Document:

EX-4.3

 Exhibit 4.3 
 BERKSHIRE HATHAWAY FINANCE CORPORATION 
 OFFICERS’ CERTIFICATE

 ESTABLISHING THE TERMS OF THE 
 2.000% SENIOR NOTES DUE 2018 
 August 15, 2013 

The undersigned, Marc D. Hamburg and Robert P. Reeson, do hereby certify pursuant to Section 3.01 of that certain Indenture, dated
as of February 1, 2010 (the “Indenture”), among Berkshire Hathaway Finance Corporation (the “Corporation”), Berkshire Hathaway Inc., as Guarantor, and The Bank of New York Mellon Trust Company, N.A., as
trustee: 
 1. They are (i) the President and (ii) the Assistant Secretary, respectively, of the Corporation.

 2. As such officers, they are authorized to execute and deliver this Officers’ Certificate on behalf of the Corporation.

 3. Attached hereto as Annex A is a true and correct copy of a specimen note representing the Corporation’s 2.000% Senior
Notes due 2018 (the “Notes”). 
 4. The Notes are a separate series of Securities under the Indenture. The form
of Notes attached hereto as Annex A is incorporated herein by reference. 
 5. The title of the Notes shall be the “2.000%
Senior Notes due 2018”. The Notes will be the Corporation’s unsecured senior obligations, will rank pari passu in right of payment with all of the Corporation’s unsubordinated, unsecured indebtedness and will be senior in right
of payment to all of the Corporation’s subordinated indebtedness. 
 6. The Notes shall be issued at the initial offering
price of 99.820% of the principal amount thereof. 
 7. The Corporation will initially issue $400,000,000 aggregate principal
amount of Notes. The Corporation may issue additional Notes from time to time after the date hereof, and such Notes will be treated as part of the same series of which the Notes are a part for all purposes under the Indenture. 

8. All of the Corporation’s obligations under the Notes will be unconditionally and irrevocably guaranteed by Berkshire Hathaway
Inc., as Guarantor. The form of Guarantee is attached to the specimen Note attached hereto as Annex A, and is incorporated herein by reference. 
 9. The principal amount of the Notes will mature on August 15, 2018. 
 10.
The Notes are issuable in minimum denominations of $2,000 and integral multiples of $1,000 in excess thereof. 

 11. Interest on the Notes will be computed on the basis of a 360 day year of twelve 30-day
months. 
 12. The Notes will bear interest from August 15, 2013 at the rate of 2.000% per annum, payable on each
February 15 and August 15, commencing on February 15, 2014, to the holders of record of the Notes at the close of business on the February 1 or August 1 (whether or not a Business Day), as the case may be, immediately
preceding such February 15 or August 15. 
 13. Payment of the principal of and premium, if any, and interest on the
Notes will be made at the office or agency of the Corporation maintained for that purpose in the City of New York, New York (or, if the Corporation does not maintain such office or agency, at the corporate trust office of the Trustee in the City of
New York or if the Trustee does not maintain an office in the City of New York, at the office of a Paying Agent in the City of New York), in such coin or currency of the United States of America as at the time of payment is legal tender for payment
of public and private debt; provided, however, that at the option of the Corporation payments of principal, premium, if any, or interest may be made by check mailed to the address of the Person entitled thereto as such address shall appear in the
Security Register, subject to surrender at such office or agency, in the case of payments of principal and premium. 
 14. The
Notes will initially be issued in the form of one or more Global Securities. The Depository Trust Company shall serve as the Depositary for such Global Securities. 
 15. The Notes shall be defeasible in whole or in part pursuant to the terms of the Indenture, including, without limitation, Section 13.02 and Section 13.03 of the Indenture. 

16. The Notes may be redeemed in whole or in part pursuant to the terms set forth in the form of Notes incorporated herein by reference.
Notwithstanding Section 11.04 of the Indenture, notice of such redemption need not set forth the Redemption Price, but only the manner of calculation thereof. The Corporation shall give the Trustee notice of such Redemption Price promptly after
the calculation thereof and the Trustee shall have no responsibility for such calculation. 
 All capitalized terms used herein
and not otherwise defined shall have the meanings given such terms in the Indenture. 
 [Remainder of page intentionally left
blank.] 

  
 - 2 -

 IN WITNESS WHEREOF, this Officers’ Certificate has been executed by the undersigned on
the Corporation’s behalf as of the date first written above. 
  

	
	
	/s/ Marc D. Hamburg
	 Name: Marc D. Hamburg

Title:   President

  

	
	
	/s/ Robert P. Reeson
	 Name: Robert P. Reeson

Title:   Assistant Secretary

 [Officer’s Certificate Establishing 2.000% Notes] 

 Annex A 
 Form of 2.000% Senior Notes due 2018 

 THIS DEBT SECURITY IS A GLOBAL SECURITY WITHIN THE MEANING OF THE INDENTURE HEREINAFTER REFERRED TO AND IS
REGISTERED IN THE NAME OF A DEPOSITARY OR A NOMINEE THEREOF. THIS DEBT SECURITY MAY NOT BE EXCHANGED IN WHOLE OR IN PART FOR A SECURITY REGISTERED, AND NO TRANSFER OF THIS DEBT SECURITY IN WHOLE OR IN PART MAY BE REGISTERED, IN THE NAME OF ANY
PERSON OTHER THAN SUCH DEPOSITARY OR A NOMINEE THEREOF, EXCEPT IN THE LIMITED CIRCUMSTANCES DESCRIBED IN THE INDENTURE. 
 UNLESS THIS
CERTIFICATE IS PRESENTED BY AN AUTHORIZED REPRESENTATIVE OF THE DEPOSITORY TRUST COMPANY, A NEW YORK CORPORATION (“DTC”), TO BERKSHIRE HATHAWAY FINANCE CORPORATION OR ITS AGENT FOR REGISTRATION OR TRANSFER, EXCHANGE, OR PAYMENT, AND ANY
CERTIFICATE ISSUED IS REGISTERED IN THE NAME OF CEDE & CO. OR IN SUCH OTHER NAME AS IS REQUESTED BY AN AUTHORIZED REPRESENTATIVE OF DTC (AND ANY PAYMENT IS MADE TO CEDE & CO. OR TO SUCH OTHER ENTITY AS IS REQUESTED BY AN AUTHORIZED
REPRESENTATIVE OF DTC), ANY TRANSFER, PLEDGE, OR OTHER USE HEREOF FOR VALUE OR OTHERWISE BY OR TO ANY PERSON IS WRONGFUL INASMUCH AS THE REGISTERED OWNER HEREOF, CEDE & CO., HAS AN INTEREST HEREIN. 

 BERKSHIRE HATHAWAY FINANCE CORPORATION 

 
  

2.000% Senior Notes due 2018 
 CUSIP: 084664BY6 
 ISIN: US084664BY67

  

			
	 No.
	  	$                

 (as revised by the Schedule of Increases and 

Decreases in Global Security attached hereto) 
 BERKSHIRE HATHAWAY FINANCE CORPORATION, a corporation duly organized and existing under the laws of the State of Delaware (herein called the “Company”, which term includes any successor Person
under the Indenture hereinafter referred to), for value received, hereby promises to pay to CEDE & CO., the registered Holder hereof, the principal sum of
                     Dollars ($            ) (as revised by the Schedule of
Increases and Decreases in Global Security attached hereto) on August 15, 2018, and to pay interest thereon from and including August 15, 2013 or from and including the most recent Interest Payment Date (as defined below) to which interest
has been paid or duly provided for, semi-annually on February 15 and August 15 in each year, commencing February 15, 2014 (each an “Interest Payment Date”), at the rate of 2.000% per annum (as adjusted, if at all,
pursuant to such Indenture, the “Interest Rate”), until the principal hereof is paid or made available for payment; provided that any principal, and any such installment of interest, which is overdue shall bear interest at the
Interest Rate (to the extent that the payment of such interest shall be legally enforceable), from the dates such amounts are due until they are paid or made available for payment, and such interest shall be payable on demand. The interest so
payable, and punctually paid or duly provided for, on any Interest Payment Date will, as provided in such Indenture, be paid to the Person in whose name this Debt Security (or one or more Predecessor Securities) is registered at the close of
business on the Regular Record Date (whether or not a Business Day) for such interest. Any such interest not so punctually paid or duly provided for will forthwith cease to be payable to the Holder on such Regular Record Date and may either be paid
to the Person in whose name this Debt Security (or one or more Predecessor Securities) is registered at the close of business on a Special Record Date for the payment of such Defaulted Interest to be fixed by the Trustee, notice whereof shall be
given to Holders of Debt Securities of this series not less than 10 days prior to such Special Record Date, or be paid at any time in any other lawful manner not inconsistent with the requirements of any securities exchange on which the Debt
Securities of this series may be listed, and upon such notice as may be required by such exchange, all as more fully provided in such Indenture. 
 Payment of the principal of and premium, if any, and interest on this Debt Security will be made at the office or agency of the Company maintained for that purpose in the City of New York, New York (or,
if the Company does not maintain such office or agency, at the corporate trust office of the Trustee in the City of New York or if the Trustee does not maintain an office in the City of New York, at the office of a Paying Agent in the City of New
York), in such coin or currency of the United States of America as at the time of payment is legal tender for payment of public and private debt; provided, however, that at the option of the Company payments of principal, premium or interest
may be made by check mailed to the address of the Person entitled thereto as such address shall appear in the Security Register, subject to surrender at such office or agency, in the case of payments of principal or premium. 

 This Debt Security may be redeemed, in whole or in part, at the option of the Company, at
any time prior to its maturity at a redemption price equal to the greater of (A) 100% of the principal amount to be redeemed or (B) as determined by the Quotation Agent, the sum of the present values of the remaining scheduled payments of
principal and interest on the portion of this Debt Security being redeemed, not including any portion of such payments of interest accrued as of the date fixed for redemption, discounted to the date fixed for redemption on a semi-annual basis
assuming a 360-day year consisting of twelve 30-day months, at the Adjusted Treasury Rate plus ten (10) basis points, plus accrued interest on the portion of this Debt Security being redeemed to the date fixed for redemption. 

In the event of redemption of this Debt Security in part only, a new Debt Security or Debt Securities of this series and of like tenor
for the unredeemed portion hereof will be issued in the name of the Holder hereof upon the cancellation hereof. 
 The Quotation
Agent will select a Comparable Treasury Issue, and the Reference Dealers will provide the Company and the Trustee with the Reference Dealer Quotations. The Company will calculate the Comparable Treasury Price. 

“Adjusted Treasury Rate” means, for any date fixed for redemption, the rate per year equal to the semi-annual equivalent yield
to maturity of the Comparable Treasury Issue assuming a price for the Comparable Treasury Issue equal to the Comparable Treasury Price for the date fixed for redemption, in each case expressed as a percentage of its principal amount. 

“Comparable Treasury Issue” means, for any date fixed for redemption, the U.S. Treasury security selected by the Quotation
Agent which has a maturity comparable to the remaining maturity of this Debt Security as of the date fixed for redemption, which would be used in accordance with customary financial practice to price new issues of corporate debt securities with a
maturity comparable to the remaining maturity of this Debt Security as of the date fixed for redemption. 
 “Comparable
Treasury Price” means, for any Comparable Treasury Issue, the price after eliminating the highest and the lowest Reference Dealer Quotations and then calculating the average of the remaining Reference Dealer Quotations; provided,
however, that if the Company obtains fewer than three Reference Dealer Quotations, the Company will, when calculating the Comparable Treasury Price, calculate the average of all the Reference Dealer Quotations and not eliminate any such
quotations. 
 “Quotation Agent” means Merrill Lynch, Pierce, Fenner & Smith Incorporated or its successor.

 “Reference Dealers” means Goldman, Sachs & Co., Merrill Lynch, Pierce, Fenner & Smith
Incorporated and a primary U.S. government securities dealer in the City of New York selected by Wells Fargo Securities, LLC or their respective successors, and one or more other primary U.S. Government securities dealers in the City of New York
appointed by the Company, provided, however, that if any of the foregoing or their respective successors ceases to be a primary U.S. Government securities dealer in the City of New York, the Company will appoint another primary U.S. Government
securities dealer in the City of New York as a substitute. 
 “Reference Dealer Quotations” means, for any Comparable
Treasury Issue, the average of the bid and asked prices for such Comparable Treasury Issue (expressed in each case as a percentage of its principal amount) quoted in writing by the Reference Dealers to the Company and the Trustee as of 5:00 p.m.
(New York City Time) on the third business day before the relevant date fixed for redemption. 

 “Regular Record Date” means, with respect to any Interest Payment Date, the
February 1 or August 1 (whether or not a Business Day), as the case may be, immediately preceding such Interest Payment Date. 
 The Company may elect to effect a redemption in accordance with these provisions at any time and on any date. However, the Company must give the Holders of this Debt Security notice, as provided in the
Indenture, of the redemption not less than 30 days or more than 60 days before the date fixed for redemption. If the Company elects to redeem fewer than all the Debt Securities of this series, the Trustee will select the particular Debt Securities
of this series to be redeemed by such method that the Trustee deems fair and appropriate. 
 Reference is hereby made to the
further provisions of this Debt Security set forth on the reverse hereof, which further provisions shall for all purposes have the same effect as if set forth at this place. 
 Unless the certificate of authentication hereon has been executed by the Trustee referred to on the reverse hereof by manual signature, this Debt Security shall not be entitled to any benefit under the
Indenture or be valid or obligatory for any purpose. 

 IN WITNESS WHEREOF, the Company has caused this instrument to be duly executed. 

 

							
	Dated:	 		 	 BERKSHIRE HATHAWAY FINANCE
 CORPORATION

				
		 		 	By:	 	 
		 		 	Name: Marc D. Hamburg
		 		 	Title: President

 Attest: 

	
	
	  
	 Name: Robert P. Reeson

Title: Assistant Secretary

 [REVERSE OF DEBT SECURITY] 

This Debt Security is one of a duly authorized series of notes of the Company (herein called the “Debt Securities”), issued and
to be issued in one or more series under an Indenture, dated as of February 1, 2010 (herein called the “Base Indenture”, and as supplemented by the Officers’ Certificate dated August 15, 2013 with respect to this Debt
Security, together with the Base Indenture, called the “Indenture”), among the Company, as issuer, Berkshire Hathaway Inc., as guarantor (herein called the “Guarantor,” which term shall include any successor Guarantor under the
Indenture), and The Bank of New York Mellon Trust Company, N.A., as Trustee (herein called the “Trustee”, which term includes any successor trustee under the Indenture), and reference is hereby made to the Indenture for a statement of the
respective rights, limitations of rights, duties and immunities thereunder of the Company, the Guarantor, the Trustee and the Holders of the Debt Securities and of the terms upon which the Debt Securities are, and are to be, authenticated and
delivered. This Debt Security is one of the series designated on the face hereof, initially limited in aggregate principal amount to $400,000,000. The Company may at any time issue additional securities under the Indenture in unlimited amounts
having the same terms as the Debt Securities of a series, provided that no additional securities of a series may be issued if at the time of issuance an Event of Default has occurred and is continuing with respect to such series of securities.

 This Debt Security does not have the benefit of any sinking fund obligation. 

In the event of redemption of this Debt Security in part only, a new Debt Security or Debt Securities of this series and of like tenor
for the unredeemed portion hereof will be issued in the name of the Holder hereof upon the cancellation hereof. 
 The Indenture
contains provisions for defeasance at any time of the entire Indebtedness of this Debt Security or of certain restrictive covenants and Events of Default with respect to this Debt Security, in each case upon compliance with certain conditions set
forth in the Indenture. 
 If an Event of Default with respect to the Debt Securities of this series shall occur and be
continuing, the principal of the Debt Securities of this series may be declared due and payable in the manner and with the effect provided in the Indenture. 
 The Indenture permits, with certain exceptions as therein provided, the amendment thereof and the modification of the rights and obligations of the Company and the Guarantor and the rights of the Holders
of the Debt Securities of each series and of Guarantees to be affected under the Indenture at any time by the Company, the Guarantor and the Trustee with the consent of the Holders of not less than 50% in principal amount of the Debt Securities at
the time Outstanding of each series to be affected (voting together as a single class). The Indenture also contains provisions permitting the Holders of specified percentages in principal amount of the Debt Securities of each series at the time
Outstanding, on behalf of the Holders of all Debt Securities of such series, to waive compliance by the Company and/or the Guarantor with certain provisions of the Indenture and certain past defaults under the Indenture and their consequences. Any
such consent or waiver by the Holder of this Debt Security shall be conclusive and binding upon such Holder and upon all future Holders of this Debt Security and of any Debt Security issued upon the registration of transfer hereof or in exchange
herefor or in lieu hereof, whether or not notation of such consent or waiver is made upon this Debt Security. 

 As provided in and subject to the provisions of the Indenture, the Holder of this Debt
Security shall not have the right to institute any proceeding with respect to the Indenture or for the appointment of a receiver or trustee or for any other remedy thereunder, unless such Holder shall have previously given the Trustee written notice
of a continuing Event of Default with respect to the Debt Securities of this series, the Holders of at least 25% in principal amount of the Debt Securities of this series at the time Outstanding shall have made written request to the Trustee to
institute proceedings in respect of such Event of Default as Trustee and offered the Trustee indemnity or security reasonably satisfactory to it, and the Trustee shall not have received from the Holders of a majority in principal amount of Debt
Securities of this series at the time Outstanding a direction inconsistent with such request, and shall have failed to institute any such proceeding, for 60 days after receipt of such notice, request and offer of indemnity. The foregoing shall not
apply to any suit instituted by the Holder of this Debt Security for the enforcement of any payment of principal hereof or any premium or interest hereon on or after the respective due dates expressed herein. 

No reference herein to the Indenture and no provision of this Debt Security or of the Indenture shall alter or impair the obligation of
the Company, which is absolute and unconditional, to pay the principal of and any interest on this Debt Security at the times, place and rate, and in the coin or currency, herein prescribed. 

As provided in the Indenture and subject to certain limitations therein set forth, the transfer of this Debt Security is registrable in
the Security Register, upon surrender of this Debt Security for registration of transfer at the office or agency of the Company in any place where the principal of and any premium and interest on this Debt Security are payable, duly endorsed by, or
accompanied by a written instrument of transfer in form satisfactory to the Company and the Security Registrar duly executed by, the Holder hereof or the Holder’s attorney duly authorized in writing, and thereupon one or more new Debt
Securities of this series and of like tenor, of authorized denominations and for the same aggregate principal amount, will be issued to the designated transferee or transferees. 

The Indenture and this Debt Security are governed by the laws of the State of New York, without regard to conflicts of laws provisions
thereof. 
 The Debt Securities of this series are issuable in registered form without coupons in minimum denominations of
$2,000 and integral multiples of $1,000 in excess thereof. As provided in the Indenture and subject to certain limitations therein set forth, Debt Securities of this series are exchangeable for a like aggregate principal amount of Debt Securities of
this series and of like tenor of a different authorized denomination, as requested by the Holder surrendering the same. 
 No
service charge shall be made to a Holder for any such registration of transfer or exchange, but the Company may require payment of a sum sufficient to cover any tax or other governmental charge payable in connection therewith. 

Prior to due presentment of this Debt Security for registration of transfer, the Company, the Guarantor, the Trustee and any agent
thereof may treat the Person in whose name this Debt Security is registered as the owner hereof for all purposes, whether or not this Debt Security be overdue, and none of the Company, the Guarantor, the Trustee or any such agent shall be affected
by notice to the contrary. 
 Except in the limited circumstances described in Section 3.05 of the Indenture, the Debt
Securities of this series shall be issued in the form of one or more Global Securities and The Depository Trust Company shall be the Depositary for such Global Security or Securities. 

All terms used in this Debt Security which are not defined herein and are defined in the Indenture shall have the meanings assigned to
them in the Indenture. 

 GUARANTEE OF 
 BERKSHIRE HATHAWAY INC. 
 FOR VALUE RECEIVED, Berkshire Hathaway Inc., a
Delaware corporation (the “Guarantor”), hereby absolutely, unconditionally and irrevocably guarantees to the holders (the “Holders”) of any security authenticated and delivered (each a “Security”) by The Bank of New
York Mellon Trust Company, N.A., as trustee (the “Trustee”) under that certain Indenture, dated as of February 1, 2010 (the “Indenture”), among the Trustee, the Guarantor and Berkshire Hathaway Finance Corporation, a
Delaware corporation (“Issuer”), the full and prompt payment when due (whether at stated maturity, by acceleration or otherwise) of all present and future payment obligations of the Issuer pursuant to the terms of such Security and/or the
Indenture, whether direct or indirect, absolute or contingent, and whether for principal, interest, fees, expenses, indemnification or otherwise (collectively, the “Obligations”). Nothing herein shall be deemed to guarantee any obligation
of the Issuer other than the Obligations. Nothing herein shall be deemed to guarantee any obligation of any person or entity other than the Issuer. 
 The Guarantor’s obligations hereunder shall be unconditional and absolute, and shall not be released, discharged or otherwise affected by (i) the existence, validity, enforceability, perfection
or extent of any collateral therefor, (ii) any lack of validity or enforceability of any provision of the Security or the Indenture, (iii) any liquidation, bankruptcy, insolvency, reorganization or other similar proceeding affecting the
Issuer or its assets, or (iv) any other circumstance relating to the Obligations that might otherwise constitute a legal or equitable discharge of, or defense to, the Guarantor. The Guarantor agrees that the Holders and/or the Trustee may
resort to the Guarantor, as primary obligor and not merely as surety, for payment of any of the Obligations whether or not the Holders or the Trustee shall have proceeded against the Issuer or any other obligor principally or secondarily obligated
with respect to any of the Obligations. Neither the Holders nor the Trustee shall be obligated to file any claim relating to any of the Obligations in the event that the Issuer becomes subject to a bankruptcy, reorganization or similar proceeding,
and the failure of the Holders or the Trustee to so file shall not affect the Guarantor’s obligations hereunder. In the event that any payment to the Holders by the Issuer in respect of any Obligations is rescinded or must otherwise be returned
for any reason whatsoever, the Guarantor shall remain liable hereunder with respect to such Obligations as if such payment had not been made. 
 The Guarantor agrees that, subject to the Indenture, the Holders and/or the Trustee may at any time and from time to time, either before or after the maturity thereof, without notice to or further consent
of the Guarantor, extend the time of payment of, exchange or surrender any collateral for, or renew any of the Obligations, and may also make any agreement with the Issuer or with any other party to or person liable on any of the Obligations or
interested therein, for the extension, renewal, payment, compromise, discharge or release thereof, in whole or in part, or for any modification of the terms thereof or of any agreement between the Holders, the Trustee and the Issuer or any such
other party or person, and that none of the foregoing shall in any way impair or affect this Guarantee. 
 The Guarantor hereby
unconditionally and irrevocably waives, to the fullest extent permitted by law, (a) notice of the acceptance of this Guarantee and of the Obligations, presentment, demand for payment, notice of dishonor and protest, (b) any requirement
that any Holder exhaust any right or take any action against the Issuer, and (c) any right to revoke this Guarantee. 

 The Guarantor agrees to pay on demand all fees and out-of-pocket expenses incurred by the
Holders or the Trustee in any way relating to the enforcement or protection of the rights of the Holders and/or the Trustee hereunder. 
 Upon payment of any of the Obligations, the Guarantor shall be subrogated to the rights of the Holders and/or the Trustee against the Issuer with respect to such Obligations, and the Holders and the
Trustee agree to take such steps, at the Guarantor’s expense, as the Guarantor may reasonably request to implement such subrogation; provided, however, that the Guarantor shall not be entitled to enforce, or to receive any payments arising out
of or based upon, such right of subrogation during any period in which any amount payable by the Issuer under the Security or the Indenture is overdue or unpaid. 
 No failure on the part of the Holders or the Trustee to exercise, and no delay in exercising, any right, remedy or power hereunder shall operate as a waiver thereof, nor shall any single or partial
exercise by the Holders or the Trustee of any right, remedy or power hereunder preclude any other or future exercise of any right, remedy or power. Each and every right, remedy and power hereby granted to the Holders or the Trustee or allowed any of
them by law or other agreement shall be cumulative and not exclusive of any other, and may be exercised by the Holders or the Trustee at any time or from time to time. 
 The Guarantor hereby represents and warrants that: 
 (a) the Guarantor is duly organized, validly
existing and in good standing as a corporation under the laws of the State of Delaware and has full corporate power to execute, deliver and perform this Guarantee; 
 (b) the execution, delivery and performance of this Guarantee have been and remain duly authorized by all necessary corporate action and do not contravene any provision of the Guarantor’s certificate
of incorporation or by-laws, as amended to date, or any law, regulation, rule, decree, order, judgment or contractual restriction binding on the Guarantor or its assets; 
 (c) all consents, licenses, clearances, authorizations and approvals of, and registrations and declarations with, any governmental authority or regulatory body necessary for the due execution, delivery
and performance of this Guarantee have been obtained and remain in full force and effect and all conditions thereof have been duly complied with, and no other action by, and no notice to or filing with, any governmental authority or regulatory body
is required in connection with the execution, delivery or performance of this Guarantee; 
 (d) this Guarantee constitutes a legal, valid and
binding obligation of the Guarantor enforceable against the Guarantor in accordance with its terms, subject to bankruptcy, insolvency, reorganization, moratorium and other laws of general applicability relating to or affecting creditors’ rights
and to general equity principles; and 
 (e) there are no actions, suits or arbitration proceedings pending or, to the knowledge of the
Guarantor, threatened against it, at law or in equity, which, individually or in the aggregate, if adversely determined, would materially adversely affect the financial condition of the Guarantor or materially impair its ability to perform its
obligations under this Guarantee. 
 The Guarantor may not assign its obligations hereunder to any person (except as permitted
by the Indenture) without the prior written consent of the Holders or the Trustee. 

 All payments by the Guarantor to the Holders or the Trustee shall be made in accordance with
the provisions of the Indenture and the Security; provided, however, that payment of any fees or expenses pursuant to the fifth paragraph hereof shall be made by wire transfer of immediately available funds to an account at a commercial bank in the
United States specified to the Guarantor at least ten (10) days in advance of any demand for payment by the Holders or the Trustee. 
 All notices or demands on the Guarantor shall be deemed effective when received, shall be in writing and shall be delivered by hand or by registered mail, or by facsimile transmission promptly confirmed
by registered mail, addressed to the Guarantor at: 
 Berkshire Hathaway Inc. 

3555 Farnam Street 
 Omaha, NE 68131 
 Attention: Chief Financial Officer 

Facsimile: (402) 346-3375 
 or to such other addresses or facsimile numbers as the Guarantor shall have notified the Holders or the Trustee in a written notice delivered in accordance with the Indenture. 

This Guarantee shall remain in full force and effect and shall be binding on the Guarantor, its successors and assigns until all of the
Obligations have been satisfied in full. 
 This Guarantee shall be governed by, and construed in accordance with, the laws of
the State of New York applicable to contracts made and to be performed solely within such State. 
 No amendment or waiver of
any provision of this Guarantee shall in any event be effective unless the same shall be in writing and signed by the Trustee and the Guarantor. 
 If for any reason any provision or provisions hereof are determined to be invalid and contrary to any existing or future law, such invalidity shall not, to the fullest extent permitted by law, impair the
operation of or effect of those portions of this Guarantee that are valid. 
 THE GUARANTOR WAIVES ANY RIGHT IT MAY HAVE TO A
JURY TRIAL IN CONNECTION WITH ANY ACTION, SUIT OR PROCEEDING ARISING OUT OF OR RELATED IN ANY WAY TO THIS GUARANTEE. 

							
	Dated:	 		 	BERKSHIRE HATHAWAY INC.
				
		 		 	By:	 	 
		 		 		 	Name: Marc D. Hamburg
		 		 		 	 Title:   Senior Vice President and
  Chief Financial Officer

 SCHEDULE OF INCREASES OR DECREASES IN GLOBAL SECURITY 

The following increases or decreases in this Debt Security have been made: 

 

									
	Date of exchange	  	Amount of decrease in
principal amount of
this Debt Security	  	Amount of increase in
principal amount of
this
Debt Security	  	Principal amount of this
Debt Security
following
such decrease or increase	  	Signature of authorized
signatory of Trustee
or
Security Custodian

 ASSIGNMENT 
 FOR VALUE RECEIVED, the undersigned assigns and transfers this Debt Security to: 
  

			
	  
	  	
	  
	  	
	(Insert assignee’s social security or tax identification number)	  	
		
	  
	  	
	  
	  	
	  
	  	
	(Insert address and zip code of assignee)	  	

 and irrevocably appoints              as agent to
transfer this Debt Security on the Security Register. The agent may substitute another to act for him or her. 
  

					
	 Dated:
	  	Signature:	  	
			
		  	Signature Guarantee:	  	

 (Sign exactly as your name appears on the other side of this Debt Security) 

Signatures must be guaranteed by an “eligible guarantor institution” meeting the requirements of the Security Registrar, which
requirements include membership or participation in the Security Transfer Agent Medallion Program (“STAMP”) or such other “signature guarantee program” as may be determined by the Security Registrar in addition to, or in
substitution for, STAMP, all in accordance with the Securities Exchange Act of 1934, as amended.EX-4.2

 Exhibit 4.2 
 EXECUTION VERSION 
 THIRD AMENDED AND RESTATED 

INVESTORS’ RIGHTS AGREEMENT 

 TABLE OF CONTENTS 

 

									
	 	 	 	  	Page	 
			
	 1.
	 	 Definitions
	  	 	1	  
			
	 2.
	 	 Registration Rights
	  	 	8	  
				
		 	 2.1
	 	 Demand Registration
	  	 	8	  
		 	 2.2
	 	 Company Registration
	  	 	9	  
		 	 2.3
	 	 Underwriting Requirements
	  	 	10	  
		 	 2.4
	 	 Obligations of the Company
	  	 	11	  
		 	 2.5
	 	 Furnish Information
	  	 	12	  
		 	 2.6
	 	 Expenses of Registration
	  	 	12	  
		 	 2.7
	 	 Delay of Registration
	  	 	13	  
		 	 2.8
	 	 Indemnification
	  	 	13	  
		 	 2.9
	 	 Reports Under Exchange Act
	  	 	15	  
		 	 2.10
	 	 Limitations on Subsequent Registration Rights
	  	 	16	  
		 	 2.11
	 	 “Market Stand-off” Agreement
	  	 	16	  
		 	 2.12
	 	 Restrictions on Transfer
	  	 	17	  
		 	 2.13
	 	 Termination of Registration Rights
	  	 	18	  
			
	 3.
	 	 Information and Observer Rights
	  	 	19	  
				
		 	 3.1
	 	 Delivery of Financial Statements
	  	 	19	  
		 	 3.2
	 	 Inspection
	  	 	20	  
		 	 3.3
	 	 Observer Rights
	  	 	20	  
		 	 3.4
	 	 Termination of Information Rights
	  	 	22	  
		 	 3.5
	 	 Confidentiality
	  	 	22	  
		 	 3.6
	 	 Board of Directors
	  	 	22	  
			
	 4.
	 	 Rights to Future Stock Issuances
	  	 	22	  
				
		 	 4.1
	 	 Right of First Offer
	  	 	22	  
		 	 4.2
	 	 Termination
	  	 	23	  
			
	 5.
	 	 Additional Covenants
	  	 	24	  
				
		 	 5.1
	 	 Insurance
	  	 	24	  
		 	 5.2
	 	 Employee Agreements
	  	 	24	  
		 	 5.3
	 	 Employee Vesting
	  	 	24	  
		 	 5.4
	 	 Certain Option Grants
	  	 	24	  
		 	 5.5
	 	 Qualified Small Business Stock
	  	 	25	  
		 	 5.6
	 	 Matters Requiring Investor Director Approval; Sale of New Securities
	  	 	25	  
		 	 5.7
	 	 Meetings of the Board of Directors
	  	 	25	  
		 	 5.8
	 	 Successor Indemnification
	  	 	25	  

  
 i 

									
		 	 5.9
	 	 Board Expenses
	  	 	25	  
		 	 5.10
	 	 Committees of the Board of Directors
	  	 	26	  
		 	 5.11
	 	 Covenant Regarding Any Future “Drag Along” Agreement
	  	 	26	  
		 	 5.12
	 	 Termination of Covenants
	  	 	26	  
			
	 6.
	 	 Miscellaneous
	  	 	26	  
				
		 	 6.1
	 	 Successors and Assigns
	  	 	26	  
		 	 6.2
	 	 Governing Law
	  	 	27	  
		 	 6.3
	 	 Counterparts; Facsimile
	  	 	27	  
		 	 6.4
	 	 Titles and Subtitles
	  	 	27	  
		 	 6.5
	 	 Notices
	  	 	27	  
		 	 6.6
	 	 Amendments and Waivers
	  	 	28	  
		 	 6.7
	 	 Severability
	  	 	29	  
		 	 6.8
	 	 Aggregation of Stock
	  	 	29	  
		 	 6.9
	 	 Additional Investors
	  	 	29	  
		 	 6.10
	 	 Entire Agreement
	  	 	29	  
		 	 6.11
	 	 Dispute Resolution
	  	 	29	  
		 	 6.12
	 	 Delays or Omissions
	  	 	30	  
		 	 6.13
	 	 Acknowledgment
	  	 	30	  

  

					
	Schedule A	 	-	  	Schedule of Investors

  
 ii 

 THIRD AMENDED AND RESTATED 

INVESTORS’ RIGHTS AGREEMENT 
 THIS THIRD AMENDED AND RESTATED INVESTORS’ RIGHTS AGREEMENT (“Agreement”) is made as of May 23, 2013, by and among Ophthotech Corporation, a Delaware corporation (the
“Company”), and each of the investors listed on Schedule A hereto, each of which is referred to in this Agreement as an “Investor”, and any additional Investor that becomes a party to this Agreement in
accordance with Section 6.9 hereof. 
 RECITALS 

WHEREAS, certain of the Investors (the “Existing Investors”) hold shares of the Company’s Series A Preferred
Stock, Series A-1 Preferred Stock, Series B Preferred Stock and Series B-1 Preferred Stock and possess registration rights, information rights, participation rights and other rights pursuant to a Second Amended and Restated Investors’ Rights
Agreement dated as of December 11, 2009 by and among the Company and such Investors, as amended by the Amendment thereto dated as of September 12, 2011, Amendment No. 2 thereto dated as of June 20, 2012, Amendment No. 3
thereto dated as of December 24, 2012 and Amendment No. 4 thereto dated as of March 15, 2013 (the “Prior Agreement”); 
 WHEREAS, the undersigned Existing Investors are holders of (i) a majority of the Series A Registrable Securities and Series A-1 Registrable Securities (as defined in the Prior Agreement
and considered together as a single class) and (ii) at least sixty percent (60%) of the Series B Registrable Securities and Series B-1 Registrable Securities (as defined in the Prior Agreement and considered together as a single class),
and desire to amend and restate the Prior Agreement in its entirety and to accept the rights created pursuant to this Agreement in lieu of the rights granted to them under the Prior Agreement; and 

WHEREAS, certain of the Investors are parties to that certain Series C Preferred Stock Purchase Agreement dated as of the date
hereof by and among the Company and certain of the Investors (as amended from time to time, the “Purchase Agreement”), under which certain of the Company’s and such Investors’ obligations are conditioned upon the execution
and delivery of this Agreement by such Investors, Existing Investors holding (i) a majority of the Series A Registrable Securities and Series A-1 Registrable Securities (as defined in the Prior Agreement and considered together as a single
class) and (ii) at least sixty percent (60%) of the Series B Registrable Securities and Series B-1 Registrable Securities (as defined in the Prior Agreement and considered together as a single class), and the Company; 

NOW, THEREFORE, the undersigned Existing Investors and the Company hereby agree that the Prior Agreement shall be amended and
restated, and the parties to this Agreement hereby agree as follows: 
 1. Definitions. For purposes of this Agreement:

 1.1 “Affiliate” means, with respect to any specified Person, any other Person who or which, directly or
indirectly, controls, is controlled by, or is under common control with such specified Person, including without limitation any general partner, officer, director, or 

  
 1 

 
manager of such Person and 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. 
 1.2 “Certificate of Incorporation” means the Company’s Certificate of Incorporation,
as amended and/or restated from time to time. 
 1.3 “Common Stock” means shares of the Company’s common
stock, $0.001 par value per share. 
 1.4 “Converted Shares” means (i) shares of Common Stock issued upon
conversion of the Series A Preferred Stock, Series B Preferred Stock and Series C Preferred Stock pursuant to the “Special Mandatory Conversion” provisions of the Company’s Certificate of Incorporation, (ii) shares of Common
Stock subject to or resulting from a “Special Mandatory Combination” pursuant to the Company’s Certificate of Incorporation and (iii) any other shares of Common Stock issued upon an optional conversion of Series A Preferred
Stock, Series B Preferred Stock or Series C Preferred Stock by an Investor subject to Special Mandatory Conversion or Special Mandatory Combination on or prior to such Special Mandatory Conversion or Special Mandatory Combination pursuant to the
Company’s Certificate of Incorporation. 
 1.5 “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. 

1.6 “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. 
 1.7 “Exchange Act” means the
Securities Exchange Act of 1934, as amended, and the rules and regulations promulgated thereunder. 
 1.8 “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. 

  
 2 

 1.9 “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. 
 1.10
“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. 
 1.11 “GAAP” means generally accepted accounting
principles in the United States. 
 1.12 “Holder” means any holder of Registrable Securities who is a party to
this Agreement and, for purposes of Sections 2.11 and 2.12 hereof only, any holder of Converted Shares who is a party to this Agreement; provided that MidCap shall, subject to the following proviso, be considered a party
hereto only for purposes of Sections 1, 2 and 6 hereof and shall not be entitled to any other rights or subject to any other obligations under this Agreement; provided further, however, that MidCap shall not
have any right to make, nor shall it be considered a “Holder” for purposes of making, any demand or request for registration under Section 2.1(a) or 2.1(b). 

1.13 “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. 
 1.14 “Initiating Holders” means, collectively, Holders who properly initiate a registration request under this Agreement. 

1.15 “IPO” means the Company’s first underwritten public offering of its Common Stock under the Securities Act.

 1.16 “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 Purchase Agreement). 

1.17 “Major Investor” means any Investor that, individually or together with such Investor’s Affiliates, holds at
least 800,000 shares of Registrable Securities (as adjusted for any stock split, stock dividend, combination, or other recapitalization or reclassification effected after the date hereof); provided, however, that, any shares of Registrable
Securities held by Affiliates of an Investor that holds Converted Shares shall not be considered for purposes of determining whether such Investor is a “Major Investor” for purposes hereof. 

1.18 “MidCap” means MidCap Financial SBIC, LP, in its capacity as a holder of (i) the Series B Warrants,
(ii) shares of Series B Preferred Stock issued upon the exercise of the Series B Warrants or (iii) Common Shares issued upon the conversion of such shares of Series B Preferred Stock. MidCap is a party to Sections 1, 2 and 6 hereof (the
“Applicable 

  
 3 

 
Sections”) and is subject to all the rights and obligations of a “Holder” and an “Investor” as such terms are defined and used in this Agreement under and with
respect to the Applicable Sections. For the avoidance of doubt, MidCap is not entitled to any other rights or subject to any additional obligations under this Agreement except in its capacity as a “Holder” or an “Investor” with
respect to the Applicable Sections. 
 1.19 “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. 
 1.20 “Person” means any individual, corporation, partnership, trust, limited liability company,
association or other entity. 
 1.21 “Preferred Director” means any director of the Company that the holders of
record of the Series A Preferred Stock or Series B Preferred Stock are entitled to elect as a separate class pursuant to the Company’s Certificate of Incorporation. 
 1.22 “Preferred Stock” means the Series A Preferred Stock, the Series A-1 Preferred Stock, the Series B Preferred Stock, the Series B-1 Preferred Stock and the Series C Preferred Stock.

 1.23 “Qualified Public Offering” means the closing of the sale of shares of the Company’s Common Stock
to the public at a price to the public of at least $2.50 per share (as adjusted for any stock split, stock dividend, combination, or other recapitalization or reclassification effected after the date hereof), in an underwritten public offering
pursuant to an effective registration statement under the Securities Act, resulting in at least $40,000,000 of gross proceeds (before deducting the underwriting discount and commissions and other offering expenses) to the Company. 

1.24 “Registrable Securities” means (i) the Common Stock issuable or issued upon conversion of the Preferred Stock,
excluding any Converted Shares; (ii) any Common Stock, or 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,
excluding any Converted Shares; (iii) any Common Stock issued or issuable upon conversion of the Series B Preferred Stock issued or issuable upon the exercise of the Series B Warrants; and (iv) 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. 
 1.25 “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. 

  
 4 

 1.26 “Restricted Securities” means the securities of the Company required
to bear the legend set forth in Section 2.12(b) hereof. 
 1.27 “SEC” means the Securities and
Exchange Commission. 
 1.28 “SEC Rule 144” means Rule 144 promulgated by the SEC under the Securities Act.

 1.29 “SEC Rule 144(b)(1)” means Rule 144(b)(1) promulgated by the SEC under the Securities Act. 

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

1.31 “Securities Act” means the Securities Act of 1933, as amended, and the rules and regulations promulgated
thereunder. 
 1.32 “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. 
 1.33 “Series A Director” means any director of the Company that the holders of
record of the Series A Preferred Stock are entitled to elect as a separate class pursuant to the Company’s Certificate of Incorporation. 
 1.34 “Series A Preferred Stock” means shares of the Company’s Series A Preferred Stock, $0.001 par value per share. 

1.35 “Series A-1 Preferred Stock” means shares of the Company’s Series A-1 Preferred Stock, $0.001 par value per
share. 
 1.36 “Series A Registrable Securities” means (i) the Common Stock issuable or issued upon
conversion of the Series A Preferred Stock, excluding any Converted Shares; and (ii) 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 clause (i) 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. 

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

  
 5 

 1.38 “Series A-1 Preferred Stock Purchase Agreement” means the Series A-1
Preferred Stock Purchase Agreement dated January 4, 2008 by and among the Company, Facet Biotech Corporation (as assignee of PDL BioPharma, Inc.), Biogen Idec MA Inc. and the Regents of the University of California in the name of
Shellwater & Co, as it may be amended from time to time. 
 1.39 “Series A-1 Registrable Securities”
means (i) the Common Stock issuable or issued upon conversion of the Series A-1 Preferred Stock; and (ii) 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 clause (i) 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. 
 1.40 “Series A-1 Registrable Securities then outstanding” means the number of shares determined
by adding the number of shares of outstanding Common Stock that are Series A-1 Registrable Securities and the number of shares of Common Stock issuable (directly or indirectly) pursuant to then exercisable and/or convertible securities that are
Series A-1 Registrable Securities. 
 1.41 “Series B Director” means any director of the Company that the
holders of record of the Series B Preferred Stock are entitled to elect as a separate class pursuant to the Company’s Certificate of Incorporation. 
 1.42 “Series B Preferred Stock” means shares of the Company’s Series B Preferred Stock, $0.001 par value per share. 

1.43 “Series B Registrable Securities” means (i) the Common Stock issuable or issued upon conversion of the Series
B Preferred Stock, excluding any Converted Shares; (ii) any Common Stock issued or issuable upon conversion of the Series B Preferred Stock issued or issuable upon the exercise of the Series B Warrants; 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 clause (i) 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. 
 1.44 “Series B Registrable Securities then outstanding” means the number of shares determined by adding the number of shares of outstanding Common Stock that are Series B Registrable
Securities and the number of shares of Common Stock issuable (directly or indirectly) pursuant to then exercisable and/or convertible securities that are Series B Registrable Securities. 

  
 6 

 1.45 “Series B Warrants” means (i) the warrant to purchase shares of
Series B Preferred Stock issued by the Company to MidCap pursuant to the Loan and Security Agreement, dated June 20, 2012, among the Company, MidCap, as administrative agent and lender, and the lenders party thereto, as such warrant is amended,
restated, amended and restated or otherwise modified and in effect from time to time, (ii) the warrant to purchase shares of Series B Preferred Stock issued by the Company to MidCap pursuant to the Amended and Restated Loan and Security
Agreement, dated December 24, 2012, among the Company, MidCap, as administrative agent and lender, and the lenders party thereto, as such warrant is amended, restated, amended and restated or otherwise modified and in effect from time to time,
and (iii) the warrants to purchase shares of Series B Preferred Stock issued by the Company to MidCap pursuant to the Second Amended and Restated Loan and Security Agreement, dated March 15, 2012, among the Company, MidCap, as
administrative agent and lender, and the lenders party thereto, as such warrants are amended, restated, amended and restated or otherwise modified and in effect from time to time. 

1.46 “Series B-1 Preferred Stock” means shares of the Company’s Series B-1 Preferred Stock, $0.001 par value per
share. 
 1.48 “Series B-1 Registrable Securities” means (i) the Common Stock issuable or issued upon
conversion of the Series B-1 Preferred Stock; and (ii) 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 clause (i) 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. 

1.49 “Series B-1 Registrable Securities then outstanding” means the number of shares determined by adding the number of
shares of outstanding Common Stock that are Series B-1 Registrable Securities and the number of shares of Common Stock issuable (directly or indirectly) pursuant to then exercisable and/or convertible securities that are Series B-1 Registrable
Securities. 
 1.50 “Series C Preferred Stock” means shares of the Company’s Series C Preferred Stock,
$0.001 par value per share. 
 1.51 “Series C Registrable Securities” means (i) the Common Stock issuable
or issued upon conversion of the Series C Preferred Stock, excluding any Converted Shares; and (ii) 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 clause (i) 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.

  
 7 

 1.52 “Series C Registrable Securities then outstanding” means the number
of shares determined by adding the number of shares of outstanding Common Stock that are Series C Registrable Securities and the number of shares of Common Stock issuable (directly or indirectly) pursuant to then exercisable and/or convertible
securities that are Series C Registrable Securities. 
 2. Registration Rights. The Company covenants and agrees as
follows: 
 2.1 Demand Registration. 
 (a) Form S-1 Demand. If at any time after one hundred eighty (180) days after the Company’s IPO, the Company receives a request from Holders of at least sixty percent (60%) of the
Registrable Securities then outstanding that the Company file a Form S-1 registration statement, then the Company shall (i) within ten (10) days after the date such request is given, give notice thereof (a “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 twenty (20) days of the date the Demand Notice is given, and in each case, subject to the limitations of Section 2.1(c) and Section 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 the Registrable Securities then outstanding that the Company file a Form S-3 registration statement with respect to outstanding Registrable Securities of such Holders having an anticipated aggregate offering price, net of Selling
Expenses, of at least $3 million, 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 twenty (20) days of the date the Demand Notice is given, and in each case, subject to the limitations of Section 2.1(c) and
Section 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 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

  
 8 

 
filing, and any time periods with respect to filing or effectiveness thereof shall be tolled correspondingly, for a period of not more than one hundred twenty (120) days after the request of
the Initiating Holders is given; provided, however, that the Company may not invoke this right more than twice 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 one hundred twenty (120) day period other than pursuant to 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 ninety (90) 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 the registration statement filed by the Company in connection with its IPO, provided,
that the Company is actively employing in good faith commercially reasonable efforts to cause such registration statement to become effective and delivers notice thereof to the Initiating Holders within thirty (30) days after the Initiating
Holders have requested registration under this Section 2.1; (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) in which case it will be deemed a request for registration under 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 two registrations pursuant to Section 2.1(b) within the twelve (12) month period immediately preceding the date of such request. 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 securities under the Securities Act in connection with the public offering of such securities solely for cash (other than in an Excluded
Registration), including in connection with the Company’s IPO, 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. 

  
 9 

 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 Company, subject
only to the reasonable approval of a majority in interest of the Initiating Holders. 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
advises 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 (i) 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 and (ii) in the event that the number of Registrable Securities held by the Holders to be included in such underwriting is
reduced, such reduction shall first apply to the Series A Registrable Securities and the Series A-1 Registrable Securities on a pro rata basis before applying to the Series B Registrable Securities, the Series B-1 Registrable Securities and the
Series C Registrable Securities on a pro rata basis. 
 (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.
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 

  
 10 

 
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 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. In the event that the number of Registrable Securities held by the selling Holders to be included in such offering is reduced, such reduction shall first apply to the Series A Registrable
Securities and the Series A-1 Registrable Securities on a pro rata basis before applying to the Series B Registrable Securities, the Series B-1 Registrable Securities and the Series C Registrable Securities on a pro rata basis. 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; 
 (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; 

  
 11 

 (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; 

(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 

  
 12 

 
disbursements of counsel for the Company; and the reasonable fees and disbursements, not to exceed $25,000, of one counsel for the selling Holders for each such registration (“Selling
Holder Counsel”), shall be borne and paid by the Company; provided, however, that the Company shall not be required to 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 Section 2.1(a) or Section 2.1(b),
as the case may be; provided further that if, at the time of such withdrawal, the Holders 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
Section 2.1(a) or Section 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 will 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 or delayed, 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 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, will 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 

  
 13 

 
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 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 any indemnity under this 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 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 of such separate counsel 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. The failure to give notice to the indemnifying party within a reasonable time of the commencement of any such action shall relieve such indemnifying party
of any liability to the indemnified party under this Section 2.8, to the extent that such failure materially prejudices the indemnifying party’s ability to defend such action. The failure to give notice to the indemnifying party
will not relieve it of any liability that it may have to any indemnified party otherwise than under this Section 2.8. 
 (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

  
 14 

 
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 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 fraud or willful misconduct 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; provided, however, that the failure of the underwriting agreement to address a provision addressed in this Agreement shall not be such a conflict. 

(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) 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),

  
 15 

 
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). 
 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 (i) a majority of the Series A Registrable Securities and Series A-1 Registrable
Securities then outstanding (voting together as a single class) and (ii) at least a majority of the Series B Registrable Securities, Series B-1 Registrable Securities and Series C Registrable Securities (voting together as a single class) 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
therein pursuant to this Agreement or (ii) to demand registration of any securities held by such holder or prospective holder; provided that this limitation shall not apply to any additional Investor who becomes a party to this Agreement
in accordance with Section 6.9. 
 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 IPO or other 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 one hundred eighty (180) days, which period
may be extended upon the request of the managing underwriter to allow it to comply with applicable regulatory restrictions (including Rule 2711(f)(4) or any successor rule or regulation of the Financial Industry Regulatory Authority) for an
additional period of up to fifteen (15) days if the Company issues or proposes to issue an earnings or other public release within fifteen (15) days of the expiration of the 180-day lockup period) (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. 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, directors, and stockholders individually owning more than one percent (1%) of the 

  
 16 

 
Company’s outstanding Common Stock (after giving effect to conversion into Common Stock of all outstanding Preferred Stock) are subject to the same restrictions. 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. 

2.12 Restrictions on Transfer. 
 (a) The Preferred Stock, the Registrable Securities and any Converted Shares shall not be sold, pledged, or otherwise transferred, and the Company shall not recognize 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, Registrable Securities or Converted Shares 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, (iii) any
Converted Shares and (iv) any other securities issued in respect of the securities referenced in clauses (i), (ii) and (iii), 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 SECURITIES 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. 

  
 17 

 (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 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 in the case of clause (y) 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 Section 2.2 shall terminate upon the earliest to occur of: 

(a) the closing of a Deemed Liquidation Event, as such term is defined in the Company’s Certificate of Incorporation; 

(b) as to any Holder, such earlier time after the IPO at which such Holder (A) can sell all shares held by it in compliance with
Rule 144(b)(1) or (B) holds one percent (1%) or less of the Company’s outstanding Common Stock and all Registrable Securities held by such Holder (together with any Affiliate of the Holder with whom such Holder must aggregate its
sales under Rule 144) can be sold in any three (3) month period without registration in compliance with Rule 144; or 

(c) five years after the Company’s IPO. 

  
 18 

 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 one hundred twenty (120) days after the end of the 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, audited and certified in accordance with GAAP by independent public accountants of nationally recognized standing selected by the Board of Directors of the Company;

 (b) as soon as practicable, but in any event within forty-five (45) 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 and a statement of stockholders’ equity 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); 

(c) concurrently with the delivery of the items described in Sections 3.1(a), (b) and (d), 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 and statement of stockholders’ equity 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”),
approved by the Board of Directors and 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;

  
 19 

 (f) with respect to the financial statements called for in Section 3.1(a),
Section 3.1(b) and Section 3.1(d), an instrument executed by the chief financial officer and chief executive officer of the Company certifying that such financial statements were prepared in accordance with GAAP consistently
applied with prior practice for earlier periods (except as otherwise set forth in Section 3.1(b) and Section 3.1(d)) and fairly present the financial condition of the Company and its results of operation for the periods
specified therein; 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. 
 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
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. 

(a) As long as SV Life Sciences Fund IV, L.P. (“SV Life Sciences”) owns shares of Preferred Stock (or Common Stock
issued upon conversion thereof), the Company shall invite a representative of SV Life Sciences 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 

  
 20 

 
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 a conflict of interest or disclosure of highly confidential proprietary
information. 
 (b) As long as Clarus Lifesciences II, L.P. (“Clarus”) owns shares of Series B Preferred Stock
(or Common Stock issued upon conversion thereof), the Company shall invite a representative of Clarus 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 a conflict of interest or disclosure of highly confidential proprietary information.

 (c) As long as Novo A/S owns shares of Preferred Stock (or Common Stock issued upon conversion thereof), the Company shall
invite a representative of Novo A/S 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 a conflict of interest or disclosure of highly confidential proprietary information. 

(d) As long as SV Life Sciences, Novo A/S, HBM Healthcare Investments (Cayman) Ltd. (“HBM”) or Clarus owns shares of
Preferred Stock (or Common Stock issued upon conversion thereof), the Company shall invite a representative of each of SV Life Sciences, Novo A/S, HBM or Clarus, as applicable, to attend all meetings of the Company’s scientific or other
advisory boards 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 the members of such boards at the same time and in the same
manner as provided to such members; 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 a conflict of interest or disclosure of highly confidential proprietary information. 

  
 21 

 3.4 Termination of Information Rights. The covenants set forth in
Section 3.1, Section 3.2, Section 3.3 and Section 3.6 shall terminate and be of no further force or effect (i) immediately before the consummation of a Qualified Public Offering, (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. 
 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 or Converted Shares from such Investor, if such prospective purchaser agrees to be bound by the
provisions of this Section 3.5; (iii) to any 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. 
 3.6 Board of Directors. On or prior to
March 31, 2014, the Board of Directors shall appoint one individual, who is not an Affiliate of the Company or of any Investor and who is approved by Novo A/S, to serve as Chairman of the Board of Directors; provided, however, that the
Company shall be deemed to be in compliance with this covenant if one or more individuals are proposed in good faith to serve in such capacity in a timely manner, Novo A/S does not approve any such individual and the Company is continuing to use
commercially reasonable efforts to identify and propose an alternative candidate that is acceptable to Novo A/S. 
 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 Major Investor. A Major 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 Major 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. 

  
 22 

 (b) By written notice to the Company within twenty (20) days after the Offer Notice
is given, each Major 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 issued and held, or
issuable (directly or indirectly) upon conversion and/or exercise, as applicable, of the Preferred Stock and any other Derivative Securities then held, by such Major Investor bears to the total Common Stock of the Company then outstanding (assuming
full conversion and/or exercise, as applicable, of all Preferred Stock and other Derivative Securities then outstanding). Each such election shall be accompanied by a representation letter that such Major Investor is an accredited investor as
defined in Rule 501(a) of Regulation D promulgated under the Securities Act. At the expiration of such twenty (20) day period, the Company shall promptly notify each Major Investor that elects to purchase or acquire all the shares available to
it (each, a “Fully Exercising Investor”) of any other Major 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 Major Investors were entitled to subscribe but that were not subscribed for by the
Major Investors which is equal to the proportion that the Common Stock issued and held, or issuable upon conversion and/or exercise, as applicable, of Preferred Stock and any other Derivative Securities then held, by such Fully Exercising Investor
bears to the Common Stock issued and held, or issuable (directly or indirectly) upon conversion and/or exercise, as applicable, of the Preferred Stock and any other Derivative Securities then held, by all Fully Exercising Investors who wish to
purchase such unsubscribed shares. The closing of any sale pursuant to this Section 4.1(b) shall occur within the later of one hundred twenty (120) 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 Major 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. 

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 Qualified Public Offering, (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. 

  
 23 

 5. Additional Covenants. 

5.1 Insurance. The Company shall at all times maintain (i) Directors and Officers Liability insurance in the amount of at
least $2,000,000 (which shall cover the directors and their affiliated entities) and (ii) term “key-person” insurance in the amount of at least $1,000,000 (or such greater amount as determined by the Board of Directors) on each of the
employees and officers deemed necessary by the Board of Directors, and will use commercially reasonable efforts to cause such insurance policies to be maintained until such time as the Board 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 of the Board of Directors including a majority of the Preferred Directors. Immediately prior to
the IPO, the Company shall, subject to approval by the Board of Directors, increase the Directors and Officers Errors and Omissions insurance to at least $5,000,000. 
 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) 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, in a form reasonably acceptable to a majority of
the Preferred 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 unanimous consent of the Preferred Directors. 
 5.3 Employee Vesting. Unless otherwise approved by the Board
of Directors, including a majority of the Preferred Directors, all future employees and consultants of the Company who purchase, receive options to purchase, or receive awards of shares of the Company’s capital stock after the date hereof shall
be required to execute restricted stock or option agreements, as applicable, providing for (i) vesting of shares over a four (4) year period, with the first twenty-five percent (25%) of such shares vesting following twelve
(12) months of continued employment or service, and the remaining shares vesting in equal monthly installments over the following thirty-six (36) months, and (ii) a market stand-off provision substantially similar to that in
Section 2.11. In addition, unless otherwise approved by the Board of Directors, including a majority of the Preferred Directors, the Company shall retain a “right of first refusal” on employee transfers until the Company’s
IPO and shall have the right to repurchase unvested shares at cost upon termination of employment of a holder of restricted stock. 
 5.4 Certain Option Grants. Any options to purchase Common Stock granted by the Company that are subject to Section 409A of the Internal Revenue Code (the “Code”) will be
granted with an exercise price of no less than 100% of the fair market value of the underlying Common Stock on the date of grant as determined by the Board of Directors based upon a third party valuation of the Common Stock that has been completed
within a reasonable period of time relative to each such grant. 

  
 24 

 5.5 Qualified Small Business Stock. The Company shall use commercially reasonable
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 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 is inconsistent with the best
interests of the Company. 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.

 5.6 Matters Requiring Investor Director Approval; Sale of New Securities. 

(a) So long as the holders of Preferred Stock are entitled to elect one or more Preferred Directors, the Company hereby covenants and
agrees with each of the Investors that it shall not, without approval of the Board of Directors, which approval must include the affirmative vote of a majority of the Preferred Directors, enter into any business arrangement that exceeds $1,000,000.

 (b) Notwithstanding anything to the contrary herein, the Company shall not consummate the sale of any New Securities (other
than Exempted Securities (as defined in the Company’s Certificate of Incorporation) but including the sale of shares of Common Stock in the IPO) unless and until (i) the sale of such New Securities is approved by a majority of the
directors then in office and (ii) the Company has satisfied its obligations under Section 1.3(c) of the Purchase Agreement. 
 5.7 Meetings of the Board of Directors. Unless otherwise determined by the vote of a majority of the directors then in office, the Board of Directors shall meet at least four (4) times per
year in accordance with an agreed-upon schedule. 
 5.8 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 maybe. 
 5.9 Board Expenses. The
Company shall reimburse the directors and board observers 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 and other events
attended on behalf of the Company. 

  
 25 

 5.10 Committees of the Board of Directors. 

(a) Series A Directors. Each of SV Life Sciences, Novo A/S and HBM shall have the right to have the Series A Director designated
by such entity pursuant to that certain Second Amended and Restated Voting Agreement of even date herewith, as such may be amended and/or restated from time to time (the “Voting Agreement”), sit on any committee of the Board of
Directors other than the compensation committee. Notwithstanding the foregoing, (i) if SV Life Sciences, Novo A/S and HBM do not exercise their rights pursuant to this Section 5.10, at least one Series A Director shall be appointed
to serve as a member of each committee of the Board of Directors other than the compensation committee. At least one Series A Director shall be appointed to serve as a member of the compensation committee, which Series A Director shall initially be
Axel Bolte. 
 (b) Series B Director. Clarus shall have the right to have the Series B Director designated by Clarus
pursuant to the Voting Agreement sit on the compensation committee of the Board of Directors. 
 5.11 Covenant Regarding Any
Future “Drag Along” Agreement. The Company shall not enter into any agreement (other than the Voting Agreement), nor permit any agreement to which the Company is a party to be amended in any manner, pursuant to which any Investor would
be compelled to transfer its ownership interest in the Company (whether directly or indirectly by way of merger or otherwise) to any acquirer thereof without such Investor’s consent, unless such agreement expressly provides (and requires to be
binding on such Investor) that in no event will any Investor be required to agree to sell, transfer, convey (whether by merger, operation of law or otherwise) its ownership interest in the Company unless the liability of security holders for
indemnification in connection with such transaction, if any, is several, not joint, is allocated pro rata in accordance with such security holder’s equity ownership of the Company, and, as to each stockholder, will not exceed the consideration
payable to such stockholder in respect of the transaction by virtue of such stockholder’s ownership of securities of the Company, if any, in such transaction (except in the case of potential liability for fraud or willful misconduct by such
Investor). 
 5.12 Termination of Covenants. The covenants set forth in this Section 5, except for
Section 5.8, shall terminate and be of no further force or effect (i) immediately before the consummation of a Qualified Public Offering, (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. 

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, partner, member, limited partner, retired partner, retired 

  
 26 

 
member, or stockholder 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 250,000 shares of Registrable Securities (subject to appropriate adjustment for stock splits, stock dividends, combinations, and other recapitalizations); 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, limited partner, retired partner, member, retired member, 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. 
 6.2 Governing Law. This Agreement shall be governed by and construed in accordance with the General Corporation Law of the State of Delaware as to matters within the scope thereof, and as to all
other matters shall be governed by and construed in accordance with the internal laws of the State of New York, without regard to its principles of conflicts of laws. 
 6.3 Counterparts; Facsimile. 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 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, requests, and other communications given or made
pursuant to this Agreement shall be in writing and shall be deemed effectively given, delivered and received (i) upon personal delivery to the party to be notified; (ii) when sent by confirmed electronic mail or facsimile if sent during
normal business hours of the recipient, and if not so confirmed, then on the 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, 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 

  
 27 

 
given in accordance with this Section 6.5. If notice is given to the Company, it shall be sent to One Penn Plaza, 35th Floor, New York, NY 10119, Attention: Chief Executive Officer; and a copy (which shall not constitute notice) shall
also be sent to Wilmer Cutler Pickering Hale and Dorr LLP, Attention: David E. Redlick, 60 State Street, Boston, MA 02109. If notice is given to the holders of Preferred Stock (or Common Stock issuable upon conversion thereof), other than Novo A/S
or HBM Healthcare Investments (Cayman) Ltd., a copy shall also be given to Mintz, Levin, Cohn, Ferris, Glovsky and Popeo P.C., Attention: Daniel T. Kajunski, One Financial Center, Boston, MA 02111. If notice is given to Novo A/S, a copy shall also
be given to Latham & Watkins LLP, Attention: B. Shayne Kennedy, 650 Town Center Drive, 20th Floor, Costa Mesa, CA 92626. If notice is given to HBM Healthcare Investments (Cayman) Ltd., a copy shall also be given to Cooley LLP, Attn: Mehdi Khodadad, 3175 Hanover Street, Palo Alto, CA 94304.

 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 (i) a majority of the Series A Registrable Securities and Series A-1
Registrable Securities (voting together as a single class) then outstanding and (ii) at least a majority of the Series B Registrable Securities, Series B-1 Registrable Securities and Series C Registrable Securities (voting together as a single
class) 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), provided however that (w) Sections 3.3(a), 3.3(d) (as it relates to
the rights of SV Life Sciences) and 5.10(a) (as it relates to the rights of SV Life Sciences) and clause (w) of this proviso may not be amended or waived without the consent of SV Life Sciences, (x) Sections 3.3(c),
3.3(d) (as it relates to the rights of Novo A/S), 5.6(b) and 5.10(a) (as it relates to the rights of Novo A/S) and clause (x) of this proviso may not be amended or waived without the consent of Novo A/S,
(y) Sections 3.3(d) (as it relates to the rights of HBM) and 5.10(a) (as it relates to the rights of HBM) and clause (y) of this proviso may not be amended or waived without the consent of HBM and (z) Sections
3.3(b) and 5.10(b) and clause (z) of this proviso may not be amended or waived without the consent of Clarus. Notwithstanding the foregoing, the provisions of Section 2.8(b), 2.8(d) and 5.11, insofar as such
sections provide for each Investor’s several, and not joint, liability in regards to obligations under this Agreement or provide for limitations on the amount of each Investor’s liability or contribution requirements under this Agreement,
shall not be amended or waived without the written consent of each Investor against which such waiver or amendment would apply. 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

  
 28 

 
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, except as set forth in the definition of “Major Investor” in Section 1. 
 6.9 Additional
Investors. Notwithstanding anything to the contrary contained herein, if the Company issues additional shares of Preferred Stock after the date hereof pursuant to the Purchase Agreement to any party not already party to this agreement, any
acquirer 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)
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, including, without
limitation, the Prior Agreement and the Term Sheet entered into prior to the date hereof between the Company and Novo A/S, is expressly canceled. 
 6.11 Dispute Resolution. The parties (a) hereby irrevocably and unconditionally submit to the jurisdiction of the state courts of the State of New York and to the jurisdiction of the United
States District Court for the Southern District of New York for the purpose of any suit, action or other proceeding arising out of or based upon this Agreement, (b) agree not to commence any suit, action or other proceeding arising out of or
based upon this Agreement except in the state courts of the State of New York, and (c) hereby waive, and agree not to assert, by way of motion, as a defense, or otherwise, in any such suit, action or proceeding, any claim that it is not subject
personally to the jurisdiction of the above-named courts, that its property is exempt or immune from attachment or execution, that the suit, action or proceeding is brought in an inconvenient forum, that the venue of the suit, action or proceeding
is improper or that this Agreement or the subject matter hereof may not be enforced in or by such court. The prevailing party shall be entitled to reasonable attorney’s fees, costs, and necessary disbursements in addition to any other relief to
which such party may be entitled. Each of the parties to this Agreement consents to personal jurisdiction for any equitable action sought in the U.S. District Court for the Southern District of New York or any court of the State of New York having
subject matter jurisdiction. 

  
 29 

 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 the 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] 

  
 30 

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

			
		 	OPHTHOTECH CORPORATION
		
	By:	 	  

	Name:	 	  

	Title:	 	  

		
	Address:	 	One Penn Plaza
		 	35th Floor
		 	New York, NY 10119

  

Signature Page to Ophthotech Corporation 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. 
  

			
	INVESTOR:
	
	NOVO A/S
	
	  

	By:	 	Thomas Dyrberg, Partner
	
	Address:
	
	Tuborg Havnevej 19
	DK-2900 Hellerup
	Denmark

  

Signature Page to Ophthotech Corporation 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. 
  

			
	INVESTOR:
	
	HBM HEALTHCARE INVESTMENTS (CAYMAN) LTD.
	
	  

	By:	 	John Arnold
	Title:	 	Chairman and Managing Director
	
	Address:
	
	Centennial Towers, 3rd Floor
	2454 West Bay Road
	Grand Cayman
	Cayman Islands

  

Signature Page to Ophthotech Corporation 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. 
  

			
	INVESTORS:
	
	SV LIFE SCIENCES FUND IV, L.P.
		
	By:	 	 SV Life Sciences Fund IV (GP), L.P.,
 its sole General Partner

	By:	 	SVLSF IV, LLC, its sole General Partner
		
	By:	 	  

	Name:	 	  

	Title:	 	  

	
	Address:
	
	 One Boston Place, Suite 3900
 Boston, MA 02108

	
	SV LIFE SCIENCES FUND IV STRATEGIC PARTNERS, L.P.
		
	By:	 	 SV Life Sciences Fund IV (GP), L.P.,
 its sole General Partner,

	By:	 	SVLSF IV, LLC, its sole General Partner
		
	By:	 	  

	Name:	 	  

	Title:	 	  

	
	Address:
	
	One Boston Place, Suite 3900
	Boston, MA 02108

  

Signature Page to Ophthotech Corporation 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. 
  

			
	INVESTORS:
	
	INTERNATIONAL BIOTECHNOLOGY TRUST PLC
		
	By:	 	  

		 	Nick Coleman
		 	IBT Authorised Signature
	
	Address:
	
	International Biotechnology Trust plc
	55 Moorgate
	London
	EC2R 6PA
	United Kingdom

  

Signature Page to Ophthotech Corporation 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. 
  

	
	INVESTORS:
	
	SAMIR C. PATEL LLC
	
	  

	
	Address:
	
	66 Witherspoon Street
	P.O. Box 214
	Princeton, NJ 08540

  

Signature Page to Ophthotech Corporation 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. 
  

			
	INVESTORS:
	
	CLARUS LIFESCIENCES II, LP
		
	By:	 	Clarus Ventures II GP, LP, its General Partner,
	By:	 	Clarus Ventures II, LLC, its General Partner
		
	By:	 	  

	Name:	 	  

	Title:	 	  

	
	Address:
	
	101 Main Street Suite 1210
	Cambridge, MA 02142

  

Signature Page to Ophthotech Corporation Third Amended and Restated Investors’ Rights Agreement 

 SCHEDULE A 
 Investors 
 SERIES A PREFERRED STOCK 

 

			
	Name	  	Number of Shares held as of
the date of this Agreement
		
	 SV Life Sciences Fund IV, L.P.
 One Boston Place, Suite 3900
 Boston, MA 02108
	  	21,000,448
		
	 SV Life Sciences Fund IV Strategic Partners, L.P.

One Boston Place, Suite 3900
 Boston, MA
02108
	  	596,220
		
	 Novo A/S
 Tuborg Havnevej 19
 DK-2900 Hellerup
 Denmark
	  	14,374,443
		
	 HBM Healthcare Investments (Cayman) Ltd.

Centennial Towers, 3rd Floor
 2454 West Bay
Road
 Grand Cayman
 Cayman
Islands
	  	14,374,443
		
	 Samir C. Patel LLC
 66 Witherspoon Street
 P.O. Box 214
 Princeton, NJ 08540
	  	1,444,446

 SCHEDULE A (continued) 
  

 Investors 
 SERIES A-1 PREFERRED STOCK 
  

			
	Name	  	Number of Shares held as of
the date of this Agreement
		
	 Archemix LLC
 300 Third Street
 Cambridge, MA 02142
	  	1,950,000
		
	 Facet Biotech Corporation
 1400 Seaport Blvd.
 Redwood City, CA 94063
	  	1,835,000
		
	 Biogen IDEC MA, Inc.
 14 Cambridge Center
 Cambridge, MA 02142
	  	1,835,000
		
	 Shellwater & Co.
 c/o Treasurer
 The Regents at University of California

PO Box 24000
 Oakland, CA
94623-1000
	  	330,000
		
	 University License Equity Holdings, Inc.

4740 Walnut St. Ste 100
 Boulder, CO
80309
	  	50,000

 SCHEDULE A (continued) 
  

 Investors 
 SERIES B PREFERRED STOCK 
  

			
	Name	  	Number of Shares held as of
the date of this Agreement
		
	 Clarus Lifesciences II, LP
 101 Main Street Suite 1210
 Cambridge, MA 02142
	  	15,000,000
		
	 SV Life Sciences Fund IV, L.P.
 One Boston Place, Suite 3900
 Boston, MA 02108
	  	6,117,974
		
	 SV Life Sciences Fund IV Strategic Partners, L.P.

One Boston Place, Suite 3900
 Boston, MA
02108
	  	173,692
		
	 International Biotechnology Trust plc

55 Moorgate
 London

EC2R 6PA
 United Kingdom
	  	1,000,000
		
	 Novo A/S
 Tuborg Havnevej 19
 DK-2900 Hellerup
 Denmark
	  	5,208,334
		
	 HBM Healthcare Investments (Cayman) Ltd.

Centennial Towers, 3rd Floor
 2454 West Bay
Road
 Grand Cayman
 Cayman
Islands
	  	2,083,334
		
	 Samir C. Patel LLC
 66 Witherspoon Street
 P.O. Box 214
 Princeton, NJ 08540
	  	416,666

 SCHEDULE A (continued) 
  

 Investors 
 SERIES B-1 PREFERRED STOCK 
  

			
	Name	  	Number of Shares held as of
the date of this Agreement
		
	 Archemix LLC
 300 Third Street
 Cambridge, MA 02142
	  	487,500
		
	 University License Equity Holdings, Inc.

4740 Walnut St. Ste 100
 Boulder, CO
80309
	  	12,500

 SCHEDULE A (continued) 
  

 Investors 
 SERIES C PREFERRED STOCK 
  

			
	Name	  	Number of Shares held as of
the date of this Agreement
		
	 Clarus Lifesciences II, LP
 101 Main Street Suite 1210
 Cambridge, MA 02142
	  	365,854
		
	 SV Life Sciences Fund IV, L.P.
 One Boston Place, Suite 3900
 Boston, MA 02108
	  	663,085
		
	 SV Life Sciences Fund IV Strategic Partners, L.P.

One Boston Place, Suite 3900
 Boston, MA
02108
	  	18,826
		
	 International Biotechnology Trust plc

55 Moorgate
 London

EC2R 6PA
 United Kingdom
	  	24,390
		
	 Novo A/S
 Tuborg Havnevej 19
 DK-2900 Hellerup
 Denmark
	  	5,146,003
		
	 HBM Healthcare Investments (Cayman) Ltd.

Centennial Towers, 3rd Floor
 2454 West Bay
Road
 Grand Cayman
 Cayman
Islands
	  	403,116
		
	 Samir C. Patel LLC
 66 Witherspoon Street
 P.O. Box 214
 Princeton, NJ 08540
	  	45,393

Source: [{"source": "alea-institute/alea-institute/kl3m-data-edgar-agreements/train-00220-of-00352.parquet"}, [{"source": "alea-institute/alea-institute/kl3m-data-edgar-agreements/train-00220-of-00352.parquet"}]]