Second Amended and Restated Investor Rights Agreement



 Exhibit 10.1 
 HYPERION THERAPEUTICS, INC. 
 SECOND AMENDED AND RESTATED INVESTOR RIGHTS
AGREEMENT 







 HYPERION THERAPEUTICS, INC. 

SECOND AMENDED AND RESTATED INVESTOR RIGHTS AGREEMENT 

THIS SECOND AMENDED AND
RESTATED INVESTOR RIGHTS AGREEMENT (the “Agreement”) is entered into as of the 29th day of June, 2009, by and among Hyperion Therapeutics, Inc., a Delaware corporation (the
“Company”) and the investors listed on Exhibit A hereto, referred to hereinafter as the “Investors” and each individually as an “Investor.” 

RECITALS 
 WHEREAS, the Investors are purchasing shares of the Company’s Series C-2 Preferred Stock (the “Series C-2 Stock”), pursuant
to that certain Series C-1 and Series C-2 Preferred Stock Purchase Agreement, of even date herewith (the “Purchase Agreement”, and such financing, the “Financing”); 

WHEREAS, the obligations in the Purchase Agreement are conditioned upon the execution and delivery of this
Agreement; 
 WHEREAS, certain of the Investors (the “Prior
Investors”) are holders of Common Stock and the Company’s Series C-1 Preferred Stock (“Series C-1 Stock” and, together with the Series C-2 Stock, “Preferred Stock”), by virtue of the
conversion of the Company’s prior Series A and Series B Preferred Stock into Common Stock, and the conversion of a Company bridge loan into Series C-1 Stock, which conversions (collectively, the “Recapitalization”) were
completed prior to the Financing or pursuant to the Purchase Agreement; 
 WHEREAS, in connection with the
consummation of the Financing, the Company and the Investors have agreed to the registration rights, information rights, and other rights as set forth below; 
 WHEREAS, the Prior Investors and the Company are parties to an Amended and Restated Investor Rights Agreement dated August 24, 2007 (the “Prior
Agreement”); and 
 WHEREAS, the parties to the Prior Agreement desire to amend and restate
the Prior Agreement in its entirety as set forth herein and to accept the rights and covenants hereof in lieu of their rights and covenants under the Prior Agreement. 
 NOW, THEREFORE, in consideration of these premises and for other good and valuable consideration, the receipt and sufficiency of which are hereby
acknowledged, the parties hereto agree that the Prior Agreement shall be superseded and replaced in its entirety by this Agreement, and the parties hereto further agree as follows: 
 SECTION 1. GENERAL. 
 1.1 Amendment and Restatement of Prior
Agreement. The Prior Agreement is hereby amended in its entirety and restated herein. Such amendment and restatement is effective 

  
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upon the execution of this Agreement by the Company and the holders of a majority of the Registrable Securities outstanding as of the date of this Agreement. Upon such execution, all provisions
of, rights granted and covenants made in the Prior Agreement are hereby waived, released and restated in their entirety as set forth herein and shall have no further force or effect, including, without limitation, all rights of first refusal and any
notice period associated therewith otherwise applicable to the transactions contemplated by the Purchase Agreement. 
 1.2
Definitions. As used in this Agreement the following terms shall have the following respective meanings: 

(a) “Exchange Act” means the Securities Exchange Act of 1934, as amended. 

(b) “Form S-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. 

(c) “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 2.9 hereof. 
 (d) “Initial Offering” means the Company’s first firm commitment underwritten public offering of its Common Stock registered under the Securities Act. 

(e) “Qualifying IPO” refers to an underwritten public offering pursuant to an effective
registration statement under the Securities Act covering the offer and sale of Common Stock for the account of the Company in which (i) the per share price is not less than $4.74 (as adjusted for any stock splits, combinations,
recapitalizations, and the like), and (ii) the gross cash proceeds to the Company (before underwriting discounts, commissions and fees) are at least $35,000,000. 

(f) “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. 

(g) “Registrable Securities” means (a) Common Stock of the Company issuable or issued
upon conversion of the Shares, (b) any Common Stock 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, and (c) Common Stock of the Company issued in the “Recapitalization” (as defined in the Purchase Agreement) to former holders of the Company’s Series A Preferred
Stock and Series B Preferred Stock. Notwithstanding the foregoing, Registrable Securities shall not include any securities (i) sold by a person to the public either pursuant to a registration statement or Rule 144, (ii) sold in a private
transaction in which the transferor’s rights under Section 2 of this Agreement are not assigned or (iii) that are Common Stock of the Company issued pursuant to a conversion of Preferred Stock pursuant to Article IV.G.5(p) of
the Company’s Amended and Restated Certificate of Incorporation. 

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

(i) “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, but excluding underwriting discounts and commissions,
reasonable fees and disbursements of a single special counsel for the selling 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). 
 (j) “SEC”
or “Commission” means the Securities and Exchange Commission. 
 (k)
“Securities Act” shall mean the Securities Act of 1933, as amended. 
 (l)
“Selling Expenses” shall mean all underwriting discounts and selling commissions applicable to the sale. 
 (m) “Shares” shall mean shares of Preferred Stock held from time to time by the Investors listed on EXHIBIT A hereto and their permitted assigns.

 (n) “Special Registration Statement” shall mean (i) a registration
statement relating to any employee benefit plan or (ii) with respect to any corporate reorganization or transaction under Rule 145 of the Securities Act, any registration statements related to the issuance or resale of securities issued in such
a transaction or (iii) a registration related to stock issued upon conversion of debt securities. 
 SECTION 2. REGISTRATION.

 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, (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. It is agreed that the Company will not require opinions of counsel for 

  
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transactions made pursuant to Rule 144, except in unusual circumstances. After its Initial Offering, the Company will not require any transferee pursuant to Rule 144 to be bound by the terms of
this Agreement if the shares so transferred do not remain Registrable Securities hereunder following such transfer. 
 (b) Notwithstanding the provisions of subsection (a) above, no such restriction shall apply to a transfer by a Holder that is (A) a partnership transferring to its partners or former
partners in accordance with partnership interests, (B) a corporation transferring to a wholly-owned subsidiary or a parent corporation that owns all of the capital stock of the Holder, (C) a limited liability company transferring to its
members or former members in accordance with their interest in the limited liability company, or (D) an individual transferring to the Holder’s family member or trust for the benefit of an individual Holder; provided that in each
case the transferee will agree in writing to be subject to the terms of this Agreement to the same extent as if he were an original Holder hereunder. 
 (c) Each certificate representing Shares or Registrable Securities shall be stamped or otherwise imprinted with legends 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. 
 THE SALE, PLEDGE, HYPOTHECATION OR TRANSFER OF THE SECURITIES REPRESENTED
BY THIS CERTIFICATE IS SUBJECT TO THE TERMS AND CONDITIONS OF A CERTAIN INVESTOR RIGHTS AGREEMENT BY AND BETWEEN THE STOCKHOLDER AND THE COMPANY. COPIES OF SUCH AGREEMENT MAY BE OBTAINED UPON WRITTEN REQUEST TO THE SECRETARY OF THE COMPANY.

 (d) The Company shall be obligated to reissue promptly unlegended certificates at the request of any
Holder thereof if the Company has completed its Initial Offering and 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 and legend, provided that the second legend listed above shall be removed only at such time as the Holder of such certificate is no-longer subject to any
restrictions hereunder. 

  
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 (e) 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. 

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 at least a majority of the Registrable Securities then outstanding
(the “Initiating Holders”) that the Company file a registration statement under the Securities Act covering a registration, then the Company shall, within ten days of the receipt thereof, give written notice of such request
to all Holders, and subject to the limitations of this Section 2.2, use its best efforts to effect the registration under the Securities Act of all Registrable Securities that all Holders request to be registered within ninety days of
receipt of such request. 
 (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 the Holders of a majority of the Registrable Securities held by all 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 that 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). Any Registrable Securities
excluded or withdrawn from such underwriting shall be withdrawn from the registration. In no event shall any Registrable Securities to be offered for sale be excluded from such underwriting unless all other securities are first excluded. 

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

 (i) prior to the expiration of the restrictions on transfer set forth in Section 2.11
following the Initial Offering; 
 (ii) after the Company has effected one registration pursuant to this
Section 2.2, and such registration has been declared or ordered effective (unless such registration 

  
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is withdrawn prior to the sale of the securities being registered because of material adverse developments at the Company); 

(iii) if within thirty 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 file a registration statement for a public offering, other than pursuant to a Special Registration Statement, within ninety days on which the
Registrable Securities covered by the request of the Initiating Holder will be included, provided that the Company uses reasonable efforts to file a registration statement within such time and provided, further, that the Company shall
not register any securities for the account of itself or any other stockholder during such ninety day period (other than pursuant to a Special Registration Statement); or 

(iv) 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 stockholders for such registration to be effected at such time, in which event the Company shall
have the right to defer the filing of the registration statement for a period of not more than sixty days after receipt of the request of the Initiating Holders under this Section 2.2; provided, that such right to delay a request
shall be exercised by the Company not more than once in any twelve month period. 
 2.3 Piggyback Registrations.
When the company proposes to register for sale any of its equity securities on a registration statement under the Securities Act (whether for its own account or otherwise and including, but not limited to, registration statements relating to
secondary offerings of securities of the Company, but excluding Special Registration Statements), the Company will promptly (but in no event later than twenty days prior to the filing of such registration statement) give written notice to the
Holders of such proposed registration and shall 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 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 of 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 include Registrable Securities 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 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 and the Holders of a majority of the
Registrable Securities proposing to distribute their 

  
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Registrable Securities through such underwriting. Notwithstanding any other provision of this 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 Holders who have elected to participate in the registration; and third, to any stockholder of the Company (other than a Holder) on a pro rata basis; provided, however, that no such reduction shall reduce the amount of securities of
the selling Holders included in the registration below 30% of the total amount of securities included in such registration, unless such offering is the Initial Offering, in which event the underwriters may determine that the amount of securities of
the selling Holders included in the registration may be less than 30% of the total amount of securities included in the registration. In no event shall any Registrable Securities to be offered for sale be excluded from such underwriting unless all
other securities are first excluded. 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 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, limited liability company or
corporation, the partners, retired partners, members, retired members and stockholders of such Holder, or the estates and family members of any such partners, retired partners, members and retired members 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 whether or not any Holder has elected to include securities in such registration, and shall promptly
notify any Holder that has elected to include shares in such registration of such termination or withdrawal. 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 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 any successor to Form S-3) 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 
 (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 days after receipt of such written notice from the Company; provided, however, that the 

  
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Company shall not be obligated to effect any such registration, qualification or compliance pursuant to this Section 2.4: 

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

(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 $1,000,000; 
 (iii) if within thirty 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 days which offering will include the shares proposed to be sold by such Holder or Holders, other than pursuant to a Special Registration Statement, provided that the Company
uses reasonable efforts to file a registration statement within such time; 
 (iv) 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
stockholders for such Form S-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 registration statement for a period of not more than sixty 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 twice in any twelve month period and provided, further, that the Company shall
not register any securities for the account of itself or any other stockholder during such sixty day period (other than pursuant to a Special Registration Statement); or 

(v) 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 unless the Company is already qualified to do business or subject to service of process in that jurisdiction and except as may be required by the
Securities Act. 
 (c) Subject to the foregoing, the Company shall file a Form S-3 registration statement
covering the Registrable Securities and other securities so requested to be registered as soon as practicable after receipt of the 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 Section 2.2. 
 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, 2.3 or 2.4 herein shall be borne by the Company,
including, without limitation, the expenses of one special counsel for the Investors. 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. Each of the Company and the Initiating Holders shall pay for one-half of expenses of any registration proceeding begun pursuant to Section 2.2, the request of which has been subsequently
withdrawn by the Initiating 

  
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Holders, provided that the Company shall pay all expenses of any such registration if (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 66% of Registrable Securities agree to deem such registration to have been effected as of the date of such withdrawal for purposes of determining whether the
Company shall be obligated pursuant to Section 2.2(c), as applicable, to undertake any subsequent registration, in which event such right shall be forfeited by all Holders. If the Holders are required to pay a portion of 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 such registration shall not be deemed to have been effected for purposes of determining whether the Company shall be obligated pursuant to
Section 2.2(c), as applicable, to undertake any subsequent registration. 
 2.6 Obligations of the Company.
Subject to the provisions of Sections 2.2(c)(iv) and 2.4(b)(iv), 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 twenty four
months or, if earlier, until the Holder or Holders have completed the distribution related thereto; provided, however, that at any time, upon written notice to the participating Holders and for a period not to exceed sixty days thereafter (the
“Suspension Period”), the Company may delay the filing or effectiveness of any registration statement or suspend the use or effectiveness of any registration statement (and the Initiating Holders hereby agree not to offer or
sell any Registrable Securities pursuant to such registration statement during the Suspension Period) if the Company reasonably believes that there is or may be in existence material nonpublic information or events involving the Company, the failure
of which to be disclosed in the prospectus included in the registration statement could result in a Violation (as defined below). In the event that the Company shall exercise its right to delay or suspend the filing or effectiveness of a
registration hereunder, the applicable time period during which the registration statement is to remain effective shall be extended by a period of time equal to the duration of the Suspension Period. The Company may extend the Suspension Period for
an additional consecutive sixty days with the consent of the holders of a majority of the Registrable Securities registered under the applicable registration statement, which consent shall not be unreasonably withheld. If so directed by the Company,
all Holders registering shares under such registration statement shall (i) not offer to sell any Registrable Securities pursuant to the registration statement during the period in which the delay or suspension is in effect after receiving
notice of such delay or suspension; and (ii) use their best efforts to deliver to the Company (at the Company’s expense) all copies, other than permanent file copies then in such Holders’ possession, of the prospectus relating to such
Registrable Securities current at the time of receipt of such notice. Notwithstanding the foregoing, the Company shall not be required to file, cause to become effective or maintain the effectiveness of any registration statement other than a
registration statement on Form S-3 that contemplates a distribution of securities on a delayed or continuous basis pursuant to Rule 415 under the Securities Act. 

  
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 (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 subsection (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 and take any and all other actions that may be reasonably necessary or advisable to enable each selling Holder
to consummate the disposition in such jurisdictions of the Registrable Securities; 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 unless the Company is already qualified to do business or subject to service of process in that jurisdiction and except as may be required by the Securities Act. 

(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. 
 (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. 

  
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 (h) Use its reasonable efforts to cause all such Registrable
Securities to be listed on each securities exchange on which securities of the Company of the same class are listed. 
 (i) Provide a transfer agent and registrar for all such Registrable Securities not later than the effective date of the relevant registration statement. 

(j) In the case of an underwritten offering, (i) enter into such agreements (including underwriting agreements
in customary form) and (ii) take all such other actions as the Holders of a majority of the Registrable Securities being sold or the underwriters, if any, reasonably request in order to expedite or facilitate the disposition of such Registrable
Securities. 
 (k) Make available for inspection by any selling Holder, any underwriter participating in
any disposition pursuant to a registration statement, and any attorney, accountant or other agent retained by the Holder or underwriter, all financial and other records, pertinent corporate documents and properties of the Company, and cause the
Company’s officers, directors, employees and independent accountants to supply all information reasonably requested by any such selling Holder, underwriter, attorney, accountant or agent in connection with such registration statement;
provided, however, that any records, information or documents that are furnished by the Company and that are non-public shall be used only in connection with such registration and shall be kept strictly confidential by any selling Holder of
Registrable Securities except to the extent disclosure of such records, information or documents is required by written order of a court or other governmental authority having jurisdiction. 

(l) If requested, use its reasonable best efforts to cause to be delivered, immediately prior to the pricing of any
underwritten offering, immediately prior to effectiveness of each registration statement (and, in the case of an underwritten offering, at the time of closing of the sale of Registrable Securities pursuant thereto), letters from the Company’s
independent registered public accountants addressed to the selling Holders and each underwriter, if any, stating that such accountants are independent public accountants within the meaning of the Securities Act and the applicable rules and
regulations adopted by the SEC thereunder, and otherwise in customary form and covering such financial and accounting matters as are customarily covered by letters of the independent registered public accountants delivered in connection with primary
underwritten public offerings. 
 (m) Make generally available to its stockholders a consolidated earnings
statement (which need not be audited) for the twelve months beginning after the effective date of a registration statement as soon as reasonably practicable after the end of such period, which earnings statement shall satisfy the requirements of an
earning statement under Section 11(a) of the Securities Act. 
 (n) Make senior executives of the
Company reasonably available to assist the underwriter(s) with respect to, and accompany the underwriter(s) on the so-called “road show” in connection with the marketing efforts for, and the distribution and sale of Registrable Securities
pursuant to a registration statement. 
 (o) Notify each selling Holder and the underwriter(s), if any:


  
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 (i) when the registration statement, any pre-effective amendment, the
prospectus or any prospectus supplement or post-effective amendment to the registration statement has been filed and, with respect to the registration statement or any post-effective amendment, when the same has become effective; 

(ii) of the notification to the Company by the SEC of its initiation of any proceeding with respect to the issuance
by the SEC of any stop order suspending the effectiveness of the registration statement; and 
 (iii) of
the receipt by the Company of any notification with respect to the suspension of the qualification of any Registrable Securities for sale under the applicable securities or blue sky laws of any jurisdiction. 

2.7 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 Section 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 reasonably 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 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. 

2.8 Indemnification. In the event any Registrable Securities are included in a registration statement under Sections 2.2,
2.3 or 22.4: 
 (a) To the extent permitted by law, the Company will indemnify and hold
harmless each Holder, the partners, members, 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 or incorporated by reference therein, 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


  
 12.









 
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 reimburse each such Holder, partner, member, 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 2.8(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, conditioned or delayed nor shall the Company be liable in any such case for any
such loss, claim, damage, liability or action to the 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, member, officer, director, underwriter or controlling person of such Holder. 

(b) To the extent permitted by law, each Holder will (severally and not jointly), if Registrable Securities held by
such Holder are included in the securities as to which such registration, qualification 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 of the following statements:
(i) any untrue statement or alleged untrue statement of a material fact contained in such registration statement or incorporated by reference therein, 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 (collectively, a “Holder Violation”), in each case to the extent (and only to the extent) that such Holder 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 reimburse 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 Holder Violation; provided, however, that the indemnity agreement contained in this Section 2.8(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 2.8 plus any contribution under this
Section 2.8 exceed the net proceeds from the offering received by such Holder. 

  
 13.









 (c) Promptly after receipt by an indemnified party under this
Section 2.8 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 2.8,
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 thereof 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 shall relieve such indemnifying party of any liability to the indemnified
party under this Section 2.8 to the extent, and only to the extent, prejudicial to its ability to defend such action, 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 2.8. 
 (d) If the
indemnification provided for in this Section 2.8 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) or Holder 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 plus any indemnification by such Holder exceed the net proceeds from the offering received by such Holder.

 (e) The obligations of the Company and Holders under this Section 2.8 shall survive
completion of any offering of Registrable Securities in a registration statement and, with respect to liability arising from an offering to which this Section 2.8 would apply that is covered by a registration filed before termination of
this Agreement, such termination. 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. 
 2.9 Assignment of Registration Rights. The rights to cause the Company to register Registrable Securities pursuant to this Section 2 may be assigned by a Holder to a transferee or
assignee of Registrable Securities in a private transaction (for so long as such shares remain 

  
 14.









 
Registrable Securities) that (a) is a subsidiary, parent, general partner, limited partner, retired partner, member or retired member, of a Holder that is a corporation, partnership or
limited liability company, (b) is a Holder’s family member or trust for the benefit of an individual Holder, (c) acquires at least 50% of the Registrable Securities issued to the original Holder thereof (as adjusted for any stock
splits, combinations, recapitalizations, and the like) or, if less than such amount, all of the Registrable Securities held by the transferring Holder, or (d) is an entity affiliated by common control (or other related entity) with such Holder;
provided, however, (i) the transferor shall promptly 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 to be subject to all restrictions set forth in this Agreement. 
 2.10 Limitation on
Subsequent Registration Rights. After the date of this Agreement, the Company shall not enter into any agreement with any holder or prospective holder of any securities of the Company that would grant such holder rights to demand the
registration of shares of the Company’s capital stock, or to include such shares in a registration statement that are more favorable to the holder or prospective holder than the rights provided to Investors in this Agreement. 

2.11 “Market Stand-Off” Agreement. 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 Stock (or other securities) of the Company held by such Holder (other than those included in the
registration) the 180-day period following the effective date of the Initial Offering; provided, that, all officers and directors of the Company and holders of at least 1% of the Company’s voting securities are bound by and have entered into
similar agreements. The obligations described in this Section 2.11 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 transaction on Form S-4 or similar forms that may be promulgated in the future. 
 2.12
Agreement to Furnish Information. Each Holder agrees to execute and deliver such other agreements as may be reasonably requested by the Company or the underwriter that are consistent with the Holder’s obligations under
Section 2.11 or that are necessary to give further effect thereto. In addition, if requested by the Company or the representative of the underwriters of Common Stock (or other securities) of the Company, each Holder shall provide, within
ten 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 Section 2.11 and this Section 2.12 shall not apply to a Special Registration Statement. The Company may impose stop-transfer instructions with respect to the shares of Common Stock
(or other securities) subject to the foregoing restriction until the end of said 180 day period. Each Holder agrees that any transferee of any shares of Registrable Securities shall be bound by Sections 2.11 and 2.12. The underwriters
of the Company’s stock are intended third party beneficiaries of Sections 2.11 and 2.12 and shall have the right, power and authority to enforce the provisions hereof as though they were a party hereto. 

  
 15.









 2.13 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 filed with the Commission; and such other reports and documents as a Holder may reasonably request in connection with availing itself of any rule or regulation of the SEC allowing
it to sell any such securities without registration. 
 2.14 Termination of Registration Rights. The right of any Holder
to request registration or inclusion of Registrable Securities in any registration pursuant to Section 2.2, Section 2.3, or Section 2.4 hereof shall terminate upon the earlier of: (i) the date five years
following a Qualifying IPO; or (ii) such time as all Registrable Securities of the Company issuable or issued upon conversion of the Shares held by and issuable to such Holder (and its affiliates) may be sold pursuant to Rule 144 during any
ninety day period. Upon such termination, such shares shall cease to be Registrable Securities hereunder for all purposes. 
 SECTION 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 U.S. generally accepted accounting principles consistently applied (except as noted therein or as disclosed to the recipients thereof),
and will set aside on its books all such proper accruals and reserves as shall be required under U.S. generally accepted accounting principles consistently applied. 

(b) The Company shall furnish to each Investor holding at least 50,000 shares of Registrable Securities, as soon as
practicable after the end of each fiscal year of the Company, and in any event within ninety days thereafter, 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 prepared in accordance with U.S. generally accepted accounting principles consistently applied (except as noted therein or as disclosed to the recipients thereof) and setting forth in each case in comparative form the figures for
the previous fiscal year, all in reasonable detail. Such financial statements shall be audited, certified and accompanied by a report and 

  
 16.









 
opinion thereon by independent public accountants selected by the Company’s Board of Directors. 
 (c) The Company will furnish each Investor holding at least 50,000 shares of Registrable Securities: (i) at least thirty days prior to the beginning of each fiscal year an annual budget and
operating plans for such fiscal year (and as soon as available, any subsequent written revisions thereto); (ii) within thirty days following the end of each month, unaudited financial statements of the Company for such month; and
(iii) within thirty days following the end of each fiscal quarter, unaudited financial statements for such fiscal quarter. 

3.2 Inspection Rights. Each Investor holding at least 50,000 shares of Registrable Securities shall have the right to visit and
inspect any of the properties of the Company or any of its subsidiaries, and to discuss the affairs, finances and accounts of the Company or any of its subsidiaries with its officers, and to review such information as is reasonably requested all at
such reasonable times and as often as may be reasonably requested; provided, however, that the Company shall not be obligated under this Section 3.2 with respect to a competitor of the Company or with respect to information which
the Board of Directors determines in good faith is confidential or attorney-client privileged and should not, therefore, be disclosed. 
 3.3 Termination of Information and Inspection Rights. The rights set forth in Section 3.1(b), 3.1(c), and 3.2 hereof shall terminate with respect to each Investor upon the
earlier of (a) a Qualifying IPO or (b) the date that the registration rights for Shares no longer apply pursuant to Section 2.14. 
 3.4 Reservation of Common Stock. The Company will at all times reserve and keep available, solely for issuance and delivery upon the conversion of the Preferred Stock, all Common Stock issuable
from time to time upon such conversion. 
 3.5 Stock Vesting. Unless otherwise approved by the Board of Directors,
including the approval of at least one director elected exclusively by the holders of the Company’s Preferred Stock, all stock options and other stock equivalents issued after the date of this Agreement to employees, directors, consultants and
other service providers shall be subject to vesting as follows: (a) 25% of such stock shall vest at the end of the first year following the earlier of the date of issuance or such person’s services commencement date with the company, and
(b) 75% of such stock shall vest over the remaining three years. Any unvested shares of restricted stock shall be repurchaseable by the Company at the lower of the exercise price of such shares or the fair market value of such shares at the
time of repurchase upon termination of employment for any reason. 
 3.6 [Reserved.] 

3.7 Director and Officer Insurance. As soon as practicable after the date hereof, the Company shall obtain and at all times
maintain in full force and effect director and officer liability insurance providing for at least $5,000,000 in coverage. 

3.8 Proprietary Information and Inventions Agreement. The Company shall require all employees and consultants to execute and
deliver a Proprietary Information and 

  
 17.









 
Inventions Agreement substantially in a form approved by the Company’s counsel or Board of Directors. 
 3.9 Directors’ Liability and Indemnification. The Company’s Certificate of Incorporation and Bylaws shall provide (a) for elimination of the liability of directors to the maximum
extent permitted by law and (b) for indemnification of directors for acts on behalf of the Company to the maximum extent permitted by law. In addition, the Company shall enter into and at all times maintain indemnification agreements with each
of its directors (and their affiliated funds) to indemnify such directors (and their affiliated funds) to the maximum extent permissible under applicable law. 
 3.10 Qualified Small Business. The Company will use reasonable efforts to comply with the reporting and recordkeeping requirements of Section 1202 of the Internal Revenue Code of 1986, as
amended (the “Code”), any regulations promulgated thereunder and any similar state laws and regulations, and agrees not to repurchase any stock of the Company if such repurchase would cause the Shares not to so qualify as
“Qualified Small Business Stock,” so long as the Company’s Board of Directors determines that it is in the best interests of and not unduly burdensome to the Company to comply with the provisions of Section 1202 of the Code.

 3.11 FIRPTA. Upon a written request by an Investor, the Company shall provide such Investor with a written statement
as to whether such Investor’s interest in the Company constitutes a United States real property interest. The Company’s determination shall comply with the requirements of Treasury Regulation Section 1.897-2(h)(1) or any successor
regulation, and the Company shall provide timely notice to the Internal Revenue Service, in accordance with and to the extent required by Treasury Regulation Section 1.897-2(h)(2) or any successor regulation, that such statement has been made.
The Company’s written statement to such Investor shall be delivered to such Investor promptly after such Investor’s written request therefor. The Company’s obligation to deliver the written statement under this
Section 3.11 shall survive the termination of this Agreement, but only with respect to periods prior to such termination. 
 3.12 Termination of Covenants. All covenants of the Company contained in Section 3 of this Agreement other than the provisions of Section 3.7 and 3.11 shall expire and
terminate as to each Investor upon the earlier of (i) the effective date of the registration statement pertaining to an Initial Offering or (ii) upon a “Liquidation Event” as defined in the Company’s
Certificate of Incorporation as in effect from time to time. 
 3.13 Reimbursement of Costs. The Company shall reimburse
all reasonable costs incurred by non-employee directors in attending Board of Directors meetings and other activities in support of Company business. 
 3.14 Director Equity Compensation. The Company shall not provide any equity compensation to a non-employee director, or to an employee director solely in connection with such person’s service
as a director, without the affirmative vote of the Board of Directors, including at least 80% of the directors nominated by the holders of Series C-1 Preferred Stock and Series C-2 Preferred Stock. 

  
 18.









 3.15 Employment Offers. The Company shall obtain the approval of the Board of
Directors of the Company prior to making any oral or written offers of employment to any employees until such time as a compensation committee of the Board of Directors of the Company is formed, and thereafter only upon the approval of such
compensation committee (provided that approval of oral or written offers of employment in respect of positions at the vice-president (or equivalent), or more senior, level shall be subject to the approval of the full Board of Directors). 

3.16 Board Committees. The Company shall establish the following committees of the Board of Directors: (a) compensation
committee and (b) audit committee. At least one director nominated pursuant to Section 1.2(c) of the Second Amended and Restated Voting Agreement, of even date herewith, by and among the parties hereto, shall have the right to be a member
of each such committee. 
 3.17 Transfers in Violation. The Company shall not, without the approval of the holders of at
least 66% percent of the outstanding Registrable Securities, permit the transfer on its stock register of any Company securities in violation of any right of an Investor contained in Section 4 of this Agreement. Should any transfer of
Company securities take place in violation of such rights, the Company shall not treat the transferee of such securities as an owner of Company securities, nor shall it accord such transferee any right to vote such Company securities or pay an such
transferee any dividends upon such Company securities. 
 SECTION 4. RIGHTS OF FIRST REFUSAL. 

4.1 Subsequent Offerings. Subject to applicable securities laws, each 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 Equity Securities excluded by Section 4.6 hereof. Each
Investor’s pro rata share is equal to the ratio of (a) the number of shares of Common Stock owned by such Investor and (A) issued or issuable to such Investor upon conversion of any Shares, (B) issued in connection with
the Recapitalization or (C) issued or issuable upon the exercise of any outstanding warrants or options of such Investor immediately prior to the issuance of such Equity Securities to (b) the total number of shares of the Company’s
outstanding Common Stock (including all shares of Common Stock issued or issuable upon conversion of the Shares or upon the exercise of any outstanding warrants or options) immediately prior to the issuance of such Equity Securities. The term
“Equity Securities” shall mean (i) any Common Stock, Preferred Stock or other security of the Company, (ii) any security convertible into or exercisable or exchangeable for, with or without consideration, any Common
Stock, Preferred Stock 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 Stock, Preferred Stock 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 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 Investor shall have forty-five 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 

  
 19.









 
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 Investor who would cause the Company to be in violation of applicable federal securities laws by virtue of such offer or sale. 
 4.3 Issuance of Equity Securities to Other Persons. If not all of the Investors elect to purchase their pro rata share of the Equity Securities, then the Company shall promptly notify in
writing the Investors who do so elect and shall offer such Investors the right to acquire such unsubscribed shares on a pro rata basis. Each such Investor shall have five days after receipt of such notice to notify the Company of its election
to purchase all or a portion thereof of the unsubscribed shares. The Company shall have thirty days thereafter to sell the Equity Securities in respect of which the Investors’ rights were not exercised, at a price not lower and upon general
terms and conditions not materially more favorable to the purchasers thereof than specified in the Company’s notice to the Investors pursuant to Section 4.2 hereof. If the Company has not sold such Equity Securities within thirty
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 Investors in the manner provided above. 

4.4 Termination and Waiver of Rights of First Refusal. The rights of first refusal established by this Section 4 shall
not apply to, and shall terminate immediately prior to the effective date of the registration statement pertaining to a Qualifying IPO. Notwithstanding Section 5.5 hereof, the rights of first refusal established by this
Section 4 may be amended, or any provision waived with and only with the written consent of the Company and the Investors holding at least 66% of the Registrable Securities held by all Investors. 

4.5 Assignment of Rights of First Refusal. The rights of first refusal of each Investor under this Section 4 may be
assigned to the same parties, subject to the same restrictions, as any transfer of registration rights pursuant to Section 2.9. 
 4.6 Excluded Securities. The rights of first refusal established by this Section 4 shall have no application to any of the following Equity Securities: 

(a) shares of Common Stock issued upon conversion of the Shares; 

(b) any shares of Common Stock and/or options, warrants or other Common Stock purchase rights issued 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 that are approved by the Board of Directors of the Company; 

(c) shares of Common Stock issued pursuant to the exercise of convertible securities outstanding as of the date of
this Agreement; 
 (d) any securities issued in connection with any stock split, stock dividend or
recapitalization by the Company; 
 (e) any securities issued pursuant to any equipment loan or leasing
arrangement, real property leasing arrangement or debt financing from a bank or similar financial 

  
 20.









 
or lending institution or other providers of goods and services to the Company approved by the Board of Directors of the Company, in an aggregate amount not to exceed 25,000; 

(f) any securities issued in connection with strategic transactions involving the Company and other entities,
including (i) joint ventures, manufacturing, marketing or distribution arrangements or (ii) technology transfer or development arrangements; provided that, in the reasonable judgment of the Board of Directors, the primary purpose of
the issuance of shares therein is not for equity financing; 
 (g) Common Stock issued pursuant to a
Qualifying IPO; 
 (h) any securities issued in any other transaction in which exemption from the
provisions of this Section 4.6 is approved by an affirmative vote of Investors holding at least 66% of the Registrable Securities held by all Investors; and 

(i) securities issued in connection with a bona fide business acquisition by the Company, whether by merger,
consolidation, sale of assets, sale or exchange of stock or otherwise, as approved by the Board of Directors of the Company. 
 SECTION 5.
MISCELLANEOUS. 
 5.1 Governing Law. This Agreement shall be governed by and construed under the laws of the State of
California in all respects as such laws are applied to agreements among California residents entered into and to be performed entirely within California, without reference to conflicts of laws principles thereof. 

5.2 Successors and Assigns. Except as otherwise expressly provided herein, the provisions hereof shall inure to the benefit of,
and be binding upon the parties hereto and their respective successors, assigns, heirs, executors, and administrators and shall inure 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. 

5.3 Entire Agreement. This Agreement, the Exhibits and Schedules hereto, the Purchase Agreement 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 oral or written representations, warranties,
covenants and agreements except as specifically set forth herein and therein. Each party expressly represents and warrants that it is not relying on any oral or written representations, warranties, covenants or agreements outside of this Agreement,
including, without limitation, those in the Prior Agreement. 
 5.4 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,


  
 21.









 
and this Agreement shall be construed as if such invalid, illegal or unenforceable provision had never been contained herein. 

5.5 Amendment and Waiver. 
 (a) Except as otherwise expressly provided, this Agreement may be amended or modified, and the obligations of the Company and the rights of the Holders under this Agreement may be waived, only upon
the written consent of the Company and the Investors holding at least 66% of the Registrable Securities held by all Investors; provided, however, that any amendment, modification, or waiver with respect to Section 4 shall require the
written consent of the Company and the Investors holding at least 77% of the Registrable Securities held by all Investors if, and only if, such amendment, modification, or waiver materially and adversely affects any holder of Series C-2 Stock as
compared to the holders of Series C-1 Stock. 
 (b) 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 stock as maintained by or on behalf of the Company. 

5.6 Delays or Omissions. It is agreed that no delay or omission to exercise any right, power, or remedy accruing to any party,
upon any breach, default or noncompliance by another party 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 party’s part of any breach, default or noncompliance under the Agreement or
any waiver on such party’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 any party, shall be cumulative and not alternative. 
 5.7 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 days after having been sent by registered or certified mail, return receipt requested, postage prepaid, or upon actual receipt by the party to be notified, if earlier, or (d) one
day after deposit with a nationally recognized overnight courier, 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 Exhibit A hereto or at such other address as such party may designate by ten days advance written notice to the other parties hereto. 
 5.8 Attorneys’ Fees. In the event that any suit or action is instituted under or in relation to this Agreement, including without limitation to enforce any provision in this Agreement, the
prevailing party in such dispute shall be entitled to recover from the losing party all fees, costs and expenses of enforcing any right of such prevailing party under or with respect to this Agreement, including without limitation, such reasonable
fees and expenses of attorneys and accountants, which shall include, without limitation, all fees, costs and expenses of appeals. 

  
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 5.9 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. 
 5.10
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. 
 5.11 Aggregation of Stock. All shares of Registrable Securities held or acquired by affiliated entities or persons or persons or entities under common management or control shall be aggregated
together for the purpose of determining the availability of any rights under this Agreement. 
 5.12 Pronouns. All
pronouns contained herein, and any variations thereof, shall be deemed to refer to the masculine, feminine or neutral, singular or plural, as to the identity of the parties hereto may require. 

5.13 Termination. This Agreement shall terminate and be of no further force or effect upon the date ten years following the
closing of the Qualifying IPO that results in the conversion of all outstanding shares of Preferred Stock. 
 [THIS SPACE
INTENTIONALLY LEFT BLANK] 

  
 23.









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

			
	 COMPANY
  

HYPERION THERAPEUTICS, INC.

		
	Signature:	 	/s/ Donald J. Santel
	Print Name:	 	Donald J. Santel
	Title:	 	Chief Executive Officer
	Address:	 	 601 Gateway Blvd., Suite 200

South San Francisco, CA 94080


 [Signature Page for Hyperion Therapeutics, Inc. Second Amended and Restated Investor Rights Agreement] 







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

					
	 INVESTORS:
  

NEW ENTERPRISE ASSOCIATES 12, LIMITED
PARTNERSHIP

	 By: NEA Partners 12, Limited Partnership

	 By: NEA 12 GP, LLC

		
	By:	 	 /s/ Charles W. Newhall III,Manager




[Signature Page for Hyperion Therapeutics, Inc. Second Amended and Restated Investor Rights Agreement] 







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

					
	 INVESTORS:
  

SOFINNOVA VENTURE PARTNERS VII, L.P.

	 By:
	 	 Sofinnova Management VII, LLC

Its General Partner

		
	By:	 	 /s/ James Healy

		 	 James Healy, Managing General Partner




[Signature Page for Hyperion Therapeutics, Inc. Second Amended and Restated Investor Rights Agreement] 







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

					
	 INVESTORS:
  

Highland Capital Partners VII Limited Partnership
  

By: Highland Management Partners VII Limited Partnership its General Partner

 
 By: Highland Management Partners VII, LLC,

its General Partner

		
	By:	 	/s/ Bijan Salehizadeh
		 	Authorized Manager
	
	 Highland Capital Partners VII-B Limited Partnership

 
 By: Highland Management Partners VII Limited Partnership, its General
Partner
  
 By: Highland Management Partners VII, LLC,

its General Partner

		
	By:	 	/s/ Bijan Salehizadeh
		 	Authorized Manager
	
	 Highland Capital Partners VII-C Limited Partnership

 
 By: Highland Management Partners VII Limited Partnership, its General
Partner
  
 By: Highland Management Partners VII, LLC,

its General Partner

		
	By:	 	/s/ Bijan Salehizadeh
		 	Authorized Manager
	
	 Highland Enterpreneurs’ Fund VII Limited Partnership

 
 By: Highland Management Partners VII Limited Partnership, its General
Partner
  
 By: Highland Management Partners VII, LLC,

its General Partner

		
	By:	 	/s/ Bijan Salehizadeh
		 	Authorized Manager


 [Signature Page for Hyperion Therapeutics, Inc. Second Amended and Restated Investor Rights
Agreement] 







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

					
	 INVESTORS:
  

BAY CITY CAPITAL FUND V, L.P.
 By: Bay City Capital Management V LLC
 Its: General Partners

By: Bay City Capital LLC
 Its: Manager

 

		
	By:	 	 /s/ Fred Craves

	 Name:
	 	 Fred Craves

	Title:	 	Managing Director
	
	 BAY CITY CAPITAL FUND V
CO-INVESTMENT FUND, L.P.
 By: Bay City Capital
Management V LLC
 Its: General Partners

By: Bay City Capital LLC

Its: Manager

 

		
	By:	 	 /s/ Fred Craves

	 Name:
	 	 Fred Craves

	Title:	 	Managing Director


 [Signature Page for Hyperion Therapeutics, Inc. Second Amended and Restated Investor Rights
Agreement] 







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

			
	 COMPANY
  

HYPERION THERAPEUTICS, INC.

		
	Signature:	 	/s/ Donald J. Santel
	Print Name:	 	Donald J. Santel
	Title:	 	Chief Executive Officer
	Address:	 	 601 Gateway Blvd., Suite 200

South San Francisco, CA 94080


 [Signature Page for Hyperion Therapeutics, Inc. Second Amended and Restated Investor Rights Agreement] 







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

					
	 INVESTORS:
  

PANORAMA CAPITAL, L.P.

		
	 By:
	 	 Panorama Capital Management, LLC
 Its General Partner

		
	By:	 	/s/ Rodney A. Ferguson
		 	Name:	 	Rodney A. Ferguson
		 	Title:	 	Managing Director


 [Signature Page
for Hyperion Therapeutics, Inc. Second Amended and Restated Investor Rights Agreement] 







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

			
	INVESTORS:
	
	WRF Capital (Washington Research Foundation)
		
	Signature:	 	/s/ Ronald S. Howell
	Print Name: Ronald S. Howell
	Title: Chief Executive Officer


 [Signature Page for Hyperion Therapeutics, Inc. Second Amended and Restated Investor Rights Agreement] 







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

			
	INVESTORS:
	
	GC&H INVESTMENTS LLC
		
	Signature:	 	/s/ Mark A. Royer
	Print Name: Mark A. Royer
	Title: CFO


 [Signature Page
for Hyperion Therapeutics, Inc. Second Amended and Restated Investor Rights Agreement] 







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

	
	INVESTORS:
	
	/s/ Klara Dickinson
	KLARA DICKINSON
	
	/s/ Chris Rivera
	CHRIS RIVERA
	
	/s/ Michael Rivera
	MICHAEL RIVERA
	
	/s/ Christine Nash
	CHRISTINE NASH


 [Signature Page for Hyperion Therapeutics, Inc. Second Amended and Restated Investor Rights Agreement] 







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

	
	INVESTOR:
	
	/s/ George Jue
	GEORGE JUE


 [Signature Page for Hyperion Therapeutics, Inc. Second Amended and Restated Investor Rights Agreement] 







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

	
	INVESTOR:
	
	/s/ Mark Blumling
	MARK BLUMLING


 [Signature Page for Hyperion Therapeutics, Inc. Second Amended and Restated Investor Rights Agreement] 







 SCHEDULE OF INVESTORS 
 Bay City Capital Fund V, L.P. 
 Bay City Capital Fund V Co-Investment Fund, L.P. 

Panorama Capital, L.P. 
 New Enterprise
Associates 12, LP 
 Highland Capital Partners VII Limited Partnership 
 Highland Capital Partners VII-B Limited Partnership 
 Highland Capital Partners VII-C Limited
Partnership 
 Highland Entrepreneurs’ Fund VII Limited Partnership 
 Sofinnova Venture Partners VII, L.P. 
 WRF Capital (Washington Research Foundation) 

GC&H Investments LLC 
 Chris Rivera

 Klara Dickinson 
 Michael Rivera

 Christine Nash 
 George Jue

 Mark Blumling