EX-4.7




 Exhibit 4.7 
  

 AMENDMENT NUMBER ONE TO 

SECOND AMENDED AND RESTATED 

RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 

THIS AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT
(the “Amendment”) is made and effective as of July 9, 2012, by and among AKEBIA THERAPEUTICS, INC., a Delaware corporation (the “Company”), and those Investors executing and delivering a counterpart signature
page hereto. Capitalized terms not defined herein have the meanings given them in that certain Second Amended and Restated Right of First Refusal and Co-Sale Agreement, dated as of April 6, 2011, by and among the Company, the Investors and the
Major Holders (as amended, the “Agreement”). 
 WHEREAS, the Company and the Investors desire to amend the Agreement to
modify the definition of Preferred Stock and add a definition for the Series X Preferred Stock of the Company; and 
 WHEREAS, the Investors
hold more than the Appropriate Percentage of the shares of Common Stock required to amend the Agreement pursuant to the provisions of Section 6.8 of the Agreement; 

NOW, THEREFORE, in consideration of the foregoing recitals and the mutual promises hereinafter set forth and set forth in the Agreement, the
parties hereby agree as follows: 
 1. Amendment. 

(a) The definition of “Preferred Stock” shall be deleted in its entirety and replaced with the following: “‘Preferred
Stock’ means all shares of Series A Preferred Stock, Series B Preferred Stock, and Series X Preferred Stock.” 
 (b) A new
definition of “Series X Preferred Stock” shall be added to the Agreement and shall read as follows: ‘”Series X Preferred Stock’ shall mean shares of the Series X Preferred Stock of the Company, par value $0.00001 per
share.” 
 2. Miscellaneous Amendments. The Agreement is amended hereby so that any reference therein to the Agreement shall be
deemed to be a reference to the Agreement as amended by this Amendment. 
 3. Continuance of Agreement. Except as specifically
amended by this Amendment, the Agreement shall remain in full force and effect. 
 4. Governing Law. The laws of the State of
Delaware govern all matters arising out of or relating to this Amendment, including, without limitation, its interpretation, construction, performance, and enforcement, without giving effect to such state’s conflicts of law principles or rules
of construction concerning the drafter hereof. 







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

[Signature Page Follows] 

  
 2 








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

			
	AKEBIA THERAPEUTICS, INC.
		
	By:	 	 /s/ Joseph H. Gardner

		 	Joseph H. Gardner, Ph.D.
		 	President and Chief Executive Officer
	
	Address:
	
	Suite 420,
	9987 Carver Road,
	Cincinnati, OH 45242



  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	JOSEPH H. GARDNER
		
	By:	 	 /s/ JOSEPH H. GARDNER

	
	Address:
	
	4060 Boomer Road
	Cincinnati, OH 45247



  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	NOVARTIS BIOVENTURES LTD.
		
	By:	 	 /s/    H. S.
Zivi        

	Name:	 	 H. S. Zivi

	Title:	 	 Deputy Chairman

		
	By:	 	 /s/    Rebecca
White        

	Name:	 	 Rebecca White

	Title:	 	 Authorized Signatory

	
	Address:
	
	NOVARTIS BIOVENTURES LTD.
	Attn: Henri Simon Zivi
	131 Front Street
	Hamilton HM 12
	Bermuda
	
	But for mail, to:
	Novartis BioVentures Ltd.
	Attn: Henri Simon Zivi
	PO Box HM 2899
	Hamilton HM LX Bermuda
	
	And, also send a copy to:
	Novartis Venture Fund
	Attn: Campbell Murray
	Five Cambridge Center, Suite 603
	Cambridge, MA 02142



  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	VENTURE INVESTORS EARLY STAGE FUND IV LIMITED PARTNERSHIP
		
	By:	 	VIESF IV GP, LLC, its General Partner
		
	By:	 	 /s/ Paul M. Weiss

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



  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	TRIATHLON MEDICAL VENTURES FUND, L.P.
		
	By:	 	Triathlon Medical Ventures, LLC
	Its:	 	General Partner
		
	By:	 	 /s/ John M. Rice

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

	Cincinnati, OH 45241



  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








 IN WITNESS WHEREOF, the parties hereto have caused this Amendment to be executed 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 1800
	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 1800
	San Francisco, CA 94108-5530



  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








 IN WITNESS WHEREOF, the parties hereto have caused this Amendment to be executed 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, Suite 1800
	San Francisco, CA 94108



  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	ATHENIAN VENTURE PARTNERS III L.P.
		
		 	/s/ Karl Elderkin
	By:	 	 Karl Elderkin, President

	Name:	 	Athenian III, Ltd
	Title:	 	General Partner
	
	Address:
	
	20 East Circle Drive, #37146
	Suite 229
	Athens, OH 45701


 
			
	
	AVP OHIO TECHNOLOGY I L.P.
		
	By:	 	AVP Ohio I, Ltd.
	Its:	 	General Partner
		
	By:	 	 /s/ Karl O. Elderkin

		 	Karl O. Elderkin
		 	President
	
	Address:
	
	20 East Circle Drive, #37146
	Suite 229
	Athens, OH 45701



  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	CINCYTECH FUND I, LLC
		
	By:	 	 /s/ Robert W. Coy, Jr

		 	Robert W. Coy, Jr
		 	President
	
	Address:
	
	30 West 3rd Street, 6th Floor
	Cincinnati, OH 45202-3559



  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	GITANA FAMILY TRUST, ELIZABETH C. ARMITAGE TRUSTEE
		
	By:	 	 /s/ Elizabeth C. Armitage

		 	Elizabeth C. Armitage
		 	Trustee
	
	Address:
	
	2207 Upland Place
	Cincinnati, OH 45206



  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	TRI-STATE GROWTH CAPITAL FUND II, L.P.
		
	By:	 	Tri-State Ventures II, LLC
	Its:	 	General Partner
		
	By:	 	Fort Washington Investment Advisors, Inc.
	Its:	 	Managing Member
		
	By:	 	 /s/ Christopher L. Baucom

		 	Christopher L. Baucom
		 	Managing Director
		
	By:	 	 /s/ Maribeth S. Rahe

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

Cincinnati, OH 45202




  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	SIGVION FUND I, LP
		
	By:	 	 /s/ J.P. Fairbank

		 	J.P. Fairbank
		 	Founding Partner
	
	Address:
	
	 1970 N HALSTED ST, 3RD FLOOR

CHICAGO, IL 60614




  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	GARDNER FAMILY TRUST, JOHN D. GARDNER TRUSTEE
		
	By:	 	 /s/ John D. Gardner

		 	John D. Gardner
		 	Trustee
	
	Address:
	
	 111 Pine Court
 Bastop, TX
78602




  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








 IN WITNESS WHEREOF, the parties hereto have caused this Amendment to be executed 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
		
	By:	 	 /s/ Muneer A. Satter

	Name:	 	Muneer A. Satter
	Title:	 	Investment Advisor
	
	 Address:
 419 Sheridan Road

Winnetka, IL 60093
 Attn: Muneer A. Satter




  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








 IN WITNESS WHEREOF, the parties hereto have caused this Amendment to be executed 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:	 	  

	
	 Address:
 219 Stratford Dr.

Chapel Hill, NC 27516




  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	DIANE H. JANUSZ TRUST
		
	By:	 	 /s/ John Janusz

	Name:	 	John Janusz
	Title:	 	Trustee
	
	 Address:
  

7385 Desert Spring Ct.
 West Chester, OH 45069




  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	AGECHEM VENTURE FUND L.P.
		
	By:	 	AgeChem Financial Inc., its General Partner
		
	By:	 	 /s/ Louis Lacasse

	Name:	 	Louis Lacasse
	Title:	 	President
	
	 Address:
  

1 Westmount Square, Suite 800
 Montreal, Quebec, Canada

H3Z 2P9




  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

	
	 /s/ Kevin Peters

	Kevin Peters
	
	 Address:
 6100 Miami Rd.

Cincinnati, OH 48243




  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	BLUE CHIP VALIDATION FUND, LTD.
		
	By:	 	 /s/ John McIlwraith

	Name:	 	John McIlwraith
	Title:	 	Managing Director
	
	 Address:
  

1100 Chiquita Center
 250 East Fifth Street

Cincinnati, OH 45202




  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	QCA FIRST FUND II
		
	By:	 	 /s/ John Habbert

	Name:	 	John Habbert
	Title:	 	 Manager

	
	 Address:
 6393 Grand Vista
Avenue
 Cincinnati, OH 45213




  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

	
	 /s/ Robert Shalwitz

	Robert Shalwitz
	
	 Address:
  

2549 Bryden Road
 Bexley, OH 43209




  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT 








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

			
	 FRED SHALWITZ TRUST,
 ROBERT
SHALWITZ, TRUSTEE

		
	By:	 	 /s/ Robert Shalwitz

	Name:	 	Robert Shalwitz
	Title:	 	Trustee
	
	 Address:
  

2549 Bryden Road
 Bexley, OH 43209




  
 SIGNATURE PAGE TO
AMENDMENT NUMBER ONE TO SECOND AMENDED AND RESTATED RIGHT OF FIRST REFUSAL AND CO-SALE AGREEMENT