Document:

EXHIBIT
      4.31.3

     

    CONSULTING
      AGREEMENT

     

    This
      Consulting Agreement (“Agreement”) is by and between KKS-TU GmbH, a German
      Corporation (“KKS”),
      located at Otfried-Müller-Str. 45, 72076 Tubingen, Germany, and Gentium, an
      Italy Corporation (“Gentium”),
      located at Piazza XX Settembre 2, Villa Gardia (Como), Italy.

     

    
      	1.	
              Description
                of Services 

            

    

     

    As
      of the
      date hereof, KKS will provide Gentium with clinical and regulatory consulting
      services (“the Services”).

     

    KKS
      will
      use its best efforts to perform the Services in accordance with the highest
      standards of its field. KKS will work closely with Gentium by meetings,
      teleconferences and project reviews to assure that the Services are performed
      as
      desired.

     

    
      	2.	
              Performance
                of Services 

            

    

     

    The
      manner in which the Services are to be performed shall be based on Estimated
      Project Budgets (Appendix A “offer A-SC-OQ04/06); KKS shall work as many hours
      as may be reasonably necessary to fulfill KKS’s obligations under this
      Agreement. For translation of documents, KKS will inform Gentium on the reason
      why translation is needed for the scope of this agreement.

     

    
      	3.	
              Payment
                to KKS 

            

    

     

    By
      the
      5th day of every month, KKS shall submit an invoice to Gentium. All invoices
      will clearly define each task performed. Gentium shall pay KKS no later than
      30
      days following receipt of each invoice.

     

    
      	4.	
              Term/Termination
                

            

    

     

    This
      Agreement shall be effective for two years from the signature date. Either
      party
      may terminate this Agreement by serving the other party with 60 days prior
      written notice to that effect. In the event of breach that the other party
      breaches this Agreement and fails to cure that breach within 30 days of receipt
      of written notice to that effect.

     

    
      	5.	
              Relationship
                of Parties 

            

    

     

    It
      is
      understood by the parties that KKS is an independent contractor with respect
      to
      Gentium, and not an employee of Gentium. Gentium will not provide fringe
      benefits, including health insurance benefits, paid vacation or any other
      employee benefit, for the benefit of KKS or KKS’s employees.

    
      
        
        

      

      
        -1-

        
          

        

      

      
        
        

      

    

    
      	6.	
              Disclosure
                

            

    

     

    KKS
      and
      Gentium recognize that KKS may work on various and similar projects for other
      clients. It is understood and agreed that KKS will not disclose to Gentium
      any
      confidential information of past and present clients. Gentium will rely on
      KKS’s
      ethical judgment to avoid conflicts of interest. Notwithstanding the above,
      KKS
      is required to disclose any outside activities or interests, including ownership
      or participation in the development of prior inventions, that conflict with
      the
      best interests of Gentium. Prompt disclosure is required under this paragraph
      if
      the activity or interest is related, directly or indirectly, to any activity
      that KKS may be involved with on behalf of Gentium.

     

    
      	7.	
              KKS’s
                Employees and Subcontractors

            

    

     

    KKS’s
      employees and professionals with whom they have contracts, if any, who perform
      services for Gentium under this Agreement, shall also be bound by the provisions
      of this Agreement.

     

    
      	8.	
              Confidentiality
                

            

    

     

    
      	 	
              a.

            	
              Gentium
                recognizes that during the discussions leading up to this Agreement
                and
                during the term of this Agreement, KKS has acquired or will acquire
                from
                Gentium information which Gentium considers to be proprietary and
                confidential. For example, Gentium has or will have products, prices,
                business affairs, future plans, trade secrets, process information,
                customer lists, technical information, product design information,
                and
                other proprietary information (collectively, “Information”) which are
                valuable, special and unique assets of Gentium. KKS agrees that KKS
                will
                not at any time or in any manner, either directly or indirectly,
                [i] use
                any Information for KKS’s own benefit, (ii) use any Information other than
                for the purpose of performing its services under this Agreement,
                or (iii)
                divulge, disclose or communicate in any manner to any third party
                any
                Information without the prior written consent of Gentium. KKS will
                protect
                the Information and treat it as strictly confidential for a period
                of 10
                years. A violation of this paragraph shall be a material violation
                of this
                Agreement. 

            

    

     

    
      	 	
              b.

            	
              Unauthorized
                Disclosure of Information 

            

    

     

    If
      it
      appears that KKS has disclosed with fault [or has threatened to disclose)
      information in violation of this Agreement, Gentium shall be entitled to an
      injunction to restrain KKS from disclosing, in whole or in part, such
      Information, or from providing any services to any party to whom such
      Information has been disclosed or may be disclosed. Gentium shall not be
      prohibited by this provision from pursuing other remedies, including a claim
      for
      losses and damages. 

     

    
      	 	
              c.

            	
              Services
                by KKS to Third Parties 

            

    

     

    The
      parties recognize that KKS may provide consulting services to third parties.
      However, KKS is bound by the confidentiality provisions of this ..\f4-
      Agreement,

    
      
        
        

      

      
        -2-

        
          

        

      

      
        
        

      

    

    and
      KKS
      may not use the information, directly or indirectly, for the benefit of third
      parties.

     

    
      	9.	
              Indemnification/Hold
                Harmless 

            

    

     

    KKS
      will
      not be held liable for any loss, injury or damage incurred by Gentium or by
      a
      third party as a result of the performance of the Services and/or as a result
      of
      Gentium’s activities, provided that such loss, injury or damage does not arise
      from KKS’s gross negligence or willful misconduct or breach of this Agreement.
      This obligation shall survive the expiration or earlier termination of this
      Agreement.

     

    
      	10.	
              Notices
                

            

    

     

    All
      notices required or permitted under this Agreement shall be in writing and
      shall
      be deemed delivered when delivered in person or sent certified and return
      receipt requested, postage prepaid, addressed as follows:

     

    
      	 	
              Gentium:

               

            	
              Gentium

              22079
                Villa Guardia (Como), Italy

            
	 	 	 
	 	
              Attn:

            	
              Massimo
                Iacobelli, Scientific Director

            
	 	 	 
	 	
              KKS:

               

            	
              KKS-TU
                GmbH 

              Otfried-Müller-Str.
                45 

              72076
                Tübingen, Germany

            
	 	 	 
	 	
              Attn:

            	
              Prof.
                Dr. C. H. Gleiter, Managing Director

            
	 	 	 

    

    Such
      addresses may be changed from time to time by either party by providing written
      notice in the manner set forth above.

     

    
      	11.	
              Entire
                Agreement 

            

    

     

    This
      Agreement contains the entire agreement of the parties and there are no other
      promises or conditions in any other agreement whether oral or written. This
      Agreement supersedes any prior written or oral agreements between the
      parties.

     

    
      	12.	
              Amendment
                

            

    

     

    This
      Agreement may be modified or amended, if the amendment is made in writing and
      is
      signed by both parties.

     

    
      	13.	
              Severability
                

            

    

     

    If
      any
      provision of this Agreement shall be held to be invalid or unenforceable for
      any
      reason, the remaining provisions shall continue to be valid and enforceable.
      If
      a court finds that any provision of this Agreement is invalid or unenforceable,
      but that by

    
      
        
        

      

      
        -3-

        
          

        

      

      
        
        

      

    

    limiting
      such provision it would become valid and enforceable, then such provision shall
      be deemed to be written, construed and enforced as so limited.

     

    
      	14.	
              Waiver
                of Contractual Right 

            

    

     

    The
      failure of either party to enforce any provision of this Agreement shall not
      be
      construed as a waiver or limitation of that party’s right to subsequently
      enforce and compel strict compliance with every provision of this
      Agreement.

     

    
      	15.	
              Governing
                law and jurisdiction 

            

    

     

    This
      Agreement shall be governed by and construed in accordance with the laws of
      Italy, without reference to any conflicts of law principles therein, and the
      courts of Como shall have sole jurisdiction in the event of a dispute arising
      between the parties in that regard.

     

    
      	16.	
              Inventions
                

            

    

     

    Any
      and
      all inventions, discoveries, reports or work product which KKS may make during
      the term of this Agreement relating to the subject matter hereafter (the
“Inventions”) and all know-how which KKS may develop in connection therewith
      shall be Gentium’s property and shall be disclosed promptly and fully to Gentium
      in writing.

     

    The
      obligations set forth in this paragraph shall survive the termination of
      expiration of this Agreement.

     

    
      	 	
              For:

            	
              Gentium.

            	 	 
	 	 	 	 	 
	 	
              By:

            	
              /s/
                Laura Iris Ferro 
                

              

              Laura
                Iris Ferro

            	
              Date:

            	
              Feb.
                13, 2006
                

              

               

            
	 	
              Title:

            	
              President

            	 	 
	 	 	 	 	 
	 	
              For:

            	
              KKS-TU
                GmbH

            	 	 
	 	 	 	 	 
	 	
              By:

            	
              /s/
                Prof. Dr. C. H. Gleiter 
                

              

              Prof.
                Dr. C. H. Gleiter

            	
              Date:

            	
              Jan.
                25, 2006
                

              

            
	 	
              Title:

            	
              Managing
                Director

            	 	 

    

     

    
      
        
        

      

      -4-Exhibit
        4.42

       

    

    GENTIUM
      S.p.A.

     

    2007
      STOCK OPTION PLAN

    

    
      
        
        

      

      
        
        

        
          

        

      

      
        
        

      

    

    TABLE
      OF CONTENTS

     

    
      
        	
                SECTION
                  1.

              	
                PURPOSE

              	
                1

              
	
                SECTION
                  2.

              	
                DEFINITIONS

              	
                1

              
	
                (a)

              	
                “ADS”

              	
                1

              
	
                (b)

              	
                “Affiliate”

              	
                1

              
	
                (c)

              	
                “Award”

              	
                1

              
	
                (d)

              	
                “Award
                  Agreement”

              	
                1

              
	
                (e)

              	
                “Board”

              	
                1

              
	
                (f)

              	
                “Change
                  In Control”

              	
                1

              
	
                (g)

              	
                “Code”

              	
                2

              
	
                (h)

              	
                “Committee”

              	
                2

              
	
                (i)

              	
                “Company”

              	
                2

              
	
                (j)

              	
                “Consultant”

              	
                2

              
	
                (k)

              	
                “Corporate
                  Transaction”

              	
                2

              
	
                (l)

              	
                “Covered
                  Employee”

              	
                3

              
	
                (m)

              	
                “Director”

              	
                3

              
	
                (n)

              	
                “Disability”

              	
                3

              
	
                (o)

              	
                “Employee”

              	
                3

              
	
                (p)

              	
                “Exchange
                  Act”

              	
                3

              
	
                (q)

              	
                “Exercise
                  Price”

              	
                3

              
	
                (r)

              	
                “Fair
                  Market Value”

              	
                3

              
	
                (s)

              	
                “Grant”

              	
                4

              
	
                (t)

              	
                “Incentive
                  Stock Option” or “ISO”

              	
                4

              
	
                (u)

              	
                “Key
                  Employee”

              	
                4

              
	
                (v)

              	
                “Non-Employee
                  Director”

              	
                4

              
	
                (w)

              	
                “Non-Employee
                  Independent Director”

              	
                4

              
	
                (x)

              	
                “Nonstatutory
                  Stock Option” or “NSO”

              	
                4

              
	
                (y)

              	
                “Option”

              	
                4

              
	
                (z)

              	
                “Optionee”

              	
                4

              
	
                (aa)

              	
                “Parent”

              	
                4

              
	
                (bb)

              	
                “Participant”

              	
                4

              
	
                (cc)

              	
                “Plan”

              	
                4

              
	
                (dd)

              	
                “Securities
                  Act”

              	
                4

              

      

       

      
        
          
          

        

        
          -i-

          
            

          

        

        
          
          

        

      

       

      
        	
                (ee)

              	
                “Service”

              	
                5

              
	
                (ff)

              	
                “Share”

              	
                5

              
	
                (gg)

              	
                “Stock
                  Option Agreement”

              	
                5

              
	
                (hh)

              	
                “Subsidiary”

              	
                5

              
	
                (ii)

              	
                “10-Percent
                  Shareholder”

              	
                5

              
	
                SECTION
                  3.

              	
                ADMINISTRATION

              	
                5

              
	
                (a)

              	
                Administration
                  by Board.

              	
                5

              
	
                (b)

              	
                Powers
                  of Board

              	
                5

              
	
                (c)

              	
                Delegation
                  to Committee

              	
                6

              
	
                (d)

              	
                Effect
                  of Board’s Decision

              	
                6

              
	
                (e)

              	
                Indemnification

              	
                6

              
	
                SECTION
                  4.

              	
                ELIGIBILITY

              	
                7

              
	
                (a)

              	
                General
                  Rules

              	
                7

              
	
                (b)

              	
                Incentive
                  Stock Options

              	
                7

              
	
                (c)

              	
                Non-Employee
                  Director Options

              	
                7

              
	
                SECTION
                  5.

              	
                SHARES
                  SUBJECT TO PLAN

              	
                8

              
	
                (a)

              	
                Basic
                  Limitation

              	
                8

              
	
                (b)

              	
                Additional
                  Shares

              	
                8

              
	
                (c)

              	
                [Limits
                  on Options

              	
                8

              
	
                SECTION
                  6.

              	
                TERMS
                  AND CONDITIONS OF OPTIONS

              	
                8

              
	
                (a)

              	
                Stock
                  Option Agreement

              	
                8

              
	
                (b)

              	
                Number
                  of Shares

              	
                8

              
	
                (c)

              	
                Exercise
                  Price

              	
                8

              
	
                (d)

              	
                Exercisability
                  and Term

              	
                8

              
	
                (e)

              	
                Modifications
                  or Assumption of Options

              	
                9

              
	
                (f)

              	
                Transferability
                  of Options

              	
                9

              
	
                (g)

              	
                Restrictions
                  on Transfer

              	
                9

              
	
                (h)

              	
                Incentive
                  Stock Option $100,000 Limitation

              	
                9

              
	
                SECTION
                  7.

              	
                PROTECTION
                  AGAINST DILUTION

              	
                9

              
	
                (a)

              	
                Adjustments

              	
                9

              
	
                (b)

              	
                Participant
                  Rights

              	
                10

              
	
                SECTION
                  8.

              	
                EFFECT
                  OF A CORPORATE TRANSACTION

              	
                10

              

      

       

      
        
          
          

        

        
          -ii-

          
            

          

        

        
          
          

        

      

       

      
        	
                (a)

              	
                Merger
                  or Reorganization

              	
                10

              
	
                (b)

              	
                Acceleration

              	
                10

              
	
                SECTION
                  9.

              	
                LIMITATIONS
                  ON RIGHTS

              	
                10

              
	
                (a)

              	
                Retention
                  Rights

              	
                10

              
	
                (b)

              	
                Shareholders’
                  Rights

              	
                10

              
	
                (c)

              	
                Regulatory
                  Requirements

              	
                10

              
	
                SECTION
                  10.

              	
                WITHHOLDING
                  TAXES

              	
                11

              
	
                (a)

              	
                Withholding
                  Obligations

              	
                11

              
	
                SECTION
                  11.

              	
                DURATION
                  AND AMENDMENTS

              	
                11

              
	
                (a)

              	
                Term
                  of the Plan

              	
                11

              
	
                (b)

              	
                Right
                  to Amend, Suspend or Terminate the Plan

              	
                11

              
	
                (c)

              	
                Right
                  to Amend Award

              	
                11

              
	
                SECTION
                  12.

              	
                EXECUTION

              	
                12

              

      

       

    

    
      
        
        

      

      
        -iii-

        
          

        

      

      
        
        

      

    

    GENTIUM
      S.p.A.

    2007
      STOCK OPTION PLAN

     

    SECTION
      1.  PURPOSE.

     

    This
      Gentium S.p.A. 2007 Stock Option Plan (the “Plan”) was adopted by the Board and
      became effective on March 26, 2007, subject to approval by the Company’s
      shareholders. 

     

    The
      purpose of the Plan is to promote the long-term success of the Company and
      the
      creation of shareholder value by offering Key Employees an opportunity to
      acquire a proprietary interest in the success of the Company, or to increase
      such interest, and to encourage such selected persons to continue to provide
      services to the Company and to attract new individuals with outstanding
      qualifications.

     

    The
      Plan
      seeks to achieve this purpose by providing for Awards in the form of Options
      (which may constitute Incentive Stock Options or Nonstatutory Stock
      Options).

     

    The
      Plan
      shall be governed by, and construed in accordance with, the laws of the Republic
      of Italy. Capitalized terms shall have the meaning provided in Section 2 of
      the
      Plan unless otherwise provided in this Plan or the applicable Award Agreement,
      or other applicable agreement. 

     

    SECTION
      2.  DEFINITIONS.

     

    (a)  “ADS”
      means
      American Depositary Shares issued pursuant to the Deposit Agreement dated June
      15, 2005 between the Company and Bank of New York, each ADS representing one
      Share.

     

    (b)  “Affiliate”
      means
      any
      entity other than a Subsidiary, if the Company and/or one or more Subsidiaries
      own not less than 50% of such entity. For purposes of determining an
      individual’s “Service,” this definition shall include any entity other than a
      Subsidiary, if the Company, a Parent and/or one or more Subsidiaries own not
      less than 50% of such entity.

     

    (c)  “Award”
      means
      any
      Grant of an Option.

     

    (d)  “Award
      Agreement” means
      a
      written agreement between the Company and a Participant evidencing the terms
      and
      conditions of a Grant of an individual Award. Each Award Agreement shall be
      subject to the terms and conditions of the Plan.

     

    (e)  “Board”
      means
      the
      Board of Directors of the Company, as constituted from time to
      time.

     

    (f)  “Change
      In Control” except
      as
      may otherwise be provided in an Award Agreement or other applicable agreement,
      means the occurrence of any of the following:

     

    
      
        
        

      

      
        1

        
          

        

      

      
        
        

      

    

     

    (i)  The
      consummation of a merger or consolidation of the Company with or into another
      entity or any other corporate reorganization, if more than 50% of the combined
      voting power of the continuing or surviving entity’s securities outstanding
      immediately after such merger, consolidation or other reorganization is owned
      by
      persons who were not shareholders of the Company immediately prior to such
      merger, consolidation or other reorganization;

     

    (ii)  The
      sale,
      transfer or other disposition of all or substantially all of the Company’s
      assets;

     

    (iii)  Any
      transaction as a result of which any person becomes the beneficiary, owner
      and/or “beneficial owner” (as defined in Rule 13d-3 under the Exchange Act),
      directly or indirectly, of securities of the Company representing at least
      20%
      of the total voting power represented by the Company’s then outstanding voting
      securities. For
      purposes of this Paragraph (iii), the term “person” shall indicate any person
      and/or entity except for:

     

    (A)  A
      trustee
      or other fiduciary holding securities under an employee benefit plan of the
      Company or a subsidiary of the Company;

     

    (B)  A
      corporation owned directly or indirectly by the shareholders of the Company
      in
      substantially the same proportions as their ownership of Shares;
      and

     

    (C)  The
      Company; or

     

    (iv)  A
      complete liquidation or dissolution of the Company.

     

    (g)  “Code”
      means
      the
      United States Internal Revenue Code of 1986, as amended.

     

    (h)  “Committee”
      means
      a
      committee consisting of one or more members of the Board that is appointed
      by
      the Board (as described in Section 3) to administer the Plan.

     

    (i)  “Company”
      means
      Gentium S.p.A., a stock corporation organized under the laws of the Republic
      of
      Italy.

     

    (j)  “Consultant”
      means
      an
      individual who performs bona fide services to the Company, a Parent, a
      Subsidiary or an Affiliate other than as an Employee or Director or Non-Employee
      Director.

     

    (k)  “Corporate
      Transaction” means
      the
      occurrence, in a single transaction or in a series of related transactions,
      of
      any one or more of the following events:

     

    (i)  a
      sale or
      other
      disposition of all or substantially all, as determined by the Board in its
      discretion, of the consolidated assets of the Company and its
      Subsidiaries;

     

    
      
        
        

      

      
        2

        
          

        

      

      
        
        

      

    

     

    (ii)  a
      merger,
      consolidation or similar transaction following which the Company is not the
      surviving corporation; or

     

    (iii)  a
      merger,
      consolidation or similar transaction following which the Company is the
      surviving corporation but the outstanding shares thereof immediately preceding
      the merger, consolidation or similar transaction are converted or exchanged
      by
      virtue of the merger, consolidation or similar transaction into other property,
      whether in the form of securities, cash or otherwise.

     

    (l)  “Covered
      Employee” means
      each of the chief executive officer and the 4 (four) other highest compensated
      officers of the Company.

     

    (m)  “Director”
      means
      a
      member of the Board who is also an Employee.

     

    (n)  “Disability”
      means
      that the Key Employee is unable to engage in any substantial gainful activity
      by
      reason of any medically determinable physical or mental impairment which can
      be
      expected to result in death or which has lasted or can be expected to last
      for a
      continuous period of not less than 12 months.

     

    (o)  “Employee”
      means
      any
      individual who is an employee of the Company, a Parent, a Subsidiary or an
      Affiliate. Mere service as a Director or payment of a director’s fee by the
      Company or an Affiliate shall not be sufficient to constitute “employment” by
      the Company or an Affiliate.

     

    (p)  “Exchange
      Act” means
      the
      United States Securities Exchange Act of 1934, as amended.

     

    (q)  “Exercise
      Price” means,
      the amount for which a Share may be purchased upon exercise of such Option,
      as
      specified in the applicable Stock Option Agreement.

     

    (r)  “Fair
      Market Value” means
      the
      market price of Shares, determined by the Committee as follows:

     

    (i)  If
      ADSs
      were traded on a stock exchange on the date in question, then the Fair Market
      Value shall be equal to the closing price reported by the applicable composite
      transactions report for such date;

     

    (ii)  If
      the
      ADSs were traded over-the-counter on the date in question and were classified
      as
      a national market issue or small cap issue, then the Fair Market Value shall
      be
      equal to the closing price quoted by the NASDAQ system for such
      date;

     

    (iii)  If
      the
      ADSs were traded over-the-counter on the date in question but were not
      classified as a national market issue, then the Fair Market Value shall be
      equal
      to the mean between the last reported representative bid and asked prices quoted
      by the applicable trading market for such date; and

     

    
      
        
        

      

      
        3

        
          

        

      

      
        
        

      

    

     

    (iv)  If
      none
      of the foregoing provisions is applicable, then the Fair Market Value shall
      be
      determined by the Committee in good faith on such basis as it deems
      appropriate.

     

    Whenever
      possible, the determination of Fair Market Value by the Committee shall be
      based
      on the prices reported in the Wall
      Street Journal.
      Such
      determination shall be conclusive and binding on all persons.

     

    (s)  “Grant”
      means
      any
      grant of an Award under the Plan.

     

    (t)  “Incentive
      Stock Option” or “ISO” means
      an
      incentive stock option described in Code section 422(b).

     

    (u)  “Key
      Employee” means
      an
      Employee, Director, Non-Employee Director or Consultant who has been selected
      by
      the Committee to receive an Award under the Plan.

     

    (v)  “Non-Employee
      Director” means
      a
      member of the Board who is not an Employee.

     

    (w)  “Non-Employee
      Independent Director” means
      a
      Non-Employee Director who: 

     

    (i)  is
      not a
      current Employee, is not a former Employee who received compensation for prior
      services in the current year, and has not been an officer of the Company;

     

    (ii)  did
      not
      directly or indirectly receive more than $60,000 in remuneration from the
      Company during the current year, other than director fees; and

     

    (iii)  did
      not
      receive more than $60,000 in remuneration from the Company during the prior
      year, other than director fees.

     

    (x)  “Nonstatutory
      Stock Option” or “NSO” means
      a
      stock option that is not an ISO.

     

    (y)  “Option”
      means
      an
      ISO or NSO granted under the Plan entitling the Optionee to purchase
      Shares.

     

    (z)   “Optionee”
      means
      an
      individual, estate or other entity that holds an Option.

     

    (aa)  “Parent”
      means
      a
“parent corporation,” whether now or hereafter existing, as defined in
      section 424(e) of the Code.

     

    (bb)  “Participant”
      means
      an
      individual or estate or other entity that holds an Award.

     

    (cc)  “Plan”
      means
      this Gentium S.p.A. 2007 Stock Option Plan as it may be amended from time to
      time.

     

    (dd)  “Securities
      Act” means
      the
      United States Securities Act of 1933, as amended.

     

    
      
        
        

      

      
        4

        
          

        

      

      
        
        

      

    

     

    (ee)  “Service”
      means
      service as an Employee, Director, Non-Employee Director or Consultant. A change
      in the capacity in which the Participant renders service to the Company or
      an
      Affiliate as an Employee, Consultant, Director or Non-Employee Director or
      a
      change in the entity for which the Participant renders such service, provided
      that there is no interruption or termination of the Participant’s service with
      the Company or an Affiliate, shall not terminate a Participant’s Service. For
      example, a change in status from an Employee of the Company to a Consultant
      of
      an Affiliate or a Director shall not constitute an interruption of
      Service.

     

    (ff)  “Share”
      means
      one
      ordinary share of the Company.

     

    (gg)  “Stock
      Option Agreement” means
      the
      agreement described in Section 6 evidencing each Grant of an
      Option.

     

    (hh)  “Subsidiary”
      means
      a
“subsidiary corporation,” whether now or hereafter existing, as defined in
      section 424(f) of the Code.

     

    (ii)  “10-Percent
      Shareholder” means
      an
      individual who owns more than ten percent (10%) of the total combined voting
      power of all classes of outstanding stock of the Company, its Parent or any
      of
      its Subsidiaries. In determining stock ownership, the attribution rules of
      section 424(d) of the Code shall be applied.

     

    SECTION
      3.  ADMINISTRATION.

     

    (a)  Administration
      by Board. The
      Board
      shall administer the Plan unless and until the Board delegates administration
      to
      a Committee, as provided in Section 3(c).

     

    (b)  Powers
      of Board. The
      Board
      shall have the power, subject to, and within the limitations of, the express
      provisions of the Plan:

     

    (i)  to
      determine from time to time which of the persons eligible under the Plan shall
      be granted Awards; when and how each Award shall be granted; the provisions
      of
      each Award granted (which need not be identical), including the time or times
      when a person shall be permitted to receive Shares pursuant to an Award; and
      the
      number of Shares with respect to which an Award shall be granted to each such
      person;

     

    (ii)  to
      construe and interpret the Plan, and Awards granted under it, and to establish,
      amend and revoke rules and regulations for its administration. The Board, in
      the
      exercise of this power, may correct any defect, omission or inconsistency in
      the
      Plan or in any Award Agreement, in a manner and to the extent it shall deem
      necessary or expedient to make the Plan fully effective;

     

    (iii)  to
      amend
      the Plan or an Award as provided in Section 11;

     

    (iv)  to
      terminate or suspend the Plan as provided in Section 11;
      and

     

    
      
        
        

      

      
        5

        
          

        

      

      
        
        

      

    

     

    (v)  generally,
      to exercise such powers and to perform such acts as the Board deems necessary
      or
      expedient to promote the best interests of the Company which are not in conflict
      with the provisions of the Plan.

     

    (c)  Delegation
      to Committee.

     

    (i)  The
      Board
      may delegate administration of the Plan to a Committee or Committees of the
      Board, and the term “Committee” shall apply to persons to whom such authority
      has been delegated. If administration is delegated to a Committee, the Committee
      shall have, in connection with the administration of the Plan, the powers
      theretofore possessed by the Board (and references in this Plan to the Board
      shall thereafter be to the Committee), subject, however, to such resolutions,
      not inconsistent with the provisions of the Plan, as may be adopted from time
      to
      time by the Board. The Board may abolish the Committee at any time and revest
      in
      the Board the administration of the Plan.

     

    (ii)  With
      respect to Awards granted to any Covered Employee, the Committee shall consist
      of those individuals who are
      outside Non-Employee Independent Directors. 

     

    The
      Board
      may also appoint one or more separate committees of the Board, each composed
      of
      directors of the Company who need not to be outside Non-Employee Independent
      Directors.

     

    Notwithstanding
      the foregoing, the Board shall constitute the Committee and shall administer
      the
      Plan with respect to all Awards granted to Non-Employee Directors.

     

    (d)  Effect
      of Board’s Decision. All
      determinations, interpretations and constructions made by the Board are not
      subject to review by any person and shall be final, binding and conclusive
      on
      all persons pursuant to the relevant Italian law provisions.

     

    (e)  Indemnification.
      Each
      member of the Committee, or of the Board, shall be indemnified and held harmless
      by the Company against and from (i) any loss, cost, liability, or expense
      that may be imposed upon or reasonably incurred by him or her in connection
      with
      or resulting from any claim, action, suit, or proceeding to which he or she
      may
      be a party or in which he or she may be involved by reason of any action taken
      or failure to act under the Plan or any Award Agreement, and (ii) from any
      and all amounts paid by him or her in settlement thereof, with the Company’s
      approval, or paid by him or her in satisfaction of any judgment in any such
      claim, action, suit, or proceeding against him or her, provided he or she shall
      give the Company an opportunity, at its own expense, to handle and defend the
      same before he or she undertakes to handle and defend it on his or her own
      behalf, except for the case of fraud or gross negligence. The foregoing right
      of
      indemnification shall not be exclusive of any other rights of indemnification
      to
      which such persons may be entitled under the Company’s Certificate of
      Incorporation or Bylaws, by contract, as a matter of law, or otherwise, or
      under
      any power that the Company may have to indemnify them or hold them
      harmless.

     

    
      
        
        

      

      
        6

        
          

        

      

      
        
        

      

    

     

    SECTION
      4.  ELIGIBILITY.

     

    (a)  General
      Rules. Only
      Employees, Directors, Non-Employee Directors and Consultants shall be eligible
      for designation as Key Employees by the Committee.

     

    (b)  Incentive
      Stock Options. A
      Key
      Employee who is a 10-Percent Shareholder shall not be eligible for the grant
      of
      an ISO unless the requirements set forth in Sections
      6(c) and 6(d) with respect to ISOs.

     

    (c)  Non-Employee
      Director Options. Non-Employee
      Directors shall also be eligible to receive Options as described in this Section
      4(c).

     

    (i)  Each
      Non-Employee Director shall automatically be granted an NSO to purchase 10,000
      Shares (subject to adjustment under Section 7(a)) as a result of his or her
      initial election as a Non-Employee Director. All NSOs granted pursuant to this
      Section 4(c)(i) shall vest and become exercisable, provided the individual
      is
      serving as a Non-Employee Director of the Company as of the vesting date, as
      follows: one-third of the total Shares subject to the NSO (rounded to nearest
      whole number) one year from the date of grant, then in 24 equal monthly
      installments commencing on the date one month and one year after the date of
      grant.

     

    (ii)  Upon
      the
      conclusion of each regular annual meeting of the Company’s shareholders
      following his or her initial election, each eligible Non-Employee Director
      who
      is re-elected as a Non-Employee Director shall receive an NSO to purchase
      5,000 Shares
      (subject to adjustment under Section 11(a)). All NSOs granted pursuant to this
      Section 4(c)(ii) shall vest and become exercisable provided the individual
      is
      serving as a Non-Employee Director of the Company as of the vesting date as
      follows: one-twelfth of the total Shares subject to the NSO (rounded to nearest
      whole number) on each monthly anniversary of the date of grant.

     

    (iii)  All
      NSOs
      granted to Non-Employee Directors under this Section 4(c) shall become
      exercisable in full in the event of Change in Control with respect to the
      Company.

     

    (iv)  The
      Exercise Price under all NSOs granted to a Non-Employee Director under this
      Section 4(c) shall be equal to one hundred percent (100%) of the Fair Market
      Value of a Share on the date of grant.

     

    (v)  All
      NSOs
      granted to a Non-Employee Director under this Section 4(c) shall terminate
      on
      the earlier of:

     

    (A)  March
      26,
      2022; or

     

    (B)  The
      date
      ninety (90) days after the termination of such Non-Employee Director’s Service
      for any reason.

     

    
      
        
        

      

      
        7

        
          

        

      

      
        
        

      

    

     

    SECTION
      5.  SHARES
      SUBJECT TO PLAN.

     

    (a)  Basic
      Limitation. Subject
      to the provisions of Section 7(a), the stock issuable under the Plan shall
      be
      authorized but unissued Shares or treasury Shares (the latter, within the limits
      set forth under Italian law). The aggregate number of Shares reserved for Awards
      under the Plan shall not exceed 1,000,000 Shares. 

     

    (b)  Additional
      Shares. If
      Awards
      are forfeited or terminate for any other reason before being exercised, then
      the
      Shares underlying such Awards shall again become available for Awards under
      the
      Plan. 

     

    SECTION
      6.  TERMS
      AND CONDITIONS OF OPTIONS.

     

    (a)  Stock
      Option Agreement. Each
      Grant of an Option under the Plan shall be evidenced by a Stock Option Agreement
      between the Optionee and the Company. Such Option shall be subject to all
      applicable terms and conditions of the Plan and may be subject to any other
      terms and conditions that are not inconsistent with the Plan and that the
      Committee deems appropriate for inclusion in a Stock Option Agreement. The
      provisions of the various Stock Option Agreements entered into under the Plan
      need not be identical. A Stock Option Agreement may provide that new Options
      will be granted automatically to the Optionee when he or she exercises the
      prior
      Options. The Stock Option Agreement shall also specify whether the Option is
      an
      ISO or an NSO.

     

    (b)  Number
      of Shares. Each
      Stock Option Agreement shall specify the number of Shares that are subject
      to
      the Option and shall provide for the adjustment of such number in accordance
      with Section 7(a).

     

    (c)  Exercise
      Price. An
      Option’s Exercise Price shall be established by the Board or the Committee and
      set forth in a Stock Option Agreement. The Exercise Price of an ISO shall not
      be
      less than the higher of (i) 100% of the Fair Market Value (110% for 10-Percent
      Shareholders) of a Share on the date of Grant, (ii) an amount corresponding,
      as
      of the date of exercise, to Euro 3.02 per Share and (iii) an amount
      corresponding, as of the date of exercise, to the nominal value of each Share.
      In the case of an NSO, a Stock Option Agreement may specify an Exercise Price
      that varies in accordance with a predetermined formula while the NSO is
      outstanding.
      All
      Exercise Prices must be paid in cash in compliance with the relevant Italian
      law
      provisions.

     

    (d)  Exercisability
      and Term. Each
      Stock Option Agreement shall specify the date when all or any installment of
      the
      Option is to become exercisable. The Stock Option Agreement shall also specify
      the term of the Option; provided that the term of an ISO shall in no event
      end
      after March 26,
      2022.
      No Option can be exercised after the expiration date provided in the applicable
      Stock Option Agreement. A Stock Option Agreement may provide for accelerated
      exercisability in the event of the Optionee’s death, Disability or retirement or
      other events and may provide for expiration prior to the end of its term in
      the
      event of the termination of the Optionee’s Service. A Stock Option Agreement may
      permit an Optionee to exercise an Option before it is vested, subject to the
      Company’s right of repurchase over any Shares acquired under the unvested
      portion of the Option (an “early exercise”), which right of repurchase shall (i)
      lapse at the same rate the Option would have vested had there been no early
      exercise and (ii) be exercised by the Company only within the strict limits
      and
      in compliance with the provisions of the Italian Civil Code. In no event shall
      the Company be required to issue fractional Shares upon the exercise of an
      Option.

     

    
      
        
        

      

      
        8

        
          

        

      

      
        
        

      

    

     

    (e)  Modifications
      or Assumption of Options. Within
      the limitations of the Plan, the Board or the Committee may modify, extend
      or
      assume outstanding stock options or may accept the cancellation of outstanding
      stock options (whether granted by the Company or by another issuer) in return
      for the grant of new Options for the same or a different number of Shares and
      at
      the same or a different Exercise Price. The foregoing notwithstanding, no
      modification of an Option shall, without the consent of the Optionee, alter
      or
      impair his or her rights or obligations under such Option.

     

    (f)  Transferability
      of Options. Except
      as
      otherwise provided in the applicable Stock Option Agreement and then only to
      the
      extent permitted by applicable law, no Option shall be transferable by the
      Optionee other than by will or by the laws of descent and distribution. Except
      as otherwise provided in the applicable Stock Option Agreement, an Option may
      be
      exercised during the lifetime of the Optionee only or by the guardian or legal
      representative of the Optionee. No Option or interest therein may be assigned,
      pledged or hypothecated by the Optionee during his or her lifetime, whether
      by
      operation of law or otherwise, or be made subject to execution, attachment
      or
      similar process.

     

    (g)  Restrictions
      on Transfer. Any
      Shares issued upon exercise of an Option shall be subject to such rights of
      repurchase, rights of first refusal and other transfer restrictions as the
      Committee may determine. Such restrictions shall apply in addition to any
      restrictions that may apply to holders of Shares generally and shall also comply
      to the extent necessary with applicable law.

     

    (h)  Incentive
      Stock Option $100,000 Limitation. To
      the
      extent that the aggregate Fair Market Value (determined at the time of grant)
      of
      the Shares with respect to which ISOs are exercisable for the first time by
      any
      Optionee during any calendar year (under all plans of the Company and its
      Affiliates) exceeds one hundred thousand dollars ($100,000), the Options or
      portions thereof which exceed such limit (according to the order in which they
      were granted) shall be treated as NSOs.

     

    SECTION
      7.  PROTECTION
      AGAINST DILUTION.

     

    (a)  Adjustments.
      In
      the
      event of a subdivision of the outstanding Shares, a declaration of a dividend
      payable in Shares, a declaration of a dividend payable in a form other than
      Shares in an amount that has a material effect on the price of Shares, a
      combination or consolidation of the outstanding Shares (by reclassification
      or
      otherwise) into a lesser number of Shares, a recapitalization, reorganization,
      merger, liquidation, spin-off or a similar occurrence (all without the receipt
      of consideration), the Board or the Committee shall make such adjustments as
      it,
      in its reasonable discretion, deems appropriate in order to prevent the dilution
      or enlargement of rights hereunder in one or more of:

     

    
      
        
        

      

      
        9

        
          

        

      

      
        
        

      

    

     

    (i)  the
      number of Shares subject to automatic Grants under Section 4(c) and the number
      of Shares available for future Awards under Section 5(a);

     

    (ii)  the
      number of Shares covered by each outstanding Award; or

     

    (iii)  the
      Exercise Price under each outstanding Option.

     

    (b)  Participant
      Rights. Except
      as
      provided in this Section 7, a recipient of an Award shall have no rights by
      reason of any issue by the Company of stock of any class or securities
      convertible into stock of any class, any subdivision or consolidation of shares
      of stock of any class, the payment of any stock dividend or any other increase
      or decrease in the number of shares of stock of any class.

     

    SECTION
      8.  EFFECT
      OF A CORPORATE TRANSACTION.

     

    (a)  Merger
      or Reorganization. In
      the
      event that the Company is a party to a Corporate Transaction, outstanding Awards
      shall be subject to the terms and conditions of the agreement memorializing
      such
      Corporate Transaction. Such agreement may provide, without limitation, for
      the
      assumption or substitution of outstanding Awards by the surviving corporation
      or
      its parent, for their continuation by the Company (if the Company is a surviving
      corporation), for accelerated vesting or for their cancellation with or without
      consideration.

     

    (b)  Acceleration.
      The
      Committee may determine, at the time of granting an Award, or thereafter, that
      such Award shall become fully exercisable as to all Shares subject to such
      Award
      in the event that a Change in Control occurs with respect to the
      Company.

     

    SECTION
      9.  LIMITATIONS
      ON RIGHTS.

     

    (a)  Retention
      Rights. Neither
      the Plan nor any Award granted under the Plan shall be deemed to give any
      individual a right to remain an Employee, Consultant or Director of the Company.
      The Company reserves the right to terminate the Service of any person at any
      time, and for any reason, subject to applicable laws, the Company’s
      Certificate of
      Incorporation and
      Bylaws and a written employment agreement (if any).

     

    (b)  Shareholders’
      Rights. A
      Participant shall have no dividend rights, voting rights or other rights as
      a
      stockholder with respect to any Shares covered by his or her Award prior to
      the
      issuance of a stock certificate, or similar means of representations of the
      Shares, for such Shares. No adjustment shall be made for cash dividends or
      other
      rights for which the record date is prior to the date when such certificate
      is
      issued, except as expressly provided in Section 7.

     

    (c)  Regulatory
      Requirements. Any
      other
      provision of the Plan notwithstanding, the obligation of the Company to issue
      Shares under the Plan shall be subject to all applicable laws, rules and
      regulations and such approval by any regulatory body as may be required. The
      Company reserves the right to restrict, in whole or in part, the delivery of
      Shares pursuant to any Award prior to the satisfaction of all legal requirements
      relating to the issuance of such Shares, to their registration, qualification
      or
      listing or to an exemption from registration, qualification or
      listing.

     

    
      
        
        

      

      
        10

        
          

        

      

      
        
        

      

    

     

    SECTION
      10.  WITHHOLDING
      TAXES.

     

    (a)  Withholding
      Obligations. The
      Company will be entitled to make any applicable withholding or deduction on
      account of any taxes or social security contributions due on benefits derived
      from the Plan pursuant to applicable law. A Participant shall make arrangements
      satisfactory to the Company for the satisfaction of such tax or social security
      obligations that arise in connection with his or her Award. The Company shall
      not be required to issue any Shares or make any cash payment under the Plan
      until such obligations are fully satisfied.

     

    SECTION
      11.  DURATION
      AND AMENDMENTS.

     

    (a)  Term
      of the Plan. The
      Plan
      shall become effective on March 26, 2007, subject to the approval of the
      Company’s shareholders. No Options shall be exercisable until such shareholder
      approval is obtained. In the event that the shareholders fail to approve the
      Plan within twelve (12) months after its adoption by the Board, any Awards
      made
      shall be null and void and no additional Awards shall be made. The Plan shall
      terminate on March 26, 2022 or on any earlier date pursuant to Section 11(b)
      of
      the Plan.

     

    (b)  Right
      to Amend, Suspend or Terminate the Plan. The
      Board
      may amend, suspend or terminate the Plan at any time and for any reason. The
      suspension or termination of the Plan, or any amendment thereof, shall not
      affect any Award previously granted under the Plan. No Awards shall be granted
      under the Plan after the Plan’s suspension or termination. An amendment of the
      Plan shall be subject to the approval of the Company’s shareholders only to the
      extent required by applicable laws, regulations or rules.

     

    (c)  Right
      to Amend Award. The
      Board
      at any time, and from time to time, may amend the terms of any one or more
      Awards; provided, however, that the rights under any Award shall not be impaired
      by any such amendment unless (i) the Company requests the consent of the
      Participant and (ii) the Participant consents in writing.

     

    
      
        
        

      

      
        11

        
          

        

      

      
        
        

      

    

     

    SECTION
      12.  EXECUTION.

     

    To
      record
      the adoption of the Plan by the Board, the Company has caused its duly
      authorized officer to execute this Plan on behalf of the Company.

    
      	 	 	 
	 	
              GENTIUM
                S.p.A.

            
	 
 	 
 	 
 
	 	By	/s/ Laura
              Iris Ferro, M.D. 
	 	 	
              

              Laura
                Iris Ferro, M.D.

            
	 	 	 
	 	Title 	President and Chief Executive
              Officer 

    

     

    
      
        
        

      

      
        12

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