Document:

2006 Stock Option and Grant Plan

 Exhibit 10.1 

AEGERION PHARMACEUTICALS, INC. 

2006 STOCK OPTION AND GRANT PLAN 
  

	SECTION 1.	GENERAL PURPOSE OF THE PLAN; DEFINITIONS 

The name of the plan is the Aegerion Pharmaceuticals, Inc. 2006 Stock Option and Grant Plan (the “Plan”). The purpose of the
Plan is to encourage and enable the officers, employees, directors and other key persons (including consultants and prospective employees) of Aegerion Pharmaceuticals, Inc. a Delaware corporation (including any successor entity, the
“Company”), and its Subsidiaries upon whose judgment, initiative and efforts the Company largely depends for the successful conduct of its business to acquire a proprietary interest in the Company. It is anticipated that providing such
persons with a direct stake in the Company’s welfare will assure a closer identification of their interests with those of the Company, thereby stimulating their efforts on the Company’s behalf and strengthening their desire to remain with
the Company. 
 The following terms shall be defined as set forth below: 

“Affiliate” of any Person means a Person that directly or indirectly, through one or more intermediaries, controls, is
controlled by or is under common control with the first mentioned Person. A Person shall be deemed to control another Person if such first Person possesses directly or indirectly the power to direct, or cause the direction of, the management and
policies of the second Person, whether through the ownership of voting securities, by contract or otherwise. 

“Award” or “Awards,” except where referring to a particular category of grant under the Plan, shall
include Incentive Stock Options, Non-Qualified Stock Options, Restricted Stock Awards, Unrestricted Stock Awards or any combination of the foregoing. 

“Bankruptcy” shall mean (i) the filing of a voluntary petition under any bankruptcy or insolvency law, or a
petition for the appointment of a receiver or the making of an assignment for the benefit of creditors, with respect to the Holder, or (ii) the Holder being subjected involuntarily to such a petition or assignment or to an attachment or other
legal or equitable interest with respect to the Holder’s assets, which involuntary petition or assignment or attachment is not discharged within sixty (60) days after its date, and (iii) the Holder being subject to a transfer of its
Issued Shares by operation of law (including by divorce, even if not insolvent), except by reason of death. 

“Board” means the Board of Directors of the Company. 

“Code” means the Internal Revenue Code of 1986, as amended, and any successor Code, and related rules, regulations and
interpretations. 

 AEGERION 2006 STOCK OPTION
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 “Committee” means the Committee of the Board referred to in
Section 2. 
 “Effective Date” means the date on which the Plan is approved by stockholders as set forth
at the end of this Plan. 
 “Exchange Act” means the Securities Exchange Act of 1934, as amended, and the rules
and regulations thereunder. 
 “Fair Market Value” of the Stock on any given date means the fair market value
of the Stock determined in good faith by the Committee. 
 “Holder” means, with respect to an Award or any
Issued Shares, the Person holding such Award or Issued Shares, including the initial recipient of the Award or any Permitted Transferee. 

“Incentive Stock Option” means any Stock Option designated and qualified as an “incentive stock option” as
defined in Section 422 of the Code. 
 “Issued Shares” means, collectively, all outstanding Shares issued
pursuant to Restricted Stock Awards, all outstanding Shares issued pursuant to Unrestricted Stock Awards, and all Option Shares. 

“Non-Qualified Stock Option” means any Stock Option that is not an Incentive Stock Option. 

“Option Shares” means outstanding shares of Stock that were issued to a Holder upon the exercise of a Stock Option.

 “Permitted Transferees” shall mean any of the following to whom a Holder may transfer Issued Shares
hereunder (as set forth in Section 9(a)(ii)(A)): the Holder’s spouse, children (natural or adopted), stepchildren, grandchildren or a trust for their sole benefit of which the Holder is the settlor; provided, however, that any such
trust does not require or permit distribution of any Issued Shares during the term of this Agreement unless subject to its terms. Upon the death of the Holder, the term Permitted Transferees shall also include such deceased Holder’s estate,
executions, administrations, personal representations, heirs, legatees and distributees, as the case may be. 

“Person” shall mean any individual, corporation, partnership (limited or general), limited liability company, limited
liability partnership, association, trust, joint venture, unincorporated organization or any similar entity. 

“Option” or “Stock Option” means any option to purchase shares of Stock granted pursuant to
Section 6. 
 “Repurchase Event” means the termination of the Award recipient’s employment or service
relationship with the Company and its Subsidiaries for Cause (as defined in the Award agreement). 
  

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 “Restricted Stock Award” means Awards granted pursuant to
Section 7 and “Restricted Stock” means Shares granted pursuant to such Awards. 
 “Sale
Event” means the consummation of (i) the dissolution or liquidation of the Company, (ii) the sale of all or substantially all of the assets of the Company and its Subsidiaries on a consolidated basis to an unrelated person or
entity, (iii) a merger, reorganization or consolidation in which the outstanding shares of Stock are converted into or exchanged for securities of the successor entity and the holders of the Company’s outstanding voting power immediately
prior to such transaction do not own at least a majority of the outstanding voting power of the successor entity immediately upon completion of such transaction (taking into account only ownership interests resulting from pre-transaction interests
in the Company), (iv) the sale, in a single transaction or series of related transactions, of all or a majority of the Stock of the Company to an unrelated person or entity, or (v) any other transaction in which the holders of the
Company’s outstanding voting power immediately prior to such transaction do not own at least a majority of the outstanding voting power of the Company or a successor entity immediately upon completion of the transaction (taking into account
only ownership interests resulting from pre-transaction interests in the Company). 
 “Securities Act” means
the Securities Act of 1933, as amended, and the rules and regulations thereunder. 
 “Shares” means shares of
Stock. 
 “Stock” means the Common Stock, par value $.001 per share, of the Company, subject to adjustments
pursuant to Section 3. 
 “Subsidiary” means any corporation or other entity (other than the Company) in
which the Company has a controlling interest, either directly or indirectly. 
 “Unrestricted Stock Award”
means any Award granted pursuant to Section 8 and “Unrestricted Stock” means Shares granted pursuant to such Awards. 
  

	SECTION 2.	ADMINISTRATION OF PLAN; COMMITTEE AUTHORITY TO SELECT GRANTEES AND DETERMINE AWARDS 

(a) Administration of Plan. The Plan shall be administered by the Board, or at the discretion of the Board, by a committee of the
Board, comprised of not less than two Directors. All references herein to the Committee shall be deemed to refer to the group then responsible for administration of the Plan at the relevant time (i.e., either the Board of Directors or a
committee or committees of the Board, as applicable). 
 (b) Powers of Committee. The Committee shall have the power and
authority to grant Awards consistent with the terms of the Plan, including the power and authority: 
 (i) to
select the Persons to whom Awards may from time to time be granted; 
  

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 (ii) to determine the time or times of grant, and the extent, if any, of
Incentive Stock Options, Non-Qualified Stock Options, Restricted Stock Awards, Unrestricted Stock Awards or any combination of the foregoing, granted to any one or more grantees; 

(iii) to determine the number of shares of Stock to be covered by any Award; 

(iv) to determine and modify from time to time the terms and conditions, including restrictions, not inconsistent with the
terms of the Plan, of any Award, which terms and conditions may differ among individual Awards and grantees, and to approve the form of written instruments evidencing the Awards; 

(v) to accelerate at any time the exercisability or vesting of all or any portion of any Award; 

(vi) to impose any limitations on Awards granted under the Plan, including limitations on transfers, repurchase provisions
and the like and to exercise repurchase rights or obligations; 
 (vii) subject to any restrictions applicable to
Incentive Stock Options, to extend at any time the period in which Stock Options may be exercised; and 
 (viii)
at any time to adopt, alter and repeal such rules, guidelines and practices for administration of the Plan and for its own acts and proceedings as it shall deem advisable; to interpret the terms and provisions of the Plan and any Award (including
related written instruments); to make all determinations it deems advisable for the administration of the Plan; to decide all disputes arising in connection with the Plan; and to otherwise supervise the administration of the Plan. 

All decisions and interpretations of the Committee shall be binding on all persons, including the Company and Plan grantees. 

(c) Indemnification. Neither the Board nor the Committee, nor any member of either or any delegatee thereof, shall be liable for
any act, omission, interpretation, construction or determination made in good faith in connection with the Plan, and the members of the Board and the Committee (and any delegatee thereof) shall be entitled in all cases to indemnification and
reimbursement by the Company in respect of any claim, loss, damage or expense (including reasonable attorneys’ fees) arising or resulting therefrom to the fullest extent permitted by law and/or under any directors’ and officers’
liability insurance coverage which may be in effect from time to time. 
  

	SECTION 3.	STOCK ISSUABLE UNDER THE PLAN; CHANGES IN STOCK; SUBSTITUTION 

(a) Stock Issuable. The maximum number of Shares reserved and available for issuance under the Plan shall be 1,555,060 Shares,
subject to adjustment as provided in Section 3(b). For purposes of this limitation, the Shares underlying any Awards which are forfeited, canceled, reacquired by the Company, satisfied without the issuance of Stock or otherwise terminated
(other than by exercise) shall be added back to the Shares available for 
  

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issuance under the Plan. Subject to such overall limitation, Shares may be issued up to such maximum number pursuant to any type or types of Award. The Shares available for issuance under the
Plan may be authorized but unissued Shares or Shares reacquired by the Company and held in its treasury. 
 (b) Changes in
Stock. Subject to Section 4, if, as a result of any reorganization, recapitalization, reclassification, stock dividend, stock split, reverse stock split or other similar change in the Company’s capital stock, the outstanding shares of
Stock are increased or decreased or are exchanged for a different number or kind of shares or other securities of the Company, or additional shares or new or different shares or other securities of the Company or other non-cash assets are
distributed with respect to such shares of Stock or other securities, or, if, as a result of any merger, consolidation or sale of all or substantially all of the assets of the Company, the outstanding shares of Stock are converted into or exchanged
for a different number or kind of securities of the Company or any successor entity (or a parent or subsidiary thereof), the Committee shall make an appropriate or proportionate adjustment in (i) the maximum number of shares reserved for
issuance under the Plan, (ii) the number and kind of shares or other securities subject to any then outstanding Awards under the Plan, (iii) the repurchase price per share subject to each outstanding Award, if any, and (iv) the
exercise price and/or exchange price for each share subject to any then outstanding Stock Options under the Plan, without changing the aggregate exercise price (i.e., the exercise price multiplied by the number of Stock Options) as to which such
Stock Options remain exercisable. The adjustment by the Committee shall be final, binding and conclusive. No fractional shares of Stock shall be issued under the Plan resulting from any such adjustment, but the Committee in its discretion may make a
cash payment in lieu of fractional shares. 
 The Committee may also adjust the number of shares subject to outstanding Awards
and the exercise price and the terms of outstanding Awards to take into consideration material changes in accounting practices or principles, extraordinary dividends, acquisitions or dispositions of stock or property or any other event if it is
determined by the Committee that such adjustment is appropriate to avoid distortion in the operation of the Plan, provided that no such adjustment shall be made in the case of an Incentive Stock Option, without the consent of the grantee, if it
would constitute a modification, extension or renewal of the Option within the meaning of Section 424(h) of the Code. 

(c) Substitute Awards. The Committee may grant Awards under the Plan in substitution for stock and stock based awards held by
employees, directors or other key persons of another corporation in connection with a merger or consolidation of the employing corporation with the Company or a Subsidiary or the acquisition by the Company or a Subsidiary of property or stock of the
employing corporation. The Committee may direct that the substitute awards be granted on such terms and conditions as the Committee considers appropriate in the circumstances. Any substitute Awards granted under the Plan shall not count against the
share limitation set forth in Section 3(a). 
  

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	SECTION 4.	TREATMENT UPON SALE EVENT OR OTHER EXTRAORDINARY TRANSACTION 

(a) Options. 

(i) In the case of and subject to the consummation of a Sale Event, the Plan and all Options issued hereunder shall
terminate upon the effective time of any such Sale Event unless provision is made in connection with the Sale Event in the sole discretion of the parties thereto for the assumption or continuation by the successor entity of Options theretofore
granted, or the substitution of such Options with new Options of the successor entity or parent thereof, with appropriate adjustment as to the number and kind of shares and, if appropriate, the per share exercise prices, as such parties shall agree.

 (ii) In the event of the termination of the Plan and all Options issued hereunder, each Holder of Options
shall be permitted, within a specified period of time prior to the consummation of the Sale Event as determined by the Committee, to exercise all such Options which are then exercisable or will become exercisable as of the effective time of the Sale
Event; provided, however, that the exercise of Options not exercisable prior to the Sale Event shall be subject to the consummation of the Sale Event. 

(iii) Notwithstanding anything to the contrary in Section 4(a)(i), in the event of a Sale Event pursuant to which
holders of the Stock of the Company will receive upon consummation thereof a cash payment for each share surrendered in the Sale Event, the Company shall have the right, but not the obligation, to make or provide for a cash payment to the grantees
holding vested Options in exchange for the cancellation thereof, in an amount equal to the difference between (A) the value as determined by the Committee of the consideration payable per share of Stock pursuant to the Sale Event (the
“Sale Price”) times the number of shares of Stock subject to outstanding vested Options (to the extent then exercisable at prices not in excess of the Sale Price) and (B) the aggregate exercise price of all such outstanding vested
Options. 
 (b) Option Shares and Restricted Stock Awards. Unless otherwise provided in an Award agreement, in the case
of and subject to the consummation of a Sale Event, Option Shares and shares of Restricted Stock shall be subject to the repurchase right set forth in Section 9(c)(i) and 9(c)(ii), respectively. 

(c) Unrestricted Stock Awards. Unless otherwise provided in an Award agreement, any shares of Unrestricted Stock shall be treated
in a Sale Event the same as all other Shares then outstanding. 
  

	SECTION 5.	ELIGIBILITY 

 Grantees
under the Plan will be such full or part-time officers and other employees, directors and key persons (including consultants and prospective employees) of the Company and its Subsidiaries as are selected from time to time by the Committee in its
sole discretion. 
  

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	SECTION 6.	STOCK OPTIONS 

 (a)
Nature of Stock Options. A Stock Option is an Award entitling the recipient to acquire, at such exercise price as determined by the Committee, shares of Stock subject to such restrictions and conditions as the Committee may determine at the
time of grant. Conditions may be based on continuing employment (or other service relationship) and/or achievement of pre-established performance goals and objectives. The grant of a Stock Option is contingent on the grantee executing the Stock
Option agreement. The terms and conditions of each such agreement shall be determined by the Committee, and such terms and conditions may differ among individual Awards and grantees. 

Stock Options granted under the Plan may be either Incentive Stock Options or Non-Qualified Stock Options. Incentive Stock Options may be
granted only to employees of the Company or any Subsidiary that is a “subsidiary corporation” within the meaning of Section 424(f) of the Code. To the extent that any Option does not qualify as an Incentive Stock Option, it shall be
deemed a Non-Qualified Stock Option. 
 No Incentive Stock Option shall be granted under the Plan after the date which is
ten (10) years from the date the Plan is approved by Board of Directors. 
 (b) Grants of Stock Options. The
Committee in its discretion may grant Stock Options to eligible directors, officers, employees and key persons of the Company or any Subsidiary. Stock Options granted under the Plan shall be subject to the following terms and conditions and shall
contain such additional terms and conditions, not inconsistent with the terms of the Plan, as the Committee shall deem desirable. If the Committee so determines, Stock Options may be granted in lieu of cash compensation at the optionee’s
election, subject to such terms and conditions as the Committee may establish. 
 (i) Exercise Price. The
exercise price per share for the Stock covered by a Stock Option granted under the Plan shall be determined by the Committee at the time of grant but shall not be less than one hundred percent (100%) of the Fair Market Value on the date of
grant. If an employee owns or is deemed to own (by reason of the attribution rules of Section 424(d) of the Code) more than ten percent (10%) of the combined voting power of all classes of stock of the Company or any parent or subsidiary
corporation and an Incentive Stock Option is granted to such employee, the option price of an Incentive Stock Option shall be not less than one hundred ten percent (110%) of the Fair Market Value on the grant date. 

(ii) Option Term. The term of each Stock Option shall be fixed by the Committee, but no Stock Option shall be
exercisable more than ten (10) years after the date the Stock Option is granted. If an employee owns or is deemed to own (by reason of the attribution rules of Section 424(d) of the Code) more than ten percent (10%) of the combined
voting power of all classes of stock of the Company or any parent or subsidiary corporation and an Incentive Stock Option is granted to such employee, the term of such Stock Option shall be no more than five (5) years from the date of grant.

 (iii) Exercisability; Rights of a Stockholder. Stock Options shall become exercisable at such time or
times, whether or not in installments, as shall be determined by the 
  

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Committee at or after the grant date. The Committee may at any time accelerate the exercisability of all or any portion of any Stock Option. An optionee shall have the rights of a stockholder
only as to shares acquired upon the exercise of a Stock Option and not as to unexercised Stock Options. An optionee shall not be deemed to have acquired any such shares unless and until a Stock Option shall have been exercised pursuant to the terms
hereof, the Company shall have issued and delivered the shares to the optionee, and the optionee’s name shall have been entered on the books of the Company as a stockholder. 

(iv) Method of Exercise. Stock Options may be exercised in whole or in part, by giving written notice of exercise
to the Company, specifying the number of shares to be purchased. Payment of the purchase price may be made by one or more of the following methods or as otherwise provided by the Committee: 

(A) In cash, by certified or bank check or other instrument acceptable to the Committee in U.S. funds payable to the order
of the Company in an amount equal to the purchase price of such Option Shares; 
 (B) By the optionee delivering
to the Company a promissory note if the Board has expressly authorized the loan of funds to the optionee for the purpose of enabling or assisting the optionee to effect the exercise of his or her Stock Option; provided that at least so much of the
exercise price as represents the par value of the Stock shall be paid other than with a promissory note if otherwise required by state law; or 

(C) If permitted by the Committee, through the delivery (or attestation to the ownership) of shares of Stock that have
been beneficially owned by the optionee for at least six (6) months and are not then subject to restrictions under any Company plan. Such surrendered shares shall be valued at Fair Market Value on the exercise date. 

Payment instruments will be received subject to collection. No certificates for shares of Stock so purchased will be issued to optionee
until the Company has completed all steps required by law to be taken in connection with the issuance and sale of the shares, including (i) receipt of a representation from the optionee at the time of exercise of the Option that the optionee is
purchasing the shares for the optionee’s own account and not with a view to any sale or distribution thereof, (ii) the legending of any certificate representing the shares to evidence the foregoing representations and restrictions, and
(iii) obtaining from optionee payment or provision for all withholding taxes due as a result of the exercise of the Option. The delivery of certificates representing the shares of Stock to be purchased pursuant to the exercise of a Stock Option
will be contingent upon receipt from the optionee (or a purchaser acting in his stead in accordance with the provisions of the Stock Option) by the Company of the full purchase price for such shares and the fulfillment of any other requirements
contained in the Option Award agreement or applicable provisions of laws. In the event an optionee chooses to pay the purchase price by previously-owned shares of Stock through the attestation method, the number of shares of Stock transferred to the
optionee upon the exercise of the Stock Option shall be net of the number of shares attested to. 
  

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 (c) Annual Limit on Incentive Stock Options. To the extent required for
“incentive stock option” treatment under Section 422 of the Code, the aggregate Fair Market Value (determined as of the time of grant) of the shares of Stock with respect to which Incentive Stock Options granted under this Plan and
any other plan of the Company or its parent and subsidiary corporations become exercisable for the first time by an optionee during any calendar year shall not exceed $100,000. To the extent that any Stock Option exceeds this limit, it shall
constitute a Non-Qualified Stock Option. 
  

	SECTION 7.	RESTRICTED STOCK AWARDS 

(a) Nature of Restricted Stock Awards. A Restricted Stock Award is an Award pursuant to which the Company may, in its sole
discretion, grant or sell, at such purchase price as determined by the Committee, in its sole discretion, shares of Stock subject to such restrictions and conditions as the Committee may determine at the time of grant, which purchase price shall be
payable in cash or other form of consideration acceptable to the Committee. Conditions may be based on continuing employment (or other service relationship) and/or achievement of pre-established performance goals and objectives. The grant of a
Restricted Stock Award is contingent on the grantee executing the Restricted Stock Award agreement. The terms and conditions of each such agreement shall be determined by the Committee, and such terms and conditions may differ among individual
Awards and grantees. 
 (b) Rights as a Stockholder. Upon execution of a written instrument setting forth the Restricted
Stock Award and payment of any applicable purchase price, a grantee shall have the rights of a stockholder with respect to the voting of the Restricted Stock, subject to such conditions contained in the written instrument evidencing the Restricted
Stock Award. 
 (c) Vesting of Restricted Stock. The Committee at the time of grant shall specify the date or dates
and/or the attainment of pre-established performance goals, objectives and other conditions on which Restricted Stock shall become vested, subject to such further rights of the Company or its assigns as may be specified in the instrument evidencing
the Restricted Stock Award. 
 (d) Record Owner; Dividends. The Holder of Restricted Stock shall be considered the record
owners of and shall be entitled to vote the Shares of Restricted Stock if and to the extent such Shares are entitled to voting rights. The Holder shall be entitled to receive all dividends and any other distributions declared on the Shares;
provided, however, that the Company is under no duty to declare any such dividends or to make any such distribution. The Restricted Stock Award agreement may require or permit the immediate payment, waiver, deferral or investment of dividends
paid on the Restricted Stock. 
  

	SECTION 8.	UNRESTRICTED STOCK AWARDS 

(a) Grant or Sale of Unrestricted Stock. The Committee may, in its sole discretion, grant (or sell at par value or such higher
purchase price determined by the Committee) an Unrestricted Stock Award to any grantee, pursuant to which such grantee may receive shares of Stock free of any vesting restrictions under the Plan. Unrestricted Stock Awards may be granted

  

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or sold as described in the preceding sentence in respect of past services or other valid consideration, or in lieu of any cash compensation due to such individual. 

(b) Elections to Receive Unrestricted Stock In Lieu of Compensation. Upon the request of a grantee and with the consent of the
Committee, each such grantee may, pursuant to an advance written election delivered to the Company no later than the date specified by the Committee, receive a portion of the cash compensation otherwise due to such grantee in the form of shares of
Unrestricted Stock either currently or on a deferred basis. 
  

	SECTION 9.	TRANSFER RESTRICTIONS; COMPANY RIGHT OF FIRST REFUSAL; COMPANY REPURCHASE RIGHTS 

(a) Restrictions on Transfer. 

(i) Options. No Stock Option shall be transferable by the optionee otherwise than by will or by the laws of descent
and distribution and all Stock Options shall be exercisable, during the optionee’s lifetime, only by the optionee, or by the optionee’s legal representative or guardian in the event of the optionee’s incapacity. The Optionee may elect
to designate a beneficiary by providing written notice of the name of such beneficiary to the Company, and may revoke or change such designation at any time by filing written notice of revocation or change with the Company, and any such beneficiary
may exercise the Optionee’s Stock Option in the event of the Optionee’s death to the extent provided herein. If the Optionee does not designate a beneficiary, or if the designated beneficiary predeceases the Optionee, the legal
representative of the Optionee may exercise this Stock Option in the event of the Optionee’s death to the extent provided herein. Notwithstanding the foregoing, the Committee, in its sole discretion, may provide in the Award agreement regarding
a given Option that the optionee may transfer, without consideration for the transfer, his or her Non-Qualified Stock Options to members of his or her immediate family, to trusts for the benefit of such family members, or to partnerships in which
such family members are the only partners, provided that the transferee agrees in writing with the Company to be bound by all of the terms and conditions of this Plan and the applicable Option. 

(ii) Issued Shares. No Issued Shares shall be sold, assigned, transferred, pledged, hypothecated, given away or in
any other manner disposed of or encumbered, whether voluntarily or by operation of law, unless (i) such transfer is in compliance with the terms of the applicable Award, all applicable securities laws (including the Securities Act), and with
the terms and conditions of this Section 9, (ii) such transfer does not cause the Company to become subject to the reporting requirements of the Exchange Act, and (iii) the transferee consents in writing to be bound by the provisions
of the Plan, including this Section 9. In connection with any proposed transfer, the Committee may require the transferor to provide at the transferor’s own expense an opinion of counsel to the transferor, satisfactory to the Committee,
that such transfer is in compliance with all foreign, federal and state securities laws (including the Securities Act). Any attempted disposition of Issued Shares not in accordance with the terms and conditions of this Section 9 shall be null
and void, and the Company shall not reflect on its records any change in record ownership of any Issued Shares as a result of any such disposition, shall otherwise refuse to recognize any such disposition and shall not in any way give effect to any
such disposition of Issued Shares. Subject to the foregoing general provisions, and unless 
  

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otherwise provided in the agreement with respect to a particular Award, Issued Shares may be transferred pursuant to the following specific terms and conditions (provided that with respect to any
transfer of Restricted Stock, all vesting and forfeiture provisions shall continue to apply only with respect to the original recipient): 

(A) Transfers to Permitted Transferees. The Holder may sell, assign, transfer or give away any or all of the Issued
Shares to Permitted Transferees; provided, however, that following such sale, assignment, or other transfer, such Issued Shares shall continue to be subject to the terms of this Plan (including this Section 9) and such Permitted
Transferee(s) shall, as a condition to any such transfer, deliver a written acknowledgment to that effect to the Company. 

(B) Transfers Upon Death. Upon the death of the Holder, any Issued Shares then held by the Holder at the time of
such death and any Issued Shares acquired thereafter by the Holder’s legal representative shall be subject to the provisions of this Plan, and the Holder’s estate, executors, administrators, personal representatives, heirs, legatees and
distributees shall be obligated to convey such Issued Shares to the Company or its assigns under the terms contemplated hereby. 

(b) Right of First Refusal. In the event that a Holder desires at any time to sell or otherwise transfer all
or any part of such Holder’s Issued Shares, the Holder first shall give written notice to the Company of the Holder’s intention to make such transfer. Such notice shall state the number of Issued Shares which the Holder proposes to sell
(the “Offered Shares”), the price and the terms at which the proposed sale is to be made and the name and address of the proposed transferee. At any time within forty-five (45) days after the receipt of such notice by the Company, the
Company or its assigns may elect to purchase all or any portion of the Offered Shares at the price and on the terms offered by the proposed transferee and specified in the notice. The Company or its assigns shall exercise this right by mailing or
delivering written notice to the Holder within the foregoing 45-day period (the “Company Exercise Notice”). If the Company or its assigns elect to exercise its purchase rights under this Section 9(b), the closing for such purchase
shall, in any event, take place on or prior to the thirtieth
(30th) day following the date of the Company Exercise
Notice. In the event that the Company or its assigns do not elect to exercise such purchase right, or in the event that the Company or its assigns do not pay the full purchase price within such 30-day period, the Holder may, within ninety (90)
days thereafter, sell the Offered Shares to the proposed transferee and at the same price and on the same terms as specified in the Holder’s notice. Any Shares purchased by such proposed transferee shall no longer be subject to the terms of the
Plan, subject to the provisions of Section 13(c) hereof. Any Shares not sold to the proposed transferee shall remain subject to the Plan. 

(c) Company’s Right of Repurchase. 

(i) Right of Repurchase for Option Shares. The Company or its assigns shall have the right and option upon a
Repurchase Event to repurchase from a Holder of Option Shares some or all (as determined by the Company) of the Option Shares held or subsequently acquired upon exercise of a Stock Option by such Holder at the price per share specified below. Such
repurchase right may be exercised by the Company within the later of (A) six (6) months following the date of such Repurchase Event or (B) seven (7) months after the acquisition of

  

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such Option Shares upon exercise of a Stock Option (the “Option Shares Repurchase Period”). The “Option Shares Repurchase Price” shall be equal to the Fair Market Value of the
Option Shares, determined as of the date the Committee elects to exercise its repurchase rights in connection with a Repurchase Event. 

(ii) Right of Repurchase With Respect to Restricted Stock and Unrestricted Stock. Unless otherwise set forth in the
agreement entered into by the recipient and the Company in connection with a Restricted Stock Award or Unrestricted Stock Award, the Company or its assigns shall have the right and option upon a Repurchase Event to repurchase from a Holder of Issued
Shares received pursuant to a Restricted Stock Award or Unrestricted Stock Award some or all (as determined by the Company) of such Issued Shares at the price per share specified below. Such repurchase right may be exercised by the Company within
six (6) months following the date of such Repurchase Event (the “Non-Option Shares Repurchase Period”). The “Non-Option Shares Repurchase Price” shall be (i) in the case of Issued Shares which are vested as of the date
of the Repurchase Event, the Fair Market Value of such Issued Shares as of the date the Committee elects to exercise its repurchase rights in connection with a Repurchase Event and (ii) in the case of Issued Shares which have not vested as of
the date of the Repurchase Event, subject to adjustment as provided in Section 3(b), the original per share purchase price paid by the recipient. 

(iii) Procedure. Any repurchase right of the Company shall be exercised by the Company or its assigns by giving the
Holder written notice on or before the last day of the Option Shares Repurchase Period or Non-Option Shares Repurchase Period, as applicable, of its intention to exercise such repurchase right. Upon such notification, the Holder shall promptly
surrender to the Company, free and clear of any liens or encumbrances, any certificates representing the Shares being purchased, together with a duly executed stock power for the transfer of such Shares to the Company or the Company’s assignee
or assignees. Upon the Company’s or its assignee’s receipt of the certificates from the Holder, the Company or its assignee or assignees shall deliver to him, her or them a check for the Option Shares Repurchase Price or the Non-Option
Shares Repurchase Price, as applicable; provided, however, that the Company may pay the Option Shares Repurchase Price or Non-Option Shares Repurchase Price, as applicable, by offsetting and canceling any indebtedness then owed by the Holder
to the Company. 
 (d) Escrow Arrangement. 

(i) Escrow. In order to carry out the provisions of Sections 9(b) and 9(c), of this Agreement more
effectively, the Company shall hold any Issued Shares in escrow together with separate stock powers executed by the Holder in blank for transfer, and any Permitted Transferee shall, as an additional condition to any transfer of Issued Shares,
execute a like stock power as to such Issued Shares. The Company shall not dispose of the Issued Shares except as otherwise provided in this Agreement. In the event of any repurchase by the Company (or any of its assigns), the Company is hereby
authorized by the Holder and any Permitted Transferee, as the Holder’s and each such Permitted Transferee’s attorney-in-fact, to date and complete the stock powers necessary for the transfer of the Issued Shares being purchased and to
transfer such Issued Shares in accordance with the terms hereof. At such time as any Issued Shares are no longer subject to the Company’s repurchase, first refusal and drag along rights, the Company

  

 12 

 AEGERION 2006 STOCK OPTION
AND GRANT PLAN 
  

 
shall, at the written request of the Holder, deliver to the Holder (or the relevant Permitted Transferee) a certificate representing such Issued Shares with the balance of the Issued Shares to be
held in escrow pursuant to this Section 9(d). 
 (ii) Remedy. Without limitation of any other
provision of this Agreement or other rights, in the event that a Holder, any Permitted Transferees or any other Person is required to sell a Holder’s Issued Shares pursuant to the provisions of Sections 9(b) or 9(c) hereof and in the
further event that he or she refuses or for any reason fails to deliver to the Company or its designated purchaser of such Issued Shares the certificate or certificates evidencing such Issued Shares together with a related stock power, the Company
or such designated purchaser may deposit the applicable purchase price for such Issued Shares with a bank designated by the Company, or with the Company’s independent public accounting firm, as agent or trustee, or in escrow, for such Holder,
any Permitted Transferees or other Person, to be held by such bank or accounting firm for the benefit of and for delivery to him, her, them or it, and/or, in its discretion, pay such purchase price by offsetting any indebtedness then owed by such
Holder as provided above. Upon any such deposit and/or offset by the Company or its designated purchaser of such amount and upon notice to the Person who was required to sell the Issued Shares to be sold pursuant to the provisions of
Sections 9(b) or 9(c), such Issued Shares shall at such time be deemed to have been sold, assigned, transferred and conveyed to such purchaser, such Holder shall have no further rights thereto (other than the right to withdraw the payment
thereof held in escrow, if applicable), and the Company shall record such transfer in its stock transfer book or in any appropriate manner. 

(e) Lockup Provision. A Holder agrees, if requested by the Company and any underwriter engaged by the Company, not to sell or
otherwise transfer or dispose of any Issued Shares (including pursuant to Rule 144 under the Securities Act) held by him or her for such period following the effective date of any registration statement of the Company filed under the Securities Act
as the Company or such underwriter shall specify reasonably and in good faith, not to exceed one hundred eighty (180) days in the case of the Company’s initial public offering (or, if required by such underwriter, such longer period of
time as is necessary to enable such underwriter to issue a research report or make a public appearance that relates to an earnings release or announcement by the Company within sixteen (16) days prior to or after the date that is one hundred
eighty (180) days after the effective date of the registration statement relating to such offering, but in any event not to exceed two hundred ten (210) days following the effective date of the registration statement relating to such
offering) or ninety (90) days in the case of any other public offering. 
 (f) Adjustments for Changes in Capital
Structure. If, as a result of any reorganization, recapitalization, reclassification, stock dividend, stock split, reverse stock split or other similar change in the Common Stock, the outstanding shares of Common Stock are increased or decreased
or are exchanged for a different number or kind of shares of the Company’s stock, the restrictions contained in this Section 9 shall apply with equal force to additional and/or substitute securities, if any, received by Holder in exchange
for, or by virtue of his or her ownership of, Issued Shares. 
 (g) Termination. The terms and provisions of
Sections 9(b) and 9(c) shall terminate upon the closing of the Company’s initial public offering or upon consummation of any Sale 

 

 13 

 AEGERION 2006 STOCK OPTION
AND GRANT PLAN 
  

 
Event, in either case as a result of which shares of the Company (or a successor entity) of the same class as the Issued Shares are registered under Section 12 of the Exchange Act and
publicly traded on NASDAQ/NMS or any national security exchange. 
  

	SECTION 10.	TAX WITHHOLDING 

 (a)
Payment by Grantee. Each grantee shall, no later than the date as of which the value of an Award or of any Stock or other amounts received thereunder first becomes includable in the gross income of the grantee for Federal income tax purposes,
pay to the Company, or make arrangements satisfactory to the Committee regarding payment of, any Federal, state, or local taxes of any kind required by law to be withheld with respect to such income. The Company and its Subsidiaries shall, to the
extent permitted by law, have the right to deduct any such taxes from any payment of any kind otherwise due to the grantee. The Company’s obligation to deliver stock certificates to any grantee is subject to and conditioned on any such tax
obligations being satisfied by the grantee. 
 (b) Payment in Stock. Subject to approval by the Committee, a grantee may
elect to have the minimum required tax withholding obligation satisfied, in whole or in part, by (i) authorizing the Company to withhold from shares of Stock to be issued pursuant to any Award a number of shares with an aggregate Fair Market
Value (as of the date the withholding is effected) that would satisfy the withholding amount due, or (ii) transferring to the Company shares of Stock owned by the grantee with an aggregate Fair Market Value (as of the date the withholding is
effected) that would satisfy the minimum withholding amount due. 
  

	SECTION 11.	AMENDMENTS AND TERMINATION 

The Board may, at any time, amend or discontinue the Plan and the Committee may, at any time, amend or cancel any outstanding Award (or
provide substitute Awards at the same or a reduced exercise or purchase price or with no exercise or purchase price) in a manner not inconsistent with the terms of the Plan, provided that such price, if any, must satisfy the requirements which would
apply to the substitute or amended Award if it were then initially granted under this Plan for the purpose of satisfying changes in law or for any other lawful purpose, but no such action shall adversely affect rights under any outstanding Award
without the holder’s consent. In addition, to the extent determined by the Committee to be required by the Code to ensure that Incentive Stock Options granted under the Plan are qualified under Section 422 of the Code, Plan amendments
shall be subject to approval by the Company’s stockholders who are entitled to vote at a meeting of stockholders. Nothing in this Section 11 shall limit the Committee’s authority to take any action permitted pursuant to
Section 3(c). 
  

	SECTION 12.	STATUS OF PLAN 

 With
respect to the portion of any Award that has not been exercised and any payments in cash, Stock or other consideration not received by a grantee, a grantee shall have no rights greater than those of a general creditor of the Company unless the
Committee shall otherwise expressly determine in connection with any Award or Awards. In its sole discretion, the Committee may authorize the creation of trusts or other arrangements to meet the Company’s

  

 14 

 AEGERION 2006 STOCK OPTION
AND GRANT PLAN 
  

 
obligations to deliver Stock or make payments with respect to Awards hereunder, provided that the existence of such trusts or other arrangements is consistent with the foregoing sentence.

  

	SECTION 13.	GENERAL PROVISIONS 

 (a)
No Distribution; Compliance with Legal Requirements. The Committee may require each person acquiring Stock pursuant to an Award to represent to and agree with the Company in writing that such person is acquiring the shares without a view to
distribution thereof. No shares of Stock shall be issued pursuant to an Award until all applicable securities law and other legal requirements have been satisfied. The Committee may require the placing of restrictive legends on certificates for
Stock and Awards as it deems appropriate. 
 (b) Delivery of Stock Certificates. Stock certificates to grantees under
this Plan shall be deemed delivered for all purposes when the Company or a stock transfer agent of the Company shall have mailed such certificates in the United States mail, addressed to the grantee, at the grantee’s last known address on file
with the Company. 
 (c) Stockholders’ Agreement. At any time that a Holder, as a result of an issuance of an Award
hereunder or as a result of a transfer of Issued Shares pursuant to Section 9 hereof, would own one percent (1%) or more of the Common Stock then outstanding (calculated on an as-converted basis, and assuming the exercise of all rights,
options and warrants and conversion of all convertible securities), as a condition to such issuance or transfer, such Holder shall execute an instrument of adherence to that certain Stockholders’ Agreement by and among the Company and the
Stockholders party thereto dated as of December 15, 2005 and as amended, restated or replaced from time to time (the “Stockholders’ Agreement”), such Holder to become bound by the provisions of the Stockholders’ Agreement as
a “Principal Stockholder”. Notwithstanding anything in this Plan to the contrary, concerning the Company’s right of first refusal as to the Holder’s Issued Shares pursuant to Section 9 hereof, any right of first refusal
continues in the Stockholders’ Agreement, to the extent applicable, to take precedent and govern the transactions contemplated by Section 9 hereof. A copy of the Stockholders’ Agreement may be obtained by any Holder at no cost by
written request to the Company. 
 (d) Other Compensation Arrangements; No Employment Rights. Nothing contained in this
Plan shall prevent the Board from adopting other or additional compensation arrangements, including trusts, and such arrangements may be either generally applicable or applicable only in specific cases. The adoption of this Plan and the grant of
Awards do not confer upon any employee any right to continued employment with the Company or any Subsidiary. 
 (e) Loans to
Award Recipients. The Company shall have the authority to make loans to recipients of Awards hereunder (including to facilitate the purchase of shares) and shall further have the authority to issue shares for promissory notes hereunder.

 (f) Designation of Beneficiary. Each grantee to whom an Award has been made under the Plan may designate a beneficiary
or beneficiaries to exercise any Award or receive any payment under any Award payable on or after the grantee’s death. Any such designation shall be on a form provided for that purpose by the Committee and shall not be effective until received
by 
  

 15 

 AEGERION 2006 STOCK OPTION
AND GRANT PLAN 
  

 
the Committee. If no beneficiary has been designated by a deceased grantee, or if the designated beneficiaries have predeceased the grantee, the beneficiary shall be the grantee’s estate.

 (g) Headings and Certain Defined Terms. Headings and captions are for convenience only and are not be used in the
interpretation of this Plan. The words “include”, “includes” and “including” will be deemed to be followed by the phrase “without limitation”. The words “herein”, “hereof” and
“hereunder”, and words of similar import, will be construed to refer to this Plan in its entirety and not to any particular provision hereof. All references herein to Sections, unless otherwise specifically provided, will be construed to
refer to Sections this Plan. 
 (h) Legend. Any certificate(s) representing the Issued Shares shall carry substantially
the following legend: 
 The transferability of this certificate and the shares of stock represented hereby are subject to the
restrictions, terms and conditions (including repurchase and restrictions against transfers) contained in the Aegerion Pharmaceuticals, Inc. 2006 Stock Option and Grant Plan and any agreement entered into thereunder by and between the company and
the holder of this certificate (a copy of which is available at the offices of the company for examination). 
  

	SECTION 14.	EFFECTIVE DATE OF PLAN 

This Plan shall become effective upon approval by the stockholders in accordance with applicable law. Subject to such approval by the
stockholders and to the requirement that no Stock may be issued hereunder prior to such approval, Stock Options and other Awards may be granted hereunder on and after adoption of this Plan by the Board. 

 

	SECTION 15.	GOVERNING LAW 

 This Plan
and all Awards and actions taken thereunder shall be governed by, and construed in accordance with, the laws of the State of Delaware, applied without regard to conflict of law principles. 

 

	SECTION 16.	DISPUTE RESOLUTION 

 (a)
Except as provided below, any dispute arising out of or relating to this Plan or any Award made hereunder, or any agreement executed in connection herewith, or the breach, termination or validity of this Plan, any such Award or any such agreement,
shall be finally settled by binding arbitration conducted expeditiously in accordance with the J.A.M.S./Endispute Comprehensive Arbitration Rules and Procedures (the “J.A.M.S. Rules”). The arbitration shall be governed by the United States
Arbitration Act, 9 U.S.C. Sections 1-16, and judgment upon the award rendered by the arbitrators may be entered by any court having jurisdiction thereof. The place of arbitration shall be New York, New York. 

 

 16 

 AEGERION 2006 STOCK OPTION
AND GRANT PLAN 
  

 (b) The arbitration shall commence within sixty (60) days of the date on which a
written demand for arbitration is filed by any party hereto. In connection with the arbitration proceeding, the arbitrator shall have the power to order the production of documents by each party and any third-party witnesses. In addition, each party
may take up to three (3) depositions as of right, and the arbitrator may in his or her discretion allow additional depositions upon good cause shown by the moving party. However, the arbitrator shall not have the power to order the answering of
interrogatories or the response to requests for admission. In connection with any arbitration, each party to the arbitration shall provide to the other, no later than seven (7) business days before the date of the arbitration, the identity of
all persons that may testify at the arbitration and a copy of all documents that may be introduced at the arbitration or considered or used by a party’s witness or expert. The arbitrator’s decision and award shall be made and delivered
within six (6) months of the selection of the arbitrator. The arbitrator’s decision shall set forth a reasoned basis for any award of damages or finding of liability. The arbitrator shall not have power to award damages in excess of actual
compensatory damages and shall not multiply actual damages or award punitive damages, and each party hereby irrevocably waives any claim to such damages. 

(c) The Company, each recipient of an Award hereunder, each party to an agreement governed hereby and any other holder of Stock issued
under this Plan (each, a “Party”) covenants and agrees that such party will participate in the arbitration in good faith. This Section 14 applies equally to requests for temporary, preliminary or permanent injunctive relief, except
that in the case of temporary or preliminary injunctive relief any party may proceed in court without prior arbitration for the limited purpose of avoiding immediate and irreparable harm. 

(d) Each Party (i) hereby irrevocably submits to the jurisdiction of any United States District Court of competent jurisdiction for
the purpose of enforcing the award or decision in any such proceeding, (ii) hereby waives, and agrees not to assert, by way of motion, as a defense, or otherwise, in any such suit, action or proceeding, any claim that it is not subject
personally to the jurisdiction of the above-named courts, that its property is exempt or immune from attachment or execution (except as protected by applicable law), that the suit, action or proceeding is brought in an inconvenient forum, that the
venue of the suit, action or proceeding is improper or that this Plan or any Award or agreement therefor or the subject matter hereof may not be enforced in or by such court, and (iii) hereby waives and agrees not to seek any review by any
court of any other jurisdiction which may be called upon to grant an enforcement of the judgment of any such court. Each Party hereby consents to service of process by registered mail at the address to which notices are to be given. Each Party
agrees that its, his or her submission to jurisdiction and its, his or her consent to service of process by mail is made for the express benefit of each other Party. Final judgment against any Party in any such action, suit or proceeding may be
enforced in other jurisdictions by suit, action or proceeding on the judgment, or in any other manner provided by or pursuant to the laws of such other jurisdiction. 

DATE APPROVED BY BOARD OF DIRECTORS: May 31, 2006 

DATE APPROVED BY STOCKHOLDERS: May 31, 2006 
  

 17 

 Aegerion Pharmaceuticals, Inc. 

Amendment No. 1 

To 
 2006
Stock Option and Grant Plan 
 Pursuant to resolutions duly adopted by the board of directors on November 9, 2007 and duly approved by
the stockholders on November 9, 2007 and in accordance with Section 11 of the 2006 Stock Option and Grant Plan (the “Plan”) of Aegerion Pharmaceuticals Inc., the Plan be and hereby is amended by deleting the clause
“1,555,060 Shares, subject to adjustment as provided in Section 3(b)” in Section 3(a) of the Plan and replacing it with “1,206,809 Shares, such number having already been adjusted to account for a 1 for 0.679674238 reverse
split of the Company’s Common Stock effected on May 25, 2007 (the “Reverse Split”) and being subject to further adjustment for subsequent events as provided in Section 3(b).” 

This Amendment No. 1 shall be effective as of the date it was adopted by the board of directors. Except as amended hereby, the Plan shall remain in
full force and effect. This Amendment No. 1 shall be governed by and interpreted in accordance with the laws of the State of Delaware, without regard to any applicable conflicts of law. 

Adopted by the Board of Directors on 

November 9, 2007 
 Adopted by the
stockholders on 
 November 9, 2007 

 Aegerion Pharmaceuticals, Inc. 

Amendment No. 2 

to 
 2006
Stock Option and Grant Plan 
 Pursuant to resolutions duly adopted by the board of directors on August 28, 2008 and duly approved by
the stockholders on August 28, 2008 and in accordance with Section 11 of the 2006 Stock Option and Grant Plan, as amended (the “Plan”), of Aegerion Pharmaceuticals, Inc., the Plan be and hereby is amended by deleting the term
“1,206,809 Shares” in Section 3(a) of the Plan and replacing it with “1,460,153 Shares.” 
 This Amendment No. 2
shall be effective as of the date it was adopted by the board of directors. Except as amended hereby, the Plan shall remain in full force and effect. This Amendment No. 2 shall be governed by and interpreted in accordance with the laws of the
State of Delaware, without regard to any applicable conflicts of law. 
 Adopted by the Board of Directors on 

August 28, 2008 
 Adopted by the
stockholders on 
 August 28, 2008 

 FORM OF 2006 STOCK OPTION AND GRANT PLAN INCENTIVE STOCK OPTION AGREEMENT 

AEGERION PHARMACEUTICALS, INC. 2006 STOCK OPTION AND GRANT PLAN 

Incentive Stock Option Agreement 

under the Aegerion Pharmaceuticals, Inc. 

2006 Stock Option and Grant Plan 
  

					
	Name of Optionee:	  	 	 	(the “Optionee”)
			
	No. of Underlying Shares:	  	 	 	Shares of Common Stock
			
	Grant Date:	  	 	 	(the “Grant Date”)
			
	Expiration Date:	  	 	 	(the “Expiration Date”)
			
	Option Exercise Price/Share:	  	 	 	(the “Option Exercise Price”)

 Pursuant to the
Aegerion Pharmaceuticals, Inc. 2006 Stock Option and Grant Plan (the “Plan”), Aegerion Pharmaceuticals, Inc., a Delaware corporation (together with all successors thereto, the “Company”), hereby grants to the Optionee, who is an
employee of the Company or any of its Subsidiaries, an Option to purchase, on or prior to the Expiration Date (or such earlier date as provided in Section 3 below), all or any part of the number of shares of Common Stock of the Company
indicated above (the “Underlying Shares,” with such shares once issued being referred to herein and in the Plan as “Option Shares”) at the Option Exercise Price per share indicated above. 

Notwithstanding anything in this Incentive Stock Option Agreement (the “Agreement”) to the contrary, this Stock Option and any Option Shares
shall be subject to, and governed by, all the terms and conditions of the Plan, including, without limitation, Section 9 thereof concerning certain restrictions on transfer of Option Shares and related matters. To the extent there is any
inconsistency between the terms of the Plan and of this Agreement, the terms of the Plan shall control. 
 All capitalized terms used in this
Agreement and not otherwise defined shall have the respective meanings given such terms in the Plan. 
 1. Vesting and
Exercisability. 
 (a) No portion of this Stock Option may be exercised until such portion shall have
vested and become exercisable. Except as set forth in Section 1(b) below, and subject to the determination of the Committee in its sole discretion to accelerate the vesting schedule hereunder, this Stock Option shall be vested and exercisable
with respect to the Underlying Shares in accordance with the following schedule: ______________________________________________ 

(b) In the case of a Sale Event, this Stock Option shall be treated as provided in Section 4(a) of the Plan.

 2. Exercise of Stock Option. Prior to the Expiration Date (or such earlier date provided in Section 3
below), the Optionee may exercise this Stock Option by delivering a Stock Option exercise notice (an “Exercise Notice”) in the form of Appendix A hereto indicating his or her election to purchase some or all of the Underlying
Shares with respect to which this Stock Option is exercisable at the time of such notice. 
  

 1 

 3. Termination of Employment. Except as the Committee may otherwise expressly
provide, or as may otherwise be expressly provided in any employment agreement between the Company and the Optionee, if the Optionee’s employment with the Company or a Subsidiary terminates, the period within which the Optionee may exercise
this Stock Option may be subject to earlier termination as set forth below: 
 (a) Termination of Employment
Due to Death or Disability. If the Optionee’s employment terminates by reason of such Optionee’s death or disability (as defined in Section 422(c) of the Code), this Stock Option may be exercised, to the extent exercisable on the
date of such termination, by the Optionee or by the Optionee’s legal representative or legatee for a period of twelve (12) months from the date of such termination or until the Expiration Date, if earlier. 

(b) Termination for Cause. If the Optionee’s employment is terminated by the Company for Cause, all Options
(unvested and vested) shall terminate immediately. “Cause” means any of the following: (i) dishonesty, embezzlement, misappropriation of assets or property of the Company; (ii) gross negligence, misconduct, neglect of duties,
theft, fraud, or breach of fiduciary duty to the Company; (iii) violation of federal or state securities laws; (iv) breach of an employment, consulting or other agreement with the Company; or (v) the conviction of a felony, or any
crime involving moral turpitude, including a plea of guilty or nolo contendre. 
 (c) Other
Termination. If the Optionee’s employment terminates for any reason other than death or disability or Cause, this Stock Option may be exercised, to the extent exercisable on the date of such termination, by the Optionee for a period of
three (3) months from the date of termination or until the Expiration Date, if earlier. 
 (d) Treatment
of Unvested Options on Termination of Employment. Any portion of this Stock Option that is not exercisable on the date of termination of the Optionee’s employment with the Company, for any reason, shall terminate immediately and be null and
void and of no further force and effect. 
 4. Incorporation of Plan. Notwithstanding anything herein to the
contrary, this Stock Option shall be subject to and governed by all the terms and conditions of the Plan. In the event of a conflict between this Agreement and the Plan, the terms of the Plan shall govern. 

5. Transferability. This Agreement is personal to the Optionee and is not transferable by the Optionee in any manner other
than by will or by the laws of descent and distribution. All transfers of Options are governed by the terms of the Plan. 

6. Status of Stock Option. The Optionee understands that, while this Stock Option is intended to qualify as an
“incentive stock option” as defined in Section 422 of the Code to the extent permitted under applicable law, the Company makes no representation or warranty that this Stock Option will, in fact, so qualify. In order to obtain the
benefits of an incentive stock option under Section 422 of the Code, the Optionee understands that this Stock Option must be exercised within three (3) months after termination of employment or within twelve (12) months after
termination of employment if such termination is due to death or disability; provided, that in no event may this Stock Option be exercised after the Expiration Date. The Optionee further understands that, to obtain such benefits, no sale or
other disposition may be made of Option Shares for which incentive stock option treatment is desired within the one-year period beginning on the day after the day of the transfer of such Option Shares to him or her,

  

 2 

 
nor within the two-year period beginning on the day after the Grant Date of this Stock Option. If the Optionee disposes (whether by sale, gift, transfer or otherwise) of any such Option Shares
within either of these periods (a “disqualifying disposition”), he or she will notify the Company within thirty (30) days after such disposition. The Optionee also agrees to provide the Company with any information concerning any such
dispositions required by the Company for tax purposes. Further, to the extent Underlying Shares and any other incentive stock options of the Optionee having an aggregate Fair Market Value in excess of $100,000 (determined as of the Grant Date) vest
in any year, such options will not qualify as incentive stock options. To the extent that any portion of the Stock Option does not qualify as an incentive stock option, whether due to a disqualifying disposition or otherwise, it shall be deemed a
non-qualified stock option. 
 7. Drag-Along Rights. As a condition to the receipt of this Stock Option and the
Option Shares, the Optionee hereby covenants and agrees to be bound by the provisions of any agreement entered into by and among the Company and at least a majority in voting interest of the stockholders of the Company that provides for certain
drag-along rights in a bona fide sale of the Company to an unaffiliated third party, whether directly or pursuant to a merger, consolidation or otherwise, a purchase of all or substantially all of the Company’s assets, or a purchase of fifty
percent (50%) or more of the Company’s outstanding shares of capital stock. As of the date hereof, the Company has entered into a certain Stockholders’ Agreement by and among the Company and the Stockholders party thereto dated as of
December 15, 2005 and as further amended, restated or replaced from time to time (the “Stockholders’ Agreement”) that provides for the following drag-along rights (with capitalized terms as defined in such agreement): 

“Drag-Along Rights. In the event that the Required Series A Majority votes to approve or consents to a bona fide sale of the
Company to an unaffiliated third party, whether directly or pursuant to a merger, consolidation or otherwise, a purchase of all or substantially all of the Company’s assets or a purchase of fifty percent (50%) or more of the Company’s
outstanding shares of capital stock (a “Company Sale”), in each case, in a single transaction or series of related transactions (collectively, a “Proposed Transaction”), then the Stockholders shall, if requested by
such Required Series A Majority, (i) if the Company Sale is structured as a sale of stock, sell all of his, her or its shares of capital stock (including all rights to purchase or exercisable for shares of capital stock) in such transaction,
(ii) if the Company Sale is structured as a sale of assets, merger, consolidation or other transaction requiring the consent or approval of the Company’s stockholders, vote such shares in favor thereof, and otherwise consent to and raise
no objection to such transaction, and waive any dissenters’ rights, appraisal rights or similar rights that such Stockholders may have in connection therewith, or (iii) if the Proposed Transaction includes the sale, contribution, exchange,
redemption, cancellation or other disposition of securities convertible into or exchangeable for capital stock of the Company, or options, warrants or other rights to purchase such capital stock or securities, sell, contribute, exchange, redeem,
cancel or otherwise dispose of such securities or options, warrants or other rights; in each such case on the same terms and for the same consideration as are applicable to the Required Series A Majority; provided, however, that
pursuant to the terms of such proposed transactions, (i) the Stockholders shall not be required to give any representations and warranties regarding the operations and conditions (financial and otherwise) of the Company and its business, assets
and liabilities (unless such Stockholders are officers of the Company and are giving such representations and warranties solely in such capacity and such officers shall not be required to give any such representations or warranties as Stockholders),
and (ii) the indemnification obligations of each Stockholder shall be limited to that Stockholder’s pro rata portion of the losses relating to such indemnity based on such Stockholder’s

  

 3 

 
percentage ownership of the Company on a fully-diluted basis. The Required Series A Majority shall give the Stockholders who are not the Required Series A Majority written notice of any Proposed
Transaction as soon as practicable but in any event at least fifteen (15) days prior to the date on which such transaction is proposed to be consummated, including the terms and conditions thereof, and the Stockholders shall have the obligation
to sell their respective shares of capital stock or vote or otherwise consent as provided above on such same terms and conditions in accordance with the instructions set forth in such notice. The Company and each Stockholder will take all reasonably
necessary and desirable actions to consummate such Proposed Transaction, including, without limitation, the execution of all agreements and other instruments and such other actions reasonably necessary to effectuate the allocation and distribution
of the aggregate consideration upon the Proposed Transaction as set forth in this Section 3.1. In such event, each Stockholder shall deliver the certificates representing his, her or its shares of capital stock (accompanied by duly
executed stock powers or other instrument of transfer duly endorsed in blank) to the Company or to an agent designated by the Company, for the purpose of effectuating the transfer of such shares, if the Company Sale is structured as a sale of stock,
or deliver such other documents that are reasonably required by the Required Series A Majority, if the Company Sale is structured as a sale of assets, merger, consolidation or other transaction requiring the consent or approval of the Company’s
stockholders, to the purchaser and the disbursement of the proceeds of such transactions to each of the Stockholders and other stockholders, as applicable. The Company may, at its option, deposit the consideration payable for such shares with a
depository designated by it and thereafter each certificate shall represent only the right to receive the consideration payable in the transaction.” 

A copy of any such agreement described in this Section 7 may be obtained by any Optionee at no cost by written request to the Company. 

8. Miscellaneous Provisions. 

(a) Change and Modifications. This Agreement may not be orally changed, modified or terminated, nor shall any oral
waiver of any of its terms be effective. This Agreement may be changed, modified or terminated only by an agreement in writing signed by the Company and the Optionee. 

(b) Governing Law. This Agreement shall be governed by and construed in accordance with the laws of Delaware
without regard to conflict of law principles. 
 (c) Equitable Relief. The parties hereto agree and
declare that legal remedies may be inadequate to enforce the provisions of this Agreement and that equitable relief, including specific performance and injunctive relief, may be used to enforce the provisions of this Agreement. 

(d) Headings. The headings are intended only for convenience in finding the subject matter and do not constitute
part of the text of this Agreement and shall not be considered in the interpretation of this Agreement. 
 (e)
Saving Clause. If any provision(s) of this Agreement shall be determined to be illegal or unenforceable, such determination shall in no manner affect the legality or enforceability of any other provision hereof. 

 

 4 

 (f) Notices. All notices, requests, consents and other communications
shall be in writing and be deemed given when delivered personally, by telex or facsimile transmission or when received if mailed by first class registered or certified mail, postage prepaid. Notices to the Company or the Optionee shall be addressed
as set forth underneath their signatures below, or to such other address or addresses as may have been furnished by such party in writing to the other. 

(g) Benefit and Binding Effect. This Agreement shall be binding upon and shall inure to the benefit of the parties
hereto, their respective successors, permitted assigns, and legal representatives. The Company has the right to assign this Agreement, and such assignee shall become entitled to all the rights of the Company hereunder to the extent of such
assignment. 
 (h) Counterparts. For the convenience of the parties and to facilitate execution, this
Agreement may be executed in two or more counterparts, each of which shall be deemed an original, but all of which shall constitute one and the same document. 

[SIGNATURE PAGE FOLLOWS] 
  

 5 

 The foregoing Agreement is hereby accepted and the terms and conditions thereof hereby
agreed to by the undersigned as of the date first above written. 
  

			
	AEGERION PHARMACEUTICALS, INC.
		
	By:	 	 
		 	Name:
		 	Title:

			
		
	Address:	 	[                    ]

The undersigned hereby acknowledges receiving and reviewing a copy of the Plan, including, without limitation, Section 9 thereof, and understands
that the Stock Option granted hereby is subject to the terms of the Plan and of this Agreement. This Agreement is hereby accepted, and the terms and conditions thereof and of the Plan hereby agreed to, by the undersigned as of the date first above
written. 
  

			
	OPTIONEE:
		
	 	 	 
	Name:	 	
		
	Address:	 	

  

			
	DESIGNATION OF BENEFICIARY:	  	____________________________________________
		
	Beneficiary’s Address:	  	

  

 6 

 SPOUSE’S
CONSENT1 

The undersigned hereby acknowledges receiving and reviewing a copy of the Plan, including, without limitation, Section 9 thereof, and understands
that the Stock Option granted hereby is subject to the terms of the Plan and of this Agreement. This Agreement is hereby accepted, and the terms and conditions hereof and of the Plan are hereby agreed to, by the undersigned as of the date first
above written. 
  

			
	SPOUSE:
		
	 	 	 
	Name:	 	
		
	Address:	 	

  

	1
	 Required only if Optionee’s state of residence is a community property state such as Arizona, California, Idaho, Louisiana, New Mexico, Nevada,
Texas, Washington or Wisconsin. 

  

 7 

 Appendix A 

STOCK OPTION EXERCISE NOTICE 

Aegerion Pharmaceuticals, Inc. 
 Attention:
Chief Financial Officer 
 ___________________________ 

___________________________ 

Pursuant to the terms of the stock option agreement between myself and Aegerion Pharmaceuticals, Inc. (the “Company”) dated
             (the “Agreement”), under the Company’s 2006 Stock Option and Grant Plan, I, [Insert Name]
                                        ,
hereby [Circle One] partially/fully exercise such Stock Option by including herein payment in the amount of $             representing the purchase price for [Fill in number of
Underlying Shares]              Option Shares. I have chosen the following form(s) of payment: 
  

					
	[ ]	  	1.	    	Cash
			
	[ ]	  	2.	    	Certified or bank check payable to Aegerion Pharmaceuticals, Inc.
			
	[ ]	  	3.	    	Other (as described in the Plan (please describe))
			
		  		    	__________________________________________________________________.

In connection with my exercise of the Stock Option as set forth above, I hereby represent and warrant to the Company as follows:

 (i) I am purchasing the Option Shares for my own account for investment only, and not for resale or with a
view to the distribution thereof. 
 (ii) I have had such an opportunity as I have deemed adequate to obtain from
the Company such information as is necessary to permit me to evaluate the merits and risks of my investment in the Company and have consulted with my own advisers with respect to my investment in the Company. 

(iii) I have sufficient experience in business, financial and investment matters to be able to evaluate the risks involved
in the purchase of the Option Shares and to make an informed investment decision with respect to such purchase. 

(iv) I can afford a complete loss of the value of the Option Shares and am able to bear the economic risk of holding such
Option Shares for an indefinite period of time. 
 (v) I understand that the Option Shares may not be registered
under the Securities Act of 1933 (it being understood that the Option Shares are being issued and sold in reliance on the exemption provided in Rule 701 thereunder) or any applicable state securities or “blue sky” laws and may not be sold
or otherwise transferred or disposed of in the absence of an effective registration statement under the Securities Act of 1933 and under any applicable state securities or “blue sky” laws (or exemptions from the registration requirements
thereof). I further acknowledge that certificates representing Option Shares will bear restrictive legends reflecting the foregoing. 
  

 8 

 (vi) I understand and agree that the Option Shares when issued will continue
to be subject to the Plan, including Section 9 thereof. 
  

	
	Sincerely yours,
	
	  
	Name:
	
	Address:
	
	 
	
	 
	
	 

  

 9 

 Form of Amendment No. 1 to Form of Incentive Stock Option Agreement

 This Amendment No. 1 to Incentive Stock Option Agreement (the “Amendment”) is made as of this
__ day of June 2007, by and between Aegerion Pharmaceuticals, Inc., a Delaware corporation (the “Company”) and _________ (the “Optionee”), parties to that certain Incentive Stock Option Agreement dated as of
_________ (the “Agreement”). Capitalized terms used herein, unless otherwise defined herein, shall have the meanings ascribed to them in the Agreement. 

WHEREAS, the Company and the Optionee desire to amend the Agreement; and 

WHEREAS, pursuant to Section 8(a) of the Agreement, a change or modification to the terms and provisions of the Agreement may
not be effected without the prior written consent of the Company and the Optionee. 
 NOW, THEREFORE in consideration of
the foregoing and intending to be legally bound, the Company and the Optionee agree as follows. 
 1. Section 7 of the Agreement shall
terminate upon an Initial Public Offering. For purposes of the Agreement, “Initial Public Offering” shall mean the Company’s initial distribution of shares of its common stock in a firm commitment underwritten public offering to the
general public pursuant to a registration statement filed with and declared effective by the Securities and Exchange Commission pursuant to the Securities Act of 1933, as amended. 

2. This Amendment shall be deemed to be a contract made under, and shall be construed in accordance with, the laws of the State of Delaware, without
giving effect to conflict of interest laws principles thereof. 
 3. This Amendment may be executed in multiple counterparts, each of which
shall be deemed an original, but all of which together shall constitute one and the same instrument. 
 4. Except to the extent amended hereby,
the terms and provisions of the Agreement shall remain in full force and effect. 
 [SIGNATURE PAGE FOLLOWS] 

 

 IN WITNESS WHEREOF, the parties hereto have executed this Amendment No. 1 to Incentive
Stock Option Agreement as of the date and year first written above. 
  

			
	AEGERION PHARMACEUTICALS, INC.
		
	By:	 	 
		 	 Name: Gerald Wisler
 Title:
President and Chief Executive Officer

	
	 
	[Optionee]Amended and Restated Investor Rights Agreement

 Exhibit 10.3 

AMENDED AND RESTATED 

INVESTOR RIGHTS AGREEMENT 

This AMENDED AND RESTATED INVESTOR RIGHTS AGREEMENT is made as of November 9, 2007 (“Investor Rights Agreement”),
by and among Aegerion Pharmaceuticals, Inc., a Delaware corporation (the “Company”), and the Investors listed on Schedule I attached hereto (the “Investors”). 

WHEREAS, the Company and certain of the Investors (the “Prior Investors”) previously entered into an Investor Rights
Agreement, dated December 15, 2005 (the “Prior Agreement”), in connection with the purchase of shares of Series A Preferred Stock of the Company, par value $0,001 per share (“Series A Preferred”); 

WHEREAS, the Prior Investors and the Company desire to induce certain of the Investors to purchase shares of Series B Preferred Stock of
the Company, par value $0,001 per share, (“Series B Preferred” and together with the Series A Preferred, the “Preferred Stock”) pursuant to the Series B Preferred Stock Purchase Agreement dated as of the date hereof
by and among the Company and certain of the Investors (the “Purchase Agreement”) by amending and restating the Prior Agreement to provide the Investors with the rights and privileges as set forth herein; and 

WHEREAS, (i) Section 6.3 of the Prior Agreement provides that it may be amended with the consent of the Company and the
holders of the Required Series A Majority (defined below) and (ii) the undersigned holders represent a Required Series A Majority. 

NOW, THEREFORE, the Company and the Investors, including the Prior Investors, each hereby agree to amend and restate the Prior Agreement
in its entirety as set forth herein, and the parties hereto further agree as follows: 
 1. GENERAL PROVISIONS 

1.1 Shares Subject to this Investor Rights Agreement. The Investors expressly agree that the terms and restrictions of this
Investor Rights Agreement shall apply to all shares of capital stock which any of them now owns or hereafter acquires by any means, including, without limitation, by purchase, assignment or operation of law, or as a result of any stock dividend,
stock split, reorganization, reclassification, whether voluntary or involuntary, or other similar transaction, and to any shares of capital stock of any successor in interest of the Company, whether by sale, merger, consolidation or other similar
transaction, or by purchase, assignment or operation of law (the “Shares”). 
 1.2 No Partnership
Relationship. Notwithstanding, but not in limitation of, any other provision of this Investor Rights Agreement, the parties understand and agree that the creation, management and operation of the Company shall not create or imply a general
partnership 

 
between or among the Investors and shall not make any Investor the agent or partner of any other Investor for any purpose. 

1.3 Certain Definitions. As used in this Investor Rights Agreement, the following terms shall have the following respective
meanings: 
 “Advent International Entity” shall mean any of the following: Advent Healthcare and Life Sciences
III Limited Partnership, Advent Healthcare and Life Sciences III-A Limited Partnership, Advent Partners HLS III Limited Partnership. 

“Affiliate” of any Person (as defined below) means, with respect to any person or entity, any other person or entity
which controls, or is controlled by, or is under common control with the subject referenced and, for the purposes hereof, the term “control” (including the terms “controlling”, “controlled by” and “under common
control with”) shall mean the possession, directly or indirectly, of the power to direct or cause the direction of the management and policies of a person or entity, whether through the ownership of voting securities or by contract or
otherwise; provided that (A) in the case of any Advent International Entity, “Affiliate” shall include any other Advent International Entity, (B) in the case of any Index Ventures Entity, “Affiliate” shall
include any other Index Ventures Entity, (C) in the case of any Alta Partners Entity, “Affiliate” shall include any other Alta Partners Entity and (D) in the case of any MVM Entity, “Affiliate” shall include any other
MVM Entity. 
 “Alta Partners Entity” shall mean any of the following: Alta BioPharma Partners III, L.P., Alta
BioPharma Partners III GmbH & Co. Beteiligungs KG, Alta Embarcadero BioPharma Partners III, LLC. 
 “Amended
and Restated Certificate of Incorporation” shall mean the Amended and Restated Certificate of Incorporation of the Company as filed with the Secretary of State of the State of Delaware on the date hereof, as amended from time to time
thereafter in accordance with its terms, the By-laws of the Company, and the laws of the State of Delaware. 

“Commission” shall mean the U.S. Securities and Exchange Commission and any successor agency of the Federal government
administering the Securities Act and the Exchange Act. 
 “Common Stock” shall mean (i) the common stock,
$.001 par value per share, of the Company, (ii) any other capital stock of the Company, however designated, authorized on or after the date hereof, which shall neither be limited to a fixed sum or percentage of par value in respect of the
rights of the holders thereof to participate in dividends nor entitled to a preference in the distribution of assets upon the voluntary or involuntary liquidation, dissolution or winding up of the Company; and (iii) any other securities into
which or for which any of the securities described in (i) or (ii) may be converted or exchanged pursuant to a plan of recapitalization, reorganization, merger, consolidation, sale of assets or other similar transaction. 

“Exchange Act” shall mean the Securities Exchange Act of 1934, as amended, and any similar or successor Federal statute,
and the rules and regulations of the Commission promulgated thereunder, all as the same shall be in effect from time to time. 
  

 2 

 “Federal” shall mean with respect to any executive, legislative or judicial
branch of government or other agency or organ of government, a branch or other agency or organ of the government of the United States. 

“Index Ventures Entity” shall mean any of the following: Index Ventures III (Jersey) L.P., Index Ventures III (Delaware)
L.P., Index Ventures III Parallel Entrepreneur Fund (Jersey) L.P. and Index Venture Management S.A. on behalf of Index Employee Investment Plan. 

“MVM Entity” shall mean any of the following: MVM International Life Sciences Fund No. 1 L.P. and MVM Executive
Limited. 
 “Person” shall mean an individual or group of individuals, a corporation, an association, a
partnership, a limited or general limited liability company, an estate, a trust, and any other entity or organization, governmental or otherwise. 

“Qualified Public Offering” shall mean a firm commitment underwritten public offering pursuant to an effective
registration statement under the Securities Act of 1933, as amended, in connection with which all of the Company’s shares of Preferred Stock convert to Common Stock. 

The terms “register,” “registered” and “registration” shall mean a registration
effected by preparing and filing a registration statement in compliance with the Securities Act and applicable rules and regulations thereunder, and the declaration or ordering of the effectiveness of such registration statement, or, as the context
may require, under the Exchange Act or applicable state securities laws. 
 “Registrable Securities” shall mean
(i) shares of Preferred Stock; (ii) shares of Common Stock or other securities issued or issuable pursuant to the conversion of the Preferred Stock; (iii) any shares of Common Stock directly held by the Investors that are
“restricted securities” within the meaning of the Securities Act; and (iv) any shares of Common Stock or other securities issued or issuable with respect to the Preferred Stock by reason of any stock split, stock dividend,
recapitalization, reorganization, merger, consolidation, sale of assets or similar event, excluding in any event securities which have been (a) registered under the Securities Act pursuant to an effective registration statement filed thereunder
and disposed of in accordance with the registration statement covering them or (b) publicly sold pursuant to Rule 144 under the Securities Act. Wherever reference is made in this Investor Rights Agreement to a request or consent of holders of a
certain percentage of Registrable Securities, the determination of such percentage shall be calculated on the basis of shares of Common Stock issued or issuable upon conversion of the Preferred Stock even if such exercise has not been effected.

 “Registration Expenses” shall mean the expenses so described in Section 4.5. 

“Required Series A/B Majority” shall mean the holders of at least two-thirds of the Series A Preferred and Series B
Preferred, voting together as a class. 
 “Securities Act” shall mean the Securities Act of 1933, as amended,
and any similar or successor Federal statute, and the rules and regulations of the Commission thereunder, all as the same shall be in effect from time to time. 
  

 3 

 “Selling Expenses” shall mean the expenses so described in
Section 4.5. 
 “Subsidiary” or “Subsidiaries” shall mean any corporation,
partnership, trust or other entity of which the Company and/or any of its other Subsidiaries directly or indirectly owns at the time a majority of the outstanding shares of any class of equity security of such corporation, partnership, trust or
other entity. 
 2. PRE-EMPTIVE RIGHTS 

2.1 Right of First Offer. Except with respect to “Exempt Issuances” as defined in Section 2.3, in the
event that the Company proposes to issue (the “New Issuance”) any (i) shares of Common Stock, (ii) warrants, options or other rights to purchase Common Stock (collectively, “Rights”), or (iii) any
debentures or other securities convertible into or exchangeable for shares of Common Stock (collectively, “Convertible Securities”), the Company will first offer to sell such securities to the Investors (the
“Offer”) and deliver a notice to the Investors (the “Offer Notice”) of such Offer, stating the price and other terms and conditions thereof (including any definitive documents relating thereto), which Offer by its
terms shall remain open and irrevocable for a period of thirty (30) days from the date it is delivered by the Company to the Investors. 

2.2 Right to Purchase Shares, Rights or Convertible Securities. 

(a) The holders of Preferred Stock (the “Preferred Holders”) shall have the right to purchase up to such number of shares
of Common Stock, Rights or Convertible Securities, as applicable, of the New Issuance at the price and on the terms stated in the Offer Notice, such price to be paid in full in cash or by check at the time of issuance of such securities to the
Preferred Holders so that, after giving effect to the New Issuance, each holder of Preferred Stock who exercises such right will continue to maintain its same proportionate ownership of Common Stock as of the date immediately preceding the Offer,
treating each Preferred Holder for the purpose of such computation as the holder of (i) the number of shares of Common Stock which would be issuable to it upon conversion, exercise and exchange of all Rights and Convertible Securities held by
it and (ii) the number of shares of Common Stock directly held by it, in each case on the date immediately preceding the Offer and assuming the like conversion, exercise and exchange of all such securities held by other persons (“Equity
Percentage”). The rights set forth in this Section 2 shall be exercised by the Preferred Holders, if at all, by written notice (the “Acceptance Notice”) to the Company delivered not later than thirty
(30) days after the receipt by the Preferred Holders of the Offer Notice in accordance with the terms and conditions stated therein, and such right shall expire at the end of the twentieth day after the day of the receipt by the Preferred
Holders of the Offer Notice. 
 (b) Each Preferred Holder shall have a right of oversubscription such that if any other
Preferred Holder fails to exercise its right to maintain its Equity Percentage, the other Preferred Holder(s), who do exercise such right, shall, among them, have the right to purchase up to the balance of such securities in the Offer not so
purchased. The Investors may exercise such right of oversubscription by electing to purchase more than their Equity Percentage of the Offer by so indicating in their Acceptance Notice. If, as a result thereof, such oversubscriptions exceed the total
number of the securities in the Offer available in respect to such oversubscription 
  

 4 

 
privilege, the oversubscribing Preferred Holders shall be cut back with respect to oversubscriptions on a pro rata basis in accordance with their Equity Percentage or as they may otherwise agree
among themselves. 
 (c) In the event that Acceptance Notices are not given by the Investors in respect of all of the New
Issuance, the Company shall have 120 days from the expiration of the foregoing thirty (30) day period to sell all or any part of such New Issuance to which the Acceptance Notices have not been given by the Investors to any other Person or
Persons, but only upon terms and conditions in all material respects, including, without limitation, share price and dividend rates, which are no more favorable to such other person or persons and no less favorable to the Company than those set
forth in the Offer. A failure by any Investor to exercise its pre-emptive rights under this Section 2 on any occasion shall not act as a waiver of any such rights in respect of future New Issuances. 

2.3 Exempt Issuances. The term “Exempt Issuances” referred to in Section 2.1 which will not give the
Preferred Holders the rights described in Section 2.2 are (a) issuances of Excluded Securities as such term is defined in Section B(2)(d)(i)(E) of the Amended and Restated Certificate of Incorporation, as such definition may be
amended from time to time, and (b) any sale prior to June 30, 2008, of Series B Preferred to one or more institutional investors, in one transaction or a series of related transactions, made solely for the purposes of capital raising and
not in connection with any strategic license, partnering or similar collaborative or strategic transaction, and in which the gross proceeds to the Company are at least $12.0 million. 

2.4 Assignment. The rights of the Investors under this Section 2 may be assigned to an Affiliate of an Investor
provided such transferee agrees in writing to be bound to the terms, agreements and restrictions contained in this Investor Rights Agreement. 

2.5 Termination. The respective rights and obligations of the parties under this Section 2 shall terminate upon the
closing of the Company’s Qualified Public Offering. 
 3. TRANSFER OF REGISTRABLE SECURITIES 

3.1 Restrictive Legend. Each certificate representing Registrable Securities shall, except as otherwise provided in this
Section 3.1 or in Section 3.2, be stamped or otherwise imprinted with a legend substantially in the following form (in addition to any legend required under applicable state securities laws): 

“The securities represented by this certificate have not been registered under the Securities Act of 1933, as amended (the
“Securities Act”), or any other securities laws. These securities have been acquired for investment and not with a view to distribution or resale. Such securities may not be offered for sale, sold, delivered after sale, transferred,
pledged or hypothecated in the absence of an effective registration statement covering such securities under the Securities Act and any other applicable securities laws, unless the holder shall have obtained an opinion of counsel reasonably
satisfactory to the corporation that such registration is not required.” 
  

 5 

 Upon the request of a holder of such a certificate, the Company shall remove the foregoing
legend from the certificate or issue to such holder a new certificate therefor free of any transfer legend, if there is an effective registration statement covering the securities represented by such certificate or, with such request, the Company
shall have received either the opinion referred to in Section 3.2(a)(1) or the “no-action” letter referred to in Section 3.2(a)(ii). 

3.2 Notice of Proposed Transfer. 

(a) Prior to any proposed sale, pledge, hypothecation or other transfer of any Registrable Securities (other than under the circumstances
described in Section 4.1, 4.2 or 4.3), the holder thereof shall give written notice to the Company of its intention to effect such sale, pledge, hypothecation or other transfer. Each such notice shall describe the manner of
the proposed sale, pledge, hypothecation or other transfer and, if requested by the Company shall be accompanied by either (i) an opinion of counsel reasonably satisfactory to the Company to the effect that the proposed sale, pledge,
hypothecation or other transfer may be effected without registration under the Securities Act or (ii) a “no action” letter from the Commission to the effect that the distribution of such securities without registration will not result
in a recommendation by the staff of the Commission that action be taken with respect thereto, whereupon the holder of such stock shall be entitled to transfer such stock in accordance with the terms of its notice; provided, however,
that no such opinion of counsel or “no action” letter shall be required (i) for a distribution to one or more partners or members of the transferor (in the case of a transferor that is a partnership or limited liability company) in
each case in respect of the beneficial interest of such partner or member; (ii) for any transfer by a Preferred Holder to any Affiliate of such Preferred Holder; or (iii) for transfers made in accordance with the provisions of Rule 144 (or
any rule permitting public sale without registration under the Securities Act) including Rule 144(k). Each certificate for Registrable Securities transferred as above provided shall bear the appropriate restrictive legend set forth in
Section 3.1, except that such certificate shall not bear such legend if (i) such transfer is in accordance with the provisions of Rule 144 (or any other rule permitting public sale without registration under the Securities Act) or
(ii) the opinion of counsel or “no-action” letter referred to above is to the further effect that the transferee and any subsequent transferee (other than an Affiliate of the Company) would be entitled to transfer such securities in a
public sale without registration under the Securities Act or that such legend is not required to establish compliance with any provisions of the Securities Act. Notwithstanding any other provision hereof, the restrictions provided for in this
Section 3.2 shall not apply to securities which are not required to bear the legend prescribed by Section 3.1 in accordance with the provisions of that Section. 

(b) No such opinion of counsel or “no action” letter from the Commission, as set forth in Section 3.2(a) above,
shall be required in the event of a sale, pledge, hypothecation or other transfer of any Registrable Securities to (i) any Affiliate of an Investor, including, without limitation, any venture capital limited partnership now existing or
hereafter formed which controls, is controlled by or is under common control with such Investor; and (ii) any successors or assigns of any of the foregoing persons, provided that the transferee agrees in writing to be subject to this Investor
Rights Agreement to the same extent as if such transferee were originally a signatory. 
  

 6 

 4. REGISTRATION 

4.1 Required Registration. 

(a) At any time after the earlier of (i) expiration of the six (6) month period following the closing of the Company’s
Qualified Public Offering and (ii) the first (1st) anniversary of the date hereof, one or more of the holders of Registrable Securities constituting a majority of the total shares of Registrable Securities then outstanding may request that
the Company register for sale under the Securities Act up to all of the shares of Registrable Securities held by such holders in the manner specified in such notice. 

(b) Following receipt of any notice under this Section 4.1(a), the Company shall immediately notify all holders of
Registrable Securities from whom notice has not been received and such holders shall then be entitled within thirty (30) days after receipt of such notice from the Company to request the Company to include in the requested registration all or
any portion of their shares of Registrable Securities. The Company shall use its best efforts to register under the Securities Act for public sale in accordance with the method of disposition specified in the notice from requesting holders described
in paragraph (a) above, within one hundred eighty (180) days of its receipt of such notice, the number of shares of Registrable Securities specified in such notice. The Company shall be obligated to register the Registrable Securities
pursuant to this Section 4.1 on one (1) occasion only. Notwithstanding anything to the contrary contained herein, no request may be made under this Section 4.1 after the effective date of a registration statement filed
by the Company covering a firm commitment underwritten public offering and prior to the later to occur of the completion of the period of distribution for such offering or ninety (90) days after the effective date of such registration
statement. 
 (c) If the holders requesting such registration intend to distribute the Registrable Securities covered by their
request by means of an underwriting, they shall so advise the Company as a part of their request made pursuant to this Section 4.1 and the Company shall include such information in the written notice referred to in paragraph
(b) above. The right of any holder to registration pursuant to this Section 4.1 shall be conditioned upon such holder’s agreeing to participate in such underwriting and to permit inclusion of such holder’s Registrable
Securities in the underwriting. The Company’s Board of Directors (the “Board”) shall designate the managing underwriter of such offering. A holder may elect to include in such underwriting all or a part of the Registrable
Securities it holds. 
 (d) A registration statement filed pursuant to this Section 4.1 may, subject to the
following provisions, include (i) shares of Common Stock for sale by the Company for its own account and (ii) shares of Common Stock held by officers or directors of the Company, in each case for sale in accordance with the method of
disposition specified by the requesting holders. If such registration shall be underwritten, the Company and such officers and directors proposing to distribute their shares through such underwriting shall enter into an underwriting agreement in
customary form with the representative of the underwriter or underwriters selected for such underwriting on terms no less favorable to such officers or directors than the terms afforded the holders of Registrable Securities. If and to the extent
that the managing underwriter determines that marketing factors require a limitation on the number of shares to be included in such registration, such exclusion, to the extent required by the managing underwriter, shall be

  

 7 

 
applied in the following order: first, to the shares held by the directors and officers and second, to the shares of Common Stock of the Company to be included for its own account. If the
managing underwriter determines that marketing factors require a further limitation of the number of Registrable Securities to be registered under this Section 4.1, then Registrable Securities shall be excluded in such manner that the
securities to be sold shall be allocated among the selling holders pro rata based on their ownership of Registrable Securities. In any event all securities to be sold other than Registrable Securities will be excluded prior to any exclusion of
Registrable Securities. If any holder of Registrable Securities, officer or director who has requested inclusion in such registration as provided above, disapproves of the terms of the underwriting, such holder of securities may elect to withdraw
therefrom by written notice to the Company and the managing underwriter. The securities so withdrawn shall also be withdrawn from registration. Except for registration statements on Form S-4, S-8 or any comparable form or successor thereto, the
Company will not file with the Commission any other registration statement with respect to its Common Stock, whether for its own account or that of other stockholders, from the date of receipt of a notice from requesting holders pursuant to this
Section 4.1 until the completion of the period of distribution of the registration contemplated thereby or one hundred twenty (120) days after the effective date of such registration, whichever is later. 

4.2 Incidental Registration. If the Company at any time (other than pursuant to Section 4.1 or
Section 4.3) proposes to register any of its securities under the Securities Act for sale to the public, whether for its own account or for the account of other security holders or both (except with respect to an initial public offering
of the Company’s securities (other than pursuant to Section 4.1) or registration statements on Forms S-4, S-8 or any successor to such forms or another form not available for registering the Registrable Securities for sale to the
public), each such time it will promptly give written notice to all holders of the Registrable Securities of its intention so to do. Upon the written request of any such holder, received by the Company within thirty (30) days after the giving
of any such notice by the Company, to register any or all of its Registrable Securities, the Company will use its best efforts to cause the Registrable Securities as to which registration shall have been so requested to be included in the securities
to be covered by the registration statement proposed to be filed by the Company, all to the extent requisite to permit the sale or other disposition by the holder (in accordance with its written request) of such Registrable Securities so registered.
If the registration of which the Company gives notice is for a registered public offering involving an underwriting, the Company shall so advise the holders of Registrable Securities as a part of the written notice given pursuant to this
Section 4.2. In such event the right of any holder of Registrable Securities to registration pursuant to this Section 4.2 shall be conditioned upon such holder’s participation in such underwriting to the extent provided
herein. All holders of Registrable Securities proposing to distribute their securities through such underwriting shall (together with the Company) enter into an underwriting agreement in customary form with the underwriter or underwriters selected
for underwriting by the Company. Notwithstanding any other provision of this Section 4.2, if the underwriter determines that marketing factors require a limitation on the number of shares to be underwritten, the Company shall so advise
all holders of securities requesting registration of any limitations on the number of shares to be underwritten, and the number of shares of securities that are entitled to be included in the registration and underwriting shall be allocated
(i) first to the Company with respect to shares of Common Stock being sold for its own account; and (ii) second, to holders of Registrable Securities requesting registration in proportion, as nearly as

  

 8 

 
practicable, to the respective amounts of securities owned by them, provided that, notwithstanding the foregoing, at least thirty percent (30%) of the shares to be included in such
registration shall be Registrable Securities. Notwithstanding the foregoing provisions, the Company may withdraw any registration statement referred to in this Section 4.2 without thereby incurring any liability to the holders of
Registrable Securities. If any holder of Registrable Securities disapproves of the terms of any such underwriting, it may elect to withdraw therefrom by written notice to the Company and the underwriter. Any Registrable Securities or other
securities excluded or withdrawn from such underwriting shall be withdrawn from such registration. 
 4.3 Registration on
Form S-3. In addition to the rights provided in Sections 4.1 and 4.2, if at any time (i) the holder or holders of Registrable Securities then outstanding request that the Company file a registration statement on Form S-3 or
any comparable or successor form thereto for a public offering of all or any portion of the shares of Registrable Securities held by such requesting holder or holders, the reasonably anticipated aggregate offering price to the public of which would
exceed $2,500,000, and (ii) the Company is a registrant entitled to use Form S-3 or any comparable or successor form thereto to register such shares, then the Company shall use its best efforts to register under the Securities Act on Form S-3
or any comparable or successor form thereto, for public sale in accordance with the method of disposition specified in such notice, the number of shares of Registrable Securities specified in such notice. Whenever the Company is required by this
Section 4.3 to use its best efforts to effect the registration of Registrable Securities, each of the procedures and requirements of Sections 4.1 and 4.4, including, but not limited to, the requirement that the Company
notify all holders of Registrable Securities from whom notice has not been received and provide them with the opportunity to participate in the offering, shall apply to such registration, provided, however, that there shall be no
limitation on the number of registrations on Form S-3 which may be requested and obtained under this Section 4.3 except that the Company shall not be required to effect more than one (1) registration pursuant to this
Section 4.3 in any twelve (12) month period. 
 4.4 Registration Procedures. If and whenever the Company
is required by the provisions of Section 4.1, 4.2 or 4.3 to use its best efforts to effect the registration of any Registrable Securities under the Securities Act, the Company will, as expeditiously as possible: 

(a) prepare and file with the Commission a registration statement (which, in the case of an underwritten public offering pursuant to
Section 4.1, shall be on Form S-l or other form of general applicability satisfactory to the managing underwriter selected as therein provided) with respect to such securities including executing an undertaking to file post-effective
amendments and use its best efforts to cause such registration statement to become and remain effective for the period of the distribution contemplated thereby; provided, that before filing a registration statement or prospectus, the Company
shall furnish to the counsel selected by the holders of a majority of the Registrable Securities covered by such registration statement copies of all such documents proposed to be filed, which documents shall be subject to reasonable review and
comment of such counsel; 
 (b) prepare and file with the Commission such amendments and supplements to such registration
statement and the prospectus used in connection therewith as may be necessary to keep such registration statement effective for the period specified herein and 

 

 9 

 
comply with the provisions of the Securities Act with respect to the disposition of all Registrable Securities covered by such registration statement in accordance with the sellers’ intended
method of disposition set forth in such registration statement for such period and to correct any statements or omissions if, at the time when a prospectus relating to such securities is required to be delivered under the Securities Act, any event
shall have occurred as the result of which any such prospectus or any other prospectus as then in effect would include an untrue statement of a material fact or omit to state any material fact necessary to make the statements therein, in light of
the circumstances in which they were made, not misleading; provided, that before filing any such amendment or supplement, the Company shall furnish to the counsel selected by the holders of a majority of the Registrable Securities covered by
such amendment or supplement copies of all such documents proposed to be filed, which documents shall be subject to the reasonable review and comment of such counsel; 

(c) prepare and file with the Commission, promptly upon the request of sellers of Registrable Securities, any amendments or supplements
to such registration statement or prospectus which, in the opinion of counsel for such sellers of Registrable Securities, is required under the Securities Act in connection with the distribution of the Registrable Securities by such sellers of
Registrable Securities; provided, however, that the Company shall only be required to file any such amendment or supplement, if counsel for the Company concurs with counsel for the sellers that there is a need for such filing to comply with the
Securities Act; 
 (d) not file any amendment or supplement to such registration statement or prospectus to which a majority in
interest of such participating sellers of Registrable Securities shall have reasonably objected on the grounds that such amendment or supplement does not comply in all material respects with the requirements of the Securities Act, in the opinion of
counsel to such sellers of Registrable Securities, after having been furnished with a copy thereof in accordance with Section 4.4(b) above, unless in the opinion of counsel for the Company the filing of such amendment or supplement is
reasonably necessary to comply with applicable federal or state law; 
 (e) furnish to each seller of Registrable Securities and
to each underwriter such number of copies of the registration statement and each such amendment and supplement thereto (in each case including all exhibits) and the prospectus included therein (including each preliminary prospectus) as such persons
reasonably may request in order to facilitate the public sale or other disposition of the Registrable Securities covered by such registration statement; 

(f) use its best efforts to register or qualify the Registrable Securities covered by such registration statement under the securities or
“blue sky” laws of such jurisdictions as the sellers of Registrable Securities or, in the case of an underwritten public offering, the managing underwriter reasonably shall request, provided, however, that the Company shall
not for any such purpose be required to qualify generally to transact business as a foreign corporation in any jurisdiction where it is not so qualified or to consent to general service of process in any such jurisdiction, unless the Company is
already subject to service in such jurisdiction; 
 (g) use its best efforts to list the Registrable Securities covered by such
registration statement with any securities exchange on which the Common Stock of the Company is then listed and, if not so listed, to be listed on the NASD automated quotation 

 

 10 

 
system and, if listed on the NASD automated quotation system, use its best efforts to secure designation of all such Registrable Securities covered by such registration statements as a NASDAQ
“national market system security” within the meaning of Rule HAa2-l of the Exchange Act or, failing that, to secure NASDAQ authorization for such Registrable Securities; 

(h) comply with all applicable rules and regulations under the Securities Act and Exchange Act; 

(i) provide a transfer agent and registrar for all such Registrable Securities and a CUSIP number for all such Registrable Securities in
each case not later than the effective date of such registration statement. 
 (j) promptly notify each seller of Registrable
Securities and each underwriter under such registration statement, at any time when a prospectus relating thereto is required to be delivered under the Securities Act, of the happening of any event of which the Company has knowledge as a result of
which the prospectus contained in such registration statement, as then in effect, includes an untrue statement of a material fact or omits to state a material fact required to be stated therein or necessary to make the statements therein not
misleading in light of the circumstances then existing, and promptly prepare and furnish to such seller a reasonable number of copies of a prospectus supplemented or amended so that, as thereafter delivered to the purchasers of such Registrable
Securities, such prospectus shall not include an untrue statement of a material fact or omit to state a material fact required to be stated therein or necessary to make the statements therein not misleading in light of the circumstances then
existing; 
 (k) if the offering is underwritten and at the request of any seller of Registrable Securities, use its best
efforts to furnish on the date that Registrable Securities are delivered to the underwriters for sale pursuant to such registration: (i) an opinion dated such date of counsel representing the Company for the purposes of such registration,
addressed to the underwriters to such effects as reasonably may be requested by counsel for the underwriters, and (ii) a letter dated such date from the independent public accountants retained by the Company, addressed to the underwriters
stating that they are independent public accountants within the meaning of the Securities Act and that, in the opinion of such accountants, the financial statements of the Company included in the registration statement or the prospectus, or any
amendment or supplement thereof, comply as to form in all material respects with the applicable accounting requirements of the Securities Act, and such letter shall additionally cover such other financial matters (including information as to the
period ending no more than five (5) business days prior to the date of such letter) with respect to such registration as such underwriters reasonably may request; 

(l) make available for inspection by each seller of Registrable Securities, any underwriter participating in any distribution pursuant to
such registration statement, and any attorney, accountant or other agent retained by such seller or underwriter, reasonable access to all financial and other records, pertinent corporate documents and properties of the Company, as such parties may
reasonably request, and cause the Company’s officers, directors and employees to supply all information reasonably requested by any such seller, underwriter, attorney, accountant or agent in connection with such registration statement;

  

 11 

 (m) cooperate with the selling holders of Registrable Securities and the managing
underwriter, if any, to facilitate the timely preparation and delivery of certificates representing Registrable Securities to be sold, such certificates to be in such denominations and registered in such names as such holders or the managing
underwriter may request at least two (2) business days prior to any sale of Registrable Securities; 
 (n) permit any
holder of Registrable Securities which holder, in the sole and exclusive judgment, exercised in good faith, of such holder, might be deemed to be a controlling person of the Company, to participate in good faith in the preparation of such
registration or comparable statement and to require the insertion therein of material, furnished to the Company in writing, which in the reasonable judgment of such holder and its counsel should be included; 

(o) in the event of the issuance of any stop order suspending the effectiveness of a registration statement, or of any order suspending
or preventing the use of any related prospectus or suspending the qualification of any common stock included in such registration statement for sale in any jurisdiction, the Company shall use its best efforts promptly to obtain the withdrawal of
such order; 
 (p) use its best efforts to cause such Registrable Securities covered by such registration statement to be
registered with or approved by such other governmental agencies or authorities as may be necessary to enable the sellers thereof to consummate the disposition of such Registrable Securities; 

(q) in the event of any underwritten public offering, enter into and perform its obligations under an underwriting agreement, in usual
and customary form, with the managing underwriter of such offering and take all such other actions as the underwriters reasonably request in order to expedite or facilitate the disposition of Registrable Securities; and 

(r) if at the time of a request for registration pursuant to Section 4.1 or Section 4.3, the Board reasonably
determines (upon advice of counsel) that registration of the Registrable Securities would interfere with any material, non-public transaction involving the Company, the Company may defer the filing of the registration statement for a period of up to
90 days after receipt of the request of the holder or holders pursuant to Section 4.1 and Section 4.3; provided, that the Company may not exercise this right more once in any twelve (12) month period;
provided, further, that the Company shall provide prior notice of such determination to the requesting holder or holders. 

For purposes of this Investor Rights Agreement, the period of distribution of Registrable Securities in a firm commitment underwritten
public offering shall be deemed to extend until each underwriter has completed the distribution of all securities purchased by it, and the period of distribution of Registrable Securities in any other registration shall be deemed to extend until the
earlier of the sale of all Registrable Securities covered thereby or one hundred eighty (180) days after the effective date thereof, provided, however, in the case of any registration of Registrable Securities on Form S-3 or a
comparable or successor form which are intended to be offered on a continuous or delayed basis, such one hundred eighty (180) day period shall be extended, if necessary, to keep the registration statement effective until all such Registrable
Securities are sold, provided that Rule 415, or any successor rule under the Securities Act, 
  

 12 

 
permits an offering on a continuous or delayed basis, and provided further that applicable rules under the Securities Act governing the obligation to file a post-effective amendment, permit, in
lieu of filing a post-effective amendment which (y) includes any prospectus required by Section 10(a)(3) of the Securities Act or (z) reflects facts or events representing a material or fundamental change in the information set
forth in the registration statement, the incorporation by reference of information required to be included in (y) and (z) above contained in periodic reports filed pursuant to Section 13 or 15(d) of the Exchange Act in
the registration statement. 
 In connection with each registration hereunder, the sellers of Registrable Securities will
furnish to the Company, in writing, such information requested by the Company with respect to themselves and the proposed distribution by them as shall be reasonably necessary in order to assure compliance with Federal and applicable state
securities laws. 
 4.5 Expenses. 

(a) All expenses other than Selling Expenses incurred by the Company in complying with Sections 4.1, 4.2 and 4.3, are
called “Registration Expenses” and shall include, without limitation, (i) all registration and filing fees, printing expenses, fees and disbursements of counsel and independent public accountants for the Company, fees and
expenses (including counsel fees) incurred in connection with complying with state securities or “blue sky” laws, fees of the National Association of Securities Dealers, Inc., transfer taxes, fees of transfer agents and registrars, costs
of any insurance which might be obtained by the Company with respect to the offering by the Company, and (ii) reasonable attorneys’ fees and disbursements of one counsel for the holders of Registrable Securities in an amount not to exceed
an aggregate of $35,000, such counsel to be selected by the holders of at least a majority of the Registrable Securities being sold. All underwriting discounts, selling commissions and other expenses not referred to above applicable to the sale of
Registrable Securities are called “Selling Expenses.” 
 (b) The Company shall pay all Registration Expenses in
connection with each registration statement under Section 4.1, 4.2 and 4.3; provided that, in the event of a registration pursuant to Section 4.1 hereof which is withdrawn at the request of the Investors other
than (i) as a result of the Company’s failure to perform its obligations hereunder, (ii) as a result of a cutback by the underwriter of such registration in the amount of Registrable Securities which may be included in such
registration by more than thirty percent (30%), (iii) as a result of information concerning a materially adverse change in the Company’s business or financial condition that is made known to the Investors after the date on which such
registration was requested, (iv) as a result of any general banking moratorium, or (v) as a result of (A) any outbreak or escalation of national or international hostilities or any crisis or calamity, (B) any change in U.S. or
international financial markets, or (C) any substantial change in U.S. or international political, financial or economic conditions, in each case, which is material and adverse and makes it impracticable to market such Registrable Securities,
the Investors shall pay the Registration Expenses with respect to such registration, in which case, the Investors will be deemed not to have used their one demand registration right under Section 4.1. All Selling Expenses in connection
with each registration statement under Section 4.1, 4.2 or 4.3 shall be borne by the participating sellers in proportion to the number of shares registered by each, or by such participating sellers other than the Company
(except to the extent the Company shall be a seller) as they may agree. 
  

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 4.6 Indemnification and Contribution. 

(a) In the event of a registration of any of the Registrable Securities under the Securities Act pursuant to Section 4.1,
4.2 or 4.3, the Company (i) will indemnify and hold harmless each holder of Registrable Securities, its officers, directors and partners, each underwriter of such Registrable Securities thereunder and each other person, if any,
who controls such holder or underwriter within the meaning of the Securities Act (each, an “Indemnitee”), against any losses, claims, damages or liabilities, joint or several, to which such Indemnitee may become subject under the
Securities Act or otherwise, insofar as such losses, claims, damages or liabilities (or actions in respect thereof) arise out of or are based upon (a) any untrue statement or alleged untrue statement of any material fact contained in any
prospectus, offering circular or other document incident to such registration (including any related notification, registration statement under which such Registrable Securities were registered under the Securities Act pursuant to
Section 4.1, 4.2 or 4.3, any preliminary prospectus or final prospectus contained therein, or any amendment or supplement thereof), (b) any blue sky application or other document executed by the Company specifically
for that purpose or based upon written information furnished by the Company filed in any state or other jurisdiction in order to qualify any or all of the Registrable Securities under the securities laws thereof (any such application, document or
information herein called a “Blue Sky Application”), (c) any omission or alleged omission to state in any such registration statement, prospectus, amendment or supplement or in any Blue Sky Applications executed or filed by the
Company, a material fact required to be stated therein or necessary to make the statements therein not misleading, (d) any violation by the Company or its agents of the Securities Act or any rule or regulation promulgated under the Securities
Act applicable to the Company or its agents and relating to action or inaction required of the Company in connection with such registration, or (e) any failure to register or qualify the Registrable Securities in any state where the Company or
its agents has affirmatively undertaken or agreed in writing that the Company (the undertaking of any underwriter chosen by the Company being attributed to the Company) will undertake such registration or qualification and (ii) will reimburse
each Indemnitee for any legal or other expenses reasonably incurred by them in connection with investigating or defending any such loss, claim, damage, liability or action, promptly after being so incurred, provided, however, that the
Company will not be liable to an Indemnitee if, and to the extent that, any such loss, claim, damage or liability (A) arises out of or is based upon an untrue statement or alleged untrue statement or omission or alleged omission so made in
conformity with written information furnished by such Indemnitee, in writing specifically stated to be for use in such registration statement or prospectus or (B) in a case where a copy of a prospectus (as amended or supplemented) is required
to be delivered by such Indemnitee, and not by any underwriter, is based upon an untrue statement or alleged untrue statement or omission or alleged omission made in a preliminary prospectus and a copy of the prospectus (as amended or supplemented)
was not sent or given by or on behalf of such Indemnitee to the person asserting any such loss, claim, liability or damage (if required by law so to have been delivered) at or prior to the written confirmation of the sale of the Registrable
Securities as required by the Securities Act and the prospectus (as so amended or supplemented) would have corrected such untrue statement or omission or alleged untrue statement or omission; provided that in clause (B), the Company had promptly
notified Indemnitee of such untrue statement or alleged untrue statement or omission or alleged omission in compliance with Section 4.4(h) herein. 
  

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 (b) In the event of a registration of any of the Registrable Securities under the Securities
Act pursuant to Section 4.1, 4.2 or 4.3, each seller of such Registrable Securities thereunder, severally and not jointly, (i) will indemnify and hold harmless the Company, each person, if any, who controls the Company
within the meaning of the Securities Act, each officer of the Company who signs the registration statement, each director of the Company, each other seller of Registrable Securities, each underwriter and each person who controls any underwriter
within the meaning of the Securities Act, against all losses, claims, damages or liabilities, joint or several, to which the Company or such officer, director, other seller, underwriter or controlling person may become subject under the Securities
Act or otherwise, insofar as such losses, claims, damages or liabilities (or actions in respect thereof) arise out of or are based upon any untrue statement or alleged untrue statement of any material fact contained in any prospectus offering
circular or other document incident to such registration (including any related notification, registration statement under which such Registrable Securities were registered under the Securities Act pursuant to Section 4.1, 4.2 or
4.3, any preliminary prospectus or final prospectus contained therein, or any amendment or supplement thereof), or any Blue Sky Application or arise out of, or are based upon, the omission or alleged omission to state therein a material fact
required to be stated therein or necessary to make the statements therein not misleading, and (ii) will reimburse the Company and each such officer, director, other seller, underwriter and controlling person for any legal or other expenses
reasonably incurred by them in connection with investigating or defending any such loss, claim, damage, liability or action, promptly after being so incurred, provided, however, that such seller will be liable hereunder in any such
case if and only to the extent that any such loss, claim, damage or liability arises out of or is based upon an untrue statement or alleged untrue statement or omission or alleged omission made in reliance upon and in conformity with information
pertaining to such seller, as such, furnished in writing to the Company by such seller specifically for use in such registration statement or prospectus; and provided further, however, that the liability of each seller hereunder shall
be limited to the proportion of any such loss, claim, damage, liability or expense which is equal to the proportion that the public offering price of the securities sold by such seller under such registration statement bears to the total public
offering price of all securities sold thereunder, but not in any event to exceed the proceeds received by such seller from the sale of Registrable Securities covered by such registration statement. Not in limitation of the foregoing, it is
understood and agreed that the indemnification obligations of any seller hereunder pursuant to any underwriting agreement entered into in connection herewith shall be limited to the obligations contained in this Section 4.6(b).

 (c) Promptly after receipt by an indemnified party hereunder of notice of the commencement of any action, such indemnified
party shall, if a claim in respect thereof is to be made against the indemnifying party hereunder, notify the indemnifying party in writing thereof, but the omission so to notify the indemnifying party shall not relieve it from any liability which
it may have to such indemnified party other than under this Section 4.6 and shall only relieve it from any liability which it may have to such indemnified party under this Section 4.6 if, and to the extent, the indemnifying
party is prejudiced by such omission. In case any such action shall be brought against any indemnified party and it shall notify the indemnifying party of the commencement thereof, the indemnifying party shall be entitled to participate in and, to
the extent it shall wish, to assume and undertake the defense thereof with counsel satisfactory to such indemnified party, and, after notice from the indemnifying party to such indemnified party of its election so to assume and undertake the defense
thereof, the indemnifying party shall not 
  

 15 

 
be liable to such indemnified party under this Section 4.6 for any legal expenses subsequently incurred by such indemnified party in connection with the defense thereof other than
reasonable costs of investigation and of liaison with counsel so selected, provided, however, that, if the defendants in any such action include both the indemnified party and the indemnifying party and the indemnified party shall have
reasonably concluded that there may be reasonable defenses available to it which are different from or additional to those available to the indemnifying party or that the interests of the indemnified party reasonably may be deemed to conflict with
the interests of the indemnifying party, the indemnified party shall have the right to select a separate counsel and to assume such legal defenses and otherwise to participate in the defense of such action, with the expenses and fees of such
separate counsel and other expenses related to such participation to be reimbursed by the indemnifying party as incurred. No indemnifying party, in the defense of any such claim or action, shall, except with the consent of each indemnified party,
consent to entry of any judgment or enter into any settlement which does not include as an unconditional term thereof the giving by the claimant or plaintiff to such indemnified party of a release from all liability in respect to such claim or
action. Each indemnified party shall furnish such information regarding itself or the claim in question as an indemnifying party may reasonably request in writing and as shall be reasonably required in connection with defense of such claim and
litigation resulting therefrom. 
 (d) In order to provide for just and equitable contribution to joint liability under the
Securities Act in any case in which either (i) any holder of Registrable Securities exercising rights under this Investor Rights Agreement, or any controlling person of any such holder, makes a claim for indemnification pursuant to this
Section 4.6 but it is judicially determined (by the entry of a final judgment or decree by a court of competent jurisdiction and the expiration of time to appeal or the denial of the last right of appeal) that such indemnification may
not be enforced in such case notwithstanding the fact that this Section 4.6 provides for indemnification in such case, or (ii) contribution under the Securities Act may be required on the part of any such selling holder or any such
controlling person in circumstances for which indemnification is provided under this Section 4.6; then, and in each such case, the Company and such holder will contribute to the aggregate losses, claims, damages or liabilities to which
they may be subject (after contribution from others) in such proportion so that such holder is responsible for the portion represented by the percentage that the public offering price of its Registrable Securities offered by the registration
statement bears to the public offering price of all securities offered by such registration statement, and the Company is responsible for the remaining portion, provided, however, that, in any such case, (A) no such holder of
Registrable Securities will be required to contribute any amount in excess of the proceeds received from the sale of all such Registrable Securities offered by it pursuant to such registration statement; and (B) no Person guilty of fraudulent
misrepresentation (within the meaning of Section 11(f) of the Securities Act) will be entitled to contribution from any Person or entity who was not guilty of such fraudulent misrepresentation. 

(e) The indemnities and obligations provided in this Section 4.6 shall survive the transfer of any Registrable Securities by
such holder. 
 4.7 Changes in Common Stock. If, and as often as, there is any change in the Common Stock by way of a
stock split, stock dividend, combination or reclassification, or through a merger, consolidation, reorganization or recapitalization, or by any other means, 

 

 16 

 
appropriate adjustment shall be made in the provisions hereof so that the rights and privileges granted hereby shall continue with respect to the Common Stock as so changed. 

4.8 Rule 144 and 144A Reporting. With a view to making available the benefits of certain rules and regulations of the Commission
which may at any time permit the sale of the Registrable Securities to the public without registration, except as provided in paragraph (c) below, at all times after any registration statement covering a public offering of securities of the
Company under the Securities Act shall have become effective, the Company agrees to: 
 (a) use its best efforts to comply with
all of the reporting requirements of the Securities Act and the Exchange Act (whether or not it shall be required to do so) and shall comply with all other public information reporting requirements of the Commission as a condition to the
availability of an exemption from the Securities Act for the sale of any of the Registrable Securities by any holder of Registrable Securities (including any such exemption pursuant to Rule 144 or Rule 144A thereof, as amended form time to time, or
any successor rule thereto or otherwise); 
 (b) cooperate with each holder of Registrable Securities in supplying such
information as may be necessary for such holder of Registrable Securities to complete and file any information reporting forms presently or hereafter required by the Commission as a condition to the availability of an exemption from the Securities
Act (under Rule 144 or Rule 144A thereunder or otherwise) for the sale of any of the Registrable Securities by any holder of Registrable Securities; and 

(c) furnish to each holder of Registrable Securities forthwith upon request a written statement by the Company as to its compliance with
the reporting requirements of such Rule 144 or Rule 144A (or any successor rule) and, at any time after it has become subject to such reporting requirements, of the Securities Act and the Exchange Act, a copy of the most recent annual or quarterly
report of the Company, and such other reports and documents so filed by the Company as such holder may reasonably request in availing itself of any rule or regulation of the Commission allowing such holder to sell any Registrable Securities without
registration. 
 4.9 “Market Stand-Off’ Agreement. Each of the Investors agrees, severally and not jointly,
that, if requested by the Company and an underwriter of Common Stock (or other securities) of the Company, not to sell or otherwise transfer or dispose of any Common Stock (or other securities) of the Company held by such Investor during a period
not to exceed one hundred and eighty (180) days (which period may be extended upon the request of the managing underwriter, in order to permit its compliance with applicable NASD rules in the event the Company issues or proposes to issue an
earnings or other public release within seventeen (17) days of the expiration of the 180-day lockup period) (the “Market Stand-Off Period”) following the effective date of the first registration statement of the Company filed
under the Securities Act and to enter into an agreement to such effect regardless of whether such Investor is participating in the offering to which the registration statement relates, provided that all directors, officers, and holders of greater
than one percent (1%) of the Common Stock (calculated on an as-converted basis, and assuming the exercise of all rights, options and warrants and conversion of all convertible securities) agree to enter into similar agreements containing terms
no more favorable to such other holders of Common Stock. Notwithstanding the foregoing, the Market Stand-Off 
  

 17 

 
Period shall be extended beyond the 180-day period referenced above for an additional one hundred and eighty-five (185) days (which period may be extended upon the request of the managing
underwriter, in order to permit its compliance with applicable NASD rules in the event the Company issues or proposes to issue an earnings or other public release within seventeen (17) days of the expiration of the 180-day lockup period) in the
event that the Company consummates a Qualified Public Offering on or before June 30, 2008, provided that all directors, officers, and holders of greater than one percent (1%) of the Common Stock (calculated on an as-converted basis, and
assuming the exercise of all rights, options and warrants and conversion of all convertible securities) are subject to the same extension. The Company may impose stop-transfer instructions with respect to the shares (or securities) subject to the
foregoing restriction until the end of said period. 
 4.10 Assignment of Registration Rights. The rights to cause the
Company to register Registrable Securities pursuant to this Section 4 may only be assigned to (a) an Affiliate of an Investor or another Investor, or (b) any other transferee who acquires at least thirty-five percent
(35%) of such transferring Investor’s shares of Preferred Stock, and in each case, only if such Affiliate or transferee agrees in writing to be bound to the terms, agreements and restrictions contained in this Investor Rights Agreement. In
no event shall such rights be assigned to a competitor of the Company as determined by the Board in its sole discretion. 
 4.11
Grant of Subsequent Registration Rights. Subsequent to the execution of this Agreement, the Company shall not grant registration rights to any holder of Common Stock (calculated on an as-converted basis, and assuming the exercise of all
rights, options and warrants and conversion of all convertible securities) of the Company unless such registration rights are subordinate to the registration rights of the Investors. 

4.12 Termination. The respective rights and obligations of the parties under this Section 4 shall terminate upon the
earlier of (i) the fifth (5th) anniversary of the closing of the Company’s Qualified Public Offering and (ii) with respect to any holder of not more than one hundred thousand (100,000) Registrable Securities, when such
holder can sell all of such holder’s shares under Rule 144 promulgated under the Securities Act without regard to time or volume limitations. 

5. AFFIRMATIVE COVENANTS OF THE COMPANY 

The Company covenants and agrees that, from the date of the Closing under the Purchase Agreement and thereafter so long as any Investor
owns at least twenty-five percent (25%) of the outstanding shares of Preferred Stock initially purchased by such Investor pursuant to the Purchase Agreement (each, a “Rights Holder”), it will perform and observe the following
covenants and provisions. 
 5.1 Corporate Existence. The Company will maintain and will cause any Subsidiary or future
Subsidiary to maintain its corporate existence in good standing and comply with all applicable laws and regulations of the United States, of any state or states thereof, any political subdivision thereof and of any governmental authority. In the
event that the Company hereafter acquires one or more Subsidiaries, the covenants of the Company set forth in this Section 5 will apply where relevant to such Subsidiary(ies) as well as to the Company. 

 

 18 

 5.2 Compliance with Laws. The Company shall comply with all applicable laws, rules,
regulations and orders, noncompliance with which could materially adversely affect its respective business or condition, financial or otherwise 

5.3 Financial Statements; Other Reports. The Company shall maintain proper books of account and records in accordance with
generally accepted accounting principles applied on a consistent basis, and shall deliver: 
 (a) to each Rights Holder who so
requests, as soon as available and in any event within thirty (30) days after the end of each month, an unaudited consolidated balance sheet of the Company and its Subsidiaries, if any, as of the end of such month and the related unaudited
statements of income and stockholders’ equity and of cash flows of the Company for the period commencing at the end of the previous fiscal year and ending with the end of such month, setting forth in each case in comparative form the
corresponding figures for the corresponding period of the preceding fiscal year and the budget for such current year, all in reasonable detail and prepared in accordance with generally accepted accounting principles consistently applied, and duly
certified (subject to year-end audit adjustments) by the chief financial officer of the Company; 
 (b) to each Rights Holder,
as soon as available and in any event within forty-five (45) days after the end of each of the first three quarters of each fiscal year of the Company, an unaudited consolidated balance sheet of the Company and its Subsidiaries, if any, as of
the end of such quarter and the related unaudited statements of income and stockholders’ equity and of cash flows of the Company for the period commencing at the end of the previous fiscal year and ending with the end of such quarter, setting
forth in each case in comparative form the corresponding figures for the corresponding period of the preceding fiscal year and the budget for such current year, all in reasonable detail and prepared in accordance with generally accepted accounting
principles consistently applied, and duly certified (subject to year-end audit adjustments) by the chief financial officer of the Company; 

(c) to each Rights Holder, as soon as available and in any event within ninety (90) days after the end of each fiscal year of the
Company, a copy of the audited financial statements for such year for the Company, prepared in accordance with generally accepted accounting principles, including therein a consolidated balance sheet of the Company and its Subsidiaries, if any, as
of the end of such fiscal year and statements of income and stockholders’ equity and of cash flows of the Company for such fiscal year, setting forth in each case in comparative form the corresponding figures for the preceding fiscal year, all
duly certified by independent public accountants selected and approved by the Board or the Audit Committee; 
 (d) to each
Rights Holder, promptly upon receipt thereof, any written report submitted to the Company by independent public accountants in connection with an annual or interim audit of the books of the Company made by such accountants; 

(e) to each Rights Holder, promptly after receipt thereof, notice of all material actions, suits and proceedings before any court or
governmental department, commission, board, bureau, agency or instrumentality, domestic or foreign, affecting the Company or any litigation-related letters, notices and filings; 

 

 19 

 (f) to each Rights Holder, at least forty-five (45) days prior to the beginning of each
fiscal year of the Company, an annual operating plan with monthly and quarterly breakdowns (the “Budget”) for each fiscal year, as soon as it is made available to the Board; and 

(g) to each Rights Holder, within seven (7) working days after coming to the attention of the Company or one of its officers, notice
of bona fide investment opportunities from outside investors as well as bona fide merger or sale opportunities. 
 Neither the
foregoing provisions of this Section nor any other provision of this Investor Rights Agreement shall be in limitation of any rights which an Investor may have with respect to the books and records of the Company, or to inspect their properties or
discuss their affairs, finances and accounts, under the laws of the jurisdictions in which they are incorporated. 
 5.4
Inspection and Other Information. Each Investor and such agents, advisors and counsel as such Investor may designate, may, at its expense, visit and inspect any of the properties of the Company, examine the books of account of the Company,
take extracts therefrom and discuss the affairs, finances and accounts of the Company with its officers and employees and public accountants (and by this provision the Company hereby authorizes said accountants to discuss with such Investors its
finances and accounts), at reasonable times and with reasonable prior notice during normal business hours, and shall have the right to organize monthly calls with management of the Company. All such visits and inspections shall be conducted in a
manner which will not unreasonably interfere with the normal business operations of the Company. The Company shall furnish to each such Investor such other information as it from time to time may reasonably request. 

5.5 Meetings and Committees of the Board of Directors. 

(a) The Company agrees that it shall cause meetings of the Board to be called not less often than once every two months until such time as
the Board determines that bi-monthly meetings are no longer required, and thereafter not less often than once each calendar quarter. The Company shall give notice of each such meeting to the Series A/B Directors (as defined in the Amended and
Restated Certificate of Incorporation) and shall provide copies of minutes of all actions taken by the Board of Directors, whether at meetings or by written consent, to the Investors. 

(b) The Board shall establish such committees as may be necessary and appropriate for conduct of its affairs, which shall include at
least a Compensation Committee and an Audit Committee. 
 (i) The Compensation Committee shall be responsible for making
recommendations on management compensation, the adoption of employee benefit plans, stock option or equity incentive plans and other similar matters. The Compensation Committee shall consist of one or more of the Series A/B Directors, one
independent director, and the Chief Executive Officer in an ex-officio, non-voting capacity. Decisions of the Compensation Committee shall be determined by a majority vote of the committee. 

(ii) The Audit Committee shall consist of at least two directors, which shall not include the Chief Executive Officer of the Company or
any director currently employed 
  

 20 

 
or retained by the Company. The Audit Committee shall be responsible for recommending an independent public accounting firm to act as auditors for the Company, meeting with the auditors to
establish the terms of their engagement and scope of the audit, and meeting with the auditors to review the results of the audit and any management letter the auditors determine necessary. The Audit Committee shall also evaluate the adequacy of the
Company’s internal accounting controls and personnel and performing such other function as are appropriate for an audit committee. 

(iii) The Company will reimburse directors and any authorized board observers for reasonable and necessary direct out-of-pocket expenses
(including travel expenses) related to attendance at Board meetings and committees of the Board in accordance with the Company’s policies and procedures in effect from time to time. 

5.6 Directors’ and Officers’ Liability Insurance; Key-Man Life Insurance. 

The Company shall maintain a directors’ and officers’ liability insurance policy on the directors and officers of the Company
with a financially sound and reputable insurance company or association in the amount of at least $3,000,000 in the aggregate. The Company has a key-man life insurance policy on the life of Gerald Wisler in the amount of $2,000,000; however,
promptly following the Closing, the Company shall increase the amount of such policy to $3,000,000. 
 5.7
Confidentiality, Non-Competition and Invention Assignment Agreements. The Company shall obtain Confidentiality, Non-Competition and Invention Assignment Agreements, in the substantially the form attached as Exhibit B to the Purchase
Agreement, from all employees of and consultants to the Company whether now or hereafter employed or engaged. 
 5.8 Use of
Proceeds. The Company shall use the proceeds from the sale of the Shares (the “Proceeds”) for general corporate and working capital purposes. 

5.9 Adverse Change. The Company will promptly advise the Investors of any event which represents a material adverse change in the
condition or business, financial or otherwise, of the Company or any subsidiary. 
 5.10 Stock Plan. With respect to all
equity issuances granted under any stock plan the Company may now have in effect or may hereafter adopt, the Company agrees that, unless a majority of the Series A/B Directors has consented otherwise: 

(a) The equity issuances shall vest according to the following 4 year schedule: twenty-five percent (25%) of such equity issuance
grant shall vest upon the first yearly anniversary of the grant date, and the remaining unvested portion of such equity issuance shall vest in equal quarterly installments thereafter; 

(b) The grant of the equity issuances shall be made at the sole and complete discretion of the Board or the Compensation Committee;

 (c) The equity issuances may not contain acceleration provisions; 

 

 21 

 (d) The holders of securities issued pursuant to any such stock plan that, as a result of
such issuance, would own one percent (1%) or more of the Common Stock then outstanding (calculated on an as-converted basis, and assuming the exercise of all rights, options and warrants and conversion of all convertible securities) shall be
subject to (i) the Rights of First Refusal in respect of such securities as set forth in Section 1 of the Amended and Restated Stockholders’ Agreement of even date herewith entered into by the Company, the Investors and certain
other stockholders (the “Stockholders’ Agreement”), or (ii) a similar contractual right of first refusal in favor of the Company; and 

(e) The holders of securities issued pursuant to any such stock plan that, as a result of such issuance or sale, would own one percent
(1%) or more of the Common Stock then outstanding (calculated on an as-converted basis, and assuming the exercise of all rights, options and warrants and conversion of all convertible securities) shall be subject to (i) the Drag Along
Rights in respect of such securities as set forth in Section 3 of the Stockholders’ Agreement, or (ii) a similar contractual Drag Along Right in favor of the Company. 

5.11 Termination of Affirmative Covenants. The covenants set forth in this Section 5 shall be of no further force or
effect upon the closing of the Company’s Qualified Public Offering. 
 6. MISCELLANEOUS 

6.1 Notices. All notices, requests, consents and other communications hereunder shall be in writing, shall be addressed to the
receiving party’s address set forth below or to such other address as a party may designate by notice hereunder, and shall be delivered (i) by hand, (ii) by telecopy or facsimile transmission, (iii) by a nationally recognized (or
substantially equivalent international) overnight courier, or (iv) by registered or certified mail, return receipt requested, postage prepaid. 
  

	 If to the Company: 
	Aegerion Pharmaceuticals, Inc. 

 CenterPointe IV

 1140 Route 22 East, Suite 304 

Bridgewater, NJ 08807 

Attn: Gerald Wisler, 

President and Chief Executive Officer 

(908) 704-1300 (Telephone) 

(908) 541-1155 (Fax) 
  

	 With a copy to: 
	Goodwin Procter LLP 

 53 State Street 

Boston, MA 02109 

Attn: Michael H. Bison, Esq. 

(617) 570-1933 (Telephone) (617)523-1231(Fax) 
  

	 If to the Investors: 
	To the addresses set forth on Schedule I. 

  

 22 

	 With a copy to: 
	Mintz, Levin, Cohn, Ferris, Glovsky and Popeo, P.C. 

 One
Financial Center 
 Boston, MA 02111 

Attn: Lewis Geffen, Esq. 

(617) 348-1834 (Telephone) 

(617) 542-2241 (Fax) 

All notices, requests, consents and other communications hereunder shall be deemed to have been given either (i) if by hand, at the
time of the delivery thereof to the receiving party at the address of such party set forth above, (ii) if made by telecopy or facsimile transmission, at the time that receipt thereof has been acknowledged by electronic confirmation or
otherwise, (iii) if sent by overnight courier, on the next business day following the day such notice is delivered to the courier service, or (iv) if sent by registered or certified mail, on the fifth business day following the day such
mailing is made. 
 6.2 Additional Investors. Notwithstanding anything to the contrary contained herein, if the Company
issues additional shares of the Company’s Preferred Stock after the date hereof, any purchaser of such shares of Preferred Stock shall (a) become a party to this Agreement by executing and delivering an additional counterpart signature
page to this Agreement and thereafter shall be deemed an Investor for all purposes hereunder and (b) become a party to a stock purchase agreement with respect to the purchase of such shares of Preferred Stock that is substantially similar to
the Purchase Agreement. 
 6.3 Entire Agreement. This Investor Rights Agreement embodies the entire agreement and
understanding between the parties hereto with respect to the subject matter hereof and supersedes all prior oral or written agreements and understandings relating to the subject matter hereof. No statement, representation, warranty, covenant or
agreement of any kind not expressly set forth in this Investor Rights Agreement shall affect, or be used to interpret, change or restrict, the express terms and provisions of this Investor Rights Agreement. 

6.4 Modifications and Amendments. This Investor Rights Agreement may not be amended or modified, and no provision hereof may be
waived, without the written consent of the Company and the Required Series A/B Majority. Any waiver or consent hereunder shall be effective only in the specific instance and for the purpose for which it was given, and shall not constitute a
continuing waiver or consent. 
 6.5 Benefit. All statements, representations, warranties, covenants and Investor Rights
Agreements in this Investor Rights Agreement shall be binding on the parties hereto and shall inure to the benefit of the respective successors and permitted assigns of each party hereto. 

6.6 Governing Law. This Investor Rights Agreement and the rights and obligations of the parties hereunder shall be construed in
accordance with and governed by the General Corporation Law of the State of Delaware as to matters within the scope thereof, and as to all other matters shall be construed in accordance with and governed by the internal laws of the State of New
Jersey, without giving effect to the conflict of law principles thereof. 
  

 23 

 6.7 Jurisdiction and Service of Process. Any legal action or proceeding with respect
to this Agreement may be brought in the federal courts of the United States of America for the Southern District of New York. By execution and delivery of this Agreement, each of the parties hereto accepts for itself and in respect of its property,
generally and unconditionally, the jurisdiction of the aforesaid courts. Each of the parties hereto irrevocably consents to the service of process of any of the aforementioned courts in any such action or proceeding by the mailing of copies thereof
by certified mail, postage prepaid, to the party at its address set forth in Section 6.1 hereof. 
 6.8 Jury
Waiver. Each of the parties hereto irrevocably waives all right to trial by jury in any action, proceeding or counterclaim arising out of or relating to this agreement or any other document. 

6.9 Severability. In the event that it is determined that any provision, or any portion thereof, contained in this Investor Rights
Agreement shall be unenforceable in any respect, then such provision shall be deemed limited to the extent that it shall be deemed enforceable and as so limited, shall remain in full force and effect. In the event that any such provision, or portion
thereof, is deemed wholly unenforceable, the remaining provisions of this Investor Rights Agreement, nevertheless, shall remain in full force and effect. 

6.10 Headings and Captions. The headings and captions of the various subdivisions of this Investor Rights Agreement are for
convenience of reference only and shall in no way modify or affect the meaning or construction of any of the terms or provisions hereof. 

6.11 Enforcement. Each of the parties hereto acknowledges and agrees that the rights acquired by each party hereunder are unique
and that irreparable damage would occur in the event that any of the provisions of this Investor Rights Agreement to be performed by the other parties were not performed in accordance with their specific terms or were otherwise breached.
Accordingly, in addition to any other remedy to which the parties hereto are entitled at law or in equity, each party hereto shall be entitled to an injunction or injunctions to prevent breaches of this Investor Rights Agreement by any other party.

 6.12 No Waiver of Rights, Powers and Remedies. No failure or delay by a party hereto in exercising any right, power or
remedy under this Investor Rights Agreement, and no course of dealing among the parties hereto, shall operate as a waiver of any such right, power or remedy of the party. No single or partial exercise of any right, power or remedy under this
Investor Rights Agreement by a party hereto, nor any abandonment or discontinuance of steps to enforce any such right, power or remedy, shall preclude such party from any other or further exercise thereof or the exercise of any other right, power or
remedy hereunder. The election of any remedy by a party hereto shall not constitute a waiver of the right of such party to pursue other available remedies. No notice to or demand on a party not expressly required under this Investor Rights Agreement
shall entitle the party receiving such notice or demand to any other or further notice or demand in similar or other circumstances or constitute a waiver of the rights of the party giving such notice or demand to any other or further action in any
circumstances without such notice or demand. 
  

 24 

 6.13 Counterparts. This Investor Rights Agreement may be executed in two or more
counterparts, and by different parties hereto on separate counterparts, each of which shall be deemed an original, but all of which together shall constitute one and the same instrument. This Investor Rights Agreement may be executed by facsimile
signatures. 
 6.14 Termination of Conflicting Rights. To the extent any of the Investors are party to a restricted stock
agreement that contains provisions that conflict with the provisions herein, the provisions herein shall supersede such original provisions and each such Investor agrees that such original provisions are hereby terminated; provided,
however, that all of the other provisions set forth in the restricted stock agreements shall remain in full force and effect. 

6.15 Assignment. Subject to Section 4.10, each Investor may assign its rights hereunder to any purchaser or transferee
of the Registrable Securities; provided, however, that such purchaser or transferee shall, as a condition to the effectiveness of such assignment, be required to execute a counterpart to the Agreement agreeing to be treated as an Investor whereupon
such purchaser or transferee shall have the benefits of, and be subject to the restrictions contained in this Agreement as if such purchaser or transferee was originally included in the definition of an Investor herein and had originally been a
party hereto. 
  

 25 

 IN WITNESS WHEREOF, the parties hereto have executed this Amended and Restated Investor
Rights Agreement or caused this Amended and Restated Investor Rights Agreement to be executed by their duly authorized representatives as of the date first written above. 

AEGERION PHARMACEUTICALS, INC. 

By:   /s/     Gerald
Wisler                              

        Gerald Wisler 

        Chief Executive Officer and President 

 
 (Counterpart Signature Pages Begin on Next Page) 

 
  

Signature Page to Amended and Restated Investor Rights Agreement 

 
  

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery
and the Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

INDEX VENTURES III (DELAWARE) L.P. 

/s/  D.A.
Hall                                         
     
 Signature of Investor 

Director 
 Index Venture Associates III Limited

 as General Partner of Index Ventures III 

(Delaware) L.P. 

By: D.A. Hall, Director 

Title: _______________________________ 

Address: No 1 Seaton Place 

                St. Helier 

                Jersey, JE484J

 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/ Gerald
Wisler                             

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

INDEX VENTURES III (JERSEY) L.P. 

/s/  D.A.
Hall                                         
        
 Signature of Investor 

Director 
 Index Venture Associates III Limited

 as General Partner of Index Ventures III 

(Jersey) L.P. 

By: D.A. Hall, Director 

Title: _______________________________ 

Address: No 1 Seaton Place 

                St. Helier 

                Jersey, JE484J

 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

INDEX VENTURES III PARALLEL ENTREPRENEUR FUND (JERSEY) L.P. 

/s/  D.A.
Hall                                         
        
 Signature of Investor 

Director 
 Index Venture Associates III Limited

 as General Partner of Index Ventures III 

Parallel Entrepreneur Fund (Jersey) L.P. 

By: D.A. Hall, Director 

Title: _______________________________ 

Address: No 1 Seaton Place 

                St. Helier 

                Jersey, JE484J

 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

YUCCA PARTNERS LP JERSEY BRANCH 

/s/  Peter LE
Breton                                        
     
 By: Ogler Employee Benefit Services Limited 

as Authorized Signatory of Yucca Partners LP 

Jersey Branch in its Capacity of Administrator of 

The Index Co-Investment Scheme 

By: Peter LE Breton 

Title: Authorized Signatory 

Address: Whitley Chambers, 

                Day Street, St. Helier,
Jersey C.I. 

                JE49WG 

Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                        
 
         Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

Advent Healthcare and Life Sciences III GP Limited Partnership  

Printed Name of Investors 

/s/  Jason
Fisherman                             

Signature of Investor 

Advent Healthcare and Life Sciences III GP Limited Partnership 

By: AHLS III GP Limited Partnership, General Partner 

By: Advent International LLC, General Partner 

By: Advent International Corporation, Manager 

By:  /s/  Jason
Fisherman                                        
                      

Vice President/Senior Vice
President                                        
 
 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerlad
Wisler                             

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

Advent Healthcare and Life Sciences III-A Limited Partnership  

Printed Name of Investors 

/s/  Jason
Fisherman                                        
     
 Signature of Investor 

Advent Healthcare and Life Sciences III-A Limited Partnership 

By: AHLS III GP Limited Partnership, General Partner 

By: Advent International L.L.C, General Partner 

By: Advent International Corporation, Manager 

By:  /s/  Jason
Fisherman                                        
             
 Vice President/Senior Vice
President                                 

Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                        
 
         Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

Advent Partners HLS III Limited Partnership 

Printed Name of Investors 

/s/  Jason
Fisherman                                     

Signature of Investor 

Advent Partners HLS III Limited Partnership 

By: Advent International Corporation, General Partner 

By:  /s/  Jason
Fisherman                                        
             
 Vice President/Senior Vice
President                                 

Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

Alta BioPharma Partners III, L.P. 
 By:
Alta BioPharma Management III, LLC 
 /s/  Hilary
Strain                                        
 
 Signature of Investor 

By: Hilary Strain 

Title: V.P. of Finance & Admin. 

Address: One Embarcadero Center, Suite 3700 

                San Francisco, CA 94111

 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

Alta BioPharma Partners III GmbH & Co. Beteiligungs KG 

By: Alta BioPharma Management III, LLC 

/s/  Hilary
Strain                                        
 
 Signature of Investor 

By: Hilary Strain 

Title: V.P. of Finance & Admin. 

Address: One Embarcadero Center, Suite 3700 

                San Francisco, CA 94111

 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

Alta Embarcadero BioPharma Partners III, LLC 

/s/  Hilary
Strain                                        
 
 Signature of Investor 

By: Hilary Strain 

Title: V.P. of Finance & Admin. 

Address: One Embarcadero Center, Suite 3700 

                San Francisco, CA 94111

 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement””) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and
delivery and the Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

MC LIFE SCIENCE VENTURES, INC. 

/s/  Tsunehiko
Yanagihara                              

Signature of Investor 

By: Tsunehiko Yanagihara 

Title: President 

Address: c/o Mitsubishi International Corporation 

                Life Science Business
Dept. 
                 655
Third Avenue 

                New York,NY 10017

 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

Red Abbey Venture Partners (QP), LP 
 By:
Red Abbey Ventures Partners, LLC, its General Partner 
 /s/  Matt
Zuga                                         
     
 By: Matt Zuga, its Managing Member 

By: Matt Zuga 

Title: Managing Member 

Address: 2330 West Joppa Road 

        Suite 330 

        Lutherville, MD 21093 

Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

Red Abbey Venture Partners, LP 
 By: Red
Abbey Ventures Partners, LLC, its General Partner 
 /s/  Matt
Zuga                                         
     
 By: Matt Zuga, its Managing Member 

By: Matt Zuga 

Title: Managing Member 

Address: 2330 West Joppa Road 

        Suite 330 

        Lutherville, MD 21093 

Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

Red Abbey CEO’s Fund, LP 
 By: Red
Abbey Ventures Partners, LLC, its General Partner 
 /s/  Matt
Zuga                                         
     
 By: Matt Zuga, its Managing Member 

By: Matt Zuga 

Title: Managing Member 

Address: 2330 West Joppa Road 

        Suite 330 

        Lutherville, MD 21093 

Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

HVM INTERNATIONAL LIFE SCIENCES FUND NO. 1 LP 

/s/  Hugo
Harrod                                        
     
 Signature of Investor 

By: Hugo Harrod 

Title: Manager HVM Life Sciences Partners LLP 

(Director) 

Address: 6 Herritta Street 

                London WCE 81M

                 United
Kingdom 
 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

HVM EXECUTIVE LIMITED 
  

/s/  Hugo
Harrod                                        
     
 Signature of Investor 

By: Hugo Harrod 

Title: Manager HVM Life Sciences Partners LLP 

(Director) 

Address: 6 Herritta Street 

                London WCE 81M

                 United
Kingdom 
 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

SERVENTIA SA 
 /s/  F.
Ghioldi                                        
         
 Signature of Investor 

By: F. Ghioldi 

Title: Director 

Address: Illegible 

Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

HERCULES TECHNOLOGY GROWTH CAPITAL, INC. 

/s/  K. Nicholas
Martitsch                              

Signature of Investor 

By: K. Nicholas Martitsch 

Title: Associate General Counsel 

Address: 400 Hamilton Ave. 

                Suite 310 

                Palo Alto, CA 94301

 Date: November 9, 2007 
  

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

Will Lewis 
 /s/  William H.
Lewis                                 

Signature of Investor 

By: William H. Lewis 

Title: _______________________________ 

Address: 22 Cantebury Lane 

                Summit, NJ 07901

 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

Jawa II LLC 
 /s/  James M.
Wisler                                     

James M. Wisler 
 Signature of Investor

 By: James M. Wisler 

Title: Manager 

Address: P.O. Box 630440 

                Highlands Ranch, CO
80124 
 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 Counterpart Signature Page For Investors 

The undersigned hereby agrees to become a party to that certain Amended and Restated investor Rights Agreement dated as of
November 9, 2007 (the “Agreement”) by and among Aegerion Pharmaceuticals, Inc. (the “Company”) and others set forth on the signature pages thereto. From and after the undersigned’s execution and delivery and the
Company’s acceptance of this Counterpart Signature Page, the undersigned shall be an “Investor”, as defined in the Agreement. 

Eileen M. Moore 
 /s/  Eileen M.
Moore                                     

Eileen M. Moore 
 Signature of Investor

 By: Eileen M. Moore 

Title: _______________________________ 

Address: 36 Weston Road 

                Weston, CT 06883

 Date: November 9, 2007 

Agreed and accepted: 
 AEGERION PHARMACEUTICALS,
INC. 
 By:  /s/  Gerald
Wisler                                 

        Name: Gerald Wisler 

        Title: President and CEO 

Date: 11/9/2007 
 Signature
Page to Amended and Restated Investor Rights Agreement 

 INVESTORS 

Index Ventures III (Delaware) L.P. 
 Index
Ventures III (Jersey) L.P. 
 Index Ventures III Parallel Entrepreneur Fund (Jersey) L.P. 

Yucca Partners LP Jersey Branch 
 Advent
Healthcare and Life Sciences III-A Limited Partnership 
 Advent Healthcare and Life Sciences III GP Limited Partnership 

Advent Partners HLS III Limited Partnership 

Alta BioPharma Partners III, L.P. 
 Alta
BioPharma Partners III GmbH & Co. Beteiligungs KG 
 Alta Embarcadero BioPharma Partners III, LLC 

MC Life Science Ventures, Inc. 
 Red Abbey
Venture Partners (QP), LP 
 Red Abbey Venture Partners, LP 

Red Abbey CEO’s Fund, LP 
 MVM International
Life Sciences Fund No. 1 LP 
 MVM Executive Limited 

Serventia SA 
 Hercules Technology Growth
Capital, Inc. 
 William Lewis 
 David
Arkowitz 
 JAWZ II LLC 
 Eileen M.
Moore

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