Document:

EX-10.166

 Exhibit 10.166 
 AMERICAN AIRLINES GROUP INC. 2013 INCENTIVE AWARD PLAN 
 ARTICLE 1

 PURPOSE 
 The purpose of the American Airlines Group Inc. 2013 Incentive Award Plan (as it may be amended or restated from time to time, the “Plan”) is to promote the success and enhance the value
of AMR Corporation, a Delaware corporation (together with its successors and assigns, the “Company”), by linking the individual interests of the members of the Board, Employees and Consultants to those of Company stockholders and by
providing such individuals with an incentive for outstanding performance to generate superior returns to Company stockholders. The Plan is further intended to provide flexibility to the Company in its ability to motivate, attract, and retain the
services of members of the Board, Employees and Consultants upon whose judgment, interest and special effort the successful conduct of the Company’s operation is largely dependent. The Plan shall be subject to and effective as of the date of a
merger (“Effective Date”) between the AMR Merger Sub, Inc., a Delaware corporation (“Merger Sub”), and US Airways, Inc., a Delaware corporation (“US Airways”), pursuant to an Agreement and Plan of
Merger dated February 13, 2013, among the Company, Merger Sub and US Airways, as amended, modified, supplemented or terminated (“Merger Agreement”). In connection with the transactions contemplated under the Merger Agreement,
AMR Corporation will be re-named as American Airlines Group Inc. as of the Effective Date. 
 ARTICLE 2 

DEFINITIONS AND CONSTRUCTION 
 Wherever the following terms are used in the Plan they shall have the meanings specified below, unless the context clearly indicates otherwise. The singular pronoun shall include the plural where the
context so indicates. 
 2.1 “Administrator” shall mean the entity that conducts the general
administration of the Plan as provided in Article 13. With reference to the duties of the Committee under the Plan which have been delegated to one or more persons pursuant to Section 13.6, or as to which the Board has assumed, the term
“Administrator” shall refer to such person(s) unless the Committee or the Board has revoked such delegation or the Board has terminated the assumption of such duties. 

2.2 “Affiliate” shall mean (a) any Subsidiary; and (b) any domestic eligible entity that is
disregarded, under Treasury Regulation Section 301.7701-3, as an entity separate from either (i) the Company or (ii) any Subsidiary. 
 2.3 “Applicable Accounting Standards” shall mean Generally Accepted Accounting Principles in the United States, International Financial Reporting Standards or such other accounting
principles or standards as may apply to the Company’s financial statements under United States federal securities laws from time to time. 

 2.4 “Award” shall mean an Option, a Restricted Stock award, a
Restricted Stock Unit award, a Performance Award, a Dividend Equivalents award, a Deferred Stock award, a Deferred Stock Unit award, a Stock Payment award or a Stock Appreciation Right, which may be awarded or granted under the Plan (collectively,
“Awards”). 
 2.5 “Award Agreement” shall mean any written notice, agreement, terms and
conditions, contract or other instrument or document evidencing an Award, including through electronic medium, which shall contain such terms and conditions with respect to an Award as the Administrator shall determine consistent with the Plan.

 2.6 “Award Limit” shall mean with respect to Awards that shall be payable in Shares or in cash, as
the case may be, the respective limit set forth in Section 3.3. 
 2.7 “Board” shall mean the Board
of Directors of the Company. 
 2.8 “Change in Control” shall mean the occurrence after the Effective
Date of any of the following: 
 (a) within any 12-month period, the individuals who constitute the Board at the
beginning of such period (the “Incumbent Board”) cease for any reason to constitute at least a majority of the Board; provided, however, that any individual becoming a director subsequent to the Effective Date whose
election, or nomination for election by the Company’s stockholders, was approved by a vote of at least two-thirds of the directors then comprising the Incumbent Board shall be considered as though such individual were a member of the Incumbent
Board; or 
 (b) any individual, entity or group (within the meaning of Section 13(d)(3) or 14(d)(2) of the
Exchange Act, other than the Company, acquires (directly or indirectly) the beneficial ownership (within the meaning of Rule 13d-3 promulgated under the Exchange Act) of more than 50% of the combined voting power of the then outstanding voting
securities of the Company entitled to vote generally in the election of directors (“Voting Power”); or 
 (c) the Company consummates a merger, consolidation or reorganization of the Company or any other similar transaction or series of related transactions (collectively, a “Transaction”)
other than (A) a Transaction in which the voting securities of the Company outstanding immediately prior thereto become (by operation of law), or are converted into or exchanged for, voting securities of the surviving corporation or its parent
corporation immediately after such Transaction that are owned by the same person or entity or persons or entities as immediately prior thereto and possess at least 50% of the Voting Power held by the voting securities of the surviving corporation or
its parent corporation, or (B) a Transaction effected to implement a recapitalization of the Company (or similar transaction) in which no person (excluding the Company or any person who held more than 50% of the Voting Power immediately prior
to such Transaction) acquires more than 50% of the Voting Power; or 
 (d) the Company sells or otherwise
disposes of, or consummates a transaction or series of related transactions providing for the sale or other disposition of, all or substantially all of the stock or assets of the Company, or enters into a plan for the complete liquidation of the
Company. 

  
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 Notwithstanding the foregoing, (i) the contemplated transactions under the Merger
Agreement shall not constitute a Change in Control and (ii) if a Change in Control constitutes a payment event with respect to any Award which provides for the deferral of compensation and is subject to Section 409A of the Code, the
transaction or event described in subsection (a), (b), (c) or (d) with respect to such Award must also constitute a “change in control event,” as defined in Treasury Regulation § 1.409A-3(i)(5) to the extent required by
Section 409A. 
 The Committee shall have full and final authority, which shall be exercised in its sole discretion, to
determine conclusively whether a Change in Control has occurred pursuant to the above definition, and the date of the occurrence of such Change in Control and any incidental matters relating thereto. 

2.9 “Code” shall mean the Internal Revenue Code of 1986, as amended from time to time, together with the
regulations and official guidance promulgated thereunder. 
 2.10 “Committee” shall mean the
Compensation Committee of the Board, another committee or subcommittee of the Board, appointed as provided in Section 13.1; provided, that if any member of the Compensation Committee does not qualify as (i) an outside director for
purposes of Section 162(m) of the Code, (ii) a non-employee director for purposes of Rule 16b-3, and (iii) an independent director for purposes of the rules of the exchange on which the Shares are traded, the remaining members of the
Compensation Committee (but not less than two members) shall be constituted as a subcommittee to act as the Committee for purposes of the Plan. 
 2.11 “Common Stock” shall mean the common stock of the Company. 
 2.12 “Company” shall have the meaning set forth in Article 1. 
 2.13 “Consultant” shall mean any consultant or adviser engaged to provide services to the Company or any Affiliate that qualifies as a consultant under the applicable rules of the
Securities and Exchange Commission for registration of shares on a Form S-8 Registration Statement. 
 2.14 “Covered
Employee” shall mean any Employee who is, or could be, a “covered employee” within the meaning of Section 162(m) of the Code. 
 2.15 “Deferred Stock” shall mean a right to receive Shares awarded under Section 10.4. 
 2.16 “Deferred Stock Unit” shall mean a right to receive Shares awarded under Section 10.5. 
 2.17 “Director” shall mean a member of the Board, as constituted from time to time. 

  
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 2.18 “Dividend Equivalent” shall mean a right to receive the
equivalent value (in cash or Shares) of dividends paid on Shares, awarded under Section 10.2. 
 2.19
“DRO” shall mean a domestic relations order as defined by the Code or Title I of the Employee Retirement Income Security Act of 1974, as amended from time to time, or the rules thereunder. 

2.20 “Effective Date” shall have the meaning set forth in Article I. 

2.21 “Eligible Individual” shall mean any person who is an Employee, a Consultant or a Non-Employee Director, as
determined by the Committee. 
 2.22 “Employee” shall mean any officer or other employee (as determined
in accordance with Section 3401(c) of the Code and the Treasury Regulations thereunder) of the Company or of any Affiliate. 
 2.23 “Equity Restructuring” shall mean a nonreciprocal transaction between the Company and its stockholders, such as a stock dividend, stock split, spin-off, rights offering or
recapitalization through a large, nonrecurring cash dividend, that affects the number or kind of Shares (or other securities of the Company) or the share price of Common Stock (or other securities) and causes a change in the per share value of the
Common Stock underlying outstanding Awards. 
 2.24 “Exchange Act” shall mean the Securities Exchange
Act of 1934, as amended from time to time. 
 2.25 “Fair Market Value” shall mean, as of any given date,
the value of a Share determined as follows: 
 (a) If the Common Stock is listed on any (i) established
securities exchange (such as the New York Stock Exchange, the NASDAQ Global Market and the NASDAQ Global Select Market), (ii) national market system or (iii) automated quotation system on which the Shares are listed, quoted or traded, its
Fair Market Value shall be the closing sales price for a Share as quoted on such exchange or system for such date or, if there is no closing sales price for a Share on the date in question, the closing sales price for a Share on the last preceding
date for which such quotation exists, as reported in The Wall Street Journal or such other source as the Administrator deems reliable; 
 (b) If the Common Stock is not listed on an established securities exchange, national market system or automated quotation system, but the Common Stock is regularly quoted by a recognized securities
dealer, its Fair Market Value shall be the mean of the high bid and low asked prices for such date or, if there are no high bid and low asked prices for a Share on such date, the high bid and low asked prices for a Share on the last preceding date
for which such information exists, as reported in The Wall Street Journal or such other source as the Administrator deems reliable; or 

  
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 (c) If the Common Stock is neither listed on an established securities
exchange, national market system or automated quotation system nor regularly quoted by a recognized securities dealer, its Fair Market Value shall be established by the Administrator in good faith. 

2.26 “Full Value Award” shall mean any Award other than (i) an Option, (ii) a Stock Appreciation Right
or (iii) any other Award for which the Holder pays the intrinsic value existing as of the date of grant (whether directly or by forgoing a right to receive a payment from the Company or any Affiliate). 

2.27 “Greater Than 10% Stockholder” shall mean an individual then owning (within the meaning of
Section 424(d) of the Code) more than 10% of the total combined voting power of all classes of stock of the Company or any subsidiary corporation (as defined in Section 424(f) of the Code) or parent corporation thereof (as defined in
Section 424(e) of the Code). 
 2.28 “Holder” shall mean a person who has been granted an Award.

 2.29 “Incentive Stock Option” shall mean an Option that is intended to qualify as an incentive stock
option and conforms to the applicable provisions of Section 422 of the Code. 
 2.30 “Non-Employee
Director” shall mean a Director of the Company who is not an Employee. 
 2.31 “Non-Employee Director
Equity Compensation Policy” shall have the meaning set forth in Section 4.6. 
 2.32 “Non-Qualified
Stock Option” shall mean an Option that is not an Incentive Stock Option. 
 2.33 “Option”
shall mean a right to purchase Shares at a specified exercise price, granted under Article 6. An Option shall be either a Non-Qualified Stock Option or an Incentive Stock Option; provided, however, that Options granted to Non-Employee
Directors and Consultants shall only be Non-Qualified Stock Options. 
 2.34 “Option Term” shall have
the meaning set forth in Section 6.4. 
 2.35 “Parent” shall mean any entity (other than the
Company), whether domestic or foreign, in an unbroken chain of entities ending with the Company if each of the entities other than the Company beneficially owns, at the time of the determination, securities or interests representing at least 50% of
the total combined voting power of all classes of securities or interests in one of the other entities in such chain. 
 2.36
“Performance Award” shall mean a cash bonus award, stock bonus award, performance award or incentive award that is paid in cash, Shares or a combination of both, awarded under Section 10.1. 

  
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 2.37 “Performance-Based Compensation” shall mean any compensation
that is intended to qualify as “performance-based compensation” as described in Section 162(m)(4)(C) of the Code. 
 2.38 “Performance Criteria” shall mean the criteria (and adjustments) that the Committee selects for an Award for purposes of establishing the Performance Goal or Performance Goals
for a Performance Period, determined as follows: 
 (a) The Performance Criteria that shall be used to establish
Performance Goals are limited to the following: (i) earnings before interest, taxes, depreciation, rent and amortization expenses (“EBITDAR”); (ii) earnings before interest, taxes, depreciation and amortization
(“EBITDA”); (iii) earnings before interest and taxes (“EBIT”); (iv) EBITDAR, EBITDA, EBIT or earnings before taxes and unusual, special or nonrecurring items as measured either against the annual budget or
as a ratio to revenue or return on total capital; (v) net earnings; (vi) earnings per share; (vii) net income (before or after taxes); (viii) profit margin; (ix) operating margin; (x) operating income; (xi) net
operating income; (xii) net operating income after taxes; (xiii) growth; (xiv) net worth; (xv) cash flow; (xvi) cash flow per share; (xvii) total stockholder return; (xviii) return on capital, assets, equity or
investment; (xix) stock price performance; (xx) revenues; (xxi) revenues per available seat mile; (xxii) costs; (xxiii) costs per available seat mile; (xxiv) working capital; (xxv) capital expenditures or
statistics; (xxvi) improvements in capital structure; (xxvii) economic value added; (xxviii) industry indices; (xxix) regulatory ratings; (xxx) customer satisfaction ratings; (xxxi) expenses and expense ratio
management; (xxxii) debt reduction; (xxxiii) profitability of an identifiable business unit or product; (xxxiv) levels of expense, cost or liability by category, operating unit or any other delineation; (xxxv) implementation or
completion of projects or processes; (xxxvi) combination of airline operating certificates within a specified period; (xxxvii) measures of operational performance (including, without limitation, U.S. Department of Transportation
performance rankings in operational areas), quality, safety, productivity or process improvement; (xxxviii) measures of employee satisfaction or employee engagement, any of which may be measured either in absolute terms or as compared to any
incremental increase or decrease or as compared to results of a peer group or to market performance indicators or indices or, where applicable, on a per-share or per seat-mile basis. 

(b) The Administrator may, in its sole discretion, provide that one or more objectively determinable adjustments shall be
made to one or more of the Performance Goals. Such adjustments may include one or more of the following: (i) items related to a change in accounting principle; (ii) items relating to financing activities; (iii) expenses for
restructuring or productivity initiatives; (iv) other non-operating items; (v) items related to acquisitions; (vi) items attributable to the business operations of any entity acquired by the Company during the Performance Period;
(vii) items related to the disposal of a business or segment of a business; (viii) items related to discontinued operations that do not qualify as a segment of a business under Applicable Accounting Standards; (ix) items attributable
to any stock dividend, stock split, combination or exchange of stock occurring during the Performance Period; (x) any other items of significant income or expense which are determined to be appropriate adjustments; (xi) items relating to

  
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unusual or extraordinary corporate transactions, events or developments, (xii) items related to amortization of acquired intangible assets; (xiii) items that are outside the scope of
the Company’s core, on-going business activities; (xiv) items related to acquired in-process research and development; (xv) items relating to changes in tax laws; (xvi) items relating to major licensing or partnership
arrangements; (xvii) items relating to asset impairment charges; (xviii) items relating to gains or losses for litigation, arbitration and contractual settlements; or (xix) items relating to any other unusual or nonrecurring events or
changes in applicable laws, accounting principles or business conditions. For all Awards intended to qualify as Performance-Based Compensation, such determinations shall be made within the time prescribed by, and otherwise in compliance with,
Section 162(m) of the Code. 
 2.39 “Performance Goals” shall mean, for a Performance Period, one
or more goals established in writing by the Administrator for the Performance Period based upon one or more Performance Criteria. Depending on the Performance Criteria used to establish such Performance Goals, the Performance Goals may be expressed
in terms of overall Company performance or the performance of a Subsidiary, division, business unit, or an individual. The achievement of each Performance Goal shall be determined, to the extent applicable, with reference to Applicable Accounting
Standards. 
 2.40 “Performance Period” shall mean one or more periods of time, which may be of varying
and overlapping durations, as the Administrator may select, over which the attainment of one or more Performance Goals will be measured for the purpose of determining a Holder’s right to, and the payment of, an Award. 

2.41 “Performance Stock Unit” shall mean a Performance Award awarded under Section 10.1 which is denominated
in units of value including dollar value of Shares. 
 2.42 “Permitted Transferee” shall mean, with
respect to a Holder, any “family member” of the Holder, as defined under the instructions to use the Form S-8 Registration Statement under the Securities Act, after taking into account any state, federal, local or foreign tax and
securities laws applicable to transferable Awards. 
 2.43 “Plan” shall have the meaning set forth in
Article 1. 
 2.44 “Program” shall mean any program adopted by the Administrator pursuant to the Plan
containing the terms and conditions intended to govern a specified type of Award granted under the Plan and pursuant to which such type of Award may be granted under the Plan. 
 2.45 “Restricted Stock” shall mean Common Stock awarded under Article 8 that is subject to certain restrictions and may be subject to risk of forfeiture or repurchase. 

2.46 “Restricted Stock Units” shall mean the right to receive Shares awarded under Article 9. 

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

2.48 “Shares” shall mean shares of Common Stock. 

  
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 2.49 “Stock Appreciation Right” shall mean a stock appreciation
right granted under Article 11. 
 2.50 “Stock Appreciation Right Term” shall have the meaning set forth
in Section 11.4. 
 2.51 “Stock Payment” shall mean (a) a payment in the form of Shares, or
(b) an option or other right to purchase Shares, as part of a bonus, deferred compensation or other arrangement, awarded under Section 10.3. 
 2.52 “Subsidiary” shall mean any entity (other than the Company), whether domestic or foreign, in an unbroken chain of entities beginning with the Company if each of the entities
other than the last entity in the unbroken chain beneficially owns, at the time of the determination, securities or interests representing at least 50% of the total combined voting power of all classes of securities or interests in one of the other
entities in such chain. 
 2.53 “Substitute Award” shall mean an Award granted under the Plan upon the
assumption of, or in substitution for, outstanding equity awards previously granted by a company or other entity in connection with a corporate transaction, such as a merger, combination, consolidation or acquisition of property or stock;
provided, however, that in no event shall the term “Substitute Award” be construed to refer to an award made in connection with the cancellation and repricing of an Option or Stock Appreciation Right. 

2.54 “Termination of Service” shall mean: 

(a) As to a Consultant, the time when the engagement of a Holder as a Consultant to the Company or an Affiliate is
terminated for any reason, with or without cause, including, without limitation, by resignation, discharge, death or retirement, but excluding terminations where the Consultant simultaneously commences or remains in employment or service with the
Company or any Affiliate. 
 (b) As to a Non-Employee Director, the time when a Holder who is a Non-Employee
Director ceases to be a Director for any reason, including, without limitation, a termination by resignation, failure to be elected, death or retirement, but excluding terminations where the Holder simultaneously commences or remains in employment
or service with the Company or any Affiliate. 
 (c) As to an Employee, the time when the employee-employer
relationship between a Holder and the Company or any Affiliate is terminated for any reason, including, without limitation, a termination by resignation, discharge, death, disability or retirement; but excluding terminations where the Holder
simultaneously commences or remains in employment or service with the Company or any Affiliate. 
 The Administrator, in its
sole discretion, shall determine the effect of all matters and questions relating to any Termination of Service, including, without limitation, the question of whether a Termination of Service resulted from a discharge for cause and all questions of
whether particular leaves of absence constitute a Termination of Service; provided, however, that, with respect to Incentive Stock Options, unless the Administrator otherwise provides in the terms of the Program, the Award Agreement or
otherwise, a leave of absence, change in status 

  
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from an employee to an independent contractor or other change in the employee-employer relationship shall constitute a Termination of Service only if, and to the extent that, such leave of
absence, change in status or other change interrupts employment for the purposes of Section 422(a)(2) of the Code and the then applicable regulations and revenue rulings under said Section. For purposes of the Plan, a Holder’s
employee-employer relationship or consultancy relations shall be deemed to be terminated in the event that the Affiliate employing or contracting with such Holder ceases to remain an Affiliate following any merger, sale of stock or other corporate
transaction or event (including, without limitation, a spin-off). 
 ARTICLE 3 

SHARES SUBJECT TO THE PLAN 
 3.1 Number of Shares. 
 (a) Subject to adjustment as
provided in Section 3.1(b) and Section 14.2, the aggregate number of Shares which may be issued or transferred pursuant to Awards under the Plan is 40,000,000. 

(b) Any Shares subject to an Option, Stock Appreciation Right, Restricted Stock or other Award, or any award granted under
any pre-existing plan described in Section 3.1(c) below, that, in either case, after the date the Plan becomes effective, is forfeited, terminated or otherwise settled, in whole or in part, without a payment being made to the Holder in the form
of Shares shall again be available for distribution in connection with future Awards under the Plan. In addition, (i) any Shares of Restricted Stock repurchased by the Company at the same or lesser price paid by the Holder will again be
available for Awards and (ii) any payment of Dividend Equivalents in cash in conjunction with any outstanding Awards shall not be counted against the Shares available for issuance under the Plan. Without limiting the generality of the preceding
sentence, upon the exercise of a Stock Appreciation Right, regardless of whether granted on a stand-alone basis or in tandem with any Stock Option, only the number of Shares actually issued in connection with the exercise of such Stock Appreciation
Right (and not the corresponding number of Shares related to the Stock Appreciation Right (or portion thereof) being exercised) shall be treated as issued under the Plan and the remaining number of Shares related to such exercised Stock Appreciation
Right (or portion thereof), including the corresponding number of Shares related to any tandem Stock Option cancelled upon such exercise, shall again be available for issuance under the Plan. For avoidance of doubt, Shares purchased on the open
market with the cash proceeds from the exercise of Options shall not be added to the Shares authorized for grant under Section 3.1(a) and will not be available for future grants of Awards. 

(c) Substitute Awards shall not reduce the Shares authorized for grant under the Plan. Additionally, in the event that a
company acquired by the Company or any Affiliate or with which the Company or any Affiliate combines has shares available under a pre-existing plan approved by stockholders and not adopted in contemplation of such acquisition or combination, the
shares available for grant pursuant to the terms of such pre-existing plan (as adjusted, to the extent appropriate, using the exchange ratio or 

  
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other adjustment or valuation ratio or formula used in such acquisition or combination to determine the consideration payable to the holders of common stock of the entities party to such
acquisition or combination) may be used for Awards under the Plan, without duplication by reason of any outstanding awards under such pre-existing plan, and shall not reduce the Shares authorized for grant under the Plan; provided, that
Awards using such available Shares shall not be made after the date awards or grants could have been made under the terms of the pre-existing plan, absent the acquisition or combination, and shall only be made to individuals who were not employed by
or providing services to the Company or its Affiliates immediately prior to such acquisition or combination. 
 3.2 Stock
Distributed. Any Shares distributed pursuant to an Award may consist, in whole or in part, of authorized and unissued Common Stock, treasury Common Stock or Common Stock purchased on the open market. 

3.3 Limitation on Number of Shares Subject to Awards. Notwithstanding any provision in the Plan to the contrary, and
subject to Section 14.2, the maximum aggregate number of Shares with respect to one or more Awards that may be granted to any one person during any calendar year shall be 2,200,000 and the maximum aggregate amount of cash that may be paid in
cash to any one person during any calendar year with respect to one or more Awards payable in cash shall be $10,000,000. 
 3.4
Full Value Award Vesting Limitations. Notwithstanding any other provision of the Plan to the contrary, Full Value Awards made to Eligible Individuals shall become vested in one or more installments over an aggregate period of not less
than three years (or, in the case of vesting based upon the attainment of Performance Goals or other performance-based objectives, over a period of not less than one year measured from the commencement of the period over which performance is
evaluated) following the date the Award is made; provided, however, that, notwithstanding the foregoing, (a) the Administrator may provide that such vesting restrictions may lapse or be waived upon the Holder’s death,
disability, retirement, any other specified Termination of Service or the consummation of a Change in Control, (b) Full Value Awards may be granted as part of Non-Employee Director retainers without respect to such minimum vesting provisions,
(c) Full Value Awards that result in the issuance of an aggregate of up to 10% of the Shares available pursuant to Section 3.1(a) may be granted to any one or more Holders without respect to such minimum vesting provisions, and
(d) Full Value Awards may be granted in accordance with the Merger Agreement and effective as of Effective Date without regard to such minimum vesting provisions. 
 ARTICLE 4 
 GRANTING OF AWARDS 

4.1 Participation. The Administrator may, from time to time, select from among all Eligible Individuals, those to whom an
Award shall be granted and shall determine the nature and amount of each Award, which shall not be inconsistent with the requirements of the Plan. Except as provided in Section 4.6 regarding the grant of Awards pursuant to the Non-Employee
Director Equity Compensation Policy, no Eligible Individual shall have any right to be granted an Award pursuant to the Plan. 

  
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 4.2 Award Agreement. Each Award shall be evidenced by an Award Agreement that
sets forth the terms, conditions and limitations for such Award, which may include the term of the Award, the provisions applicable in the event of the Holder’s Termination of Service, and the Company’s authority to unilaterally or
bilaterally amend, modify, suspend, cancel or rescind an Award. Award Agreements evidencing Awards intended to qualify as Performance-Based Compensation shall contain such terms and conditions as may be necessary to meet the applicable provisions of
Section 162(m) of the Code. Award Agreements evidencing Incentive Stock Options shall contain such terms and conditions as may be necessary to meet the applicable provisions of Section 422 of the Code. 

4.3 Limitations Applicable to Section 16 Persons. Notwithstanding any other provision of the Plan, the Plan, and any
Award granted or awarded to any individual who is then subject to Section 16 of the Exchange Act, shall be subject to any additional limitations set forth in any applicable exemptive rule under Section 16 of the Exchange Act (including
Rule 16b-3 of the Exchange Act and any amendments thereto) that are requirements for the application of such exemptive rule. To the extent permitted by applicable law, the Plan and Awards granted or awarded hereunder shall be deemed amended to the
extent necessary to conform to such applicable exemptive rule. 
 4.4 At-Will Employment: Voluntary Participation.
Nothing in the Plan or in any Program or Award Agreement hereunder shall confer upon any Holder any right to continue in the employ of, or as a Director or Consultant for, the Company or any Affiliate, or shall interfere with or restrict in any way
the rights of the Company and any Affiliate, which rights are hereby expressly reserved, to discharge any Holder at any time for any reason whatsoever, with or without cause, and with or without notice, or to terminate or change all other terms and
conditions of employment or engagement, except to the extent expressly provided otherwise in a written agreement between the Holder and the Company or any Affiliate. Participation by each Holder in the Plan shall be voluntary and nothing in the Plan
shall be construed as mandating that any Eligible Individual shall participate in the Plan. 
 4.5 Foreign
Holders. Notwithstanding any provision of the Plan to the contrary, in order to comply with the laws in countries other than the United States in which the Company and its Affiliates operate or have Employees, Non-Employee Directors or
Consultants, or in order to comply with the requirements of any foreign securities exchange, the Administrator, in its sole discretion, shall have the power and authority to: (a) determine which Affiliates shall be covered by the Plan;
(b) determine which Eligible Individuals outside the United States are eligible to participate in the Plan; (c) modify the terms and conditions of any Award granted to Eligible Individuals outside the United States to comply with
applicable foreign laws or listing requirements of any such foreign securities exchange; (d) establish subplans and modify exercise procedures and other terms and procedures, to the extent such actions may be necessary or advisable (any such
subplans and/or modifications shall be attached to the Plan as appendices); provided, however, that no such subplans and/or modifications shall increase the Share limitations contained in Sections 3.1 and 3.3; and (e) take any
action, before or after an Award is made, that it deems advisable to obtain approval or comply with any necessary local governmental regulatory exemptions or approvals or listing requirements of any such foreign securities exchange. Notwithstanding
the foregoing, the Administrator may not take any actions hereunder, and no Awards shall be granted, that would violate the Code, the Exchange Act, the 

  
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Securities Act, any other securities law or governing statute, the rules of the securities exchange or automated quotation system on which the Shares are listed, quoted or traded or any other
applicable law. For purposes of the Plan, all references to foreign laws, rules, regulations or taxes shall be references to the laws, rules, regulations and taxes of any applicable jurisdiction other than the United States or a political
subdivision thereof. 
 4.6 Non-Employee Director Awards. The Administrator may, in its sole discretion, provide
that Awards granted to Non-Employee Directors shall be granted pursuant to a written non-discretionary formula established by the Administrator (the “Non-Employee Director Equity Compensation Policy”), subject to the limitations of
the Plan. The Non-Employee Director Equity Compensation Policy shall set forth the type of Award(s) to be granted to Non-Employee Directors, the number of Shares to be subject to Non-Employee Director Awards, the conditions on which such Awards
shall be granted, become exercisable and/or payable and expire, and such other terms and conditions as the Administrator shall determine in its sole discretion. The Non-Employee Director Equity Compensation Policy may be modified by the
Administrator from time to time in its sole discretion. 
 4.7 Stand-Alone and Tandem Awards. Awards granted
pursuant to the Plan may, in the sole discretion of the Administrator, be granted either alone, in addition to, or in tandem with, any other Award granted pursuant to the Plan. Awards granted in addition to or in tandem with other Awards may be
granted either at the same time as or at a different time from the grant of such other Awards. 
 ARTICLE 5 

PROVISIONS APPLICABLE TO AWARDS INTENDED TO QUALIFY AS 
 PERFORMANCE-BASED COMPENSATION. 
 5.1 Purpose. The Committee, in its
sole discretion, may determine at the time an Award is granted or at any time thereafter whether such Award is intended to qualify as Performance-Based Compensation. If the Committee, in its sole discretion, decides to grant an Award to an Eligible
Individual that is intended to qualify as Performance-Based Compensation, then the provisions of this Article 5 shall control over any contrary provision contained in the Plan. The Administrator may in its sole discretion grant Awards to Eligible
Individuals that are based on Performance Criteria or Performance Goals but that do not satisfy the requirements of this Article 5 and that are not intended to qualify as Performance-Based Compensation. Unless otherwise specified by the
Administrator at the time of grant, the Performance Criteria with respect to an Award intended to be Performance-Based Compensation payable to a Covered Employee shall be determined on the basis of Applicable Accounting Standards. 

5.2 Applicability. The grant of an Award to an Eligible Individual for a particular Performance Period shall not require
the grant of an Award to such Individual in any subsequent Performance Period and the grant of an Award to any one Eligible Individual shall not require the grant of an Award to any other Eligible Individual in such period or in any other period.

 5.3 Types of Awards. Notwithstanding anything in the Plan to the contrary, the Committee may grant any Award to
an Eligible Individual intended to qualify as Performance-

  
 12 

 
Based Compensation, including, without limitation, Restricted Stock the restrictions with respect to which lapse upon the attainment of specified Performance Goals, Restricted Stock Units that
vest and become payable upon the attainment of specified Performance Goals and any Performance Awards described in Article 10 that vest or become exercisable or payable upon the attainment of one or more specified Performance Goals. 

5.4 Procedures with Respect to Performance-Based Awards. To the extent necessary to comply with the requirements of
Section 162(m)(4)(C) of the Code, with respect to any Award granted to one or more Eligible Individuals which is intended to qualify as Performance-Based Compensation, no later than 90 days following the commencement of any Performance Period
or any designated fiscal period or period of service (or such earlier time as may be required under Section 162(m) of the Code), the Committee shall, in writing, (a) designate one or more Eligible Individuals, (b) select the
Performance Criteria applicable to the Performance Period, (c) establish the Performance Goals, and amounts of such Awards, as applicable, which may be earned for such Performance Period based on the Performance Criteria, and (d) specify
the relationship between Performance Criteria and the Performance Goals and the amounts of such Awards, as applicable, to be earned by each Covered Employee for such Performance Period. Following the completion of each Performance Period, the
Committee shall certify in writing whether and the extent to which the applicable Performance Goals have been achieved for such Performance Period. In determining the amount earned under such Awards, the Committee shall have the right to reduce or
eliminate (but not to increase) the amount payable at a given level of performance to take into account additional factors that the Committee may deem relevant, including the assessment of individual or corporate performance for the Performance
Period. 
 5.5 Payment of Performance-Based Awards. Unless otherwise provided in the applicable Program or Award
Agreement and only to the extent otherwise permitted by Section 162(m)(4)(C) of the Code, as to an Award that is intended to qualify as Performance-Based Compensation, the Holder must be employed by the Company or an Affiliate throughout the
Performance Period. Unless otherwise provided in the applicable Performance Goals, Program or Award Agreement, a Holder shall be eligible to receive payment pursuant to such Awards for a Performance Period only if and to the extent the Performance
Goals for such period are achieved. 
 5.6 Additional Limitations. Notwithstanding any other provision of the Plan
and except as otherwise determined by the Administrator, any Award which is granted to an Eligible Individual and is intended to qualify as Performance-Based Compensation shall be subject to any additional limitations set forth in
Section 162(m) of the Code or any regulations or rulings issued thereunder that are requirements for qualification as Performance-Based Compensation, and the Plan, the applicable Program and Award Agreement shall be deemed amended to the extent
necessary to conform to such requirements. 

  
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 ARTICLE 6 
 GRANTING OF OPTIONS 
 6.1 Granting of Options to Eligible
Individuals. The Administrator is authorized to grant Options to Eligible Individuals from time to time, in its sole discretion, on such terms and conditions as it may determine which shall not be inconsistent with the Plan. 

6.2 Qualification of Incentive Stock Options. No Incentive Stock Option shall be granted to any person who is not an
Employee of the Company or any subsidiary corporation (as defined in Section 424(f) of the Code) of the Company. No person who qualifies as a Greater Than 10% Stockholder may be granted an Incentive Stock Option unless such Incentive Stock
Option conforms to the applicable provisions of Section 422 of the Code. Any Incentive Stock Option granted under the Plan may be modified by the Administrator, with the consent of the Holder, to disqualify such Option from treatment as an
“incentive stock option” under Section 422 of the Code. To the extent that the aggregate Fair Market Value of stock with respect to which “incentive stock options” (within the meaning of Section 422 of the Code, but
without regard to Section 422(d) of the Code) are exercisable for the first time by a Holder during any calendar year under the Plan, and all other plans of the Company and any parent or subsidiary corporation thereof (each as defined in
Section 424(e) and (f) of the Code, respectively), exceeds $100,000, the Options shall be treated as Non-Qualified Stock Options to the extent required by Section 422 of the Code. The rule set forth in the immediately preceding
sentence shall be applied by taking Options and other “incentive stock options” into account in the order in which they were granted and the Fair Market Value of stock shall be determined as of the time the respective options were granted.

 6.3 Option Exercise Price. The exercise price per Share subject to each Option shall be set by the
Administrator, but shall not be less than 100% of the Fair Market Value of a Share on the date the Option is granted (or, as to Incentive Stock Options, on the date the Option is modified, extended or renewed for purposes of Section 424(h) of
the Code). In addition, in the case of Incentive Stock Options granted to a Greater Than 10% Stockholder, such price shall not be less than 110% of the Fair Market Value of a Share on the date the Option is granted (or the date the Option is
modified, extended or renewed for purposes of Section 424(h) of the Code). 
 6.4 Option Term. The term of
each Option (the “Option Term”) shall be set by the Administrator in its sole discretion; provided, however, that the Option Term shall not be more than 10 years from the date the Option is granted, or five years from
the date an Incentive Stock Option is granted to a Greater Than 10% Stockholder. The Administrator shall determine the time period, including the time period following a Termination of Service, during which the Holder has the right to exercise the
vested Options, which time period may not extend beyond the last day of the Option Term. Except as limited by the requirements of Section 409A or Section 422 of the Code and regulations and rulings thereunder, the Administrator may extend
the Option Term of any outstanding Option, and may extend the time period during which vested Options may be exercised, in connection with any Termination of Service of the Holder, and may amend, subject to Section 14.1, any other term or
condition of such Option relating to such a Termination of Service. 

  
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 6.5 Option Vesting. 

(a) The period during which the right to exercise, in whole or in part, an Option vests in the Holder shall be set by the
Administrator and the Administrator may determine that an Option may not be exercised in whole or in part for a specified period after it is granted. Such vesting may be based on service with the Company or any Affiliate, any of the Performance
Criteria, or any other criteria selected by the Administrator. Except as limited by the Plan, at any time after grant of an Option, the Administrator may, in its sole discretion and subject to whatever terms and conditions it selects, accelerate the
period during which an Option vests. 
 (b) No portion of an Option which is unexercisable at a Holder’s
Termination of Service shall thereafter become exercisable, except as may be otherwise provided by the Administrator either in the applicable Program, the Award Agreement evidencing the grant of an Option, or by action of the Administrator following
the grant of the Option. 
 6.6 Substitute Awards. Notwithstanding the foregoing provisions of this Article 6 to
the contrary, in the case of an Option that is a Substitute Award, the price per share of the Shares subject to such Option may be less than the Fair Market Value per share on the date of grant; provided that the excess of (a) the
aggregate Fair Market Value (as of the date such Substitute Award is granted) of the shares subject to the Substitute Award, over (b) the aggregate exercise price thereof does not exceed the excess of: (x) the aggregate fair market value
(as of the time immediately preceding the transaction giving rise to the Substitute Award, such fair market value to be determined by the Administrator) of the shares of the predecessor entity that were subject to the grant assumed or substituted
for by the Company, over (y) the aggregate exercise price of such shares. 
 6.7 Substitution of Stock Appreciation
Rights. The Administrator may provide in the applicable Program or the Award Agreement evidencing the grant of an Option that the Administrator, in its sole discretion, shall have the right to substitute a Stock Appreciation Right for such
Option at any time prior to or upon exercise of such Option; provided that such Stock Appreciation Right shall be exercisable with respect to the same number of Shares for which such substituted Option would have been exercisable, and shall
also have the same exercise price, vesting schedule and remaining Option Term as the substituted Option. 
 ARTICLE 7 

EXERCISE OF OPTIONS 
 7.1 Partial Exercise. An exercisable Option may be exercised in whole or in part. However, an Option shall not be exercisable with respect to fractional Shares and the Administrator may
require that, by the terms of the Option, a partial exercise must be with respect to a minimum number of Shares. 

  
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 7.2 Manner of Exercise. All or a portion of an exercisable Option shall be
deemed exercised upon delivery of all of the following to the Secretary of the Company, the stock administrator of the Company or such other person or entity designated by the Administrator, or his, her or its office, as applicable: 

(a) A written or electronic notice complying with the applicable rules established by the Administrator stating that the
Option, or a portion thereof, is exercised. The notice shall be signed by the Holder or other person then entitled to exercise the Option or such portion of the Option; 

(b) Such representations and documents as the Administrator, in its sole discretion, deems necessary or advisable to
effect compliance with all applicable provisions of the Securities Act and any other federal, state or foreign securities laws or regulations, the rules of any securities exchange or automated quotation system on which the Shares are listed, quoted
or traded or any other applicable law. The Administrator may, in its sole discretion, also take whatever additional actions it deems appropriate to effect such compliance including, without limitation, placing legends on share certificates and
issuing stop-transfer notices to agents and registrars; 
 (c) In the event that the Option shall be exercised
pursuant to Section 12.3 by any person or persons other than the Holder, appropriate proof of the right of such person or persons to exercise the Option, as determined in the sole discretion of the Administrator; and 

(d) Full payment of the exercise price and applicable withholding taxes to the stock administrator of the Company for the
Shares with respect to which the Option, or portion thereof, is exercised, in a manner permitted by Section 12.1 and 12.2. 

7.3 Notification Regarding Disposition. The Holder shall give the Company prompt written or electronic notice of any
disposition of Shares acquired by exercise of an Incentive Stock Option which occurs within (a) two years from the date of granting (including the date the Option is modified, extended or renewed for purposes of Section 424(h) of the Code)
such Option to such Holder, or (b) one year after the transfer of such Shares to such Holder. 
 ARTICLE 8 

AWARD OF RESTRICTED STOCK 
 8.1 Award of Restricted Stock. 
 (a) The
Administrator is authorized to grant Restricted Stock to Eligible Individuals, and shall determine the terms and conditions, including the restrictions applicable to each award of Restricted Stock, which terms and conditions shall not be
inconsistent with the Plan, and may impose such conditions on the issuance of such Restricted Stock as it deems appropriate. 
 (b) The Administrator shall establish the purchase price, if any, and form of payment for Restricted Stock; provided, however, that if a purchase price is charged, such purchase price shall
be no less than the par value, if any, of the Shares to be purchased, unless otherwise permitted by applicable law. In all cases, legal consideration shall be required for each issuance of Restricted Stock. 

  
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 8.2 Rights as Stockholders. Subject to Section 8.4, upon issuance of
Restricted Stock, the Holder shall have, unless otherwise provided by the Administrator, all the rights of a stockholder with respect to said Shares, subject to the restrictions in the applicable Program or in each individual Award Agreement,
including the right to receive all dividends and other distributions paid or made with respect to the Shares; provided, however, that, in the sole discretion of the Administrator, any extraordinary distributions with respect to the
Shares shall be subject to the restrictions set forth in Section 8.3. In addition, with respect to a share of Restricted Stock with performance-based vesting, dividends which are paid prior to vesting shall only be paid out to the Holder to the
extent that the performance-based vesting conditions are subsequently satisfied and the share of Restricted Stock vests. 
 8.3
Restrictions. All shares of Restricted Stock (including any shares received by Holders thereof with respect to shares of Restricted Stock as a result of stock dividends, stock splits or any other form of recapitalization) shall, in the
terms of the applicable Program or in each individual Award Agreement, be subject to such restrictions and vesting requirements as the Administrator shall provide. Such restrictions may include, without limitation, restrictions concerning voting
rights and transferability and such restrictions may lapse separately or in combination at such times and pursuant to such circumstances or based on such criteria as selected by the Administrator, including, without limitation, criteria based on the
Holder’s duration of employment, directorship or consultancy with the Company, the Performance Criteria, Company performance, individual performance or other criteria selected by the Administrator. By action taken after the Restricted Stock is
issued, the Administrator may, on such terms and conditions as it may determine to be appropriate, accelerate the vesting of such Restricted Stock by removing any or all of the restrictions imposed by the terms of the applicable Program or Award
Agreement. Restricted Stock may not be sold or encumbered until all restrictions are terminated or expire. 
 8.4
Repurchase or Forfeiture of Restricted Stock. Except as otherwise determined by the Administrator at the time of the grant of the Award or thereafter, if no price was paid by the Holder for the Restricted Stock, upon a Termination of
Service during the applicable restriction period, the Holder’s rights in unvested Restricted Stock then subject to restrictions shall lapse, and such Restricted Stock shall be surrendered to the Company and cancelled without consideration. If a
price was paid by the Holder for the Restricted Stock, upon a Termination of Service during the applicable restriction period, the Company shall have the right to repurchase from the Holder the unvested Restricted Stock then subject to restrictions
at a cash price per share equal to the price paid by the Holder for such Restricted Stock or such other amount as may be specified in the applicable Program or Award Agreement. Notwithstanding the foregoing, except as otherwise provided by
Section 3.4, the Administrator in its sole discretion may provide that upon certain events, including a Change in Control, the Holder’s death, retirement or disability or any other specified Termination of Service or any other event, the
Holder’s rights in unvested Restricted Stock shall not lapse, such Restricted Stock shall vest and, if applicable, the Company shall not have a right of repurchase. 

  
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 8.5 Certificates for Restricted Stock. Restricted Stock granted pursuant to
the Plan may be evidenced in such manner as the Administrator shall determine. Certificates or book entries evidencing shares of Restricted Stock shall include an appropriate legend referring to the terms, conditions, and restrictions applicable to
such Restricted Stock. The Company may, in its sole discretion, (a) retain physical possession of any stock certificate evidencing shares of Restricted Stock until the restrictions thereon shall have lapsed and/or (b) require that the
stock certificates evidencing shares of Restricted Stock be held in custody by a designated escrow agent (which may but need not be the Company) until the restrictions thereon shall have lapsed, and that the Holder deliver a stock power, endorsed in
blank, relating to such Restricted Stock. 
 8.6 Section 83(b) Election. If a Holder makes an election under
Section 83(b) of the Code to be taxed with respect to the Restricted Stock as of the date of transfer of the Restricted Stock rather than as of the date or dates upon which the Holder would otherwise be taxable under Section 83(a) of the
Code, the Holder shall be required to deliver a copy of such election to the Company promptly after filing such election with the Internal Revenue Service along with proof of the timely filing thereof with the Internal Revenue Service. 

ARTICLE 9 
 AWARD
OF RESTRICTED STOCK UNITS 
 9.1 Grant of Restricted Stock Units. The Administrator is authorized to grant Awards
of Restricted Stock Units to any Eligible Individual selected by the Administrator in such amounts and subject to such terms and conditions as determined by the Administrator. 
 9.2 Term. Except as otherwise provided herein, the term of a Restricted Stock Unit award shall be set by the Administrator in its sole discretion. 

9.3 Purchase Price. The Administrator shall specify the purchase price, if any, to be paid by the Holder to the Company
with respect to any Restricted Stock Unit award; provided, however, that value of the consideration shall not be less than the par value of a Share, unless otherwise permitted by applicable law. 

9.4 Vesting of Restricted Stock Units. At the time of grant, the Administrator shall specify the date or dates on which the
Restricted Stock Units shall become fully vested and nonforfeitable, and may specify such conditions to vesting as it deems appropriate, including, without limitation, vesting based upon the Holder’s duration of service to the Company or any
Affiliate, one or more Performance Criteria, Company performance, individual performance or other criteria, in each case on a specified date or dates or over any period or periods, as determined by the Administrator, subject to Section 3.4.

 9.5 Maturity and Payment. At the time of grant, the Administrator shall specify the maturity date applicable to
each grant of Restricted Stock Units which shall be no earlier than the vesting date or dates of the Award and may be determined at the election of the Holder (if permitted by the applicable Award Agreement); provided that, except as
otherwise determined by the Administrator, set forth in any applicable Award Agreement, and subject to compliance with Section 409A of the Code, in no event shall the maturity date relating to each Restricted

  
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Stock Unit occur following the later of (a) the 15th day of the third month following the end of calendar year in which the Restricted Stock Unit vests; or (b) the 15th day of the third
month following the end of the Company’s fiscal year in which the Restricted Stock Unit vests. On the maturity date, the Company shall, subject to Section 12.4(e), transfer to the Holder one unrestricted, fully transferable Share for each
Restricted Stock Unit scheduled to be paid out on such date and not previously forfeited, or in the sole discretion of the Administrator, an amount in cash equal to the Fair Market Value of such Shares on the maturity date or a combination of cash
and Common Stock as determined by the Administrator. 
 9.6 Payment upon Termination of Service. An Award of
Restricted Stock Units shall only be payable while the Holder is an Employee, a Consultant or a member of the Board, as applicable; provided, however, that the Administrator, in its sole and absolute discretion may provide (in an Award
Agreement or otherwise) that a Restricted Stock Unit award may be paid subsequent to a Termination of Service in certain events, including a Change in Control, the Holder’s death, retirement or disability or any other specified Termination of
Service. 
 9.7 No Rights as a Stockholder. Unless otherwise determined by the Administrator, a Holder who is
awarded Restricted Stock Units shall possess no incidents of ownership with respect to the Shares represented by such Restricted Stock Units, unless and until the same are transferred to the Holder pursuant to the terms of this Plan and the Award
Agreement. 
 9.8 Dividend Equivalents. Subject to Section 10.2, the Administrator may, in its sole
discretion, provide that Dividend Equivalents shall be earned by a Holder of Restricted Stock Units based on dividends declared on the Common Stock, to be credited as of dividend payment dates during the period between the date an Award of
Restricted Stock Units is granted to a Holder and the maturity date of such Award. 
 ARTICLE 10 

AWARD OF PERFORMANCE AWARDS, DIVIDEND EQUIVALENTS, STOCK 
 PAYMENTS, DEFERRED STOCK, DEFERRED STOCK UNITS 
 10.1 Performance
Awards. 
 (a) The Administrator is authorized to grant Performance Awards, including Awards of
Performance Stock Units, to any Eligible Individual and to determine whether such Performance Awards shall be Performance-Based Compensation. The value of Performance Awards, including Performance Stock Units, may be linked to any one or more of the
Performance Criteria or other criteria determined by the Administrator, in each case on a specified date or dates or over any period or periods determined by the Administrator. Performance Awards, including Performance Stock Unit awards may be paid
in cash, Shares, or a combination of cash and Shares, as determined by the Administrator. 
 (b) Without limiting
Section 10.1(a), the Administrator may grant Performance Awards to any Eligible Individual in the form of a cash bonus payable upon the attainment of objective Performance Goals, or such other criteria, whether or not

  
 19 

 
objective, which are established by the Administrator, in each case on a specified date or dates or over any period or periods determined by the Administrator. Any such bonuses paid to a Holder
which are intended to be Performance-Based Compensation shall be based upon objectively determinable bonus formulas established in accordance with the provisions of Article 5. 
 10.2 Dividend Equivalents. 
 (a) Dividend Equivalents
may be granted by the Administrator based on dividends declared on the Common Stock, to be credited as of dividend payment dates during the period between the date an Award is granted to a Holder and the date such Award vests, is exercised, is
distributed or expires, as determined by the Administrator. Such Dividend Equivalents shall be converted to cash or additional Shares by such formula and at such time and subject to such limitations as may be determined by the Administrator. In
addition, Dividend Equivalents with respect to an Award with performance-based vesting that are based on dividends paid prior to the vesting of such Award shall only be paid out to the Holder to the extent that the performance-based vesting
conditions are subsequently satisfied and the Award vests. 
 (b) Notwithstanding the foregoing, no Dividend
Equivalents shall be payable with respect to Options or Stock Appreciation Rights. 
 10.3 Stock Payments. The
Administrator is authorized to make Stock Payments to any Eligible Individual. The number or value of shares of any Stock Payment shall be determined by the Administrator and may be based upon one or more Performance Criteria or any other criteria,
including service to the Company or any Affiliate, determined by the Administrator. Shares underlying a Stock Payment which is subject to a vesting schedule or other conditions or criteria set by the Administrator shall not be issued until those
conditions have been satisfied. Unless otherwise provided by the Administrator, a Holder of a Stock Payment shall have no rights as a Company stockholder with respect to such Stock Payment until such time as the Stock Payment has vested and the
Shares underlying the Award have been issued to the Holder. Stock Payments may, but are not required to, be made in lieu of base salary, bonus, fees or other cash compensation otherwise payable to such Eligible Individual. 

10.4 Deferred Stock. The Administrator is authorized to grant Deferred Stock to any Eligible Individual. The number of
shares of Deferred Stock shall be determined by the Administrator and may (but is not required to) be based on one or more Performance Criteria or other criteria, including service to the Company or any Affiliate, as the Administrator determines, in
each case on a specified date or dates or over any period or periods determined by the Administrator. Shares underlying a Deferred Stock award which is subject to a vesting schedule or other conditions or criteria set by the Administrator will be
issued on the vesting date(s) or date(s) that those conditions and criteria have been satisfied, as applicable. Unless otherwise provided by the Administrator, a Holder of Deferred Stock shall have no rights as a Company stockholder with respect to
such Deferred Stock until such time as the Award has vested and any other applicable conditions and/or criteria have been satisfied and the Shares underlying the Award have been issued to the Holder. 

  
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 10.5 Deferred Stock Units. The Administrator is authorized to grant Deferred
Stock Units to any Eligible Individual. The number of Deferred Stock Units shall be determined by the Administrator and may (but is not required to) be based on one or more Performance Criteria or other criteria, including service to the Company or
any Affiliate, as the Administrator determines, in each case on a specified date or dates or over any period or periods determined by the Administrator. Each Deferred Stock Unit shall entitle the Holder thereof to receive one Share on the date the
Deferred Stock Unit becomes vested or upon a specified settlement date thereafter (which settlement date may (but is not required to) be the date of the Holder’s Termination of Service). Shares underlying a Deferred Stock Unit award which is
subject to a vesting schedule or other conditions or criteria set by the Administrator will not be issued until on or following the date that those conditions and criteria have been satisfied. Unless otherwise provided by the Administrator, a Holder
of Deferred Stock Units shall have no rights as a Company stockholder with respect to such Deferred Stock Units until such time as the Award has vested and any other applicable conditions and/or criteria have been satisfied and the Shares underlying
the Award have been issued to the Holder. 
 10.6 Term. The term of a Performance Award, Dividend Equivalent
award, Stock Payment award, Deferred Stock award and/or Deferred Stock Unit award shall be established by the Administrator in its sole discretion. 
 10.7 Purchase Price. The Administrator may establish the purchase price of a Performance Award, Shares distributed as a Stock Payment award, Shares of Deferred Stock or Shares distributed
pursuant to a Deferred Stock Unit award; provided, however, that value of the consideration shall not be less than the par value of a Share, unless otherwise permitted by applicable law. 

10.8 Termination of Service. A Performance Award, Stock Payment award, Dividend Equivalent award, Deferred Stock award
and/or Deferred Stock Unit award is distributable only while the Holder is an Employee, Director or Consultant, as applicable. The Administrator, however, in its sole discretion may provide that the Performance Award, Dividend Equivalent award,
Stock Payment award, Deferred Stock award and/or Deferred Stock Unit award may be distributed subsequent to a Termination of Service in certain events, including a Change in Control, the Holder’s death, retirement or disability or any other
specified Termination of Service. 
 ARTICLE 11 
 AWARD OF STOCK APPRECIATION RIGHTS 
 11.1 Grant of Stock Appreciation
Rights. 
 (a) The Administrator is authorized to grant Stock Appreciation Rights to Eligible Individuals
from time to time, in its sole discretion, on such terms and conditions as it may determine which shall not be inconsistent with the Plan. 
 (b) A Stock Appreciation Right shall entitle the Holder (or other person entitled to exercise the Stock Appreciation Right pursuant to the Plan) to exercise all or a

  
 21 

 
specified portion of the Stock Appreciation Right (to the extent then exercisable pursuant to its terms) and to receive from the Company an amount determined by multiplying the difference
obtained by subtracting the exercise price per share of the Stock Appreciation Right from the fair market value at the time of exercise of the Stock Appreciation Right by the number of Shares with respect to which the Stock Appreciation Right shall
have been exercised, subject to any limitations the Administrator may impose. Except as described in (c) below, the exercise price per Share subject to each Stock Appreciation Right shall be set by the Administrator, but shall not be less than
100% of the Fair Market Value on the date the Stock Appreciation Right is granted. 
 (c) Notwithstanding the
foregoing provisions of Section 11.1(b) to the contrary, in the case of an Stock Appreciation Right that is a Substitute Award, the price per share of the Shares subject to such Stock Appreciation Right may be less than the Fair Market Value
per share on the date of grant; provided that the excess of: (i) the aggregate Fair Market Value (as of the date such Substitute Award is granted) of the shares subject to the Substitute Award, over (ii) the aggregate exercise price
thereof does not exceed the excess of: (x) the aggregate fair market value (as of the time immediately preceding the transaction giving rise to the Substitute Award, such fair market value to be determined by the Administrator) of the shares of
the predecessor entity that were subject to the grant assumed or substituted for by the Company, over (y) the aggregate exercise price of such shares. 
 11.2 Stock Appreciation Right Vesting. 
 (a) The
period during which the right to exercise, in whole or in part, a Stock Appreciation Right vests in the Holder shall be set by the Administrator and the Administrator may determine that a Stock Appreciation Right may not be exercised in whole or in
part for a specified period after it is granted. Such vesting may be based on service with the Company or any Affiliate, any of the Performance Criteria, or any other criteria selected by the Administrator. Except as limited by the Plan, at any time
after grant of a Stock Appreciation Right, the Administrator may, in its sole discretion and subject to whatever terms and conditions it selects, accelerate the period during which a Stock Appreciation Right vests. 

(b) No portion of a Stock Appreciation Right which is unexercisable at a Holder’s Termination of Service shall
thereafter become exercisable, except as may be otherwise provided by the Administrator either in the applicable Program, the Award Agreement evidencing the grant of a Stock Appreciation Right, or by action of the Administrator following the grant
of the Stock Appreciation Right. 
 11.3 Manner of Exercise. All or a portion of an exercisable Stock Appreciation
Right shall be deemed exercised upon delivery of all of the following to the Secretary of the Company, the stock plan administrator of the Company, or such other person or entity designated by the Administrator, or his, her or its office, as
applicable: 
 (a) A written or electronic notice complying with the applicable rules established by the
Administrator stating that the Stock Appreciation Right, or a portion 

  
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thereof, is exercised. The notice shall be signed by the Holder or other person then entitled to exercise the Stock Appreciation Right or such portion of the Stock Appreciation Right; 

(b) Such representations and documents as the Administrator, in its sole discretion, deems necessary or advisable to
effect compliance with all applicable provisions of the Securities Act and any other federal, state or foreign securities laws or regulations, the rules of any securities exchange or automated quotation system on which the Shares are listed, quoted
or traded or any other applicable law. The Administrator may, in its sole discretion, also take whatever additional actions it deems appropriate to effect such compliance including, without limitation, placing legends on share certificates and
issuing stop-transfer notices to agents and registrars; 
 (c) In the event that the Stock Appreciation Right
shall be exercised pursuant to this Section 11.3 by any person or persons other than the Holder, appropriate proof of the right of such person or persons to exercise the Stock Appreciation Right, as determined in the sole discretion of the
Administrator; and 
 (d) Full payment of the exercise price and applicable withholding taxes to the stock
administrator of the Company for the Shares with respect to such the Stock Appreciation Rights, or portion thereof, is exercised, in a manner permitted by Section 12.1 and 12.2. 

11.4 Stock Appreciation Right Term. The term of each Stock Appreciation Right (the “Stock Appreciation Right
Term”) shall be set by the Administrator in its sole discretion; provided, however, that the Stock Appreciation Right Term shall not be more than 10 years from the date the Stock Appreciation Right is granted. The
Administrator shall determine the time period, including the time period following a Termination of Service, during which the Holder has the right to exercise the vested Stock Appreciation Rights, which time period may not extend beyond the last day
of the Stock Appreciation Right Term. Except as limited by the requirements of Section 409A of the Code and regulations and rulings thereunder, the Administrator may extend the Stock Appreciation Right Term of any outstanding Stock Appreciation
Right, and may extend the time period during which vested Stock Appreciation Rights may be exercised, in connection with any Termination of Service of the Holder, and may amend, subject to Section 14.1, any other term or condition of such Stock
Appreciation Right relating to such a Termination of Service. 
 11.5 Payment. Payment of the amounts payable with
respect to Stock Appreciation Rights pursuant to this Article 11 shall be in cash, Shares (based on its fair market value as of the time the Stock Appreciation Right is exercised), or a combination of both, as determined by the Administrator.

  
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 ARTICLE 12 
 ADDITIONAL TERMS OF AWARDS 
 12.1 Payment. The Administrator shall
determine the methods by which payments by any Holder with respect to any Awards granted under the Plan shall be made, including, without limitation: (a) cash or check, (b) Shares (including, in the case of payment of the exercise price of
an Award, Shares issuable pursuant to the exercise of the Award) or Shares held for such period of time as may be required by the Administrator in order to avoid adverse accounting consequences, in each case, having a fair market value at the time
of delivery equal to the aggregate payments required, (c) delivery of a written or electronic notice that the Holder has placed a market sell order with a broker with respect to Shares then issuable upon exercise or vesting of an Award, and
that the broker has been directed to pay a sufficient portion of the net proceeds of the sale to the Company in satisfaction of the aggregate payments required; provided that payment of such proceeds is then made to the Company upon
settlement of such sale, or (d) other form of legal consideration acceptable to the Administrator in its sole discretion. The Administrator shall also determine the methods by which Shares shall be delivered or deemed to be delivered to
Holders. Notwithstanding any other provision of the Plan to the contrary, no Holder who is a Director or an “executive officer” of the Company within the meaning of Section 13(k) of the Exchange Act shall be permitted to make payment
with respect to any Awards granted under the Plan, or continue any extension of credit with respect to such payment, with a loan from the Company or a loan arranged by the Company in violation of Section 13 (k) of the Exchange Act.

 12.2 Tax Withholding. The Company or any Affiliate shall have the authority and the right to deduct or
withhold, or require a Holder to remit to the Company, an amount sufficient to satisfy federal, state, local and foreign taxes (including the Holder’s FICA, employment tax or other social security contribution obligation) required by law to be
withheld with respect to any taxable event concerning a Holder arising as a result of the Plan. The Administrator may in its sole discretion and in satisfaction of the foregoing requirement allow a Holder to elect to have the Company withhold Shares
otherwise issuable under an Award (or allow the surrender of Shares). The number of Shares which may be so withheld or surrendered shall be limited to the number of Shares which have a fair market value at the time of withholding or repurchase equal
to the aggregate amount of such liabilities based on the minimum statutory withholding rates for federal, state, local and foreign income tax and payroll tax purposes that are applicable to such supplemental taxable income. The Administrator shall
determine the fair market value of the Shares, consistent with applicable provisions of the Code, for tax withholding obligations due in connection with a broker-assisted cashless Option or Stock Appreciation Right exercise involving the sale of
Shares to pay the Option or Stock Appreciation Right exercise price or any tax withholding obligation. 
 12.3
Transferability of Awards. 
 (a) Except as otherwise provided in Section 12.3(b): 

(i) No Award under the Plan may be sold, pledged, assigned or transferred in any manner other than by will or the laws of
descent and 

  
 24 

 
distribution or, subject to the consent of the Administrator, pursuant to a DRO, unless and until such Award has been exercised, or the Shares underlying such Award have been issued, and all
restrictions applicable to such Shares have lapsed; 
 (ii) No Award or interest or right therein shall be liable
for the debts, contracts or engagements of the Holder or his successors in interest or shall be subject to disposition by transfer, alienation, anticipation, pledge, hypothecation, encumbrance, assignment or any other means whether such disposition
be voluntary or involuntary or by operation of law by judgment, levy, attachment, garnishment or any other legal or equitable proceedings (including bankruptcy), and any attempted disposition thereof shall be null and void and of no effect, except
to the extent that such disposition is permitted by the preceding sentence; and 
 (iii) During the lifetime of
the Holder, only the Holder may exercise an Award (or any portion thereof) granted to him under the Plan, unless it has been disposed of pursuant to a DRO; after the death of the Holder, any exercisable portion of an Award may, prior to the time
when such portion becomes unexercisable under the Plan or the applicable Program or Award Agreement, be exercised by his personal representative or by any person empowered to do so under the deceased Holder’s will or under the then applicable
laws of descent and distribution. 
 (b) Notwithstanding Section 12.3(a), the Administrator, in its sole
discretion, may determine to permit a Holder to transfer an Award other than an Incentive Stock Option to any one or more Permitted Transferees, subject to the following terms and conditions: (i) an Award transferred to a Permitted Transferee
shall not be assignable or transferable by the Permitted Transferee other than by will or the laws of descent and distribution; (ii) an Award transferred to a Permitted Transferee shall continue to be subject to all the terms and conditions of
the Award as applicable to the original Holder (other than the ability to further transfer the Award); (iii) any transfer of a Non-Qualified Stock Option to a Permitted Transferee shall be without consideration, except as required by applicable
law and (iv) the Holder and the Permitted Transferee shall execute any and all documents requested by the Administrator, including, without limitation documents to (A) confirm the status of the transferee as a Permitted Transferee,
(B) satisfy any requirements for an exemption for the transfer under applicable federal, state and foreign securities laws and (C) evidence the transfer. 

(c) Notwithstanding Section 12.3(a), a Holder may, in the manner determined by the Administrator, designate a
beneficiary to exercise the rights of the Holder and to receive any distribution with respect to any Award upon the Holder’s death. A beneficiary, legal guardian, legal representative, or other person claiming any rights pursuant to the Plan is
subject to all terms and conditions of the Plan and any Program or Award Agreement applicable to the Holder, except to the extent the Plan, the Program and the Award Agreement otherwise provide, and to any additional restrictions deemed necessary or
appropriate by the Administrator. If the Holder is married and resides in a 

  
 25 

 
community property state, a designation of a person other than the Holder’s spouse as his or her beneficiary with respect to more than 50% of the Holder’s interest in the Award shall
not be effective without the prior written or electronic consent of the Holder’s spouse. If no beneficiary has been designated or survives the Holder, payment shall be made to the person entitled thereto pursuant to the Holder’s will or
the laws of descent and distribution. Subject to the foregoing, a beneficiary designation may be changed or revoked by a Holder at any time; provided that the change or revocation is filed with the Administrator prior to the Holder’s
death. 
 12.4 Conditions to Issuance of Shares. 

(a) Notwithstanding anything herein to the contrary, the Company shall not be required to issue or deliver any
certificates or make any book entries evidencing Shares pursuant to the exercise of any Award, unless and until the Board or the Committee has determined, with advice of counsel, that the issuance of such Shares is in compliance with all applicable
laws, regulations of governmental authorities and, if applicable, the requirements of any exchange on which the Shares are listed or traded, and the Shares are covered by an effective registration statement or applicable exemption from registration.
In addition to the terms and conditions provided herein, the Board or the Committee may require that a Holder make such reasonable covenants, agreements, and representations as the Board or the Committee, in its discretion, deems advisable in order
to comply with any such laws, regulations, or requirements. 
 (b) All Share certificates delivered pursuant to
the Plan and all Shares issued pursuant to book entry procedures are subject to any stop-transfer orders and other restrictions as the Administrator deems necessary or advisable to comply with federal, state, or foreign securities or other laws,
rules and regulations and the rules of any securities exchange or automated quotation system on which the Shares are listed, quoted, or traded. The Administrator may place legends on any Share certificate or book entry to reference restrictions
applicable to the Shares. 
 (c) The Administrator shall have the right to require any Holder to comply with any
timing or other restrictions with respect to the settlement, distribution or exercise of any Award, including a window-period limitation, as may be imposed in the sole discretion of the Administrator. 

(d) No fractional Shares shall be issued and the Administrator shall determine, in its sole discretion, whether cash shall
be given in lieu of fractional Shares or whether such fractional Shares shall be eliminated by rounding down. 

(e) Notwithstanding any other provision of the Plan, unless otherwise determined by the Administrator or required by any
applicable law, rule or regulation, the Company shall not deliver to any Holder certificates evidencing Shares issued in connection with any Award and instead such Shares shall be recorded in the books of the Company (or, as applicable, its transfer
agent or stock plan administrator). 

  
 26 

 12.5 Forfeiture and Claw-Back Provisions. Pursuant to its general authority to
determine the terms and conditions applicable to Awards under the Plan, the Administrator shall have the right to provide, in an Award Agreement or otherwise, or to require a Holder to agree by separate written or electronic instrument, that:

 (a) (i) Any proceeds, gains or other economic benefit actually or constructively received by the Holder upon
any receipt or exercise of the Award, or upon the receipt or resale of any Shares underlying the Award, must be paid to the Company, and (ii) the Award shall terminate and any unexercised portion of the Award (whether or not vested) shall be
forfeited, if (x) a Termination of Service occurs prior to a specified date, or within a specified time period following receipt or exercise of the Award, or (y) the Holder at any time, or during a specified time period, engages in any
activity in competition with the Company, or which is inimical, contrary or harmful to the interests of the Company, as further defined by the Administrator or (z) the Holder incurs a Termination of Service for “cause” (as such term
is defined in the sole discretion of the Administrator, or as set forth in a written agreement relating to such Award between the Company and the Holder); and 
 (b) All Awards (including any proceeds, gains or other economic benefit actually or constructively received by the Holder upon any receipt or exercise of any Award or upon the receipt or resale of any
Shares underlying the Award) shall be subject to the provisions of any claw-back obligation imposed by applicable law or any policy implemented by the Company, including, without limitation, any claw-back policy adopted to comply with the
requirements of the Dodd-Frank Wall Street Reform and Consumer Protection Act and any rules or regulations promulgated thereunder, to the extent set forth in such claw-back policy and/or in the applicable Award Agreement. 

12.6 Prohibition on Repricing. Subject to Section 14.2, the Administrator shall not, without the approval of the
stockholders of the Company, (i) authorize the amendment of any outstanding Option or Stock Appreciation Right to reduce its price per share, or (ii) cancel any Option or Stock Appreciation Right in exchange for cash or another Award when
the Option or Stock Appreciation Right price per share exceeds the Fair Market Value of the underlying Shares. Subject to Section 14.2, the Administrator shall have the authority, without the approval of the stockholders of the Company, to
amend any outstanding Award to increase the price per share or to cancel and replace an Award with the grant of an Award having a price per share that is greater than or equal to the price per share of the original Award. 

ARTICLE 13 

ADMINISTRATION 

13.1 Administrator. The Committee (or another committee or a subcommittee of the Board assuming the functions of the
Committee under the Plan) shall administer the Plan (except as otherwise permitted herein) and, unless otherwise determined by the Board, shall consist solely of two or more Non-Employee Directors appointed by and holding office at the pleasure of
the Board, each of whom is intended to qualify as both a “non-employee director” as defined by Rule 16b-3 of the Exchange Act or any successor rule, an “outside director” for purposes of

  
 27 

 
Section 162(m) of the Code and an “independent director” under the rules of any securities exchange or automated quotation system on which the Shares are listed, quoted or traded;
provided that any action taken by the Committee shall be valid and effective, whether or not members of the Committee at the time of such action are later determined not to have satisfied the requirements for membership set forth in this
Section 13.1 or otherwise provided in any charter of the Committee. Except as may otherwise be provided in any charter of the Committee, appointment of Committee members shall be effective upon acceptance of appointment. Committee members may
resign at any time by delivering written or electronic notice to the Board. Vacancies in the Committee may only be filled by the Board. Notwithstanding the foregoing, (a) the full Board, acting by a majority of its members in office, shall
conduct the general administration of the Plan with respect to Awards granted to Non-Employee Directors and, with respect to such Awards, the terms “Administrator” and “Committee” as used in the Plan shall be deemed to refer to
the Board and (b) the Board or Committee may delegate its authority hereunder to the extent permitted by Section 13.6. 
 13.2 Duties and Powers of Committee. It shall be the duty of the Committee to conduct the general administration of the Plan in accordance with its provisions. The Committee shall have the
power to interpret the Plan, the Program and the Award Agreement, and to adopt such rules for the administration, interpretation and application of the Plan as are not inconsistent therewith, to interpret, amend or revoke any such rules and to amend
any Program or Award Agreement; provided that the rights or obligations of the Holder of the Award that is the subject of any such Program or Award Agreement are not affected adversely by such amendment, unless the consent of the Holder is
obtained or such amendment is otherwise permitted under Section 14.10. Any such grant or award under the Plan need not be the same with respect to each Holder. Any such interpretations and rules with respect to Incentive Stock Options shall be
consistent with the provisions of Section 422 of the Code. In its sole discretion, the Board may at any time and from time to time exercise any and all rights and duties of the Committee under the Plan except with respect to matters which under
Rule 16b-3 under the Exchange Act or any successor rule, or Section 162(m) of the Code, or any regulations or rules issued thereunder, or the rules of any securities exchange or automated quotation system on which the Shares are listed, quoted
or traded are required to be determined in the sole discretion of the Committee. 
 13.3 Action by the Committee.
Unless otherwise established by the Board or in any charter of the Committee, a majority of the Committee shall constitute a quorum and the acts of a majority of the members present at any meeting at which a quorum is present, and acts approved in
writing by all members of the Committee in lieu of a meeting, shall be deemed the acts of the Committee. Each member of the Committee is entitled to, in good faith, rely or act upon any report or other information furnished to that member by any
officer or other employee of the Company or any Affiliate, the Company’s independent certified public accountants, or any executive compensation consultant or other professional retained by the Company to assist in the administration of the
Plan. 
 13.4 Authority of Administrator. Subject to the Company’s Bylaws, the Committee’s Charter and
any specific designation in the Plan, the Administrator has the exclusive power, authority and sole discretion to: 
 (a) Designate Eligible Individuals to receive Awards; 

  
 28 

 (b) Determine the type or types of Awards to be granted to each Eligible
Individual; 
 (c) Determine the number of Awards to be granted and the number of Shares to which an Award will
relate; 
 (d) Determine the terms and conditions of any Award granted pursuant to the Plan, including, but not
limited to, the exercise price, grant price, or purchase price, any performance criteria, any restrictions or limitations on the Award, any schedule for vesting, lapse of forfeiture restrictions or restrictions on the exercisability of an Award, and
accelerations or waivers thereof, and any provisions related to non-competition and recapture of gain on an Award, based in each case on such considerations as the Administrator in its sole discretion determines; 

(e) Determine whether, to what extent, and pursuant to what circumstances an Award may be settled in, or the exercise
price of an Award may be paid in cash, Shares, other Awards, or other property, or an Award may be canceled, forfeited, or surrendered; 
 (f) Prescribe the form of each Award Agreement, which need not be identical for each Holder; 
 (g) Decide all other matters that must be determined in connection with an Award; 
 (h) Establish, adopt, or revise any rules and regulations as it may deem necessary or advisable to administer the Plan; 

(i) Interpret the terms of, and any matter arising pursuant to, the Plan, any Program or any Award Agreement; 

(j) Make all other decisions and determinations that may be required pursuant to the Plan or as the Administrator deems
necessary or advisable to administer the Plan; and 
 (k) Accelerate wholly or partially the vesting or lapse of
restrictions of any Award or portion thereof at any time after the grant of an Award, subject to whatever terms and conditions it selects and Sections 3.4 and 14.2. 
 13.5 Decisions Binding. The Administrator’s interpretation of the Plan, any Awards granted pursuant to the Plan, any Program, any Award Agreement and all decisions and determinations by
the Administrator with respect to the Plan are final, binding, and conclusive on all parties. 
 13.6 Delegation of
Authority. To the extent permitted by applicable law or the rules of any securities exchange or automated quotation system on which the Shares are listed, quoted or traded, the Board or Committee may from time to time delegate to a committee
of one or more members of the Board or one or more officers of the Company the authority to grant or amend Awards or to take other administrative actions pursuant to Article 13; provided, however, that in

  
 29 

 
no event shall an officer of the Company be delegated the authority to grant awards to, or amend awards held by, the following individuals: (a) individuals who are subject to Section 16
of the Exchange Act, (b) Covered Employees, or (c) officers of the Company (or Directors) to whom authority to grant or amend Awards has been delegated hereunder; provided, further, that any delegation of administrative
authority shall only be permitted to the extent it is permissible under Section 162(m) of the Code and applicable securities laws or the rules of any securities exchange or automated quotation system on which the Shares are listed, quoted or
traded. Any delegation hereunder shall be subject to the restrictions and limits that the Board or Committee specifies at the time of such delegation, and the Board may at any time rescind the authority so delegated or appoint a new delegatee. At
all times, the delegatee appointed under this Section 13.6 shall serve in such capacity at the pleasure of the Board and the Committee. 
 ARTICLE 14 
 MISCELLANEOUS PROVISIONS 

14.1 Amendment, Suspension or Termination of the Plan. Except as otherwise provided in this Section 14.1, the Plan may
be wholly or partially amended or otherwise modified, suspended or terminated at any time or from time to time by the Board or the Committee. However, without approval of the Company’s stockholders given within twelve (12) months before or
after the action by the Administrator, no action of the Administrator may, except as provided in Section 14.2, (a) increase the limits imposed in Section 3.1 on the maximum number of Shares which may be issued under the Plan, or
(b) reduce the price per Share of any outstanding Option or Stock Appreciation Right granted under the Plan or take any action prohibited under Section 12.6, or (c) cancel any Option or Stock Appreciation Right in exchange for cash or
another Award when the Option or Stock Appreciation Right price per Share exceeds the Fair Market Value of the underlying Shares. Except as provided in Sections 12.5 and 14.10, no amendment, suspension or termination of the Plan shall, without the
consent of the Holder, impair any rights or obligations under any Award theretofore granted or awarded, unless the Award itself otherwise expressly so provides. No Awards may be granted or awarded during any period of suspension or after termination
of the Plan, and in no event may any Award be granted under the Plan after the tenth anniversary of the date the Plan was approved by the Board. 
 14.2 Changes in Common Stock or Assets of the Company Acquisition or Liquidation of the Company and Other Corporate Events. 

(a) In the event of any stock dividend, stock split, combination or exchange of shares, merger, consolidation or other
distribution (other than normal cash dividends) of Company assets to stockholders, or any other change affecting the shares of the Company’s stock or the share price of the Company’s stock other than an Equity Restructuring, the
Administrator shall make equitable adjustments, if any, to reflect such change with respect to (i) the aggregate number and kind of shares that may be issued under the Plan (including, but not limited to, adjustments of the limitations in
Section 3.1 on the maximum number and kind of shares which may be issued under the Plan and adjustments of the Award Limit); (ii) the number and kind of Shares (or other securities or property) subject to outstanding Awards; (iii) the
number and kind of Shares (or other 

  
 30 

 
securities or property) for which automatic grants are subsequently to be made to new and continuing Non-Employee Directors pursuant to Section 4.6 and the Non-Employee Director Equity
Compensation Policy; (iv) the terms and conditions of any outstanding Awards (including, without limitation, any applicable performance targets or criteria with respect thereto); and (v) the grant or exercise price per Share for any
outstanding Awards under the Plan. Any adjustment affecting an Award intended as Performance-Based Compensation shall be made consistent with the requirements of Section 162(m) of the Code. 

(b) In the event of any transaction or event described in Section 14.2(a) or any unusual or nonrecurring transactions
or events affecting the Company, any Affiliate of the Company, or the financial statements of the Company or any Affiliate, or of changes in applicable laws, regulations or accounting principles, the Administrator, in its sole discretion, and on
such terms and conditions as it deems appropriate, either by the terms of the Award or by action taken prior to the occurrence of such transaction or event and either automatically or upon the Holder’s request, is hereby authorized to take any
one or more of the following actions whenever the Administrator determines that such action is appropriate in order to prevent dilution or enlargement of the benefits or potential benefits intended to be made available under the Plan or with respect
to any Award under the Plan, to facilitate such transactions or events or to give effect to such changes in laws, regulations or principles: 
 (i) To provide for either (A) termination of any such Award in exchange for an amount of cash, if any, equal to the amount that would have been attained upon the exercise of such Award or realization
of the Holder’s rights (and, for the avoidance of doubt, if as of the date of the occurrence of the transaction or event described in this Section 14.2 the Administrator determines in good faith that no amount would have been attained upon
the exercise of such Award or realization of the Holder’s rights, then such Award may be terminated by the Company without payment) or (B) the replacement of such Award with other rights or property selected by the Administrator in its
sole discretion having an aggregate value not exceeding the amount that could have been attained upon the exercise of such Award or realization of the Holder’s rights had such Award been currently exercisable or payable or fully vested;

 (ii) To provide that such Award be assumed by the successor or survivor corporation, or a parent or subsidiary
thereof, or shall be substituted for by similar options, rights or awards covering the stock of the successor or survivor corporation, or a parent or subsidiary thereof, with appropriate adjustments as to the number and kind of shares and prices;

 (iii) To make adjustments in the number and type of shares of the Company’s stock (or other securities or
property) subject to outstanding Awards, and in the number and kind of outstanding Restricted Stock or Deferred Stock and/or in the terms and conditions of (including the grant or exercise price), and the criteria included in, outstanding Awards and
Awards which may be granted in the future; 

  
 31 

 (iv) To provide that such Award shall be exercisable or payable or fully
vested with respect to all Shares covered thereby, notwithstanding anything to the contrary in the Plan or the applicable Program or Award Agreement; and 
 (v) To provide that the Award cannot vest, be exercised or become payable after such event. 
 (c) In connection with the occurrence of any Equity Restructuring, and notwithstanding anything to the contrary in Sections 14.2(a) and 14.2(b): 

(i) The number and type of securities subject to each outstanding Award and the exercise price or grant price thereof, if
applicable, shall be equitably adjusted; and/or 
 (ii) The Administrator shall make such equitable adjustments,
if any, as the Administrator in its sole discretion may deem appropriate to reflect such Equity Restructuring with respect to the aggregate number and kind of shares that may be issued under the Plan (including, but not limited to, adjustments of
the limitations in Section 3.1 on the maximum number and kind of shares which may be issued under the Plan and adjustments of the Award Limit). The adjustments provided under this Section 14.2(c) shall be nondiscretionary and shall be
final and binding on the affected Holder and the Company. 
 (d) In the event of a Change in Control, or if the
Company is otherwise a party to a consolidation or a merger in which the Company is not the surviving corporation, a transaction that results in the acquisition of substantially all of the Company’s outstanding stock by a single person or
entity, or a sale or transfer of substantially all of the Company’s assets occurs (in any such case, a “Corporate Event”), then the Committee (or, with respect to an Award granted to a Non-Employee Director, the Board) may take
any actions with respect to outstanding Awards as it deems appropriate, consistent with applicable provisions of the Code and any applicable federal or state securities laws. 

(e) Notwithstanding anything in the Plan to the contrary, the Committee (or, with respect to an Award granted to a
Non-Employee Director, the Board) may take the foregoing actions without the consent of any Participant, and its determination shall be conclusive and binding on all persons and for all purposes. 

(f) With respect to Awards which are granted to Covered Employees and are intended to qualify as Performance-Based
Compensation, no adjustment or action described in this Section 14.2 or in any other provision of the Plan shall be authorized to the extent that such adjustment or action would cause such Award to fail to so qualify as Performance-Based
Compensation, unless the Administrator determines that the Award should not so qualify. No adjustment or action described in this Section 14.2 or in any other provision of the Plan shall be authorized to the extent that such adjustment or
action would cause the Plan to violate Section 422(b)(1) of the Code. Furthermore, no such adjustment or action shall be authorized to the extent such adjustment or action would 

  
 32 

 
result in short-swing profits liability under Section 16 or violate the exemptive conditions of Rule 16b-3 unless the Administrator determines that the Award is not to comply with such
exemptive conditions. 
 (g) The existence of the Plan, the Program, the Award Agreement and the Awards granted
hereunder shall not affect or restrict in any way the right or power of the Company or the stockholders of the Company to make or authorize any adjustment, recapitalization, reorganization or other change in the Company’s capital structure or
its business, any merger or consolidation of the Company, any issue of stock or of options, warrants or rights to purchase stock or of bonds, debentures, preferred or prior preference stocks whose rights are superior to or affect the Common Stock or
the rights thereof or which are convertible into or exchangeable for Common Stock, or the dissolution or liquidation of the Company, or any sale or transfer of all or any part of its assets or business, or any other corporate act or proceeding,
whether of a similar character or otherwise. 
 (h) No action shall be taken under this Section 14.2 which
shall cause an Award to fail to be exempt from or comply with Section 409A of the Code or the Treasury Regulations thereunder. 
 (i) In the event of any pending stock dividend, stock split, combination or exchange of shares, merger, consolidation or other distribution (other than normal cash dividends) of Company assets to
stockholders, or any other change affecting the shares of Common Stock or the share price of the Common Stock including any Equity Restructuring, for reasons of administrative convenience, the Administrator in its sole discretion may refuse to
permit the exercise of any Award during a period of up to 30 days prior to the consummation of any such transaction. 
 14.3
No Stockholder Rights. Except as otherwise provided herein, a Holder shall have none of the rights of a stockholder with respect to Shares covered by any Award until the Holder becomes the record owner of such Shares. 

14.4 Paperless Administration. In the event that the Company establishes, for itself or using the services of a third
party, an automated system for the documentation, granting or exercise of Awards, such as a system using an internet website or interactive voice response, then the paperless documentation, granting or exercise of Awards by a Holder may be permitted
through the use of such an automated system. 
 14.5 Effect of Plan upon Other Compensation Plans. The adoption of
the Plan shall not affect any other compensation or incentive plans in effect for the Company or any Affiliate. Nothing in the Plan shall be construed to limit the right of the Company or any Affiliate: (a) to establish any other forms of
incentives or compensation for Employees, Directors or Consultants of the Company or any Affiliate, or (b) to grant or assume options or other rights or awards otherwise than under the Plan in connection with any proper corporate purpose
including without limitation, the grant or assumption of options in connection with the acquisition by purchase, lease, merger, consolidation or otherwise, of the business, stock or assets of any corporation, partnership, limited liability company,
firm or association. 

  
 33 

 14.6 Compliance with Laws. The Plan, the granting and vesting of Awards under
the Plan and the issuance and delivery of Shares and the payment of money under the Plan or under Awards granted or awarded hereunder are subject to compliance with all applicable federal, state, local and foreign laws, rules and regulations
(including but not limited to state, federal and foreign securities law and margin requirements), the rules of any securities exchange or automated quotation system on which the Shares are listed, quoted or traded, and to such approvals by any
listing, regulatory or governmental authority as may, in the opinion of counsel for the Company, be necessary or advisable in connection therewith. Any securities delivered under the Plan shall be subject to such restrictions, and the person
acquiring such securities shall, if requested by the Company, provide such assurances and representations to the Company as the Company may deem necessary or desirable to assure compliance with all applicable legal requirements. To the extent
permitted by applicable law, the Plan and Awards granted or awarded hereunder shall be deemed amended to the extent necessary to conform to such laws, rules and regulations. 
 14.7 Titles and Headings, References to Sections of the Code or Exchange Act. The titles and headings of the Sections in the Plan are for convenience of reference only and, in the event of
any conflict, the text of the Plan, rather than such titles or headings, shall control. References to sections of the Code or the Exchange Act shall include any amendment or successor thereto. 

14.8 Governing Law. The Plan and any agreements hereunder shall be administered, interpreted and enforced under the
internal laws of the State of Delaware without regard to conflicts of laws thereof or of any other jurisdiction. 
 14.9
Section 409A. To the extent that the Administrator determines that any Award granted under the Plan is subject to Section 409A of the Code, the Program pursuant to which such Award is granted and the Award Agreement
evidencing such Award shall incorporate the terms and conditions required by Section 409A of the Code. To the extent applicable, the Plan, the Program and any Award Agreements shall be interpreted in accordance with Section 409A of the
Code and Department of Treasury regulations and other interpretive guidance issued thereunder, including without limitation any such regulations or other guidance that may be issued after the Effective Date. Notwithstanding any provision of the Plan
to the contrary, in the event that following the Effective Date the Administrator determines that any Award may be subject to Section 409A of the Code and related Department of Treasury guidance (including such Department of Treasury guidance
as may be issued after the Effective Date), the Administrator may adopt such amendments to the Plan and the applicable Program and Award Agreement or adopt other policies and procedures (including amendments, policies and procedures with retroactive
effect), or take any other actions, that the Administrator determines are necessary or appropriate to (a) exempt the Award from Section 409A of the Code and/or preserve the intended tax treatment of the benefits provided with respect to
the Award, or (b) comply with the requirements of Section 409A of the Code and related Department of Treasury guidance and thereby avoid the application of any penalty taxes under such Section. 

14.10 No Rights to Awards. No Eligible Individual or other person shall have any claim to be granted any Award pursuant to
the Plan, and neither the Company nor the Administrator is obligated to treat Eligible Individuals, Holders or any other persons uniformly. 

  
 34 

 14.11 Unfunded Status of Awards. The Plan is intended to be an
“unfunded” plan for incentive compensation. With respect to any payments not yet made to a Holder pursuant to an Award, nothing contained in the Plan or any Program or Award Agreement shall give the Holder any rights that are greater than
those of a general creditor of the Company or any Affiliate. 
 14.12 Indemnification. To the extent allowable
pursuant to applicable law, each member of the Committee or of the Board shall be indemnified and held harmless by the Company from any loss, cost, liability, or expense that may be imposed upon or reasonably incurred by such member in connection
with or resulting from any claim, action, suit, or proceeding to which he or she may be a party or in which he or she may be involved by reason of any action or failure to act pursuant to the Plan and against and from any and all amounts paid by him
or her in satisfaction of judgment in such action, suit, or proceeding against him or her; provided he or she gives the Company an opportunity, at its own expense, to handle and defend the same before he or she undertakes to handle and defend
it on his or her own behalf. The foregoing right of indemnification shall not be exclusive of any other rights of indemnification to which such persons may be entitled pursuant to the Company’s Certificate of Incorporation or Bylaws, as a
matter of law, or otherwise, or any power that the Company may have to indemnify them or hold them harmless. 
 14.13
Relationship to other Benefits. No payment pursuant to the Plan shall be taken into account in determining any benefits under any pension, retirement, savings, profit sharing, group insurance, welfare or other benefit plan of the
Company or any Affiliate except to the extent otherwise expressly provided in writing in such other plan or an agreement thereunder. 
 14.14 Expenses. The expenses of administering the Plan shall be borne by the Company and its Affiliates. 

  
 35EX-10.1

 Exhibit 10.1 
 SHAREHOLDERS AGREEMENT - EXECUTION COPY 
 SHAREHOLDERS AGREEMENT 

AMENDMENT NO. 2 2012 

between 

Prosensa Holding B.V. 
 and 
 All Shareholders of Prosensa Holding B.V. 

Dated 16 January 2012 

 SHAREHOLDERS AGREEMENT - EXECUTION COPY 
 Contents 
  

							
	Clause	 	 	  	Page	 
			
		 	 CHAPTER 1
	  	 	5	  
			
	 1
	 	 AMENDMENT TO THE 2010 SHAREHOLDERS AGREEMENT
	  	 	5	  
			
		 	 CHAPTER II - RESTATED SHAREHOLDERS AGREEMENT
	  	 	6	  
			
	 1
	 	 DEFINITIONS AND OTHER RULES OF CONSTRUCTION
	  	 	6	  
			
	 2
	 	 ARTCLES. OWNERSHIP STRUCTURE
	  	 	6	  
			
	 2.1
	 	 Articles of the Company
	  	 	6	  
			
	 2.2
	 	 Existing subscription and purchase rights
	  	 	7	  
			
	 2.3
	 	 Ownership structure
	  	 	7	  
			
	 3
	 	 CAPITAL AND SHARES
	  	 	10	  
			
	 3.1
	 	 Type of shares
	  	 	10	  
			
	 3.2
	 	 Tranche 2 Default
	  	 	10	  
			
	 3.3
	 	 Rights attached to the Shares
	  	 	10	  
			
	 3.4
	 	 Special rights Preferred Shares
	  	 	17	  
			
		 	 Exemptions
	  	 	17	  
			
		 	 Pay-to-play
	  	 	18	  
			
	 4
	 	 USE OF PROCEEDS. DIVIDEND POLICY
	  	 	18	  
			
	 4.1
	 	 Use of proceeds
	  	 	18	  
			
	 4.2
	 	 Dividend policy
	  	 	18	  
			
	 5
	 	 ISSUE OF SHARES. PRE-EMPTIVE RIGHTS
	  	 	18	  
			
	 5.1
	 	 Issue of Shares
	  	 	18	  
			
	 5.2
	 	 Pre-emptive right
	  	 	18	  
			
	 5.3
	 	 Restriction/cancellation pre-emptive right
	  	 	19	  
			
	 6
	 	 TRANSFER OF SHARES
	  	 	19	  
			
	 6.1
	 	 Restrictions in general
	  	 	19	  
			
	 6.2
	 	 Permitted transfers
	  	 	19	  
			
	 6.3
	 	 Right of First Refusal
	  	 	20	  
			
	 6.4
	 	 Tag-along Right
	  	 	23	  
			
	 6.5
	 	 Drag-along Right
	  	 	23	  
			
	 6.6
	 	 Transfer of Shares and New Articles
	  	 	24	  
			
	 6.7
	 	 Transfer of shares in personal holding companies
	  	 	25	  
			
	 7
	 	 MANAGEMENT BOARD
	  	 	26	  
			
	 7.1
	 	 Management Board. Number of Managing Directors
	  	 	26	  
			
	 7.2
	 	 Appointment of the Managing Directors
	  	 	27	  
			
	 7.3
	 	 Term
	  	 	27	  
			
	 7.4
	 	 Remuneration
	  	 	27	  

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	 7.5
	 	 Decision making
	  	 	27	  
			
		 	 Voting at Management Board meetings
	  	 	27	  
			
		 	 Tie in votes
	  	 	27	  
			
		 	 Resolutions without holding a meeting
	  	 	27	  
			
		 	 Further rules and regulations
	  	 	27	  
			
	 7.6
	 	 Approvals by the Supervisory Board
	  	 	28	  
			
	 7.7
	 	 Approvals by the Class AB Meeting, the Class A Meeting, and/or the Class B Meeting
	  	 	29	  
			
	 7.8
	 	 Implementation at subsidiaries
	  	 	30	  
			
	 8
	 	 SUPERVISORY BOARD
	  	 	30	  
			
	 8.1
	 	 Supervisory Board. Members
	  	 	30	  
			
	 8.2
	 	 Nominations and Shareholder voting
	  	 	32	  
			
	 8.3
	 	 Term
	  	 	32	  
			
	 8.4
	 	 Remuneration
	  	 	32	  
			
	 8.5
	 	 Decision making by the Supervisory Board
	  	 	32	  
			
		 	 Meetings
	  	 	32	  
			
		 	 Voting at Supervisory Board meetings
	  	 	33	  
			
		 	 Tie in votes
	  	 	33	  
			
		 	 Notice of meetings
	  	 	33	  
			
		 	 Resolutions outside a meeting
	  	 	33	  
			
		 	 Committees
	  	 	33	  
			
	 8.6
	 	 Information for the benefit of the Supervisory Board
	  	 	33	  
			
	 9
	 	 GENERAL MEETING OF SHAREHOLDERS
	  	 	34	  
			
	 9.1
	 	 General Meeting
	  	 	34	  
			
	 9.2
	 	 Shareholder delegates
	  	 	34	  
			
	 9.3
	 	 Decision making
	  	 	34	  
			
		 	 Voting rights per Share
	  	 	34	  
			
		 	 Resolutions outside a meeting
	  	 	35	  
			
		 	 Shareholder voting
	  	 	35	  
			
	 9.4
	 	 Information rights
	  	 	35	  
			
	 10
	 	 EXIT STRATEGY. REGISTRATION RIGHTS
	  	 	36	  
			
	 10.1
	 	 Exit strategy
	  	 	36	  
			
	 10.2
	 	 Registration Rights
	  	 	36	  
			
	 11
	 	 INCENTIVE PLANS
	  	 	37	  
			
	 11.1
	 	 Incentive Plans
	  	 	37	  
			
	 11.2
	 	 Number of Incentive Shares
	  	 	37	  
			
	 11.3
	 	 Stichting AK Board
	  	 	38	  
			
	 12
	 	 VARIOUS
	  	 	38	  
			
	 12.1
	 	 Giving effect to this Agreement
	  	 	38	  
			
		 	 Undertaking by all Parties
	  	 	38	  

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		 	 Undertakings by the Company
	  	 	38	  
			
	 12.2
	 	 Conflict with New Articles
	  	 	39	  
			
	 12.3
	 	 Entire Agreement
	  	 	39	  
			
	 12.4
	 	 Severability
	  	 	39	  
			
	 12.5
	 	 Termination
	  	 	40	  
			
	 12.6
	 	 Consequences of termination. Survival
	  	 	40	  
			
	 12.7
	 	 Notices
	  	 	40	  
			
	 12.8
	 	 Restrictions on announcements
	  	 	46	  
			
	 12.9
	 	 Confidential information
	  	 	46	  
			
		 	 Non-disclosure
	  	 	46	  
			
		 	 Exceptions
	  	 	47	  
			
	 12.10
	 	 Amendments
	  	 	47	  
			
	 12.11
	 	 Conflicts of interests
	  	 	47	  
			
	 12.12
	 	 Assignment
	  	 	48	  
			
	 12.13
	 	 No partnership
	  	 	48	  
			
	 12.14
	 	 Language
	  	 	48	  
			
	 12.15
	 	 Governing law
	  	 	48	  
			
	 12.16
	 	 Dispute resolution
	  	 	48	  
			
	 12.17
	 	 Counterparts
	  	 	48	  

 SHAREHOLDERS AGREEMENT - EXECUTION COPY 
 THIS (AMENDMENT TO THE) SHAREHOLDERS AGREEMENT is made on     January 2012 (the “Agreement”) 
 BETWEEN: 
  

	(1)	Prosensa Holding B.V., a private limited liability company (besloten vennootschap met beperkte aansprakelijkheid) organized and existing under the
laws of the Netherlands, with its corporate seat in Leiden, The Netherlands, with address J.H. Oortweg 21, 2333 CH Leiden, the Netherlands, registered with the Trade Register of the Chamber of Commerce under file number 28076693 (the
“Company’’); 

  

	(2)	LSP III Omni Investment Coöperatief U.A., a co-operative (coöperatie met uitgesloten aansprakelijkheid) organized and existing under the
laws of the Netherlands, with its corporate seat in Amsterdam, The Netherlands with address at Johannes Vermeerplein 9, 1071 DV Amsterdam, The Netherlands, registered with the Trade Register of the Chamber of Commerce under file number 34259327
(“LSP III Omni”); 

  

	(3)	Coöperatief LSP IV U.A., a cooperative with excluded liability for its members, with corporate seat in Amsterdam, the Netherlands and address at:
1071 DV Amsterdam, the Netherlands, Johannes Vermeerplein 9, number Trade Register 34329760 (“LSP IV”), and in that capacity is representing LSP IV; 

 

	(4)	ABV IV Holdings N.V., a limited liability company (naamloze vennootschap met beperkte aansprakelijkheid) organized and existing under the laws of
Curaçao, with its statutory seat at Curaçao, with address at Landhuis Groot Kwartier, Groot Kwartierweg 12, Curaçao, registered with the Commercial Register of the Curaçao Chamber of Commerce & Industry
under file number 83355 (“ABV Holdings NV”); 

  

	(5)	MedSciences Prosensa Holding B.V., a private limited liability company (besloten vennootschap met beperkte aansprakelijkheid) organized and existing under
the laws of the Netherlands, with its corporate seat in Amsterdam, The Netherlands, with address Beethovenstraat 300, 1077 WZ Amsterdam, The Netherlands, registered with the Trade Register of the Chamber of Commerce under file number 24298136
(“MedSciences BV”); 

  

	(6)	 Idinvest Partners (formerly named AGF Private Equity), a French company registered in Paris with registered number RCS 414 735 175 and
whose registered office is situated at 117 Avenue des Champs-Elysées, 75008 Paris, France (“Idinvest”) acting on behalf of and representing: (i) FCPI Allianz Innovation 8 (former name: FCPI AGF Innovation 8), an
investment fund 

  
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(Fonds Commun de Placement dans I’Innovation), formed and existing under the laws of France (“FCPI 8”), (ii) FCPI Capital Croissance 3, an investment fund (Fonds
Commun de Placement dans I’Innovation) (“FCPI Croissance”) and (iii) FCPI Objectif Innovation Patrimoine 3, an investment fund (Fonds Commun de Placement dans I’Innovation) (“FCPI Patrimoine”);

  

	(7)	Gimv NV, a limited liability company, organized and existing under the laws of Belgium, having its corporate seat at Karel Oomsstraat 37, 2018 Antwerpen,
Belgium, with company number VAT BE 0220.324.117 (“Gimv NV”); 

  

	(8)	Adviesbeheer Gimv Life Sciences 2007 NV, a limited liability company, organized and existing under the laws of Belgium, having its corporate seat at Karel
Oomsstraat 37, 2018 Antwerpen, Belgium, with company number VAT BE 0887.140.224 (“Adviesbeheer NV”); 

  

	(9)	Arriwan Holding B.V., a private limited liability company (besloten vennootschap met beperkte aansprakelijkheid) organized and existing under the laws of
the Netherlands, with its corporate seat in Roelofarendsveen (municipality of Alkemade), The Netherlands, with address Weper 6, 8431 RH Oosterwolde, The Netherlands, registered with the Trade Register of the Chamber of Commerce under file number
280832 15 (“Arriwan BV”); 

  

	(10)	Cure Duchenne, a non-profit public benefit corporation, incorporated under the laws of California, United States of America, having its registered office
address at 3334 East Coast Hwy., #157, Corona del Mar, CA 92625. United States of America (“Cure Duchenne”); 

  

	(11)	Charley’s Fund Inc., a not-for-profit organization, organized and existing under the laws of Massachusetts, United States of America, with its
registered office at P.O. Box 83, Stockbridge, MA 01230, United States of America (“Charley’s Fund”); 

  

	(12)	Dordtwijck I B.V., a private limited liability company (besloten vennootschap met beperkte aansprakelijkheid) organized and existing under the laws of the
Netherlands, with its corporate seat in Wassenaar, The Netherlands, with address Groot Haesebroekseweg 49, 2243 EE Wassenaar, The Netherlands, registered with the Trade Register of the Chamber of Commerce under file number 27252134
(“Dordtwijck BV”); 

  

	(13)	Mrs. Catharine Margarethe van den Brink, residing at Kruislaan 184, 1098 SK Amsterdam, The Netherlands, born on June 18, 1964
(“Brink”); 

  

	(14)	 Libertatis Ergo Holding B.V., a private limited liability company (besloten vennootschap met beperkte aansprakelijkheid) organized and
existing under the laws of the Netherlands., with its corporate seat in Leiden, The Netherlands, 

  
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with address Rijnsburgerweg 10, 2333 AA Leiden, The Netherlands, registered with the Trade Register of the Chamber of Commerce under file number 28071688 (“Libertatis BV”);

  

	(15)	Mr. Gerardus Johannes Platenburg, residing at Wijngaardenlaan 56, 2252 XR Voorschoten, The Netherlands, born on February 24, 1964
(“Platenburg”); 

  

	(16)	Mrs. Elisabeth Maria Hubertina Cecilia van Boom-Husken, residing at Pieter de Hoochlaan 14, 2343 CR Oegstgeest, The Netherlands, born on
March 10, 1942 (“Boom”); 

  

	(17)	Mr. Peter Frank Ekhart, residing at Niersstraat 61-1, 1078 VJ Amsterdam, The Netherlands, born on July 24, 1966 (“Ekhart”);

  

	(18)	Mr. Charles Jan Peter Maria van Megen, residing at Kruislaan 184, 1098 SK Amsterdam, The Netherlands, born on March 14, 1958
(“Megen”); 

  

	(19)	Mr. Daniël Jan Ellens, residing at Kriesbaumen 288, CH 3157, Milken. Switzerland, born on July 31, 1948 (“Ellens”);

  

	(20)	Mr. Herbert Louis Heijneker, residing at 2244 Steiner Street, San Francisco, CA 94115, United States of America, born on January 22, 1944
(“Heijneker”); 

  

	(21)	Stichting Administratiekantoor Prosensa Holding, a foundation (stichting) organized and existing under the laws of the Netherlands, with its seat
at Leiden, The Netherlands, address Wassenaarseweg 72, 2333 AL Leiden, The Netherlands, registered with the Trade Register of the Chamber of Commerce under file number 27330683 (“Stichting AK”); and 

 

	(22)	New Enterprise Associates 13, L.P., a limited partnership organized and existing under the laws of the Cayman Islands, with registered address c/o Maples
Corporate Services, PO Box 309, Ugland House, Grand Cayman KY1-1104, Cayman Islands (“NEA”). 

 All parties
hereinafter collectively and individually referred to as: “Parties” and “Party” respectively. FCPI 8, FCPI Croissance and FCPI Patrimoine are hereinafter collectively also referred to as the “Idinvest
Funds”, and the parties under (7) and (8) hereinafter collectively also referred to as the “Gimv Parties”. 

WHEREAS: 
  

	(A)	The Company and its wholly owned subsidiaries (the “Subsidiaries”) are involved in the development and commercialization of RNA modulating
products, in particular in relation to genetic muscular disorders (the “Business”). 

  
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	(B)	On the 26 November, 2008, the relevant Parties (among other parties) have executed a subscription agreement (the “2008 Subscription
Agreement”) with regard to the Company’s series B financing round (the “Series B Round”), pursuant to which LSP III Omni, ABV Holdings NV, MedSciences BV, the Idinvest Funds and the GIMV Parties have (i) acquired
4.153,845 Class B Shares, at a total issue price of EUR 9,000,000,— (nine million euro) (the “2008 First Installment Shares”), (ii) acquired a further 4,153,845 Class B Shares, at a total issue price of EUR
9,000,000.— (nine million euro) (the “2008 Second InstalIment Shares”). 

  

	(C)	Further to the 2008 Subscription Agreement, the relevant Parties and the Company have, among other things, executed on the 8th day of December, 2008, a
shareholders agreement setting forth the terms and conditions between them as shareholders of the Company (the “Shareholders”) and the terms and conditions in relation to the operations of the Company (the “2008 Shareholders
Agreement”). 

  

	(D)	Subsequent to the 2008 Subscription Agreement, the ownership of shares in the Company have changed, pursuant to a sale and transfer of shares by Arriwan to
Platenburg on 12 February 2009, the sale and transfer of shares by BioPartner Start-up Ventures C.V. and Mrs. Petra van Kuik-Romeijn, and the issue of shares on 15 October 2009 to MedSciences BV pursuant to the exercise by MedSciences
BV of its option right. Furthermore, the 2008 Second Installment Shares have been issued on 18 January 2010. 

  

	(E)	On 6 October 2009, the Company has entered into a research, development, collaboration and license agreement with Glaxo Group Limited.

  

	(F)	Pursuant to the development of the Company, including the share transactions and the transaction with Glaxo Group Limited, the relevant Parties have updated and
amended the 2008 Shareholders Agreement by the first amendment to the 2008 Shareholders Agreement, executed on 29 June 2010 (the “2010 Shareholders Agreement”). 

 

	(G)	On     January 2012, the relevant Parties have executed a subscription agreement (the “2012 Subscription Agreement”) with
regard to: 

  

	 	(i)	a new issue of class B2 preferred shares, pursuant to which LSP IV, ABV Holdings NV, MedSciences BV, FCPI Croissance, FCPI Patrimoine, the Gimv Parties and NEA have
acquired 5,000,004 Class B2 Shares, at a total issue price of EUR 11,500,009.20; and 

  

	 	(ii)	a new issue of class B3 preferred shares, pursuant to which LSP IV, ABV Holdings NV, MedSciences BV, FCPI Croissance, FCPI Patrimoine, the Gimv Parties and NEA intend
to acquire 4,107,140 Class B3 Shares, at a total issue price of EUR 11,499,992. 

  

	(H)	Pursuant to the 2012 Subscription Agreement, the Parties now wish to hereby update and amend the terms and conditions between them as Shareholders of the
Company. 

  
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 THEREFOR IT IS HEREBY AGREED AS FOLLOWS: 

CHAPTER 1 
  

	1	AMENDMENT TO THE 2010 SHAREHOLDERS AGREEMENT 

  

	1.1	The Parties hereby agree, confirm and ratify that subject only to this Agreement (including the attachments hereto), the 2010 Shareholders Agreement, including
the schedules thereto, shall remain in full force and effect and that any rights and obligations pursuant to the 2010 Shareholders Agreement shall remain unaffected, subject only to the amendments expressly contained herein.

  

	1.2	The following provisions and schedules of the 2010 Shareholders Agreement shall be amended as follows: 

 

	 	•	 	 Schedule 1.1 (Definitions), in order to update it with any amended or new defined terms; 

 

	 	•	 	 Parties, updated to reflect that NEA and LSP IV are new parties to the shareholders agreement; 

 

	 	•	 	 Clause 2.1, in order to reflect the Company’s amended articles of association; 

 

	 	•	 	 Clause 2.2, in order to reflect the current existing and purchase rights; 

 

	 	•	 	 Clause 2.3, in order to reflect the current share ownership on a fully diluted basis and the share ownership after the new issue of Class B2 Shares and
Class B3 Shares; 

  

	 	•	 	 Clause 3, in order to reflect the new issue of Class B2 Shares and Class B3 Shares; 

 

	 	•	 	 Clause 4, in order to reflect the use of proceeds of the issue of Class B Shares; 

 

	 	•	 	 Clause 6.2, in order to reflect the permitted transfers regarding the LSP Funds; 

 

	 	•	 	 Clause 6.5, in order to reflect the further details of the drag along right as agreed between the Parties; 

 

	 	•	 	 Clause 7.6, in order to reflect the agreed amendment of the thresholds; 

  
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	 	•	 	 Clause 8, in order to reflect the new composition of the Supervisory Board; 

 

	 	•	 	 Clause 10.1, in order to reflect the exit strategy; 

  

	 	•	 	 Clause 11.2, in order to reflect the current number of Stock Option Shares; 

 

	 	•	 	 Clause 11.3, in order to reflect the new composition of the Stichting AK Board. 

CHAPTER II - RESTATED SHAREHOLDERS AGREEMENT 
 The parties hereby agree, confirm and ratify that the 2010 Shareholders Agreement in which the amendments as referred to in Chapter I have been inserted shall henceforth read as follows: 

 

	1	DEFINITIONS AND OTHER RULES OF CONSTRUCTION 

  

	1.1	Definitions. In this Agreement, unless the context otherwise requires, the words and expressions shall have the meanings set out in Schedule 1.1.

 Documents. References to any document, including this Agreement, are references to that document as
amended, supplemented, novated or replaced from time to time. 
 Recitals. Clauses. Paragraphs and Schedules. References
in this Agreement to Recitals, Clauses, Paragraphs and Schedules are to clauses and paragraphs in and recitals and schedules to this Agreement. The Recitals and Schedules to this Agreement shall be deemed to form part of this Agreement. 

Headings. Headings are inserted for convenience only and shall not affect the construction of this Agreement. 

 

	2	ARTCLES. OWNERSHIP STRUCTURE 

  

	2.1	Articles of the Company 

The articles of association of the Company as currently in force are set forth in a deed executed on 16 January 2012, before (a substitute
of) a civil law notary in Amsterdam, the Netherlands, at De Brauw Blackstone Westboek N.V. (the “New Articles”). 

  
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	2.2	Existing subscription and purchase rights. 

 The Parties acknowledge that, other than the rights and obligations pursuant to the 2012 Subscription Agreement, the following rights to subscribe for or purchase shares in the capital of the Company or
depositary receipts thereof exist: 
  

	 	(a)	pursuant to the Company’s employee stock option plans and the 2010 Equity Incentive Plan (the “Incentive Plans”), the Company has a pool of rights
to acquire a total of up to 2.241.454 depositary receipts for Common Shares to be issued by the Company (granted and not yet granted rights, including the agreed extension assumed as 500,000 depositary receipts), of which rights to acquire a total
of 1,418,232 depositary receipts have been granted to employees but not yet exercised, whilst rights to acquire a total of 823,222 depositary receipts are still available. The Common Shares that may be issued in connection with the Incentive Plans
will hereinafter be referred to as the “Incentive Shares”. 

  

	 	(b)	the Supervisory Board shall have the right to have Arriwan BV grant rights to purchase up to 56,250 Common Shares, at a price of EUR 1.82, to members of the scientific
advisory board (if and when installed) and members of senior management of the Company. Furthermore, Arriwan BV has granted a call option right to Mrs. J. van Deutekom to purchase a total of 33,750 Common Shares, at a purchase price of EUR
1.82 per Common Share. The above options shall be exercisable on or before October 1, 2016. 

  

	2.3	Ownership structure 

 As
per the date hereof and if all Incentive Shares would have been issued, as well as the voting interests, the ownership structure shall be as follows: 
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	 Shareholder
	  	Common
Shares	 	  	Class O
Shares	 	  	Class A
Shares	 	  	Class B1
Shares	 	  	Class B2
Shares	 	  	Voting
Interest
(rounded)	 
	 Arriwan BV
	  	 	784,002	  	  				  				  				  				  	 	2.96	% 
	 MedSciences BV
	  	 	42,026	  	  	 	470,533	  	  	 	824,175	  	  	 	461,538	  	  	 	108,696	  	  	 	7,21	% 
	 Cure Duchenne
	  	 	337,128	  	  				  				  				  				  	 	1,27	% 
	 Charley’s Fund
	  	 	196,452	  	  				  				  				  				  	 	0,74	% 
	 Dordtwijck BV
	  	 	16,335	  	  				  				  				  				  	 	0,06	% 
	 Brink
	  	 	14,265	  	  				  				  				  				  	 	0,05	% 
	 Libertatis BV
	  	 	279,720	  	  				  				  				  				  	 	1,06	% 
	 Platenburg
	  	 	311,898	  	  				  				  				  				  	 	1,18	% 
	 Boom
	  	 	202,500	  	  				  				  				  				  	 	0,77	% 
	 Ekhart
	  	 	27,090	  	  				  				  				  				  	 	0,10	% 
	 Megen
	  	 	3,420	  	  				  				  				  				  	 	0,01	% 
	 Ellens
	  	 	67,500	  	  				  				  				  				  	 	0,26	% 
	 Heijneker
	  	 	90,000	  	  				  				  				  				  	 	0,34	% 
	 LSP Funds*
	  	 	32,491	  	  	 	52,073	  	  	 	3,296,703	  	  	 	1,961,538	  	  	 	478,262	  	  	 	22,0	% 
	 ABV Holdings NV
	  	 	32,491	  	  	 	52,073	  	  	 	3,296,703	  	  	 	1,961,538	  	  	 	478,262	  	  	 	22,0	% 
	 Idinvest Funds**
	  	 	32,491	  	  	 	52,073	  	  				  	 	1,961,538	  	  	 	315,218	  	  	 	8,93	% 
	 Gimv Funds***
	  	 	32,491	  	  	 	52,073	  	  				  	 	1,961,538	  	  	 	315,218	  	  	 	8,93	% 
	 NEA
	  				  				  				  				  	 	3,304,348	  	  	 	12,49	% 
	 Stichting AK
	  	 	564,131	  	  				  				  				  				  	 	2,11	% 
	 Unissued ESOP Shares
	  	 	1,418,232	  	  				  				  				  				  	 	5,36	% 
	 Unallocated Incentive Shares
	  	 	573,222	  	  				  				  				  				  	 	2,16	% 
		  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 
	 Total
	  	 	5,051,135	  	  	 	678,825	  	  	 	7,417,581	  	  	 	8,307,690	  	  	 	5,000,004	  	  	 	100	% 
		  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 

  

	*	LSP Funds: LSP III Omni Investment Coöperatief U.A. and LSP IV 

	**	Idinvest Funds: FCPI Allianz Innovation 8, FCPI Capital Croissance and FCPI Patrimoine 

	***	Gimv Funds: Gimv NV and Adviesbeheer NV 

  
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 Immediately upon the issue of Class B3 Shares to LSP IV, ABV Holdings NV, MedSciences
BV, FCPI Croissance, FCPI Patrimoine, the Gimv Parties and NEA in accordance with the 2012 Subscription Agreement (the “Tranche 2 Issue”) and, assuming that all Incentive Shares have been issued and no shares have been cancelled or
converted, the ownership structure shall be as follows: 
  

																													
	 Shareholder
	  	Common
Shares	 	  	Class O
Shares	 	  	Class A
Shares	 	  	Class B1
Shares	 	  	Class B2
Shares	 	  	Class B3
Shares	 	  	VI	 
	 Arriwan BV
	  	 	784,002	  	  				  				  				  				  				  	 	2.54	  
	 MedSciences BV
	  	 	42,026	  	  	 	470,533	  	  	 	824,175	  	  	 	461,538	  	  	 	108,696	  	  	 	89,285	  	  	 	6.48	  
	 Cure Duchenne
	  	 	337,128	  	  				  				  				  				  				  	 	1.09	  
	 Charley’s Fund
	  	 	196,452	  	  				  				  				  				  				  	 	0.64	  
	 Dordtwijck BV
	  	 	16,335	  	  				  				  				  				  				  	 	0.05	  
	 Brink
	  	 	14,265	  	  				  				  				  				  				  	 	0.05	  
	 Libertatis BV
	  	 	279,720	  	  				  				  				  				  				  	 	0.91	  
	 Platenburg
	  	 	311,898	  	  				  				  				  				  				  	 	1.01	  
	 Boom
	  	 	202,500	  	  				  				  				  				  				  	 	0.66	  
	 Ekhart
	  	 	27,090	  	  				  				  				  				  				  	 	0.09	  
	 Megen
	  	 	3,420	  	  				  				  				  				  				  	 	0.01	  
	 Ellens
	  	 	67,500	  	  				  				  				  				  				  	 	0.22	  
	 Heijneker
	  	 	90,000	  	  				  				  				  				  				  	 	0.29	  
	 LSP Funds
	  	 	32,491	  	  	 	52,073	  	  	 	3,296,703	  	  	 	1,961,538	  	  	 	478,262	  	  	 	392,857	  	  	 	20.17	  
	 ABV Holdings NV
	  	 	32,491	  	  	 	52,073	  	  	 	3,296,703	  	  	 	1,961,538	  	  	 	478,262	  	  	 	392,857	  	  	 	20.17	  
	 Idinvest Funds
	  	 	32,491	  	  	 	52,073	  	  				  	 	1,961,538	  	  	 	315,218	  	  	 	258,928	  	  	 	8.5	  
	 Gimv Funds
	  	 	32,491	  	  	 	52,073	  	  				  	 	1,961,538	  	  	 	315,218	  	  	 	258,928	  	  	 	8.5	  
	 NEA
	  				  				  				  				  	 	3,304,348	  	  	 	2,714,285	  	  	 	19.53	  
	 Stichting AK
	  	 	564,131	  	  				  				  				  				  				  	 	1.81	  
	 Unissued ESOP Shares
	  	 	1,418,232	  	  				  				  				  				  				  	 	4.6	  
	 Unallocated Incentive Shares
	  	 	823,222	  	  				  				  				  				  				  	 	2.67	  
		  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 
	 Total
	  	 	5,307,885	  	  	 	678,825	  	  	 	7,417,581	  	  	 	8,307,690	  	  	 	5,000,004	  	  	 	4,107,140	  	  	 	100	  
		  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 

  
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 As soon as practicable after the execution of the deed of issue of the Class B3 Shares
in accordance with the 2012 Subscription Agreement (the “Tranche 2 Completion Date”) or a Tranche 2 Default (as referred to under paragraph 3.1), the Company will attach a chart reflecting the updated ownership structure to this
Agreement and inform the Parties thereof. 
  

	3	CAPITAL AND SHARES 

  

	3.1	Type of shares 

 In
accordance with the New Articles, the Company’s capital is divided into the following types of shares (hereinafter collectively and individually referred to as the “Shares” and the “Share” respectively):

  

	 	•	 	 ordinary Shares, being the Common Shares; 

  

	 	•	 	 junior preferred Shares, being the Class O Shares; 

  

	 	•	 	 preferred Shares, being the Class A Shares; and 

  

	 	•	 	 senior preferred Shares, being the Class B Shares, sub-divided into the Class B1 Shares, consisting of 8,307,690 shares, Class B2 Shares, consisting of
5,000,004 shares and, as a result of the Tranche 2 Issue, Class B3 Shares, consisting of 4,107,140 shares, 

each Share with a nominal value of EUR 0.01. 
 The Class A Shares and the Class B Shares (for the avoidance of doubt, consisting of the Class B1 Shares, the Class B2 Shares and the Class B3 Shares) will jointly also be referred to as the
“Class AB Shares” and the Class AB Shares and the Class O Shares will jointly be referred to as the “Preferred Shares”). 
  

	3.2	Tranche 2 Default 

Pursuant to the 2012 Subscription Agreement, if LSP IV, ABV Holdings NV, MedSciences BV, FCPI Croissance, FCPI Patrimoine, the Gimv
Parties or NEA fails to comply with one or more of its “Tranche 2 Completion Obligations”, as described in the 2012 Subscription Agreement (a “Defaulting Investor”), then, in addition to the remedies that the Company and
any other Parties may have, the Class B2 Shares of that or those Defaulting Investor(s) shall be converted into Common Shares, in accordance with the terms of the 2012 Subscription Agreement. 

 

	3.3	Rights attached to the Shares 

 Each Share shall entitle its holder to cast one vote. 

  
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 The Common Shares and the Preferred Shares shall have the rights as allocated to them in
this Agreement and the New Articles. 
  

	3.4	Special rights Preferred Shares 

  

	3.4.1	Dividend rights 

 The
Preferred Shares shall have the dividend rights as set forth in the New Articles, meaning in summary that: 
  

	 	(i)	each Class B Share will carry an annual non-cumulative profit entitlement of 8% of the amount paid on such Share (nominal value plus share premium attributed to such
Share). Said rights attached to the Class B Shares are at all times senior to any rights of the Class A Shares, the Class O Shares, set out below and in the New Articles, and the Common Shares; 

 

	 	(ii)	each Class A Share will carry an annual non-cumulative profit entitlement of 8% of the amount paid on such Shares (nominal value plus share premium attributed to
such series). Said rights attached to the Class A Shares are at all times senior to any rights of the Class O Shares, set out below and in the New Articles, and the Common Shares; and 

 

	 	(iii)	each Class O Share will carry an annual non-cumulative profit entitlement of 6% of the amount paid on such Share (nominal value plus share premium). Said rights
attached to the Class O Shares and the rights attached to the Common Shares are at all times junior to the rights of the Class B Shares and the Class A Shares as set out above and in the New Articles, but the rights of the Class O Shares are at
all times senior to the rights of the Common Shares. 

 All profits allocated to the Class B Shares, the
Class A Shares and the Class O Shares will be credited to separate profit reserves for such Shares, or in case of the Class B Shares, to separate profit reserves for each the Class B1 Shares, the Class B2 Shares and the Class B3 Shares, whilst
any and all remaining profits will be credited to the Company’s general profit reserve. Without the approval of the joint meeting of the holders of the Class AB Shares, which shall adopt its resolutions in accordance with Clause 9.3 (the
“Class AB Meeting”), no dividends will be paid on the Common Shares or Class O Shares, so long as Class AB Shares are outstanding. 

  
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	3.4.2	Liquidation rights Preferred Shares 

  

	 	1.	Class AB Shares Liquidation Rights 

 Exclusively upon the occurrence of any of the following, events (each a “Liquidation Event”): 
  

	 	(i)	a liquidation or the winding-up of the Company (ontbinding); or 

  

	 	(ii)	a merger or other event pursuant to which the Shareholders will have 50% or less of the total voting power of the surviving or acquiring company; or

  

	 	(iii)	a sale, lease transaction, transfer or other disposition of all Shares (including pursuant to the exercise of the drag-along right as referred to in Clause 6.5), or of
all or substantially all of the Company’s assets or of the Subsidiaries’ assets, including, without limitation, the core intellectual property rights owned, co-owned or controlled by the Company and the Subsidiaries (such as, for example,
the intellectual property rights licensed by the Company from Leiden University Medical Center), or the grant to a single third party, in a field of use comprising at least Duchenne Muscular Dystrophy, of an exclusive (sub)license under such core
intellectual property rights; 

 the holders of Class AB Shares (the “AB-Shareholders”) shall be
entitled to receive an amount equal to the original issue price (which is the price per Share at which the relevant tranche / installment of Shares, were originally issued, comprising of the formal nominal share capital plus share premium paid per
Share, hereinafter the “Original Issue Price”) of the relevant AB Shares, increased by an amount equal to any accrued but unpaid dividends (and which have therefore been allocated to the profit reserve for such class of shares in
accordance with the New Articles) on the relevant Class AB Shares until the date of the Liquidation Event in cash or securities (“Class AB Liquidation Proceeds”), for the calculation of which 8% return interim payments (e.g.
dividends previously paid and share premium repayments previously made) made by the Company to the AB-Shareholders shall reduce the balance of such accrued but unpaid dividends. For the avoidance of doubt, it is hereby confirmed and agreed that
(i) the calculation of the Class AB Liquidation Proceeds shall be separately made for any issue of Class AB Shares, and (ii) upon full receipt of the Class AB Liquidation Proceeds, the AB-Shareholders shall have no further entitlement
pursuant to their preferred dividend rights (but shall remain entitled to distributions of Remaining Liquidation Proceeds as provided in subclause 3 below). 
 In case insufficient proceeds are available to fully pay the Class AB Liquidation Proceeds, the available proceeds shall be paid first to the holders of Class B Shares (the
“B-Shareholders”), in preference over the holders of Class A Shares (the “A-Shareholders”), while any amounts available for the holders of a class of Shares, shall be paid to each of them based upon the
relative amount actually invested in such class. 

  
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 In order to effectuate a Liquidation Event a resolution must be adopted to that effect
by the Class AB Meeting. 
  

	 	2.	Class O Liquidation Rights 

After full payment of the Class AB Liquidation Proceeds the holders of Class O Shares (the “O-Shareholders”) shall be
entitled to receive, in preference over the holders of Common Shares (the “Common-Shareholders”), from the remaining proceeds (if any) an amount equal to the Original Issue Price of the relevant Class O Shares, increased by an
amount equal to any accrued but unpaid dividends (and which have therefore been allocated to the profit reserve O in accordance with the New Articles) (“Class O Liquidation Proceeds”), for the calculation of which 6% return interim
payments (e.g. dividends previously paid and share premium repayments previously made) made by the Company to the O-Shareholders Shares shall reduce the balance of such accrued but unpaid dividends. For the avoidance of doubt, it is hereby confirmed
and agreed that upon full receipt of the Class O Liquidation Proceeds, the O-Shareholders shall have no further entitlement pursuant to their preferred dividend rights (but shall remain entitled to distributions of Remaining Liquidation Proceeds as
provided in subclause 3 below). 
 In case insufficient proceeds are available to fully pay the Class O Liquidation Proceeds, the
available proceeds shall be paid to the O-Shareholders pro rata to the amounts that should have been paid if sufficient proceeds would be available. 
  

	 	3.	Remaining Liquidation Proceeds 

After full payment of the Class AB Liquidation Proceeds and the Class O Liquidation Proceeds, any remaining amounts thereafter shall be
paid to all holders of Shares (whether of Common Shares, Class O Shares, Class A Shares or Class B Shares), in proportion to the number of Shares held, whereby the Preferred Shares shall be taken into account on an as if converted basis (the
“Remaining Liquidation Proceeds”). 
 In case the Liquidation Event is caused by the grant to a single third
party, in a field of use comprising at least Duchenne Muscular Dystrophy, of an exclusive (sub)license under the Company’s core intellectual property rights, the Parties shall in exercising the above liquidation rights refrain from causing the
Company to distribute cash or otherwise withdraw funds from the Company until such time as such distribution and/or withdrawal will not prevent the Company from performing its obligations under the relevant (sub)license transaction. 

  
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	 	4.	Contingent consideration 

 In the
event of a Liquidation Event if any portion of the consideration payable to the shareholders of the Company is placed into escrow and/or is payable to the shareholders of the Company subject to contingencies, the relevant transaction agreements
shall provide that (a) the portion of such consideration that is not placed in escrow and not subject to any contingencies (the “Initial Consideration”) shall be allocated among the holders of Shares of the Company in
accordance with clauses 1 through 3 of this clause 3.4.2 as if the Initial Consideration were the only consideration payable in connection with such Liquidation Event and (b) any additional consideration which becomes payable to the
shareholders of the Company upon release from escrow or satisfaction of contingencies shall be allocated among the holders of Shares in accordance with clauses 1 through 3 of this clause 3.4.2 after taking into account the previous payment of the
Initial Consideration as part of the same transaction. 
  

	3.4.3	Conversion rights 

  

	 	1.	Conversion events 

 The issued
Preferred Shares (as well as any accrued and unpaid dividends) are convertible into Common Shares only. 
 Conversion of
Preferred Shares shall occur with regard to all the issued Preferred Shares, in the following events: 
  

	 	(i)	the Class AB Meeting has adopted a resolution to convert all (and not fewer then all) of the issued Preferred Shares, it being understood, however, that if such
conversion occurs in connection with an intended Liquidation Event of which the proceeds amounts to less than EUR 54,600,000, the prior approval of the meeting of the holders of Class B Shares (the “Class B Meeting”) shall also be
required; and 

  

	 	(ii)	immediately prior to the completion of an initial public offering of shares of the Company at an organized or regulated public market (an “IPO”),
provided that the aggregate gross proceeds shall amount to at least EUR 30,000,000 and a public offering price per Share of not less than EUR 6.95. 

  
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	 	2.	(Adjustment of) Conversion Rate 

The conversion shall take place at a rate (the “Conversion Rate”) calculated by dividing the Original Issue Price of the
relevant Preferred Shares by the applicable conversion price (the “Conversion Price”). The applicable Conversion Price shall be equal to the Original Issue Price of the relevant Preferred Shares, subject, however, to an adjustment
in the following events after the date hereof: 
  

	 	•	 	 any subdivision (stock split) or combination of Shares; 

 

	 	•	 	 any distribution by the Company of a dividend in Shares to Shareholders; 

 

	 	•	 	 all other events that are similar to the above events; and 

 

	 	•	 	 in relation to the Class AB Shares, in case the Anti-Dilution Protection (set forth in the below paragraph 3.4.4) applies.

  

	3.4.4	Anti-dilution protection AB-Shareholders 

 The AB-Shareholders shall be protected against dilution of their shareholding in the Company on a broad-based weighted average basis as described below (“Anti-Dilution Protection”), such
that in the event the Company issues additional shares or options or securities which are convertible into Shares against a price (the “Dilutive Price”) which is less than the applicable Conversion Price of the relevant Class A
and/or Class B Shares issued in the relevant tranche, the Conversion Price shall be reduced to the price resulting from the this broad based weighted average adjustment, it being understood, however, that in case the Anti-Dilution Protection applies
to both the Class A Shares and the Class B Shares, first a calculation shall be made in relation to the Class B Shares, and subsequently in relation to the Class A Shares. 

The Conversion Price of the Class B Shares (“CPb”) will hence be calculated in accordance with the formula below:

 CPb = [(Pb * Qpre) + (Pnew * Qnew)] / (Qpre + Qnew) 

 

			
	where: Pb	  	= the Original Issue Price of the Class B1 Shares, the Class B2 Shares or the Class B3 Shares, at the case may be

 Qpre = the total number of Shares pre money of the new dilutive round, on a fully diluted basis

 Pnew = the Dilutive Price (being the subscription price in the new round) 

Qnew = the number of Shares issued in the new round al the Dilutive Price 

  
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 Subsequently, the Conversion price of the Class A Shares (“CPa”)
will hence be calculated in accordance with the formula attached below: 
 CPa = [(Pa * (Qpre+ Qbplus) + (Pnew * Q new)] / (Qpre
+ Qbplus + Qnew) 
 where: Pa = the Original Issue Price of the Class A Shares 

Qpre = the total number of Shares pre money of the new dilutive round, on a fully diluted basis 

Qbplus = the total number of anti-dilution Class B Shares which will be calculated pursuant to (Pb* original number of Class B Shares /
CPb) 
 - original number of Class B Shares 
 Pnew = the Dilutive Price (being the subscription price in the new round) 
 Qnew =
the number of Shares issued in the new round at the Dilutive Price 
 Instead of adjusting the Conversion Price, the relevant
AB-Shareholders, at their sole discretion, may cause the Company to issue additional Shares of the same class and, in the event of the issue of Class B Shares, the issue of Class B1 Shares, the issue of Class B2 shares or the issue of Class B3
Shares, as the case may be, as to which the Anti-Dilution Protection applies (with exclusion of any pre-emptive rights of all other shareholders), to the extent that the Company is lawfully able to do so. The issue to the AB-Shareholders shall occur
by way of capitalisation of the Company’s share premium account or otherwise in accordance with all applicable laws and in a manner approved by the AB-Shareholders, of such number of additional Shares of the relevant class, creating a similar
anti-dilution protection as pursuant to adjustment of the Conversion Price. If the reserves of the Company are not sufficient to fully pay up the additional Shares to which the AB-Shareholders are entitled, all of the Shareholders shall effectuate a
transfer of Shares among them in order to achieve the correct percentages of ownership. Furthermore, the Shareholders shall effectuate an amendment of the New Articles to the extent that such amendment should be required in order to create
sufficient Shares as needed for the share issue. 
 If and to the extent the rights of the A-Shareholders and the B-Shareholders
cannot be fully awarded, the rights of the B-Shareholders shall be given preference to the rights of the A-Shareholders. 

  
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 Exemptions. 

The Anti-Dilution Protection will not apply in the event of issuance of (i) Common Shares issued or issuable upon conversion of
Preferred Shares or issued or issuable to employees, consultants or directors of the Company directly or pursuant to the Incentive Plans that have been approved by the Supervisory Board; (ii) in exceptional cases and subject to prior approval
of the Class AB Meeting, Common Shares (and/or options or warrants therefore) issued or issuable to parties that are actual or potential suppliers, distributors or customers, strategic partners investing in connection with a commercial relationship
with the Company or providing the Company with loans, credit lines, guarantees of indebtedness or similar transactions; and (iii) Common Shares issued or issuable (a) in a public offering before or in connection with which all outstanding
Preferred Shares will be converted to Common Shares or (b) upon exercise of warrants or rights granted to underwriters in connection with such a public offering. 
 Pay-to-play. 
 In the event of any dilutive issuance of shares up to an
aggregate subscription amount of EUR 20,000,000 reflecting an issue price (nominal value and share premium) below EUR 1.82, and therefore below the issue price of both the Class A Shares and the Class B Shares (the “A&B Down
Round”), the AB-Shareholders are required to participate in the A&B Down Round (at least in the part reserved to the AB-Shareholders) to the extent of their pro rata equity interest in the Class A Shares and Class B Shares (which
for the purpose of this provision, will be considered as one single class). In the event and to the extent that an AB-Shareholder fails to participate in accordance with the previous sentence, the Class A Shares or Class B Shares held by such
AB-Shareholder will automatically and proportionally, lose their Anti-Dilution Protection. 
 In the event of any dilutive
issuance of shares up to an aggregate amount of EUR 20,000,000 reflecting an issue price (nominal value and share premium) below P (as defined below), but an issue price per share equal or greater than EUR 1.82 (the “B Down Round”),
the B-Shareholders are required to participate in the B Down Round (at least in the part reserved to the B-Shareholders) to the extent of their pro rata equity interest in the Class B Shares. In the event and to the extent that a B-Shareholder fails
to participate in accordance with the previous sentence, the Class B Shares held by such B-Shareholder will automatically and proportionally, lose their Anti-Dilution Protection. 

P will be calculated in accordance with the formula below: 
 P = (Qb2* EUR 2.30) + (Qb3 * EUR 2.80) / (Qb2 + Qb3) 

  
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	Where:	 	Qb2 = the number of issued Class B2 Shares; and
		
		 	Qb3 = the number of issued Class B3 Shares

  

	4	USE OF PROCEEDS. DIVIDEND POLICY 

  

	4.1	Use of proceeds 

 The
Company will apply the net proceeds of the issue of Class B Shares exclusively to the development and operation of the Company. 
  

	4.2	Dividend policy 

 The
Parties acknowledge that the Company may only distribute profits to the shareholders provided that the Company has distributable reserves available for distribution in accordance with section 2:216 of the Netherlands Civil Code. 

Distributions may only be made pursuant to a resolution by the General Meeting, with the prior approval of the Class AB Meeting (in
absence of which approval no distributions may be made). 
  

	5	ISSUE OF SHARES. PRE-EMPTIVE RIGHTS 

  

	5.1	Issue of Shares 

 Without
prejudice to Clause 5.2, the Company shall only issue Shares pursuant to a resolution by the General Meeting adopted with a simple majority of the votes cast in the meeting, provided that such resolution shall require the prior approval of the Class
AB Meeting. 
  

	5.2	Pre-emptive right 

Without prejudice to the Anti-Dilution Protection, upon the issue of new Shares all Shareholders (irrespective of the Class of Shares held
by these Shareholders) shall have a pre-emptive right to subscribe for the newly issued Shares in proportion to their respective ownership interest prior to such issue of Shares, subject to article 6 of the New Articles. 

In accordance with said article 6 of the New Articles, the following shall apply to the pre-emptive right upon an issue of Shares:

  

	 	•	 	 in case Shares are being issued under the Incentive Plans, as defined in Clause 11, whether directly to employees, managing directors or supervisory
directors of the Company or of group companies or to an entity that shall hold the Shares for such employees, the other Shareholders shall not have any pre-emptive rights; 

  
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	 	•	 	 the eligible employees and the entity holding shares under the Incentive Plans shall not have any pre-emptive rights upon the issue of Shares;

  

	 	•	 	 in relation to a particular issue of Shares, the pre-emptive rights may be limited or excluded pursuant to a resolution passed in the same manner as
the resolution to issue Shares (resolution by the General Meeting adopted with a simple majority of the votes cast in the meeting, provided that such resolution shall require the prior approval of the Class AB Meeting), provided, however, that the
pre-emptive rights of the B-Shareholders may only be limited or excluded with the prior approval of the Class B Meeting; 

  

	 	•	 	 if pre-emptive rights may be exercised upon an issue of Shares, the Company shall notify the Shareholders in writing at the address stated by them, of
the period during which such right may be exercised, which period – in accordance with Dutch law shall be at least four weeks after the day of sending the notice. 

 

	5.3	Restriction/cancellation pre-emptive right 

 The General Meeting may only restrict or cancel the pre-emptive rights of the Shareholders in accordance with this Agreement and the New Articles. 

 

	6	TRANSFER OF SHARES 

  

	6.1	Restrictions in general 

No Party shall transfer any Shares in a manner inconsistent with this Agreement. A Share transfer is only permitted if the party that
acquires the Shares agrees to be bound by this Agreement, and any related agreements referred to herein, if applicable, and agrees to fulfil all obligations of the transferor in that respect, as its own. 

 

	6.2	Permitted transfers 

Without prejudice to any other rights under this Agreement, any Shareholder may transfer all of its Shares to one of its wholly owned
subsidiaries, and any Shareholder may transfer all of its Shares to a (direct or indirect) wholly owned subsidiary of any Shareholder’s ultimate parent company, provided, however, that a Shareholder may not transfer any Shares to (designated
holding companies for specific) portfolio companies. If a Shareholder is the personal holding company, all Shares held by such Shareholder or all Shares in such Shareholder may be transferred to another, successive, personal holding company. In
addition, Dordtwijck BV is entitled to transfer all of its Shares to its managing director and sole shareholder Mr. J.C.H.L Pauli. In case of a transfer permitted under the previous sentences, the transferring Shareholder shall

  
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cause the transferee to accede to this Agreement and to assume any and all obligations and liabilities of the transferring Shareholder under this Agreement. The transferring Shareholder shall
remain jointly and severally liable with the transferee, provided that at the request of the transferring Shareholder the other Shareholders agree to waive the benefit of said joint and several liability, which waiver shall not be unreasonably
withheld if the transferee as a sustainable financial capability at least equal to that of the transferring Shareholder. In addition, each of the AB-Shareholders that is an investment fund or a fund manager, shall be entitled to transfer (some or
all of) its Shares to a fund or entity that is under the same (ultimate) management as itself (it being understood that LSP III Omni and LSP IV are under the same (ultimate) management, ABV Holdings NV is under the management of Abingworth
Management Ltd and/or Abingworth LLP, FCPI 8, FCPI Croissance and FCPI Patrimoine are each under the management of Idinvest. In case of a transfer permitted under the previous sentence, the transferor shall cause the transferee to accede to this
Agreement and to assume any and all obligations and liabilities of the transferring Shareholder under this Agreement (or in case of a partial transfer, a proportionate part of the obligations and liabilities). Upon the accession by the transferee,
the transferor shall be released from its liabilities assumed by the transferee. In addition, LSP III Omni and LSP IV are entitled to transfer all of their Shares to a newly incorporated legal entity under the same (ultimate) management as LSP III
Omni and LSP IV (and rewind this transfer), provided that (for the avoidance of doubt) LSP III Omni and LSP IV will in all circumstances remain to be treated as separate parties and shareholders for the purpose of Clause 3.4.4 of this Agreement. In
order to benefit or continue to benefit from the participation exemption benefits provided by Dutch tax law, each Shareholder (the “Transferor”) shall be permitted to transfer its Shares to a legal person (the
“Transferee”) that may or may not be an existing Shareholder, provided that the indirect and beneficial ownership of the relevant Shareholders shall not change. In case of a transfer permitted under the previous sentence, and if the
Transferee is not an existing Shareholder, the Transferor shall cause the Transferee and the Transferee shall be required to accede to this Agreement and to assume any and all obligations and liabilities of the Transferor under this Agreement.

 In case Shares are transferred in accordance with this Clause 6.2, neither the Right of First Refusal, nor the Tag-along Right
nor the Drag-along Right shall apply. 
  

	6.3	Right of First Refusal 

If at any time a Shareholder (“Proposed Seller”) shall have identified a bona fide third party (“Proposed
Purchaser”) who has proposed in writing to purchase and acquire Shares held by the Proposed Seller (“Offered Shares”), the Proposed Seller shall give notice in writing (“Sale Notice”) to the Company and all
other Shareholders (the “Offerees”) specifying: 
  

	 	(i)	the name of the Proposed Purchaser; 

  
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	 	(ii)	the number of Offered Shares; and 

  

	 	(iii)	the price and other terms and conditions of the proposed disposal of the Offered Shares to the Proposed Purchaser. 

The Sale Notice shall constitute an offer to the Offerees, to purchase the Offered Shares (the “Offer”). 

Upon receipt of the Sale Notice, the Offerees in the aggregate shall have the right to purchase all (and not fewer than all) of the
Offered Shares at the price and on the other terms and conditions set out in the Sale Notice (the “Right of First Refusal”), by serving a notice to the Proposed Seller, with a copy to the Company and all other Shareholders
(“Exercise Notice”). A qualified acceptance of the Offer, or a failure by the Offerees to timely accept the Offer, shall be deemed a rejection of the Offer. The procedure in relation to the Offer is set out below. 

Within a period of twenty-one (21) days following the date of receipt of the Sale Notice, each of the AB-Shareholders shall have the
first right to purchase the Offered Shares, by serving an Exercise Notice, stating the number of Shares he wishes to purchase. The receipt of an Exercise Notice from an AB-Shareholder shall constitute a binding agreement between the Proposed Seller
and the accepting AB-Shareholder, provided, however, that if the AB-Shareholders in the aggregate wish to purchase more than the number of Offered Shares, the Offered Shares shall be allocated first to the accepting B-Shareholders (and among them in
proportion to the number of Class B Shares held by them), and only if thereafter any Offered Shares remain such remaining Offered Shares shall be allocated to the accepting A-Shareholders (and among them in proportion to the number of Class A
Shares held by them). An accepting AB-Shareholder cannot be allocated more Shares than he wished to purchase in accordance with his Exercise Notice. 
 If the AB-Shareholders in the aggregate have served Exercise Notices in relation to fewer than all Offered Shares, each AB-Shareholder who has already served an Exercise Notice shall have the right to
purchase the Offered Shares for which no Exercise Notices have been received (the “Remaining Shares”) by serving an Exercise Notice within a fourteen (14) days’ period following lapse of the previously mentioned twenty-one
(21) days’ period, stating the number of Remaining Shares he wishes to purchase. 

  
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 In case the relevant Offerees in the aggregate wish to purchase all Remaining Shares,
the receipt of the Exercise Notices shall constitute a binding agreement between the Proposed Seller and each of the accepting Offerees. 
 If the relevant Offerees in the aggregate wish to purchase more than the number of Remaining Shares, the Remaining Shares shall be allocated among them in proportion to the number of Shares held by them,
provided, however, that the Remaining Shares shall be allocated first to the accepting B-Shareholders (and among them in proportion to the number of Class B Shares held by them), and only if thereafter any Remaining Shares remain such Shares shall
be allocated to the accepting A-Shareholders (and among them in proportion to the number of Class A Shares held by them), provided always, however, that an accepting Offeree cannot be allocated more Shares than he wished to purchase in
accordance with his Exercise Notice. 
 If pursuant to the above the Offerees in the aggregate have served Exercise Notices in
relation to fewer than all Remaining Shares within the fourteen (14) days’ period, each of the Offerees other than the AB-Shareholders shall have the right to purchase the Remaining Shares for which no Exercise Notices from the
AB-Shareholders have been received by serving an Exercise Notice within a seven (7) days’ period following lapse of the previously mentioned fourteen (14) days’ period, stating the additional number of Offered Shares he wishes to
purchase, provided however, that if the relevant Offerees in the aggregate wish to purchase more than the number of such Remaining Shares, such Remaining Shares shall be allocated among them in proportion to the number of Shares held by them,
provided, however, that any Exercise Notices by O-Shareholders shall have priority over those of Common-Shareholders, and provided further that an accepting Offeree cannot be allocated more Shares than he wished to purchase in accordance with his
Exercise Notice. 
 If pursuant to the above, the Offerees have submitted Exercise Notices in relation to all and not fewer than
all of the Offered Shares, the transfer of the Offered Shares to the accepting Offerees shall take place no later than twenty-one (21) days from the final date on which acceptance notices may be sent and the requested approval as mentioned in
article 13 of the New Articles shall be given by the Class AB Meeting. 
 If pursuant to the above, the Offerees have not, or not
timely, submitted Exercise Notices in relation to all and not fewer than all of the Offered Shares, the Right of First Refusal and all Exercise Notices shall lapse and become void. In such event, the Proposed Seller shall be free to transfer the
Offered Shares to the Proposed Purchaser at the price and on the terms and conditions set out in the Sale Notice, subject, however, to the Tag-along Right. 

  
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	6.4	Tag-along Right 

 In the
event that a Proposed Seller, with due observance of the Right of First Refusal pursuant to which, however, the Offered Shares are not purchased by the Offerees, wishes to transfer the Offered Shares to the Proposed Purchaser, each of the holders of
Preferred Shares (the “P-Shareholders”) shall have the right to require the Proposed Purchaser to purchase from it a proportionate number of Shares (the ‘‘Tag-along Right”), and the Proposed Seller shall ensure
that the Proposed Purchaser shall be prepared to buy a proportionate number of Shares from such P-Shareholders, at the price and on the other terms and conditions set out in the Sale Notice, provided, however, that the P-Shareholders shall have the
right to convert any of their Class B Shares, Class A Shares and/or Class O Shares into Common Shares prior to transferring their Shares to the Proposed Purchaser, and provided further that the number of Shares to be sold by the Proposed Seller
and each of the P-Shareholders exercising the Tag-along Right shall be proportionate to the equity investment into the Company by each of such Parties. The price and terms and conditions of such sale shall be identical in every respect and no
further agreements, arrangements or understandings of any nature (for example “kick-back arrangements”) shall be made between the Proposed Purchaser and the Proposed Seller. 

If a P-Shareholder wishes to exercise its Tag-along Right, it shall serve on the Proposed Seller and the Proposed Purchaser a written
notice (the “Tag-along Notice”) within ten (10) business days from the date that the Right of First Refusal has been fully observed. 
 If a P-Shareholder has served an Exercise Notice or has failed to serve its Tag-along Notice within said ten (10) business days’ period, it shall be deemed to have waived its Tag-along Right.

  

	6.5	Drag-along Right 

 In the
event a Proposed Purchaser has been identified who is willing to acquire all Shares from all Shareholders under terms and conditions accepted by a resolution of the Class AB Meeting, the Proposed Seller will have the right (the “Drag Along
Right”) to require the remaining Shareholders to sell and transfer their Shares to said Proposed Purchaser under the terms and conditions accepted by the Class AB Meeting, without prejudice, however, to the liquidation rights set forth in
Clause 3, and provided that the consideration shall be paid in cash, cash equivalents and/or marketable securities (the “Proposed Sale”). 
 If the Drag-along Right is exercised, a notice shall be served to the Company (“Drag Along Notice”), specifying the name of the Proposed Purchaser and the

  
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price and other terms and conditions of the Proposed transaction. The Company shall inform the other Shareholders of the Drag Along Notice and its contents. Consequently, the Proposed Seller
shall be entitled to transfer (all but not fewer than all of) the Offered Shares to the Proposed Purchaser at the price and on the other terms and conditions set out in the Drag Along Notice, and the other Shareholders hereby grant power of attorney
to the Company in order to simultaneously sell and transfer all their Shares on the same terms and conditions to the Proposed Purchaser. 
 Notwithstanding the foregoing, a Shareholder will not be required to comply with this Clause 6.5 unless: 
  

	 	(i)	the liability for indemnification, if any, of such Shareholder in the Proposed Sale and for the inaccuracy of any representations and warranties made by the Company or
its Shareholders in connection with such Proposed Sale, is several and not joint with any other Person (except to the extent that funds may be paid out of an escrow established to cover breach of representations, warranties and covenants of the
Company as well as breach by any Shareholder of any of identical representations, warranties and covenants provided by all Shareholders), is pro rata in proportion to, and does not exceed, the amount of consideration paid to such Shareholder in
connection with such Proposed Sale; 

  

	 	(ii)	liability shall be limited to such Shareholder’s applicable share (determined based on the respective proceeds payable to each Shareholder in connection with such
Proposed Sale in accordance with the liquidation rights set forth in Clause 3) of a negotiated aggregate indemnification amount that applies equally to all Shareholders but that in no event exceeds the amount of consideration otherwise payable to
such Shareholder in connection with such Proposed Sale, except with respect to claims related to fraud by such Shareholder, the liability for which need not be limited as to such Shareholder; and 

 

	 	(iii)	upon the consummation of the Proposed Sale, (a) each holder of each class or series of the Company’s Shares will receive the same form of consideration for
their shares of such class or series as is received by other holders in respect of their shares of such same class or series of Shares. 

  

	6.6	Transfer of Shares and New Articles 

 The Shareholders shall at all times exercise their voting rights with respect to their Shares and shall at all times exercise or waive any other rights under the New Articles in such manner as to give
full effect to this Clause 6. Pursuant to 

  
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the foregoing, the AB Shareholders shall apply the transfer restrictions contained in the New Articles (approval by the Class AB Meeting) in a way consistent with and giving effect to this Clause
6. 
 If, for example, a transfer qualifies as a Permitted Transfer, the Class AB meeting shall approve the transfer to the
permitted transferee and the Right of First Refusal, Tag-Along Right and Drag-Along Right are not applicable. 
 If, for
example, the Offerees have not served an Exercise Notice, the Class AB Meeting shall approve the transfer of the Offered Shares to the Proposed Purchaser in accordance with the Sale Notice; if, however, the Offerees have served Exercise Notices in
relation to all Offered Shares, the Class AB Meeting shall approve the transfer of the Offered Shares to the Offerees in accordance with the Exercise Notice and at the same time shall resolve not to approve the sale to the Proposed Purchaser and
shall simultaneously inform the Proposed Seller that the Offered Shares shall be sold and transferred to the Offerees in accordance with the Offer, and the Proposed Seller shall waive any right to have the value of the Offered Shares determined by
independent experts. 
 Any transfer of Shares permitted under this Agreement shall be formalized by means of a notarial
transfer deed, in accordance with Dutch law and the New Articles. 
  

	6.7	Transfer of shares in personal holding companies 

 The Parties acknowledge that for the purposes of this Clause 6, the relevant incorporator / shareholder of Arriwan BV or of Dordtwijck BV shall be deemed to be a shareholder of the Company, in such manner
that in case any of these persons (the “Indirect Shareholders”) wishes to transfer his or her shares or indirect interest in his/her relevant personal holding company, respectively, each of the other Shareholders may require the
relevant direct Shareholder of the Company (the relevant personal holding company) to offer for sale such number of Shares that corresponds to the percentage of the issued share capital of the relevant personal holding company that is being
transferred, and provided further that in case more than 50% of the shares in the personal holding company, whether pursuant to one transaction or a series of transactions, shall be held by other persons than the present Indirect Shareholder(s), the
relevant direct shareholder shall be under the obligation to offer all his Shares to the other Shareholders. 
 If for
example, Mr. De Boer wishes to transfer 30% of his shares in Arriwan BV, any of the Shareholders may require Arriwan BV to offer 

  
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30% of its Shares for sale to the other Shareholders, provided, however, that in case Mr. De Boer wishes to transfer 51% of his shares in Arriwan BV, Arriwan BV may be required to offer all
its Shares for sale. 
 In addition, the Tag-along Right shall apply mutatis mutandis, such that the relevant purchaser may
be required to purchase a pro rata number of Shares from the other Shareholders. 
 Any offer of Shares pursuant to the above
shall occur in accordance with Clause 6.3 (Right of First Refusal). 
 The Parties acknowledge that in the event of a permitted
transfer as described in Clause 6.2, this Clause 6.7 shall not apply; in such case this Clause 6.7 shall apply to the successive personal holding company. 
  

	7	MANAGEMENT BOARD 

  

	7.1	Management Board. Number of Managing Directors 

 The management of the Company shall be entrusted with the Management Board consisting of one or more Managing Directors. The Company shall be represented by two Managing Directors acting jointly. The
Management Board shall be constituted and regulated in accordance with articles 14 up to and including 21 of the New Articles. 

The Parties acknowledge that as per the date hereof, the Management Board shall consist of the following Managing Directors: 

 

	 	•	 	 Mr. Hans Schikan (Chief Executive Officer); 

  

	 	•	 	 Mr. Berndt Modig, (Chief Financial Officer); 

  

	 	•	 	 Mr. Luc Dochez, (Chief Business Officer and Senior Vice-President Business Development); and 

 

	 	•	 	 Mr. Giles Campion, (Chief Medical Officer and Senior Vice-President R&D). 

The Company shall have a management team, which shall be responsible for the day-to-day management of the Company. The management team
shall consist of the Managing Directors, as well as such other key employees of the Company as designated by Management Board and approved by the Supervisory Board. The management team shall report directly or indirectly to the Chief Executive
Officer. Any rules and regulations governing said management team shall be prepared by the Management Board and shall be subject to the prior approval of the Supervisory Board. 

  
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	7.2	Appointment of the Managing Directors 

 The General Meeting, upon a non-binding recommendation by the Supervisory Board as set forth in article 15 of the New Articles, shall appoint the Managing Directors. 

 

	7.3	Term 

 The Managing
Directors shall be appointed for an indefinite period, unless the General Meeting resolves otherwise. 
  

	7.4	Remuneration 

 The
Supervisory Board shall determine the remuneration of the Managing Directors. The Managing Directors shall be entitled to be paid or reimbursed for their reasonable expenses incurred in the discharge of their duties as Managing Directors, subject to
production of all necessary vouchers and receipts. 
  

	7.5	Decision making 

Voting at Management Board meetings. 
 At each meeting of the Management Board and in respect of each resolution proposed to the Management Board at a meeting of the Management Board each Managing Director shall have one (1) vote. All
resolutions of the Management Board at a meeting of the Management Board shall be passed by an absolute majority of the votes cast, unless this Agreement and/or the New Articles require a greater majority. 

Tie in votes. 
 In the case of an equality of votes at any meeting of the Management Board in relation to a proposal on a matter requiring the absolute majority of the votes, each Managing Director shall be authorized to
refer the matter to the Supervisory Board. The relevant proposal shall be deemed adopted in case the Supervisory Board adopts a resolution to that effect. 
 Resolutions without holding a meeting. 
 The Management Board may adopt
resolutions without holding a meeting of the Management Board, provided that such resolutions shall only be validly passed if the text of the resolution has been signed by all Managing Directors. 

Further rules and regulations. 
 Subject to the prior approval from the Supervisory Board, the Management Board may adopt further rules and regulations as to its decision making process, which rules and regulations may, without
limitation, regard the frequency and location of meetings and the notice period. 

  
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	7.6	Approvals by the Supervisory Board 

 Any resolution by the Management Board with respect to the matters described below or any other matters the Supervisory Board deems necessary in its own discretion (the “Supervisory Board Approval
Matters”) shall require the approval by the Supervisory Board, in the absence of which approval any such resolution shall be void: 
  

	 	(a)	implementation of an Employee Stock Option Plan and granting any rights thereunder; 

 

	 	(b)	appointment of employees with a yearly base salary in excess of EUR 125,000, or a lower amount determined by the Supervisory Board; 

 

	 	(c)	disposal or acquisition of any securities in the capital of any other company or establishment of any new branch or subsidiary of the Company; 

 

	 	(d)	exercise of voting rights in the shareholders’ meeting of any subsidiary or affiliate, if any; 

 

	 	(e)	conduct of any litigation with a financial interest exceeding EUR 150,000 on behalf of the Company, or a lower amount determined by the Supervisory Board;

  

	 	(f)	entering into a guarantee or indemnity or otherwise committing the Company for an amount exceeding 100,000, or a lower amount determined by the Supervisory Board (other
than in the ordinary course of business); 

  

	 	(g)	provision of any loan or advance or any credit (other than in the ordinary course of business) to any person; 

 

	 	(h)	changing the accounting policies; 

  

	 	(i)	any transaction relating to intellectual property rights owned, co-owned or controlled by the Company and/or any of the Subsidiaries (including without limitation, the
intellectual property rights licensed by the Company and/or any of the Subsidiaries from Leiden University Medical Centre); 

  

	 	(j)	any transaction relating to intellectual property rights owned, co-owned or controlled by a third party (including, without limitation, inlicensing);

  
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	 	(k)	any material modification to the license agreement between the Company and/or any of the Subsidiaries and Leiden University Medical Centre; 

 

	 	(l)	undertaking any such legal acts as will be determined and clearly defined by the Supervisory Board and notified to the Management Board in writing;

  

	 	(m)	engagement in any new line of business or jurisdiction where the Company is managed and controlled; 

 

	 	(n)	approval of the annual budget and any non-budgeted expenses in excess of EUR 150,000, or a lower amount determined by the Supervisory Board; 

 

	 	(o)	entering into any transactions with related parties; 

  

	 	(p)	entering into any agreements, contracts or arrangements that are not of an at arm’s length nature; and 

 

	 	(q)	establishment of a scientific advisory board and the appointment, dismissal and remuneration of its members. 

 

	7.7	Approvals by the Class AB Meeting, the Class A Meeting, and/or the Class B Meeting 

Any resolution by the Management Board with respect to the matters described below (the “Class AB Meeting Approval
Matters”) shall require the approval by the Class AB Meeting, whether or not such matter is also a Supervisory Board Approval Matter, in the absence of which approval any such resolution shall be void, and without prejudice (to the extent
applicable) to the rights of other Shareholders or of the General Meeting: 
  

	 	(a)	issuance of any securities ranking senior to or pari passu with (as to dividend rights, redemption rights, liquidation preference and other rights) or convertible into
securities ranking senior to or pari passu with the Class A Shares, including the issuance of subordinated debt; 

  

	 	(b)	declaration and/or payment of any and all dividends by the Company; 

  

	 	(c)	entering into any merger, consolidation, recapitalisation, change of control, or sale of all or substantially all of the assets of the Company;

  

	 	(d)	undertaking of any filing for bankruptcy or insolvency by or against the Company; 

  
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	 	(e)	increase of the authorised number of Shares; 

  

	 	(f)	engagement in any transaction that constitutes a deemed dividend according to the relevant tax laws; 

 

	 	(g)	the application by the Company to have Shares listed at a stock exchange as well as any actions to prepare for such listing-, and 

 

	 	(h)	the terms and conditions of a purchase by a Proposed Purchaser in the event of a drag-along situation as described in Clause 6.5. 

Any amendment to the articles of association/charter/bylaws of the Company or any other action that adversely impacts or is likely to
adversely impact the Class A Shares and/or the Class B Shares, including by way of merger, consolidation or otherwise, or any transaction and/or resolution by the Management Board that will or may have an adverse effect on the specific rights
of the A-Shareholders and/or the B-Shareholders shall require the approval of the meeting of the holders of Class A Shares (the “Class A Meeting”) (if the Class A Shares are affected) and/or Class B Meeting (if the Class B
Shares are affected), it being understood, however, that if a matter is subject to approval of the Class AB Meeting as mentioned under a. up to and including h. above and such approval has been obtained, no approval from the Class A Meeting
and/or Class B Meeting, shall additionally be required. 
  

	7.8	Implementation at subsidiaries 

 The Parties shall cause the Company to implement such procedures at the level of the Subsidiaries and any future subsidiaries in such manner that each such company cannot resolve upon any of the
Supervisory Board Approval Matters and/or Class AB Meeting Approval Matters without the prior written approval of the Supervisory Board, the Class A Meeting, the Class B Meeting, and Class AB Meeting, respectively, as contemplated by Clauses
7.6 and 7.7. 
  

	8	SUPERVISORY BOARD 

  

	8.1	Supervisory Board. Members 

The Supervisory Board of the Company shall supervise the policies of the Management Board and shall have all such other tasks as assigned
to it in this Agreement and/or the New Articles. The Supervisory Board shall be constituted and regulated in accordance with articles 22 up to and including 27 of the New Articles. 

  
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 The Supervisory Board shall consist of (up to) eight (8) members, appointed in
accordance with the following rules: 
  

	 	•	 	 one (1) member appointed upon the binding nomination of LSP III Omni (unless it holds only Common Shares in the capital of the Company, in which
case it shall not have a right of binding nomination); 

  

	 	•	 	 one (1) member appointed upon the binding nomination of ABV Holdings NV (unless it holds only Common Shares in the capital of the Company, in
which case it shall not have a right of binding nomination); 

  

	 	•	 	 one (1) member appointed upon the binding nomination of Idinvest Partners (unless it holds only Common Shares in the capital of the Company, in
which case it shall not have a right of binding nomination); 

  

	 	•	 	 one (1) member appointed upon the binding nomination of Gimv NV (unless it holds only Common Shares in the capital of the Company, in which case
it shall not have a right of binding nomination); 

  

	 	•	 	 one (1) member appointed upon the binding nomination of NEA (unless it holds only Common Shares in the capital of the Company, in which case it
shall not have a right of binding nomination); and 

  

	 	•	 	 up to three (3) industry experts, who shall be appointed by the General Meeting upon the binding nomination of the Supervisory Board.

 The Parties acknowledge that as per the date hereof the Supervisory Board shall consist of the following
persons: 
 1. Martijn Kleijwegt (nominated by LSP III Omni); 

2. Stephen Bunting (nominated by ABV Holdings NV); 
 3. Rémi Droller (nominated by Idinvest); 
 4. Jim Van heusden (nominated by
Gimv NV); 
 5. David Mott (nominated by NEA); 
 6. Daniël Jan Ellens (industry expert); 
 7. Peter Neville Goodfellow
(industry expert); and 
 8. [industry expert vacancy to be fulfilled] 

The Parties acknowledge that as per the date hereof, Mr. Daniël Jan Ellens is the chairman, it being understood, however, that
the chairman of the Supervisory Board can be replaced by another industry expert, upon the agreement by the Supervisory Board. 

The Supervisory Board shall at its own discretion have the right to invite experts that may attend meetings of the Supervisory Board.

  
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	8.2	Nominations and Shareholder voting 

 The Shareholders shall at all times exercise their voting rights with respect to their Shares in such a manner that the person nominated in accordance with this Clause (or the first person on the list, in
case the nomination consists of more than one person) shall be appointed. Furthermore, the person nominated by a company body shall be suspended and removed by the General Meeting at that company body’s request. 

Without prejudice to the relevant Shareholders’ ultimate and own discretion to bindingly recommend a person for appointment in
accordance with the above, each of the Parties undertakes to reasonably consult the other Parties prior to making a recommendation in order to reach an agreement on the persons to be appointed. 

 

	8.3	Term 

 The Supervisory
Directors will be appointed for a term of (approximately) one year, running from an annual general meeting of shareholders until the next annual general meeting of shareholders. Each Supervisory Director may be re-appointed. 

 

	8.4	Remuneration 

 All
Supervisory Directors shall be entitled to be paid or reimbursed for their reasonable expenses incurred in attending meetings of the Supervisory Board, including committee meetings, or otherwise acting for the Company, subject to production of all
necessary vouchers and receipts. 
 The General Meeting shall determine the remuneration of the Supervisory Directors. In
principle, said remuneration shall be an equal amount for all members, provided that any deviation from such equal amount shall be approved by the Class AB Meeting. 
 Each member of the Supervisory Board shall be indemnified by the Company for any claims in accordance with the form of the indemnity agreement attached hereto as Schedule 8.4. 

 

	8.5	Decision making by the Supervisory Board 

 Meetings. 
 The Supervisory Board shall meet as often as it sees fit but in
any case at least once every two months to discuss the affairs of the Company and the performance of the Management Board. The meetings may be held by telephone and videoconference if so agreed by all members of the Supervisory Board. 

  
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 Voting at Supervisory Board meetings. 

At each meeting of the Supervisory Board and in respect of each resolution proposed to the Supervisory Board at a meeting of the
Supervisory Board each Supervisory Director shall have one (1) vote. All resolutions of the Supervisory Board at a meeting of the Supervisory Board shall be passed by an absolute majority of the votes cast, unless this Agreement and/or the New
Articles require a greater majority. 
 Tie in votes. 

In the case of an equality of votes at any meeting of the Supervisory Board in relation to a proposal on a matter requiring the absolute
majority of the votes, each Supervisory Director shall be authorized to refer the matter to and for resolution by the General Meeting. 
 Notice of meetings. 
 Unless waived by all of the Supervisory Directors, not
less than ten (10) business days’ notice of all meetings of the Supervisory Board shall be given to each Supervisory Director and shall be accompanied by an agenda of the business to be transacted at such meeting together with all papers
to be circulated or presented to the same. Within no more than ten (10) business days after each such meeting, a copy of the minutes of that meeting shall be delivered to each Supervisory Director. 

Resolutions outside a meeting. 
 The Supervisory Board may adopt resolutions outside a meeting, provided that such resolutions shall only be validly passed if all Supervisory Directors have signed the text of the resolution. 

Committees. 
 The Supervisory Board may install committees from among its members and determine the tasks of each committee, subject, however, to the prior approval of the Class AB Meeting. 

 

	8.6	Information for the benefit of the Supervisory Board 

 The Management Board shall provide members of the Supervisory Board with the following information regarding the Company: 
  

	 	(a)	Notices, agendas and minutes of meetings of the Management Board and the management team, Supervisory Board and the Shareholders Meetings; 

  
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	 	(b)	Quarterly reports (balance sheet, profit and loss statement, cash-flow statements, explanatory notes and a report of the Management Board, including estimates for the
remaining part of the current financial year) within thirty (30) days of the end of the respective quarter, 

  

	 	(c)	Annual budgets (no later than thirty (30) days before the end of the current financial year, to be submitted for approval), as well as all such other information
as is reasonably requested; and 

  

	 	(d)	All such other information as the Supervisory Baard may reasonably request from time to time. 

 

	9	GENERAL MEETING OF SHAREHOLDERS 

  

	9.1	General Meeting 

 The
General Meeting shall have all powers that are not specifically assigned to the meeting of Common-Shareholders (the “Common Meeting”), the Class AB Meeting, the Management Board or the Supervisory Board. The General Meeting will be
regulated in accordance with articles 33 up to and including 43 of the New Articles. 
  

	9.2	Shareholder delegates 

Each Shareholder being a legal entity shall give notice to the other Shareholders which natural person or persons shall represent it for
purposes of the meetings of the Shareholders. 
  

	9.3	Decision making 

Voting rights per Share. 
 Each fully paid up Share will entitle the registered holder thereof to vote on all matters to be decided by the General Meeting. Unless specifically agreed otherwise in this Agreement or in the New
Articles, the General Meeting shall adopt its resolutions by a simple majority of the votes cast, in a meeting in which at least 50% of all issued Shares are represented, in case such quorum is not met, a second meeting may be held in which
resolutions may be passed on the relevant agenda items, without a quorum being required. 
 Resolutions to be adopted by the
meeting of the holders of a particular class of Shares or a combination of two or more classes of Shares shall be adopted by a majority of at least 60% of the votes cast, in a meeting in which at least 50% of all issued Shares of the relevant class
are represented. In case such quorum is not met, a second meeting may he held in which resolutions may be passed on the relevant agenda items, with said 60% majority but without a quorum being required. 

  
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 Resolutions outside a meeting. 

The General Meeting may adopt resolutions outside a meeting if the proposed written resolution is circulated to and approved in writing by
all of the Shareholders. Resolutions may be circulated by facsimile. Such resolutions shall constitute a valid and binding resolution of the Shareholders when signed by the last to sign of all the Shareholders. 

Shareholder voting. 
 The Shareholders agree that when called to a vote at the General Meeting they shall vote in accordance with the terms and conditions of this Agreement and to ensure that none of the Parties to this
Agreement shall be deprived of its rights pursuant to this Agreement. 
  

	9.4	Information rights 

 As
long as an AB-Shareholder (provided that it is not a competitor of the Company or otherwise holds conflicting interests) continues to hold at least 5% of the outstanding share capital, it will be granted access to Company facilities and personnel
during normal business hours and with reasonable advance notification. 
 The Company will deliver to each holder of Preferred
Shares: 
  

	 	(i)	audited financial statements within one hundred and twenty (120) days after the end of the calendar year; 

 

	 	(ii)	quarterly financial statements within thirty (30) days after such period, with a report on the progress in the development of the Business, and other information
as determined by the Supervisory Board; 

  

	 	(iii)	fifteen (15) days prior to the end of each fiscal year, a comprehensive operating budget forecasting the Company’s revenues, expenses, and cash position on a
month-to-month basis for the upcoming fiscal year; and 

  

	 	(iv)	promptly following the end of each quarter an up-to-date capitalisation table, certified by the Management Board. 

The foregoing provisions will terminate upon an IPO. 

  
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	10	EXIT STRATEGY. REGISTRATION RIGHTS 

  

	10.1	Exit strategy 

 The object
of the Business is to develop a growing and prosperous company, so that the interested Shareholders will be able to dispose of, on favourable terms, in approximately two (2) to four (4) years from January 2012 their interests in the
Company and its subsidiaries (the “Exit”). An Exit could, for example, occur by means of: (i) a sale of Shares to a Third Party, or (ii) a public offering of Shares. Furthermore, the Exit may also occur by the Company
disposing of its interest in an intermediate holding Company or a public offering of shares in an intermediate holding Company, after which the proceeds received by the Company shall be distributed to the Shareholders. The Parties shall cooperate in
order to optimise the proceeds of an Exit, without prejudice, for the avoidance of doubt, to the entitlement of the relevant Shareholders to receive the relevant liquidation proceeds, if the proceeds of the Exit so allow. 

 

	10.2	Registration Rights 

 In
the event that the Company consummates a public offering of its Shares at a US stock exchange, the Shareholders will enter into a registration rights agreement pursuant to which the AB-Shareholders will have registration rights including two demand
registration rights, unlimited “piggy-back” registration rights, S-3 registration rights, transfer of registration rights, proportionate underwriter cut-backs, and other typical registration rights. 

The AB-Shareholders will agree in connection with the public offering, if requested by the managing underwriter, not to sell or transfer
any Shares of the Company (excluding shares acquired in or following the public offering) for a period of up to one hundred and eighty (180) days following the public offering provided all managing directors and supervisory directors of the
Company and Shareholders holding two and one half of a per cent (2.5%) or more of the issued and outstanding share capital of the Company agree to the same lock-up. 
 In the event that the Company consummates a public offering of its Shares on a stock exchange outside the US, then the AB-Shareholders will be entitled to registration rights equivalent to the rights and
obligations contained in this provision (or as equivalent as possible given differences in applicable laws and regulations). 

  
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	11	INCENTIVE PLANS 

  

	11.1	Incentive Plans 

 Pursuant
to the Incentive Plans, the Company’s managing directors, supervisory directors, employees, consultants and other eligible persons as described in the Incentive Plans are and may further be granted the right to acquire depositary receipts for
Incentive Shares, whereby legal title to the Incentive Shares shall be held by Stichting AK, which shall issue (non-voting) depositary receipts to the managing directors, supervisory directors, and employees. 

The Incentive Plans as currently in force consist of the 2007 Employee Stock Option Plan and the 2010 Equity Incentive Plan. 

Under said 2010 Equity Incentive Plan, which was adopted at the Company’s 2010 annual general meeting of shareholders (the
“2010 AGM”), the Company may also grant the relevant eligible persons with stock appreciation rights. Restricted stock, restricted stock units and other stock based awards. At the 2010 AGM, certain changes have been approved in
relation to said 2007 Employee Stock Option Plan, the implementation of which changes is subject to the matters discussed at the 2010 AGM. 
  

	11.2	Number of Incentive Shares 

Pursuant to the Incentive Plans: 
 after the Tranche 1 Issue: 
  

	 	(a)	the Company has a pool of rights to acquire a total of up to 1,991,454 depositary receipts for Common Shares to be issued by the Company (granted and not yet granted
rights, including the agreed extension with 250,000 depositary receipts), 

 and after the Tranche 2 Issue:

  

	 	(b)	the Company has a pool of rights to acquire a total of up to 2.241.454 depositary receipts for Common Shares to be issued by the Company (granted and not yet granted
rights, including the agreed extension with 250,000 depositary receipts), 

 of which rights to acquire a total of
1,418,232 depositary receipts have been granted to employees but not yet exercised, whilst rights to acquire a total of 823,222 depositary receipts are still available. 

  
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	11.3	Stichting AK Board 

 The
board of directors of the Stichting AK shall be appointed by the Supervisory Board and shall comprise of (i) a person appointed upon the nomination of the Common Meeting, (ii) a person appointed upon the nomination of the Class AB Meeting
and (iii) a member of the management team. The board of directors may adopt resolutions by a simple majority of the votes cast, which majority shall at least include the board member nominated by the Class AB Meeting. 

As per the date hereof, the board of directors of the Stichting AK consists of the following persons: 

1. Ruut van Dam (nominated by the Common Meeting); 
 2. Martijn Kleijwegt (nominated by the Class AB Meeting); and 
 3. Richard Holslag
(management team member). 
  

	12	VARIOUS 

  

	12.1	Giving effect to this Agreement 

 Undertaking by all Parties 
 The Parties shall exercise all voting and other
rights and powers available to them so as to give effect to the provisions of this Agreement to the fullest extent possible under law. The Parties waive, and shall from time to time waive, any rights that they may have, under the New Articles or
otherwise, which may be inconsistent with the terms of this Agreement, and to the extent such rights cannot be waived, they shall exercise such rights in a way consistent with and to the fullest extent possible under the law in order to effect to
this Agreement. 
 The Parties undertake, among other things, to do everything within their power, to the fullest extent possible
under the law, to procure that the Management Board and the Supervisory Board shall be composed and constituted in accordance with this Agreement, and that the Management Board and the Supervisory Board shall exercise their powers in a manner
consistent with the objects, wishes and intentions of the Shareholders as expressed in this Agreement. 
 Undertakings by the
Company 
 By executing this Agreement, the Company undertakes to the Shareholders that to the fullest extent permitted under
law, it shall at all times: 
  

	 	(a)	 implement such procedures at the level of all of its Subsidiaries and its future subsidiaries, if any, in such a way that each such company

  
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cannot resolve upon any of the Supervisory Board Approval Matters and/or Class AB Meeting Approval Matters without the prior written approval of the Supervisory Board and Class AB Meeting,
respectively, as contemplated by Clauses 7.6 and 7.7; 

  

	 	(b)	generally act in accordance with the terms of this Agreement, including but not limited to obtaining the required approvals in relation to the Supervisory Board
Approval Matters and/or Class AB Meeting Approval Matters; and 

  

	 	(c)	indemnify to the fullest extent possible under law and maintain a D&O insurance (covering at least an amount of EUR two (2) million per occurrence), for all
Supervisory Directors, in connection with the performance by them of their tasks as Supervisory Director of the Company. 

  

	12.2	Conflict with New Articles 

The Parties agree that in the event of a conflict between the terms of this Agreement and the New Articles, the provisions of this
Agreement shall prevail as far as possible between the Parties. The Parties undertake to cause the New Articles to be consistent with this Agreement, and to the extent such is not the case, all Parties undertake, at any Party’s first request,
to amend the New Articles in order to make them consistent with this Agreement. If pursuant to law such amendment is not possible, the Parties shall use all their rights to the fullest extent possible in such manner as to give effect to this
Agreement. The New Articles shall not be amended in any way if such amendment would cause any explicit rights of the Parties under this Agreement (particularly the rights to dividends and rights to liquidation proceeds as described in Clause 3.4) to
be terminated or negatively affected. 
  

	12.3	Entire Agreement 

 This
Agreement (together with any documents referred to herein or executed contemporaneously in connection herewith) constitutes the whole agreement between the Parties and supersedes any previous agreements or arrangements between them relating to the
subject matter of this Agreement. 
  

	12.4	Severability 

 If any
provision or part of a provision of this Agreement shall be, or be found by any authority or court of competent jurisdiction to be invalid or unenforceable, such invalidity or unenforceability shall not affect the other provisions or parts of such
provisions of this Agreement, all of which shall remain in full force and effect, and the Parties hereto shall consult with each other in order to replace the invalid or unenforceable provisions by provisions which comply with the objects, wishes
and intentions of the Parties as expressed in this Agreement. 

  
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	12.5	Termination 

 The Parties
acknowledge and agree that this Agreement shall only terminate in the event of an Exit by all Shareholders, and with respect to individual Parties, from the date such Party ceases to hold any Shares. 

 

	12.6	Consequences of termination. Survival 

 Upon termination of this Agreement the provisions of this Agreement shall cease to have effect save in relation to any existing claims which may have arisen prior to termination, and provided, however,
that Clause 12.7 (Notices), Clause 12.9 (Confidentiality), Clause 12.15 (Governing Law) and Clause 12.16 (Dispute Resolution) shall survive the termination. 
  

	12.7	Notices 

 Each notice,
demand or other communication given or made under this Agreement shall be in writing and delivered or sent to the relevant Party at its address set out below (or such other address or fax number as the addressee has specified to the other Parties by
five (5) days’ prior written notice), provided, that notices shall be delivered by international express courier (such as DHL): 
 To: Prosensa Holding B.V. 
 J.H. Oortweg 21 

2333 CH Leiden 

Attn.: Managing Director 
 To: ABV IV Holdings N.V. 
 Landhuis Groot Kwartier 

Groot Kwartierweg 12, Curacao 
 Attn. Managing Director 

  
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 CC to: 
 Abingworth Management Ltd. 
 Princes House, 38 Jermyn Street 

London SW1Y 6DN, UK 
 Attn.: General Counsel 
 To: FCPI Capital Croissance 3 

c/o Idinvest Partners 
 117 Avenue des Champs-Elysées 
 75008 Paris, France 

and to: 
 KLS
Partners 
 507, rue de Monttessuy 
 F-75340 Paris cedex 07 
 France 

contact@kls-partners.com 
 To: Gimv NV 
 Karel Oomsstraat 37 

2018 Antwerpen, Belgium 
 Attn.: Managing Director 
 To: Arriwan Holding B.V.  

Weper 6 
 8431 RH
Oosterwolde 
 Attn.: Managing Director 

  
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 To: Cure Duchenne 

3334 East Coast Hwy., # 157 
 Corona del Mar 
 CA 92625, United States of America 

Attn.: President 

To: Dordtwijck I B.V. 
 Groot Haesebroekseweg 49 
 2243 EE Wassenaar 

Attn.: Managing Director 
 To: LSP III Omni Investment Coöperatief U.A. 
 Johannes Vermeerplein 9

 1071 DV Amsterdam 
 Attn.: Managing Director 
 To: Coöperatief LSP IV U.A. 

Johannes Vermeerplein 9 
 1071 DV Amsterdam 
 Attn.: Managing Director 

  
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 To: MedSciences Prosensa Holding B.V. 

Beethovenstraat 300 
 1077 WZ Amsterdam 
 Attn.: Managing Director 

To: FCPI Allianz Innovation 8 
 c/o Idinvest Partners 
 117 Avenue des Champs-Elysées 

75008 Paris, France 
 and to: 
 KLS Partners 

507, rue de Monttessuy 
 F-75340 Paris cedex 07 
 France 

contact@kls-partners.com 
 To: FCPI Objectif Innovation Patrimoine 3 
 c/o Idinvest Partners

 117 Avenue des Champs-Elysées 
 75008 Paris, France 
 and to: 

KLS Partners 

507, rue de Monttessuy 
 F-75340 Paris cedex 07 
 France 

contact@kls-partners.com 

  
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 To: Adviesbeheer Gimv Life Sciences 2007 NV 

Karel Oomsstraat 37 
 2018 Antwerpen. Belgium 
 Attn.: Managing Director 

To: Charley’s Fund, Inc. 
 P.O. Box 83 
 Stockbridge 

MA 01230, United States of America 
 Attn.: President 
 To: Mrs. C.M. van den Brink 

Kruislaan 184 

1098 SK Amsterdam 
 To: Mr. G.J. Platenburg 
 Wijngaardenlaan 56 

2252 XR Voorschoten 
 To: Libertatis Ergo Holding B.V. 
 Rijnsburgerweg 10 

  
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 2333 AA Leiden 
 Attn.: Managing Director 
 To: Mrs. E.M.H.C. van Boom – Husken

 Pieter de Hoochlaan 14 
 2343 CR Oegstgeest 
 To: Mr. P.F. Ekhart 

Niersstraat 61-I 

1078 VJ Amsterdam 
 To: Stichting Administratiekantoor Prosensa Holding 
 Wassenaarseweg 72

 2333 AL Leiden 
 Attn. Managing Director 
 To: Mr. C.J.P.M. van Megen 

Kruislaan 184 

1098 SK Amsterdam 
 To: Mr. H.L. Heijneker 
 2244 Steiner Street 

San Francisco 

CA 941 15, United States of America 

  
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 To: Mr. D.J. Ellens 

Kriesbaumen 288 

CH 3157 Milken, Switzerland 
 To: New Enterprise Associates 
 c/o New Enterprise Associates 

1954 Greenspring Drive, Suite 600 
 Timonium MD 21093 
 Attn: Louis Citron 

Each Party will give written notice of any change in its address above to the other Parties. 

Any notice, demand or other communication so addressed to the relevant Party shall be deemed to have been delivered (a) if given or
made by letter, when actually delivered to the relevant address; or (b) if given or made by fax, when dispatched. 
  

	12.8	Restrictions on announcements 

 Each of the Parties, hereto undertake that it will not (save as required by law) make any announcement in connection with this Agreement or with respect to other Parties, unless the Investors and the
Company have given their written consent to such announcement (which consent may not be unreasonably and may be given either generally or in a specific case or cases and may be subject to conditions). 

 

	12.9	Confidential information 

Non-disclosure. 
 The Parties undertake that they shall treat as strictly confidential any and all data and information, whether provided orally, in writing or electronically, received or obtained by them or their
employees, agents or advisers as a result of entering into or performing this Agreement including information relating to the provisions of this Agreement, the negotiations leading up to this Agreement, the subject matter of this Agreement or the
business or affairs of each of the 

  
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Parties or the Company and its subsidiaries, whether or not marked or expressly stated as “confidential” (the “Confidential Information”) and that they will not at any
time hereafter make use of or disclose or divulge to any person any such Confidential Information and shall use their best endeavours to prevent the publication or disclosure of any such information. The Parties acknowledge that they have received
Confidential Information with regard to the Company and its subsidiaries during the review process and negotiations leading up to this Agreement and that such information may not be used for any other purposes. 

In addition, each Party confirms that it will respect and protect any rights of the Company and its future subsidiaries related to the
latter’s know-how, patents and other intellectual property rights (whether registered or unregistered). The previous sentence does not provide a right of action by any Party other than the Company. 

Exceptions. 
 The restrictions contained in this Clause 12.9 shall not apply so as to prevent the Parties from making any disclosure (i) to the Shareholders of the Company. (ii) with regard to customary
information to the investors of the Investors, (iii) as otherwise required by law, or as required by any securities exchange or supervisory or regulatory or governmental body pursuant to rules to which the relevant Party is subject; or from
making any disclosure to any professional adviser for the purposes of obtaining advice (provided always that the provisions of this Clause shall apply to and the Parties shall procure that they apply to, and are observed in relation to, the use or
disclosure by such professional adviser of the information provided to him) nor shall said restrictions apply in respect of any information which collies into the public domain other than by a breach of this Clause by any of the Parties. 

 

	12.10	Amendments 

 No amendment
or variation of this Agreement shall be effective unless it is made or confirmed in a written document signed by all of the Parties. 
  

	12.11	Conflicts of interests 

Notwithstanding anything contained in this Agreement, no Shareholder, Supervisory Director or Supervisory Board observer shall receive any
relevant Confidential Information from the Company as soon as he, or the person represented by him and/or at whose nomination/recommendation he has been appointed has a conflict of interest with the Company. Such conflicts of interest could arise,
for example, in case the person who nominated a Supervisory Director is involved in a potential acquisition of the Company or a substantial portion of its assets, or in case a Shareholder acquires an interest in another

  
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company that directly competes with the Company by using similar technology or wishes to set up a competing business by using similar technology as used by the Company. In case a (potential)
conflict of interest arises, the involved person shall report such fact to the Chairman of the Supervisor) Board and shall provide all relevant information. The Chairman of the Supervisory Board shall then assess whether and, if so, to which extent
Confidential Information shall henceforth be withheld from the involved person. 
  

	12.12	Assignment 

 Except as
expressly contemplated in this Agreement, none of the Parties shall be entitled to assign or transfer its rights and/or obligations under this Agreement to any Third Party without the prior written consent of the other Parties. 

 

	12.13	No partnership 

 Nothing
in this Agreement shall constitute or be deemed to constitute a partnership between the Shareholders and/or between any of them and the Company nor constitute any Party the agent of any other for any purpose. 

 

	12.14	Language 

 The language of
this Agreement and the transactions envisaged by it is English and all notices, demands, requests, statements, certificates or other documents or communications issued pursuant to, or which relate to, this Agreement shall be in English. 

 

	12.15	Governing law 

 This
Agreement shall be governed by and construed in accordance with the laws of The Netherlands. 
  

	12.16	Dispute resolution 

 All
disputes arising in connection with this Agreement, or further agreements or contracts resulting thereof, shall in first instance be referred exclusively to the Court of Amsterdam. 

 

	12.17	Counterparts 

 This
Agreement may be executed in one or more counterparts, each of which shall be deemed an original but all of which together shall constitute one and the same instrument. 

  
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 IN WITNESS WHEREOF this Agreement was signed in the manner set out below.

 – signature pages to follow – 

  
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	 /s/ Berndt Modig
	 		 	  

			
	Prosensa Holding B.V.	 		 	Prosensa Holding B.V.
					
	By:	 	 Berndt Modig
	 		 	By:	 	  

	Title:	 	 CFO
	 		 	Title:	 	  

	Date:	 	 13 January
	 	2012	 		 	Date:	 	  
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Martijn Kleijwegt
	 		 	 /s/ RR Kuijten

			
	LSP III Omni Investment Coöperatief U.A.	 		 	LSP III Omni Investment Coöperatief U.A.
					
	By:	 	LSP III Management B.V.	 		 	By:	 	LSP III Management B.V.
					
	By:	 	 M. Kleijwegt
	 		 	By:	 	 RR Kuijten

	Title:	 	 Managing Director
	 		 	Title:	 	 Managing Director

	Date:	 	 13 January
	 	2012	 		 	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Martijn Kleijwegt
	 		 	 /s/ RR Kuijten

			
	Coöperatief LSP IV U.A.	 		 	Coöperatief LSP IV U.A.
					
	By:	 	LSP IV Management B.V.	 		 	By:	 	LSP IV Management B.V.
	Title:	 	 Managing Director
	 		 	Title:	 	 Managing Director

	Date:	 	 13 January
	 	2012	 		 	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Sonja Hartsuijker

	
	ABV IV Holdings N.V.
		
	By:	 	 Sonja Hartsuijker

	Title:	 	 Managing Director

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	MedSciences Prosensa Holding B.V.	 		 	MedSciences Prosensa Holding B.V.
					
	By:	 	MedSciences Capital Management B.V.	 		 	By:	 	MedSciences Capital Management B.V.
					
	By:	 	Kempen Capital Management N.V.	 		 	By:	 	Kempen Capital Management N.V.
					
	By:	 	 /s/ Paul Gerla
	 		 	By:	 	  

	Title:	 	 Managing Director
	 		 	Title:	 	  

	Date:	 	 13 January
	 	2012	 		 	Date:	 	  
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Benoist Grossman
	 		 	 /s/ Benoist Grossman

			
	FCPI Allianz Innovation 8	 		 	FCPI Capital Croissance 3
					
	By:	 	Idinvest Partners	 		 	By:	 	Idinvest Partners
					
	By:	 	 Benoist Grossman
	 		 	By:	 	 Benoist Grossman

	Title:	 	 Managing Partner
	 		 	Title:	 	 Managing Partner

	Date:	 	 13 January
	 	2012	 		 	Date:	 	 13 January
	 	2012

  

					
	 /s/ Benoist Grossman

	
	FCPI Objectif Innovation Patrimoine 3
		
	By:	 	 Benoist Grossman

	Title:	 	 Managing Partner

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Alex Brabers
	 		 	 /s/ Edmond Bastijns

			
	Gimv NV	 		 	Gimv NV
					
	By:	 	Alex Brabers	 		 	By:	 	Edmond Bastijns
	Title:	 	Gimv Partner	 		 	Title:	 	Gimv Partner
	Date:	 	13 January 2012	 		 	Date:	 	13 January 2012

  

									
	 /s/ Alex Brabers
	 		 	 /s/ Edmond Bastijns

			
	Adviesbeheer Gimv Life Sciences 2007 NV	 		 	Adviesbeheer Gimv Life Sciences 2007 NV
					
	By:	 	Alex Brabers	 		 	By:	 	Edmond Bastijns
	Title:	 	Gimv Partner	 		 	Title:	 	Gimv Partner
	Date:	 	13 January 2012	 		 	Date:	 	13 January 2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig

	
	Arriwan Holding B.V.
		
	By:	 	 Berndt Modig

	Title:	 	 Representative

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig

	
	Cure Duchenne
		
	By:	 	 Berndt Modig

	Title:	 	 Representative

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig

	
	Charley’s Fund Inc.
		
	By:	 	 Berndt Modig

	Title:	 	 Representative

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig

	
	Dordtwijck I B.V.
		
	By:	 	 Berndt Modig

	Title:	 	 Representative

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig

	
	C.M. van den Brink
			
	[By:	 	Berndt Modig	 	]
		 	  

					
	Title:	 	 Representative

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig

	
	Libertatis Ergo Holding B.V.
		
	By:	 	 Berndt Modig

	Title:	 	 Representative

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig

	
	G.J. Platenburg
			
	[By:	 	Berndt Modig	 	]
		 	  

					
	Title:	 	 Representative

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig

	
	E.M.H.C. van Boom-Husken
			
	[By:	 	Berndt Modig	 	]
		 	  

					
	Title:	 	 Representative

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig

	
	P.F. Ekhart
			
	[By:	 	Berndt Modig	 	]
		 	  

					
	Title:	 	 Representative

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig

	
	C.J.P.M. van Megen
			
	[By:	 	Berndt Modig	 	]
		 	  

					
	Title:	 	 Representative

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig

	
	D.J. Ellens
			
	[By:	 	Berndt Modig	 	]
		 	  

					
	Title:	 	 Representative

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig

	
	H.L. Heijneker
			
	[By:	 	Berndt Modig	 	]
		 	  

					
	Title:	 	 Representative

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	 /s/ Berndt Modig
	 		 	  

			
	Stichting Administratiekantoor Prosensa Holding	 		 	Stichting Administratiekantoor Prosensa Holding
					
	By:	 	 Berndt Modig
	 		 	By:	 	  

	Title:	 	 Representative
	 		 	Title:	 	  

	Date:	 	 13 January
	 	2012	 		 	Date:	 	  
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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	New Enterprise Associates 13, L.P.
		
	By:	 	NEA Partners 13, Limited Partnership, its general partner
		
	By:	 	NEA 13 GP, LTD, its general partner
		
	By:	 	 /s/ Louis A. Citron

	Title:	 	 Chief Legal Officer

	Date:	 	 13 January
	 	2012

 Signature page shareholders agreement amendment no. 2, 2012. 

This shareholders agreement will become effective as per the day of the execution of the notarial deed of issuance pursuant to which
new Class B2 shares will be issued to the Investors as defined in the 2012 Subscription Agreement. 

  
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 Schedule 1.1 

[Prosensa Shareholders Agreement] 
 Definitions 
 The below capitalized terms used in the Shareholders Agreement shall have the
meaning as set forth opposite such term or as defined in the article of the Shareholders Agreement mentioned opposite such term. 
  

			
	2008 First Installment Shares	  	pre-amble
		
	2008 Second Installment Shares	  	pre-amble
		
	2008 Shareholders Agreement	  	pre-amble
		
	2008 Subscription Agreement	  	pre-amble
		
	2010 AGM	  	as defined and described in Clause 11.1
		
	2010 Shareholders Agreement	  	pre-amble
		
	2011 Subscription Agreement	  	pre-amble
		
	A – Shareholders	  	as defined and described in Clause 3.4.2.1
		
	A&B Down Round	  	as defined and described in Clause 3.4.4
		
	AB – Shareholders	  	as defined and described in Clause 3.4.2.1
		
	ABV Holdings NV	  	see Parties
		
	Adviesbeheer NV	  	see Parties
		
	Agreement	  	this Shareholders Agreement
		
	Anti-Dilution Protection	  	as defined and described in Clause 3.4.4
		
	Arriwan BV	  	see Parties
		
	B-Shareholders	  	as defined and described in Clause 3.4.2.1

  
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	B Down Round	  	as defined and described in Clause 3.4.4
		
	Boom	  	see Parties
		
	Brink	  	see Parties
		
	Business	  	pre-amble
		
	Charley’s Fund	  	see Parties
		
	Class A Meeting	  	as defined and described in Clause 7.7
		
	Class AB Liquidation Proceeds	  	as defined and described in Clause 3.4.2.1
		
	Class AB Meeting Approval Matters	  	as defined and described in Clause 7.7
		
	Class AB Meeting	  	as defined and described in Clause 3.4.1
		
	Class AB Shares	  	as defined and described in Clause 3.1
		
	Class B Meeting	  	as defined and described in Clause 3.4.3.1
		
	Class O Liquidation Proceeds	  	as defined and described in Clause 3.4.2.2
		
	Common Meeting	  	as defined and described in Clause 9.1
		
	Common-Shareholders	  	as defined and described in Clause 3.4.2.2
		
	Company	  	see Parties
		
	Confidential Information	  	as defined and described in Clause 12.9
		
	Conversion Price	  	as defined and described in Clause 3.4.3.2
		
	Conversion Rate	  	as defined and described in Clause 3.4.3.2

  
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	Cpa	  	as defined and described in Clause 3.4.4
		
	CPb	  	as defined and described in Clause 3.4.4
		
	Cure Duchenne	  	see Parties
		
	Defaulting Investor	  	as defined and described in Clause 2.3
		
	Dilutive Price	  	as defined and described in Clause 3.4.4
		
	Dordtwijck BV	  	see Parties
		
	Drag Along Notice	  	as defined and described in Clause 6.5
		
	Drag Along Right	  	as defined and described in Clause 6.5
		
	Ekhart	  	see Parties
		
	Ellens	  	see Parties
		
	Exercise Notice	  	as defined and described in Clause 6.3
		
	Exit	  	as defined and described in Clause 10.1
		
	FCPI 8	  	see Parties
		
	FCPI Croissance	  	see Parties
		
	FCPI Patrimoine	  	see Parties
		
	General Meeting	  	the general meeting of shareholders of the Company
		
	GIMV NV	  	see Parties
		
	GIMV Parties	  	see Parties
		
	Heijneker	  	see Parties

  
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	Idinvest Funds	  	see Parties
		
	Incentive Plans	  	as defined and described in Clause 2.2
		
	Incentive Shares	  	as defined and described in Clause 2.2
		
	Indirect Shareholders	  	as defined and described in Clause 6.7
		
	Initial Consideration	  	as defined and described in Clause 3.4.2.4
		
	IPO	  	as defined and described in Clause 3.4.3.1
		
	Libertatis BV	  	see Parties
		
	Liquidation Event	  	as defined and described in Clause 3.4.2.1
		
	LSP III Omni	  	see Parties
		
	LSP IV	  	see Parties
		
	Management Board	  	the management board of the Company
		
	MedSciences BV	  	see Parties
		
	Megen	  	see Parties
		
	New Articles	  	as defined and described in Clause 2.1
		
	O-Shareholders	  	as defined and described in Clause 3.4.2.2
		
	Offer/Offered Shares	  	as defined and described in Clause 6.3
		
	Offerees	  	as defined and described in Clause 6.3
		
	Original Issue Price	  	as defined and described in Clause 3.4.2.1
		
	P-Shareholders	  	as defined and described in Clause 6.4
		
	Party/Parties	  	the party/parties of this Shareholders Agreement

  
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	Platenburg	  	see parties
		
	Preferred Shares	  	as defined and described in Clause 3.1
		
	Proposed Purchaser	  	as defined and described in Clause 6.3
		
	Proposed Sale	  	As defined in Clause 6.5
		
	Proposed Seller	  	as defined and described in Clause 6.3
		
	Remaining Liquidation Proceeds	  	as defined and described in Clause 3.4.2.3
		
	Remaining Shares	  	as defined and described in Clause 6.3
		
	Right of First Refusal	  	as defined and described in Clause 6.3
		
	Sale Notice	  	as defined and described in Clause 6.3
		
	Series B Round	  	pre-amble
		
	Shares	  	as defined and described in Clause 3.1
		
	Shareholders	  	pre-amble
		
	Stichting AK	  	see Parties
		
	Subscription Agreement	  	pre-amble
		
	Subsidiaries	  	pre-amble
		
	Supervisory Board Approval Matters	  	as defined and described in Clause 7.6
		
	Tag-along Notice	  	as defined and described in Clause 6.4
		
	Tag-along Right	  	as defined and described in Clause 6.4
		
	Tranche 2 Completion Date	  	as defined and described in Clause 2.3
		
	Tranche 2 Issue	  	as defined and described in Clause 2.3
		
	Transferee	  	as defined and described in Clause 6.2
		
	Transferor	  	as defined and described in Clause 6.2

  
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