Document:

Investors' Rights Agreement

 Exhibit 4.2 

Execution Version 
 AUDENTES
THERAPEUTICS, INC. 
 AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 

This Amended and Restated Investors’ Rights Agreement (this “Agreement”) is made and entered into as of
October 8, 2015 by and among Audentes Therapeutics, Inc., a Delaware corporation (the “Company”), and each of the investors listed on Schedule A hereto, each of which is referred to in this Agreement as an
“Investor,” any subsequent investor that becomes a party to this Agreement in accordance with Section 7.14 hereof and any holder of a Lender Warrant that becomes a party to this Agreement in accordance with
Section 7.14 hereof. 
 RECITALS 

WHEREAS, certain of the Investors (the “Prior Investors”) are holders of (i) outstanding shares of the
Company’s Series Seed Preferred Stock (the “Series Seed Preferred Stock”) issued by the Company to such Prior Investors pursuant to a Series Seed Preferred Stock Purchase Agreement, by and among the Company and certain
of the Prior Investors, dated December 24, 2012, as amended from time to time, (ii) the Company’s Series A Preferred Stock (the “Series A Preferred Stock”) issued by the Company to such Prior Investors
pursuant to a Series A Preferred Stock Purchase Agreement, by and among the Company and certain of the Prior Investors, dated July 16, 2013, and/or (iii) the Company’s Series B Preferred Stock (the “Series B
Preferred Stock”) issued by the Company to such Prior Investors pursuant to a Series B Preferred Stock Purchase Agreement, by and among the Company and certain of the Prior Investors, dated November 21, 2014, and have also
been granted certain rights under a Second Amended and Restated Investors’ Rights Agreement, by and among the Company, the Prior Investors and certain other parties thereto, dated November 21, 2014, as amended by Amendment No. 1 to
the Investors’ Rights Agreement (the “Prior Rights Agreement”). 
 WHEREAS, certain of the Investors (the
“Series C Investors”) have agreed to purchase from the Company, and the Company has agreed to sell to the Series C Investors, shares of the Company’s Series C Preferred Stock (the “Series C Preferred
Stock” and together with the Series Seed Preferred Stock, the Series A Preferred Stock, and the Series B Preferred Stock, the “Preferred Stock”) on the terms and conditions set forth in that certain Series C
Preferred Stock Purchase Agreement dated of even date herewith, by and among the Company and the Series C Investors, as amended from time to time (the “Purchase Agreement”). 

WHEREAS, the Company and the Prior Investors desire to enter into this Agreement in order to amend, restate and replace their rights and
obligations under the Prior Rights Agreement with the rights and obligations set forth in herein. 
 NOW, THEREFORE, in consideration of the
foregoing recitals and the mutual promises hereinafter set forth, the parties hereto hereby agree as follows: 
 1.
DEFINITIONS. For purposes of this Agreement: 
 “Affiliate” means, with respect to any specified Person,
or any other Person who or which, directly or indirectly, controls, is controlled by, or is under common control with such 

 
Person including without limitation any general partner, managing partner, managing member, officer or director of such Person or any venture capital or other investment fund now or hereafter
existing that is controlled by one or more general partners or managing members of, or shares the same management company or investment advisor (or sub-advisor) with, such Person. For purposes of this definition, the terms
“controlling,” “controlled by,” or “under common control with” shall mean the possession, directly or indirectly, of (a) the power to direct or cause the direction of the
management and policies of a Person, whether through the ownership of voting securities, by contract, or otherwise, or (b) the power to elect or appoint at least fifty percent (50%) of the directors, managers, general partners, or persons
exercising similar authority with respect to such Person. 
 “Automatic Shelf Registration Statement” shall have the
meaning given to that term in SEC Rule 405. 
 “business day” means a weekday on which banks are open for general
banking business in San Francisco, California. 
 “Cardiogen Holders” means those stockholders of Cardiogen
Sciences, Inc. (“Cardiogen”), whose shares of Cardiogen capital stock were exchanged for shares of the Company’s capital stock pursuant to the Merger Agreement. 

“Cardiogen Registrable Securities” means the shares of Common Stock originally issued by the Company, and the shares
of Common Stock issued or issuable upon conversion of the shares of Series B Preferred Stock originally issued by the Company, to the Cardiogen Holders pursuant to that certain Agreement and Plan of Merger and Reorganization by and among the
Company, Commodus Acquisition Corp, Inc., a Delaware corporation and a wholly-owned subsidiary of the Company, Maximus Acquisition Sub, LLC, a Delaware limited liability company and a wholly-owned subsidiary of the Company, Cardiogen Sciences, Inc.,
Delaware corporation, and Louis Lange as the Stockholders Agent (as defined therein), dated as of August 17, 2015 (the “Merger Agreement”). 

“Code” means the Internal Revenue Code of 1986, as amended. 

“Common Stock” means shares of the Company’s common stock. 

“Damages” means any loss, damage, 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, or liability (or any action in respect thereof) arises out of or is based upon (a) 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, and any free-writing prospectus and any issuer information (as defined
in Rule 433 of the Securities Act) filed or required to be filed pursuant to Rule 433(d) under the Securities Act or any other document incident to such registration prepared by or on behalf of the Company or used or referred to by the Company;
(b) 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 (c) 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. 

  
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 “Demand Notice” means notice sent by the Company to the Holders
specifying that a demand registration has been requested as provided in Section 3.1.1. 
 “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. 

“Deemed Liquidation Event” has the meaning set forth for such term in the certificate of incorporation of the Company
most recently filed with the Delaware Secretary of State that contains such a definition, whether or not the holders of outstanding shares of Preferred Stock elect otherwise by written notice sent to the Company as provided in such definition. 

“Exchange Act” means the Securities Exchange Act of 1934, as amended, and the rules and regulations promulgated
thereunder. 
 “Excluded Registration” means (a) a registration relating to the sale of securities to employees
of the Company or a subsidiary pursuant to an equity incentive, stock option, stock purchase, or similar plan; (b) a registration relating to an SEC Rule 145 transaction; (c) 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; or (d) a registration in which the only Common Stock being registered is Common Stock issuable upon conversion
of debt securities that are also being registered. 
 “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. 

“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. 

“Free Writing Prospectus” means a free-writing prospectus, as defined in Rule 405 under the Securities Act. 

“Fully Exercising Investor” shall have the meaning set forth in Section 4.2. 

“GAAP” means generally accepted accounting principles in the United States. 

“Genethon” means Genethon, a French non-profit organization organized under the French law of July 1, 1901. 

“Genethon Registrable Securities” means the Common Stock issuable or issued pursuant to that certain Common Stock
Purchase Agreement, by and between the Company and Genethon, dated as of January 24, 2014. 
 “Holder” means
any holder of Registrable Securities who is a party to this Agreement. 

  
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 “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. 

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

 “Investor Notice” shall have the meaning set forth in Section 4.2. 

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

“Lender Registrable Securities” means (a) the Common Stock issuable or issued upon the exercise of any Lender
Warrant and (b) the Common Stock issuable or issued upon conversion of the Preferred Stock issuable or issued pursuant to the exercise of any Lender Warrant; provided, however, that before the holder of any Lender
Warrant shall be entitled to exercise any rights under this Agreement, such holder must either (i) become a party to this Agreement as a “Lender” or (ii) agree to be bound by the terms of this Agreement related to registration
rights applicable to the Lender Registrable Securities in a separate written agreement between such holder and the Company (including, without limitation, in a Lender Warrant). 

“Lender Warrant” means any warrant to purchase shares of capital stock of the Company issued to banks, equipment
lessors or other financial institutions pursuant to a debt financing or equipment leasing transaction where the Company’s Board of Directors (the “Board”) has approved the grant to the holder thereof of
“piggyback” registration rights. 
 “Major Investor” means any Investor that, individually or together
with such Investor’s Affiliates, holds at least 500,000 shares of Registrable Securities (as adjusted for any stock split, stock dividend, combination, or other recapitalization or reclassification effected after the date hereof). 

“New Securities” means, collectively, equity securities of the Company, whether or not currently authorized,
Derivative Securities and any 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 (in each case, directly or indirectly)
such equity securities; provided however, that “New Securities” shall exclude: (a) Exempted Securities (as defined in the Restated Certificate); and (b) shares of Series C Preferred Stock issued pursuant
to the Purchase Agreement. 
 “Offer Notice” shall have the meaning set forth in Section 4.1. 

“Person” means any individual, corporation, partnership, trust, limited liability company, association or other
entity. 
 “Preferred Stock” means shares of the Company’s Series Seed Preferred Stock, Series A Preferred
Stock, Series B Preferred Stock, and Series C Preferred Stock and all outstanding shares of any other series of the Company’s Preferred Stock that may hereafter be issued by the Company. 

  
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 “Pro Rata Amount” means, for each Major Investor, that portion of the New
Securities identified in an Offer Notice 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 Preferred Stock and any other Derivative
Securities then held, by such Major Investor bears to the total Common Stock of the Company then outstanding (assuming full conversion and/or exercise, as applicable, of all Preferred Stock and other Derivative Securities). 

“Registrable Securities” means (a) the Common Stock issuable or issued upon conversion of shares of the Preferred
Stock held by the Investors; (b) the Lender Registrable Securities, provided, however, that such Lender Registrable Securities shall not be deemed Registrable Securities and the Lenders shall not be deemed Holders
for the purposes of Sections 2.1, 2.2, 3.1, 3.10, 4 and 7.6; (c) the Genethon Registrable Securities, provided, however, that, for purposes of Section 4 only, (x) such Genethon Registrable Securities shall
not be deemed Registrable Securities and (y) Genethon shall not be deemed a Holder; (d) the Cardiogen Registrable Securities, provided, however, that such Cardiogen Registrable Securities shall not be deemed Registrable Securities and the
Cardiogen Holders shall not be deemed Holders for the purposes of Sections 2.1, 2.2, 3.1, 3.10, 4, 5 and 7.6; and (e) 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 (a) through (d) 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 Section 7.1, and excluding for purposes of Section 3 any shares for which registration rights have terminated pursuant to Section 6.2 of this
Agreement. Notwithstanding the foregoing, the Company shall in no event be obligated to register any Preferred Stock of the Company, and Holders of Registrable Securities will not be required to convert their Preferred Stock into Common Stock in
order to exercise the registration rights granted hereunder, until immediately before the closing of the offering to which the registration relates. 

“Registrable Securities then outstanding” means the number of shares determined by adding the number of shares of
outstanding Common Stock that are Registrable Securities and the number of shares of Common Stock issuable (directly or indirectly) pursuant to then exercisable and/or convertible securities that are Registrable Securities. 

“Requisite Investors” means the holders of at least 66.67% of the shares of Common Stock (x) issued or issuable
upon conversion of the then outstanding shares of the Preferred Stock held by the Investors and (y) then held by Genethon (voting as a single class and on an as-converted basis). 

“Restated Certificate” means the Company’s Restated Certificate of Incorporation (as may be amended from time to
time). 
 “Restricted Securities” means the securities of the Company required to bear the legend set forth in
Section 3.12.2 hereof. 
 “SEC” means the Securities and Exchange Commission. 

“SEC Rule 144” means Rule 144 promulgated by the SEC under the Securities Act. 

  
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 “SEC Rule 145” means Rule 145 promulgated by the SEC under the Securities
Act. 
 “SEC Rule 405” means Rule 405 promulgated by the SEC under the Securities Act. 

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

 “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 3.6. 

“Selling Holder Counsel” means one counsel for the selling Holders. 

“Standoff Period” means the period commencing on the date of the final prospectus relating to an underwritten public
offering of the Company’s Common Stock under the Securities Act and ending on the date specified by the Company and the managing underwriter (such period not to exceed one hundred eighty (180) days). 

“Stock Sale” means a sale by the Company’s stockholders, in one transaction or series of related transactions, of
equity securities that represent, immediately prior to such transaction or transactions, at least a majority by voting power of the equity securities of the Company pursuant to an agreement approved by the Board and entered into by the Company. 

“T. Rowe Price Investor” means any Investor that that receives, directly or indirectly, investment management or
investment advisory services from T. Rowe Price Associates, Inc. (or its Affiliates and successors) (“T. Rowe Price”) with respect to its ownership interest in the Company. 

“Voting Agreement” means that certain Amended and Restated Voting Agreement dated of even date hereof by and among the
Company and the Investors. 
 2. INFORMATION RIGHTS. 

2.1 Delivery of Financial Statements. 

2.1.1 Information to be Delivered. The Company shall deliver the following to each Major Investor (provided that the
Board has not reasonably determined that such Major Investor is a competitor of the Company) and to Genethon: 
 (a) As soon as
practicable, but in any event within fifteen (15) days of being made available to the Company after the end of each fiscal year of the Company, the Company shall deliver, (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 of which shall be prepared in accordance with GAAP (except that such financial statements may (x) be subject to
normal year-end audit adjustments and (y) not contain all notes thereto that may be required in accordance with GAAP), provided, however, that such financial statements shall be audited and certified by independent
public accountants of nationally recognized standing selected by the Company. 

  
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 (b) As soon as practicable, but in any event within
forty-five (45) days after the end of each quarter of each fiscal year of the Company, the Company shall deliver, 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) Consolidation. 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 Section 2.1.1 shall be the consolidated and consolidating financial statements
of the Company and all such consolidated subsidiaries. 
 2.1.2 Suspension or Termination. Notwithstanding anything else in this
Section 2.1 to the contrary but subject to Section 6.1, the Company may cease providing the information set forth in this Section 2.1 during the period starting with the date sixty (60) 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 2.1 shall be reinstated at such time as the Company is no longer actively employing its reasonable efforts to cause such registration statement to become effective. 

2.2 Inspection. The Company shall permit each Major Investor, at such Major Investor’s expense, and on such Major
Investor’s written request, 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 2.2 to provide access to any information that it reasonably and
in good faith considers to be confidential information (unless covered by an enforceable confidentiality agreement, in form reasonably acceptable to the Company), a trade secret or the disclosure of which would adversely affect the attorney-client
privilege between the Company and its counsel.  
 2.3 Confidentiality. Each Investor (which term, solely for purposes
of this Section 2.3, shall include Genethon) 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 Section 2 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 2.3 by such Investor),
(b) is or has been independently developed or conceived by the Investor without use of the Company’s confidential information, (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, or (d) is possessed by Investor on a non-confidential basis from a source not subject to an obligation of confidentiality to the Company before receipt of such confidential
information from the Company; provided, however, that an Investor may disclose confidential information (i) to its attorneys, accountants, consultants, investment advisors (and sub-advisors) and other professionals to the
extent necessary to obtain their services in connection with monitoring its 

  
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investment in the Company; (ii) to any existing Affiliate, partner, limited partner, general partner, member, stockholder or wholly owned subsidiary or prospective limited partner of such
Investor in the ordinary course of business, but only if such Investor informs such Person that such information is confidential and directs such Person to maintain the confidentiality of such information; (iii) as may otherwise be required by
law if the Investor promptly notifies the Company of such disclosure and takes reasonable steps to minimize the extent of any such required disclosure; (iv) as required by any court or other governmental body, provided that the Investor
promptly notifies the Company of such disclosure and takes reasonable steps to minimize the extent of any such required disclosure; or (v) to comply with applicable law, statutes, rules or regulations or pursuant to any direction, request or
requirement (whether or not having the force of law but if not having the force of law being of a type with which institutional investors in the relevant jurisdiction are accustomed to comply) of any self-regulating organization or any governmental,
fiscal, monetary or other authority if the Investor promptly notifies the Company of such disclosure and takes reasonable steps to minimize the extent of any such required disclosure. The Company further acknowledges that certain of the Investors
are in the business of venture capital investing and/or are investment funds and therefore review, and/or are advised by entities that review, the business plans and related proprietary information of many enterprises, including enterprises which
may have products or services which compete directly or indirectly with those of the Company. Nothing in this 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. Nothing in this Agreement shall in any way restrict or impair the ability of T. Rowe Price to report the investment of the T. Rowe Price Investors in the Company in
accordance with applicable laws and regulations, without any requirement of prior notice to the Company. 
 3. REGISTRATION
RIGHTS. 
 3.1 Demand Registration. 

3.1.1 Form S-1 Demand. If at any time after the earlier of (a) five (5) years after the date of this Agreement or
(b) one hundred eighty (180) days after the effective date of the registration statement for the IPO, the Company receives a request from the Requisite Investors that the Company file a Form S-1 registration statement with respect to any
Registrable Securities then outstanding (and the Registrable Securities subject to such request have an anticipated aggregate offering price, net of Selling Expenses, of at least $25,000,000), 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) use commercially reasonable efforts to as soon as practicable, and in any event within ninety (90) 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 3.1.3 and Section 3.3. 
 3.1.2 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 any Holder of the Registrable 

  
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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
price, net of Selling Expenses, of at least $5,000,000, then the Company shall (a) within ten (10) days after the date such request is given, give a Demand Notice to all Holders other than the Initiating Holders; and (b) use
commercially reasonable efforts to 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 of the date the Demand Notice is given, and in each case, subject
to the limitations of Section 3.1.3 and Section 3.3. 
 3.1.3 Delay. Notwithstanding the foregoing obligations, if the
Company furnishes to Holders requesting a registration pursuant to this Section 3.1 a certificate signed by the Company’s chief executive officer stating that in the good faith judgment of the Board 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 (a) materially
interfere with a significant acquisition, corporate reorganization, or other similar transaction involving the Company; (b) require premature disclosure of material information that the Company has a bona fide business purpose for preserving as
confidential; or (c) 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 ninety (90) days after the request of the Initiating Holders is given; provided, however, that (i) the Company may not
invoke this right more than once in any twelve (12) month period and (ii) the Company shall not register any securities for its own account or that of any other stockholder during such ninety (90) day period other than an Excluded
Registration. 
 3.1.4 Limitations. The Company shall not be obligated to effect, or to take any action to effect, any registration
pursuant to Section 3.1.1: (a) during the period that is sixty (60) days before the Company’s good faith estimate of the date of filing of, and ending on a date that is one hundred eighty (180) days after the effective date
of, a Company-initiated registration, provided, that the Company is actively employing in good faith commercially reasonable efforts to cause such registration statement to become effective; (b) after the Company has effected two
(2) registrations pursuant to Section 3.1.1; or (c) 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 3.1.2. The Company shall not be obligated to effect, or to take any action to effect, any registration pursuant to Section 3.1.2: (i) during the period that is thirty (30) days before the Company’s good faith
estimate of the date of filing of, and ending on a date that is ninety (90) days after the effective date of, a Company-initiated registration, provided, that the Company is actively employing in good faith commercially reasonable efforts to
cause such registration statement to become effective; (ii) if the Company has effected two (2) registrations pursuant to Section 3.1.2 within the twelve (12) month period immediately preceding the date of such request; or
(iii) the registration is in any jurisdiction in which the Company would be required to qualify to do business as a foreign corporation or execute a general consent to service of process to effect such

  
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registration. A registration shall not be counted as “effected” for purposes of this Section 3.1.4 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, elect not to pay the registration expenses therefor, and forfeit their right to one registration on Form S-1 or S-3, as applicable, pursuant to
Section 3.6, in which case such withdrawn registration statement shall be counted as “effected” for purposes of this Section 3.1.4. 

3.2 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 3.3, 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 3.2 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 3.6. 
 3.3 Underwriting Requirements. 

3.3.1 Inclusion. If, pursuant to Section 3.1, 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 3.1, and the Company shall include such information in the Demand Notice. The underwriter(s) will be selected by the
Company, subject only to the reasonable approval of the holders of a majority of Registrable Securities held by 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. All Holders proposing to distribute their securities through such underwriting shall (together with the Company as provided in Section 3.4(e)) enter into an
underwriting agreement in customary form with the underwriter(s) selected for such underwriting. Notwithstanding any other provision of this Section 3.3, if the managing underwriter(s) advise(s) 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 of Registrable Securities 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 of Registrable Securities, including the Initiating Holders, in proportion (as nearly as practicable) to the number of Registrable Securities owned or held by each Holder
or in such other proportion as shall mutually be agreed to by all such selling Holders; provided, however, that the number of Registrable Securities owned or held 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 one hundred (100) shares. 

  
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 3.3.2 Underwriter Cutback. In connection with any offering involving an underwriting of
shares of the Company’s capital stock pursuant to Section 3.2, the Company shall not be required to include any of the Holders’ Registrable Securities in such underwriting unless the Holders accept the terms of the underwriting as
agreed upon between the Company and its underwriters. If the total number of securities, including Registrable Securities, requested by stockholders 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 or held 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 one hundred (100) shares. Notwithstanding the foregoing, in no event shall (a) 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 (b) 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 3.3.2 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 or held by all Persons included in such “selling Holder,” as defined in this sentence. 

3.3.3 Registration Not Effected. For purposes of Section 3.1, a registration shall not be counted as “effected” if, as
a result of an exercise of the underwriter’s cutback provisions in Section 3.3.1, fewer than fifty percent (50%) of the total number of Registrable Securities that Holders have requested to be included in such registration statement
are actually included. 
 3.4 Obligations of the Company. Whenever required under this Section 3 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 as promptly as practicable, and, upon the request of the Holders of a
majority of the Registrable Securities registered thereunder, keep such 

  
 11 

 
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 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 Common Stock (or other securities) of 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 120-day period shall be extended for up to sixty (60) 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, the prospectus and, if required, any Free
Writing 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 and any Free Writing
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 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 managing 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; 

  
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 (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 or Free-Writing Prospectus forming a part of such registration statement has been filed; 

(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 or Free-Writing Prospectus; 
 (k) use its commercially reasonable efforts to obtain
for the underwriters one or more “cold comfort” letters, dated the effective date of the related registration statement (and, if such registration includes an underwritten public offering, dated the date of the closing under the
underwriting agreement), signed by the Company’s independent public accountants in customary form and covering such matters of the type customarily covered by “cold comfort” letters; 

(l) use its commercially reasonable efforts to obtain for the underwriters on the date such securities are delivered to the underwriters for
sale pursuant to such registration a legal opinion of the Company’s outside counsel with respect to the registration statement, each amendment and supplement thereto, the prospectus included therein (including the preliminary prospectus) and
such other documents relating thereto in customary form and covering such matters of the type customarily covered by legal opinions of such nature; 

(m) to the extent the Company is a well-known seasoned issuer (as defined in SEC Rule 405 at the time any request for registration is
submitted to the Company in accordance with Section 3.1, if so requested, file an Automatic Shelf Registration Statement to effect such registration; and 

(n) if at any time when the Company is required to re-evaluate its well-known seasoned issuer status for purposes of an outstanding Automatic
Shelf Registration Statement used to effect a request for registration in accordance with Section 3.1.2 the Company determines that it is not a well-known seasoned issuer and (i) the registration statement is required to be kept effective
in accordance with this Agreement and (ii) the registration rights of the applicable Holders have not terminated, use commercially reasonable efforts to promptly amend the registration statement on a form the Company is then eligible to use or
file a new registration statement on such form, and keep such registration statement effective in accordance with the requirements otherwise applicable under this Agreement. 

3.5 Furnish Information. It shall be a condition precedent to the obligations of the Company to take any action pursuant to this
Section 3 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. 

  
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 3.6 Expenses of Registration. All expenses (other than Selling Expenses) incurred
in connection with registrations, filings, or qualifications pursuant to Section 3, 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 Selling Holder Counsel, not to exceed $30,000, shall be borne and paid by the Company; provided, however, that (a) the Company shall not be required to pay for any expenses of any
registration proceeding begun pursuant to Section 3.1 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 3.1.1 or Section 3.1.2, as the case may be, and (b) if, at the time of such withdrawal, the Holders shall have learned of a material adverse change in the condition, business, or prospects of the Company not 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 3.1.1 or Section 3.1.2. All Selling Expenses relating to Registrable Securities registered pursuant to this Section 3 shall be borne and paid by the Holders pro rata on the basis of the number of
Registrable Securities registered on their behalf. 
 3.7 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 3. 

3.8 Indemnification. If any Registrable Securities are included in a registration statement under this Section 3: 

3.8.1 Company Indemnification. 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 3.8.1 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, conditioned, or delayed 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. 
 3.8.2 Selling Holder
Indemnification. To the extent permitted by law, each selling Holder, severally and not jointly, will indemnify and hold harmless the Company, 

  
 14 

 
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 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 (a) the indemnity agreement contained in this Section 3.8.2 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, conditioned or delayed, and (b) that in no event shall the aggregate amounts payable by any Holder by way of indemnity or contribution
under Sections 3.8.2 and 3.8.4 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. 

3.8.3 Procedures. Promptly after receipt by an indemnified party under this Section 3.8 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 3.8, 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 3.8, solely to the extent that such failure prejudices the indemnifying party’s ability to defend such
action. 
 3.8.4 Contribution. To provide for just and equitable contribution to joint liability under the Securities Act in any
case in which either (a) any party otherwise entitled to indemnification hereunder makes a claim for indemnification pursuant to this Section 3.8 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 3.8 provides for indemnification in
such case, or (b) contribution under the Securities Act may be required on the part of any party hereto for which indemnification is provided under this Section 3.8, 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 

  
 15 

 
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: 

(i) in any such case, (A) no Holder will be required to contribute any amount in excess of the public offering price of all such
Registrable Securities offered and sold by such Holder pursuant to such registration statement, and (B) 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 
 (ii) in no event shall a Holder’s
liability pursuant to this Section 3.8.4, when combined with the amounts paid or payable by such Holder pursuant to Section 3.8.2, 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. 
 3.8.5 Underwriting Agreement Controls. 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. 
 3.8.6 Survival. 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 3.8 shall survive the completion of any offering of Registrable Securities in a registration under this
Section 3, and otherwise shall survive the termination of this Agreement. 
 3.9 Reports under the 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) use commercially reasonable efforts to 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 

  
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 (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); and (ii) 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). 
 3.10 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 to include such securities in any registration if such agreement (a) would allow such holder or prospective holder to include a portion of its securities in any “piggyback” registration if such inclusion could
reduce the number of Registrable Securities that selling Holders could be entitled to include in such registration under Sections 3.2 and 3.3.2 hereof or (b) would allow such holder or prospective holder to initiate a demand for registration of
any of its securities at a time earlier than the Holders of Registrable Securities can demand registration under Section 3.1 hereof. This Section 3.10 shall not apply with respect to the grant of “piggyback” registration rights
to a holder of a Lender Warrant. 
 3.11 “Market Stand-off” Agreement. Each
Holder hereby agrees that, during the Standoff Period, such Holder will not, without the prior written consent of the Company or the managing underwriter, 

(a) 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 
 (b) 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 (a) or (b) above is to be settled by delivery of Common Stock or other
securities, in cash, or otherwise. 
 The foregoing provisions of this Section 3.11 shall not apply to Registrable Securities included in the
registration statement for such offering, to shares acquired by an Investor or Genethon (as applicable) in an underwritten public offering or in an open market transaction following the underwritten public offering or to the transfer of any shares
to any trust for the direct or indirect 

  
 17 

 
benefit of the Holder or the immediate family of the Holder, provided that the trustee of the trust agrees to be bound in writing by the restrictions set forth herein, and provided further that
any such transfer shall not involve a disposition for value, 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 similarly bound with respect to the securities of the Company they hold that are not included in such registration statement. For purposes of this
Section 3.11, the term “Company” shall include any wholly-owned subsidiary of the Company into which the Company merges or consolidates. In order to enforce the foregoing covenant, the Company shall have the right to place restrictive
legends on the certificates representing the shares subject to this Section 3.11 and to impose stop transfer instructions with respect to such shares until the end of such period. The underwriters in connection with such registration are
intended third-party beneficiaries of this Section 3.11 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 3.11 or that are necessary to give further effect thereto. 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. 

3.12 Restrictions on Transfer. 

3.12.1 Agreement Binding. The Preferred Stock and the Registrable Securities shall not be sold, pledged, or otherwise transferred, and
the Company shall not recognize and shall issue stop-transfer instructions to its transfer agent with respect to 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. 
 3.12.2 Legends. Each certificate or instrument
representing (a) the Preferred Stock, (b) the Registrable Securities, and (c) any other securities issued in respect of the securities referenced in clauses (a) and (b), upon any stock split, stock dividend,
recapitalization, merger, consolidation, or similar event, shall (unless otherwise permitted by the provisions of Section 3.12.3) be stamped or otherwise imprinted with a legend substantially in the following form: 

THE SHARES REPRESENTED BY THIS CERTIFICATE HAVE NOT BEEN REGISTERED UNDER THE SECURITIES ACT OF 1933, AS AMENDED, AND HAVE BEEN ACQUIRED FOR
INVESTMENT AND NOT WITH A VIEW TO, OR IN CONNECTION WITH, THE SALE OR DISTRIBUTION THEREOF. NO TRANSFER MAY BE EFFECTED WITHOUT AN EFFECTIVE REGISTRATION STATEMENT RELATED THERETO OR AN OPINION OF COUNSEL IN A FORM REASONABLY SATISFACTORY TO THE
COMPANY THAT SUCH REGISTRATION IS NOT REQUIRED UNDER THE SECURITIES ACT OF 1933, AS AMENDED. 

  
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 THE SHARES REPRESENTED BY THIS CERTIFICATE 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 3.12. 

3.12.3 Procedure. The holder of each certificate representing Restricted Securities, by acceptance thereof, agrees to comply in all
respects with the provisions of this Section 3. 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 (a) 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; (b) 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 (c) 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 (i) in any transaction in compliance with SEC Rule 144 or (ii) 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 3.12. 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 3.12.2, 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. Until the IPO, no Holder shall transfer any
Restricted Securities to any person or entity that is determined to be a competitor of the Company, in the good faith judgment of the Board. 

4. RIGHTS TO FUTURE STOCK ISSUANCES. Subject to the terms and conditions of this Section 4 and applicable securities laws,
if the Company proposes to sell any New Securities, the Company shall offer to sell a portion of New Securities to each Major Investor as described in this Section 4. A Major Investor shall be entitled to apportion the right of first refusal
hereby granted to it among itself and its Affiliates in such proportions as it deems appropriate. The right of first refusal in this Section 4 shall not be applicable with respect to any Major Investor, if at the time of such subsequent
securities issuance, the Major Investor is not an “accredited investor,” as that term is then defined in Rule 501(a) under the Securities Act. 

  
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 4.1 Company Notice. The Company shall give notice (the “Offer
Notice”) to each Major Investor, stating (a) its bona fide intention to sell such New Securities, (b) the number of such New Securities to be sold and (c) the price and terms, if any, upon which it proposes to sell such
New Securities. 
 4.2 Investor Right. By written notice (the “Investor Notice”) 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 such Major Investor’s Pro Rata Amount. In addition, each
Major Investor that elects to purchase or acquire all of its Pro Rata Amount (each, a “Fully Exercising Investor”) may, in the Investor Notice, elect to purchase or acquire, in addition to its Pro Rata Amount, a portion of
the New Securities, if any, for which other Major Investors were entitled to subscribe but that are not subscribed for by such Major Investors. The amount of such overallotment that each Fully Exercising Investor shall be entitled to purchase is
equal to the proportion that the Common Stock issued and held, or issuable (directly or indirectly) upon conversion and/or exercise, as applicable, of Preferred Stock and any other Derivative Securities then held, by such Fully Exercising Investor
bears to the Common Stock issued and held, or issuable (directly or indirectly) upon conversion and/or exercise, as applicable, of the Preferred Stock and any other Derivative Securities then held, by all Fully Exercising Investors who wish to
purchase such unsubscribed shares. A Major Investor’s election may be conditioned on the consummation of the transaction described in the Offer Notice. The closing of any sale pursuant to this Section 4.2 shall occur on the earlier of one
hundred and twenty (120) days after the date that the Offer Notice is given and the date of initial sale of New Securities pursuant to Section 4.3. 

4.3 Sale of Securities. If all New Securities referred to in the Offer Notice are not elected to be purchased or acquired as
provided in Section 4.2, the Company may, during the ninety (90) day period following the expiration of the periods provided in Section 4.2, 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. 
 4.4 Alternate Procedure. Notwithstanding any provision hereof to the contrary, in lieu of complying with
the provisions of Sections 4.1 and 4.2, the Company may elect to give notice to the Major Investors within thirty (30) days after the issuance of New Securities. Such notice shall describe the type, price, and terms of the New Securities, and
the identities of the Persons to whom the New Securities were sold. Each Major Investor shall have twenty (20) days from the date notice is given to elect to purchase up to the number of New Securities that would, if purchased by such Major
Investor, maintain such Major Investor’s Pro Rata Amount before giving effect to the issuance of such New Securities. Any Major Investors electing to purchase such New Securities shall also have rights of oversubscription to purchase New
Securities that were purchasable by other Major Investors pursuant to the foregoing sentence but were not so purchased, and such rights of oversubscription shall be apportioned in a manner consistent with the apportionment among Fully Exercising
Investors described in Section 4.2. The closing of such sale shall occur within sixty (60) days of the date notice is given to the Major Investors. 

  
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 5. ADDITIONAL COVENANTS. 

5.1 Stock Options. All stock and stock equivalents issued to employees, directors, consultants and other service providers
(other than equity securities issued and outstanding as of the date of this Agreement to Matthew Patterson, Thomas Schuetz or OrbiMed Private Investments IV, LP) will be subject to vesting as follows (unless different vesting is approved by the
Board, including at least two (2) of the Preferred Directors (as defined in the Restated Certificate)): 25% to vest at the end of the first year following continued employment or service (or the date of such issuance in the case of a grant to
an existing employee or service provider), with the remaining 75% to vest quarterly over the next three (3) years. If employees or other service providers are permitted to exercise unvested options, the Company shall issue restricted stock to
such employees or service providers, whereby upon termination of the employment or other services of such stockholder, with or without cause, the Company or its assignee (to the extent permissible under applicable securities law qualification) shall
have the right to repurchase at cost (or if less, at the fair market value) any unvested shares of such stock held by such stockholder.  

6. TERMINATION. 

6.1 Generally. The covenants set forth in Section 2.1, Section 2.2 and Section 4 shall terminate and be of no
further force or effect upon the earliest to occur of: (a) immediately before the consummation of the IPO in connection with which all of the outstanding Preferred Stock of the Company is converted into Common Stock; (b) when the Company
first becomes subject to the periodic reporting requirements of Section 12(g) or 15(d) of the Exchange Act; or (c) upon a Deemed Liquidation Event or a Stock Sale. 

6.2 Registration Rights. The right of any Holder to request registration or inclusion of Registrable Securities in any
registration pursuant to Section 3.1 or Section 3.2 shall terminate upon the earliest to occur of: (a) when all of such Holder’s Registrable Securities could be sold without any restriction on volume or manner of sale in any
three (3) month period under SEC Rule 144 or any successor; (b) upon a Deemed Liquidation Event or a Stock Sale; and (c) the fifth (5th) anniversary of the IPO. 

7. GENERAL PROVISIONS. 

7.1 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 (a) is an Affiliate, partner, member, limited partner, retired or former partner, retired or former member, or stockholder of a Holder or such Holder’s Affiliate; (b) 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; (c) after such transfer, holds at least two percent (2%) of the shares of Registrable Securities (or if
the transferring Holder owns less than two percent (2%) of the Registrable Securities, then all Registrable Securities held by the transferring Holder); or (d) is a venture capital or other investment fund that is controlled by or under
common control with one or more general partners or managing partners or managing members 

  
 21 

 
of, or shares the same management company or investment advisor (or sub-advisor) with, the Holder; provided, however, that (i) 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 (ii) 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 3.11. For the purposes of determining the number of shares of Registrable Securities held by a transferee,
the holdings of a transferee (A) that is an Affiliate, limited partner, retired or former partner, member, retired or former member, or stockholder of a Holder or such Holder’s Affiliate; (B) who is a Holder’s Immediate Family
Member; or (C) 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. 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. 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. 

7.2 Governing Law. This Agreement shall be governed by, and construed in accordance with, the laws of the State of California,
regardless of the laws that might otherwise govern under applicable principles of conflicts of laws. 
 7.3 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. 

7.4 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. 
 7.5 Notices. All notices, requests, and other communications given, made or
delivered pursuant to this Agreement shall be in writing and shall be deemed effectively given, made or delivered upon the earlier of actual receipt or: (a) personal delivery to the party to be notified; (b) when sent, if sent by facsimile
during the recipient’s normal business hours, and if not sent during normal business hours, then on the recipient’s next business day; (c) five (5) days after having been sent by registered or certified mail, return receipt
requested, postage prepaid; or (d) one (1) business day after the business day of deposit with a nationally recognized overnight courier, freight prepaid, 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
address or facsimile number as subsequently modified by written notice given in accordance with this Section 7.5. If notice is given to Genethon, it shall be sent to the address set forth on Genethon’s signature page hereto. If notice is
given to the Company, it shall be sent to 101 Montgomery Street, Suite 2650, San Francisco, CA 94104, marked “Attention: Chief Executive Officer”; and a copy (which shall not constitute notice) shall also be sent to Fenwick & West
LLP, 555 California Street, 12th Floor, San Francisco, California 94104, Attn: Matthew Rossiter. If no facsimile number is listed on Schedule A for a party (or above in the case of the Company), notices and communications given or made by
facsimile shall not be deemed effectively given to such party. 

  
 22 

 7.6 Amendments and Waivers. This Agreement may only be amended or terminated and
the observance of any term hereof may be waived (either generally or in a particular instance, and either retroactively or prospectively) only by a written instrument executed by the Company and the holders of at least 66.67% of the shares of Common
Stock issued or issuable upon conversion of the then outstanding shares of the Preferred Stock held by the Investors (voting together as a single class and on an as-converted basis); provided, that (i) the Company may in its sole
discretion waive compliance with Section 3.12 (and the Company’s failure to object promptly in writing after notification of a proposed assignment allegedly in violation of Section 3.12 shall be deemed to be a waiver); (ii) any
provision hereof may be waived by any waiving party on such party’s own behalf, without the consent of any other party; (iii) any amendment, termination or waiver of Section 2.1.1, Section 2.3 or Section 3 shall require the
approval of the Company and the Requisite Investors, and (iv) the Company may, without the consent or approval of any other party hereto, cause additional persons to become party to this Agreement as Investors or Lenders pursuant to
Section 7.14 hereto and amend Schedule A hereto accordingly. Notwithstanding the foregoing, this Agreement may not be amended or terminated and the observance of any term hereof may not be waived if such amendment, termination or waiver
materially adversely affects the rights of any Investor or Genethon in a manner which is not the same as or similar in all material respects to the way in which it affects the respective rights of the other Investors or Genethon (as applicable)
without the consent of such Investor or Genethon (as applicable) (it being agreed that a waiver of the provisions of Section 4 with respect to a particular transaction shall not be deemed to apply similarly to Investors in all material respects
if certain Investors, by agreement with the Company, purchase securities in such transaction). Any amendment, termination, or waiver effected in accordance with this Section 7.6 shall be binding on each party hereto and all of such party’s
successors and permitted assigns, regardless of whether or not any such party, successor or assignee entered into or approved such amendment, termination, or waiver. 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. 

7.7 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. 
 7.8 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 persons may apportion such rights as among themselves in any
manner they deem appropriate. 
 7.9 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
and replaced with this Agreement. 

  
 23 

 7.10 Third Parties. Nothing in this Agreement, express or implied, is intended to
confer upon any person, other than the parties hereto and their successors and assigns, any rights or remedies under or by reason of this Agreement. 

7.11 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. 
 7.12 Dispute Resolution.
The parties (a) hereby irrevocably and unconditionally submit to the jurisdiction of the federal or state courts located in the Northern District of California (or the State of Delaware with respect to Genethon) for the purpose of any suit,
action or other proceeding arising out of or based upon this Agreement, (b) agree not to commence any suit, action or other proceeding arising out of or based upon this Agreement except in the federal or state courts located in the Northern
District of California (or the State of Delaware with respect to Genethon), and (c) hereby waive, and agree not to assert, by way of motion, as a defense, or otherwise, in any such suit, action or proceeding, any claim that a party is not
subject to the jurisdiction of the above-named courts, that its property is exempt or immune from attachment or execution based upon judgment or order of such court(s), that any suit, action or proceeding arising out of or based upon this Agreement
commenced in the federal or state courts located in the Northern District of California (or the State of Delaware with respect to Genethon) is brought in an inconvenient forum, that the venue of such suit, action or proceeding is improper or that
this Agreement or the subject matter hereof may not be enforced in or by such court. Should any party commence a suit, action or other proceeding arising out of or based upon this Agreement in a forum other than the federal or state courts located
in the Northern District of California (or the State of Delaware with respect to Genethon), or should any party otherwise seek to transfer or dismiss such suit, action or proceeding from such court(s), that party shall indemnify and reimburse the
other party for all legal costs and expenses incurred in enforcing this provision. 
 7.13 Attorneys’ Fees. If any action
at law or in equity is necessary to enforce or interpret the terms of this Agreement, the non-prevailing party shall pay all costs and expenses incurred by the prevailing party, including, without limitation, all reasonable attorneys’ fees.

 7.14 Additional Investors and Lenders. 

7.14.1 Notwithstanding anything to the contrary contained herein, if the Company issues additional shares of the Company’s Series C
Preferred Stock after the date hereof, any purchaser of such shares of Series C Preferred Stock may become a party to this Agreement by executing and delivering an additional counterpart signature page to this Agreement, and thereafter shall be
deemed an “Investor” for all purposes hereunder. 

  
 24 

 7.14.2 Notwithstanding anything to the contrary contained herein, if the Company issues any
Lender Warrant, any recipient of a Lender Warrant may become a party to this Agreement by executing and delivering an additional counterpart signature page to this Agreement, and thereafter shall be deemed a “Lender” for all purposes
hereunder. 
 7.14.3 Notwithstanding anything to the contrary contained herein, each Cardiogen Holder may become a party to this Agreement
by executing and delivering an additional counterpart signature page to this Agreement and thereafter shall be deemed an “Investor” for all purposes hereunder. 

7.14.4 No action or consent by the Investors shall be required for such joinder to this Agreement by such additional Investor or Lender, so
long as such additional Investor or Lender has agreed in writing to be bound by all of the obligations as an “Investor” or a “Lender” hereunder, as applicable. 

7.15 Prior Rights Agreement. The Prior Rights Agreement is hereby amended and restated to read as set forth in this Agreement
and the Prior Rights Agreement is hereby terminated, waived, released, replaced and superseded in its entirety by this Agreement and shall have no further force or effect. 

[SIGNATURE PAGES FOLLOW] 

  
 25 

 Execution Version 
  

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

			
	COMPANY:
	
	AUDENTES THERAPEUTICS, INC.
		
	By:	 	 /s/ Matthew Patterson

		
	Name:	 	 Matthew Patterson

		
	Title:	 	 President and Chief Executive Officer

  
 [SIGNATURE PAGE TO
AUDENTES THERAPEUTICS, INC. AMENDED AND 
 RESTATED INVESTORS’ RIGHTS AGREEMENT] 

 Execution Version 
  

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

			
	INVESTORS:
	
	ORBIMED PRIVATE INVESTMENTS IV, LP
		
	By:	 	 /s/ Jonathan Silverstein

		
	Name:	 	 Jonathan Silverstein

		
	Title:	 	 Member

  
 [SIGNATURE PAGE TO
AUDENTES THERAPEUTICS, INC. AMENDED AND 
 RESTATED INVESTORS’ RIGHTS AGREEMENT] 

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

			
	INVESTORS:
	
	5AM VENTURES III, L.P.
	
	By: 5AM Partners III, LLC, its General Partner
		
	By:	 	 /s/ Scott Rocklage

	Name:	 	Scott M. Rocklage
	Title:	 	Managing Member

  

			
	Address:	 	
		 	

  

			
	5AM CO-INVESTORS III, L.P.
	
	By: 5AM Partners III, LLC, its General Partner
		
	By:	 	 /s/ Scott Rocklage

	Name:	 	Scott M. Rocklage
	Title:	 	Managing Member

  

			
	Address:	 	
		 	

  
 [SIGNATURE PAGE TO
AUDENTES THERAPEUTICS, INC. AMENDED AND 
 RESTATED INVESTORS’ RIGHTS AGREEMENT] 

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

			
	INVESTORS:
	
	VERSANT VENTURE CAPITAL IV, L.P.
	VERSANT SIDE FUND IV, L.P.
		
	By:	 	Versant Ventures IV, LLC
	Its:	 	General Partner
		
	By:	 	 /s/ Tom Woiwode

		
	Name:	 	 Tom Woiwode

		
	Title:	 	 Managing Director

  
 [SIGNATURE PAGE TO
AUDENTES THERAPEUTICS, INC. AMENDED AND 
 RESTATED INVESTORS’ RIGHTS AGREEMENT] 

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

			
	INVESTORS:
	
	VENROCK HEALTHCARE CAPITAL PARTNERS II, L.P.
		
	By:	 	VHCP Management II, LLC
	Its:	 	General Partner
		
	By:	 	 /s/ David L. Stepp

		
	Name:	 	 David L. Stepp

		
	Title:	 	 Authorized Signatory

	
	VHCP CO-INVESTMENT HOLDINGS II, LLC
		
	By:	 	VHCP Management II, LLC
	Its:	 	Manager
		
	By:	 	 /s/ David L. Stepp

		
	Name:	 	 David L. Stepp

		
	Title:	 	 Authorized Signatory

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

			
	INVESTORS:
	
	SOFINNOVA VENTURE PARTNERS IX, L.P.
		
	By:	 	Sofinnova Management IX, L.L.C.
	Its:	 	General Partner
		
	By:	 	 /s/ James Healy

		
	Name:	 	 James Healy

		
	Title:	 	 Managing Member

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

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

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

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

			
	INVESTORS:
	
	COWEN PRIVATE INVESTMENTS LP
		
	By:	 	Cowen Private Investments GP LLC
	Its:	 	General Partner
		
	By:	 	 /s/ Owen Littman

		
	Name:	 	 Owen Littman

		
	Its:	 	 Authorized Signatory

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

			
	INVESTORS:
	
	FORESITE CAPITAL FUND III, L.P.
		
	By:	 	Foresite Capital Management III, LLC
	Its:	 	General Partner
		
	By:	 	 /s/ Dennis D. Ryan

	Name:	 	Dennis D. Ryan
	Its:	 	Chief Financial Officer

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

			
	INVESTORS:
	
	RA CAPITAL HEALTHCARE FUND, L.P.
		
	By:	 	RA Capital Management, LLC
	Its:	 	General Partner
		
	By:	 	 /s/ Peter Kolchinsky

		
	Name:	 	 Peter Kolchinsky

		
	Its:	 	 Authorized Signatory

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

									
	INVESTORS:	 		 	
			
	BLACKWELL PARTNERS LLC – SERIES A	 		 	
					
	By:	 	 /s/ Justin B. Nixon
	 		 	By:	 	 /s/ Janine M. Lall

					
	Name:	 	 Investment Manager
	 		 	Name:	 	 Assistant Treasurer

					
		 	 DUMAC, Inc.
	 		 		 	 DUMAC, Inc.

					
		 	 Authorized Agent
	 		 		 	 Authorized Agent

					
	Its:	 	 Authorized Signatory
	 		 	Its:	 	 Authorized Signatory

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

			
	INVESTORS:
	
	ROCK SPRINGS CAPITAL MASTER FUND LP
		
	By:	 	Rock Springs GP LLC
	Its:	 	General Partner
		
	By:	 	 /s/ Graham McPhail

	Name:	 	Graham McPhail
	Its:	 	Managing Director

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

							
	INVESTORS:
	
	DEERFIELD SPECIAL SITUATIONS FUND, L.P.
		
	By:	 	Deerfield Mgmt, L.P.
		 	General Partner
		 	By:	 	J.E. Flynn Capital, LLC
		 		 	General Partner
			
		 	By:	 	 /s/ David J. Clark

		 		 	Name:	 	David J. Clark
		 		 	Title:	 	Authorized Signatory
	
	DEERFIELD PRIVATE DESIGN FUND III, L.P.
		
	By:	 	Deerfield Mgmt III, L.P.
		 	General Partner
		 	By:	 	J.E. Flynn Capital III, LLC
		 		 	General Partner
			
		 	By:	 	 /s/ David J. Clark

		 		 	Name:	 	David J. Clark
		 		 	Title:	 	Authorized Signatory

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

			
	INVESTORS:
	
	T. ROWE PRICE HEALTH SCIENCES FUND, INC.
	TD MUTUAL FUNDS – TD HEALTH SCIENCES FUND
	VALIC COMPANY I – HEALTH SCIENCES FUND
	T. ROWE PRICE HEALTH SCIENCES PORTFOLIO
	JOHN HANCOCK VARIABLE INSURANCE TRUST – HEALTH SCIENCES TRUST
	JOHN HANCOCK FUNDS II – HEALTH SCIENCES FUND

			
	
	Each fund, severally and not jointly
		
	By:	 	T. Rowe Price Associates, Inc., Investment Adviser or Subadviser, as applicable
		
	By:	 	 /s/ Ziad Bakri

		
	Name:	 	 /s/ Ziad Bakri

		
	Title:	 	 Vice President

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

			
	INVESTORS:
	
	REDMILE CAPITAL FUND, LP
		
	By:	 	 /s/ Jeremy Green

	Name:	 	 Jeremy Green

	Its:	 	 Managing Member of the General Partner and the Investment Manager

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

			
	INVESTORS:
	
	REDMILE CAPITAL OFFSHORE FUND, LTD.
		
	By:	 	 /s/ Jeremy Green

	Name:	 	 Jeremy Green

	Its:	 	 Managing Member of the Investment Manager

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

			
	INVESTORS:
	
	REDMILE CAPITAL OFFSHORE FUND II, LTD.
		
	By:	 	 /s/ Jeremy Green

	Name:	 	 Jeremy Green

	Its:	 	 Managing Member of the Investment Manager

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

			
	INVESTORS:
	
	REDMILE SPECIAL OPPORTUNITIES FUND, LTD.
		
	By:	 	 /s/ Jeremy Green

	Name:	 	 Jeremy Green

	Its:	 	 Managing Member of the Investment Manager

  

[SIGNATURE PAGE TO AUDENTES THERAPEUTICS, INC.
AMENDED AND 
 RESTATED INVESTORS’ RIGHTS
AGREEMENT] 

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

			
	INVESTORS:
	
	REDMILE BIOTECHNOLOGIES INVESTMENTS I AF, LP
		
	By:	 	 /s/ Jeremy Green

	Name:	 	 Jeremy Green

	Its:	 	 Managing Member of the Investment Mngr./Mngt. Company (the Managing Member of the GP)

 SCHEDULE A 

List of Investors 
  

	
	 Name and Address of Investor

	Orbimed Private Investments IV, LP
	
	5AM Ventures III, L.P.
	
	5AM Co-Investors III, L.P.
	
	Versant Venture Capital IV, L.P.
	
	Versant Side Fund IV, L.P.
	
	Deerfield Special Situations Fund, L.P.
	
	Deerfield Private Design Fund III, L.P.
	
	Venrock Healthcare Capital Partners II, L.P.
	
	Genethon
	
	VHCP Co-Investment Holdings II, LLC
	
	Sofinnova Venture Partners IX, L.P.
	
	Redmile Capital Fund, LP
	
	Redmile Capital Offshore Fund, Ltd.
	
	Redmile Capital Offshore Fund II, Ltd.

	
	 Name and Address of Investor

	Redmile Special Opportunities Fund, Ltd.
	
	Redmile Biotechnologies Investments I AF, LP
	
	Cowen Private Investments LP
	
	Foresite Capital Fund III, L.P.
	
	Rock Springs Capital Master Fund LP
	
	RA Capital Healthcare Fund, L.P.
	
	Blackwell Partners LLC – Series A
	
	Cormorant Global Healthcare Master Fund, LP
	
	 T. Rowe Price Health Sciences Fund, Inc.

	
	 TD Mutual Funds – TD Health Sciences Fund

	
	 VALIC Company I – Health Sciences Fund

	
	 T. Rowe Price Health Sciences Portfolio

	
	 John Hancock Variable Insurance Trust – Health Sciences Trust

	
	 Name and Address of Investor

	 John Hancock Funds II – Health Sciences Fund

	
	Lange Minors’ Trust
	
	Peter M. Joost & Lindsay M. Joost, Trustees U/T/A dated April 11, 2002
	
	Oliver Reynolds Joost 1992 Trust
	
	Caroline Lapham Joost 1994 Trust
	
	Annabel Ward Joost 1997 Trust
	
	J. Leighton Read
	
	Adjuvant Partners, LLC
	
	Louis G. Lange
	
	Camp Lowell, LLC
	
	Asset Management Ventures Fund, L.P.
	
	The Diamond Revocable Trust
	
	Amygdala Lange Trust
	
	Alberto Auricchio2012 Equity Incentive Plan

 Exhibit 10.2 

AUDENTES THERAPEUTICS, INC. 

2012 EQUITY INCENTIVE PLAN 

As Adopted on December 21, 2012 

1. PURPOSE. The purpose of this Plan is to provide incentives to attract, retain and motivate eligible persons whose present and
potential contributions are important to the success of the Company, its Parent and Subsidiaries by offering eligible persons an opportunity to participate in the Company’s future performance through the grant of Awards covering Shares.
Capitalized terms not defined in the text are defined in Section 14 hereof. Although this Plan is intended to be a written compensatory benefit plan within the meaning of Rule 701, grants may be made pursuant to this Plan that do not
qualify for exemption under Rule 701 or Section 25102(o). Any requirement of this Plan that is required in law only because of Section 25102(o) need not apply if the Committee so provides. 

2. SHARES SUBJECT TO THE PLAN. 

2.1 Number of Shares Available. Subject to Sections 2.2 and 11 hereof, the total number of Shares reserved and available
for grant and issuance pursuant to this Plan will be 400,000 Shares. Subject to Sections 2.2 and 11 hereof, Shares subject to Awards that are cancelled, forfeited, settled in cash, used to pay withholding obligations or pay the exercise price of an
Option or that expire by their terms at any time will again be available for grant and issuance in connection with other Awards. In the event that Shares previously issued under the Plan are reacquired by the Company pursuant to a forfeiture
provision, right of first refusal, or repurchase by the Company, such Shares shall be added to the number of Shares then available for issuance under the Plan. At all times the Company will reserve and keep available a sufficient number of Shares as
will be required to satisfy the requirements of all Awards granted and outstanding under this Plan. In no event shall the total number of Shares issued (counting each reissuance of a Share that was previously issued and then forfeited or repurchased
by the Company as a separate issuance) under the Plan upon exercise of ISOs exceed 4,000,000 Shares (adjusted in proportion to any adjustments under Section 2.2 hereof) over the term of the Plan (the “ISO Limit”).
Subject to Sections 2.2 and 11 hereof, in the event that the number of Shares reserved for issuance under the Plan is increased, the ISO Limit shall be automatically increased by such number of Shares such that the ISO Limit equals (a) ten
(10) multiplied by (b) the number of Shares reserved for issuance under the Plan. 
 2.2 Adjustment of Shares. In
the event that the number of outstanding shares of the Company’s Common Stock is changed by a stock dividend, recapitalization, stock split, reverse stock split, subdivision, combination, reclassification or other change in the capital
structure of the Company affecting Shares without consideration, then in order to prevent diminution or enlargement of the benefits or potential benefits intended to be made available under the Plan (a) the number of Shares reserved for
issuance under this Plan, (b) the Exercise Prices of and number of Shares subject to outstanding Options and SARs, and (c) the Purchase Prices of and/or number of Shares subject to other outstanding Awards will be proportionately adjusted,
subject to any required action by the Board or the stockholders of the 

 
Company and compliance with applicable securities laws; provided, however, that fractions of a Share will not be issued but will either be paid in cash at the Fair
Market Value of such fraction of a Share or will be rounded down to the nearest whole Share, as determined by the Committee. 
 3.
PLAN FOR BENEFIT OF SERVICE PROVIDERS. 
 3.1 Eligibility. The Committee will have the authority to select persons
to receive Awards. ISOs (as defined in Section 4 hereof) may be granted only to employees (including officers and directors who are also employees) of the Company or of a Parent or Subsidiary of the Company. NQSOs (as defined in Section 4
hereof) and all other types of Awards may be granted to employees, officers, directors and consultants of the Company or any Parent or Subsidiary of the Company; provided such consultants render bona fide services not in connection
with the offer and sale of securities in a capital-raising transaction when Rule 701 is to apply to the Award granted for such services. A person may be granted more than one Award under this Plan. 

3.2 No Obligation to Employ. Nothing in this Plan or any Award granted under this Plan will confer or be deemed to confer on any
Participant any right to continue in the employ of, or to continue any other relationship with, the Company or any Parent or Subsidiary or limit in any way the right of the Company or any Parent or Subsidiary to terminate Participant’s
employment or other relationship at any time, with or without Cause. 
 4. OPTIONS. The Committee may grant Options to
eligible persons described in Section 3 hereof and will determine whether such Options will be Incentive Stock Options within the meaning of the Code (“ISOs”) or Nonqualified Stock Options
(“NQSOs”), the number of Shares subject to the Option, the Exercise Price of the Option, the period during which the Option may be exercised, and all other terms and conditions of the Option, subject to the following. 

4.1 Form of Option Grant. Each Option granted under this Plan will be evidenced by an Award Agreement which will expressly
identify the Option as an ISO or an NQSO (“Stock Option Agreement”), and will be in such form and contain such provisions (which need not be the same for each Participant) as the Committee may from time to time approve, and
which will comply with and be subject to the terms and conditions of this Plan. 
 4.2 Date of Grant. The date of grant of an
Option will be the date on which the Committee makes the determination to grant such Option, unless a later date is otherwise specified by the Committee. The Stock Option Agreement and a copy of this Plan will be delivered to the Participant within
a reasonable time after the granting of the Option. 
 4.3 Exercise Period. Options may be exercisable within the time or upon
the events determined by the Committee in the Award Agreement and may be awarded as immediately exercisable but subject to repurchase pursuant to Section 10 hereof or may be exercisable within the times or upon the events determined by the
Committee as set forth in the Stock Option Agreement governing such Option; provided, however, that (a) no Option will be exercisable after the expiration of ten (10) years from the date the Option is granted;
and (b) no 

 
ISO granted to a person who directly or by attribution owns more than ten percent (10%) of the total combined voting power of all classes of stock of the Company or of any Parent or
Subsidiary (“Ten Percent Stockholder”) will be exercisable after the expiration of five (5) years from the date the ISO is granted. The Committee also may provide for Options to become exercisable at one time or from
time to time, periodically or otherwise, in such number of Shares or percentage of Shares as the Committee determines. 
 4.4 Exercise
Price. The Exercise Price of an Option will be determined by the Committee when the Option is granted and shall not be less than the Fair Market Value per Share unless expressly determined in writing by the Committee on the Option’s
date of grant; provided that the Exercise Price of an ISO granted to a Ten Percent Stockholder will not be less than one hundred ten percent (110%) of the Fair Market Value of the Shares on the date of grant. Payment for the
Shares purchased must be made in accordance with Section 8 hereof. 
 4.5 Method of Exercise. Options may be exercised
only by delivery to the Company of a written stock option exercise agreement (the “Exercise Agreement”) in a form approved by the Committee (which need not be the same for each Participant). The Exercise Agreement will state
(a) the number of Shares being purchased, (b) the restrictions imposed on the Shares purchased under such Exercise Agreement, if any, and (c) such representations and agreements regarding Participant’s investment intent and
access to information and other matters, if any, as may be required or desirable by the Company to comply with applicable securities laws. Each Participant’s Exercise Agreement may be modified by (i) agreement of Participant and the
Company or (ii) substitution by the Company, upon becoming a public company, in order to add the payment terms set forth in Section 8.1 that apply to a public company and such other terms as shall be necessary or advisable in order to
exercise a public company option. Upon exercise of an Option, Participant shall execute and deliver to the Company the Exercise Agreement then in effect, together with payment in full of the Exercise Price for the number of Shares being purchased
and payment of any applicable taxes. No adjustment will be made for a dividend or other right for which the record date is prior to the date the Shares are issued, except as provided in Section 2.2 of the Plan. Exercising an Option in any
manner will decrease the number of Shares thereafter available, both for purposes of the Plan and for sale under the Option, by the number of Shares as to which the Option is exercised. 

4.6 Termination. Subject to earlier termination pursuant to Sections 11 and 13.1 hereof and notwithstanding the exercise
periods set forth in the Stock Option Agreement, exercise of an Option will always be subject to the following terms and conditions. 

4.6.1 Other than Death or Disability or for Cause. If the Participant is Terminated for any reason other than death, Disability or for
Cause, then the Participant may exercise such Participant’s Options only to the extent that such Options are exercisable as to Vested Shares upon the Termination Date or as otherwise determined by the Committee. Such Options must be exercised
by the Participant, if at all, as to all or some of the Vested Shares calculated as of the Termination Date or such other date determined by the Committee, within three (3) months after the Termination Date (or within such shorter time period,
not less than thirty (30) days, or within such longer time period after the Termination Date as may be determined by the Committee, with any exercise beyond three (3) months after the Termination Date deemed to be an NQSO) but in any
event, no later than the expiration date of the Options. 

 4.6.2 Death or Disability. If the Participant is Terminated because of Participant’s
death or Disability (or the Participant dies within three (3) months after a Termination other than for Cause), then Participant’s Options may be exercised only to the extent that such Options are exercisable as to Vested Shares by
Participant on the Termination Date or as otherwise determined by the Committee. Such options must be exercised by Participant (or Participant’s legal representative or authorized assignee), if at all, as to all or some of the Vested Shares
calculated as of the Termination Date or such other date determined by the Committee, within twelve (12) months after the Termination Date (or within such shorter time period, not less than six (6) months, or within such longer time
period, after the Termination Date as may be determined by the Committee, with any exercise beyond (a) three (3) months after the Termination Date when the Termination is for any reason other than the Participant’s death or
disability, within the meaning of Section 22(e)(3) of the Code, or (b) twelve (12) months after the Termination Date when the Termination is for Participant’s disability, within the meaning of Section 22(e)(3) of the Code,
deemed to be an NQSO) but in any event no later than the expiration date of the Options. 
 4.6.3 For Cause. If the Participant is
terminated for Cause, the Participant may exercise such Participant’s Options, but not to an extent greater than such Options are exercisable as to Vested Shares upon the Termination Date and Participant’s Options shall expire on such
Participant’s Termination Date, or at such later time and on such conditions as are determined by the Committee. 
 4.7
Limitations on Exercise. The Committee may specify a reasonable minimum number of Shares that may be purchased on any exercise of an Option, provided that such minimum number will not prevent Participant from exercising the
Option for the full number of Shares for which it is then exercisable. 
 4.8 Limitations on ISOs. The aggregate Fair Market
Value (determined as of the date of grant) of Shares with respect to which ISOs are exercisable for the first time by a Participant during any calendar year (under this Plan or under any other incentive stock option plan of the Company or any Parent
or Subsidiary of the Company) will not exceed One Hundred Thousand Dollars ($100,000). If the Fair Market Value of Shares on the date of grant with respect to which ISOs are exercisable for the first time by a Participant during any calendar year
exceeds One Hundred Thousand Dollars ($100,000), then the Options for the first One Hundred Thousand Dollars ($100,000) worth of Shares to become exercisable in such calendar year will be ISOs and the Options for the amount in excess of One Hundred
Thousand Dollars ($100,000) that become exercisable in that calendar year will be NQSOs. In the event that the Code or the regulations promulgated thereunder are amended after the Effective Date (as defined in Section 13.1 hereof) to provide
for a different limit on the Fair Market Value of Shares permitted to be subject to ISOs, then such different limit will be automatically incorporated herein and will apply to any Options granted after the effective date of such amendment. 

 4.9 Modification, Extension or Renewal. The Committee may modify, extend or renew
outstanding Options and authorize the grant of new Options in substitution therefor, provided that any such action may not, without the written consent of a Participant, impair any of such Participant’s rights under any Option
previously granted. Any outstanding ISO that is modified, extended, renewed or otherwise altered will be treated in accordance with Section 424(h) of the Code. Subject to Section 4.10 hereof, the Committee may reduce the Exercise Price of
outstanding Options without the consent of Participants by a written notice to them; provided, however, that the Exercise Price may not be reduced below the minimum Exercise Price that would be permitted under
Section 4.4 hereof for Options granted on the date the action is taken to reduce the Exercise Price. 
 4.10 No
Disqualification. Notwithstanding any other provision in this Plan, no term of this Plan relating to ISOs will be interpreted, amended or altered, nor will any discretion or authority granted under this Plan be exercised, so as to disqualify
this Plan under Section 422 of the Code or, without the consent of the Participant, to disqualify any Participant’s ISO under Section 422 of the Code. 

5. RESTRICTED STOCK. A Restricted Stock Award is an offer by the Company to sell to an eligible person Shares that are subject
to certain specified restrictions. The Committee will determine to whom an offer will be made, the number of Shares the person may purchase, the Purchase Price, the restrictions to which the Shares will be subject, and all other terms and conditions
of the Restricted Stock Award, subject to the following terms and conditions. 
 5.1 Form of Restricted Stock Award. All
purchases under a Restricted Stock Award made pursuant to this Plan will be evidenced by an Award Agreement (“Restricted Stock Purchase Agreement”) that will be in such form (which need not be the same for each Participant)
as the Committee will from time to time approve, and will comply with and be subject to the terms and conditions of this Plan. The Restricted Stock Award will be accepted by the Participant’s execution and delivery of the Restricted Stock
Purchase Agreement and full payment for the Shares to the Company within thirty (30) days from the date the Restricted Stock Purchase Agreement is delivered to the person. If such person does not execute and deliver the Restricted Stock
Purchase Agreement along with full payment for the Shares to the Company within such thirty (30) days, then the offer will terminate, unless otherwise determined by the Committee. 

5.2 Purchase Price. The Purchase Price of Shares sold pursuant to a Restricted Stock Award will be determined by the Committee
on the date the Restricted Stock Award is granted or at the time the purchase is consummated. Payment of the Purchase Price must be made in accordance with Section 8 hereof. 

5.3 Dividends and Other Distributions. Participants holding Restricted Stock will be entitled to receive all dividends and other
distributions paid with respect to such Shares, unless the Committee provides otherwise at the time of award. If any such dividends or distributions are paid in Shares, the Shares will be subject to the same restrictions on transferability and
forfeitability as the Shares of Restricted Stock with respect to which they were paid. 
 5.4 Restrictions. Restricted Stock
Awards may be subject to the restrictions set forth in Sections 9 and 10 hereof or, with respect to a Restricted Stock Award to which Section 25102(o) is to apply, such other restrictions not inconsistent with Section 25102(o). 

 6. RESTRICTED STOCK UNITS. 

6.1 Awards of Restricted Stock Units. A Restricted Stock Unit (“RSU”) is an Award covering a number of
Shares that may be settled in cash, or by issuance of those Shares at a date in the future. No Purchase Price shall apply to an RSU settled in Shares. All grants of Restricted Stock Units will be evidenced by an Award Agreement that will be in such
form (which need not be the same for each Participant) as the Committee will from time to time approve, and will comply with and be subject to the terms and conditions of this Plan. 

6.2 Form and Timing of Settlement. To the extent permissible under applicable law, the Committee may permit a Participant to
defer payment under a RSU to a date or dates after the RSU is earned, provided that the terms of the RSU and any deferral satisfy the requirements of Section 409A of the Code (or any successor) and any regulations or rulings
promulgated thereunder. Payment may be made in the form of cash or whole Shares or a combination thereof, all as the Committee determines. 

7. STOCK APPRECIATION RIGHTS. 

7.1 Awards of SARs. Stock Appreciation Rights (“SARs”) may be settled in cash, or Shares (which may
consist of Restricted Stock or RSUs), having a value equal to the value determined by multiplying the difference between the Fair Market Value on the date of exercise over the Exercise Price and the number of Shares with respect to which the SAR is
being settled. All grants of SARs made pursuant to this Plan will be evidenced by an Award Agreement that will be in such form (which need not be the same for each Participant) as the Committee will from time to time approve, and will comply with
and be subject to the terms and conditions of this Plan. 
 7.2 Exercise Period and Expiration Date. A SAR will be exercisable
within the times or upon the occurrence of events determined by the Committee and set forth in the Award Agreement governing such SAR. The Award Agreement shall set forth the Expiration Date; provided that no SAR will be exercisable
after the expiration of ten years from the date the SAR is granted. 
 7.3 Exercise Price. The Committee will determine the
Exercise Price of the SAR when the SAR is granted, and which may not be less than the Fair Market Value on the date of grant and may be settled in cash or in Shares. 

7.4 Termination. Subject to earlier termination pursuant to Sections 11 and 13.1 hereof and notwithstanding the exercise
periods set forth in the Award Agreement, exercise of SARs will always be subject to the following terms and conditions. 
 7.4.1 Other
than Death or Disability or for Cause. If the Participant is Terminated for any reason other than death, Disability or for Cause, then the Participant may exercise such Participant’s SARs only to the extent that such SARs are exercisable as
to vested 

 
Shares upon the Termination Date or as otherwise determined by the Committee. SARs must be exercised by the Participant, if at all, as to all or some of the vested Shares calculated as of the
Termination Date or such other date determined by the Committee, within three (3) months after the Termination Date (or within such shorter time period, not less than thirty (30) days, or within such longer time period after the
Termination Date as may be determined by the Committee) but in any event, no later than the expiration date of the SARs. 
 7.4.2 Death
or Disability. If the Participant is Terminated because of Participant’s death or Disability (or the Participant dies within three (3) months after a Termination other than for Cause), then Participant’s SARs may be exercised only
to the extent that such SARs are exercisable as to vested Shares by Participant on the Termination Date or as otherwise determined by the Committee. Such SARs must be exercised by Participant (or Participant’s legal representative or authorized
assignee), if at all, as to all or some of the vested Shares calculated as of the Termination Date or such other date determined by the Committee, within twelve (12) months after the Termination Date (or within such shorter time period, not
less than six (6) months, or within such longer time period after the Termination Date as may be determined by the Committee) but in any event no later than the expiration date of the SARs. 

7.4.3 For Cause. If the Participant is terminated for Cause, the Participant may exercise such Participant’s SARs, but not to an
extent greater than such SARs are exercisable as to vested Shares upon the Termination Date and Participant’s SARs shall expire on such Participant’s Termination Date, or at such later time and on such conditions as are determined by the
Committee. 
 8. PAYMENT FOR PURCHASES AND EXERCISES. 

8.1 Payment in General. Payment for Shares acquired pursuant to this Plan may be made in cash (by check) or, where expressly
approved for the Participant by the Committee and where permitted by law: 
 (a) by cancellation of indebtedness of the Company owed to the
Participant; 
 (b) by surrender of shares of the Company that are clear of all liens, claims, encumbrances or security interests and:
(i) for which the Company has received “full payment of the purchase price” within the meaning of SEC Rule 144 (and, if such shares were purchased from the Company by use of a promissory note, such note has been fully paid with
respect to such shares) or (ii) that were obtained by Participant in the public market; 
 (c) by tender of a full recourse promissory
note having such terms as may be approved by the Committee and bearing interest at a rate sufficient to avoid imputation of income under Sections 483 and 1274 of the Code; provided, however, that Participants who are
not employees or directors of the Company will not be entitled to purchase Shares with a promissory note unless the note is adequately secured by collateral other than the Shares; provided, further, that the portion of
the Exercise Price or Purchase Price, as the case may be, equal to the par value (if any) of the Shares must be paid in cash or other legal consideration permitted by the laws under which the Company is then incorporated or organized; 

 (d) by waiver of compensation due or accrued to the Participant from the Company for services
rendered; 
 (e) by participating in a formal cashless exercise program implemented by the Committee in connection with the Plan; 

(f) subject to compliance with applicable law and solely in the discretion of the Committee, provided that a public market for the
Company’s Common Stock exists, by exercising through a “same day sale” commitment from the Participant and a broker-dealer whereby the Participant irrevocably elects to exercise the Award and to sell a portion of the Shares so
purchased sufficient to pay the total Exercise Price or Purchase Price, and whereby the broker-dealer irrevocably commits upon receipt of such Shares to forward the total Exercise Price or Purchase Price directly to the Company; or 

(g) by any combination of the foregoing or any other method of payment approved by the Committee. 

8.2 Withholding Taxes. 

8.2.1 Withholding Generally. Whenever Shares are to be issued in satisfaction of Awards granted under this Plan, the Company may
require the Participant to remit to the Company an amount sufficient to satisfy applicable tax withholding requirements prior to the delivery of any certificate or certificates for such Shares. Whenever, under this Plan, payments in satisfaction of
Awards are to be made in cash by the Company, such payment will be net of an amount sufficient to satisfy applicable tax withholding requirements. 

8.2.2 Stock Withholding. When, under applicable tax laws, a Participant incurs tax liability in connection with the exercise or
vesting of any Award that is subject to tax withholding and the Participant is obligated to pay the Company the amount required to be withheld, the Committee may in its sole discretion allow the Participant to satisfy the minimum tax withholding
obligation by electing to have the Company withhold from the Shares to be issued up to the minimum number of Shares having a Fair Market Value on the date that the amount of tax to be withheld is to be determined that is not more than the minimum
amount to be withheld; or to arrange a mandatory “sell to cover” on Participant’s behalf (without further authorization) but in no event will the Company withhold Shares or “sell to cover” if such withholding would result in
adverse accounting consequences to the Company. Any elections to have Shares withheld or sold for this purpose will be made in accordance with the requirements established by the Committee for such elections and be in writing in a form acceptable to
the Committee. 
 9. RESTRICTIONS ON AWARDS. 

9.1 Transferability. Except as permitted by the Committee, Awards granted under this Plan, and any interest therein, will not be
transferable or assignable by Participant, other than by will or by the laws of descent and distribution, and, with respect to NQSOs, by instrument to an inter vivos or testamentary trust in which the NQSOs are to be passed to beneficiaries upon the
death of the trustor (settlor), or by gift to “family member” as that term is defined in Rule 701, and may not be made subject to execution, attachment or similar process. 

 
For the avoidance of doubt, the prohibition against assignment and transfer applies to a stock option and, prior to exercise, the shares to be issued on exercise of a stock option, and pursuant
to the foregoing sentence shall be understood to include, without limitation, a prohibition against any pledge, hypothecation, or other transfer, including any short position, any “put equivalent position” or any “call equivalent
position” (in each case, as defined in Rule 16a-1 promulgated under the Exchange Act). During the lifetime of the Participant an Award will be exercisable only by the Participant or Participant’s legal representative and any elections with
respect to an Award may be made only by the Participant or Participant’s legal representative. The terms of an Option shall be binding upon the executor, administrator, successors and assigns of the Participant who is a party thereto. 

9.2 Securities Law and Other Regulatory Compliance. Although this Plan is intended to be a written compensatory benefit plan
within the meaning of Rule 701 promulgated under the Securities Act, grants may be made pursuant to this Plan that do not qualify for exemption under Rule 701 or Section 25102(o). Any requirement of this Plan which is required in law only
because of Section 25102(o) need not apply with respect to a particular Award to which Section 25102(o) will not apply. An Award will not be effective unless such Award is in compliance with all applicable federal and state securities
laws, rules and regulations of any governmental body, and the requirements of any stock exchange or automated quotation system upon which the Shares may then be listed or quoted, as they are in effect on the date of grant of the Award and also on
the date of exercise or other issuance. Notwithstanding any other provision in this Plan, the Company will have no obligation to issue or deliver certificates for Shares under this Plan prior to (a) obtaining any approvals from governmental
agencies that the Company determines are necessary or advisable, and/or (b) compliance with any exemption, completion of any registration or other qualification of such Shares under any state or federal law or ruling of any governmental body
that the Company determines to be necessary or advisable. The Company will be under no obligation to register the Shares with the SEC or to effect compliance with the exemption, registration, qualification or listing requirements of any state
securities laws, stock exchange or automated quotation system, and the Company will have no liability for any inability or failure so do. 

9.3 Exchange and Buyout of Awards. The Committee may, at any time or from time to time, authorize the Company, with the consent
of the respective Participants, to issue new Awards in exchange for the surrender and cancellation of any or all outstanding Awards. Without prior stockholder approval the Committee may reprice Options or SARs (and where such repricing is a
reduction in the Exercise Price of outstanding Options or SARs, the consent of the affected Participants is not required provided written notice is provided to them). The Committee may at any time buy from a Participant an Award previously granted
with payment in cash, Shares (including Restricted Stock) or other consideration, based on such terms and conditions as the Committee and the Participant may agree. 

10. RESTRICTIONS ON SHARES. 

10.1 Privileges of Stock Ownership. No Participant will have any of the rights of a stockholder with respect to any Shares until
such Shares are issued to the Participant. After Shares are issued to the Participant, the Participant will be a stockholder and have all the rights of a stockholder with respect to such Shares, including the right to vote and receive all

 
dividends or other distributions made or paid with respect to such Shares; provided, that if such Shares are Restricted Stock, then any new, additional or different securities the
Participant may become entitled to receive with respect to such Shares by virtue of a stock dividend, stock split or any other change in the corporate or capital structure of the Company will be subject to the same restrictions as the Restricted
Stock. The Participant will have no right to retain such stock dividends or stock distributions with respect to Unvested Shares that are repurchased as described in this Section 10. 

10.2 Rights of First Refusal and Repurchase. At the discretion of the Committee, the Company may reserve to itself and/or its
assignee(s) in the Award Agreement (a) a right of first refusal to purchase all Shares that a Participant (or a subsequent transferee) may propose to transfer to a third party, provided that such right of first refusal terminates
upon the Company’s initial public offering of Common Stock pursuant to an effective registration statement filed under the Securities Act and (b) a right to repurchase Unvested Shares held by a Participant for cash and/or cancellation of
purchase money indebtedness owed to the Company by the Participant following such Participant’s Termination at any time. 
 10.3
Escrow; Pledge of Shares. To enforce any restrictions on a Participant’s Shares, the Committee may require the Participant to deposit all certificates representing Shares, together with stock powers or other instruments of transfer
approved by the Committee, appropriately endorsed in blank, with the Company or an agent designated by the Company to hold in escrow until such restrictions have lapsed or terminated. The Committee may cause a legend or legends referencing such
restrictions to be placed on the certificate. Any Participant who is permitted to execute a promissory note as partial or full consideration for the purchase of Shares under this Plan will be required to pledge and deposit with the Company all or
part of the Shares so purchased as collateral to secure the payment of Participant’s obligation to the Company under the promissory note; provided, however, that the Committee may require or accept other or
additional forms of collateral to secure the payment of such obligation and, in any event, the Company will have full recourse against the Participant under the promissory note notwithstanding any pledge of the Participant’s Shares or other
collateral. In connection with any pledge of the Shares, Participant will be required to execute and deliver a written pledge agreement in such form as the Committee will from time to time approve. The Shares purchased with the promissory note may
be released from the pledge on a pro rata basis as the promissory note is paid. 
 10.4 Securities Law Restrictions. All
certificates for Shares or other securities delivered under this Plan will be subject to such stock transfer orders, legends and other restrictions as the Committee may deem necessary or advisable, including restrictions under any applicable
federal, state or foreign securities law, or any rules, regulations and other requirements of the SEC or any stock exchange or automated quotation system upon which the Shares may be listed or quoted. 

11. CORPORATE TRANSACTIONS. 

11.1 Acquisitions or Other Combinations. In the event that the Company is subject to an Acquisition or Other Combination,
outstanding Awards acquired under the Plan shall be subject to the agreement evidencing the Acquisition or Other Combination, which need 

 
not treat all outstanding Awards in an identical manner. Such agreement, without the Participant’s consent, shall provide for one or more of the following with respect to all outstanding
Awards as of the effective date of such Acquisition or Other Combination: 
 (a) The continuation of such outstanding Awards by the Company
(if the Company is the successor entity). 
 (b) The assumption of outstanding Awards by the successor or acquiring entity (if any) in such
Acquisition or Other Combination (or by any of its Parents, if any), which assumption, will be binding on all Participants; provided that the exercise price and the number and nature of shares issuable upon exercise of any such option or stock
appreciation right, or any award that is subject to Section 409A of the Code, will be adjusted appropriately pursuant to Section 424(a) and Section 409A of the Code. For the purposes of this Section 10, an Award will be
considered assumed if, following the Acquisition or Other Combination, the Award confers the right to purchase or receive, for each Share subject to the Award immediately prior to the Acquisition or Other Combination, the consideration (whether
stock, cash, or other securities or property) received in the Acquisition or Other Combination by holders of Shares for each Share held on the effective date of the transaction (and if holders were offered a choice of consideration, the type of
consideration chosen by the holders of a majority of the outstanding Shares); provided, however, that if such consideration received in the Acquisition or Other Combination is not solely common stock of the successor corporation or its Parent, the
Committee may, with the consent of the successor corporation, provide for the consideration to be received upon the exercise of an Option or Stock Appreciation Right or upon the payout of a Restricted Stock Unit, for each Share subject to such
Award, to be solely common stock of the successor corporation or its Parent equal in fair market value to the per share consideration received by holders of Common Stock in the Acquisition or Other Combination. 

(c) The substitution by the successor or acquiring entity in such Acquisition or Other Combination (or by any of its Parents, if any) of
equivalent awards with substantially the same terms for such outstanding Awards (except that the exercise price and the number and nature of shares issuable upon exercise of any such option or stock appreciation right, or any award that is subject
to Section 409A of the Code, will be adjusted appropriately pursuant to Section 424(a) of the Code). 
 (d) The full or partial
exercisability or vesting and accelerated expiration of outstanding Awards. 
 (e) The settlement of the full value of such outstanding
Award (whether or not then vested or exercisable) in cash, cash equivalents, or securities of the successor entity (or its Parent, if any) with a Fair Market Value equal to the required amount, followed by the cancellation of such Awards; provided
however, that such Award may be cancelled without consideration if such Award has no value, as determined by the Committee, in its discretion. Subject to Section 409A of the Code, such payment may be made in installments and may be deferred
until the date or dates when the Award would have become exercisable or vested. Such payment may be subject to vesting based on the Participant’s continued service, provided that without the Participant’s consent, the vesting schedule
shall 

 
not be less favorable to the Participant than the schedule under which the Award would have become vested or exercisable. For purposes of this Section 11.1(e), the Fair Market value of any
security shall be determined without regard to any vesting conditions that may apply to such security. 
 (f) The cancellation of
outstanding Awards in exchange for no consideration. 
 Immediately following an Acquisition or Other Combination, outstanding Awards shall
terminate and cease to be outstanding, except to the extent such Awards, have been continued, assumed or substituted, as described in Sections 11.1(a), (b) and/or (c). 

11.2 Assumption of Awards by the Company. The Company, from time to time, also may substitute or assume outstanding awards
granted by another entity, whether in connection with an acquisition of such other entity or otherwise, by either (a) granting an Award under this Plan in substitution of such other entity’s award or (b) assuming and/or converting
such award as if it had been granted under this Plan if the terms of such assumed award could be applied to an Award granted under this Plan. Such substitution or assumption will be permissible if the holder of the substituted or assumed award would
have been eligible to be granted an Award under this Plan if the other entity had applied the rules of this Plan to such grant. In the event the Company assumes an award granted by another entity, the terms and conditions of such award will remain
unchanged (except that the exercise price and the number and nature of shares issuable upon exercise of any such option or stock appreciation right, or any award that is subject to Section 409A of the Code, will be adjusted appropriately
pursuant to Section 424(a) of the Code). In the event the Company elects to grant a new Option or SAR rather than assuming an existing option or stock appreciation right, such new Option or SAR may be granted with a similarly adjusted Exercise
Price. 
 12. ADMINISTRATION. 

12.1 Committee Authority. This Plan will be administered by the Committee or the Board if no Committee is created by the Board.
Subject to the general purposes, terms and conditions of this Plan, and to the direction of the Board, the Committee will have full power to implement and carry out this Plan. Without limitation, the Committee will have the authority to: 

(a) construe and interpret this Plan, any Award Agreement and any other agreement or document executed pursuant to this Plan; 

(b) prescribe, amend, expand, modify and rescind or terminate rules and regulations relating to this Plan; 

(c) approve persons to receive Awards; 

(d) determine the form and terms of Awards; 

(e) determine the number of Shares or other consideration subject to Awards granted under this Plan; 

 (f) determine the Fair Market Value in good faith and interpret the applicable provisions of
this Plan and the definition of Fair Market Value in connection with circumstances that impact the Fair Market Value, if necessary; 
 (g)
determine whether Awards will be granted singly, in combination with, in tandem with, in replacement of, or as alternatives to, other Awards under this Plan or awards under any other incentive or compensation plan of the Company or any Parent or
Subsidiary of the Company; 
 (h) grant waivers of any conditions of this Plan or any Award; 

(i) determine the terms of vesting, exercisability and payment of Awards to be granted pursuant to this Plan; 

(j) correct any defect, supply any omission, or reconcile any inconsistency in this Plan, any Award, any Award Agreement, any Exercise
Agreement or any Restricted Stock Purchase Agreement; 
 (k) determine whether an Award has been earned; 

(l) extend the vesting period beyond a Participant’s Termination Date; 

(m) adopt rules and/or procedures (including the adoption of any subplan under this Plan) relating to the operation and administration of the
Plan to accommodate requirements of local law and procedures outside of the United States; 
 (n) delegate any of the foregoing to a
subcommittee consisting of one or more executive officers pursuant to a specific delegation as may otherwise be permitted by applicable law; and 

(o) make all other determinations necessary or advisable in connection with the administration of this Plan. 

12.2 Committee Composition and Discretion. The Board may delegate full administrative authority over the Plan and Awards to a
Committee consisting of at least one member of the Board (or such greater number as may then be required by applicable law). Unless in contravention of any express terms of this Plan or Award, any determination made by the Committee with respect to
any Award will be made in its sole discretion either (a) at the time of grant of the Award, or (b) subject to Section 4.9 hereof, at any later time. Any such determination will be final and binding on the Company and on all persons
having an interest in any Award under this Plan. To the extent permitted by applicable law, the Committee may delegate to one or more officers of the Company the authority to grant an Award under this Plan, provided that each such
officer is a member of the Board. 
 12.3 Nonexclusivity of the Plan. Neither the adoption of this Plan by the Board, the
submission of this Plan to the stockholders of the Company for approval, nor any provision of this Plan will be construed as creating any limitations on the power of the Board to 

 
adopt such additional compensation arrangements as it may deem desirable, including, without limitation, the granting of stock options and other equity awards otherwise than under this Plan, and
such arrangements may be either generally applicable or applicable only in specific cases. 
 12.4 Governing Law. This Plan
and all agreements hereunder shall be governed by and construed in accordance with the laws of the State of California, without giving effect to that body of laws pertaining to conflict of laws. 

13. EFFECTIVENESS, AMENDMENT AND TERMINATION OF THE PLAN. 

13.1 Adoption and Stockholder Approval. This Plan will become effective on the date that it is adopted by the Board (the
“Effective Date”). This Plan will be approved by the stockholders of the Company (excluding Shares issued pursuant to this Plan), consistent with applicable laws, within twelve (12) months before or after the Effective
Date. Upon the Effective Date, the Board may grant Awards pursuant to this Plan; provided, however, that: (a) no Option or SAR may be exercised prior to initial stockholder approval of this Plan; (b) no Option
or SAR granted pursuant to an increase in the number of Shares approved by the Board shall be exercised prior to the time such increase has been approved by the stockholders of the Company; (c) in the event that initial stockholder approval is
not obtained within the time period provided herein, all Awards for which only the exemption from California’s securities qualification requirements provided by Section 25102(o) can apply shall be canceled, any Shares issued pursuant to
any such Award shall be canceled and any purchase of such Shares issued hereunder shall be rescinded; and (d) Awards (to which only the exemption from California’s securities qualification requirements provided by Section 25102(o) can
apply) granted pursuant to an increase in the number of Shares approved by the Board which increase is not approved by stockholders within the time then required under Section 25102(o) shall be canceled, any Shares issued pursuant to any such
Awards shall be canceled, and any purchase of Shares subject to any such Award shall be rescinded. 
 13.2 Term of Plan.
Unless earlier terminated as provided herein, this Plan will automatically terminate ten (10) years after the later of (i) the Effective Date, or (ii) the most recent increase in the number of Shares reserved under Section 2 that
was approved by stockholders. 
 13.3 Amendment or Termination of Plan. Subject to Section 4.9 hereof, the Board may at
any time (a) terminate or amend this Plan in any respect, including without limitation amendment of any form of Award Agreement or instrument to be executed pursuant to this Plan and (b) terminate any and all outstanding Options or SARs
upon a dissolution or liquidation of the Company, followed by the payment of creditors and the distribution of any remaining funds to the Company’s stockholders; provided, however, that the Board will not, without
the approval of the stockholders of the Company, amend this Plan in any manner that requires such stockholder approval pursuant to Section 25102(o) or pursuant to the Code or the regulations promulgated under the Code as such provisions apply
to ISO plans. The termination of the Plan, or any amendment thereof, shall not affect any Share previously issued or any Award previously granted under the Plan. 

 14. DEFINITIONS. For all purposes of this Plan, the following terms will have the
following meanings. 
 “Acquisition,” for purposes of Section 11, means: 

(a) any consolidation or merger in which the Company is a constituent entity or is a party in which the voting stock and other voting
securities of the Company that are outstanding immediately prior to the consummation of such consolidation or merger represent, or are converted into, securities of the surviving entity of such consolidation or merger (or of any Parent of such
surviving entity) that, immediately after the consummation of such consolidation or merger, together possess less than fifty percent (50%) of the total voting power of all voting securities of such surviving entity (or of any of its Parents, if
any) that are outstanding immediately after the consummation of such consolidation or merger; 
 (b) a sale or other transfer by the
holders thereof of outstanding voting stock and/or other voting securities of the Company possessing more than fifty percent (50%) of the total voting power of all outstanding voting securities of the Company, whether in one transaction or in a
series of related transactions, pursuant to an agreement or agreements to which the Company is a party and that has been approved by the Board, and pursuant to which such outstanding voting securities are sold or transferred to a single person or
entity, to one or more persons or entities who are Affiliates of each other, or to one or more persons or entities acting in concert; or 

(c) the sale, lease, transfer or other disposition, in a single transaction or series of related transactions, by the Company and/or any
Subsidiary or Subsidiaries of the Company, of all or substantially all the assets of the Company and its Subsidiaries taken as a whole, (or, if substantially all of the assets of the Company and its Subsidiaries taken as a whole are held by one or
more Subsidiaries, the sale or disposition (whether by consolidation, merger, conversion or otherwise) of such Subsidiaries of the Company), except where such sale, lease, transfer or other disposition is made to the Company or one or more wholly
owned Subsidiaries of the Company (an “Acquisition by Sale of Assets”). 
 “Affiliate”of a
specified person means a person that directly, or indirectly through one or more intermediaries, controls or is controlled by, or is under common control with, the person specified (where, for purposes of this definition, the term
“control” (including the terms controlling, controlled by and under common control with) means the possession, direct or indirect, of the power to direct or cause the direction
of the management and policies of a person, whether through the ownership of voting securities, by contract, or otherwise. 

“Award” means any award pursuant to the terms and conditions of this Plan, including any Option, Restricted Stock
Unit, Stock Appreciation Right or Restricted Stock Award. 
 “Award Agreement” means, with respect to each Award,
the signed written agreement between the Company and the Participant setting forth the terms and conditions of the Award as approved by the Committee. 

“Board” means the Board of Directors of the Company. 

 “Cause” means Termination because of (a) Participant’s
unauthorized misuse of the Company or a Parent or Subsidiary of the Company’s trade secrets or proprietary information, (b) Participant’s conviction of or plea of nolo contendere to a felony or a crime involving moral turpitude,
(c) Participant’s committing an act of fraud against the Company or a Parent or Subsidiary of the Company or (d) Participant’s gross negligence or willful misconduct in the performance of his or her duties that has had or will
have a material adverse effect on the Company or Parent or Subsidiary of the Company’ reputation or business. 

“Code” means the Internal Revenue Code of 1986, as amended. 

“Committee” means the committee created and appointed by the Board to administer this Plan, or if no committee is
created and appointed, the Board. 
 “Company” means Audentes Therapeutics, Inc., or any successor corporation. 

“Disability” means a disability, whether temporary or permanent, partial or total, as determined by the Committee.

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

“Exercise Price” means the price per Share at which a holder of an Option may purchase Shares issuable upon exercise
of the Option. 
 “Fair Market Value” means, as of any date, the value of a share of the Company’s Common Stock
determined as follows: 
 (a) if such Common Stock is then publicly traded on a national securities exchange, its closing price on the date
of determination on the principal national securities exchange on which the Common Stock is listed or admitted to trading as reported in The Wall Street Journal; 

(b) if such Common Stock is publicly traded but is not listed or admitted to trading on a national securities exchange, the average of the
closing bid and asked prices on the date of determination as reported by The Wall Street Journal (or, if not so reported, as otherwise reported by any newspaper or other source as the Committee may determine); or 

(c) if none of the foregoing is applicable to the valuation in question, by the Committee in good faith. 

“Option” means an award of an option to purchase Shares pursuant to Section 4 of this Plan. 

“Other Combination” for purposes of Section 11 means any (a) consolidation or merger in which the Company is
a constituent entity and is not the surviving entity of such consolidation or merger or (b) any conversion of the Company into another form of entity; provided that such consolidation, merger or conversion does not constitute an
Acquisition. 

 “Parent” of a specified entity means, any entity that, either directly or
indirectly, owns or controls such specified entity, where for this purpose, “control” means the ownership of stock, securities or other interests that possess at least a majority of the voting power of such specified entity
(including indirect ownership or control of such stock, securities or other interests). 
 “Participant” means a
person who receives an Award under this Plan. 
 “Plan” means this 2012 Equity Incentive Plan, as amended from time
to time. 
 “Purchase Price” means the price at which a Participant may purchase Restricted Stock pursuant to this
Plan. 
 “Restricted Stock” means Shares purchased pursuant to a Restricted Stock Award under this Plan. 

“Restricted Stock Award” means an award of Shares pursuant to Section 5 hereof. 

“Restricted Stock Unit” or “RSU” means an award made pursuant to Section 6 hereof. 

“Rule 701” means Rule 701 et seq. promulgated by the Commission under the Securities Act. 

“SEC” means the Securities and Exchange Commission. 

“Section 25102(o)” means Section 25102(o) of the California Corporations Code. 

“Securities Act” means the Securities Act of 1933, as amended. 

“Shares” means shares of the Company’s Common Stock reserved for issuance under this Plan, as adjusted pursuant
to Sections 2.2 and 11 hereof, and any successor security. 
 “Stock Appreciation Right” or
“SAR” means an award granted pursuant to Section 7 hereof. 
 “Subsidiary” means any
entity (other than the Company) in an unbroken chain of entities beginning with the Company if each of the entities other than the last entity in the unbroken chain owns stock or other equity securities representing fifty percent (50%) or more
of the total combined voting power of all classes of stock or other equity securities in one of the other entities in such chain. 

“Termination” or “Terminated” means, for purposes of this Plan with respect to a Participant,
that the Participant has for any reason ceased to provide services as an employee, officer, director or consultant to the Company or a Parent or Subsidiary of the Company. A Participant will not be deemed to have ceased to provide services in the
case of sick leave, 

 
military leave, or any other leave of absence approved by the Committee; provided that such leave is for a period of not more than ninety (90) days (a) unless
reinstatement (or, in the case of an employee with an ISO, reemployment) upon the expiration of such leave is guaranteed by contract or statute, or (b) unless provided otherwise pursuant to formal policy adopted from time to time by the
Company’s Board and issued and promulgated in writing. In the case of any Participant on sick leave, military leave or an approved leave of absence, the Committee may make such provisions respecting suspension of vesting of the Award while on
leave from the Company or a Parent or Subsidiary of the Company as it may deem appropriate, except that in no event may an Option be exercised after the expiration of the term set forth in the Stock Option Agreement. The Committee will have sole
discretion to determine whether a Participant has ceased to provide services and the effective date on which the Participant ceased to provide services (the “Termination Date”). 

“Unvested Shares” means “Unvested Shares” as defined in the Award Agreement for an Award. 

“Vested Shares” means “Vested Shares” as defined in the Award Agreement. 

* * * * * * * * * * * 

 NOTICE OF STOCK OPTION GRANT 

AUDENTES THERAPEUTICS, INC. 

2012 EQUITY INCENTIVE PLAN 

The Optionee named below (“Optionee”) has been granted an option (this “Option”) to purchase shares of Common
Stock, $0.00001 par value per share (the “Common Stock”), of Audentes Therapeutics, Inc., a Delaware corporation (the “Company”), pursuant to the Company’s 2012 Equity Incentive Plan, as amended
from time to time (the “Plan”) on the terms, and subject to the conditions, described below and in the Stock Option Agreement attached hereto as Exhibit A, including its annexes (the “Stock
Option Agreement”). 
  

					
	Optionee:	 	  
	  	
			
	Maximum Number of Shares Subject to this Option (the “Shares”):	 	  
	  	
		
	Exercise Price Per Share:	 	 $         per share

			
	Date of Grant:	 	  
	  	
			
	Vesting Start Date:	 	  
	  	
		
	Exercise Schedule:	 	 This Option will become exercisable during its term with respect to portions of the Shares in accordance with the Vesting
Schedule set forth below.

		
	Expiration Date:	 	The date ten (10) years after the Date of Grant set forth above, subject to earlier expiration in the event of Termination as provided in Section 3 of the Stock Option Agreement.
		
	 Tax Status of Option:
 (Check
Only One Box):
	 	  ̈ Incentive Stock Option (To the fullest extent permitted by the Code)

 ̈ Nonqualified Stock Option.

(If neither box is checked, this Option is a Nonqualified Stock Option).

 Vesting Schedule: For so long as Optionee continuously provides services to the Company (or any Subsidiary or Parent of
the Company) as an employee, officer, director, contractor or consultant, this Option will vest (that is, become exercisable) with respect to the Shares as follows: (a) prior to the first one (1) year anniversary of the Vesting Start Date
this Option will not be vested or exercisable as to any of the Shares; (b) this Option will become vested and exercisable with respect to 1/4th of the Shares on the one (1) year
anniversary of the Vesting Start Date; and (c) thereafter, this Option will become vested and exercisable with respect to an additional 1/16th of the Shares at the end of each quarter after
the one (1) year anniversary of the Vesting Start Date. 
 General; Agreement: By their signatures below, Optionee and the Company agree that
this Option is granted under and governed by this Notice of Stock Option Grant (this “Grant Notice”) and by the provisions of the Plan and the Stock Option Agreement. The Plan and the Stock Option Agreement are incorporated
herein by reference. Capitalized terms used but not defined herein shall have the meanings given to them in the Plan or in the Stock Option Agreement, as applicable. By signing below, Optionee acknowledges receipt of a copy of this Grant Notice, the
Plan and the Stock Option Agreement, represents that Optionee has carefully read and is familiar with their provisions, and hereby accepts the Option subject to all of their respective terms and conditions. Optionee acknowledges that there may be
adverse tax consequences upon exercise of the Option or disposition of the Shares and that Optionee should consult a tax adviser prior to such exercise or disposition. 

 Execution and Delivery: This Grant Notice may be executed and delivered electronically whether via the
Company’s intranet or the Internet site of a third party or via email or any other means of electronic delivery specified by the Company. By Optionee’s acceptance hereof (whether written, electronic or otherwise), Optionee agrees, to the
fullest extent permitted by law, that in lieu of receiving documents in paper format, Optionee accepts the electronic delivery of any documents that the Company (or any third party the Company may designate), may deliver in connection with this
grant (including the Plan, this Grant Notice, the Stock Option Agreement, the information described in Rules 701(e)(2), (3), (4) and (5) under the Securities Act (the “701 Disclosures”), account statements, or other
communications or information) whether via the Company’s intranet or the Internet site of such third party or via email or such other means of electronic delivery specified by the Company. 

Audentes Therapeutics, Inc. 
  

									
	By /Signature:	 	  
	 		 	Optionee Signature:	 	  

					
	Typed Name:	 	 Matthew Patterson
	 		 	Optionee’s Name:	 	  

					
	Title:	 	 Chief Executive Officer
	 		 		 	

 ATTACHMENT: Exhibit A – Stock Option Agreement 

 Exhibit A 

Stock Option Agreement 

 EXHIBIT A 

STOCK OPTION AGREEMENT 

AUDENTES THERAPEUTICS, INC. 

2012 EQUITY INCENTIVE PLAN 

This Stock Option Agreement (this “Agreement”) is made and entered into as of the date of grant (the “Date
of Grant”) set forth on the Notice of Stock Option Grant attached as the facing page to this Agreement (the “Grant Notice”) by and between Audentes Therapeutics, Inc., a Delaware corporation (the
“Company”), and the optionee named on the Grant Notice (the “Optionee”). Capitalized terms not defined in this Agreement shall have the meaning ascribed to them in the Company’s 2012 Equity
Incentive Plan, as amended from time to time (the “Plan”), or in the Grant Notice, as applicable. 
 1. GRANT OF
OPTION. The Company hereby grants to Optionee an option (this “Option”) to purchase up to the total number of shares of Common Stock of the Company, $0.00001 par value per share (the “Common
Stock”), set forth in the Grant Notice as the Shares (the “Shares”) at the Exercise Price Per Share set forth in the Grant Notice (the “Exercise Price”), subject to all of the terms and
conditions of the Grant Notice, this Agreement and the Plan. If designated as an Incentive Stock Option in the Grant Notice, this Option is intended to qualify as an incentive stock option (the “ISO”) within the meaning of
Section 422 of the Internal Revenue Code of 1986, as amended (the “Code”), except that if on the Date of Grant Optionee is not subject to U.S. income tax, then this Option shall be a NQSO. 

2. EXERCISE PERIOD. 

2.1 Exercise Period of Option. This Option is considered to be “vested” with respect to any particular Shares when
this Option is exercisable with respect to such Shares. This Option will become vested during its term as to portions of the Shares in accordance with the Vesting Schedule set forth in the Grant Notice. Notwithstanding any provision in the Plan or
this Agreement to the contrary, on or after Optionee’s Termination Date, this Option may not be exercised with respect to any Shares that are Unvested Shares on Optionee’s Termination Date. 

2.2 Vesting of Option Shares. Shares with respect to which this Option is vested and exercisable at a given time pursuant to the
Vesting Schedule set forth in the Grant Notice are “Vested Shares.” Shares with respect to which this Option is not vested and exercisable at a given time pursuant to the Vesting Schedule set forth in the Grant Notice
are “Unvested Shares.” 
 2.3 Expiration. The Option shall expire on the Expiration Date
set forth in the Grant Notice or earlier as provided in Section 3 below. 
 3. TERMINATION. 

3.1 Termination for Any Reason Except Death, Disability or Cause. Except as provided in subsection 3.2 in a case in which
Optionee dies within three (3) months after Optionee is Terminated other than for Cause, if Optionee is Terminated for any reason (other than Optionee’s death or Disability or for Cause), then (a) on and after Optionee’s
Termination Date, this Option shall expire immediately with respect to any Shares that are Unvested Shares and may not be exercised with respect to any Shares that are Unvested Shares on Optionee’s Termination Date and (b) this Option to
the extent (and only to the extent) that it is exercisable with respect to Vested Shares on Optionee’s Termination Date, may be exercised by Optionee no later than three (3) months after Optionee’s Termination Date (but in no event
may this Option be exercised after the Expiration Date). 
 3.2 Termination Because of Death or Disability. If Optionee is
Terminated because of Optionee’s death or Disability (or if Optionee dies within three (3) months of the date of 

 
Optionee’s Termination for any reason other than for Cause), then (a) on and after Optionee’s Termination Date, this Option shall expire immediately with respect to any Shares that
are Unvested Shares and may not be exercised with respect to any Shares that are Unvested Shares on Optionee’s Termination Date and (b) this Option, to the extent (and only to the extent) that it is exercisable with respect to Vested
Shares on Optionee’s Termination Date, may be exercised by Optionee (or Optionee’s legal representative) no later than twelve (12) months after Optionee’s Termination Date, but in no event later than the Expiration Date. Any
exercise of this Option beyond (i) three (3) months after Optionee’s Termination Date when Optionee’s Termination is for any reason other than Optionee’s death or disability, within the meaning of Section 22(e)(3) of
the Code; or (ii) twelve (12) months after Optionee’s Termination Date when the termination is for Optionee’s disability, within the meaning of Section 22(e)(3) of the Code, is deemed to be an NQSO. 

3.3 Termination for Cause. If Optionee is Terminated for Cause, then Optionee may exercise this Option, but only with respect to
any Shares that are Vested Shares on Optionee’s Termination Date, and this Option shall expire on Optionee’s Termination Date, or at such later time and on such conditions as may be affirmatively determined by the Committee. On and after
Optionee’s Termination Date, this Option shall expire immediately with respect to any Shares that are Unvested Shares and may not be exercised with respect to any Shares that are Unvested Shares on Optionee’s Termination Date. 

3.4 No Obligation to Employ. Nothing in the Plan or this Agreement shall confer on Optionee any right to continue in the employ
of, or other relationship with, the Company or any Parent or Subsidiary of the Company, or limit in any way the right of the Company or any Parent or Subsidiary of the Company to terminate Optionee’s employment or other relationship at any
time, with or without Cause. 
 4. MANNER OF EXERCISE. 

4.1 Stock Option Exercise Notice and Agreement. To exercise this Option, Optionee (or in the case of exercise after
Optionee’s death or incapacity, Optionee’s executor, administrator, heir or legatee, as the case may be) must deliver to the Company an executed Stock Option Exercise Notice and Agreement in the form attached hereto as
Annex A, or in such other form as may be approved by the Committee from time to time (the “Exercise Agreement”) and payment for the shares being purchased in accordance with this Agreement. The Exercise
Agreement shall set forth, among other things, (i) Optionee’s election to exercise this Option, (ii) the number of Shares being purchased, (iii) any representations, warranties and agreements regarding Optionee’s investment
intent and access to information as may be required by the Company to comply with applicable securities laws in connection with any exercise of this Option, (iv) any other agreements required by the Company and (v) Optionee’s
obligation to execute and deliver certain Stock Powers and Assignments Separate from Stock Certificate to the Company. If someone other than Optionee exercises this Option, then such person must submit documentation reasonably acceptable to the
Company verifying that such person has the legal right to exercise this Option and such person shall be subject to all of the restrictions contained herein as if such person were Optionee. 

4.2 Limitations on Exercise. This Option may not be exercised unless such exercise is in compliance with all applicable federal
and state securities laws, as they are in effect on the date of exercise. 
 4.3 Payment. The Exercise Agreement shall be
accompanied by full payment of the Exercise Price for the shares being purchased in cash (by check or wire transfer), or where permitted by law: 

(a) by cancellation of indebtedness of the Company owed to Optionee; 

  
 3 

 (b) by surrender of shares of the Company that are free and clear of all security interests,
pledges, liens, claims or encumbrances and: (i) for which the Company has received “full payment of the purchase price” within the meaning of SEC Rule 144 (and, if such shares were purchased from the Company by use of a
promissory note, such note has been fully paid with respect to such shares) or (ii) that were obtained by Optionee in the public market; 

(c) by participating in a formal cashless exercise program implemented by the Committee in connection with the Plan; 

(d) provided that a public market for the Common Stock exists and subject to compliance with applicable law, by exercising as set forth
below, through a “same day sale” commitment from Optionee and a broker-dealer whereby Optionee irrevocably elects to exercise this Option and to sell a portion of the Shares so purchased sufficient to pay the total Exercise Price, and
whereby the broker-dealer irrevocably commits upon receipt of such Shares to forward the total Exercise Price directly to the Company; or 

(e) by any combination of the foregoing or any other method of payment approved by the Committee that constitutes legal consideration for the
issuance of Shares. 
 4.4 Tax Withholding. Prior to the issuance of the Shares upon exercise of the Option, Optionee must pay
or provide for any applicable federal, state and local withholding obligations of the Company. If the Committee permits, Optionee may provide for payment of withholding taxes upon exercise of the Option by requesting that the Company retain the
minimum number of Shares with a Fair Market Value equal to the minimum amount of taxes required to be withheld; or to arrange a mandatory “sell to cover” on Participant’s behalf (without further authorization); but in no event will
the Company withhold Shares or “sell to cover” if such withholding would result in adverse accounting consequences to the Company. In case of stock withholding or a sell to cover, the Company shall issue the net number of Shares to the
Optionee by deducting the Shares retained from the Shares issuable upon exercise. 
 4.5 Issuance of Shares.
Provided that the Exercise Agreement and payment are in form and substance satisfactory to counsel for the Company, the Company shall issue the Shares issuable upon a valid exercise of this Option registered in the name of Optionee, Optionee’s
authorized assignee, or Optionee’s legal representative, and shall deliver certificates representing the Shares with the appropriate legends affixed thereto. 

5. COMPLIANCE WITH LAWS AND REGULATIONS. The Plan and this Agreement are intended to comply with
Section 25102(o) and Rule 701. Any provision of this Agreement that is inconsistent with Section 25102(o) or Rule 701 shall, without further act or amendment by the Company or the Committee, be reformed to comply with the requirements of
Section 25102(o) and/or Rule 701. The exercise of this Option and the issuance and transfer of Shares shall be subject to compliance by the Company and Optionee with all applicable requirements of federal and state securities laws and with all
applicable requirements of any stock exchange on which the Common Stock may be listed at the time of such issuance or transfer. Optionee understands that the Company is under no obligation to register or qualify the Shares with the SEC, any state
securities commission or any stock exchange to effect such compliance. 
 6. NONTRANSFERABILITY OF OPTION. This Option may
not be transferred in any manner other than by will or by the laws of descent and distribution, and, with respect to NQSOs, by instrument to a testamentary trust in which the options are to be passed to beneficiaries upon the death of the trustor
(settlor) or a revocable trust, or by gift to “immediate family” as that term is defined in 17 C.F.R. 240.16a-1(e), and may be exercised during the lifetime of Optionee only by Optionee or in the event of Optionee’s incapacity, by
Optionee’s legal representative. The terms of this Option shall be binding upon the executors, administrators, successors and assigns of Optionee. 

  
 4 

 7. RESTRICTIONS ON TRANSFER. 

7.1 Disposition of Shares. Optionee hereby agrees that Optionee shall make no disposition of any of the Shares (other than as
permitted by this Agreement) unless and until: 
 (a) Optionee shall have notified the Company of the proposed disposition and
provided a written summary of the terms and conditions of the proposed disposition; 
 (b) Optionee shall have complied with all
requirements of this Agreement applicable to the disposition of the Shares; 
 (c) Optionee shall have provided the Company with written
assurances, in form and substance satisfactory to counsel for the Company, that (i) the proposed disposition does not require registration of the Shares under the Securities Act or under any applicable state securities laws or (ii) all
appropriate actions necessary for compliance with the registration requirements of the Securities Act or of any exemption from registration available under the Securities Act (including Rule 144) or applicable state securities laws have been taken;
and 
 (d) Optionee shall have provided the Company with written assurances, in form and substance satisfactory to the Company, that the
proposed disposition will not result in the contravention of any transfer restrictions applicable to the Shares pursuant to the provisions of the regulations promulgated under Section 25102(o), Rule 701 or under any other applicable securities
laws or adversely affect the Company’s ability to rely on the exemption(s) from registration under the Securities Act or under any other applicable securities laws for the grant of the Option, the issuance of Shares thereunder or any other
issuance of securities under the Plan. 
 7.2 Restriction on Transfer. Optionee shall not transfer, assign, grant a lien or
security interest in, pledge, hypothecate, encumber or otherwise dispose of any of the Shares which are subject to the Company’s Right of First Refusal described below, except as permitted by this Agreement. 

7.3 Transferee Obligations. Each person (other than the Company) to whom the Shares are transferred by means of one of the
permitted transfers specified in this Agreement must, as a condition precedent to the validity of such transfer, acknowledge in writing to the Company that such person is bound by the provisions of this Agreement and that the transferred Shares are
subject to (i) the Company’s Right of First Refusal granted hereunder and (ii) the market stand-off provisions of Section 8 below, to the same extent such Shares would be so subject if retained by Optionee. 

8. MARKET STANDOFF AGREEMENT. Optionee agrees that, subject to any early release provisions that apply pro rata to stockholders of the
Company according to their holdings of Common Stock (determined on an as-converted into Common Stock basis), Optionee will not, if requested by the managing underwriter(s) in the initial underwritten sale of Common Stock of the Company to the public
pursuant to a registration statement filed with, and declared effective by, the SEC under the Securities Act (the “IPO”), for a period of up to one hundred eighty (180) days (plus up to an additional thirty five
(35) days to the extent reasonably requested by the Company or such underwriter(s) to accommodate regulatory restrictions on the publication or other distribution of research reports or earnings releases by the Company, including NASD and NYSE
rules) following the effective date of the registration statement relating to such IPO, directly or indirectly sell, offer to sell, grant any option for the sale of, or otherwise dispose of any Common Stock or securities convertible into Common
Stock, except for: (i) transfers of Shares permitted under Section 9.6 hereof so long as such transferee furnishes to the Company and the managing underwriter their written consent to be bound by this Section 8 as a
condition precedent to such transfer; and (ii) sales of any securities to be included in the registration statement for the IPO. For the avoidance of doubt, the provisions of this Section shall only apply to the IPO. The restricted period shall
in any event terminate two (2) years after the closing date of the IPO. In order to enforce the foregoing covenant, the Company shall have the right to place restrictive legends on the 

  
 5 

 
certificates representing the Shares subject to this Section and to impose stop transfer instructions with respect to the Shares until the end of such period. Optionee further agrees to enter
into any agreement reasonably required by the underwriters to implement the foregoing restrictions on transfer. For the avoidance of doubt, the foregoing provisions of this Section shall not apply to any registration of securities of the Company
(a) under an employee benefit plan or (b) in a merger, consolidation, business combination or similar transaction. 
 9.
COMPANY’S RIGHT OF FIRST REFUSAL. Before any Shares held by Optionee or any transferee of such Shares (either sometimes referred to herein as the “Holder”) may be sold or otherwise transferred
(including, without limitation, a transfer by gift or operation of law), the Company and/or its assignee(s) will have a right of first refusal to purchase the Shares to be sold or transferred (the “Offered
Shares”) on the terms and conditions set forth in this Section (the “Right of First Refusal”). 

9.1 Notice of Proposed Transfer. The Holder of the Offered Shares will deliver to the Company a written notice (the
“Notice”) stating: (i) the Holder’s bona fide intention to sell or otherwise transfer the Offered Shares; (ii) the name and address of each proposed purchaser or other transferee (the
“Proposed Transferee”); (iii) the number of Offered Shares to be transferred to each Proposed Transferee; (iv) the bona fide cash price or other consideration for which the Holder proposes to transfer
the Offered Shares (the “Offered Price”); and (v) that the Holder acknowledges this Notice is an offer to sell the Offered Shares to the Company and/or its assignee(s) pursuant to the Company’s Right
of First Refusal at the Offered Price as provided for in this Agreement. 
 9.2 Exercise of Right of First Refusal. At
any time within thirty (30) days after the date of the Notice, the Company and/or its assignee(s) may, by giving written notice to the Holder, elect to purchase all (or, with the consent of the Holder, less than all) the Offered Shares proposed
to be transferred to any one or more of the Proposed Transferees named in the Notice, at the purchase price, determined as specified below.  

9.3 Purchase Price. The purchase price for the Offered Shares purchased under this Section will be the Offered Price,
 provided that if the Offered Price consists of no legal consideration (as, for example, in the case of a transfer by gift) then the purchase price will be the fair market value of the Offered Shares as determined in
good faith by the Committee. If the Offered Price includes consideration other than cash, then the value of the non-cash consideration, as determined in good faith by the Committee, will conclusively be deemed to be the cash equivalent value of such
non-cash consideration. 
 9.4 Payment. Payment of the purchase price for the Offered Shares will be payable, at the
option of the Company and/or its assignee(s) (as applicable), by check or by cancellation of all or a portion of any outstanding purchase money indebtedness owed by the Holder to the Company (or to such assignee, in the case of a purchase of Offered
Shares by such assignee) or by any combination thereof. The purchase price will be paid without interest within sixty (60) days after the Company’s receipt of the Notice, or, at the option of the Company and/or its assignee(s), in the
manner and at the time(s) set forth in the Notice. 
 9.5 Holder’s Right to Transfer. If all of the Offered Shares
proposed in the Notice to be transferred to a given Proposed Transferee are not purchased by the Company and/or its assignee(s) as provided in this Section, then the Holder may sell or otherwise transfer such Offered Shares to each Proposed
Transferee at the Offered Price or at a higher price, provided that (i) such sale or other transfer is consummated within ninety (90) days after the date of the Notice, (ii) any such sale or other transfer
is effected in compliance with all applicable securities laws, and (iii) each Proposed Transferee agrees in writing that the provisions of this Section will continue to apply to the Offered Shares in the hands of such Proposed Transferee. If
the Offered Shares described in the Notice are not transferred to each Proposed Transferee within such ninety (90) day period, then a new Notice must be given to the Company pursuant to which the Company will again be offered the Right of First
Refusal before any Shares held by the Holder may be sold or otherwise transferred. 

  
 6 

 9.6 Exempt Transfers. Notwithstanding anything to the contrary in this Section, the
following transfers of Shares will be exempt from the Right of First Refusal: (i) the transfer of any or all of the Shares during Optionee’s lifetime by gift or on Optionee’s death by will or intestacy to any member(s) of
Optionee’s “Immediate Family” (as defined below) or to a trust for the benefit of Optionee and/or member(s) of Optionee’s Immediate Family, provided that each transferee or other recipient agrees in a writing
satisfactory to the Company that the provisions of this Section will continue to apply to the transferred Shares in the hands of such transferee or other recipient; (ii) any transfer of Shares made pursuant to a statutory merger, statutory
consolidation of the Company with or into another corporation or corporations or a conversion of the Company into another form of legal entity (except that the Right of First Refusal will continue to apply thereafter to such Shares, in which case
the surviving corporation of such merger or consolidation or the resulting entity of such conversion shall succeed to the rights of the Company under this Section unless the agreement of merger or consolidation or conversion expressly otherwise
provides); or (iii) any transfer of Shares pursuant to the winding up and dissolution of the Company. As used herein, the term “Immediate Family” will mean Optionee’s spouse, the lineal descendant or antecedent,
father, mother, brother or sister, child, adopted child, grandchild or adopted grandchild of Optionee or Optionee’s spouse, or the spouse of any of the above or Spousal Equivalent, as defined herein. As used herein, a person is deemed to be a
“Spousal Equivalent” provided the following circumstances are true: (i) irrespective of whether or not the Optionee and the Spousal Equivalent are the same sex, they are the sole spousal equivalent of the other for the
last twelve (12) months, (ii) they intend to remain so indefinitely, (iii) neither are married to anyone else, (iv) both are at least 18 years of age and mentally competent to consent to contract, (v) they are not related by
blood to a degree of closeness that which would prohibit legal marriage in the state in which they legally reside, (vi) they are jointly responsible for each other’s common welfare and financial obligations, and (vii) they reside
together in the same residence for the last twelve (12) months and intend to do so indefinitely. 
 9.7 Termination of Right of
First Refusal. The Right of First Refusal will terminate as to all Shares: (i) on the effective date of the first sale of Common Stock of the Company to the general public pursuant to a registration statement filed with and declared
effective by the SEC under the Securities Act (other than a registration statement relating solely to the issuance of Common Stock pursuant to a business combination or an employee incentive or benefit plan); (ii) on any transfer or conversion
of Shares made pursuant to a statutory merger or statutory consolidation of the Company with or into another corporation or corporations if the common stock of the surviving corporation or any direct or indirect parent corporation thereof is
registered under the Exchange Act; or (iii) on any transfer or conversion of Shares made pursuant to a statutory conversion of the Company into another form of legal entity if the common equity (or comparable equity security) of entity
resulting from such conversion is registered under the Exchange Act. 
 9.8 Encumbrances on Shares. Optionee may grant a lien
or security interest in, or pledge, hypothecate or encumber Shares only if each party to whom such lien or security interest is granted, or to whom such pledge, hypothecation or other encumbrance is made, agrees in a writing satisfactory to the
Company that: (i) such lien, security interest, pledge, hypothecation or encumbrance will not adversely affect or impair the Right of First Refusal or the rights of the Company and/or its assignee(s) with respect thereto and will not apply to
such Shares after they are acquired by the Company and/or its assignees under this Section; and (ii) the provisions of this Agreement will continue to apply to such Shares in the hands of such party and any transferee of such party. 

10. RIGHTS AS A STOCKHOLDER. Subject to the terms and conditions of this Agreement, Optionee will have all of the rights of a
stockholder of the Company with respect to the Shares from and after the date that Shares are issued to Optionee pursuant to, and in accordance with, the terms of the Exercise Agreement until such time as Optionee disposes of the Shares or the
Company and/or its assignee(s) exercise(s) the Right of First Refusal. Upon an exercise of the Right of First Refusal, Optionee will have no further rights as a holder of the Shares so purchased upon such exercise, other than the right to receive
payment for the Shares so purchased in accordance with the provisions of this Agreement, and Optionee will promptly surrender the stock certificate(s) evidencing the Shares so purchased to the Company for transfer or cancellation. 

  
 7 

 11. ESCROW. As security for Optionee’s faithful performance of this Agreement,
Optionee agrees, immediately upon receipt of the stock certificate(s) evidencing the Shares, to deliver such certificate(s), together with two (2) copies of a blank Stock Power and Assignment Separate from Stock Certificate in the form attached
to the Exercise Agreement (the “Stock Powers”), both executed by Purchaser (and Purchaser’s spouse, if any) (with the transferee, certificate number, date and number of Shares left blank), to the Secretary of the Company
or other designee of the Company (the “Escrow Holder”), who is hereby appointed to hold such certificate(s) and Stock Powers in escrow and to take all such actions and to effectuate all such transfers and/or releases of such
Shares as are in accordance with the terms of this Agreement. Optionee and the Company agree that Escrow Holder will not be liable to any party to this Agreement (or to any other party) for any actions or omissions unless Escrow Holder is grossly
negligent or intentionally fraudulent in carrying out the duties of Escrow Holder under this Agreement. Escrow Holder may rely upon any letter, notice or other document executed with any signature purported to be genuine and may rely on the advice
of counsel and obey any order of any court with respect to the transactions contemplated by this Agreement and will not be liable for any act or omission taken by Escrow Holder in good faith reliance on such documents, the advice of counsel or a
court order. The Shares will be released from escrow upon termination of the Right of First Refusal. 
 12. RESTRICTIVE LEGENDS AND
STOP-TRANSFER ORDERS. 
 12.1 Legends. Optionee understands and agrees that the Company will place the legends set forth
below or similar legends on any stock certificate(s) evidencing the Shares, together with any other legends that may be required by state or U.S. Federal securities laws, the Company’s Certificate of Incorporation or Bylaws, any other agreement
between Optionee and the Company, or any agreement between Optionee and any third party (and any other legend(s) that the Company may become obligated to place on the stock certificate(s) evidencing the Shares under the terms of any agreement to
which the Company is or may become bound or obligated): 
 (a) THE SECURITIES REPRESENTED HEREBY HAVE NOT BEEN REGISTERED UNDER THE
SECURITIES ACT OF 1933, AS AMENDED (THE “SECURITIES ACT”), OR UNDER THE SECURITIES LAWS OF CERTAIN STATES. THESE SECURITIES ARE SUBJECT TO RESTRICTIONS ON TRANSFERABILITY AND RESALE AND MAY NOT BE TRANSFERRED OR RESOLD EXCEPT AS PERMITTED
UNDER THE SECURITIES ACT AND APPLICABLE STATE SECURITIES LAWS, PURSUANT TO REGISTRATION OR EXEMPTION THEREFROM. INVESTORS SHOULD BE AWARE THAT THEY MAY BE REQUIRED TO BEAR THE FINANCIAL RISKS OF THIS INVESTMENT FOR AN INDEFINITE PERIOD OF TIME. THE
ISSUER OF THESE SECURITIES MAY REQUIRE AN OPINION OF COUNSEL IN FORM AND SUBSTANCE SATISFACTORY TO THE ISSUER TO THE EFFECT THAT ANY PROPOSED TRANSFER OR RESALE IS IN COMPLIANCE WITH THE SECURITIES ACT AND ANY APPLICABLE STATE SECURITIES LAWS. 

(b) THE SHARES REPRESENTED BY THIS CERTIFICATE ARE SUBJECT TO CERTAIN RESTRICTIONS ON RESALE AND TRANSFER, INCLUDING THE RIGHT OF FIRST
REFUSAL HELD BY THE ISSUER AND/OR ITS ASSIGNEE(S) AS SET FORTH IN A STOCK OPTION AGREEMENT BETWEEN THE ISSUER AND THE ORIGINAL HOLDER OF THESE SHARES, A COPY OF WHICH MAY BE OBTAINED AT THE PRINCIPAL OFFICE OF THE ISSUER. SUCH SALE AND TRANSFER
RESTRICTIONS, INCLUDING THE RIGHT OF FIRST REFUSAL, ARE BINDING ON TRANSFEREES OF THESE SHARES. 
 (c) THE SHARES REPRESENTED BY THIS
CERTIFICATE ARE SUBJECT TO A MARKET STANDOFF RESTRICTION AS SET FORTH IN A CERTAIN STOCK OPTION AGREEMENT BETWEEN THE ISSUER AND THE ORIGINAL HOLDER OF THESE 

  
 8 

 
SHARES, A COPY OF WHICH MAY BE OBTAINED AT THE PRINCIPAL OFFICE OF THE ISSUER. AS A RESULT OF SUCH AGREEMENT, THESE SHARES MAY NOT BE TRADED PRIOR TO 180 DAYS (AND POSSIBLY LONGER) AFTER THE
EFFECTIVE DATE OF CERTAIN PUBLIC OFFERINGS OF THE COMMON STOCK OF THE ISSUER HEREOF. SUCH RESTRICTION IS BINDING ON TRANSFEREES OF THESE SHARES. 

12.2 Stop-Transfer Instructions. Optionee agrees that, to ensure compliance with the restrictions imposed by this Agreement, the
Company may issue appropriate “stop-transfer” instructions to its transfer agent, if any, and if the Company transfers its own securities, it may make appropriate notations to the same effect in its own records. 

12.3 Refusal to Transfer. The Company will not be required (i) to transfer on its books any Shares that have been sold or
otherwise transferred in violation of any of the provisions of this Agreement or (ii) to treat as owner of such Shares, or to accord the right to vote or pay dividends to any purchaser or other transferee to whom such Shares have been so
transferred. 
 13. CERTAIN TAX CONSEQUENCES. Set forth below is a brief summary as of the Effective Date of the Plan of some of the
federal tax consequences of exercise of the Option and disposition of the Shares. THIS SUMMARY IS NECESSARILY INCOMPLETE, AND THE TAX LAWS AND REGULATIONS ARE SUBJECT TO CHANGE. OPTIONEE SHOULD CONSULT A TAX ADVISER BEFORE EXERCISING THE OPTION OR
DISPOSING OF THE SHARES. 
 13.1 Exercise of ISO. If the Option qualifies as an ISO, there will be no regular federal income
tax liability upon the exercise of the Option, although the excess, if any, of the Fair Market Value of the Shares on the date of exercise over the Exercise Price will be treated as a tax preference item for federal alternative minimum tax purposes
and may subject the Optionee to the alternative minimum tax in the year of exercise. 
 13.2 Exercise of Nonqualified Stock
Option. If the Option does not qualify as an ISO, there may be a regular federal income tax liability upon the exercise of the Option. Optionee will be treated as having received compensation income (taxable at ordinary income tax rates)
equal to the excess, if any, of the Fair Market Value of the Shares on the date of exercise over the Exercise Price. If Optionee is a current or former employee of the Company, the Company may be required to withhold from Optionee’s
compensation or collect from Optionee and pay to the applicable taxing authorities an amount equal to a percentage of this compensation income at the time of exercise. 

13.3 Disposition of Shares. The following tax consequences may apply upon disposition of the Shares. 

(a) Incentive Stock Options. If the Shares are held for more than twelve (12) months after the date of purchase of the Shares
pursuant to the exercise of an ISO and are disposed of more than two (2) years after the Date of Grant, any gain realized on disposition of the Shares will be treated as long term capital gain for federal income tax purposes. If Shares
purchased under an ISO are disposed of within the applicable one (1) year or two (2) year period, any gain realized on such disposition will be treated as compensation income (taxable at ordinary income rates in the year of the
disposition) to the extent of the excess, if any, of the Fair Market Value of the Shares on the date of exercise over the Exercise Price. 

(b) Nonqualified Stock Options. If the Shares are held for more than twelve (12) months after the date of purchase of the Shares
pursuant to the exercise of an NQSO, any gain realized on disposition of the Shares will be treated as long term capital gain. 
 14.
PRIVILEGES OF STOCK OWNERSHIP. Optionee shall not have any of the rights of a stockholder with respect to any Shares unless and until such Shares are issued to Optionee. 

  
 9 

 15. GENERAL PROVISIONS. 

15.1 Interpretation. Any dispute regarding the interpretation of this Agreement shall be submitted by Optionee or the Company to
the Committee for review. The resolution of such a dispute by the Committee shall be final and binding on the Company and Optionee. 

15.2 Entire Agreement. The Plan, the Grant Notice and the Exercise Agreement are each incorporated herein by reference. This
Agreement, the Grant Notice, the Plan and the Exercise Agreement constitute the entire agreement of the parties with respect to the subject matter hereof and supersede all prior undertakings and agreements with respect to such subject matter. 

16. NOTICES. Any and all notices required or permitted to be given to a party pursuant to the provisions of this Agreement will be in
writing and will be effective and deemed to provide such party sufficient notice under this Agreement on the earliest of the following: (i) at the time of personal delivery, if delivery is in person; (ii) at the time of transmission by
facsimile, addressed to the other party at its facsimile number specified herein (or hereafter modified by subsequent notice to the parties hereto), with confirmation of receipt made by both telephone and printed confirmation sheet verifying
successful transmission of the facsimile; (iii) one (1) business day after deposit with an express overnight courier for United States deliveries, or two (2) business days after such deposit for deliveries outside of the United
States, with proof of delivery from the courier requested; or (iv) three (3) business days after deposit in the United States mail by certified mail (return receipt requested) for United States deliveries. Any notice for delivery outside
the United States will be sent by facsimile or by express courier. Any notice not delivered personally or by facsimile will be sent with postage and/or other charges prepaid and properly addressed to the party to be notified at the address or
facsimile number set forth below the signature lines of this Agreement, or at such other address or facsimile number as such other party may designate by one of the indicated means of notice herein to the other parties hereto. Notices to the Company
will be marked “Attention: Chief Financial Officer.” Notices by facsimile shall be machine verified as received. 
 17.
SUCCESSORS AND ASSIGNS. The Company may assign any of its rights under this Agreement including its rights to purchase Shares under the Right of First Refusal. This Agreement shall be binding upon and inure to the benefit of the successors and
assigns of the Company. Subject to the restrictions on transfer set forth herein, this Agreement shall be binding upon Optionee and Optionee’s heirs, executors, administrators, legal representatives, successors and assigns. 

18. GOVERNING LAW. This Agreement shall be governed by and construed in accordance with the internal laws of the State of California as
such laws are applied to agreements between California residents entered into and to be performed entirely within California. If any provision of this Agreement is determined by a court of law to be illegal or unenforceable, then such provision will
be enforced to the maximum extent possible and the other provisions will remain fully effective and enforceable. 
 19. FURTHER
ASSURANCES. The parties agree to execute such further documents and instruments and to take such further actions as may be reasonably necessary to carry out the purposes and intent of this Agreement. 

20. TITLES AND HEADINGS. The titles, captions and headings of this Agreement are included for ease of reference only and will be
disregarded in interpreting or construing this Agreement. Unless otherwise specifically stated, all references herein to “sections” and “exhibits” will mean “sections” and “exhibits” to this Agreement. 

21. COUNTERPARTS. This Agreement may be executed in any number of counterparts, each of which when so executed and delivered will be
deemed an original, and all of which together shall constitute one and the same agreement. 

  
 10 

 22. SEVERABILITY. If any provision of this Agreement is determined by any court or
arbitrator of competent jurisdiction to be invalid, illegal or unenforceable in any respect, such provision will be enforced to the maximum extent possible given the intent of the parties hereto. If such clause or provision cannot be so enforced,
such provision shall be stricken from this Agreement and the remainder of this Agreement shall be enforced as if such invalid, illegal or unenforceable clause or provision had (to the extent not enforceable) never been contained in this Agreement.
Notwithstanding the forgoing, if the value of this Agreement based upon the substantial benefit of the bargain for any party is materially impaired, which determination as made by the presiding court or arbitrator of competent jurisdiction shall be
binding, then both parties agree to substitute such provision(s) through good faith negotiations. 
 * * * * * 

Attachment: Annex A: Form of Stock Option Exercise Notice and Agreement 

  
 11 

 ANNEX A 

FORM OF STOCK OPTION EXERCISE NOTICE AND AGREEMENT 

 STOCK OPTION EXERCISE NOTICE AND AGREEMENT 

AUDENTES THERAPEUTICS, INC. 

2012 EQUITY INCENTIVE PLAN 

*NOTE: You must sign this Notice on Page 3 before submitting it to Audentes Therapeutics, Inc. (the
“Company”). 
 OPTIONEE INFORMATION: Please provide the following information about
yourself (“Optionee”): 
  

									
	Name:	  	  
	 		  	Social Security Number:	  	  

					
	Address:	  	  
	 		  	Employee Number:	  	  

					
		  	  
	 		  		  	

 OPTION INFORMATION: Please provide this information on the option being
exercised (the “Option”): 
  

			
	Grant No.	  	
		
	Date of Grant:	  	Type of Stock Option:
		
	Option Price per Share: $        	  	 ̈ Nonqualified (NQSO)
		
	Total number of shares of Common Stock of the Company subject to the Option:	  	 ̈ Incentive (ISO)

 EXERCISE INFORMATION: 

Number of shares of Common Stock of the Company for which the Option is now being exercised
                . (These shares are referred to below as the “Purchased Shares.”) 

Total Exercise Price Being Paid for the Purchased Shares: $         

Form of payment enclosed [check all that apply]: 
  

			
	 ̈	  	Check for $        , payable to “Audentes Therapeutics, Inc..”
		
	 ̈	  	Certificate(s) for                 shares of Common Stock of the Company. These shares will be valued as of the date this notice is received by the
Company. [Requires Company consent.]

 AGREEMENTS, REPRESENTATIONS AND ACKNOWLEDGMENTS
OF OPTIONEE: By signing this Stock Option Exercise Notice and Agreement, Optionee hereby agrees with, and represents to, the Company as follows: 

 

	1.	Terms Governing. I acknowledge and agree with the Company that I am acquiring the Purchased Shares by exercise of this Option subject to all other terms and conditions of the Notice of Stock Option Grant and the
Stock Option Agreement that govern the Option, including without limitation the terms of the Company’s 2012 Equity Incentive Plan, as it may be amended (the “Plan”). 

 

	2.	 Investment Intent; Securities Law Restrictions. I represent and warrant to the Company that I am acquiring and will hold the Purchased Shares
for investment for my account only, and not with a view to, or for resale in connection with, any “distribution” of the Purchased Shares within the meaning of the Securities Act of 1933, as amended (the “Securities
Act”). I understand that the Purchased Shares have not been registered under the Securities Act by reason of a specific exemption from such 

	 	
registration requirement and that the Purchased Shares must be held by me indefinitely, unless they are subsequently registered under the Securities Act or I obtain an opinion of counsel (in form
and substance satisfactory to the Company and its counsel) that registration is not required. I acknowledge that the Company is under no obligation to register the Purchased Shares under the Securities Act or under any other securities law.

  

	3.	Restrictions on Transfer: Rule 144. I will not sell, transfer or otherwise dispose of the Purchased Shares in violation of the Securities Act, the Securities Exchange Act of 1934, or the rules promulgated
thereunder (including Rule 144 under the Securities Act described below “Rule 144”)) or of any other applicable securities laws. I am aware of Rule 144, which permits limited public resales of securities acquired in a non-public
offering, subject to satisfaction of certain conditions, which include (without limitation) that: (a) certain current public information about the Company is available; (b) the resale occurs only after the holding period required by
Rule 144 has been met; (c) the sale occurs through an unsolicited “broker’s transaction”; and (d) the amount of securities being sold during any three-month period does not exceed specified limitations. I understand
that the conditions for resale set forth in Rule 144 have not been satisfied and that the Company has no plans to satisfy these conditions in the foreseeable future. 

 

	4.	Access to Information; Understanding of Risk in Investment. I acknowledge that I have received and had access to such information as I consider necessary or appropriate for deciding whether to invest in the
Purchased Shares and that I had an opportunity to ask questions and receive answers from the Company regarding the terms and conditions of the issuance of the Purchased Shares. I am aware that my investment in the Company is a speculative investment
that has limited liquidity and is subject to the risk of complete loss. I am able, without impairing my financial condition, to hold the Purchased Shares for an indefinite period and to suffer a complete loss of my investment in the Purchased
Shares. 

  

	5.	Rights of First Refusal; Market Stand-off. I acknowledge that the Purchased Shares remain subject to the Company’s Right of First Refusal and the market stand-off covenants (sometimes referred to as the
“lock-up”), all in accordance with the applicable Notice of Stock Option Grant and the Stock Option Agreement that govern the Option. 

  

	6.	Form of Ownership. I acknowledge that the Company has encouraged me to consult my own adviser to determine the form of ownership of the Purchased Shares that is appropriate for me. In the event that I choose to
transfer my Purchased Shares to a trust, I agree to sign a Stock Transfer Agreement. In the event that I choose to transfer my Purchased Shares to a trust that is not an eligible revocable trust, I also acknowledge that the transfer will be treated
as a “disposition” for tax purposes. As a result, the favorable ISO tax treatment will be unavailable and other unfavorable tax consequences may occur. 

  

	7.	Investigation of Tax Consequences. I acknowledge that the Company has encouraged me to consult my own adviser to determine the tax consequences of acquiring the Purchased Shares at this time. 

 

	8.	Other Tax Matters. I agree that the Company does not have a duty to design or administer the Plan or its other compensation programs in a manner that minimizes my tax liabilities. I will not make any claim
against the Company or its Board, officers or employees related to tax liabilities arising from my options or my other compensation. In particular, I acknowledge that my options (including the Option) are exempt from section 409A of the
Internal Revenue Code only if the exercise price per share is at least equal to the fair market value per share of the Common Stock at the time the option was granted by the Board. Since shares of the Common Stock are not traded on an established
securities market, the determination of their fair market value was made by the Board and/or by an independent valuation firm retained by the Company. I acknowledge that there is no guarantee in either case that the Internal Revenue Service will
agree with the valuation, and I will not make any claim against the Company or its Board, officers or employees in the event that the Internal Revenue Service asserts that the valuation was too low. 

  
 3 

	9.	Spouse Consent. I agree to seek the consent of my spouse to the extent required by the Company to enforce the foregoing. 

  

	10.	Tax Withholding. As a condition of exercising this Option, I agree to make adequate provision for foreign, federal, state or other tax withholding obligations, if any, which arise upon the grant, vesting or
exercise of this Option, or disposition of the Purchased Shares, whether by withholding, direct payment to the Company, or otherwise. 

 The
undersigned hereby executes and delivers this Stock Option Exercise Notice and Agreement to agrees to be bound by its terms 
  

											
	SIGNATURE:	 		  		  	DATE:	  		  	
				
		  		  		  	
	  
	  		  	  
	  	
	Optionee’s Name:	 		  		  		  		  	

 [Signature Page to Stock Option Exercise Notice and Agreement] 

  
 4

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