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

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

SIGNATURE PAGE TO 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.

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

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

SIGNATURE PAGE TO 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/ Xxxx X. Xxxxx
Name:		Xxxx X. Xxxxx, PhD
Title:		Managing Director
Address:
000 Xxxxx Xxxx Xxxx
Xxxxxxx, XX 00000-0000
Attn: Xxxx Xxxxx, Managing Director
Phone: (000) 000-0000
Fax: (000) 000-0000
Email: xxxx@xxxxxxxxxxxxxxxx.xxx

SIGNATURE PAGE TO 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/ Xxxx X. Xxxx
		Xxxx X. Xxxx
		Managing Partner
Address:
000 X-Xxxxxxxx Xxx Xxxxx 000
Xxxxxxxxxx, XX 00000

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

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.

XXXXXX XXXXXX HEALTHCARE VENTURE PARTNERS, L.P.
By:		Xxxxxx Xxxxxx Capital Management LLC
Title:		its Managing Member
By:		/s/ Xxxxxx Xxxxx
Name:		Xxxxxx Xxxxx
Title:		Managing Director
Address:
00 Xxxxxx Xxxxxx, Xxxxx 0000
Xxx Xxxxxxxxx, XX 00000

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/ Xxxx Xxxxxxxx
By:		Xxxx Xxxxxxxx, President
Name:		Athenian III, Ltd
Title:		General Partner
Address:
00 Xxxx Xxxxxx Xxxxx, #00000
Xxxxx 000
Xxxxxx, XX 00000

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

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/ Xxxxxx X. Xxx, Xx
		Xxxxxx X. Xxx, Xx
		President
Address:
00 Xxxx 0xx Xxxxxx, 0xx Xxxxx
Xxxxxxxxxx, XX 00000-0000

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

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/ Xxxxxxxxxxx X. Xxxxxx
		Xxxxxxxxxxx X. Xxxxxx
		Managing Director
By:		/s/ Xxxxxxxx X. Xxxx
		Xxxxxxxx X. Xxxx
		President and Chief Executive Officer
Address:
000 Xxxxxxxx, Xxxxx 0000 Xxxxxxxxxx, XX 00000

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/ X.X. Xxxxxxxx
		X.X. Xxxxxxxx
		Founding Partner
Address:
0000 X XXXXXXX XX, 0XX XXXXX XXXXXXX, XX 00000

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.

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

SIGNATURE PAGE TO 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.

XXXXXX X. XXXXXX REVOCABLE TRUST
By:		/s/ Xxxxxx X. Xxxxxx
Name:		Xxxxxx X. Xxxxxx
Title:		Trustee
THE XXXXXX FOUNDATION
By:		/s/ Xxxxxx X. Xxxxxx
Name:		Xxxxxx X. Xxxxxx
Title:		Trustee
XXXXXX FAMILY TRUST
By:		/s/ Xxxxxx X. Xxxxxx
Name:		Xxxxxx X. Xxxxxx
Title:		Investment Advisor
XXXXXX CHILDREN’S TRUST
By:		/s/ Xxxxxx X. Xxxxxx
Name:		Xxxxxx X. Xxxxxx
Title:		Investment Advisor
Address: 000 Xxxxxxxx Xxxx Xxxxxxxx, XX 00000 Attn: Xxxxxx X. Xxxxxx

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

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.

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

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/ Xxxxx Xxxxxxx
Name:		Xxxxx Xxxxxxx
Title:		President
Address: 0 Xxxxxxxxx Xxxxxx, Xxxxx 000 Xxxxxxxx, Xxxxxx, Xxxxxx X0X 0X0

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/ Xxxxx Xxxxxx

Xxxxx Xxxxxx

Address:

0000 Xxxxx Xx.

Xxxxxxxxxx, XX 00000

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/ Xxxx XxXxxxxxxx
Name:		Xxxx XxXxxxxxxx
Title:		Managing Director
Address: 1100 Xxxxxxxx Center 000 Xxxx Xxxxx Xxxxxx Xxxxxxxxxx, XX 00000

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/ Xxxx Xxxxxxx
Name:		Xxxx Xxxxxxx
Title:		Manager
Address: 0000 Xxxxx Xxxxx Xxxxxx Xxxxxxxxxx, XX 00000

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/ Xxxxxx Xxxxxxxx

Xxxxxx Xxxxxxxx

Address:

0000 Xxxxxx Xxxx

Xxxxxx, XX 00000

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.

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

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