THIRD AMENDED AND RESTATED VOTING AGREEMENT

Exhibit 4.2

EXECUTION VERSION

THIRD AMENDED AND RESTATED

VOTING AGREEMENT

TABLE OF CONTENTS

		Page
1. Voting Provisions Regarding Board of Directors			2
1.1. Size of the Board			2
1.2. Board Composition			2
1.3. Failure to Designate a Board Member			3
1.4. Removal of Board Members			3
1.5. No Liability for Election of Recommended Directors			4
1.6. Co-Chairs and Committee Rights			4
2. Vote to Increase Authorized Common Stock			4
3. Drag-Along Right			4
3.1. Definitions			4
3.2. Actions to be Taken			5
3.3. Exceptions			6
3.4. Restrictions on Sales of Control of the Company			7
4. Remedies			7
4.1. Covenants of the Company			7
4.2. [Reserved]			7
4.3. Specific Enforcement			7
4.4. Remedies Cumulative			8
5. Term			8
6. Miscellaneous			8
6.1. Additional Parties			8
6.2. Transfers			8
6.3. Successors and Assigns			8
6.4. Governing			9
6.5. Counterparts; Facsimile or PDF			9
6.6. Titles and Subtitles			9
6.7. Notices			9
6.8. Consent Required to Amend, Terminate or Waive			10
6.9. Delays or Omissions			11
6.10. Severability			11
6.11. Entire Agreement			11

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6.12. Legend on Share Certificates			11
6.13. Stock Splits, Stock Dividends, etc.			12
6.14. Manner of Voting			12
6.15. Further Assurances			12
6.16. [Reserved]			12
6.17. [Reserved]			12
6.18. Aggregation of Stock			12
Schedule A - Investors
Schedule B - Key Holders
Exhibit A - Adoption Agreement

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THIRD AMENDED AND RESTATED VOTING AGREEMENT

THIS THIRD AMENDED AND RESTATED VOTING AGREEMENT is made and entered into as of this 10th day of May, 2013 by and among AKEBIA THERAPEUTICS, INC., a Delaware corporation (the “Company”), each holder of the Company’s Series C Preferred Stock, $0.00001 par value per share (“Series C Preferred Stock”), the Company’s Series B Preferred Stock, $0.00001 par value per share (“Series B Preferred Stock”) and Series A Preferred Stock, $0.00001 par value per share (“Series A Preferred Stock”) listed on Schedule A (together with any subsequent investors, or transferees, who become parties hereto as “Investors” pursuant to Sections 6.1 or 6.2 below, the “Investors”) and those certain stockholders of the Company listed on Schedule B (together with any subsequent stockholders or option holders, or any transferees, who become parties hereto as “Key Holders” pursuant to Sections 6.1 or 6.2 below, the “Key Holders”, and together collectively with the Investors, the “Stockholders”).

RECITALS

WHEREAS, certain of the Investors have previously purchased equity securities of the Company;

WHEREAS, the Company and certain of the Investors are parties to the Series C Preferred Stock Purchase Agreement, dated as of the date hereof (as it may be amended, restated, supplemented or otherwise modified from time to time, the “Series C Purchase Agreement”);

WHEREAS, the Company, certain of the Investors, the Key Holders and certain other Persons (as defined below) previously entered into a Voting Agreement, dated as of January 23, 2008, as amended and restated as of July 15, 2009 and April 6, 2011 (the “Prior Agreement”);

WHEREAS, the Company and certain of the Investors are parties to the Series B Preferred Stock Purchase Agreement, dated as of April 6, 2011;

WHEREAS, the Company and certain Investors entered into Series A Preferred Stock Purchase Agreements dated January 23, 2008 and July 15, 2009 in connection with the purchase of shares of Series A Preferred Stock;

WHEREAS, the Company and its existing Investors desire to induce the Investors to purchase shares of Series C Preferred Stock of the Company, pursuant to the Series C Purchase Agreement, by amending and restating the Prior Agreement to provide the Investors with the rights and privileges as set forth herein; and

WHEREAS, the Eighth Amended and Restated Certificate of Incorporation of the Company (the “Restated Certificate”) provides that (a) the holders of record of the shares of the Company’s Series A Preferred Stock, Series B Preferred Stock and Series C Preferred Stock (collectively, “Preferred Stock”), exclusively and together as a single class (separate from other classes), voting on a pari passu basis, shall be entitled to elect six (6) directors of the Company (the “Preferred Directors”), and (b) the holders of shares of Common Stock (including, on an as-converted to Common Stock basis, all shares of Preferred Stock convertible into Common Stock) shall be entitled to elect three (3) directors of the Company (the “Common Directors”).

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For purposes of this Agreement, “Common Stock” means the Common Stock of the Company, par value $0.00001 per share.

NOW, THEREFORE, the parties agree as follows:

1. Voting Provisions Regarding Board of Directors.

1.1. Size of the Board. Each Stockholder agrees to vote, or cause to be voted, all Shares (as defined below) owned by such Stockholder, or over which such Stockholder has voting control, from time to time and at all times, in whatever manner as shall be necessary to ensure that the size of the Board shall be set and remain at nine (9) directors (subject to reduction for (i) the vacancy described in Section 1.2(e) below should Xx. Xxxx no longer be a Board member and (ii) any vacancies for Board seats not ultimately filled pursuant to Section 1.2(d) below) and may be increased only with the written consent of holders of more than the Appropriate Percentage (as defined below) of the shares of Common Stock then issuable upon conversion of the then outstanding shares of Preferred Stock. For purposes of this Agreement, the term “Shares” shall mean and include any securities of the Company the holders of which are entitled to vote for members of the Board, including without limitation, all shares of Common Stock and Preferred Stock, by whatever name called, now owned or subsequently acquired by a Stockholder, however acquired, whether through stock splits, stock dividends, reclassifications, recapitalizations, similar events or otherwise. For the purposes of this Agreement, the term “Appropriate Percentage” means fifty percent (50%).

1.2. Board Composition. Each Stockholder agrees to vote, or cause to be voted, all Shares owned by such Stockholder, or over which such Stockholder has voting control, from time to time and at all times, in whatever manner as shall be necessary to ensure that at each annual or special meeting of stockholders at which an election of directors is held or pursuant to any written consent of the stockholders, the following persons shall be elected to the Board:

(a) As a Preferred Director, one person designated by Xxxxxx Investment Management, LLC, which individual shall initially be Xxxxxx X. Xxxxxx.

(b) As Preferred Directors, two persons designated by Novartis Bioventures Ltd., which individuals shall initially be Xxxxxxxx Xxxxxx and Xxxxxxxx Xxxxxxx.

(c) As a Preferred Director, one person designated by Kearny Venture Partners, which individual shall initially be Xxxxxx Xxxxx.

(d) If an Investor that (together with its Affiliates) does not purchase shares of Series C Preferred Stock at Closing 1 purchases at least $5,000,000 of Series C Preferred Stock at an Additional Closing (as defined in the Series C Purchase Agreement), such Investor may designate (i) one Preferred Director if such investment is at least $5,000,000, but less than $8,400,000 and (ii) two Preferred Directors if such investment is at least $8,400,000 (each, a “New Investor Director”); provided that the Board seats reserved for such New Investor Directors shall remain vacant unless and until such New Investor Directors are designated pursuant to the terms of this Section 1.2(d).

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(e) As Common Directors, two persons, one of whom shall be an independent outside director, to be designated by a majority of the Board members, and the other who shall be Xxxxx Xxxx; provided that the Board seat reserved for Xx. Xxxx shall not be filled at any time after the date hereof on which a majority of the Board or Xx. Xxxx determines that Xx. Xxxx shall not serve on the Board or his earlier death or disability, and each of the Stockholders shall promptly vote their respective Shares to remove Xx. Xxxx from the Board upon any such occurrence.

(f) As a Common Director, the Company’s Chief Executive Officer, who shall initially be Xxxxxx Xxxxxxx (the “CEO Director”), provided that if for any reason the CEO Director shall cease to serve as the Chief Executive Officer of the Company, each of the Stockholders shall promptly vote their respective Shares (i) to remove the former Chief Executive Officer from the Board if such person has not resigned as a member of the Board and (ii) to elect such person’s replacement as Chief Executive Officer of the Company as the new CEO Director.

Except with respect to clauses (d) and (e) above, to the extent that any of clauses (a) through (f) above shall not be applicable, any member of the Board who would otherwise have been designated in accordance with the terms thereof shall instead be voted upon by all the stockholders of the Company entitled to vote thereon in accordance with, and pursuant to, the Company’s Restated Certificate.

For purposes of this Agreement, an individual, firm, corporation, partnership, association, limited liability company, trust or any other entity (collectively, a “Person”) shall be deemed an “Affiliate” of another Person who, directly or indirectly, controls, is controlled by or is under common control with such Person, including, without limitation, any general partner, managing member, officer or director of such Person or any venture capital fund now or hereafter existing that is controlled by one or more general partners or managing members of, or shares the same management company with, such Person, and provided that Xxxxxx Investment Management, LLC, Xxxxxx X. Xxxxxx and all Persons for which Xx. Xxxxxx or any of his immediate family members serves as trustee or investment advisor or any similar capacity (and their respective Affiliates) and any account held for the benefit of any such Person shall be Affiliates of one another, regardless of whether they would otherwise be deemed Affiliates hereunder.

1.3. Failure to Designate a Board Member. In the absence of any designation from the Persons or groups with the right to designate a director as specified above, the director previously designated by them and then serving shall be reelected if still eligible to serve as provided herein.

1.4. Removal of Board Members. Each Stockholder also agrees to vote, or cause to be voted, all Shares owned by such Stockholder, or over which such Stockholder has voting control, from time to time and at all times, in whatever manner as shall be necessary to ensure that:

(a) no director elected pursuant to Sections 1.2 or 1.3 of this Agreement may be removed from office unless (i) such removal is directed or approved by the affirmative vote of the Person entitled under Section 1.2 to designate that director or (ii) the Persons originally entitled to designate or approve such director or occupy such Board seat pursuant to Section 1.2 is no longer so entitled to designate or approve such director or occupy such Board seat;

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(b) any vacancies created by the resignation, removal or death of a director elected pursuant to Sections 1.2 or 1.3 shall be filled (or, with respect to Xx. Xxxx and the New Investor Directors, result in a reduction in the authorized size of the Board as described in Section 1.1 above) pursuant to the provisions of this Section 1; and

(c) upon the request of any party entitled to designate a director as provided in Section 1.2(a) through (f) to remove such director, such director shall be removed.

All Stockholders agree to execute any written consents required to perform the obligations of this Agreement, and the Company agrees at the request of any party entitled to designate directors to call a special meeting of stockholders for the purpose of electing directors.

1.5. No Liability for Election of Recommended Directors. No Stockholder, nor any Affiliate of any Stockholder, shall have any liability as a result of designating a person for election as a director for any act or omission by such designated person in his or her capacity as a director of the Company, nor shall any Stockholder have any liability as a result of voting for any such designee in accordance with the provisions of this Agreement.

1.6. Co-Chairs and Committee Rights. Each Stockholder agrees to vote or cause to be voted all Shares owned by such Stockholder, or over which such Stockholder has voting control, from time to time and at all times, in whatever manner as shall be necessary to cause and the Company agrees to take whatever associated actions are necessary to cause (a) the director elected pursuant to Section 1.2(a) and whichever of the two directors who is elected pursuant to Section 1.2(b) who is thereafter also designated by Novartis Bioventures Ltd. to be elected and maintain the positions of co-Chairmen of the Board (which, for the avoidance of doubt, shall be Board governance positions and not positions as an officer of the Company), and (b) the director elected pursuant to Section 1.2(a) and one of the directors selected by Novartis Bioventures Ltd. pursuant to Section 1.2(b) to have the right to serve on any and all committees of the Board. The Company shall cause management to participate in frequent and regular conference calls with one or both of the co-Chairmen of the Board.

2. Vote to Increase Authorized Common Stock. Each Stockholder agrees to vote or cause to be voted all Shares owned by such Stockholder, or over which such Stockholder has voting control, from time to time and at all times, in whatever manner as shall be necessary to increase the number of authorized shares of Common Stock from time to time to ensure that there will be sufficient shares of Common Stock available for conversion of all of the shares of Preferred Stock outstanding at any given time.

3. Drag-Along Right.

3.1. Definitions. A “Sale of the Company” shall mean either: (a) a transaction or series of related transactions in which a Person, or a group of related Persons, acquires from stockholders of the Company shares representing a majority of the outstanding voting power of the Company (a “Stock Sale”); or (b) a transaction that qualifies as a “Deemed Liquidation Event” as defined in the Restated Certificate.

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3.2. Actions to be Taken. In the event that (i) the holders of more than the Appropriate Percentage of the shares of Common Stock then issuable upon conversion of the then outstanding shares of Preferred Stock (the “Selling Investors”) and (ii) the Board of Directors approve a Sale of the Company in writing, specifying that this Section 3 shall apply to such transaction, then each Stockholder hereby agrees:

(a) if such transaction requires stockholder approval, with respect to all Shares that such Stockholder owns or over which such Stockholder otherwise exercises voting power, to vote (in person, by proxy or by action by written consent, as applicable) all Shares in favor of, and adopt, such Sale of the Company (together with any related amendment to the Restated Certificate required in order to implement such Sale of the Company) and to vote in opposition to any and all other proposals that could reasonably be expected to delay or impair the ability of the Company to consummate such Sale of the Company;

(b) if such transaction is a Stock Sale, to sell the same proportion of shares of capital stock of the Company beneficially held by such Stockholder as is being sold by the Selling Investors to the Person to whom the Selling Investors propose to sell their Shares, and, except as permitted in Section 3.3 below, on the same terms and conditions as the Selling Investors;

(c) to execute and deliver all related documentation and take such other action in support of the Sale of the Company as shall reasonably be requested by the Company or the Selling Investors in order to carry out the terms and provision of this Section 3, including without limitation executing and delivering instruments of conveyance and transfer, and any purchase agreement, merger agreement, indemnity agreement, escrow agreement, consent, waiver, governmental filing, share certificates duly endorsed for transfer (free and clear of impermissible liens, claims and encumbrances) and any similar or related documents;

(d) not to deposit, and to cause their Affiliates not to deposit, except as provided in this Agreement, any Shares of the Company owned by such party or Affiliate in a voting trust or subject any Shares to any arrangement or agreement with respect to the voting of such Shares, unless specifically requested to do so by the acquiror in connection with the Sale of the Company;

(e) to refrain from exercising any dissenters’ rights or rights of appraisal under applicable law at any time with respect to such Sale of the Company; and

(f) if the consideration to be paid in exchange for the Shares pursuant to this Section 3 includes any securities and due receipt thereof by any Stockholder would require under applicable law (x) the registration or qualification of such securities or of any person as a broker or dealer or agent with respect to such securities or (y) the provision to any Stockholder of any information other than such information as a prudent

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issuer would generally furnish in an offering made solely to “accredited investors” as defined in Regulation D promulgated under the Securities Act of 1933, as amended, the Company may cause to be paid to any such Stockholder in lieu thereof, against surrender of the Shares which would have otherwise been sold by such Stockholder, an amount in cash equal to the fair value (as determined in good faith by the Company) of the securities which such Stockholder would otherwise receive as of the date of the issuance of such securities in exchange for the Shares.

3.3. Exceptions. Notwithstanding the foregoing, a Stockholder will not be required to comply with Section 3.2 above in connection with any proposed Sale of the Company (the “Proposed Sale”) unless:

(a) [Reserved.]

(b) the Stockholder shall not be liable for the inaccuracy of any representation or warranty made by any other Person in connection with the Proposed Sale, other than the Company (except to the extent that funds may be paid out of an escrow established to cover breach of representations, warranties and covenants of the Company as well as breach by any stockholder of any of identical representations, warranties and covenants provided by all stockholders);

(c) the liability for indemnification, if any, of such Stockholder in the Proposed Sale and for the inaccuracy of any representations and warranties made by the Company in connection with such Proposed Sale, is several and not joint with any other Person (except to the extent that funds may be paid out of an escrow established to cover breach of representations, warranties and covenants of the Company as well as breach by any stockholder of any of identical representations, warranties and covenants provided by all stockholders), and is pro rata in proportion to the amount of consideration paid to such Stockholder in connection with such Proposed Sale (in accordance with the provisions of the Restated Certificate);

(d) liability shall be limited to such Stockholder’s applicable share (determined based on the respective proceeds payable to each Stockholder in connection with such Proposed Sale in accordance with the provisions of the Restated Certificate) of a negotiated aggregate indemnification amount that applies equally to all Stockholders but that in no event exceeds the amount of consideration otherwise payable to such Stockholder in connection with such Proposed Sale, except with respect to claims related to fraud by such Stockholder, the liability for which need not be limited as to such Stockholder;

(e) upon the consummation of the Proposed Sale, (i) each holder of each class or series of the Company’s stock will receive the same form of consideration for their shares of such class or series as is received by other holders in respect of their shares of such same class or series of stock, (ii) each holder of a series of Preferred Stock will receive the same amount of consideration per share of such series of Preferred Stock as is received by other holders in respect of their shares of such same series, (iii) each holder of Common Stock will receive the same amount of consideration per share of

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Common Stock as is received by other holders in respect of their shares of Common Stock, and (iv) unless the holders of more than the Appropriate Percentage of the shares of Common Stock then issuable upon conversion of the then outstanding shares of Preferred Stock elect otherwise by written notice given to the Company at least five (5) days prior to the effective date of any such Proposed Sale, the aggregate consideration receivable by all holders of the Preferred Stock and Common Stock shall be allocated among the holders of Preferred Stock and Common Stock on the basis of the relative liquidation preferences to which the holders of each respective series of Preferred Stock and the holders of Common Stock are entitled in a Deemed Liquidation Event (assuming for this purpose that the Proposed Sale is a Deemed Liquidation Event) in accordance with the Company’s Certificate of Incorporation in effect immediately prior to the Proposed Sale; and

(f) subject to clause (e) above, requiring the same form of consideration to be available to the holders of any single class or series of capital stock, if any holders of any capital stock of the Company are given an option as to the form and amount of consideration to be received as a result of the Proposed Sale, all holders of such capital stock will be given the same option.

3.4. Restrictions on Sales of Control of the Company. No Stockholder shall be a party to any Stock Sale unless all holders of Preferred Stock are allowed to participate in such transaction and the consideration received pursuant to such transaction is allocated among the parties thereto in the manner specified in the Company’s Certificate of Incorporation in effect immediately prior to the Stock Sale (as if such transaction were a Deemed Liquidation Event), unless the holders of more than the Appropriate Percentage of the shares of Common Stock then issuable upon conversion of the then outstanding shares of Preferred Stock elect otherwise by written notice given to the Company at least five (5) days prior to the effective date of any such transaction or series of related transactions.

4. Remedies.

4.1. Covenants of the Company. The Company agrees to use its best efforts, within the requirements of applicable law, to ensure that the rights granted under this Agreement are effective and that the parties enjoy the benefits of this Agreement. Such actions include, without limitation, the use of the Company’s best efforts to cause the nomination and election of the directors as provided in this Agreement.

4.2. [Reserved].

4.3. Specific Enforcement. Each party acknowledges and agrees that each party hereto will be irreparably damaged in the event any of the provisions of this Agreement are not performed by the parties in accordance with their specific terms or are otherwise breached. Accordingly, it is agreed that each of the Company and the Stockholders shall be entitled to an injunction to prevent breaches of this Agreement, and to specific enforcement of this Agreement and its terms and provisions in any action instituted in any court of the United States or any state having subject matter jurisdiction.

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4.4. Remedies Cumulative. All remedies, either under this Agreement or by law or otherwise afforded to any party, shall be cumulative and not alternative.

5. Term. This Agreement shall be effective as of the date hereof and shall continue in effect until and shall terminate upon the earliest to occur of (a) the consummation of the Company’s first underwritten public offering of its Common Stock (other than a registration statement relating either to the sale of securities to employees of the Company pursuant to its stock option, stock purchase or similar plan or an SEC Rule 145 transaction); (b) the consummation of a Sale of the Company and distribution of proceeds to or escrow for the benefit of the Stockholders in accordance with the Restated Certificate, provided that the provisions of Section 3 hereof will continue after the closing of any Sale of the Company to the extent necessary to enforce the provisions of Section 3 with respect to such Sale of the Company; or (c) termination of this Agreement in accordance with Section 6.8 below. The provisions contained in Sections 1.2(a)-(c) shall expire if the Person otherwise entitled to designate or approve such director, and his, her or its Affiliates collectively hold no shares of Preferred Stock.

6. Miscellaneous.

6.1. Additional Parties. In the event that after the date of this Agreement, the Company enters into an agreement with any Person to issue shares of capital stock to such Person, then, the Company shall cause such Person, as a condition precedent to entering into such agreement, to become a party to this Agreement by executing and delivering (i) the Adoption Agreement attached to this Agreement as Exhibit A, or (ii) a counterpart signature page hereto agreeing to be bound by and subject to the terms of this Agreement as a Stockholder and thereafter such person shall be deemed a Stockholder for all purposes under this Agreement.

6.2. Transfers. Each transferee or assignee of any Shares subject to this Agreement shall continue to be subject to the terms hereof, and, as a condition precedent to the Company’s recognizing such transfer, each transferee or assignee shall agree in writing to be subject to each of the terms of this Agreement by executing and delivering an Adoption Agreement substantially in the form attached hereto as Exhibit A. Upon the execution and delivery of an Adoption Agreement by any transferee, such transferee shall be deemed to be a party hereto as if such transferee were the transferor and such transferee’s signature appeared on the signature pages of this Agreement and shall be deemed to be an Investor and Stockholder, or Key Holder and Stockholder, as applicable. The Company shall not permit the transfer of the Shares subject to this Agreement on its books or issue a new certificate representing any such Shares unless and until such transferee shall have complied with the terms of this Section 6.2. Each certificate representing the Shares subject to this Agreement if issued on or after the date of this Agreement shall be endorsed by the Company with the legend set forth in Section 6.12.

6.3. Successors and Assigns. The terms and conditions of this Agreement shall inure to the benefit of and be binding upon the respective successors and assigns of the parties. Nothing in this Agreement, express or implied, is intended to confer upon any party other than the parties hereto or their respective successors and assigns any rights, remedies, obligations, or liabilities under or by reason of this Agreement, except as expressly provided in this Agreement.

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6.4. Governing. This Agreement shall be governed by, and construed in accordance with, the laws of the State of Delaware, regardless of the laws that might otherwise govern under applicable principles of conflicts of law.

6.5. Counterparts; Facsimile or PDF. This Agreement may be executed and delivered by facsimile or PDF signature and in two or more counterparts, each of which shall be deemed an original, but all of which together shall constitute one and the same instrument.

6.6. 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.

6.7. Notices. All notices and other communications given or made pursuant to this Agreement shall be in writing and shall be deemed effectively given upon the earlier of actual receipt or: (a) personal delivery to the party to be notified, (b) when sent, if sent by electronic mail or facsimile during normal business hours of the recipient, and if not sent during normal business hours, then on the recipient’s next business day, (c) five (5) days after having been sent by registered or certified mail, return receipt requested, postage prepaid, or (d) one (1) business day after the business day of deposit with a nationally recognized overnight courier, freight prepaid, specifying next business day delivery, with written verification of receipt. All communications shall be sent to the respective parties at their address as set forth on Schedule A or Schedule B hereto, or to such email address, facsimile number or address as subsequently modified by written notice given in accordance with this Section 6.7.

If notice is given to the Company, a copy shall also be sent to:

Xxxxxxxx Xxxx LLP

000 Xxxxxx Xxxxxx, 00xx Xxxxx

Xxxxxxxxxx, XX 00000-0000

Attn: Xxxxx X. Willbrand

xxxxx.xxxxxxxxx@xxxxxxxxxxxx.xxx

If notice is given to Stockholders, a copy shall also be sent to:

Xxxxxxxx & Xxxxx LLP

000 X. XxXxxxx Xx.

Xxxxxxx, XX 00000

Attn:   Xxx X. Xxxx, P.C.

  Xxxxx X. Xxxxxx

E-mail: xxx.xxxx@xxxxxxxx.xxx

xxxxx.xxxxxx@xxxxxxxx.xxx

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and

Xxxxxxx Xxxxxxx Xxxxxx LLP

Attn: Xxxxxx X. Xxxxx

000 Xxxxxxxxxx Xxxxxx

Xxxxxx, XX 00000 XXX

xxxxxx@xxxxxxxxxxxxxx.xxx

6.8. Consent Required to Amend, Terminate or Waive. This Agreement may be amended or terminated and the observance of any term hereof may be waived (either generally or in a particular instance and either retroactively or prospectively) only by a written instrument executed by (a) the Company; and (b) the holders of more than the Appropriate Percentage of the shares of Common Stock then issuable upon conversion of the then outstanding shares of Preferred Stock. Notwithstanding the foregoing:

(i) this Agreement may not be amended or terminated and the observance of any term of this Agreement may not be waived with respect to any Stockholder without the written consent of such Stockholder unless such amendment, termination or waiver applies to all Stockholders in the same fashion;

(ii) Schedules A and B hereto may be amended by the Company from time to time to add information regarding additional Investors (as defined in the Series B Purchase Agreement) without the consent of the other parties hereto;

(iii) any provision hereof may be waived by the waiving party on such party’s own behalf, without the consent of any other party; and

(iv) Section 1.2(a) of this Agreement shall not be amended or waived without the written consent of Xxxxxx Investment Management, LLC, Section 1.2(b) of this Agreement shall not be amended or waived without the written consent of Novartis Bioventures Ltd., Section 1.2(c) shall not be amended or waived without the written consent of Kearny Venture Partners, Section 1.2(d) shall not be amended or waived without the written consent of the new Investor (if any) having the right to designate any New Investor Director thereunder, Section 1.2(e) shall not be amended or waived without the written consent of the holders of a majority of the outstanding shares of Common Stock (including, on an as-converted to Common Stock basis, all shares of Preferred Stock convertible into Common Stock) and Section 1.6 shall not be amended or waived without the written consent of Xxxxxx Investment Management, LLC and Novartis Bioventures Ltd. Notwithstanding the foregoing, if any amendment, modification, termination or waiver of this section or any other section or subsection of this Agreement would deprive Xxxxxx Investment Management, LLC, Novartis Bioventures Ltd., Kearny Venture Partners or any applicable new Investor (in the case of Section 1.2(d)) of its director designation rights (or of Xxxxx Xxxx’x right to be named to the Board) under Section 1.2, then the written consent of such party or such person (in the case of Xx. Xxxx) shall be required.

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The Company shall give prompt written notice of any amendment, termination or waiver hereunder to any party that did not consent in writing thereto. Any amendment, termination or waiver effected in accordance with this Section 6.8 shall be binding on each party and all of such party’s successors and permitted assigns, whether or not any such party, successor or assignee entered into or approved such amendment, termination or waiver.

6.9. Delays or Omissions. No delay or omission to exercise any right, power or remedy accruing to any party under this Agreement, upon any breach or default of any other party under this Agreement, shall impair any such right, power or remedy of such non-breaching or non-defaulting party nor shall it be construed to be a waiver of any such breach or default, or an acquiescence therein, or of or in any similar breach or default thereafter occurring; nor shall any waiver of any single breach or default be deemed a waiver of any other breach or default previously or thereafter occurring. Any waiver, permit, consent or approval of any kind or character on the part of any party of any breach or default under this Agreement, or any waiver on the part of any party of any provisions or conditions of this Agreement, must be in writing and shall be effective only to the extent specifically set forth in such writing. All remedies, either under this Agreement or by law or otherwise afforded to any party, shall be cumulative and not alternative.

6.10. Severability. The invalidity or unenforceability of any provision hereof shall in no way affect the validity or enforceability of any other provision.

6.11. Entire Agreement. This Agreement (including the Exhibits hereto), and the Restated Certificate and the other Transaction Agreements (as defined in the Series C Purchase Agreement) constitute the full and entire understanding and agreement between the parties with respect to the subject matter hereof, and any other written or oral agreement relating to the subject matter hereof existing between the parties is expressly canceled.

6.12. Legend on Share Certificates. Each certificate representing any Shares issued after the date hereof shall be endorsed by the Company with a legend reading substantially as follows:

“THE SHARES EVIDENCED HEREBY ARE SUBJECT TO A VOTING AGREEMENT, AS MAY BE AMENDED FROM TIME TO TIME, (A COPY OF WHICH MAY BE OBTAINED UPON WRITTEN REQUEST FROM THE COMPANY), AND BY ACCEPTING ANY INTEREST IN SUCH SHARES THE PERSON ACCEPTING SUCH INTEREST SHALL BE DEEMED TO AGREE TO AND SHALL BECOME BOUND BY ALL THE PROVISIONS OF THAT VOTING AGREEMENT, INCLUDING CERTAIN RESTRICTIONS ON TRANSFER AND OWNERSHIP SET FORTH THEREIN.”

The Company, by its execution of this Agreement, agrees that it will cause the certificates evidencing the Shares issued after the date hereof to bear the legend required by this Section 6.12 of this Agreement, and it shall supply, free of charge, a copy of this Agreement to any holder of a certificate evidencing Shares upon written request from such holder to the Company at its principal office. The parties to this Agreement do hereby agree that the failure to cause the certificates evidencing the Shares to bear the legend required by this Section 6.12 herein and/or the failure of the Company to supply, free of charge, a copy of this Agreement as provided hereunder shall not affect the validity or enforcement of this Agreement.

-11-

6.13. Stock Splits, Stock Dividends, etc. In the event of any issuance of Shares of the Company’s voting securities hereafter to any of the Stockholders (including, without limitation, in connection with any stock split, stock dividend, recapitalization, reorganization, or the like), such Shares shall become subject to this Agreement and shall be endorsed with the legend set forth in Section 6.12.

6.14. Manner of Voting. The voting of Shares pursuant to this Agreement may be effected in person, by proxy, by written consent or in any other manner permitted by applicable law.

6.15. Further Assurances. At any time or from time to time after the date hereof, the parties agree to cooperate with each other, and at the request of any other party, to execute and deliver any further instruments or documents and to take all such further action as the other party may reasonably request in order to evidence or effectuate the consummation of the transactions contemplated hereby and to otherwise carry out the intent of the parties hereunder.

6.16. [Reserved]

6.17. [Reserved]

6.18. Aggregation of Stock. All Shares held or acquired by a Stockholder and/or its Affiliates shall be aggregated together for the purpose of determining the availability of any rights under this Agreement, and such Affiliated persons may apportion such rights as among themselves in any manner they deem appropriate.

[Signature Page Follows]

-12-

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

AKEBIA THERAPEUTICS, INC.
By:		/s/ Xxxxxx X. Xxxxxxx
		Xxxxxx X. Xxxxxxx, Ph.D.
		President and Chief Executive Officer
Address: Suite 420, 0000 Xxxxxx Xxxx, Xxxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

AGECHEM VENTURE FUND L.P.
By:		/s/ Xxxxx Xxxxxxx
		Name: Xxxxx Xxxxxxx
		Title: President
Address: Attn: Xxxxx Xxxxxxx, President 0 Xxxxxxxxx Xxxxxx, Xxxxx 000 Xxxxxxxx, Xxxxxx, Xxxxxx X0X 0X0

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

ATHENIAN VENTURE PARTNERS III L.P. By:   Athenian III, Ltd Its:   General Partner
By:		/s/ Xxxx X. Xxxxxxxx
		Name: Xxxx X. Xxxxxxxx
		Title: President

AVP OHIO TECHNOLOGY I L.P. By:   AVP Ohio I, Ltd. Its:   General Partner
By:		/s/ Xxxx Xxxxxxxx
		Name: Xxxx X. Xxxxxxxx
		Title: President
Address: 000 Xxxx Xxxxx Xxxxxx Xxxx 00/Xxxxx 000X Xxxxxx XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

BLUE CHIP VALIDATION FUND, LTD. By:   Blue Chip Venture Company, LTD Its:   Manager
By:		/s/ Xxxx XxXxxxxxxx
		Xxxx XxXxxxxxxx
		Managing Director
Address: 000 Xxxxxx Xxxxxx Xxxxx 0000 Xxxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

CINCINNATI CORNERSTONE INVESTORS AKB, LLC
By:		/s/ Xxxxxx X. Xxx, Xx.
		Xxxxxx X. Xxx, Xx.
		President
Address: 00 Xxxx 0xx Xxxxxx, 0xx Xxxxx Xxxxxxxxxx, XX 00000-0000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXXXXX XXXX

/s/ Xxxxxxx Xxxx

Xxxxxxx Xxxx

Address:

00 Xxx Xxxxxxx

Xxx Xxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXXX X. XXXXXX TRUST
By:		/s/ Xxxx Xxxxxx
		Name: Xxxx Xxxxxx
		Title: Trustee
XXXX XXXXXX
		/s/ Xxxx Xxxxxx
		Xxxx Xxxxxx
Address: 0000 Xxxxxx Xxxxxx Xxxxx Xxxx Xxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

FAMILY AKEBIA INVESTMENTS LLC
By:		/s/ Xxxxxx Xxxxxxxxx
		Name: Xxxxxx Xxxxxxxxx
		Title: Managing Member
Address: 0000 Xxxx Xxxxxx #00X Xxx Xxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXX XXXXXXX

/s/ Xxxx Xxxxxxx

Xxxx Xxxxxxx

Address:

0000 Xxxxxxxxxx Xx.

Xxxxxxxxxx, XX 00000-0000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXX XXXXXXXX TRUST, XXXXXX XXXXXXXX, TRUSTEE
By:		/s/ Xxxxxx Xxxxxxxx
		Name: Xxxxxx Xxxxxxxx
		Title: Trustee
Address: 0000 Xxxxxx Xxxx Xxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above

XXXXXXX FAMILY TRUST, XXXX X. XXXXXXX TRUSTEE
By:		/s/ Xxxx X. Xxxxxxx
		Xxxx X. Xxxxxxx Trustee
Address: 000 Xxxx Xxxxx Xxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

GITANA FAMILY TRUST, XXXXXXXXX X. XXXXXXXX TRUSTEE
By:		/s/ Xxxxxxxxx X. Xxxxxxxx
		Xxxxxxxxx X. Xxxxxxxx Trustee
Address: 0000 Xxxxxx Xxxxx Xxxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXXXX X. XXXXXXX
By:		/s/ Xxxxxx X. Xxxxxxx
Address: 0000 Xxxxxx Xxxx Xxxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

IAN X. X. XXXXX, XXX, STERLING TRUST CUSTODIAN
By:		/s/ Ian X. X. Xxxxx
Name: Ian X. X. Xxxxx
Title: Trustee
IAN X. X. XXXXX
/s/ Xxx X. X. Xxxxx
Xxx X. X. Xxxxx
Address: 000 Xxxxxxxxx Xxxxx Xxxxxx Xxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

KEARNY VENTURE PARTNERS, L.P.
By:		/s/ Xxxxxx Xxxxx
		Name: Kearny Venture Associates, LLC
		Title: its General Partner
Address: Attn:Xxxxxx Xxxxx Kearny Venture Associates LLC 00 Xxxxxx Xxxxxx, Xxxxx 000 Xxx Xxxxxxxxx, XX 00000-0000

KEARNY VENTURE PARTNERS ENTREPRENEURS FUND, L.P.
By:		/s/ Xxxxxx Xxxxx
Name: Kearny Venture Associates, LLC
Title: its General Partner
Address: Attn:Xxxxxx Xxxxx Kearny Venture Associates LLC 00 Xxxxxx Xxxxxx, Xxxxx 000 Xxx Xxxxxxxxx, XX 00000-0000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

MCIL WRAITH INVESTMENTS, LLC
By:		/s/ Xxxx XxXxxxxxxx
		Name: Xxxx XxXxxxxxxx
		Title: Manager
Address: Attn: Xxxx XxXxxxxxxx 0000 Xxxxxxx Xxxx Xxxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

MRK INTERNATIONAL, LLC
By:		/s/ Xxxxxxx X. Xxxxx
Name: Xxxxxxx X. Xxxxx
Title: Principal Member
Address: 0000 Xxxxxxxx Xxxxx Xxxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

NOVARTIS BIOVENTURES LTD.
By:		/s/ X.X. Xxxx
Name: X. X. Xxxx Title: Deputy Chairman
			By:		/s/ Xxxxxxx Xxxxx
Name: Xxxxxxx Xxxxx Title: Authorized Signatory
			Address: NOVARTIS BIOVENTURES LTD. Attn: Xxxxx Xxxxx Zivi 000 Xxxxx Xxxxxx Xxxxxxxx XX 12 Bermuda But for mail, to: Novartis BioVentures Ltd. Attn: Xxxxx Xxxxx Zivi PO Box HM 2899 Xxxxxxxx XX LX Bermuda And, also send a copy to: Novartis Venture Fund Attn: Xxxxxxxx Xxxxxx Five Xxxxxxxxx Xxxxxx, Xxxxx 000 Xxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXXX XXXXXX

/s/ Xxxxx Xxxxxx

Xxxxx Xxxxxx

Address:

0000 Xxxxx Xxxx

Xxxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

QCA FIRST FUND II
By:		/s/ Xxxx Xxxxxxx
Name: Xxxx Xxxxxxx Title: Manager
			Address: 000 Xxxxxxxx Xx. Xxxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXXXX X. XXXXXX REVOCABLE TRUST
By:		/s/ Xxxxxx X. Xxxxxx
Name: Xxxxxx X. Xxxxxx
Title: Trustee

THE XXXXXX FOUNDATION
By:		/s/ Xxxxxx X. Xxxxxx
Name: Xxxxxx X. Xxxxxx
Title: Trustee

XXXXXX FAMILY TRUST
By:		/s/ Xxxxxx X. Xxxxxx
Name: Xxxxxx X. Xxxxxx
Title: Investment Advisor

XXXXXX CHILDREN’S TRUST I
By:		/s/ Xxxxxx X. Xxxxxx
Name: Xxxxxx X. Xxxxxx
Title: Investment Advisor
Address: c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXXXXX XXXXXX XXXXXX REVOCABLE TRUST
By:		/s/ Xxxxxxx Xxxxxx Xxxxxx
Name: Xxxxxxx Xxxxxx Xxxxxx
Title:   Trustee
Address:
c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXX-XXXXXX XXXXXX INSURANCE TRUST
By:		/s/ Xxxxxx X. Xxxxxx
Name: Xxxxxx X. Xxxxxx
Title:   Trustee
ROSE XXXXXXX XXXXX INSURANCE TRUST
By:		/s/ Xxxxxx X. Xxxxxx
Name: Xxxxxx X. Xxxxxx
Title:   Investment Advisor
XXXX X. XXXXXX INSURANCE TRUST
By:		/s/ Xxxxxx X. Xxxxxx
Name: Xxxxxx X. Xxxxxx
Title:   Investment Advisor
XXXX XXXX TRUST
By:		/s/ Xxxxxx X. Xxxxxx
Name: Xxxxxx X. Xxxxxx
Title:   Investment Advisor
Address:
c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXXX XXXXXX INSURANCE TRUST
By:		/s/ Xxxxxx X. Xxxxxx
Name: Xxxxxx X. Xxxxxx
Title:   Trustee
XXXXXX AND XXXXXXX XXXX XXXXXX INSURANCE TRUST
By:		/s/ Xxxxxx X. Xxxxxx
Name: Xxxxxx X. Xxxxxx
Title:   Trustee
Address:
c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXXXX XXXXXXXX
By:		/s/ Xxxxxx Xxxxxxxx
Address:
0000 Xxxxxx Xxxx Xxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

SIGVION FUND I, LP
By:		/s/ X.X. Xxxxxxxx
		X.X. Xxxxxxxx
		Founding Partner
Address:
000 Xxxx Xxxxxxxxxx Xxxxxx, Xxxxx 000 Xxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXXXX XXXXXX HEALTHCARE VENTURE PARTNERS, L.P.
By:		Xxxxxx Xxxxxx Capital Management LLC
Title:		its Managing Member

By:		/s/ Xxxxxx Xxxxx
Name: Xxxxxx Xxxxx
Title:   Managing Director
Address:
00 Xxxxxx Xxxxxx, 0xx Xxxxx Xxx Xxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

TRIATHLON MEDICAL VENTURES FUND, L.P.
By:		Triathlon Medical Ventures, LLC
Its:		General Partner
By:		/s/ Xxxx X. Xxxx
		Xxxx X. Xxxx
		Managing Partner
Address:
000 X-Xxxxxxxx Xxx Xxxxx 000 Xxxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

TRI-STATE GROWTH CAPITAL FUND II, L.P.
By:		Tri-State Ventures II, LLC
Its:		General Partner
By:		Fort Washington Investment Advisors, Inc.
Its:		Managing Member
By:		/s/ Xxxxx Xxxxx
Name: Xxxxx Xxxxx
Title:   Managing Director
By:		/s/ Xxxxxxxx X. Xxxx
Name: Xxxxxxxx X. Xxxx
Title:   President and Chief Executive Officer
Address:
000 Xxxxxxxx, Xxxxx 0000 Xxxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

VENTURE INVESTORS EARLY STAGE FUND IV LIMITED PARTNERSHIP
By:		VIESF IV GP, LLC, its General Partner
By:		/s/ Xxxx X. Xxxxx
Name: Xxxx X. Xxxxx, PhD
Title:   Managing Director
Address:
000 Xxxxx Xxxx Xxxx Xxxxxxx, XX 00000-0000 Attn: Xxxx Xxxxx, Managing Director Phone: (000) 000-0000 Fax: (000) 000-0000 Email: xxxx@xxxxxxxxxxxxxxxx.xxx

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Voting Agreement as of the date first written above.

XXXX X. XXXXX

/s/ Xxxx X. Xxxxx

Xxxx X. Xxxxx

Address:

Blue Chip Venture Company

1120 Scripps Center

000 Xxxxxx Xxxxxx

Xxxxxxxxxx, XX 00000

SIGNATURE PAGE TO THIRD AMENDED AND RESTATED VOTING AGREEMENT

SCHEDULE A¹

INVESTORS

Name and Address		Number of Shares of Common Stock				Number of Shares of Series A Preferred Stock				Number of Shares of Series B Preferred Stock				Number of Shares of Series C Preferred Stock
Triathlon Medical Ventures Fund, L.P. Attn:    Xxxx X. Xxxx             Managing Partner 000 X-Xxxxxxxx Xxx, Xxxxx 000 Xxxxxxxxxx, XX 00000			20,000.00				128,095.46				124,501.72				118,397.71
Novartis BioVentures Ltd. Attn:    Xxxxx Xxxxx Zivi 000 Xxxxx Xxxxxx Xxxxxxxx XX 12 Bermuda But for mail, to: Novartis BioVentures Ltd. Attn:    Xxxxx Xxxxx Zivi PO Box HM 2899 Xxxxxxxx XX LX Bermuda And, also send a copy to: Novartis Venture Fund Attn:    Xxxxxxxx Xxxxxx Five Xxxxxxxxx Xxxxxx, Xxxxx 000 Xxxxxxxxx, XX 00000 and Xxxxxxx Xxxxxx Xxxxxx & Dodge LLP Attn:    Xx Xxxxx 000 Xxxxxxxxxx Xxxxxx Xxxxxx, XX 00000 xxxxxx@xxxxxxx.xxx			0				257,031.16				347,830.73				708,647.43

¹  The share amounts set forth in this Schedule A and on Schedule B reflect the effects of the 1-for-100 reverse stock split described in the Restated Certificate.

Schedule A-1

Name and Address		Number of Shares of Common Stock				Number of Shares of Series A Preferred Stock				Number of Shares of Series B Preferred Stock				Number of Shares of Series C Preferred Stock
Venture Investors Early Stage Fund IV Limited Partnership Attn:    Xxxx Xxxxx             Managing Director 000 Xxxxx Xxxx Xxxx Xxxxxxx, XX 00000-0000			0				153,434.52				173,915.36				201,811.43
Kearny Venture Partners, L.P. Attn:    Xxxxxx Xxxxx Kearny Venture Associates, LLC 00 Xxxxxx Xxxxxx, Xxxxx 000 Xxx Xxxxxxxxx, XX 00000-0000			0				44,606.15				43,354.71				308,448.29
Kearny Venture Partners Entrepreneurs Fund, L.P. Attn:    Xxxxxx Xxxxx Kearny Venture Associates, LLC 00 Xxxxxx Xxxxxx, Xxxxx 000 Xxx Xxxxxxxxx, XX 00000-0000			0				909.78				884.26				6,290.57
Xxxxxx Xxxxxx Healthcare Venture Partners, L.P. 00 Xxxxxx Xxxxxx, 0xx Xxxxx Xxx Xxxxxxxxx, XX 00000			0				45,515.99				44,239.03				74,475.86
The Procter & Xxxxxx Company Attn:    Xxxxx Xx Xxxxx             Director, Corporate             Acquisitions, Divestitures and             Equity Ventures 0 Xxxxxxx & Xxxxxx Xxxxx Xxxxxxxxxx, XX 00000			72,047.44				0				8,475.71				0
Athenian Venture Partners III L.P. Attn:    Xxxx X. Xxxxxxxx             President             Athenian III, Ltd. 000 Xxxx Xxxxx Xxxxxx Xxxx 00/Xxxxx 000X Xxxxxx, XX 00000			0				22,964.77				31,575.85				124,832.79

Schedule A-2

Name and Address		Number of Shares of Common Stock				Number of Shares of Series A Preferred Stock				Number of Shares of Series B Preferred Stock				Number of Shares of Series C Preferred Stock
AVP Ohio Technology I L.P. Attn:    Xxxx X. Xxxxxxxx             President             AVP Ohio I, Ltd. 000 Xxxx Xxxxx Xxxxxx Xxxx 00/Xxxxx 000X Xxxxxx, XX 00000			0				7,654.92				9,004.41				24,194.21
Sigvion Fund I, LP Attn:    X. X. Xxxxxxxx             Founding Partner 000 X. Xxxxxx Xxxxxx Xxxxxxx, XX 00000			8,000.00				13,660.69				13,277.43				21,402.57
Cincinnati Cornerstone Investors AKB, LLC Attn:    Xxxxxx X. Xxx, Xx.             President 00 Xxxx 0xx Xxxxxx, 0xx Xxxxx Xxxxxxxxxx, XX 00000-0000			0				13,122.75				12,754.59				118,391.79
Tri-State Growth Capital Fund II, L.P. Attn:    Xxxxx Xxxxx 000 Xxxxxxxx, Xxxxx 0000 Xxxxxxxxxx, XX 00000			0				12,804.65				12,445.41				21,488.36
Blue Chip Validation Fund, Ltd. Attn:    Xxxx XxXxxxxxxx             Managing Director 1100 Xxxxxxxx Center 000 Xxxx Xxxxx Xxxxxx Xxxxxxxxxx, XX 00000			0				3,402.09				0				0
QCA First Fund II Attn:    Xxxx Xxxxxxx 0000 Xxxxxx Xxxxxx, XX #000 Xxxxxxxxxx, XX 00000			0				3,375.00				0				0
Gitana Family Trust, Xxxxxxxxx X. Xxxxxxxx Trustee Attn:    Xxxxxxxxx X. Xxxxxxxx             Trustee 0000 Xxxxxx Xxxxx Xxxxxxxxxx, XX 00000			0				2,765.57				828.17				1,551.57

Schedule A-3

Name and Address		Number of Shares of Common Stock				Number of Shares of Series A Preferred Stock				Number of Shares of Series B Preferred Stock				Number of Shares of Series C Preferred Stock
Xxxxxx Xxxxxxxx 0000 Xxxxxx Xxxx Xxxxxx, XX 00000			127,637.41				849.53				2,070.42				2,500.00
Xxxx Xxxxxxxx Trust, Xxxxxx Xxxxxxxx, Trustee Attn:    Xxxxxx Xxxxxxxx             Trustee 0000 Xxxxxx Xxxx Xxxxxx, XX 00000			0				3,403.13				0				0
Xxxxxx X. Xxxxxxx 0000 Xxxxxx Xxxx Xxxxxxxxxx, XX 00000			161,423.79				9,038.87				11,594.35				26,383.21
Xxxxxxx Family Trust, Xxxx X. Xxxxxxx Trustee Attn:    Xxxx X. Xxxxxxx             Trustee 000 Xxxx Xxxxx Xxxxxx, XX 00000			0				6,901.99				7,288.07				2,485.00
Ian X. X. Xxxxx, XXX, Sterling Trust Custodian Attn:    Ian X. X. Xxxxx             Trustee 000 Xxxxxxxxx Xxxxx Xxxxxx Xxxx, XX 00000			0				5,000.00				5,797.18				7,142.00
Ian X. X. Xxxxx 000 Xxxxxxxxx Xxxxx Xxxxxx Xxxx, XX 00000			46,925.51				0				0				2,902.21
Xxxxx Xxxxxx 0000 Xxxxx Xxxx Xxxxxxxxxx, XX 00000			46,925.51				0				1,449.29				3,473.00
Xxxxxxx Xxxx 00 Xxx Xxxxxxx Xxx Xxxxxxxx, XX 00000			60,810.00				0				0				13,488.86

Schedule A-4

Name and Address		Number of Shares of Common Stock				Number of Shares of Series A Preferred Stock				Number of Shares of Series B Preferred Stock				Number of Shares of Series C Preferred Stock
Xxxxxx X. Xxxxxx Revocable Trust c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx			0				0				115,943.58				182,551.50
Xxxx Xxxx Trust c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx			0				0				0				3,571.00
The Xxxxxx Foundation c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx			0				0				57,971.78				191,275.86
Xxxxxx X Xxxxxx XXX, Millennium Trust Company, Custodian			0				0				0				14,285.00
Xxxxxx Children’s Trust I c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx			0				0				57,971.78				66,275.86
Xxxxxxx Xxxxxx Xxxxxx Revocable Trust c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx			0				0				0				7,142.00
Xxxx-Xxxxxx Xxxxxx Insurance Trust c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx			0				0				0				7,142.00

Schedule A-5

Name and Address		Number of Shares of Common Stock				Number of Shares of Series A Preferred Stock				Number of Shares of Series B Preferred Stock				Number of Shares of Series C Preferred Stock
Rose Xxxxxxx Xxxxx Insurance Trust c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx			0				0				0				7,142.00
Xxxx X. Xxxxxx Insurance Trust c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx			0				0				0				7,142.00
Xxxxx Xxxxxx Insurance Trust c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx			0				0				0				3,571.00
Xxxxxx and Xxxxxxx Xxxx Xxxxxx Insurance Trust c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx			0				0				0				3,571.00
Xxxxxx Family Trust c/x Xxxxxx Investment Management, LLC 000 X. Xxxxxxxx Xxxxxx, Xxxxx 0000 Xxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx			0				0				28,985.89				31,351.93
AgeChem Venture Fund L.P. Attn:    Xxxxx Xxxxxxx             President 0 Xxxxxxxxx Xxxxxx, Xxxxx 000 Xxxxxxxx, Xxxxxx, Xxxxxx X0X 0X0			0				0				173,915.35				123,143.86

Schedule A-6

Name and Address		Number of Shares of Common Stock				Number of Shares of Series A Preferred Stock				Number of Shares of Series B Preferred Stock				Number of Shares of Series C Preferred Stock
Xxxxx X. Xxxxxx Trust Attn:    Xxxx Xxxxxx             Trustee 0000 Xxxxxx Xxxxxx Xxxxx Xxxx Xxxxxxx, XX 00000			0				0				1,449.29				139.00
Xxxx Xxxxxx 0000 Xxxxxx Xxxxxx Xxxxx Xxxx Xxxxxxx, XX 00000			0				0				0				1,670.79
MRK International, LLC Attn:    Xxxxxxx X. Xxxxx             Principal Member 0000 Xxxxxxxx Xxxxx Xxxxxxxxxx, XX 00000			0				0				0				396.00
McIlwraith Investments, LLC Attn:    Xxxx XxXxxxxxxx             Manager 0000 Xxxxxxx Xxxx Xxxxxxxxxx, XX 00000			0				0				0				4,597.00
Xxxx X. Xxxxx Blue Chip Venture Company 1120 Scripps Center 000 Xxxxxx Xxxxxx Xxxxxxxxxx, XX 00000			0				0				0				1,039.36
Xxxx Xxxxxxx 0000 Xxxxxxxxxx Xxxxx Xxxxxxxxxx, XX 00000-0000			0				0				0				1,428.00
Family Akebia Investments LLC Attn:    Xxxxxx Xxxxxxxxx 0000 Xxxx Xxxxxx #00X Xxx Xxxx, XX 00000			0				0				0				142,858.00
Xxxx X/X Xxxx Xxxxxxxx Xxxxxx Xxxxxxxx 00 DK - 2900 - Hellerup			0				0				0				714,285.00

Schedule A-7

SCHEDULE B

KEY HOLDERS Name and Address		Number of Shares Held
The Procter & Xxxxxx Company Xxx Xxxxxxx & Xxxxxx Xxxxx Xxxxxxxxxx, XX 00000		7,204,744 shares of Common Stock

Schedule B-1

EXHIBIT A

ADOPTION AGREEMENT

This Adoption Agreement (“Adoption Agreement”) is executed on             , 20             , by the undersigned (the “Holder”) pursuant to the terms of that certain Voting Agreement dated as of May [__], 2013 (the “Agreement”), by and among the Company and certain of its Investors, as such Agreement may be amended or amended and restated hereafter. Capitalized terms used but not defined in this Adoption Agreement shall have the respective meanings ascribed to such terms in the Agreement. By the execution of this Adoption Agreement, the Holder agrees as follows.

1.1 Acknowledgement. Holder acknowledges that Holder is acquiring certain shares of the capital stock of the Company (the “Stock”)[ or options, warrants or other rights to purchase such Stock (the “Options”)], for one of the following reasons (Check the correct box):

¨ as a transferee of Shares from a party in such party’s capacity as an “Investor” bound by the Agreement, and after such transfer, Holder shall be considered an “Investor” and a “Stockholder” for all purposes of the Agreement.

¨ as a new Investor in accordance with Section 6.1 of the Agreement, in which case Holder will be an “Investor” and a “Stockholder” for all purposes of the Agreement.

1.2 Agreement. Holder hereby (a) agrees that the Stock [Options], and any other shares of capital stock or securities required by the Agreement to be bound thereby, shall be bound by and subject to the terms of the Agreement and (b) adopts the Agreement with the same force and effect as if Holder were originally a party thereto.

1.3 Notice. Any notice required or permitted by the Agreement shall be given to Holder at the address or facsimile number listed below Holder’s signature hereto.

HOLDER:						ACCEPTED AND AGREED:
By:						AKEBIA THERAPEUTICS, INC.
Name and Title of Signatory
Address:						By:
						Title:
Facsimile Number:

Exhibit A-1