUTICALS INC. 
 [†]
DESIGNATES PORTIONS OF THIS DOCUMENT THAT HAVE BEEN OMITTED PURSUANT TO A REQUEST FOR CONFIDENTIAL TREATMENT FILED SEPARATELY WITH THE COMMISSION 

 

 
 EXECUTION VERSION 
  

 TABLE OF CONTENTS 

 

							
	ARTICLE 1	 	DEFINITIONS	  	 	1	  
			
	ARTICLE 2	 	RESEARCH PROGRAM	  	 	8	  
			
	 2.1
	 	General.	  	 	8	  
	 2.2
	 	Research Program Term.	  	 	8	  
	 2.3
	 	Conduct of Research.	  	 	9	  
	 2.4
	 	Principal Scientists.	  	 	9	  
	 2.5
	 	Research Program.	  	 	10	  
	 2.6
	 	Ownership of Technology.	  	 	10	  
	 2.7
	 	Joint Steering Committee.	  	 	11	  
	 2.8
	 	Decision-Making.	  	 	11	  
	 2.9
	 	JSC Responsibilities.	  	 	11	  
	 2.10
	 	Meetings.	  	 	12	  
	 2.11
	 	Records.	  	 	12	  
	 2.12
	 	Copies and Inspection of Records.	  	 	12	  
	 2.13
	 	Quarterly Reports.	  	 	13	  
	 2.14
	 	Annual Report.	  	 	13	  
			
	ARTICLE 3	 	MERCK OPTION	  	 	13	  
			
	 3.1
	 	Merck Option.	  	 	13	  
	 3.2
	 	Exercise of Merck Option.	  	 	13	  
	 3.3
	 	Transfer by Xenon.	  	 	13	  
	 3.4
	 	Option Exercise Fee.	  	 	14	  
	 3.5
	 	Xenon Option.	  	 	14	  
	 3.6
	 	Merck Research Program Technology.	  	 	14	  
	 3.7
	 	Annual Report.	  	 	15	  
	 3.8
	 	Joint Development Committee.	  	 	15	  
	 3.9
	 	Co-Funded Products – Reversion Rights and Credited Payments..	  	 	15	  
	 3.10
	 	[†].	  	 	15	  
	 3.11
	 	[†].	  	 	16	  
			
	ARTICLE 4	 	LICENSE; EXCHANGE OF INFORMATION; DEVELOPMENT AND COMMERCIALIZATION	  	 	16	  
			
	 4.1
	 	License Grants.	  	 	16	  
	 4.2
	 	Internal Research Purposes.	  	 	17	  
	 4.3
	 	Exchange of Information.	  	 	17	  
	 4.4
	 	No Implied Licenses.	  	 	17	  
	 4.5
	 	Development and Commercialization.	  	 	17	  
			
	ARTICLE 5	 	CONFIDENTIALITY AND PUBLICATION	  	 	17	  

  
 i 

[†] DESIGNATES PORTIONS OF THIS DOCUMENT THAT HAVE BEEN OMITTED PURSUANT TO A REQUEST FOR CONFIDENTIAL TREATMENT FILED SEPARATELY WITH THE COMMISSION

 

 
 EXECUTION VERSION 
  

							
	 5.1
	 	Nondisclosure Obligation.	  	 	17	  
	 5.2
	 	Publication.	  	 	19	  
	 5.3
	 	Publicity/Use of Names.	  	 	19	  
			
	ARTICLE 6	 	PAYMENTS; ROYALTIES AND REPORTS	  	 	20	  
			
	 6.1
	 	Research Program Costs.	  	 	20	  
	 6.2
	 	FTE Funding.	  	 	20	  
	 6.3
	 	Milestone Payments.	  	 	20	  
	 6.4
	 	Royalties.	  	 	22	  
	 6.5
	 	Reports; Payment of Royalty.	  	 	24	  
	 6.6
	 	Audits.	  	 	24	  
	 6.7
	 	Payment Exchange Rate.	  	 	25	  
	 6.8
	 	Income Tax Withholding.	  	 	25	  
			
	ARTICLE 7	 	REPRESENTATIONS AND WARRANTIES	  	 	25	  
			
	 7.1
	 	Representations and Warranties of Each Party.	  	 	25	  
	 7.2
	 	Xenon Representations and Warranties.	  	 	25	  
	 7.3
	 	Merck Representations and Warranties.	  	 	26	  
	 7.4
	 	Covenants.	  	 	26	  
	 7.5
	 	Disclaimer of Warranties.	  	 	26	  
	 7.6
	 	Limitation of Liability.	  	 	27	  
	 7.7
	 	Indemnification.	  	 	27	  
	 7.8
	 	Insurance.	  	 	28	  
			
	ARTICLE 8	 	PATENT PROVISIONS	  	 	28	  
			
	 8.1
	 	Filing, Prosecution and Maintenance of Patent Rights During the Research Program Term.	  	 	28	  
	 8.2
	 	Exercise of the Merck Option.	  	 	29	  
	 8.3
	 	 Filing, Prosecution and Maintenance of Patent Rights Following Expiration of the Research Program Term.
	  	 	30	  
	 8.4
	 	Enforcement and Defense.	  	 	30	  
	 8.5
	 	Cooperation; Patent Term Restoration; Pediatric Study Extensions.	  	 	32	  
	 8.6
	 	Inventorship.	  	 	32	  
			
	ARTICLE 9	 	TERM AND TERMINATION	  	 	32	  
			
	 9.1
	 	Term and Expiration.	  	 	32	  
	 9.2
	 	Termination By Merck.	  	 	33	  
	 9.3
	 	Termination for Cause.	  	 	33	  
	 9.4
	 	Effect of Expiration or Termination; Survival.	  	 	34	  
	 9.5
	 	[†].	  	 	35	  

  
 ii 

[†] DESIGNATES PORTIONS OF THIS DOCUMENT THAT HAVE BEEN OMITTED PURSUANT TO A REQUEST FOR CONFIDENTIAL TREATMENT FILED SEPARATELY WITH THE COMMISSION

 

 
 EXECUTION VERSION 
  

							
			
	ARTICLE 10	 	MISCELLANEOUS	  	 	35	  
			
	 10.1
	 	Force Majeure.	  	 	35	  
	 10.2
	 	Assignment.	  	 	36	  
	 10.3
	 	Severability.	  	 	36	  
	 10.4
	 	Notices.	  	 	36	  
	 10.5
	 	Applicable Law.	  	 	37	  
	 10.6
	 	Dispute Resolution.	  	 	38	  
	 10.7
	 	Entire Agreement; Amendments.	  	 	39	  
	 10.8
	 	Headings.	  	 	39	  
	 10.9
	 	Independent Contractors.	  	 	39	  
	 10.10
	 	Waiver.	  	 	39	  
	 10.11
	 	Cumulative Remedies.	  	 	39	  
	 10.12
	 	Waiver of Rule of Construction.	  	 	39	  
	 10.13
	 	Certain Conventions.	  	 	40	  
	 10.14
	 	Business Day Requirements.	  	 	40	  
	 10.15
	 	Counterparts.	  	 	40	  
	 Exhibit 1.56
	 	MERCK PATENT RIGHTS	  	 	41	  
	 Exhibit 1.78
	 	RESEARCH PLAN	  	 	42	  
	 Exhibit 1.100
	 	XENON PATENT RIGHTS	  	 	43	  
	 Exhibit 1.102
	 	XENON TARGET VALIDATION CRITERIA	  	 	44	  
	 Exhibit 5.3
	 	PRESS RELEASE	  	 	45	  

  
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 EXCLUSIVE COLLABORATIVE RESEARCH AND OPTION AGREEMENT 

This Exclusive Collaborative Research and Option Agreement (the “Agreement”) is effective as of June 10, 2009 (the “Effective
Date”) and is entered into by and between MERCK SHARP & DOHME RESEARCH LTD., a company organized and existing under the laws of Bermuda (“Merck”), and XENON PHARMACEUTICALS INC., a corporation organized and
existing under the laws of Canada (“Xenon”). 
 RECITALS: 

WHEREAS, Xenon is a research-based company focused on new drug discovery and development of pharmaceutical products; 

WHEREAS, Merck discovers, develops, manufactures and markets vaccines and medicines for the treatment of human diseases and/or conditions; and 

WHEREAS, Xenon and Merck wish to enter into this Agreement for the purpose of carrying out collaborative research activities to: (i) validate
genes and proteins as therapeutic targets in the field of [†]; and (ii) identify, optimize and develop modulators of such therapeutic targets, and to enable Merck to develop and commercialize products that are discovered and developed as a
result of such research activities, all upon the terms set out in this Agreement. 
 NOW, THEREFORE, in consideration of the foregoing premises and
the mutual covenants herein contained, and for other good and valuable consideration, the receipt and sufficiency of which are hereby acknowledged, Merck and Xenon hereby agree as follows: 

ARTICLE 1 DEFINITIONS 
 The following
terms, whether used in the singular or plural, shall have the respective meanings set forth below. 
  

	1.1	“Act” means, as applicable, the United States Federal Food, Drug and Cosmetic Act, 21 U.S.C. §§ 301 et seq., as such may be amended from time-to-time. 

 

	1.2	“Affiliate” of a Party means: (1) any corporation or other Person of which fifty percent (50%) or more of the securities or other ownership interests representing the equity, the voting stock
or general partnership interest are owned, controlled or held, directly or indirectly, by such Party; or (2) any corporation or other Person which, directly or indirectly, owns, controls or holds fifty percent (50%) (or the maximum
ownership interest permitted by law) or more of the securities or other ownership interests representing the equity, the voting stock or general partnership interest of such Party; or (3) any corporation or other Person of which fifty percent
(50%) or more of the securities or other ownership interests representing the equity, the voting stock or general partnership interest are owned, controlled or held, directly or indirectly, by a corporation or other Person described in
(1) or (2). 

  
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	1.3	“Agreement” means this Exclusive Collaborative Research and Option Agreement, including all exhibits, attachments and research plans and any amendments made pursuant to the provisions of Section 10.7.

  

	1.4	“Agreement Finalization Period” shall have the meaning given such term in Section 3.10.5. 

  

	1.5	“Approval by Merck” or “Approved by Merck” means, with respect to any Milestone event described herein as requiring approval by Merck, the approval of such event by the internal
committee of Merck that is responsible for the research and development activities that include such event, in each case applying the criteria for approval that is customarily applied by that internal committee of Merck. 

 

	1.6	“Calendar Quarter” means the respective periods of three (3) consecutive calendar months ending on March 31, June 30, September 30 and December 31. 

 

	1.7	“Calendar Year” means each successive period of twelve (12) months commencing on January 1 and ending on December 31. 

 

	1.8	“CFR” means the United States Code of Federal Regulations, as amended and in effect from time-to-time. 

  

	1.9	“Change of Control” shall have the meaning given such term in Section 9.5.2. 

  

	1.10	“Clinical Trial” means a Phase I Clinical Trial, Phase II Clinical Trial or Phase III Clinical Trial, as applicable. 

 

	1.11	“Co-Funded Product” shall have the meaning given such term in Section 3.5. 

  

	1.12	“Combination Product” means a Product which includes one or more active ingredients, other than a Compound, in combination with a Compound. [†]. 

 

	1.13	“Commencement of Lead Optimization” means the Approval by Merck of the Lead Optimization Package for a Lead Compound to enter the lead optimization stage of the discovery process. 

 

	1.14	“Commercialization” or “Commercialize” means activities directed to marketing, advertising, promoting, distributing, importing, exporting and selling. 

 

	1.15	“Commercially Reasonable Efforts” means, with respect to the efforts to be expended by a Party with respect to any objective hereunder, [†]. 

 

	1.16	“Compound” means any chemical entity that modulates a Target [†] and is either: (i) [†]; or (ii) [†]. 

 

	1.17	“Control”, “Controls” or “Controlled by” means, with respect to any item of or right under Xenon Know-How or Xenon Patent Rights or Merck Know-How or Merck Patent
Rights, the possession of (whether by ownership or license, other than pursuant to this Agreement) or the ability of a Party to grant access to, or a license or sublicense of, such item or right as provided for herein without violating the terms of
any agreement or other arrangement with any Third Party existing at the time such Party would be required hereunder to grant the other Party such access or license or sublicense. 

  
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	1.18	“Covering”, “Cover”, or “Covered” means, with respect to a Patent Right, that, but for a license granted to a party under a claim included in such Patent Right, the
practice by such party of an invention claimed in such Patent Right would infringe such claim or in the case of a Patent Right that is a patent application, would infringe a claim in such patent application if it were to issue as a patent.

  

	1.19	“Effective Date” shall have the meaning given such term in the preamble to this Agreement. 

  

	1.20	“Election Notice” shall have the meaning given such term in Section 3.10.3. 

  

	1.21	“EMEA” means the European Medicines Evaluation Agency and the Committee for Proprietary Medicinal Products or any successor agencies thereof or, to the extent the mutual recognition procedure is used
for the Compound or Product in the EU, any governmental authority having the authority to regulate the sale of medicinal or pharmaceutical products in any country in the EU. 

 

	1.22	“EU” means all countries that are member states of the European Union as of the Effective Date of this Agreement. 

  

	1.23	“Exclusive Review Period” shall have the meaning given such term in Section 3.10.3. 

  

	1.24	“FDA” means the United States Food and Drug Administration. 

  

	1.25	“Field” means [†]. 

  

	1.26	“Filing” of an NDA means the acceptance by a Regulatory Authority of an NDA for filing. 

  

	1.27	“First Commercial Sale” means, with respect to any Product, the first sale for end use or consumption of such Product in any country in the Territory; excluding, however, any sale or other distribution
for use in a Clinical Trial. 

  

	1.28	“FTE Rate” shall mean the amount Merck will pay to Xenon over a consecutive twelve (12) month period during the Research Program to support one (1) Xenon FTE dedicated to the Research Program.
The FTE Rate shall be [†] Dollars ($[†]) per FTE. The FTE Rate shall [†]. 

  

	1.29	“Full-Time Equivalent” or “FTE” means the equivalent of a full-time scientist’s work time over a twelve (12) month period (including normal vacations, sick days and holidays).
The portion of an FTE year devoted by a scientist to the Research Program shall be determined by [†]. 

  

	1.30	“GLP” or “Good Laboratory Practice” means the applicable then-current standards for laboratory activities for pharmaceuticals or biologicals, as set forth in the Act and any regulations
or guidance documents promulgated thereunder, as amended from time-to-time, together with any similar standards of good laboratory practice as are required by any Regulatory Authority in the Territory. 

 

	1.31	 “GMP” means, in respect of the manufacture of a Compound or Product, the then current Good Manufacturing Practices as such term is
defined from time-to-time by the FDA, and provided for in 

  
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21 CFR Parts 210-211, or other relevant Regulatory Authority having jurisdiction over the development, manufacture or Commercialization of the Compound or Product in the Territory pursuant to its
regulations, guidelines or otherwise. 

  

	1.32	“Identification of First Lead Compound for a Target” means the Approval by Merck of the first Compound as a Lead Compound, [†]. 

 

	1.33	“IND” means an investigational new drug application, clinical study application, clinical trial exemption, or similar application or submission for approval to conduct human clinical investigations
filed with or submitted to a Regulatory Authority in conformance with the requirements of such Regulatory Authority. 

  

	1.34	“Indication” means [†]. 

  

	1.35	“Information” means any and all information and data, including without limitation all Merck Know-How, all Xenon Know-How, and all other scientific, pre-clinical, clinical, regulatory, manufacturing,
marketing, financial and commercial information or data, whether communicated in writing or orally or by any other method, which is provided by one Party to the other Party in connection with this Agreement. 

 

	1.36	“Initiates”, “Initiated” or “Initiation” means, with respect to a Clinical Trial, the administration of the first dose to the first patient in such Clinical Trial.

  

	1.37	“Insolvency Event” means the filing or institution of bankruptcy, liquidation or receivership proceedings before a court or tribunal with proper jurisdiction under or pursuant to the laws of Canada.

  

	1.38	“Internal Research Purposes” means [†]. 

  

	1.39	“Invention” means any [†]. 

  

	1.40	“JDC” shall have the meaning given such term in Section 3.8. 

  

	1.41	“Joint Know-How” means all information and materials, including, but not limited to, discoveries, improvements, processes, methods, protocols, formulas, data, Inventions, know-how and trade secrets,
patentable or otherwise, that are discovered, developed, created or invented: (1) [†]and (2) [†]. 

  

	1.42	“Joint Patent Rights” means Patent Rights that claim or Cover any Joint Know-How. 

  

	1.43	“Joint Research Program Technology” means Joint Know-How and Joint Patent Rights. 

  

	1.44	“JSC” shall have the meaning given such term in Section 2.7. 

  

	1.45	“Know-How Royalty Rate” shall have the meaning given such term in Section 6.4.1(b). 

  

	1.46	“Know-How Royalty Term” shall have the meaning given such term in Section 6.4.1(b). 

  
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	1.47	“Lead Compound” means a Compound Approved by Merck as a Compound that satisfies the Lead Compound Criteria. 

  

	1.48	“Lead Compound Criteria” means [†]. 

  

	1.49	[†]. 

  

	1.50	“Marketing Authorization” means all approvals from the relevant Regulatory Authority necessary to market and sell a Product in any country in the Territory (including without limitation all applicable
pricing and governmental reimbursement approvals even if not legally required to sell the Product in a country). 

  

	1.51	“Merck” shall have the meaning given such term in the preamble to this Agreement. 

  

	1.52	“Merck Background Technology” means Merck Know-How and Merck Patent Rights. 

  

	1.53	“Merck Know-How” means [†], which: (1) [†], (2) [†], (3) [†], and (4) [†]. 

  

	1.54	“Merck Option” means the option granted to Merck pursuant to Section 3.1. 

  

	1.55	“Merck Option Period” means, subject to earlier termination of this Agreement pursuant to Sections 9.2 or 9.3, the period commencing on the Effective Date and ending on that date that is [†] days
following the expiration of the Research Program Term. 

  

	1.56	“Merck Patent Rights” means Patent Rights which [†] (1) [†], and (2) [†]. 

  

	1.57	“Merck Research Program Technology” means [†] (1) [†] and (2) [†]. 

  

	1.58	“Merck Technology” means Merck Background Technology and Merck Research Program Technology. 

  

	1.59	“Milestone” means each of the milestones listed in Sections 6.3.1, 6.3.2 and 6.3.3. 

  

	1.60	“NDA” means a New Drug Application, Biologics License Application, Community-Wide Marketing Application, Marketing Authorization Application, filing pursuant to Section 510(k) of the Act, or
similar application or submission for Marketing Authorization of a Product filed with a Regulatory Authority to obtain marketing approval for a biological or pharmaceutical product in that country or in that group of countries. 

 

	1.61	“NDA Approval” means approval of an NDA by the FDA, EMEA or other applicable Regulatory Authority in any country in the Territory. 

 

	1.62	“Net Sales” means [†] 

  

	 	1.62.1	[†]; 

  

	 	1.62.2	[†]; 

  
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	 	1.62.3	[†]; and 

  

	 	1.62.4	[†]. 

 With respect to sales of Combination Products, Net Sales shall be [†]. 

 

	1.63	“Option Exercise Fee” shall have the meaning given such term in Section 3.4. 

  

	1.64	“Party” means Merck or Xenon, individually, and “Parties” means Merck and Xenon, collectively. 

  

	1.65	“Person” means any individual, partnership, corporation, trust or any other entity that has legal capacity to own property in its own name or to sue or be sued. 

 

	1.66	“Patent Rights” means any and all patents and patent applications in the Territory (which for the purpose of this Agreement shall be deemed to include certificates of invention and applications for
certificates of invention), including divisionals, continuations, continuations-in-part, reissues, renewals, substitutions, registrations, re-examinations, revalidations, extensions, supplementary protection certificates and other governmental
actions that extend any of the patents and patent applications, and all foreign equivalents of the foregoing. 

  

	1.67	“Patent Royalty Rate” shall have the meaning given such term in Section 6.4.1(a)(iii). 

  

	1.68	“Patent Royalty Term” shall have the meaning given such term in Section 6.4.1(a)(iii). 

  

	1.69	“Phase I Clinical Trial” means a human clinical trial of a Product or Compound in any country in the Territory that would satisfy the requirements of 21 CFR 312.21(a). 

 

	1.70	“Phase II Clinical Trial” means a human clinical trial in any country in the Territory performed in a patient population with the primary goal being to estimate the clinical efficacy effect of a
Compound or Product. 

  

	1.71	“Phase III Clinical Trial” means a human clinical trial in any country in the Territory that would satisfy the requirements of 21 CFR 312.21(c). 

 

	1.72	[†]. 

  

	1.73	[†]. 

  

	1.74	“Product” means [†] (1) [†]; or (2) [†]. 

  

	1.75	“Proposed Transaction” means: 

  

	 	(a)	[†]; or 

  

	 	(b)	[†]. 

  
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	1.76	“Regulatory Authority” means any applicable government regulatory authority involved in granting approvals for the manufacturing, marketing, sale, reimbursement and/or pricing of a Product in the
Territory, including, in the United States, the FDA and any successor governmental authority having substantially the same function. 

  

	1.77	“Related Party” means, in respect of Merck, each of its Affiliates, and each of the respective licensees and sublicensees of Merck and its Affiliates (which term does not include distributors), as
applicable. 

  

	1.78	“Research Plan” means the preclinical research plan attached hereto as Exhibit 1.78. The Research Plan provides, among other things, that [†]. In the event of a conflict between the terms of
this Agreement and the Research Plan, the terms of this Agreement shall govern. 

  

	1.79	“Research Program” means the research activities undertaken by the Parties as set forth in Article 2 and includes, without limitation, the research activities described in the Research Plan and in
each Target Validation Plan and Target Drug Discovery Plan. 

  

	1.80	“Research Program Technology” means Xenon Research Program Technology, Merck Research Program Technology, and Joint Research Program Technology, collectively. 

 

	1.81	“Research Program Term” means the duration of the Research Program as described in Section 2.2. 

  

	1.82	[†]. 

  

	1.83	“Target” means any human protein or gene: (1) [†] or (2) [†]. 

  

	1.84	“Target Candidate” shall have the meaning given such term in Section 2.5.1. 

  

	1.85	“Target Drug Discovery Plan” means the written research plan, [†]. In the event of a conflict between the terms of this Agreement and a Target Drug Discovery Plan, the terms of this Agreement shall
govern. 

  

	1.86	“Target Product Profile shall have the meaning given such term in Section 2.5.3. 

  

	1.87	“Target Termination Notice” shall have the meaning given such term in Section 3.9.1. 

  

	1.88	“Target Validation Plan” means the written research plan, [†]. In the event of a conflict between the terms of this Agreement and a Target Validation Plan, the terms of this Agreement shall govern.

  

	1.89	“Term Sheet Finalization Period” shall have the meaning given such term in Section 3.10.4. 

  

	1.90	“Territory” means all of the countries in the world, and their territories and possessions. 

  

	1.91	“Third Party” means any Person other than Merck and its Related Parties, and Xenon and its Affiliates. 

  

	1.92	“Transferred Product” shall have the meaning given such term in Section 3.9.1. 

  
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	1.93	“Valid Patent Claim” means [†]. 

  

	1.94	“Xenon” shall have the meaning given such term in the preamble to this Agreement. 

  

	1.95	“Xenon Background Technology” means Xenon Know-How and Xenon Patent Rights. 

  

	1.96	“Xenon Extreme Genetics” means [†]. 

  

	1.97	“Xenon Know-How” means [†]. 

  

	1.98	“Xenon Option” shall have the meaning given such term in Section 3.5. 

  

	1.99	“Xenon Patent Prosecution” shall have the meaning given such term in Section 8.1.1(c). 

  

	1.100	“Xenon Patent Rights” means [†]. 

  

	1.101	“Xenon Research Program Technology” means [†]. 

  

	1.102	“Xenon Target Validation Criteria” means the written criteria for validation of a Target Candidate by Xenon [†]. Such Xenon Target Validation Criteria shall be attached hereto as Exhibit
1.102 and become part of this Agreement. 

  

	1.103	“Xenon Technology” means Xenon Background Technology and Xenon Research Program Technology. 

  

	1.104	“Xenon-Validated Target” means [†]. 

 ARTICLE 2 RESEARCH PROGRAM

  

	2.1	General. 

 Xenon and Merck shall conduct research activities pursuant to the provisions
of this Agreement and the Research Program to validate Target Candidates in the Field and to identify, optimize and develop modulators of Targets. It is intended that the Research Program will be conducted as a unified, collaborative effort with the
Parties’ activities carried out primarily at each Party’s respective facilities. The Research Program shall be comprised of the Research Plan, a Target Validation Plan, approved by the JSC pursuant to Section 2.5.2, for each Target,
and/or a Target Drug Discovery Plan, approved by the JSC pursuant to Section 2.5.2, for each Target. The Parties may amend the Research Plan by mutual written agreement. In the event of a conflict between the terms of this Agreement and the
Research Plan, the terms of this Agreement shall govern. 
  

	2.2	Research Program Term. 

 Except as otherwise provided herein, the term of the Research
Program shall commence on the Effective Date and continue for an initial period of [†] years. The Parties may extend the term of the 

  
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Research Program on a year-by-year basis, by mutual written agreement of authorized representatives of each Party, initially at least [†] days prior to the [†] anniversary of the
Effective Date and, thereafter, at least [†] days prior to each subsequent anniversary of the Effective Date, and shall, in such case, amend any exhibits as necessary. 
  

	2.3	Conduct of Research. 

  

	 	2.3.1	Each Party shall use its Commercially Reasonable Efforts to perform its obligations pursuant to the Research Program and, without limitation, each Party shall perform the work set out in the Research Program by using
its good faith efforts to allocate sufficient time, effort, equipment and facilities to the Research Program and to use personnel with sufficient skills and experience as are required to accomplish the objectives of the Research Program.

  

	 	2.3.2	Each Party shall conduct the Research Program in compliance with all applicable laws, rules and regulations, including, without limitation, Good Laboratory Practice. In addition, if animals are used in research
hereunder, the Parties shall comply with the United States Animal Welfare Act or any other applicable local, state, national and international laws and regulations relating to the care and use of laboratory animals. Each Party encourages the other
Party to use the highest standards, such as those set forth in the Guide for the Care and Use of Laboratory Animals (NRC, 1996), for the humane handling, care and treatment of such research animals. Any animals which are used in the course of the
Research Program, or products derived from those animals, such as eggs or milk, shall not be used for food purposes, nor shall these animals be used for commercial breeding purposes. Each Party shall notify the other Party in writing of any
deviations from applicable regulatory or legal requirements. 

  

	 	2.3.3	Each Party hereby agrees that it shall not employ or otherwise use in any capacity, the services of any person debarred under United States law, including but not limited to Section 21 USC 335a, in performing any
portion of the Research Program. 

  

	 	2.3.4	Merck shall be entitled to utilize the services of its Affiliates and Third Parties to perform its Research Program activities. Xenon shall be entitled to utilize the services of Third Parties to perform its Research
Program activities only with the prior written consent of the JSC or as specifically set forth in the applicable Target Validation Plan or Target Drug Discovery Plan. [†]. 

 

	2.4	Principal Scientists. 

 The Parties shall each designate a principal scientist for the
Research Program, and/or a principal scientist for a Target Validation Plan and Target Drug Discovery Plan, and all work assignments to be performed by Xenon and Merck shall be carried out under the direction and supervision of the respective
principal scientists selected by the Parties. Each Party shall notify the other Party as soon as practicable upon the changing of a principal scientist. 

  
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	2.5	Research Program. 

  

	 	2.5.1	Either Party may submit a human gene or protein (a “Target Candidate”) in writing to the JSC for consideration by the JSC as the subject of a Target Validation Plan or Target Drug Discovery Plan, and
shall include a proposed Target Product Profile. Each of the Parties shall disclose to the JSC such Information within the Party’s Control relating to the Target Candidate that is necessary (as determined by the disclosing Party) to evaluate
the Target Candidate, and shall cooperate with the JSC to review and analyze such Information and, if the Target Candidate is approved by the JSC for the purpose of a Target Validation Plan or if a Target Candidate is approved for a Target Drug
Discovery Plan, jointly assist the JSC to develop the plan. All such Information disclosed by a Party to the JSC shall be considered confidential Information and shall be subject to the provisions of Article 5. 

 

	 	2.5.2	A Target Validation Plan or Target Drug Discovery Plan shall be based upon the Research Plan and may only be approved by the JSC [†]. 

 

	 	2.5.3	The Parties agree that each Target Drug Discovery Plan will include [†] (collectively, the “Target Product Profile”). 

 

	 	2.5.4	As part of its obligations under the Research Program, [†]. 

  

	 	2.5.5	The JSC may elect to develop any number of Target Drug Discovery Plans during the Research Program Term; provided, however, that commencing upon the [†] anniversary [†] Merck and Xenon shall
[†]. 

  

	 	2.5.6	If a Party (the “Rejecting Party”) rejects a Target Candidate proposed by the other Party (the “Proposing Party”) pursuant to Section 2.5.1 [†]. 

 

	2.6	Ownership of Technology. 

  

	 	2.6.1	Subject to the provisions of Section 2.6.2 and Section 3.1, the entire right, title and interest in: 

  

	 	(i)	Xenon Technology shall be owned solely by Xenon; 

  

	 	(ii)	Merck Technology shall be owned solely by Merck; and 

  

	 	(iii)	Joint Research Program Technology shall be owned [†]; 

 and, except as expressly set out
in this Agreement, neither Party acquires any right, license or other interest in such technology owned by the other Party. 
  

	 	2.6.2	Notwithstanding the provisions of Section 2.6.1 and subject to Section 3.1, with regard to Compounds which: (1) [†]; (2) [†]; (3) [†] (4) [†] Xenon shall, without
further act by either Party, have a perpetual (which for greater certainty, shall survive the expiration or termination of this Agreement), royalty-free, exclusive (even as to Merck) license in the Territory, under [†] to [†] and Xenon
shall have no financial obligation, or other obligation, to Merck or to any of its Affiliates, arising from the exercise of such right. 

  
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	 	2.6.3	[†]or more frequently if requested by Merck, Xenon shall promptly disclose in writing, to an in-house patent attorney of Merck, designated by Merck, the development, making, conception or reduction to practice of
Xenon Research Program Technology and Joint Research Program Technology including, but not limited to, Inventions discovered or created and resulting from the Research Program. In addition, Xenon shall disclose such Xenon Research Program Technology
and Joint Research Program Technology to Merck in writing at the next succeeding JSC meeting or more frequently if requested by Merck. 

  

	2.7	Joint Steering Committee. 

 Within [†] days after the Effective Date, the Parties
shall establish a Joint Steering Committee (“JSC”) to oversee the Research Program. The JSC shall be comprised [†] but in no event shall exceed [†] representatives of each Party. Each Party may change its representatives
to the JSC from time-to-time in its sole discretion, effective upon notice to the other Party of such change. Each of the representatives shall have appropriate technical credentials, experience and knowledge, familiarity with the Research Program
and appropriate decision-making authority. Additional representatives or consultants may from time-to-time, by mutual consent of the Parties, be invited to attend JSC meetings, subject to such representative’s or consultant’s written
agreement to comply with the requirements of Section 5.1. Each Party shall bear its own expenses related to the attendance of such meetings by its representatives or consultants. 

 

	2.8	Decision-Making. 

 Decisions of the JSC shall be made unanimously by the members of the
JSC. In the event that the JSC cannot or does not, after good faith efforts, reach agreement on an issue, then [†]. 
  

	2.9	JSC Responsibilities. 

 The JSC shall oversee the Research Program and its
responsibilities shall include, but not be limited to, the following functions: 
  

	 	(a)	[†]; 

  

	 	(b)	Defining minimum quality standards of sequence criteria for sequence data prior to commencement of sequencing of Target Candidates by Xenon; 

 

	 	(c)	Acceptance of sequencing data for Target Candidates; 

  

	 	(d)	Developing and approving each Target Validation Plan and Target Drug Discovery Plan pursuant to the provisions of Section 2.5.2, including all Xenon Target Validation Criteria; 

 

	 	(e)	Approving a Target Candidate proposed by a Party as the subject of a Target Validation Plan or Target Drug Discovery Plan; 

  
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	 	(f)	Review relevant data, consider and advise on any technical issues that arise, consider issues of priority, and review and advise on any budget matters relating to the Research Program or to any individual Target
Validation Plan or Target Drug Discovery Plan; 

  

	 	(g)	Reviewing progress of the Research Program, including Compound identification; 

  

	 	(h)	Recommending modifications to the Research Program; 

  

	 	(i)	[†]; 

  

	 	(j)	Making recommendations to Merck as to whether to terminate a Target Validation Plan or Target Drug Discovery Plan; and 

  

	 	(k)	Performing such other tasks as are specifically set forth in this Agreement. 

  

	2.10	Meetings. 

  

	 	2.10.1	The JSC shall be chaired by a representative of Merck. 

  

	 	2.10.2	The JSC shall meet [†] in accordance with a schedule established by mutual written agreement of the Parties. 

  

	 	2.10.3	The JSC shall hold an initial meeting within [†] days following the Effective Date and shall thereafter meet in accordance with a schedule established by mutual written agreement of the Parties, but no less
frequently than once per Calendar Quarter. 

  

	 	2.10.4	The JSC may meet by means of teleconference, videoconference or other similar communications equipment, but in any event shall meet in person at least [†] with the location for in-person meetings alternating
between Xenon and Merck facilities. 

  

	2.11	Records. 

 Each Party shall maintain records, in sufficient detail and in good scientific
manner appropriate for patent and regulatory purposes, which shall fully and properly reflect all work done and results achieved in the performance of the Research Program. 
  

	2.12	Copies and Inspection of Records. 

 Merck shall have the right, during normal business
hours and upon reasonable notice, to inspect and copy all such records of Xenon referred to in Section 2.11. Xenon shall maintain such records and the information disclosed therein in confidence in accordance with Section 5.1. Merck shall
have the right to arrange for its employee(s) and/or consultant(s) involved in the activities contemplated hereunder to visit the offices and laboratories of Xenon and any of its Third Party contractors as permitted under Section 2.3.4 during
normal business hours and upon reasonable notice, and to discuss the Research Program work and its results in detail with the technical personnel and consultant(s) of Xenon. Upon request, Xenon shall provide to Merck copies of the records described
in Section 2.11. 

  
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	2.13	Quarterly Reports. 

 Within [†] days following the end of each Calendar Quarter
during the Research Program Term, Xenon shall provide to the JSC a written progress report which shall describe, in reasonable detail, the work performed to date on the Research Program, evaluate the work performed in relation to the goals of the
Research Program and the individual Target Validation Plans and Target Drug Discovery Plans, and provide such other information required by the Research Program or reasonably requested by the JSC or Merck. 

 

	2.14	Annual Report. 

 Until the expiration of the Research Program Term, on the annual
anniversary of the Effective Date (and at the expiration of the Research Program Term), Xenon shall prepare, on a Target-by-Target basis, and provide to Merck: (1) an annual report, written in reasonable detail, of material activities performed
under the Research Program the previous year, and (2) plans, written in reasonable detail, for activities to be undertaken under the Research Program in the subsequent year. 

ARTICLE 3 MERCK OPTION 
  

	3.1	Merck Option. 

 With regard to each Target, Xenon hereby grants to Merck an option, on a
Target-by-Target basis, to acquire from Xenon: 
  

	 	(a)	an exclusive (even as to Xenon), worldwide, royalty-bearing license under [†]; and 

  

	 	(b)	[†]; 

 to research, develop, make, have made, use, offer to sell, sell and/or import
Compounds and Products that [†] the Target[†], subject to the terms of this Agreement. 
  

	3.2	Exercise of Merck Option. 

 Merck may exercise the Merck Option at any time, and from
time-to-time, during the Merck Option Period by providing notice in writing to Xenon that specifies the Target, and upon receipt by Xenon of such notice Merck shall, without further act by either of the Parties, be granted the licenses set out in
Section 3.1. 
  

	3.3	Transfer by Xenon. 

 As soon as practicable following Merck’s exercise of the Merck
Option for a Target, but in no event later than [†] days thereafter, [†] shall, within a reasonable period of time after exercise of the Merck Option, but in any event not longer than [†] days thereafter, transfer all Information on
the Target to Merck so that Merck may proceed with research, development and Commercialization relating to the Target and to Compounds and Products relating to the Target including, but not limited to, [†] pursuant to the terms of this
Agreement. 

  
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	3.4	Option Exercise Fee. 

 If Merck elects to exercise the Merck Option, then Merck shall pay
to Xenon an option exercise fee of Two Million Dollars ($2,000,000) (the “Option Exercise Fee”) for each of the first [†] optioned Targets and there shall be no Option Exercise Fee for Targets optioned by Merck after the first
[†] Targets. The Parties agree that: (1) there is no limit on the number of Targets which Merck may option, and (2) the total of all Option Exercise Fees paid by Merck to Xenon pursuant to this Agreement shall not, under any
circumstances, exceed [†]. Merck shall pay the Option Exercise Fee to Xenon within thirty (30) days of Merck’s exercise of the Merck Option for each of the first [†] optioned Targets. 

 

	3.5	Xenon Option. 

 If Merck exercises the Merck Option and develops Compounds or Products,
then Xenon shall have an option, subject to Section 3.6, with regard to Compounds or Products that utilise or are derived from Xenon Research Program Technology or Joint Research Program Technology, on a Compound-by-Compound and
Product-by-Product basis, to co-fund fifty percent (50%) of the combined development costs of (a) and (b) below for each Compound or Product for which it exercises such option (the “Xenon Option” and each Product, a
“Co-Funded Product”): 
  

	 	(a)	all Phase I Clinical Trials, until completion of the first Phase II Clinical Trial; and 

  

	 	(b)	the cost of the first Phase II Clinical Trial. 

 Merck shall provide to Xenon a copy of each
such Compound’s or Product’s first IND (or other equivalent regulatory filing) and summary clinical development plans, including estimated costs through the end of the first Phase II Clinical Trial. [†]Xenon shall have [†] days
after receipt of such costs in which to inform Merck in writing if Xenon exercises the Xenon Option. 
 If Xenon exercises the Xenon Option,
then Xenon shall pay to Merck, [†], fifty percent (50%) of [†] with payment due [†] days after the end of [†]. Each [†], the Parties shall reconcile any variance between actual costs and estimated costs. If the actual
development costs exceed the estimated development costs by [†] percent ([†]%) or less, then Xenon shall remit to Merck fifty percent (50%) of such underpayment within forty-five (45) days of notice of such underpayment. If the
actual development costs exceed the estimated development costs by more than [†]percent ([†]%), then Merck shall deduct Xenon’s share of the underpayment in excess of [†]percent ([†]%) from future royalties payable to Xenon.

  

	3.6	Merck Research Program Technology. 

 The Parties acknowledge and agree that Xenon shall
not have an option to co-fund Compounds or Products that do not utilise or are not derived from Xenon Research Program Technology or Joint Research Program Technology, but Xenon shall be eligible to receive royalties and milestones as provided in
Sections 6.3.2 and 6.4.1(a)(i) in respect of such Compounds and Products. 

  
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	3.7	Annual Report. 

 Following exercise of the Merck Option, Merck shall prepare, on a
Target-by-Target and Product-by-Product and Compound-by-Compound basis, a summary annual report for Xenon which will include a description, in reasonable detail, of material research, pre-clinical, clinical and Commercialization activities
undertaken by Merck during the prior year and planned for the next year. 
  

	3.8	Joint Development Committee. 

 Following exercise of the Merck Option, if Xenon exercised
the Xenon Option, the Parties shall establish a joint development committee (“JDC”) for each Co-Funded Product under development, which committee shall be constituted and shall carry out its duties as follows: 

 

	 	3.8.1	The principal function of the JDC shall be to keep Xenon informed, in reasonable detail, of development activities relating to each Co-Funded Product until NDA Approval of such Co-Funded Product. 

 

	 	3.8.2	The JDC shall be comprised of [†]. Each Party may change its representatives to the JDC from time-to-time in its sole discretion, effective upon notice to the other Party of such change. Each of the representatives
shall have appropriate technical credentials, experience and knowledge, familiarity with the development of the Compound or Product and appropriate decision-making authority. Additional representatives or consultants may from time-to-time, by mutual
consent of the Parties, be invited to attend JDC meetings, subject to such representative’s or consultant’s written agreement to comply with the requirements of Section 5.1. 

 

	 	3.8.3	The JDC shall meet [†], with the first such meeting at the facilities of Merck and thereafter to alternate between the facilities of the Parties. 

 

	 	3.8.4	A representative of Merck shall chair each meeting. 

  

	3.9	Co-Funded Products – Reversion Rights and Credited Payments. 

  

	 	3.9.1	Reversion Rights. If, following the expiration of the Research Program Term and if Merck has exercised the Merck Option, [†]. 

 

	 	3.9.2	Credited Payments. If the Research Program Term expires and Merck ceases clinical development activities, for [†], on a Co-Funded Product [†] then Merck shall credit Xenon with an amount equal to the
co-funding amounts received by Merck from Xenon for such dropped Co-Funded Product. Merck shall apply such credited amount to [†]. 

  

	3.10	[†]. 

  

	 	3.10.1	[†]. 

  

	 	3.10.2	[†]. 

  
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	 	3.10.3	[†]. 

  

	 	3.10.4	[†]. 

  

	 	3.10.5	[†]. 

  

	 	3.10.6	[†]. 

  

	 	3.10.7	[†]. 

  

	3.11	[†]. 

 [†]: 

 

	 	(a)	[†] 

  

	 	(b)	[†] 

 ARTICLE 4 LICENSE; EXCHANGE OF INFORMATION; DEVELOPMENT AND
COMMERCIALIZATION 
  

	4.1	License Grants. 

  

	 	4.1.1	License Grants from Xenon to Merck. 

  

	 	(a)	Xenon hereby grants to Merck a non-exclusive license in the Field and in the Territory under Xenon Background Technology solely to perform research pursuant to the Research Plan and/or the applicable Target Validation
Plan or Target Drug Discovery Plan during the Research Program Term. 

  

	 	(b)	Xenon hereby grants to Merck a co-exclusive license in the Field and in the Territory under Xenon Research Program Technology and Xenon’s interest in Joint Research Program Technology solely to perform research
pursuant to the Research Plan and/or the applicable Target Validation Plan or Target Drug Discovery Plan during the Research Program Term. 

  

	 	(c)	Upon Merck’s exercise of the Merck Option, Xenon hereby grants to Merck the licenses provided for in Section 3.1. 

  

	 	4.1.2	License Grants from Merck to Xenon. 

  

	 	(a)	Merck hereby grants to Xenon a non-exclusive license in the Field and in the Territory under Merck Background Technology solely to perform research pursuant to the Research Plan and/or the applicable Target Validation
Plan or Target Drug Discovery Plan during the Research Program Term. 

  
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	 	(b)	Merck hereby grants to Xenon a co-exclusive license in the Field and in the Territory under Merck Research Program Technology and Merck’s interest in Joint Research Program Technology solely to perform research
pursuant to the Research Plan and/or the applicable Target Validation Plan or Target Drug Discovery Plan during the Research Program Term. 

  

	 	4.1.3	Joint Research Program Technology. Subject to the Merck Option, upon the expiration of the Research Program Term, [†]. 

 

	4.2	Internal Research Purposes. 

 During the term of this Agreement, [†] shall be
entitled to use [†] for Internal Research Purposes. 
  

	4.3	Exchange of Information. 

 During the Research Program Term, Merck shall provide to
Xenon, in English and in writing or in an electronic format, the Merck Background Technology for Xenon to undertake its activities described in the Research Program. During the term of this Agreement, Xenon shall provide to Merck, in English and in
writing or in an electronic format, the Xenon Background Technology for Merck to undertake its activities described in the Research Program and for Merck to exercise its rights upon exercise of the Merck Option. 

 

	4.4	No Implied Licenses. 

 Except as specifically set forth in this Agreement, neither Party
shall acquire any license or other intellectual property interest, by implication or otherwise, in any Information disclosed to it under this Agreement or under any patents or patent applications Controlled by the other Party or its Affiliates. 

 

	4.5	Development and Commercialization. 

 Upon Merck’s exercise of the Merck Option in
respect of a Target, Merck shall use Commercially Reasonable Efforts in the development and Commercialization of Compound(s) and Product(s) that modulate such optioned Target. 

 

	4.6	Excused Performance. 

 [†]. 

ARTICLE 5 CONFIDENTIALITY AND PUBLICATION 
  

	5.1	Nondisclosure Obligation. 

 All Information disclosed by one Party to the other Party
hereunder shall be maintained in confidence by the receiving Party and shall not be disclosed to any Third Party or used for any purpose except as set forth herein without the prior written consent of the disclosing Party, except to the extent that
such Information: 
  

	 	5.1.1	is known by the receiving Party at the time of its receipt, and not through a prior disclosure by the disclosing Party, as documented by the receiving Party’s business records; 

  
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	 	5.1.2	is in the public domain by use and/or publication before its receipt from the disclosing Party, or thereafter enters the public domain through no fault of the receiving Party; 

 

	 	5.1.3	is subsequently disclosed to the receiving Party by a Third Party who may lawfully do so and is not under an obligation of confidentiality to the other Party; 

 

	 	5.1.4	is developed by the receiving Party independently of Information received from the disclosing Party, as documented by the receiving Party’s business records; 

 

	 	5.1.5	is disclosed to governmental or other regulatory agencies in order to obtain patents or to gain or maintain approval to conduct clinical trials or to market Compounds or Products, but such disclosure may be only to the
extent reasonably necessary to obtain patents or authorizations; 

  

	 	5.1.6	is deemed necessary by either Party to be disclosed to Related Parties, agent(s), or other Third Parties (who are approved pursuant to Section 2.3.4) in connection with the performance of its obligations pursuant
to this Agreement, on the condition that such Third Parties agree to be bound by confidentiality and non-use obligations that substantially are no less stringent than those confidentiality and non-use provisions contained in this Agreement;
provided, however, that the term of confidentiality for such Third Parties shall be no less than ten (10) years; or 

  

	 	5.1.7	is deemed necessary by counsel to the receiving Party to be disclosed to such Party’s attorneys, independent accountants or financial advisors for the sole purpose of enabling such attorneys, independent
accountants or financial advisors to provide advice to the receiving Party, on the condition that such attorneys, independent accountants and financial advisors agree to be bound by the confidentiality and non-use obligations contained in this
Agreement; provided, however, that the term of confidentiality for such attorneys, independent accountants and financial advisors shall be no less than ten (10) years. 

Any combination of features or disclosures shall not be deemed to fall within the foregoing exclusions merely because individual features are published or
available to the general public or in the rightful possession of the receiving Party unless the combination itself and principle of operation are published or available to the general public or in the rightful possession of the receiving Party. 

If a Party is required by judicial or administrative process to disclose Information that is subject to the non-disclosure provisions of this Section 5.1
or Section 5.2, such Party shall promptly inform the other Party of the disclosure that is being sought in order to provide the other Party an opportunity to challenge or limit the disclosure obligations. Information that is disclosed by
judicial or administrative process shall remain otherwise subject to the confidentiality and non-use provisions of this Section 5.1 and Section 5.2, and the Party disclosing Information pursuant to law or court order shall take all steps
reasonably necessary, including without limitation obtaining an order of confidentiality, to ensure the continued confidential treatment of such Information. 

  
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	5.2	Publication. 

  

	 	5.2.1	Subject to the provisions of Section 5.1 and 5.2.3, Merck shall have the right, as of the Effective Date to publish: (1) Merck Research Program Technology; (2) any Xenon Research Program Technology under
which Merck acquires a license pursuant to the exercise of the Merck Option; and (3) [†]. 

  

	 	5.2.2	Following the expiration or termination of the Research Program Term, but not before, and subject to the provisions of Section 5.1 and 5.2.3, Xenon shall have the right to publish: (1) Xenon Research Program
Technology (excluding any Xenon Research Program Technology under which Merck acquires a license pursuant to the exercise of the Merck Option); and (2) [†]. 

 

	 	5.2.3	Merck and Xenon each acknowledge the other Party’s interest in publishing the results of its research in order to obtain recognition within the scientific community and to advance the state of scientific knowledge.
Each Party also recognizes the mutual interest in obtaining valid patent protection and in protecting business interests and trade secret information. Consequently, except for disclosures permitted pursuant to Section 5.1, if either Party
decides that public presentation or publication of Research Program Technology is desirable pursuant to Sections 5.2.1 or 5.2.2, either Party, its Affiliates, its employee(s) or consultant(s) wishing to make a publication or presentation shall
deliver to the other Party a copy of the proposed written publication or an outline of an oral disclosure at least sixty (60) days prior to submission for publication or presentation. The reviewing Party shall have the right (a) to propose
modifications to the publication or presentation for patent reasons, trade secret reasons or business reasons, or (b) to request a reasonable delay in publication or presentation in order to protect patentable information. If the reviewing
Party requests a delay, the publishing Party shall delay submission or presentation for a period of ninety (90) days to enable patent applications protecting each Party’s rights in such information to be filed in accordance with
Article 8. Upon expiration of such ninety (90) days, the publishing Party shall be free to proceed with the publication or presentation. If the reviewing Party requests modifications to the publication or presentation, the publishing Party
shall edit such publication to prevent disclosure of trade secret or proprietary business information prior to submission of the publication or presentation. 

  

	5.3	Publicity/Use of Names. 

 Upon execution of this Agreement by the Parties, each Party may
issue a press release as set out in Exhibit 5.3. In addition, each Party may, in its public and confidential disclosures to Third Parties and after execution of this Agreement, refer to the name of the other Party and the information set
out in Exhibit 5.3. Otherwise, except as permitted by Section 5.1, no disclosure of the existence, or the terms, of this Agreement may be made by either Party, and no Party shall use the name, trademark, trade name or logo of the
other Party, its Affiliates or their respective employee(s) in any publicity, promotion, news release or disclosure relating to this Agreement or its subject matter, without the prior express written permission of the other Party, except as may be
required by law. 

  
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 ARTICLE 6 PAYMENTS; ROYALTIES AND REPORTS 

 

	6.1	Research Program Costs. 

 Other than as provided in Section 6.2, each Party shall be
responsible for the costs it incurs in carrying out its responsibilities under the Research Program. 
  

	6.2	FTE Funding. 

 For the duration of the initial [†] year Research Program Term, Merck
shall fund, on a [†] basis (pro-rated for any period of less than [†] months at the beginning or end of the Research Program Term), in advance, a minimum of [†] FTEs of Xenon at the FTE Rate. The number of funded FTEs may be increased
during the initial [†] year Research Program Term by mutual written consent of both Parties. For extensions to the Research Program Term beyond the initial [†] year term, the FTEs may be increased or decreased by mutual written consent of
both Parties. After the initial [†] year Research Program Term, the FTE cost will be adjusted in accordance with the local consumer price index. 
  

	6.3	Milestone Payments. 

 Subject to the terms and conditions of this Agreement, Merck shall
pay to Xenon the following milestone payments: 
  

	 	6.3.1	[†] Milestones: 

  

	 	(a)	Delivery of [†] pursuant to Section 2.9(a): 

 Two Million Five Hundred Thousand
Dollars ($2,500,000) 
  

	 	(b)	Delivery of [†] pursuant to Section 2.9(a): 

 Two Million Five Hundred Thousand
Dollars ($2,500,000) 
  

	 	6.3.2	Compounds and Products for which Xenon has Not Exercised the Xenon Option (i.e., Compounds and Products Which are Not Co-Funded Products): 

 

	 	(a)	Milestone #1: Identification of first Lead Compound for a Target: 

 One Million Dollars
($1,000,000) 
  

	 	(b)	Milestone #2: [†]: 

 [†] Dollars ($[†]) 

 

	 	(c)	Milestone #3: [†]: 

 [†] Dollars ($[†]) 

  
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	 	(d)	Milestone #4: [†]: 

 [†] Dollars ($[†]) 

 

	 	(e)	Milestone #5: [†]: 

 [†] Dollars ($[†]) 

 

	 	(f)	Milestone #6: [†]: 

 [†] Dollars ($[†]) 

 

	 	(g)	Milestone #7: [†]: 

 [†] Dollars ($[†]) 

 

	 	(h)	Milestone #8: [†]: 

 [†] Dollars ($[†]) 

 

	 	(i)	Milestone #9: [†]: 

 [†] Dollars ($[†]) 

 

	 	6.3.3	Compounds and Products for which Xenon has Exercised the Xenon Option (i.e., Co-Funded Products): 

  

	 	(a)	Milestone #1: [†]: 

 [†] Dollars ($[†]) 

 

	 	(b)	Milestone #2: [†]: 

 [†] Dollars ($[†]) 

 

	 	(c)	Milestone #3: [†]: 

 [†] Dollars ($[†]) 

 

	 	(d)	Milestone #4: [†]: 

 [†] Dollars ($[†]) 

 

	 	(e)	Milestone #5: [†]: 

 [†] Dollars ($[†]) 

  
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	 	(f)	Milestone #6: [†]: 

 [†] Dollars ($[†]) 

 

	 	(g)	Milestone #7: [†]: 

 [†] Dollars ($[†]) 

 

	 	(h)	Milestone #8: [†]: 

 [†] Dollars ($[†]) 

 

	 	(i)	Milestone #9: [†]: 

 [†] Dollars ($[†]) 

 

	 	6.3.4	General Milestone Provisions. 

  

	 	(a)	Merck shall notify Xenon in writing within [†] days following the achievement of each Milestone, and shall make the corresponding Milestone payment within [†] days after the achievement of such Milestone.

  

	 	(b)	[†] Milestones as provided for in Section 6.3.1 are payable [†]. 

  

	 	(c)	Milestones as provided for under Section 6.3.2 and Section 6.3.3 are payable [†]. 

  

	 	(d)	Each milestone payment under Section 6.3.2 and Section 6.3.3 is payable [†]. 

  

	 	(e)	[†]. 

  

	 	(f)	[†]. 

  

	6.4	Royalties. 

  

	 	6.4.1	Royalties Payable By Merck. Subject to the terms and conditions of this Agreement, Merck shall pay royalties to Xenon, [†]. 

 

	 	(a)	Patent Royalties. 

  

	 	(i)	Royalty Tiers for Products which are Not Co-Funded Products. Merck shall pay Xenon royalties in an amount equal to the following percentage of Net Sales by Merck and its Related Parties[†]:

  

	 	(A)	[†] percent ([†]%) of Net Sales [†] ($[†]); 

  

	 	(B)	[†] percent ([†]%) of Net Sales [†]; 

  
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	 	(C)	[†] percent ([†]%) of Net Sales [†]; and 

  

	 	(D)	[†] percent ([†]%) of Net Sales [†]. 

  

	 	(ii)	Royalty Tiers for Co-Funded Products. Merck shall pay Xenon royalties in an amount equal to the following percentage of Net Sales of Co-Funded Products by Merck or its Related Parties[†]: 

 

	 	(A)	[†] percent ([†]%) of Net Sales [†] 

  

	 	(B)	[†] percent ([†]%) of Net Sales [†] 

  

	 	(C)	[†] percent ([†]%) of Net Sales [†]and 

  

	 	(D)	[†] percent ([†]%) of Net Sales [†] 

  

	 	(iii)	Royalties on each Product at the rates set forth in Section 6.4.1(a)(i) and (ii) above (the “Patent Royalty Rate”) shall continue on a country-by-country basis until the expiration of the
last-to-expire Valid Patent Claim Covering the Product (the “Patent Royalty Term”). 

  

	 	(b)	Know-How Royalty. Notwithstanding the provisions of Section 6.4.1(a), in countries where the sale of a Product by Merck or its Related Parties would not infringe a Valid Patent Claim, Merck shall pay royalty
rates that shall be set at fifty percent (50%) of the applicable Patent Royalty Rate determined according to Section 6.4.1(a)(i) or (ii), as applicable (tier based on worldwide annual net sales) (the “Know-How Royalty
Rate”). Such royalties shall be calculated after first calculating royalties under Section 6.4.1(a)(i) or (ii), as applicable. Such Know-How Royalty shall be payable for ten (10) years following the First Commercial Sale of such
Product (the “Know-How Royalty Term”). 

  

	 	(c)	Calculation of Royalties. [†]. 

  

	 	(d)	General Royalty Provisions. 

  

	 	(i)	All royalties are subject to the following conditions: 

  

	 	(A)	[†] shall be due with respect to [†]; 

  

	 	(B)	[†]shall be due upon the sale or other transfer [†]; 

  

	 	(C)	no royalties shall accrue on the sale or other disposition of a Compound or Product by Merck or its Related Parties for use in a Clinical Trial; and 

 

	 	(D)	[†]. 

  
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	 	6.4.2	Change in Sales Practices. The Parties acknowledge that during the term of this Agreement, [†]. 

  

	 	6.4.3	Compulsory Licenses. If a compulsory license is granted to a Third Party[†]. 

  

	 	6.4.4	Third Party Licenses. In the event that one or more [†]. 

  

	 	6.4.5	Non-Monetary Consideration. In the event that Merck or any of its Related Parties receives any non-monetary consideration in connection with the sale or other disposition for value of Compounds or Products,
including barter or counter-trade (but excluding transactions described in Section 6.4.1(d)(i)(D)), the Net Sales of such Compound or Product shall be calculated [†]. 

 

	6.5	Reports; Payment of Royalty. 

 During the term of this Agreement following the First
Commercial Sale of a Product, Merck shall furnish to Xenon a [†]written report for [†]showing the Net Sales of all Products subject to royalty payments sold by Merck and its Related Parties in the Territory during the reporting period and
the royalties payable under this Agreement. Reports shall be due on the [†]day following [†]. Royalties shown to have accrued by each royalty report shall be due and payable on the date such royalty report is due. Merck shall keep complete
and accurate records in sufficient detail to enable the royalties payable hereunder to be determined. 
  

	6.6	Audits. 

  

	 	6.6.1	Upon the written request of Xenon and not more than once [†], Merck shall permit an independent certified public accounting firm of nationally recognized standing selected by Xenon and reasonably acceptable to
Merck, at Xenon’s expense, to have access during normal business hours to such of the records of Merck as may be reasonably necessary to verify the accuracy of the royalty reports hereunder for any [†] ending not more than [†] prior
to the date of such request. The accounting firm shall disclose to Xenon only whether the royalty reports are correct or incorrect and the amount of any discrepancy. No other information shall be provided to Xenon. 

 

	 	6.6.2	If such accounting firm correctly identifies a discrepancy made during such period, the appropriate Party shall pay the other Party the amount of the discrepancy within [†] days of the date Xenon delivers to Merck
such accounting firm’s written report so correctly concluding, or as otherwise agreed upon by the Parties. The fees charged by such accounting firm shall be paid by [†]. 

 

	 	6.6.3	Merck shall include in each sublicense granted by it pursuant to this Agreement a provision requiring the sublicensee to make reports to Merck, to keep and maintain records of sales made pursuant to such sublicense and
to grant access to such records by Xenon’s independent accountant to the same extent required of Merck under this Agreement. 

  

	 	6.6.4	Upon the expiration of [†]following[†], the calculation of royalties payable with respect to [†]. 

  
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	 	6.6.5	Xenon shall treat all financial information subject to review under this Section 6.6 or under any sublicense agreement in accordance with the confidentiality and non-use provisions of this Agreement, and shall
cause its accounting firm to enter into an acceptable confidentiality agreement with Merck and/or its Related Parties obligating it to retain all such information in confidence pursuant to such confidentiality agreement. 

 

	6.7	Payment Exchange Rate. 

 All payments to be made by Merck to Xenon under this Agreement
shall be made in United States dollars and may be paid by check made to the order of Xenon or bank wire transfer in immediately available funds to such bank account in the United States as may be designated in writing by Xenon from time-to-time. In
the case of sales outside the United States, the rate of exchange to be used in computing the monthly amount of currency equivalent in United States dollars due Xenon shall be made at the monthly rate of exchange utilized by Merck in its worldwide
accounting system, prevailing on the third to the last business day of the month preceding the month in which such sales are recorded by Merck. 
  

	6.8	Income Tax Withholding. 

 [†]. 

ARTICLE 7 REPRESENTATIONS AND WARRANTIES 
  

	7.1	Representations and Warranties of Each Party. 

 Each Party represents and warrants to the
other Party that as of the Effective Date: 
  

	 	(a)	it is duly-incorporated, validly existing and in good standing under the laws of the jurisdiction of its incorporation; 

  

	 	(b)	it has the full right, power and authority to enter into this Agreement and to perform its obligations hereunder; and 

  

	 	(c)	this Agreement has been duly-executed by it and is legally binding upon it, enforceable in accordance with its terms, and does not conflict with any agreement, instrument or understanding, oral or written, to which it
is a party or by which it may be bound, nor violate any material law or regulation of any court, governmental body or administrative or other agency having jurisdiction over it. 

 

	7.2	Xenon Representations and Warranties. 

 Xenon represents and warrants to Merck that as of
the Effective Date: 
  

	 	(a)	[†]; 

  
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	 	(b)	[†]; 

  

	 	(c)	[†]; 

  

	 	(d)	[†]; and 

  

	 	(e)	Xenon has not received notice of any claims, judgments or settlements against or owed by Xenon, nor of any pending or threatened claims or litigation, relating to the Xenon Patent Rights or Xenon Know-How.

  

	7.3	Merck Representations and Warranties. 

 Merck represents and warrants to Xenon that as of
the Effective Date: 
  

	 	(a)	[†]; 

  

	 	(b)	[†]; 

  

	 	(c)	[†]; and 

  

	 	(d)	Merck has not received notice of any claims, judgments or settlements against or owed by Merck nor of pending or threatened claims or litigation relating to the Merck Patent Rights or Merck Know-How. 

 

	7.4	Covenants. 

  

	 	7.4.1	[†]. 

  

	 	7.4.2	[†]. 

  

	 	7.4.3	[†]. 

  

	 	7.4.4	[†]. 

  

	 	7.4.5	[†]. 

  

	7.5	Disclaimer of Warranties. 

 The warranties expressly provided in this Agreement are the
sole warranties given by the Parties hereunder, and are made expressly in lieu of, and exclude, any implied warranties of merchantability, fitness for a particular purpose, non-infringement or otherwise, and all other express or implied
representations and warranties provided by common law, statute or otherwise are hereby disclaimed by both Parties. 

  
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	7.6	Limitation of Liability. 

 [†]; provided, however, the foregoing
limitation of liability shall not apply to the liabilities arising from: (a) fraud or fraudulent misrepresentation of a Party or its Affiliate, or (b) the gross negligence or wilful misconduct of a Party or its Affiliate. Furthermore, this
Section 7.6 shall not be construed to limit either Party’s indemnification obligations under Section 7.7 or a Party’s right to obtain such damages for a breach of Article 5. 

 

	7.7	Indemnification. 

  

	 	7.7.1	Indemnification By Merck. Merck shall indemnify, defend and hold Xenon, its Affiliates and its and their respective agents, employees, officers and directors (each a “Xenon Indemnitee”) harmless
from and against any and all claims, suits, actions, demands, liabilities, expenses and/or loss, including reasonable legal expense and attorneys’ fees (collectively, “Losses”) to which any Xenon Indemnitee may become subject
as a result of any claim, demand, action or other proceeding by any Person other than a Party or its Affiliates to the extent such Losses arise out of: (a) Merck’s, its Related Parties’ or subcontractors’ performance of
Merck’s obligations under this Agreement; (b) the manufacture, use, handling, storage, sale or other disposition of Compounds or Products by Merck and its Related Parties, only to the extent such manufacture, use, handling, storage, sale
or other disposition activities (or any component thereof by Xenon for Merck) are not performed by, or on behalf of, Xenon and/or its Affiliates; (c) the use by a Third Party of any Compound or Product sold by or otherwise provided by Merck or
its Related Parties; or (d) a material breach by Merck or its Related Parties of any covenant, representation, warranty or other Agreement made by Merck in this Agreement; except, in each case, to the extent such Losses result from the material
breach by Xenon, its Affiliates, sublicensees or subcontractors of any covenant, representation, warranty or other agreement made by Xenon in this Agreement or the negligence or wilful misconduct of any Xenon Indemnitee. 

 

	 	7.7.2	Indemnification by Xenon. Xenon shall indemnify, defend and hold Merck, its Affiliates, Related Parties and its and their respective agents, employees, officers and directors (each a “Merck
Indemnitee”) harmless from and against any and all Losses, to which any Merck Indemnitee may become subject as a result of any claim, demand, action or other proceeding by any Person other than a Party or its Affiliates to the extent such
Losses arise out of: (a) Xenon’s, its Affiliates’, sublicensees’ or subcontractors’ performance of Xenon’s obligations under this Agreement; (b) the manufacture, use, handling, storage, sale or other disposition of
Compounds or Products by, or on behalf or, Xenon and/or its Affiliates, including the use of Compounds or Products in connection with Clinical Trials; (c) the use by a Third Party of any Compound or Product sold by or otherwise provided by
Xenon, its Affiliates, its sublicensees or subcontractors; or (d) a material breach by Xenon, its Affiliates, sublicensees or subcontractors of any covenant, representation or warranty or other agreement made by Xenon in this Agreement; except,
in each case, to the extent such Losses result from the material breach by Merck, its Related Party or subcontractors of any covenant, representation, warranty or other agreement made by Merck in this Agreement or the negligence or wilful misconduct
of any Merck Indemnitee. 

  
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	 	7.7.3	Notice of Indemnification Obligation and Defense. Any Party entitled to indemnification under Section 7.7.1 or 7.7.2 shall give notice to the indemnifying Party of any Losses that may be subject to
indemnification, promptly after learning of such Losses, but the omission to so notify the indemnifying Party promptly will not relieve the indemnifying Party from any liability under Section 7.7.1 or 7.7.2 except to the extent that the
indemnifying Party shall have been prejudiced as a result of the failure or delay in providing such notice. The indemnifying Party shall assume the defense of such Losses with counsel reasonably satisfactory to the indemnified Party. If such defense
is assumed by the indemnifying Party, the indemnifying Party will not be subject to any liability for any settlement of such Losses made by the indemnified Party without its consent (but such consent will not be unreasonably withheld or delayed),
and will not be obligated to pay the fees and expenses of any separate counsel retained by the indemnified Party with respect to such Losses. The indemnified Party shall provide the indemnifying Party with all information in its possession and all
assistance reasonably necessary to enable the indemnifying Party to carry on the defense of any such Losses. 

  

	7.8	Insurance. 

  

	 	7.8.1	Xenon’s Insurance Obligations. During the term of this Agreement, Xenon shall maintain, at its cost, adequate insurance against liability and other risks associated with its activities contemplated by this
Agreement, including but not limited to its indemnification obligations herein, in such amounts and on such terms as are customary for prudent practices in the pharmaceutical industry for the activities to be conducted by it under this Agreement.
Xenon shall furnish to Merck evidence of such insurance upon request by Merck. 

  

	 	7.8.2	Merck’s Insurance Obligations. Merck hereby represents and warrants to Xenon that it is self-insured against liability and other risks associated with its activities and obligations under this Agreement in
such amounts and on such terms as are customary for a company in the pharmaceutical industry. 

 ARTICLE 8 PATENT
PROVISIONS 
  

	8.1	Filing, Prosecution and Maintenance of Patent Rights During the Research Program Term. 

  

	 	8.1.1	During the Research Program Term: 

  

	 	(a)	Merck shall be responsible for, and shall bear the cost of, the preparation, filing, prosecution and maintenance, in its sole discretion, of: 

 

	 	(i)	the Merck Patent Rights; and 

  

	 	(ii)	all Patent Rights Covering Merck Research Program Technology. 

  

	 	(b)	Subject to the provisions of Sections 8.1.1(c) and (d) and Section 8.2.1, Xenon shall be responsible for the preparation, filing, prosecution and maintenance, of: 

 

	 	(i)	the Xenon Patent Rights; 

  
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	 	(ii)	all Patent Rights Covering Xenon Research Program Technology; and 

  

	 	(iii)	all Patent Rights Covering Joint Research Program Technology. 

  

	 	(c)	With respect to the preparation, filing, prosecution and maintenance of the Patent Rights set out in Section 8.1.1(b) (“Xenon Patent Prosecution”): 

 

	 	(i)	Xenon shall consult from time-to-time with Merck with respect to its choice of patent counsel; 

  

	 	(ii)	Xenon shall provide to Merck, as often as reasonably requested, written reports listing the jurisdictions for Xenon Patent Prosecution and summarizing Xenon Patent Prosecution activities; 

 

	 	(iii)	Xenon shall provide to Merck, for its review and comment, drafts of all patent applications, and all other material correspondence, submissions and documents, relating to Joint Patent Rights; and 

 

	 	(iv)	Xenon shall bear the costs in respect of the Xenon Patent Rights and the Parties shall share equally the costs in respect of the Patent Rights Covering Xenon Research Program Technology and the Patent Rights Covering
Joint Research Program Technology. 

  

	 	(d)	[†]. 

  

	 	(e)	[†]. 

  

	8.2	Exercise of the Merck Option. 

  

	 	8.2.1	On each occasion on which Merck exercises the Merck Option, but subject to the provisions of Section 8.2.3, Merck shall thereafter have the sole right, and the obligation, at its cost, to prepare, file, prosecute
and maintain, throughout the Territory, all Patent Rights Covering the Xenon Research Program Technology and Joint Research Program Technology, in each case that is the subject of the license granted to Merck pursuant to Section 3.1.

  

	 	8.2.2	With respect to the preparation, filing, prosecution and maintenance of the Patent Rights set out in Section 8.2.1 (“Merck Patent Prosecution”), Merck shall: 

 

	 	(i)	consult from time-to-time with Xenon with respect to its choice of patent counsel; and 

  

	 	(ii)	provide to Xenon, so often as reasonably requested, written reports listing the jurisdictions for Merck Patent Prosecution and summarizing Merck Patent Prosecution activities. 

  
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	 	8.2.3	Merck shall not cease the prosecution or maintenance of any Patent Rights set out in Section 8.2.1, without first [†]. 

  

	 	8.2.4	In the event that [†]. 

  

	8.3	Filing, Prosecution and Maintenance of Patent Rights Following Expiration of the Research Program Term. 

  

	 	8.3.1	Following the expiration of the Research Program Term: 

  

	 	(a)	Merck and Xenon shall have the right, each in its sole discretion and at its own cost, to prepare, file, prosecute and maintain, respectively, the Merck Patent Rights and the Xenon Patent Rights. 

 

	 	(b)	Merck shall have the right, in its sole discretion, and at its cost, to prepare, file, prosecute and maintain all Patent Rights Covering Merck Research Program Technology. 

 

	 	(c)	Subject to the provisions of Section 8.2.1, Xenon shall have the right, in its sole discretion, and at its cost, to prepare, file, prosecute and maintain: 

 

	 	(i)	[†]; and 

  

	 	(ii)	subject to the provisions of [†]. 

  

	 	(d)	[†]. 

  

	 	(e)	[†]. 

  

	8.4	Enforcement and Defense. 

  

	 	8.4.1	Enforcement and Defense Relating to Xenon Patent Rights or Xenon Know-How. 

  

	 	(a)	During the Research Program Term: 

  

	 	(i)	each Party shall give to the other notice of any infringement of any Patent Rights Covering Joint Research Program Technology or the misuse or misappropriation of Joint Know-How that comes to such Party’s
attention; 

  

	 	(ii)	Xenon shall give Merck notice of (A) any infringement of [†]; 

  

	 	(iii)	[†]; 

  

	 	(iv)	Xenon, upon notice to Merck, shall have the first right to initiate and prosecute such legal action in its own name or in the name of Xenon and Merck, or to control the defense of any declaratory judgment action
relating to any such Patent Rights; 

  
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	 	(v)	Xenon shall promptly inform Merck if it elects not to exercise such first right and Merck shall thereafter have the right to either initiate and prosecute such action or to control the defense of such declaratory
judgment action in the name of Merck and, if necessary, Xenon; 

  

	 	(vi)	the costs of any such actions [†]; and 

  

	 	(vii)	each Party shall have the right to be represented by counsel of its own choice. 

  

	 	(b)	Following the expiration of the Research Program Term: 

  

	 	(i)	Each Party shall give to the other notice of: 

  

	 	(A)	any infringement of any Patent Rights Covering Joint Research Program Technology; 

  

	 	(B)	misuse or misappropriation of Joint Know-How; and 

  

	 	(C)	in the event that Merck has exercised the Merck Option, any infringement of any Patent Rights Covering the Xenon Research Program Technology that is the subject of the license granted pursuant to Section 3.1.

  

	 	(ii)	[†]; 

  

	 	(iii)	[†]; 

  

	 	(iv)	[†]; and 

  

	 	(v)	each Party shall have the right to be represented by counsel of its own choice, at its cost. 

  

	 	(c)	For any action contemplated in this Section 8.4.1, in the event that either Party is unable to initiate or prosecute such action solely in its own name, the other Party shall join such action voluntarily and shall
execute and cause its Affiliates to execute all documents necessary to initiate litigation to prosecute and maintain such action. In connection with any action, each Party shall cooperate fully and shall provide each other with any information or
assistance that either may reasonably request. Each Party shall keep the other informed of developments in any action or proceeding, including, to the extent permissible by law, consultation on and approval of any settlement, the status of any
settlement negotiations and the terms of any offer related thereto. 

  

	 	(d)	Any recovery obtained by either or both Merck and Xenon in connection with or as a result of any action contemplated by this Section, whether by settlement or otherwise, shall be shared in order as follows:

  

	 	(i)	[†]; 

  
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	 	(ii)	[†]; and 

  

	 	(iii)	[†]. 

  

	 	(e)	Xenon shall inform Merck of any certification regarding any Xenon Patent Rights it has received pursuant to either 21 U.S.C. §§355(b)(2)(A)(iv) or (j)(2)(A)(vii)(IV) or its successor provisions
[†]provided, however, that [†]. 

  

	8.5	Cooperation; Patent Term Restoration; Pediatric Study Extensions. 

 The Parties agree to
cooperate and to take reasonable actions to maximize the protections available under the safe harbor provisions of 35 U.S.C. 103(c) for U.S. patents and patent applications. The Parties shall cooperate with each other, including, without limitation,
to provide necessary information and assistance as the other Party may reasonably request, in obtaining patent term restoration or supplemental protection certificates or their equivalents, or pediatric study extensions, in any country in the
Territory where applicable to Xenon Patent Rights, Xenon Research Program Technology, Merck Patent Rights, Merck Research Program Technology and/or Joint Patent Rights. 
  

	8.6	Inventorship. 

 Inventorship of all patent applications filed under this Agreement shall
be determined in accordance with U.S. patent law. 
 ARTICLE 9 TERM AND TERMINATION 

 

	9.1	Term and Expiration. 

  

	 	9.1.1	This Agreement shall be effective as of the Effective Date and, unless terminated earlier pursuant to Sections 9.2 or 9.3, this Agreement shall continue in full force and effect until the expiration of all royalty
obligations hereunder. 

  

	 	9.1.2	In the event that Merck has exercised the Merck Option, upon expiration of this Agreement the license granted to Merck pursuant to Section 3.1(a) shall thereupon become a fully paid-up, non-exclusive license, and
each Party shall have the right, both in and outside of the Field, under Joint Research Program Technology, with a right to grant licenses under their respective interests in Joint Research Program Technology, to [†], and shall have no
financial obligation to the other Party, or other obligation to account to the other Party, arising from the exercise of such right. 

  

	 	9.1.3	In the event that Merck has not exercised the Merck Option, upon expiration of this Agreement, [†], and shall have no financial obligation to the other Party, or other obligation to account to the other Party,
arising from the exercise of such right. 

  
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	9.2	Termination By Merck. 

  

	 	9.2.1	Notwithstanding anything contained herein to the contrary, Merck shall have the right to terminate this Agreement, in its sole discretion, by giving notice in writing (a “Termination Notice”) to Xenon
at any time following[†], specifying the effective date of such termination (the “Termination Date”) and, except for the surviving provisions set forth in Section 9.4 and subject to the following provisions of this
Section 9.2, all of the rights and obligations of the Parties hereunder shall terminate as of the Termination Date: 

  

	 	(a)	[†]; 

  

	 	(b)	[†]; 

  

	 	(c)	Each Party shall have the right to fully use and exploit its interest in all Joint Research Program Technology both in and outside of the Field, without limitation, and to grant licenses under its interest in Joint
Research Program Technology, all without any obligation to account to the other Party for any consideration received by such Party from the exercise of such right; 

 

	 	(d)	The Parties shall continue to prosecute, maintain, enforce, and bear the costs of, all Patent Rights Covering Joint Research Program Technology in accordance with the provisions of Article 8; 

 

	 	(e)	[†]; 

  

	 	(f)	[†]; and 

  

	 	(g)	No later than thirty (30) days after the Termination Date, each Party shall return or cause to be returned to the other Party all Information in tangible form received from the other Party and all copies thereof;
provided, however, that each Party may retain any Information reasonably necessary for such Party’s continued practice under any license(s) which do not terminate pursuant to this Section, and may keep one copy of Information received from the
other Party in its confidential files for record purposes. 

  

	9.3	Termination for Cause. 

  

	 	9.3.1	Cause for Termination. This Agreement may be terminated at any time during the term of this Agreement: 

  

	 	(a)	upon written notice by either Party if the other Party is in breach of its material obligations hereunder by causes and reasons within its control and has not cured such breach within ninety (90) days after notice
requesting cure of the breach; provided, however, in the event of a good faith dispute with respect to the existence of a material breach, the ninety (90) day cure period shall be tolled until such time as the dispute is resolved
pursuant to Section 10.6; or 

  
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	 	(b)	by either Party upon the filing or institution of bankruptcy, reorganization, liquidation or receivership proceedings, or upon an assignment of a substantial portion of the assets for the benefit of creditors by the
other Party; provided, however, that in the case of any involuntary bankruptcy proceeding such right to terminate shall only become effective if the Party consents to the involuntary bankruptcy or such proceeding is not dismissed
within [†] days after the filing thereof. 

  

	 	9.3.2	Effect of Termination for Cause on License. 

  

	 	(a)	If Merck terminates this Agreement under Section 9.3.1(a), then Merck’s licenses pursuant to Sections 4.1.1 and 4.1.3 shall become fully paid-up, perpetual licenses, Xenon’s licenses pursuant to Sections
4.1.2 and 4.1.3 shall terminate as of such termination date and Xenon shall, within thirty (30) days after the effective date of such termination return or cause to be returned to Merck all Products, Compounds, all Information in tangible form,
and all substances or compositions delivered or provided by Merck, as well as any other material provided by Merck in any medium. If Xenon terminates this Agreement under Section 9.3.1(a), then Merck’s licenses pursuant to Sections 4.1.1
and 4.1.3 shall terminate as of such termination date and Merck shall, within thirty (30) days after the effective date of such termination, return or cause to be returned to Xenon all Information in tangible form and substances or compositions
delivered or provided by Xenon, as well as any other material provided by Xenon in any medium. 

  

	 	(b)	Upon termination of this Agreement by Merck pursuant to Section 9.2, or by Xenon pursuant to Section 9.3.1(a), Merck and its Affiliates, sublicensees and distributors shall be entitled, during the twelve
(12) month period immediately following the effective date of termination, to finish any work-in-progress and to sell any Products or Compounds remaining in inventory, in accordance with the terms of this Agreement. 

 

	 	(c)	In the event that this Agreement is terminated by Merck under Section 9.3.1(b), then, in addition to the provisions of Section 9.3.2(a), this Section 9.3.2(c) shall also apply. In the event that this
Agreement is terminated due to the rejection of this Agreement by or on behalf of Xenon due to an Insolvency Event[†]. 

The foregoing provisions of Section 9.3.2(c) are without prejudice to any rights Merck may have arising under the Code or other
applicable law. 
  

	9.4	Effect of Expiration or Termination; Survival. 

 Expiration or termination of this
Agreement shall not relieve the Parties of any obligation accruing prior to such expiration or termination. Any expiration or termination of this Agreement shall be without prejudice to the rights of either Party against the other accrued or
accruing under this Agreement prior to expiration or termination, including, without limitation, the obligation to pay royalties for Products or Compounds sold prior to such expiration or termination. The provisions of Article 5 shall survive
the expiration or termination of this Agreement and shall continue in effect for 

  
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ten (10) years. The provisions of Articles 1, 7, and 9 (except Section 9.5), the provisions of Article 8 governing the prosecution and enforcement of Patent Rights Covering Joint
Research Program Technology, and Sections 2.6.2, 7.6, 7.7.1, 10.3, 10.4 and 10.5 shall survive the expiration or termination of this Agreement. 
  

	9.5	[†]. 

  

	 	9.5.1	In the event that there is a Change of Control [†] shall provide written notice to [†] and: 

  

	 	(a)	[†]; and 

  

	 	(b)	[†] shall have the right, at [†] election at any time after such Change of Control, to implement some or all of the following revisions to this Agreement: 

 

	 	(i)	[†]; 

  

	 	(ii)	[†]; 

  

	 	(iii)	[†]; 

  

	 	(iv)	[†]; and 

  

	 	(v)	[†]. 

  

	 	9.5.2	“Change of Control” of a Party means: (a) the sale of all or substantially all of the Party’s assets or business relating to this Agreement; (b) a merger, amalgamation, reorganization or
consolidation involving the Party in which the voting securities of such Party outstanding immediately prior thereto cease to represent at least fifty percent (50%) of the combined voting power of the surviving entity immediately after such
merger, reorganization or consolidation; or (c) the acquisition by a Person, or group of Persons acting in concert, of more than fifty percent (50%) of the voting equity securities or management control of the Party. 

ARTICLE 10 MISCELLANEOUS 
  

	10.1	Force Majeure. 

 Neither Party shall be held liable to the other Party nor be
deemed to have defaulted under or breached this Agreement for failure or delay in performing any obligation under this Agreement to the extent such failure or delay is caused by or results from causes beyond the reasonable control of the affected
Party, potentially including, but not limited to, embargoes, war, acts of war (whether war be declared or not), acts of terrorism, insurrections, riots, civil commotions, strikes, lockouts or other labor disturbances, fire, floods, or other acts of
God, or acts, omissions or delays in acting by any governmental authority or the other Party. The affected Party shall notify the other Party of such force majeure circumstances as soon as reasonably practical, and shall promptly undertake all
reasonable efforts necessary to cure such force majeure circumstances. 

  
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 EXECUTION VERSION 
  

	10.2	Assignment. 

 Except as hereinafter provided in this Section 10.2, this Agreement
shall not be assigned or otherwise transferred, in whole or in part, nor may any right or obligation hereunder be assigned or transferred, by either Party without the prior written consent of the other Party, provided, however, that:

  

	 	10.2.1	Merck may assign this Agreement and its rights and obligations hereunder, without Xenon’s consent: 

  

	 	(i)	in whole or in part to any Affiliate of Merck, provided that Merck notifies Xenon in writing within a reasonable amount of time after such assignment; or 

 

	 	(ii)	in whole or in part in connection with a Change of Control of Merck or any Affiliate of Merck, provided that Merck gives notice in writing to Xenon within twenty (20) business days following the completion of such
event. 

  

	 	10.2.2	Xenon may assign this Agreement and its rights and obligations hereunder, without Merck’s consent in whole or in part in connection with a Change of Control of Xenon, provided that Xenon gives notice in writing to
Merck within ten (10) business days following the completion of such event. 

  

	 	10.2.3	Any attempt by either Party to assign this Agreement in a manner which is not in accordance with this Section 10.2 shall be null and void and of no effect. In addition, no assignment shall release any Party from
responsibility for the performance of any accrued obligation of such Party hereunder. Furthermore, this Agreement shall be binding upon and enforceable against the successor to or any permitted assignees from either of the Parties hereto.

  

	10.3	Severability. 

 If any one or more of the provisions contained in this Agreement is held
invalid, illegal or unenforceable in any respect, the validity, legality and enforceability of the remaining provisions contained herein shall not in any way be affected or impaired thereby, unless the absence of the invalidated provision(s)
adversely affects the substantive rights of the Parties. The Parties shall, in such an instance, use their best efforts to replace the invalid, illegal or unenforceable provision(s) with valid, legal and enforceable provision(s) which, insofar as
practical, implement the purposes of this Agreement. 
  

	10.4	Notices. 

 All notices which are required or permitted hereunder shall be in writing and
sufficient if delivered personally, sent by facsimile (and promptly confirmed by personal delivery, registered or certified mail or overnight courier), sent by nationally-recognized overnight courier or sent by registered or certified mail, postage
prepaid, return receipt requested, addressed as follows: 

  
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 EXECUTION VERSION 
  

			
	 if to Xenon, to:
	  	Xenon Pharmaceuticals Inc.
		  	3650 Gilmore Way
		  	Burnaby, British Columbia
		  	Canada V5G 4W8
		
		  	Attention: President and Chief Executive Officer
		  	Facsimile No.: 604-484-3450
		
		  	and
		
		  	Attention: General Counsel
		  	Facsimile No.: 604-484-3450
		
	 if to Merck, to:
	  	Merck Sharp & Dohme Research Ltd.
		  	Chesney House
		  	96 Pitts Bay Road
		  	Pembroke HM 08, Bermuda
		  	Attention: Wesley Toavs
		  	Facsimile No.: 441-294-1551
		
	 and:
	  	Merck & Co., Inc.
		  	One Merck Drive
		  	P.O. Box 100, WS3A-65
		  	Whitehouse Station, NJ 08889-0100
		  	Attention: Office of Secretary
		  	Facsimile No.: (908)735-1246
		
	 and:
	  	Merck & Co., Inc.
		  	One Merck Drive
		  	P.O. Box 100, WS2A-30
		  	Whitehouse Station, NJ 08889-0100
		  	Attention: Chief Licensing Officer
		  	Facsimile No.: (908)735-1214

 or to such other address(es) as the Party to whom notice is to be given may have furnished to the other Party
in writing in accordance herewith. Any such notice shall be deemed to have been given: (a) when delivered if personally delivered or sent by facsimile on a business day (or if delivered or sent on a non-business day, then on the next business
day); (b) on the business day after dispatch if sent by nationally-recognized overnight courier; or (c) on the fifth (5th) business day following the date of mailing, if sent by mail. 

 

	10.5	Applicable Law. 

 This Agreement shall be governed by and construed in accordance with
the laws of [†] without reference to any rules of conflict of laws or renvoi. 

  
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 EXECUTION VERSION 
  

	10.6	Dispute Resolution. 

  

	 	10.6.1	The Parties shall negotiate in good faith and use reasonable efforts to settle any dispute, controversy or claim arising from or related to this Agreement or the breach thereof. If the Parties do not fully settle, and a
Party wishes to pursue the matter, each such dispute, controversy or claim that is not an “Excluded Claim” (as defined below) shall be finally resolved by binding arbitration in accordance with the Commercial Arbitration Rules and
Supplementary Procedures for Large Complex Disputes of the American Arbitration Association (“AAA”), and judgment on the arbitration award may be entered in any court having jurisdiction thereof. 

 

	 	10.6.2	The arbitration shall be conducted by a panel of three (3) persons experienced in the pharmaceutical business. Within thirty (30) days after initiation of arbitration, each Party shall select one person to act
as arbitrator and the two Party-selected arbitrators shall select a third arbitrator within thirty (30) days of their appointment. If the arbitrators selected by the Parties are unable or fail to agree upon the third arbitrator, the third
arbitrator shall be appointed by the AAA. The place of arbitration shall be [†], and all proceedings and communications shall be in English. 

  

	 	10.6.3	Either Party may apply to the arbitrators for interim injunctive relief until the arbitration award is rendered or the controversy is otherwise resolved. Either Party also may, without waiving any remedy under this
Agreement, seek from any court having jurisdiction any injunctive or provisional relief necessary to protect the rights or property of that Party pending the arbitration award. The arbitrators shall have no authority to award punitive or any other
type of damages not measured by a Party’s compensatory damages. [†]. 

  

	 	10.6.4	Except to the extent necessary to confirm an award or as may be required by law, neither a Party nor an arbitrator may disclose the existence, content, or results of an arbitration without the prior written consent of
both Parties. In no event shall an arbitration be initiated after the date when commencement of a legal or equitable proceeding based on the dispute, controversy or claim would be barred by the applicable [†] statute of limitations.

  

	 	10.6.5	The Parties agree that, in the event of a dispute over the nature or quality of performance under this Agreement, neither Party may terminate this Agreement until final resolution of the dispute through arbitration or
other judicial determination. The Parties further agree that any payments made pursuant to this Agreement pending resolution of the dispute shall be refunded if an arbitrator or court determines that such payments are not due. 

 

	 	10.6.6	As used in this Section, the term “Excluded Claim” means a dispute, controversy or claim that concerns [†]. 

  

	 	10.6.7	 In the event that any matter involving the determination of any amounts due to either Party pursuant to the audit process set out in Section 6.6
has not been resolved pursuant to the procedures set out in Section 10.6.1, then the Parties shall (i) use reasonable efforts to reach agreement on the appointment of one (1) internationally-recognized independent accounting firm to
determine the matter, (ii) if the Parties cannot reach agreement on such accounting firm within [†] business days, then each Party shall appoint one
(1) internationally-

  
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 EXECUTION VERSION 
  

	 	
recognized accounting firm to determine the matter, and (iii) if such firms cannot reach agreement within [†] business days from their appointment, such firms shall choose a third
internationally-recognized independent accounting firm who shall make the final determination as promptly as possible, which determination shall be final and binding on the Parties. 

 

	10.7	Entire Agreement; Amendments. 

 This Agreement, together with the Schedules and Exhibits
hereto, contains the entire understanding of the Parties with respect to the subject matter hereof. Any other express or implied agreements and understandings, negotiations, writings and commitments, either oral or written, in respect to the subject
matter hereof are superseded by the terms of this Agreement. The Schedules and Exhibits to this Agreement are incorporated herein by reference and shall be deemed a part of this Agreement. This Agreement may be amended, or any term hereof modified,
only by a written instrument duly-executed by authorized representative(s) of both Parties hereto. 
  

	10.8	Headings. 

 The captions to the several Articles, Sections and subsections hereof are not
a part of this Agreement, but are merely for convenience to assist in locating and reading the several Articles and Sections hereof. 
  

	10.9	Independent Contractors. 

 It is expressly agreed that Xenon and Merck shall be
independent contractors and that the relationship between the two Parties shall not constitute a partnership, joint venture or agency. Neither Xenon nor Merck shall have the authority to make any statements, representations or commitments of any
kind, or to take any action, which shall be binding on the other Party, without the prior written consent of the other Party. 
  

	10.10	Waiver. 

 The waiver by either Party hereto of any right hereunder, or of any failure of
the other Party to perform, or of any breach by the other Party, shall not be deemed a waiver of any other right hereunder or of any other breach by or failure of such other Party whether of a similar nature or otherwise. 

 

	10.11	Cumulative Remedies. 

 No remedy referred to in this Agreement is intended to be
exclusive, but each shall be cumulative and in addition to any other remedy referred to in this Agreement or otherwise available under law. 
  

	10.12	Waiver of Rule of Construction. 

 Each Party has had the opportunity to consult with
counsel in connection with the review, drafting and negotiation of this Agreement. Accordingly, the rule of construction that any ambiguity in this Agreement shall be construed against the drafting Party shall not apply. 

  
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 EXECUTION VERSION 
  

	10.13	Certain Conventions. 

 Any reference in this Agreement to an Article, Section,
subsection, paragraph, clause, Schedule or Exhibit shall be deemed to be a reference to an Article, Section, subsection, paragraph, clause, Schedule or Exhibit, of or to, as the case may be, this Agreement, unless otherwise indicated. Unless the
context of this Agreement otherwise requires, (a) words of any gender include each other gender, (b) words such as “herein”, “hereof”, and “hereunder” refer to this Agreement as a whole
and not merely to the particular provision in which such words appear, (c) words using the singular shall include the plural, and vice versa. 
  

	10.14	Business Day Requirements. 

 In the event that any notice or other action or omission is
required to be taken by a Party under this Agreement on a day that is not a business day, then such notice or other action or omission shall be deemed required to be taken on the next occurring business day. 

 

	10.15	Counterparts. 

 This Agreement may be executed in two or more counterparts, each
of which shall be deemed an original, but all of which together shall constitute one and the same instrument. 
 IN WITNESS WHEREOF, the Parties have
executed this Agreement as of the Effective Date. 
  

									
	MERCK SHARP & DOHME RESEARCH LTD.	 		 	XENON PHARMACEUTICALS INC.
					
	BY:	 	 /s/ Wesley Toavs
	 		 	BY:	 	 /s/ Simon Pimstone

		 	Wesley Toavs	 		 		 	Simon N. Pimstone, MD, PhD, FRCPC
					
	TITLE:	 	Assistant Treasurer	 		 	TITLE:	 	President & CEO

  
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 EXECUTION VERSION 
  

 EXHIBIT 1.56 

MERCK PATENT RIGHTS  

  
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 EXECUTION VERSION 
  

 EXHIBIT 1.78 

RESEARCH PLAN 

  
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 Xenon Pharmaceuticals Inc. 

and 
  

 
 Research Plan 

June 10, 2009 

  
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 Highly Confidential 

 
 table of contents 

 

					
	 1. background and scope of document
	  	 	3	  
	 2. CONCEPT
	  	 	3	  
	 2A. INITIAL LEAD OPTIMIZATION PROGRAM
	  	 	4	  
	 2B. TARGET VALIDATION PROGRAM
	  	 	8	  
	 2C. ADVANCING PROGRAMS INTO LEAD
IDENTIFICATION / LEAD OPTIMIZATION
	  	 	10	  
		
	 3. conclusion
	  	 	15	  

 [†] [Redaction continued for 14 pages] 
  

 

  
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 EXECUTION VERSION 
  

 EXHIBIT 1.100 

XENON PATENT RIGHTS 

  
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 EXECUTION VERSION 
  

 EXHIBIT 1.102 

XENON TARGET VALIDATION CRITERIA 

  
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 EXECUTION VERSION 
  

 Exhibit 5.3 

PRESS RELEASE 
  

					
	

	 		 	FOR IMMEDIATE RELEASE

 Vancouver, Canada, 11th June 2009 

Xenon Enters Cardiovascular Disease Collaboration with Merck & Co., Inc. 

Xenon today announced a strategic alliance with Merck & Co., Inc., through an affiliate, to discover and develop novel small molecule candidates for
the potential treatment of cardiovascular disease. 
 “We are very excited to be collaborating with Merck to define new therapeutics in the area of
cardiovascular diseases,” said Simon Pimstone, President and CEO of Xenon. “With this deal, Xenon is continuing its strategy of risk mitigation by select partnering, while retaining ownership of other programs.” 

In collaboration with Merck, Xenon will perform validation studies using its clinical genetics platform, as well as drug discovery and select preclinical
development of small molecule compounds for those targets selected by a joint steering committee. 
 Under the terms of the agreement, Merck has the option
to exclusively license targets and compounds from Xenon for development and commercialization. In return, Xenon receives research funding and is eligible for option exercise fees, research, development and regulatory milestone payments of up to
US$94.5 million for the first target and up to US$89.5 million for each subsequent target selected for drug discovery. In addition, Merck will pay Xenon undisclosed royalties on sales of products resulting from the collaboration. Xenon retains the
right to develop and commercialize certain compounds for which Merck does not exercise its option. 

  
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 EXECUTION VERSION 
  

 Michael Hayden, CSO of Xenon added: “We recognize that Merck is a leading pharmaceutical company with
significant presence in and commitment to the cardiovascular space and they are an ideal strategic partner for Xenon. This new alliance, which represents our fifth partnership with a major pharmaceutical company, once again highlights Xenon’s
R&D capabilities and validates our drug discovery platform.” 

  
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 EXECUTION VERSION 
  

 About Xenon Pharmaceuticals Inc. (Xenon) 

Xenon is a privately owned, clinical genetics-based drug discovery and development company engaged in developing small molecule therapies based on the genetic
causes of select metabolic, neurological and cardiovascular diseases. For more information, visit the Company’s website at http://www.xenon-pharma.com. 

For more information regarding this press release, contact: 

Dr. Robin Sherrington, Senior Director, Strategic Alliances (604) 484-3363 ddunn@xenon-pharma.com 

This release contains forward-looking statements that are not based on historical fact. These forward-looking statements involve risks, uncertainties and
other factors that may cause the actual results, events or developments to be materially different from those expressed or implied by such forward-looking statements. Readers are cautioned not to place undue reliance on such forward-looking
statements. 

  
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 EXECUTION VERSION 

GUARANTEE 
 THIS GUARANTEE is made
effective as of June 10, 2009 
 BETWEEN: 

MERCK & CO., INC., a corporation organized and existing under the laws of New Jersey 

(“Merck”) 
 AND 

XENON PHARMACEUTICALS INC., a corporation organized and existing under the laws of Canada 

(“Xenon”) 

RECITALS 
 WHEREAS: 

 

	A.	Xenon has entered into an Exclusive Collaborative Research and Option Agreement (the “Agreement”) with Merck Sharp & Dohme Research Ltd. (“Merck Research”), a wholly-owned
subsidiary of Merck, upon the condition that Merck guarantee the performance of the financial obligations of Merck Research under the Agreement. 

  

	B.	Merck is prepared to guarantee the performance of the financial obligations of Merck Research under the Agreement upon the terms set out herein. 

IN CONSIDERATION of Xenon entering into the Agreement with Merck Research, and for other good and valuable consideration, the receipt and sufficiency
of which is hereby acknowledged, Merck hereby unconditionally guarantees to Xenon the due and punctual performance by Merck Research of all financial obligations of Merck Research under the Agreement, and promises on demand to pay to the order of
Xenon all indebtedness and liability of Merck Research under the Agreement, in the amounts, at the times and in the manner set forth in the Agreement. The following terms apply to this Guarantee: 

 

	(a)	Merck hereby unconditionally, absolutely and irrevocably guarantees and covenants to Xenon the full performance, observance, satisfaction, and payment of, any and all payment obligations as and when due by Merck
Research to Xenon under the Agreement (the “Guaranteed Obligations), provided that Merck Research has failed to make a payment when due under the Agreement and Xenon has provided a notice and demand for payment to Merck Research.

  
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	(b)	The liability of Merck pursuant to this Guarantee shall not be discharged, limited or released by any extensions of time for payment of any Guaranteed Obligations granted by Xenon to Merck Research.

  

	(c)	If any default shall be made in the performance, observance, satisfaction and payment of any of the Guaranteed Obligations, Merck covenants and agrees with Xenon to perform, observe, satisfy and pay to Xenon forthwith
any and all of the Guaranteed Obligations in respect of which such default will have occurred. 

  

	(d)	Until there has been full performance, observance, satisfaction and payment of all of the Guaranteed Obligations, the rights of Xenon and the obligations of Merck under this Guarantee shall remain in full force and
effect without regard to, and shall not be released, discharged or in any way affected or impaired, terminated or prejudiced by, the dissolution, winding-up or other cessation of existence of Merck Research, the amalgamation of Merck Research with
another corporation, the appointment of a custodian, liquidator, receiver or trustee in respect of the assets or undertaking, in whole or in part, of Merck Research, any arrangement, bankruptcy, composition, insolvency, liquidation, readjustment,
receivership, reorganization or other similar proceeding or occurrence relating to Merck Research, or any assignment by Merck Research for the benefit of creditors. 

 

	(e)	The foregoing guarantee shall be fully enforceable against Merck without Xenon first bringing legal process against or exhausting any remedy against Merck Research. 

 

	(f)	Xenon may assign, grant, pledge or transfer its interest in this Guarantee or any of the guaranteed liabilities or any power, remedy or right of Xenon hereunder on the same terms upon which Xenon may assign its interest
in the Agreement. 

  

	(g)	No waiver on the part of Xenon to exercise, and no delay in exercising, any right hereunder will operate as a waiver of this Guarantee, nor will any single or partial exercise of any right hereunder preclude the other
or further exercise thereof or the exercise of any other right. The remedies provided hereunder are not exclusive of any remedies provided at law. 

  

	(h)	This Agreement shall be governed by and construed in accordance with the laws of the State of New York without reference to any rules of conflict of laws or renvoi. 

(Signature page follows.) 

  
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 EXECUTION VERSION 

IN WITNESS WHEREOF the parties hereto have executed this Guarantee on the dates stated below. 

 

			
	MERCK & CO., INC.
		
		 	/s/ Mervyn Turner
	Per:	 	 Mervyn Turner

		 	 Chief Strategy Officer, Merck & Co., Inc. and Senior Vice President, Worldwide Licensing &
External Research, Merck Research Laboratories

		
	Date:	 	 June 10, 2009

	
	XENON PHARMACEUTICALS INC.
		
		 	/s/ Simon N. Pimstone
	Per:	 	 Simon N. Pimstone, MD, PhD, FRCPC

		 	 President & CEO

		
	Date:	 	 June 10, 2009

 This is the Execution Page to Guarantee dated effective June 10, 2009. 

  
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 C Confldential 

EXECUTION VERSION 
 First Amendment
to the 
 Exclusive Collaborative Research and Option Agreement between 

Essex Chemie AG (formerly Merck Sharp & Dohme Research Ltd.) and 

Xenon Pharmaceuticals Inc. 
 This First Amendment
(“First Amendment”) is made effective as of June 9, 2012 (the “First Amendment Effective Date”) by and between Essex Chemie AG (formerly Merck Sharp & Dohme Research Ltd.) (“ECAG”) and Xenon
Pharmaceuticals Inc. (“Xenon”) respecting that certain Exclusive Collaborative Research and Option Agreement between the Parties dated June 10, 2009 (the “Agreement”). 

WHEREAS, Xenon and ECAG entered into the Agreement for the purpose of carrying out certain collaborative research activities under the terms and conditions
set forth in the Agreement which may result in Research Program Technology; and 
 WHEREAS, Xenon and ECAG now wish to amend the agreement to extend the
Research Program Term and to modify the Research Plan, as set forth herein; 
 NOW, THEREFORE, in consideration of the foregoing and the mutual promises
contained hereinafter, the sufficiency of which is hereby acknowledged, Xenon and ECAG hereby agree as follows: 
  

	 	1.	All references to “Merck” in the Agreement shall be read and construed as references to “ECAG”. 

  

	 	2.	In Article 1, Definitions, the following definitions shall be deleted and replaced in their entirety as follows: 

1.25 “Field” means [†]. 

1.78 “Research Plan” means the preclinical research plan attached to the Agreement as Exhibit 1.78, including any written
modifications to same that were approved in writing by the JSC during the Research Program Term. Such modifications to the Research Plan include the eight (8) plans attached hereto as Exhibit 1.78 (including Exhibit 1.78A). The
“Research Plan” includes the “Extended Research Plan” which is attached hereto as Exhibit 1.78A. 

  
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 The Research Plan provides, among other things, that the JSC may approve a Target Validation Plan
or Target Drug Discovery Plan for each Target. The Research Plan during the Initial Research Program Term requires Xenon to [†]. In addition to modifications made by the JSC as provided above, the Parties may also amend the Research Plan by
mutual written agreement. In the event of a conflict between the terms of the Agreement, as amended herein, and the Research Plan, the terms of the Agreement, as amended herein, shall govern. 

1.81 “Research Program Term” means the combined duration of (i) the “Initial Research Program Term” and
(ii) the “Extended Research Program Term”, each as described in Section 2.2 as amended. 
  

	 	3.	In Article 2, Research Program, the following Sections shall be amended as follows: 

  

	 	3.1	Section 2.1, General, shall be deleted and replaced m its entirety as follows: 

“2.1 Xenon and ECAG shall conduct research activities pursuant to the provisions of this Agreement and the Research Program. Xenon shall
perform studies to produce data with the goal of [†]. For clarity, 
 (i) during the Initial Research Program Term (as defined below),
the Parties shall conduct the activities in the Field as described in the Research Plan set forth in Exhibit 1.78, including any written modifications to same that are approved in writing by the JSC; and 

(ii) during the Extended Research Program Term, the Parties shall conduct solely the activities agreed to in the “Extended Research
Plan” which is attached hereto as Exhibit 1.78A, including any written modifications to same that are approved in writing by the JSC, 

each of (i) and (ii) in accordance with the terms set out herein. 

It is intended that the Research Program be conducted as a unified, collaborative effort with the Parties’ activities carried out
primarily at each Party’s respective facilities. The Research Program shall be comprised of the Research Plan, one or more Target Validation Plans approved by the JSC pursuant to Section 2.5.2, and one or more Target Drug Discovery Plans
approved by the JSC pursuant to Section 2.5.2. In addition to modifications approved by the JSC in writing as provided in this Section 2.1 above, the Parties may also amend the Research Plan by mutual written agreement. In the event of a conflict
between the terms of the Agreement and the Research Plan, the terms of this Agreement, as amended herein, shall govern. 
 For avoidance of
doubt, the Parties hereto confirm and agree that the Research Plan , including the Extended Research Plan, is an integral part of the Research Program for all purposes herein.” 

  
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	 	3.2	Section 2.2, Research Program Term, shall be deleted and replaced in its entirety as follows: 

  

	 	“2.2	Except as otherwise provided herein, the term of the Research Program shall commence on the Effective Date of the Agreement and continue up to and including [†] (the “Initial Research Program Term”), and
shall further continue during the period commencing on [†] and continue up to and including December 9, 2012 (the “Extended Research Program Term”).” 

 

	 	3.3	Section 2.5.4 shall be deleted and replaced in its entirety as follows: 

 “2.5.4 As
part of its obligations under the Research Program , [†].” 
  

	 	3.4	Section 2.5.6 shall be deleted and replaced in its entirety as follows : 

 “2.5.6 If
a Party (the “Rejecting Party”) rejects in writing a Target Candidate proposed by the other Party (the “Proposing Party”) pursuant to Section 2.5.l [†].” 

 

	 	3.5	The Parties hereto confirm and agree that as of the First Amendment Effective Date: (i) [†]; and 

(ii) no Target Candidates have been rejected under Section 2.5.6. 

 

	 	3.6	In Article 2, Research Program, a new section, Section 2.5.7, shall be added as follows: 

“2.5.7 Notwithstanding anything to the contrary in the Agreement, the Parties agree that all Xenon Research Program Technology generated,
developed or invented during the Research Program Term regarding [†] shall be deemed to be “Joint Research Program Technology”.” 
  

	 	3.7	In Article 2, Section 2.9 (h) shall be deleted and replaced in its entirety as follows: 

“(h) [†];” 
  

	 	4.	In Article 4, License Grants, Section 4.1.3 shall be deleted and replaced in its entirety as follows: 

“4.1.3 Joint Research Program Technology. Subject to the Merck Option, upon the expiration of the Research Program Term,
[†].” 

  
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	 	5.	In Article 6, Payments; Royalties and Reports, the following Section shall be amended as follows: 

5.1 Section 6.2, FTE Funding, shall be deleted and replaced in its entirety as follows: 

“6.2 For the duration of the Initial Research Program Term, ECAG shall fund, on a [†] basis (pro-rated for any period of less than
[†] months at the beginning or end of the Initial Research Program Term), in advance, a minimum often [†] FTEs of Xenon, but no more than [†] FTEs of Xenon, as approved by the JSC in writing, at the FTE Rate. 

For the duration of the Extended Research Program Term, Xenon shall undertake the work as provided in the Extended Research Plan, and:
(i) ECAG shall fund, on a [†] basis (pro-rated for any period of less than [†] months at the beginning or end of the Extended Research Program Term), in advance, for the period of [†] through and including [†] FTEs of Xenon
at the FTE Rate, and (ii) Xenon shall fund, for the period of [†] through and including [†], up to [†] FTEs of Xenon.” 
  

	 	6.	In Article 7, Representations and Warranties , a new section, Section 7.9, shall be added as follows: 

“7.9 Compliance with Laws and Business Ethics. Xenon acknowledges that ECAG’s corporate policy requires that ECAG’s business
must be conducted within the letter and spirit of the law. By signing this Agreement, and any associated amendments, each Party agrees to conduct the activities contemplated herein in a manner which is consistent with both law and good business
ethics. Neither Party shall make any payment, either directly or indirectly, of money or other assets (collectively , “Payment”) to government or political party officials, officials of international public organizations , candidates for
public office, or representatives of other businesses or persons acting on behalf of any of the foregoing (“Officials”) where such Payment would constitute a violation of any law. In addition, regardless of legality , neither Party shall
make any Payment either directly or indirectly to Officials if such Payment is for the purpose of influencing decisions or actions with respect to the subject matter of this Agreement or any other aspect of the other Party’s business. Each
Party acknowledges that no employee of the other Party shall have authority to give any direction, either written or oral, relating to the making of any commitment by a Party or its agents to any Third Party in violation of terms of this
provision.” 
  

	 	7.	In Article 9, Section 9.1.2 and Section 9.1.3, to the extent that each such Sections relate to the Parties’ rights under Joint Research Program Technology, shall each be deemed to be amended to reflect
the same rights under Joint Research Program Technology as provided under Section 4.1.3, as herein amended. 

  

	 	8.	In Article 9, Change of Control, a new subsection, Section 9.5.l(c), shall be added as follows: 

“9.5.1(c) In the event of a Change of Control of [†].” 

  
 [†] DESIGNATES PORTIONS OF THIS
DOCUMENT THAT HAVE BEEN OMITTED PURSUANT TO A REQUEST FOR CONFIDENTIAL TREATMENT FILED SEPARATELY WITH THE COMMISSION 

	 	9.	This First Amendment shall be read and interpreted as being additive to and in harmony with and not as replacing or contradicting the terms and conditions of the Agreement except to the extent modified by the terms and
conditions of this First Amendment. All capitalized terms used and not otherwise defined in this First Amendment shall have the meanings set forth in the Agreement. 

 

	 	10.	All other terms and conditions to the Agreement remain unchanged and in full force and effect except to the extent modified by the terms and conditions of this First Amendment. 

 

	 	11.	The Agreement, as modified by this First Amendment, contains the entire understanding of the Parties with respect to the subject matter contemplated herein. 

 

	 	12.	As of the First Amendment Effective Date, all terms contained in this First Amendment shall be in full force and effect; provided, however, that the modifications to Sections 1.25, 1.78 (solely with respect to
the terms therein providing that written modifications to the Research Plan and/or Research Program be approved by the JSC in writing), 2.1 (solely with respect to the terms therein 

  
 [†] DESIGNATES PORTIONS OF THIS
DOCUMENT THAT HAVE BEEN OMITTED PURSUANT TO A REQUEST FOR CONFIDENTIAL TREATMENT FILED SEPARATELY WITH THE COMMISSION 

 

 
 providing that written modifications to the Research Plan and/or Research Program be approved by the JSC in
writing), 2.5.4, 2.5.6, 2.5.7 and 2.9(h) (solely with respect to the terms therein providing that written modifications to the Research Plan and/or Research Program be approved by the JSC in writing) of the Agreement as set forth above in this
Amendment shall be effective as of the Effective Date. 
 IN WITNESS WHEREOF, the Parties have caused this First Amendment to be executed by
their duly-authorized representatives, effective as set forth above. 
  

									
	Essex Chemie AG	 		 	Xenon Pharmaceuticals Inc.
					
	BY:	 	 /s/ Christoph Brombacher
	 		 	BY:	 	 /s/ KAREN G. CORRAINI

		 	Christoph Brombacher	 		 		 	KAREN G. CORRAINI
					
	TITLE:	 	Director	 		 	TITLE:	 	GENERAL COUNSEL & CORPORATE SECRETARY
					
	DATE:	 	July 20, 2012	 		 	DATE:	 	26 - July - 2012

  
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 C Confidential 

EXHIBIT 1.78 
 Research Plan 

Research Plan dated November 24, 2009 

[†] Target Drug Discovery Plan dated February 2, 2010 

[†] Target Validation Plan dated March 16, 2011 

[†] Target Drug Discovery Plan dated March 28, 2011 

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 C Confidential 

EXHIBIT 1.78A 
 Extended Research
Plan 
 [†] Target Validation Plan dated June 19, 2012 

[†] Research Plan dated June 19, 2012 

[†] Research Plan dated June 19, 2012 

[†] Research Plan dated June 19, 2012 

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