Document:

Form of Medium-Term Notes, Series K, Notes Linked to the Dow Jones Industrial

 Exhibit 4.4 
 [Face of Note] 
 Unless this certificate is presented by an authorized
representative of The Depository Trust Company, a New York corporation (“DTC”), to the Company or its agent for registration of transfer, exchange or payment, and any certificate issued is registered in the name of Cede &
Co. or in such other name as requested by an authorized representative of DTC (and any payment is made to Cede & Co. or such other entity as is requested by an authorized representative of DTC), ANY TRANSFER, PLEDGE OR OTHER USE HEREOF FOR
VALUE OR OTHERWISE BY OR TO ANY PERSON IS WRONGFUL inasmuch as the registered owner hereof, Cede & Co., has an interest herein. 
  

			
	 CUSIP NO. 94986RHA2
 REGISTERED
NO.
	  	FACE AMOUNT: $                    

 WELLS FARGO & COMPANY 

MEDIUM-TERM NOTE, SERIES K 
 Due Nine Months or More From Date of Issue 
 Notes
Linked to the Dow Jones Industrial AverageSM

 due August 7, 2018 
 WELLS FARGO & COMPANY, a corporation duly organized and existing under the laws of the State of Delaware (hereinafter called the “Company,” which term includes any successor
corporation under the Indenture hereinafter referred to), for value received, hereby promises to pay to CEDE & Co., or registered assigns, an amount equal to the Redemption Amount (as defined below), in such coin or currency of the United
States of America as at the time of payment is legal tender for payment of public and private debts, on the Stated Maturity Date. The “Initial Stated Maturity Date” shall be August 7, 2018. If no Market Disruption Event (as
defined below) occurs or is continuing with respect to the Index (as defined below) on the final scheduled Calculation Day (as defined below), the Initial Stated Maturity Date will be the “Stated Maturity Date.” If a Market
Disruption Event occurs or is continuing with respect to the Index on the final scheduled Calculation Day, the “Stated Maturity Date” shall be the later of (i) three Business Days (as defined below) after the postponed final
Calculation Day and (ii) the Initial Stated Maturity Date. This Security shall not bear any interest. 
 Any payments on
this Security at Maturity will be made against presentation of this Security at the office or agency of the Company maintained for that purpose in the City of Minneapolis, Minnesota and at any other office or agency maintained by the Company for
such purpose. 
 “Face Amount” shall mean, when used with respect to this Security, the amount set forth on the
face of this Security as its “Face Amount.” 

 Determination of Redemption Amount 

The “Redemption Amount” of this Security will equal: 

 

	 	•	 	 If the Average Ending Level is greater than the Starting Level: the Face Amount plus the greater of: 

(i) the Minimum Return; and 
  

			
	(ii)	  	

  

	 	•	 	 If the Average Ending Level is less than or equal to the Starting Level: the Face Amount plus the Minimum Return. 

“Index” shall mean the Dow Jones Industrial AverageSM. 

The “Pricing Date” shall mean January 31, 2012. 

The “Starting Level” is 12632.91, the Closing Level of the Index on the Pricing Date. 

The “Closing Level” of the Index on any Trading Day means the official closing level of the Index as reported by the
Index Sponsor on such Trading Day. 
 The “Average Ending Level” will be the arithmetic average of the Closing
Level of the Index on the Calculation Days. 
 The “Participation Rate” is 100%. 

The “Minimum Return” is 5% of the Face Amount of this Security. 

“Index Sponsor” shall mean CME Group Index Services LLC, as assignee of Dow Jones & Company. 

“Business Day” shall mean a day, other than a Saturday or Sunday, that is neither a legal holiday nor a day on which
banking institutions are authorized or required by law or regulation to close in New York, New York or Minneapolis, Minnesota. 

A “Trading Day” with respect to the Index means a day, as determined by the Calculation Agent, on which (i) the
Relevant Exchanges (as defined below) with respect to the securities underlying the Index are open for trading for their regular trading sessions and (ii) the exchanges on which futures or options contracts related to the Index or successor
thereto, if applicable, are traded, are open for trading for their respective regular trading sessions. 

  
 2 

 The “Calculation Days” shall be semi-annually, on the last Trading Day of
each January and July, commencing July 2012 and ending July 2018. A Calculation Day is subject to postponement due to the occurrence of a Market Disruption Event. If a Market Disruption Event occurs or is continuing with respect to the Index on a
Calculation Day, such Calculation Day will be postponed to the first succeeding Trading Day on which a Market Disruption Event has not occurred and is not continuing. If such first succeeding Trading Day has not occurred as of the eighth scheduled
Trading Day after an originally scheduled Calculation Day, that eighth scheduled Trading Day shall be deemed a Calculation Day. If a Calculation Day has been postponed eight scheduled Trading Days after an originally scheduled Calculation Day and
such eighth scheduled Trading Day is not a Trading Day, or if a Market Disruption Event occurs or is continuing with respect to the Index on such eighth scheduled Trading Day, the Calculation Agent will determine the Closing Level of the Index on
such eighth scheduled Trading Day in accordance with the formula for and method of calculating the Closing Level of the Index last in effect prior to commencement of the Market Disruption Event, using the closing price (or, with respect to any of
the relevant securities, if such date is not a Trading Day or a Market Disruption Event has occurred, its good faith estimate of the closing price that would have prevailed for such securities) on such date of each security most recently included in
the Index. See “—Market Disruption Events.” As used herein, “closing price” means, with respect to any security on any date, the last reported sales price regular way on such date or, in case no such reported sale
takes place on such date, the average of the reported closing bid and asked prices regular way on such date, in either case on the primary organized exchange or trading system on which such security is then listed or admitted to trading. 

“Calculation Agent Agreement” shall mean the Calculation Agent Agreement dated as of February 7, 2012 between the
Company and the Calculation Agent, as amended from time to time. 
 “Calculation Agent” shall mean the Person
that has entered into the Calculation Agent Agreement with the Company providing for, among other things, the determination of the Average Ending Level and the Redemption Amount, which term shall, unless the context otherwise requires, include its
successors under such Calculation Agent Agreement. The initial Calculation Agent shall be Wells Fargo Securities, LLC. Pursuant to the Calculation Agent Agreement, the Company may appoint a different Calculation Agent from time to time after the
initial issuance of this Security without the consent of the Holder of this Security and without notifying the Holder of this Security. 

Discontinuance Of The Index; Alteration Of Method Of Calculation 
 If the Index Sponsor discontinues publication of the Index, and the Index Sponsor or another entity publishes a successor or substitute equity index that the Calculation Agent determines, in its sole
discretion, to be comparable to the Index (a “Successor Equity Index”), then, upon the Calculation Agent’s notification of that determination to the Trustee and the Company, the Calculation Agent will substitute the Successor
Equity Index as calculated by the relevant Index Sponsor or any other entity and calculate the Average Ending Level as described above. Upon any selection by the Calculation Agent of a Successor Equity Index, the Company will cause notice to be
given to the Holder of this Security. 

  
 3 

 In the event that the Index Sponsor discontinues publication of the Index and the
Calculation Agent does not select a Successor Equity Index, the Calculation Agent will compute a substitute level for the Index in accordance with the procedures last used to calculate the Index before any discontinuance. If a Successor Equity Index
is selected or the Calculation Agent calculates a level as a substitute for the Index, the Successor Equity Index or level will be used as a substitute for the Index for all purposes, including the purpose of determining whether a Market Disruption
Event exists. 
 If at any time the Index Sponsor makes a material change in the formula for or the method of calculating the
Index, or in any other way materially modifies the Index so that the Index does not, in the opinion of the Calculation Agent, fairly represent the level of the Index had those changes or modifications not been made, then, from and after that time,
the Calculation Agent will, at the close of business in New York, New York, on the date that the Closing Level of the Index is to be calculated, make any adjustments as, in the good faith judgment of the Calculation Agent, may be necessary in order
to arrive at a value of an equity index comparable to the Index as if those changes or modifications had not been made, and calculate the level of the Index with reference to such equity index, as so adjusted. Accordingly, if the method of
calculating the Index is modified so that the level of the Index is a fraction or a multiple of what it would have been if it had not been modified, then the Calculation Agent will adjust the Index in order to arrive at a level of the Index as if it
had not been modified. 
 Market Disruption Events 
 A “Market Disruption Event” means, with respect to the Index, any of the following events as determined by the Calculation Agent in its sole discretion: 

 

	 	(A)	A material suspension or material limitation of trading in the securities which then comprise 20% or more of the level of the Index or any Successor Equity Index has
been imposed by the Relevant Exchanges on which those securities are traded, at any time during the one-hour period preceding the Close of Trading on such day, whether by reason of movements in price exceeding limits permitted by those Relevant
Exchanges or otherwise. 

  

	 	(B)	A material suspension or material limitation of trading has occurred on that day, in each case during the one-hour period preceding the Close of Trading in options or
futures contracts related to the Index or any Successor Equity Index, on the primary exchange or quotation system on which those options or futures contracts are traded, whether by reason of movements in price exceeding levels permitted by the
exchange, the quotation system or otherwise. 

  

	 	(C)	Any event, other than an early closure, that materially disrupts or impairs the ability of market participants in general to effect transactions in, or obtain market
values for, the securities that then comprise 20% or more of the level of the Index or any Successor Equity Index, at any time during the one-hour period that ends at the Close of Trading on that day. 

  
 4 

	 	(D)	Any event, other than an early closure, that materially disrupts or impairs the ability of market participants in general to effect transactions in, or obtain market
values for, the futures or options contracts relating to the Index or any Successor Equity Index on the primary exchange or quotation system on which those futures or options contracts are traded, at any time during the one-hour period that ends at
the Close of Trading on that day. 

  

	 	(E)	The closure of the Relevant Exchanges on which the securities that then comprise 20% or more of the level of the Index or any Successor Equity Index are traded or the
primary exchange or quotation system on which futures or options contracts relating to the Index or any Successor Equity Index are traded prior to its scheduled Close of Trading unless the earlier closing time is announced by the Relevant Exchanges,
the primary exchange or the quotation system, as applicable, at least one hour prior to the earlier of (1) the actual closing time for the regular trading session on the Relevant Exchanges, the primary exchange or the quotation system, as
applicable, and (2) the submission deadline for orders to be entered into the relevant exchanges, the primary exchange or the quotation system, as applicable, for execution at the Close of Trading on that day. 

For purposes of determining whether a Market Disruption Event has occurred: 

 

	 	(1)	the relevant percentage contribution of a security to the level of the Index or any Successor Equity Index will be based on a comparison of (x) the portion of the
level of the Index attributable to that security and (y) the overall level of the Index or Successor Equity Index, in each case immediately before the occurrence of the Market Disruption Event; 

 

	 	(2)	“Close of Trading” means in respect of any Relevant Exchange, primary exchange or quotation system, the scheduled weekday closing time on a day on
which such Relevant Exchange, primary exchange or quotation system is scheduled to be open for trading for its respective regular trading session, without regard to after hours or any other trading outside the regular trading session hours; and

  

	 	(3)	“Relevant Exchange” for any security (or any combination thereof then underlying the Index or any Successor Equity Index) means the primary exchange or
quotation system on which such security is traded, as determined by the Calculation Agent. 

 Calculation Agent

 The Calculation Agent will determine the Redemption Amount and the Average Ending Level. In addition, the Calculation
Agent will (i) determine if adjustments are required to the Closing Level of the Index under the circumstances described in this Security, (ii) if publication of the Index is discontinued, select a Successor Equity Index or, if no
Successor Equity Index is available, determine the Closing Level of the Index under the circumstances described in this Security, and (iii) determine whether a Market Disruption Event has occurred. 

  
 5 

 The Company covenants that, so long as this Security is Outstanding, there shall at all
times be a Calculation Agent (which shall be a broker-dealer, bank or other financial institution) with respect to this Security. 
 All determinations made by the Calculation Agent with respect to this Security will be at the sole discretion of the Calculation Agent and, in the absence of manifest error, will be conclusive for all
purposes and binding on the Company and the Holder of this Security. All percentages and other amounts resulting from any calculation with respect to this Security will be rounded at the Calculation Agent’s discretion. 

Redemption and Repayment 
 This Security is not subject to redemption at the option of the Company or repayment at the option of the Holder hereof prior to August 7, 2018. This Security is not entitled to any sinking fund.

 Acceleration 
 If an Event of Default, as defined in the Indenture, with respect to this Security shall occur and be continuing, the Redemption Amount (calculated as set forth in the next sentence) of this Security may
be declared due and payable in the manner and with the effect provided in the Indenture. The amount payable to the Holder hereof upon any acceleration permitted under the Indenture will be equal to the Redemption Amount hereof calculated as provided
herein; provided, however, that the Redemption Amount will be calculated using (i) the Closing Level(s) ascertained on the Calculation Day(s) that occurred before the date of acceleration and (ii) the Closing Level(s) ascertained on each
of the Trading Days on which a Market Disruption Event has not occurred or is not continuing leading up to the date of acceleration in such number equal to the number of Calculation Days scheduled to occur after the date of acceleration. 

 
  

Reference is hereby made to the further provisions of this Security set forth on the reverse hereof, which further provisions shall for
all purposes have the same effect as if set forth at this place. 
 Unless the certificate of authentication hereon has been
executed by the Trustee referred to on the reverse hereof by manual signature or its duly authorized agent under the Indenture referred to on the reverse hereof by manual signature, this Security shall not be entitled to any benefit under the
Indenture or be valid or obligatory for any purpose. 
 [The remainder of this page has been left intentionally blank]

  
 6 

 IN WITNESS WHEREOF, the Company has caused this instrument to be duly executed under its
corporate seal. 
 DATED:
                             

 

					
	WELLS FARGO & COMPANY
		
	By:	 	 
		 	 
		 	Its:	 	 

 [SEAL] 
  

					
		
	Attest:	 	 
		 	 
		 	Its:	 	 

 TRUSTEE’S CERTIFICATE OF 
 AUTHENTICATION 
 This is one of the Securities of the 

series designated therein described 
 in the
within-mentioned Indenture. 
 CITIBANK, N.A., 
         as Trustee 

			
		
	By:	 	 
		 	Authorized Signature
		
		 	OR

  

			
	 WELLS FARGO BANK, N.A.,
     as Authenticating Agent for the Trustee

		
	By:	 	 
		 	Authorized Signature

  
 7 

 [Reverse of Note] 
 WELLS FARGO & COMPANY 
 MEDIUM-TERM NOTE, SERIES K

 Due Nine Months or More From Date of Issue 

Notes Linked to the Dow Jones Industrial AverageSM 
 due August 7, 2018 
 This Security is one of a duly authorized issue
of securities of the Company (herein called the “Securities”), issued and to be issued in one or more series under an indenture dated as of July 21, 1999, as amended or supplemented from time to time (herein called the
“Indenture”), between the Company and Citibank, N.A., as Trustee (herein called the “Trustee,” which term includes any successor trustee under the Indenture), to which Indenture and all indentures supplemental
thereto reference is hereby made for a statement of the respective rights, limitations of rights, duties and immunities thereunder of the Company, the Trustee and the Holders of the Securities, and of the terms upon which the Securities are, and are
to be, authenticated and delivered. This Security is one of the series of the Securities designated as Medium-Term Notes, Series K, of the Company, which series is limited to an aggregate principal amount or face amount, as applicable, of
$25,000,000,000 or the equivalent thereof in one or more foreign or composite currencies. The amount payable on the Securities of this series may be determined by reference to the performance of one or more equity-, commodity- or currency-based
indices, exchange traded funds, securities, commodities, currencies, statistical measures of economic or financial performance, or a basket comprised of two or more of the foregoing, or any other market measure or may bear interest at a fixed rate
or a floating rate. The Securities of this series may mature at different times, be redeemable at different times or not at all, be repayable at the option of the Holder at different times or not at all and be denominated in different currencies.

 Article Sixteen of the Indenture shall not apply to this Security. 

The Securities are issuable only in registered form without coupons and will be either (a) book-entry securities represented by one
or more Global Securities recorded in the book-entry system maintained by the Depositary or (b) certificated securities issued to and registered in the names of, the beneficial owners or their nominees. 

The Company agrees, to the extent permitted by law, not to voluntarily claim the benefits of any laws concerning usurious rates of
interest against a Holder of this Security. 
 Modification and Waivers 

The Indenture permits, with certain exceptions as therein provided, the amendment thereof and the modification of the rights and
obligations of the Company and the rights of the Holders of the Securities of each series to be affected under the Indenture at any time by the Company and the Trustee with the consent of the Holders of a majority in principal amount of the
Securities at the 

  
 8 

 
time Outstanding of all series to be affected, acting together as a class. The Indenture also contains provisions permitting the Holders of a majority in principal amount of the Securities of all
series at the time Outstanding affected by certain provisions of the Indenture, acting together as a class, on behalf of the Holders of all Securities of such series, to waive compliance by the Company with those provisions of the Indenture. Certain
past defaults under the Indenture and their consequences may be waived under the Indenture by the Holders of a majority in principal amount of the Securities of each series at the time Outstanding, on behalf of the Holders of all Securities of such
series. Solely for the purpose of determining whether any consent, waiver, notice or other action or Act to be taken or given by the Holders of Securities pursuant to the Indenture has been given or taken by the Holders of Outstanding Securities in
the requisite aggregate principal amount, the principal amount of this Security will be deemed to be equal to the amount set forth on the face hereof as the “Face Amount” hereof. Any such consent or waiver by the Holder of this Security
shall be conclusive and binding upon such Holder and upon all future Holders of this Security and of any Security issued upon the registration of transfer hereof or in exchange herefor or in lieu hereof, whether or not notation of such consent or
waiver is made upon this Security. 
 Defeasance 
 Section 403 and Article Fifteen of the Indenture and the provisions of clause (ii) of Section 401(1)(B) of the Indenture, relating to defeasance at any time of (a) the entire
indebtedness on this Security and (b) certain restrictive covenants and certain Events of Default, upon compliance by the Company with certain conditions set forth therein, shall not apply to this Security. The remaining provisions of
Section 401 of the Indenture shall apply to this Security. 
 Authorized Denominations 

This Security is issuable only in registered form without coupons in denominations of $1,000 or any amount in excess thereof which is an
integral multiple of $1,000. 
 Registration of Transfer 
 Upon due presentment for registration of transfer of this Security at the office or agency of the Company in the City of Minneapolis, Minnesota, a new Security or Securities of this series, with the same
terms as this Security, in authorized denominations for an equal aggregate Face Amount will be issued to the transferee in exchange herefor, as provided in the Indenture and subject to the limitations provided therein and to the limitations
described below, without charge except for any tax or other governmental charge imposed in connection therewith. 
 This
Security is exchangeable for definitive Securities in registered form only if (x) the Depositary notifies the Company that it is unwilling or unable to continue as Depositary for this Security or if at any time the Depositary ceases to be a
clearing agency registered under the Securities Exchange Act of 1934, as amended, and a successor depositary is not appointed within 90 days after the Company receives such notice or becomes aware of such ineligibility, (y) the Company in
its sole discretion determines that this Security shall be exchangeable for definitive Securities in registered form and notifies the Trustee thereof or (z) an Event of Default with respect to the Securities represented hereby has occurred and
is continuing. If this Security is exchangeable pursuant to the preceding sentence, it shall be exchangeable for definitive Securities in registered form, having the same date of issuance, Stated Maturity Date and other terms and of authorized
denominations aggregating a like amount. 

  
 9 

 This Security may not be transferred except as a whole by the Depositary to a nominee of the
Depositary or by a nominee of the Depositary to the Depositary or another nominee of the Depositary or by the Depositary or any such nominee to a successor of the Depositary or a nominee of such successor. Except as provided above, owners of
beneficial interests in this Global Security will not be entitled to receive physical delivery of Securities in definitive form and will not be considered the Holders hereof for any purpose under the Indenture. 

Prior to due presentment of this Security for registration of transfer, the Company, the Trustee and any agent of the Company or the
Trustee may treat the Person in whose name this Security is registered as the owner hereof for all purposes, whether or not this Security be overdue, and neither the Company, the Trustee nor any such agent shall be affected by notice to the
contrary. 
 Obligation of the Company Absolute 
 No reference herein to the Indenture and no provision of this Security or the Indenture shall alter or impair the obligation of the Company, which is absolute and unconditional, to pay the Redemption
Amount at the times, place and rate, and in the coin or currency, herein prescribed, except as otherwise provided in this Security. 
 No
Personal Recourse 
 No recourse shall be had for the payment of the Redemption Amount, or for any claim based hereon, or
otherwise in respect hereof, or based on or in respect of the Indenture or any indenture supplemental thereto, against any incorporator, stockholder, officer or director, as such, past, present or future, of the Company or any successor corporation,
whether by virtue of any constitution, statute or rule of law, or by the enforcement of any assessment or penalty or otherwise, all such liability being, by the acceptance hereof and as part of the consideration for the issuance hereof, expressly
waived and released. 
 Defined Terms 
 All terms used in this Security which are defined in the Indenture shall have the meanings assigned to them in the Indenture unless otherwise defined in this Security. 

Governing Law 

This Security shall be governed by and construed in accordance with the law of the State of New York, without regard to principles of
conflicts of laws. 

  
 10 

 ABBREVIATIONS 

The following abbreviations, when used in the inscription on the face of this instrument, shall be construed as though they were written
out in full according to applicable laws or regulations: 

							
				
	 TEN COM
	  	—	  	as tenants in common	  	
				
	 TEN ENT
	  	—	  	as tenants by the entireties	  	
				
	 JT TEN
	  	—	  	 as joint tenants with right

of survivorship and not
 as tenants in
common
	  	

  

							
	UNIF GIFT MIN ACT —	  	 	  	Custodian    	  	 
		  	(Cust)	  		  	(Minor)

  

			
	Under Uniform Gifts to Minors Act
	
	 
		 	(State)

  
 Additional abbreviations may also
be used though not in the above list. 
 FOR VALUE RECEIVED, the undersigned hereby sell(s) and transfer(s) unto

 Please Insert Social Security or 

Other Identifying Number of Assignee 

			
	
	 
		 	

  
  

 
  
  

 
  
  

(PLEASE PRINT OR TYPE NAME AND
ADDRESS INCLUDING POSTAL ZIP CODE OF ASSIGNEE) 

  
 11 

 the within Security of WELLS FARGO & COMPANY and does hereby irrevocably constitute and appoint
                     attorney to transfer the said Security on the books of the Company, with full power of substitution in the premises. 

 

					
	Dated:
                                         
       	 		 	
			
		 		 	 
		 		 	
		 		 	 

 NOTICE: The signature to this assignment must correspond with the name as written upon the face of the within instrument
in every particular, without alteration or enlargement or any change whatever. 

  
 12Stock Purchase Agreement

 Exhibit 10.1 
 STOCK PURCHASE AGREEMENT 
 among 

ENOBIA PHARMA CORP., 
 ALEXION PHARMACEUTICALS, INC. 
 and 

THE SELLERS LISTED ON 
 SCHEDULE I ATTACHED HERETO 

 TABLE OF CONTENTS 

 

									
	 Section
	 	 	 	 	  	Page	 
	1.	 	 Purchase and Sale of the Shares
	  	 	1	  
		 	 1.01.
	 	 Purchase of the Shares from the Sellers
	  	 	2	  
		 	 1.02.
	 	 Certificates
	  	 	2	  
		 	 1.03.
	 	 Further Assurances
	  	 	2	  
		 	 1.04.
	 	 Purchase Price for the Shares
	  	 	2	  
		 	 1.05.
	 	 Closing
	  	 	3	  
		 	 1.06.
	 	 Withholding Rights
	  	 	3	  
	2.	 	 Representations of the Sellers
	  	 	3	  
		 	 2.01.
	 	 Shares
	  	 	3	  
		 	 2.02.
	 	 Authority; No Conflict; Required Filings and Consents
	  	 	4	  
		 	 2.03.
	 	 Litigation
	  	 	4	  
		 	 2.04.
	 	 Tax Advisors
	  	 	5	  
		 	 2.05.
	 	 Consultation with Counsel; Information
	  	 	5	  
		 	 2.06.
	 	 Escrow and Future Consideration
	  	 	5	  
	3.	 	 Representations of the Buyer
	  	 	5	  
		 	 3.01.
	 	 Organization, Standing and Power
	  	 	5	  
		 	 3.02.
	 	 Authority; No Conflict; Required Filings and Consents
	  	 	5	  
		 	 3.03.
	 	 Litigation
	  	 	6	  
		 	 3.04.
	 	 Investment Representation
	  	 	6	  
	4.	 	 Conditions to Obligations of the Buyer and the Sellers
	  	 	7	  
		 	 4.01.
	 	 Satisfaction of Closing Conditions Under Merger Agreement
	  	 	7	  
	5.	 	 Conditions to Obligations of the Buyer
	  	 	7	  
		 	 5.01.
	 	 Continued Truth of Representations and Warranties of the Sellers
	  	 	7	  
		 	 5.02.
	 	 Closing Deliveries
	  	 	7	  
	6.	 	 Conditions to Obligations of the Sellers
	  	 	7	  
		 	 6.01.
	 	 Continued Truth of Representations and Warranties of the Buyer
	  	 	7	  
	7.	 	 Indemnification
	  	 	8	  
		 	 7.01.
	 	 Indemnification by the Sellers
	  	 	8	  
		 	 7.02.
	 	 Indemnification by the Buyer
	  	 	8	  
		 	 7.03.
	 	 Claims for Indemnification
	  	 	8	  
		 	 7.04.
	 	 Survival of Representations
	  	 	8	  
	8.	 	 Other Agreements
	  	 	8	  
		 	 8.01.
	 	 Release of Claims
	  	 	8	  
		 	 8.02.
	 	 Tax Reimbursements
	  	 	9	  
		 	 8.03.
	 	 Treatment as Company Equityholder
	  	 	9	  
	9.	 	 Termination of Agreement
	  	 	9	  
		 	 9.01.
	 	 Termination of Merger Agreement
	  	 	9	  
		 	 9.02.
	 	 Termination by Agreement of the Parties
	  	 	9	  
		 	 9.03.
	 	 Effect of Termination
	  	 	9	  

  
 - i -

  

									
	10.	 	 Miscellaneous
	  	 	9	  
		 	 10.01.
	 	 Notices
	  	 	9	  
		 	 10.02.
	 	 Entire Agreement
	  	 	10	  
		 	 10.03.
	 	 No Third Party Beneficiaries
	  	 	10	  
		 	 10.04.
	 	 Assignment
	  	 	10	  
		 	 10.05.
	 	 Severability
	  	 	10	  
		 	 10.06.
	 	 Counterparts and Signature
	  	 	10	  
		 	 10.07.
	 	 Interpretation
	  	 	10	  
		 	 10.08.
	 	 Governing Law
	  	 	11	  
		 	 10.09.
	 	 Remedies
	  	 	11	  
		 	 10.10.
	 	 Submission to Jurisdiction.
	  	 	11	  
		 	 10.11.
	 	 Foreign Exchange Conversions
	  	 	12	  

  
 - ii -

 Exhibits 
  

					
	 A
	  	—	  	Agreement and Plan of Merger dated as of December 28, 2011, as amended by a First Amendment to Agreement and Plan of Merger dated the date hereof.

  

					
	 B
	  	—	  	Form of Promissory Note.

 Schedules to be provided by the Sellers 

 

					
	I	  	—	  	Shares to be Sold by Sellers

  
 - iii -

 STOCK PURCHASE AGREEMENT 

This Stock Purchase Agreement (the “Agreement”) made as of the 1st day of February, 2012, by and among ENOBIA PHARMA
CORP, a Delaware corporation (the “Company”), ALEXION PHARMACEUTICALS, INC., a Delaware corporation (the “Buyer”), and the individuals listed on Schedule I attached hereto (individually, a
“Seller” and collectively, the “Sellers”), who hold Company Options of the Company. 

Preliminary Statement 
 1. The Buyer, EMRD Corporation, a Delaware corporation and a wholly owned subsidiary of the Buyer (the “Transitory Subsidiary”), M. Luc Mainville, Jonathan Silverstein, Robert Heft and
David Bonita, solely in their capacity as the Stockholder Representatives (the “Stockholder Representatives”), and the Company entered into an Agreement and Plan of Merger dated as of December 28, 2011, as amended by a First
Amendment to Agreement and Plan of Merger dated the date hereof (as so amended, the “Merger Agreement”), attached hereto as Exhibit A. 
 2. Capitalized terms used herein and not defined herein shall have the meaning set forth in the Merger Agreement. 
 3. Each Seller is the holder of the Company Options set forth opposite his or her name on Schedule I attached hereto. 
 4. Following the execution and delivery of this Agreement, but prior to the Share Closing (as hereinafter defined), each of the Sellers will exercise such Company Option and become the owner of the number
of shares of Company Common Stock (collectively, the “Shares”) set forth opposite his or her name on Schedule I attached hereto resulting from the exercise of such Company Option (it being understood that the Shares include
only the Shares of Company Stock obtained by Seller upon exercise of each such Company Option and do not include any other shares of Company Stock owned by such Seller prior to, or acquired by such Seller after, the time of the exercise of such
Company Option). Each Seller shall pay the exercise price of its Company Options by delivery to the Company of a Promissory Note in the form attached hereto as Exhibit B (the “Promissory Note”). 

5. The Buyer desires to purchase, and the Sellers desire to sell, the Shares for the consideration set forth below, subject to the terms
and conditions of this Agreement. 
 NOW, THEREFORE, in consideration of the mutual promises hereinafter set forth and other
good and valuable consideration, the receipt of which is hereby acknowledged, the parties hereby agree as follows: 
 1.
Purchase and Sale of the Shares 

  
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 1.01. Purchase of the Shares from the Sellers. The Buyer and each Seller agree to
effect the following transactions in the following order: 
 (a) On the day prior to the Closing Date, each Seller shall
exercise all Company Options held by such Seller; and 
 (b) On the Closing Date, prior to the Effective Time, each Seller shall
sell, transfer, convey, assign and deliver to the Buyer, and the Buyer shall purchase, acquire and accept from each Seller, all of the Shares owned by such Seller, as set forth opposite such Seller’s name on Schedule I attached hereto
(the “Share Closing”). 
 1.02. Certificates. At the Share Closing, each Seller shall deliver to the
Buyer certificates evidencing the Shares owned by such Seller duly endorsed in blank or with stock powers duly executed by such Seller. In order to facilitate the Share Closing, each Seller hereby grants to the Secretary of the Company an
irrevocable power of attorney to endorse the Shares for transfer to the Buyer in accordance with the terms and conditions of this Agreement. 
 1.03. Further Assurances. At any time and from time to time after the Share Closing, at the Buyer’s request and without further consideration, each of the Sellers shall, at the Buyer’s
expense, promptly execute and deliver such instruments of sale, transfer, conveyance, assignment and confirmation, and take all such other action as the Buyer may reasonably request, more effectively to transfer, convey and assign to the Buyer, and
to confirm the Buyer’s title to, all of the Shares owned by such Seller. 
 1.04. Purchase Price for the Shares.

 (a) The purchase price to be paid by the Buyer for each Share (the “Purchase Price”) shall be the portion of
the Total Consideration payable from time to time in accordance with the terms and conditions of the Merger Agreement with respect to a share of Company Common Stock owned by a Company Equityholder immediately prior to the Effective Time. The
Purchase Price shall be payable by the Buyer to each Seller at the time, and in the manner, payments of the Total Consideration are otherwise payable by the Buyer or the Transitory Subsidiary in accordance with the terms and conditions of the Merger
Agreement. The Purchase Price shall be subject to reduction and withholding as set forth in this Section 1.04. 
 (b) Each
Seller authorizes and instructs Buyer to withhold from the payment of the Purchase Price to such Seller an amount necessary to satisfy and pay in full any amounts due under such Seller’s Promissory Note, each Seller directs Buyer to remit such
amount to the Company in satisfaction in full of all amounts due under such Promissory Note and each Seller directs the Paying Agent to pay to the Company such amount as shall be payable by such Seller with respect to taxes due as a result of the
exercise of Company Options (the “Tax Amount”) and to deduct the Tax Amount from the amounts otherwise payable to such Seller. 

  
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 (c) Each Seller agrees that he or she shall pay his or her pro rata share (as determined
below) of any costs or expenses incurred by the Buyer, the Company or their respective Subsidiaries on or prior to the date hereof in connection with the negotiation, preparation and execution of this Agreement, the First Amendment to Agreement and
Plan of Merger dated the date hereof, or any tax analysis and advice obtained and all documents entered into by the Buyer, the Company or their respective Subsidiaries in connection therewith (the “Transaction Expenses”). Each
Seller’s pro rata share of the Transaction Expenses (the “Expenses Share”) shall be determined by multiplying the Transaction Expenses by a fraction, the numerator of which is the number of Shares being sold by such Seller to
the Buyer under this Agreement and the denominator of which is the total number of Shares being sold to the Buyer under this Agreement. The Transaction Expenses shall be paid by the Buyer to the parties and in the amounts set forth on the Closing
Payment Certificate and each Seller agrees that the Purchase Price otherwise payable to such Seller shall be reduced by such Seller’s Expenses Share. 
 1.05. Closing. The Share Closing shall take place on the Closing Date, prior to the Effective Time, at the offices of Wilmer Cutler Pickering Hale and Dorr LLP, 60 State Street, Boston,
Massachusetts 02109, unless another date, place or time is agreed to in writing by the Buyer and the Sellers. Following the Share Closing, the Sellers shall have no further ownership rights in the Shares sold to the Buyer and shall only have the
right to receive the Purchase Price. 
 1.06. Withholding Rights. Each of the Paying Agent, the Buyer and the Escrow
Agent shall be entitled to deduct and withhold from any consideration otherwise payable pursuant to this Agreement to the Sellers such amounts as it is required to deduct and withhold with respect to the making of such payment under the Code, or any
other applicable state, local or non-U.S. Tax law. To the extent that amounts are so deducted or withheld by the Paying Agent, the Buyer or the Escrow Agent, as the case may be, such withheld amounts shall be (i) remitted by the Paying Agent,
the Buyer or the Escrow Agent, as the case may be, to the applicable Governmental Entity, and (ii) treated for all purposes of this Agreement as having been paid to such Seller in respect of which such deduction and withholding was made by the
Paying Agent, the Buyer or the Escrow Agent, as the case may be. 
 2. Representations of the Sellers 

Each Seller severally represents and warrants to the Buyer as follows: 

2.01. Shares. 
 (a) Upon the exercise of the Seller’s Company Option, such Seller shall have good and marketable title to the Shares, free and clear of any and all Liens. Schedule I attached hereto sets
forth a true and correct description of all Shares to be owned by such Seller upon the exercise of such Company Option. 

  
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 (b) Such Seller has the full right, power and authority to enter into this Agreement and to
transfer, convey and sell to the Buyer at the Share Closing the Shares to be sold by such Seller hereunder and, upon consummation of the purchase contemplated hereby, the Buyer will acquire from such Seller good and marketable title to such Shares,
free and clear of all Liens. 
 (c) No broker of finder has acted for such Seller in connection with this Agreement or the
transactions contemplated hereby, and no broker or finder is entitled to any brokerage or finder’s fee or other commissions in respect of such transactions based upon agreements, arrangements or understandings made by or on behalf of such
Seller. 
 2.02. Authority; No Conflict; Required Filings and Consents. 

(a) This Agreement has been duly executed and delivered by such Seller and constitutes the valid and binding obligation of such Seller,
enforceable against such Seller in accordance with its terms. 
 (b) The execution and delivery of this Agreement by such Seller
do not, and the consummation by such Seller of the transactions contemplated by this Agreement shall not, (i) conflict with, or result in any violation or breach of, or constitute (with or without notice or lapse of time, or both) a default (or
give rise to a right of termination, modification, amendment, cancellation or acceleration of any obligation or loss of any material benefit or to increased, additional, accelerated or guaranteed rights or entitlements of any Person) under, require
a consent or waiver under, constitute a change in control under, require the payment of a penalty under or result in the imposition of any Lien on such Seller’s assets under, any of the terms, conditions or provisions of any lease, license,
contract or other agreement, instrument or obligation to which such Seller is a party or by which he or she or any of his or her properties or assets may be bound, or (ii) conflict with or violate any permit, concession, franchise, license,
judgment, injunction, order, decree, statute, law, ordinance, rule or regulation applicable to such Seller or any of his or her properties or assets. 
 (c) No action by, consent, approval, license, permit, order or authorization of, or registration, declaration, notice or filing with, any Governmental Entity is required by or with respect to such Seller
in connection with the execution and delivery of this Agreement by such Seller or the consummation by such Seller of the transactions contemplated by this Agreement. 
 2.03. Litigation. There is no litigation, action, suit, proceeding, claim, arbitration or investigation pending or, to the knowledge of such Seller, threatened, against such Seller, and such Seller
is not subject to any outstanding order, writ, judgment, injunction or decree of any Governmental Entity that, in either case, would, individually or in the aggregate, (a) prevent or materially delay the consummation by such Seller of the
transactions contemplated by this Agreement or (b) otherwise prevent or materially delay performance by such Seller of any of its material obligations under this Agreement. 

  
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 2.04. Tax Advisors. Such Seller has been advised to, and has had the opportunity to,
consult with his or her own tax advisors regarding the tax consequences of the exercise of the Company Options and the transactions contemplated by this Agreement. With respect to such matters, such Seller has not relied on any statements or
representations of the Company or any of its employees, directors or agents, written or oral. Such Seller understands that he or she (and not the Company) will be solely responsible for his or her own tax liability that may arise as a result of his
or her exercise of the Company Options and the sale of Shares pursuant to this Agreement. 
 2.05. Consultation with Counsel;
Information. Such Seller has had the opportunity to retain and consult with legal counsel of such Seller’s choosing and to ask questions of, and receive answers from, persons acting on behalf of the Company or the Buyer in each case as
necessary to understand the terms and conditions of this Agreement, and to obtain any additional information desired by such Seller with respect to the transactions contemplated by this Agreement. 

2.06. Escrow and Future Consideration. Such Seller acknowledges that the receipt of Future Payments is subject to the achievement
of the Milestones and there is no assurance that any Milestones will be achieved or that any Future Payments will be made. Such Seller also acknowledges that a portion of the Total Consideration payable to such Seller will be reduced by amounts to
be paid into an escrow fund in order to secure certain indemnification obligations under, and in accordance with the terms of, the Merger Agreement. 
 3. Representations of the Buyer 
 The Buyer represents and warrants to each
Seller as follows: 
 3.01. Organization, Standing and Power. The Buyer is a company duly organized, validly existing and
in good standing under the laws of the jurisdiction of its incorporation, has all requisite corporate power and authority to own, lease and operate its properties and assets and to carry on its business as now being conducted, and is duly qualified
to do business and, where applicable as a legal concept, is in good standing as a foreign corporation in each jurisdiction in which the character of the properties it owns, operates or leases or the nature of its activities makes such qualification
necessary, except for such failures to be so qualified or in good standing that, individually or in the aggregate, would not reasonably be expected to have a Buyer Material Adverse Effect. For purposes of this Agreement, “Buyer Material
Adverse Effect” means any material adverse Change with respect to, or any material adverse effect on, the ability of the Buyer to consummate, including any material delay in the Buyer’s ability to consummate, the transactions
contemplated by this Agreement. 
 3.02. Authority; No Conflict; Required Filings and Consents. 

(a) The Buyer has all requisite corporate power and authority to enter into this Agreement and to consummate the transactions
contemplated by this Agreement. The execution and delivery of this Agreement and the consummation of the transactions contemplated by this 

  
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Agreement by the Buyer have been duly authorized by all necessary corporate action on the part of the Buyer. This Agreement has been duly executed and delivered by the Buyer and constitutes the
valid and binding obligation of the Buyer, enforceable against the Buyer in accordance with its terms, subject to the Bankruptcy and Equity Exception. 
 (b) The execution and delivery of this Agreement by the Buyer do not, and the consummation by the Buyer of the transactions contemplated by this Agreement shall not, (i) conflict with, or result in
any violation or breach of, any provision of the certificate of incorporation or by-laws of the Buyer, (ii) conflict with, or result in any violation or breach of, or constitute (with or without notice or lapse of time, or both) a default (or
give rise to a right of termination, modification, amendment, cancellation or acceleration of any obligation or loss of any material benefit or to increased, additional, accelerated or guaranteed rights or entitlements of any Person) under, require
a consent or waiver under, constitute a change in control under, require the payment of a penalty under or result in the imposition of any Lien on the Buyer’s assets under, any of the terms, conditions or provisions of any lease, license,
contract or other agreement, instrument or obligation to which the Buyer is a party or by which it or any of its properties or assets may be bound, or (iii) conflict with or violate any permit, concession, franchise, license, judgment,
injunction, order, decree, statute, law, ordinance, rule or regulation applicable to the Buyer or any of its properties or assets, except in the case of clauses (ii) and (iii) of this Section 3.02(b) for any such conflicts,
violations, breaches, defaults, terminations, modifications, amendments, cancellations, accelerations, losses, rights, entitlements, penalties or Liens, and for any consents or waivers not obtained, that, individually or in the aggregate, would not
reasonably be expected to have a Buyer Material Adverse Effect. 
 (c) No action by, consent, approval, license, permit, order
or authorization of, or registration, declaration, notice or filing with, any Governmental Entity is required by or with respect to the Buyer in connection with the execution and delivery of this Agreement by the Buyer or the consummation by the
Buyer of the transactions contemplated by this Agreement. 
 (d) No vote of the holders of any class or series of the
Buyer’s capital stock or other securities is necessary for the consummation by the Buyer of the transactions contemplated by this Agreement. 
 3.03. Litigation. As of the date hereof, there is no litigation, action, suit, proceeding, claim, arbitration or investigation pending or, to the knowledge of the Buyer, threatened, against the
Buyer, and the Buyer is not subject to any outstanding order, writ, judgment, injunction or decree of any Governmental Entity that, in either case, would, individually or in the aggregate, (a) prevent or materially delay the consummation by the
Buyer of the transactions contemplated by this Agreement or (b) otherwise prevent or materially delay performance by the Buyer of any of its material obligations under this Agreement. 

  
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 3.04. Investment Representation. The Buyer is acquiring the Shares from each Seller
for its own account for investment and not with a view to, or for sale in connection with, any distribution thereof, nor with any present intention of distributing or selling the same; and, except as contemplated by this Agreement and the agreements
contemplated herein, the Buyer has no present or contemplated agreement, undertaking, arrangement, obligation, indebtedness or commitment providing for the disposition thereof, in each case, except in compliance with applicable securities laws.

 4. Conditions to Obligations of the Buyer and the Sellers 

The obligations of the Buyer and the Sellers under this Agreement are subject to the fulfillment, prior to the Share Closing, of the
following conditions precedent: 
 4.01. Satisfaction of Closing Conditions Under Merger Agreement. Each of the
conditions to Closing set forth in Article VII of the Merger Agreement (other than the delivery of items to be delivered at the Closing and other than satisfaction of those conditions that by their nature are to be satisfied at the Closing) shall
have been satisfied or waived in accordance with the provisions of the Merger Agreement. 
 5. Conditions to Obligations of
the Buyer 
 The obligations of the Buyer under this Agreement are subject to the fulfillment, prior to the Share Closing,
of the following additional conditions precedent: 
 5.01. Continued Truth of Representations and Warranties of the
Sellers. The representations and warranties of the Sellers shall be true in all material respects on and as of the date of the Share Closing as though such representations and warranties were made on and as of such date. 

5.02. Closing Deliveries. The Buyer shall have received at or prior to the Share Closing the stock certificates representing the
Shares duly endorsed in accordance with Subsection 1.01 of this Agreement. 
 6. Conditions to Obligations of the
Sellers 
 The obligations of the Sellers under this Agreement are subject to the fulfillment, prior to the Share Closing,
of the following additional conditions precedent: 
 6.01. Continued Truth of Representations and Warranties of the
Buyer. The representations and warranties of the Buyer shall be true in all material respects on and as of the date of the Share Closing as though such representations and warranties were made on and as of such date. 

  
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 7. Indemnification 

7.01. Indemnification by the Sellers. Subject to the terms and conditions of this Section 7, from and after the Share
Closing, each Seller, severally and not jointly, shall indemnify and hold the Buyer harmless from and against any and all Damages incurred or suffered by the Buyer or any of its Subsidiaries (including the Company) to the extent arising out of,
resulting from or constituting any breach or inaccuracy as of the date hereof or as of the date of the Share Closing of any representation, warranty or covenant or other agreement made by such Seller in this Agreement. 

7.02. Indemnification by the Buyer. Subject to the terms and conditions of this Section 7, from and after the Share Closing,
the Buyer shall indemnify and hold harmless each Seller from and against any and all Damages incurred or suffered by any Seller to the extent resulting from or constituting any breach of a representation or warranty of the Buyer contained in this
Agreement (in each case, as such representation or warranty would read if all qualifications as to materiality, including each reference to the defined term “Buyer Material Adverse Effect”, were deleted therefrom). 

7.03. Claims for Indemnification. All claims for indemnification made under this Agreement shall be made in accordance with the
procedures set forth in Section 9.3 of the Merger Agreement. 
 7.04. Survival of Representations. All
representations and warranties made by the Sellers and the Buyer in this Agreement shall survive the Closing without limitation. 
 8. Other Agreements. 
 8.01. Release of Claims. From and after the
Share Closing, each of the Sellers hereby releases and forever discharges the Buyer, the Transitory Subsidiary, the Company, the Surviving Corporation and any and all of their past, present and future affiliated entities, partners, shareholders,
independent contractors, parent corporations, subsidiary corporations, successors, agents, directors, officers, assigns, employees, representatives, insurers, attorneys, and all others (the “Released Parties”) of and from all
claims, demands, Damages, obligations, actions and causes of action such Seller has had, now has or might now have against the Released Parties, or any of them, including those arising in connection with the grant or exercise of such Seller’s
Company Options and the Tax treatment arising from the exercise thereof, including, without limitation, any withholding Tax paid or payable by any Released Party in connection with the exercise thereof and the amount of Tax payable by each of the
Sellers. Each of the Sellers acknowledges and agrees that, as a condition 

  
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to the execution and delivery of this Agreement, such Seller has not relied on any Tax advice provided by any of the Released Parties at any time whatsoever. The foregoing release expressly
excludes any and all obligations of (a) the Buyer set forth in this Agreement, (b) any Released Party, to the extent that any of the organizational documents of the Company or any of its Subsidiaries or Affiliates confer rights to
indemnification and/or advancement of expenses, arising under, or in connection with such rights, (c) any Released Party, arising from or in connection with such Seller’s employment by the Company or any of its Subsidiaries or affiliates,
and (d) any Released Party for directors’ meeting fees and expenses for or in connection with such Seller’s services as a director of the Company or any of its Subsidiaries. 

8.02. Tax Reimbursements. Each Seller agrees that, except as otherwise agreed in writing by the Buyer, any obligation of the
Company to reimburse such Seller for any taxes and tax services is hereby terminated and each Seller releases and forever discharges the Released Parties from any obligation to reimburse such Seller for any such payments. 

8.03. Treatment as Company Equityholder. Each Seller agrees that, except for the provisions for the payment for the Shares set
forth herein (which shall be governed in accordance with the provisions of this Agreement), the Seller shall be a Company Equityholder for all purposes of the Merger Agreement. Without limiting the generality of the foregoing, each Seller appoints
and constitutes the Stockholder Representatives to act in their capacity as such under Section 2.4 of the Merger Agreement. 
 9. Termination of Agreement 
 9.01. Termination of Merger Agreement.
This Agreement shall automatically terminate and the obligations of each party hereto shall cease upon the termination of the Merger Agreement. 
 9.02. Termination by Agreement of the Parties. This Agreement may be terminated by the mutual written agreement of the parties hereto. 

9.03. Effect of Termination. In the event of any termination of this Agreement in accordance with Section 9.01 or
Section 9.02, the Buyer shall have no further obligation or liability to the Sellers under this Agreement, and the Sellers shall have no further obligation or liability to the Buyer under this Agreement; provided that any such
termination shall not relieve any party from liability for damages for any willful breach of this Agreement. 
 10.
Miscellaneous 
 10.01. Notices. All notices and other communications hereunder shall be in writing and shall be
sent in accordance with the provisions of the Merger Agreement. Notices to each of the Sellers shall be sent to the address set forth below their names on the signature pages hereto. 

  
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 10.02. Entire Agreement. This Agreement and the Merger Agreement constitute the
entire agreement among the parties to this Agreement and supersede any prior understandings, agreements or representations by or among the parties hereto, or any of them, written or oral, with respect to the subject matter hereof. 

10.03. No Third Party Beneficiaries. This Agreement is not intended to, and shall not, confer upon any other Person or entity any
rights or remedies hereunder. 
 10.04. Assignment. Neither this Agreement nor any of the rights, interests or
obligations under this Agreement may be assigned or delegated, in whole or in part, by operation of law or otherwise by any of the parties hereto without the prior written consent of the other parties, and any such assignment without such prior
written consent shall be null and void, except that the Buyer may assign its rights under this Agreement to a wholly-owned Subsidiary (which may assume any of Buyer’s obligations under this Agreement) and may make a collateral assignment of its
rights under this Agreement to any Financing Sources; provided that any such assignment or assumption shall not relieve the Buyer of its primary liability for its obligations hereunder. Subject to the preceding sentence, this Agreement shall
be binding upon, inure to the benefit of, and be enforceable by, the parties hereto and their respective successors and permitted assigns. 
 10.05. Severability. Any term or provision of this Agreement that is invalid or unenforceable in any situation in any jurisdiction shall not affect the validity or enforceability of the remaining
terms and provisions hereof or the validity or enforceability of the offending term or provision in any other situation or in any other jurisdiction. If the final judgment of a court of competent jurisdiction declares that any term or provision
hereof is invalid or unenforceable, the parties hereto agree that the court making such determination shall have the power to limit the term or provision, to delete specific words or phrases, or to replace any invalid or unenforceable term or
provision with a term or provision that is valid and enforceable and that comes closest to expressing the intention of the invalid or unenforceable term or provision, and this Agreement shall be enforceable as so modified. In the event such court
does not exercise the power granted to it in the prior sentence, the parties hereto agree to replace such invalid or unenforceable term or provision with a valid and enforceable term or provision that will achieve, to the extent possible, the
economic, business and other purposes of such invalid or unenforceable term. 
 10.06. Counterparts and Signature. This
Agreement may be executed in two (2) or more counterparts, each of which shall be deemed an original but all of which together shall be considered one and the same agreement and shall become effective when counterparts have been signed by each
of the parties hereto and delivered to the other parties, it being understood that all parties need not sign the same counterpart. This Agreement may be executed and delivered by facsimile or .pdf transmission. 

10.07. Interpretation. Except where expressly stated otherwise in this Agreement, the following rules of interpretation apply to
this Agreement: (a) “either” and “or” are not exclusive and “include,” “includes” and “including” are not limiting; (b) “hereof,” “hereto,” “hereby,”

  
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“herein” and “hereunder” and words of similar import when used in this Agreement refer to this Agreement as a whole and not to any particular provision of this Agreement;
(c) “date hereof” refers to the date set forth in the initial caption of this Agreement; (d) “extent” in the phrase “to the extent” means the degree to which a subject or other thing extends, and such phrase
does not mean simply “if”; (e) descriptive headings, the table of defined terms and the table of contents are inserted for convenience only and do not affect in any way the meaning or interpretation of this Agreement;
(f) definitions contained in this Agreement are applicable to the singular as well as the plural forms of such terms; (g) references to a Person or entity are also to its permitted successors and assigns; (h) references to an
“Article,” “Section,” “Exhibit” or “Schedule” refer to an Article or Section of, or an Exhibit or Schedule to, this Agreement; (ix) references to “$” or otherwise to dollar amounts refer to the
lawful currency of the United States; (x) references to a federal, state, local or foreign statute or law include any rules, regulations and delegated legislation issued thereunder; and (i) references to a communication by a regulatory
agency include a communication by the staff of such regulatory agency. 
 10.08. Governing Law. This Agreement shall be
governed by and construed in accordance with the internal laws of the State of Delaware without giving effect to any choice or conflict of law provision or rule (whether of the State of Delaware or any other jurisdiction) that would cause the
application of laws of any jurisdictions other than those of the State of Delaware. 
 10.09. Remedies. Except as
otherwise provided herein, any and all remedies herein expressly conferred upon a party will be deemed cumulative with and not exclusive of any other remedy conferred hereby, or by law or equity upon such party, and the exercise by a party of any
one (1) remedy will not preclude the exercise of any other remedy. The parties hereto agree that irreparable damage would occur in the event that any of the provisions of this Agreement were not performed in accordance with their specific terms
or were otherwise breached. It is accordingly agreed that the parties shall be entitled to an injunction or injunctions to prevent breaches of this Agreement and to enforce specifically the terms and provisions of this Agreement, this being in
addition to any other remedy to which they are entitled at law or in equity. Each party shall (and shall cause its Affiliates to) use commercially reasonable efforts to pursue all legal rights and remedies available in order to minimize the damages
for which it seeks recovery under this Agreement. 
 10.10. Submission to Jurisdiction. 

(a) Each of the parties to this Agreement (i) consents to submit itself to the exclusive personal jurisdiction of the Court of
Chancery of the State of Delaware, New Castle County, or, if that court does not have jurisdiction, a federal court sitting in Wilmington, Delaware in any action or proceeding arising out of or relating to this Agreement or any of the transactions
contemplated by this Agreement, (ii) agrees that all claims in respect of such action or proceeding may be heard and determined in any such court, (iii) agrees that it shall not attempt to deny or defeat such personal jurisdiction by
motion or other request for leave from any such 

  
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court and (iv) agrees not to bring any action or proceeding arising out of or relating to this Agreement or any of the transactions contemplated by this Agreement in any other court. Each of
the parties hereto waives any defense of inconvenient forum to the maintenance of any action or proceeding so brought and waives any bond, surety or other security that might be required of any other party with respect thereto. Any party hereto may
make service on another party by sending or delivering a copy of the process to the party to be served at the address and in the manner provided for the giving of notices in Section 10.01. Nothing in this Section 10.10, however, shall
affect the right of any party to serve legal process in any other manner permitted by law. 
 (b) EACH PARTY ACKNOWLEDGES AND
AGREES THAT ANY CONTROVERSY WHICH MAY ARISE UNDER THIS AGREEMENT IS LIKELY TO INVOLVE COMPLICATED AND DIFFICULT ISSUES, AND THEREFORE EACH SUCH PARTY HEREBY IRREVOCABLY AND UNCONDITIONALLY WAIVES ANY RIGHT SUCH PARTY MAY HAVE TO A TRIAL BY JURY IN
RESPECT OF ANY LITIGATION DIRECTLY OR INDIRECTLY ARISING OUT OF OR RELATING TO THIS AGREEMENT, OR THE TRANSACTIONS CONTEMPLATED BY THIS AGREEMENT. EACH PARTY CERTIFIES AND ACKNOWLEDGES THAT (A) NO REPRESENTATIVE, AGENT OR ATTORNEY OF ANY OTHER
PARTY HAS REPRESENTED, EXPRESSLY OR OTHERWISE, THAT SUCH OTHER PARTY WOULD NOT, IN THE EVENT OF LITIGATION, SEEK TO ENFORCE THE FOREGOING WAIVER, (B) EACH PARTY UNDERSTANDS AND HAS CONSIDERED THE IMPLICATIONS OF THIS WAIVER, (C) EACH PARTY
MAKES THIS WAIVER VOLUNTARILY, AND (D) EACH PARTY HAS BEEN INDUCED TO ENTER INTO THIS AGREEMENT BY, AMONG OTHER THINGS, THE MUTUAL WAIVERS AND CERTIFICATIONS IN THIS SECTION 10.10. 

10.11. Foreign Exchange Conversions. If any amount to be paid, transferred, allocated, reimbursed or calculated pursuant to, or in
accordance with, the terms of this Agreement or any Exhibit or Schedule referred to herein is originally stated or expressed in a currency other than United States Dollars, then, for the purpose of determining the amount to be so paid, transferred,
allocated, reimbursed or calculated, such amount shall be converted into United States Dollars at the Bank of Canada’s noon exchange rate as of the Business Day immediately prior to (or, if no such quote exists on such Business Day, on the
closest Business Day prior to) the day on which the party required to make such payment, transfer, reimbursement or calculation first becomes obligated to do so hereunder. 
 [Remainder of page intentionally left blank] 

  
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 IN WITNESS WHEREOF, this Agreement has been duly executed by the parties hereto as of and on
the date first above written. 
  

			
	COMPANY:
	
	 ENOBIA PHARMA CORP.

		
	By:	 	/s/    Robert Heft        
		 	 Title: President and CEO

  

			
	 BUYER:

	
	 ALEXION PHARMACEUTICALS, INC.

		
	By:	 	/s/    Vikas Sinha        
		 	Title: Senior Vice President and CFO

  
 [Signature
Page to Stock Purchase Agreement] 

  

			
	SELLERS:
	
	ANDREA ACEL
		
		 	/s/    Andrea Acel        

  

			
	MOHAMAD AHMAD
		
		 	/s/    Mohamad Ahmad        

  

			
	SOPHIE BINETTE
		
		 	/s/    Sophie Binette        

  

			
	GUY BOILEAU
		
		 	/s/    Guy Boileau        

  

			
	ISABEL BOIVIN
		
		 	/s/    Isabel Boivin        

  

			
	FANNY BONIN
		
		 	/s/    Fanny Bonin        

  

			
	NATHALIE BRISSETTE
		
		 	/s/    Nathalie Brissette        
		
		 	

  
 [Signature
Page to Stock Purchase Agreement] 

  

			
	ALEXANDRE CARON
		
		 	/s/    Alexandre Caron        

  

			
	PAOLA CHABOT
		
		 	/s/    Paola Chabot        

  

			
	HELEN CHAN
		
		 	/s/    Helen Chan        

  

			
	LUC COURNOYER
		
		 	/s/    Luc Cournoyer        

  

			
	PHILIPPE CRINE
		
		 	/s/    Philippe Crine        

  

			
	OUMAR SALL DIALLO
		
		 	/s/    Oumar Sall Diallo        

  

			
	ANNIE DULUDE
		
		 	/s/    Annie Dulude        

  

			
	BERNHARD EGGIMANN
		
		 	/s/    Bernhard Eggimann        
		
		 	

  
 [Signature
Page to Stock Purchase Agreement] 

  

			
	NATACHA FIRENZE
		
		 	/s/    Natacha Firenze        

  

			
	JACQUELINE YEP GARCIA
		
		 	/s/    Jacqueline Yep Garcia        

  

			
	GEORGIA GERONTAKOS
		
		 	/s/    Georgia Gerontakos        

  

			
	FRANCINE GERVAIS
		
		 	/s/    Francine Gervais        

  

			
	JULIE GUIMOND
		
		 	/s/    Julie Guimond        

  

			
	ROBERT HEFT
		
		 	/s/    Robert Heft        

  

			
	GENEVIEVE HELIE
		
		 	/s/    Genevieve Helie        

  

			
	FRANCOIS HUDON-DAVID
		
		 	/s/    Francois Hudon-David        
		
		 	

  
 [Signature
Page to Stock Purchase Agreement] 

  

			
	SIMON JOUBERT
		
		 	/s/    Simon Joubert        

  

			
	MOUNIA KHYARI
		
		 	/s/    Mounia Khyari        

  

			
	ANNIE LEBLANC
		
		 	/s/    Annie Leblanc        

  

			
	ERIC LEBLANC
		
		 	/s/    Eric Leblanc        

  

			
	ISABELLE LEMIRE
		
		 	/s/    Isabelle Lemire        

  

			
	PIERRE LEONARD
		
		 	/s/    Pierre Leonard        

  

			
	LINE LESPERANCE
		
		 	/s/    Line Lesperance        

  

			
	THOMAS LOISEL
		
		 	/s/    Thomas Loisel        
		
		 	

  
 [Signature
Page to Stock Purchase Agreement] 

  

			
	LUC MAINVILLE
		
		 	/s/    Luc Mainville        

  

			
	CAROLINE MOREL
		
		 	/s/    Caroline Morel        

  

			
	MOWAFAK NASSANI
		
		 	/s/    Mowafak Nassani        

  

			
	DONALD PAQUETTE
		
		 	/s/    Donald Paquette        

  

			
	MARIE PARAT
		
		 	/s/    Marie Parat        

  

			
	JOHANNE PION
		
		 	/s/    Johanne Pion        

  

			
	TRUDIE RESCH
		
		 	/s/    Trudie Resch        

  

			
	ANNIE SALESSE
		
		 	/s/    Annie Salesse        
		
		 	

  
 [Signature
Page to Stock Purchase Agreement] 

  

			
	CLAUDE SARRAZIN
		
		 	/s/    Claude Sarrazin        

  

			
	CLAIRE VEZINA
		
		 	/s/    Claire Vezina        

  

			
	ANDREA WAKEFIELD
		
		 	/s/    Andrea Wakefield        
		
		 	

  
 [Signature
Page to Stock Purchase Agreement] 

 Exhibit A 
 Agreement and Plan of Merger 
 The Agreement and Plan of Merger was filed as Exhibit 2.1 to the
Current Report on Form 8-K filed by the Buyer on January 4, 2012. The First Amendment to the Agreement and Plan of Merger was filed concurrently with this Stock Purchase Agreement, as Exhibit 2.1 to the Current Report on Form 8-K. 

 Exhibit B 
 Form of Promissory Note 

 PROMISSORY NOTE 

 

					
	$                        	  		  	As of                     , 2012

 Reference is made to the Agreement and Plan of Merger dated as of December 28, 2011, as amended by a
First Amendment to Agreement and Plan of Merger dated January __, 2012 (as so amended, the “Merger Agreement”) by and among Enobia Pharma Corp., a Delaware corporation (the “Company”), Alexion Pharmaceuticals,
Inc., a Delaware corporation (the “Buyer”), EMRD Corporation, a Delaware corporation and a wholly owned subsidiary of the Buyer (the “Transitory Subsidiary”), and M. Luc Mainville, Jonathan Silverstein, Robert
Heft and David Bonita, solely in their capacity as the Stockholder Representatives (the “Stockholder Representatives”). 
 The undersigned,
                                         
                             (the “Option Holder”) has executed a Stock Option
Agreement with the Company dated                     , 20            , (the
“Stock Option Agreement”) and has outstanding options to purchase                         shares of
common stock, par value $0.001, of the Company (the “Options”). The Option Holder has irrevocably elected to exercise all of the Option Holder’s Options. The Company has agreed to accept this Note from the Option Holder
providing for the payment of the exercise price for the shares to be issued upon exercise of the Options. 
 Capitalized terms
unless otherwise defined herein shall have the same meaning as set forth in the Merger Agreement. 
 FOR VALUE RECEIVED, the
Option Holder promises to pay to the order of the Company, its successors and assigns, at its principal offices at 55 Cambridge Parkway, Suite 800, Cambridge, MA 02142, the principal sum
of                                        
                              and 00/100 United States Dollars
(US$                    ) with interest on the unpaid principal balance from the date hereof, at the annual rate of
                    percent (            %) per annum, but in no event
in excess of the maximum rate permitted by law. 
 1. PAYMENTS AND PREPAYMENTS. 

(a) Unless the maturity of this Note shall otherwise have been accelerated pursuant to paragraph 2 hereof, this Note shall mature and
all unpaid amounts shall be due and payable in full on the earliest to occur of (i) the Closing under the Merger Agreement, (ii) upon demand at any time after the termination of the Merger Agreement and (iii) December 31, 2015
(the “Maturity Date”). 
 (b) Payments and prepayments of principal and interest on this Note shall be made at
the offices of Enobia at 55 Cambridge Parkway, Suite 800, Cambridge, MA 02142, or such other place or places within the United States as may be specified in writing by the holder of this Note. 

 (c) If any payment on this Note becomes due and payable on a day other than a Business Day,
the maturity hereof shall be extended to the next succeeding Business Day. 
 (d) Option Holder shall not have the right to
prepay this Note in whole or in part. 
 2. Events of Default. In the event that: 

(a) Option Holder fails to make any payment of principal or interest required to be made on this Note when due; or 

(b) Option Holder (i) commences any case, proceeding or other action under any existing or future law of any jurisdiction, domestic
or foreign, relating to bankruptcy, insolvency, reorganization or relief of debtors, seeking to have an order for relief entered with respect to it, or seeking to adjudicate it a bankrupt or insolvent, or seeking reorganization, arrangement,
adjustment, winding-up, liquidation, dissolution, composition or other relief with respect to it or its debts, or seeking appointment of a receiver, trustee, custodian or other similar official for it or for all or any substantial part of its
assets, or shall make a general assignment for the benefit of its creditors, or (ii) is the debtor named in any other case, proceeding or other action of a nature referred to in clause (i) above which results in the entry of an order for
relief or any such adjudication or appointment or remains undismissed or unbonded for a period of sixty (60) days, or (iii) takes any action in furtherance of, or indicating its consent to, approval of, or acquiescence in, any of the acts
set forth in clause (i) or (ii) above, or (iv) shall generally not, or shall be unable to, or shall admit in writing its inability to pay his or her debts as they become due; 

then, and in any such event, and at any time thereafter, if such event shall then be continuing for more than ten (10) days after
receipt of written notice of such default by Option Holder, any holder of this Note may, by written notice to Option Holder, declare this Note due and payable, whereupon the entire unpaid balance and other indebtedness on account of this obligation
due together with accrued but unpaid interest, if any, of this Note shall be due and payable without presentment, demand, protest or other notice of any kind, all of which are hereby expressly waived. 

3. Miscellaneous: 
 (a) No failure by the holder of this Note to exercise any right under this Note, including any rights resulting from an event of default, shall operate as a waiver or otherwise prevent the holder from
exercising any of its rights under this Note at any other time, including the exercise by the holder of any rights at any time during the continuance of such event of default or on the occurrence of a subsequent event of default. 

 (b) If this Note or any part of this Note is not paid when due and if the holder incurs any
expenses in connection with its collection, Option Holder agrees to pay all reasonable expenses so incurred, including reasonable attorneys’ fees, paralegal fees, costs and expenses. 

(c) This Note and the rights and obligations of Option Holder and each holder hereunder shall be construed in accordance with and be
governed by the laws of the State of Delaware. 
 (d) Upon payment in full of all outstanding principal and interest due under
this Note, Option Holder’s obligations in respect of payment of this Note shall terminate and the holder shall promptly return this Note to Option Holder. 
 (e) The Option Holder expressly agrees that if the Maturity Date arises by virtue of the occurrence of the Closing under the Merger Agreement, then (i) this Note shall be paid in full from the
consideration otherwise payable to the Option Holder at the Share Closing under that certain Stock Purchase Agreement dated the date hereof by and among the Buyer, the Option Holder and certain other option holders of Enobia listed on Schedule
I attached thereto (the “Stock Purchase Agreement”) and (ii) the Option Holder expressly authorizes the Buyer to deduct the total principal amount and all accrued interest due under this Note from the consideration payable
to the Option Holder at the Share Closing and to pay said amount to the Company in satisfaction of this Note. 
 (f) From and
after the Closing, the Option Holder hereby releases and forever discharges the Buyer, the Transitory Subsidiary, the Company, the Surviving Corporation and any and all of their past, present and future affiliated entities, partners, shareholders,
independent contractors, parent corporations, subsidiary corporations, successors, agents, directors, officers, assigns, employees, representatives, insurers, attorneys, and all others (the “Released Parties”) of and from all claims,
demands, Damages, obligations, actions and causes of action, arising in connection with the grant or exercise of the Option Holder’s Company Options and the Tax treatment arising from the exercise thereof, including any withholding Tax paid or
payable by any Released Party in connection with the exercise thereof. The Option Holder acknowledges and agrees that, as a condition to the execution and delivery of this Note, the Option Holder has not relied on any Tax advice provided by any of
the Released Parties at any time whatsoever. The foregoing release expressly excludes any and all obligations of (a) the Buyer under the Stock Purchase Agreement, (b) any Released Party, to the extent that any of the organizational
documents of the Company or any of its Subsidiaries or Affiliates confer rights to indemnification and/or advancement of expenses, arising under, or in connection with such rights, (c) any Released Party, arising from or in connection with such
Seller’s employment by the Company or any of its Subsidiaries or affiliates, and (d) any Released Party for directors’ meeting fees and expenses for or in connection with such Seller’s services as a director of the Company or any
of its Subsidiaries. 

 IN WITNESS WHEREOF, Option Holder has caused this Note to be duly executed and delivered by
its officer thereunto duly authorized as of the date first above written. 
  

			
	“OPTION HOLDER”
	
	
	Signature
		
	Printed Name:	 	 
		
	Address:	 	 
		
		 	 
		
		 	 

 Schedule I

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