Document:

EX-4.2

 Exhibit 4.2 

MYOKARDIA, INC. 
 SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 
 THIS SECOND AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT
(“Agreement”) is made as of April 20, 2015, by and among MyoKardia, Inc., a Delaware corporation (the “Company”), and each investor listed on Schedule A hereto, each of which is referred to in this
Agreement as an “Investor.” 
 RECITALS: 

WHEREAS, certain of the Investors (the “Existing Investors”) hold shares of the Company’s Series A Preferred
Stock and/or Series A-1 Preferred Stock and possess registration rights, information rights, rights of first offer, and other rights pursuant to an Amended and Restated Investors’ Rights Agreement dated as of August 1, 2014, by and among
the Company and such Existing Investors (as amended, the “Prior Agreement”); 
 WHEREAS, the Existing Investors a
party thereto are holders of a majority of the Registrable Securities of the Company (as defined in the Prior Agreement), and desire to amend and restate the Prior Agreement in its entirety and to accept the rights created pursuant to this Agreement
in lieu of the rights granted to them under the Prior Agreement; 
 WHEREAS, the Company and certain Investors are parties to the
Series B Preferred Stock Purchase Agreement of even date herewith (the “Purchase Agreement”), under which certain of the Company’s and such Investors’ obligations are conditioned upon the execution and delivery of this
Agreement by such Investors, Existing Investors holding at least such amount of the Registrable Securities (as defined in the Prior Agreement) as would be required to amend and restate the Prior Agreement and the Company; and 

WHEREAS, in order to induce the Company to enter into the Purchase Agreement and to induce the Investors a party to the Purchase
Agreement to invest funds in the Company pursuant to the Purchase Agreement, the Investors and the Company hereby agree that this Agreement shall govern the rights of the Investors to cause the Company to register shares of Common Stock issuable to
the Investors, to receive certain information from the Company, and to participate in certain future equity offerings by the Company, and shall govern certain other matters as set forth in this Agreement. 

AGREEMENT: 

NOW, THEREFORE, the Existing Investors hereby agree that the Prior Agreement shall be amended and restated in its entirety by this
Agreement, and the parties to this Agreement further agree as follows: 
 1. Definitions. 

For purposes of this Agreement: 

1.1 “Affiliate” means, with respect to any specified Person, any other Person who or which, directly or indirectly, controls,
is controlled by, or is under common control with such specified Person, including, without limitation, any general partner, officer, director or manager, member or employee of such Person and any venture capital fund now or hereafter existing that
is controlled by or under common control with one or more general partners or managing members of, or shares the same management company or investment advisor with, such Person. 

 1.2 “Board of Directors” means the Company’s Board of Directors. 

1.3 “Collaboration Agreement” means that certain License and Collaboration Agreement entered into by and between the Company
and the Strategic Investor on the date of this Agreement, as the same may be amended and/or restated from time to time. 
 1.4
“Common Stock” means shares of the Company’s common stock, par value $0.0001 per share. 
 1.5 “Company’s
Certificate of Incorporation” means the Third Amended and Restated Certificate of Incorporation of the Company, as amended and/or restated from time to time. 

1.6 “Damages” means any loss, damage, claim or liability (joint or several) to which a party hereto may become subject under
the Securities Act, the Exchange Act, or other federal or state law, insofar as such loss, damage, claim or liability (or any action in respect thereof) arises out of or is based upon (i) any untrue statement or alleged untrue statement of a
material fact contained in any registration statement of the Company, including any preliminary prospectus or final prospectus contained therein or any amendments or supplements thereto; (ii) an 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 indemnifying party (or any of its agents or Affiliates) 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. 

1.7 “Derivative Securities” means any securities or rights convertible into, or exercisable or exchangeable for (in each
case, directly or indirectly), Common Stock, including options and warrants. 
 1.8 “Exchange Act” means the
Securities Exchange Act of 1934, as amended, and the rules and regulations promulgated thereunder. 
 1.9 “Excluded
Registration” means (i) a registration relating to the sale of securities to employees of the Company or a subsidiary pursuant to a stock option, stock purchase, or similar plan; (ii) a registration relating to an SEC Rule 145
transaction; or (iii) a registration on any form that does not include substantially the same information as would be required to be included in a registration statement covering the sale of the Registrable Securities. 

1.10 “Form S-1” means such form under the Securities Act as in effect on the date
hereof or any successor registration form under the Securities Act subsequently adopted by the SEC. 
 1.11 “Form S-3” means such form under the Securities Act as in effect on the date hereof or any registration form under the Securities Act subsequently adopted by the SEC that permits incorporation of substantial
information by reference to other documents filed by the Company with the SEC. 
 1.12 “GAAP” means generally accepted
accounting principles in the United States. 
 1.13 “Holder” means any holder of Registrable Securities who is a party to
this Agreement. 

  
 2 

 1.14 “Immediate Family Member” means a child, stepchild, grandchild, parent,
stepparent, grandparent, spouse, sibling, mother-in-law, father-in-law, son-in-law, daughter-in-law, brother-in-law, or sister-in-law, including adoptive relationships, of a natural person referred to herein. 

1.15 “Initiating Holders” means, collectively, Holders who properly initiate a registration request under this Agreement.

 1.16 “IPO” means the Company’s first underwritten public offering of its Common Stock under the Securities Act.

 1.17 “Key Employee” means any executive-level employee (including division director and vice president-level positions).

 1.18 “Major Investor” means any Investor that, individually or together with such Investor’s Affiliates, holds at
least 1,000,000 shares of Registrable Securities (subject to appropriate adjustment in the event of any stock dividend, stock split, combination or other similar recapitalization); provided, however, that solely for purposes of
Section 4 of this Agreement the Strategic Investor shall not be deemed a Major Investor (regardless of the number of shares of Registrable Securities held) until the date on which the Strategic Investor (or any of its Affiliates) has
purchased shares of Registrable Securities with an aggregate value at least equal to the Minimum Purchase Amount (under and as defined in the Collaboration Agreement), rounded down as permitted pursuant to Section 9.3 of the Collaboration
Agreement. 
 1.19 “New Securities” means, collectively, equity securities of the Company, whether or not currently
authorized, as well as rights, options, or warrants to purchase such equity securities, or securities of any type whatsoever that are, or may become, convertible or exchangeable into or exercisable for such equity securities, other than Exempted
Securities (as such term is defined in the Company’s Certificate of Incorporation). 
 1.20 “Person” means any
individual, corporation, partnership, trust, limited liability company, association or other entity. 
 1.21 “Preferred
Stock” means the Series A Preferred Stock, the Series A-1 Preferred Stock and/or the Series B Preferred Stock. 
 1.22
“Purchase Agreement” means the Series B Preferred Stock Purchase Agreement dated as of the date of this Agreement, as may be amended from time to time, by and among the Company and certain of the Investors set forth on Exhibit A
thereto. 
 1.23 “QPO” shall have the meaning set forth in the Company’s Certificate of Incorporation. 

1.24 “Registrable Securities” means (i) the Common Stock issuable or issued upon conversion of the Preferred Stock held
from time to time by the Investors or their permitted assigns to whom rights are properly transferred; (ii) any Common Stock, or any Common Stock issued or issuable (directly or indirectly) upon conversion and/or exercise of any other
securities of the Company, held by the Investors or acquired by the Investors after the date hereof; and (iii) any Common Stock issued as (or issuable upon the conversion or exercise of any warrant, right, or other security that is issued as) a
dividend or other distribution with respect to, or in exchange for or in replacement of, the shares referenced in clauses (i) and (ii) above; excluding in all cases, however, any Registrable Securities sold by a Person
in a transaction in which the applicable rights under this Agreement are not assigned pursuant to 

  
 3 

 
Section 6.1, and excluding for purposes of Section 2 any shares for which registration rights have terminated pursuant to Section 2.13 of this Agreement and
(iv) any other Common Stock subsequently acquired by an Investor, without duplication of any securities referred to in clauses (i) through (iii) hereof. 

1.25 “Registrable Securities then outstanding” means the number of shares at a point in time determined by adding the number
of shares of outstanding Common Stock that are Registrable Securities at such time and the number of shares of Common Stock issuable (directly or indirectly) at such time pursuant to then exercisable and/or convertible securities that are
Registrable Securities. 
 1.26 “Restricted Securities” means the securities of the Company required to bear the legend set
forth in Section 2.12(b) hereof. 
 1.27 “SEC” means the Securities and Exchange Commission. 

1.28 “SEC Rule 144” means Rule 144 promulgated by the SEC under the Securities Act, or any successor provisions. 

1.29 “SEC Rule 145” means Rule 145 promulgated by the SEC under the Securities Act, or any successor provisions. 

1.30 “Securities Act” means the Securities Act of 1933, as amended, and the rules and regulations promulgated thereunder.

 1.31 “Selling Expenses” means all underwriting discounts, selling commissions, and stock transfer taxes applicable to
the sale of Registrable Securities, and fees and disbursements of counsel for any Holder, except for the fees and disbursements of the Selling Holder Counsel borne and paid by the Company as provided in Section 2.6. 

1.32 “Selling Holder Counsel” shall have the meaning assigned to it in Section 2.6. 

1.33 “Series A Directors” means the directors of the Company that have been solely designated by the holders of record of the
Series A Preferred Stock pursuant to the Company’s Certificate of Incorporation, the Stockholders Agreement or otherwise. 
 1.34
“Series A Preferred Stock” means shares of the Company’s Series A Preferred Stock, par value $0.0001 per share. 

1.35 “Series A-1 Preferred Stock” means shares of the Company’s Series A-1 Preferred Stock, par value $0.0001 per share.

 1.36 “Series B Preferred Stock” means shares of the Company’s Series B Preferred Stock, par value $0.0001 per
share. 
 1.37 “Specified Preferred Stock” means any shares of Preferred Stock purchased by the Strategic Investor (or one
of its designated Affiliates) pursuant to Section 9.3(a)(ii), Section 9.3(b)(i) or Section 9.3(b)(ii) of the Collaboration Agreement. 

1.38 “Stockholders Agreement” means the Second Amended and Restated Stockholders Agreement, dated as of the date hereof and
as may be amended from time to time, by and among the Company, the Investors, and Key Holders (as defined therein). 

  
 4 

 1.39 “Strategic Investor” means Aventis Inc. 

1.40 “Strategic Investor Director” means the directors of the Company that have been designated by the Strategic Investor
pursuant to the Stockholders Agreement or otherwise. 
 2. Registration Rights. The Company covenants and agrees as follows: 

2.1 Demand Registration. 

(a) Form S-1 Demand. Beginning upon the earlier of (i) five (5) years after the date of this Agreement or (ii) six
(6) months after the effective date of the registration statement for the IPO, if the Company receives a request from Holders of at least twenty-five percent (25%) of the Registrable Securities then outstanding that the Company file a Form
S-1 registration statement with respect to at least twenty-five percent (25%) of the Registrable Securities then outstanding, having the anticipated aggregate offering amount of at least $3 million, then the Company shall (i) within ten
(10) days after the date such request is given, give notice thereof (the “Demand Notice”) to all Holders other than the Initiating Holders; and (ii) as soon as practicable, and in any event within sixty (60) days
after the date such request is given by the Initiating Holders, file a Form S-1 registration statement under the Securities Act covering all Registrable Securities that the Initiating Holders requested to be registered and any additional Registrable
Securities requested to be included in such registration by any other Holders, as specified by notice given by each such Holder to the Company within twenty (20) days after the date the Demand Notice is given, and in each case, subject to the
limitations of Section 2.1(c) and Section 2.3. 
 (b) Form S-3 Demand. If at any time when it is eligible to
use a Form S-3 registration statement, the Company receives a request from Holders of at least twenty-five percent (25%) of the Registrable Securities then outstanding that the Company file a Form S-3 registration statement with respect to
outstanding Registrable Securities of such Holders having an anticipated aggregate offering amount, net of Selling Expenses, of at least $1 million, then the Company shall (i) within ten (10) days after the date such request is given, give
a Demand Notice to all Holders other than the Initiating Holders; and (ii) as soon as practicable, and in any event within forty-five (45) days after the date such request is given by the Initiating Holders, file a Form S-3 registration
statement under the Securities Act covering all Registrable Securities requested to be included in such registration by any other Holders, as specified by notice given by each such Holder to the Company within twenty (20) days after the date
the Demand Notice is given, and in each case, subject to the limitations of Section 2.1(c) and Section 2.3. 
 (c)
Notwithstanding the foregoing obligations, if the Company furnishes to Holders requesting a registration pursuant to this Section 2.1 a certificate signed by the Company’s chief executive officer or other most senior executive
officer then in office stating that in the good faith judgment of the Board of Directors it would be materially detrimental to the Company and its stockholders for such registration statement to either become effective or remain effective for as
long as such registration statement otherwise would be required to remain effective, because such action would (i) materially interfere with a significant acquisition, corporate reorganization, or other similar transaction involving the
Company; (ii) require premature disclosure of material information that the Company has a bona fide business purpose for preserving as confidential; or (iii) render the Company unable to comply with requirements under the Securities Act or
Exchange Act, then the Company shall have the right to defer taking action with respect to such filing, and any time periods with respect to filing or effectiveness thereof shall be tolled correspondingly, for a period of not more than one hundred
twenty (120) days after the request of the Initiating Holders is given; provided, however, that the Company may not invoke this right more than once in any twelve (12) month period, nor shall the Company invoke this right more than
twice in all periods; and provided further that the Company shall not register any securities for its own account or that of any other stockholder during either one hundred twenty (120) day period other than an Excluded Registration.

  
 5 

 (d) The Company shall not be obligated to effect, or to take any action to effect, any
registration pursuant to Section 2.1(a) (i) if it delivers notice to the Holders within thirty (30) days after any registration request of its intent to file a registration statement for a public offering within ninety
(90) days; (ii) during the period that is one hundred eighty (180) days after commencing a Company-initiated registration; (iii) after the Company has effected two (2) registrations pursuant to Section 2.1(a); or
(iv) if the Initiating Holders propose to dispose of shares of Registrable Securities that may be immediately registered on Form S-3 pursuant to a request made pursuant to Section 2.1(b). The Company shall not be obligated to
effect, or to take any action to effect, any registration pursuant to Section 2.1(b) if the Company has effected two (2) registrations pursuant to Section 2.1(b) within the twelve (12) month period immediately
preceding the date of such request. A registration shall not be counted as “effected” for purposes of this Section 2.1(d) until such time as the applicable registration statement has been declared effective by the SEC, unless
the Initiating Holders withdraw their request for such registration (other than as a result of a material adverse change to the Company), elect not to pay the registration expenses therefor, and forfeit their right to one demand registration
statement pursuant to Section 2.6, in which case such withdrawn registration statement shall be counted as “effected” for purposes of this Section 2.1(d). 

2.2 Company Registration. If the Company proposes to register (including, for this purpose, a registration effected by the Company for
stockholders other than the Holders) any of its Common Stock under the Securities Act in connection with the public offering of such securities solely for cash (other than in an Excluded Registration), the Company shall, at such time, promptly give
each Holder notice of such registration. Upon the request of each Holder given within twenty (20) days after such notice is given by the Company, the Company shall, subject to the provisions of Section 2.3, cause to be registered
all of the Registrable Securities that each such Holder has requested to be included in such registration. The Company shall have the right to terminate or withdraw any registration initiated by it under this Section 2.2 before the
effective date of such registration, whether or not any Holder has elected to include Registrable Securities in such registration. The expenses (other than Selling Expenses) of such withdrawn registration shall be borne by the Company in accordance
with Section 2.6. 
 2.3 Underwriting Requirements. 

(a) If, pursuant to Section 2.1, 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 Section 2.1, and the Company shall include such information in the Demand Notice. The underwriter(s) will be selected by the
Company and shall be reasonably acceptable to a majority in interest of the Initiating Holders. In such event, the right of any Holder to include such Holder’s 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
(together with the Company as provided in Section 2.4(e)) enter into an underwriting agreement in customary form with the underwriter(s) selected for such underwriting. Notwithstanding any other provision of this Section 2.3,
if the managing underwriter(s) advise the Initiating Holders in writing that marketing factors require a limitation on the number of shares to be underwritten, then the Initiating Holders shall so advise all Holders that otherwise would be
underwritten pursuant hereto, and the number of Registrable Securities that may be included in the underwriting shall be allocated among such Holders, including the Initiating Holders, in proportion (as nearly as practicable) to the number of
Registrable Securities owned by each, or in such other proportion as shall mutually be agreed to by all such selling Holders; provided, however, that the number of Registrable Securities held

  
 6 

 
by the Holders to be included in such underwriting shall not be reduced unless all other securities are first entirely excluded from the underwriting. To facilitate the allocation of shares in
accordance with the above provisions, the Company or the underwriters may round the number of shares allocated to any Holder to the nearest 100 shares. 

(b) In connection with any offering involving an underwriting of shares of the Company’s capital stock pursuant to
Section 2.2, the Company shall not be required to include any of the Registrable Securities in such underwriting unless the Holders accept the terms of the underwriting as agreed upon between the Company and its underwriters, and then
only in such quantity as the underwriters in their sole discretion determine will not jeopardize the success of the offering by the Company. If the total number of securities, including Registrable Securities, requested by Holders to be included in
such offering exceeds the number of securities to be sold (other than by the Company) that the underwriters in their reasonable discretion determine is compatible with the success of the offering, then the Company shall be required to include in the
offering only that number of such securities, including Registrable Securities, which the underwriters and the Company in their sole discretion determine will not jeopardize the success of the offering. If the underwriters determine that less than
all of the Registrable Securities requested to be registered can be included in such offering, then the Registrable Securities that are included in such offering shall be allocated among the selling Holders in proportion (as nearly as practicable)
to the number of Registrable Securities owned by each selling Holder or in such other proportions as shall mutually be agreed to by all such selling Holders. To facilitate the allocation of shares in accordance with the above provisions, the Company
or the underwriters may round the number of shares allocated to any Holder to the nearest 100 shares. Notwithstanding the foregoing, in no event shall (i) the number of Registrable Securities included in the offering be reduced unless all other
securities (other than securities to be sold by the Company) are first entirely excluded from the offering, or (ii) the number of Registrable Securities included in the offering be reduced below thirty percent (30%) of the total number of
securities included in such offering, unless such offering is the IPO, in which case the selling Holders may be excluded further if the underwriters make the determination described above and no other stockholder’s securities are included in
such offering. For purposes of the provision in this Section 2.3(b) concerning apportionment, for any selling Holder that is a partnership, limited liability company, or corporation, the partners, members, retired partners, retired
members, stockholders, and Affiliates of such Holder, or the estates and Immediate Family Members of any such partners, retired partners, members, and retired members and any trusts for the benefit of any of the foregoing Persons, shall be deemed to
be a single “selling Holder,” and any pro rata reduction with respect to such “selling Holder” shall be based upon the aggregate number of Registrable Securities owned by all Persons included in such “selling Holder,”
as defined in this sentence. 
 (c) For purposes of Section 2.1, a registration shall not be counted as “effected”
if, as a result of an exercise of the underwriter’s cutback provisions in Section 2.3(a) and (b), less than the total number of Registrable Securities that Holders have requested to be included in such registration statement are
actually included. 
 2.4 Obligations of the Company. Whenever required under this Section 2 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 its commercially 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 a period of up to one hundred twenty (120) days or, if earlier, until the distribution contemplated in the registration statement has been completed; provided,
however, that (i) such one hundred twenty (120) day period shall be extended for a period of time equal to the period the Holder refrains, at the request of an underwriter of 

  
 7 

 
the Company, from selling any securities included in such registration, and (ii) in the case of any registration of Registrable Securities on Form S-3 that are intended to be offered on a
continuous or delayed basis, subject to compliance with applicable SEC rules, such one hundred twenty (120) day period shall be extended for up to one hundred eighty (180) days, if necessary, to keep the registration statement effective
until all such Registrable Securities are sold; 
 (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 Securities Act in order to enable the disposition of all securities covered by such registration statement; 

(c) furnish to the selling Holders such numbers of copies of a prospectus, including a preliminary prospectus, as required by the Securities
Act, and such other documents as the Holders may reasonably request in order to facilitate their disposition of their Registrable Securities; 

(d) use its commercially 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 selling Holders; provided that the Company shall not be required 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 subject to service in such 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 underwriter(s) of such offering; 
 (f) use its commercially reasonable efforts to cause all such Registrable
Securities covered by such registration statement to be listed on a national securities exchange or trading system and each securities exchange and trading system (if any) on which similar securities issued by the Company are then listed; 

(g) provide a transfer agent and registrar for all Registrable Securities registered pursuant to this Agreement and provide a CUSIP number
for all such Registrable Securities, in each case not later than the effective date of such registration; 
 (h) promptly make available
for inspection by the selling Holders, any underwriter(s) participating in any disposition pursuant to such registration statement, and any attorney or accountant or other agent retained by any such underwriter or selected by the selling Holders,
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
seller, underwriter, attorney, accountant, or agent, in each case, as necessary or advisable to verify the accuracy of the information in such registration statement and to conduct appropriate due diligence in connection therewith; 

(i) notify each selling Holder, promptly after the Company receives notice thereof, of the time when such registration statement has been
declared effective or a supplement to any prospectus forming a part of such registration statement has been filed; and 
 (j) after such
registration statement becomes effective, notify each selling Holder of any request by the SEC that the Company amend or supplement such registration statement or prospectus. 

  
 8 

 2.5 Furnish Information. It shall be a condition precedent to the obligations of the
Company to take any action pursuant to this Section 2 with respect to the Registrable Securities of any selling Holder that such Holder shall furnish to the Company such information regarding itself, the Registrable Securities held by
it, and the intended method of disposition of such securities as is reasonably required to effect the registration of such Holder’s Registrable Securities. 

2.6 Expenses of Registration. All expenses (other than Selling Expenses) incurred in connection with registrations, filings, or
qualifications pursuant to Section 2, including all registration, filing, and qualification fees; printers’ and accounting fees; fees and disbursements of counsel for the Company; and the reasonable fees and disbursements of one
counsel for the selling Holders (“Selling Holder Counsel”) selected by the Holders of a majority of the Registrable Securities, shall be borne and paid by the Company; provided, however, that the Company shall not be required
to pay for any expenses of any registration proceeding begun pursuant to Section 2.1 if the registration request is subsequently withdrawn at the request of the Holders of a majority of the Registrable Securities to be registered (in
which case all selling Holders shall bear such expenses pro rata based upon the number of Registrable Securities that were to be included in the withdrawn registration), unless the Holders of a majority of the Registrable Securities agree to forfeit
their right to one registration pursuant to Section 2.1(a) or Section 2.1(b), as the case may be; provided further that if, at the time of such withdrawal, the Holders have learned of a material adverse change in the
condition, business, or prospects of the Company from that known to the Holders at the time of their request and have withdrawn the request with reasonable promptness after learning of such information, then the Holders shall not be required to pay
any of such expenses and shall not forfeit their right to one registration pursuant to Section 2.1(a) or Section 2.1(b). All Selling Expenses relating to Registrable Securities registered pursuant to this
Section 2 shall be borne and paid by the Holders pro rata on the basis of the number of Registrable Securities registered on their behalf. 

2.7 Delay of Registration. No Holder shall have any right to obtain or seek an injunction restraining or otherwise delaying any
registration pursuant to this Agreement as the result of any controversy that might arise with respect to the interpretation or implementation of this Section 2. 

2.8 Indemnification. If any Registrable Securities are included in a registration statement under this Section 2: 

(a) To the extent permitted by law, the Company will indemnify and hold harmless each selling Holder, and the partners, members, officers,
directors, and stockholders of each such Holder; legal counsel and accountants for each such Holder; any underwriter (as defined in the Securities Act) for each 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 Damages, and the Company will pay to each such Holder, underwriter, controlling Person, or other aforementioned Person any legal or other expenses reasonably incurred thereby in
connection with investigating or defending any claim or proceeding from which Damages may result, as such expenses are incurred; provided, however, that the indemnity agreement contained in this Section 2.8(a) shall not apply to
amounts paid in settlement of any such claim or proceeding if such settlement is effected without the consent of the Company, which consent shall not be unreasonably withheld, nor shall the Company be liable for any Damages to the extent that they
arise out of or are based upon actions or omissions made in reliance upon and in conformity with written information furnished by or on behalf of any such Holder, underwriter, controlling Person, or other aforementioned Person expressly for use in
connection with such registration. 
 (b) To the extent permitted by law, each selling Holder, severally and not jointly, will indemnify
and hold harmless the Company, and each of its directors, each of its officers who has signed the registration statement, each Person (if any), who controls the Company within the meaning 

  
 9 

 
of the Securities Act, legal counsel and accountants for the Company, any underwriter (as defined in the Securities Act), any other Holder selling securities in such registration statement, and
any controlling Person of any such underwriter or other Holder, against any Damages, in each case only to the extent that such Damages arise out of or are based upon actions or omissions made in reliance upon and in conformity with written
information furnished by or on behalf of such selling Holder expressly for use in connection with such registration; and each such selling Holder will pay to the Company and each other aforementioned Person any legal or other expenses reasonably
incurred thereby in connection with investigating or defending any claim or proceeding from which Damages may result, as such expenses are incurred; provided, however, that the indemnity agreement contained in this Section 2.8(b)
shall not apply to amounts paid in settlement of any such claim or proceeding if such settlement is effected without the consent of the Holder, which consent shall not be unreasonably withheld; and provided further that in no event shall any
indemnity under this Section 2.8(b) exceed the proceeds from the offering received by such Holder (net of any Selling Expenses paid by such Holder), except in the case of fraud or willful misconduct by such Holder. 

(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) for which a party may be entitled to indemnification hereunder, such indemnified party will, if a claim in respect thereof is to be made against any indemnifying party under this Section 2.8, give the
indemnifying party notice of the commencement thereof. The indemnifying party shall have the right to participate in such action and, to the extent the indemnifying party so desires, participate jointly with any other indemnifying party to which
notice has been given, and to assume the defense thereof with counsel mutually satisfactory to the parties; provided, however, that an indemnified party (together with all other indemnified parties that may be represented without conflict by
one counsel) shall have the right to retain one separate 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 action. The failure to give 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 that such failure materially prejudices the indemnifying party’s ability to defend such action. The
failure to give 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) To provide for just and equitable contribution to joint liability under the Securities Act in any case in which either (i) any party
otherwise entitled to indemnification hereunder makes a claim for indemnification pursuant to this Section 2.8 but it is judicially determined (by the entry of a final judgment or decree by a court of competent jurisdiction and the
expiration of time to appeal or the denial of the last right of appeal) that such indemnification may not be enforced in such case, notwithstanding the fact that this Section 2.8 provides for indemnification in such case, or
(ii) contribution under the Securities Act may be required on the part of any party hereto for which indemnification is provided under this Section 2.8, then, and in each such case, such parties will contribute to the aggregate
losses, claims, damages, liabilities, or expenses to which they may be subject (after contribution from others) in such proportion as is appropriate to reflect the relative fault of each of the indemnifying party and the indemnified party in
connection with the statements, omissions, or other actions that resulted in such loss, claim, damage, liability, or expense, as well as to reflect any other relevant equitable considerations. The relative fault of the indemnifying party and of the
indemnified party shall be determined by reference to, among other things, whether the untrue or allegedly untrue statement of a material fact, or the omission or alleged omission of 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, however, that, in any such case, (x) no
Holder will be required to contribute any amount in 

  
 10 

 
excess of the public offering price of all such Registrable Securities offered and sold by such Holder pursuant to such registration statement, and (y) no Person guilty of fraudulent
misrepresentation (within the meaning of Section 11(f) of the Securities Act) will be entitled to contribution from any Person who was not guilty of such fraudulent misrepresentation; and provided further that in no event shall a
Holder’s liability pursuant to this Section 2.8(d), when combined with the amounts paid or payable by such Holder pursuant to Section 2.8(b), exceed the proceeds from the offering received by such Holder (net of any
Selling Expenses paid by such Holder), except in the case of willful misconduct or fraud by such Holder. 
 (e) Notwithstanding the
foregoing, to the extent that the provisions on indemnification and contribution contained in the underwriting agreement entered into in connection with the underwritten public offering are in conflict with the foregoing provisions, the provisions
in the underwriting agreement shall control; provided, however, that the failure of the underwriting agreement to provide for or address a matter provided for or addressed by the foregoing provisions shall not be a conflict, and, in such event, the
foregoing provisions shall control. 
 (f) Unless otherwise superseded by an underwriting agreement entered into in connection with the
underwritten public offering, the obligations of the Company and Holders under this Section 2.8 shall survive the completion of any offering of Registrable Securities in a registration under this Section 2, and otherwise
shall survive the termination of this Agreement. 
 2.9 Reports Under Exchange Act. With a view to making available to the Holders
the benefits of SEC Rule 144 and any other rule or regulation of the SEC that may at any time permit a Holder to sell securities of the Company to the public without registration or pursuant to a registration on Form
S-3, the Company shall: 
 (a) make and keep available adequate current public information, as
those terms are understood and defined in SEC Rule 144, at all times after the effective date of the registration statement filed by the Company for the IPO; 

(b) use commercially reasonable efforts to file with the SEC in a timely manner all reports and other documents required of the Company under
the Securities Act and the Exchange Act (at any time after the Company has become subject to such reporting requirements); and 
 (c)
furnish to any Holder, so long as the Holder owns any Registrable Securities, forthwith upon request (i) to the extent accurate, a written statement by the Company that it has complied with the reporting requirements of SEC Rule 144 (at any
time after ninety (90) days after the effective date of the registration statement filed by the Company for the IPO), the Securities Act, and the Exchange Act (at any time after the Company has become subject to such reporting requirements), or
that it qualifies as a registrant whose securities may be resold pursuant to Form S-3 (at any time after the Company so qualifies); (ii) a copy of the most recent annual or quarterly report of the Company
and such other reports and documents so filed by the Company; and (iii) such other information as may be reasonably requested in availing any Holder of any rule or regulation of the SEC that permits the selling of any such securities without
registration (at any time after the Company has become subject to the reporting requirements under the Exchange Act) or pursuant to Form S-3 (at any time after the Company so qualifies to use such form). 

2.10 Limitations on Subsequent Registration Rights. From and after the date of this Agreement, the Company shall not, without the prior
written consent of the Holders of a majority of the Registrable Securities then outstanding, enter into any agreement with any holder or prospective holder of any securities of the Company that would allow such holder or prospective holder
(i) to include such securities in any registration unless, under the terms of such agreement, such holder or prospective holder 

  
 11 

 
may include such securities in any such registration only to the extent that the inclusion of such securities will not reduce the number of the Registrable Securities of the Holders that are
included or (ii) to demand registration of any securities held by such holder or prospective holder. 
 2.11 “Market Stand-off” Agreement. Each Holder hereby agrees that, if required by the managing underwriter, it will not, during the period commencing on the date of the final prospectus relating to the IPO and ending on
the date specified by the Company and the managing underwriter (such period not to exceed one hundred eighty (180) days in the case of the IPO, which period may be extended upon the request of the managing underwriter for such longer period of
time as is necessary to enable such underwriter to issue a research report or to make a public appearance that relates to an earnings release or announcement by the Company within fifteen (15) days prior to or after the day that is one hundred
eighty (180) days after the effective date of the registration statement relating to such offering, but in any event not to exceed two hundred ten (210) days following the effective date of the registration statement relating to such
offering), (i) lend; offer; pledge; sell; contract to sell; sell any option or contract to purchase; purchase any option or contract to sell; grant any option, right, or warrant to purchase; or otherwise transfer or dispose of, directly or
indirectly, any shares of Common Stock or any securities convertible into or exercisable or exchangeable (directly or indirectly) for Common Stock held immediately before the effective date of the registration statement for such offering or
(ii) enter into any swap or other arrangement that transfers to another, in whole or in part, any of the economic consequences of ownership of such securities, whether any such transaction described in clause (i) or (ii) above is to
be settled by delivery of Common Stock or other securities, in cash, or otherwise. The foregoing provisions of this Section 2.11 shall apply only to the IPO, shall not apply to the sale of any shares to an underwriter pursuant to an
underwriting agreement, and shall be applicable to the Holders only if all officers, directors, and stockholders individually owning more than one percent (1%) of the Company’s outstanding Common Stock (after giving effect to conversion
into Common Stock of all outstanding Preferred Stock) are subject to the same restrictions. The underwriters in connection with such registration are intended third-party beneficiaries of this
Section 2.11 and shall have the right, power, and authority to enforce the provisions hereof as though they were a party hereto. Each Holder further agrees to execute such agreements as may be reasonably requested by the underwriters in
connection with such registration that are consistent with this Section 2.11 or that are necessary to give further effect thereto. The Company agrees to use its reasonable efforts to obtain the agreement of the managing underwriter to
periodic early releases of portions of the securities subject to such lock-up agreements upon the request of a Holder to such early release, provided that in the event of any early release, all Holders will be released on a pro rata basis from such
agreements. Any discretionary waiver or termination of the restrictions of any or all of such agreements by the Company or the underwriters shall apply pro rata to all Holders subject to such agreements, based on the number of shares subject to such
agreements. 
 2.12 Restrictions on Transfer. 

(a) The Preferred Stock and the Registrable Securities shall not be sold, pledged, or otherwise transferred, and the Company shall not
recognize any such sale, pledge, or transfer, except upon the conditions specified in this Agreement, which conditions are intended to ensure compliance with the provisions of the Securities Act. A transferring Holder will cause any proposed
purchaser, pledgee, or transferee of the Preferred Stock and the Registrable Securities held by such Holder to agree to take and hold such securities subject to the provisions and upon the conditions specified in this Agreement. 

(b) Each certificate or instrument representing (i) the Preferred Stock, (ii) the Registrable Securities, and (iii) any other
securities issued in respect of the securities referenced in clauses (i) and (ii), upon any stock split, stock dividend, recapitalization, merger, consolidation, or similar event, shall (unless otherwise permitted by the provisions of
Section 2.12(c)) be stamped or otherwise imprinted with a legend substantially in the following form: 

  
 12 

 THE SECURITIES REPRESENTED HEREBY HAVE BEEN ACQUIRED FOR INVESTMENT AND HAVE NOT BEEN REGISTERED
UNDER THE SECURITIES ACT OF 1933, AS AMENDED. SUCH SHARES MAY NOT BE SOLD, PLEDGED, OR TRANSFERRED IN THE ABSENCE OF SUCH REGISTRATION OR A VALID EXEMPTION FROM THE REGISTRATION AND PROSPECTUS DELIVERY REQUIREMENTS OF SAID ACT. 

THE SECURITIES REPRESENTED HEREBY MAY BE TRANSFERRED ONLY IN ACCORDANCE WITH THE TERMS OF AN AGREEMENT BETWEEN THE COMPANY AND THE STOCKHOLDER,
A COPY OF WHICH IS ON FILE WITH THE SECRETARY OF THE COMPANY. 
 The Holders consent to the Company making a notation in its records and giving instructions
to any transfer agent of the Restricted Securities in order to implement the restrictions on transfer set forth in this Section 2.12. 

(c) The holder of each certificate representing Restricted Securities, by acceptance thereof, agrees to comply in all respects with the
provisions of this Section 2. Before any proposed sale, pledge, or transfer of any Restricted Securities, unless there is in effect a registration statement under the Securities Act covering the proposed transaction, the Holder thereof
shall give notice to the Company of such Holder’s intention to effect such sale, pledge, or transfer. Each such notice shall describe the manner and circumstances of the proposed sale, pledge, or transfer in sufficient detail and, if reasonably
requested by the Company, shall be accompanied at such Holder’s expense by either (i) a written opinion of legal counsel who shall, and whose legal opinion shall, be reasonably satisfactory to the Company, addressed to the Company, to the
effect that the proposed transaction may be effected without registration under the Securities Act; (ii) a “no action” letter from the SEC to the effect that the proposed sale, pledge, or transfer of such Restricted Securities without
registration will not result in a recommendation by the staff of the SEC that action be taken with respect thereto; or (iii) any other evidence reasonably satisfactory to counsel to the Company to the effect that the proposed sale, pledge, or
transfer of the Restricted Securities may be effected without registration under the Securities Act, whereupon the Holder of such Restricted Securities shall be entitled to sell, pledge, or transfer such Restricted Securities in accordance with the
terms of the notice given by the Holder to the Company. The Company will not require such a legal opinion or “no action” letter (x) in any transaction in compliance with SEC Rule 144 or (y) in any transaction in which such Holder
distributes Restricted Securities to an Affiliate of such Holder for no consideration; provided that each transferee agrees in writing to be subject to the terms of this Section 2.12. Each certificate or instrument evidencing the
Restricted Securities transferred as above provided shall bear, except if such transfer is made pursuant to SEC Rule 144, the appropriate restrictive legend set forth in Section 2.12(b), except that such certificate shall not bear
such restrictive legend if, in the opinion of counsel for such Holder and the Company, such legend is not required in order to establish compliance with any provisions of the Securities Act. 

2.13 Termination of Registration Rights. The right of any Holder to request registration or inclusion of Registrable Securities in any
registration pursuant to Section 2.1 or Section 2.2 shall terminate upon the earliest to occur of: 
 (a) the
closing of a Sale of the Company (as defined in the Stockholders Agreement); 

  
 13 

 (b) following the IPO, such time as SEC Rule 144 or another similar exemption under the
Securities Act is available for the sale of all of such Holder’s shares without limitation during a three-month period without registration; and 

(c) the third (3rd) anniversary of the IPO. 

3. Information and Observer Rights. 

3.1 Delivery of Financial Statements. The Company shall deliver to each Major Investor the required items listed below: 

(a) as soon as practicable, but in any event within one hundred twenty (120) days after the end of each fiscal year of the Company,
(i) a balance sheet as of the end of such year, (ii) statements of income and of cash flows for such year, and (iii) a statement of stockholders’ equity as of the end of such year, all such financial statements audited and
certified by independent public accountants of nationally or regionally recognized standing selected by the Company and approved by the Board of Directors; 

(b) as soon as practicable but in any event within forty-five (45) days after the end of each of the first three (3) quarters of
each fiscal year of the Company, unaudited statements of income and of cash flows for such fiscal quarter, and an unaudited balance sheet and a statement of stockholders’ equity as of the end of such fiscal quarter, all prepared in accordance
with GAAP (except that such financial statements may (i) be subject to normal year-end audit adjustments and (ii) not contain all notes thereto that may be required in accordance with GAAP); 

(c) if requested in writing by a Major Investor with respect to a month within five (5) business days of the end of such month, as soon
as practicable, but in any event within thirty (30) days after the date of such request, an unaudited income statement and statement of cash flows for such month, and an unaudited balance sheet and statement of stockholders’ equity as of
the end of such month, all prepared in accordance with GAAP (except that such financial statements may (i) be subject to normal year-end audit adjustments and (ii) not contain all notes thereto that may be required in accordance with
GAAP); 
 (d) as soon as practicable, but in any event within forty-five (45) days after the end of each of the first three
(3) quarters of each fiscal year of the Company, a statement showing the number of shares of each class and series of capital stock and securities convertible into or exercisable for shares of capital stock outstanding at the end of the period,
the Common Stock issuable upon conversion or exercise of any outstanding securities convertible or exercisable for Common Stock and the exchange ratio or exercise price applicable thereto, and the number of shares of issued stock options and stock
options not yet issued but reserved for issuance, if any, all in sufficient detail as to permit each Major Investor to calculate their respective percentage equity ownership in the Company, and certified by the chief financial officer or chief
executive officer of the Company as being true, complete, and correct; 
 (e) as soon as practicable, but in any event thirty
(30) days before the end of each fiscal year, a budget and business plan for the next fiscal year (collectively, the “Budget”), approved by the Board of Directors and prepared on a monthly basis, including balance sheets,
income statements, and statements of cash flow for such months and, promptly after prepared, any other budgets or revised budgets prepared by the Company; and 

(f) such other information relating to the financial condition, business, or corporate affairs of the Company as any Major Investor (provided
that the Board of Directors has not 

  
 14 

 
reasonably determined that such Investor is a competitor of the Company) may from time to time reasonably request; provided, however, that the Company shall not be obligated under
this Section 3.1 to provide information (i) that the Company reasonably determines in good faith to be a trade secret or confidential information (unless covered by an enforceable confidentiality agreement, in form acceptable to the
Company) or (ii) the disclosure of which would adversely affect the attorney-client privilege between the Company and its counsel. 

(g) If, for any period, the Company has any subsidiary whose accounts are consolidated with those of the Company, then in respect of such
period the financial statements delivered pursuant to the foregoing sections shall be the consolidated and consolidating financial statements of the Company and all such consolidated subsidiaries. Each party to this Agreement hereby waives any
breach of the Prior Agreement as may have existed with respect to Section 3.1 of the Prior Agreement. 
 (h) Notwithstanding
anything else in this Section 3.1 to the contrary, the Company may cease providing the information set forth in this Section 3.1 during the period starting with the date thirty (30) days before the Company’s
good-faith estimate of the date of filing of a registration statement if it reasonably concludes it must do so to comply with the SEC rules applicable to such registration statement and related offering; provided that the Company’s covenants
under this Section 3.1 shall be reinstated at such time as the Company is no longer actively employing its commercially reasonable efforts to cause such registration statement to become effective. 

3.2 Inspection. The Company shall permit each Major Investor (provided that the Board of Directors has not reasonably determined that
such Investor is a competitor of the Company), at such Major Investor’s expense, to visit and inspect the Company’s properties; examine its books of account and records; and discuss the Company’s affairs, finances, and accounts with
its officers, during normal business hours of the Company as may be reasonably requested by the Major Investor; provided, however, that the Company shall not be obligated pursuant to this Section 3.2 to provide access to
any information that it reasonably considers to be a trade secret or confidential information (unless covered by an enforceable confidentiality agreement, in form acceptable to the Company) or the disclosure of which would adversely affect the
attorney-client privilege between the Company and its counsel. 
 3.3 Termination of Information Rights. 

The covenants set forth in Sections 3.1 and 3.2 shall terminate and be of no further force or effect (i) immediately before
the consummation of the IPO, (ii) when the Company first becomes subject to the periodic reporting requirements of Section 12(g) or 15(d) of the Exchange Act, or (iii) upon the closing of a Sale of the Company, as such term is defined
in the Stockholders Agreement. 
 3.4 Confidentiality. Each Investor agrees that such Investor will keep confidential and will not
disclose, divulge, or use for any purpose (other than to monitor its investment in the Company) any confidential information obtained from the Company pursuant to the terms of this Agreement (including notice of the Company’s intention to file
a registration statement), unless such confidential information (a) is known or becomes known to the public in general (other than as a result of a breach of this Section 3.4 by such Investor), (b) is or has been independently
developed or conceived by the Investor without use of the Company’s confidential information, or (c) is or has been made known or disclosed to the Investor by a third party without a breach of any obligation of confidentiality such third
party may have to the Company; provided, however, that an Investor may disclose confidential information (i) to its attorneys, accountants, consultants, and other professionals to the extent necessary to obtain their services in
connection with monitoring its investment in the Company; (ii) to any prospective purchaser of any Registrable Securities from such Investor, if such prospective purchaser agrees to be bound by the provisions of this Section 3.4;
(iii) to any existing or prospective Affiliate, partner, member, 

  
 15 

 
stockholder, or wholly owned subsidiary of such Investor in the ordinary course of business, provided that such Investor informs such Person that such information is confidential and directs such
Person to maintain the confidentiality of such information; or (iv) as may otherwise be required by law, provided that the Investor promptly notifies the Company of such disclosure and takes reasonable steps to minimize the extent of any such
required disclosure. 
 4. Rights to Future Stock Issuances. 

4.1 Right of First Offer. Subject to the terms and conditions of this Section 4.1 and applicable securities laws, if the
Company proposes to offer or sell any New Securities, the Company shall first offer such New Securities to each Major Investor. A Major Investor shall be entitled to apportion the right of first offer hereby granted to it among itself and its
Affiliates in such proportions as it deems appropriate. 
 (a) The Company shall give notice (the “Offer Notice”) to each
Major Investor, stating (i) its bona fide intention to offer such New Securities, (ii) the number of such New Securities to be offered, and (iii) the price and terms, if any, upon which it proposes to offer such New Securities. 

(b) By notification to the Company within twenty (20) days after the Offer Notice is given, each Major Investor may elect to purchase or
otherwise acquire, at the price and on the terms specified in the Offer Notice, up to that portion of such New Securities which equals the proportion that the Common Stock issued and held, or issuable (directly or indirectly) upon conversion and/or
exercise, as applicable, of the Series A Preferred Stock or Series B Preferred Stock and any other Derivative Securities then held, by such Major Investor bears to the total number of shares of Common Stock of the Company then outstanding (assuming
full conversion and/or exercise, as applicable, of all Series A Preferred Stock and Series B Preferred Stock and other Derivative Securities). At the expiration of such twenty (20) day period, the Company shall promptly notify each Major
Investor that elects to purchase or acquire all the shares available to it (each, a “Fully Exercising Investor”) of any other Major Investor’s failure to do likewise. During the ten (10) day period commencing after the
Company has given such notice, each Fully Exercising Investor may, by giving notice to the Company, elect to purchase or acquire, in addition to the number of shares specified above, up to that portion of the New Securities for which Major Investors
were entitled to subscribe but that were not subscribed for by the Major Investors, which is equal to the proportion that the Common Stock issued and held, or issuable upon conversion of Series A Preferred Stock and Series B Preferred Stock and any
other Derivative Securities then held, by such Fully Exercising Investor bears to the total number of shares of Common Stock issued and held, or issuable (directly or indirectly) upon conversion of the Series A Preferred Stock and Series B Preferred
Stock and any other Derivative Securities then held, by all Fully Exercising Investors who wish to purchase such unsubscribed shares. The closing of any sale pursuant to this Section 4.1(b) shall occur within the later of one hundred
twenty (120) days after the date that the Offer Notice is given and the date of initial sale of New Securities pursuant to Section 4.1(c). 

(c) If all New Securities referred to in the Offer Notice are not elected to be purchased or acquired as provided in
Section 4.1(b), the Company may, during the ninety (90) day period following the expiration of the periods provided in Section 4.1(b), offer and sell the remaining unsubscribed portion of such New Securities to any
Person or Persons at a price not less than, and upon terms no more favorable to the offeree than, those specified in the Offer Notice. If the Company does not enter into an agreement for the sale of the New Securities within such period, or if such
agreement is not consummated within thirty (30) days of the execution thereof, the right provided hereunder shall be deemed to be revived and such New Securities shall not be offered unless first reoffered to the Major Investors in accordance
with this Section 4.1. 

  
 16 

 (d) The right of first offer in this Section 4.1 shall not be applicable to
(i) Exempted Securities (as defined in the Company’s Certificate of Incorporation), (ii) shares of Common Stock issued in the IPO, (iii) shares of Specified Preferred Stock and (iv) shares of Series B Preferred Stock issued
to the Strategic Investor pursuant to the Purchase Agreement. 
 4.2 Termination. The covenants set forth in Section 4.1
shall terminate and be of no further force or effect (i) immediately before but subject to the consummation of an IPO, (ii) when the Company first becomes subject to the periodic reporting requirements of Section 12(g) or 15(d) of the
Exchange Act, or (iii) upon a Sale of the Company, as such term is defined in the Stockholders Agreement. 
 5. Additional
Covenants. 
 5.1 Insurance. Within a reasonable period of time after requested by the Board of Directors, the Company shall
obtain from financially sound and reputable insurers Directors and Officers Errors and Omissions insurance in an amount satisfactory to the Board of Directors, and will use commercially reasonable efforts to cause such insurance policies to be
maintained until such time as the Board of Directors determines that such insurance should be discontinued. 
 5.2 Employee
Agreements. The Company will cause (i) each person now or hereafter employed by it or by any subsidiary (or engaged by the Company or any subsidiary as a consultant/independent contractor) with access to confidential information and/or
trade secrets to enter into a nondisclosure and proprietary rights assignment agreement and (ii) each Key Employee to enter into a one (1) year noncompetition and nonsolicitation agreement (to the extent permitted by law), substantially in
the form approved by the Board of Directors, including one of the Series A Directors. In addition, the Company shall not amend, modify, terminate, waive, or otherwise alter, in whole or in part, any of the above-referenced agreements or any
restricted stock agreement between the Company and any employee, without the approval by the Board of Directors, including one of the Series A Directors. 

5.3 Employee Vesting. Unless otherwise approved by the Board of Directors, which approval shall include the Series A Directors, all
current and future employees and consultants of the Company who purchase, receive options to purchase, or receive awards of shares of the Company’s capital stock after the date hereof shall be required to execute restricted stock or option
agreements, as applicable, providing for (i) vesting of shares not faster than over a four (4) year period, with the first twenty-five percent (25%) of such shares vesting following twelve (12) months of continued employment or
service, and the remaining shares vesting in equal monthly installments over the following three (3) years, and (ii) a market stand-off provision substantially similar to that in Section 2.11. In addition, unless otherwise
approved by the Board of Directors, including one of the Series A Directors, the Company shall retain a “right of first refusal” on employee transfers until the Company’s IPO and shall have the right to repurchase unvested shares at
cost upon termination of employment of a holder of restricted stock. 
 5.4 Matters Requiring Investor Director Approval. The Company
hereby covenants and agrees with each of the Investors that it shall not, without first obtaining the approval of the Board of Directors, which approval must include, for so long as any shares of Series A Preferred Stock remain outstanding, the
affirmative vote of at least two (2) Series A Directors (or at least one (1) Series A Director, at any time when there are fewer than two (2) Series A Directors then serving): 

(i) make, or permit any subsidiary to make, any loan or advance to, or own any stock or other securities of, any subsidiary or other
corporation, partnership, or other entity unless it is wholly owned by the Company; 

  
 17 

 (ii) make, or permit any subsidiary to make, any loan or advance to any Person, including,
without limitation, any employee or director of the Company or any subsidiary, except advances and similar expenditures in the ordinary course of business or under the terms of an employee stock or option plan approved by the Board of Directors;

 (iii) guarantee, directly or indirectly, or permit any subsidiary to guarantee, directly or indirectly, any indebtedness except for
trade accounts of the Company or any subsidiary arising in the ordinary course of business; 
 (iv) make any investment inconsistent with
any investment policy approved by the Board of Directors; 
 (v) incur any indebtedness in excess of $100,000 in the aggregate that is not
covered by the Budget, other than trade credit incurred in the ordinary course of business; 
 (vi) otherwise enter into or be a party to
any transaction with any director, officer or employee of the Company or any “associate” (as defined in Rule 12b-2 promulgated under the Exchange Act) of any such Person; 

(vii) hire, terminate, or change the compensation of the executive officers, including approving or amending the terms of any option grants
or stock awards to executive officers; 
 (viii) change the principal business of the Company, or enter into a new line of business, or
exit the existing line of business of the Company; 
 (ix) sell, assign, license, pledge or encumber material technology or intellectual
property other than licenses granted in the ordinary course of business; or 
 (x) enter into any corporate strategic relationship
involving the payment contribution or assignment by the Company or to the Company of money or assets greater than $250,000. 
 5.5
Meetings of the Board of Directors; Committees. Unless otherwise determined at least by the vote of a majority of the directors then in office, the Board of Directors shall meet at least four (4) times per year, and at least once per
quarter, in accordance with an agreed-upon schedule, unless otherwise agreed by a vote of the majority of the directors. Each non-employee director shall be entitled at the Board’s discretion to be a member of any committee of the Board of
Directors. 
 5.6 Successor Indemnification. If the Company or any of its successors or assignees consolidates with or merges into
any other Person and is not the continuing or surviving corporation or entity of such consolidation or merger, then to the extent necessary, proper provision shall be made so that the successors and assignees of the Company assume the obligations of
the Company with respect to indemnification of members of the Board of Directors as in effect immediately before such transaction, whether such obligations are contained in the Company’s By-laws, its Certificate of Incorporation, or elsewhere,
as the case may be. 
 5.7 Board Expenses. The Company shall reimburse all non-employee directors for all reasonable out-of-pocket
expenses incurred (consistent with the Company’s policies) in connection with their role as a director of the Company. 

  
 18 

 5.8 Directors’ Liability and Indemnification. 

(a) The Company’s Certificate of Incorporation and Bylaws (as such Certificate of Incorporation and Bylaws of the Company may be amended
from time to time) shall provide (i) for limitation of the liability of directors to the maximum extent permitted by law, and (ii) for indemnification of directors for acts on behalf of the Company to the maximum extent permitted by law.
In the event any suit is filed or claim is asserted against a director or former director of the Company as a result of such director’s or former director’s service on the Board of Directors, the Company will provide such director or
former director access to all records and files of the Company as he or she may reasonably request in defending against or preparing to defend against any such suit or claim. 

(b) The Company hereby acknowledges that one or more of the directors nominated by holders of Series A Preferred Stock may have certain
rights to indemnification, advancement of expenses and/or insurance provided by one or more of the Investors and certain of their affiliates (collectively, the “Fund Indemnitors”) for alleged acts or omissions in their capacities as
directors of the Company. The Company hereby agrees (i) that it is the indemnitor of first resort (i.e., its obligations to any such director are primary and any obligation of the Fund Indemnitors to advance expenses or to provide
indemnification for the same expenses or liabilities incurred by such director are secondary), (ii) that it shall be required to advance the full amount of expenses incurred by such director and shall be liable for the full amount of all
expenses, judgments, penalties, fines and amounts paid in settlement by or on behalf of any such director to the extent legally permitted and as required by the Company’s Certificate of Incorporation or the Bylaws of the Company (or any
agreement between the Company and such director), without regard to any rights such director may have against the Fund Indemnitors, and, (iii) that it irrevocably waives, relinquishes and releases the Fund Indemnitors from any and all claims
against the Fund Indemnitors for contribution, subrogation or any other recovery of any kind in respect thereof. The Company further agrees that no advancement or payment by the Fund Indemnitors on behalf of any such director with respect to any
claim for which such director has sought indemnification from the Company shall affect the foregoing and the Fund Indemnitors shall have a right of contribution and/or be subrogated to the extent of such advancement or payment to all of the rights
of recovery of such director against the Company. 
 5.9 Termination of Covenants. The covenants set forth in this
Section 5, except for Sections 5.6 and 5.8, shall terminate and be of no further force or effect (i) immediately before but subject to the consummation of an IPO, (ii) when the Company first becomes subject to the
periodic reporting requirements of Section 12(g) or 15(d) of the Exchange Act, or (iii) upon a Sale of the Company, as such term is defined in the Stockholders Agreement, whichever event occurs first. 

6. Miscellaneous. 
 6.1
Successors and Assigns. The rights under this Agreement may be assigned (but only with all related obligations) by a Holder to a transferee of Registrable Securities that (i) is an Affiliate, partner, member, limited partner, retired
partner, retired member, or stockholder of a Holder; (ii) is a Holder’s Immediate Family Member or trust for the benefit of an individual Holder or one or more of such Holder’s Immediate Family Members; or (iii) after such
transfer, holds at least 1,000,000 shares of Registrable Securities (subject to appropriate adjustment for stock splits, stock dividends, combinations, and other recapitalizations); provided, however, that (x) the Company is, within a
reasonable time after such transfer, furnished with written notice of the name and address of such transferee and the Registrable Securities with respect to which such rights are being transferred; and (y) such transferee agrees in a written
instrument delivered to the Company to be bound by and subject to the terms and conditions of this Agreement, including the provisions of Section 2.11. For the purposes of determining the number of shares of Registrable Securities held
by a transferee, the holdings of a transferee (1) that is an Affiliate, 

  
 19 

 
limited partner, retired partner, member, retired member, or stockholder of a Holder; (2) who is a Holder’s Immediate Family Member; or (3) that is a trust for the benefit of an
individual Holder or such Holder’s Immediate Family Member shall be aggregated together and with those of the transferring Holder; provided further that all transferees who would not qualify individually for assignment of rights shall have a
single attorney-in-fact for the purpose of exercising any rights, receiving notices, or taking any action under this Agreement. The terms and conditions of this Agreement inure to the benefit of and are binding upon the respective successors and
permitted assignees of the parties, including without limitation, the Investor’s affiliated partnership or funds management by such Investor or any of its respective directors, officers or partners. Nothing in this Agreement, express or
implied, is intended to confer upon any party other than the parties hereto or their respective successors and permitted assignees any rights, remedies, obligations or liabilities under or by reason of this Agreement, except as expressly provided
herein. 
 6.2 Governing Law. This Agreement shall be governed by and construed in accordance with the General Corporation Law of the
State of Delaware as to matters within the scope thereof, and as to all other matters shall be governed by and construed in accordance with the internal laws of the State of California, without regard to conflict of law principles that would result
in the application of any law other than the law of the State of California. 
 6.3 Counterparts; Facsimile. 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. This Agreement may also be executed and delivered by facsimile signature and 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. 
 6.4
Titles and Subtitles. The titles and subtitles used in this Agreement are for convenience only and are not to be considered in construing or interpreting this Agreement. 

6.5 Notices. All notices, requests, and other communications given or made pursuant to this Agreement shall be in writing and shall be
deemed effectively given, delivered and received (i) upon personal delivery to the party to be notified; (ii) when sent by confirmed electronic mail or facsimile if sent during normal business hours of the recipient, and if not so
confirmed, then on the next business day; (iii) five (5) days after having been sent by registered or certified mail, return receipt requested, postage prepaid; or (iv) one (1) business day after the business day of deposit with
a nationally recognized overnight courier, specifying next-day delivery, with written verification of receipt. All communications shall be sent to the respective parties at their addresses as set forth on Schedule A hereto, or to the
principal office of the Company and to the attention of the Chief Executive Officer, in the case of the Company, or to such email address, facsimile number, or address as subsequently modified by written notice by the applicable party given in
accordance with this Section 6.5. If notice is given to the Company, a copy which shall not constitute notice shall also be sent to Mitchell S. Bloom, Esq. at Goodwin Procter LLP, 53 State St., Exchange Place, Boston, MA 02109. If notice
is given to the Purchasers, a copy, which shall not constitute notice, shall be sent to Steven C. Browne, Esq. at Morgan, Lewis & Bockius LLP, One Federal Street, Boston, MA 02110 or by facsimile to (617) 345-5069. 

6.6 Amendments and Waivers. Any term of this Agreement, including without limitation Section 4.1, may be amended and the
observance of any term of this Agreement 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 holders of at least a majority of the Registrable
Securities held by the Investors; provided that the Company may in its sole discretion waive compliance with Section 2.12(c)  

  
 20 

 
(and the Company’s failure to object promptly in writing after notification of a proposed assignment allegedly in violation of Section 2.12(c) shall be deemed to be a waiver);
and provided further that any provision hereof may be waived by any waiving party on such party’s own behalf, without the consent of any other party. Notwithstanding the foregoing, this Agreement may not be amended or terminated and the
observance of any term hereof may not be waived with respect to any Investor without the written consent of such Investor, unless such amendment, termination, or waiver applies to all Investors in the same fashion (it being agreed that a waiver of
the provisions of Section 4 with respect to a particular transaction shall be deemed to apply to all Investors in the same fashion if such waiver does so by its terms, notwithstanding the fact that certain Investors may nonetheless, by
agreement with the Company, purchase securities in such transaction); provided, that, if Investors holding at least a majority of the then-outstanding Series B Preferred Stock do not provide such waiver with respect to a particular transaction, the
holders of the Series B Preferred Stock that do not provide such waiver shall have the right to participate in such transaction on substantially similar terms to the Investors providing such waiver. Notwithstanding the foregoing,
Section 2.11 and this sentence of Section 6.6 may not be amended in a manner that adversely affects the Investors without the consent of Investors holding at least a majority of the then outstanding Series B Preferred Stock.
The Company shall give prompt notice of any amendment or termination hereof or waiver hereunder to any party hereto that did not consent in writing to such amendment, termination, or waiver. Any amendment, termination, or waiver effected in
accordance with this Section 6.6 shall be binding on all parties hereto, regardless of whether any such party has consented thereto. No waivers of or exceptions to any term, condition, or provision of this Agreement, in any one or more
instances, shall be deemed to be or construed as a further or continuing waiver of any such term, condition, or provision. 
 6.7
Severability. In case any one or more of the provisions contained in this Agreement is for any reason held to be invalid, illegal or unenforceable in any respect, such invalidity, illegality, or unenforceability shall not affect any other
provision of this Agreement, and such invalid, illegal, or unenforceable provision shall be reformed and construed so that it will be valid, legal, and enforceable to the maximum extent permitted by law. 

6.8 Aggregation of Stock. All shares of Registrable Securities held or acquired by Affiliates shall be aggregated together for the
purpose of determining the availability of any rights under this Agreement and such Affiliated person may apportion such rights as among themselves in any manner they deem appropriate. 

6.9 Amendment and Restatement of Prior Agreement; Entire Agreement. This Agreement (including any Schedules and Exhibits hereto)
constitutes the full and entire understanding and agreement among the parties with respect to the subject matter hereof, and any other written or oral agreement relating to the subject matter hereof existing between the parties is expressly
canceled. Upon the effectiveness of this Agreement, the Prior Agreement shall be deemed amended and restated and superseded and replaced in its entirety by this Agreement, and shall be of no further force or effect. 

6.10 Delays or Omissions. No delay or omission to exercise any right, power, or remedy accruing to any party under this Agreement, upon
any breach or default of any other party under this Agreement, shall impair any such right, power, or remedy of such nonbreaching or nondefaulting party, nor shall it be construed to be a waiver of or acquiescence to any such breach or default, or
to any similar breach or default thereafter occurring, nor shall any waiver of any single breach or default be deemed a waiver of any other breach or default theretofore or thereafter occurring. All remedies, whether under this Agreement or by law
or otherwise afforded to any party, shall be cumulative and not alternative. 

  
 21 

 6.11 Submission to Jurisdiction. The parties hereto submit to the exclusive jurisdiction
of any federal or state court located within the State of California over any dispute arising out of or relating to the Agreement or any of the transactions contemplated hereby and each party hereby agree that all claims in respect of such dispute
or any suit, action or proceeding related thereto may be heard and determined in such courts. The parties waive, to the fullest extent permitted by applicable law, any objection which they may not or hereafter have to the laying of venue of any such
dispute brought in such court or any defense of inconvenient forum for the maintenance of such dispute. Each of the parties hereto agrees that a judgment in any such dispute may be enforced in other jurisdictions by suit on the judgment or in any
other manner provided by law. 
 6.12 Other Investments. The Company hereby agrees and acknowledges that the Investors invest in
numerous portfolio companies, some of which may be deemed competitive with the Company’s business (as currently conducted or as currently proposed to be conducted). Nothing in this Agreement or the other Transaction Agreements (as defined in
the Purchase Agreement) shall preclude or in any way restrict the Investors from investing or participating in any particular enterprise whether or not such enterprise has products or services which compete with those of the Company. The Company
hereby further agrees that, to the extent permitted by applicable law, the Investors shall not be liable to the Company for any claim arising out of, or based upon, (a) the investment by the Investors in any entity competitive with the Company,
or (b) actions taken by any partner, officer or other representative of the Investors to assist any such competitive company, whether or not such action was taken as a member of the board of directors of such competitive company or otherwise,
and whether or not such action has a detrimental effect on the Company; provided, however that the foregoing shall not relieve (x) the Investors or any party from liability associated with the willful misuse of the Company’s confidential
information, or (ii) any director or officer of the Company from any liability associated with breaches of his or her fiduciary duties to the Company. 

[Remainder of Page Intentionally Left Blank] 

  
 22 

 IN WITNESS WHEREOF, the parties have executed this Second Amended and Restated Investors’
Rights Agreement as of the date first written above. 
  

			
	COMPANY:
	
	MYOKARDIA, INC.
		
	By:	 	 /s/ T. Anastasios Gianakakos

		 	Name: T. Anastasios Gianakakos
		 	Title: President and Chief Executive Officer
	
	Address:
	333 Allerton Ave.
	South San Francisco, CA 94080
	Attention: President

 [SIGNATURE PAGE TO SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 IN WITNESS WHEREOF, the parties have executed this Second Amended and Restated Investors’
Rights Agreement as of the date first written above. 
  

			
	INVESTOR:
	
	THIRD ROCK VENTURES II, L.P.
		
	By:	 	Third Rock Ventures GP II, L.P., its general partner
		
	By:	 	TRV GP II, LLC, its general partner
		
	By:	 	 /s/ Kevin Gillis

		 	 Name: Kevin Gillis
 Title: CFO

 [SIGNATURE PAGE TO SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 IN WITNESS WHEREOF, the parties have executed this Second Amended and Restated Investors’
Rights Agreement as of the date first written above. 
  

			
	INVESTOR:
	
	THIRD ROCK VENTURES III, L.P.
		
	By:	 	Third Rock Ventures GP III, L.P.,
	its General Partner
		
	By:	 	TRV GP III, LLC, its General
	Partner
		
	By:	 	 /s/ Kevin Gillis

	Name: Kevin Gillis
	Title: CFO

 [SIGNATURE PAGE TO SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 IN WITNESS WHEREOF, the parties have executed this Second Amended and Restated Investors’
Rights Agreement as of the date first written above. 
  

			
	 INVESTOR:

	
	 AVENTIS INC.

		
	 By:
	 	 /s/ M. McClellan

		 	 Name: M. McClellan

		 	 Title: CFO North America

	
	 Address:

	 c/o Sanofi S.A.

	 54 rue La Boetie, 75008 Paris, France

	 Facsimile: +33 1 53 77 44 53

	 Attention: Vice President, Legal Operations

 [SIGNATURE PAGE TO SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 IN WITNESS WHEREOF, the parties have executed this Second Amended and Restated Investors’
Rights Agreement as of the date first written above. 
  

			
	INVESTOR:
	
	COWEN MK INVESTMENT LLC
		
	By:	 	 /s/ Stephen Lasota

		 	Name: Stephen Lasota
		 	Title: Chief Financial Officer
		 	          Cowen Group, Inc.

 

			
	Address:	 	599 Lexington Avenue
		 	New York, NY 10022
		 	Attn: Owen Littman

 [SIGNATURE PAGE TO SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 IN WITNESS WHEREOF, the parties have executed this Second Amended and Restated Investors’
Rights Agreement as of the date first written above. 
  

			
	INVESTOR:
	
	BRIDGEBIO LLC
		
	By:	 	 /s/ Neil Kumar

		 	Name: Neil Kumar
		 	Title: Partner

  

			
	Address:	 	1 Maritime Plaza, Suite 1940
		 	San Francisco, CA 94111

 [SIGNATURE PAGE TO SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 IN WITNESS WHEREOF, the parties have executed this Second Amended and Restated Investors’
Rights Agreement as of the date first written above. 
  

			
	INVESTOR:
	
	PERCEPTIVE LIFE SCIENCES MASTER FUND LTD
		
	By:	 	 /s/ James H. Mannix

		 	Name: James H. Mannix
		 	Title: COO

  

			
	Address:	 	499 Park Avenue, 25th Floor
		 	New York, NY 10022
		 	Attn: James H. Mannix
		 	Phone: (646) 205-5340
		 	Fax: (646) 205-5301

 [SIGNATURE PAGE TO SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 IN WITNESS WHEREOF, the parties have executed this Second Amended and Restated Investors’
Rights Agreement as of the date first written above. 
  

			
	INVESTOR:
	
	TITAN PERC, LLC
		
	By:	 	 /s/ Thomas Holliday

		 	Name: Thomas Holliday
		 	Title: CIO

  

			
	Address:	 	750 Washington Blvd., 10th Floor
		 	Stamford, CT 06901
		 	Attn: Darren Ross
		 	Phone: (203) 327-8650
		 	Email: dross@titanadvisors.com
		 	Fax: (203) 327-8599

 [SIGNATURE PAGE TO SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 IN WITNESS WHEREOF, the parties have executed this Second Amended and Restated Investors’
Rights Agreement as of the date first written above. 
  

			
	INVESTOR:
	
	CORMORANT GLOBAL HEALTHCARE MASTER FUND, LP
		
	By:	 	 /s/ Bihua Chen

		 	Name: Bihua Chen
		 	Title: Managing Member of the General Partner

  

			
	Address:	 	Cormorant Global Healthcare Master Fund, LP
		 	200 Clarendon Street 52nd Floor
		 	Boston, MA 02115

 [SIGNATURE PAGE TO SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 IN WITNESS WHEREOF, the parties have executed this Second Amended and Restated Investors’
Rights Agreement as of the date first written above. 
  

			
	INVESTOR:
	
	FIDELITY SELECT PORTFOLIOS: BIOTECHNOLOGY PORTFOLIO
		
	By:	 	 /s/ Stacie M. Smith

		 	Name: Stacie M. Smith
		 	Title: Authorized Signatory
	
	 Address:
 Brown Brothers
Harriman & Co.
 525 Washington Blvd
 Jersey City NJ
07310
 Attn: Michael Lerman 15th Floor
 Corporate Actions

Email: michael.lerman@bbh.com
 Fax number: 617
772-2418

 [SIGNATURE PAGE TO SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 IN WITNESS WHEREOF, the parties have executed this Second Amended and Restated Investors’
Rights Agreement as of the date first written above. 
  

			
	INVESTOR:
	
	FIDELITY ADVISOR SERIES VII: FIDELITY ADVISOR BIOTECHNOLOGY FUND
		
	By:	 	 /s/ Stacie M. Smith

		 	Name: Stacie M. Smith
		 	Title: Authorized Signatory
	
	 Address:
 State Street
Bank & Trust
 PO Box 5756
 Boston, Massachusetts
02206
 Attn: Bangle & Co fbo Fidelity Advisor Series VII: Fidelity Advisor Biotechnology Fund

Email: SSBCORPACTIONS@StateStreet.com
 Fax number:
617-988-9110

 [SIGNATURE PAGE TO SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 IN WITNESS WHEREOF, the parties have executed this Second Amended and Restated Investors’
Rights Agreement as of the date first written above. 
  

			
	INVESTOR:
	
	CASDIN PARTNERS MASTER FUND LP
		
	By:	 	 /s/ Eli Casdin

	Name: Eli Casdin
	Title: Managing Partner
	
	 Address:
 1345
Avenue of the Americas, Suite 1140
 New York, NY 10019

 [SIGNATURE PAGE TO SECOND
AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 SCHEDULE A 

Name and Contact of Investors 

THIRD ROCK VENTURES II, L.P. 

29 Newbury Street; 3rd Floor 

Boston, MA 02116 
 Phone:
(617)585-2000 
 Fax: (617) 859-2891 

http://www.thirdrockventures.com 

ALEXANDRIA EQUITIES, LLC, 

Alexandria Real Estate Equities, Inc. 

385 East Colorado Blvd., Suite 299 

Pasadena, CA 91101 
 Attn: Natasha
Moon 
 Phone: (626) 578-0777, x. 1619 

Fax: (626) 578-7252 
 http:/
/www.are.com 
 THIRD ROCK VENTURES III, L.P. 

29 Newbury Street; 3rd Floor 

Boston, MA 02116 
 Phone:
(617)585-2000 
 Fax: (617) 859-2891 

http://www.thirdrockventures.com 

AVENTIS INC. 
 c/o Sanofi
S.A. 
 54 rue La Boetie, 75008 Paris, France 

Facsimile: +33 1 53 77 44 53 

Attention: Vice President, Legal Operations 

BRIDGEBIO LLC 
 1 Maritime
Plaza, Suite 1940 
 San Francisco, CA 94111 

CORMORANT ASSET MANAGEMENT HEALTHCARE MASTER FUND, LP 

200 Clarendon Street 52nd Floor 

Boston, MA 02116 
 COWEN MK
INVESTMENT LLC 
 599 Lexington Avenue 

New York, NY 10022 
 Attn: Owen
Littman 
 FIDELITY SELECT PORTFOLIOS: BIOTECHNOLOGY PORTFOLIO 

Brown Brothers Harriman & Co. 

525 Washington Blvd 
 Jersey City NJ
07310 

 Attn: Michael Lerman 15th Floor 

Corporate Actions 
 Email:
michael.lerman@bbh.com 
 Fax number: 617 772-2418 

FIDELITY ADVISOR SERIES VII: FIDELITY ADVISOR BIOTECHNOLOGY FUND 

State Street Bank & Trust 

PO Box 5756 
 Boston, Massachusetts
02206 
 Attn: Bangle & Co fbo Fidelity Advisor Series VII: Fidelity Advisor Biotechnology Fund 

Email: SSBCORPACTIONS@StateStreet.com 

Fax number: 617-988-9110 

PERCEPTIVE LIFE SCIENCES MASTER FUND LTD 

499 Park Avenue, 25th Floor 
 New
York, NY 10022 
 Attn: James H. Mannix 

Phone: (646) 205-5340 
 Fax:
(646) 205-5301 
 TITAN PERC, LLC 

750 Washington Blvd., 10th Floor 

Stamford, CT 06901 
 Attn: Darren
Ross 
 Phone: (203) 327-8650 

Email: dross@titanadvisors.com 

Fax: (203) 327-8599 

Casdin Partners Master Fund LP 

1345 Avenue of the Americas, Suite 1140 

New York, NY 10019EX-4.3

 Exhibit 4.3 

AMENDMENT NO. 1 TO SECOND AMENDED AND RESTATED 

INVESTORS’ RIGHTS AGREEMENT 

This Amendment No. 1 to Second Amended and Restated Investors’ Rights Agreement (this “Amendment”) is made as of
April 20, 2015, by and among MyoKardia, Inc., a Delaware corporation (the “Company”), and the parties listed on the signature pages hereto. Capitalized terms used herein and not otherwise defined herein shall have the meanings
ascribed to such terms in the Second Amended and Restated Investors’ Rights Agreement, dated as of April 20, 2015, by and among the Company and the parties named therein (the “Agreement”). 

WHEREAS, pursuant to the terms of the Purchase Agreement, the Strategic Investor may, at its option, purchase certain shares of Series B
Preferred Stock in accordance with the terms of the Purchase Agreement (the “Strategic Investor Investment”); 
 WHEREAS,
pursuant to Section 6.6 of the Agreement, and subject to the qualifications set forth therein, the Agreement may be amended by a written instrument executed by (i) the Company and (ii) the holders of at least a majority of the
Registrable Securities then held by the Investors; and 
 WHEREAS, any such amendment so effected shall be binding on all Parties, even if
they do not execute such consent. 
 NOW, THEREFORE, for good and valuable consideration, the receipt and sufficiency of which are hereby
acknowledged, the undersigned hereby agree as follows: 
 1. The defined term “Major Investor” is hereby deleted in its entirety from
Section 1.18 of the Agreement and replaced with the following: “1.18 Major Investor” means any Investor that, individually or together with such Investor’s Affiliates, holds at least 1,000,000 shares of Registrable
Securities (subject to appropriate adjustment in the event of any stock dividend, stock split, combination or other similar recapitalization); provided, however, that the Strategic Investor shall only be deemed a Major Investor for
purposes of Section 4 of this Agreement so long as (x) the Strategic Investor (together with its Affiliates) holds at least 1,000,000 shares of Registrable Securities (subject to appropriate adjustment in the event of any stock
dividend, stock split, combination or other similar recapitalization), (y) the Collaboration Agreement remains in full force and effect and (z) for any offering of New Securities after December 31, 2016, the Strategic Investor (or any
of its Affiliates) shall have taken all actions required under Section 9.3(a) of the Collaboration Agreement on or before January 31, 2017 (the conditions in clauses (x), (y) and (z) of the foregoing, the “Strategic
Investor Conditions”). For the avoidance of doubt, (A) if the Strategic Investor Conditions are not satisfied, the Strategic Investor shall not be deemed a Major Investor solely for purposes of Section 4 of this Agreement
and (B) clause (z) shall only be a Strategic Investor Condition following December 31, 2016.” 
 2. Section 4.1(b) of the Agreement
is hereby deleted in its entirety and replaced with the following: “By notification to the Company within twenty (20) days after the Offer Notice is given, each Major Investor may elect to purchase or otherwise acquire, at the price and on
the terms specified in the Offer Notice, up to that portion of such New Securities which equals the proportion that the Common Stock issued and held, or issuable (directly or indirectly) upon conversion and/or exercise, as applicable, of the Series
A Preferred Stock, Series A-1 Preferred Stock or Series B Preferred Stock and any other Derivative Securities (excluding Specified Preferred Stock) then held, by such Major Investor bears to the total number of shares of Common Stock of the Company
then outstanding (assuming full conversion and/or exercise, as applicable, of all Series A Preferred Stock, Series A-1 Preferred Stock and Series B Preferred Stock and other Derivative Securities). At the expiration of such twenty (20) day
period, the Company shall promptly notify each Major Investor that elects to purchase or acquire all the shares available to it (each, a “Fully Exercising Investor”) of any other Major Investor’s failure to do likewise. During
the ten (10) day period commencing after the Company 

 
has given such notice, each Fully Exercising Investor may, by giving notice to the Company, elect to purchase or acquire, in addition to the number of shares specified above, up to that portion
of the New Securities for which Major Investors were entitled to subscribe but that were not subscribed for by the Major Investors, which is equal to the proportion that the Common Stock issued and held, or issuable upon conversion of Series A
Preferred Stock, Series A-1 Preferred Stock and Series B Preferred Stock and any other Derivative Securities (excluding Specified Preferred Stock) then held, by such Fully Exercising Investor bears to the total number of shares of Common Stock
issued and held, or issuable (directly or indirectly) upon conversion of the Series A Preferred Stock, Series A-1 Preferred Stock and Series B Preferred Stock and any other Derivative Securities (excluding Specified Preferred Stock) then
held, by all Fully Exercising Investors who wish to purchase such unsubscribed shares. The closing of any sale pursuant to this Section 4.1(b) shall occur within the later of one hundred twenty (120) days after the date that the Offer
Notice is given and the date of initial sale of New Securities pursuant to Section 4.1(c).” 
 3. The Agreement is hereby amended to add a new
Section 4.3 as follows: “4.3 Strategic Investor Limitation. The Investors and the Company hereby agree that if the Strategic Investor exercises its right to participate in a financing of the Company as contemplated in
Section 9.3 of the Collaboration Agreement, then for such financing, such participation rights will be in lieu of the Strategic Investor’s participation rights in an offer of New Securities as contemplated by this Section 4, and the
Strategic Investor shall not be considered a Major Investor under this Section 4 solely for purposes of such financing.” 
 4. This Amendment
shall become effective upon the consummation of the Strategic Investor Investment. If the Strategic Investor Investment is not consummated on or prior to the Strategic Investor Outside Date (as defined in the Purchase Agreement) in accordance with
the terms of the Purchase Agreement, this Amendment shall be void ab initio and the Agreement shall remain in full force and effect without the amendments contemplated herein. 

5. Except as expressly amended herein, the Agreement shall remain in full force and effect. 

6. This Amendment shall be governed by and construed in accordance with the General Corporation Law of the State of Delaware as to matters within the scope
thereof, and as to all other matters shall be governed by and construed in accordance with the internal laws of the State of California, without regard to conflict of law principles that would result in the application of any law other than the law
of the State of California. 
 7. This Amendment 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. This Amendment may also be executed and delivered by facsimile signature and 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. 
 8. Sections 6.1, 6.5, 6.7, 6.8, 6.10 and 6.11 of the Agreement are hereby incorporated by reference (mutatis
mutandis) as if set forth herein 
 [Remainder of Page Intentionally Left Blank]. 

  
 2 

 IN WITNESS WHEREOF, the parties have executed this Amendment as of the date first above written.

  

			
	MYOKARDIA, INC.
		
	By:	 	 /s/ T. Anastasios Gianakakos

		 	Name: T. Anastasios Gianakakos
		 	Title: President and Chief Executive Officer

 
			
	INVESTOR:
	
	FIDELITY ADVISOR SERIES VII: FIDELITY ADVISOR BIOTECHNOLOGY FUND
		
	By:	 	 /s/ Kenneth Robins

		 	Name: Kenneth Robins
		 	Title: Authorized Signatory
	
	Address:
	 State Street Bank & Trust

PO Box 5756
 Boston, Massachusetts 02206

Attn: Bangle & Co fbo Fidelity Advisor Series VII: Fidelity Advisor Biotechnology Fund

Email: SSBCORPACTIONS@StateStreet.com
 Fax number:
617-988-9110

 
			
	INVESTOR:
	
	FIDELITY SELECT PORTFOLIOS: BIOTECHNOLOGY PORTFOLIO
		
	By:	 	 /s/ Kenneth Robins

		 	Name: Kenneth Robins
		 	Title: Authorized Signatory
	
	Address:
	 Brown Brothers Harriman & Co.

525 Washington Blvd

Jersey City NJ 07310

Attn: Michael Lerman 15th Floor

Corporate Actions

Email: michael.lerman@bbh.com

Fax number: 617 772-2418

 
					
	 INVESTOR:

	
	 THIRD ROCK VENTURES II, L.P.

		
		 	By: Third Rock Ventures GP II, L.P., its general partner
		 	By: TRV GP II, LLC, its general partner
			
		 	By:	 	 /s/ Kevin Gillis

		 		 	Name: Kevin Gillis
		 		 	Title: CFO
			
	 Address:
	 		 	    29 Newbury Street; 3rd Floor
		 		 	     Boston, MA 02116

		 		 	     Phone: (617) 585-2000

		 		 	     Fax: (617) 859-2891

 
					
	 INVESTOR:

	
	 THIRD ROCK VENTURES III, L.P.

		
		 	By: Third Rock Ventures GP II, L.P., its general partner
		 	By: TRV GP II, LLC, its general partner
			
		 	By:	 	 /s/ Kevin Gillis

		 		 	Name: Kevin Gillis
		 		 	Title: CFO
			
	 Address:
	 		 	    29 Newbury Street; 3rd Floor
		 		 	     Boston, MA 02116

		 		 	     Phone: (617) 585-2000

		 		 	     Fax: (617) 859-2891

 SCHEDULE A 

Name and Contact of Investors 

THIRD ROCK VENTURES II, L.P. 

29 Newbury Street; 3rd Floor 

Boston, MA 02116 
 Phone:
(617)585-2000 
 Fax: (617) 859-2891 

http://www.thirdrockventures.com 

ALEXANDRIA EQUITIES, LLC, 

Alexandria Real Estate Equities, Inc. 

385 East Colorado Blvd., Suite 299 

Pasadena, CA 91101 
 Attn: Natasha
Moon 
 Phone: (626) 578-0777, x. 1619 

Fax: (626) 578-7252 
 http:/
/www.are.com 
 THIRD ROCK VENTURES III, L.P. 

29 Newbury Street; 3rd Floor 

Boston, MA 02116 
 Phone:
(617)585-2000 
 Fax: (617) 859-2891 

http://www.thirdrockventures.com 

AVENTIS INC. 
 c/o Sanofi
S.A. 
 54 rue La Boetie, 75008 Paris, France 

Facsimile: +33 1 53 77 44 53 

Attention: Vice President, Legal Operations 

BRIDGEBIO LLC 
 1 Maritime
Plaza, Suite 1940 
 San Francisco, CA 94111 

CORMORANT ASSET MANAGEMENT HEALTHCARE MASTER FUND, LP 

200 Clarendon Street 52nd Floor 

Boston, MA 02116 
 COWEN MK
INVESTMENT LLC 
 599 Lexington Avenue 

New York, NY 10022 
 Attn: Owen
Littman 
 FIDELITY SELECT PORTFOLIOS: BIOTECHNOLOGY PORTFOLIO 

Brown Brothers Harriman & Co. 

525 Washington Blvd 
 Jersey City NJ
07310 

 Attn: Michael Lerman 15th Floor 

Corporate Actions 
 Email:
michael.lerman@bbh.com 
 Fax number: 617 772-2418 

FIDELITY ADVISOR SERIES VII: FIDELITY ADVISOR BIOTECHNOLOGY FUND 

State Street Bank & Trust 

PO Box 5756 
 Boston, Massachusetts
02206 
 Attn: Bangle & Co fbo Fidelity Advisor Series VII: Fidelity Advisor Biotechnology Fund 

Email: SSBCORPACTIONS@StateStreet.com 

Fax number: 617-988-9110 

PERCEPTIVE LIFE SCIENCES MASTER FUND LTD 

499 Park Avenue, 25th Floor 
 New
York, NY 10022 
 Attn: James H. Mannix 

Phone: (646) 205-5340 
 Fax:
(646) 205-5301 
 TITAN PERC, LLC 

750 Washington Blvd., 10th Floor 

Stamford, CT 06901 
 Attn: Darren
Ross 
 Phone: (203) 327-8650 

Email: dross@titanadvisors.com 

Fax: (203) 327-8599 

Casdin Partners Master Fund LP 

1345 Avenue of the Americas, Suite 1140 

New York, NY 10019

Source: [{"source": "alea-institute/alea-institute/kl3m-data-edgar-agreements/train-00249-of-00352.parquet"}, [{"source": "alea-institute/alea-institute/kl3m-data-edgar-agreements/train-00249-of-00352.parquet"}]]