Document:

EX-4.2

 Exhibit 4.2 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 

THIS AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT is made as of the 4th day of
December, 2013, by and among Eleven Biotherapeutics, Inc., a Delaware corporation (the “Company”), and each of the investors listed on Schedule A hereto, each of which is referred to in this Agreement as an
“Investor,” and any Additional Purchaser (as defined in the Purchase Agreement) that becomes a party to this Agreement in accordance with Section 6.9 hereof. 

RECITALS 

WHEREAS, certain of the Investors (the “Existing Investors”) hold shares of the Company’s Series A Preferred
Stock and/or shares of Common Stock issued upon conversion thereof and possess registration rights, information rights, rights of first offer, and other rights pursuant to an Investors’ Rights Agreement dated as of February 9, 2010, as
amended by Amendment No. 1 dated April 23, 2012 and Amendment No. 2 dated June 28, 2013, between the Company and such Investors (the “Prior Agreement”); and 

WHEREAS, the Existing Investors are holders of at least seventy percent (70%) of the Registrable Securities of the Company (as
defined in the Prior Agreement), and desire to amend and restate the Prior Agreement in its entirety and to accept the rights created pursuant to this Agreement in lieu of the rights granted to them under the Prior Agreement; and 

WHEREAS, certain of the Investors are parties to that certain Securities Purchase Agreement of even date herewith between the Company
and certain of the Investors (the “Purchase Agreement”), under which certain of the Company’s and such Investors’ obligations are conditioned upon the execution and delivery of this Agreement by such Investors, Existing
Investors holding at least seventy percent (70%) of the Registrable Securities, and the Company. 
 NOW, THEREFORE, the parties
hereby agree as follows: 
 1. Definitions. For purposes of this Agreement: 

1.1 “Affiliate” means, with respect to any specified Person, any other Person who, directly or indirectly, controls, is
controlled by, or is under common control with such Person, including without limitation any general partner, managing member, officer or director of such Person or any venture capital fund now or hereafter existing that is controlled by or under
common control with one or more general partners or managing members of, or shares the same management company with, such Person. 
 1.2
“Common Stock” means shares of the Company’s common stock, par value $.001 per share. 
 1.3 “Common Stock
Warrants” has the meaning ascribed to such term in the Purchase Agreement. 
 1.4 “Damages” means any loss,
damage, or liability (joint or several) to which a party hereto may become subject under the Securities Act, the Exchange Act, or other federal or 

 
state law, insofar as such loss, damage, or liability (or any action in respect thereof) arises out of or is based upon (i) any untrue statement or alleged untrue statement of a material
fact contained in any registration statement of the Company, including any preliminary prospectus or final prospectus contained therein or any amendments or supplements thereto; (ii) an omission or alleged omission to state therein a material
fact required to be stated therein, or necessary to make the statements therein not misleading; or (iii) any violation or alleged violation by the indemnifying party (or any of its agents or Affiliates) of the Securities Act, the Exchange Act,
any state securities law, or any rule or regulation promulgated under the Securities Act, the Exchange Act, or any state securities law. 

1.5 “Derivative Securities” means any securities or rights convertible into, or exercisable or exchangeable for (in each
case, directly or indirectly), Common Stock, including without limitation options, warrants and Preferred Stock. 
 1.6 “Exchange
Act” means the Securities Exchange Act of 1934, as amended, and the rules and regulations promulgated thereunder. 
 1.7
“Excluded Registration” means (i) a registration relating to the sale of securities to employees of the Company or a subsidiary pursuant to a stock option, stock purchase, or similar plan; (ii) a registration relating to
an SEC Rule 145 transaction; (iii) a registration on any form that does not include substantially the same information as would be required to be included in a registration statement covering the sale of the Registrable Securities; or
(iv) a registration in which the only Common Stock being registered is Common Stock issuable upon conversion of debt securities that are also being registered. 

1.8 “Form S-1” means such form under the Securities Act as in effect on the date hereof or any successor registration form
under the Securities Act subsequently adopted by the SEC. 
 1.9 “Form S-3” means such form under the Securities Act as in
effect on the date hereof or any registration form under the Securities Act subsequently adopted by the SEC that permits incorporation of substantial information by reference to other documents filed by the Company with the SEC. 

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

1.11 “Holder” means any holder of Registrable Securities, or holder of Derivative Securities convertible into, or exercisable
or exchangeable for, Registrable Securities, who is a party to this Agreement. For purposes hereof, a holder of Derivative Securities convertible into, or exercisable or exchangeable for, Registrable Securities, shall be deemed to hold such
Registrable Securities. 
 1.12 “Immediate Family Member” means a child, stepchild, grandchild, parent, stepparent,
grandparent, spouse, sibling, mother-in-law, father-in-law, son-in-law, daughter-in-law, brother-in-law, or sister-in-law, including adoptive relationships, of a natural person referred to herein. 

  
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 1.13 “Initiating Holders” means, collectively, Holders who properly initiate a
registration request under this Agreement. 
 1.14 “IPO” means the Company’s first underwritten public offering of its
Common Stock under the Securities Act. 
 1.15 “June Warrants” means the warrants exercisable for shares of Common Stock
issued in connection with the sale of 7% convertible promissory notes with an aggregate principal amount of up to $3,500,000 by the Company to certain investors pursuant to subscription agreements dated as of June 28, 2013. 

1.16 “Key Employee” means any executive-level employee (including division director and vice president-level positions) as
well as any employee who, either alone or in concert with others, develops, invents, programs, or designs any Company Intellectual Property (as defined in the Purchase Agreement). 

1.17 “Major Investor” means any Investor that, individually or together with such Investor’s Affiliates, holds at least
1,000,000 shares of Registrable Securities (as adjusted for any stock split, stock dividend, combination, or other recapitalization or reclassification effected after the date hereof). 

1.18 “New Securities” means, collectively, equity securities of the Company, whether or not currently authorized, as well as
rights, options, or warrants to purchase such equity securities, or securities of any type whatsoever that are, or may become, convertible or exchangeable into or exercisable for such equity securities. 

1.19 “Person” means any individual, corporation, partnership, trust, limited liability company, association or other entity.

 1.20 “Preferred Director” means any director of the Company that the holders of record of the Preferred Stock are
entitled to elect pursuant to the Restated Certificate of Incorporation. 
 1.21 “Preferred Stock” means Series A Preferred
Stock and Series B Preferred Stock. 
 1.22 “Qualified Public Offering” means the consummation of a the Company’s
first sale of shares of Common Stock to the public at a price of (i) at least $5.00 per share (subject to appropriate adjustment in the event of any stock dividend, stock split, combination or other similar recapitalization with respect to the
Common Stock) in a firm-commitment underwritten public offering pursuant to an effective registration statement under the Securities Act, resulting in at least $30,000,000 of gross proceeds to the Company or (ii) in any other underwritten
public offering pursuant to an effective registration statement under the Securities Act at a price less than $5.00 per share (subject to appropriate adjustment in the event of any stock dividend, stock split, combination or other similar
recapitalization with respect to the Common Stock) and/or resulting in gross proceeds to the Company of less than $30,000,000 approved by the vote or written consent of the Requisite Holders. 

  
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 1.23 “Registrable Securities” means (i) the Common Stock issuable or issued
upon conversion of the Preferred Stock or upon exercise of the June Warrants or the Common Stock Warrants; and (ii) any shares of Common Stock issued as (or issuable upon the conversion or exercise of any warrant, right, or other security that
is issued as) a dividend or other distribution with respect to, or in exchange for or in replacement of, the shares referenced in clause (i) above; excluding in all cases, however, any Registrable Securities sold by a Person in a transaction in
which the applicable rights under this Agreement are not assigned pursuant to Section 6.1, and excluding for purposes of Section 2 any shares for which registration rights have terminated pursuant to Section 2.13
of this Agreement. 
 1.24 “Registrable Securities then outstanding” means the number of shares determined by adding the
number of shares of outstanding Common Stock that are Registrable Securities and the number of shares of Common Stock issuable (directly or indirectly) pursuant to outstanding then convertible, exercisable and/or exchangeable Derivative Securities
that are Registrable Securities. 
 1.25 “Requisite Holders” means the Holders of seventy percent (70%) of the
Registrable Securities then outstanding. 
 1.26 “Restated Certificate of Incorporation” means the Restated Certificate of
Incorporation of the Company, as the same may be further amended or restated from time to time. 
 1.27 “Restricted
Securities” means the securities of the Company required to bear the legend set forth in Section 2.12(b) hereof. 

1.28 “SEC” means the Securities and Exchange Commission. 

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

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

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

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

1.33 “Series A Preferred Stock” means shares of the Company’s Series A Preferred Stock, par value $.001 per share. 

1.34 “Series B Preferred Stock” means shares of the Company’s Series B Preferred Stock, par value $.001 per share. 

  
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 2. Registration Rights. The Company covenants and agrees as follows: 

2.1 Demand Registration. 

(a) Form S-l Demand. If at any time after the earlier of (i) five (5) after the date of this Agreement or (ii) one
hundred eighty (180) days after the effective date of the registration statement for the IPO, the Company receives a request from Holders of at least twenty-five percent (25%) of the Registrable Securities then outstanding that the Company
file a Form S-l registration statement with respect to at least twenty-five (25%) of the Registrable Securities then outstanding, which Registrable Securities represent an anticipated aggregate offering price, net of Selling Expenses, of at
least $5 million), then the Company shall (i) within ten (10) days after the date such request is given, give notice thereof (the “Demand Notice”) to all Holders other than the Initiating Holders; and (ii) as soon as
practicable, and in any event within sixty (60) days after the date such request is given by the Initiating Holders, file a Form S-1 registration statement under the Securities Act covering all Registrable Securities that the Initiating Holders
requested to be registered and any additional Registrable Securities requested to be included in such registration by any other Holders, as specified by notice given by each such Holder to the Company within twenty (20) days of the date the
Demand Notice is given, and in each case, subject to the limitations of Section 2.1(c) and Section 2.3. 
 (b)
Form S-3 Demand. If at any time when it is eligible to use a Form S-3 registration statement, the Company receives a request from Holders of at least ten percent (10%) of the Registrable Securities then outstanding that the Company file
a Form S-3 registration statement with respect to outstanding Registrable Securities of such Holders having an anticipated aggregate offering price, net of Selling Expenses, of at least $1 million, then the Company shall (i) within ten
(10) days after the date such request is given, give a Demand Notice to all Holders other than the Initiating Holders; and (ii) as soon as practicable, and in any event within forty-five (45) days after the date such request is given
by the Initiating Holders, file a Form S-3 registration statement under the Securities Act covering all Registrable Securities requested to be included in such registration by any other Holders, as specified by notice given by each such Holder to
the Company within twenty (20) days of the date the Demand Notice is given, and in each case, subject to the limitations of Section 2.l(c) and Section 2.3. 

(c) Notwithstanding the foregoing obligations, if the Company furnishes to Holders requesting a registration pursuant to this
Section 2.1 a certificate signed by the Company’s chief executive officer stating that in the good faith judgment of the Company’s Board of Directors it would be materially detrimental to the Company and its stockholders for
such registration statement to either become effective or remain effective for as long as such registration statement otherwise would be required to remain effective, because such action would (i) materially interfere with a significant
acquisition, corporate reorganization, or other similar transaction involving the Company; (ii) require premature disclosure of material information that the Company has a bona fide business purpose for preserving as confidential; or
(iii) render the Company unable to comply with requirements under the Securities Act or Exchange Act, then the Company shall have the right to defer taking action with respect to such filing, and any time periods with respect to filing or
effectiveness thereof shall be tolled correspondingly, for a period of not more than one hundred twenty (120) days after the request of the Initiating Holders is given; provided, however, that the Company may not invoke this right

  
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more than once in any twelve (12) month period; and provided further that the Company shall not register any securities for its own account or that of any other stockholder during
such one hundred twenty (120) day period other than an Excluded Registration. 
 (d) The Company shall not be obligated to effect, or
to take any action to effect, any registration pursuant to Section 2.1(a) (i) during the period that is sixty (60) days before the Company’s good faith estimate of the date of filing of, and ending on a date that is one
hundred eighty (180) days after the effective date of, a Company-initiated registration, provided, that the Company is actively employing in good faith commercially reasonable efforts to cause such registration statement to become
effective; (ii) after the Company has effected two registrations pursuant to Section 2.1(a); or (iii) if the Initiating Holders propose to dispose of shares of Registrable Securities that may be immediately registered on Form
S-3 pursuant to a request made pursuant to Section 2.1(b). The Company shall not be obligated to effect, or to take any action to effect, any registration pursuant to Section 2.1(b) (i) during the period that is thirty
(30) days before the Company’s good faith estimate of the date of filing of, and ending on a date that is ninety (90) days after the effective date of, a Company-initiated registration, provided, that the Company is actively employing
in good faith commercially reasonable efforts to cause such registration statement to become effective; or (ii) if the Company has effected two registrations pursuant to Section 2.1(b) within the twelve (12) month period
immediately preceding the date of such request. A registration shall not be counted as “effected” for purposes of this Section 2.1(d) until such time as the applicable registration statement has been declared effective by the
SEC, unless the Initiating Holders withdraw their request for such registration (other than as a result of a material adverse change to the Company), elect not to pay the registration expenses therefor, and forfeit their right to one demand
registration statement pursuant to Section 2.6, in which case such withdrawn registration statement shall be counted as “effected” for purposes of this Section 2.1(d). 

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

(a) If, pursuant to Section 2.1, the Initiating Holders intend to distribute the Registrable Securities covered by their request
by means of an underwriting, they shall so advise the Company as a part of their request made pursuant to Section 2.1, and the Company shall include such information in the Demand Notice. The underwriter(s) will be selected by the
Company and shall be reasonably acceptable to a majority in interest of the Initiating Holders. 

  
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In such event, the right of any Holder to include such Holder’s Registrable Securities in such registration shall be conditioned upon such Holder’s participation in such underwriting
and the inclusion of such Holder’s Registrable Securities in the underwriting to the extent provided herein. All Holders proposing to distribute their securities through such underwriting shall (together with the Company as provided in
Section 2.4(e)) enter into an underwriting agreement in customary form with the underwriter(s) selected for such underwriting. Notwithstanding any other provision of this Section 2.3, if the managing underwriter(s) advise(s)
the Initiating Holders in writing that marketing factors require a limitation on the number of shares to be underwritten, then the Initiating Holders shall so advise all Holders of Registrable Securities that otherwise would be underwritten pursuant
hereto, and the number of Registrable Securities that may be included in the underwriting shall be allocated among such Holders of Registrable Securities, including the Initiating Holders, in proportion (as nearly as practicable) to the number of
Registrable Securities owned by each Holder or in such other proportion as shall mutually be agreed to by all such selling Holders; provided, however, that the number of Registrable Securities held by the Holders to be included in such
underwriting shall not be reduced unless all other securities are first entirely excluded from the underwriting. To facilitate the allocation of shares in accordance with the above provisions, the Company or the underwriters may round the number of
shares allocated to any Holder to the nearest 100 shares. 
 (b) In connection with any offering involving an underwriting of shares of the
Company’s capital stock pursuant to Section 2.2, the Company shall not be required to include any of the Holders’ Registrable Securities in such underwriting unless the Holders accept the terms of the underwriting as agreed
upon between the Company and its underwriters, and then only in such quantity as the underwriters in their sole discretion determine will not jeopardize the success of the offering by the Company. If the total number of securities, including
Registrable Securities, requested by stockholders to be included in such offering exceeds the number of securities to be sold (other than by the Company) that the underwriters in their reasonable discretion determine is compatible with the success
of the offering, then the Company shall be required to include in the offering only that number of such securities, including Registrable Securities, which the underwriters and the Company in their sole discretion determine will not jeopardize the
success of the offering. If the underwriters determine that less than all of the Registrable Securities requested to be registered can be included in such offering, then the Registrable Securities that are included in such offering shall be
allocated among the selling Holders in proportion (as nearly as practicable to) the number of Registrable Securities owned by each selling Holder or in such other proportions as shall mutually be agreed to by all such selling Holders. To facilitate
the allocation of shares in accordance with the above provisions, the Company or the underwriters may round the number of shares allocated to any Holder to the nearest 100 shares. Notwithstanding the foregoing, in no event shall (i) the number
of Registrable Securities included in the offering be reduced unless all other securities (other than securities to be sold by the Company) are first entirely excluded from the offering, or (ii) the number of Registrable Securities included in
the offering be reduced below twenty percent (20%) of the total number of securities included in such offering, unless such offering is the IPO, in which case the selling Holders may be excluded further if the underwriters make the
determination described above and no other stockholder’s securities are included in such offering For purposes of the provision in this Section 2.3(b) concerning apportionment, for any selling Holder that is a partnership, limited
liability company, or corporation, the partners, members, retired partners, retired members, stockholders, and Affiliates of such Holder, or the estates and 

  
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Immediate Family Members of any such partners, retired partners, members, and retired members and any trusts for the benefit of any of the foregoing Persons, shall be deemed to be a single
“selling Holder,” and any pro rata reduction with respect to such “selling Holder” shall be based upon the aggregate number of Registrable Securities owned by all Persons included in such “selling Holder,” as defined in
this sentence. 
 (c) For purposes of Section 2.1(a), a registration shall not be counted as “effected” if, as a
result of an exercise of the underwriter’s cutback provisions in Section 2.3(a), fewer than all of the Registrable Securities that Holders have requested to be included in such registration statement are actually included. 

2.4 Obligations of the Company. Whenever required under this Section 2 to effect the registration of any Registrable
Securities, the Company shall, as expeditiously as reasonably possible: 
 (a) prepare and file with the SEC a registration statement with
respect to such Registrable Securities and use its commercially reasonable efforts to cause such registration statement to become effective and, upon the request of the Holders of a majority of the Registrable Securities registered thereunder, keep
such registration statement effective for a period of up to one hundred twenty (120) days or, if earlier, until the distribution contemplated in the registration statement has been completed; provided, however, that (i) such one
hundred twenty (120) day period shall be extended for a period of time equal to the period the Holder refrains, at the request of an underwriter of Common Stock (or other securities) of the Company, from selling any securities included in such
registration; 
 (b) prepare and file with the SEC such amendments and supplements to such registration statement, and the prospectus used
in connection with such registration statement, as may be necessary to comply with the Securities Act in order to enable the disposition of all securities covered by such registration statement; 

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

(d) use its commercially reasonable efforts to register and qualify the securities covered by such registration statement under such other
securities or blue-sky laws of such jurisdictions as shall be reasonably requested by the selling Holders; provided that the Company shall not be required to qualify to do business or to file a general consent to service of process in any
such states or jurisdictions, unless the Company is already subject to service in such jurisdiction and except as may be required by the Securities Act; 

(e) in the event of any underwritten public offering, enter into and perform its obligations under an underwriting agreement, in usual and
customary form, with the underwriter(s) of such offering; 
 (f) use its commercially reasonable efforts to cause all such Registrable
Securities covered by such registration statement to be listed on a national securities exchange or trading system and each securities exchange and trading system (if any) on which similar securities issued by the Company are then listed; 

  
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 (g) provide a transfer agent and registrar for all Registrable Securities registered pursuant to
this Agreement and provide a CUSIP number for all such Registrable Securities, in each case not later than the effective date of such registration; 

(h) promptly make available for inspection by the selling Holders, any managing underwriter(s) participating in any disposition pursuant to
such registration statement, and any attorney or accountant or other agent retained by any such underwriter or selected by the selling Holders, all financial and other records, pertinent corporate documents, and properties of the Company, and cause
the Company’s officers, directors, employees, and independent accountants to supply all information reasonably requested by any such seller, underwriter, attorney, accountant, or agent, in each case, as necessary or advisable to verify the
accuracy of the information in such registration statement and to conduct appropriate due diligence in connection therewith; 
 (i) notify
each selling Holder, promptly after the Company receives notice thereof, of the time when such registration statement has been declared effective or a supplement to any prospectus forming a part of such registration statement has been filed; and

 (j) after such registration statement becomes effective, notify each selling Holder of any request by the SEC that the Company amend or
supplement such registration statement or prospectus. 
 2.5 Furnish Information. It shall be a condition precedent to the
obligations of the Company to take any action pursuant to this Section 2 with respect to the Registrable Securities of any selling Holder that such Holder shall furnish to the Company such information regarding itself, the Registrable
Securities held by it, and the intended method of disposition of such securities as is reasonably required to effect the registration of such Holder’s Registrable Securities. 

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

  
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such information then the Holders shall not be required to pay any of such expenses and shall not forfeit their right to one registration pursuant to Section 2.1(a) or
Section 2.1(b). All Selling Expenses relating to Registrable Securities registered pursuant to this Section 2 shall be borne and paid by the Holders pro rata on the basis of the number of Registrable Securities registered on
their behalf. 
 2.7 Delay of Registration. No Holder shall have any right to obtain or seek an injunction restraining or otherwise
delaying any registration pursuant to this Agreement as the result of any controversy that might arise with respect to the interpretation or implementation of this Section 2. 

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

(a) To the extent permitted by law, the Company will indemnify and hold harmless each selling Holder, and the partners, members, officers,
directors, and stockholders of each such Holder; legal counsel and accountants for each such Holder; any underwriter (as defined in the Securities Act) for each such Holder; and each Person, if any, who controls such Holder or underwriter within the
meaning of the Securities Act or the Exchange Act, against any Damages, and the Company will pay to each such Holder, underwriter, controlling Person, or other aforementioned Person any legal or other expenses reasonably incurred thereby in
connection with investigating or defending any claim or proceeding from which Damages may result, as such expenses are incurred; provided, however, that the indemnity agreement contained in this Section 2.8(a) shall not apply to
amounts paid in settlement of any such claim or proceeding if such settlement is effected without the consent of the Company, which consent shall not be unreasonably withheld, nor shall the Company be liable for any Damages to the extent that they
arise out of or are based upon actions or omissions made in reliance upon and in conformity with written information furnished by or on behalf of any such Holder, underwriter, controlling Person, or other aforementioned Person expressly for use in
connection with such registration. 
 (b) To the extent permitted by law, each selling Holder, severally and not jointly, will indemnify
and hold harmless the Company, and each of its directors, each of its officers who has signed the registration statement, each Person (if any), who controls the Company within the meaning of the Securities Act, legal counsel and accountants for the
Company, any underwriter (as defined in the Securities Act), any other Holder selling securities in such registration statement, and any controlling Person of any such underwriter or other Holder, against any Damages, in each case only to the extent
that such Damages arise out of or are based upon actions or omissions made in reliance upon and in conformity with written information furnished by or on behalf of such selling Holder expressly for use in connection with such registration; and each
such selling Holder will pay to the Company and each other aforementioned Person any legal or other expenses reasonably incurred thereby in connection with investigating or defending any claim or proceeding from which Damages may result, as such
expenses are incurred; provided, however, that the indemnity agreement contained in this Section 2.8(b) shall not apply to amounts paid in settlement of any such claim or proceeding if such settlement is effected without the
consent of the Holder, which consent shall not be unreasonably withheld; and provided further that in no event shall the aggregate amounts payable by any Holder by way 

  
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of indemnity or contribution under Sections 2.8(b) and 2.8(d) exceed the proceeds from the offering received by such Holder (net of any Selling Expenses paid by such Holder), except
in the case of fraud or willful misconduct by such Holder. 
 (c) Promptly after receipt by an indemnified party under this
Section 2.8 of notice of the commencement of any action (including any governmental action) for which a party may be entitled to indemnification hereunder, such indemnified party will, if a claim in respect thereof is to be made against
any indemnifying party under this Section 2.8, give the indemnifying party notice of the commencement thereof. The indemnifying party shall have the right to participate in such action and, to the extent the indemnifying party so
desires, participate jointly with any other indemnifying party to which notice has been given, and to assume the defense thereof with counsel mutually satisfactory to the parties; provided, however, that an indemnified party (together with
all other indemnified parties that may be represented without conflict by one counsel) shall have the right to retain one separate counsel, with the fees and expenses to be paid by the indemnifying party, if representation of such indemnified party
by the counsel retained by the indemnifying party would be inappropriate due to actual or potential differing interests between such indemnified party and any other party represented by such counsel in such action. The failure to give notice to the
indemnifying party within a reasonable time of the commencement of any such action shall relieve such indemnifying party of any liability to the indemnified party under this Section 2.8, to the extent that such failure materially
prejudices the indemnifying party’s ability to defend such action. The failure to give notice to the indemnifying party will not relieve it of any liability that it may have to any indemnified party otherwise than under this
Section 2.8. 
 (d) To provide for just and equitable contribution to joint liability under the Securities Act in any case in
which either (i) any party otherwise entitled to indemnification hereunder makes a claim for indemnification pursuant to this Section 2.8 but it is judicially determined (by the entry of a final judgment or decree by a court of
competent jurisdiction and the expiration of time to appeal or the denial of the last right of appeal) that such indemnification may not be enforced in such case, notwithstanding the fact that this Section 2.8 provides for
indemnification in such case, or (ii) contribution under the Securities Act may be required on the part of any party hereto for which indemnification is provided under this Section 2.8, then, and in each such case, such parties will
contribute to the aggregate losses, claims, damages, liabilities, or expenses to which they may be subject (after contribution from others) in such proportion as is appropriate to reflect the relative fault of each of the indemnifying party and the
indemnified party in connection with the statements, omissions, or other actions that resulted in such loss, claim, damage, liability, or expense, as well as to reflect any other relevant equitable considerations. The relative fault of the
indemnifying party and of the indemnified party shall be determined by reference to, among other things, whether the untrue or allegedly untrue statement of a material fact, or the omission or alleged omission of a material fact, relates to
information supplied by the indemnifying party or by the indemnified party and the parties’ relative intent, knowledge, access to information, and opportunity to correct or prevent such statement or omission; provided, however, that, in
any such case, (x) no Holder will be required to contribute any amount in excess of the public offering price of all such Registrable Securities offered and sold by such Holder pursuant to such registration statement, and (y) no Person
guilty of fraudulent misrepresentation (within the meaning of Section 11(f) of the Securities Act) will be entitled to contribution from any Person who was not guilty of such fraudulent 

  
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misrepresentation; and provided further that in no event shall a Holder’s liability pursuant to this Section 2.8(d), when combined with the amounts paid or payable by such
Holder pursuant to Section 2.8(b), exceed the proceeds from the offering received by such Holder (net of any Selling Expenses) paid by such Holder), except in the case of willful misconduct or fraud by such Holder. 

(e) Notwithstanding the foregoing, to the extent that the provisions on indemnification and contribution contained in the underwriting
agreement entered into in connection with the underwritten public offering are in conflict with the foregoing provisions, the provisions in the underwriting agreement shall control. 

(f) Unless otherwise superseded by an underwriting agreement entered into in connection with the underwritten public offering, the
obligations of the Company and Holders under this Section 2.8 shall survive the completion of any offering of Registrable Securities in a registration under this Section 2, and otherwise shall survive the termination of this
Agreement. 
 2.9 Reports Under Exchange Act. With a view to making available to the Holders the benefits of SEC Rule 144 and any
other rule or regulation of the SEC that may at any time permit a Holder to sell securities of the Company to the public without registration or pursuant to a registration on Form S-3, the Company shall: 

(a) make and keep available adequate current public information, as those terms are understood and defined in SEC Rule 144, at all times
after the effective date of the registration statement filed by the Company for the IPO; 
 (b) use commercially reasonable efforts to file
with the SEC in a timely manner all reports and other documents required of the Company under the Securities Act and the Exchange Act (at any time after the Company has become subject to such reporting requirements); and 

(c) furnish to any Holder, so long as the Holder owns any Registrable Securities, forthwith upon request (i) to the extent accurate, a
written statement by the Company that it has complied with the reporting requirements of SEC Rule 144 (at any time after ninety (90) days after the effective date of the registration statement filed by the Company for the IPO), the Securities
Act, and the Exchange Act (at any time after the Company has become subject to such reporting requirements), or that it qualifies as a registrant whose securities may be resold pursuant to Form S-3 (at any time after the Company so qualifies);
(ii) a copy of the most recent annual or quarterly report of the Company and such other reports and documents so filed by the Company; and (iii) such other information as may be reasonably requested in availing any Holder of any rule or
regulation of the SEC that permits the selling of any such securities without registration (at any time after the Company has become subject to the reporting requirements under the Exchange Act) or pursuant to Form S-3 (at any time after the Company
so qualifies to use such form). 
 2.10 Limitations on Subsequent Registration Rights. From and after the date of this Agreement, the
Company shall not, without the prior written consent of the Requisite Holders, enter into any agreement with any holder or prospective holder of any securities of the 

  
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Company that would (i) allow such holder or prospective holder to include such securities in any registration unless, under the terms of such agreement, such holder or prospective holder may
include such securities in any such registration only to the extent that the inclusion of such securities will not reduce the number of the Registrable Securities of the Holders that are included or (ii) allow such holder or prospective holder
to initiate a demand for registration of any securities held by such holder or prospective holder; provided that this limitation shall not apply to any additional Investor who becomes a party to this Agreement in accordance with
Section 6.9. 
 2.11 “Market Stand-off” Agreement. Each Holder hereby agrees that, if required by the managing
underwriter and the Company, it will not, without the prior written consent of the managing underwriter, during the period commencing on the date of the final prospectus relating to the IPO and ending on the date specified by the Company and the
managing underwriter (such period not to exceed one hundred eighty (180) days, which period may be extended upon the request of the managing underwriter, to the extent required by any FINRA rules, for an additional period of up to fifteen
(15) days if the Company issues or proposes to issue an earnings or other public release within fifteen (15) days of the expiration of the 180-day lockup period, (i) lend; offer; pledge; sell; contract to sell; sell any option or
contract to purchase; purchase any option or contract to sell; grant any option, right, or warrant to purchase; or otherwise transfer or dispose of, directly or indirectly, any shares of Common Stock or any Derivative Securities (whether such shares
or any such Derivative Securities are then owned by the Holder or are thereafter acquired) or (ii) enter into any swap or other arrangement that transfers to another, in whole or in part, any of the economic consequences of ownership of such
securities, whether any such transaction described in clause (i) or (ii) above is to be settled by delivery of Common Stock or other securities, in cash, or otherwise. The foregoing provisions of this Section 2.11 shall apply
only to the IPO, shall not apply to the sale of any shares to an underwriter pursuant to an underwriting agreement, and shall be applicable to the Holders only if all officers and directors, and all stockholders individually owning more than one
percent (1%) of the Company’s outstanding Common Stock (after giving effect to conversion into Common Stock of all outstanding Preferred Stock), are subject to similar restrictions. The underwriters in connection with such registration are
intended third-party beneficiaries of this Section 2.11 and shall have the right, power, and authority to enforce the provisions hereof as though they were a party hereto. Each Holder further agrees to execute such agreements as may be
reasonably requested by the underwriters in connection with such registration that are consistent with this Section 2.11 or that are necessary to give further effect thereto. Notwithstanding the foregoing, the Company shall use
commercially reasonable efforts to obtain from the managing underwriter(s) an agreement, and the underwriters may, in their sole discretion agree, to waive these restrictions in order to provide for periodic early releases of portions of the
aforesaid securities upon the occurrence of certain specified events, any such release to apply pro rata to all Holders subject to this Section 2.11, based on the number of securities (determined on an as-converted basis) subject to the
restrictions set forth in this Section 2.11. 
 2.12 Restrictions on Transfer. 

(a) The Preferred Stock and the Registrable Securities shall not be sold, pledged, or otherwise transferred, and the Company shall not
recognize and shall issue stop-transfer 

  
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instructions to its transfer agent with respect to any such sale, pledge, or transfer, except upon the conditions specified in this Agreement, which conditions are intended to ensure compliance
with the provisions of the Securities Act. A transferring Holder will cause any proposed purchaser, pledgee, or transferee of the Preferred Stock and the Registrable Securities held by such Holder to agree to take and hold such securities subject to
the provisions and upon the conditions specified in this Agreement. 
 (b) Each certificate or instrument representing (i) the
Preferred Stock, (ii) the Registrable Securities, and (iii) any other securities issued in respect of the securities referenced in clauses (i) and (ii), upon any stock split, stock dividend, recapitalization, merger, consolidation, or
similar event, shall (unless otherwise permitted by the provisions of Section 2.12(c)) be stamped or otherwise imprinted with a legend substantially in the following form: 

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

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

(c) The holder of each certificate representing Restricted Securities, by acceptance thereof, agrees to comply in all respects with the
provisions of this Section 2. Before any proposed sale, pledge, or transfer of any Restricted Securities, unless there is in effect a registration statement under the Securities Act covering the proposed transaction, the Holder thereof
shall give notice to the Company of such Holder’s intention to effect such sale, pledge, or transfer. Each such notice shall describe the manner and circumstances of the proposed sale, pledge, or transfer in sufficient detail and, if reasonably
requested by the Company, shall be accompanied at such Holder’s expense by either (i) a written opinion of legal counsel who shall, and whose legal opinion shall, be reasonably satisfactory to the Company, addressed to the Company, to the
effect that the proposed transaction may be effected without registration under the Securities Act; (ii) a “no action” letter from the SEC to the effect that the proposed sale, pledge, or transfer of such Restricted Securities without
registration will not result in a recommendation by the staff of the SEC that action be taken with respect thereto; or (iii) any other evidence reasonably satisfactory to counsel to the Company to the effect that the proposed sale, pledge, or
transfer of the Restricted Securities may be effected without registration under 

  
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the Securities Act, whereupon the Holder of such Restricted Securities shall be entitled to sell, pledge, or transfer such Restricted Securities in accordance with the terms of the notice given
by the Holder to the Company. The Company will not require such a legal opinion or “no action” letter (x) in any transaction in compliance with SEC Rule 144 or (y) in any transaction in which such Holder distributes Restricted
Securities to an Affiliate of such Holder for no consideration; provided that each transferee agrees in writing to be subject to the terms of this Section 2.12. Each certificate or instrument evidencing the Restricted Securities
transferred as above provided shall bear, except if such transfer is made pursuant to SEC Rule 144, the appropriate restrictive legend set forth in Section 2.12(b), except that such certificate shall not bear such restrictive legend if,
in the opinion of counsel for such Holder and the Company, such legend is not required in order to establish compliance with any provisions of the Securities Act. 

2.13 Termination of Registration Rights. The right of any Holder to request registration or inclusion of Registrable Securities in any
registration pursuant to Section 2.1 or Section 2.2 shall terminate upon the earliest to occur of: 
 (a) the
closing of a Deemed Liquidation Event, as such term is defined in the Restated Certificate of Incorporation; 
 (b) the date on which all
of such Holder’s Registrable Securities have been sold; or 
 (c) the fifth anniversary of the Qualified Public Offering. 

3. Information and Observer Rights. 
 3.1
Delivery of Financial Statements. The Company shall deliver to each Major Investor: 
 (a) as soon as practicable, but in any event
within one hundred eighty (180) days after the end of each fiscal year of the Company, (i) a balance sheet as of the end of such year, (ii) statements of income and of cash flows for such year, and a comparison between (x) the
actual amounts as of and for such fiscal year and (y) the comparable amounts for the prior year and as included in the Budget (as defined in Section 3.1(e)) for such year, with an explanation of any material differences between such
amounts and a schedule as to the sources and applications of funds for such year, and (iii) a statement of stockholders’ equity as of the end of such year, all such financial statements audited and certified by independent public
accountants of nationally recognized standing selected by the Company and approved by the Board of Directors, which approval shall include the approval of the Preferred Directors; 

(b) as soon as practicable, but in any event within thirty (30) days after the end of each quarter of each fiscal year of the Company,
unaudited statements of income and of cash flows for such fiscal quarter, and an unaudited balance sheet as of the end of such fiscal quarter, all prepared in accordance with GAAP (except that such financial statements may (i) be subject to
normal year-end audit adjustments and (ii) not contain all notes thereto that may be required in accordance with GAAP); 

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

(f) such other information relating to the financial condition, business, prospects, or corporate affairs of the Company as any Major
Investor may from time to time reasonably request; provided, however, that the Company shall not be obligated under this Section 3.1 to provide information (i) that the Company reasonably determines in good faith to be a
trade secret or confidential information (unless covered by an enforceable confidentiality agreement, in form acceptable to the Company) or (ii) the disclosure of which would adversely affect the attorney-client privilege between the Company
and its counsel. 
 If, for any period, the Company has any subsidiary whose accounts are consolidated with those of the Company, then in respect of such
period the financial statements delivered pursuant to the foregoing sections shall be the consolidated and consolidating financial statements of the Company and all such consolidated subsidiaries. 

Notwithstanding anything else in this Section 3.1 to the contrary, the Company may cease providing the information set forth in this
Section 3.1 at any time during the period starting with the date sixty (60) days before the Company’s good-faith estimate of the date of filing of a registration statement if it reasonably concludes it must do so to comply with
the SEC rules applicable to such registration statement and related offering; provided that the Company’s covenants under this Section 3.1 shall be reinstated at such time as the Company is no longer actively employing its
commercially reasonable efforts to cause such registration statement to become effective. 
 3.2 Inspection. The Company shall permit
each Major Investor, at such Major Investor’s expense, to visit and inspect the Company’s properties; examine its books of account 

  
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and records; and discuss the Company’s affairs, finances, and accounts with its officers, during normal business hours of the Company as may be reasonably requested by the Major Investor;
provided, however, that the Company shall not be obligated pursuant to this Section 3.2 to provide access to any information that it reasonably and in good faith considers to be a trade secret or confidential information (unless
covered by an enforceable confidentiality agreement, in form acceptable to the Company) or the disclosure of which would adversely affect the attorney-client privilege between the Company and its counsel. 

3.3 Observer Rights. As long as JAFCO Super V3 Investment Limited Partnership and/or its Affiliates (“JAFCO”) owns not
less than fifteen percent (15%) of the shares of the Preferred Stock it purchased under that certain Series A Preferred Stock Purchase Agreement of the Company dated as of April 23, 2012 and the Purchase Agreement (or an equivalent amount
of Common Stock issued upon conversion thereof), the Company shall invite a representative of JAFCO to attend all meetings of its Board of Directors in a nonvoting observer capacity and, in this respect, shall give such representative copies of all
notices, minutes, consents, and other materials that it provides to its directors at the same time and in the same manner as provided to such directors; provided, however, that such representative shall agree to hold in confidence and trust
and to act in a fiduciary manner with respect to all information so provided; and provided further, that the Company reserves the right to withhold any information and to exclude such representative from any meeting or portion thereof if
access to such information or attendance at such meeting would reasonably be expected to adversely affect the attorney-client privilege between the Company and its counsel or result in disclosure of trade secrets or a conflict of interest, or if
such Investor or its representative is a competitor of the Company. 
 3.4 Termination of Information. The covenants set forth in
Sections 3.1, 3.2 and 3.3 shall terminate and be of no further force or effect (i) immediately before the consummation of the Qualified Public Offering, or (ii) upon a Deemed Liquidation Event, as such term is defined
in the Restated Certificate of Incorporation, whichever event occurs first. 
 3.5 Confidentiality. Each Investor agrees that such
Investor will keep confidential and will not disclose, divulge, or use for any purpose (other than to monitor its investment in the Company) any confidential information obtained from the Company pursuant to the terms of this Agreement (including
notice of the Company’s intention to file a registration statement), unless such confidential information (a) is known or becomes known to the public in general (other than as a result of a breach of this Section 3.5 by such
Investor), (b) is or has been independently developed or conceived by the Investor without use of the Company’s confidential information, or (c) is or has been made known or disclosed to the Investor by a third party without a breach
of any obligation of confidentiality such third party may have to the Company; provided, however, that an Investor may disclose confidential information (i) to its attorneys, accountants, consultants, and other professionals to the
extent necessary to obtain their services in connection with monitoring its investment in the Company; (ii) to any prospective purchaser of any Registrable Securities from such Investor, if such prospective purchaser agrees to be bound by the
provisions of this Section 3.5; (iii) to any existing or prospective Affiliate, partner, member, stockholder, or wholly owned subsidiary of such Investor in the ordinary course of business, provided that such Investor informs
such Person that such information is confidential and directs such Person to maintain the confidentiality of such information; or (iv) as may otherwise be required by law, provided that the Investor promptly notifies the Company of such
disclosure and takes reasonable steps to minimize the extent of any such required disclosure. 

  
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 4. Rights to Future Stock Issuances. 

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

(b) By notification to the Company within twenty (20) days after the Offer Notice is given, each Major Investor may elect to purchase or
otherwise acquire, at the price and on the terms specified in the Offer Notice, up to that portion of such New Securities which equals the proportion that the Common Stock issued and held, or issuable (directly or indirectly) upon conversion,
exercise or exchange, as applicable, of Derivative Securities then held, by such Major Investor bears to the total Common Stock of the Company then outstanding (assuming full conversion, exercise or exchange, as applicable, of all Derivative
Securities). At the expiration of such twenty (20) day period, the Company shall promptly notify each Major Investor that elects to purchase or acquire all the shares available to it (each, a “Fully Exercising Investor”) of any
other Major Investor’s failure to do likewise. During the ten (10) day period commencing after the Company has given such notice, each Fully Exercising Investor may, by giving notice to the Company, elect to purchase or acquire, in
addition to the number of shares specified above, up to that portion of the New Securities for which Major Investors were entitled to subscribe but that were not subscribed for by the Major Investors which is equal to the proportion that the Common
Stock issued and held, or issuable (directly or indirectly) upon conversion, exercise or exchange, as applicable, of Derivative Securities then held, by such Fully Exercising Investor bears to the Common Stock issued and held, or issuable (directly
or indirectly) upon conversion, exercise or exchange, as applicable, of Derivative Securities then held, by all Fully Exercising Investors who wish to purchase such unsubscribed shares. The closing of any sale pursuant to this
Section 4.1(b) shall occur within the later of ninety (90) days of the date that the Offer Notice is given and the date of initial sale of New Securities pursuant to Section 4.l(c). 

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

  
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 4.2 Termination. The covenants set forth in Section 4.1 shall terminate and be
of no further force or effect (i) immediately before the consummation of the Qualified Public Offering, or (ii) upon a Deemed Liquidation Event, as such term is defined in the Restated Certificate of Incorporation, whichever event occurs
first. 
 5. Additional Covenants. 

5.1 Insurance. The Company shall use its commercially reasonable efforts to obtain, within ninety (90) days of the date hereof,
from financially sound and reputable insurers Directors and Officers liability insurance in an amount and on terms and conditions satisfactory to the Board of Directors, including the Preferred Directors, and will use commercially reasonable efforts
to cause such insurance policy to be maintained until such time as the Board of Directors, including the Preferred Directors, determines that such insurance should be discontinued. 

5.2 Employee Agreements. The Company will cause (i) each person now or hereafter employed by it or by any subsidiary (or engaged
by the Company or any subsidiary as a consultant or independent contractor, including any member of the Company’s scientific advisory board) with access to confidential information and/or trade secrets to enter into a nondisclosure and
proprietary rights assignment agreement and, to the extent permitted by law, (ii) each Key Employee to enter into a one (1) year noncompetition and nonsolicitation agreement, substantially in the form approved by the Board of Directors,
including the Preferred Directors, all subject to the policies of any academic or research institution with which any such person may be affiliated. 

5.3 Employee Stock. Unless otherwise approved by the Board of Directors, including the Preferred Directors, all future employees and
consultants of the Company who purchase, receive options to purchase, or receive awards of shares of the Company’s capital stock after the date hereof shall be required to execute restricted stock or option agreements, as applicable, providing
for (i) vesting of shares over a four (4) year period, with the first twenty-five percent (25%) of such shares vesting following twelve (12) months of continued employment or service, and the remaining shares vesting in equal
quarterly installments over the following thirty-six (36) months, and (ii) a market stand-off provision substantially similar to that in Section 2.11. In addition, unless otherwise approved by the Board of Directors, including
the Preferred Directors, the Company shall retain a “right of first refusal” on employee transfers until the Company’s IPO and shall have the right to repurchase unvested shares at cost upon termination of employment of a holder of
restricted stock. 
 5.4 Qualified Small Business Stock. The Company shall use its best commercial efforts to cause the shares of
Preferred Stock, as well as any shares into which such shares are 

  
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converted, within the meaning of Section 1202(f) of the Internal Revenue Code (the “Code”), to constitute “qualified small business stock” as defined in
Section 1202(c) of the Code; provided, however, that such requirement shall not be applicable if the Board of Directors of the Company determines, in its good-faith business judgment, that such qualification is inconsistent with the best
interests of the Company. The Company shall submit to its stockholders (including the Investors) and to the Internal Revenue Service any reports that may be required under Section 1202(d)(l)(C) of the Code and the regulations promulgated
thereunder. In addition, within twenty (20) business days after any Investor’s written request therefor, the Company shall, at its option, either (i) deliver to such Investor a written statement indicating whether (and what portion
of) such Investor’s interest in the Company constitutes “qualified small business stock” as defined in Section 1202(c) of the Code or (ii) deliver to such Investor such factual information in the Company’s possession as
is reasonably necessary to enable such Investor to determine whether (and what portion of) such Investor’s interest in the Company constitutes “qualified small business stock” as defined in Section 1202(c) of the Code. 

5.5 Matters Requiring Investor Director Approval. So long as the holders of Preferred Stock are entitled to elect a Preferred Director,
the Company hereby covenants and agrees with each of the Investors that it shall not, without approval of the Board of Directors, which approval must include the affirmative vote of a majority of the Preferred Directors: 

(a) subject to Section 1.1 of that certain Second Amended and Restated Voting Agreement, dated as of the date hereof, by and among the
Company and certain of its stockholders, increase the size of the Board of Directors above seven (7) members; 
 (b) make, or permit
any subsidiary to make, any loan or advance to, or own any stock or other securities of, any subsidiary or other corporation, partnership, or other entity unless it is wholly owned by the Company; 

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

(d) make any loan or advance to, or own any stock or other securities of, any subsidiary of any other corporation, partnership or other
entity unless it is wholly owned by the Company; 
 (e) guarantee, directly or indirectly, or permit any subsidiary to guarantee, directly
or indirectly, any indebtedness except for trade accounts of the Company or any subsidiary arising in the ordinary course of business; 

(f) incur any aggregate indebtedness (excluding trade credit incurred in the ordinary course of business) in excess of $100,000 that is not
already included in a Budget for the fiscal year in which such indebtedness is incurred; 
 (g) make any investment other than investments
in prime commercial paper, money market funds, certificates of deposit in any United States bank having a net worth in excess of $100,000,000, or obligations issued or guaranteed by the United States of America, in each case having a maturity not in
excess of one year; 

  
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 (h) enter into any lines of business that is not primarily related to the business of the
Company as conducted as of the Closing, exit the business of the Company as conducted as of the Closing, or change the principal business of the Company; 

(i) grant any exclusive license to any of the Company’s material intellectual property rights; 

(j) acquire all or substantially all of the properties, assets or stock of any other company or entity; 

(k) otherwise enter into or be a party to any transaction with any director, officer, or employee of the Company or any “associate”
(as defined in Rule 12b-2 promulgated under the Exchange Act) of any such Person, except for transactions contemplated by the Purchase Agreement; or 

(l) hire, terminate, or change the compensation of any senior executive officer, including approving the payment of bonuses to any senior
executive officer, except in accordance with the Budget or such senior executive officer’s employment agreement. 
 5.6 Board
Matters. The Company shall reimburse its directors for all reasonable out-of-pocket travel expenses incurred (consistent with the Company’s travel policy) in connection with attending meetings of, or in otherwise in relation to their
service as members of, the Board of Directors provided, however, that travel expenses for any individual board member shall not exceed $25,000 in any 12-month period. Each committee of the Board of Directors shall include at least one Preferred
Director. 
 5.7 Successor Indemnification. If the Company or any of its successors or assignees consolidates with or merges into any
other Person and is not the continuing or surviving corporation or entity of such consolidation or merger, then to the extent necessary, proper provision shall be made so that the successors and assignees of the Company assume the obligations of the
Company with respect to indemnification of members of the Board of Directors as in effect immediately before such transaction, whether such obligations are contained in the Company’s Bylaws, the Restated Certificate of Incorporation, or
elsewhere, as the case may be. 
 5.8 Termination of Covenants. The covenants set forth in this Section 5, except for
Section 5.7, shall terminate and be of no further force or effect (i) immediately before the consummation of the Qualified Public Offering or (ii) upon a Deemed Liquidation Event, as such term is defined in the Restated
Certificate of Incorporation, whichever event occurs first. 
 6. Miscellaneous. 

6.1 Successors and Assigns. The rights under this Agreement may be assigned (but only with all related obligations) by a Holder to a
transferee of Registrable Securities that (i) is an Affiliate of a Holder; (ii) is a Holder’s Immediate Family Member or trust for the benefit of 

  
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an individual Holder or one or more of such Holder’s Immediate Family Members; or (iii) after such transfer, holds at least 50,000 shares of Registrable Securities (subject to
appropriate adjustment for stock splits, stock dividends, combinations, and other recapitalizations); provided, however, that (x) the Company is, within a reasonable time after such transfer, furnished with written notice of the name and
address of such transferee and the Registrable Securities with respect to which such rights are being transferred; and (y) such transferee agrees in a written instrument delivered to the Company to be bound by and subject to the terms and
conditions of this Agreement, including the provisions of Section 2.11. For the purposes of determining the number of shares of Registrable Securities held by a transferee, the holdings of a transferee (1) that is an Affiliate or
stockholder of a Holder; (2) who is a Holder’s Immediate Family Member; or (3) that is a trust for the benefit of an individual Holder or such Holder’s Immediate Family Member shall be aggregated together and with those of the
transferring Holder; provided further that all transferees who would not qualify individually for assignment of rights shall have a single attorney-in-fact for the purpose of exercising any rights, receiving notices, or taking any action under this
Agreement. The terms and conditions of this Agreement inure to the benefit of and are binding upon the respective successors and permitted assignees of the parties. Nothing in this Agreement, express or implied, is intended to confer upon any party
other than the parties hereto or their respective successors and permitted assignees any rights, remedies, obligations or liabilities under or by reason of this Agreement, except as expressly provided herein. 

6.2 Governing Law. This Agreement and any controversy arising out of or relating to this Agreement shall be governed by and construed
in accordance with the General Corporation Law of the State of Delaware as to matters within the scope thereof, and as to all other matters shall be governed by and construed in accordance with the internal laws of The Commonwealth of Massachusetts,
without regard to conflict of law principles that would result in the application of any law other than the law of The Commonwealth of Massachusetts. 

6.3 Counterparts; Facsimile. This Agreement may be executed in two or more counterparts, each of which shall be deemed an original, but
all of which together shall constitute one and the same instrument. This Agreement may also be executed and delivered by facsimile signature and in two or more counterparts, each of which shall be deemed an original, but all of which together shall
constitute one and the same instrument. 
 6.4 Titles and Subtitles. The titles and subtitles used in this Agreement are for
convenience only and are not to be considered in construing or interpreting this Agreement. 
 6.5 Notices. All notices and other
communications given or made pursuant to this Agreement shall be in writing and shall be deemed effectively given upon the earlier of actual receipt or: (i) personal delivery to the party to be notified; (ii) when sent, if sent by
electronic mail or facsimile during the recipient’s normal business hours, and if not sent during normal business hours, then on the recipient’s next business day; (iii) for deliveries within the United States, five (5) days
after having been sent by registered or certified mail, return receipt requested, postage prepaid; or (iv) one (1) business day after the business day of deposit with a nationally recognized overnight courier, specifying next business day
delivery for United States deliveries or three (3) business days after such deposit with an internationally recognized express overnight courier for deliveries outside of the United States, in either case, freight prepaid, with written
verification of receipt. All communications shall be sent to the respective parties at their 

  
 - 22 - 

 
addresses as set forth on Schedule A hereto, or to the principal office of the Company and to the attention of the Chief Executive Officer, in the case of the Company, or to such email address,
facsimile number, or address as subsequently modified by written notice given in accordance with this Section 6.5. If notice is given to the Company, a copy (which shall not constitute notice) shall also be sent to Wilmer Cutler Pickering Hale
and Dorr LLP, 60 State Street, Boston, Massachusetts 02109, Attention: David E. Redlick. If notice is given to the Investors, a copy (which shall not constitute notice) shall also be given to Sheppard Mullin Richter & Hampton LLP, 12275 El
Camino Real, Suite 200, San Diego, California 92130, Attention: Kirt W. Shuldberg and to Faber Daeufer Itrato & Cabot PC, Bay Colony Corporate Center, 950 Winter Street, Suite 4500, Waltham, MA 02451, Attention: Kurt Machemer. 

6.6 Amendments and Waivers. Any term of this Agreement may be amended and the observance of any term of this Agreement may be waived
(either generally or in a particular instance, and either retroactively or prospectively) only with the written consent of the Company and the Requisite Holders; provided that the Company may in its sole discretion waive compliance with
Section 2.12(c) (and the Company’s failure to object promptly in writing after notification of a proposed assignment allegedly in violation of Section 2.12(c) shall be deemed to be a waiver); and provided further that
any provision hereof may be waived by any waiving party on such party’s own behalf, without the consent of any other party. Notwithstanding the foregoing, this Agreement may not be amended or terminated and the observance of any term hereof may
not be waived with respect to any Investor without the written consent of such Investor, unless such amendment, termination, or waiver applies to all Investors in the same fashion (it being agreed that a waiver of the provisions of
Section 4 with respect to a particular transaction shall be deemed to apply to all Investors in the same fashion if such waiver does so by its terms, notwithstanding the fact that certain Investors may nonetheless, by agreement with the
Company, purchase securities in such transaction). The Company shall give prompt notice of any amendment or termination hereof or waiver hereunder to any party hereto that did not consent in writing to such amendment, termination, or waiver. Any
amendment, termination, or waiver effected in accordance with this Section 6.6 shall be binding on all parties hereto, regardless of whether any such party has consented thereto. No waivers of or exceptions to any term, condition, or
provision of this Agreement, in any one or more instances, shall be deemed to be or construed as a further or continuing waiver of any such term, condition, or provision. 

6.7 Severability. In case any one or more of the provisions contained in this Agreement is for any reason held to be invalid, illegal
or unenforceable in any respect, such invalidity, illegality, or unehforceability shall not affect any other provision of this Agreement, and such invalid, illegal, or unenforceable provision shall be reformed and construed so that it will be valid,
legal, and enforceable to the maximum extent permitted by law. 
 6.8 Aggregation of Stock. All shares of Registrable Securities held
or acquired by Affiliates shall be aggregated together for the purpose of determining the availability of any rights under this Agreement and such Affiliated persons may apportion such rights as among themselves in any manner they deem appropriate.

 6.9 Additional Investors. Notwithstanding anything to the contrary contained herein, if the Company issues additional shares of
the Series B Preferred Stock after the date hereof, any purchaser of such shares of Series B Preferred Stock may become a party to this 

  
 - 23 - 

 
Agreement by executing and delivering an additional counterpart signature page to this Agreement, and thereafter shall be deemed an “Investor” for all purposes hereunder. No action or
consent by the Investors shall be required for such joinder to this Agreement by such additional Investor, so long as such additional Investor has agreed in writing to be bound by all of the obligations as an “Investor” hereunder. 

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

6.11 Delays or Omissions. No delay or omission to exercise any right, power, or remedy accruing to any party under this Agreement, upon
any breach or default of any other party under this Agreement, shall impair any such right, power, or remedy of such nonbreaching or nondefaulting party, nor shall it be construed to be a waiver of or acquiescence to any such breach or default, or
to any similar breach or default thereafter occurring, nor shall any waiver of any single breach or default be deemed a waiver of any other breach or default theretofore or thereafter occurring. All remedies, whether under this Agreement or by law
or otherwise afforded to any party, shall be cumulative and not alternative. 
 6.12 Acknowledgment. The Company acknowledges that
the Investors are in the business of venture capital investing and therefore review the business plans and related proprietary information of many enterprises, including enterprises which may have products or services which compete directly or
indirectly with those of the Company. Nothing in this Agreement shall preclude or in any way restrict the Investors from investing or participating in any particular enterprise whether or not such enterprise has products or services which compete
with those of the Company. 
 [Remainder of Page Intentionally Left Blank] 

  
 - 24 - 

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

  

			
	COMPANY:
	
	ELEVEN BIOTHERAPEUTICS, INC.
		
	By:	 	 /s/ Abbie Celniker

	Name:	 	Abbie Celniker
	Title:	 	Chief Executive Officer

  
 — Signature Page
to Series B Investors’ Rights Agreement — 

 
			
	INVESTORS:
	
	FLAGSHIP VENTURES FUND 2007, L.P.
		
	By:	 	Flagship Ventures 2007 General Partner, L.L.C., its general partner
		
	By:	 	 /s/ Noubar Afeyan

	Name:	 	Noubar Afeyan
	Title:	 	
	
	FLAGSHIP VENTURES FUND IV, L.P.
		
	By:	 	Flagship Ventures Fund IV General Partner LLC, its General Partner
		
	By:	 	 /s/ Noubar Afeyan

		 	Manager
	
	FLAGSHIP VENTURES FUND IV-RX, L.P.
		
	By:	 	Flagship Ventures Fund IV General Partner LLC, its General Partner
		
	By:	 	 /s/ Noubar Afeyan

		 	Manager

  
 — Signature Page
to Series B Investors’ Rights Agreement — 

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

	Name:	 	Kevin Gillis
	Title:	 	Partner/CFO
	
	JAFCO SUPER V3 INVESTMENT LIMITED PARTNERSHIP
		
	By:	 	JAFCO Co., Ltd., its general partner
		
	By:	 	 /s/ Shinichi Fuki

	Name:	 	Shinichi Fuki
	Title:	 	President and CEO
	
	Address:
	Otemachi First Square, West Tower 11F
	1-5-1 Otemachi, Chiyoda-ku
	Tokyo 100-0004 JAPAN
	Attention: Dr. Kenji Harada, Ph.D.
	Principal
	Life Science Investment Group
	Investment Division

  
 — Signature Page
to Series B Investors’ Rights Agreement — 

 SCHEDULE A 

Investors 
 Flagship Ventures Fund 2007,
L.P. 
 Flagship Ventures Fund IV, L.P. 
 Flagship ventures Fund
IV-Rx, L.P. 
 One Memorial Drive, 7th Floor 
 Cambridge,
Massachusetts 02142 
 Attn: Edwin M. Kania, Jr. 
 Third Rock
Ventures, L.P. 
 29 Newbury Street 
 Boston, MA 02116 

Attn.: Kevin Starr 
 JAFCO Super V3 Investment Limited
Partnership 
 Otemachi First Square 
 West Tower 11F 

1-5-1 Otemachi, Chiyoda-ku 
 Tokyo 100-0004 Japan 

Attn: Dr. Kenji Harada, Ph.D. 
 Principal 

Life Science Investment Group 
 Investment Division 

JAFCO Co., Ltd. 

 AMENDMENT NO. 1 TO 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 

This AMENDMENT NO. 1 TO THE AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT (this “Amendment”) is made as of the 17th day of December, 2013 by and among Eleven Biotherapeutics, Inc., a Delaware corporation (the “Company”), and each of the signatories hereto (collectively, the
“Investors”). The Company and the Investors are individually each referred to herein as a “Party” and are collectively referred to herein as the “Parties.” Capitalized terms not defined herein shall have
the meanings given to such terms in the Investors’ Rights Agreement (as defined below). 
 RECITALS 

WHEREAS, the Investors are parties to the Amended and Restated Investors’ Rights Agreement, dated December 4, 2013, by and among the
Company and the parties thereto (the “Investors’ Rights Agreement”); 
 WHEREAS, the Investors’ Rights Agreement
may be amended pursuant to Section 6.6 thereof only with the written consent of the Company and the Requisite Holders; and 
 WHEREAS,
the Investors executing this Amendment constitute the Requisite Holders and the Parties hereto desire to amend the Investors’ Rights Agreement as set forth herein. 

NOW, THEREFORE, in consideration of the mutual covenants contained herein and for other valuable consideration, the receipt of which is hereby
acknowledged, the Parties agree as follows: 
 1. Amendment of Section 1.22. Section 1.22 of the Investors’ Rights
Agreement is hereby amended by deleting Section 1.22 in its entirety and substituting in lieu thereof the following: 
 “1.22
Qualified Public Offering” means the consummation of the Company’s first sale of shares of Common Stock to the public (i) at a price of at least $5.00 per share (subject to appropriate adjustment in the event of any stock
dividend, stock split, combination or other similar recapitalization with respect to the Common Stock) in a firm-commitment underwritten public offering pursuant to an effective registration statement under the Securities Act, resulting in at least
$30,000,000 of gross proceeds to the Company or (ii) in any other underwritten public offering pursuant to an effective registration statement under the Securities Act at a price less than $5.00 per share (subject to appropriate adjustment in
the event of any stock dividend, stock split, combination or other similar recapitalization with respect to the Common Stock) and/or resulting in gross proceeds to the Company of less than $30,000,000 approved by the vote or written consent of the
Requisite Holders.” 

 2. Amendment of Section 3. Section 3 of the Investors’ Rights Agreement is
hereby amended by: 
 (a) deleting Section 3.1(d) in its entirety and substituting in lieu thereof the following: 

“(d) INTENTIONALLY OMITTED.” 

(b) deleting Section 3.1(e) in its entirety and substituting in lieu thereof the following: 

“(e) as soon as practicable, but in any event no later than sixty (60) days after the end of each fiscal year, a
budget and business plan for the next fiscal year (collectively, the “Budget”), approved by the Board of Directors and prepared on a quarterly basis, including balance sheets, income statements, and statements of cash flow for such
months and, promptly after prepared, any other budgets or revised budgets prepared by the Company; and” 
 3. Effectiveness of
Amendment. Except as expressly amended hereby, all terms, conditions and provisions of the Investors’ Rights Agreement shall remain in full force and effect in accordance with the Investors’ Rights Agreement. 

[Remainder of Page Intentionally Left Blank] 

  
 - 2 - 

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

  

			
	COMPANY:
	
	ELEVEN BIOTHERAPEUTICS, INC.
		
	By:	 	 /s/ Abbie Celniker

	Name:	 	Abbie Celniker
	Title:	 	Chief Executive Officer

 [Signature Page to Amendment No. 1 to the Amended and Restated Investors’ Rights Agreement]

			
	INVESTORS:
	
	FLAGSHIP VENTURES FUND 2007, L.P.
		
	By:	 	Flagship Ventures 2007 General Partner, L.L.C., its general partner
		
	By: 	 	 /s/ Noubar Afeyan

	Name:	 	Noubar Afeyan
	Title:	 	
	
	FLAGSHIP VENTURES FUND IV, L.P.
		
	By:	 	Flagship Ventures Fund IV General Partner LLC, its General Partner
		
	By:	 	 /s/ Noubar Afeyan

		 	Manager
	
	FLAGSHIP VENTURES FUND IV-RX, L.P.
		
	By:	 	Flagship Ventures Fund IV General Partner LLC, its General Partner
		
	By:	 	 /s/ Noubar Afeyan

		 	Manager

 [Signature Page to Amendment No. 1 to the Amended and Restated Investors’ Rights Agreement]

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

	Name:	 	Kevin Gillis
	Title:	 	CFO
	
	JAFCO SUPER V3 INVESTMENT LIMITED PARTNERSHIP
		
	By:	 	JAFCO Co., Ltd., its general partner
		
	By:	 	 /s/ Shinichi Fuki

	Name:	 	Shinichi Fuki
	Title:	 	President and CEO
	
	Address:
	Otemachi First Square, West Tower 11F
	1-5-1 Otemachi, Chiyoda-ku
	Tokyo 100-0004 JAPAN
	Attention: Dr. Kenji Harada, Ph.D.
	Principal
	Life Science Investment Group
	Investment Division
	JAFCO Co., Ltd.

 [Signature Page to Amendment No. 1 to the Amended and Restated Investors’ Rights Agreement]EX-10.1

 Exhibit 10.1 

ELEVEN BIOTHERAPEUTICS, INC. 

Amended and Restated 2009 Stock Incentive Plan 

1. Purpose. 
 The purpose
of this plan (the “Plan”) is to secure for Eleven Biotherapeutics, Inc., a Delaware corporation (the “Company”) and its shareholders the benefits arising from capital stock ownership by employees, officers and
directors of, and consultants or advisors to, the Company and its parent and subsidiary corporations who are expected to contribute to the Company’s future growth and success. Under the Plan recipients may be awarded both (i) Options (as
defined in Section 2.1) to purchase the Company’s common stock, par value $0.0001 (“Common Stock”) and (ii) shares of Common Stock (“Restricted Stock Awards”). Except where the context
otherwise requires, the term “Company” shall include any parent and all present and future subsidiaries of the Company as defined in Sections 424(e) and 424(f) of the Internal Revenue Code of 1986, as amended or replaced from time to time
(the “Code”). Those provisions of the Plan which make express reference to Section 422 of the Code shall apply only to Incentive Stock Options (as that term is defined below). Appendix A to this Plan shall apply only to
participants in the Plan who are residents of the State of California. 
 2. Types of Awards and Administration. 

Options. Options granted pursuant to the Plan (“Options”) shall be authorized by action of the board of directors of
the Company (the “Board”) and may be either incentive stock options (“Incentive Stock Options”) meeting the requirements of Section 422 of the Code or nonstatutory Options which are not intended to meet the
requirements of Section 422. All Options when granted are intended to be non-statutory Options, unless the applicable Option Agreement (as defined in Section 5) explicitly states that the Option is intended to be an Incentive Stock
Option. The vesting of Options may be conditioned upon the completion of a specified period of employment with the Company and/or such other conditions or events as the Board may determine. The Board may also provide that Options are immediately
exercisable subject to certain repurchase rights in the Company dependent upon the continued employment of the optionee and/or such other conditions or events as the Board may determine. 

Incentive Stock Options. Incentive Stock Options may only be granted to employees of the Company. For so long as the Code shall so
provide, Options granted to any employee under the Plan (and any other incentive stock option plans of the Company) which are intended to constitute Incentive Stock Options shall not constitute Incentive Stock Options to the extent that such
Options, in the aggregate, become exercisable for the first time in any one calendar year for shares of Common Stock with an aggregate fair market value (determined as of the respective date or dates of grant) of more than $100,000. If an Option is
intended to be an Incentive Stock Option, and if for any reason such Option (or any portion thereof) shall not qualify as an Incentive Stock Option, then, to the extent of such nonqualification, such Option (or portion thereof) shall be regarded as
a non-statutory Option appropriately granted under the Plan provided that such Option (or portion thereof) otherwise meets the Plan’s requirements relating to non-statutory Options. 

 Restricted Stock Awards. The Board in its discretion may grant Restricted Stock Awards,
entitling the recipient to acquire, for a purchase price determined by the Board, shares of Common Stock subject to such restrictions and conditions as the Board may determine at the time of grant (“Restricted Stock”), including
continued employment and/or achievement of pre-established performance goals and objectives. 
 Administration. The Plan shall be
administered by the Board, whose construction and interpretation of the terms and provisions of the Plan shall be final and conclusive. The Board may in its sole discretion authorize issuance of Restricted Stock, the grant of Options and the
issuance of shares upon exercise of such Options as provided in the Plan. The Board shall have authority, subject to the express provisions of the Plan, to construe Restricted Stock Agreements, Option Agreements and the Plan, to prescribe, amend and
rescind rules and regulations relating to the Plan, to determine the terms and provisions of Restricted Stock Agreements and Option Agreements, and to make all other determinations in the judgment of the Board necessary or desirable for the
administration of the Plan. The Board may correct any defect or supply any omission or reconcile any inconsistency in the Plan or in any Restricted Stock Agreement or Option Agreement in the manner and to the extent it shall deem expedient to carry
the Plan into effect and it shall be the sole and final judge of such expediency. No director or person acting pursuant to authority delegated by the Board shall be liable for any action or determination under the Plan made in good faith. The Board
may, to the full extent permitted by of consistent with applicable laws or regulations, delegate any or all of its powers under the Plan to a committee (the “Committee”) appointed by the Board, and if the Committee is so appointed,
to the extent of such delegation, all references to the Board in the Plan shall mean and relate to such Committee, other than references to the Board in this sentence and in Section 18 (as to amendment or termination of the Plan) and
Section 22. 
 3. Eligibility. 

Options may be granted, and Restricted Stock may be issued, to persons who are, at the time of such grant or issuance, employees, officers or
directors of, or consultants or advisors to, the Company; provided, that the class of persons to whom Incentive Stock Options may be granted shall be limited to employees of the Company. 

10% Shareholder. If any employee to whom an Incentive Stock Option is to be granted is, at the time of the grant of such Option, the
owner of stock possessing more than 10% of the total combined voting power of all classes of stock of the Company (after taking into account the attribution of stock ownership rules of Section 424(d) of the Code) (a “Greater Than 10%
Shareholder”), any Incentive Stock Option granted to such individual must: (i) have an exercise price per share of not less than 110% of the fair market value of one share of Common Stock at the time of grant; and (ii) expire by
its terms not more than five years from the date of grant. 
 4. Stock Subject to Plan. 

Subject to adjustment as provided in Section 14 below, the maximum number of shares of Common Stock which may be issued under the
Plan is 8,800,000 shares. If an Option shall expire or terminate for any reason without having been exercised in full, the unpurchased shares subject 

  
 - 2 - 

 
to such Option shall again be available for subsequent Option grants or Restricted Stock Awards under the Plan. If shares of Restricted Stock shall be forfeited to, or otherwise repurchased by,
the Company pursuant to a Restricted Stock Agreement, such repurchased shares shall again be available for subsequent Option grants or Restricted Stock Awards under the Plan. If shares issued upon exercise of an Option are tendered to the Company in
payment of the exercise price of an Option, such tendered shares shall again be available for subsequent Option grants or Restricted Stock Awards under the Plan. 

5. Forms of Restricted Stock Agreements and Option Agreements. 

Option Agreement. Each recipient of an Option shall execute an option agreement (“Option Agreement”) in such form not
inconsistent with the Plan as may be approved by the Board. Such Option Agreements may differ among recipients. 
 Restricted
Stock Agreement. Each recipient of a grant of Restricted Stock shall execute an agreement (“Restricted Stock Agreement”) in such form not inconsistent with the Plan as may be approved by the Board. Such Restricted Stock
Agreements may differ among recipients. 
 “Lock-Up” Agreement. Unless the Board specifies otherwise, each
Restricted Stock Agreement and Option Agreement shall provide that upon the request of the Company or the managing underwriter(s) of any offering of securities of the Company that is the subject of a registration statement filed under the United
States Securities Act of 1933, as amended from time to time (the “Act”), the holder of any Option or the purchaser of any Restricted Stock shall, in connection therewith, agree in writing (in such form as the Company or such
managing underwriter(s) shall request) to the general effect that for a period of time (not to exceed 180 days, plus such additional number of days (not to exceed 35) as may reasonably be requested to enable the underwriter(s) of such offering to
comply with Rule 2711(f) of the Financial Industry Regulatory Authority or any amendment or successor thereto) from the effective date of the registration statement under the Act for such offering, the holder or purchaser will not sell, make any
short sale of, loan, grant any option for the purchase of, or otherwise dispose of any shares of the common stock of the Company owned or controlled by him or her. 

6. Purchase Price. 

General. The purchase price per share of Restricted Stock and per share of stock deliverable upon the exercise of an Option shall be
determined by the Board, provided, however, that in the case of any Option, the exercise price shall not be less than 100% of the fair market value of such stock, as determined by the Board, at the time of grant of such Option, or less than 110% of
such fair market value in the case of any Incentive Stock Option granted to a Greater Than 10% Shareholder. 
 Payment of Purchase
Price. Option Agreements may provide for the payment of the exercise price by delivery of cash or a check to the order of the Company in an amount equal to the exercise price of such Options, or, to the extent provided in the applicable Option
Agreement, by one of the following methods: 
  

	 	(i)	with the consent of the Board, by delivery to the Company of shares of Common Stock; such surrendered shares shall have a fair market value equal in amount to the exercise price of the Options being exercised,

  
 - 3 - 

	 	(ii)	with the consent of the Board, a personal recourse note issued by the optionee to the Company in a principal amount equal to such aggregate exercise price and with such other terms, including interest rate and maturity,
as the Company may determine in its discretion; provided, however, that the interest rate borne by such note shall not be less than the lowest applicable federal rate, as defined in Section 1274(d) of the Code, 

 

	 	(iii)	with the consent of the Board, if the class of Common Stock is registered under the Securities Exchange Act of 1934 at such time, subject to rules as may be established by the Board, by delivery to the Company of a
properly executed exercise notice along with irrevocable instructions to a broker to promptly deliver to the Company cash or a check payable and acceptable to the Company for the purchase price, 

 

	 	(iv)	with the consent of the Board, by reducing the number of Option shares otherwise issuable to the optionee upon exercise of the Option by a number of shares of Common Stock having a fair market value equal to such
aggregate exercise price, 

  

	 	(v)	with the consent of the Board, by any combination of such methods of payment. 

 The fair market
value of any shares of Common Stock or other non-cash consideration which may be delivered upon exercise of an Option shall be determined by the Board. Restricted Stock Agreements may provide for the payment of any purchase price in any manner
approved by the Board at the time of authorizing the issuance thereof. 
 7. Option Period. 

Notwithstanding any other provision of the Plan or any Option Agreement, each Option and all rights thereunder shall expire on the date
specified in the applicable Option Agreement, provided that such date shall not be later than ten years after the date on which the Option is granted (or five years in the case of an Incentive Stock Option granted to a Greater Than 10% Shareholder),
and in either case, shall be subject to earlier termination as provided in the Plan or Option Agreement. 
 8. Exercise of Options.

 General. Each Option shall be exercisable either in full or in installments at such time or times and during such period as shall
be set forth in the Option Agreement evidencing such Option, subject to the provisions of the Plan. To the extent not exercised, installments shall accumulate and be exercisable, in whole or in part, at any time after becoming exercisable, but not
later than the date the Option expires. 

  
 - 4 - 

 Notice of Exercise. An Option may be exercised by the optionee by delivering to the
Company on any business day a written notice specifying the number of shares of Common Stock the optionee then desires to purchase and specifying the address to which the certificates for such shares are to be mailed (the “Notice”),
accompanied by payment for such shares. In addition, the Company may require any individual to whom an Option is granted, as a condition of exercising such Option, to give written assurances (the “Investment Letter”) in a substance
and form satisfactory to the Company to the effect that such individual is acquiring the Common Stock subject to the Option for his or her own account for investment and not with a view to the resale or distribution thereof, and to such other
effects as the Company deems necessary or advisable in order to comply with any securities law(s). 
 Delivery. As promptly as
practicable after receipt of the Notice, the Investment Letter (if required) and payment, the Company shall deliver or cause to be delivered to the optionee certificates for the number of shares with respect to which such Option has been so
exercised, issued in the optionee’s name; provided, however, that such delivery shall be deemed effected for all purposes when the Company or a stock transfer agent shall have deposited such certificates in the United States mail, addressed to
the optionee, at the address specified in the Notice. 
 9. Nontransferability of Options. 

No Option shall be assignable or transferable by the person to whom it is granted, either voluntarily or by operation of law, except by will or
the laws of descent and distribution. During the life of an optionee, an Option shall be exercisable only by the optionee. 
 10.
Termination of Employment; Disability; Death. Except as may be otherwise expressly provided in the terms and conditions of the Option Agreement, Options shall terminate on the earliest to occur of: 

the date of expiration thereof; 

immediately after termination of the optionee’s employment with, or provision of services to, the Company by the Company for Cause (as
hereinafter defined); 
 90 days after the date of voluntary termination of the optionee’s employment with, or provision of services
to, the Company by the optionee (other than for death or permanent disability as defined below); or 
 90 days after the date of
termination of the optionee’s employment with, or provision of services to, the Company by the Company without Cause (other than for death or permanent disability as defined below). 

Until the date on which the Option so expires, the optionee may exercise that portion of his or her Option which is exercisable at the time of
termination of the employment or service relationship. 
 An employment or service relationship between the Company and the optionee shall
be deemed to exist during any period during which the optionee is employed by or providing services 

  
 - 5 - 

 
to the Company. Whether an authorized leave of absence or an absence due to military or government service shall constitute termination of the employment relationship between the Company and the
optionee shall be determined by the Board at the time thereof. 
 For purposes of this Section 10, the term
“Cause” shall mean (a) any material breach by the optionee of any agreement to which the optionee and the Company are both parties, (b) any act (other than retirement) or omission to act by the optionee which may have a
material and adverse effect on the Company’s business or on the optionee’s ability to perform services for the Company, including, without limitation, the commission of any crime (other than minor traffic violations), or (c) any
material misconduct or material neglect of duties by the optionee in connection with the business or affairs of the Company. An optionee’s employment shall be deemed to have been terminated for Cause if the Company determines within thirty
(30) days of the termination of employment (whether such termination was voluntary or involuntary) that termination for Cause was warranted. 

In the event of the permanent and total disability or death of an optionee while in an employment or other relationship with the Company, any
Option held by such optionee shall terminate on the earlier of the date of expiration of the Option or 180 days following the date of such disability or death. After disability or death, the optionee (or in the case of death, his or her executor,
administrator or any person or persons to whom this option may be transferred by will or by laws of descent and distribution) shall have the right, at any time prior to such termination of an Option, to exercise the Option to the extent the optionee
was entitled to exercise such Option as of the date of his or her disability or death. An optionee is permanently and totally disabled if he or she is unable to engage in any substantial gainful activity by reason of any medically determinable
physical or mental impairment which can be expected to last for a continuous period of not less than 12 months; permanent and total disability shall be determined in accordance with Section 22(e)(3) of the Code and the regulations issued
thereunder. 
 11. Rights as a Shareholder. The holder of an Option shall have no rights as a shareholder with respect to any shares
covered by the Option (including, without limitation, any rights to receive dividends or non-cash distributions with respect to such shares) until the date of issue of a stock certificate to him or her for such shares. No adjustment shall be made
for dividends or other rights for which the record date is prior to the date such stock certificate is issued. 
 12. Additional
Provisions. The Board may, in its sole discretion, include additional provisions in Restricted Stock Agreements and Option Agreements, including, without limitation, restrictions on transfer, rights of the Company to repurchase shares of
Restricted Stock or shares of Common Stock acquired upon exercise of Options, commitments to pay cash bonuses, to make, arrange for or guaranty loans or to transfer other property to optionees upon exercise of Options, or such other provisions as
shall be determined by the Board; provided that such additional provisions shall not be inconsistent with any other term or condition of the Plan and such additional provisions shall not be such as to cause any Incentive Stock Option to fail to
qualify as an Incentive Stock Option within the meaning of Section 422 of the Code. 
 13. Acceleration, Extension, Etc. The
Board may, in its sole discretion, (i) accelerate the date or dates on which all or any particular Option or Options may be exercised or (ii) extend the period or periods of time during which all, or any particular, Option or Options may
be exercised. 

  
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 14. Adjustment Upon Changes in Capitalization. 

No Effect of Options upon Certain Corporate Transactions. The existence of outstanding Options shall not affect in any way the right or
power of the Company to make or authorize any or all adjustments, recapitalizations, reorganizations or other changes in the Company’s capital structure or its business, or any merger or consolidation, or any issue of Common Stock, or any issue
of bonds, debentures, preferred or prior preference stock ahead of or affecting the Common Stock or the rights thereof, 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. 
 Adjustment Provisions. If, through or as a
result of any merger, consolidation, sale of all or substantially all of the assets of the Company, reorganization, recapitalization, reclassification, stock dividend, stock split, reverse stock split or other similar transaction, (i) the
outstanding shares of Common Stock are increased, decreased or exchanged for a different number or kind of shares or other securities of the Company, or (ii) additional shares or new or different shares or other securities of the Company or
other non-cash assets are distributed with respect to such shares of Common Stock or other securities, an appropriate and proportionate adjustment shall be made in (a) the maximum number and kind of shares reserved for issuance under the Plan,
(b) the number and kind of shares or other securities subject to any then outstanding Options, and (c) the price for each share or other security subject to any then outstanding Options, so that upon exercise of such Options, in lieu of
the shares of Common Stock for which such Options were then exercisable, the relevant optionee shall be entitled to receive, for the same aggregate consideration, the same total number and kind of shares or other securities, cash or property that
the owner of an equal number of outstanding shares of Common Stock immediately prior to the event requiring adjustment would own as a result of the event. If any such event shall occur, appropriate adjustment shall also be made in the application of
the provisions of this Section 14 and Section 15 with respect to Options and the rights of optionees after the event so that the provisions of such Sections shall be applicable after the event and be as nearly equivalent as practicable in
operation after the event as they were before the event. 
 No Adjustment in Certain Cases. Except as hereinbefore expressly
provided, the issue by the Company of shares of stock of any class, or securities convertible into shares of stock of any class, for cash or property or for labor or services, either upon direct sale or upon the exercise of rights or warrants to
subscribe therefor, or upon conversion of shares or obligations of the Company convertible into such shares or other securities, shall not affect, and no adjustment by reason thereof shall be made with respect to, the number or price of shares of
Common Stock then subject to outstanding options. 
 Board Authority to Make Adjustments. Any adjustments under this
Section 14 will be made by the Board, whose determination as to what adjustments, if any, will be made and the extent thereof will be final, binding and conclusive. No fractional shares will be issued under the Plan on account of any such
adjustments. 

  
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 15. Effect of Certain Transactions. 

General. Except as provided in any Option Agreement or Restricted Stock Agreement to the contrary, if the Company is merged with or
into or consolidated with another corporation under circumstances where the stockholders of the Company immediately prior to such merger or consolidation do not own after such merger or consolidation shares representing at least fifty percent
(50%) of the voting power of the Company or the surviving or resulting corporation, as the case may be, or if shares representing fifty percent (50%) or more of the voting power of the Company are transferred to an Unrelated Third Party,
as hereinafter defined, or if the Company is liquidated, or sells or otherwise disposes of all or substantially all its assets (each such transaction is referred to herein as a “Change in Control Transaction”), the Board, or the board of
directors of any corporation assuming the obligations of the Company, may, in its discretion, take any one or more of the following actions, as to some or all outstanding Options or Restricted Stock Awards (and need not take the same action as to
each such Option or Restricted Stock Award): (i) provide that such Options shall be assumed, or equivalent Options shall be substituted, by the acquiring or succeeding corporation (or an affiliate thereof), provided that any such Options
substituted for Incentive Stock Options shall meet the requirements of Section 424(a) of the Code, (ii) upon written notice to the optionees, provide that all unexercised Options will terminate immediately prior to the consummation of the
Change in Control Transaction unless exercised by the optionee to the extent otherwise then exercisable within a specified period following the date of such notice, (iii) upon written notice to the grantees, provide that all unvested shares of
Restricted Stock shall be repurchased at cost, (iv) make or provide for a cash payment to the optionees equal to the difference between (A) the fair market value of the per share consideration (whether cash, securities or other property or
any combination of the above) the holder of a share of Common Stock will receive upon consummation of the Change in Control Transaction (the “Per Share Transaction Price”) times the number of shares of Common Stock subject to outstanding
vested Options (to the extent then exercisable at prices not equal to or in excess of the Per Share Transaction Price) and (B) the aggregate exercise price of such outstanding vested Options, in exchange for the termination of such Options, or
(v) provide that all or any outstanding Options shall become exercisable and all or any outstanding Restricted Stock Awards shall vest in part or in full immediately prior to such event. To the extent that any Options are exercisable at a price
equal to or in excess of the Per Share Transaction Price, the Board may provide that such Options shall terminate immediately upon the consummation of the Change in Control Transaction without any payment being made to the holders of such Options.
“Unrelated Third Party” shall mean any person who is not, on the date of adoption of this Plan by the Board, a holder of stock of any class or preference or any stock option of the Company. 

Substitute Options. The Company may grant Options in substitution for options held by employees, officers or directors of, or
consultants or advisors to, another corporation who become employees, officers or directors of, or consultants or advisors to, the Company, as the result of a merger or consolidation of the employing corporation with the Company or as a result of
the acquisition by the Company, of property or stock of the employing corporation. The Company may direct that substitute Options be granted on such terms and conditions as the Board considers appropriate in the circumstances. 

Restricted Stock. In the event of a business combination or other transaction of the type detailed in Section 15.1, any
securities, cash or other property received in 

  
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exchange for shares of Restricted Stock shall continue to be governed by the provisions of any Restricted Stock Agreement pursuant to which they were issued, including any provision regarding
vesting, and such securities, cash, or other property may be held in escrow on such terms as the Board may direct, to insure compliance with the terms of any such Restricted Stock Agreement. 

16. No Special Employment Rights. Nothing contained in the Plan or in any Option Agreement or Restricted Stock Agreement shall confer
upon any optionee or holder of Restricted Stock any right with respect to the continuation of his or her employment by the Company or interfere in any way with the right of the Company at any time to terminate such employment or to increase or
decrease his or her compensation. 
 17. Other Employee Benefits. The amount of any compensation deemed to be received by an employee
as a result of the issuance of shares of Restricted Stock or the grant or exercise of an Option or the sale of shares received upon issuance of a Restricted Stock Award or exercise of an Option will not constitute compensation with respect to which
any other employee benefits of such employee are determined, including, without limitation, benefits under any bonus, pension, profit-sharing, life insurance or salary continuation plan, except as otherwise specifically determined by the Board. 

18. Amendment of the Plan. 

The Board may at any time, and from time to time, modify or amend in any respect or terminate the Plan. If shareholder approval is not obtained
within twelve months after any amendment increasing the number of shares authorized under the Plan or changing the class of persons eligible to receive Options under the Plan, no Options granted pursuant to such amendments shall be deemed to be
Incentive Stock Options and no Incentive Stock Options shall be issued pursuant to such amendments thereafter. 
 The termination or any
modification or amendment of the Plan shall not, without the consent of an optionee or the holder of Restricted Stock, adversely affect his or her rights under an Option or Restricted Stock Award previously granted to him or her. With the consent of
the recipient of Restricted Stock or optionee affected, the Board may amend outstanding Restricted Stock Agreements or Option Agreements in a manner not inconsistent with the Plan. 

19. Withholding. The Company shall have the right to deduct from payments of any kind otherwise due to the optionee or recipient of
Restricted Stock, any federal, state or local taxes of any kind required by law to be withheld with respect to issuance of any shares of Restricted Stock or shares issued upon exercise of Options. Prior to delivery of any Common Stock pursuant to
the terms of this Plan, the Board has the right to require that the optionee or recipient of Restricted Stock remit to the Company an amount sufficient to satisfy any minimum tax withholding obligation. Subject to the prior approval of the Company,
which may be withheld by the Company in its sole discretion, the obligor may elect to satisfy any minimum withholding obligations, in whole or in part, (i) by causing the Company to withhold shares of Common Stock otherwise issuable, or
(ii) by delivering to the Company a sufficient number of shares of Common Stock. The shares so withheld shall have a fair market value equal to such minimum withholding obligation. The fair market value of the shares used to satisfy such
minimum withholding 

  
 - 9 - 

 
obligation shall be determined by the Company as of the date that the amount of tax to be withheld is to be determined. A person who has made an election pursuant to this Section 19 may only
satisfy his or her withholding obligation with shares of Common Stock which are not subject to any repurchase, forfeiture, unfulfilled vesting or other similar restrictions. 

20. Effective Date and Duration of the Plan. 

Effective Date. The Plan shall become effective when adopted by the Board. If shareholder approval is not obtained within twelve months
after the date of the Board’s adoption of the Plan, no Options previously granted under the Plan shall be deemed to be Incentive Stock Options and no Incentive Stock Options shall be granted thereafter. Amendments to the Plan not requiring
shareholder approval shall become effective when adopted by the Board. Amendments requiring shareholder approval shall become effective when adopted by the Board, but if shareholder approval is not obtained within twelve months of the Board’s
adoption of such amendment, any Incentive Stock Options granted pursuant to such amendment shall be deemed to be non-statutory Options provided that such Options are authorized by the Plan. Subject to this limitation, Options may be granted under
the Plan at any time after the effective date and before the date fixed for termination of the Plan. 
 Termination. Unless
sooner terminated by action of the Board, the Plan shall terminate upon the close of business on the day next preceding the tenth anniversary of the date of its adoption by the Board. 

21. Provision for Foreign Participants. The Board may, without amending the Plan, modify the terms of Option Agreements or Restricted
Stock Agreements to differ from those specified in the Plan with respect to participants who are foreign nationals or employed outside the United States to recognize differences in laws, rules, regulations or customs of such foreign jurisdictions
with respect to tax, securities, currency, employee benefit or other matters. 
 22. Requirements of Law. The Company shall not be
required to sell or issue any shares under any Option or Restricted Stock Award if the issuance of such shares shall constitute a violation by the optionee, the Restricted Stock Award recipient, or by the Company of any provision of any law or
regulation of any governmental authority. In addition, in connection with the Act, the Company shall not be required to issue any shares upon exercise of any Option unless the Company has received evidence satisfactory to it to the effect that the
holder of such Option will not transfer such shares except pursuant to a registration statement in effect under the Act or unless an opinion of counsel satisfactory to the Company has been received by the Company to the effect that such registration
is not required in connection with any such transfer. Any determination in this connection by the Board shall be final, binding and conclusive. In the event the shares issuable on exercise of an Option are not registered under the Act or under the
securities laws of each relevant state or other jurisdiction, the Company may imprint on the certificate(s) appropriate legends that counsel for the Company considers necessary or advisable to comply with the Act or any such state or other
securities law. The Company may register, but in no event shall be obligated to register, any securities covered by the Plan pursuant to the Act; and in the event any shares are so registered the Company may remove any legend on certificates
representing such shares. The Company shall not be obligated to take any affirmative action in order to cause the exercise of an Option, the grant of any Restricted Stock Award or the issuance of shares pursuant thereto to comply with any law or
regulation of any governmental authority. 

  
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 23. Conversion of Incentive Stock Options into Non-Qualified Options; Termination. The
Board, with the consent of any optionee, may in its discretion take such actions as may be necessary to convert such optionee’s Incentive Stock Options (or any installments or portions of installments thereof) that have not been exercised on
the date of conversion into non-statutory Options at any time prior to the expiration of such Incentive Stock Options, regardless of whether the optionee is an employee of the Company or a parent or subsidiary of the Company at the time of such
conversion. At the time of such conversion, the Board (with the consent of the optionee) may impose such conditions on the exercise of the resulting non-statutory Options as the Board in its discretion may determine, provided that such conditions
shall not be inconsistent with this Plan. Nothing in this Plan shall be deemed to give any optionee the right to have such optionee’s Incentive Stock Options converted into non-statutory Options, and no such conversion shall occur until and
unless the Board takes appropriate action. The Board, with the consent of the optionee, may also terminate any portion of any Incentive Stock Option that has not been exercised at the time of such termination. 

24. Non-Exclusivity of this Plan; Non-Uniform Determinations. Neither the adoption of this Plan by the Board nor the approval of this
Plan by the stockholders of the Company shall be construed as creating any limitations on the power of the Board to adopt such other incentive arrangements as it may deem desirable, including, without limitation, the granting of stock options
otherwise than under this Plan, and such arrangements may be either applicable generally or only in specific cases. 
 The determinations of
the Board under this Plan need not be uniform and may be made by it selectively among persons who receive or are eligible to receive Options or Restricted Stock Awards under this Plan (whether or not such persons are similarly situated). Without
limiting the generality of the foregoing, the Board shall be entitled, among other things, to make non-uniform and selective determinations, and to enter into non-uniform and selective Option Agreements and Restricted Stock Agreements, as to
(a) the persons to receive Options or Restricted Stock Awards under this Plan, (b) the terms and provisions of Options or Restricted Stock Awards, (c) the exercise by the Board of its discretion in respect of the exercise of Options
pursuant to the terms of this Plan, and (d) the treatment of leaves of absence pursuant to Section 10 hereof. 
 25.
Governing Law. This Plan and each Option or Restricted Stock Award shall be governed by the laws of The Commonwealth of Massachusetts, without regard to its principles of conflicts of law. 

Originally Adopted: August 27, 2009 

  
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 APPENDIX A 

TO ELEVEN BIOTHERAPEUTICS, INC. 2009 STOCK INCENTIVE PLAN 

FOR CALIFORNIA RESIDENTS ONLY 

This Appendix to the Eleven Biotherapeutics, Inc. 2009 Stock Incentive Plan (the “Plan”) shall have application only to
participants in the Plan who are residents of the State of California. Capitalized terms contained herein shall have the same meanings given to them in the Plan, unless otherwise provided in this Appendix. Notwithstanding any provision contained
in the Plan to the contrary and to the extent required by applicable law, the following terms and conditions shall apply to all Options and Restricted Stock Awards (collectively “Awards”) granted to residents of the State of California,
until such time as the Common Stock becomes subject to registration under the Securities Act of 1933: 
 1. Awards shall be
nontransferable other than by will or the laws of descent and distribution. Notwithstanding the foregoing, and to the extent permitted by Section 422 of the Code, the Board, in its discretion, may permit distribution of an Award to an inter
vivos or testamentary trust in which the Award is to be passed to beneficiaries upon the death of the trustor (settlor), or by gift to “immediate family” as that term is defined in Rule 16a-1(e) of the United States Exchange Act of 1934.

 2. Unless employment is terminated for Cause, the right to exercise an Option in the event of termination of employment, to the extent
that the optionee is otherwise entitled to exercise an Option on the date employment terminates, shall be 
 (a) at least six months from
the date of termination of employment if termination was caused by death or permanent disability; and 
 (b) at least 30 days from the date
of termination if termination of employment was caused by other than death or permanent disability; 
 (c) but in no event later than the
remaining term of the Option. 
 3. Any Award exercised before shareholder approval is obtained shall be rescinded if shareholder approval
is not obtained within 12 months of the Board’s adoption of the Plan. 

  
 A-1

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