Document:

Form of Warrant to Purchase Stock

 Exhibit 4.9 
 THIS WARRANT AND THE SHARES ISSUABLE HEREUNDER HAVE NOT BEEN REGISTERED UNDER THE SECURITIES ACT OF 1933, AS AMENDED (THE “ACT”), OR THE SECURITIES LAWS OF ANY STATE AND, EXCEPT AS SET FORTH IN
SECTIONS 5.3 AND 5.4 BELOW, MAY NOT BE OFFERED, SOLD, PLEDGED OR OTHERWISE TRANSFERRED UNLESS AND UNTIL REGISTERED UNDER SAID ACT AND LAWS OR IN FORM AND SUBSTANCE SATISFACTORY TO THE COMPANY, SUCH OFFER, SALE, PLEDGE OR OTHER TRANSFER IS EXEMPT
FROM SUCH REGISTRATION. 
 FORM OF 
 WARRANT TO PURCHASE STOCK 
 Company: HYPERION THERAPEUTICS, INC. 

Number of Shares: 231,343 
 Type/Series
of Stock: Common Stock 
 Warrant Price: $0.67 per share 
 Issue Date: April 19, 2012 
 Expiration Date: April 18, 2022 See also
Section 5.1(b). 
  

			
	Credit Facility:	  	This Warrant to Purchase Stock (“Warrant”) is issued in connection with that certain Loan and Security Agreement of even date herewith between Silicon
Valley Bank, Leader Lending, LLC—Series B and the Company (the “Loan Agreement”).

 THIS WARRANT CERTIFIES THAT, for good and valuable consideration,
             (together with any successor or permitted assignee or transferee of this Warrant or of any shares issued upon exercise hereof, “Holder”) is
entitled to purchase the number of fully paid and non-assessable shares (the “Shares”) of the above-stated Type/Series of Stock (the “Class”) of the above-named company (the
“Company”) at the above-stated Warrant Price, all as set forth above and as adjusted pursuant to Section 2 of this Warrant, subject to the provisions and upon the terms and conditions set forth in this Warrant. Reference
is made to Section 5.4 of this Warrant whereby Silicon Valley Bank shall transfer this Warrant to its parent company, SVB Financial Group. 
 SECTION 1. EXERCISE. 
 1.1 Method of Exercise. Holder may at any
time and from time to time exercise this Warrant, in whole or in part, by delivering to the Company the original of this Warrant together with a duly executed Notice of Exercise in substantially the form attached hereto as Appendix 1 and, unless
Holder is exercising this Warrant pursuant to a cashless exercise set forth in Section 1.2, a check, wire transfer of same-day funds (to an account designated by the Company), or other form of payment acceptable to the Company for the aggregate
Warrant Price for the Shares being purchased. 
 1.2 Cashless Exercise. On any exercise of this Warrant, in lieu of
payment of the aggregate Warrant Price in the manner as specified in Section 1.1 above, but otherwise in accordance with the requirements of Section 1.1, Holder may elect to receive Shares equal to the value of this Warrant, or portion
hereof as to which this Warrant is being exercised. Thereupon, the Company shall issue to the Holder such number of fully paid and non-assessable Shares as are computed using the following formula: 

  
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	 	X  =	Y(A-B)/A 

 where: 

 

	 	X  =	the number of Shares to be issued to the Holder; 

  

	 	Y  =	the number of Shares with respect to which this Warrant is being exercised (inclusive of the Shares surrendered to the Company in payment of the aggregate Warrant
Price); 

  

	 	A  =	the Fair Market Value (as determined pursuant to Section 1.3 below) of one Share; and 

 

	 	B  =	the Warrant Price. 

 1.3 Fair
Market Value. If the Company’s common stock is then traded or quoted on a nationally recognized securities exchange, inter-dealer quotation system or over-the-counter market (a “Trading Market”) the fair market value
of a Share shall be the closing price or last sale price of a share of common stock reported for the Business Day immediately before the date on which Holder delivers this Warrant together with its Notice of Exercise to the Company. If the
Company’s common stock is not traded in a Trading Market, the Board of Directors of the Company shall determine the fair market value of a Share in its reasonable good faith judgment. 

1.4 Delivery of Certificate and New Warrant. Within a reasonable time after Holder exercises this Warrant in the manner set forth
in Section 1.1 or 1.2 above, the Company shall deliver to Holder a certificate representing the Shares issued to Holder upon such exercise and, if this Warrant has not been fully exercised and has not expired, a new warrant of like tenor
representing the Shares not so acquired. 
 1.5 Replacement of Warrant. On receipt of evidence reasonably satisfactory to
the Company of the loss, theft, destruction or mutilation of this Warrant and, in the case of loss, theft or destruction, on delivery of an indemnity agreement reasonably satisfactory in form, substance and amount to the Company or, in the case of
mutilation, on surrender of this Warrant to the Company for cancellation, the Company shall, within a reasonable time, execute and deliver to Holder, in lieu of this Warrant, a new warrant of like tenor and amount. 

1.6 Treatment of Warrant Upon Acquisition of Company. 
 (a) Acquisition. For the purpose of this Warrant, “Acquisition” means any transaction or series of related transactions involving: (i) the sale, lease, exclusive license, or
other disposition of all or substantially all of the assets of the Company (ii) any merger or consolidation of the Company into or with another person or entity (other than a merger or consolidation effected exclusively to change the
Company’s domicile), or any other corporate reorganization, in which the stockholders of the Company in their capacity as such immediately prior to such merger, consolidation or reorganization, own less than a majority of the Company’s (or
the surviving or successor entity’s) outstanding voting power immediately after such merger, consolidation or reorganization; or (iii) any sale or other transfer by the stockholders of the Company of shares representing at least a majority
of the Company’s then-total outstanding combined voting power. 
 (b) Treatment of Warrant at Acquisition. In the event of
an Acquisition in which the consideration to be received by the Company’s stockholders consists solely of cash, solely of Marketable 

  
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Securities or a combination of cash and Marketable Securities (a “Cash/Public Acquisition”), either (i) Holder shall exercise this Warrant pursuant to Section 1.1
and/or 1.2 and such exercise will be deemed effective immediately prior to and contingent upon the consummation of such Acquisition or (ii) if Holder elects not to exercise the Warrant, this Warrant will expire immediately prior to the
consummation of such Acquisition. 
 (c) The Company shall provide Holder with written notice of its request relating to the
Cash/Public Acquisition (together with such reasonable information as Holder may reasonably require regarding the treatment of this Warrant in connection with such contemplated Cash/Public Acquisition giving rise to such notice), which is to be
delivered to Holder not less than seven (7) Business Days prior to the closing of the proposed Cash/Public Acquisition. In the event the Company does not provide such notice, then if, immediately prior to the Cash/Public Acquisition, the fair
market value of one Share (or other security issuable upon the exercise hereof) as determined in accordance with Section 1.3 above would be greater than the Warrant Price in effect on such date, then this Warrant shall automatically be deemed
on and as of such date to be exercised pursuant to Section 1.2 above as to all Shares (or such other securities) for which it shall not previously have been exercised, and the Company shall promptly notify the Holder of the number of Shares (or
such other securities) issued upon such exercise to the Holder and Holder shall be deemed to have restated each of the representations and warranties in Section 4 of the Warrant as the date thereof. 

(d) Upon the closing of any Acquisition other than a Cash/Public Acquisition defined above, the acquiring, surviving or successor entity
shall assume the obligations of this Warrant, and this Warrant shall thereafter be exercisable for the same securities and/or other property as would have been paid for the Shares issuable upon exercise of the unexercised portion of this Warrant as
if such Shares were outstanding on and as of the closing of such Acquisition, subject to further adjustment from time to time in accordance with the provisions of this Warrant. 

(e) As used in this Warrant, “Marketable Securities” means securities meeting all of the following requirements:
(i) the issuer thereof is then subject to the reporting requirements of Section 13 or Section 15(d) of the Securities Exchange Act of 1934, as amended (the “Exchange Act”), and is then current in its filing of
all required reports and other information under the Act and the Exchange Act; (ii) the class and series of shares or other security of the issuer that would be received by Holder in connection with the Acquisition were Holder to exercise this
Warrant on or prior to the closing thereof is then traded in Trading Market, and (iii) Holder would be able to publicly re-sell, within six (6) months following the closing of such Acquisition, all of the issuer’s shares and/or other
securities that would be received by Holder in such Acquisition were Holder to exercise this Warrant in full on or prior to the closing of such Acquisition. 
 SECTION 2. ADJUSTMENTS TO THE SHARES AND WARRANT PRICE. 
 2.1 Stock
Dividends, Splits, Etc. If the Company declares or pays a dividend or distribution on the outstanding shares of the Class payable in common stock or other securities or property (other than cash), then upon exercise of this Warrant, for each
Share acquired, Holder shall receive, without additional cost to Holder, the total number and kind of securities and property which Holder would have received had Holder owned the Shares of record as of the date the dividend or distribution
occurred. If the Company subdivides the outstanding shares of the Class by reclassification or otherwise into a greater number of shares, the number of Shares purchasable hereunder shall be proportionately increased and the Warrant Price shall be
proportionately decreased. If the outstanding shares of the Class are combined or consolidated, by reclassification or otherwise, into a lesser number of shares, the Warrant Price shall be proportionately increased and the number of Shares shall be
proportionately decreased. 

  
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 2.2 Reclassification, Exchange, Combinations or Substitution. Upon any event whereby
all of the outstanding shares of the Class are reclassified, exchanged, combined, substituted, or replaced for, into, with or by Company securities of a different class and/or series, then from and after the consummation of such event, this Warrant
will be exercisable for the number, class and series of Company securities that Holder would have received had the Shares been outstanding on and as of the consummation of such event, and subject to further adjustment thereafter from time to time in
accordance with the provisions of this Warrant. The provisions of this Section 2.2 shall similarly apply to successive reclassifications, exchanges, combinations substitutions, replacements or other similar events. 

2.3 No Fractional Share. No fractional Share shall be issuable upon exercise of this Warrant and the number of Shares to be issued
shall be rounded down to the nearest whole Share. If a fractional Share interest arises upon any exercise of the Warrant, the Company shall eliminate such fractional Share interest by paying Holder in cash the amount computed by multiplying the
fractional interest by (i) the fair market value (as determined in accordance with Section 1.3 above) of a full Share, less (ii) the then-effective Warrant Price. 

2.4 Notice/Certificate as to Adjustments. Upon each adjustment of the Warrant Price, Class and/or number of Shares, the Company,
at the Company’s expense, shall notify Holder in writing within a reasonable time setting forth the adjustments to the Warrant Price, Class and/or number of Shares and facts upon which such adjustment is based. The Company shall, upon written
request from Holder, furnish Holder with a certificate of its Chief Financial Officer, including computations of such adjustment and the Warrant Price, Class and number of Shares in effect upon the date of such adjustment. 

SECTION 3. REPRESENTATIONS AND COVENANTS OF THE COMPANY. 
 3.1 Representations and Warranties. The Company represents and warrants to, and agrees with, the Holder as follows: 
 (a) All Shares which may be issued upon the exercise of this Warrant, shall, upon issuance, be duly authorized, validly issued, fully paid and non-assessable, and free of any liens and encumbrances except
for restrictions on transfer provided for herein or under applicable federal and state securities laws. The Company covenants that it shall at all times cause to be reserved and kept available out of its authorized and unissued capital stock such
number of shares of common stock as will be sufficient to permit the exercise in full of this Warrant. 
 (b) The Company’s
capitalization table attached hereto as Schedule 1 is true and complete, in all material respects, as of the Issue Date. 
 3.2
Notice of Certain Events. If the Company proposes at any time to: 
 (a) declare any dividend or distribution upon the
outstanding shares of the Class, whether in cash, property, stock, or other securities and whether or not a regular cash dividend; 
 (b) offer for subscription or sale pro rata to the holders of the outstanding shares of the Class any additional shares of any class or series of the Company’s stock (other than pursuant to
contractual pre-emptive rights); 
 (c) effect any reclassification, exchange, combination, substitution, reorganization or
recapitalization of the outstanding shares of the Class; or 
 (d) effect an Acquisition or to liquidate, dissolve or wind up;

  
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then, in connection with each such event, the Company shall give Holder: 

(1) at least seven (7) Business Days prior written notice of the date on which a record will be taken for such dividend,
distribution, or subscription rights (and specifying the date on which the holders of outstanding shares of the Class will be entitled thereto) or for determining rights to vote, if any, in respect of the matters referred to in (a) and
(b) above; and 
 (2) in the case of the matters referred to in (c) and (d) above at least seven
(7) Business Days prior written notice of the date when the same will take place (and specifying the date on which the holders of outstanding shares of the Class will be entitled to exchange their shares for the securities or other property
deliverable upon the occurrence of such event); 
 Company will also provide information requested by Holder that is reasonably necessary to
enable Holder to comply with Holder’s accounting or reporting requirements. 
 SECTION 4. REPRESENTATIONS, WARRANTIES OF
THE HOLDER. 
 The Holder represents and warrants to the Company as follows: 

4.1 Purchase for Own Account. This Warrant and the securities to be acquired upon exercise of this Warrant by Holder are being
acquired for investment for Holder’s account, not as a nominee or agent, and not with a view to the public resale or distribution within the meaning of the Act. Holder also represents that it has not been formed for the specific purpose of
acquiring this Warrant or the Shares. 
 4.2 Disclosure of Information. Holder is aware of the Company’s business
affairs and financial condition and has received or has had full access to all the information it considers necessary or appropriate to make an informed investment decision with respect to the acquisition of this Warrant and its underlying
securities. Holder further has had an opportunity to ask questions and receive answers from the Company regarding the terms and conditions of the offering of this Warrant and its underlying securities and to obtain additional information (to the
extent the Company possessed such information or could acquire it without unreasonable effort or expense) necessary to verify any information furnished to Holder or to which Holder has access. 

4.3 Investment Experience. Holder understands that the purchase of this Warrant and its underlying securities involves substantial
risk. Holder has experience as an investor in securities of companies in the development stage and acknowledges that Holder can bear the economic risk of such Holder’s investment in this Warrant and its underlying securities and has such
knowledge and experience in financial or business matters that Holder is capable of evaluating the merits and risks of its investment in this Warrant and its underlying securities and/or has a preexisting personal or business relationship with the
Company and certain of its officers, directors or controlling persons of a nature and duration that enables Holder to be aware of the character, business acumen and financial circumstances of such persons. 

4.4 Accredited Investor Status. Holder is an “accredited investor” within the meaning of Regulation D promulgated under
the Act. 
 4.5 The Act. Holder understands that this Warrant and the Shares issuable upon exercise hereof have not been
registered under the Act in reliance upon a specific exemption therefrom, which exemption depends upon, among other things, the bona fide nature of the Holder’s investment intent as expressed herein. Holder understands that this Warrant and the
Shares issued upon any exercise 

  
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hereof must be held indefinitely unless subsequently registered under the Act and qualified under applicable state securities laws, or unless exemption from such registration and qualification
are otherwise available. Holder is aware of the provisions of Rule 144 promulgated under the Act. 
 4.6 Market Stand-off
Agreement. The Holder agrees that the Shares shall be subject to the Market Standoff provisions in Section 2.11 of the Second Amended and Restated Investor Rights Agreement or by and between the Company and the investors listed on
Exhibit A thereto dated June 29, 2009, as such may be amended from time to time. 
 4.7 No Voting Rights.
Holder, as a Holder of this Warrant, will not have any voting rights until the exercise of this Warrant. 
 SECTION 5.
MISCELLANEOUS. 
 5.1 Term and Automatic Conversion Upon Expiration. 

(a) Term. Subject to the provisions of Section 1.6 above, this Warrant is exercisable in whole or in part at any time and from
time to time on or before 6:00 PM, Pacific time, on the Expiration Date and shall be void thereafter. 
 (b) Automatic
Cashless Exercise upon Expiration. In the event that, upon the Expiration Date, the fair market value of one Share (or other security issuable upon the exercise hereof) as determined in accordance with Section 1.3 above is greater than the
Warrant Price in effect on such date, then this Warrant shall automatically be deemed on and as of such date to be exercised pursuant to Section 1.2 above as to all Shares (or such other securities) for which it shall not previously have been
exercised, and the Company shall, within a reasonable time, deliver a certificate representing the Shares (or such other securities) issued upon such exercise to Holder. 
 5.2 Legends. The Shares (and the securities issuable, directly or indirectly, upon conversion of the Shares, if any) shall be imprinted with a legend in substantially the following form:

 THE SHARES EVIDENCED BY THIS CERTIFICATE HAVE NOT BEEN REGISTERED UNDER THE SECURITIES ACT OF 1933, AS AMENDED (THE
“ACT”), OR THE SECURITIES LAWS OF ANY STATE AND, EXCEPT AS SET FORTH IN THAT CERTAIN WARRANT TO PURCHASE STOCK ISSUED BY THE ISSUER TO SILICON VALLEY BANK DATED APRIL __, 2012, MAY NOT BE OFFERED, SOLD, PLEDGED OR OTHERWISE
TRANSFERRED UNLESS AND UNTIL REGISTERED UNDER SAID ACT AND LAWS OR IN FORM AND SUBSTANCE SATISFACTORY TO THE ISSUER, SUCH OFFER, SALE, PLEDGE OR OTHER TRANSFER IS EXEMPT FROM SUCH REGISTRATION. 

5.3 Compliance with Securities Laws on Transfer. This Warrant and the Shares issuable upon exercise of this Warrant (and the
securities issuable, directly or indirectly, upon conversion of the Shares, if any) may not be transferred or assigned in whole or in part except in compliance with applicable federal and state securities laws by the transferor and the transferee
(including, without limitation, the delivery of investment representation letters and legal opinions reasonably satisfactory to the Company, as reasonably requested by the Company). The Company shall not require Holder to provide an opinion of
counsel if the transfer is to SVB Financial Group (Silicon Valley Bank’s parent company) or any other affiliate of Holder, provided that any such transferee is an “accredited investor” as

  
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defined in Regulation D promulgated under the Act. Additionally, the Company shall also not require an opinion of counsel if there is no material question as to the availability of Rule 144
promulgated under the Act. 
 5.4 Transfer Procedure. After receipt by Silicon Valley Bank of the executed Warrant,
Silicon Valley Bank will transfer all of this Warrant to its parent company, SVB Financial Group. By its acceptance of this Warrant, SVB Financial Group hereby makes to the Company each of the representations and warranties set forth in
Section 4 hereof and agrees to be bound by all of the terms and conditions of this Warrant as if the original Holder hereof. Subject to the provisions of Section 5.3 and upon providing the Company with written notice, SVB Financial Group
and any subsequent Holder may transfer all or part of this Warrant or the Shares issuable upon exercise of this Warrant (or the securities issuable directly or indirectly, upon conversion of the Shares, if any) to any transferee, provided, however,
in connection with any such transfer, SVB Financial Group or any subsequent Holder will give the Company notice of the portion of the Warrant being transferred with the name, address and taxpayer identification number of the transferee and Holder
will surrender this Warrant to the Company for reissuance to the transferee(s) (and Holder if applicable); and provided further, that any subsequent transferee other than SVB Financial Group shall agree in writing with the Company to be bound by all
of the terms and conditions of this Warrant. Notwithstanding any contrary provision herein, at all times prior to the IPO, Holder may not, without the Company’s prior written consent, transfer this Warrant or any portion hereof, or any Shares
issued upon any exercise hereof, or any shares or other securities issued upon any conversion of any Shares issued upon any exercise hereof, to any person or entity who directly competes with the Company, except in connection with an Acquisition of
the Company by such a direct competitor. 
 5.5 Notices. All notices and other communications hereunder from the Company
to the Holder, or vice versa, shall be deemed delivered and effective (i) when given personally, (ii) on the third (3rd) Business Day after being mailed by first-class registered or certified mail, postage prepaid, (iii) upon
actual receipt if given by facsimile or electronic mail and such receipt is confirmed in writing by the recipient, or (iv) on the first Business Day following delivery to a reliable overnight courier service, courier fee prepaid, in any case at
such address as may have been furnished to the Company or Holder, as the case may be, in writing by the Company or such Holder from time to time in accordance with the provisions of this Section 5.5. All notices to Holder shall be addressed as
follows until the Company receives notice of a change of address in connection with a transfer or otherwise: 
  

									
		  	 	  		  		  	
					
		  	 	  		  		  	
					
		  	 	  		  		  	

 Notice to the Company shall be addressed as follows until Holder receives notice of a change in address:

 HYPERION THERAPEUTICS, INC. 
 Attn: Donald Santel 
 601 Gateway Blvd., Suite 200 

South San Francisco, CA 94080 
 Telephone: (650) 745-7802 
 Facsimile: (650) 871-7029 

Email: don.santel@hyperiontx.com 

  
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 With a copy (which shall not constitute notice) to: 

HOGAN LOVELLS US LLP 
 Attn: Laura Berezin 
 525 University Avenue, 3rd Floor 

Palo Alto, CA 94301 
 Telephone: (650) 463-4194 
 Facsimile: (650) 463-4199 

Email: laura.berezin@hoganlovells.com 
 5.6 Waiver. This Warrant and any term hereof may be changed, waived, discharged or terminated (either generally or in a particular instance and either retroactively or prospectively) only by an
instrument in writing signed by the party against which enforcement of such change, waiver, discharge or termination is sought. 

5.7 Attorney’s Fees. In the event of any dispute between the parties concerning the terms and provisions of this Warrant, the
party prevailing in such dispute shall be entitled to collect from the other party all costs incurred in such dispute, including reasonable attorneys’ fees. 
 5.8 Counterparts; Facsimile/Electronic Signatures. This Warrant may be executed in counterparts, all of which together shall constitute one and the same agreement. Any signature page delivered
electronically or by facsimile shall be binding to the same extent as an original signature page with regards to any agreement subject to the terms hereof or any amendment thereto. 

5.9 Governing Law. This Warrant shall be governed by and construed in accordance with the laws of the State of California, without
giving effect to its principles regarding conflicts of law. 
 5.10 Headings. The headings in this Warrant are for
purposes of reference only and shall not limit or otherwise affect the meaning of any provision of this Warrant. 
 5.11
Business Days. “Business Day” is any day that is not a Saturday, Sunday or a day on which Silicon Valley Bank is closed. 
 [Remainder of page left blank intentionally] 
 [Signature page follows] 

  
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 IN WITNESS WHEREOF, the parties have caused this Warrant to Purchase Stock to be executed by
their duly authorized representatives effective as of the Issue Date written above. 
  

	
	 “COMPANY”

	
	 HYPERION THERAPEUTICS, INC.

	
	 By:

	
	 Name:

	
	             (Print)

	
	 Title:

	
	 “HOLDER”

	
	 SILICON VALLEY BANK

	
	 By:

	
	 Name:

	
	             (Print)

	
	 Title:

  

  
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 APPENDIX 1  
 NOTICE OF EXERCISE 
 1. The undersigned Holder hereby exercises its right
purchase             shares of the Common Stock of HYPERION THERAPEUTICS, INC. (the “Company”) in accordance with the attached Warrant To Purchase Stock, and tenders
payment of the aggregate Warrant Price for such shares as follows: 
 [    ]    
check in the amount of $             payable to order of the Company enclosed herewith 
 [    ]     Wire transfer of immediately available funds to the Company’s account 

[    ]     Cashless Exercise pursuant to Section 1.2 of the Warrant 

[    ]     Other [Describe]
                                         
                                        

2. Please issue a certificate or certificates representing the Shares in the name specified below: 

 

			
		 	
		 	  
 Holder’s
Name

		 	  

		
		 	  

		 	(Address)

  
 3. By its execution below and for the
benefit of the Company, Holder hereby restates each of the representations and warranties in Section 4 of the Warrant to Purchase Stock as of the date hereof. 
  

			
		 	 HOLDER:

 

		
		 	 By:

		
		 	 Name:

		
		 	 Title:

		
		 	 Date:

  
 Appendix 1

 SCHEDULE 1  

Company Capitalization Table 

  
 Schedule 1Restated Omnibus Amendment to Convertible Note and Warrant Purchase Agreement

 Exhibit 10.3 
 HYPERION THERAPEUTICS, INC. 
 RESTATED OMNIBUS AMENDMENT TO

 CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT 

DATED APRIL 1, 2011, 
 CONVERTIBLE UNSECURED PROMISSORY NOTES 
 DATED APRIL 1, 2011, MAY 2, 2011,
MAY 4, 2011 AND MAY 10, 2011 
 AND 
 WARRANTS TO PURCHASE SHARES OF COMMON STOCK 
 DATED APRIL 1, 2011, MAY 2,
2011, MAY 4, 2011 AND MAY 10, 2011 
 This Restated Omnibus Amendment (the “Amendment”) to the
Convertible Note and Warrant Purchase Agreement dated April 1, 2011, the Convertible Unsecured Promissory Notes dated April 1, 2011, May 2, 2011, May 4, 2011 and May 10, 2011 and the Warrants to Purchase Shares of
Common Stock, each dated April 1, 2011, May 2, 2011, May 4, 2011 and May 10, 2011 is made as of April [    ], 2012 (the “Effective Date”) by and among Hyperion Therapeutics,
Inc., a Delaware company (the “Company”) and the other parties listed on the signature pages hereto (the “Prior Purchasers”). All capitalized terms set forth herein shall have the meanings given to
such terms in the Prior Purchase Agreement, April/May 2011 Notes and April/May 2011 Warrants (each as defined below), unless otherwise defined herein. 
 RECITALS 
 A. The Company and the Prior Purchasers are parties to
that certain Convertible Note and Warrant Purchase Agreement dated April 1, 2011 (the “Prior Purchase Agreement”) between the Company and the Prior Purchasers, those certain Convertible Unsecured Promissory Notes dated
April 1, 2011, May 2, 2011, May 4, 2011 and May 10, 2011 issued to the Prior Purchasers pursuant to the Prior Purchase Agreement (the “April/May 2011 Notes”) and those certain Warrants to
Purchase Shares of Common Stock dated April 1, 2011, May 2, 2011, May 4, 2011 and May 10, 2011 issued to the Prior Purchasers pursuant to the Prior Purchase Agreement (the “April/May 2011
Warrants”). 
 B. The Company and the Prior Purchasers desire to amend a provision of the Prior Purchase Agreement
regarding a second closing of the sale of April/May 2011 Notes and April/May 2011 Warrants. 
 C. The Company and the Prior
Purchasers desire to amend a provision of the April/May 2011 Notes to extend the Maturity Date (as defined in the April/May 2011 Notes). 
 D. The Company and the Prior Purchasers desire to amend a provision of the April/May 2011 Warrants to correct a typographical error. 

E. The Prior Purchase Agreement, the April/May 2011 Notes and the April/May 2011 Warrants may be amended only upon the written consent of
the Company and the holders of at least sixty-six percent (66%) of the principal amount of the April/May 2011 Notes then outstanding. 

  
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 AGREEMENT 

In consideration of the mutual promises contained herein and other good and valuable consideration, receipt of which is hereby
acknowledged, the parties agree as follows: 
 1. Amendment of Prior Purchase Agreement. Section 2.3 of the Prior
Purchase Agreement is hereby amended to add to the end thereof the following: 
 “In the event the Second Closing has not
occurred by October 26, 2011, the Company’s obligation to issue and sell Notes in the Second Closing, and the Purchasers’ rights to elect to purchase and the obligations to purchase such Notes in the Second Closing shall terminate and
be of no further force and effect. 
 2. Amendment of the April/May 2011 Notes. Section 1 of each of the April/May
2011 Notes is amended in its entirety to read as follows: 
 “Maturity. Unless earlier converted into equity
securities of the Company pursuant to Section 3 of the Purchase Agreement, the principal amount of this Note, together with any accrued interest thereon, shall be due and payable in full on the earlier of (i) the demand of a Purchaser
Majority (as defined below), which shall not be earlier than December 31, 2012, and (ii) the occurrence of an Event of Default (as defined below) (such date, the “Maturity Date”).” 

3. Amendment of the April/May 2011 Warrants. Section 1(a)(ii) of each of the April/May 2011 Warrants is amended in its
entirety to read as follows: 
 “(ii) in the event that the Holder’s Note(s) have been converted into shares of Series
C-2 Preferred pursuant to Section 3.2 or 3.5 of the Purchase Agreement or into Common Stock pursuant to Section 3.3 of the Purchase Agreement, the number of Exercise Shares shall be equal to the quotient of (A) thirty percent
(30%) of the principal amount of the Note issued to such Purchaser at the Closing, divided by (B) the Series C-2 Original Issue Price (as defined in the Company’s Amended and Restated Certificate of Incorporation, as may be amended
from time to time (the “Restated Certificate”)); or” 
 4. No Other Amendments. Except as expressly
set forth above, all of the terms and conditions of the Prior Purchase Agreement, the April/May 2011 Notes and the April/May 2011 Warrants remain in full force and effect. 
 5. Governing Law. This Amendment will be governed by and construed in accordance with the laws of the State of California, regardless of the laws that might otherwise govern under applicable
principles of conflicts of law. 
 6. Counterparts. This Amendment may be executed in any number of counterparts, each of
which when so executed and delivered will be deemed an original, and all of which together shall constitute one and the same instrument. 
 7. Facsimile and Electronic Signatures. This Amendment may be executed and delivered by facsimile or electronic transmission and upon such delivery the facsimile or electronic signature will be
deemed to have the same effect as if the original signature had been delivered to the other party. The original signature copy shall be subsequently delivered to the other parties. The failure to deliver the

  
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original signature copy and/or the non-receipt of the original signature copy shall have no effect upon the binding and enforceable nature of this Amendment. 

[SIGNATURE PAGES FOLLOW] 

  
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 IN WITNESS WHEREOF, the parties hereto
have executed the AMENDMENT as of the date set forth in the first paragraph hereof. 
 COMPANY: 

Hyperion Therapeutics, Inc. 
  

			
		
	 Signature:
	 	 /s/ Donald J. Santel

		
	 Print Name:
	 	 Donald J. Santel

		
	 Title:
	 	 CEO

		
	 Address:
	 	 601 Gateway Blvd., Suite 200

		
		 	 South San Francisco, CA 94080

  
 [SIGNATURE
PAGE TO HYPERION THERAPEUTICS, INC. RESTATED OMNIBUS AMENDMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the AMENDMENT as of the date set forth in the first paragraph hereof. 
  

									
	PURCHASER:	 		 	NEW ENTERPRISE ASSOCIATES 12, LIMITED
			
		 		 	PARTNERSHIP
					
		 		 		 	By:	 	NEA Partners 12, Limited Partnership
					
		 		 		 	By:	 	NEA 12 GP, LLC
					
		 		 		 	By:	 	/s/ Louis S. Citron, General
Counsel                    

  
 [SIGNATURE
PAGE TO HYPERION THERAPEUTICS, INC. RESTATED OMNIBUS AMENDMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the AMENDMENT as of the date set forth in the first paragraph hereof. 
  

									
	PURCHASERS:	 		 	Highland Capital Partners VII Limited Partnership
					
		 		 		 	By:	 	Highland Management Partners VII Limited Partnership, its General Partner
					
		 		 		 	By:	 	Highland Management Partners VII, LLC, its General Partner
					
		 		 		 	By:	 	/s/ Corey Mulloy
		 		 	Authorized Manager

  

									
		 		 	Highland Capital Partners VII-B Limited Partnership
					
		 		 		 	By:	 	Highland Management Partners VII Limited Partnership, its General Partner
					
		 		 		 	By:	 	Highland Management Partners VII, LLC, its General Partner
					
		 		 		 	By:	 	/s/ Corey Mulloy
		 		 	Authorized Manager

  

									
		 		 	Highland Capital Partners VII-C Limited Partnership
					
		 		 		 	By:	 	Highland Management Partners VII Limited Partnership, its General Partner
					
		 		 		 	By:	 	Highland Management Partners VII, LLC, its General Partner
					
		 		 		 	By:	 	/s/ Corey Mulloy
		 		 	Authorized Manager

  
 [SIGNATURE
PAGE TO HYPERION THERAPEUTICS, INC. RESTATED OMNIBUS AMENDMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the AMENDMENT as of the date set forth in the first paragraph hereof. 
  

									
	PURCHASER:	 		 	Highland Entrepreneurs’ Fund VII Limited Partnership
					
		 		 		 	By:	 	Highland Management Partners VII Limited Partnership, its General Partner
					
		 		 		 	By:	 	Highland Management Partners VII, LLC, its General Partner
					
		 		 		 	By:	 	/s/ Corey Mulloy
		 		 	Authorized Manager

  
 [SIGNATURE
PAGE TO HYPERION THERAPEUTICS, INC. RESTATED OMNIBUS AMENDMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the AMENDMENT as of the date set forth in the first paragraph hereof. 
  

									
	PURCHASER:	 		 	SOFINNOVA VENTURE PARTNERS VII, L.P.
					
		 		 		 	By:	 	Sofinnova Management VII, LLC
					
		 		 		 		 	Its General Partner
					
		 		 		 	By:	 	/s/ James Healy
					
		 		 		 	Name:	 	James Healy
					
		 		 		 	Title:	 	Managing General Partner

  
  

[SIGNATURE PAGE TO HYPERION THERAPEUTICS, INC.
RESTATED OMNIBUS AMENDMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the AMENDMENT as of the date set forth in the first paragraph hereof. 
  

									
	PURCHASER:	 		 	WRF CAPITAL (WASHINGTON RESEARCH FOUNDATION)
					
		 		 		 	Signature:	 	/s/ Ronald S. Howell
					
		 		 		 	Print Name:	 	Ronald S. Howell
					
		 		 		 	Title:	 	CEO

  
 [SIGNATURE
PAGE TO HYPERION THERAPEUTICS, INC. RESTATED OMNIBUS AMENDMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the AMENDMENT as of the date set forth in the first paragraph hereof. 
  

									
	PURCHASER:	 		 	BAY CITY CAPITAL FUND V, L.P.
					
		 		 		 	By:	 	Bay City Capital Management V LLC, its General Partner
					
		 		 		 	By:	 	Bay City Capital LLC, its Manager
					
		 		 		 	By:	 	/s/ Fred Craves
		 		 	Name:	 	Fred Craves
		 		 	Title:	 	Manager and Managing Director

  
 [SIGNATURE
PAGE TO HYPERION THERAPEUTICS, INC. RESTATED OMNIBUS AMENDMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the AMENDMENT as of the date set forth in the first paragraph hereof. 
  

									
	PURCHASER:	 		 	BAY CITY CAPITAL FUND V CO-INVESTMENT FUND, L.P.
					
		 		 		 	By:	 	Bay City Capital Management V LLC, its General Partner
					
		 		 		 	By:	 	Bay City Capital LLC, its Manager
					
		 		 		 	By:	 	/s/ Fred Craves
		 		 	Name:	 	Fred Craves
		 		 	Title:	 	Manager and Managing Director

  
 [SIGNATURE
PAGE TO HYPERION THERAPEUTICS, INC. RESTATED OMNIBUS AMENDMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the AMENDMENT as of the date set forth in the first paragraph hereof. 
  

									
	PURCHASER:	 		 	PANORAMA CAPITAL, L.P.
					
		 		 		 	By:	 	 Panorama Capital Management, LLC

Its General Partner

					
		 		 		 	By:	 	/s/ Gaurav Aggarwal
					
		 		 		 	Name:	 	Gaurav Aggarwal
					
		 		 		 	Title:	 	Partner

  
 [SIGNATURE
PAGE TO HYPERION THERAPEUTICS, INC. RESTATED OMNIBUS AMENDMENT]

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