Document:

Amended and Restated Investors' Rights Agreement

 Exhibit 4.02 
 ACUCELA INC. 
 AMENDED AND RESTATED 

INVESTORS’ RIGHTS AGREEMENT 
 This Amended and Restated Investors’ Rights Agreement (the “Agreement”) is made as of the 31st day of May, 2006 by and among Acucela Inc., a Washington corporation (the
“Company”), and each of the investors listed on Schedule A hereto (each, an “Investor,” and collectively, the “Investors”). 

RECITALS 

A. The Company and the Investors listed on Schedule A hereto under the heading Series C Investors have entered into a Series
C Preferred Stock Purchase Agreement (the “Series C Purchase Agreement”) pursuant to which the Company desires to sell to the Investors and the Investors desire to purchase from the Company shares of the Company’s Series C
Preferred Stock (the “Series C Preferred Stock”). A condition to the Investors’ obligations under the Series C Purchase Agreement is that the Company and each Investor enter into a counterpart of this Agreement in order to
provide the Investors with certain rights to register shares of the Company’s Common Stock issuable upon conversion of the Series C Preferred Stock held by the Investors. Capitalized terms not otherwise defined herein shall have the meanings
ascribed to such terms in the Series C Purchase Agreement. 
 B. The Company and the Investors listed on Schedule A
hereto under the heading Series A Investors, each of whom holds shares of the Company’s Series A Preferred Stock (the “Series A Preferred Stock”), and the Investors listed on Schedule A hereto under the heading
Series B Investors, each of whom holds shares of the Company’s Series B Preferred Stock (the “Series B Preferred Stock”), are parties to that certain Amended and Restated Investors’ Rights Agreement dated August 31,
2004 (the “Prior Agreement”) and desire to amend and restate the Prior Agreement in its entirety to read as set forth in this Agreement. The Prior Agreement may be amended with the written consent of the Company and holders of a
majority of the Registrable Securities (as defined in the Prior Agreement) then outstanding. 
 C. The Company and the Investors
each desire to facilitate the purchase by certain of the Investors of shares of Series C Preferred Stock pursuant to the Series C Purchase Agreement by agreeing to the terms and conditions set forth herein. 

AGREEMENT 

The parties hereby agree as follows: 
 1. Registration Rights. The Company and the Investors covenant and agree as follows: 
 1.1 Definitions. For purposes of this Agreement: 
 (a) The terms
“register,” “registered,” and “registration” refer to a registration effected by preparing and filing a registration statement or similar document in compliance with the Securities Act of 1933, as
amended (the “Securities Act”), and the declaration or ordering of effectiveness of such registration statement or document; 

 (b) The term “Registrable Securities” means (i) the shares of Common
Stock issuable or issued upon conversion of the Series A Preferred Stock, Series B Preferred Stock or the Series C Preferred Stock and (ii) any other shares of Common Stock of the Company issued as (or issuable upon the conversion or exercise
of any warrant, right or other security which is issued as) a dividend or other distribution with respect to, or in exchange for or in replacement of, the shares listed in clauses (i) and (ii) of this Section 1.1(b); provided,
however, that the foregoing definition shall exclude in all cases any Registrable Securities sold by a person in a transaction in which his, her or its rights under this Agreement are not assigned. Notwithstanding the foregoing, Common Stock
of other securities shall only be treated as Registrable Securities if and so long as they have not been (A) sold to or through a broker or dealer or underwriter in a public distribution or a public securities transaction or (B) sold in a
transaction exempt from the registration and prospectus delivery requirements of the Securities Act under Section 4(1) thereof so that all transfer restrictions, and restrictive legends with respect thereto, if any, are removed upon the
consummation of such sale; 
 (c) The number of shares of “Registrable Securities then outstanding” shall be
determined by the number of shares of Common Stock outstanding that are, and the number of shares of Common Stock issuable pursuant to then exercisable or convertible securities that are, Registrable Securities; 

(d) The term “Holder” means any person owning or having the right to acquire Registrable Securities or any assignee
thereof in accordance with Section 1.10; 
 (e) The term “Form S-3” means such form under the Securities
Act as in effect on the date hereof or any successor form under the Securities Act; 
 (f) The term “SEC”
means the Securities and Exchange Commission; and 
 (g) The term “Qualified IPO” means a firm commitment
underwritten public offering by the Company of shares of its Common Stock pursuant to a registration statement under the Securities Act, the public offering price of which is not less than $3.50 per share (appropriately adjusted for any stock split,
dividend, combination or other recapitalization) and which results in aggregate cash proceeds to the Company of $25,000,000 (net of underwriting discounts and commissions). 
 1.2 Company Registration. If (but without any obligation to do so) the Company proposes to register (including for this purpose a registration effected by the Company for shareholders other
than the Holders) any of its stock under the Securities Act in connection with the public offering of such securities solely for cash (other than the initial public offering of the Company’s Common Stock, a registration relating solely to the
sale of securities to participants in a Company stock plan or a transaction covered by Rule 145 under the Securities Act, a registration in which the only stock being registered is Common Stock issuable upon conversion of debt securities which
are also being registered, or any registration on any form that 

  
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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 on Form S-4 and
Form S-8), the Company shall, at such time, promptly give each Holder written notice of such registration. Upon the written request of each Holder made within twenty (20) days after mailing of such notice by the Company in accordance with
Section 4.3, the Company shall, subject to the provisions of Section 1.6, cause to be registered under the Securities Act all of the Registrable Securities that each such Holder has requested to be registered. 

1.3 Obligations of the Company. Whenever required under this Section 1 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 best efforts to cause such registration statement to become effective, and, upon the request of the Holders of a majority of the Registrable Securities registered thereunder, keep
such registration statement effective for up to one hundred twenty (120) days. The Company shall not be required to file, cause to become effective or maintain the effectiveness of any registration statement that contemplates a distribution of
securities on a delayed or continuous basis pursuant to Rule 415 under the Securities Act. 
 (b) Prepare and file with
the SEC such amendments and supplements to such registration statement and the prospectus used in connection with such registration statement as may be necessary to comply with the provisions of the Securities Act with respect to the disposition of
all securities covered by such registration statement for up to one hundred twenty (120) days. 
 (c) Furnish to the
Holders such numbers of copies of a prospectus, including a preliminary prospectus, in conformity with the requirements of the Securities Act, and such other documents as they may reasonably request in order to facilitate the disposition of
Registrable Securities owned by them. 
 (d) Use its reasonable best efforts to register and qualify the securities covered by
such registration statement under such other securities or Blue Sky laws of such jurisdictions as shall be reasonably requested by the Holders; provided, however, that the Company shall not be required in connection therewith or as a
condition thereto to qualify to do business or to file a general consent to service of process in any such states or jurisdictions. 
 (e) In the event of any underwritten public offering, enter into and perform its obligations under an underwriting agreement, in usual and customary form, with the managing underwriter of such offering.
Each Holder participating in such underwriting shall also enter into and perform its obligations under such an agreement. 

(f) Notify each Holder of Registrable Securities covered by such registration statement at any time when a prospectus relating thereto
is required to be delivered under the Securities Act of the happening of any event as a result of which the prospectus included in such registration statement, as then in effect, includes an untrue statement of a material fact or omits to state a
material fact required to be stated therein or necessary to make the statements therein not misleading in the light of the circumstances then existing, such obligation to continue for one hundred twenty (120) days. 

  
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 (g) Cause all such Registrable Securities registered pursuant hereunder to be listed on
each securities exchange on which similar securities issued by the Company are then listed. 
 (h) Provide a transfer agent and
registrar for all Registrable Securities registered pursuant hereunder and a CUSIP number for all such Registrable Securities, in each case not later than the effective date of such registration statement. 

(i) Use its reasonable best efforts to furnish, at the request of any Holder requesting registration of Registrable Securities pursuant
to this Section 1, on the date that such Registrable Securities are delivered to the underwriters for sale in connection with a registration pursuant to this Section 1, if such securities are being sold through underwriters, or, if such
securities are not being sold through underwriters, on the date that the registration statement with respect to such securities becomes effective, (i) an opinion, dated such date, of the counsel representing the Company for the purposes of such
registration, in form and substance as is customarily given to underwriters in an underwritten public offering, addressed to the underwriters, if any, and to the Holders of the Registrable Securities included on the registration statement and
(ii) a letter dated such date, from the independent certified public accountants of the Company, in form and substance as is customarily given by independent certified public accountants to underwriters in an underwritten public offering,
addressed to the underwriters, if any, and to the Holders of the Registrable Securities included on the registration statement. 

1.4 Furnish Information. It shall be a condition precedent to the obligations of the Company to take any action pursuant to
this Section 1 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 shall be required to effect the registration of such Holder’s Registrable Securities. 
 1.5
Expenses of Registration. All expenses other than underwriting discounts and commissions incurred in connection with registrations, filings or qualifications of Registrable Securities pursuant to Section 1.2 for each Holder (which
right may be assigned as provided in Section 1.10), including, without limitation, all registration, filing and qualification fees, printers’ and accounting fees, fees and disbursements of counsel for the Company and the reasonable fees
and disbursements, not to exceed $35,000, of special counsel for the selling Holder or Holders selected by them with the approval of the Company, which approval shall not be unreasonably withheld, shall be borne by the Company. 

1.6 Underwriting Requirements. In connection with any offering involving an underwriting of shares of the Company’s
capital stock, the Company shall not be required under Section 1.2 to include any of the Holders’ securities in such underwriting unless they accept the terms of the underwriting as agreed upon between the Company and the underwriters
selected by it (or by other persons entitled to select the underwriters), and then only in such quantity as the underwriters determine in their sole discretion will not jeopardize the success of

  
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the offering by the Company. If the total amount of securities, including Registrable Securities, requested by shareholders to be included in such offering exceeds the amount of Securities sold
other than by the Company that the underwriters determine in their sole discretion 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 determine in their sole discretion will not jeopardize the success of the offering (the securities so included to be apportioned pro rata among the selling shareholders according to the total amount of
securities entitled to be included therein owned by each selling shareholder or in such other proportions as shall mutually be agreed to by such selling shareholders) but in no event shall the amount of securities of the selling Holders included in
the offering be reduced below ten percent (10%) of the total amount of securities included in such offering, unless such offering is a primary-public offering of the Company’s securities, in which case, the selling shareholders may be
excluded if the underwriters make the determination described above and no other shareholder’s securities are included. For purposes of the preceding parenthetical concerning apportionment, for any selling shareholder which is a holder of
Registrable Securities and which is a partnership or corporation, the partners, retired partners and shareholders of such holder, or the estates and family members of any such partners and retired partners and any trusts for the benefit of any of
the foregoing persons, shall be deemed to be a single “selling shareholder,” and any pro-rata reduction with respect to such “selling shareholder” shall be based upon the aggregate amount of shares carrying registration
rights owned by all entities and individuals included in such “selling shareholder,” as defined in this sentence. 

1.7 Delay of Registration. No Holder shall have any right to obtain or seek an injunction restraining or otherwise delaying
any such registration as the result of any controversy that might arise with respect to the interpretation or implementation of this Section 1. 
 1.8 Indemnification. In the event any Registrable Securities are included in a registration statement under this Section 1: 

(a) To the extent permitted by law, the Company will indemnify and hold harmless each Holder, any underwriter (as defined in the
Securities Act) for such Holder and each person, if any, who controls such Holder or underwriter within the meaning of the Securities Act or the Securities Exchange Act of 1934, as amended (the “Exchange Act”), against any losses,
claims, damages or liabilities (joint or several) to which they may become subject under the Securities Act, the Exchange Act or other federal or state law, insofar as such losses, claims, damages or liabilities (or actions in respect thereof) arise
out of or are based upon any of the following statements, omissions or violations (collectively a “Violation”): (i) any untrue statement or alleged untrue statement of a material fact contained in such registration statement,
including any preliminary prospectus or final prospectus contained therein or any amendments or supplements thereto, (ii) the omission or alleged omission to state therein a material fact required to be stated therein, or necessary to make the
statements therein not misleading, or (iii) any violation or alleged violation by the Company of the Securities Act, 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; and the Company will pay to each such Holder, underwriter or controlling person, as incurred, any legal or other expenses reasonably incurred by them in connection with investigating or defending any such loss, claim,
damage, 

  
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liability, or action; provided, however, that the indemnity agreement contained in this Section 1.8(a) shall not apply to amounts paid in settlement of any such loss, claim,
damage, liability or action if such settlement is effected without the consent of the Company (which consent shall not be unreasonably withheld), nor shall the Company be liable to any Holder, underwriter or controlling person for any such loss,
claim, damage, liability or action to the extent that it arises out of or is based upon a Violation that occurs in reliance upon and in conformity with written information furnished expressly for use in connection with such registration by any
Holder, underwriter or controlling person. 
 (b) To the extent permitted by law, each selling Holder will indemnify and hold
harmless the Company, 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, any underwriter, any other Holder selling securities
in such registration statement and any controlling person of any such underwriter or other Holder against any losses, claims, damages or liabilities (joint or several) to which any of the foregoing persons may become subject, under the Securities
Act, the Exchange Act or other federal or state law, insofar as such losses, claims, damages or liabilities (or actions in respect thereto) arise out of or are based, upon any Violation, in each case to the extent (and only to the extent) that such
Violation occurs in reliance upon and in conformity with written information furnished by such Holder expressly for use in connection with such registration; and each such Holder will pay, as incurred, any legal or other expenses reasonably incurred
by any person intended to be indemnified pursuant to this Section 1.8(b), in connection with investigating or defending any such loss, claim, damage, liability, or action; provided, however, that the indemnity agreement contained
in this Section 1.8(b) shall not apply to amounts paid in settlement of any such loss, claim, damage, liability or action if such settlement is effected without the consent of the Holder, which consent shall not be unreasonably withheld;
provided, further, that in no event shall any indemnity under this Section 1.8(b) exceed the net proceeds from the offering received by such Holder, except in the case of willful fraud by such Holder. 

(c) Promptly after receipt by an indemnified party under this Section 1.8 of notice of the commencement of any action (including
any governmental action), such indemnified party will, if a claim in respect thereof is to be made against any indemnifying party under this Section 1.8, deliver to the indemnifying party a written notice of the commencement thereof and the
indemnifying party shall have the right to participate in, and, to the extent the indemnifying party so desires, jointly with any other indemnifying party similarly noticed, to assume the defense thereof with counsel mutually satisfactory to the
parties; provided, however, that an indemnified party (together with all other indemnified parties which may be represented without conflict by one counsel) shall have the right to retain one separate counsel, with the reasonable 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 proceeding. The failure to deliver written notice to the indemnifying party within a reasonable time of the commencement of any such action, if prejudicial to its ability to defend such action,
shall relieve such indemnifying party of any liability to the indemnified party under this Section 1.8, but the omission so to deliver written notice to the indemnifying party will not relieve it of any liability that it may have to any
indemnified party otherwise than under this Section 1.8. 

  
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 (d) If the indemnification provided for in this Section 1.8 is held by a court of
competent jurisdiction to be unavailable to an indemnified party with respect to any loss, liability, claim, damage or expense referred to therein, then the indemnifying party, in lieu of indemnifying such indemnified party hereunder, shall
contribute to the amount paid or payable by such indemnified party as a result of such loss, liability, claim, damage, or expense in such proportion as is appropriate to reflect the relative fault of the indemnifying party on the one hand and of the
indemnified party on the other in connection with the statements or omissions that resulted in such loss, liability, claim, damage or expense, as well as any other relevant equitable considerations; provided, however, that in no event
shall any contribution by a Holder under this Section 1.8(d) exceed the net proceeds from the offering received by such Holder, except in the case of willful fraud by such Holder. 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 alleged untrue statement of a material fact or the omission to state a material fact relates to information supplied by the indemnifying party or by the
indemnified party and the parties’ relative intent, knowledge, access to information, and opportunity to correct or prevent such statement or omission. 
 (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) The
obligations of the Company and the Holders under this Section 1.8 shall survive the completion of any offering of Registrable Securities in a registration statement under this Section 1, and otherwise. 

1.9 Reports Under Securities Exchange Act of 1934. With a view to making available to the Holders the benefits of Rule 144
promulgated under the Securities Act 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 statement on Form S-3, the
Company agrees to: 
 (a) make and keep public information available, as those terms are understood and defined in Rule 144
under the Securities Act, at all times after ninety (90) days after the effective date of the first registration statement filed by the Company for the offering of its securities to the general public so long as the Company remains subject to
the periodic reporting requirements under Section 13 or 15(d) of the Exchange Act; 
 (b) take such action, including the
voluntary registration of its Common Stock under Section 12 of the Exchange Act, as is necessary to enable the Holders to utilize Form S-3 for the sale of their Registrable Securities, such action to be taken as soon as practicable after
the end of the fiscal year in which the first registration statement filed by the Company for the offering of its securities to the general public is declared effective; 
 (c) 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; and 

  
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 (d) furnish to any Holder, so long as the Holder owns any Registrable Securities, forthwith
upon request (i) a written statement by the Company that it has complied with the reporting requirements of Rule 144 (at any time after ninety (90) days after the effective date of the first registration statement filed by the
Company), the Securities Act and the Exchange Act (at any time after it 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 it 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 or pursuant to such form. 
 1.10 Assignment of Registration Rights. The rights to cause the Company to register Registrable Securities pursuant to this Section 1 may be assigned (but only with all related
obligations) by a Holder to a transferee or assignee of at least the lesser of (a) twenty percent (20%) of the original Holder’s shares of such securities or (b) all such securities then held by such Holder, provided the Company
is, within a reasonable time after such transfer, furnished with written notice of the name and address of such transferee or assignee and the securities with respect to which such registration rights are being assigned, and provided further
that such assignment shall be effective only if immediately following such transfer the further disposition of such securities by the transferee or assignee is restricted under the Securities Act. For the purposes of determining the number of shares
of Registrable Securities held by a transferee or assignee, the holdings of transferees and assignees of a partnership who are partners or retired partners of such partnership (including spouses and ancestors, lineal descendants and siblings of such
partners or spouses who acquire Registrable Securities by gift, will or intestate succession) shall be aggregated together and with the partnership; provided, however, that all assignees and transferees who would not qualify
individually for assignment of registration rights shall have a single attorney-in-fact for the purpose of exercising any rights, receiving notices or taking any action under this Section 1. 

1.11 Market-Standoff Agreement. 
 (a) Market-Standoff Period; Agreement. In connection with the initial public offering of the Company’s Securities and upon request of the Company or the underwriters managing such
offering of the Company’s securities, each Holder agrees not to sell, make any short sale of, loan, grant any option for the purchase of, or otherwise dispose of any securities of the Company (other than those included in the registration)
without the prior written consent of the Company or such underwriters, as the case may be, for such period of time (not to exceed 180 days) from the effective date of such registration as may be requested by the Company or such managing underwriters
and to execute an agreement reflecting the foregoing as may be requested by the underwriters at the time of the Company’s initial public offering. In addition, the Holder agrees to be bound by similar restrictions, and to sign a similar
agreement, in connection with no more than one additional registration statement filed within twelve months after the closing date of the initial public offering, provided that the duration of the market-standoff period with respect to such
additional registration shall not exceed ninety (90) days from the effective date of such additional registration statement. 

  
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 (b) Limitations. The obligations described in Section 1.11(a) shall
apply only if all officers and directors of the Company enter into similar agreements, and shall not apply to a registration relating solely to employee benefit plans, or to a registration relating solely to a transaction pursuant to Rule 145
under the Securities Act. 
 (c) Stop-Transfer Instructions. In order to enforce the foregoing covenants, the
Company may impose stop-transfer instructions with respect to the securities of each Holder (and the securities of every other person subject to the restrictions in Section 1.11(a)). 

(d) Transferees Bound. Each Holder agrees that prior to the Company’s initial public offering it will not transfer
securities of the Company unless each transferee agrees in writing to be bound by all of the provisions of this Section 1.11. 
 1.12 Termination of Registration Rights. No Holder shall be entitled to exercise any right provided for in this Section 1 after the earliest of: 

(a) five (5) years following the consummation of a Qualified IPO; 

(b) with respect to any Holder who is not an affiliate of the Company at such time, such time as Rule 144 (without reference to
subsection (k) thereof) or another similar exemption under the Securities Act is available for the sale of all of such Holder’s shares during any three (3) month period without registration; 

(c) the closing of any merger or consolidation of the Company in which the shareholders of the Company immediately prior to such merger
or consolidation retain less than fifty percent (50%) of the voting power of the surviving corporation immediately following such merger or consolidation; provided, however, that this Section 1.12(c) shall not apply to a
merger effected exclusively for the purpose of changing the domicile of the Company; or 
 (d) any sale of all or substantially
all of the Company’s assets. 
 2. Information Rights. 

2.1 Delivery of Financial Statements. So long as (i) at least twenty percent (20%) of the shares of Series A
Preferred Stock, at least twenty percent (20%) of the shares of Series B Preferred Stock and at least twenty percent (20%) of the shares of Series C Preferred Stock originally issued to the Investors remains outstanding and
(ii) the Company is not subject to the periodic reporting requirements of the Exchange Act, the Company shall deliver to each Investor: 
 (a) as soon as practicable, but in any event within ninety (90) days after the end of each fiscal year of the Company, an income statement for such fiscal year, a balance sheet of the Company and
statement of shareholder’s equity as of the end of such year, and a statement of cash flows for such year, such year-end financial reports to be in reasonable detail and prepared in accordance with generally accepted accounting principles
(“GAAP”); 

  
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 (b) as soon as practicable, but in any event within forty-five (45) days after the end
of each of the first three (3) quarters of each fiscal year of the Company, an unaudited profit or loss statement, a statement of cash flows for such fiscal quarter and an unaudited balance sheet as of the end of such fiscal quarter;

 (c) within thirty (30) days of the end of each month, an unaudited income statement and a statement of cash flows and
balance sheet for and as of the end of such month, in reasonable detail; 
 (d) as soon as practicable, but in any event no
later than thirty (30) days prior to the end of each fiscal year, a budget and business plan for the next fiscal year, prepared on a monthly basis, and, as soon as prepared, any other budgets or revised budgets prepared by the Company; and

 (e) with respect to the financial statements called for in subsections (b) and (c) of this Section 2.1, an
instrument executed by the Chief Financial Officer or President of the Company and certifying that such financials were prepared in accordance with GAAP consistently applied with prior practice for earlier periods (with the exception of footnotes
that may be required by GAAP) and fairly present the financial condition of the Company and its results of operation for the period specified, subject to year-end audit adjustment, provided that the foregoing shall not restrict the right of the
Company to change its accounting principles consistent with GAAP, if the Company’s Board of Directors determines that it is in the best interest of the Company to do so. 
 2.2 Inspection. The Company shall permit a single representative of the Investors, at the Investors’ expense, to visit and inspect the Company’s properties, to examine its books of
account and records and to discuss the Company’s affairs, finances and accounts with its officers, all at such reasonable times as may be requested by the Investors; provided, however, that the Company shall not be obligated pursuant to this
Section 2.2 to provide access to any information that it reasonably considers to be a trade secret or similar confidential information. The rights under this Section 2.2 may not be assigned or otherwise conveyed without the prior written
consent of the Company and may not be exercised more than twice in any calendar year. 
 2.3 Termination of Information
Rights. The covenants in this Section 2 shall terminate upon the earliest to occur of any one of the following events: 
 (a) a Qualified IPO; 
 (b) the Company becoming subject to the periodic reporting
requirements of the Exchange Act; 
 (c) the sale, conveyance or disposal of all or substantially all of the Company’s
property or business or the Company’s merger with or into or consolidation with any other corporation (other than a wholly owned subsidiary corporation) or if the Company effects any other transaction or series of related transactions in which
more than fifty percent (50%) of the voting power of the Company is disposed of and the Company is not the survivor; provided, however, that this Section 2.3(c) shall not apply to a merger effected exclusively for the purpose of changing
the domicile of the Company; 

  
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 (d) the liquidation, dissolution or indefinite cessation of the business operations of the
Company; or 
 (e) the execution by the Company of a general assignment for the benefit of creditors or the appointment of a
receiver or trustee to take possession of the property and assets of the Company. 
 3. Right of First Offer.

 3.1 Notice and Exercise of Right of First Offer. Subject to the terms and conditions specified in this
Section 3, the Company hereby grants to (i) each Investor holding at least 500,000 shares of Series A Preferred Stock, Series B Preferred Stock or Series C Preferred Stock and (ii) each holder of those certain
promissory notes issued by the Company as of May 29, 2006 (each Investor and holder referred to in the foregoing clauses (i) and (ii), a “Major Investor”) a right of first offer (the “Right of First
Offer”) with respect to future sales by the Company of its New Shares (as hereinafter defined). A Major Investor may designate as purchasers under such right itself or its partners or affiliates in such proportions as it deems appropriate.
Each time the Company proposes to offer any shares of, or securities convertible into or exercisable for any shares of, any class of its capital stock (“New Shares”), the Company shall first make an offering of such New Shares to
each Major Investor in accordance with the following provisions: 
 (a) The Company shall deliver a notice by certified mail
(“Notice”) to the Major Investors stating (i) its bona fide intention to offer such New Shares, (ii) the number of such New Shares to be offered, and (iii) the price and terms, if any, upon which it proposes to offer
such New Shares. 
 (b) Within fifteen (15) days after delivery of the Notice, each Major Investor may elect to purchase
or obtain, at the price and on the terms specified in the Notice, up to that portion of such New Shares that equals the proportion that the number of shares of Common Stock issued and held, or issuable upon conversion and exercise of all convertible
or exercisable securities then held, by such Major Investor bears to the total number of shares of Common Stock then outstanding (assuming full conversion and exercise of all convertible or exercisable securities). 

(c) The Company may, during the forty-five (45) day period following the expiration of the period provided in Section 3.1(b)
hereof, offer the remaining unsubscribed portion of the New Shares 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 Notice. If the Company does not enter into an
agreement for the sale of the New Shares within such period, or if such agreement is not consummated within sixty (60) days of the execution thereof, the right provided hereunder shall be deemed to be revived and such New Shares shall not be
offered unless first reoffered to the Major Investors in accordance herewith. 
 (d) The Right of First Offer in this
Section 3 shall not be applicable to the issuance or sale of (i) New Shares issued or issuable upon conversion of the Series A Preferred Stock, Series B Preferred Stock or Series C Preferred Stock; (ii) up to 2,000,000 New

  
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Shares issued or issuable to officers, directors, employees or consultants pursuant to stock option, stock grant, stock purchase or similar plans or arrangements approved by the Company’s
Board of Directors, including without limitation upon the exercise of stock options thereunder; (iii) capital stock, or options or warrants to purchase capital stock, issued to financial institutions or lessors in connection with commercial
credit arrangements, equipment financings, commercial property lease transactions or similar transactions approved by the Company’s Board of Directors; (iv) New Shares issuable pursuant to warrants, notes or other rights to acquire
securities of the Company outstanding as of the date hereof; (v) New Shares issued in connection with a stock combination, consolidated or subdivision, as a dividend or other distribution, or a recapitalization; (vi) New Shares issued or
issuable in a Qualified IPO or any subsequent public offering; (vii) New Shares issued or issuable in connection with a merger or acquisition transaction involving the Company; (viii) New Shares issued or issuable to strategic commercial
partners or counterparties of the Company; (ix) New Shares issued or issuable in offerings to persons other than existing holders of the Company’s securities that are approved unanimously by the Company’s Board of Directors; or
(x) New Shares issued or issuable with the affirmative vote of at least a majority of the then outstanding shares of Series A Preferred Stock, Series B Preferred Stock and Series C Preferred Stock, voting together as a single class. 

(e) The non-exercise of the rights of an Investor hereunder to purchase New Shares offered by the Company shall terminate such
Investor’s right to participate in subsequent offerings of New Shares by the Company. 
 3.2 Termination of Right of
First Offer. The covenants in this Section 3 shall terminate upon the earliest to occur of any one of the following events: 
 (a) a Qualified IPO; 
 (b) the Company becoming subject to the periodic reporting
requirements of the Exchange Act; 
 (c) the sale, conveyance or disposal of all or substantially all of the Company’s
property or business or the Company’s merger with or into or consolidation with any other corporation (other than a wholly owned subsidiary corporation) or if the Company effects any other transaction or series of related transactions in which
more than fifty percent (50%) of the voting power of the Company is disposed of and the Company is not the survivor; provided, however, that this Section 3.2(c) shall not apply to a merger effected exclusively for the purpose
of changing the domicile of the Company; 
 (d) the liquidation, dissolution or indefinite cessation of the business operations
of the Company; or 
 (e) the execution by the Company of a general assignment for the benefit of creditors or the appointment
of a receiver or trustee to take possession of the property and assets of the Company. 

  
 12 

 4. Miscellaneous. 

4.1 Successors and Assigns. Except as otherwise provided in this Agreement, the terms and conditions of this Agreement
shall inure to the benefit of and be binding upon the respective permitted successors and assigns of the parties (including transferees of any of the Series A Preferred Stock, Series B Preferred Stock, Series C Preferred Stock or any Common Stock
issued upon conversion thereof). Nothing in this Agreement, express or implied, is intended to confer upon any party other than the parties hereto or their respective successors and assigns any rights, remedies, obligations or liabilities under or
by reason of this Agreement, except as expressly provided in this Agreement. 
 4.2 Amendments and Waivers. Any
term of this Agreement may be amended or waived only with the written consent of the Company and the holders of a majority of the Registrable Securities then outstanding. Any amendment or waiver effected in accordance with this paragraph shall be
binding upon each holder of any Registrable Securities then outstanding, each future holder of all such Registrable Securities, and the Company. Notwithstanding the foregoing, this Agreement may be amended to add Additional Investors (as defined in
the Series C Purchase Agreement) purchasing the Company’s Series C Preferred Stock issued and sold pursuant to the Series C Purchase Agreement who have executed a counterpart signature page in the form attached hereto as Exhibit 1 and the
Company shall, concurrently therewith, amend Schedule A hereto to reflect the addition of such Additional Investors to this Agreement. Each such Additional Investor so joined as a party hereto shall be deemed an “Investor” hereunder.

 4.3 Notices. Unless otherwise provided, any notice required or permitted by this Agreement shall be in writing
and shall be deemed sufficient upon delivery, when delivered personally or by overnight courier or sent by telegram or fax, or forty-eight (48) hours after being deposited in the U.S. mail, as certified or registered mail, with postage prepaid,
and addressed to the party to be notified at such party’s address or fax number as set forth below or on Schedule A hereto, or as subsequently modified by written notice. 

4.4 Consent to Electronic Delivery of Shareholder Notices. Each of the undersigned Investors who has provided a facsimile
number or email address on his, her or its respective signature page hereto consents to the delivery to such Investor by the Company of all future notices and other communications to directors, if applicable, and shareholders by email to the address
set forth on such signature page and/or by facsimile transmission to the number set forth on such signature page. Each Investor understands that he, she or it may revoke this consent at any time by giving notice to the Secretary of the Company.

 4.5 Severability. If one or more provisions of this Agreement are held to be unenforceable under applicable
law, the parties agree to renegotiate such provision in good faith. In the event that the parties cannot reach a mutually agreeable and enforceable replacement for such provision, then (a) such provision shall be excluded from this Agreement,
(b) the balance of this Agreement shall be interpreted as if such provision were so excluded, and (c) the balance of this Agreement shall be enforceable in accordance with its terms. 

  
 13 

 4.6 Governing Law and Venue. This Agreement and all acts and transactions
pursuant hereto shall be governed, construed and interpreted in accordance with the laws of the state of Washington, without giving effect to principles of conflicts of laws. Each party hereto expressly consents to the exclusive personal
jurisdiction of and venue in the U.S. federal and state courts located in King County, Washington. 
 4.7
Counterparts. 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. 

4.8 Titles and Subtitles. The titles and subtitles used in this Agreement are used for convenience only and are not to be
considered in construing or interpreting this Agreement. 
 4.9 Aggregation of Stock. All shares of the Series A
Preferred Stock, Series B Preferred Stock or Series C Preferred Stock held or acquired by affiliated entities or persons shall be aggregated together for the purpose of determining the availability of any rights under this Agreement. 

4.10 Confidentiality. Anything in this Agreement to the contrary notwithstanding, no Holder by reason of this Agreement
shall have access to any trade secrets or similar confidential information of the Company. Each Holder hereby agrees to hold in confidence and trust and not to misuse or disclose to any third party any confidential information received by such
Holder pursuant to this Agreement. 
 4.11 Amendment of Prior Agreement. This Agreement amends and restates the
Prior Agreement in its entirety and the Prior Agreement shall be of no further force or effect. 
 [Signature Page Follows]

  
 14 

 The parties have executed this Agreement as of the date first above written. 

COMPANY: 
  

			
	ACUCELA INC.
		
	By:	 	/s/ Ryo Kubota
		 	 Ryo Kubota, M.D., Ph.D.

President and Chief Executive Officer

		
	Address:	 	454 North 34th Street
		 	Seattle, WA 98103
	Telephone:	 	(206) 812-1530
	Facsimile:	 	(206) 545-3885

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

					
		 	FUJI PHOTO FILM CO., LTD.
			
		 	By: 	  	/s/ Yuzo Toda
		 	Print Name:	  	Yuzo Toda
		 	Title:	  	 Corporate Vice President, General
 Manager, Life Science Products Div.

			
		 	 Address:    
	  	 26-30 Nishiazabu 2-Chome
 Minato-ku,Tokyo 106-8620

JAPAN       

		 	Tel: 	  	 
		 	Fax: 	  	 
		 	Email:	  	 
		
		 	JPE MEDIBIC I L.P.
			
		 	By:	  	JPE Capital Management Ltd.,
		 		  	its General Partner
			
		 	By: 	  	/s/ Franco Ng
		 	Print Name:	  	Ng Tsz Shun
		 	Title:	  	Director
			
		 	 Address:  
	  	 P.O. Box 3093 GT Ugland House

South Church Street
 George Town, Grand
Cayman
 CAYMAN ISLANDS

		 	Tel:	  	 
		 	Fax:	  	 
		 	Email:	  	 
		
		 	HIROSHI KONISHI
		
		 	/s/ Hiroshi Konishi
			
		 	 Address:    
	  	 
		 	Tel:	  	 
		 	Fax:	  	 
		 	Email:	  	 

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

					
		 	MEDIBIC ALLIANCE
			
		 	By: 	  	/s/ Hikaru Kobayashi
		 	Print Name:	  	Hikaru Kobayashi
		 	Title:	  	Chief Executive Officer
			
		 	 Address:    
	  	 Daido Kasumigaseki Bldg. 8F

1-4-2 Kasumigaseki
 Chiyoda-ku,Tokyo 100-0013

JAPAN       

		 	Tel:	  	 
		 	Fax:	  	 
		 	Email:	  	 
		
		 	HIKARU SHIMURA
		
		 	/s/ Hikaru Shimura
			
		 	 Address:    
	  	 
			
		 	Tel:	  	 
		 	Fax:	  	 
		 	Email:	  	 
		
		 	SHIN NIHON JITSUGYO CO., LTD.
			
		 	By: 	  	/s/ Hiroshi Kinoshi
		 	Print Name:	  	Hiroshi Kinoshi
		 	Title:	  	President
			
		 	 Address:    
	  	 6-6-5, Ginza
 Chuo-ku,    Tokyo 104-0061

JAPAN       

			
		 	Attn:	  	Rika Sugino
		 	Tel: 	  	 
		 	Fax: 	  	 
		 	Email:	  	 

 PRIOR INVESTORS: 

 

			
	 AQUA RIMCO BIOTECHNOLOGY NO. 2
 INVESTMENT PARTNERSHIP

		
	By:	 	/s/ Yoshihiko Takamiya
	Print Name:	 	Yoshihiko Takamiya
	Title:	 	 President of Aqua RIMCO, Ltd.

of its General Partner

		
	 Address:    
	 	 Kawate Building 1-5-8 Nishi
 Shimbashi 
 Minato-ku,Tokyo 105-0003

JAPAN       

		
	Attn:	 	Yoshihiko Takamiya
	Tel:	 	 
	Fax:	 	 
	Email:	 	 
	
	BEREVNO CORPORATION
		
	By:	 	 
	Print Name:	 	 
	Title:	 	 
		
	 Address:    
	 	 4-2, Ohtemachi 1-Chome
 Chiyoda-ku,Tokyo 100-8088

JAPAN       

	Tel:	 	 
	Fax:	 	 
	Email:	 	 

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 PRIOR INVESTORS: 

 

			
	BIOVISION LIFE SCIENCE FUND 1
		
	By:	 	 Softbank Investment Corporation,

its General Partner

		
	By: 	 	 /s/ [illegible]

	Print Name:	 	  

	Title:	 	  

		
	Address:	 	 1-6-1, Roppongi
 Minato-ku,
Tokyo 106-6020
 JAPAN
 Attn: Yoshitaka
Kitao

		
	Tel:	 	 
	Fax:	 	 
	Email:	 	 
	
	DNP HOLDING USA CORPORATION
		
	By:	 	  

	Print Name:	 	  

	Title:	 	  

		
	Address:	 	 335 Madison Avenue, Third Floor

New York, NY 10017

	Tel:	 	  

	Fax:	 	  

	Email:	 	  

	
	IKEGIN CAPITAL CO., LTD.
		
	By: 	 	 /s/ Toshiaki Jimbow

	Print Name:	 	Toshiaki Jimbow
	Title: 	 	President
		
	Address:	 	 4-1-9, Minami Semba
 Chuo-ku,
Osaka 542-0081
 JAPAN
 Attn: Toshiaki
Jimbow

		
	Tel: 	 	 
	Fax: 	 	 
	Email:	 	 

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 PRIOR INVESTORS: 

 

			
	JAPAN ASIA INVESTMENT CO., LTD.
		
	By:	 	  

	Print Name:	 	  

	Title:	 	  

		
	Address:	 	 2-13-5, Nagata-cho
 Chiyoda-ku,
Tokyo 100-8972
 JAPAN
 Attn: Toyoji
Tatsuoka

		
	Tel:	 	  

	Fax:	 	  

	Email:	 	  

	
	MARUBENI CORPORATION
		
	By: 	 	 /s/ Toshihide Kubo

	Print Name:	 	Toshihide Kubo
	Title:	 	General Manager, Business Incubation Dept.
		
	Address:	 	 4-2, Ohtemachi 1-Chome

Chiyoda-ku, Tokyo 100-8088

JAPAN

		
	Tel:	 	  

	Fax:	 	  

	Email:	 	  

	
	MARUBENI VENTURE CAPITAL FUND 1 L.P.
		
	By:	 	 Sigma Capital Corporation,
 its
General Partner

		
	By:	 	  

	Print Name:	 	  

	Title:	 	  

		
	Address:	 	 1-4-1, Uchikanda, Chiyoda-ku

Tokyo, 101-0047
 JAPAN

		
	Tel:	 	  

	Fax:	 	  

	Email:	 	  

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 PRIOR INVESTORS: 

 

			
	MITSUBISHI UFJ CAPITAL CO., LTD.
		
	By: 	 	 /s/ Kazuhiko Tokita

	Print Name:	 	Kazuhiko Tokita
	Title: 	 	President
		
	Address:	 	 14-1 2-Chome, Kyobashi

Chuo-ku, Tokyo 104-0031

JAPAN

		
	Tel: 	 	  

	Fax: 	 	  

	Email:	 	  

	
	 MITSUI SUMITOMO INSURANCE C
 GLOBAL TECHNOLOGY VENTURE
 CAPITAL INVESTMENT LIMITED

PARTNERSHIP

		
	By:	 	  

	Print Name:	 	  

	Title:	 	  

		
	Address:	 	 Technology Venture Capital

Investment Limited Partnership
 Yaesu Nagoya
Bldg. 3F, 2-10, Yaesu
 2-Chome

Chuo-ku, Tokyo, 104-0028

JAPAN

		
	Tel:	 	  

	Fax:	 	  

	Email:	 	  

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 PRIOR INVESTORS: 

 

			
	MIZUHO CAPITAL NO. 1 LIMITED PARTNERSHIP
		
	By:	 	 Mizuho Capital Co., Ltd.,
 its
General Partner

		
	By: 	 	 /s/ Osamu Kita

	Print Name:	 	Osamu Kita
	Title:	 	 President of Mizuho Capital Co., Ltd.
 Acting as General Partner

		
	Address:	 	 4-3, Nihombashi-kabutocho

Chuo-ku, Tokyo 103-0026
 JAPAN

Attn: Osamu Kita

		
	Tel: 	 	  

	Fax: 	 	  

	Email:	 	  

	
	 NVCC KANSAI NO. 3 VENTURE
 CAPITAL INVESTMENT LIMITED
 PARTNERSHIP

		
	By:	 	 Nippon Venture Capital Co., Ltd.,
 its General Partner

		
	By:	 	  

	Print Name:	 	  

	Title:	 	  

		
	Address:	 	 7-1-16 Akasaka
 Minato-ku,
Tokyo 107-0052
 JAPAN

		
	Tel:	 	  

	Fax:	 	  

	Email:	 	  

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 PRIOR INVESTORS: 

 

			
	 NVCC NO. 4 VENTURE CAPITAL
 INVESTMENT LIMITED PARTNERSHIP

		
	By:	 	 Nippon Venture Capital Co., Ltd.,
 its General Partner

		
	By:	 	  

	Print Name:	 	  

	Title:	 	  

		
	Address:	 	 7-1-16 Akasaka
 Minato-ku,
Tokyo 107-0052
 JAPAN

		
	Tel:	 	  

	Fax:	 	  

	Email:	 	  

	
	OLYMPUS CORPORATION
		
	By:	 	  

	Print Name:	 	  

	Title:	 	  

		
	Address:	 	 2-3 Kuboyama-cho

Hachioji-shi
 Tokyo 192-8512

JAPAN

		
	Tel:	 	+81-426-91-7111
	Fax:	 	+81-426-91-7932
	Email:	 	  

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 PRIOR INVESTORS: 

 

			
	ORIX FUND NO. 7
	By:	 	ORIX Capital Corporation,
		 	its General Partner
		
	By:	 	  

	Print Name:	 	  

	Title:	 	  

		
	Address:	 	 2-4-1, Hamamatsu-cho

Minato-ku
 Tokyo 105-6132

JAPAN
 Attn: Akira Hirose

		
	Tel:	 	  

	Fax:	 	  

	Email:	 	  

	
	ORIX FUND NO. 8
		
	By:	 	 ORIX Capital Corporation,
 its
Executive Partner

		
	By:	 	  

	Print Name:	 	  

	Title:	 	  

		
	Address:	 	 2-4-1, Hamamatsu-cho

Minato-ku
 Tokyo 105-6132

JAPAN
 Attn: Akira Hirose

		
	Tel:	 	  

	Fax:	 	  

	Email:	 	  

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 PRIOR INVESTORS: 

 

			
	RESONA CAPITAL CO., LTD.
		
	By:	 	 
	Print Name: 	 	 
	Title:	 	 
		
	 Address:    
	 	 3-1, Kyobashi 1-Chome
 Chuo-Ku,
Tokyo
 JAPAN
 Attn: Hirohide
Takahashi

		
	Tel:	 	 
	Fax:	 	 
	Email:	 	 
	
	SHIN NIPPON BIOMEDICAL LABORATORIES, LTD.
		
	By:	 	 
	Print Name: 	 	 
	Title:	 	 
		
	 Address:    
	 	 Toho Twin Tower Building, 6th FL.
 1-5-2 Yurakucho
 Chiyoda-ku
 Tokyo, 100-0006
 JAPAN
 Attn: Ryoichi Nagata, M.D., Ph.D., FFPM

		
	Tel:	 	 
	Fax:	 	 
	Email:	 	 

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 PRIOR INVESTORS: 

 

			
	SHIN-ETSU CHEMICAL CO., LTD.
		
	By: 	 	/s/ Hiroaki Okamoto
	Print Name:	 	Hiroaki Okamoto
	Title:	 	General Manager, R&D Dept.
		
	 Address:    
	 	 6-1 Ohtemachi 2-chome

Chiyoda-ku
 Tokyo, 100-0004

JAPAN

		
	Tel: 	 	 
	Fax: 	 	 
	Email:	 	 
	
	 SMBC CAPITAL NO 6 VENTURE
 CAPITAL INVESTMENT LIMITED PARTNERSHIP

		
	By:	 	 NIF SMBC Ventures Co., Ltd.,

its General Partner

		
	By:	 	/s/ [illegible]
	Print Name:	 	[illegible]
	Title:	 	[illegible]
		
	 Address:    
	 	 1-2-1 Kyobashi Chuo-ku
 Tokyo,
104-0031 JAPAN
 Attn: Michihiro Matsuda

		
	Tel:	 	 
	Fax:	 	 
	Email:	 	 

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 PRIOR INVESTORS: 

 

			
	SOFTBANK INTERNET FUND
	By:	 	 Soft Trend Capital Corp.,
 its
General Partner

		
	By: 	 	/s/ Takashi Nakagawa
	Print Name: 	 	 
	Title:	 	 
		
	 Address:    
	 	 1-6-1, Roppongi
 Minato-ku,
Tokyo 106-6020
 JAPAN
 Attn: Yoshitaka
Kitao

		
	Tel:	 	 
	Fax:	 	 
	Email:	 	 
	
	TRANS-SCIENCE NO. 1 INVESTMENT LIMITED PARTNERSHIP
		
	By: 	 	/s/ Kiyoshi Inoue
	Print Name:	 	Kiyoshi Inoue
	Title:	 	 President & CEO

Trans-Science, Inc.

		
	 Address:    
	 	 1-1-1 Uchisaiwai-cho, Chiyoda-Ku

Tokyo 100-0011
 JAPAN

Attn: Kiyoshi Inoue

		
	Tel: 	 	 
	Fax: 	 	 
	Email:	 	 

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 PRIOR INVESTORS: 

 

			
	TRANS-SCIENCE NO. 2A INVESTMENT LIMITED PARTNERSHIP
		
	By:	 	 /s/ Kiyoshi Inoue

	Print Name:	 	Kiyoshi Inoue
	Title:	 	 President & CEO

Trans-Science, Inc.

		
	 Address:    
	 	 1-1-1 Uchisaiwai-cho, Chiyoda-Ku

Tokyo 100-0011
 JAPAN

Attn: Kiyoshi Inoue

		
	Tel: 	 	  

	Fax: 	 	  

	Email:	 	  

	
	UOB JAIC VENTURE BIO INVESTMENTS LTD.
		
	By:	 	  

	Print Name:	 	  

	Title:	 	  

		
	 Address:    
	 	 50 Raffles Place #24-03

Singapore Land Tower 5
 SINGAPORE
048623
 Attn: Toyoji Tatsuoka

		
	Tel:	 	  

	Fax:	 	  

	Email:	 	  

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 EXHIBIT 1 
 COUNTERPART SIGNATURE PAGE TO 
 AMENDED AND RESTATED INVESTORS’
RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of
April     , 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the
conditions of, the Amended and Restated Investors’ Rights Agreement, dated as of April     , 2006 (the “Investors’ Rights Agreement”), by and among the Company and the Investors, as an
“Investor” thereunder. 
 The undersigned has executed this Counterpart to the Investors’ Rights Agreement as of
            , 200    . 
  

			
	INVESTOR:
	
	 MITSUI SUMITOMO INSURANCE C
 GLOBAL TECHNOLOGY VENTURE
 CAPITAL INVESTMENT LIMITED PARTNERSHIP

	(Print name of individual or corporate Investor)
		
	By:	 	 /s/ Takeaki Mori

		 	Name:   Takeaki Mori
		 	Title:     President
		
	Address:	 	Yaesu Nagoya Bldg. 3F, 2-10,Yaesu 2-Chome
		 	Chuo-ku, Tokyo, 104-0028
		
	Telephone:	 	
	Facsimile:	 	

  

			
	Accepted and Agreed:
	
	ACUCELA INC.
		
	By:	 	 
	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	 454 North 34th Street
 Seattle,
WA 98103

		
	Telephone:	 	(206) 812-1530
	Facsimile:	 	(206) 545-3885

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 EXHIBIT 1 
 COUNTERPART SIGNATURE PAGE TO 
 AMENDED AND RESTATED INVESTORS’
RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of
May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and
Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 

The undersigned has executed this Counterpart to the Investors’ Rights Agreement as of June     ,
2006. 
  

			
	FUJI PHOTO FILM CO., LTD.
		
	By:	 	/s/ Yuzo Toda
	Print Name:	 	Yuzo Toda
	Title:	 	Corporate Vice President, General Manager, Life Science Products Div.
		
	Address:	 	 26-30 Nishiazabu 2-Chome

Minato-ku
 Tokyo, 106-8620

JAPAN

		
	Telephone:	 	
	Facsimile:	 	
		 	

  

			
	Accepted and Agreed:
	
	ACUCELA INC.
		
	By:	 	 
	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	 454 North 34th Street
 Seattle,
WA 98103

		
	Telephone:	 	(206) 812-1530
	Facsimile:	 	(206) 545-3885

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 COUNTERPART SIGNATURE PAGE TO 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined
therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights
Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 
 The undersigned has
executed this Counterpart to the Investors’ Rights Agreement as of June 30, 2006. 
  

			
	INVESTOR:
	
	BRAIN STAFF CONSULTANTS INC.
		
	By:	 	/s/ Toyonori Tanaka
		 	Name: Toyonori Tanaka
		 	Title: Representative Director
		
	 Address:
	 	10-15, 1-Chome, Shimanouchi
		 	 Chuo-Ku, Osaka-city 542-0082

		 	 JAPAN

	 Telephone:
	 	
	 Facsimile:
	 	

  

			
	Accepted and Agreed:
	
	ACUCELA INC.
		
	By:	 	/s/ Ryo Kubota
	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	454 North 34th Street
		 	Seattle, WA 98103
		
	Telephone:	 	(206) 812-1530
	Facsimile:	 	(206) 545-3885

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 COUNTERPART SIGNATURE PAGE TO 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined
therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights
Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 
 The undersigned has
executed this Counterpart to the Investors’ Rights Agreement as of June 30, 2006. 
  

			
	INVESTOR:
	
	JIKEI NO.1 LIMITED PARTNERSHIP FUND
		
	By:	 	JIK CO., LTD., its General Partner
		
	By:	 	/s/ Hiroki Hideshima
		 	Name: Hiroki Hideshima
		 	Title: Representative Director
		
	Address:	 	11-30, 1-Chome, Shimanouchi,
		 	Chuo-Ku, Osaka 542-0082
		 	JAPAN
		
	Telephone:	 	
	Facsimile:	 	

  

			
	Accepted and Agreed:
	
	ACUCELA INC.
		
	By:	 	/s/ Ryo Kubota
	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	454 North 34th Street
		 	Seattle, WA 98103
		
	Telephone:	 	(206) 812-1530
	Facsimile:	 	(206) 545-3885

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 COUNTERPART SIGNATURE PAGE TO 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined
therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights
Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 
 The undersigned has
executed this Counterpart to the Investors’ Rights Agreement as of July 14, 2006. 
  

			
	INVESTOR:
	
	TOKYO WELD CO., LTD.
		
	By:	 	 /s/ Yoshiro Kubota

		 	Name: Yoshiro Kubota
		 	Title: President and Representative Director
		
	Address:	 	28-1, Kitamagome 2-chome
		 	Ohta-ku, Tokyo 143-0021
		 	JAPAN
		
	Telephone:	 	  

	Facsimile:	 	  

  

			
	Accepted and Agreed:
	
	ACUCELA INC.
		
	By:	 	/s/ Ryo Kubota
	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	21720 23rd Drive SE, Suite 120
		 	Bothell, WA 98021
		
	Telephone:	 	(425) 524-3260
	Facsimile:	 	(425) 527-3156

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 COUNTERPART SIGNATURE PAGE TO 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined
therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights
Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 
 The undersigned has
executed this Counterpart to the Investors’ Rights Agreement as of September 6, 2006. 
  

			
	INVESTOR:
	
	 HSBC INSTITUTIONAL TRUST SERVICES

(ASIA) LIMITED
 INSTITUTIONAL FUND SERVICES TRUST
 ACCOUNT
006-124937-471
 YAMATODAMASHII FUND

		
	By:	 	/s/ Naoko Takemura
		 	Name: Naoko Takemura
		 	Title: President, Moon Light Capital Limited
		
		 	 on behalf of HSBC Institutional Trust Services
 (Asia) Limited
 Institutional Fund Services Trust Account 006-

124937-471 Yamatodamashii Fund

		
	Address:	 	39/F, Dorset House, Taikoo Place
		 	979 King’s Road
		 	HONG KONG
	Telephone:	 	
	Facsimile:	 	

  

			
	Accepted and Agreed:
	
	ACUCELA INC.
		
	By:	 	/s/ Ryo Kubota
	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	21720 23rd Drive SE, Suite 120
		 	Bothell, WA 98021
		
	Telephone:	 	(425) 527-3260
	Facsimile:	 	(425) 527-3156

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 COUNTERPART SIGNATURE PAGE TO 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined
therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights
Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 
 The undersigned has
executed this Counterpart to the Investors’ Rights Agreement as of September 14, 2006. 
  

			
	INVESTOR:
	
	KYT INC.
		
	By:	 	/s/ Osami Takahashi
		 	Name: Osami Takahashi
		 	Title: President
		
	Address:	 	2-3-3 Kyobashi
		 	Chuo-ku
		 	Tokyo 104-0031
		 	JAPAN
	Telephone:	 	
	Facsimile:	 	

  

			
	Accepted and Agreed:
	
	ACUCELA INC.
		
	By:	 	/s/ Ryo Kubota
	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	21720 23rd Drive SE, Suite 120
		 	Bothell, WA 98021
		
	Telephone:	 	(425) 527-3260
	Facsimile:	 	(425) 527-3156

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 COUNTERPART SIGNATURE PAGE TO 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined
therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights
Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 
 The undersigned has
executed this Counterpart to the Investors’ Rights Agreement as of September 14, 2006. 
  

			
	INVESTOR:
	
	 JAPAN NEW TECHNOLOGY FUND 2
 PARTNERSHIP

		
	By:	 	JNT Ventures Inc., its General Partner
		
	By:	 	/s/ Yoshiyuki Hongoh
		 	Name: Yoshiyuki Hongoh
		 	Title: President
		
	Address:	 	Cosmo Court Kosugi 1-B
		 	3-3-28 Shimokodanaka
		 	Nakahara-ku
		 	Kawasaki City, Kanagawa 211-0041
		 	JAPAN
	Telephone:	 	
	Facsimile:	 	

  

			
	 Accepted and Agreed:

	
	ACUCELA INC.
		
	By:	 	/s/ Ryo Kubota
	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	21720 23rd Drive SE, Suite 120
		 	Bothell, WA 98021
		
	Telephone:	 	(425) 527-3260
	Facsimile:	 	(425) 527-3156

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 COUNTERPART SIGNATURE PAGE TO 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined
therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights
Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 
 The undersigned has
executed this Counterpart to the Investors’ Rights Agreement as of November 28, 2006. 
  

					
	INVESTOR:
	
	SBI BIO LIFE SCIENCE INVESTMENT LPS.
		
	By:	 	SOFTBANK INVESTMENT CORPORATION,
		 	its General Partner
		
	By:	 	 /s/ Yoshitaka Kitao

		 	Name:	 	Yoshitaka Kitao
		 	Title:	 	Representative Director and CEO
		
	Address:	 	Izumi Garden Tower 19F
		 	1-6-1, Roppongi, Minato-ku
		 	Tokyo 106-6019
		 	JAPAN
	Telephone:	 	
	Facsimile:	 	

  

			
	Accepted and Agreed:
	
	ACUCELA INC.
		
	By: 	 	 /s/ Ryo Kubota

	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	21720 23rd Drive SE, Suite 120
		 	Bothell, WA 98021
		
	Telephone:	 	(425) 527-3260
	Facsimile:	 	(425) 527-3156

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 COUNTERPART SIGNATURE PAGE TO 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined
therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights
Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 
 The undersigned has
executed this Counterpart to the Investors’ Rights Agreement as of November 28, 2006. 
  

					
	INVESTOR:
	
	SBI BB MEDIA INVESTMENT LIMITED PARTNERSHIP
		
	By:	 	SOFTBANK INVESTMENT CORPORATION,
		 	its General Partner
		
	By:	 	 /s/ Yoshitaka Kitao

		 	Name:	 	Yoshitaka Kitao
		 	Title:	 	Representative Director and CEO
		
	Address:	 	Izumi Garden Tower 19F
		 	1-6-1, Roppongi, Minato-ku
		 	Tokyo 106-6019
		 	JAPAN
	Telephone:	 	
	Facsimile:	 	

  

			
	Accepted and Agreed:
	
	ACUCELA INC.
		
	By: 	 	 /s/ Ryo Kubota

	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	21720 23rd Drive SE, Suite 120
		 	Bothell, WA 98021
		
	Telephone:	 	(425) 527-3260
	Facsimile:	 	(425) 527-3156

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 COUNTERPART SIGNATURE PAGE TO 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined
therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights
Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 
 The undersigned has
executed this Counterpart to the Investors’ Rights Agreement as of November 28, 2006. 
  

			
	 INVESTOR:

	
	SBI BROADBAND FUND No1 LIMITED PARTNERSHIP
		
	 By:
	 	 SOFTBANK INVESTMENT CORPORATION,

		 	 its General Partner

		
	 By:
	 	/s/ Yoshitaka Kitao
		 	 Name:   Yoshitaka Kitao

		 	Title:     Representative Director and CEO
		
	 Address:
	 	 Izumi Garden Tower 19F

		 	 1-6-1, Roppongi, Minato-ku

		 	 Tokyo 106-6019

		 	 JAPAN

	 Telephone:
	 	
	 Facsimile:
	 	

  

			
	Accepted and Agreed:
	
	ACUCELA INC.
		
	By:	 	/s/ Ryo Kubota
	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	21720 23rd Drive SE, Suite 120
		 	Bothell, WA 98021
		
	Telephone:	 	(425) 527-3260
	Facsimile:	 	(425) 527-3156

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 COUNTERPART SIGNATURE PAGE TO 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined
therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights
Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 
 The undersigned has
executed this Counterpart to the Investors’ Rights Agreement as of November 28, 2006. 
  

			
	INVESTOR:
	
	SBI BROADBAND CAPITAL K.K.
		
	By:	 	/s/ Takashi Nakagawa
		 	Name: Takashi Nakagawa
		 	Title: Representative Director
		
	Address:	 	Izumi Garden Tower 19F
		 	1-6-1, Roppongi, Minato-ku
		 	Tokyo 106-6019
		 	JAPAN
	Telephone:	 	
	Facsimile:	 	

  

			
	 Accepted and Agreed:

	
	ACUCELA INC.
		
	By:	 	/s/ Ryo Kubota
	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	21720 23rd Drive SE, Suite 120
		 	Bothell, WA 98021
		
	Telephone:	 	(425) 527-3260
	Facsimile:	 	(425) 527-3156

  
 Signature Page
to Amended and Restated Investor’s Rights Agreement 

 COUNTERPART SIGNATURE PAGE TO 

AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined
therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights
Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 
 The undersigned has
executed this Counterpart to the Investors’ Rights Agreement as of March 28, 2007. 
  

			
	 INVESTOR:

	
	 OTSUKA PHARMACEUTICAL FACTORY, INC.

		
	By:	 	 /s/ Ichiro Otsuka

	Name:	 	Ichiro Otsuka
	Title:	 	President
		
	Address:	 	115 Kuguhara
		 	 Tateiwa, Muya-cho

		 	 Naruto, Tokushima 772-8601

		 	 JAPAN

	Telephone:	 	
	Facsimile:	 	

  

			
	 Accepted and Agreed:

 
 ACUCELA INC.

		
	 By:
	 	/s/ Ryo Kubota
	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	 Address:
	 	21720 23rd Drive SE, Suite 120 Bothell, WA 98021
		
	 Telephone:
	 	(425) 527-3260
	 Facsimile:
	 	(425) 527-3156

  
 Counterpart
Signature Page to Amended and Restated Investors’ Rights Agreement 

 SCHEDULE A 
 SERIES A INVESTORS 
  

					
	 Investor Name and Address
	  	Number of Shares of
Series B Preferred
Stock	 
	 Olympus Corporation

2-3 Kuboyama-cho
 Hachioji-shi
 Tokyo 192-8512

JAPAN
 Tel: 
 Fax: 

Email:
                                    
	  	 	1,333,333	  
	 Shin-Etsu Chemical Co., Ltd.

6-1 Ohtemachi 2-chome

Chiyoda-ku
 Tokyo, 100-0004
 JAPAN

Tel: 
 Fax: 
 Email: 
	  	 	666,667	  
	 Berevno Corporation

4-2, Ohtemachi 1-Chome

Chiyoda-ku
 Tokyo, 100-8088
 JAPAN

Tel:
                                    

Fax:
                                    

Email:
                                    
	  	 	67,667	  
	 Trans-Science No. 1 Investment Limited Partnership

1-1-1, Uchisaiwai-cho, Chiyoda-ku

Tokyo 100-0011
 JAPAN
 Tel:
                                    

Fax:
                                    

Email:
                                    
	  	 	666,667	  
		  	  
	  
	 
	 Total
	  	 	2,734,334	  
		  	  
	  
	 

 SCHEDULE A (cont.) 

SERIES B INVESTORS 
  

  
  

					
	 Investor Name and Address
	  	Number of Shares of
Series B Preferred
Stock	 
	 First Closing: August 31, 2004
	  			
	 Trans-Science No. 1 Investment Limited Partnership

1-1-1, Uchisaiwai-cho, Chiyoda-ku

Tokyo 100-0011
 JAPAN
 Attn: Kiyoshi Inoue
	  	 	457,784	  
	 Trans-Science No. 2A Investment Limited Partnership

1-1-1, Uchisaiwai-cho, Chiyoda-ku

Tokyo 100-0011
 JAPAN
 Attn: Kiyoshi Inoue
	  	 	208,883	  
	 Mizuho Capital No. 1 Limited Partnership

4-3, Nihombashi-kabutocho

Chuo-ku, Tokyo 103-0026

JAPAN
 Attn: Osamu Kita
	  	 	666,667	  
	 SMBC Capital No. 6 Venture Capital Investment Limited Partnership

1-2-1 Kyobashi Chuo-ku

Tokyo, 104-0031
 JAPAN
 Attn: Michihiro Matsuda
	  	 	666,666	  
	 DNP Holding USA Corporation

335 Madison Avenue, Third Floor

New York, NY 10017
	  	 	666,667	  
		  	  
	  
	 
	 Subtotal First Closing
	  	 	2,666,667	  
		  	  
	  
	 

 SCHEDULE A (cont.) 

SERIES B INVESTORS 
  

  

					
	 Investor Name and Address
	  	Number of Shares of
Series B Preferred
Stock	 
	 Second Closing: September 30, 2004
	  			
	 Shin Nippon Biomedical Laboratories, Ltd.

Toho Twin Tower Building, 6th FL.

1-5-2 Yurakucho, Chiyoda-ku

Tokyo, 100-0006
 JAPAN
 Attn: Ryoichi Nagata, M.D., Ph.D.,
FFPM
	  	 	666,666	  
	 Japan Asia Investment Co., Ltd.

2-13-5, Nagata-cho, Chiyoda-ku

Tokyo, 100-8972
 JAPAN
 Attn: Toyoji Tatsuoka
	  	 	600,000	  
	 Marubeni Venture Capital Fund 1 L.P.

1-4-1, Uchikanda, Chiyoda-ku

Tokyo, 101-0047
 JAPAN
	  	 	133,333	  
	 UOB JAIC Venture Bio Investments Ltd.

50 Raffles Place #24-03

Singapore Land Tower 5

SINGAPORE 048623
 Attn: Toyoji Tatsuoka
	  	 	600,000	  
	 Aqua RIMCO Biotechnology No. 2

Investment Partnership

Kawate Building 1-5-8 Nishi Shimbashi

Minato-ku, Tokyo 105-0003 JAPAN

Attn: Yoshihiko Takamiya
	  	 	333,333	  
	 Marubeni Corporation

4-2, Ohtemachi 1-Chome

Chiyoda-ku, Tokyo 100-8088

JAPAN
	  	 	533,334	  

  

					
	 Investor Name and Address
	  	Number of Shares of
Series B Preferred
Stock	 
	 ORIX Fund No. 7

2-4-1, Hamamatsu-cho

Minato-ku
 Tokyo 105-6132
 JAPAN

Attn: Akira Hirose
	  	 	222,222	  
	 ORIX Fund No. 8

2-4-1, Hamamatsu-cho

Minato-ku
 Tokyo 105-6132
 JAPAN

Attn: Akira Hirose
	  	 	444,444	  
	 Resona Capital Co., Ltd.

3-1, Kyobashi 1-chome

Chuo-Ku, Tokyo
 JAPAN
 Attn: Hirohide Takahashi
	  	 	666,667	  
	 MITSUI SUMITOMO INSURANCE C

Global Technology Venture Capital Investment Limited Partnership

Yaesu Nagoya Bldg. 3F, 2-10,

Yaesu 2-Chome
 Chuo-ku, Tokyo, 104-0028
 JAPAN
	  	 	666,666	  
	 BIOVISION Life Science Fund 1

1-6-1, Roppongi
 Minato-ku, Tokyo 106-6020 JAPAN
 Attn: Yoshitaka
Kitao
	  	 	4,000,000	  
	 SOFTBANK INTERNET FUND

1-6-1, Roppongi
 Minato-ku, Tokyo 106-6020
 JAPAN

Attn: Yoshitaka Kitao
	  	 	666,667	  

  

					
	 Investor Name and Address
	  	Number of Shares of
Series B Preferred
Stock	 
	 Ikegin Capital Co., Ltd.

4-1-9, Minami Semba, Chuo-ku

Osaka, 542-0081
 JAPAN
 Attn: Toshiaki Jimbow
	  	 	133,333	  
	 NVCC Kansai NO. 3 Venture Capital Investment Limited Partnership

7-1-16 Akasaka
 Minato-ku, Tokyo 107-0052
 JAPAN
	  	 	600,000	  
	 NVCC NO. 4 Venture Capital Investment Limited Partnership

7-1-16 Akasaka
 Minato-ku, Tokyo 107-0052
 JAPAN
	  	 	300,000	  
	 MITSUBISHI UFJ CAPITAL Co., LTD.

14-1 2-Chome, Kyobashi

Chuo-ku, Tokyo 104-0031

JAPAN
	  	 	666,666	  
		  	  
	  
	 
	 Subtotal Second Closing
	  	 	11,233,331	  
		  	  
	  
	 

 SCHEDULE A (cont.) 

SERIES B INVESTORS 
  

  

					
	 Investor Name and Address
	  	Number of Shares of
Series B Preferred
Stock	 
	 Third Closing: December 24, 2004
	  			
	 BIOVISION Life Science Fund 1

1-6-1, Roppongi
 Minato-ku, Tokyo 106-6020
 JAPAN

Attn: Yoshitaka Kitao
	  	 	1,333,333	  
	 SOFTBANK INTERNET FUND

1-6-1, Roppongi
 Minato-ku, Tokyo 106-6020
 JAPAN

Attn: Yoshitaka Kitao
	  	 	2,666,667	  
	 Subtotal Third Closing
	  	 	4,000,000	  
		  	  
	  
	 
	 Total All Three Closings
	  	 	17,899,998	  
		  	  
	  
	 

 SCHEDULE A (cont.) 

SERIES C INVESTORS 
  

  

					
	 Investor Name and Address
	  	Number of Shares of
Series C Preferred
Stock	 
	 First Closing: May 31, 2006
	  			
	 JPE Medibic I L.P.

JPE Capital Management

PO Box 3093 GT Ugland House

South Church Street

Georgetown, Grand Cayman

CAYMAN ISLANDS
	  	 	200,000	  
	 Hiroshi Konishi
	  	 	181,819	  
	 MediBIC Alliance

Daido Kasumigaseki Bldg. 8F

1-4-2 Kasumigaseki

Chiyoda-ku
 Tokyo, 100-0013
 JAPAN
	  	 	100,000	  
	 Mr. Hikaru Shimura
	  	 	454,546	  
	 Shin Nihon Jitsugyo Co., Ltd.

6-6-5, Ginza, Chuo-ku

Tokyo, 104-0061
 JAPAN
 Attn: Rika Sugino
	  	 	727,273	  
		  	  
	  
	 
	 Subtotal First Closing
	  	 	1,663,638	  
		  	  
	  
	 

 SCHEDULE A (cont.) 

SERIES C INVESTORS 
  

  

					
	 Investor Name and Address
	  	Number of Shares of
Series C Preferred
Stock	 
	 Second Closing: June 16, 2006
	  			
	 Fuji Photo Film Co., Ltd.

26-30 Nishiazabu 2-Chome

Minato-ku, Tokyo, 106-8620

JAPAN
 Attn: Yuzo Toda
	  	 	454,546	  
		  	  
	  
	 
	 Subtotal Second Closing
	  	 	454,546	  
		  	  
	  
	 
	 Total First and Second Closings
	  	 	2,118,184	  
		  	  
	  
	 

 SCHEDULE A (cont.) 

SERIES C INVESTORS 
  

  

					
	 Investor Name and Address
	  	Number of Shares 
of
Series C Preferred
Stock	 
	 Third Closing: June 30, 2006
	  			
	 JIKEI NO.1 LIMITED PARTNERSHIP FUND

11-30, 1-Chome, Shimanouchi,

Chuo-Ku, Osaka 542-0082

JAPAN
 Attn: Hiroki Hideshima
	  	 	550,000	  
	 Brain Staff Consultants Inc.

10-15, 1-Chome, Shimanouchi

Chuo-Ku, Osaka-city 542-0082

JAPAN
 Attn: Toyonori Tanaka
	  	 	800,000	  
		  	  
	  
	 
	 Subtotal Third Closing
	  	 	1,350,000	  
		  	  
	  
	 
	 Total First Through Third Closings
	  	 	3,468,184	  
		  	  
	  
	 

 SCHEDULE A (cont.) 

SERIES C INVESTORS 
  

  

					
	 Investor Name and Address
	  	Number of Shares 
of
Series C Preferred
Stock	 
	 Fourth Closing: July 14, 2006
	  			
	 Tokyo Weld Co., Ltd.

28-1, Kitamagome
 Ohta-ku, Tokyo 143-0021
 JAPAN

Attn: Yoshiro Kubota
	  	 	454,546	  
		  	  
	  
	 
	 Subtotal Fourth Closing
	  	 	454,546	  
		  	  
	  
	 
	 Total First Through Fourth Closings
	  	 	3,922,730	  
		  	  
	  
	 

 SCHEDULE A (cont.) 

SERIES C INVESTORS 
  

  

					
	 Investor Name and Address
	  	Number of Shares 
of
Series C Preferred
Stock	 
	 Fifth Closing: September 6, 2006
	  			
	 HSBC Institutional Trust Services

(Asia) Limited
 Institutional Fund Services
 Trust Account
006-124937-471
 Yamatodamashii Fund

39/F, Dorset House, Taikoo Place

979 King’s Road

HONG KONG
 Attn: Ms. Naoko Takemura
 President, Moon Light
Capital Limited
	  	 	909,091	  
		  	  
	  
	 
	 Subtotal Fifth Closing
	  	 	909,091	  
		  	  
	  
	 
	 Total First Through Fifth Closings
	  	 	4,831,821	  
		  	  
	  
	 

 SCHEDULE A (cont.) 

SERIES C INVESTORS 
  

  
  

					
	 Investor Name and Address
	  	Number of Shares 
of
Series C Preferred
Stock	 
	 Sixth Closing: September 14, 2006
	  			
	 KYT Inc.

2-3-3 Kyobashi
 Chuo-ku
 Tokyo 104-0031

JAPAN
 Attn: Osami Takahashi
	  	 	272,728	  
	 Japan New Technology Fund 2

Partnership
 c/o JNT Ventures Inc.
 Cosmo Court Kosugi 1-B

3-3-28 Shimokodanaka

Nakahara-ku
 Kawasaki City, Kanagawa 211-0041
 JAPAN

Attn: Yoshiyuki Hongoh
	  	 	818,182	  
		  	  
	  
	 
	 Subtotal Sixth Closing
	  	 	1,090,910	  
		  	  
	  
	 
	 Total First Through Sixth Closings
	  	 	5,922,731	  
		  	  
	  
	 

 SCHEDULE A (cont.) 

SERIES C INVESTORS 
  

  

					
	 Investor Name and Address
	  	Number of Shares of
Series C Preferred
Stock	 
	 Seventh Closing: November 28, 2006
	  			
	 SBI BROADBAND FUND No1 LIMITED PARTNERSHIP

c/o Softbank Investment Corporation

Izumi Garden Tower 19F

1-6-1, Roppongi, Minato-ku

Tokyo 106-6019
 JAPAN
 Attn: Akihiro Yamamoto
	  	 	224,855	  
	 SBI BROADBAND CAPITAL K.K.

c/o Softbank Investment Corporation

Izumi Garden Tower 19F

1-6-1, Roppongi, Minato-ku

Tokyo 106-6019
 JAPAN
 Attn: Akihiro Yamamoto
	  	 	137,814	  
	 SBI BB MEDIA INVESTMENT LIMITED PARTNERSHIP

c/o Softbank Investment Corporation

Izumi Garden Tower 19F

1-6-1, Roppongi, Minato-ku

Tokyo 106-6019
 JAPAN
 Attn: Akihiro Yamamoto
	  	 	362,669	  
	 SBI BIO LIFE SCIENCE INVESTMENT LPS.

c/o Softbank Investment Corporation

Izumi Garden Tower 19F

1-6-1, Roppongi, Minato-ku

Tokyo 106-6019
 JAPAN
 Attn: Akihiro Yamamoto
	  	 	613,748	  
		  	  
	  
	 
	 Subtotal Seventh Closing
	  	 	1,339,086	  
		  	  
	  
	 
	 Total First Through Seventh Closings
	  	 	7,261,817	  
		  	  
	  
	 

 SCHEDULE A (cont.) 

SERIES C INVESTORS 
  

  
  

					
	 Investor Name and Address
	  	Number of Shares of
Series C Preferred
Stock	 
	 Eighth Closing: March 28, 2007
	  			
	 Otsuka Pharmaceutical Factory, Inc.

115 Kuguhara
 Tateiwa, Muya-cho
 Naruto, Tokushima 772-8601

JAPAN
 Attn: Mr. Ichiro Otsuka
 President
	  	 	4,545,455	  
		  	  
	  
	 
	 Subtotal Eighth Closing
	  	 	4,545,455	  
		  	  
	  
	 
	 Total First Through Eighth Closings
	  	 	11,807,272	  
		  	  
	  
	 

 EXHIBIT 1 
 COUNTERPART SIGNATURE PAGE TO 
 AMENDED AND RESTATED INVESTORS’
RIGHTS AGREEMENT 
 Pursuant to Section 1.2(c) of the Series C Preferred Stock Purchase Agreement, dated as of
May 31, 2006, by and among Acucela Inc. (the “Company”) and the Investors (as defined therein), the undersigned hereby agrees to be a party to, and be bound by the terms and subject to the conditions of, the Amended and
Restated Investors’ Rights Agreement, dated as of May 31, 2006 (the “Investors’ Rights Agreement”), by and among the Company and the Investors, as an “Investor” thereunder. 

The undersigned has executed this Counterpart to the Investors’ Rights Agreement as of
            , 200    . 
  

					
	INVESTOR:
	
	  
	  
 (Print name of
individual or corporate Investor)

		
	By:	 	  

		 	Name:	 	  

		 	Title:	 	  

		
	Address:	 	  

		 	  

		 	  

	Telephone:	 	  

	Facsimile:	 	  

  

			
	Accepted and Agreed:
	
	ACUCELA INC.
		
	By: 	 	 /s/ Ryo Kubota

	Name:	 	Ryo Kubota, M.D., Ph.D.
	Title:	 	President and Chief Executive Officer
		
	Address:	 	21720 23rd Drive SE, Suite 120
		 	Bothell, WA 98021
		
	Telephone:	 	(425) 527-3260
	Facsimile:	 	(425) 527-3156

  
 Counterpart
Signature Page to Amended and Restated Investors’ Rights Agreement2002 Stock Option/Restricted Stock Plan

 Exhibit 10.02 
 ACUCELA INC. 
 2002 STOCK OPTION AND RESTRICTED STOCK PLAN

 EFFECTIVE AS OF JUNE 28, 2002 
 AMENDED APRIL 11, 2011 

 TABLE OF CONTENTS 

 

					
	 	  	Page	 
	 SECTION 1. INTRODUCTION
	  	 	1	  
		
	 SECTION 2. DEFINITIONS
	  	 	1	  
		
	 (a) “Affiliate”
	  	 	1	  
	 (b) “Award”
	  	 	1	  
	 (c) “Board”
	  	 	1	  
	 (d) “Change In Control”
	  	 	1	  
	 (e) “Code”
	  	 	2	  
	 (f) “Committee”
	  	 	2	  
	 (g) “Common Stock”
	  	 	2	  
	 (h) “Company”
	  	 	2	  
	 (i) “Consultant”
	  	 	2	  
	 (j) “Director”
	  	 	2	  
	 (k) “Disability”
	  	 	2	  
	 (l) “Employee”
	  	 	2	  
	 (m) “Exchange Act”
	  	 	2	  
	 (n) “Exercise Price”
	  	 	2	  
	 (o) “Fair Market Value”
	  	 	2	  
	 (p) “Grant”
	  	 	3	  
	 (q) “Incentive Stock Option” or “ISO”
	  	 	3	  
	 (r) “Key Employee”
	  	 	3	  
	 (s) “Non-Employee Director”
	  	 	3	  
	 (t) “Nonstatutory Stock Option” or “NSO”
	  	 	3	  
	 (u) “Option”
	  	 	3	  
	 (v) “Optionee”
	  	 	3	  
	 (w) “Parent”
	  	 	3	  
	 (x) “Participant”
	  	 	3	  
	 (y) “Plan”
	  	 	3	  
	 (z) “Restricted Stock”
	  	 	3	  
	 (aa) “Restricted Stock Agreement”
	  	 	3	  
	 (bb) “Securities Act”
	  	 	4	  
	 (cc) “Service”
	  	 	4	  
	 (dd) “Share”
	  	 	4	  
	 (ee) “Stock Option Agreement”
	  	 	4	  
	 (ff) “Subsidiary”
	  	 	4	  
	 (gg) “10-Percent Shareholder”
	  	 	4	  
		
	 SECTION 3. ADMINISTRATION
	  	 	4	  
		
	 (a) Committee Composition
	  	 	4	  
	 (b) Authority of the Committee
	  	 	5	  
	 (c) Indemnification
	  	 	5	  
	 (d) Financial Reports
	  	 	5	  
		
	 SECTION 4. ELIGIBILITY
	  	 	5	  
		
	 (a) General Rules
	  	 	5	  
	 (b) Incentive Stock Options
	  	 	5	  

  
 -i-

					
		
	 SECTION 5. SHARES SUBJECT TO PLAN
	  	 	6	  
		
	 (a) Basic Limitation
	  	 	6	  
	 (b) Additional Shares
	  	 	6	  
	 (c) Dividend Equivalents
	  	 	6	  
		
	 SECTION 6. TERMS AND CONDITIONS OF OPTIONS
	  	 	6	  
		
	 (a) Stock Option Agreement
	  	 	6	  
	 (b) Number of Shares
	  	 	6	  
	 (c) Exercise Price
	  	 	6	  
	 (d) Exercisability and Term
	  	 	6	  
	 (e) Modifications or Assumption of Options
	  	 	7	  
	 (f) Transferability of Options
	  	 	7	  
	 (g) Restrictions on Transfer
	  	 	7	  
		
	 SECTION 7. PAYMENT FOR OPTION SHARES
	  	 	8	  
		
	 (a) General Rule
	  	 	8	  
	 (b) Surrender of Stock
	  	 	8	  
	 (c) Promissory Note
	  	 	8	  
	 (d) Other Forms of Payment
	  	 	8	  
		
	 SECTION 8. TERMS AND CONDITIONS FOR AWARDS OF RESTRICTED STOCK
	  	 	8	  
		
	 (a) Time, Amount and Form of Awards
	  	 	8	  
	 (b) Restricted Stock Agreement
	  	 	8	  
	 (c) Payment for Restricted Stocks
	  	 	8	  
	 (d) Vesting Conditions
	  	 	8	  
	 (e) Assignment or Transfer of Restricted Stocks
	  	 	9	  
	 (f) Trusts
	  	 	9	  
	 (g) Voting and Dividend Rights
	  	 	9	  
		
	 SECTION 9. PROTECTION AGAINST DILUTION
	  	 	9	  
		
	 (a) Adjustments
	  	 	9	  
	 (b) Participation Rights
	  	 	9	  
		
	 SECTION 10. EFFECT OF A CHANGE IN CONTROL
	  	 	10	  
		
	 (a) Merger or Reorganization
	  	 	10	  
	 (b) Accleration
	  	 	10	  
		
	 SECTION 11. LIMITATIONS ON RIGHTS
	  	 	10	  
		
	 (a) Retention Rights
	  	 	10	  
	 (b) Shareholders’Rights
	  	 	10	  
	 (c) Regulatory Requirements
	  	 	10	  

  
 -ii-

					
		
	 SECTION 12. WITHHOLDING TAXES
	  	 	11	  
		
	 (a) General
	  	 	11	  
	 (b) Share Withholding
	  	 	11	  
		
	 SECTION 13. DURATION AND AMENDMENTS
	  	 	11	  
		
	 (a) Term of the Plan
	  	 	11	  
	 (b) Right to Amend or Terminate the Plan
	  	 	11	  

  
 -iii-

 ACUCELA INC. 
 2002 STOCK OPTION AND RESTRICTED STOCK PLAN 
 EFFECTIVE AS OF JUNE 28,
2002 
 AMENDED APRIL 11, 2011 
 SECTION 1. INTRODUCTION. 
 The Company’s Board of Directors adopted the
Acucela Inc. 2002 Stock Option and Restricted Stock Plan on June 28, 2002, subject to approval by the Company’s shareholders. 
 The purpose of the Plan is to promote the long-term success of the Company and the creation of shareholder value by offering Key Employees an opportunity to acquire a proprietary interest in the success
of the Company, or to increase such interest, and to encourage such selected persons to continue to provide services to the Company and to attract new individuals with outstanding qualifications. 

The Plan seeks to achieve this purpose by providing for Options (which may constitute Incentive Stock Options or Nonstatutory Stock
Options) and Awards of Restricted Stock. 
 The Plan shall be governed by, and construed in accordance with, the laws of the
state of Washington (except its choice-of-law provisions). Capitalized terms shall have the meaning provided in Section 2 unless otherwise provided in this Plan or Stock Option Agreement or Restricted Stock Agreement. 

SECTION 2. DEFINITIONS. 

(a) “Affiliate” means any entity other than a Subsidiary, if the Company and/or one or more Subsidiaries own not less
than 50% of such entity. For purposes of determining an individual’s “Service,” this definition shall include any entity other than a Subsidiary, if the Company, a Parent and/or one or more Subsidiaries own not less than 50% of such
entity. 
 (b) “Award” means any award of an Option or Restricted Stock under the Plan. 

(c) “Board” means the Board of Directors of the Company, as constituted from time to time. 

(d) “Change in Control” except as may otherwise be provided in the Stock Option Agreement, means any merger or
consolidation of the Company into or with another corporation or other entity, or the sale, transfer or other disposition of all or substantially all of the assets or capital stock of the Company, or any reorganization, recapitalization or like
transaction or series of related transactions having substantially equivalent effect and purpose, at the conclusion of which such merger, consolidation, sale, transfer, disposition, reorganization, recapitalization or like transaction the holders of
the capital stock of the 

  
 1 

 
Company entitled to vote for the election of directors or similar governing body immediately prior to such transaction or series of related transactions own less than a majority of the capital
stock entitled to vote for the election of directors or similar governing body of the acquiring entity or entity surviving or resulting from such transaction or series of related transactions immediately thereafter; provided that a merger
effected exclusively for the purpose of changing the domicile of the Company shall not be deemed to constitute a “Change in Control”. 
 (e) “Code” means the Internal Revenue Code of 1986, as amended. 

(f) “Committee” means a committee consisting of two or more members of the Board that is appointed by the Board (as
described in Section 3) to administer the Plan. 
 (g) “Common Stock” means the Company’s common
stock. 
 (h) “Company” means Acucela Inc., a Washington corporation. 

(i) “Consultant” means an individual who performs bona fide services to the Company, a Parent, a Subsidiary or an
Affiliate other than as an Employee or Director or Non-Employee Director. 
 (j) “Director” means a member of
the Board who is also an Employee. 
 (k) “Disability” means that the Key Employee is unable to engage in any
substantial gainful activity by reason of any medically determinable physical or mental impairment which can be expected to result in death or which has lasted or can be expected to last for a continuous period of not less than 12 months.

 (l) “Employee” means any individual who is a common-law employee of the Company, a Parent, a Subsidiary or an
Affiliate. 
 (m) “Exchange Act” means the Securities Exchange Act of 1934, as amended. 

(n) “Exercise Price” means the amount for which a Share may be purchased upon exercise of such Option, as specified in
the applicable Stock Option Agreement. 
 (o) “Fair Market Value” means the market price of Shares, determined
by the Committee as follows: 
 (i) If the Shares were traded over-the-counter on the date in question but were
not classified as a national market issue, then the Fair Market Value shall be equal to the mean between the last reported representative bid and asked prices quoted by the NASDAQ system for such date; 

(ii) If the Shares were traded over-the-counter on the date in question and were classified as a national market issue,
then the Fair Market Value shall be equal to the last-transaction price quoted by the NASDAQ system for such date; 

  
 2 

 (iii) If the Shares were traded on a stock exchange on the date in question,
then the Fair Market Value shall be equal to the closing price reported by the applicable composite transactions report for such date; and 
 (iv) If none of the foregoing provisions is applicable, then the Fair Market Value shall be determined by the Committee in good faith on such basis as it deems appropriate. 

Whenever possible, the determination of Fair Market Value by the Committee shall be based on the prices reported in the Wall Street
Journal. Such determination shall be conclusive and binding on all persons. 
 (p) “Grant” means any grant
of an Option under the Plan. 
 (q) “Incentive Stock Option” or “ISO” means an incentive stock
option described in Code section 422(b). 
 (r) “Key Employee” means an Employee, Director, Non-Employee
Director or Consultant who has been selected by the Committee to receive an Award under the Plan. 
 (s) “Non-Employee
Director” means a member of the Board who is not an Employee. 
 (t) “Nonstatutory Stock Option” or
“NSO” means a stock option that is not an ISO. 
 (u) “Option” means an ISO or NSO granted
under the Plan entitling the Optionee to purchase Shares. 
 (v) “Optionee” means an individual, estate or other
entity that holds an Option. 
 (w) “Parent” means any corporation (other than the Company) in an unbroken chain
of corporations ending with the Company, if each of the corporations other than the Company owns stock possessing fifty percent (50%) or more of the total combined voting power of all classes of stock in one of the other corporations in such
chain. A corporation that attains the status of a Parent on a date after the adoption of the Plan shall be considered a Parent commencing as of such date. 
 (x) “Participant” means an individual or estate or other entity that holds an Award. 
 (y) “Plan” means this Acucela Inc. 2002 Stock Option and Restricted Stock Plan as it may be amended from time to time. 

(z) “Restricted Stock” means a Share awarded under the Plan. 

(aa) “Restricted Stock Agreement” means the agreement described in Section 8 evidencing each Award of Restricted
Stock. 

  
 3 

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

 (cc) “Service” means service as an Employee, Director, Non-Employee Director or Consultant. 

(dd) “Share” means one share of Common Stock. 
 (ee) “Stock Option Agreement” means the agreement described in Section 6 evidencing each Grant of an Option. 
 (ff) “Subsidiary” means any corporation (other than the Company) in an unbroken chain of corporations beginning with the Company, if each of the corporations other than the last
corporation in the unbroken chain owns stock possessing fifty percent (50%) or more of the total combined voting power of all classes of stock in one of the other corporations in such chain. A corporation that attains the status of a Subsidiary
on a date after the adoption of the Plan shall be considered a Subsidiary commencing as of such date. 
 (gg) “10-Percent
Shareholder” means an individual who owns more than ten percent (10%) of the total combined voting power of all classes of outstanding stock of the Company, its Parent or any of its subsidiaries. In determining stock ownership, the
attribution rules of section 424(d) of the Code shall be applied. 
 SECTION 3. ADMINISTRATION. 

(a) Committee Composition. A Committee appointed by the Board shall administer the Plan. The Board shall designate one of the
members of the Committee as chairperson. If no Committee has been approved, the entire Board shall constitute the Committee. Members of the Committee shall serve for such period of time as the Board may determine and shall be subject to removal by
the Board at any time. The Board may also at any time terminate the functions of the Committee and reassume all powers and authority previously delegated to the Committee. 
 Effective upon the Company’s initial public offering, the Committee shall consist either of (i) those individuals who shall satisfy the requirements of Rule 16b-3 (or its successor) under the
Exchange Act with respect to Options to persons who are officers or directors of the Company under Section 16 of the Exchange Act or (ii) the Board itself. 
 The Board may also appoint one or more separate committees of the Board, each composed of one or more directors of the Company who need not qualify under Rule 16b-3, who may administer the Plan with
respect to Key Employees who are not considered officers or directors of the Company under Section 16 of the Exchange Act, may grant Awards under the Plan to such Key Employees and may determine all terms of such Awards. 

To the extent consistent with applicable law, the Board may authorize a senior executive officer of the Company to grant Awards to
individuals eligible to receive Awards under the Plan, within the limits specifically prescribed by the Board. 

  
 4 

 (b) Authority of the Committee. Subject to the provisions of the Plan, the Committee
shall have full authority and discretion to take any actions it deems necessary or advisable for the administration of the Plan. Such actions shall include: 
  

	 	(i)	selecting Key Employees who are to receive Awards under the Plan; 

  

	 	(ii)	determining the type, number, vesting requirements and other features and conditions of such Awards; 

 

	 	(iii)	interpreting the Plan; and 

  

	 	(iv)	making all other decisions relating to the operation of the Plan. 

 The Committee may adopt such rules or guidelines, as it deems appropriate to implement the Plan. The Committee’s determinations under the Plan shall be final and binding on all persons. 

(c) Indemnification. Each member of the Committee, or of the Board, shall be indemnified and held harmless by the Company against
and from (i) any loss, cost, liability, or expense that may be imposed upon or reasonably incurred by him or her in connection with or resulting from any claim, action, suit, or proceeding to which he or she may be a party or in which he or she
may be involved by reason of any action taken or failure to act under the Plan or any Stock Option Agreement or any Restricted Stock Agreement, and (ii) from any and all amounts paid by him or her in settlement thereof, with the Company’s
approval, or paid by him or her in satisfaction of any judgment in any such claim, action, suit, or proceeding against him or her, provided he or she shall give the Company an opportunity, at its own expense, to handle and defend the same before he
or she undertakes to handle and defend it on his or her own behalf. The foregoing right of indemnification shall not be exclusive of any other rights of indemnification to which such persons may be entitled under the Company’s Articles of
Incorporation or Bylaws, by contract, as a matter of law, or otherwise, or under any power that the Company may have to indemnify them or hold them harmless. 
 (d) Financial Reports. To the extent required by applicable law, the Company shall furnish to Participants the Company’s summary financial information including a balance sheet regarding the
Company’s financial condition and results of operations, unless such Participants have duties with the Company that assure them access to equivalent information. Such financial statements need not be audited. 

SECTION 4. ELIGIBILITY. 

(a) General Rules. Only Employees, Directors, Non-Employee Directors and Consultants shall be eligible for designation as Key
Employees by the Committee. 
 (b) Incentive Stock Options. Only Key Employees who are common-law employees of the
Company, a Parent or a Subsidiary shall be eligible for the grant of ISOs. In addition, a Key Employee who is a 10-Percent Shareholder shall not be eligible for the grant of an ISO unless the requirements set forth in section 422(c)(5) of the Code
are satisfied. 

  
 5 

 SECTION 5. SHARES SUBJECT TO PLAN. 

(a) Basic Limitation. The stock issuable under the Plan shall be authorized but unissued Shares or treasury Shares. The aggregate
number of Shares reserved for Awards under the Plan shall not exceed Two Million Three Hundred Thousand (2,300,000) Shares, subject to adjustment pursuant to Section 9. 

(b) Additional Shares. If Awards are forfeited or terminate for any other reason before being exercised, then the Shares underlying
such Awards shall again become available for Awards under the Plan. 
 (c) Dividend Equivalents. Any dividend equivalents
distributed under the Plan shall not be applied against the number of Shares available for Awards. 
 SECTION 6. TERMS AND CONDITIONS OF
OPTIONS. 
 (a) Stock Option Agreement. Each Grant under the Plan shall be evidenced by a Stock Option Agreement
between the Optionee and the Company. Such Option shall be subject to all applicable terms and conditions of the Plan and may be subject to any other terms and conditions that are not inconsistent with the Plan and that the Committee deems
appropriate for inclusion in a Stock Option Agreement. The provisions of the various Stock Option Agreements entered into under the Plan need not be identical. A Stock Option Agreement may provide that new Options will be granted automatically to
the Optionee when he or she exercises the prior Options. The Stock Option Agreement shall also specify whether the Option is an ISO or an NSO. 
 (b) Number of Shares. Each Stock Option Agreement shall specify the number of Shares that are subject to the Option and shall provide for the adjustment of such number in accordance with
Section 9. 
 (c) Exercise Price. An Option’s Exercise Price shall be established by the Committee and set forth
in a Stock Option Agreement. To the extent required by applicable law the Exercise Price of an ISO shall not be less than 100% of the Fair Market Value (110% for 10-Percent Shareholders) of a Share on the date of Grant. In the case of an NSO, a
Stock Option Agreement may specify an Exercise Price that varies in accordance with a predetermined formula while the NSO is outstanding. To the extent required by applicable law, the Exercise Price for an NSO shall not be less than 85% of the Fair
Market Value (110% for 10-Percent Shareholders) of a Share on the date of Grant. 
 (d) Exercisability and Term. Each
Stock Option Agreement shall specify the date when all or any installment of the Option is to become exercisable. Except as otherwise set forth in the Stock Option Agreement, an Option shall vest as to 25% of the total number of Shares on the first
anniversary of the Vesting Start Date, as defined in the Stock Option Agreement, provided that the Optionee is providing Service as of such anniversary. Thereafter, the Option shall vest monthly at the rate of 2.0833% of the total number of Shares
until the Option is 100% vested and exercisable four years from the Vesting Start Date, as defined in the Stock Option Agreement, provided the Optionee is providing Service as of such date. In no event shall more than 100% of the Shares, as defined
the Stock 

  
 6 

 
Option Agreement, become vested. The Stock Option Agreement shall also specify the term of the Option; provided that the term of an ISO, and to the extent required by applicable law a NSO, shall
in no event exceed ten (10) years from the date of Grant. An ISO that is granted to a 10-Percent Shareholder shall have a maximum term of five (5) years. To the extent required by applicable law, vested Options shall be exercisable for a
minimum period of six (6) months and a maximum period of twelve (12) months following termination of Services due to death or Disability, and vested Options shall be exercisable for a minimum of thirty (30) days and a maximum of three
(3) months following termination of Services not due to death or Disability (other than terminations for cause, as defined in the Company’s personnel policies). Notwithstanding the previous sentence, no Option can be exercised after the
expiration date provided in the applicable Stock Option Agreement. A Stock Option Agreement may provide for accelerated exercisability in the event of the Optionee’s death, Disability or retirement or other events and may provide for expiration
prior to the end of its term in the event of the termination of the Optionee’s service. A Stock Option Agreement may permit an Optionee to exercise an Option before it is vested, subject to the Company’s right of repurchase over any Shares
acquired under the unvested portion of the Option (an “early exercise”), which right of repurchase shall lapse at the same rate the Option would have vested had there been no early exercise. In no event shall the Company be required to
issue fractional Shares upon the exercise of an Option. 
 (e) Modifications or Assumption of Options. Within the
limitations of the Plan, the Committee may modify, extend or assume outstanding options or may accept the cancellation of outstanding options (whether granted by the Company or by another issuer) in return for the grant of new Options for the same
or a different number of Shares and at the same or a different Exercise Price. The foregoing notwithstanding, no modification of an Option shall, without the consent of the Optionee, alter or impair his or her rights or obligations under such
Option. 
 (f) Transferability of Options. Except as otherwise provided in the applicable Stock Option Agreement and then
only to the extent permitted by applicable law, no Option shall be transferable by the Optionee other than by will or by the laws of descent and distribution. Except as otherwise provided in the applicable Stock Option Agreement, an Option may be
exercised during the lifetime of the Optionee only or by the guardian or legal representative of the Optionee. No Option or interest therein may be assigned, pledged or hypothecated by the Optionee during his lifetime, whether by operation of law or
otherwise, or be made subject to execution, attachment or similar process. 
 (g) Restrictions on Transfer. Any Shares
issued upon exercise of an Option shall be subject to such rights of repurchase, rights of first refusal and other transfer restrictions as the Committee may determine. Such restrictions shall apply in addition to any restrictions that may apply to
holders of Shares generally and shall also comply to the extent necessary with applicable law. 

  
 7 

 SECTION 7. PAYMENT FOR OPTION SHARES. 

(a) General Rule. The entire Exercise Price of Shares issued upon exercise of Options shall be payable in cash at the time when
such Shares are purchased, except as follows: 
 (i) In the case of an ISO granted under the Plan, payment shall
be made only pursuant to the express provisions of the applicable Stock Option Agreement. The Stock Option Agreement may specify that payment may be made in any form(s) described in this Section 7. 

(ii) In the case of an NSO granted under the Plan, the Committee may in its discretion at any time accept payment in any
form(s) described in this Section 7. 
 (b) Surrender of Stock. To the extent that this Section 7(b) is
applicable, payment for all or any part of the Exercise Price may be made with Shares which have already been owned by the Optionee for such duration as shall be specified by the Committee. Such Shares shall be valued at their Fair Market Value on
the date when the new Shares are purchased under the Plan. 
 (c) Promissory Note. To the extent that this
Section 7(c) is applicable, payment for all or any part of the Exercise Price may be made with a full-recourse promissory note. 
 (d) Other Forms of Payment. To the extent that this Section 7(d) is applicable, payment may be made in any other form that is consistent with applicable laws, regulations and rules.

 SECTION 8. TERMS AND CONDITIONS FOR AWARDS OF RESTRICTED STOCK. 

(a) Time, Amount and Form of Awards. Awards under the Plan may be granted in the form of Restricted Stock. 

(b) Restricted Stock Agreement. Each Award of Restricted Stock under the Plan shall be evidenced by a Restricted Stock Agreement
between the Participant and the Company. Such Award shall be subject to all applicable terms and conditions of the Plan and may be subject to any other terms and conditions that are not inconsistent with the Plan and that the Committee deems
appropriate for inclusion in a Restricted Stock Agreement. The provisions of the various Restricted Stock Agreements entered into under the Plan need not be identical. 
 (c) Payment for Restricted Stock. Restricted Stock may be issued with or without cash consideration under the Plan. 
 (d) Vesting Conditions. Each Award of Restricted Stock shall become vested, in full or in installments, upon satisfaction of the conditions specified in the Restricted Stock Agreement. A Restricted
Stock Agreement may provide for accelerated vesting in the event of the Participant’s death, Disability or retirement or other events. 

  
 8 

 (e) Assignment or Transfer of Restricted Stock. Except as provided in
Section 12, or in a Restricted Stock Agreement, or as required by applicable law, Restricted Stock granted under the Plan shall not be anticipated, assigned, attached, garnished, optioned, transferred or made subject to any creditor’s
process, whether voluntarily, involuntarily or by operation of law. Any act in violation of this Section 8(e) shall be void. However, this Section 8(e) shall not preclude a Participant from designating a beneficiary who will receive any
outstanding Restricted Stock in the event of the Participant’s death, nor shall it preclude a transfer of Restricted Stock by will or by the laws of descent and distribution. 

(f) Trusts. Neither this Section 8 nor any other provision of the Plan shall preclude a Participant from transferring or
assigning Restricted Stock to (a) the trustee of a trust that is revocable by such Participant alone, both at the time of the transfer or assignment and at all times thereafter prior to such Participant’s death, or (b) the trustee of
any other trust to the extent approved in advance by the Committee in writing. A transfer or assignment of Restricted Stock from such trustee to any person other than such Participant shall be permitted only to the extent approved in advance by the
Committee in writing, and Restricted Stock held by such trustee shall be subject to all of the conditions and restrictions set forth in the Plan and in the applicable Restricted Stock Agreement, as if such trustee were a party to such Agreement.

 (g) Voting and Dividend Rights. The holders of Restricted Stock awarded under the Plan shall have the same voting,
dividend and other rights as the Company’s other shareholders. A Restricted Stock Agreement, however, may require that the holders of Restricted Stock invest any cash dividends received in additional Restricted Stock. Such additional Restricted
Stock shall be subject to the same conditions and restrictions as the Award with respect to which the dividends were paid. Such additional Restricted Stock shall not reduce the number of Shares available under Section 5. 

SECTION 9. PROTECTION AGAINST DILUTION. 
 (a) Adjustments. In the event of a subdivision of the outstanding Shares, a declaration of a dividend payable in Shares, a declaration of a dividend payable in a form other than Shares in an amount
that has a material effect on the price of Shares, a combination or consolidation of the outstanding Shares (by reclassification or otherwise) into a lesser number of Shares, a recapitalization, a spin-off or a similar occurrence, the Committee
shall make such adjustments as it, in its sole discretion, deems appropriate in one or more of: 
  

	 	(i)	the number of Shares available for future Awards under Section 5; 

  

	 	(ii)	the number of Shares covered by each outstanding Award; or 

  

	 	(iii)	the Exercise Price under each outstanding Option. 

 (b) Participant Rights. Except as provided in this Section 9, a Participant shall have no rights by reason of any issue by the Company of stock of any class or securities convertible into
stock of any class, any subdivision or consolidation of shares of stock of any class, the payment of any stock dividend or any other increase or decrease in the number of shares of stock of any class. 

  
 9 

 SECTION 10. EFFECT OF A CHANGE IN CONTROL. 

(a) Merger or Reorganization. In the event that the Company is a party to a merger or other reorganization, outstanding Awards
shall be subject to the agreement of merger or reorganization. Such agreement may provide, without limitation, for the assumption of outstanding Awards or replacement Awards of substantially equivalent value by the surviving corporation or its
parent, for their continuation by the Company (if the Company is a surviving corporation), for accelerated vesting or for their cancellation with or without consideration, in all cases without the consent of the Participant. 

(b) Acceleration. Except as otherwise provided in the applicable Stock Option Agreement or Restricted Stock Agreement, in the event
that a Change in Control occurs with respect to the Company and the applicable agreement of merger or reorganization provides for assumption or continuation of Awards pursuant to Section 10(a), or the Awards otherwise continue, no acceleration
of vesting shall occur. In the event that a Change in Control occurs with respect to the Company and there is no assumption or continuation of Awards, all Awards shall vest and become immediately exercisable immediately before the Change in Control.

 SECTION 11. LIMITATIONS ON RIGHTS. 
 (a) Retention Rights. Neither the Plan nor any Award granted under the Plan shall be deemed to give any individual a right to remain an employee, consultant or director of the Company, a Parent, a
Subsidiary or an Affiliate. The Company and its Parents and Subsidiaries and Affiliates reserve the right to terminate the Service of any person at any time, and for any reason, subject to applicable laws, the Company’s Articles of
Incorporation and Bylaws and a written employment agreement (if any). 
 (b) Shareholders’ Rights. A Participant
shall have no dividend rights, voting rights or other rights as a shareholder with respect to any Shares covered by his or her Award prior to the issuance of a stock certificate for such Shares. No adjustment shall be made for cash dividends or
other rights for which the record date is prior to the date when such certificate is issued, except as expressly provided in Section 9. 
 (c) Regulatory Requirements. Any other provision of the Plan notwithstanding, the obligation of the Company to issue Shares under the Plan shall be subject to all applicable laws, rules and
regulations and such approval by any regulatory body as may be required. The Company reserves the right to restrict, in whole or in part, the delivery of Shares pursuant to any Award prior to the satisfaction of all legal requirements relating to
the issuance of such Shares, to their registration, qualification or listing or to an exemption from registration, qualification or listing. 

  
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 SECTION 12. WITHHOLDING TAXES. 

(a) General. A Participant shall make arrangements satisfactory to the Company for the satisfaction of any withholding tax
obligations that arise in connection with his or her Award. The Company shall not be required to issue any Shares or make any cash payment under the Plan until such obligations are satisfied. 

(b) Share Withholding. If a public market for the Company’s Shares exists, the Committee may permit a Participant to satisfy
all or part of his or her withholding or income tax obligations by having the Company withhold all or a portion of any Shares that otherwise would be issued to him or her or by surrendering all or a portion of any Shares that he or she previously
acquired. Such Shares shall be valued at their Fair Market Value on the date when taxes otherwise would be withheld in cash. Any payment of taxes by assigning Shares to the Company may be subject to restrictions, including, but not limited to, any
restrictions required by rules of the Securities and Exchange Commission. 
 SECTION 13. DURATION AND AMENDMENTS. 

(a) Term of the Plan. The Plan, as set forth herein, shall become effective on the date of its adoption by the Board, subject to
the approval of the Company’s shareholders. No Options shall be exercisable until such shareholder approval is obtained. In the event that the shareholders fail to approve the Plan within twelve (12) months after its adoption by the Board,
any ISO granted under the Plan shall revert to an NSO. To the extent required by applicable law, the Plan shall terminate on the date that is ten (10) years after its adoption by the Board and may be terminated on any earlier date pursuant to
Section 13(b). 
 (b) Right to Amend or Terminate the Plan. The Board may amend or terminate the Plan at any time and
for any reason. The termination of the Plan, or any amendment thereof, shall not affect any Award previously granted under the Plan. No Awards shall be granted under the Plan after the Plan’s termination. An amendment of the Plan shall be
subject to the approval of the Company’s shareholders only to the extent required by applicable laws, regulations or rules. 

********************************************************************** 

Adopted by the Board of Directors on June 28, 2002 and approved by the shareholders on August 7, 2002. 

Amended by the Board of Directors on April 11, 2011 to increase available shares from 2,000,000 to 2,300,000 and by shareholders
on May 20, 2011, 
 Expired on June 28, 2012. 

  
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