Document:

The October 2011 Purchase Agreement

 Exhibit 10.4 
 HYPERION THERAPEUTICS, INC. 
 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT 
 This Convertible Note and Warrant Purchase Agreement (this “Agreement”) is made as of
October 26, 2011 (the “Effective Date”) by and between Hyperion Therapeutics, Inc., a Delaware corporation (the “Company”), and each of the purchasers listed on Exhibit A attached to this Agreement (each
a “Purchaser” and together the “Purchasers”). 
 RECITALS 

The Company desires to issue and sell, severally and not jointly, and the Purchasers desire to purchase, severally and not jointly,
(i) convertible unsecured promissory notes in substantially the form attached to this Agreement as Exhibit B (individually, a “Note” and collectively, the “Notes”) in the aggregate principal amount of up
to fifteen million thirty thousand dollars ($15,030,000), which shall be convertible on the terms stated herein into equity securities of the Company and (ii) warrants to purchase stock of the Company in substantially the form attached to this
Agreement as Exhibit C (individually, a “Warrant” and collectively, the “Warrants”) for the purchase price set forth on Exhibit A. The Notes, Warrants and the equity securities issuable upon conversion
or exercise thereof (and the securities issuable upon conversion of such equity securities) are collectively referred to herein as the “Securities.” This Agreement, the Notes and the Warrants are collectively referred to herein as
the “Loan Documents.” 
 AGREEMENT 

In consideration of the mutual promises contained herein and other good and valuable consideration, receipt of which is hereby
acknowledged, the parties to this Agreement agree as follows: 
 1. PURCHASE AND SALE OF NOTES AND WARRANT.

 1.1 Issuance of Notes. On the terms of and subject to the conditions specified in this Agreement, the Company
hereby agrees to issue and sell to the Purchasers, severally and not jointly, and each Purchaser agrees, severally and not jointly, to purchase from the Company, the Notes on the terms and conditions included herein in such principal amounts at each
Closing (as defined below) as set forth on the Schedule of Purchasers attached as Exhibit A hereto. The Company’s obligations to the Purchasers shall be evidenced by the Notes delivered to the Purchasers on the date of the applicable
Closing. 
 1.2 Interest Rate. Interest shall accrue solely to the extent as set forth in the Notes. 

1.3 Term. All outstanding principal and interest due under any Note shall be due and payable as set forth therein.
Payment of all or any portion of the outstanding principal amount of any Note and all interest thereon shall be pari passu in right of payment and in all other respects to the other Notes. If the Company pays all or any portion of the outstanding
principal amount of any Note or any accrued and unpaid interest thereon, such payment shall be made at the same time as and pro rata with payment on each other Note. 
 1.4 No Usury. This Agreement and each Note issued pursuant to the terms of this Agreement are hereby expressly limited so that in no event whatsoever, whether by reason of deferment or
advancement of loan proceeds, acceleration of maturity of the loan evidenced hereby, or otherwise, shall the amount paid or agreed to be paid to the Purchasers hereunder for the loan, use, forbearance or detention of money exceed the maximum
interest rate permitted by the laws of the State of California. If 

 
at any time the performance of any provision hereof or any Note involves a payment exceeding the limit of the price that may be validly charged for the loan, use, forbearance or detention of
money under applicable law, then automatically and retroactively, ipso facto, the obligation to be performed shall be reduced to such limit, it being the specific intent of the Company and the Purchasers hereof that all payments under this Agreement
or any Note are to be credited first to interest as permitted by law, but not in excess of (i) the agreed rate of interest set forth in the Note, or (ii) that permitted by law, whichever is the lesser, and the balance toward the reduction
of principal. The provisions of this Section 1.4 shall never be superseded or waived and shall control every other provision of this Agreement and any Note. 
 1.5 Warrant Issuance. On the terms of and subject to the conditions specified in this Agreement, the Company further agrees to issue and sell to the Purchasers, severally and not jointly,
and each Purchaser agrees, severally and not jointly, to purchase from the Company, the Warrants on the terms and conditions included herein at each Closing for the aggregate purchase price set forth on the Schedule of Purchasers attached as
Exhibit A hereto. The number of shares of stock that the Investor shall be entitled to purchase upon exercise of the Warrant shall be determined in accordance with the terms and conditions of the Warrant. 

1.6 Warrant Purchase Price. In consideration for the issuance of the Warrants, at each Closing, the Purchasers shall
pay to the Company by check or wire transfer an amount for each Warrant equal to 0.01% of the principal amount of each Note it is issued. 
 1.7 The Company and the Purchasers, as a result of arm’s length bargaining, agree that: 
 (a) Neither the Purchasers nor any affiliated company has rendered any services to the Company in connection with this Agreement; 
 (b) The Warrants are not being issued as compensation; 
 (c) The aggregate fair
market value of the Notes, if issued apart from the Warrants, is equal to the aggregate of the total note amounts as listed on Exhibit A; and the aggregate fair market value of the Warrants, if issued apart from the Notes, is equal to the
aggregate total warrant amounts as listed on Exhibit A; and 
 (d) All tax returns and other return information of each
party relative to this Agreement and the Notes and Warrants issued pursuant hereto shall, as applicable, consistently reflect the matters agreed to in (a) through (d) above. 

2. CLOSINGS. 
 2.1 Initial Closing. The initial sale and purchase of the Notes and Warrants shall take place in a closing (the “Initial Closing”) to be held at the offices of Hogan Lovells
U.S. LLP, 525 University Ave., 3rd Floor, Palo Alto,
California 94301 (“Hogan Lovells”) on the date hereof or at such other time and place as the Company and the Purchasers having agreed to purchase a majority of the principal amount of the Notes at the Initial Closing to be purchased
hereunder mutually agree upon, orally or in writing. The Company will issue and sell to the Purchasers, and each Purchaser agrees, severally and not jointly, to purchase from the Company, Notes with an aggregate principal amount of up to seven
million five hundred thousand dollars ($7,500,000) in such principal amounts as is set forth under the heading “Initial Closing Note Amount” on the Schedule of Purchasers attached as Exhibit A hereto (the “Initial Closing
Notes”). 

  
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 2.2 Subsequent Initial Closing. For up to thirty (30) days following the
Initial Closing, the Company may sell and issue Notes with an aggregate principal amount of up to twelve thousand seven hundred fifty dollars ($12,750) (the “Subsequent Initial Closing Notes”) to one or more new Purchasers who is a
holder of preferred stock of the Company (other than those preferred stockholders that participated in the Initial Closing) (each a “New Purchaser”), pursuant to Section 9.3 hereof, in a subsequent closing (the
“Subsequent Initial Closing”). Any purchaser of Notes in the Subsequent Initial Closing shall become party to this Agreement and shall become subject to the terms and conditions of this Agreement, including, but not limited to
Section 2.3 hereof. The Subsequent Initial Closing shall be held at Hogan Lovells at a time and date specified by the Company or at such other time and place as the Company and the Purchasers having agreed to purchase a majority of the
principal amount of the Notes at the applicable Closing to be purchased hereunder mutually agree upon, orally or in writing. 

2.3 Second Closing (and Investor “Put” Right). So long as there has not been a Qualified Financing, Change in
Control or Initial Public Offering (each as defined below) on or before June 30, 2012, or in the event that the Notes issued in the Initial Closing or Subsequent Initial Closing have not previously been converted pursuant to Section 3
below, upon: (i) the election and approval, in writing, of at least 66% of the principal amount of the then-outstanding Notes (the “Purchaser Majority”) in their sole discretion, (ii) with advance notice provided to all
Purchasers, and (iii) subject to the satisfaction or waiver of the conditions set forth in Section 7 hereof, the Company will issue and sell to the Purchasers, severally and not jointly, and each Purchaser agrees, severally and not
jointly, to purchase from the Company, Notes with an aggregate principal amount of (a) seven million five hundred thousand dollars ($7,500,000) or (b) in the event that all or a portion of the Subsequent Initial Closing Notes have been
issued, up to seven million five hundred twelve thousand seven hundred fifty dollars ($7,512,750) (subject to the provisions of Section 9.3), in such principal amounts as is set forth under the heading “Second Closing Note Amount” on
the Schedule of Purchasers attached as Exhibit A hereto (the “Second Closing”); provided, however, in the event that the Notes issued in the Initial Closing or Subsequent Initial Closing will be converted
pursuant to Section 3.2 or 3.3 and the Second Closing has not yet occurred, the Company shall provide each Purchaser with twenty (20) days notice prior to the consummation of the Change of Control (as defined below) or Initial Public
Offering , as applicable. The Second Closing shall be held at Hogan Lovells at a time and date specified by the Company or at such other time and place as the Company and the Purchasers having agreed to purchase a majority of the principal amount of
the Notes at the Second Closing mutually agree upon, orally or in writing; provided, that in the event the Company has provided the Purchasers with notice of the consummation of a Change of Control or Initial Public Offering, as applicable, the
Second Closing shall occur no later than ten (10) days prior to the consummation of the Change of Control or Initial Public Offering, as applicable. The Initial Closing, Subsequent Initial Closing and the Second Closing are each referred to
herein as a “Closing.” Any sale of Notes at a Closing shall be upon the same terms and conditions as those contained herein; and such persons or entities, by delivery of the appropriate executed signature pages, shall become parties
to this Agreement and shall have the rights and obligations of a Purchaser hereunder. 
 2.4 Delivery. At each
Closing, the Company will deliver to each Purchaser a Note in the principal amount set forth opposite such Purchaser’s name on Exhibit A against receipt by the Company from each Purchaser of the principal amount set forth opposite
such Purchaser’s name on Exhibit A by check or wire transfer. Notwithstanding Section 11.7 herein, Exhibit A to this Agreement may be amended by the Company without the consent of Purchasers from time to time to reflect the
addition of new Purchasers and the principal amount of Notes to be purchased by such Purchasers in any Closing. 

  
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 3. CONVERSION. 

3.1 Qualified Financing. If, after the date hereof and prior to conversion pursuant to Sections 3.2, 3.3, 3.4 or 3.5, a
Qualified Financing occurs, the entire unpaid principal amount and unpaid accrued interest (if any) of each Note shall be automatically converted into that number of shares of the series of the Company’s preferred stock issued in the Qualified
Financing (the “New Preferred Stock”) equal to the quotient of (i) the outstanding principal amount plus unpaid accrued interest (if any) divided by (ii) the price per share paid by the investors purchasing New Preferred
Stock in the Qualified Financing. For purposes of the Loan Documents, “Qualified Financing” shall mean the issuance and sale by the Company of New Preferred Stock in consideration of which the Company receives aggregate gross
proceeds of not less than forty million dollars ($40,000,000) (including conversion of the Notes) and “New Investor” shall mean a venture capital, institutional or corporate investor that is not, immediately prior to the closing of
the Qualified Financing, (i) a holder of the preferred stock of the Company and (ii) that invests at least five million dollars ($5,000,000) in the Qualified Financing. For purposes of clarity, the issuance of forty million dollars
($40,000,000) in Notes (including the April 2011 Bridge Notes, as defined below) shall not (or the conversion of any such notes into equity shall not) constitute a Qualified Financing. Shares of New Preferred Stock issuable upon conversion of the
Notes shall be issued on the same terms and conditions as the shares sold to the investors in the Qualified Financing. 
 3.2
Conversion Upon Change of Control. If , after the date hereof and prior to conversion pursuant to Sections 3.1, 3.3, 3.4 or 3.5, a Change of Control occurs, immediately prior to the effective date of such Change of Control, the entire
unpaid principal amount and unpaid accrued interest (if any) of each Note shall be automatically converted into that number of shares of a new series of preferred stock (the “Series X Preferred”) equal to the quotient of
(i) the outstanding principal amount plus accrued interest (if any) divided by (ii) the Series C-2 Original Issue Price (as defined in the Company’s Amended and Restated Certificate of Incorporation (the “Restated
Certificate”)). Prior to the effective date of such Change of Control, the Company shall amend its Restated Certificate to authorize the Series X Preferred. The Series X Preferred Stock shall have rights, preferences and privileges
identical to the Company’s existing Series C-1 Preferred Stock and Series C-2 Preferred Stock (together, the “Preferred Stock”) and shall be pari passu with such Preferred Stock, provided, however, that upon a Liquidation Event
(as defined in the Restated Certificate), before any distribution or payment is made to the holders of Preferred Stock or the Company’s common stock (“Common Stock”), the Series X Preferred shall be entitled to be paid out of
the assets of the Company legally available for distribution an amount per share of Series X Preferred Stock equal to three times the Series C-2 Original Issue Price. For purposes of the Loan Documents, “Change of Control” shall
mean (i) a merger with or into or consolidation with any other corporation, limited liability company or other entity (other than a wholly owned subsidiary of the Company) or any other transaction or series of related transactions in which in
excess of fifty percent (50%) of the Company’s voting power is transferred; or (ii) a sale, lease, exclusive license or other disposition of all or substantially all of the assets or intellectual property of the Company in a single
transaction or series of related transactions; provided, however, that a Change of Control of the Company shall not include any transaction or series of transactions principally for bona fide equity financing purposes in which cash is
received by the Company or any successor or indebtedness of the Company is cancelled or converted or a combination thereof. The Company shall provide each of the Purchasers written notice no less than twenty (20) days’ notice prior to the
effective date of such Change of Control transaction. 
 3.3 Conversion upon Initial Public Offering. If, after
the date hereof and prior to conversion pursuant to Sections 3.1, 3.2, 3.4 or 3.5, an Initial Public Offering occurs, immediately prior to the closing of such Initial Public Offering, the entire unpaid principal amount and unpaid accrued interest
(if any) of each Note shall be automatically converted into that number of shares of the Company’s Common Stock equal to the quotient of (i) the outstanding principal amount of the note plus accrued

  
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interest (if any) divided by (ii) the price to the public of the shares of Common Stock to be sold in the Initial Public Offering. For purposes of the Loan Documents, “Initial Public
Offering” shall mean the Company’s first firm commitment underwritten public offering of its Common Stock pursuant to a registration statement declared effective under the Securities Act of 1933, as amended (the “Securities
Act”). 
 3.4 Optional Conversion. If, after the date hereof and prior to conversion pursuant to Sections
3.1, 3.2, 3.3 or 3.5, the Company consummates an equity financing, other than a Qualified Financing (a “Non-Qualified Financing”), each Holder shall have the option to covert its respective Note(s) into a number of shares of equity
securities issued in the Non-Qualified Financing, equal to the quotient of (i) the outstanding principal amount of the note plus unpaid accrued interest (if any) divided by (ii) the lowest price per share paid by the investor in the
Non-Qualified Financing. In addition, upon the election of the Purchaser Majority, the entire unpaid principal amount and unpaid accrued interest (if any) of each Note shall be automatically converted into that number of shares of equity securities
issued in the Non-Qualified Financing in the same manner as specified in the foregoing sentence. 
 3.5 Conversion at
Maturity. If the Notes have not been previously converted prior to the Maturity Date (as such term is defined in the Notes), upon the written election of the Purchaser Majority the entire unpaid principal amount and unpaid accrued interest
(if any) of each Note shall be automatically converted into that number of shares of the Company’s Series C-2 Preferred equal to the quotient of (i) the outstanding principal amount plus unpaid accrued interest (if any) divided by
(ii) the Series C-2 Original Issue Price. 
 3.6 Mechanics and Effect of Conversion. No fractional shares of
the Company’s capital stock will be issued upon conversion of the Notes. In lieu of any fractional share to which a holder of a Note would otherwise be entitled, the Company will pay to such holder in cash the amount of the unconverted
principal balance and accrued interest balance (if any) of such Note that would otherwise be converted into such fractional share. Upon conversion of the Notes pursuant to this Section 3, each holder shall surrender its Note(s), duly endorsed,
at the principal offices of the Company or any transfer agent of the Company or provide evidence and indemnity reasonably satisfactory to the Company of the loss, theft, destruction or mutilation of its Note(s). At its expense, the Company will, as
soon as practicable thereafter, issue and deliver to such holder, at such principal office, a certificate or certificates for the number of shares to which such holder is entitled upon such conversion and a check or wire transfer payable to such
holder for any cash amounts payable as described herein. Upon conversion of the Notes, the Company will be forever released from all of its obligations and liabilities under the Loan Documents including without limitation the obligation to pay the
principal amount and accrued interest of the Notes. 
 3.7 Further Assurances. Each Purchaser understands that the
conversion of the Notes into, and the sale and purchase of, equity securities of the Company may require such Purchaser’s execution of, among other things, (i) written actions for the amendment of the Company’s Restated Certificate,
to increase the existing authorized capital stock of the Company and/or authorize a new series of preferred stock, and (ii) certain agreements relating to the purchase and sale of such securities as well as registration, co-sale and voting
rights, if any, relating to such equity securities, and each Purchaser hereby agrees to execute such written actions and agreements and to take such further actions necessary or advisable to consummate the transactions contemplated hereby upon
request by the Company after the time of conversion. 

  
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 4. REPRESENTATIONS AND WARRANTIES OF THE COMPANY. 

The Company represents and warrants to the Purchasers as follows: 

4.1 Organization, Good Standing and Qualification. The Company is a corporation duly organized, validly existing and in
good standing under the laws of the State of Delaware and has all requisite corporate power and authority to own and operate its properties and assets and to carry on its business as presently conducted. The Company is duly qualified to transact
business and is in good standing in each jurisdiction in which the failure so to qualify would have a material adverse effect on its business or properties. 
 4.2 Authorization. Except for: (a) filing an amendment to the Restated Certificate to authorize a new series of Preferred Stock and additional shares of Common Stock issuable upon
conversion of Preferred Stock issued upon conversion of the Notes or exercise of the Warrants and (b) the reservation of (i) shares of Preferred Stock issuable upon conversion of the Notes or exercise of the Warrants or (ii) shares of
Common Stock issuable upon conversion of the Preferred Stock into which the Notes have been converted or the Warrants have been exercised, all corporate action on the part of the Company, its officers, directors and stockholders necessary for
(a) execution and delivery of the Loan Documents, (b) the authorization, sale, issuance and delivery of the Notes and Warrants, (c) the issuance of the capital stock of the Company into which the Notes are convertible (the
“Conversion Stock”), (d) the issuance of the Common Stock into which the Warrants are exercisable, (e) the issuance of the Common Stock underlying the Conversion Stock (the “Underlying Common”) and
(f) the performance of all obligations of the Company under the Loan Documents required to be taken before the applicable Closing has been taken or will be taken prior to the applicable Closing. The Loan Documents, when executed and delivered
by the Company, shall constitute valid and legally binding obligations of the Company, enforceable against the Company in accordance with their terms, except (i) as limited by applicable bankruptcy, insolvency, reorganization, moratorium,
fraudulent conveyance and other laws of general application affecting enforcement of creditors’ rights generally, (ii) as limited by laws relating to the availability of specific performance, injunctive relief or other equitable remedies
and (iii) to the extent any indemnification provisions may be limited by applicable federal or state securities laws. The terms of the transactions contemplated by the Loan Documents have been recommended to the Company’s Board of
Directors by the Company’s management and unanimously approved by all directors not having an interest in the transactions contemplated by the Loan Documents. 
 4.3 Governmental Consent. No consent, approval, order or authorization of, or registration, qualification, designation, declaration or filing with, any federal, state or local governmental
authority on the part of the Company is required in connection with the valid execution, delivery and performance by the Company of the Loan Documents and the transactions contemplated thereby, except for filings pursuant to Section 25102(f) of
the California Corporate Securities Law of 1968, as amended, and the rules thereunder, other applicable state securities laws and Regulation D of the Securities Act, all of which filings will be effected after the applicable Closing.

 4.4 Compliance with Other Instruments. The Company is not in violation or default of any provision of the
Restated Certificate or its Bylaws as currently in effect. The Company is not in violation of, or default under any provision of any instrument, mortgage, deed of trust, loan, contract, commitment or obligation to which it is a party or by which it
or any of its properties are bound, which violations or defaults, individually or in the aggregate, would materially adversely affect the business, properties or condition (financial or otherwise) of the Company. The Company is not in violation of
any provision of any federal, state or local statute, rule or governmental regulation which would materially adversely affect the business, properties or condition (financial or otherwise) of the Company or any judgment, decree or order to which it
is a party, in any material respect. The Company 

  
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has all franchises, permits, licenses and any similar authority necessary for the conduct of its business, the lack of which could materially and adversely affect the business, properties or
condition (financial or otherwise) of the Company. The Company is not in default in any material respect under any of such franchises, permits, licenses or other similar authority. 

4.5 Litigation. There is no action, suit, proceeding or investigation pending or, to the Company’s knowledge,
currently threatened against the Company that questions the validity of the Loan Documents or the right of the Company to enter into any of such Loan Document, or to consummate the transactions contemplated hereby or thereby, nor is the Company
aware that there is any basis for the foregoing. Except for the arbitration proceeding with Medicis Pharmaceutical Corporation, there is no action, suit, proceeding or investigation pending or, to the Company’s knowledge, currently threatened
against the Company that might result, either individually or in the aggregate, in any material adverse effect on the business, properties or condition (financial or otherwise) of the Company, or any change in the current equity ownership of the
Company, nor is the Company aware that there is any basis for the foregoing. The foregoing includes, without limitation, actions pending or threatened (or any basis therefor known to the Company) involving the prior employment of any of the
Company’s employees, their use in connection with the Company’s business of any information or techniques allegedly proprietary to any of their former employers, or their obligations under any agreements with prior employers. The Company
is not a party or subject to the provisions of any order, writ, injunction, judgment or decree of any court or government agency or instrumentality. There is no action, suit, proceeding or investigation by the Company currently pending or that the
Company intends to initiate. 
 4.6 Capitalization. 

(a) The authorized capital stock of the Company, immediately prior to the Initial Closing, consists of (i) 80,000,000 shares of
Common Stock, par value $0.0001 per share, 2,858,251 shares of which are issued and outstanding and (ii) 66,000,000 shares of Preferred Stock, par value $0.0001 per share, 11,647,769 of which are designated Series C-1 Preferred Stock, all of
which are issued and outstanding and 53,673,645 of which are designated Series C-2 Preferred Stock, 28,397,980 of which are issued and outstanding. 
 (b) The Company has reserved 8,823,187 shares of Common Stock for issuance pursuant to the Company’s 2006 Equity Incentive Plan (the “Plan”), of which (i) 400,546 shares of
Common Stock have been issued pursuant to the exercise of outstanding options and are included in the number of shares of Common Stock outstanding as reflected in Section 4.6(a)(i) above, (ii) options to purchase 7,920,296 shares of
Common Stock have been approved for grant, are issued and are unexercised, and (iii) 502,345 shares of Common Stock remain available for future grant and issuance to officers, directors, employees and consultants of the Company. The Company
reserved (i) 11,647,769 shares of Common Stock for conversion of the Series C-1 Preferred Stock, (ii) 28,397,980 shares of Common Stock for conversion of the outstanding Series C-2 Preferred Stock. 

(c) Other than the shares reserved for issuance under the Plan, 1,810 shares of Common Stock reserved for issuance pursuant to
outstanding warrants to purchase Common Stock, $17.5 million principal amount of Convertible Unsecured Promissory Notes issued on April 1, 2011 (the “April 2011 Bridge Notes”) pursuant to the Convertible Note and Warrant
Purchase Agreement, dated April 1, 2011 between the Company and the Purchasers (the “Prior Purchase Agreement”) and Warrants to purchase shares of Common Stock issued on April 1, 2011 pursuant to the Prior Purchase
Agreement (the “April 2011 Bridge Warrants”), there are no outstanding options, warrants, rights (including conversion or preemptive rights and rights of first refusal), proxy or stockholder agreements, or agreements of any kind for
the purchase or acquisition from the Company of any of its securities. 

  
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 (d) As of the date hereof, each outstanding share of Preferred Stock is convertible into
one share of Common Stock, and the issuance of the Notes and Warrants contemplated hereunder will not result in any anti-dilution adjustment or other similar adjustment to the conversion rate of the outstanding shares of Preferred Stock. 

4.7 Financial Statements. The Company has delivered to the Purchasers true and complete copies of (i) the audited
balance sheets of the Company as of December 31, 2009 and 2010; (ii) the audited statements of income and cash flows of the Company for each of the three years ended December 31, 2008, 2009 and 2010; (iii) the unaudited balance
sheet of the Company as of September 30, 2011 and (iv) unaudited statements of income and cash flows of the Company for the nine-month period ended September 30, 2011 (all of such financial statements, collectively, the
“Financial Statements”). The Financial Statements were prepared in accordance with GAAP consistently applied, and fairly present the financial condition and results of operations and cash flows of the Company as of the stated date
and for the periods referred to therein, except that the unaudited Financial Statements may not contain all footnotes required by generally accepted accounting principles. 
 4.8 Full Disclosure. To the Company’s knowledge, neither this Agreement nor the Notes or Warrants, nor any other document delivered by the Company to Purchasers or their attorneys or
agents in connection herewith or therewith at the applicable Closing or with the transactions contemplated hereby or thereby, contain any untrue statement of a material fact nor, to the Company’s knowledge, omit to state a material fact
necessary in order to make the statements contained herein or therein not misleading. 
 5. REPRESENTATIONS AND WARRANTIES
OF THE PURCHASERS. 
 Each Purchaser, severally and not jointly, hereby represents and warrants to the Company as to
itself only that: 
 5.1 Requisite Power and Authority. The Purchaser has all necessary power and authority under
all applicable provisions of law to execute and deliver the Loan Documents and to carry out their provisions. All action on the Purchaser’s part required for the lawful execution and delivery of the Loan Documents has been taken. Upon their
execution and delivery, the Loan Documents will constitute a valid and legally binding obligation of the Purchaser, enforceable in accordance with their terms, except (i) as limited by applicable bankruptcy, insolvency, reorganization,
moratorium, fraudulent conveyance and other laws of general application affecting enforcement of creditors’ rights generally, (ii) as limited by laws relating to the availability of specific performance, injunctive relief or other
equitable remedies and (iii) to the extent any indemnification provisions may be limited by applicable federal or state securities laws. 
 5.2 Experience. The Purchaser is experienced in investing in the securities of development stage companies such as the Company and acknowledges that investment in the Securities involves a
number of significant risks, it is able to fend for itself, it can bear the economic risk of its investment, including the full loss of its investment, and it has such knowledge and experience in financial or business matters that it is capable of
evaluating the merits and risks of the investment in the Securities. The Purchaser also represents it was not organized solely for the purpose of acquiring the Securities. 
 5.3 Accredited Investor. The Purchaser represents that it is an “accredited investor” within the meaning of Rule 501(a) of the Securities Act. 

  
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 5.4 Foreign Investors. If the Purchaser is not a United States person (as
defined by Section 7701(a)(30) of the Internal Revenue Code of 1986, as amended), the Purchaser hereby represents that it has satisfied itself as to the full observance of the laws of its jurisdiction in connection with any invitation to
subscribe for the Securities or any use of this Agreement, including (i) the legal requirements within its jurisdiction for the purchase of the Securities, (ii) any foreign exchange restrictions applicable to such purchase, (iii) any
government or other consents that may need to be obtained, and (iv) the income tax and other tax consequences, if any, that may be relevant to the purchase, holding, redemption, sale or transfer of the Securities. The Purchaser’s
subscription and payment for and continued beneficial ownership of the Securities will not violate any applicable securities or other laws of the Purchaser’s jurisdiction. 

5.5 Residence. The Purchaser’s principal residence or office location is in the state identified in the address of the
Purchaser set forth beneath its signature hereto. 
 5.6 Disclosure of Information. The Purchaser has received and
reviewed information about the Company and has had an opportunity to discuss the Company’s business, management and financial affairs with its management and to review the Company’s facilities. The Purchaser understands and acknowledges
that such discussions, as well as any written information issued by the Company, (i) were intended to describe the aspects of the Company’s business which the Company believes to be material, but were not necessarily an exhaustive
description, and (ii) may have contained forward-looking statements involving known and unknown risks and uncertainties which may cause the Company’s actual results in future periods or plans for future periods to differ materially from
what was anticipated and that no representations or warranties were or are being made with respect to any such forward-looking statements or the probability of achieving any of the results projected in any of such forward-looking statements. The
foregoing, however, does not limit or modify the representations and warranties of the Company in Section 4 of this Agreement or the right of the Purchaser to rely thereon. 

5.7 Purchase Entirely for Own Account. This Agreement is made with the Purchaser in reliance upon the Purchaser’s
representation to the Company, which by the Purchaser’s execution of this Agreement the Purchaser hereby confirms, that the Securities to be received by the Purchaser will be acquired for investment for the Purchaser’s own account, not as
a nominee or agent, and not with a view to the resale or distribution of any part thereof, and that the Purchaser has no present intention of selling, granting any participation in, or otherwise distributing the same. The Purchaser further
represents that it does not have any contract, undertaking, agreement or arrangement with any person to sell, transfer or grant participations to such person or to any third person, with respect to the Securities. 

5.8 Restricted Securities. The Purchaser understands that the Securities are characterized as “restricted
securities” under the federal securities laws inasmuch as they are being acquired from the Company in a transaction not involving a public offering and that under such laws and applicable regulations such Securities may be resold without
registration under the Securities Act only in certain limited circumstances. In this connection, the Purchaser represents that it is familiar with Rule 144, as presently in effect, and understands the resale limitations imposed thereby and by the
Securities Act. The Purchaser must bear the economic risk of this investment indefinitely unless the Securities are registered pursuant to the Securities Act, or an exemption from registration is available. The Purchaser understands that the Company
has no present intention of registering the Securities. The Purchaser also understands that there is no assurance that any exemption from registration under the Securities Act will be available and that, even if available, such exemption may not
allow the Purchaser to transfer all or any portion of the Securities under the circumstances, in the amounts or at the times the Purchaser might propose. 

  
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 5.9 No Public Market. The Purchaser understands that no public market now
exists for any of the securities issued by the Company and that the Company has made no assurances that a public market will ever exist for the Securities. 
 5.10 Special Mandatory Conversion. Each Purchaser acknowledges and agrees that if a Purchaser holds, at the time of delivery of the Issuance Notice (as defined in the Restated Certificate),
an aggregate of at least 60,000 shares of the Company’s Series C-1 Preferred Stock (the “Series C-1 Preferred”) or the Company’s Series C-2 Preferred Stock (the “Series C-2 Preferred”) (as adjusted for any
stock dividends, combinations, splits, recapitalizations and the like) and fails to purchase (a) in the Initial Closing and (b) the Second Closing, if any, a Note in the principal amount set forth on Exhibit A under “Initial
Closing Note Amount” and “Second Closing Note Amount,” as applicable, then (i) all of the total shares of Series C-1 Preferred and Series C-2 Preferred then-held by such Purchaser shall automatically be converted into shares of
the Company’s Common Stock on a 10-for-1 basis in accordance with Article IV.D.5(p) of the Restated Certificate and (ii) to the extent that the Series C-1 Preferred Conversion Price (as defined in the Restated Certificate) or Series C-2
Conversion Price (as defined in the Restated Certificate) has been adjusted pursuant to a previously Qualifying Dilutive Issuance (as defined in the Restated Certificate) the conversion of the Series C-1 Preferred and/or Series C-2 Preferred, as
applicable, shall apply as if no such previous adjustment(s) had been applied to the Series C-1 Conversion Price or Series C-2 Conversion Price, and (iii) all rights with respect to the Series C-1 Preferred and/or Series C-2 Preferred converted
pursuant to Article IV.D.5(p) of the Restated Certificate will terminate at the time of such conversion (notwithstanding the failure of the holder or holders thereof to surrender the certificates for such shares on or prior to such time), except
only the rights of the holders thereof, upon surrender of the certificate or certificates therefor, to receive a certificate or certificates for the number of full shares of Common Stock issuable on such conversion. Any person or entity to whom
shares of Series C-1 Preferred and/or Series C-2 Preferred are transferred by a Purchaser, whether voluntarily or by operation of law, shall be bound by this Section 5.10, and such shares shall remain subject to the conversion provisions
hereunder and under the Restated Certificate, as may be amended from time to time, until such time as all Notes that the transferring Purchaser was required to purchase at the Closings are purchased. In addition, in the event that a Purchaser fails
to purchase Notes in the principal amount allocated to it in the Initial Closing or Second Closing such Notes shall be made available for the Purchasers who participate in such Closing to purchase on a pro rata basis (based on the principal amount
of the Notes such participating Investors have purchased in the Initial Closing or Second Closing, as applicable). 
 5.11
Legends. The Purchaser understands that the Securities, and any securities issued in respect thereof or exchange therefor, may bear one or all of the following legends: 

(a) “THESE SECURITIES HAVE NOT BEEN REGISTERED UNDER THE SECURITIES ACT OF 1933, AS AMENDED, OR ANY APPLICABLE STATE SECURITIES
LAWS AND HAVE BEEN ACQUIRED FOR INVESTMENT AND NOT WITH A VIEW TO, OR IN CONNECTION WITH, THE SALE OR DISTRIBUTION THEREOF. NO SUCH SALE OR DISTRIBUTION MAY BE EFFECTED WITHOUT AN EFFECTIVE REGISTRATION STATEMENT OR QUALIFICATION RELATED THERETO OR
AN OPINION OF COUNSEL IN A FORM SATISFACTORY TO THE COMPANY THAT SUCH REGISTRATION OR QUALIFICATION IS NOT REQUIRED UNDER THE SECURITIES ACT OF 1933, AS AMENDED, OR ANY APPLICABLE STATE SECURITIES LAWS.” 

(b) “THESE SECURITIES ARE SUBJECT TO, AND MAY BE TRANSFERRED ONLY IN COMPLIANCE WITH, THAT CERTAIN CONVERTIBLE NOTE AND WARRANT
PURCHASE AGREEMENT BETWEEN THE ISSUER AND THE HOLDER, A COPY OF WHICH IS ON FILE AT THE PRINCIPAL OFFICE OF THE ISSUER.” 

  
 10 

 (c) Any legend required by the blue sky laws of any state to the extent such laws are
applicable to the securities represented by the certificate or other document so legended, and any legends placed on the New Preferred Stock in connection with the Qualified Financing or such other equity securities issued upon conversion of the
Notes. 
 6. CONDITIONS OF THE PURCHASERS’ OBLIGATIONS AT THE INITIAL CLOSING. 

The obligations of each Purchaser to the Company under this Agreement are subject to the fulfillment, on or before the Initial Closing,
of each of the following conditions, unless waived by the Purchaser Majority of the principal amount of the Notes to be issued in the Initial Closing: 
 6.1 Representations and Warranties; Performance of Obligations. The representations and warranties of the Company contained in Section 4 shall be true on and as of the Initial Closing,
and the Company shall have performed all obligations and conditions herein required to be performed or observed by it on or prior to the Initial Closing. 
 6.2 Qualifications. All authorizations, approvals or permits, if any, of any governmental authority or regulatory body of the United States or of any state that are required in connection
with the lawful issuance and sale of the Securities pursuant to this Agreement shall be obtained and effective as of the Initial Closing. 
 6.3 Restated Certificate. The Company’s Restated Certificate shall continue to be in full force and effect as of the Initial Closing. 

6.4 Compliance Certificate. The Company shall have delivered to the Purchasers a Compliance Certificate, executed by the
Chief Executive Officer of the Company, dated the date of the Initial Closing, to the effect that the conditions specified in Section 6.1 have been satisfied. 
 6.5 Secretary’s Certificate. The Purchasers shall have received from the Company’s Secretary, a certificate having attached thereto (i) the Company’s Amended and Restated
Certificate of Incorporation as in effect at the time of the Initial Closing; (ii) the Company’s Bylaws as in effect at the time of the Initial Closing; (iii) resolutions approved by the Board of Directors authorizing the transactions
contemplated hereby and (iv) good standing certificates (including tax good standing) with respect to the Company from the applicable authority(ies) in Delaware and any other jurisdiction in which the Company is qualified to do business, dated
a recent date before the date of the Initial Closing. 
 7. CONDITIONS OF THE PURCHASERS’ OBLIGATIONS AT THE
ADDITIONAL CLOSINGS. 
 The obligations of each Purchaser to the Company under this Agreement are subject to the
fulfillment, on or before the Subsequent Initial Closing or Second Closing, as applicable (the “Additional Closing”), of the following conditions, unless waived by the Purchaser Majority of the principal amount of the Notes to be
issued in such Closing: 
 7.1 Representations and Warranties; Performance of Obligations. The representations and
warranties of the Company contained in Section 4 shall be true on and as of such Closing, and the Company shall have performed all obligations and conditions herein required to be performed or observed by it on or prior to the Additional
Closing. 

  
 11 

 7.2 Qualifications. All authorizations, approvals or permits, if any, of any
governmental authority or regulatory body of the United States or of any state that are required in connection with the lawful issuance and sale of the Securities pursuant to this Agreement shall be obtained and effective as of the Additional
Closing. 
 7.3 Compliance Certificate. The Company shall have delivered to the Purchasers a Compliance
Certificate, executed by the Chief Executive Officer of the Company, dated the date of such Closing, to the effect that the conditions specified in Sections 7.1 have been satisfied. 

7.4 Secretary’s Certificate. The Purchasers shall have received from the Company’s Secretary, a certificate
having attached thereto (i) the Company’s Amended and Restated Certificate of Incorporation as in effect at the time of the Additional Closing; (ii) the Company’s Bylaws as in effect at the time of the Additional Closing;
(iii) resolutions approved by the Board of Directors authorizing the transactions contemplated hereby; and (iv) and good standing certificates (including tax good standing) with respect to the Company from the applicable authority(ies) in
Delaware and any other jurisdiction in which the Company is qualified to do business, dated a recent date before the date of the Additional Closing. 
 8. CONDITIONS OF THE COMPANY’S OBLIGATIONS AT EACH CLOSING. 

The obligations of the Company to each Purchaser under this Agreement are subject to the fulfillment, on or before the applicable
Closing, of each of the following conditions, unless otherwise waived: 
 8.1 Representations and Warranties. The
representations and warranties of each Purchaser contained in Section 4 shall be true on and as of the applicable Closing with the same effect as though such representations and warranties had been made on and as of the applicable Closing.

 8.2 Qualifications. All authorizations, approvals or permits, if any, of any governmental authority or
regulatory body of the United States or of any state that are required in connection with the lawful issuance and sale of the Securities pursuant to this Agreement shall be obtained and effective as of the applicable Closing. 

9. COVENANTS OF THE COMPANY. 
 9.1 Indebtedness. For so long as any of the Notes remain outstanding, the Company will not, except with the approval of the Purchaser Majority, incur any obligation, liability, or
indebtedness (absolute, accrued, contingent or other), other than (i) trade payables incurred in the ordinary course of business consistent with past practice, and (ii) those related to or contemplated by the Loan Documents. 

9.2 Change of Control. The Company shall not undergo a Change of Control without the prior written consent of the Purchaser
Majority. 
 9.3 Rights Offering. To the extent permitted by applicable securities laws, the Company shall
provide each holder of preferred stock of the Company (other than those preferred stockholders that participated in the Initial Closing) with a written offer to participate in the financing contemplated by this Agreement in an aggregate amount equal
to fifteen million ($15,000,000) multiplied by the ratio of the total number of shares of preferred stock held by such stockholder immediately prior to the Initial Closing divided by the total number of shares of the Company’s preferred stock
outstanding immediately prior to the Initial Closing and close such investment within thirty (30) days of the Initial 

  
 12 

 
Closing; provided, however, that any amounts subscribed for by any such preferred stockholder shall be funded in two tranches (50% of the aggregate investment in the Subsequent
Initial Closing and 50% of the aggregate investment in the Second Closing). 
 10. “MARKET STAND OFF”
AGREEMENT. 
 Each Purchaser hereby agrees that such Purchaser shall not sell, transfer, make any short sale of, grant
any option for the purchase of, or enter into any hedging or similar transaction with the same economic effect as a sale, any Common Stock (or other securities) of the Company held by such Purchaser (other than those included in the registration)
the 180-day period following the effective date of the Initial Public Offering; provided, that, all officers and directors of the Company and holders of at least 1% of the Company’s voting securities are bound by and have entered into similar
agreements. The obligations described in this Section 10 shall not apply to a registration relating solely to employee benefit plans on Form S-1 or Form S-8 or similar forms that may be promulgated in the future, or a registration
relating solely to a transaction on Form S-4 or similar forms that may be promulgated in the future. 
 11.
MISCELLANEOUS. 
 11.1 Fees and Expenses. The Company shall pay all reasonable legal fees and
out-of-pocket expenses of a single legal counsel to the Purchasers, Gunderson Dettmer Stough Villeneuve Franklin & Hachigian, LLP, incurred in connection with the transactions contemplated in this Agreement; provided that such legal
fees and out-of-pocket expenses shall not exceed $15,000 in the aggregate. 
 11.2 Successors and Assigns. The
terms and conditions of this Agreement shall inure to the benefit of and be binding upon the respective successors and assigns 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 assigns any rights, remedies, obligations, or liabilities under or by reason of this Agreement, except as expressly provided in this Agreement. 

11.3 Governing Law; Venue. This Agreement and all acts and transactions pursuant hereto and the rights and obligations of
the parties hereto shall be governed, construed and interpreted in accordance with the laws of the State of California, without giving effect to principles of conflicts of law. Each party hereto consents to exclusive jurisdiction and venue in the
county of San Mateo, California, if in state court, and in the United States District Court for the Northern District of California, if in United States federal court, for any suit or proceeding relating to, arising out of or arising under this
Agreement; such courts shall have the sole and exclusive in personam, subject matter and other jurisdiction in connection with such suit or proceeding and venue shall be appropriate for all purposes in such courts. 

11.4 Counterparts. This Agreement may be executed in two or more counterparts, each of which shall be deemed an original
and all of which together shall constitute one instrument. 
 11.5 Titles and Subtitles. The titles and subtitles
used in this Agreement are used for convenience only and are not to be considered in construing or interpreting this Agreement. 

11.6 Notices. All notices and other communications required or permitted hereunder shall be in writing and shall be deemed
effectively given upon personal delivery, upon three (3) business days after deposit with the United States Post Office, by registered or certified mail, return receipt requested, postage prepaid, one (1) business day after deposit with a
nationally recognized air courier, upon receipt of confirmation with regard to delivery by facsimile or upon twelve (12) hours after delivery by electronic mail and addressed: (i) if to the Purchasers, at the Purchasers’ address,
facsimile number or 

  
 13 

 
electronic mail address as set forth on the signature page, or at such other address, facsimile number or electronic mail address as the Purchasers shall have furnished to the Company in writing,
or (ii) if to the Company, at its current address or at such other address, facsimile number or electronic mail address as the Company shall have furnished to the Purchasers in writing. 

11.7 Waivers and Amendments. 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 Purchaser Majority; provided, however, no consent of the Purchaser Majority shall be required to
add Purchasers participating in an Additional Closing (in accordance with the Company’s obligations pursuant to Section 9.3 hereof) and any New Purchasers participating in an Additional Closing as parties to this Agreement and to
Exhibit A hereof. Any amendment or waiver effected in accordance with this Section 11.7 shall be binding upon each Purchaser of Notes and the Company. 
 11.8 Severability. If one or more provisions of this Agreement are held to be unenforceable under applicable law, the parties agree to renegotiate such provision in good faith in order to
maintain the economic position enjoyed by each party as close as possible to that under the provision rendered unenforceable. In the event that the parties cannot reach a mutually agreeable and enforceable replacement for such provision, then
(i) such provision shall be excluded from this Agreement, (ii) the balance of the Agreement shall be interpreted as if such provision were so excluded and (iii) the balance of the Agreement shall be enforceable in accordance with its
terms. 
 11.9 Entire Agreement. This Agreement, and the documents referred to herein constitute the entire
agreement between the parties hereto pertaining to the subject matter hereof, and any and all other written or oral agreements existing between the parties hereto are expressly canceled. 

11.10 Corporate Securities Law. THE SALE OF THE SECURITIES WHICH ARE THE SUBJECT OF THIS AGREEMENT HAS NOT BEEN QUALIFIED
WITH THE COMMISSIONER OF CORPORATIONS OF THE STATE OF CALIFORNIA AND THE ISSUANCE OF THE SECURITIES OR THE PAYMENT OR RECEIPT OF ANY PART OF THE CONSIDERATION THEREFOR PRIOR TO THE QUALIFICATION IS UNLAWFUL, UNLESS THE SALE OF SECURITIES IS EXEMPT
FROM THE QUALIFICATION BY SECTION 25100, 25102 OR 25105 OF THE CALIFORNIA CORPORATIONS CODE. THE RIGHTS OF ALL PARTIES TO THIS AGREEMENT ARE EXPRESSLY CONDITIONED UPON THE QUALIFICATION BEING OBTAINED UNLESS THE SALE IS SO EXEMPT. 

11.11 Conflict with Terms of the Convertible Notes. In the event that any of the terms of this Agreement conflict with the
terms contained in the Notes and except for any conflict with Section 1.6 of this Agreement which shall govern and control, such terms contained in the Notes shall govern and control. 

11.12 Implied Covenant of Good Faith and Fair Dealing. This Agreement shall be (to the extent necessary to satisfy the
requirements of Section 22062(b)(3)(D) of the California Financial Code) subject to the implied covenant of good faith and fair dealing arising under Section 1655 of the California Civil Code. 

[Signature Pages Follow] 

  
 14 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT 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 Boulevard, Suite 200

		
		 	 South San Francisco, CA 94080

  

[SIGNATURE PAGE TO HYPERION THERAPEUTICS, INC.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT 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 Citron

				
		 		 	Name:	 	 Louis Citron

				
		 		 	Title:	 	 Chief Legal Officer

  

[SIGNATURE PAGE TO HYPERION THERAPEUTICS, INC.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT 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/ Bijan Salehizadeh

		 		 	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/ Bijan Salehizadeh

		 		 	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/ Bijan Salehizadeh

		 		 	Authorized Manager

  

[SIGNATURE PAGE TO HYPERION THERAPEUTICS, INC.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT 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/ Bijan Salehizadeh

		 		 	Authorized Manager

  

[SIGNATURE PAGE TO HYPERION THERAPEUTICS, INC.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT 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

  

[SIGNATURE PAGE TO HYPERION THERAPEUTICS, INC.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT 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.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT 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/ Carl Goldfischer

		 		 	Name:	 	Carl Goldfischer
		 		 	Title: Manager and Managing Director

  

[SIGNATURE PAGE TO HYPERION THERAPEUTICS, INC.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT 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/ Carl Goldfischer

		 		 	Name:	 	Carl Goldfischer
		 		 	Title: Manager and Managing Director

  

[SIGNATURE PAGE TO HYPERION THERAPEUTICS, INC.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT 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.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT as of the date set forth in the first paragraph hereof. 

 

			
	PURCHASER:	 	 /s/ Chris Rivera

		 	CHRIS RIVERA

  

[SIGNATURE PAGE TO HYPERION THERAPEUTICS, INC.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT as of the date set forth in the first paragraph hereof. 

 

			
	PURCHASER:	 	 /s/ Mark Blumling

		 	MARK BLUMLING

  

[SIGNATURE PAGE TO HYPERION THERAPEUTICS, INC.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT as of the date set forth in the first paragraph hereof. 

 

			
	PURCHASER:	 	 /s/ Michael Rivera

		 	MICHAEL RIVERA

  

[SIGNATURE PAGE TO HYPERION THERAPEUTICS, INC.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 IN WITNESS WHEREOF, the parties hereto
have executed the CONVERTIBLE NOTE AND WARRANT PURCHASE AGREEMENT as of the date set forth in the first paragraph hereof. 

 

			
	PURCHASER:	 	 /s/ George Jue

		 	GEORGE JUE

  

[SIGNATURE PAGE TO HYPERION THERAPEUTICS, INC.

 CONVERTIBLE NOTE AND WARRANT PURCHASE
AGREEMENT] 

 EXHIBIT A 

SCHEDULE OF PURCHASERS 
 INVESTORS IN INITIAL CLOSING AND SECOND CLOSING 

 

																													
	 INVESTOR
	 	INITIAL
CLOSING NOTE

AMOUNT	 	 	SECOND
CLOSING NOTE

AMOUNT	 	 	TOTAL NOTE
AMOUNTS	 	 	INITIAL
CLOSING

WARRANT
PURCHASE
PRICE	 	 	SECOND
CLOSING
WARRANT
PURCHASE
PRICE
	 	 	TOTAL
WARRANT
AMOUNTS	 	 	TOTAL
INVESTMENT
AMOUNT	 
	 New Enterprise Associates 12, Limited Partnership 

 
 1954 Greenspring Drive, Suite 600

Timonium, MD 21093

Attn: Pam Clark
	 	 $
	 1,725,188.12
	   
	 	 $
	 1,725,188.12
	   
	 	$	3,450,376.24	  	 	 $
	 172.52
	   
	 	$	172.52	  	 	$	345.04	  	 	$	3,450,721.28	  
	 Highland Capital Partners VII Limited Partnership

 
 One Broadway,

16th Floor
 Cambridge, MA 02142
 Attn: Patrick
Cammarata
	 	$	915,968.94	  	 	$	915,968.94	  	 	$	1,831,937.88	  	 	$	91.60	  	 	$	91.60	  	 	$	183.20	  	 	$	1,832,121.08	  
	 Highland Capital Partners VII-B Limited Partnership

 
 One Broadway,

16th Floor
 Cambridge, MA 02142
 Attn: Patrick
Cammarata
	 	$	221,957.12	  	 	$	221,957.12	  	 	$	443,914.24	  	 	$	22.20	  	 	$	22.20	  	 	$	44.40	  	 	$	443,958.64	  

																													
	 INVESTOR
	  	INITIAL
CLOSING
NOTE
AMOUNT	 	  	SECOND
CLOSING
NOTE
AMOUNT	 	  	TOTAL NOTE
AMOUNTS	 	  	INITIAL
CLOSING

WARRANT
PURCHASE
PRICE	 	  	SECOND
CLOSING
WARRANT
PURCHASE
PRICE
	 	  	TOTAL
WARRANT
AMOUNTS	 	  	TOTAL
INVESTMENT
AMOUNT	 
	 Highland Capital Partners VII-C Limited Partnership

 
 One Broadway,

16th Floor
 Cambridge, MA 02142
 Attn: Patrick
Cammarata
	  	$	323,239.63	  	  	$	323,239.63	  	  	$	646,479.26	  	  	$	32.32	  	  	$	32.32	  	  	$	64.64	  	  	$	646,543.90	  
	 Highland Entrepreneurs’ Fund VII Limited Partnership

 
 One Broadway,

16th Floor
 Cambridge, MA 02142
 Attn: Patrick
Cammarata
	  	$	28,701.40	  	  	$	28,701.40	  	  	$	57,402.80	  	  	$	2.87	  	  	$	2.87	  	  	$	5.74	  	  	$	57,408.54	  
	 Sofinnova Venture Partners VII, L.P.

 
 2800 Sand Hill Road, Suite 150

Menlo Park, CA 94025

Attn: Hooman Shahlavi
	  	$	1,725,188.12	  	  	$	1,725,188.12	  	  	$	3,450,376.24	  	  	$	172.52	  	  	$	172.52	  	  	$	345.04	  	  	$	3,450,721.28	  
	 WRF Capital (Washington Research Foundation)

 
 2815 Eastlake Avenue E., Suite
300
 Seattle, WA 98102

Attn: Thong Le
	  	$	185,013.32	  	  	$	185,013.32	  	  	$	370,026.64	  	  	$	18.50	  	  	$	18.50	  	  	$	37.00	  	  	$	370,063.64	  

																													
	 INVESTOR
	  	INITIAL
CLOSING
NOTE
AMOUNT	 	  	SECOND
CLOSING
NOTE
AMOUNT	 	  	TOTAL NOTE
AMOUNTS	 	  	INITIAL
CLOSING

WARRANT
PURCHASE
PRICE	 	  	SECOND
CLOSING
WARRANT
PURCHASE
PRICE
	 	  	TOTAL
WARRANT
AMOUNTS	 	  	TOTAL
INVESTMENT
AMOUNT	 
	 Bay City Capital Fund V, L.P.

 
 750 Battery Street

Suite 400
 San Francisco, CA 94111
	  	$	1,398,201.37	  	  	$	1,398,201.37	  	  	$	2,796,402.74	  	  	$	139.82	  	  	$	139.82	  	  	$	279.64	  	  	$	2,796,682.38	  
	 Bay City Capital Fund V Co-Investment Fund, L.P.

 
 750 Battery Street

Suite 400
 San Francisco, CA 94111
	  	$	26,644.56	  	  	$	26,644.56	  	  	$	53,289.12	  	  	$	2.66	  	  	$	2.66	  	  	$	5.32	  	  	$	53,294.44	  
	 Panorama Capital, L.P.

 
 2440 Sand Hill Road

Suite 302
 Menlo Park, CA 94025
	  	$	949,897.41	  	  	$	949,897.41	  	  	$	1,899,794.82	  	  	$	94.99	  	  	$	94.99	  	  	$	189.98	  	  	$	1,899,984.80	  
		  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 
								
	 TOTAL:
	  	$	7,499,999.99	  	  	$	7,499,999.99	  	  	$	14,999,999.98	  	  	$	750.00	  	  	$	750.00	  	  	$	1,500.00	  	  	$	15,001,499.98	  

 INVESTORS IN SUBSEQUENT
INITIAL CLOSING AND SECOND CLOSING 
  

																													
	 INVESTOR
	  	SUBSEQUENT
INITIAL
CLOSING 
NOTE
AMOUNT	 	  	SECOND
CLOSING 
NOTE
AMOUNT	 	  	TOTAL NOTE
AMOUNTS	 	  	SUBSEQUENT
INITIAL
CLOSING
WARRANT
PURCHASE
PRICE	 	  	SECOND
CLOSING
WARRANT
PURCHASE
PRICE
	 	  	TOTAL
WARRANT
AMOUNTS	 	  	TOTAL
INVESTMENT
AMOUNT	 
	 Chris Rivera

22016 238th Place SE
 Maple Valley, WA 98038
  
	  	$	2,396.70	  	  	$	2,396.70	  	  	$	4,793.40	  	  	$	0.24	  	  	$	0.24	  	  	$	0.48	  	  	$	4,793.88	  
	 Michael Rivera

2804 Clifftop Lane

Whistler, BC V0N 1B2

 
	  	$	345.73	  	  	$	345.73	  	  	$	691.46	  	  	$	0.03	  	  	$	0.03	  	  	$	0.06	  	  	$	691.52	  
	 George Jue

1709 Westbrook Avenue

Los Altos, CA 94024

 
	  	$	571.22	  	  	$	571.22	  	  	$	1,142.44	  	  	$	0.06	  	  	$	0.06	  	  	$	0.12	  	  	$	1,142.56	  
	 Mark Blumling

5463 Paradise Drive

Corte Madera, CA 94925

 
	  	$	237.10	  	  	$	237.10	  	  	$	474.20	  	  	$	0.02	  	  	$	0.02	  	  	$	0.04	  	  	$	474.24	  
		  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 
								
	 TOTAL:
	  	$	3,550.75	  	  	$	3,550.75	  	  	$	7,101.50	  	  	$	0.35	  	  	$	0.35	  	  	$	0.70	  	  	$	7,102.20	  

 TOTAL INVESTMENT OF ALL
INVESTORS 
  

																											
	 TOTAL NOTE

AMOUNT IN INITIAL

CLOSING
	 	  	TOTAL NOTE
AMOUNT
IN
SUBSEQUENT
CLOSING	 	  	TOTAL
NOTE
AMOUNT IN SECOND
CLOSING	 	  	TOTAL
WARRANT
AMOUNT IN INITIAL
CLOSING	 	  	TOTAL
WARRANT
AMOUNT IN
SUBSEQUENT
CLOSING	 	  	TOTAL
WARRANT
AMOUNT IN SECOND
CLOSING	 	  	TOTAL INVESTMENT
AMOUNT	 
							
	$	7,499,999.99	  	  	$	3,550.75	  	  	$	7,503,550.74	  	  	$	750.00	  	  	$	0.35	  	  	$	750.35	  	  	$	15,008,602.18	  

 EXHIBIT B 

FORM OF CONVERTIBLE UNSECURED PROMISSORY NOTE 

 EXHIBIT C 

FORM OF WARRANT TO PURCHASE STOCKForm of Indemnification Agreement

 Exhibit 10.5 
 FORM OF 
 INDEMNIFICATION AGREEMENT 

THIS AGREEMENT (the “Agreement”) is made and entered into as of ____, 2012 between Hyperion Therapeutics, Inc., a
Delaware corporation (the “Company”), and ____________ (“Indemnitee”). This Agreement will become effective only upon the effectiveness of the Company’s registration statement on Form S-1 in connection with the
Company’s initial public offering. This Agreement supersedes and replaces any and all previous agreements between the Company and Indemnitee covering the subject matter of this Agreement. 

WITNESSETH THAT: 
 WHEREAS, Indemnitee performs a valuable service for the Company; 
 WHEREAS, the
Board of Directors of the Company (the “Board”) has adopted Bylaws (the “Bylaws”) providing for the indemnification of the officers and directors of the Company to the maximum extent authorized by the Delaware
General Corporation Law; 
 WHEREAS, the Bylaws and Delaware General Corporation Law (the “DGCL”)
Section 145, as amended (“Law”), by their nonexclusive nature, permit contracts between the Company and the officers or directors of the Company with respect to indemnification of such officers or directors; 

WHEREAS, this Agreement is supplemental to and in furtherance of the Bylaws and any resolutions adopted pursuant thereto, and shall not
be deemed a substitute therefor, nor to diminish or abrogate any rights of Indemnitee thereunder; 
 WHEREAS, in accordance with
the authorization as provided by the Law, the Company may purchase and maintain a policy or policies of directors’ and officers’ liability insurance, covering certain liabilities which may be incurred by its officers or directors in the
performance of their obligations to the Company; [and] 
 WHEREAS, in order to induce Indemnitee to continue to serve as an
officer or director of the Company, the Company has determined and agreed to enter into this contract with Indemnitee[; and 

WHEREAS, Indemnitee is a representative of ________________ and has certain rights to indemnification and/or insurance provided by
________________ which Indemnitee and ________________ intend to be secondary to the primary obligation of the Company to indemnify Indemnitee as provided herein, with the Company’s acknowledgement and agreement to the foregoing being a
material condition to Indemnitee’s willingness to serve on the Board]. 
 NOW, THEREFORE, in consideration of
Indemnitee’s service as an officer or director after the date hereof, the parties hereto agree as follows: 
 1.
Indemnification of Indemnitee. The Company hereby agrees to hold harmless and indemnify Indemnitee to the full extent authorized or permitted by the provisions of the Law, as such may be amended from time to time, and the Bylaws, as such may
be amended. In furtherance of the foregoing indemnification, and without limiting the generality thereof: 
 (a) Proceedings
Other Than Proceedings by or in the Right of the Company. The Company shall indemnify Indemnitee in accordance with the provisions of this Section 1(a) if, by 

 reason of Indemnitee’s Corporate Status (as defined in Section 12 hereof), Indemnitee is,
or is threatened to be made, a party to or participant in any Proceeding (as defined in Section 12 hereof) other than a Proceeding by or in the right of the Company to procure a judgment in its favor. Pursuant to this
Section 1(a), Indemnitee shall be indemnified to the fullest extent permitted by applicable law against all Expenses (as defined in Section 12 hereof), judgments, fines and amounts paid in settlement (including all interest,
assessments and other charges paid or payable in connection with or in respect of such Expenses, judgments, fines and amounts paid in settlement) actually and reasonably incurred by Indemnitee, or on Indemnitee’s behalf, in connection with such
Proceeding or any claim, issue or matter therein, if Indemnitee acted in good faith and in a manner Indemnitee reasonably believed to be in or not opposed to the best interests of the Company, and with respect to any criminal Proceeding, had no
reasonable cause to believe Indemnitee’s conduct was unlawful. The parties hereto intend that this Agreement shall provide to the fullest extent permitted by law for indemnification in excess of that expressly permitted by statute, including,
without limitation, any indemnification provided by the Certificate of Incorporation of the Company (the “Certificate of Incorporation”), the Bylaws, vote of its stockholders or disinterested directors or applicable law. 

(b) Proceedings by or in the Right of the Company. The Company shall indemnify Indemnitee in accordance with the provisions of
this Section 1(b) if, by reason of Indemnitee’s Corporate Status, Indemnitee is, or is threatened to be made, a party to or participant in any Proceeding brought by or in the right of the Company to procure a judgment in its favor.
Pursuant to this Section 1(b), Indemnitee shall be indemnified to the fullest extent permitted by applicable law against all Expenses actually and reasonably incurred by Indemnitee, or on Indemnitee’s behalf, in connection with such
Proceeding or any claim, issue or matter therein, if Indemnitee acted in good faith and in a manner Indemnitee reasonably believed to be in or not opposed to the best interests of the Company. No indemnification for Expenses shall be made under
this Section 1(b) in respect of any claim, issue or matter as to which Indemnitee shall have been finally adjudged by a court to be liable to the Company, unless and only to the extent that the Court of Chancery of the State of Delaware
or any court in which the Proceeding was brought shall determine upon application that, despite the adjudication of liability but in view of all the circumstances of the case, Indemnitee is fairly and reasonably entitled to indemnification.

 (c) Indemnification for Expenses of a Party Who is Wholly or Partly Successful. Notwithstanding any other provision of
this Agreement, to the fullest extent permitted by applicable law and to the extent that Indemnitee is, by reason of Indemnitee’s Corporate Status, a party to (or a participant in) and is successful, on the merits or otherwise, in any
Proceeding or in defense of any claim, issue or matter therein, in whole or in part, the Company shall indemnify Indemnitee against all Expenses actually and reasonably incurred by Indemnitee or on Indemnitee’s behalf in connection therewith.
If Indemnitee is not wholly successful in such Proceeding but is successful, on the merits or otherwise, as to one or more but less than all claims, issues or matters in such Proceeding, the Company shall indemnify Indemnitee against all Expenses
actually and reasonably incurred by Indemnitee or on Indemnitee’s behalf in connection with each successfully resolved claim, issue or matter to the fullest extent permitted by law. For purposes of this Section 1(c) and without
limitation, the termination of any claim, issue or matter in such a Proceeding by dismissal, with or without prejudice, shall be deemed to be a successful result as to such claim, issue or matter. 

2. Additional Indemnity. 
 (a) In addition to, and without regard to any limitations on, the indemnification provided for in Section 1 hereof, the Company shall and hereby does indemnify and hold harmless Indemnitee, to
the fullest extent permitted by applicable law, against all Expenses, judgments, penalties, fines and amounts paid in settlement actually and reasonably incurred by Indemnitee or on 

  
 2 

 
Indemnitee’s behalf if, by reason of Indemnitee’s Corporate Status, Indemnitee is, or is threatened to be made, a party to or participant in any Proceeding (including a Proceeding by or
in the right of the Company), including, without limitation, all liability arising out of the negligence or active or passive wrongdoing of Indemnitee. The only limitation that shall exist upon the Company’s obligations pursuant to this
Agreement shall be that the Company shall not be obligated to make any payment to Indemnitee that is finally determined (under the procedures, and subject to the presumptions, set forth in Sections 6 and 7 hereof) to be unlawful under
Delaware law. 
 (b) For the purposes of Section 2(a), the meaning of the phrase “to the fullest extent
permitted by applicable law” shall include, but not be limited to: 
 (i) to the fullest extent permitted by the provision
of the DGCL that authorizes or contemplates additional indemnification by agreement, or the corresponding provision of any amendment to or replacement of the DGCL, and 
 (ii) to the fullest extent authorized or permitted by any amendments to or replacements of the DGCL adopted after the date of this Agreement that increase the extent to which a corporation may indemnify
its officers and directors. 
 3. Indemnification for Expenses of a Witness. Notwithstanding any other provision of this
Agreement, to the fullest extent permitted by applicable law and to the extent that Indemnitee is, by reason of Indemnitee’s Corporate Status, a witness or otherwise asked to participate in any Proceeding to which Indemnitee is not a party,
Indemnitee shall be indemnified against all Expenses actually and reasonably incurred by Indemnitee or on Indemnitee’s behalf in connection therewith. 
 4. Advancement of Expenses. Notwithstanding any provision of this Agreement to the contrary (other than Section 6), the Company shall advance, to the extent not prohibited by law, the
Expenses incurred by or on behalf of Indemnitee in connection with any Proceeding (or any part of any Proceeding) not initiated by Indemnitee. Such advancement shall be made within ten (10) days after the receipt by the Company of a statement
or statements from Indemnitee requesting such advance or advances from time to time, whether prior to or after final disposition of such Proceeding. Such statement or statements shall reasonably evidence the Expenses incurred by Indemnitee and shall
include or be preceded or accompanied by an undertaking by or on behalf of Indemnitee to repay any Expenses advanced if it shall ultimately be determined that Indemnitee is not entitled to be indemnified against such Expenses. Any advances and
undertakings to repay pursuant to this Section 4 shall be unsecured and interest free. Notwithstanding the foregoing, the obligation of the Company to advance Expenses pursuant to this Section 4 shall be subject to the
condition that, if, when and to the extent that the Company determines that Indemnitee would not be permitted to be indemnified under applicable law, the Company shall be entitled to be reimbursed, within thirty (30) days of such determination,
by Indemnitee (who hereby agrees to reimburse the Company) for all such amounts theretofore paid; provided, however, that if Indemnitee has commenced or thereafter commences legal proceedings in a court of competent jurisdiction to
secure a determination that Indemnitee should be indemnified under applicable law, any determination made by the Company that Indemnitee would not be permitted to be indemnified under applicable law shall not be binding and Indemnitee shall not be
required to reimburse the Company for any advance of Expenses until a final judicial determination is made with respect thereto (and as to which all rights of appeal therefrom have been exhausted or lapsed). No other form of undertaking shall be
required other than the execution of this Agreement. 
 5. Procedures and Presumptions for Determination of Entitlement to
Indemnification. It is the intent of this Agreement to secure for Indemnitee rights of indemnity that are as favorable as may be permitted under the Law and public policy of the State of Delaware. Accordingly,

  
 3 

 
the parties agree that the following procedures and presumptions shall apply in the event of any question as to whether Indemnitee is entitled to indemnification under this Agreement: 

(a) To obtain indemnification (including, but not limited to, the advancement of Expenses and contribution by the Company) under this
Agreement, Indemnitee shall submit to the Company a written request, including therein or therewith such documentation and information as is reasonably available to Indemnitee and is reasonably necessary to determine whether and to what extent
Indemnitee is entitled to indemnification. The Secretary of the Company shall, promptly upon receipt of such a request for indemnification, advise the Board in writing that Indemnitee has requested indemnification. 

(b) Upon written request by Indemnitee for indemnification pursuant to the first sentence of Section 5(a) hereof, a
determination, if required by applicable law, with respect to Indemnitee’s entitlement thereto shall be made in the specific case by one of the following three methods, which shall be at the election of Indemnitee: (i) by a majority vote
of the disinterested directors, even though less than a quorum, (ii) by Independent Counsel in a written opinion or (iii) by the stockholders. 
 (c) If the determination of entitlement to indemnification is to be made by Independent Counsel pursuant to Section 5(b) hereof, the Independent Counsel shall be selected as provided in this
Section 5(c). The Independent Counsel shall be selected by Indemnitee (unless Indemnitee requests that such selection be made by the Board). Indemnitee or the Company, as the case may be, may, within ten (10) days after such written
notice of selection shall have been given, deliver to the Company or to Indemnitee, as the case may be, a written objection to such selection; provided, however, that such objection may be asserted only on the ground that the
Independent Counsel so selected does not meet the requirements of “Independent Counsel” as defined in Section 12 hereof, and the objection shall set forth with particularity the factual basis of such assertion. Absent a
proper and timely objection, the person so selected shall act as Independent Counsel. If a written objection is made and substantiated, the Independent Counsel selected may not serve as Independent Counsel unless and until such objection is
withdrawn or a court has determined that such objection is without merit. If, within twenty (20) days after the later of submission by Indemnitee of a written request for indemnification pursuant to Section 5(a) hereof and the final
disposition of the Proceeding, no Independent Counsel shall have been selected and not objected to, either the Company or Indemnitee may petition the Court of Chancery of the State of Delaware or other court of competent jurisdiction for resolution
of any objection which shall have been made by the Company or Indemnitee to the other’s selection of Independent Counsel and/or for the appointment as Independent Counsel of a person selected by the court or by such other person as the court
shall designate, and the person with respect to whom all objections are so resolved or the person so appointed shall act as Independent Counsel under Section 5(b) hereof. The Company shall pay any and all reasonable fees and expenses of
Independent Counsel incurred by such Independent Counsel in connection with acting pursuant to Section 5(b) hereof, and the Company shall pay all reasonable fees and expenses incident to the procedures of this Section 5(c),
regardless of the manner in which such Independent Counsel was selected or appointed. 
 (d) In making a determination with
respect to entitlement to indemnification hereunder, the person, persons or entity making such determination shall, to the fullest extent not prohibited by law, presume that Indemnitee is entitled to indemnification under this Agreement. Anyone
seeking to overcome this presumption shall have the burden of proof and the burden of persuasion by clear and convincing evidence. 
 (e) For the purposes of any determination of good faith, Indemnitee shall be deemed to have acted in good faith if Indemnitee’s action is based on the records or books of account of the Enterprise,
including financial statements, or on information supplied to Indemnitee by the directors 

  
 4 

 
or officers of the Enterprise (as hereinafter defined) in the course of their duties, or on the advice of legal counsel for the Enterprise or on information or records given or reports made to
the Enterprise by an independent certified public accountant or by an appraiser or other expert selected with reasonable care by the Enterprise. In addition, the knowledge and/or actions, or failure to act, of any director, officer, agent or
employee of the Enterprise shall not be imputed to Indemnitee for purposes of determining the right to indemnification under this Agreement. Whether or not the foregoing provisions of this Section 5(e) are satisfied, it shall in any
event be presumed that Indemnitee has at all times acted in good faith and in a manner Indemnitee reasonably believed to be in or not opposed to the best interests of the Company. Anyone seeking to overcome this presumption shall have the burden of
proof and the burden of persuasion by clear and convincing evidence. 
 (f) If the person, persons or entity empowered or
selected under this Section 5 to determine whether Indemnitee is entitled to indemnification shall not have made a determination within thirty (30) days after receipt by the Company of the request therefor, the requisite
determination of entitlement to indemnification shall, to the fullest extent not prohibited by law, be deemed to have been made and Indemnitee shall be entitled to such indemnification absent (i) a misstatement by Indemnitee of a material fact,
or an omission of a material fact necessary to make Indemnitee’s statement not materially misleading, in connection with the request for indemnification, or (ii) a prohibition of such indemnification under applicable law; provided,
however, that such thirty (30)-day period may be extended for a reasonable time, not to exceed an additional fifteen (15) days, if the person, persons or entity making such determination with respect to entitlement to indemnification in
good faith requires such additional time to obtain or evaluate documentation and/or information relating thereto; and provided, further, that the foregoing provisions of this Section 5(f) shall not apply if the
determination of entitlement to indemnification is to be made by the stockholders pursuant to Section 5(b) hereof and if (x) within fifteen (15) days after receipt by the Company of the request for such determination, the Board
or the Disinterested Directors, if appropriate, resolve to submit such determination to the stockholders for their consideration at an annual meeting thereof to be held within seventy-five (75) days after such receipt and such determination is
made thereat, or (y) a special meeting of stockholders is called within fifteen (15) days after such receipt for the purpose of making such determination, such meeting is held for such purpose within sixty (60) days after having been
so called and such determination is made thereat. 
 (g) Indemnitee shall cooperate with the person, persons or entity making
such determination with respect to Indemnitee’s entitlement to indemnification, including providing to such person, persons or entity upon reasonable advance request any documentation or information which is not privileged or otherwise
protected from disclosure and which is reasonably available to Indemnitee and reasonably necessary to such determination. Any Independent Counsel, member of the Board or stockholder of the Company shall act reasonably and in good faith in making a
determination regarding the Indemnitee’s entitlement to indemnification under this Agreement. Any costs or expenses (including attorneys’ fees and disbursements) incurred by Indemnitee in so cooperating with the person, persons or entity
making such determination shall be borne by the Company (irrespective of the determination as to Indemnitee’s entitlement to indemnification) and the Company hereby indemnifies and agrees to hold Indemnitee harmless therefrom. 

(h) The Company acknowledges that a settlement or other disposition short of final judgment may be successful if it permits a party to
avoid expense, delay, distraction, disruption and uncertainty. In the event that any action, claim or proceeding to which Indemnitee is a party is resolved in any manner other than by adverse judgment against Indemnitee (including, without
limitation, settlement of such action, claim or proceeding with or without payment of money or other consideration) it shall be presumed that Indemnitee has been successful on the merits or otherwise in such action, suit or

  
 5 

 
proceeding. Anyone seeking to overcome this presumption shall have the burden of proof and the burden of persuasion by clear and convincing evidence. 

6. Remedies of Indemnitee. 
 (a) In the event that (i) a determination is made pursuant to Section 5 hereof that Indemnitee is not entitled to indemnification under this Agreement, (ii) advancement of Expenses
is not timely made pursuant to Section 4 hereof, (iii) no determination of entitlement to indemnification is made pursuant to Section 5(b) hereof within ninety (90) days after receipt by the Company of the request
for indemnification, (iv) payment of indemnification is not made pursuant to this Agreement within ten (10) days after receipt by the Company of a written request therefor, (v) payment of indemnification is not made within ten
(10) days after a determination has been made that Indemnitee is entitled to indemnification or such determination is deemed to have been made pursuant to Section 5 hereof, or (vi) in the event that the Company or any other
person takes or threatens to take any action to declare this Agreement void or unenforceable, or institutes any litigation or other action or Proceeding designed to deny, or to recover from the Indemnitee the benefits provided or intended to be
provided to the Indemnitee hereunder, Indemnitee shall be entitled to an adjudication in an appropriate court of the State of Delaware, or in any other court of competent jurisdiction, of Indemnitee’s entitlement to such indemnification or
advancement of Expenses. Alternatively, Indemnitee, at Indemnitee’s option, may seek an award in arbitration to be conducted by a single arbitrator pursuant to the Commercial Arbitration Rules of the American Arbitration Association. Indemnitee
shall commence such proceeding seeking an adjudication or an award in arbitration within one hundred eighty (180) days following the date on which Indemnitee first has the right to commence such proceeding pursuant to this
Section 6(a). The Company shall not oppose Indemnitee’s right to seek any such adjudication or award in arbitration. 
 (b) In the event that a determination shall have been made pursuant to Section 5(b) hereof that Indemnitee is not entitled to indemnification, any judicial proceeding commenced pursuant to
this Section 6 shall be conducted in all respects as a de novo trial, or arbitration, on the merits, and Indemnitee shall not be prejudiced by reason of the adverse determination under Section 5(b) hereof. In any
judicial proceeding or arbitration commenced pursuant to this Section 6 the Company shall have the burden of proving Indemnitee is not entitled to indemnification or advancement of Expenses, as the case may be. 

(c) If a determination shall have been made pursuant to Section 5(b) hereof that Indemnitee is entitled to indemnification,
the Company shall be bound by such determination in any judicial proceeding or arbitration commenced pursuant to this Section 6, absent (i) a misstatement by Indemnitee of a material fact, or an omission of a material fact necessary
to make Indemnitee’s statement not materially misleading, in connection with the request for indemnification, or (ii) a prohibition of such indemnification under applicable law. 

(d) In the event that Indemnitee, pursuant to this Section 6, seeks a judicial adjudication of Indemnitee’s rights
under, or to recover damages for breach of, this Agreement, or to recover under any directors’ and officers’ liability insurance policies maintained by the Company, the Company shall pay on Indemnitee’s behalf, in advance, any and all
expenses (of the types described in the definition of Expenses in Section 12 hereof) actually and reasonably incurred by Indemnitee in such judicial adjudication, regardless of whether Indemnitee ultimately is determined to be entitled
to such indemnification, advancement of expenses or insurance recovery. 
 (e) The Company shall, to the fullest extent not
prohibited by law, be precluded from asserting in any judicial proceeding or arbitration commenced pursuant to this Section 6 that the procedures and presumptions of this Agreement are not valid, binding and enforceable and shall

  
 6 

 
stipulate in any such court or before any such arbitrator that the Company is bound by all the provisions of this Agreement. 

(f) Notwithstanding anything in this Agreement to the contrary, no determination as to entitlement of Indemnitee to indemnification under
this Agreement shall be required to be made prior to the final disposition of the Proceeding. 
 7. Non-Exclusivity; Survival
of Rights; Insurance; Primacy of Indemnification; Subrogation. 
 (a) The rights of indemnification and to receive
advancement of Expenses as provided by this Agreement shall not be deemed exclusive of any other rights to which Indemnitee may at any time be entitled under applicable law, the Certificate of Incorporation, the Bylaws, any agreement, a vote of
stockholders, a resolution of directors or otherwise. No amendment, alteration or repeal of this Agreement or of any provision hereof shall limit or restrict any right of Indemnitee under this Agreement in respect of any action taken or omitted by
such Indemnitee in Indemnitee’s Corporate Status prior to such amendment, alteration or repeal. To the extent that a change in the Law, whether by statute or judicial decision, permits greater indemnification or advancement of Expenses than
would be afforded currently under the Bylaws and this Agreement, it is the intent of the parties hereto that Indemnitee shall enjoy by this Agreement the greater benefits so afforded by such change. No right or remedy herein conferred is intended to
be exclusive of any other right or remedy, and every other right and remedy shall be cumulative and in addition to every other right and remedy given hereunder or now or hereafter existing at law or in equity or otherwise. The assertion or
employment of any right or remedy hereunder, or otherwise, shall not prevent the concurrent assertion or employment of any other right or remedy. 
 (b) To the extent that the Company maintains an insurance policy or policies providing liability insurance for directors, officers, employees, or agents or fiduciaries of the Company or of any other
corporation, partnership, joint venture, trust, employee benefit plan or other enterprise that such person serves at the request of the Company, Indemnitee shall be covered by such policy or policies in accordance with its or their terms to the
maximum extent of the coverage available for any director, officer, employee, agent or fiduciary under such policy or policies. 

(c) [Except as provided in subparagraph (e) below,] in the event of any payment under this Agreement, the Company shall be
subrogated to the extent of such payment to all of the rights of recovery of Indemnitee (other than against the Fund Indemnitors), who shall execute all papers required and take all action necessary to secure such rights, including execution of such
documents as are necessary to enable the Company to bring suit to enforce such rights. 
 (d) [Except as provided in
subparagraph (e) below,] the Company shall not be liable under this Agreement to make any payment of amounts otherwise indemnifiable hereunder (or for which advancement is provided hereunder) if and to the extent that Indemnitee has
otherwise actually received such payment under any insurance policy, contract, agreement or otherwise. 
 (e) [The Company
hereby acknowledges that Indemnitee has certain rights to indemnification, advancement of expenses and/or insurance provided by                     
and/or certain of its affiliates (collectively, the “Fund Indemnitors”). The Company hereby agrees (i) that it is the indemnitor of first resort (i.e., its obligations to Indemnitee are primary and any obligation of the Fund
Indemnitors to advance expenses or to provide indemnification for the same expenses or liabilities incurred by Indemnitee are secondary), (ii) that it shall be required to advance the full amount of expenses incurred by Indemnitee and shall be
liable for the full amount of all Expenses, judgments, penalties, fines and amounts paid in settlement to the extent legally permitted and as required by the Certificate of 

  
 7 

 
Incorporation or Bylaws of the Company (or any agreement between the Company and Indemnitee), without regard to any rights Indemnitee may have against the Fund Indemnitors, and, (iii) that
it irrevocably waives, relinquishes and releases the Fund Indemnitors from any and all claims against the Fund Indemnitors for contribution, subrogation or any other recovery of any kind in respect thereof. The Company further agrees that no
advancement or payment by the Fund Indemnitors on behalf of Indemnitee with respect to any claim for which Indemnitee has sought indemnification from the Company shall affect the foregoing and the Fund Indemnitors shall have a right of contribution
and/or be subrogated to the extent of such advancement or payment to all of the rights of recovery of Indemnitee against the Company. The Company and Indemnitee agree that the Fund Indemnitors are express third party beneficiaries of the terms
hereof.] 
 8. Liability Insurance. The Company shall maintain liability insurance applicable to directors, officers,
employees, or agents, and Indemnitee shall be covered by such policies in such a manner as to provide such Indemnitee the same rights and benefits as are provided to the most favorably insured of the Company’s directors. The Company shall
notify Indemnitee of any change, lapse or cancellation of such coverage. 
 9. Exception to Right of Indemnification.
Notwithstanding any other provision of this Agreement, Indemnitee shall not be entitled to indemnification under this Agreement with respect to any Proceeding brought by Indemnitee, or any claim therein, unless (a) the bringing of such
Proceeding or making of such claim shall have been approved by the Board or (b) such Proceeding is being brought by Indemnitee to assert, interpret or enforce Indemnitee’s rights under this Agreement. 

10. Duration of Agreement. All agreements and obligations of the Company contained herein shall continue during the period
Indemnitee is an officer or director of the Company (or is or was serving at the request of the Company as a director, officer, employee or agent of another corporation, partnership, joint venture, trust or other enterprise) and shall continue
thereafter so long as Indemnitee shall be subject to any Proceeding (or any proceeding commenced under Section 6 hereof) by reason of Indemnitee’s Corporate Status, whether or not Indemnitee is acting or serving in any such capacity
at the time any liability or expense is incurred for which indemnification can be provided under this Agreement. This Agreement shall be binding upon and inure to the benefit of and be enforceable by the parties hereto and their respective
successors (including any direct or indirect successor by purchase, merger, consolidation or otherwise to all or substantially all of the business or assets of the Company), assigns, spouses, heirs, executors and personal and legal representatives.

 11. Security. To the extent requested by Indemnitee and approved by the Board, the Company may at any time and from
time to time provide security to Indemnitee for the Company’s obligations hereunder through an irrevocable bank line of credit, funded trust or other collateral. Any such security, once provided to Indemnitee, may not be revoked or released
without the prior written consent of the Indemnitee. 
 12. Definitions. For purposes of this Agreement: 

(a) “Corporate Status” means the status of a person who is or was a director (including, without limitation, serving as
a member of any committee or subcommittee of the Board), officer, employee, agent or fiduciary of the Company (or any subsidiary of the Company) or of any other corporation, limited liability company, partnership, joint venture, trust, employee
benefit plan or other enterprise that such person is or was serving at the express written request of the Company. 
 (b)
“Disinterested Director” means a director of the Company who is not and was not a party to the Proceeding in respect of which indemnification is sought by Indemnitee. 

  
 8 

 (c) “Enterprise” means the Company and any other corporation, partnership,
joint venture, trust, employee benefit plan or other enterprise that Indemnitee is or was serving at the express written request of the Company as a director, officer, employee, agent or fiduciary. 

(d) “Expenses” means all reasonable attorneys’ fees, retainers, court costs, transcript costs, fees of experts,
witness fees, travel expenses, duplicating costs, printing and binding costs, telephone charges, postage, delivery service fees , any federal, state, local or foreign taxes imposed on Indemnitee as a result of the actual or deemed receipt of any
payments under this Agreement, ERISA excise taxes and penalties, and all other disbursements or expenses of the types customarily incurred in connection with prosecuting, defending, preparing to prosecute or defend, investigating, participating, or
being or preparing to be a witness in a Proceeding. Expenses also shall include (i) Expenses incurred in connection with any appeal resulting from any Proceeding, including without limitation the premium, security for, and other costs relating
to any cost bond, supersedeas bond, or other appeal bond or its equivalent, and (ii) for purposes of Section 6(d) only, Expenses incurred by Indemnitee in connection with the interpretation, enforcement or defense of
Indemnitee’s rights under this Agreement, by litigation or otherwise. The parties agree that for the purposes of any advancement of Expenses for which Indemnitee has made written demand to the Company in accordance with this Agreement, all
Expenses included in such demand that are certified by affidavit of Indemnitee’s counsel as being reasonable shall be presumed conclusively to be reasonable. Expenses, however, shall not include amounts paid in settlement by Indemnitee or the
amount of judgments or fines against Indemnitee. 
 (e) “Independent Counsel” means a law firm, or a member of
a law firm, that is experienced in matters of corporation law and neither presently is, nor in the past five (5) years has been, retained to represent: (i) the Company or Indemnitee in any matter material to either such party (other than
with respect to matters concerning Indemnitee under this Agreement, or of other indemnitees under similar indemnification agreements), or (ii) any other party to the Proceeding giving rise to a claim for indemnification hereunder.
Notwithstanding the foregoing, the term “Independent Counsel” shall not include any person who, under the applicable standards of professional conduct then prevailing, would have a conflict of interest in representing either the Company or
Indemnitee in an action to determine Indemnitee’s rights under this Agreement. The Company agrees to pay the reasonable fees and expenses of the Independent Counsel referred to above and to fully indemnify such counsel against any and all
Expenses, claims, liabilities and damages arising out of or relating to this Agreement or its engagement pursuant hereto. 
 (f)
“Proceeding” means any threatened, pending or completed action, suit, claim, counterclaim, cross claim, arbitration, mediation, alternate dispute resolution mechanism, investigation, inquiry, administrative hearing or any other
actual, threatened or completed proceeding, whether brought by or in the right of the Company or otherwise and whether of a civil, criminal, administrative, legislative or investigative (formal or informal) nature, including any appeal therefrom, in
which Indemnitee was, is or will be involved as a party, potential party, non-party witness or otherwise by reason of the fact that Indemnitee is or was an officer or director of the Company, by reason of any action taken by Indemnitee or of any
inaction on Indemnitee’s part while acting as an officer or director of the Company, or by reason of the fact that Indemnitee is or was serving at the request of the Company as a director, officer, employee, agent or fiduciary of another
corporation, partnership, joint venture, trust or other Enterprise or of any action (or failure to act) on Indemnitee’s part while acting pursuant to Indemnitee’s Corporate Status; in each case whether or not Indemnitee is acting or
serving in such capacity at the time any liability or Expense is incurred for which indemnification, reimbursement, or advancement of Expenses can be provided under this Agreement; including one pending on or before the date of this Agreement, but
excluding one initiated by an Indemnitee pursuant to Section 6 hereof to enforce Indemnitee’s rights under this Agreement. If the Indemnitee believes in good faith that a given

  
 9 

 
situation may lead to or culminate in the institution of a Proceeding, this shall be considered a Proceeding under this paragraph. 

13. Severability. If any provision or provisions of this Agreement shall be held by a court of competent jurisdiction to be
invalid, void, illegal or otherwise unenforceable for any reason whatsoever: (a) the validity, legality and enforceability of the remaining provisions of this Agreement (including, without limitation, each portion of any section of this
Agreement containing any such provision held to be invalid, illegal or unenforceable that is not itself invalid, illegal or unenforceable) shall not in any way be affected or impaired thereby and shall remain enforceable to the fullest extent
permitted by law; and (b) to the fullest extent possible, the provisions of this Agreement (including, without limitation, each portion of any section of this Agreement containing any such provision held to be invalid, illegal or unenforceable
that is not itself invalid, illegal or unenforceable) shall be construed so as to give effect to the intent manifested thereby. Without limiting the generality of the foregoing, this Agreement is intended to confer upon Indemnitee indemnification
rights to the fullest extent permitted by applicable laws. In the event any provision hereof conflicts with any applicable law, such provision shall be deemed modified, consistent with the aforementioned intent, to the extent necessary to resolve
such conflict. 
 14. Enforcement. 
 (a) The Company expressly confirms and agrees that it has entered into this Agreement and assumed the obligations imposed on it hereby in order to induce Indemnitee to serve as a director or officer of
the Company, and the Company acknowledges that Indemnitee is relying upon this Agreement in serving as a director or officer of the Company. 
 (b) This Agreement constitutes the entire agreement between the parties hereto with respect to the subject matter hereof and supersedes all prior agreements and understandings, oral, written and implied,
between the parties hereto with respect to the subject matter hereof; provided, however, that this Agreement is a supplement to and in furtherance of the Certificate of Incorporation, the Bylaws and applicable law, and shall not be
deemed a substitute therefor, nor to diminish or abrogate any rights of Indemnitee thereunder. 
 15. Modification and
Waiver. No supplement, modification, termination or amendment of this Agreement shall be binding unless executed in writing by both of the parties hereto. No waiver of any of the provisions of this Agreement shall be deemed or shall constitute a
waiver of any other provisions hereof (whether or not similar) nor shall such waiver constitute a continuing waiver. 
 16.
Notice By Indemnitee. Indemnitee agrees promptly to notify the Company in writing upon being served with or otherwise receiving any summons, citation, subpoena, complaint, indictment, information or other document relating to any Proceeding
or matter which may be subject to indemnification covered hereunder. The failure to so notify the Company shall not relieve the Company of any obligation which it may have to Indemnitee under this Agreement or otherwise unless and only to the extent
that such failure or delay materially prejudices the Company. 
 17. Notices. All notices, requests, demands and other
communications hereunder shall be in writing and shall be deemed to have been duly given if (i) delivered by hand and receipted for by the party to whom said notice or other communication shall have been directed, or (ii) mailed by
certified or registered mail with postage prepaid, on the third business day after the date on which it is so mailed: 

  
 10 

 (a) If to Indemnitee, to the address set forth below Indemnitee signature hereto.

 (b) If to the Company, to: 
       601 Gateway Blvd, Suite 200 

      South San Francisco, CA 94080 
       Attention: Chief Executive Officer 
 or to such other address
as may have been furnished to Indemnitee by the Company or to the Company by Indemnitee, as the case may be. 
 18.
Contribution. To the fullest extent permissible under applicable law, if the indemnification provided for in this Agreement is unavailable to Indemnitee for any reason whatsoever, the Company, in lieu of indemnifying Indemnitee, shall
contribute to the amount incurred by Indemnitee, whether for judgments, fines, penalties, excise taxes, amounts paid or to be paid in settlement and/or for Expenses, in connection with any claim relating to an indemnifiable event under this
Agreement, in such proportion as is deemed fair and reasonable in light of all of the circumstances of such Proceeding in order to reflect (i) the relative benefits received by the Company and Indemnitee as a result of the event(s) and/or
transaction(s) giving cause to such Proceeding; and/or (ii) the relative fault of the Company (and its directors, officers, employees and agents) and Indemnitee in connection with such event(s) and/or transaction(s). 

19. Identical Counterparts. This Agreement may be executed in one or more counterparts, each of which shall for all purposes be
deemed to be an original but all of which together shall constitute one and the same Agreement. Only one such counterpart signed by the party against whom enforceability is sought needs to be produced to evidence the existence of this Agreement.

 20. Headings. The headings of the paragraphs of this Agreement are inserted for convenience only and shall not be
deemed to constitute part of this Agreement or to affect the construction thereof. 
 21. Governing Law. The parties
agree that this Agreement shall be governed by, and construed and enforced in accordance with, the laws of the State of Delaware without application of the conflict of laws principles thereof. 

22. Gender. Use of the masculine pronoun shall be deemed to include usage of the feminine pronoun where appropriate. 

  
 11 

 IN WITNESS WHEREOF, the parties hereto have executed this Agreement on and as of the day and
year first above written. 
  

							
		 		 	HYPERION THERAPEUTICS, INC.
				
		 		 	By:	 	 
				
		 		 	Name:	 	 
				
		 		 	Title:	 	 
			
		 		 	INDEMNITEE
			
		 		 	[type Indemnitee name]
			
		 		 	 
			
		 	Address:	 	 
			
		 		 	 
			
		 		 	 

  
 SIGNATURE
PAGE TO INDEMNIFICATION AGREEMENT

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