Unassociated Document


    REGISTRATION
      RIGHTS AGREEMENT

     

    This
      Registration Rights Agreement (this “Agreement”)
      is
      made and entered into as of February 9, 2007, among Gentium S.p.A., an Italian
      joint stock company (the “Company”),
      and
      the purchasers signatory hereto (each such purchaser is a “Purchaser”
and
      collectively, the “Purchasers”).

     

    This
      Agreement is made pursuant to the Securities Subscription Agreement, dated
      as of
      February 6, 2007, among the Company and the Purchasers (the “Subscription
      Agreement”).

     

    The
      Company and the Purchasers hereby agree as follows:

     

    1.            
      Definitions.
      Capitalized terms used and not otherwise defined herein that are defined in
      the
      Subscription Agreement shall have the meanings given such terms in the
      Subscription Agreement. As used in this Agreement, the following terms shall
      have the following meanings:

     

    “ADSs”
means
      American Depository Shares, each a security representing one Ordinary Share
      deposited by the Company with The Bank of New York pursuant to the terms of
      the
      Deposit Agreement, issued by The Bank of New York pursuant to the terms of
      the
      Deposit Agreement.

     

    “Advice”
shall
      have the meaning set forth in Section 6(c).

     

    “Effectiveness
      Date”
      means
      the
      90th
      calendar
      day following the Closing, extended by a period of 30 additional days if the
      Commission reviews the Registration Statement; provided,
      however,
      in the
      event that the Company is notified by the Commission that the Registration
      Statement will not be reviewed or is no longer subject to further review and
      comments, the Effectiveness Date as to the Registration Statement shall be
      the
      fifth Trading Day following the date on which the Company is so notified if
      such
      date precedes the dates required above, unless such fifth Trading Day is after
      March 31, 2007, in which case such Effectiveness Date will be the 90th
      calendar
      day following the Closing.

     

    “Effectiveness
      Period”
shall
      have the meaning set forth in Section 2(a).

     

    “Filing
      Date”
means
      the 30th
      calendar
      day following the Closing.

     

    “Holder”
or
      “Holders”
means
      the holder or holders, as the case may be, from time to time of Registrable
      Securities.

     

    “Indemnified
      Party”
shall
      have the meaning set forth in Section 5(c).

     

    “Indemnifying
      Party”
shall
      have the meaning set forth in Section 5(c).

     

    “Liquidated
      Damages”
shall
      have the meaning set forth in Section 2(b).

     

    
      
         

      

      
         

        
          

        

      

      
         

      

    

     

    “Losses”
shall
      have the meaning set forth in Section 5(a).

     

    “Plan
      of Distribution”
shall
      have the meaning set forth in Section 2(a).

     

    “Proceeding”
means
      an action, claim, suit, investigation or proceeding (including, without
      limitation, an investigation or partial proceeding, such as a deposition),
      whether commenced or threatened.

     

    “Prospectus”
means
      the prospectus included in the Registration Statement (including, without
      limitation, a prospectus that includes any information previously omitted from
      a
      prospectus filed as part of an effective registration statement in reliance
      upon
      Rule 430A promulgated under the Securities Act), as amended or supplemented
      by
      any prospectus supplement, with respect to the terms of the offering of any
      portion of the Registrable Securities covered by the Registration Statement,
      and
      all other amendments and supplements to the Prospectus, including post-effective
      amendments, and all material incorporated by reference or deemed to be
      incorporated by reference in such Prospectus.

     

    “Registrable
      Securities”
means
      all of (i) the Shares, including ADSs representing the same, and (ii) any
      Shares, including ADSs representing the same, issued or issuable upon any stock
      split, dividend or other distribution, recapitalization or similar event with
      respect to the foregoing.

     

    “Registration
      Statement”
means
      the registration statement required to be filed hereunder, including the
      Prospectus, amendments and supplements to such registration statement or
      Prospectus, including pre- and post-effective amendments, all exhibits thereto,
      and all material incorporated by reference or deemed to be incorporated by
      reference in such registration statement.

     

    “Registration
      Default”
shall
      have the meaning set forth in Section 2(b).

     

    “Registration
      Default Date”
shall
      have the meaning set forth in Section 2(b).

     

    “Rule
      415”
means
      Rule 415 promulgated by the Commission pursuant to the Securities Act, as such
      Rule may be amended from time to time, or any similar rule or regulation
      hereafter adopted by the Commission having substantially the same purpose and
      effect as such Rule.

     

    “Rule
      424”
means
      Rule 424 promulgated by the Commission pursuant to the Securities Act, as such
      Rule may be amended from time to time, or any similar rule or regulation
      hereafter adopted by the Commission having substantially the same purpose and
      effect as such Rule.

     

    “Selling
      Security Holder Questionnaire”
shall
      mean the Questionnaire attached to this form as Annex B.

     

    “Shares”
shall
      mean the Ordinary Shares issued pursuant to the Subscription
      Agreement.

     

    
      
         

      

      
        2

        
          

        

      

      
         

      

    

     

    2.  Shelf
      Registration.

     

    (a)  On
      or
      prior to the Filing Date, the Company shall prepare and file with the Commission
      the Registration Statement covering the resale of the Registrable Securities
      (including the ADSs) for an offering to be made on a continuous basis pursuant
      to Rule 415. The Registration Statement shall be on Form F-3 (except if the
      Company is not then eligible to register for resale the Registrable Securities
      on Form F-3, in which case such registration shall be on another appropriate
      form in accordance herewith) and shall contain (unless otherwise directed by
      the
      Holders) substantially the “Plan
      of Distribution”
      attached hereto as Annex
      A.
      Subject
      to the terms of this Agreement, the Company shall use its best efforts to cause
      the Registration Statement to be declared effective under the Securities Act
      as
      promptly as possible after the filing thereof, but in any event prior to the
      Effectiveness Date, and shall use its best efforts to keep the Registration
      Statement continuously effective under the Securities Act until all Registrable
      Securities covered by the Registration Statement have been sold or may be sold
      without volume restrictions pursuant to Rule 144 as determined by the counsel
      to
      the Company pursuant to a written opinion letter to such effect, addressed
      and
      acceptable to the Company’s transfer agent and the affected Holders (the
“Effectiveness
      Period”).
      The
      Company shall immediately notify the Holders via facsimile or electronic mail
      of
      the effectiveness of the Registration Statement on the same Trading Day that
      the
      Company telephonically confirms effectiveness with the Commission, which shall
      be the date requested for effectiveness of the Registration Statement. The
      Company shall, by 9:30 AM Eastern Time on the Trading Day after the Effective
      Date (as defined in the Subscription Agreement), file a Form 424(b)(5) with
      the
      Commission. Each Holder agrees to furnish to the Company a completed Selling
      Security Holder Questionnaire simultaneously with the execution of this
      Agreement.

     

    
      
         

      

      
        3

        
          

        

      

      
         

      

    

     

    (b)  If
      (i)
      the Registration Statement is not filed on or prior to the Filing Date, or
      (ii)
      the Company fails to file with the Commission a request for acceleration in
      accordance with Rule 461 promulgated under the Securities Act, within five
      Trading Days of the date that the Company is notified (orally or in writing,
      whichever is earlier) by the Commission that the Registration Statement will
      not
      be “reviewed,” or not subject to further review (unless such fifth Trading Day
      is after March 31, 2007) or (iii) prior to the Effectiveness Date, the Company
      fails to file a pre-effective amendment and otherwise respond in writing to
      comments made by the Commission in respect of the Registration Statement within
      twenty Trading Days after the receipt of comments by or notice from the
      Commission that such amendment is required in order for the Registration
      Statement to be declared effective or (iv) the Registration Statement is not
      declared effective by the Commission by the Effectiveness Date or (v) the
      Registration Statement ceases to be effective or usable at any time during
      the
      Effectiveness Period (without being succeeded on the same date immediately
      by a
      post-effective amendment or supplement to the Registration Statement that cures
      such failure and that is itself, in the case of a post-effective amendment,
      immediately declared effective) or the Holders are not permitted to utilize
      the
      Prospectus therein to resell such Registrable Securities for twenty consecutive
      Trading Days or in any individual case an aggregate of thirty Trading Days
      during any twelve month period (which need not be consecutive Trading Days)
      (any
      of the foregoing being a “Registration
      Default”
and
      for
      purposes of clause (i) or (iv) the date on which such Registration Default
      occurs, or for purposes of clause (ii) the date on which such five Trading
      Day
      period is exceeded, or for purposes of clause (iii) the date which such twenty
      Trading Day period is exceeded, or for purposes of clause (v) the date on which
      such twenty or thirty Trading Day period, as applicable, is exceeded, each
      being
      a “Registration
      Default Date”)
      then,
      subject to Section 3(j), the Company shall pay to each Holder an amount in
      cash,
      as liquidated damages and not a penalty (“Liquidated
      Damages”)
      equal
      to 1% of the aggregate purchase price paid by such Holder pursuant to the
      Subscription Agreement for any Registrable Securities then held by such Holder
      per month of time between the Registration Default Date and the date such
      Registration Default is cured, prorated for any period less than one month;
      provided
      that
      such
      Liquidated Damages paid to each Holder may not exceed more than 10% of the
      purchase price paid by such Holder for its Registrable Securities. The foregoing
      represents the sole monetary remedy to any Holder in connection with any
      Registration Default. In no event shall the Company be required to pay
      Liquidated Damages in excess of the applicable amount set forth above,
      regardless of whether one or multiple Registration Defaults exists. The Company
      shall pay the Holders any Liquidated Damages accrued for the first month after
      a
      Registration Default Date within seven calendar days after the end of such
      month, and any Liquidated Damages accrued for any subsequent month within seven
      calendar days after the end of such month. If the Company fails to pay any
      liquidated damages pursuant to this Section in full within seven days after
      the
      date payable, the Company will pay interest thereon at a rate of 15% per annum
      (or such lesser maximum amount that is permitted to be paid by applicable law)
      to the Holder, accruing daily from the date such liquidated damages are due
      until such amounts, plus all such interest thereon, are paid in full. A
      Registration Default under clause (i) above shall be cured on the date that
      the
      Registration Statement is filed with the SEC; a Registration Default under
      clause (ii) above shall be cured on the date that the Company files such request
      for acceleration; a Registration Default under clause (iii) above shall be
      cured
      when the Company files such pre-effective amendment and otherwise respond in
      writing to comments made by the Commission; a Registration Default under clause
      (iv) above shall be cured on the date that the Registration Statement is
      declared effective by the SEC; and a Registration Default under clause (v)
      above
      shall be cured on the date that the Registration Statement is declared effective
      by the SEC or otherwise usable.

     

    (c)  Prior
      to
      the Effective Date of the Registration Statement the Company is required to
      file
      hereunder, the Company will file a Form F-6 if necessary to register or list
      any
      of the Registrable Securities thereunder, and will file such an F-6 in enough
      time so that such F-6 is effective on or prior to the Effective Date of the
      Registration Statement.

     

    3.  
Registration
      Procedures

     

    In
      connection with the Company’s registration obligations hereunder, the Company
      shall:

     

    
      
         

      

      
        4

        
          

        

      

      
         

      

    

     

    (a) (i)
      Prepare and file with the Commission such amendments, including post-effective
      amendments, to the Registration Statement and the Prospectus used in connection
      therewith as may be necessary to keep the Registration Statement continuously
      effective as to the applicable Registrable Securities for the Effectiveness
      Period; (ii) cause the related Prospectus to be amended or supplemented by
      any
      required Prospectus supplement (subject to the terms of this Agreement), and
      as
      so supplemented or amended to be filed pursuant to Rule 424; (iii) respond
      as
      promptly as reasonably possible to any comments received from the Commission
      with respect to the Registration Statement or any amendment thereto; and (iv)
      comply in all material respects with the provisions of the Securities Act and
      the Exchange Act with respect to the disposition of all Registrable Securities
      covered by the Registration Statement during the applicable period in accordance
      (subject to the terms of this Agreement) with the intended methods of
      disposition by the Holders thereof set forth in the Registration Statement
      as so
      amended or in such Prospectus as so supplemented.

     

    (b) Notify
      the Holders of Registrable Securities to be sold (which notice shall, pursuant
      to clauses (ii) through (v) hereof, be accompanied by an instruction to suspend
      the use of the Prospectus until the requisite changes have been made) as
      promptly as reasonably possible and (if requested by any such Person) confirm
      such notice in writing no later than one Trading Day following the day (i)(A)
      when a Prospectus or any Prospectus supplement or post-effective amendment
      to
      the Registration Statement is filed; and (B) with respect to the Registration
      Statement or any post-effective amendment, when the same has become effective;
      (ii) of any request by the Commission or any other Federal or state governmental
      authority for amendments or supplements to the Registration Statement or
      Prospectus or for additional information; (iii) of the issuance by the
      Commission or any other federal or state governmental authority of any stop
      order suspending the effectiveness of the Registration Statement covering any
      or
      all of the Registrable Securities or the initiation of any Proceedings for
      that
      purpose; (iv) of the receipt by the Company of any notification with respect
      to
      the suspension of the qualification or exemption from qualification of any
      of
      the Registrable Securities for sale in any jurisdiction, or the initiation
      or
      threatening of any Proceeding for such purpose; and (v) of the occurrence of
      any
      event or passage of time that makes the financial statements included in the
      Registration Statement ineligible for inclusion therein or any statement made
      in
      the Registration Statement or Prospectus or any document incorporated or deemed
      to be incorporated therein by reference untrue in any material respect or that
      requires any revisions to the Registration Statement, Prospectus or other
      documents so that, in the case of the Registration Statement or the Prospectus,
      as the case may be, it will not contain any untrue statement of a material
      fact
      or omit to state any material fact required to be stated therein or necessary
      to
      make the statements therein, in light of the circumstances under which they
      were
      made, not misleading; provided
      that any
      and all of such information provided pursuant to clause (v) above shall remain
      confidential to each Holder until such information otherwise becomes public,
      unless disclosure by a Holder is required by law; provide,
      further,
      notwithstanding each Holder’s agreement to keep such information confidential,
      the Holders make no acknowledgement that any such information is material,
      non-public information.

     

    (c) Use
      its
      best efforts to avoid the issuance of, or, if issued, obtain the withdrawal
      of
      (i) any order suspending the effectiveness of the Registration Statement, or
      (ii) any suspension of the qualification (or exemption from qualification)
      of
      any of the Registrable Securities for sale in any jurisdiction, at the earliest
      practicable moment.

     

    
      
         

      

      
        5

        
          

        

      

      
         

      

    

     

    (d) Furnish
      to each Holder, upon written request of such Holder, without charge, at least
      one conformed copy of the Registration Statement and each amendment thereto,
      including financial statements and schedules, all documents incorporated or
      deemed to be incorporated therein by reference to the extent requested by such
      Person, and all exhibits to the extent requested by such Person (including
      those
      previously furnished or incorporated by reference).

     

    (e) Promptly
      deliver to each Holder, upon written request of such Holder, without charge,
      as
      many copies of the Prospectus or Prospectuses (including each form of
      prospectus) and each amendment or supplement thereto as such Persons may
      reasonably request in connection with resales by the Holder of Registrable
      Securities. Subject to the terms of this Agreement, the Company hereby consents
      to the use of such Prospectus and each amendment or supplement thereto by each
      of the selling Holders in connection with the offering and sale of the
      Registrable Securities covered by such Prospectus and any amendment or
      supplement thereto, except after the giving of any notice pursuant to Section
      3(b).

     

    (f) Prior
      to
      any resale of Registrable Securities by a Holder, use its commercially
      reasonable efforts to register or qualify or cooperate with the selling Holders
      in connection with the registration or qualification (or exemption from the
      Registration or qualification) of such Registrable Securities for the resale
      by
      the Holder under the securities or Blue Sky laws of such jurisdictions within
      the United States as any Holder reasonably requests in writing, to keep each
      registration or qualification (or exemption therefrom) effective during the
      Effectiveness Period and to do any and all other acts or things reasonably
      necessary to enable the disposition in such jurisdictions of the Registrable
      Securities covered by each Registration Statement; provided that the Company
      shall not be required to qualify generally to do business in any jurisdiction
      where it is not then so qualified or subject the Company to any material tax
      in
      any such jurisdiction where it is not then so subject or file a general consent
      to service of process in any such jurisdiction.

     

    (g) If
      requested by the Holders, use its best efforts to cause its transfer agent
      to
      prepare and deliver certificates representing Registrable Securities to a
      transferee pursuant to the Registration Statement within three (3) Trading
      Days
      of delivery to the transfer agent of certificates bearing restrictive legends,
      which certificates shall be free, to the extent permitted by the Subscription
      Agreement, of all restrictive legends, and to enable such Registrable Securities
      to be in such denominations and registered in such names as any such Holders
      may
      request.

     

    (h) Upon
      the
      occurrence of any event contemplated by this Section 3, as promptly as
      reasonably possible under the circumstances taking into account the Company’s
      good faith assessment of any adverse consequences to the Company and its
      shareholders of the premature disclosure of such event, prepare a supplement
      or
      amendment, including a post-effective amendment, to the Registration Statement
      or a supplement to the related Prospectus or any document incorporated or deemed
      to be incorporated therein by reference, and file any other required document
      so
      that, as thereafter delivered, neither the Registration Statement nor such
      Prospectus will contain an untrue statement of a material fact or omit to state
      a material fact required to be stated therein or necessary to make the
      statements therein, in light of the circumstances under which they were made,
      not misleading. If the Company notifies the Holders in accordance with clauses
      (ii) through (v) of Section 3(b) above to suspend the use of any Prospectus
      until the requisite changes to such Prospectus have been made, then the Holders
      shall suspend use of such Prospectus. The Company will use its best efforts
      to
      ensure that the use of the Prospectus may be resumed as promptly as is
      practicable. The Company shall be entitled to exercise its right under this
      Section 3(h) to suspend the availability of the Registration Statement and
      Prospectus for a period not to exceed 90 days (which need not be consecutive
      days) in any 365 day period.

     

    
      
         

      

      
        6

        
          

        

      

      
         

      

    

     

    (i) Comply
      with all applicable rules and regulations of the Commission and the Trading
      Market.

     

    (j) The
      Company may require each selling Holder to furnish to the Company a certified
      statement as to the number of Ordinary Shares or ADSs beneficially owned by
      such
      Holder and, if required by the Commission, the person thereof that has voting
      and dispositive control over the Shares. During any periods that the Company
      is
      unable to meet its obligations hereunder with respect to the registration of
      the
      Registrable Securities solely because any Holder fails to furnish such
      information within three Trading Days of the Company’s request, any Liquidated
      Damages that are accruing at such time as to such Holder only shall be tolled
      and any Registration Default that may otherwise occur solely because of such
      delay shall be suspended as to such Holder only, until such information is
      delivered to the Company.

     

    (k) File
      such
      public reports as shall be required to satisfy the current public information
      requirements set forth in Rule 144 of the Securities Act until the earlier
      of
      the sale of the Registrable Securities under the Registration Statement or
      such
      time as the Registrable Securities are eligible for sale under Rule
      144(k).

    


    (l) If
      requested by a Purchaser, the Company shall (i) as soon as practicable
      incorporate in a prospectus supplement or post-effective amendment such
      information as such Purchaser reasonably requests to be included therein
      relating to the sale and distribution of Registrable Securities, including,
      without limitation, information with respect to the number of Registrable
      Securities being offered or sold, the purchase price being paid therefor and
      any
      other terms of the offering of the Registrable Securities to be sold in such
      offering, and (ii) as soon as practicable make all required filings of such
      prospectus supplement or post-effective amendment after being notified of the
      matters to be incorporated in such prospectus supplement or post-effective
      amendment.

    


    4.  Registration
      Expenses.
      All
      fees and expenses incident to the performance of or compliance with this
      Agreement by the Company shall be borne by the Company whether or not any
      Registrable Securities are sold pursuant to the Registration Statement. The
      fees
      and expenses referred to in the foregoing sentence shall include, without
      limitation, (i) all registration and filing fees (including, without limitation,
      fees and expenses (A) with respect to filings required to be made with the
      Trading Market on which the ADSs are then listed for trading and (B) in
      compliance with applicable state securities or Blue Sky laws reasonably agreed
      to by the Company in writing (including, without limitation, fees and
      disbursements of counsel for the Company in connection with Blue Sky
      qualifications or exemptions of the Registrable Securities and determination
      of
      the eligibility of the Registrable Securities for investment under the laws
      of
      such jurisdictions as requested by the Holders), (ii) printing expenses
      (including, without limitation, expenses of printing certificates for
      Registrable Securities and of printing prospectuses for a Holder if the printing
      of prospectuses is reasonably requested by such Holder), (iii) messenger,
      telephone and delivery expenses related to the Company's obligations hereunder,
      (iv) fees and disbursements of counsel for the Company, (v) Securities Act
      liability insurance, if the Company so desires such insurance, and (vi) fees
      and
      expenses of all other Persons retained by the Company in connection with the
      consummation of the transactions contemplated by this Agreement. In addition,
      the Company shall be responsible for all of its internal expenses incurred
      in
      connection with the consummation of the transactions contemplated by this
      Agreement (including, without limitation, all salaries and expenses of its
      officers and employees performing legal or accounting duties), the expense
      of
      any annual audit and the fees and expenses incurred in connection with the
      listing of the Registrable Securities on any securities exchange as required
      hereunder, including all fees and expenses of the Depositary. In no event shall
      the Company be responsible for any broker or similar commissions or, except
      to
      the extent provided for in the Transaction Documents, including Section 5.2
      of
      the Subscription Agreement, any legal fees or other costs of the
      Holders.

     

    
      
         

      

      
        7

        
          

        

      

      
         

      

    

     

    5.  Indemnification

     

    (a)  Indemnification
      by the Company.
      The
      Company shall, notwithstanding any termination of this Agreement, indemnify
      and
      hold harmless each Holder, the officers, directors, members, partners, agents,
      brokers (including brokers who offer and sell Registrable Securities as
      principal as a result of a pledge or any failure to perform under a margin
      call
      of Ordinary Shares), investment advisors and employees of each of them, each
      Person who controls any such Holder (within the meaning of Section 15 of the
      Securities Act or Section 20 of the Exchange Act) and the officers, directors,
      members, partners, agents and employees of each such controlling Person, to
      the
      fullest extent permitted by applicable law, from and against any and all losses,
      claims, damages, liabilities, judgments, fines, penalties, charges, costs
      (including, without limitation, reasonable attorneys’ fees) and expenses
      (collectively, “Losses”),
      as
      incurred, arising out of or relating to (i) any untrue or alleged untrue
      statement of a material fact contained in the Registration Statement, any
      Prospectus or any form of prospectus (including an issuer free-writing
      prospectus) or in any amendment or supplement thereto or in any preliminary
      prospectus, or arising out of or relating to any omission or alleged omission
      of
      a material fact required to be stated therein or necessary to make the
      statements therein (in the case of any Prospectus or form of prospectus or
      supplement thereto, in light of the circumstances under which they were made)
      not misleading, except to the extent, but only to the extent, that (a) such
      untrue statements or omissions are based solely upon information regarding
      such
      Holder furnished in writing to the Company by such Holder expressly for use
      therein, or to the extent that such information relates to such Holder or such
      Holder’s proposed method of distribution of Registrable Securities as set forth
      in Annex
      A
      hereto
      or any changes to Annex
      A
      hereto
      that are expressly approved in writing by such Holder expressly for use in
      the
      Registration Statement, such Prospectus or such form of Prospectus or in any
      amendment or supplement thereto or (b) in the case of an occurrence of an event
      of the type specified in Section 3(b)(ii)-(v), the use by such Holder of an
      outdated or defective Prospectus after the Company has notified such Holder
      in
      writing that the Prospectus is outdated or defective and prior to the receipt
      by
      such Holder of the Advice contemplated in Section 6(c);
      (ii)
      any violation by the Company of the Securities Act, or any other law, including,
      without limitation, any state securities laws, or any rule or regulation
      thereunder relating to the offer or sale of the Registrable Securities pursuant
      to the Registration Statement; or (iii) any material violation of this Agreement
      by the Company, its agents or representatives.
      The
      Company shall notify the Holders promptly of the institution, threat or
      assertion of any Proceeding arising from or in connection with the transactions
      contemplated by this Agreement of which the Company is aware.

     

    
      
         

      

      
        8

        
          

        

      

      
         

      

    

     

    (b)  Indemnification
      by Holders.
      Each
      Holder shall, notwithstanding any termination of this Agreement, severally
      and
      not jointly, indemnify and hold harmless the Company, its directors, officers,
      agents and employees, each Person who controls the Company (within the meaning
      of Section 15 of the Securities Act and Section 20 of the Exchange Act), and
      the
      directors, officers, agents or employees of such controlling Persons, to the
      fullest extent permitted by applicable law, from and against all Losses as
      to
      the extent arising solely out of or based solely upon (i) such Holder’s failure
      to comply with the prospectus delivery requirements of the Securities Act;
      or
      (ii) upon any untrue or alleged untrue statement of a material fact contained
      in
      any Registration Statement, any Prospectus, or any form of prospectus, or in
      any
      amendment or supplement thereto or in any preliminary prospectus, or arising
      out
      of or relating to any omission or alleged omission of a material fact required
      to be stated therein or necessary to make the statements therein not misleading
      to the extent that (1) such untrue statements or omissions are based solely
      upon
      information regarding such Holder furnished in writing to the Company by such
      Holder expressly for use therein, or to the extent that such information relates
      to such Holder or such Holder’s proposed method of distribution of Registrable
      Securities as set forth in Annex
      A
      hereto
      or any changes to Annex
      A
      hereto
      that are expressly approved in writing by such Holder expressly for use in
      the
      Registration Statement, such Prospectus or such form of Prospectus or in any
      amendment or supplement thereto or (2) in the case of an occurrence of an event
      of the type specified in Section 3(b)(ii)-(v), the use by such Holder of an
      outdated or defective Prospectus after the Company has notified such Holder
      in
      writing that the Prospectus is outdated or defective and prior to the receipt
      by
      such Holder of the Advice contemplated in Section 6(c). In no event shall the
      liability of any selling Holder hereunder be greater in amount than the dollar
      amount of the net proceeds received by such Holder upon the sale of the
      Registrable Securities covered by such Registration Statement giving rise to
      such indemnification obligation.

     

    (c)  Conduct
      of Indemnification Proceedings.
      If any
      Proceeding shall be brought or asserted against any Person entitled to indemnity
      hereunder (an “Indemnified
      Party”),
      such
      Indemnified Party shall promptly notify the Person from whom indemnity is sought
      (the “Indemnifying
      Party”)
      in
      writing, and the Indemnifying Party shall have the right to assume the defense
      thereof, including the employment of counsel reasonably satisfactory to the
      Indemnified Party and the payment of all fees and expenses incurred in
      connection with defense thereof; provided, that the failure of any Indemnified
      Party to give such notice shall not relieve the Indemnifying Party of its
      obligations or liabilities pursuant to this Agreement, except (and only) to
      the
      extent that it shall be finally determined by a court of competent jurisdiction
      (which determination is not subject to appeal or further review) that such
      failure shall have prejudiced the Indemnifying Party.

     

    
      
         

      

      
        9

        
          

        

      

      
         

      

    

     

    An
      Indemnified Party shall have the right to employ separate counsel in any such
      Proceeding and to participate in the defense thereof, but the fees and expenses
      of such counsel shall be at the expense of such Indemnified Party or Parties
      unless: (1) the Indemnifying Party has agreed in writing to pay such fees and
      expenses; (2) the Indemnifying Party shall have failed promptly to assume the
      defense of such Proceeding; (3) the Indemnifying Party shall have failed
      promptly to employ counsel reasonably satisfactory to such Indemnified Party
      in
      any such Proceeding; or (4) the named parties to any such Proceeding (including
      any impleaded parties) include both such Indemnified Party and the Indemnifying
      Party, and such Indemnified Party shall reasonably believe that a material
      conflict of interest is likely to exist if the same counsel were to represent
      such Indemnified Party and the Indemnifying Party (in which case, if such
      Indemnified Party notifies the Indemnifying Party in writing that it elects
      to
      employ separate counsel at the expense of the Indemnifying Party, the reasonable
      fees and expenses of one separate counsel shall be at the expense of the
      Indemnifying Party). The Indemnifying Party shall not be liable for any
      settlement of any such Proceeding effected without its written consent, which
      consent shall not be unreasonably withheld. No Indemnifying Party shall, without
      the prior written consent of the Indemnified Party, effect any settlement of
      any
      pending Proceeding in respect of which any Indemnified Party is a party, unless
      such settlement includes an unconditional release of such Indemnified Party
      from
      all liability on claims that are the subject matter of such
      Proceeding.

     

    The
      Indemnified Party shall promptly reimburse the Indemnifying Party for that
      portion of such fees and expenses applicable to such actions for which such
      Indemnified Party is not entitled to indemnification hereunder, determined
      based
      upon the relative faults of the parties.

     

    (d)  Contribution.
      If the
      indemnification under Section 5(a) or 5(b) is unavailable to an Indemnified
      Party or insufficient to hold an Indemnified Party harmless for any Losses,
      then
      each Indemnifying Party shall contribute to the amount paid or payable by such
      Indemnified Party, in such proportion as is appropriate to reflect the relative
      fault of the Indemnifying Party and Indemnified Party in connection with the
      actions, statements or omissions that resulted in such Losses as well as any
      other relevant equitable considerations. The relative fault of such Indemnifying
      Party and Indemnified Party shall be determined by reference to, among other
      things, whether any action in question, including any untrue or alleged untrue
      statement of a material fact or omission or alleged omission of a material
      fact,
      has been taken or made by, or relates to information supplied by, such
      Indemnifying Party or Indemnified Party, and the parties’ relative intent,
      knowledge, access to information and opportunity to correct or prevent such
      action, statement or omission. The amount paid or payable by a party as a result
      of any Losses shall be deemed to include, subject to the limitations set forth
      in Section 5(c), any reasonable attorneys’ or other reasonable fees or expenses
      incurred by such party in connection with any Proceeding to the extent such
      party would have been indemnified for such fees or expenses if the
      indemnification provided for in this Section was available to such party in
      accordance with its terms.

     

    
      
         

      

      
        10

        
          

        

      

      
         

      

    

     

    The
      parties hereto agree that it would not be just and equitable if contribution
      pursuant to this Section 5(d) were determined by pro rata allocation or by
      any
      other method of allocation that does not take into account the equitable
      considerations referred to in the immediately preceding paragraph.
      Notwithstanding the provisions of this Section 5(d), no Holder shall be required
      to contribute, in the aggregate, any amount in excess of the amount by which
      the
      proceeds actually received by such Holder from the sale of the Registrable
      Securities subject to the Proceeding exceeds the amount of any damages that
      such
      Holder has otherwise been required to pay by reason of such untrue or alleged
      untrue statement or omission or alleged omission, except in the case of fraud
      by
      such Holder.

     

    The
      indemnity and contribution agreements contained in this Section are in addition
      to any liability that the Indemnifying Parties may have to the Indemnified
      Parties.

     

    6.         
      Miscellaneous

     

    (a)  Remedies.
      In the
      event of a breach by the Company or by a Holder, of any of their obligations
      under this Agreement, each Holder or the Company, as the case may be, in
      addition to being entitled to exercise all rights granted by law and under
      this
      Agreement, including recovery of damages, will be entitled to specific
      performance of its rights under this Agreement. The Company and each Holder
      agree that monetary damages would not provide adequate compensation for any
      losses incurred by reason of a breach by it of any of the provisions of this
      Agreement and hereby further agrees that, in the event of any action for
      specific performance in respect of such breach, it shall waive the defense
      that
      a remedy at law would be adequate.

     

    (b)  Compliance.
      Each
      Holder covenants and agrees that it will comply with the prospectus delivery
      requirements of the Securities Act as applicable to it in connection with sales
      of Registrable Securities pursuant to the Registration Statement.

     

    (c)  Discontinued
      Disposition.
      Each
      Holder agrees by its acquisition of such Registrable Securities that, upon
      receipt of a notice from the Company of the occurrence of any event of the
      kind
      described in Section 3(b)(ii) through Section 3(c)(v), such Holder will
      forthwith discontinue disposition of such Registrable Securities under the
      Registration Statement until such Holder’s receipt of the copies of the
      supplemented Prospectus and/or amended Registration Statement or until it is
      advised in writing (the “Advice”)
      by the
      Company that the use of the applicable Prospectus may be resumed, and, in either
      case, has received copies of any additional or supplemental filings that are
      incorporated or deemed to be incorporated by reference in such Prospectus or
      Registration Statement. The
      Company agrees and acknowledges that any periods during which the Holder is
      required to discontinue the disposition of the Registrable Securities hereunder
      shall be subject to the provisions of Section 2(b). The
      Company will use its best efforts to ensure that the use of the Prospectus
      may
      be resumed as promptly as it practicable. 

     

    
      
         

      

      
        11

        
          

        

      

      
         

      

    

     

    (d)  Piggy-Back
      Registrations.
      If at
      any time during the Effectiveness Period there is not an effective Registration
      Statement covering all of the Registrable Securities and the Company shall
      determine to prepare and file with the Commission a registration statement
      relating to an offering for its own account or the account of others under
      the
      Securities Act of any of its equity securities, other than on Form F-4 or Form
      F-8 (each as promulgated under the Securities Act) or their then equivalents
      relating to equity securities to be issued solely in connection with any
      acquisition of any entity or business or equity securities issuable in
      connection with the stock option or other employee benefit plans, then the
      Company shall send to each Holder a written notice of such determination and,
      if
      within fifteen days after the date of such notice, any such Holder shall so
      request in writing, the Company shall include in such registration statement
      all
      or any part of such Registrable Securities such Holder requests to be
      registered; provided,
      however,
      that,
      the Company shall not be required to register any Registrable Securities
      pursuant to this Section 6(d) that are eligible for resale pursuant to Rule
      144(k) promulgated under the Securities Act or that are the subject of a
      then-effective Registration Statement.

     

    (e)  Amendments
      and Waivers.
      The
      provisions of this Agreement, including the provisions of this sentence, may
      not
      be amended, modified or supplemented, and waivers or consents to departures
      from
      the provisions hereof may not be given, unless the same shall be in writing
      and
      signed by the Company and each Holder of the then outstanding Registrable
      Securities. Notwithstanding the foregoing, a waiver or consent to depart from
      the provisions hereof with respect to a matter that relates exclusively to
      the
      rights of Holders and that does not directly or indirectly affect the rights
      of
      other Holders may be given by Holders of all of the Registrable Securities
      to
      which such waiver or consent relates; provided,
      however,
      that
      the provisions of this sentence may not be amended, modified, or supplemented
      except in accordance with the provisions of the immediately preceding
      sentence.

     

    (f)  Notices.
      Any and
      all notices or other communications or deliveries required or permitted to
      be
      provided hereunder shall be delivered as set forth in the Subscription
      Agreement.

     

    (g)  Successors
      and Assigns.
      This
      Agreement shall inure to the benefit of and be binding upon the successors
      and
      permitted assigns of each of the parties and shall inure to the benefit of
      each
      Holder. The Company may not assign its rights or obligations hereunder without
      the prior written consent of all of the Holders of the then-outstanding
      Registrable Securities. Each Holder may assign their respective rights hereunder
      in the manner and to the Persons as permitted under the Subscription
      Agreement.

     

    (h)  No
      Inconsistent Agreements.
      Neither
      the Company nor any of its subsidiaries has entered, as of the date hereof,
      nor
      shall the Company or any of its subsidiaries, on or after the date of this
      Agreement, enter into any agreement with respect to its securities, that would
      have the effect of impairing the rights granted to the Holders in this
      Agreement. 

     

    
      
         

      

      
        12

        
          

        

      

      
         

      

    

     

    (i)  Execution
      and Counterparts.
      This
      Agreement may be executed in any number of counterparts, each of which when
      so
      executed shall be deemed to be an original and, all of which taken together
      shall constitute one and the same Agreement. In the event that any signature
      is
      delivered by facsimile transmission, such signature shall create a valid binding
      obligation of the party executing (or on whose behalf such signature is
      executed) the same with the same force and effect as if such facsimile signature
      were the original thereof.

     

    (j)  Governing
      Law.
      All
      questions concerning the construction, validity, enforcement and interpretation
      of this Agreement shall be determined with the provisions of the Subscription
      Agreement.

     

    (k)  Cumulative
      Remedies.
      The
      remedies provided herein are cumulative and not exclusive of any remedies
      provided by law.

     

    (l)  Severability.
      If any
      term, provision, covenant or restriction of this Agreement is held by a court
      of
      competent jurisdiction to be invalid, illegal, void or unenforceable, the
      remainder of the terms, provisions, covenants and restrictions set forth herein
      shall remain in full force and effect and shall in no way be affected, impaired
      or invalidated, and the parties hereto shall use their commercially reasonable
      efforts to find and employ an alternative means to achieve the same or
      substantially the same result as that contemplated by such term, provision,
      covenant or restriction. It is hereby stipulated and declared to be the
      intention of the parties that they would have executed the remaining terms,
      provisions, covenants and restrictions without including any of such that may
      be
      hereafter declared invalid, illegal, void or unenforceable.

     

    (m)  Headings.
      The
      headings in this Agreement are for convenience of reference only and shall
      not
      limit or otherwise affect the meaning hereof.

     

    (n)  Independent
      Nature of Holders’ Obligations and Rights.
      The
      obligations of each Holder hereunder are several and not joint with the
      obligations of any other Holder hereunder, and no Holder shall be responsible
      in
      any way for the performance of the obligations of any other Holder hereunder.
      Nothing contained herein or in any other agreement or document delivered at
      any
      closing, and no action taken by any Holder pursuant hereto or thereto, shall
      be
      deemed to constitute the Holders as a partnership, an association, a joint
      venture or any other kind of entity, or create a presumption that the Holders
      are in any way acting in concert with respect to such obligations or the
      transactions contemplated by this Agreement. Each Holder shall be entitled
      to
      protect and enforce its rights, including without limitation the rights arising
      out of this Agreement, and it shall not be necessary for any other Holder to
      be
      joined as an additional party in any proceeding for such purpose.

     

    *************************

    


    
      
         

      

      
        13

        
          

        

      

      
         

      

    


    IN
      WITNESS WHEREOF, the parties have executed this Registration Rights Agreement
      as
      of the date first written above.

    
      	 	 	 
	 	
              GENTIUM
                S.p.A. 

            
	 
 	 
 	 
 
	 	By:  	/s/
              Laura Iris Ferro,
              M.D.                        
              
	 	Name: Laura Iris Ferro, M.D.
	 	
              Title:
                President and Chief Executive
                Officer

            


    

    
 

    [SIGNATURE
      PAGE OF HOLDERS FOLLOWS]


    


    


    
      
         

      

      
         

        
          

        

      

      
         

      

    



    Alexandra
      Global Master Fund Ltd.

    By:
      Alexandra Investment Management, LLC, as Investment Advisor

    


    /s/
      Mikhail Filimonov    

    Title:
      Chairman and Chief Executive Officer

    


    Atlas
      Master Fund, Ltd.     

    


    /s/
      Francis Rainsford    

    Title:
      Chief Financial Officer   

    


    Caxton
      Advantage Life Sciences Fund, L.P. 

    By:
      Caxton Advantage Venture Partners, L.P., its General Partner

    By:
      Advantage Life Sciences Partners, LLC, its Managing General Partner  

    


    /s/
      Eric W. Roberts     

    Title:
      Member

    


    Caxton
      International Limited 

    


    /s/
      Joseph Kelly     

    Title:
      Vice President and Treasurer  

    


    /s/
      Maxwell Quinn     

    Title:
      Vice President and Secretary   

     

    Chaumiere
      Consultadoria E Servicos Unipessoal LdA 

    


    /s/
      Roberto Carlos De Castro Abreu  

    Title:
      Director

    


    Credit
      Suisse Securities (USA) LLC 

    


    /s/
      Onu Odim     

    Title:
      Managing Director

    


    DAFNA
      LifeScience Ltd. 

    By:
      DAFNA
      Capital Management, LLC, its Investment Manager

    


    /s/
      Nathan Fischel, MD, CFA   

    Title:
      Managing Member

     

    
      
         

      

      
        A

        
          

        

      

      
         

      

    

     

    DAFNA
      LifeScience Market Neutral Ltd. 

    By:
      DAFNA
      Capital Management, LLC, its Investment Manager

    


    /s/
      Nathan Fischel, MD, CFA   

    Title:
      Managing Member

    


    DAFNA
      LifeScience Select Ltd. 

    By:
      DAFNA
      Capital Management, LLC, its Investment Manager

    


    /s/
      Nathan Fischel, MD, CFA   

    Title:
      Managing Member  

     

    Defiante
      Farmaceutica LdA 

    


    /s/
      Paulo Viegas     

    Title:
      Director - General Manager

    


    GLG
      North American Opportunity Fund

    By:
      GLG
      Partners LP, its Investment Manager 

    


    /s/
      Tim Kuschill     

    Title:
      Legal Counsel

    


    /s/
      Victoria Parry     

    Title:
      Senior Legal Counsel

    


    Inverlochy
      Consultadoria E Servicos, LdA 

    


    /s/
      Roberto Carlos De Castro Abreu  

    Title:
      Director 

    


    JP
      Morgan Securities Inc.   

    


    /s/
      Avik Roy      

    Title:
      Vice President

    


    Millennium
      Partners, L.P.

    By:
      Millennium Management L.L.C.

    


    /s/
      Terry Feeney     

    Title:
      Chief Operating Officer

    


    Morgan
      Stanley & Co. Incorporated

    


    /s/
      Andrew C. Pipa     

    Title:
      Managing Director

    


    Clipperbay
      & Co. 

    fbo
      SMALLCAP World Fund, Inc.    

    By:
      Capital Research and Management Company, 

    its
      investment adviser 

    


    /s/
      Michael J. Downer    

    Title:
      Vice President and Secretary    

     

    
      
         

      

      
        B

        
          

        

      

      
         

      

    

    


    Symphonia
      Sicav Comparto Italia Flessibile    

    


    /s/
      Guido Crivellaro     

    Title:
      Head of Investment Managers’ Team  

    


    Symphonia
      Sicav Comparto Italia Small Cap    

    


    /s/
      Guido Crivellaro     

    Title:
      Head of Investment Managers’ Team  

    


    UBS
      O’Connor

    Fbo
      O’Connor PIPES Corporate Strategies Master Limited    

    


    /s/
      Nick Nocerino     

    Title:
      Managing Director   

    


    Visium
      Balanced Fund, LP     

    


    /s/
      Mark Gottlieb     

    Title:
      Chief Communications Officer    

    


    Visium
      Balanced Offshore Fund, Ltd.   

    


    /s/
      Mark Gottlieb     

    Title:
      Chief Communications Officer    

    


    Visium
      Long Bias Fund, LP     

    


    /s/
      Mark Gottlieb     

    Title:
      Chief Communications Officer    

    


    Visium
      Long Bias Offshore Fund, Ltd.   

    


    /s/
      Mark Gottlieb     

    Title:
      Chief Communications Officer 

    


    Windmill
      Master Fund LP 

    By:
      Duquesne Capital Management, LLC, as Investment Manager   

    


    /s/
      Darren Martian     

    Title:
      Vice President

     

    
      
         

      

      
        C

        
          

        

      

      
         

      

    


    ANNEX
      A

     

    Plan
      of Distribution

     

    Each
      selling security holder and any of their pledgees, assignees and
      successors-in-interest may, from time to time, sell any or all of their ADSs
      on
      the Nasdaq Global Market System or any other stock exchange, market or trading
      facility on which the ADSs are traded or in private transactions. These sales
      may be at fixed or negotiated prices. A selling security holder may use any
      one
      or more of the following methods when selling ADSs:

     

    ·  ordinary
      brokerage transactions and transactions in which the broker-dealer solicits
      purchasers;

     

    ·  block
      trades in which the broker-dealer will attempt to sell the shares as agent
      but
      may position and resell a portion of the block as principal to facilitate the
      transaction;

     

    ·  purchases
      by a broker-dealer as principal and resale by the broker-dealer for its
      account;

     

    ·  an
      exchange distribution in accordance with the rules of the applicable
      exchange;

     

    ·  public
      or
      privately negotiated transactions;

     

    ·  settlement
      of short sales entered into after the effective date of the registration
      statement of which this prospectus is a part;

     

    ·  on
      the
      Nasdaq National Market System (or through facilities of any national securities
      exchange or US inter-dealer quotation system of a registered national securities
      association on which the ADSs are then listed, admitted to unlisted trading
      privileges or included for quotation);

     

    ·  broker-dealers
      may agree with the selling security holders to sell a specified number of such
      shares at a stipulated price per share;

     

    ·  through
      underwriters, brokers or dealers (who may act as agents or principals) or
      directly to one or more purchasers;

     

    ·  through
      the writing or settlement of options or other hedging transactions, whether
      through an options exchange or otherwise; 

     

    ·  a
      combination of any such methods of sale; or

     

    ·  any
      other
      method permitted pursuant to applicable law.

     

    The
      selling security holders may also sell shares under Rule 144 under the
      Securities Act of 1933, as amended, if available, rather than under this
      prospectus.

     

    
      
         

      

      
         

        
          

        

      

      
         

      

    


    Broker-dealers
      engaged by the selling security holders may arrange for other brokers-dealers
      to
      participate in sales. Broker-dealers may receive commissions or discounts from
      the selling security holders (or, if any broker-dealer acts as agent for the
      purchaser of shares, from the purchaser) in amounts to be negotiated, but,
      except as set forth in a supplement to this Prospectus, in the case of an agency
      transaction not in excess of a customary brokerage commission in compliance
      with
      NASDR Rule 2440; and in the case of a principal transaction a markup or markdown
      in compliance with NASDR IM-2440. 

     

    In
      connection with the sale of the ADSs or interests therein, the selling security
      holders may enter into hedging transactions with broker-dealers or other
      financial institutions, which may in turn engage in short sales of the ADSs
      in
      the course of hedging the positions they assume. The selling security holders
      may also sell shares of the ADSs short and deliver these securities to close
      out
      their short positions, or loan or pledge the ADSs to broker-dealers that in
      turn
      may sell these securities. The selling security holders may also enter into
      option or other transactions with broker-dealers or other financial institutions
      or the creation of one or more derivative securities which require the delivery
      to such broker-dealer or other financial institution of shares offered by this
      prospectus, which shares such broker-dealer or other financial institution
      may
      resell pursuant to this prospectus (as supplemented or amended to reflect such
      transaction). The selling security holders may also pledge shares to a
      broker-dealer or other financial institution which, upon default, they may
      in
      turn resell.

     

    In
      addition to the foregoing methods, the selling security holders may offer their
      shares from time to time in transactions involving principals or brokers not
      otherwise contemplated above, in a combination of such methods or described
      above or any other lawful methods. The selling security holders may also
      transfer, donate or assign their shares to lenders, family members and others
      and each of such persons will be deemed to be a selling security holder for
      purposes of this prospectus. The security holders or their successors in
      interest may from time to time pledge or grant a security interest in some
      or
      all of the shares of common stock, and if the selling security holders default
      in the performance of their secured obligations, the pledgees or secured parties
      may offer and sell the shares of common stock from to time under this
      prospectus; provided however in the event of a pledge or then default on a
      secured obligation by the selling security holder, in order for the shares
      to be
      sold under this registration statement, unless permitted by law, we must
      distribute a prospectus supplement and/or amendment to this registration
      statement amending the list of selling security holders to include the pledgee,
      secured party or other successors in interest of the security holder under
      this
      prospectus.

     

    The
      selling security holders may also sell their shares pursuant to Rule 144 under
      the Securities Act, which permits limited resale of shares purchased in a
      private placement subject to the satisfaction of certain conditions, including,
      among other things, the availability of certain current public information
      concerning the issuer, the resale occurring following the required holding
      period under Rule 144 and the number of shares being sold during any three-month
      period not exceeding certain limitations.

     

    Sales
      through brokers may be made by any method of trading authorized by any stock
      exchange or market on which the shares may be listed or quoted, including block
      trading in negotiated transactions. Without limiting the foregoing, such brokers
      may act as dealers by purchasing any or all of the shares covered by this
      prospectus, either as agents for others or as principals for their own accounts,
      and reselling such shares pursuant to this prospectus. The selling security
      holders may effect such transactions directly, or indirectly through
      underwriters, broker-dealers or agents acting on their behalf. In effecting
      sales, broker-dealers or agents engaged by the selling security holders may
      arrange for other broker-dealers to participate. Broker-dealers or agents may
      receive commissions, discounts or concessions from the selling security holders,
      in amounts to be negotiated immediately prior to the sale (which compensation
      as
      to a particular broker-dealer might be in excess of customary commissions for
      routine market transactions).

     

    
      
         

      

      
         

        
          

        

      

      
         

      

    

     

    The
      selling security holders and any broker-dealers or agents that are involved
      in
      selling the shares may be deemed to be “underwriters” within the meaning of the
      Securities Act in connection with such sales. In such event, any profits
      received by the selling security holders or such broker-dealers or agents and
      any profit on the resale of the shares purchased by them may be deemed to be
      underwriting commissions or discounts under the Securities Act. 

     

    We
      are
      required to pay certain fees and expenses incurred by us incident to the
      registration of the shares. We have agreed to indemnify the selling security
      holders against certain losses, claims, damages and liabilities, including
      liabilities under the Securities Act. 

     

    We
      agreed
      to keep this prospectus effective until the earlier of (i) the date on which
      the
      shares may be resold by the selling security holders without registration and
      without regard to any volume limitations by reason of Rule 144(e) under the
      Securities Act or any other rule of similar effect or (ii) all of the shares
      have been sold pursuant to the prospectus or Rule 144 under the Securities
      Act
      or any other rule of similar effect.

     

    Under
      applicable rules and regulations under the Exchange Act, any person engaged
      in
      the distribution of the resale shares may not simultaneously engage in market
      making activities with respect to the ordinary shares or ADSs for the applicable
      restricted period, as defined in Regulation M, prior to the commencement of
      the
      distribution. In addition, the selling security holders will be subject to
      applicable provisions of the Exchange Act and the rules and regulations
      thereunder, including Regulation M, which may limit the timing of purchases
      and
      sales of shares of the ordinary shares or ADSs by the selling security holders
      or any other person. We will make copies of this prospectus available to the
      selling security holders and have informed them of the need to deliver a copy
      of
      this prospectus to each purchaser at or prior to the time of the
      sale.

     

    
      
         

      

      
         

        
          

        

      

      
         

      

    

    


    Annex
      B

     

    Gentium
      S.p.A. 

     

    Selling
      Security Holder Notice and Questionnaire

     

    The
      undersigned beneficial owner of ADSs representing the ordinary shares, par
      value
€1.00 per share (the “Ordinary
      Shares”),
      of
      Gentium S.p.A., an Italian joint stock company (the “Company”),
      (the
“Registrable
      Securities”)
      understands that the Company has filed or intends to file with the Securities
      and Exchange Commission (the “Commission”)
      a
      registration statement on Form F-3 (the “Registration
      Statement”)
      for
      the registration and resale under Rule 415 of the Securities Act of 1933, as
      amended (the “Securities
      Act”),
      of
      the Registrable Securities, in accordance with the terms of the Registration
      Rights Agreement, dated as of February 9, 2007 (the “Registration
      Rights Agreement”),
      among
      the Company and the Purchasers named therein. A copy of the Registration Rights
      Agreement is available from the Company upon request at the address set forth
      below. All capitalized terms not otherwise defined herein shall have the
      meanings ascribed thereto in the Registration Rights Agreement.

     

    Certain
      legal consequences arise from being named as a selling security holder in the
      Registration Statement and the related prospectus. Accordingly, holders and
      beneficial owners of Registrable Securities are advised to consult their own
      securities law counsel regarding the consequences of being named or not being
      named as a selling security holder in the Registration Statement and the related
      prospectus.

     

    NOTICE

     

    The
      undersigned beneficial owner (the “Selling
      Security Holder”)
      of
      Registrable Securities hereby elects to include the Registrable Securities
      owned
      by it and listed below in Item 3 (unless otherwise specified under such Item
      3)
      in the Registration Statement.

     

    
      
         

      

      
         

        
          

        

      

      
         

      

    


    The
      undersigned hereby provides the following information to the Company and
      represents and warrants that such information is accurate:

     

    QUESTIONNAIRE

     

    1. Name.

     

    
      	 	
              (a)

            	
              Full
                Legal Name of Selling Security
                Holder:

            


    

     

    
      	 	 
	 	 


    

    


    
      	 	
              (b)

            	
              Full
                Legal Name of Registered Holder (if not the same as (a) above) through
                which Registrable Securities Listed in Item 3 below are
                held:

            


    

     

    
      	 	 
	 	 


    

    


    
      	 	
              (c)

            	
              Full
                Legal Name of Natural Control Person (which means a natural person
                who
                directly or indirectly alone or with others has power to vote or
                dispose
                of the securities covered by the
                questionnaire):

            


    

     

    
      	 	 
	 	 


    

    


     

    2.
      Address for Notices to Selling Security Holder:

     

    
      	 
	 
	 
	
              Telephone: 

            	 
	
              Fax: 

            	 
	
              Contact
                Person: 

            	 


    

    


    3.
      Beneficial Ownership of Registrable Securities:

     

    
      	 	
              (a)

            	
              Type
                and Number of Registrable Securities beneficially
                owned:

            


    

     

    
      	 	 
	 	 
	 	 
	 	 


    

    


    
      
         

      

      
         

        
          

        

      

      
         

      

    


    4.
      Broker-Dealer Status:

     

    
      	 	
              (a)

            	
              Are
                you a broker-dealer?

            


    

     

    Yes
o No
o

     

    
      	 	
              (b)

            	
              If
                “yes” to Section 4(a), did you receive your Registrable Securities as
                compensation for investment banking services to the
                Company.

            


    

     

    Yes
o No
o

     

    
      	 	
              Note:

            	
              If
                no, the Commission’s staff has indicated that you should be identified as
                an underwriter in the Registration
                Statement.

            


    

     

    
      	 	
              (c)

            	
              Are
                you an affiliate of a
                broker-dealer?

            


    

     

    Yes
o No
o

     

    
      	 	
              (d)

            	
              If
                you are an affiliate of a broker-dealer, do you certify that you
                bought
                the Registrable Securities in the ordinary course of business, and
                at the
                time of the purchase of the Registrable Securities to be resold,
                you had
                no agreements or understandings, directly or indirectly, with any
                person
                to distribute the Registrable
                Securities?

            


    

     

    Yes
o No
o

     

    
      	 	
              Note:

            	
              If
                no, the Commission’s staff has indicated that you should be identified as
                an underwriter in the Registration
                Statement.

            


    

     

    5.
      Beneficial Ownership of Other Securities of the Company Owned by the Selling
      Security Holder.

     

    Except
      as set forth below in this Item 5, the undersigned is not the beneficial or
      registered owner of any securities of the Company other than the Registrable
      Securities listed above in Item 3.

     

    
      	 	
              (a)

            	
              Type
                and Amount of Other Securities beneficially owned by the Selling
                Security
                Holder:

            


    

     

    
      	 	 
	 	 
	 	 


    

    


    
      
         

      

      
         

        
          

        

      

      
         

      

    


    6.
      Relationships with the Company:

     

    Except
      as set forth below, neither the undersigned nor any of its affiliates, officers,
      directors or principal equity holders (owners of 5% of more of the equity
      securities of the undersigned) has held any position or office or has had any
      other material relationship with the Company (or its predecessors or affiliates)
      during the past three years.

     

    State
      any
      exceptions here:

     

    
      	 	 
	 	 
	 	 


    

    


     

    The
      undersigned agrees to promptly notify the Company of any inaccuracies or changes
      in the information provided herein that may occur subsequent to the date hereof
      at any time while the Registration Statement remains effective.

     

    By
      signing below, the undersigned consents to the disclosure of the information
      contained herein in its answers to Items 1 through 6 and the inclusion of such
      information in the Registration Statement and the related prospectus
and
      any
      amendments or supplements thereto.
      The
      undersigned understands that such information will be relied upon by the Company
      in connection with the preparation or amendment of the Registration Statement
      and the related prospectus.

     

    IN
      WITNESS WHEREOF the undersigned, by authority duly given, has caused this Notice
      and Questionnaire to be executed and delivered either in person or by its duly
      authorized agent.

     

     

    
      	Dated: ____________________ 	 	Beneficial Owner:
              ____________________
	 	 	 
	 	 	By:_______________________________ 
	 	 	
               
Name: 

            
	 	 	
               
Title:

            


    

       

    


    PLEASE
      FAX A COPY OF THE COMPLETED AND EXECUTED NOTICE AND QUESTIONNAIRE, AND RETURN
      THE ORIGINAL BY OVERNIGHT MAIL, TO:

    


    Karen
      F.
      Connors, Esq.

    Epstein
      Becker & Green, P.C.

    250
      Park
      Avenue

    New
      York,
      NY 10177

    Tel:
      (212) 351-4786

    Fax:
      (212) 878-8794

    E-mail:
      kconnors@ebglaw.com