EX-4.2



 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 Mr. Nash 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 Satter Investment Management, LLC, which individual shall initially be
Muneer A. Satter. 
 (b) As Preferred Directors, two persons designated by Novartis Bioventures Ltd., which individuals shall
initially be Campbell Murray and Giovanni Ferrara. 
 (c) As a Preferred Director, one person designated by Kearny Venture
Partners, which individual shall initially be Anupam Dalal. 
 (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 Duane Nash; provided that the Board seat reserved for Mr. Nash shall not be filled at any time after the date hereof on which a majority of the Board or
Mr. Nash determines that Mr. Nash 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 Mr. Nash from the Board upon any such occurrence.

 (f) As a Common Director, the Company’s Chief Executive Officer, who shall initially be Joseph Gardner (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 Satter Investment Management, LLC, Muneer A. Satter and all Persons for which Mr. Satter 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 Mr. Nash 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: 

Thompson Hine LLP 
 312 Walnut
Street, 14th Floor 
 Cincinnati, OH 45202-4089 

Attn: David J. Willbrand 

david.willbrand@thompsonhine.com 

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

Kirkland & Ellis LLP 

300 N. LaSalle St. 
 Chicago, IL
60654 

	 	Attn:	  Ted H. Zook, P.C. 


	 	    	  Roger D. Rhoten 

 E-mail: ted.zook@kirkland.com 

roger.rhoten@kirkland.com 

  
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 and 

Edwards Wildman Palmer LLP 

Attn: Albert L. Sokol 
 111
Huntington Avenue 
 Boston, MA 02199 USA 

asokol@edwardswildman.com 
 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
Satter 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 Satter 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 Satter 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 Duane Nash’s 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 Mr. Nash) 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/ Joseph H. Gardner
		 	Joseph H. Gardner, Ph.D.
		 	President and Chief Executive Officer
	  
 Address:

 
 Suite 420,

9987 Carver Road,
 Cincinnati, OH 45242




  
 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/ Louis Lacasse
		 	Name: Louis Lacasse
		 	Title: President
	
	 Address:
  

Attn: Louis Lacasse, President
 1 Westmount Square, Suite 800

Montreal, Quebec, Canada
 H3Z 2P9




  
 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/ Karl O. Elderkin
		 	Name: Karl O. Elderkin
		 	Title: President


  

			
	 AVP OHIO TECHNOLOGY I L.P.
  

By:   AVP Ohio I, Ltd.

Its:   General Partner

		
	By:	 	/s/ Karl Elderkin
		 	Name: Karl O. Elderkin
		 	Title: President
	
	 Address:
  

340 West State Street
 Unit 29/Suite 137D

Athens OH 45701




  
 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/ John McIlwraith
		 	John McIlwraith
		 	Managing Director
	  
 Address:

 
 312 Walnut Street

Suite 1120
 Cincinnati, OH 45202




  
 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/ Robert W. Coy, Jr.
		 	Robert W. Coy, Jr.
		 	President
	  
 Address:

 
 30 West 3rd Street, 6th Floor
 Cincinnati, OH 45202-3559




  
 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. 
  

			
	WILLIAM DALY
		
		 	/s/ William Daly
		 	William Daly
	  
 Address:

 
 13 Via Abrazar

San Clemente, CA 92673




  
 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. 
  

			
	DIANE H. JANUSZ TRUST
		
	By:	 	/s/ John Janusz
		 	Name: John Janusz
		 	Title: Trustee
	
	JOHN JANUSZ
		
		 	/s/ John Janusz
		 	John Janusz
	  
 Address:

 
 7385 Desert Spring Court

West Chester, OH 45069




  
 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/ Milton Berlinski
		 	Name: Milton Berlinski
		 	Title: Managing Member
	  
 Address:

 
 1185 Park Avenue #11G

New York, NY 10128




  
 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. 
  

			
	ALAN FISHMAN
		
		 	/s/ Alan Fishman
		 	Alan Fishman
	  
 Address:

 
 6900 Stonehenge Dr.

Cincinnati, OH 45242-6204




  
 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. 
  

			
	 FRED SHALWITZ TRUST,
 ROBERT
SHALWITZ, TRUSTEE

		
	By:	 	/s/ Robert Shalwitz
		 	Name: Robert Shalwitz
		 	Title: Trustee
	  
 Address:

 
 2549 Bryden Road

Bexley, OH 43209




  
 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 
  

			
	 GARDNER FAMILY TRUST, JOHN D.

GARDNER TRUSTEE

		
	By:	 	/s/ John D. Gardner
		 	 John D. Gardner
 Trustee

	  
 Address:

 
 111 Pine Court

Bastop, TX 78602




  
 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,
 ELIZABETH C.
ARMITAGE TRUSTEE

		
	By:	 	/s/ Elizabeth C. Armitage
		 	 Elizabeth C. Armitage
 Trustee

	  
 Address:

 
 2207 Upland Place

Cincinnati, OH 45206




  
 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. 
  

			
	JOSEPH H. GARDNER
		
	By:	 	/s/ Joseph H. Gardner
	  
 Address:

 
 4060 Boomer Road

Cincinnati, OH 45247




  
 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 A. W. HOWES, IRA, STERLING TRUST CUSTODIAN
		
	By:	 	/s/ Ian A. W. Howes
	Name: Ian A. W. Howes
	Title: Trustee
	
	IAN A. W. HOWES
	
	 /s/ Ian A. W. Howes

	Ian A. W. Howes
	  
 Address:

 
 219 Stratford Drive

Chapel Hill, NC 27516




  
 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/ Anupam Dalal
		 	Name: Kearny Venture Associates, LLC
		 	Title: its General Partner
	  
 Address:

 
 Attn:Anupam Dalal

Kearny Venture Associates LLC

88 Kearny Street, Suite 200

San Francisco, CA 94108-5530



  

			
	KEARNY VENTURE PARTNERS ENTREPRENEURS FUND, L.P.
		
	By:	 	/s/ Anupam Dalal
	Name: Kearny Venture Associates, LLC
	Title: its General Partner
	  
 Address:

 
 Attn:Anupam Dalal

Kearny Venture Associates LLC
 88 Kearny Street, Suite 200

San Francisco, CA 94108-5530




  
 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/ John McIlwraith
		 	Name: John McIlwraith
		 	Title: Manager
	  
 Address:

 
 Attn: John McIlwraith

7680 Foxgate Lane
 Cincinnati, OH 45243




  
 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/ Richard L. Kiley
	Name: Richard L. Kiley
	Title: Principal Member
	  
 Address:

 
 7800 Tecumseh Trail

Cincinnati, OH 45243




  
 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/ H.S. Zivi
	 Name: H. S. Zivi
 Title:
Deputy Chairman


		
	By:	 	/s/ Rebecca White
	 Name: Rebecca White

Title: Authorized Signatory


	  
 Address:

 
 NOVARTIS BIOVENTURES LTD.

Attn: Henri Simon Zivi
 131 Front Street

Hamilton HM 12
 Bermuda

 
 But for mail, to: 

 
 Novartis BioVentures Ltd.

Attn: Henri Simon Zivi
 PO Box HM 2899

Hamilton HM LX Bermuda
  

And, also send a copy to: 
  

Novartis Venture Fund
 Attn: Campbell Murray

Five Cambridge Center, Suite 603
 Cambridge, MA
02142




  
 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. 
  

			
	KEVIN PETERS
		
		 	/s/ Kevin Peters
		 	Kevin Peters
	  
 Address:

 
 9160 Given Road

Cincinnati, OH 45243




  
 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/ John Habbert
	 Name: John Habbert

Title: Manager


	  
 Address:

 
 109 Bentwood Ct.

Cincinnati, OH 45241




  
 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. 
  

			
	MUNEER A. SATTER REVOCABLE TRUST
		
	By:	 	/s/ Muneer A. Satter
	Name: Muneer A. Satter
	Title: Trustee


  

			
	THE SATTER FOUNDATION
		
	By:	 	/s/ Muneer A. Satter
	Name: Muneer A. Satter
	Title: Trustee


  

			
	SATTER FAMILY TRUST
		
	By:	 	/s/ Muneer A. Satter
	Name: Muneer A. Satter
	Title: Investment Advisor


  

			
	SATTER CHILDREN’S TRUST I
		
	By:	 	/s/ Muneer A. Satter
	Name: Muneer A. Satter
	Title: Investment Advisor
	  
 Address:

 
 c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000
 Chicago, IL 60611

Attn: Muneer A. Satter




  
 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. 
  

			
	KRISTEN HAYLER HERTEL REVOCABLE TRUST
		
	By:	 	 /s/ Kristen Hayler Hertel

	Name: Kristen Hayler Hertel
	Title:   Trustee
	
	Address:
	
	 c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000
 Chicago, IL 60611

Attn: Muneer A. Satter




  
 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. 
  

			
	ANNE-CAROLE WITORT INSURANCE TRUST
		
	By:	 	 /s/ Muneer A. Satter

	Name: Muneer A. Satter
	Title:   Trustee
	
	ROSE SHEREEN FUQUA INSURANCE TRUST
		
	By:	 	 /s/ Muneer A. Satter

	Name: Muneer A. Satter
	Title:   Investment Advisor
	
	RABI H. SATTER INSURANCE TRUST
		
	By:	 	 /s/ Muneer A. Satter

	Name: Muneer A. Satter
	Title:   Investment Advisor
	
	JOHN WOOD TRUST
		
	By:	 	 /s/ Muneer A. Satter

	Name: Muneer A. Satter
	Title:   Investment Advisor
	
	Address:
	
	 c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000
 Chicago, IL 60611

Attn: Muneer A. Satter




  
 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. 
  

			
	ABDUS SATTER INSURANCE TRUST
		
	By:	 	 /s/ Muneer A. Satter

	Name: Muneer A. Satter
	Title:   Trustee
	
	GORDON AND BARBARA ANNE HERTEL INSURANCE TRUST
		
	By:	 	 /s/ Muneer A. Satter

	Name: Muneer A. Satter
	Title:   Trustee
	
	Address:
	
	 c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000
 Chicago, IL 60611

Attn: Muneer A. Satter




  
 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. 
  

			
	ROBERT SHALWITZ
		
	By:	 	 /s/ Robert Shalwitz

	
	Address:
	
	 2549 Bryden Road
 Bexley, OH
43209




  
 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/ J.P. Fairbank

		 	    J.P. Fairbank
		 	    Founding Partner
	
	Address:
	
	 806 West Washington Street, Suite 204

Chicago, IL 60607




  
 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. 
  

			
	THOMAS WEISEL HEALTHCARE VENTURE PARTNERS, L.P.
		
	By:	 	Thomas Weisel Capital Management LLC
	Title:	 	its Managing Member


 
			
		
	By:	 	 /s/ Anupam Dalal

	Name: Anupam Dalal
	Title:   Managing Director
	
	Address:
	
	 88 Kearny Street, 4th Floor

San Francisco, CA 94108




  
 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/ John M. Rice

		 	John M. Rice
		 	Managing Partner
	
	Address:
	
	 300 E-Business Way
 Suite 200

Cincinnati, OH 45241




  
 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/ Steve Baker

	Name: Steve Baker
	Title:   Managing Director
		
	By:	 	 /s/ Maribeth S. Rahe

	Name: Maribeth S. Rahe
	Title:   President and Chief Executive Officer
	
	Address:
	
	 303 Broadway, Suite 1200

Cincinnati, OH 45202




  
 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/ Paul M. Weiss

	Name: Paul M. Weiss, PhD
	Title:   Managing Director
	
	Address:
	
	 505 South Rosa Road
 Madison, WI
53719-1262
 Attn: Paul Weiss, Managing Director
 Phone:
(608) 441-2700
 Fax: (608) 441-2727
 Email:
paul@ventureinvestors.com




  
 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. 
  

			
	JOHN H. WYANT
	
	 /s/ John H. Wyant

	John H. Wyant
	
	Address:
	
	 Blue Chip Venture Company
 1120
Scripps Center
 312 Walnut Street
 Cincinnati, OH
45202




  
 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:    John M. Rice

            Managing Partner

300 E-Business Way, Suite 200

Cincinnati, OH 45241
	  	 	20,000.00	  	  	 	128,095.46	  	  	 	124,501.72	  	  	 	118,397.71	  
					
	 Novartis BioVentures Ltd.

Attn:    Henri Simon Zivi

131 Front Street

Hamilton HM 12

Bermuda
  

But for mail, to: 

Novartis BioVentures Ltd.

Attn:    Henri Simon Zivi

PO Box HM 2899

Hamilton HM LX Bermuda
  

And, also send a copy to: 

Novartis Venture Fund

Attn:    Campbell Murray

Five Cambridge Center, Suite 603

Cambridge, MA 02142
  

and
  

Edwards Angell Palmer & Dodge LLP

Attn:    Al Sokol

111 Huntington Avenue

Boston, MA 02199

asokol@eapdlaw.com
	  	 	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:    Paul Weiss

            Managing Director

505 South Rosa Road

Madison, WI 53719-1262
	  	 	0	  	  	 	153,434.52	  	  	 	173,915.36	  	  	 	201,811.43	  
					
	 Kearny Venture Partners, L.P.

Attn:    Anupam Dalal

Kearny Venture Associates, LLC

88 Kearny Street, Suite 200

San Francisco, CA 94108-5530
	  	 	0	  	  	 	44,606.15	  	  	 	43,354.71	  	  	 	308,448.29	  
					
	 Kearny Venture Partners Entrepreneurs

Fund, L.P.

Attn:    Anupam Dalal

Kearny Venture Associates, LLC

88 Kearny Street, Suite 200

San Francisco, CA 94108-5530
	  	 	0	  	  	 	909.78	  	  	 	884.26	  	  	 	6,290.57	  
					
	 Thomas Weisel Healthcare Venture

Partners, L.P.

88 Kearny Street, 4th Floor

San Francisco, CA 94108
	  	 	0	  	  	 	45,515.99	  	  	 	44,239.03	  	  	 	74,475.86	  
					
	 The Procter & Gamble Company

Attn:    David Le Neveu

            Director, Corporate

            Acquisitions, Divestitures and

            Equity Ventures

1 Procter & Gamble Plaza

Cincinnati, OH 45202
	  	 	72,047.44	  	  	 	0	  	  	 	8,475.71	  	  	 	0	  
					
	 Athenian Venture Partners III L.P.

Attn:    Karl O. Elderkin

            President

            Athenian III, Ltd.

340 West State Street

Unit 29/Suite 137D

Athens, OH 45701
	  	 	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:    Karl O. Elderkin

            President

            AVP Ohio I, Ltd.

340 West State Street

Unit 29/Suite 137D

Athens, OH 45701
	  	 	0	  	  	 	7,654.92	  	  	 	9,004.41	  	  	 	24,194.21	  
					
	 Sigvion Fund I, LP

Attn:    J. P. Fairbank

            Founding Partner

738 W. Belden Avenue

Chicago, IL 60614
	  	 	8,000.00	  	  	 	13,660.69	  	  	 	13,277.43	  	  	 	21,402.57	  
					
	 Cincinnati Cornerstone Investors AKB, LLC

Attn:    Robert W. Coy, Jr.

            President

30 West 3rd Street,
6th Floor
 Cincinnati, OH 45202-3559
	  	 	0	  	  	 	13,122.75	  	  	 	12,754.59	  	  	 	118,391.79	  
					
	 Tri-State Growth Capital Fund II, L.P.

Attn:    Steve Baker

303 Broadway, Suite 1200

Cincinnati, OH 45202
	  	 	0	  	  	 	12,804.65	  	  	 	12,445.41	  	  	 	21,488.36	  
					
	 Blue Chip Validation Fund, Ltd.

Attn:    John McIlwraith

            Managing Director

1100 Chiquita Center

250 East Fifth Street

Cincinnati, OH 45202
	  	 	0	  	  	 	3,402.09	  	  	 	0	  	  	 	0	  
					
	 QCA First Fund II

Attn:    John Habbert

1776 Mentor Avenue, MB #302

Cincinnati, OH 45212
	  	 	0	  	  	 	3,375.00	  	  	 	0	  	  	 	0	  
					
	 Gitana Family Trust, Elizabeth C.

Armitage Trustee

Attn:    Elizabeth C. Armitage

            Trustee

2207 Upland Place

Cincinnati, OH 45206
	  	 	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	 
	 Robert Shalwitz

2549 Bryden Road

Bexley, OH 43209
	  	 	127,637.41	  	  	 	849.53	  	  	 	2,070.42	  	  	 	2,500.00	  
					
	 Fred Shalwitz Trust, Robert Shalwitz,

Trustee

Attn:    Robert Shalwitz

            Trustee

2549 Bryden Road

Bexley, OH 43209
	  	 	0	  	  	 	3,403.13	  	  	 	0	  	  	 	0	  
					
	 Joseph H. Gardner

4060 Boomer Road

Cincinnati, OH 45247
	  	 	161,423.79	  	  	 	9,038.87	  	  	 	11,594.35	  	  	 	26,383.21	  
					
	 Gardner Family Trust, John D. Gardner

Trustee

Attn:    John D. Gardner

            Trustee

111 Pine Court

Bastop, TX 78602
	  	 	0	  	  	 	6,901.99	  	  	 	7,288.07	  	  	 	2,485.00	  
					
	 Ian A. W. Howes, IRA, Sterling Trust

Custodian

Attn:    Ian A. W. Howes

            Trustee

219 Stratford Drive

Chapel Hill, NC 27516
	  	 	0	  	  	 	5,000.00	  	  	 	5,797.18	  	  	 	7,142.00	  
					
	 Ian A. W. Howes

219 Stratford Drive

Chapel Hill, NC 27516
	  	 	46,925.51	  	  	 	0	  	  	 	0	  	  	 	2,902.21	  
					
	 Kevin Peters

9160 Given Road

Cincinnati, OH 45243
	  	 	46,925.51	  	  	 	0	  	  	 	1,449.29	  	  	 	3,473.00	  
					
	 William Daly

13 Via Abrazar

San Clemente, CA 92673
	  	 	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	 
	 Muneer A. Satter Revocable Trust

c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000

Chicago, IL 60611

Attn: Muneer A. Satter
	  	 	0	  	  	 	0	  	  	 	115,943.58	  	  	 	182,551.50	  
					
	 John Wood Trust

c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000

Chicago, IL 60611

Attn: Muneer A. Satter
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	3,571.00	  
					
	 The Satter Foundation

c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000

Chicago, IL 60611

Attn: Muneer A. Satter
	  	 	0	  	  	 	0	  	  	 	57,971.78	  	  	 	191,275.86	  
					
	 Muneer A Satter IRA, Millennium Trust

Company, Custodian
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	14,285.00	  
					
	 Satter Children’s Trust I

c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000

Chicago, IL 60611

Attn: Muneer A. Satter
	  	 	0	  	  	 	0	  	  	 	57,971.78	  	  	 	66,275.86	  
					
	 Kristen Hayler Hertel Revocable Trust

c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000

Chicago, IL 60611

Attn: Muneer A. Satter
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	7,142.00	  
					
	 Anne-Carole Witort Insurance Trust

c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000

Chicago, IL 60611

Attn: Muneer A. Satter
	  	 	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 Shereen Fuqua Insurance Trust

c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000

Chicago, IL 60611

Attn: Muneer A. Satter
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	7,142.00	  
					
	 Rabi H. Satter Insurance Trust

c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000

Chicago, IL 60611

Attn: Muneer A. Satter
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	7,142.00	  
					
	 Abdus Satter Insurance Trust

c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000

Chicago, IL 60611

Attn: Muneer A. Satter
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	3,571.00	  
					
	 Gordon and Barbara Anne Hertel

Insurance Trust

c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000

Chicago, IL 60611

Attn: Muneer A. Satter
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	3,571.00	  
					
	 Satter Family Trust

c/o Satter Investment Management, LLC

676 N. Michigan Avenue, Suite 4000

Chicago, IL 60611

Attn: Muneer A. Satter
	  	 	0	  	  	 	0	  	  	 	28,985.89	  	  	 	31,351.93	  
					
	 AgeChem Venture Fund L.P.

Attn:    Louis Lacasse

            President

1 Westmount Square, Suite 800

Montreal, Quebec, Canada

H3Z 2P9
	  	 	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	 
	 Diane H. Janusz Trust

Attn:    John Janusz

            Trustee

7385 Desert Spring Court

West Chester, OH 45069
	  	 	0	  	  	 	0	  	  	 	1,449.29	  	  	 	139.00	  
					
	 John Janusz

7385 Desert Spring Court

West Chester, OH 45069
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	1,670.79	  
					
	 MRK International, LLC

Attn:    Richard L. Kiley

            Principal Member

7800 Tecumseh Trail

Cincinnati, OH 45243
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	396.00	  
					
	 McIlwraith Investments, LLC

Attn:    John McIlwraith

            Manager

7680 Foxgate Lane

Cincinnati, OH 45243
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	4,597.00	  
					
	 John H. Wyant

Blue Chip Venture Company

1120 Scripps Center

312 Walnut Street

Cincinnati, OH 45202
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	1,039.36	  
					
	 Alan Fishman

6900 Stonehenge Drive

Cincinnati, OH 45242-6204
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	1,428.00	  
					
	 Family Akebia Investments LLC

Attn:    Milton Berlinski

1185 Park Avenue #11G

New York, NY 10128
	  	 	0	  	  	 	0	  	  	 	0	  	  	 	142,858.00	  
					
	 Novo A/S

Novo Ventures

Tuborg Havnevej 19

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 & Gamble Company

One Procter & Gamble Plaza

Cincinnati, OH 45202
	  	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