EX-4.4



 Exhibit 4.4 

EXECUTION VERSION 
 THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 







 TABLE OF CONTENTS 

 

					
	 	  	Page	 
	 1.      Definitions
	  	 	2	  
		
	 2.      Registration Rights
	  	 	5	  
	 2.1.     Demand Registration
	  	 	5	  
	 2.2.     Company Registration
	  	 	6	  
	 2.3.     Underwriting Requirements
	  	 	7	  
	 2.4.     Obligations of the Company
	  	 	8	  
	 2.5.     Furnish Information
	  	 	9	  
	 2.6.     Expenses of Registration
	  	 	9	  
	 2.7.     Delay of Registration
	  	 	10	  
	 2.8.     Indemnification
	  	 	10	  
	 2.9.     Reports Under Exchange Act
	  	 	12	  
	 2.10.   Limitations on Subsequent Registration Rights
	  	 	13	  
	 2.11.   “Market Stand-off” Agreement
	  	 	13	  
	 2.12.   Restrictions on Transfer
	  	 	14	  
	 2.13.   Termination of Registration Rights
	  	 	15	  
		
	 3.      Information and Observer Rights
	  	 	15	  
	 3.1.     Delivery of Financial Statements
	  	 	15	  
	 3.2.     Inspection
	  	 	17	  
	 3.3.     Observer Rights
	  	 	17	  
	 3.4.     Termination of Information and Observer Rights
	  	 	17	  
	 3.5.     Confidentiality
	  	 	18	  
		
	 4.      Rights to Future Stock Issuances
	  	 	18	  
	 4.1.     Right of First Offer
	  	 	18	  
	 4.2.     Termination
	  	 	19	  
		
	 5.      Additional Covenants
	  	 	19	  
	 5.1.     Insurance
	  	 	19	  
	 5.2.     Employee Agreements
	  	 	20	  
	 5.3.     Qualified Small Business Stock
	  	 	20	  
	 5.4.     Matters Requiring Investor Approval
	  	 	20	  
	 5.5.     Board Matters
	  	 	21	  


  
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	 5.6.     Successor Indemnification
	  	 	21	  
	 5.7.     Termination of Covenants
	  	 	22	  
	 5.8.     Limitation of Liability
	  	 	22	  
		
	 6.      Miscellaneous
	  	 	22	  
	 6.1.     Successors and Assigns
	  	 	22	  
	 6.2.     Governing Law
	  	 	23	  
	 6.3.     Counterparts; Facsimile or PDF
	  	 	23	  
	 6.4.     Titles and Subtitles
	  	 	23	  
	 6.5.     Notices
	  	 	23	  
	 6.6.     Amendments and Waivers
	  	 	24	  
	 6.7.     Severability
	  	 	24	  
	 6.8.     Aggregation of Stock
	  	 	24	  
	 6.9.     Additional Investors
	  	 	25	  
	 6.10.   Entire Agreement
	  	 	25	  
	 6.11.   [Reserved]
	  	 	25	  
	 6.12.   Delays or Omissions
	  	 	25	  
	 6.13.   Acknowledgment
	  	 	25	  

 Schedule A - Schedule of Investors 

  
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 Exhibit 4.4 

THIRD AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT 

THIS THIRD AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT (this “Agreement”) is made as of the 10th day of May, 2013 by and
among AKEBIA THERAPEUTICS, INC., a Delaware corporation (the “Company”), each of the investors listed on Schedule A hereto, each of which is referred to in this Agreement as an “Investor” and collectively, the
“Investors”), and any Additional Investor that becomes a party to this Agreement in accordance with Section 6.9 hereof. 

RECITALS 
 WHEREAS,
certain of the Investors have previously purchased equity securities of the Company; 
 WHEREAS, the Company and certain Investors are
parties to the “Series A Preferred Stock Purchase Agreements” dated as of January 23, 2008 and July 15, 2009; 

WHEREAS, the Company and certain Investors are parties to the Series B Preferred Stock Purchase Agreement, dated as of April 6, 2011, by
and among the Company and certain of the Investors (as it may be amended, restated, supplemented or otherwise modified from time to time, the “Series B Purchase Agreement”); 

WHEREAS, the Company and certain Investors are parties to the Series C Preferred Stock Purchase Agreement, dated as of the date hereof, by and
among the Company and certain of the Investors (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 and certain other Persons (as defined below) previously entered into an Investors’ Rights
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 its existing Investors desire to induce the Investors to purchase shares of Series C Preferred Stock of the Company,
pursuant to the Series C Preferred Stock Purchase Agreement by amending and restating the Prior Agreement to provide the Investors with the rights and privileges as set forth herein; and 

WHEREAS, in order to induce the Company to enter into the Series C Purchase Agreement and to induce the Investors to invest funds in the
Company pursuant to the Series C Purchase Agreement, the Investors and the Company hereby agree that this Agreement shall govern the rights of the Investors to cause the Company to register shares of Common Stock issuable to the Investors, to
receive certain information from the Company, and to participate in future equity offerings by the Company, and shall govern certain other matters as set forth in this Agreement; 







 NOW, THEREFORE, the parties hereby agree as follows: 

1. Definitions. For purposes of this Agreement: 

“Affiliate” means, with respect to any specified Person, any other Person who, directly or indirectly, controls, is controlled
by, or is under common control with such Person, including without limitation any general partner, managing member, officer or director of such Person or any venture capital fund now or hereafter existing that is controlled by 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. 
 “Appropriate Percentage” means fifty percent (50%). 

“Common Stock” means shares of the Company’s common stock, par value $0.00001 per share. 

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

“Derivative Securities” means any securities or rights convertible into, or exercisable or exchangeable for (in each case,
directly or indirectly), Common Stock, including options and warrants. 
 “Exchange Act” means the Securities Exchange Act
of 1934, as amended, and the rules and regulations promulgated thereunder. 
 “Excluded Registration” means (i) a
registration relating to the sale of securities to employees of the Company or a subsidiary pursuant to a stock option, stock purchase, or similar plan; (ii) a registration relating to an SEC Rule 145 transaction; (iii) a registration on
any form that does not include substantially the same information as would be required to be included in a registration statement covering the sale of the Registrable Securities; or (iv) a registration in which the only Common Stock being
registered is Common Stock issuable upon conversion of debt securities that are also being registered. 
 “Form S-1” means
such form under the Securities Act as in effect on the date hereof or any successor registration form under the Securities Act subsequently adopted by the SEC. 

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

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

“Holder” means any holder of Registrable Securities who is a party to this Agreement. 

“Immediate Family Member” means a child, stepchild, grandchild, parent, stepparent, grandparent, spouse, sibling,
mother-in-law, father-in-law, son-in-law, daughter-in- law, brother-in-law, or sister-in-law, including adoptive relationships, of a natural person referred to herein. 

“Initiating Holders” means, collectively, Holders who properly initiate a registration request under this Agreement. 

“Investor” means the persons named on Schedule A hereto, each person who hereafter becomes a signatory to this
Agreement pursuant to Section 6.9 and any one of them, as the context may require; provided, however, that any such person shall cease to be considered an Investor for purposes of this Agreement at any time such person and
his, her or its Affiliates collectively hold no shares of Preferred Stock. 
 “IPO” means the Company’s first
underwritten public offering of its Common Stock under the Securities Act. 
 “Key Employee” means any executive-level
employee (including division director and vice president-level positions) as well as any employee who, either alone or in concert with others, develops, invents, programs, or designs any Company Intellectual Property (as defined in the Series C
Purchase Agreement). 
 “Major Investor” means any Investor that, individually or together with such Investor’s
Affiliates, holds at least 27,500 shares of Registrable Securities (as adjusted for any stock split, stock dividend, combination, or other recapitalization or reclassification effected after the date hereof). 

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

“P&G License Agreement” means that certain License Agreement dated as of September 4, 2007 between the Company and
The Procter & Gamble Company. 
 “Person” means any individual, corporation, partnership, trust, limited liability
company, association, foundation or other entity. 

  
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 “Preferred Director” means any director of the Company that the holders of
record of the Preferred Stock are entitled to elect pursuant to the Company’s Certificate of Incorporation. 
 “Preferred
Stock” means shares of the Company’s Series A Preferred Stock, Series B Preferred Stock and Series C Preferred Stock. 

“Prior Agreement” has the meaning set forth in the Recitals to this Agreement. 

“Registrable Securities” means (i) the Common Stock issuable or issued upon conversion of the Preferred Stock;
(ii) any Common Stock issued or issuable (directly or indirectly) upon conversion and/or exercise of any other securities of the Company, acquired by the Investors after the date hereof; and (iii) any Common Stock issued as (or issuable
upon the conversion or exercise of any warrant, right, or other security that is issued as) a dividend or other distribution with respect to, or in exchange for or in replacement of, the shares referenced in clauses (i) and (ii) above;
excluding in all cases, however, any Registrable Securities sold by a Person in a transaction in which the applicable rights under this Agreement are not assigned pursuant to Section 6.1, and excluding for purposes of
Section 2 any shares for which registration rights have terminated pursuant to Section 2.13 of this Agreement. 

“Registrable Securities then outstanding” means the number of shares determined by adding the number of shares of outstanding
Common Stock that are Registrable Securities and the number of shares of Common Stock issuable (directly or indirectly) pursuant to then exercisable and/or convertible securities that are Registrable Securities. 

“Restricted Securities” means the securities of the Company required to bear the legend set forth in
Section 2.12(b) hereof. 
 “SEC” means the Securities and Exchange Commission. 

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

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

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

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

“Series A Preferred Stock” means shares of the Company’s Series A Preferred Stock, par value $0.00001 per share. 

  
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 “Series B Preferred Stock” means shares of the Company’s Series B Preferred
Stock, par value $0.00001 per share. 
 “Series C Preferred Stock” means shares of the Company’s Series C Preferred
Stock, par value $0.00001 per share. 
 “Series C Purchase Agreement” has the meaning set forth in the Recitals to this
Agreement. 
 2. Registration Rights. The Company covenants and agrees as follows: 

2.1. Demand Registration. 

(a) Form S-1 Demand. If at any time after the earlier of (i) five (5) years after the date hereof or (ii) one hundred
eighty (180) days after the effective date of the registration statement for the IPO the Company receives a request from Holders of thirty percent (30%) of the Registrable Securities then outstanding that the Company file a Form S-1
registration statement with respect to Registrable Securities then outstanding, then the Company shall (i) within ten (10) days after the date such request is given, give notice thereof (the “Demand Notice”) to all Holders
other than the Initiating Holders; and (ii) as soon as practicable, and in any event within sixty (60) days after the date such request is given by the Initiating Holders, file a Form S-1 registration statement under the Securities Act
covering all Registrable Securities that the Initiating Holders requested to be registered and any additional Registrable Securities requested to be included in such registration by any other Holders, as specified by notice given by each such Holder
to the Company within ten (10) days of the date the Demand Notice is given, and in each case, subject to the limitations of Sections 2.1(c) and 2.3. 

(b) Form S-3 Demand. If at any time when it is eligible to use a Form S-3 registration statement, the Company receives a request from
Holders of at least thirty percent (30%) of the Registrable Securities then outstanding that the Company file a Form S-3 registration statement with respect to outstanding Registrable Securities of such Holders, then the Company shall
(i) within ten (10) days after the date such request is given, give a Demand Notice to all Holders other than the Initiating Holders; and (ii) as soon as practicable, and in any event within forty-five (45) days after the date
such request is given by the Initiating Holders, file a Form S-3 registration statement under the Securities Act covering all Registrable Securities requested to be included in such registration by any other Holders, as specified by notice given by
each such Holder to the Company within ten (10) days of the date the Demand Notice is given, and in each case, subject to the limitations of Sections 2.1(c) and 2.3. 

(c) Notwithstanding the foregoing obligations, if the Company furnishes to Holders requesting a registration pursuant to this
Section 2.1 a certificate signed by the Company’s chief executive officer stating that in the good faith judgment of the Company’s Board of Directors it would be materially detrimental to the Company and its stockholders for
such registration statement to either become effective or remain effective for as long as such registration statement otherwise would be required to remain effective, because such action would (i) materially interfere with a significant
acquisition, corporate reorganization, or other similar transaction involving the Company; (ii) require premature disclosure of material 

  
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information that the Company has a bona fide business purpose for preserving as confidential; or (iii) render the Company unable to comply with requirements under the Securities Act or
Exchange Act, then the Company shall have the right to defer taking action with respect to such filing, and any time periods with respect to filing or effectiveness thereof shall be tolled correspondingly, for a period of not more than sixty
(60) days after the request of the Initiating Holders is given; provided, however, that the Company may not invoke this right more than once in any twelve (12) month period; and provided, further, that the
Company shall not register any securities for its own account or that of any other stockholder during such sixty (60) day period other than an Excluded Registration. 

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

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

  
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 2.3. Underwriting Requirements. 

(a) If, pursuant to Section 2.1, the Initiating Holders intend to distribute the Registrable Securities covered by their request
by means of an underwriting, they shall so advise the Company as a part of their request made pursuant to Section 2.1, and the Company shall include such information in the Demand Notice. The underwriter(s) will be selected by the
Initiating Holders, subject only to the reasonable approval of the Company. In such event, the right of any Holder to include such Holder’s Registrable Securities in such registration shall be conditioned upon such Holder’s participation
in such underwriting and the inclusion of such Holder’s Registrable Securities in the underwriting to the extent provided herein. All Holders proposing to distribute their securities through such underwriting shall (together with the Company as
provided in Section 2.4(e)) enter into an underwriting agreement in customary form with the underwriter(s) selected for such underwriting. Notwithstanding any other provision of this Section 2.3, if the managing
underwriter(s) advise(s) the Initiating Holders in writing that marketing factors require a limitation on the number of shares to be underwritten, then the Initiating Holders shall so advise all Holders of Registrable Securities that otherwise would
be underwritten pursuant hereto, and the number of Registrable Securities that may be included in the underwriting shall be allocated among such Holders of Registrable Securities, including the Initiating Holders, in proportion (as nearly as
practicable) to the number of Registrable Securities owned by each Holder or in such other proportion as shall mutually be agreed to by all such selling Holders; provided, however, that the number of Registrable Securities held by the
Holders to be included in such underwriting shall not be reduced unless all other securities are first entirely excluded from the underwriting. To facilitate the allocation of shares in accordance with the above provisions, the Company or the
underwriters may round the number of shares allocated to any Holder to the nearest 100 shares. 
 (b) In connection with any offering
involving an underwriting of shares of the Company’s capital stock pursuant to Section 2.2, the Company shall not be required to include any of the Holders’ Registrable Securities in such underwriting unless the Holders accept
the terms of the underwriting as agreed upon between the Company and its underwriters, and then only in such quantity as the underwriters in their sole discretion determine will not jeopardize the success of the offering by the Company. If the total
number of securities, including Registrable Securities, requested by stockholders to be included in such offering exceeds the number of securities to be sold (other than by the Company) that the underwriters in their reasonable discretion determine
is compatible with the success of the offering, then the Company shall be required to include in the offering only that number of such securities, including Registrable Securities, which the underwriters and the Company in their sole discretion
determine will not jeopardize the success of the offering. If the underwriters determine that less than all of the Registrable Securities requested to be registered can be included in such offering, then the Registrable Securities that are included
in such offering shall be allocated among the selling Holders in proportion (as nearly as practicable to) the number of Registrable Securities owned by each selling Holder or in such other proportions as shall mutually be agreed to by all such
selling Holders. To facilitate the allocation of shares in accordance with the above provisions, the Company or the underwriters may round the number of shares allocated to any Holder to the nearest 100 shares. Notwithstanding the foregoing, in no
event shall (i) the number of Registrable Securities included in the offering be reduced unless all other securities (other than securities to be sold by the Company) are first entirely excluded from the offering, or (ii) the number of
Registrable Securities included in the offering be reduced below thirty percent (30%) of the total number of securities included in such offering, unless such offering is the IPO, in 

  
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which case the selling Holders may be excluded further if the underwriters make the determination described above and no other stockholder’s securities are included in such offering. For
purposes of the provision in this Section 2.3(b) concerning apportionment, for any selling Holder that is a partnership, limited liability company, or corporation, the partners, members, retired partners, retired members, stockholders,
and Affiliates of such Holder, or the estates and Immediate Family Members of any such partners, retired partners, members, and retired members and any trusts for the benefit of any of the foregoing Persons, shall be deemed to be a single
“selling Holder,” and any pro rata reduction with respect to such “selling Holder” shall be based upon the aggregate number of Registrable Securities owned by all Persons included in such “selling Holder,” as defined in
this sentence. 
 (c) For purposes of Section 2.1, a registration shall not be counted as “effected” if, as a result
of an exercise of the underwriter’s cutback provisions in Section 2.3(a), fewer than fifty percent (50%) of the total number of Registrable Securities that Holders have requested to be included in such registration statement
are actually included. 
 2.4. Obligations of the Company. Whenever required under this Section 2 to effect the
registration of any Registrable Securities, the Company shall, as expeditiously as reasonably possible: 
 (a) prepare and file with the SEC
a registration statement with respect to such Registrable Securities and use its commercially reasonable efforts to cause such registration statement to become effective and, upon the request of the Holders of a majority of the Registrable
Securities registered thereunder, keep such registration statement effective for a period of up to one hundred twenty (120) days or, if earlier, until the distribution contemplated in the registration statement has been completed;
provided, however, that (i) such one hundred twenty (120) day period shall be extended for a period of time equal to the period the Holder refrains, at the request of an underwriter of Common Stock (or other securities) of
the Company, from selling any securities included in such registration, and (ii) in the case of any registration of Registrable Securities on Form S-3 that are intended to be offered on a continuous or delayed basis, subject to compliance with
applicable SEC rules, such one hundred twenty (120) day period shall be extended for up to sixty (60) days, if necessary, to keep the registration statement effective until all such Registrable Securities are sold; 

(b) prepare and file with the SEC such amendments and supplements to such registration statement, and the prospectus used in connection with
such registration statement, as may be necessary to comply with the Securities Act in order to enable the disposition of all securities covered by such registration statement; 

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

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

  
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 (e) in the event of any underwritten public offering, enter into and perform its obligations
under an underwriting agreement, in usual and customary form, with the underwriter(s) of such offering; 
 (f) use its commercially
reasonable efforts to cause all such Registrable Securities covered by such registration statement to be listed on a national securities exchange or trading system and each securities exchange and trading system (if any) on which similar securities
issued by the Company are then listed; 
 (g) provide a transfer agent and registrar for all Registrable Securities registered pursuant to
this Agreement and provide a CUSIP number for all such Registrable Securities, in each case not later than the effective date of such registration; 

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

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

2.6. Expenses of Registration. All expenses (other than Selling Expenses) incurred in connection with registrations, filings, or
qualifications pursuant to Section 2, including all registration, filing, and qualification fees; printers’ and accounting fees; fees and disbursements of counsel for the Company; and the reasonable fees and disbursements, not to
exceed $75,000, of one counsel for the selling Holders (“Selling Holder Counsel”), shall be borne and paid by the Company; provided, however, that the Company shall not be required to


  
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pay for any expenses of any registration proceeding begun pursuant to Section 2.1 if the registration request is subsequently withdrawn at the request of the Holders of a majority of
the Registrable Securities to be registered (in which case all selling Holders shall bear such expenses pro rata based upon the number of Registrable Securities that were to be included in the withdrawn registration), unless the Holders of a
majority of the Registrable Securities agree to forfeit their right to one registration pursuant to Sections 2.1(a) or 2.1(b), as the case may be; provided, further, that if, at the time of such withdrawal, the Holders
shall have learned of a material adverse change in the condition, business, or prospects of the Company from that known to the Holders at the time of their request and have withdrawn the request with reasonable promptness after learning of such
information then the Holders shall not be required to pay any of such expenses and shall not forfeit their right to one registration pursuant to Sections 2.1(a) or 2.1(b). All Selling Expenses relating to Registrable Securities
registered pursuant to this Section 2 shall be borne and paid by the Holders pro rata on the basis of the number of Registrable Securities registered on their behalf. 

2.7. Delay of Registration. No Holder shall have any right to obtain or seek an injunction restraining or otherwise delaying any
registration pursuant to this Agreement as the result of any controversy that might arise with respect to the interpretation or implementation of this Section 2. 

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

(a) To the extent permitted by law, the Company shall indemnify and hold harmless each selling Holder, and the partners, members, officers,
directors, and stockholders of each such Holder; legal counsel and accountants for each such Holder; any underwriter (as defined in the Securities Act) for each such Holder; and each Person, if any, who controls such Holder or underwriter within the
meaning of the Securities Act or the Exchange Act, against any Damages, and the Company will pay to each such Holder, underwriter, controlling Person, or other aforementioned Person any legal or other expenses reasonably incurred thereby in
connection with investigating or defending any claim or proceeding from which Damages may result, as such expenses are incurred; provided, however, that the indemnity agreement contained in this Section 2.8(a) shall not
apply to amounts paid in settlement of any such claim or proceeding if such settlement is effected without the consent of the Company, which consent shall not be unreasonably withheld, nor shall the Company be liable for any Damages to the extent
that they arise out of or are based upon actions or omissions made in reliance upon and in conformity with written information furnished by or on behalf of any such Holder, underwriter, controlling Person, or other aforementioned Person expressly
for use in connection with such registration. 
 (b) To the extent permitted by law, each selling Holder, severally and not jointly, shall
indemnify and hold harmless the Company, and each of its directors, each of its officers who has signed the registration statement, each Person (if any), who controls the Company within the meaning of the Securities Act, legal counsel and
accountants for the Company, any underwriter (as defined in the Securities Act), any other Holder selling securities in such registration statement, and any controlling Person of any such underwriter or other Holder, against any Damages, in each
case only to the extent that such Damages arise out of or 

  
 10 








 
are based upon actions or omissions made in reliance upon and in conformity with written information furnished by or on behalf of such selling Holder expressly for use in connection with such
registration; and each such selling Holder will pay to the Company and each other aforementioned Person any legal or other expenses reasonably incurred thereby in connection with investigating or defending any claim or proceeding from which Damages
may result, as such expenses are incurred; provided, however, that the indemnity agreement contained in this Section 2.8(b) shall not apply to amounts paid in settlement of any such claim or proceeding if such settlement is
effected without the consent of the Holder, which consent shall not be unreasonably withheld; and provided, further, that in no event shall the aggregate amounts payable by any Holder by way of indemnity or contribution under
Sections 2.8(b) and 2.8(d) exceed the proceeds from the offering received by such Holder (net of any Selling Expenses paid by such Holder), except in the case of fraud or willful misconduct by such Holder. 

(c) Promptly after receipt by an indemnified party under this Section 2.8 of notice of the commencement of any action (including
any governmental action) for which a party may be entitled to indemnification hereunder, such indemnified party will, if a claim in respect thereof is to be made against any indemnifying party under this Section 2.8, give the
indemnifying party notice of the commencement thereof. The indemnifying party shall have the right to participate in such action and, to the extent the indemnifying party so desires, participate jointly with any other indemnifying party to which
notice has been given, and to assume the defense thereof with counsel mutually satisfactory to the parties; provided, however, that an indemnified party (together with all other indemnified parties that may be represented without
conflict by one counsel) shall have the right to retain one separate counsel, with the fees and expenses to be paid by the indemnifying party, if representation of such indemnified party by the counsel retained by the indemnifying party would be
inappropriate due to actual or potential differing interests between such indemnified party and any other party represented by such counsel in such action. 

(d) To provide for just and equitable contribution to joint liability under the Securities Act in any case in which either (i) any party
otherwise entitled to indemnification hereunder makes a claim for indemnification pursuant to this Section 2.8 but it is judicially determined (by the entry of a final judgment or decree by a court of competent jurisdiction and the
expiration of time to appeal or the denial of the last right of appeal) that such indemnification may not be enforced in such case, notwithstanding the fact that this Section 2.8 provides for indemnification in such case, or
(ii) contribution under the Securities Act may be required on the part of any party hereto for which indemnification is provided under this Section 2.8, then, and in each such case, such parties will contribute to the aggregate
losses, claims, damages, liabilities, or expenses to which they may be subject (after contribution from others) in such proportion as is appropriate to reflect the relative fault of each of the indemnifying party and the indemnified party in
connection with the statements, omissions, or other actions that resulted in such loss, claim, damage, liability, or expense, as well as to reflect any other relevant equitable considerations. The relative fault of the indemnifying party and of the
indemnified party shall be determined by reference to, among other things, whether the untrue or allegedly untrue statement of a material fact, or the omission or alleged omission of a material fact, relates to information supplied by the
indemnifying party or by the indemnified party and the parties’ relative intent, knowledge, access to information, and opportunity to correct or prevent such statement or omission; provided, however, that, in any such case,
(x) no Holder will be required 

  
 11 








 
to contribute any amount in excess of the public offering price of all such Registrable Securities offered and sold by such Holder pursuant to such registration statement, and (y) no Person
guilty of fraudulent misrepresentation (within the meaning of Section 11(f) of the Securities Act) will be entitled to contribution from any Person who was not guilty of such fraudulent misrepresentation; and provided, further,
that in no event shall a Holder’s liability pursuant to this Section 2.8(d), when combined with the amounts paid or payable by such Holder pursuant to Section 2.8(b), exceed the proceeds from the offering received by
such Holder (net of any Selling Expenses) paid by such Holder), except in the case of willful misconduct or fraud by such Holder. 
 (e)
Notwithstanding the foregoing, to the extent that the provisions on indemnification and contribution contained in the underwriting agreement entered into in connection with the underwritten public offering are in conflict with the foregoing
provisions, the provisions in the underwriting agreement shall control. 
 (f) Unless otherwise superseded by an underwriting agreement
entered into in connection with the underwritten public offering, the obligations of the Company and Holders under this Section 2.8 shall survive the completion of any offering of Registrable Securities in a registration under this
Section 2, and otherwise shall survive the termination of this Agreement. 
 2.9. Reports Under Exchange Act. With a view
to making available to the Holders the benefits of SEC Rule 144 and any other rule or regulation of the SEC that may at any time permit a Holder to sell securities of the Company to the public without registration or pursuant to a registration on
Form S-3, the Company shall: 
 (a) make and keep available adequate current public information, as those terms are understood and defined
in SEC Rule 144, at all times after the effective date of the registration statement filed by the Company for the IPO; 
 (b) use
commercially reasonable efforts to file with the SEC in a timely manner all reports and other documents required of the Company under the Securities Act and the Exchange Act (at any time after the Company has become subject to such reporting
requirements); and 
 (c) furnish to any Holder, so long as the Holder owns any Registrable Securities, forthwith upon request (i) to
the extent accurate, a written statement by the Company that it has complied with the reporting requirements of SEC Rule 144 (at any time after ninety (90) days after the effective date of the registration statement filed by the Company for the
IPO), the Securities Act, and the Exchange Act (at any time after the Company has become subject to such reporting requirements), or that it qualifies as a registrant whose securities may be resold pursuant to Form S-3 (at any time after the Company
so qualifies); (ii) a copy of the most recent annual or quarterly report of the Company and such other reports and documents so filed by the Company; and (iii) such other information as may be reasonably requested in availing any Holder of
any rule or regulation of the SEC that permits the selling of any such securities without registration (at any time after the Company has become subject to the reporting requirements under the Exchange Act) or pursuant to Form S-3 (at any time after
the Company so qualifies to use such form). 

  
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 2.10. Limitations on Subsequent Registration Rights. From and after the date of this
Agreement, the Company shall not, without the prior written consent of the Holders of more than the Appropriate Percentage of the Registrable Securities then outstanding, enter into any agreement with any holder or prospective holder of any
securities of the Company that would allow such holder or prospective holder (i) to include such securities in any registration unless, under the terms of such agreement, such holder or prospective holder may include such securities in any such
registration only to the extent that the inclusion of such securities will not reduce the number of the Registrable Securities of the Holders that are included or (ii) allow such holder or prospective holder to initiate a demand for
registration of any securities held by such holder or prospective holder. 
 2.11. “Market Stand-off” Agreement. Each
Holder hereby agrees that it will not, without the prior written consent of the managing underwriter, during the period commencing on the date of the final prospectus relating to the registration by the Company of shares of its Common Stock or any
other equity securities under the Securities Act on a registration statement on Form S-1 or Form S-3, and ending on the date specified by the Company and the managing underwriter (such period not to exceed (x) one hundred eighty (180) days
in the case of the IPO, or (y) if requested by the managing underwriter and approved by Holders of more than the Appropriate Percentage of the Registrable Securities, ninety (90) days in the case of any registration other than the IPO,
(i) lend; offer; pledge; sell; contract to sell; sell any option or contract to purchase; purchase any option or contract to sell; grant any option, right, or warrant to purchase; or otherwise transfer or dispose of, directly or indirectly, any
shares of Common Stock or any securities convertible into or exercisable or exchangeable (directly or indirectly) for Common Stock held immediately before the effective date of the registration statement for such offering or (ii) enter into any
swap or other arrangement that transfers to another, in whole or in part, any of the economic consequences of ownership of such securities, whether any such transaction described in clause (i) or (ii) above is to be settled by delivery of
Common Stock or other securities, in cash, or otherwise. Notwithstanding clause (i) and (ii) above, each Holder may distribute any or all of its shares of Common Stock or any securities convertible into or exercisable or exchangeable
(directly or indirectly) for Common Stock to any of its limited partners. Provided, however, that such limited partners who receive the distribution of any or all shares of Common Stock or any securities convertible into or exercisable
or exchangeable (directly or indirectly) for Common Stock shall still be subject to the other provisions of this Section 2.11. The foregoing provisions of this Section 2.11 shall not apply to the sale of any shares to an
underwriter pursuant to an underwriting agreement, and shall be applicable to the Holders only if all officers and directors are subject to the same restrictions and the Company uses commercially reasonable efforts to obtain a similar agreement from
all stockholders individually owning more than one percent (1%) of the Company’s outstanding Common Stock (after giving effect to conversion into Common Stock of all outstanding Preferred Stock). The underwriters in connection with such
registration are intended third-party beneficiaries of this Section 2.11 and shall have the right, power, and authority to enforce the provisions hereof as though they were a party hereto. Each Holder further agrees to execute such
agreements as may be reasonably requested by the underwriters in connection with such registration that are consistent with this Section 2.11 or that are necessary to give further effect thereto. Any discretionary waiver or termination
of the restrictions of any or all of such agreements by the Company or the underwriters shall apply pro rata to all Holders subject to such agreements, based on the number of shares subject to such agreements. 

  
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 2.12. Restrictions on Transfer. 

(a) The Preferred Stock and the Registrable Securities shall not be sold, pledged, or otherwise transferred, and the Company shall not
recognize and shall issue stop-transfer instructions to its transfer agent with respect to any such sale, pledge, or transfer, except upon the conditions specified in this Agreement, which conditions are intended to ensure compliance with the
provisions of the Securities Act. A transferring Holder will cause any proposed purchaser, pledgee, or transferee of the Preferred Stock and the Registrable Securities held by such Holder to agree to take and hold such securities subject to the
provisions and upon the conditions specified in this Agreement. 
 (b) Each certificate or instrument representing (i) the Preferred
Stock, (ii) the Registrable Securities, and (iii) any other securities issued in respect of the securities referenced in clauses (i) and (ii), upon any stock split, stock dividend, recapitalization, merger, consolidation, or similar
event, shall (unless otherwise permitted by the provisions of Section 2.12(c)) be stamped or otherwise imprinted with a legend substantially in the following form: 

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

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

(c) The holder of each certificate representing Restricted Securities, by acceptance thereof, agrees to comply in all respects with the
provisions of this Section 2. Before any proposed sale, pledge, or transfer of any Restricted Securities, unless there is in effect a registration statement under the Securities Act covering the proposed transaction, the Holder thereof
shall give notice to the Company of such Holder’s intention to effect such sale, pledge, or transfer. Each such notice shall describe the manner and circumstances of the proposed sale, pledge, or transfer in sufficient detail and, if reasonably
requested by the Company, shall be accompanied at such Holder’s expense by either (i) a written opinion of legal counsel who shall, and whose legal opinion shall, be reasonably satisfactory to the Company, addressed to the Company, to the
effect that the proposed transaction may be effected without registration under 

  
 14 








 
the Securities Act; (ii) a “no action” letter from the SEC to the effect that the proposed sale, pledge, or transfer of such Restricted Securities without registration will not
result in a recommendation by the staff of the SEC that action be taken with respect thereto; or (iii) any other evidence reasonably satisfactory to counsel to the Company to the effect that the proposed sale, pledge, or transfer of the
Restricted Securities may be effected without registration under the Securities Act, whereupon the Holder of such Restricted Securities shall be entitled to sell, pledge, or transfer such Restricted Securities in accordance with the terms of the
notice given by the Holder to the Company. The Company will not require such a legal opinion or “no action” letter (x) in any transaction in compliance with SEC Rule 144 or (y) in any transaction in which such Holder distributes
Restricted Securities to an Affiliate of such Holder for no consideration; provided, that each transferee agrees in writing to be subject to the terms of this Section 2.12. Each certificate or instrument evidencing the Restricted
Securities transferred as above provided shall bear, except if such transfer is made pursuant to SEC Rule 144, the appropriate restrictive legend set forth in Section 2.12(b), except that such certificate shall not bear such restrictive
legend if, in the opinion of counsel for such Holder and the Company, such legend is not required in order to establish compliance with any provisions of the Securities Act. 

2.13. Termination of Registration Rights. The right of any Holder to request registration or inclusion of Registrable Securities in any
registration pursuant to Section 2.1 or 2.2 shall terminate upon the closing of a Deemed Liquidation Event, as such term is defined in the Company’s Certificate of Incorporation. 

3. Information and Observer Rights. 

3.1. Delivery of Financial Statements. The Company shall deliver to each Major Investor: 

(a) as soon as practicable, but in any event within ninety (90) days after the end of each fiscal year of the Company, (i) a balance
sheet as of the end of such year, (ii) statements of income and of cash flows for such year, and a comparison between (x) the actual amounts as of and for such fiscal year and (y) the comparable amounts for the prior year and as
included in the Budget (as defined in Section 3.1(e)) for such year, with an explanation of any material differences between such amounts and a schedule as to the sources and applications of funds for such year, and (iii) a
statement of stockholders’ equity as of the end of such year, all such financial statements audited and certified by independent public accountants of nationally recognized standing selected by the Company; 

(b) as soon as practicable, but in any event within thirty (30) days after the end of each of the first three (3) quarters of each
fiscal year of the Company, unaudited statements of income and of cash flows for such fiscal quarter, and an unaudited balance sheet as of the end of such fiscal quarter, all prepared in accordance with GAAP (except that such financial statements
may (i) be subject to normal year-end audit adjustments and (ii) not contain all notes thereto that may be required in accordance with GAAP); 

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

(f) with respect to the financial statements called for in Sections 3.1(a) and 3.1(b), a management discussion and analysis by
the chief executive officer of the Company that includes updates and status of the Company’s material developments and activities; and 

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

Notwithstanding anything else in this Section 3.1 to the contrary, the Company may cease providing the information set forth in this
Section 3.1 during the period starting with the date thirty (30) days before the Company’s good-faith estimate of the date of filing of a registration statement if it reasonably concludes it must do so to comply with the SEC
rules applicable to such registration statement and related offering; provided, that the Company’s covenants under this Section 3.1 shall be reinstated at such time as the Company is no longer actively employing its
commercially reasonable efforts to cause such registration statement to become effective. 

  
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 3.2. Inspection. The Company shall permit each Major Investor, at such Major
Investor’s expense, to visit and inspect the Company’s properties; examine its books of account and records; and discuss the Company’s affairs, finances, and accounts with its officers, during normal business hours of the Company as
may be reasonably requested by the Major Investor; provided, however, that the Company shall not be obligated pursuant to this Section 3.2 to provide access to any information that it reasonably and in good faith considers
to be a trade secret or confidential information (unless covered by an enforceable confidentiality agreement, in form acceptable to the Company) or the disclosure of which would adversely affect the attorney-client privilege between the Company and
its counsel. 
 3.3. Observer Rights. As long as each of Venture Investors Early Stage Fund IV Limited Partnership, Athenian Venture
Partners, AgeChem Venture Fund L.P., Cincinnati Cornerstone Investors AKB, LLC and Family Akebia Investments LLC (a) purchases shares of the Company’s Series C Preferred Stock for at least $1,499,000 in cash (excluding any conversion of
Series X Preferred Stock) pursuant to the Series C Purchase Agreement and (b) continues to own not less than fifty percent (50%) of the aggregate number of shares of Preferred Stock that it purchased under the Series A Preferred Stock
Purchase Agreements and the Series B Purchase Agreement and that it is purchasing (or acquiring as a result of the conversion of its shares of Series X Preferred Stock) under the Series C Purchase Agreement (or an equivalent number of shares of
Common Stock issued upon conversion thereof), and as long as Triathlon Medical Ventures Fund, L.P. continues to own not less than fifty percent (50%) of the aggregate number of shares of Preferred Stock that it purchased under the Series A
Preferred Stock Purchase Agreements and the Series B Purchase Agreement and that it is purchasing (or acquiring as a result of the conversion of its shares of Series X Preferred Stock) under the Series C Purchase Agreement (or an equivalent number
of shares of Common Stock issued upon conversion thereof), the Company shall invite a representative of each such Person (provided that the representative of Triathlon Medical Ventures Fund, L.P. must be John M. Rice), as applicable, to attend all
meetings of its Board of Directors in a nonvoting observer capacity and, in this respect, shall give such representative copies of all notices, minutes, consents, and other materials that it provides to its directors at the same time and in the same
manner as provided to such directors; provided, however, that such representative shall agree to hold in confidence and trust and to act in a fiduciary manner with respect to all information so provided; and provided,
further, that the Company reserves the right to withhold any information and to exclude such representative from any meeting or portion thereof if access to such information or attendance at such meeting could adversely affect the
attorney-client privilege between the Company and its counsel or result in disclosure of trade secrets or a conflict of interest, or if such Investor or its representative is a competitor of the Company. 

3.4. Termination of Information and Observer Rights. The covenants set forth in Sections 3.1, 3.2, and 3.3 shall
terminate and be of no further force or effect (i) immediately before the consummation of the IPO, (ii) when the Company first becomes subject to the periodic reporting requirements of Section 12(g) or 15(d) of the Exchange Act, or
(iii) upon a Deemed Liquidation Event, as such term is defined in the Company’s Certificate of Incorporation, whichever event occurs first. 

  
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 3.5. Confidentiality. Each Investor agrees that such Investor will keep confidential and
will not disclose, divulge, or use for any purpose (other than to monitor its investment in the Company) any confidential information obtained from the Company pursuant to the terms of this Agreement (including notice of the Company’s intention
to file a registration statement), unless such confidential information (a) is known or becomes known to the public in general (other than as a result of a breach of this Section 3.5 by such Investor), (b) is or has been
independently developed or conceived by the Investor without use of the Company’s confidential information, or (c) is or has been made known or disclosed to the Investor by a third party without a breach of any obligation of
confidentiality such third party may have to the Company; provided, however, that an Investor may disclose confidential information (i) to its attorneys, accountants, consultants, and other professionals to the extent necessary to
obtain their services in connection with monitoring its investment in the Company; (ii) to any prospective purchaser of any Registrable Securities from such Investor, if such prospective purchaser agrees to be bound by the provisions of this
Section 3.5; (iii) to any existing or prospective Affiliate, partner, member, stockholder, or wholly owned subsidiary of such Investor in the ordinary course of business; provided, that such Investor informs such Person that
such information is confidential and directs such Person to maintain the confidentiality of such information; or (iv) as may otherwise be required by law; provided, that the Investor promptly notifies the Company of such disclosure and
takes reasonable steps to minimize the extent of any such required disclosure. 
 4. Rights to Future Stock Issuances. 

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

(b) By notification to the Company within twenty (20) days after the Offer Notice is given, each Investor may elect to purchase or
otherwise acquire, at the price and on the terms specified in the Offer Notice, up to that portion of such New Securities which equals the proportion that the Common Stock then held by such Investor (assuming full conversion and/or exercise, as
applicable, of all Preferred Stock and other Derivative Securities then held by such Investor) bears to the total shares of Common Stock of the Company then outstanding (assuming full conversion and/or exercise, as applicable, of all Preferred Stock
and other Derivative Securities). At the expiration of such twenty (20) day period, the Company shall promptly notify each Investor that elects to purchase or acquire all the shares available to it (each, a “Fully Exercising
Investor”) of any other Investor’s failure to do likewise. During the ten (10) day period commencing after the Company has given such notice, each Fully Exercising Investor may, by giving notice to the Company, elect to purchase
or acquire, in addition to the number of shares specified above, up to that portion of the New Securities for which Investors were entitled to subscribe but that were not subscribed for by the Investors which is equal to the proportion that the
Common Stock then held by such Fully Exercising Investor (assuming full conversion and/or exercise, as applicable, of all Preferred Stock and other Derivative Securities 

  
 18 








 
then held by such Fully Exercising Investor) bears to the Common Stock then held by all Fully Exercising Investors who wish to purchase such unsubscribed shares (assuming full conversion and/or
exercise, as applicable, of all Preferred Stock and other Derivative Securities then held by all such Fully Exercising Investors). The closing of any sale pursuant to this Section 4.1(b) shall occur within the later of ninety
(90) days of the date that the Offer Notice is given and the date of initial sale of New Securities pursuant to Section 4.1(c). 

(c) If all New Securities referred to in the Offer Notice are not elected to be purchased or acquired as provided in
Section 4.1(b), the Company may, during the ninety (90) day period following the expiration of the periods provided in
 Section 4.1(b), offer and sell the remaining unsubscribed portion of such New Securities to any
Person or Persons at a price not less than, and upon terms no more favorable to the offeree than, those specified in the Offer Notice. If the Company does not enter into an agreement for the sale of the New Securities within such period, or if such
agreement is not consummated within thirty (30) days of the execution thereof, the right provided hereunder shall be deemed to be revived and such New Securities shall not be offered unless first reoffered to the Investors in accordance with
this Section 4.1. 
 (d) The right of first offer in this Section 4.1 shall not be applicable to (i) Exempted
Securities (as defined in the Company’s Certificate of Incorporation); and (ii) shares of Common Stock issued in the IPO. 
 (e)
[Reserved] 
 (f) Notwithstanding any provision hereof to the contrary, in lieu of complying with the provisions of this
Section 4.1, the Company may elect to give notice to the Investors within thirty (30) days after the issuance of New Securities. Such notice shall describe the type, price, and terms of the New Securities. Each Investor shall have
twenty (20) days from the date notice is given to elect to purchase up to the number of New Securities that would, if purchased by such Investor, maintain such Investor’s percentage-ownership position, calculated as set forth in
Section 4.1(b) before giving effect to the issuance of such New Securities. The closing of such sale shall occur within sixty (60) days of the date notice is given to the Investors. 

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

5. Additional Covenants. 

5.1. Insurance. The Company shall use its commercially reasonable efforts to obtain, within ninety (90) days of the date hereof,
from financially sound and reputable insurers Directors and Officers liability insurance and term “key-person” insurance on each of Joseph Gardner, Robert Shalwitz and William Daly, each in the amount of $2,000,000 or such lesser amount
determined by the Board of Directors (including a majority of the Preferred Directors) and on terms and conditions satisfactory to the Board of Directors, and will use commercially reasonable efforts to cause such insurance policies to be maintained
until such time as the Board 

  
 19 








 
of Directors determines that such insurance should be discontinued. The key-person policy shall name the Company as loss payee, and neither policy shall be cancelable by the Company without prior
approval by the Board of Directors and 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. 

5.2. Employee Agreements. The Company will cause (i) each person now or hereafter employed by it or by any subsidiary (or engaged
by the Company or any subsidiary as a consultant/independent contractor) with access to confidential information and/or trade secrets to enter into a nondisclosure and proprietary rights assignment agreement and (ii) each Key Employee to enter
into a one (1) year noncompetition and nonsolicitation agreement, substantially in the form approved by the Board of Directors. In addition, the Company shall not amend, modify, terminate, waive, or otherwise alter, in whole or in part, any of
the above-referenced agreements or any restricted stock agreement between the Company and any employee, without the consent of a majority of the Preferred Directors. 

5.3. Qualified Small Business Stock. The Company shall use reasonable best efforts to cause the shares of Preferred Stock, as well as
any shares into which such shares are converted, within the meaning of Section 1202(f) of the Internal Revenue Code (the “Code”), to constitute “qualified small business stock” as defined in Section 1202(c) of
the Code; provided, however, that such requirement shall not be applicable if the Board of Directors of the Company determines, in its good-faith business judgment, that such qualification would unreasonably defer a sale or liquidation
of the Company by virtue of the applicable “minimum holding period” requirements. The Company shall submit to its stockholders (including the Investors) and to the Internal Revenue Service any reports that may be required under
Section 1202(d)(1)(C) of the Code and the regulations promulgated thereunder. In addition, within twenty (20) business days after any Investor’s written request therefor, the Company shall, at its option, either (i) deliver to
such Investor a written statement indicating whether (and what portion of) such Investor’s interest in the Company constitutes “qualified small business stock” as defined in Section 1202(c) of the Code or (ii) deliver to
such Investor such factual information in the Company’s possession as is reasonably necessary to enable such Investor to determine whether (and what portion of) such Investor’s interest in the Company constitutes “qualified small
business stock” as defined in Section 1202(c) of the Code. 
 5.4. Matters Requiring Investor Approval. So long as at least
twenty percent (20%) of the originally issued Series A Preferred Stock, at least twenty percent (20%) of the originally issued Series B Preferred Stock or at least twenty percent (20%) of the originally issued Series C Preferred Stock
remains outstanding, the Company hereby covenants and agrees with each of the Investors that it shall not, without the approval of the Board of Directors, which approval must include the affirmative vote of a majority of the Preferred Directors then
in office: 
 (a) make, or permit any subsidiary to make, any loan or advance in an aggregate amount in excess of $100,000 to, or own any
stock or other securities of, any subsidiary or other corporation, partnership, or other entity unless it is wholly owned by the Company; 

  
 20 








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

 (c) guarantee, directly or indirectly, or permit any subsidiary to guarantee, directly or indirectly, any indebtedness in an aggregate
amount in excess of $100,000, except for trade accounts of the Company or any subsidiary arising in the ordinary course of business; 
 (d)
make any investment inconsistent with any investment policy approved by the Board of Directors; 
 (e) incur any aggregate indebtedness in
excess of $250,000, other than trade credit incurred in the ordinary course of business; 
 (f) otherwise enter into or be a party to any
transaction with any director, officer, or employee of the Company or any “associate” (as defined in Rule 12b-2 promulgated under the Exchange Act) of any such Person, except for transactions contemplated by this Agreement, the Series A
Purchase Agreements, the Series B Purchase Agreement, and the P&G License Agreement in effect as of the date of this Agreement; 
 (g)
hire, terminate, or change the compensation of the executive officers, including approving any option grants or stock awards to executive officers; 

(h) change the principal business of the Company, enter new lines of business, or exit the current line of business; 

(i) sell, assign, license, pledge, or encumber material technology or intellectual property, other than licenses granted in the ordinary
course of business; 
 (j) make any capital expenditure in excess of $100,000; or 

(k) adopt the Budget. 
 5.5.
Board Matters. Unless otherwise determined by the vote of a majority of the directors then in office, the Board of Directors shall meet at least bi-monthly in accordance with an agreed-upon schedule. The Company shall reimburse the
nonemployee directors for all reasonable out-of-pocket travel expenses incurred (consistent with the Company’s travel policy) in connection with attending meetings of the Board of Directors. The Company shall cause to be established, as soon as
practicable after such request, and will maintain, an audit and compensation committee, each of which shall consist solely of non-management directors. Each non-employee director shall be entitled in such person’s discretion to be a member of
any Board committee. 

  
 21 








 5.6. Successor Indemnification. If the Company or any of its successors or assignees
consolidates with or merges into any other Person and is not the continuing or surviving corporation or entity of such consolidation or merger, then to the extent necessary, proper provision shall be made so that the successors and assignees of the
Company assume the obligations of the Company with respect to indemnification of members of the Board of Directors as in effect immediately before such transaction, whether such obligations are contained in the Company’s Bylaws, its Certificate
of Incorporation, or elsewhere, as the case may be. 
 5.7. Termination of Covenants. The covenants set forth in this
Section 5, except for Section 5.6, shall terminate and be of no further force or effect (i) immediately before the consummation of the IPO, (ii) when the Company first becomes subject to the periodic reporting
requirements of Section 12(g) or 15(d) of the Exchange Act, or (iii) upon a Deemed Liquidation Event, as such term is defined in the Company’s Certificate of Incorporation, whichever event occurs first. 

5.8. Limitation of Liability. The Company shall not enter into any agreement that could reasonably be expected to result in requiring
such Investor in any series of the Company’s Preferred Stock, without the written consent of such Investor, to participate in any transaction which would expose such Investor to liability (a) in excess of the proceeds actually received by
such Investor under such transaction or (b) where the share of such liability of such Investor is higher than its pro rata portion of the proceeds actually received by such Investor under such transaction. 

6. Miscellaneous. 
 6.1.
Successors and Assigns. The rights under this Agreement may be assigned (but only with all related obligations) by a Holder to a transferee of Registrable Securities that (i) is an Affiliate of a Holder; (ii) is a Holder’s
Immediate Family Member or trust for the benefit of an individual Holder or one or more of such Holder’s Immediate Family Members; or (iii) after such transfer, holds at least 1,000 shares of Registrable Securities (subject to appropriate
adjustment for stock splits, stock dividends, combinations, and other recapitalizations after the date hereof); provided, however, that (x) the Company is, within a reasonable time after such transfer, furnished with written
notice of the name and address of such transferee and the Registrable Securities with respect to which such rights are being transferred; and (y) such transferee agrees in a written instrument delivered to the Company to be bound by and subject
to the terms and conditions of this Agreement, including the provisions of Section 2.11. For the purposes of determining the number of shares of Registrable Securities held by a transferee, the holdings of a transferee (1) that is
an Affiliate or stockholder of a Holder; (2) who is a Holder’s Immediate Family Member; or (3) that is a trust for the benefit of an individual Holder or such Holder’s Immediate Family Member shall be aggregated together and with
those of the transferring Holder; provided, further, that all transferees who would not qualify individually for assignment of rights shall have a single attorney-in-fact for the purpose of exercising any rights, receiving notices, or
taking any action under this Agreement. The terms and conditions of this Agreement inure to the benefit of and are binding upon the respective successors and permitted assignees of the parties. Nothing in this Agreement, express or implied, is
intended to confer upon any party other than the parties hereto or their respective successors and permitted assignees any rights, remedies, obligations or liabilities under or by reason of this Agreement, except as expressly provided herein. 

  
 22 








 6.2. Governing Law. 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. 

6.3. Counterparts; Facsimile or PDF. This Agreement may be executed in two or more counterparts, each of which shall be deemed an
original, but all of which together shall constitute one and the same instrument. This Agreement may also be executed and delivered by facsimile 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. 
 6.4. Titles and Subtitles. The titles and subtitles used in this
Agreement are for convenience only and are not to be considered in construing or interpreting this Agreement. 
 6.5. Notices. All
notices and other communications given or made pursuant to this Agreement shall be in writing and shall be deemed effectively given upon the earlier of actual receipt or: (i) personal delivery to the party to be notified; (ii) when sent,
if sent by electronic mail or facsimile during the recipient’s normal business hours, and if not sent during normal business hours, then on the recipient’s next business day; (iii) five (5) days after having been sent by
registered or certified mail, return receipt requested, postage prepaid; or (iv) one (1) business day after the business day of deposit with a nationally recognized overnight courier, freight prepaid, specifying next-day delivery, with
written verification of receipt. All communications shall be sent to the respective parties at their addresses as set forth on Schedule A hereto, or to the principal office of the Company and to the attention of the Chief Executive Officer,
in the case of the Company, or to such email address, facsimile number, or address as subsequently modified by written notice given in accordance with this Section 6.5. 

If notice is given to the Company, a copy 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 the Investors, 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 

and 
 Edwards Wildman Palmer LLP

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

asokol@edwardswildman.com 

  
 23 








 6.6. Amendments and Waivers. Any term of this Agreement may be amended and the observance
of any term of this Agreement may be waived (either generally or in a particular instance, and either retroactively or prospectively) only with the written consent of the Company and the holders of more than the Appropriate Percentage of the
Registrable Securities then outstanding; provided, that the Company may in its sole discretion waive compliance with Section 2.12(c) (and the Company’s failure to object promptly in writing after notification of a proposed
assignment allegedly in violation of Section 2.12(c) shall be deemed to be a waiver); and provided, further, that any provision hereof may be waived by any waiving party on such party’s own behalf, without the consent
of any other party. Notwithstanding the foregoing, this Agreement may not be amended or terminated and the observance of any term hereof may not be waived with respect to any Investor without the written consent of such Investor, unless such
amendment, termination, or waiver applies to all Investors in the same fashion (it being agreed that a waiver of the provisions of Section 4 with respect to a particular transaction shall be deemed to apply to all Investors in the same
fashion if such waiver does so by its terms, notwithstanding the fact that certain Investors may nonetheless, by agreement with the Company, purchase securities in such transaction). Notwithstanding the foregoing, the provisions of
(a) Section 3.3 may not be amended to terminate a designated right therein to appoint an observer to the Board of Directors without the written consent of the Company and at least fifty percent (50%) of the shares of Common
Stock issuable upon conversion of the then outstanding shares of Preferred Stock held by the Persons entitled to appoint such observer, (b) Sections 4.1 and 4.2 may not be waived without the written consent of the holders of more
than seventy percent (70%) of the shares of Common Stock issuable upon conversion of the then outstanding shares of Preferred Stock and (c) Section 5.8 may not be amended or waived without the written consent of the Company and
the holders of ninety percent (90%) of the shares of Common Stock issuable upon conversion of the then outstanding shares of Preferred Stock. The Company shall give prompt notice of any amendment or termination hereof or waiver hereunder to any
party hereto that did not consent in writing to such amendment, termination, or waiver. Any amendment, termination, or waiver effected in accordance with this Section 6.6 shall be binding on all parties hereto, regardless of whether any
such party has consented thereto. No waivers of or exceptions to any term, condition, or provision of this Agreement, in any one or more instances, shall be deemed to be or construed as a further or continuing waiver of any such term, condition, or
provision. 
 6.7. Severability. In case any one or more of the provisions contained in this Agreement is for any reason held to be
invalid, illegal or unenforceable in any respect, such invalidity, illegality, or unenforceability shall not affect any other provision of this Agreement, and such invalid, illegal, or unenforceable provision shall be reformed and construed so that
it will be valid, legal, and enforceable to the maximum extent permitted by law. 
 6.8. Aggregation of Stock. All shares of
Registrable Securities held or acquired by Affiliates shall be aggregated together for the purpose of determining the availability of any rights under this Agreement and such Affiliated persons may apportion such rights as among themselves in any
manner they deem appropriate. 

  
 24 








 6.9. Additional Investors. Notwithstanding anything to the contrary contained herein, if
the Company issues additional shares of the Company’s Preferred Stock after the date hereof, whether pursuant to the Series C Purchase Agreement or otherwise or if any Investor transfers any shares of the Company’s Preferred Stock to any
other Person, any purchaser or new holder of such shares of Preferred Stock may become a party to this Agreement by executing and delivering an additional counterpart signature page to this Agreement, and thereafter shall be deemed an
“Investor” for all purposes hereunder. No action or consent by the Investors shall be required for such joinder to this Agreement by such additional Investor, so long as such additional Investor has agreed in writing to be bound by all of
the obligations as an “Investor” hereunder. 
 6.10. Entire Agreement. This Agreement (including any Schedules and Exhibits
hereto) amends and restates in its entirety the Prior Agreement, constitutes the full and entire understanding and agreement among the parties with respect to the subject matter hereof, and any other written or oral agreement relating to the subject
matter hereof existing between the parties is expressly canceled. 
 6.11. [Reserved]. 

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

  
 25 








 Exhibit 4.4 

IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’ Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	AGECHEM VENTURE FUND L.P.
		
	By:	 	 /s/ Louis Lacasse

		 	Name: Louise Lacasse
		 	Title: President
	
	Address:
	
	 Attn: Louis Lacasse, President
 1
Westmount Square, Suite 800
 Montreal, Quebec, Canada
 H3Z
2P9




  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	ATHENIAN VENTURE PARTNERS III L.P.
		
	By:	 	/s/ Karl O. Elderkin
		 	Name: Karl O. Elderkin
		 	Title: President
	
	AVP OHIO TECHNOLOGY I L.P.
		
	By:	 	/s/ Karl O. Elderkin
		 	Name: Karl O. Elderkin
		 	Title: President
	
	Address:
	
	340 West State Street
	Unit 29/Suite I37D
	Athens OH 45701



  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	BLUE CHIP VALIDATION FUND, LTD.
		
	By:	 	/s/ John McIlwraith
		 	John McIlwraith
		 	Managing Director
	
	Address:
	
	312 Walnut Street
	Suite 1120
	Cincinnati, OH 45202



  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	WILLIAM DALY
		
	By:	 	/s/ William Daly
		 	William Daly
	
	Address:
	
	13 Via Abrazar
	San Clemente, CA 92673



  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	DIANE H. JANUSZ TRUST
		
	By:	 	/s/ John Janusz
		 	Name: John Janusz
		 	Title: Trustee
	
	JOHN JANUSZ
		
	By:	 	/s/ John Janusz
		 	John Janusz
	
	Address:
	
	7385 Desert Spring Court
	West Chester, OH 45069



  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	ALAN FISHMAN
		
	By:	 	/s/ Alan Fishman
		 	Alan Fishman
	
	Address:
	
	6900 Stonehenge Dr.
	Cincinnati, OH 45242-6204



  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	FRED SHALWITZ TRUST,
	ROBERT SHALWITZ, TRUSTEE
		
	By:	 	/s/ Robert Schalwitz
		 	Name: Robert Schalwitz
		 	Title: Trustee
	
	Address:
	
	2549 Bryden Road
	Bexley, OH 43209



  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	GHANA FAMILY TRUST, ELIZABETH C. ARMITAGE TRUSTEE
		
	By:	 	/s/ Elizabeth C. Armitage
		 	Elizabeth C. Armitage
		 	Trustee
	
	Address:
	
	2207 Upland Place
	Cincinnati, OH 45206



  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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
		
	By:	 	/s/ Ian A. W. Howes
		 	Ian A. W. Howes
	
	Address:
	
	219 Stratford Drive
	Chapel Hill, NC 27516



  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	MCILWRAITH INVESTMENTS, LLC
		
	By:	 	/s/ John McIlwraith
		 	Name: John McIlwraith
		 	Title: Manager
	
	Address:
	
	Attn: John McIlwraith
	7680 Foxgate Lane
	Cincinnati, OH 45243



  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 Bio Ventures 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	KEVIN PETERS
		
	By:	 	/s/ Kevin Peters
		 	Kevin Peters
	
	Address:
	
	9160 Given Road
	Cincinnati, OH 45243



  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	ROBERT A. SHALWITZ
		
	By:	 	/s/ Robert A. Shalwitz
	
	Address:
	
	2549 Bryden Road
	Bexley, OH 43209



  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	TRIATHLON MEDICAL VENTURES FUND, L.P.
	
	 By: Triathlon Medical Ventures, LLC

Title: 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

			
	TRI-STATE GROWTH CAPITAL FUND II, L.P.
	
	 By: Tri-State Ventures II, LLC

Title: General Partner

	
	 By: Fort Washington Investment Advisors, Inc.

Title: 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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 IN WITNESS WHEREOF, the parties have executed this Third Amended and Restated Investors’
Rights 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, Ph.D.
		 	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 OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








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

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



  
 SIGNATURE PAGE OF THIRD
AMENDED AND RESTATED 
 INVESTORS’ RIGHTS AGREEMENT 








 Exhibit 4.4 

SCHEDULE A 
 Investors

 Name and Address 
 Triathlon
Medical Ventures Fund, L.P. 
 Attn: John M. Rice 

 Managing Partner 
 300 E-Business Way, Suite
200 
 Cincinnati, OH 45241 
 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 

Venture Investors Early Stage Fund IV Limited Partnership 
 Attn:
Paul Weiss 
  Managing Director 
 505
South Rosa Road 
 Madison, WI 53719-1262 

  
 Schedule A-1 








 Kearny Venture Partners, L.P. 

Attn: Anupam Dalal 
 Kearny Venture Associates, LLC 

88 Kearny Street, Suite 200 
 San Francisco, CA 94108-5530 

Kearny Venture Partners Entrepreneurs Fund, L.P. 
 Attn: Anupam
Dalal 
 Kearny Venture Associates, LLC 
 88 Kearny Street,
Suite 200 
 San Francisco, CA 94108-5530 
 Thomas Weisel
Healthcare Venture Partners, L.P. 
 88 Kearny Street, 4th Floor 

San Francisco, CA 94108 
 The Procter & Gamble Company

 Attn: David Le Neveu 
  Director,
Corporate Acquisitions, Divestitures and Equity Ventures 
 1 Procter & Gamble Plaza 

Cincinnati, OH 45202 
 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 
 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 

Sigvion Fund I, LP 
 Attn: J. P. Fairbank 

 Founding Partner 
 738 W. Belden Avenue 

Chicago, IL 60614 

  
 Schedule A-2 








 Cincinnati Cornerstone Investors AKB, LLC 

Attn: Robert W. Coy, Jr. 
  President 

30 West 3rd Street, 6th Floor 

Cincinnati, OH 45202-3559 
 Tri-State Growth Capital Fund II,
L.P. 
 Attn: Steve Baker 
 303 Broadway, Suite 1200 

Cincinnati, OH 45202 
 Blue Chip Validation Fund, Ltd. 

Attn: John McIlwraith 
   Managing
Director 
 1100 Chiquita Center 
 250 East Fifth Street 

Cincinnati, OH 45202 
 QCA First Fund II 

Attn: John Habbert 
 1776 Mentor Avenue, MB #302 

Cincinnati, OH 45212 
 Gitana Family Trust, Elizabeth C. Armitage
Trustee 
 Attn: Elizabeth C. Armitage 

 Trustee 
 2207 Upland Place 

Cincinnati, OH 45206 
 Robert Shalwitz 

2549 Bryden Road 
 Bexley, OH 43209 

Fred Shalwitz Trust, Robert Shalwitz, Trustee 
 Attn: Robert
Shalwitz 
  Trustee 
 2549 Bryden Road

 Bexley, OH 43209 
 Joseph H. Gardner 

4060 Boomer Road 
 Cincinnati, OH 45247 

  
 Schedule A-3 








 Gardner Family Trust, John D. Gardner Trustee 

Attn: John D. Gardner 
  Trustee 

111 Pine Court 
 Bastop, TX 78602 

Ian A. W. Howes, IRA, Sterling Trust Custodian 
 Attn: Ian A. W.
Howes 
  Trustee 
 219 Stratford Drive

 Chapel Hill, NC 27516 
 Ian A. W. Howes 

219 Stratford Drive 
 Chapel Hill, NC 27516 

Kevin Peters 
 9160 Given Road 

Cincinnati, OH 45243 
 William Daly 

13 Via Abrazar 
 San Clemente, CA 92673 

Muneer A. Satter Revocable Trust 
 c/o Satter Investment
Management, LLC 
 676 N. Michigan Avenue, Suite 4000 
 Chicago,
IL 60611 
 Attn: Muneer A. Satter 
 John Wood Trust 

c/o Satter Investment Management, LLC 
 676 N. Michigan Avenue,
Suite 4000 
 Chicago, IL 60611 
 Attn: Muneer A. Satter 

The Satter Foundation 
 c/o Satter Investment Management, LLC

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

Attn: Muneer A. Satter 
 Muneer A Satter IRA, Millennium Trust
Company, Custodian 

  
 Schedule A-4 








 Satter Children’s Trust I 

c/o Satter Investment Management, LLC 
 676 N. Michigan Avenue,
Suite 4000 
 Chicago, IL 60611 
 Attn: Muneer A. Satter 

Kristen Hayler Hertel Revocable Trust 
 c/o Satter Investment
Management, LLC 
 676 N. Michigan Avenue, Suite 4000 
 Chicago,
IL 60611 
 Attn: Muneer A. Satter 
 Anne-Carole Witort
Insurance Trust 
 c/o Satter Investment Management, LLC 
 676
N. Michigan Avenue, Suite 4000 
 Chicago, IL 60611 
 Attn:
Muneer A. Satter 
 Rose Shereen Fuqua Insurance Trust 
 c/o
Satter Investment Management, LLC 
 676 N. Michigan Avenue, Suite 4000 

Chicago, IL 60611 
 Attn: Muneer A. Satter 

Rabi H. Satter Insurance Trust 
 c/o Satter Investment
Management, LLC 
 676 N. Michigan Avenue, Suite 4000 
 Chicago,
IL 60611 
 Abdus Satter Insurance Trust 
 c/o Satter
Investment Management, LLC 
 676 N. Michigan Avenue, Suite 4000 

Chicago, IL 60611 
 Attn: Muneer A. Satter 

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 

  
 Schedule A-5 








 Satter Family Trust 

c/o Satter Investment Management, LLC 
 676 N. Michigan Avenue,
Suite 4000 
 Chicago, IL 60611 
 Attn: Muneer A. Satter 

AgeChem Venture Fund L.P. 
 Attn: Louis Lacasse 

 President 
 1 Westmount Square, Suite 800

 Montreal, Quebec, Canada 
 H3Z 2P9 

Diane H. Janusz Trust 
 Attn: John Janusz 

 Trustee 
 7385 Desert Spring Court 

West Chester, OH 45069 
 John Janusz 

7385 Desert Spring Court 
 West Chester, OH 45069 

MRK International, LLC 
 Attn: Richard L. Kiley 

 Principal Member 
 7800 Tecumseh Trail 

Cincinnati, OH 45243 
 McIlwraith Investments, LLC 

Attn: John McIlwraith 
  Manager 

7680 Foxgate Lane 
 Cincinnati, OH 45243 

John H. Wyant 
 Blue Chip Venture Company 

1120 Scripps Center 
 312 Walnut Street 

Cincinnati, OH 45202 
 Alan Fishman 

6900 Stonehenge Drive 
 Cincinnati, OH 45242-6204 

  
 Schedule A-6 








 Family Akebia Investments LLC 

1185 Park Avenue #11G 
 New York, NY 10128 

Novo A/S Novo Ventures 
 Tuborg Havnevej 19 

DK - 2900 - Hellerup 

  
 Schedule A-7