2006 Stock Option and Grant Plan, as amended



 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
AND GRANT PLAN 
  

 “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

  

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 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 

 

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 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

  

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 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 
  

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 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. 

 

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 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 







 Aegerion Pharmaceuticals, Inc. 

Amendment No. 3 

to 
 2006
Stock Option and Grant Plan 
 Pursuant to resolutions duly adopted by the board of directors on September 15, 2010 and duly approved by the
stockholders on                          , 2010, 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 term “1,460,153 Shares” in Section 3(a) of the Plan and replacing it with “4,710,153 Shares.” 

This Amendment No. 3 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. 3 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
	September 15, 2010
	
	Approved by the stockholders on
	                         ,
2010








 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]