Document:

QuickLinks
 -- Click here to rapidly navigate through this document

 
 

Exhibit 4.2.2    
    

        THE WARRANTS EVIDENCED BY THIS CERTIFICATE AND THE SECURITIES ISSUABLE UPON EXERCISE HEREOF HAVE NOT BEEN REGISTERED UNDER THE SECURITIES
ACT OF 1933, AS AMENDED (THE "ACT"), OR ANY STATE SECURITIES LAWS AND NEITHER SUCH SECURITIES NOR ANY INTEREST THEREIN MAY BE OFFERED, SOLD, PLEDGED,
ASSIGNED OR OTHERWISE TRANSFERRED UNLESS (1) A REGISTRATION STATEMENT WITH RESPECT THERETO IS EFFECTIVE UNDER THE ACT AND ANY APPLICABLE STATE SECURITIES LAWS, OR (2) ISSUER RECEIVES AN
OPINION OF COUNSEL TO THE HOLDER OF SUCH SECURITIES, WHICH COUNSEL AND OPINION ARE REASONABLY SATISFACTORY TO ISSUER, THAT SUCH SECURITIES MAY BE OFFERED, SOLD, PLEDGED, ASSIGNED OR TRANSFERRED IN THE
MANNER CONTEMPLATED WITHOUT AN EFFECTIVE REGISTRATION STATEMENT UNDER THE ACT OR APPLICABLE STATE SECURITIES LAWS.

	No. W-

Issue Date:	 	Warrants

New York, New York

GENTIUM S.P.A.  

 WARRANT TO PURCHASE SHARES OF

COMMON STOCK, PAR VALUE €1 PER SHARE  

        This Warrant Certificate certifies that, for value received,
                        , is the registered holder (the "Holder") of Warrants ("Warrants") to purchase, at any
time commencing on the earlier to occur of (i) the closing of a registered public offering on any of Nasdaq National or SmallCap Market, the American Stock Exchange or the New York Stock
Exchange (an "Initial Public Offering") and (ii) the one year anniversary of the date hereof as specified above (the "Issue Date"), subject to the terms and conditions hereof, until
5:00 p.m., New York City time on the date that is the later of (w) five (5) years and three (3) months after the Issue Date and (x) four (4) years and three
(3) months following the closing of the Initial Public Offering (the "Expiration Date"), the number of shares of fully-paid and non-assessable shares of common stock,
€1 par value per share (the "Common Stock"), of GENTIUM S.P.A., a joint stock company (società per azioni) incorporated
and organized under the laws of the Republic of Italy (the "Company"), equal to $[            ] [coverage percentage multiplied by dollars invested]
divided by the lower of (i) ten dollars ($10.00) per share and (ii) the price per share of Common Stock sold by the Company in an Initial Public Offering (but not less than $6.00 per
share) (in each case subject to adjustment as set forth herein, the "Shares"). The per share exercise price for the shares of Common Stock that may be purchased hereunder shall be one hundred ten
percent (110%) of the price per share of Common Stock sold by the Company in an Initial Public Offering (but not less than $6.00 per share); provided, however, that if the Company shall not have
completed an Initial Public Offering on or prior to [                        ] [one year anniversary of the Issue Date], the
exercise price shall be six dollars
($6.00) per share (such exercise price, as adjusted pursuant to the terms of this Warrant, the "Exercise Price"). In order to exercise all or a part of this Warrant, the Holder shall surrender this
Warrant Certificate and pay the Exercise Price at the principal office of the Company. Payment of the Exercise Price shall be made by wire transfer or as otherwise provided in Section 1(b) or
1(f) hereof. The Warrants represented by this Warrant Certificate is one of a series of similar warrants (the "Investor Warrants") issued pursuant to those certain Subscription Agreements, dated as of
the date hereof, by and among the Company and the Holder and the Company and certain other holders (each, a "Subscription Agreement" and collectively, the "Subscription Agreements"). Capitalized terms
used herein without definition have the meanings ascribed to such terms in the Subscription Agreements. The rights of the Holder of this Warrant Certificate shall be subject to the following terms and
conditions and to the text of the official English translation of the resolution relating to the issuance of this Warrant Certificate approved by the extraordinary shareholders' meeting of the Company
on September 30, 2004 as amended and supplemented by an additional extraordinary 

 

shareholders'
meeting of the Company on October 8, 2004 (the "Company Resolution"), herewith enclosed as Exhibit A: 

        SECTION
1.    Method of Exercise of Warrants; Exercise Price; Issuance of Certificates.    

        (a)   The
Holder of this Warrant Certificate may exercise the Warrants evidenced hereby, in whole or in part, at any time, and from time to time, prior to 5:00 p.m.,
New York City time, on the Expiration Date, by a duly executed facsimile copy of the Election to Purchase attached hereto as Exhibit B, to the Company at its principal office as set forth in
the Investors' Rights Agreement. Within ten (10) days from the delivery to the Company of the Election to Purchase by facsimile copy, the Holder of this Warrant Certificate shall surrender this
Warrant Certificate by registered or certified mail, return receipt requested, or by a nationally-recognized overnight mail carrier, or by hand delivery against written receipt thereof, together with
payment of the Exercise Price for each Share as to which the Warrants are exercised. Each Warrant, to the extent not exercised prior to 5:00 p.m., New York City time, on the Expiration Date
shall become void and all rights thereunder shall cease as of such time. 

        (b)   The
aggregate Exercise Price shall be payable by wire transfer of immediately available funds, by means of a cashless exercise pursuant to Section 1(f) hereof, or
by any other means consented to by the Company. 

        (c)   Upon
receipt of this Warrant Certificate representing exercisable Warrants, with the Form of Election to Purchase attached hereto duly executed, accompanied by payment
of the aggregate Exercise Price for the Shares to be purchased and an amount equal to any applicable transfer tax required to be paid by the Holder in accordance with Section 6(c) hereof, the
Company shall thereupon promptly, and in no event later than five (5) business days thereafter, cause to be issued to the Holder a certificate for the number of whole shares of Common Stock so
purchased. 

        (d)   If
the Holder of this Warrant Certificate shall exercise less than all the Warrants evidenced hereby, a new Warrant Certificate, substantially in the form hereof,
evidencing Warrants equivalent to the Warrants remaining unexercised shall be issued by the Company to the Holder of this Warrant Certificate or to its duly authorized assigns, subject to the
provisions of Section 5(a) hereof. 

        (e)   The
Warrant Certificates and the certificates representing the shares of Common Stock shall be executed on behalf of the Company in accordance with the Company's
Articles of Association. Warrant Certificates shall be dated the date of execution by the Company upon initial issuance, division, exchange, substitution or transfer. Each Warrant Certificate may be
signed on behalf of the Company by any person who, at the actual date of the execution of such Warrant Certificate, shall be the proper officer of the Company to sign such Warrant Certificate. If any
officer whose signature is on a Warrant Certificate no longer holds that office after the date of issuance of the Warrant Certificate, such Warrant Certificate shall be valid nevertheless. 

        (f)    Notwithstanding
any other provision in this Warrant to the contrary, if on or prior to the one (1) year anniversary of the Initial Public Offering, the Company
has not yet filed with the Securities and Exchange Commission a registration statement (the "Registration Statement") with respect to the resale by the Holder of the Shares, or if a Registration
Statement has been so filed but is not effective at any time following the fourteen (14) month anniversary of the Initial Public Offering, then for as long as no such Registration Statement is
effective with a current prospectus available, this Warrant may also be exercised, in whole or in part, at any time prior to the Expiration Date, by means of a "cashless exercise" in which the Holder
shall be entitled to receive a certificate for the number of Shares equal to the quotient obtained by dividing [(A-B)(X)] by (A), where: 

        (A)  =
the closing bid price on the trading day preceding the date of such election; 

        (B)  =
the Exercise Price of the Warrants, as adjusted; and 

2

 

        (X)  =
the number of Shares issuable upon exercise of the Warrants in accordance with the terms of this Warrant. 

        SECTION
2.    Split Up, Combination and Exchange of Warrant Certificates; Mutilated, Destroyed, Lost or Stolen Warrant Certificates; Warrant
Register.    

        (a)   Subject
to the provisions of Section 5(a) hereof, at or prior to the Expiration Date, this Warrant Certificate, with or without other Warrant Certificates, may be
split up, combined or exchanged for another Warrant Certificate or Warrant Certificates, entitling the Holder to purchase a like number of shares of Common Stock as the Warrant Certificate or Warrant
Certificates surrendered then entitled the Holder to purchase. Any Holder desiring to split up, combine or exchange this Warrant Certificate shall make such request in writing delivered to the
Company, and shall surrender the Warrant Certificate or Warrant Certificates to be split up, combined or exchanged at the office of the Company maintained for such purpose as set forth below.
Thereupon the Company shall sign and deliver to the person or entity entitled thereto a Warrant Certificate or Warrant Certificates, as the case may be, as so requested. The Company may require
payment of a sum sufficient to cover any transfer tax that may be imposed in connection with any split-up, combination or exchange of Warrant Certificates. 

        (b)   Upon
receipt by the Company of evidence reasonably satisfactory to it of the loss, theft, destruction or mutilation of this Warrant Certificate, and, in case of loss,
theft or destruction, of indemnity or security reasonably satisfactory to it and reimbursement to the Company of all reasonable expenses incidental thereto, and upon surrender and cancellation of the
Warrant Certificate if mutilated, the Company will make and deliver a new Warrant Certificate of like tenor to the Holder in lieu of the Warrant Certificate so lost, stolen, destroyed or mutilated. 

        (c)   The
Company will maintain at its principal office a Warrant register upon which transfers and exchanges of Warrants shall be recorded. Such office is currently located
at Piazza XX Settembre, n.2, 22079, Villa Guardia, Como, Italy. 

        SECTION
3.    Subsequent Issue of Warrant Certificates.    Subsequent to their original issuance, no Warrant
Certificates shall be issued except (a) Warrant Certificates issued upon any transfer, combination, split-up or exchange of Warrants pursuant to the terms, conditions and provisions
hereof, (b) Warrant Certificates issued in replacement of mutilated, destroyed, lost or stolen Warrant Certificates pursuant to Section 2 hereof and (c) Warrant Certificates
issued pursuant to Section 1(d) hereof upon the partial exercise of any Warrant Certificate to evidence the unexercised portion of such Warrant Certificate. 

        SECTION
4.    Cancellation and Destruction of Warrant Certificates.    All Warrant Certificates surrendered for the
purpose of exercise, exchange, substitution, transfer, split-up or combination shall be cancelled by the Company, and no Warrant Certificates shall be issued in lieu thereof except as
expressly permitted by any of the provisions of this Warrant Certificate. The Company shall cancel and retire any other Warrant Certificates purchased or acquired by the Company otherwise than upon
the exercise thereof. 

        SECTION
5.    Ownership; Restrictions on Transfer; Legends.    

        (a)   The
Holder of this Warrant Certificate, by its acceptance hereof, covenants and agrees that the Warrants evidenced hereby are being acquired as an investment and not
with a view to the distribution thereof and that the Warrants may not be sold, transferred, assigned, hypothecated or otherwise disposed of in any way, in whole or in part, except in compliance with
the Securities Act of 1933, as amended (the "Act") and the provisions of this Warrant Certificate and that certain Investors' Rights Agreement, dated as of the date hereof, by and among the Company
and the investors listed as parties thereto (the "Investors' Rights Agreement"). To be effective under this Warrant Certificate, any transfer, sale or other disposition of the Warrants shall be
registered with the Company by submission 

3

 

to
it of this Warrant Certificate, together with a notice of transfer in the form of the transfer form (the "Transfer Form") attached hereto as Exhibit B, duly completed and executed, and
payment of any and all applicable taxes, expenses or charges in respect of such transaction pursuant to Section 6(c) hereof. Within five business days after the Company's receipt of the Warrant
Certificate, the Transfer Form so completed and executed and the payment of such taxes, expenses or charges, if any, the Company will issue and deliver to the transferee a new Warrant Certificate
representing the portion of the Warrants transferred and otherwise having the same terms and provisions as this Warrant Certificate, which the Company will register in the new Holder's name. In the
event of a partial transfer of the Warrants, the Company shall concurrently issue and deliver to the transferring Holder a new Warrant Certificate that entitles the transferring Holder to the balance
of the Warrants not so transferred and that otherwise is upon the same terms and provisions as this Warrant Certificate. Upon the due delivery of this Warrant Certificate for transfer, the transferee
shall be deemed for all purposes to have become the Holder of the new Warrant Certificate issued in respect of the Warrants transferred, effective immediately prior to the close of business on the
date of such delivery, irrespective of the date of actual delivery of such new Warrant Certificate representing such transferred Warrants. 

        (b)   Each
Warrant Certificate (including each Warrant Certificate issued upon the transfer of such Warrant Certificate) shall be stamped or otherwise imprinted with a legend
in substantially the form of the legend on the face hereof. 

        (c)   The
Warrants, and the shares of Common Stock issuable upon exercise of the Warrants, have not been registered under the Act. Upon exercise, in part or in whole, of the
Warrants, certificates representing the shares of Common Stock issuable upon exercise of the Warrants shall be stamped or otherwise imprinted with a legend in substantially the following form: 

"THE
SECURITIES REPRESENTED HEREBY HAVE NOT BEEN REGISTERED UNDER THE SECURITIES ACT OF 1933, AS AMENDED (THE "ACT"), OR ANY STATE SECURITIES LAWS AND
NEITHER SUCH SECURITIES NOR ANY INTEREST THEREIN MAY BE OFFERED, SOLD, PLEDGED, ASSIGNED OR OTHERWISE TRANSFERRED UNLESS (1) A REGISTRATION STATEMENT WITH RESPECT THERETO IS EFFECTIVE UNDER THE
ACT AND ANY APPLICABLE STATE SECURITIES LAWS, OR (2) ISSUER RECEIVES AN OPINION OF COUNSEL TO THE HOLDER OF SUCH SECURITIES, WHICH COUNSEL AND OPINION ARE REASONABLY SATISFACTORY TO ISSUER,
THAT SUCH SECURITIES MAY BE OFFERED, SOLD, PLEDGED, ASSIGNED OR TRANSFERRED IN THE MANNER
CONTEMPLATED WITHOUT AN EFFECTIVE REGISTRATION STATEMENT UNDER THE ACT OR APPLICABLE STATE SECURITIES LAWS. 

        SECTION
6.    Reservation and Availability of Shares of Common Stock; Taxes.    

        (a)   The
Company shall at all times have authorized and reserved, free from any preemptive or similar rights, and shall keep available out of its authorized and unissued
shares of Common Stock, the number of shares of Common Stock that will be sufficient to permit the exercise in full of all outstanding Warrants from time to time. 

        (b)   The
Company shall take all such action as may be necessary to ensure that all shares of Common Stock delivered upon exercise of Warrants shall, at the time of delivery
of the certificates for such shares (subject to payment of the Exercise Price), be duly and validly authorized and issued, fully paid and non-assessable, and free and clear of any and all
taxes, liens and charges with respect to the issuance thereof, and the issuance of such shares of Common Stock shall not be subject to any preemptive or similar right of the Company or any other
person or entity (all of which rights being hereby waived irrevocably). 

4

 

        (c)   The
Company shall pay when due and payable any and all issue or other taxes and charges that may be payable in respect of the initial issuance or delivery of this
Warrant Certificate or of the issuance or delivery of any shares of Common Stock upon the exercise of Warrants, except as set forth in the immediately following sentence. The Company shall not,
however, be required to pay any tax which may be payable in respect of any transfer involved in the issue or delivery of this Warrant Certificate to a person or entity other than, or the issuance or
delivery of certificates for Common Stock in a name other than that of, the Holder of the Warrant Certificate evidencing Warrants surrendered for exercise or to issue or deliver any certificates for
shares of Common Stock upon the exercise of any Warrants until any such tax shall have been paid (any such tax being payable by the Holder of such Warrant Certificate at the time of surrender) or
until it has been established to the Company's satisfaction that no such tax is due. 

        SECTION
7.    Common Stock Record Date.    Each person or entity in whose name any certificate for shares of Common
Stock is issued upon the exercise of Warrants shall for all purposes be deemed to have become the holder of record of the Common Stock represented thereby on, and such certificate shall be dated, the
close of business on the date upon which the Warrant Certificate evidencing such Warrants was duly surrendered and payment of the Exercise Price (and any applicable transfer taxes) was made;  provided,
however, that if the date of such surrender and payment is a date upon which the Common Stock
transfer books of the Company are closed, such person or entity shall be deemed to have become the record holder of such shares on, and such certificate shall be dated, the opening of business on the
next succeeding business day on which the Common Stock transfer books of the Company are open. 

        SECTION
8.    Adjustments to Exercise Price, Number of Shares and Number of Warrants.    

        (a)    Special Definitions.    For purposes of this Section 8, the following definitions apply: 

        (1)   "Options" shall mean rights, options, or warrants to subscribe for, purchase or otherwise acquire either Common Stock or
Convertible Securities (defined below). 

        (2)   "Convertible Securities" shall mean any evidences of indebtedness, shares (other than Common Stock) or other securities
convertible into or exchangeable for Common Stock. 

        (3)   "Additional Shares of Common Stock" shall mean all shares of Common Stock issued (or pursuant to Section 8(c),
deemed to be issued) by the Company after the Issue Date, other than shares of Common Stock issued or issuable: 

        (A)  to
officers, directors or employees of, or consultants to, the Company pursuant to any compensation agreement, plan or arrangement or the issuance of Common Stock upon
the exercise of any such options or warrants; 

        (B)  upon
conversion of existing convertible securities outstanding as of the Issue Date; 

        (C)  upon
exercise of outstanding warrants existing as of the Issue Date (including, without limitation, the Investor Warrants); 

        (D)  to
an institution or bank lender in connection with a loan transaction or equipment lease; 

        (E)  to
any persons or entities as consideration for an acquisition by the Company of any entity or business, or other similar transaction; or 

        (F)  upon
exercise of Investor Warrants or conversion of the Notes, in each case whether or not existing as of the Issue Date. 

        (b)    No Adjustment of Exercise Price.    Any provision herein to the contrary notwithstanding, no adjustment in the
Exercise Price shall be made in respect of the issuance of Additional Shares of 

5

 

Common
Stock unless the consideration per share (determined pursuant to Section 8(e) hereof) for an Additional Share of Common Stock issued or deemed to be issued by the Company is less than
the Exercise Price in effect on the date of, and immediately prior to such issue. 

        (c)    Deemed Issue of Additional Shares of Common Stock.    In the event the Company at any time or from time to time
after the Issue Date shall issue any Options or Convertible Securities or shall fix a record date for the determination of holders of any class of securities then entitled to receive any such Options
or Convertible Securities, then the maximum number of shares (as set forth in the instrument relating thereto without regard to any provisions contained therein designed to protect against dilution)
of Common Stock issuable upon the exercise of such Options or, in the case of Convertible Securities and Options therefor, the conversion or exchange of such Convertible Securities, shall be deemed to
be Additional Shares of Common Stock issued as of the time of such issue or, in case such a record date shall have been fixed, as of the close of business on such record date, provided further that in
any such case in which Additional Shares of Common Stock are deemed to be issued: 

        (1)   no
further adjustments in the Exercise Price shall be made upon the subsequent issue of Convertible Securities or shares of Common Stock upon the exercise of such
Options or conversion or exchange of such Convertible Securities; 

        (2)   if
such Options or Convertible Securities by their terms provide, with the passage of time or otherwise, for any increase in the consideration payable to the Company, or
decrease in the number of shares of Common Stock issuable, upon the exercise, conversion or exchange thereof, the Exercise Price computed upon the original issue thereof (or upon the occurrence of a
record date with respect thereto), and any subsequent adjustments based thereon, shall, upon any such increase or decrease becoming effective, be recomputed to reflect such increase or decrease
insofar as it affects such Options or the rights of conversion or exchange under such Convertible Securities; 

        (3)   no
readjustment pursuant to clause (2) above shall have the effect of increasing the Exercise Price to an amount which exceeds the lower of (A) the
Exercise Price on the original adjustment date, or (B) the Exercise Price that would have resulted from any issuance of Additional Shares of Common Stock between the original adjustment date
and such readjustment date. 

        (d)    Adjustment of Exercise Price Upon Issuance of Additional Shares of Common Stock.    In the event the Company,
at any time after the Issue Date shall issue Additional Shares of Common Stock (including Additional Shares of Common Stock deemed to be issued pursuant to Section 8(c)) without consideration
or for a consideration per share less than the Exercise Price in effect on the date of and immediately prior to such issue, then and in such event, the Exercise Price shall be reduced, concurrently
with such issue, (x) if the issuance or deemed issuance occurs prior to the completion of the Initial Public Offering, to a price (calculated to the nearest cent) equal to the price per share
received by the Company upon the issuance of Additional Shares of Common Stock or (y) if the issuance occurs following the completion of the Initial Public Offering, to a price (calculated to
the nearest cent) determined by multiplying such Exercise Price by a fraction, the numerator of which shall be the number of shares of Common Stock outstanding immediately prior to such issue plus the
number of shares of Common Stock which the aggregate consideration received by the Company for the total number of Additional Shares of Common Stock so issued would purchase at such Exercise Price in
effect immediately prior to such issuance, and the denominator of which shall be the number of shares of Common Stock outstanding immediately prior to such issue plus the number of such Additional
Shares of Common Stock so issued. For the purpose of the above calculation, the number of shares of Common Stock outstanding immediately prior to such issue shall be calculated on a fully diluted
basis, as if all Convertible Securities had been fully converted into shares of Common Stock and any outstanding warrants, options or other rights for the purchase of shares of stock or convertible 

6

 

securities
had been fully exercised (and the resulting securities fully converted into shares of Common Stock, if so convertible) as of such date. 

        (e)    Determination of Consideration.    For purposes of this Section 8, the consideration received by the
Company for the issue of any Additional Shares of Common Stock shall be computed as follows: 

        (1)    Cash and Property.    Such consideration shall: 

        (A)  insofar
as it consists of cash, be computed at the aggregate amount of cash received by the Company excluding amounts paid or payable for accrued interest or accrued
dividends; 

        (B)  insofar
as it consists of property other than cash, be computed at the fair value thereof at the time of such issue, as determined in good faith by the Board of
Directors of the Company; and 

        (C)  in
the event Additional Shares of Common Stock are issued together with other shares or securities or other assets of the Company for consideration which covers both, be
the proportion of such consideration so received, computed as provided in clauses (A) and (B) above, as determined in good faith by the Board of Directors of the Company. 

        (2)    Options and Convertible Securities.    The consideration per share received by the Company for Additional
Shares of Common Stock deemed to have been issued pursuant to Section 8(c), relating to Options and Convertible Securities shall be determined by dividing 

        (A)  the
total amount, if any, received or receivable by the Company as consideration for the issue of such Options or Convertible Securities, plus the minimum aggregate
amount of additional consideration (as set forth in the instruments relating thereto, without regard to any provision contained therein designed to protect against dilution) payable to the Company
upon the exercise of such Options or the conversion or exchange of such Convertible Securities, or in the case of Options for Convertible Securities, the exercise of such Options for Convertible
Securities and the conversion or exchange of such Convertible Securities by 

        (B)  the
maximum number of shares of Common Stock (as set forth in the instruments relating thereto, without regard to any provision contained therein designed to protect
against the dilution) issuable upon the exercise of such Options or conversion or exchange of such Convertible Securities. 

        SECTION
9.    Elimination of Fractional Interests.    The Company shall not be required to issue certificates
representing fractions of shares of Common Stock upon the exercise of the Warrants, nor shall it be required to issue scrip or pay cash in lieu of fractional interests, it being the intent of the
parties that all fractional interests shall be eliminated by rounding any fraction up to the nearest whole number of shares of Common Stock, or other securities, properties or rights. 

        SECTION
10.    Registrations; Listing of Securities.    The Company covenants and agrees that if any shares of capital
stock to be reserved for the purpose of the issuance of shares upon the exercise of the Warrants requires registration with or approval of any governmental or other authority under any federal or
state law before such shares may be validly issued or delivered upon exercise, then the Company will in good faith and as expeditiously as possible endeavor to secure such registration or approval, as
the case may be. If and so long as the Common Stock issuable upon the exercise of the Warrants is listed on any national securities exchange or automated quotation system, the Company will, if
permitted by the rules of such exchange or automated quotation system, as applicable, list and keep listed on such exchange or automated quotation system, as applicable, upon official notice of
issuance, all shares of such Common Stock issuable upon exercise of the Warrants. 

7

 

        SECTION
11.    Rights of Warrant Holder.    If at any time prior to the expiration of the Warrants and their exercise
any of the following actions, activities, events, circumstances, conditions or transactions shall be proposed: 

        (a)   the
Company shall issue (by distribution or otherwise) or sell or transfer any Common Stock or any other capital stock of the Company or rights, warrants or options or
convertible, exercisable or exchangeable securities containing the right to subscribe for, convert, exercise or exchange into or
purchase any additional shares of Common Stock or shares of any other capital stock of the Company or any other securities, rights, warrants or options; 

        (b)   the
Company shall effect any reorganization, recapitalization or reclassification of its Common Stock or its other capital stock; 

        (c)   the
Company shall effect any reorganization, consolidation, merger or sale, transfer, lease or other disposition of all or substantially all of the property, assets,
stock or business of the Company; 

        (d)   the
Company shall effect an initial public offering of Company Stock; 

        (e)   the
Company shall apply any property or assets to the purchase, acquisition, redemption or retirement of any of its Common Stock or its other capital stock; or 

        (f)    the
Company shall effect a dissolution, liquidation or winding up of the Company, 

then
in any one or more of said events, the Company shall give written notice of such event to the Holder at least thirty (30) days prior to the date fixed as a record date or the date of
closing the transfer books for the determination of the stockholders entitled to any such asset or to vote on such proposed action, activity, event, circumstance, condition or transaction, or the
consummation of such action, activity, event, circumstance, condition or transaction if no vote of stockholders is required. 

        SECTION
12.    Limitation of Rights.    No mere enumeration herein of the rights or privileges of the Holder shall
give rise to any rights or privileges of the Holder as a stockholder of the Company solely by reason of its ownership of the Warrants. 

        SECTION
13.    Notices.    Except as otherwise expressly specified herein, all notices, requests and other
communications required or permitted hereunder shall be in writing and shall be sent by an internationally recognized overnight courier service; by certified or registered mail, return receipt
requested (or, in the case of a notice sent to an address in Italy, by international express mail, return receipt requested); by facsimile transmission or by hand delivery. 

	if to the Company:	 	Gentium S.p.A.

Piazza XX Settembre, n.2

22079 Villa Guardia Como

Italy
	

 	
 	

Attention: Dott. Sauro Carsana

Fax:            +39 031 385333

and,
if to the Holder, to the name and address specified with respect to such person or entity in the Warrant register maintained by the Company pursuant to Section 2(c) hereof. 

Any
party may designate a different notice address, contact person, telephone number or facsimile number with respect to such party by providing a notice describing such changes to the other party
hereto in accordance with the provisions of this Section 13. Any notice sent by internationally recognized overnight mail courier service shall be deemed to be delivered to the address shown on
the mailing receipt on the expected date of delivery upon proper evidence of mailing for purposes of this Section 13. Any notice sent by certified or registered mail, return receipt requested
(or, in the case of a 

8

 

notice
sent to an address in Italy, by international express mail, return receipt requested), shall be deemed to be delivered five business days after mailing. Any notice sent by facsimile
transmission shall be deemed delivered as of the open of business on the business day following the date on which sent provided the sender receives written confirmation of transmission and provided
that within 24 hours such notice is also sent by regular mail or by an internationally-recognized overnight mail courier service to the appropriate address specified above. Any notice sent by
hand delivery shall be deemed delivered as of the date of delivery. 

        SECTION
14.    Successors.    This Warrant Certificate shall be binding upon and inure to the benefit of the Company
and the Holder and their respective successors and permitted assigns; provided that the Company may not assign, delegate or otherwise transfer any of
its rights or obligations under this Warrant Certificate without the written consent of the Holder. Holders may assign its rights hereunder in the manner and to the persons permitted under the
Investors' Rights Agreement. 

        SECTION
15.    Governing Law; Submission to Jurisdiction.    

        (a)   This
Warrant Certificate shall be construed in accordance with and governed by the laws of the State of New York without regard to principles of conflicts of laws,
except for the application of the provisions of Italian law concerning the proper authorization and issuance of warrant certificates. 

        (b)   In
case of any inconsistencies between the text of this Warrant Certificate and the text of the Company Resolution, the text of the Company Resolution shall govern. 

        (c)   IN
THE EVENT THAT A JUDICIAL PROCEEDING IS NECESSARY, THE EXCLUSIVE FORUMS FOR RESOLVING DISPUTES ARISING OUT OF OR RELATING TO THIS AGREEMENT ARE EITHER THE SUPREME
COURT OF THE STATE OF NEW YORK IN AND FOR THE COUNTRY OF NEW YORK OR THE FEDERAL COURTS FOR SUCH STATE AND COUNTRY, AND ALL RELATED APPELLATE COURTS, THE PARTIES HEREBY IRREVOCABLY CONSENT TO THE
JURISDICTION OF SUCH COURTS AND AGREE TO SAID VENUE. 

        SECTION
16.    WAIVER OF TRIAL BY JURY.    TO THE EXTENT IT MAY
LEGALLY DO SO, THE COMPANY EXPRESSLY WAIVES ANY RIGHT TO TRIAL BY JURY OF ANY CLAIM, DEMAND, ACTION, CAUSE OF ACTION OR PROCEEDING ARISING UNDER OR WITH RESPECT TO THIS WARRANT CERTIFICATE OR IN ANY
WAY CONNECTED WITH, OR RELATED TO, OR INCIDENTAL TO, THE DEALINGS OF THE COMPANY AND THE HOLDER WITH RESPECT TO THIS WARRANT CERTIFICATE OR THE TRANSACTIONS RELATED HERETO, IN EACH CASE WHETHER NOW
EXISTING OR HEREAFTER ARISING, AND IRRESPECTIVE OF WHETHER SOUNDING IN CONTRACT, TORT OR OTHERWISE. TO THE EXTENT IT MAY LEGALLY DO SO, THE COMPANY AGREES THAT ANY SUCH CLAIM, DEMAND, ACTION, CAUSE OF
ACTION OR PROCEEDING SHALL BE DECIDED BY A COURT TRIAL WITHOUT A JURY AND THAT THE HOLDER MAY FILE AN ORIGINAL COUNTERPART OR A COPY OF THIS SECTION 16 WITH ANY COURT AS WRITTEN EVIDENCE OF THE
CONSENT OF THE COMPANY TO WAIVER OF ITS RIGHT TO TRIAL BY JURY.

        SECTION
17.    Amendments; Modifications.    This Warrant Certificate may be amended or modified only with the written
consent of the Company and the holders representing a majority-in-interest of the then outstanding Investor Warrants which holders shall include Generation Capital Associates,
and any provision of this Warrant Certificate may be waived only by the party (or in the case of the Holder, by holders representing a majority-in-interest of the
then-outstanding Investor Warrants which holders shall include Generation Capital Associates) against whom enforcement of any waiver is sought; provided, that no such amendment,
modification or waiver shall be binding on a Holder without such Holder's consent in the event that the amendment, modification or waiver 

9

 

(i) increases
the Exercise Price of any Investor Warrant; or (ii) reduces the amount of Shares covered by any Investor Warrant. 

        SECTION
18.    Severability.    The holding of any provision of this Warrant to be invalid or unenforceable by a court
of competent jurisdiction shall not affect any other provision of this Warrant, which shall remain in full force and effect. If any provision of this Warrant shall be declared by a court of competent
jurisdiction to be invalid, illegal or incapable of being enforced in whole or in part, such provision shall be interpreted so as to remain enforceable to the maximum extent permissible consistent
with applicable law and the remaining conditions and provisions or portions thereof shall nevertheless remain in full force and effect and enforceable to the extent they are valid, legal and
enforceable, and no provisions shall be deemed dependent upon any other covenant or provision unless so expressed herein. 

        SECTION
19.    Specific Performance.    The Company acknowledges that irreparable damage would occur to the Holder in
the event that any of the provisions of this Warrant Certificate were not performed by the Company in accordance with their specific terms or were otherwise breached by the Company and that money
damages would not provide an adequate remedy to the Holder. It is accordingly agreed that the Holder shall be entitled to an injunction and other equitable remedies to prevent breaches by the Company
of this Warrant Certificate and to enforce specifically the terms and provisions hereof in any court of the United States or any state thereof or any other court having jurisdiction, this being in
addition to any other remedy to which the Holder may be entitled at law or in equity or otherwise. 

        SECTION
20.    Captions.    The section headings used herein are for convenience only and shall not affect the
construction hereof. 

        IN
WITNESS WHEREOF, the Company has caused this Warrant Certificate to be duly executed under its corporate seal as of the Issue Date specified on the face hereof. 

	 	 	GENTIUM S.P.A.
	

 	
 	

By:	
 	

 
	 	 	 	 	
 Name:

Title:

The
issuer hereby expressly agrees that the competent jurisdiction for any proceedings concerning this agreement is exclusively either the Supreme Court of the State of New York in and for the County
of New York or the federal courts for such State and County, and all related appellate courts. 

	 	 	GENTIUM S.P.A.
	

 	
 	

By:	
 	

 
	 	 	 	 	
 Name:

Title:

10

EXHIBIT A  

REGULATIONS FOR THE GENTIUM S.P.A.

ORDINARY SHARE WARRANTS  

Article 1—"Gentium S.p.A." ordinary share warrants  

        The extraordinary shareholders' meeting of "gentium S.p.A." (hereinafter also referred to as the "Company") resolved on September 30, 2004 and
October 8, 2004, among other things, to increase the company capital, in a divisible manner, by a nominal maximum of 881,100 Euros by means of the issue, which also may occur in more than one
installment, of a maximum number of 881,100 ordinary shares with a nominal value of 1 Euro each, destined exclusively and irrevocably, within the terms specified below in article 2,
section I, for the exercise of the purchase option for the holders of a maximum of of 8,010= warrants for ordinary "gentium S.p.A." shares (hereinafter referred to as the "Warrants") combined
free-of-charge with the maximum of 8,010= convertible notes which form the Note known as the "gentium S.p.A. Convertible Note," whose issue was decided during the same
extraordinary Board Meeting. 

        On
the basis of this decision, the holders of Warrants will have the right to subscribe, following the methods and terms indicated in these Regulations in Article 2 below, a
maximum number of 881,100 ordinary "Gentium S.p.A." with-coupon shares, again at the price indicated below in Article 2, par. I, lett. B. 

        The
securities are nominative; the Warrants may circulate separately from the notes to which they are combined starting from the issue date, this date being understood as the date
indicated on the corresponding certificate (hereinafter referred to as the "Issue Date"). 

        Note
the following: 

        (i)    the
Warrants may be placed exclusively with qualified investors in the United States of America and in Europe (in compliance with the applicable law and regulations in
force in Italy and in the other countries of the European Union), while it is expressly forbidden any public solicitation in Italy by the Company; 

        (ii)   the
proper authorization and the issuance of the Warrants are governed by Italian Law; with regard to circulation of the Warrants, however, the Company Board of
Directors, subject to the aforesaid limits, may add to, expand and modify this Regulation, also to ensure that it complies with the current legal regulations and standards of the United States of
America. 

        The
Warrants issued under this Regulations and the securities which may be issued upon their exercise are not registered under the Securities Act of 1933 and subsequent modifications
(hereinafter referred to as the "Act") or any state securities laws and neither these securities nor any interest therein may be offered, sold, used as guarantee, disposed of or otherwise transferred
unless (1) a registration statement with respect thereto is valid under the terms of the Act and any applicable state securities laws or (2) the Issuer receives the opinion of counsel to
the holder of these securities, the counsel and the opinion being reasonably satisfactory to the Issuer, stating that these securities may be offered, sold, used as guarantee, disposed of or
transferred in the manner contemplated without an effective registration statement under the Act or any applicable state securities laws. 

Article 2—Warrant use methods—issuing the certificates  

        The holder of the Warrant may ask to subscribe ordinary "Gentium S.p.A." shares for every Warrant owned, in accordance with the number and subscription price
specified in this article: 

	I)
	Number

	•
	for
the notes and the corresponding Warrants subscribed by October 15, 2004, a number of shares corresponding to 66% of the nominal value of the notes subscribed,
divided by the lower of the following: $10.00 and the price of the initial public offering of the shares of common stock of the Company on Nasdaq National or SmallCap Market, American Stock Exchange
or 

 

New
York Stock Exchange (hereinafter referred to as the "Initial Public Offering" or "IPO"), provided that such price shall not be less than $6.00 per share. 

	•
	for
the notes and the corresponding Warrants subscribed after October 15, 2004, a number of shares corresponding to 40% of the nominal value of the notes subscribed,
divided by the lower of the following: $10.00 and the price of the IPO (but not less than $6.00 per share).

	II)
	Date

        The
option may be exercised starting from the first of the following dates: 

        (i)    the
closing of the Initial Public Offering; 

        (ii)   one
year after the Warrant Issue Date; 

and
until 5 p.m. (New York City time) on the last of the following dates (hereinafter referred to as the "Expiry Date"): 

        (i)    five
years and three months after the Warrant Issue Date; 

        (ii)   four
years and three months after the completion of the IPO. 

	III)
	Price

        The
Subscription Price for the exercise of the right incorporated within the Warrants for the subscription of newly-issued ordinary "Gentium S.p.A." shares for every Warrant owned shall
be: 

        (i)    110%
of the price per share of the shares placed by the Company in the IPO (but not less than $6.00 per share); or 

        (ii)   US$6
if the Issuer Company has not completed the IPO within one year of the Warrant Issue Date. 

	IV)
	The
holder of the Warrant may exercise the subscription option, in full or in part, at any time, before 5 p.m. (New York City time) on the Expiry Date, by sending the
corresponding request (Purchase Option) by fax to the legal headquarters of the Company, drafted in full compliance with the model prepared by the Company, duly signed and following these methods: 

        (i)    within
10 (ten) days of the delivery of the Purchase Option to the Company, the holder of the Warrant must deliver to the Company the corresponding Warrant certificate
by means of registered letter with notification of receipt, by courier service or by hand with hand-written receipt; 

        (ii)   this
delivery must be accompanied by full payment of the price for each share for which the Warrants are being exercised, by means of wire transfer or by the other
payment methods permitted by the Company. 

        Within
5 (five) working says of receipt of the certificate for the Warrant, accompanied by payment of the price and payment of any duties due for the transfer of the securities, the
Company must arrange that the Warrant holder is issued with the nominative share certificate representing the ordinary Company shares purchased as illustrated above. 

	V)
	The
Warrants and the share certificates must be issued by the Company in compliance with the Company Bylaws. 

        The
Warrants must indicate the corresponding issue date and be signed, on behalf of the Company, by a legal representative of the Company. 

        If
the legal representative of the Company who has signed a Warrant no longer has this role after the Issue Date, this Warrant is still valid. 

2

 

	VI)
	If
the holder of the Warrant does not fully exercise the rights assigned to him by the security, at the request of the holder himself, a new Warrant corresponding to the rights not
exercised will be issued by the Company.

	VII)
	Each
Warrant, if the right is not exercised before 5 p.m. (New York City time) on the Expiry Date, will be considered null and void and all the corresponding rights will be
null and void from that time.

	VIII)
	The
current legal headquarters of the Company is Piazza XX Settembre 2, Villa Guardia, 22079 Como, Italy. 

        A
register of the Warrant holders, which must indicate all the corresponding issues and transfers will be set up and maintained by the Company at the legal headquarters. 

Article 3—Rights of the Warrant holders in the event of "Gentium S.p.A." company capital operations  

        If the Company enters into extraordinary transactions on the company capital which affect the Warrant issue and use terms and conditions, the number of shares
which may be obtained if the Warrants are used and the corresponding price per share will be modified to implement the agreements between the Company and the Warrant subscribers, in compliance with
the applicable Laws and standards. 

Article 4—Using the Warrant while registration as per the Act is pending  

        Notwithstanding any other provision in this Regulation to the contrary, if on or prior to the one (1) year anniversary of the Initial Public Offering, the
Company has not yet filed with the Securities and Exchange Commission a registration statement with respect to the resale by the holder of the shares, or if a registration statement has been so filed
but is not effective at any time following the fourteen (14) month anniversary of the Initial Public Offering, then for as long as no such registration statement is effective with a current
prospectus available, the warrants may also be exercised, in whole or in part, at any time prior to the Expiration Date, by means of a calculation method in which the holder shall be entitled to
receive a certificate for the number of shares equal to the quotient obtained by dividing [(A-B)(X)] by (A), where: 

	(A)
	=
the closing bid price on the trading day preceding the date of such election;

	(B)
	=
the exercise price of the warrants, as adjusted in accordance with the methods specified in article 3 above; and

	(X)
	=
the number of shares issuable upon exercise of the warrants in accordance with the terms of this Regulation. 

Article 5—Rights of the Warrant holders  

        If, at any time before the expiration date of the Warrants and their exercise thereof, any of the following actions, activities, events, circumstances,
conditions, or transactions should be proposed: 

        (a)   the
Company issues (by distribution or with other methods), sells or transfers any ordinary shares or other Company capital, rights, warrants, options or convertible,
usable or exchangeable securities containing the right to subscribe, convert, use, exchange or purchase additional ordinary shares or shares in any other Company capital or any other security, right,
warrant or option; 

        (b)   the
Company reorganizes, recapitalizes or reclassifies its ordinary shares or its Company capital; 

3

 

        (c)   the
Company reorganizes, consolidates, incorporates, sells, transfers, leases or in any other way disposes of all or a quite substantial part of its property, goods,
Company capital or Company activities; 

        (d)   the
Company organizes a public share issue; 

        (e)   the
Company uses any property or activity to purchase, acquire, reimburse or withdraw any of its ordinary shares or its Company capital; 

        (f)    the
Company winds up or liquidates the Company, 

the
Company must send a written note to the holder of the Warrant at least 30 (thirty) days before the date set for the implementation of the corresponding decisions, actions, activities, events,
circumstances, conditions or transactions, even if no Shareholder vote must be required by Law for these. 

Article 6—Tax system  

        The capital gains deriving from the sale of subscription warrants for shareholdings in companies resident in Italy, unless attained within the exercise of arts
and professions or business, are considered as income not of a financial nature. 

        These
capital gains are subject to taxation as follows: 

	•
	the
capital gains deriving from the transfer of warrants, also possibly sold to different subjects over 12 months, even if pertaining to different taxation periods,
and which permit the acquisition of a qualified shareholding as defined by Article 67, clause I, letter c) of Italian Presidential Decree 917/1986 (T.U.I.R.—Single
Income Tax Law Text), also considering for these purposes the direct transfer of the shareholdings and other rights carried out in the same
12-month period, are taxable at 40% and are used, net of 40% of any capital losses in the same taxation period or in the 4 previous taxation periods, to establish the overall income of the
taxpayer in his income tax returns;

	•
	the
capital losses deriving from the transfer of warrants which, again sold over 12 months, also, possibly, to different subjects, do not permit, even together with
the direct transfer of the shareholdings and other rights, the acquisition of a qualified shareholding are fully taxable and are subject, net of any capital losses other than those for qualified
shareholdings, to a substitute tax at a rate of 12.5%. 

        The
capital gains earned by subjects not resident in Italy, if pertaining to non-qualified shareholdings, created by the transfer of warrants, negotiated in regulated Italian
or foreign markets, are not subject to this substitute tax referred to above. 

        If,
on the other hand, the capital gains are non-qualified gains created by the transfer of warrants for non-listed companies, these capital gains are not taxable
only if the transferor is resident in a Country which permits an adequate exchange of information with Italy (Article 5, clause. 5, Italian Legal Decree 461/97). 

        The
capital gains related to qualified shareholdings created by the same subjects are usually taxable in Italy, notwithstanding the application of more favorable conventional systems
deriving from the standards put in place to prevent double taxation and agreed by Italy with the Countries in which the taxpayer reside. 

        These
agreements generally envisage, to comply with certain conditions, the taxation of the capital gains in the Country of the transferor. 

        For
further references to and details about the taxation systems for the incomes referred to above and any eventual corresponding clashes with the separate regulations for capital
income, refer to Italian 

4

 

Legal
Decree 461 of 21/11/1997, as subsequently modified, and to the Single Income Tax Law Text (T.U.I.R.), and any further related administrative standards and legislation. 

Article 7—Notices  

        Except where otherwise specified in this document, all the notices and other communications required or permitted by these Regulations must be in written form and
sent by international courier with delivery within 24h, by registered or certified letter with notification of receipt (or, for a notice sent to an Italian address, by express international post with
notification of receipt), by fax or hand-delivered: 

To the Company:

Gentium, S.p.A.

Piazza XX Settembre 2

22079 Villa Guardia, Como

Italy

Attn.: Dott. Sauro Carsana

Fax: +39 031 385333 

To the Warrant holder:

        The
name and address specified in the Warrant Register maintained by the Company. 

        Each
of the Parties may select a different address, contact and telephone or fax number by sending a notice which informs the other Party of these changes in accordance with the
specifications in this article. 

        Any
notice sent by express international courier with delivery within 24h will be considered to be delivered to the address indicated in the postal address on the envisaged delivery date
with adequate proof of postal delivery as specified in this article. 

        Any
notice sent by registered or certified letter with notification of receipt (or, for a notice sent to an Italian address, by express international post with notification of receipt)
will be considered to be delivered five working days after it is sent. 

        Any
notice sent by fax will be considered to be delivered by the time of the start of the activities one working day after the date on which it was sent, provided the sender receives
written confirmation of
successful transmission and provided this notice is also sent by ordinary post or by express international courier with delivery within 24h to the address specified above within 24 hours. 

        Any
hand-delivered notice will be considered to be delivered on the delivery date. 

Article 8—Various  

        Ownership of the Warrants requires full acceptance of all the conditions established in these Regulations. 

        For
any disputes involving the Warrants and this Regulation, the exclusive forums are either the Supreme Court of the State of New York and for the County of New York, or the Federal
Courts for such State and County, and all related Appellate Courts. 

	/s/  Laura Iris Ferro      
 Laura Iris Ferro	 	 
	

/s/  Massimo Caspani      
 Notary Massimo Caspani	
 	

 

5

EXHIBIT B  

FORM
OF ELECTION TO PURCHASE 

        The
undersigned hereby irrevocably elects to exercise the right, represented by this Warrant Certificate, to
purchase                        shares of Common Stock, par value
€1 per share, of Gentium S.p.A. (the "Company") and herewith tenders payment of the exercise price, together with all applicable transfer taxes, if any. 

        Payment
shall take the form of (check applicable box): 

        [    ]
wire transfer pursuant to the provisions of Section 1(b) of the Warrant Certificate in the amount of
$                        , all in accordance
with the terms thereof; or 

        [    ]
the cancellation of such number of Shares as is necessary, in accordance with the formula set forth in Section 1(f), to exercise this
Warrant with respect to the maximum number of Shares purchasable pursuant to the cashless exercise procedure set forth in Section 1(f). 

        The
undersigned requests that a certificate for such shares of Common Stock be registered in the name of                        whose
address is                        and that such certificate be
delivered to                        whose address
is                        . 

	Dated:	 	Signature	 	 
	 	 	 	 	

	 	 	 	 	(Signature must conform in all respects to name of holder as specified on the face of the Warrant Certificate.)

(Insert
Social Security or Other

Identifying Number of Holder) 

EXHIBIT C  

TRANSFER
FORM 

        For
value received, the undersigned Holder of the within Warrant Certificate hereby sells, assigns and transfers unto the transferee whose name and address are set forth below all of the
right, title and interest of the undersigned in, to and under the within Warrants (to the extent of the portion of the within Warrant Certificate being transferred hereby, which portion is
                        Warrants). 

        Name
of Transferee: 

        State
of Organization (if applicable): 

        Federal
Tax Identification or Social Security Number: 

        Address:

        If
such portion of the Warrants being transferred shall not consist of all of the within Warrant Certificate, then the undersigned hereby requests that, as provided in Section 5
of the within Warrant Certificate, a new Warrant Certificate of like tenor respecting the balance of the Warrants not being transferred pursuant hereto be issued in the name of and delivered to the
undersigned. 

	Dated:	 	[HOLDER]
	

 	
 	

By:	

 
	 	 	 	
 Name:

Title:
	

 	
 	

(Signature must conform in all respects to name of holder as specified on the face of the Warrant Certificate.)

QuickLinks

Exhibit 4.2.2QuickLinks
 -- Click here to rapidly navigate through this document

 
 

Exhibit 4.2.4    
    

 
 

INVESTORS' RIGHTS AGREEMENT    
    

October 15,
2004 

 
 
 

Table of Contents    
    

	 
	 	 
	 	 
	 	Page

	1.	 	Registration Rights	 	1
	

 	
 	

1.1.	
 	

Definitions	
 	

1
	

 	
 	

1.2.	
 	

Request for Registration	
 	

2
	

 	
 	

1.3.	
 	

Company Registration	
 	

3
	

 	
 	

1.4.	
 	

Obligations of the Company	
 	

4
	

 	
 	

1.5.	
 	

Furnish Information	
 	

5
	

 	
 	

1.6.	
 	

Expenses of Demand Registration	
 	

5
	

 	
 	

1.7.	
 	

Expenses of Company Registration	
 	

5
	

 	
 	

1.8.	
 	

Underwriting Requirements	
 	

5
	

 	
 	

1.9.	
 	

Delay of Registration	
 	

6
	

 	
 	

1.10.	
 	

Indemnification	
 	

6
	

 	
 	

1.11.	
 	

Reports Under Securities Exchange Act of 1934	
 	

8
	

 	
 	

1.12.	
 	

Form F-3 Registration	
 	

8
	

 	
 	

1.13.	
 	

Assignment of Registration Rights	
 	

9
	

 	
 	

1.14.	
 	

"Market Stand-Off" Agreement	
 	

10
	

 	
 	

1.15.	
 	

Termination of Registration Rights	
 	

10
	

2.	
 	

Covenants of the Company	
 	

10
	

 	
 	

2.1.	
 	

Delivery of Financial Statements	
 	

10
	

 	
 	

2.2.	
 	

Inspection	
 	

11
	

 	
 	

2.3.	
 	

Termination of Information and Inspection Covenants	
 	

11
	

 	
 	

2.4.	
 	

Right of First Refusal on Dispositions	
 	

11
	

 	
 	

2.5.	
 	

Drag-Along Rights	
 	

13
	

3.	
 	

Miscellaneous	
 	

13
	

 	
 	

3.1.	
 	

Successors and Assigns	
 	

13
	

 	
 	

3.2.	
 	

Governing Law	
 	

13
	

 	
 	

3.3.	
 	

Counterparts	
 	

14
	

 	
 	

3.4.	
 	

Titles and Subtitles	
 	

14
	

 	
 	

3.5.	
 	

Notices	
 	

14
	

 	
 	

3.6.	
 	

Expenses	
 	

14
	

 	
 	

3.7.	
 	

Amendments and Waivers	
 	

14
	

 	
 	

3.8.	
 	

Severability	
 	

14
	

 	
 	

3.9.	
 	

Entire Agreement	
 	

15
	

Schedule A Schedule of Investors	
 	

 

i

  

 
 

INVESTORS' RIGHTS AGREEMENT    
    

        THIS INVESTORS' RIGHTS AGREEMENT is made as of the 15th day of October, 2004, by and between Gentium S.p.A., a joint stock company  (società per
azioni) incorporated and organized under the laws of the Republic of Italy (the "Company"), and the investors listed on
Schedule A hereto, each of which is herein referred to as an "Investor." 

 
 

RECITALS    
    

        WHEREAS, each Investor has executed and delivered to the Company, and the Company has accepted from each Investor, a Subscription Agreement (a "Subscription
Agreement" and collectively, the "Subscription Agreements"); 

        WHEREAS,
in order to induce the Company to enter into the Subscription Agreements and to induce the Investors to invest funds in the Company pursuant to the Subscription Agreements, the
Investors and the Company hereby agree that this Agreement shall govern the rights of the Investors to cause the Company to register the shares of Common Stock underlying the notes and warrants issued
to the Investors pursuant to the Subscription Agreements and certain other matters as set forth herein; 

        NOW,
THEREFORE, THE PARTIES HEREBY AGREE AS FOLLOWS: 

        1.    Registration Rights.    The Company covenants and agrees as follows: 

        1.1.    Definitions.    For purposes of this Agreement: 

        (a)   The
term "Act" means the Securities Act of 1933, as amended. 

        (b)   The
term "Common Stock" means the common stock, par value €1 per share, of the Company. 

        (c)   The
term "Form F-3" means such form under the Act as in effect on the date hereof or any registration form under the Act subsequently adopted by the
SEC which permits inclusion or incorporation of substantial information by reference to other documents filed by the Company with the SEC. 

        (d)   The
term "Holder" means the Investors and any person owning or having the right to acquire Registrable Securities or any person to whom the rights under Section 1
have been transferred in accordance with Section 1.13 hereof. 

        (e)   The
term "1934 Act" means the Securities Exchange Act of 1934, as amended. 

        (f)    The
term "Majority Shareholder" means Finanziaria Sirton S.p.A. 

        (g)   The
term "Notes" means those certain Series A Senior Convertible Notes of the Company issued by the Company, and purchased by the Investors, pursuant to the
Subscription Agreements. 

        (h)   The
term "register," "registered," and "registration" refer to a registration effected by preparing and filing a registration statement or similar document in compliance
with the Act, and the declaration or ordering of effectiveness of such registration statement or document. 

        (i)    The
term "Registrable Securities" means (i) the Shares, (ii) any additional shares of Common Stock issuable upon adjustment to the exercise price of the
Warrants or the conversion price of the Notes (the "Additional Shares"), and (iii) any other securities issued or issuable with respect to or in exchange of any of the securities referenced in
(i), (ii) or (iii), subject to appropriate adjustment for any stock split, stock dividend, recapitalization, merger or other reorganization; provided, however, that securities shall only be
treated as Registrable 

1

 

Securities
if and only for so long as they (A) have not been disposed of pursuant to a registration statement declared effective by the SEC; (B) have not been sold in a transaction
exempt from the registration and prospectus delivery requirements of the Act so that all transfer restrictions and restrictive legends with respect thereto are removed upon the consummation of such
sale; (C) are held by a Holder or a permitted transferee pursuant to Section 1.13; or (D) have not become eligible for sale pursuant to Rule 144(k) (or any successor
thereto) under the Act. 

        (j)    The
number of shares of "Registrable Securities then outstanding" shall be determined by the number of shares of Common Stock outstanding which are, and the number of
shares of Common Stock issuable pursuant to then exercisable or convertible securities which are, Registrable Securities. 

        (k)   The
term "SEC" shall mean the Securities and Exchange Commission. 

        (l)    The
term "Securities" means the Notes, the Warrants and the Shares. 

        (m)  The
term "Shares" means (i) the shares of Common Stock issued or issuable upon exercise of the Warrants; and (ii) the shares of Common Stock issued or
issuable upon conversion of the Notes. 

        (n)   The
term "Warrants" means those certain warrants to purchase shares of Common Stock issued by the Company, and purchased by the Investors, pursuant to the Subscription
Agreements. 

        1.2.    Request for Registration.    

        (a)   If
the Company shall receive at any time after one (1) year following the effective date of the first registration statement for a public offering of securities
of the Company (other than a registration statement relating either to the sale of securities to employees of the Company pursuant to a stock option, stock purchase or similar plan or an SEC
Rule 145 transaction), a written request from the Holders of a majority of the Registrable Securities then outstanding that the Company file a registration statement under the Act
covering the registration of the Registrable Securities then outstanding with an aggregate offering price, net of underwriting discounts and commissions of no less than two million five hundred
thousand dollars ($2,500,000), then the Company shall: 

          (i)  within
ten (10) days of the receipt thereof, give written notice of such request to all Holders in accordance with Section 3.5; and 

         (ii)  effect
as soon as practicable the registration under the Act of all Registrable Securities which the Holders request to be registered, subject to the limitations of
subsection 1.2(b), within twenty (20) days of the mailing of such notice by the Company in accordance with Section 3.5. 

        (b)   If
the Holders initiating the registration request hereunder ("Initiating Holders") intend to distribute the Registrable Securities covered by their request by means of
an underwriting, they shall so advise the Company as a part of their request made pursuant to subsection 1.2(a), and the Company shall include such information in the written notice referred to in
subsection 1.2(a). The underwriter will be selected by the Company and shall be reasonably acceptable to a majority in interest of the Initiating Holders. In such event, the right of any Holder to
include his Registrable Securities in such registration shall be conditioned upon such Holder's participation in such underwriting and the inclusion of such Holder's Registrable Securities in the
underwriting (unless otherwise mutually agreed by a majority in interest of the Initiating Holders and such Holder) to the extent provided herein. All Holders proposing to distribute their securities
through such underwriting shall (together 

2

 

with
the Company as provided in subsection 1.4(e)) enter into an underwriting agreement in customary form with the underwriter or underwriters selected for such underwriting. Notwithstanding any other
provision of this Section 1.2, if the underwriter advises the Initiating Holders in writing that marketing factors require a limitation of the number of shares to be underwritten, then the
Initiating Holders shall so advise the Company, and the Company shall so advise all Holders of Registrable Securities which would otherwise be underwritten pursuant hereto, and the number of shares of
Registrable Securities that may be included in the underwriting shall be allocated among all Holders thereof in accordance with Section 1.8 hereof. 

        (c)   Notwithstanding
the foregoing, if the Company shall furnish to Holders requesting a registration statement pursuant to this Section 1.2, a certificate signed by
the Chief Executive Officer of the Company stating that in the good faith judgment of the Board of Directors of the Company, it would be seriously detrimental to the Company and its shareholders for
such registration statement to be filed and it is therefore essential to defer the filing of such registration statement, the Company shall have the right to defer taking action with respect to such
filing for a period of not more than 120 days after receipt of the request of the Initiating Holders; provided, however, that the Company may not utilize this right more than once in any
twelve-month period. 

        (d)   In
addition, the Company shall not be obligated to effect, or to take any action to effect, any registration pursuant to this Section 1.2: 

          (i)  After
the Company has effected two registrations pursuant to this Section 1.2 and such registrations have been declared or ordered effective; 

         (ii)  During
the period starting with the date sixty (60) days prior to the Company's good faith estimate of the date of filing of, and ending on a date one hundred
eighty (180) days (or, if shorter, the date the market stand-off time period expires pursuant to Section 1.14) after the effective date of, a registration subject to
Section 1.3 hereof; provided that the Company is actively employing in good faith all reasonable efforts to cause such registration statement to become effective; or 

        (iii)  If
the Initiating Holders propose to dispose of shares of Registrable Securities that are eligible to be registered on Form F-3 pursuant to a
request made pursuant to Section 1.12 below. 

        1.3.    Company Registration.    If (but without any obligation to do so) the Company proposes to register (including
for this purpose a registration effected by the Company for shareholders other than the Holders) any of its stock or other securities under the Act in connection with the public offering (other than
an initial public offering) of such securities solely for cash (other than a registration relating solely to the sale of securities to participants in a Company stock plan, a registration on any form
which does not include substantially the same information as would be required to be included in a registration statement covering the sale of the Registrable Securities or a registration in which the
only Common Stock being registered is Common Stock issuable upon conversion of debt securities which are also being registered), the Company shall, at such time, promptly give each Holder written
notice of such registration in accordance with Section 3.5. Upon the written request of each Holder given within twenty (20) days after mailing of such notice by the Company in
accordance with Section 3.5, the Company shall, subject to the provisions of Section 1.8, cause to be registered under the Act all of the Registrable Securities that each such Holder has
requested to be registered; provided, however, that the Company shall not be obligated to effect any such registration pursuant to this Section 1.3 if the Holders propose to sell Registrable
Securities at an aggregate price to the public (net of any underwriters' discounts or commissions) of less than one million dollars ($1,000,000). 

3

 

        1.4.    Obligations of the Company.    Whenever required under this Section 1 to effect the registration of any
Registrable Securities, the Company shall, as expeditiously as reasonably possible: 

        (a)   Prepare
and file with the SEC a registration statement with respect to such Registrable Securities and use its reasonable commercial efforts to cause such registration
statement to become effective, and, upon the request of the Holders of a majority of the Registrable Securities registered thereunder, keep such registration statement effective for a period of up to
one hundred twenty (120) days or until the distribution contemplated in the Registration Statement has been completed; provided, however, that (i) such 120-day period shall
be extended for a period of time equal to the period the Holder refrains from selling any securities included in such registration at the request of an underwriter of Common Stock (or other
securities) of the Company; and (ii) in the case of any registration of Registrable Securities on Form F-3 which are intended to be offered on a continuous or delayed basis,
such 120-day period shall be extended, if necessary, to keep the registration statement effective until all such Registrable Securities are sold, provided that Rule 415, or any
successor rule under the Act, permits an offering on a continuous or delayed basis, and provided further that applicable rules under the Act governing the obligation to file a
post-effective amendment permit, in lieu of filing a post-effective amendment which (I) includes any prospectus required by Section 10(a)(3) of the Act or
(II) reflects facts or events representing a material or fundamental change in the information set forth in the registration statement, the incorporation by reference of information required to
be included in (I) and (II) above to be contained in periodic reports filed pursuant to Section 13 or 15(d) of the 1934 Act in the registration statement. 

        (b)   Prepare
and file with the SEC such amendments and supplements to such registration statement and the prospectus used in connection with such registration statement as
may be necessary to comply with the provisions of the Act with respect to the disposition of all securities covered by such registration statement. 

        (c)   Furnish
to the Holders such numbers of copies of a prospectus, including a preliminary prospectus, in conformity with the requirements of the Act, and such other
documents as they may reasonably request in order to facilitate the disposition of Registrable Securities owned by them. 

        (d)   Use
its reasonable commercial efforts to register and qualify the securities covered by such registration statement under such other securities or Blue Sky laws of such
jurisdictions as shall be reasonably requested by the Holders; provided that the Company shall not be required in connection therewith or as a condition thereto to qualify to do business or to
file a general consent to service of process in any such states or jurisdictions. 

        (e)   In
the event of any underwritten public offering, enter into and perform its obligations under an underwriting agreement, in usual and customary form, with the managing
underwriter of such offering. Each Holder participating in such underwriting shall also enter into and perform its obligations under such an agreement. 

        (f)    Notify
each Holder of Registrable Securities covered by such registration statement at any time when a prospectus relating thereto is required to be delivered under the
Act of the happening of any event as a result of which the prospectus included in such registration statement, as then in effect, includes an untrue statement of a material fact or omits to state a
material fact required to be stated therein or necessary to make the statements therein not misleading in the light of the circumstances then existing. 

4

 

        (g)   Cause
all such Registrable Securities registered pursuant hereunder to be listed on each securities exchange on which similar securities issued by the Company are then
listed. 

        (h)   Provide
a transfer agent and registrar for all Registrable Securities registered pursuant hereunder and a CUSIP number for all such Registrable Securities, in each case
not later than the effective date of such registration. 

        (i)    Furnish,
at the request of any Holder requesting registration of Registrable Securities pursuant to this Section 1, on the date that such Registrable Securities
are delivered to the underwriters for sale in connection with a registration pursuant to this Section 1, if such securities are being sold through underwriters, (i) an opinion, dated
such date, of the counsel representing the Company for the purposes of such registration, in form and substance as is customarily given to underwriters in an underwritten public offering, addressed to
the underwriters, if any, and to the Holders requesting registration of Registrable Securities and (ii) a letter dated such date, from the independent certified public accountants of the
Company, in form and substance as is customarily given by independent certified public accountants to underwriters in an underwritten public offering, addressed to the underwriters and to the Holders
requesting registration of Registrable Securities. 

        1.5.    Furnish Information.    It shall be a condition precedent to the obligations of the Company to take any action
pursuant to this Section 1 with respect to the Registrable Securities of any selling Holder that such Holder shall furnish to the Company such information regarding itself, the Registrable
Securities held by it, and the intended method of disposition of such securities as shall be required to effect the registration of such Holder's Registrable Securities. 

        1.6.    Expenses of Demand Registration.    All expenses other than underwriting discounts and commissions incurred in
connection with registrations, filings or qualifications pursuant to Section 1.2, including (without limitation) all registration, filing and qualification fees, printers' and accounting fees,
fees and disbursements of counsel for the Company (including fees and disbursements of counsel for the Company in its capacity as counsel to the selling Holders hereunder; if Company counsel does not
make itself available for this purpose, the Company will pay the reasonable fees and disbursements of one counsel for the selling Holders) shall be borne by the Company; provided, however, that the
Company shall not be required to pay for any expenses of any registration proceeding begun pursuant to Section 1.2 if the registration request is subsequently withdrawn at the request of the
Holders of a majority of the Registrable Securities to be registered (in which case all participating holders shall bear such expenses), unless the Holders of a majority of the Registrable Securities
agree to forfeit their right to one demand registration pursuant to Section 1.2. 

        1.7.    Expenses of Company Registration.    The Company shall bear and pay all expenses incurred in connection with
any registration, filing or qualification of Registrable Securities with respect to the registrations pursuant to Section 1.3 for each Holder (which right may be assigned as provided in
Section 1.13), including (without limitation) all registration, filing, and qualification fees, printers and accounting fees relating or apportionable thereto and the fees and disbursements of
counsel for the Company in its capacity as counsel to the selling Holders hereunder; if Company counsel does not make itself available for this purpose, the Company will pay the reasonable fees and
disbursements of one counsel for the selling Holders selected by them, but excluding underwriting discounts and commissions relating to Registrable Securities. 

        1.8.    Underwriting Requirements.    In connection with any offering involving an underwriting of shares of the
Company's capital stock, the Company shall not be required under Section 1.2 or 1.3 to include any of the Holders' securities in such underwriting unless they accept the terms of the
underwriting as agreed upon between the Company and the underwriters selected by it (or by other persons entitled to select the underwriters), and then only in such quantity as the 

5

 

underwriters
determine in their sole discretion will not, jeopardize the success of the offering by the Company. If the total amount of securities, including Registrable Securities, requested by
shareholders to be included in such offering exceeds the amount of securities sold other than by the Company that the underwriters determine in their sole discretion is compatible with the success of
the offering, then the Company shall be required to include in the offering only that number of such securities, including Registrable Securities, which the underwriters determine in their sole
discretion will not jeopardize the success of the offering (the securities so included to be apportioned pro rata among the selling shareholders according to the total amount of securities entitled to
be included therein owned by each selling shareholder or in such other proportions as shall mutually be agreed to by such selling shareholders) but in no event shall the amount of securities of the
selling Holders included in the offering be reduced below fifty percent (50%) of the total amount of Registrable Securities requested by such selling Holders to be included in the offering, unless
there are no selling shareholders, other than the selling Holders, in the offering, in which case the amount of securities of the selling Holders included in the offering may be reduced below fifty
percent (50%). For purposes of the preceding parenthetical concerning apportionment, for any selling shareholder which is a holder of Registrable Securities and which is a partnership or corporation,
the partners, retired partners and shareholders of such holder, or the estates and family members of any such partners and retired partners and any trusts for the benefit of any of the foregoing
persons shall be deemed to be a single "selling shareholder," and any pro-rata reduction with respect to such "selling shareholder" shall be based upon the aggregate amount of shares
carrying registration rights owned by all entities and individuals included in such "selling shareholder," as defined in this sentence. 

        1.9.    Delay of Registration.    No Holder shall have any right to obtain or seek an injunction restraining or
otherwise delaying any such registration as the result of any controversy that might arise with respect to the interpretation or implementation of this Section 1. 

        1.10.    Indemnification.    In the event any Registrable Securities are included in a registration statement under
this Section 1: 

        (a)   To
the extent permitted by law, the Company will indemnify and hold harmless each Holder, any underwriter (as defined in the Act) for such Holder and each person, if
any, who controls such Holder or underwriter within the meaning of the Act or the 1934 Act, against any losses, claims, damages, or liabilities (joint or several) to which they may become subject
under the Act, or the 1934 Act, insofar as such losses, claims, damages, or liabilities (or actions in respect thereof) arise out of or are based upon any of the following statements, omissions or
violations (collectively a "Violation"): (i) any untrue statement or alleged untrue statement of a material fact contained in such registration statement, including any preliminary prospectus
or final prospectus contained therein or any amendments or supplements thereto, (ii) the omission or alleged omission to state therein a material fact required to be stated therein, or
necessary to make the statements therein not misleading, or (iii) any violation or alleged violation by the Company of the Act, the 1934 Act, or any rule or regulation promulgated under the
Act, or the 1934 Act; and the Company will pay to each such Holder, underwriter or controlling person any legal or other expenses reasonably incurred by them in connection with investigating or
defending any such loss, claim, damage, liability, or action; provided, however, that the indemnity agreement contained in this subsection 1.10(a) shall not apply to amounts paid in settlement of any
such loss, claim, damage, liability, or action if such settlement is effected without the consent of the Company (which consent shall not be unreasonably withheld), nor shall the Company be liable in
any such case for any such loss, claim, damage, liability, or action to the extent that it arises out of or is based upon a Violation which occurs in reliance upon and in conformity with written 

6

 

information
furnished expressly for use in connection with such registration by any such Holder, underwriter or controlling person. 

        (b)   To
the extent permitted by law, each selling Holder will indemnify and hold harmless the Company, each of its directors, each of its officers who has signed the
registration statement, each person, if any, who controls the Company within the meaning of the Act, any underwriter, any other Holder selling securities in such registration statement and any
controlling person of any such underwriter or other Holder, against any losses, claims, damages, or liabilities (joint or several) to which any of the foregoing persons may become subject, under the
Act or the 1934 Act insofar as such losses, claims, damages, or liabilities (or actions in respect thereto) arise out of or are based upon any Violation, in each case to the extent (and only to the
extent) that such Violation occurs in reliance upon and in conformity with written information furnished by such Holder expressly for use in connection with such registration; and each such Holder
will pay any legal or other expenses reasonably incurred by any person intended to be indemnified pursuant to this subsection 1.10(b), in connection with investigating or defending any such loss,
claim, damage, liability, or action; provided, however, that the indemnity agreement contained in this subsection 1.10(b) shall not apply to amounts paid in settlement of any such loss, claim, damage,
liability or action if such settlement is effected without the consent of the Holders of a majority-in-interest of the then outstanding Registrable Securities, which consent
shall not be unreasonably withheld; provided, that, in no event shall any indemnity under this subsection 1.10(b) exceed the gross proceeds from the offering received by such Holder. 

        (c)   Promptly
after receipt by an indemnified party under this Section 1.10 of notice of the commencement of any action (including any governmental action), such
indemnified party will, if a claim in respect thereof is to be made against any indemnifying party under this Section 1.10, deliver to the indemnifying party a written notice of the
commencement thereof and the indemnifying party shall
have the right to participate in, and, to the extent the indemnifying party so desires, jointly with any other indemnifying party similarly noticed, to assume the defense thereof with counsel mutually
satisfactory to the parties; provided, however, that an indemnified party (together with all other indemnified parties which may be represented without conflict by one counsel) shall have the right to
retain one separate counsel, with the reasonable fees and expenses to be paid by the indemnifying party, if representation of such indemnified party by the counsel retained by the indemnifying party
would be inappropriate due to actual or potential differing interests between such indemnified party and any other party represented by such counsel in such proceeding. The failure to deliver written
notice to the indemnifying party within a reasonable time of the commencement of any such action, if prejudicial to its ability to defend such action, shall relieve such indemnifying party of any
liability to the indemnified party under this Section 1.10, but the omission so to deliver written notice to the indemnifying party will not relieve it of any liability that it may have to any
indemnified party otherwise than under this Section 1.10. 

        (d)   If
the indemnification provided for in this Section 1.10 is held by a court of competent jurisdiction to be unavailable to an indemnified party with respect to
any loss, liability, claim, damage, or expense referred to therein, then the indemnifying party, in lieu of indemnifying such indemnified party hereunder, shall contribute to the amount paid or
payable by such indemnified party as a result of such loss, liability, claim, damage, or expense in such proportion as is appropriate to reflect the relative fault of the indemnifying party on the one
hand and of the indemnified party on the other in connection with the statements or omissions that resulted in such loss, liability, claim, damage, or expense as well as any other relevant equitable
considerations. The relative fault of the indemnifying party and of the 

7

 

indemnified
party shall be determined by reference to, among other things, whether the untrue or alleged untrue statement of a material fact or the omission to state a material fact relates to
information supplied by the indemnifying party or by the indemnified party and the parties' relative intent, knowledge, access to information, and opportunity to correct or prevent such statement or
omission. 

        (e)   Notwithstanding
the foregoing, to the extent that the provisions on indemnification and contribution contained in the underwriting agreement entered into in connection
with the underwritten public offering are in conflict with the foregoing provisions, the provisions in the underwriting agreement shall control. 

        (f)    The
obligations of the Company and Holders under this Section 1.10 shall survive the completion of any offering of Registrable Securities in a registration
statement under this Section 1, and otherwise. 

        1.11.    Reports Under Securities Exchange Act of 1934.    With a view to making available to the Holders the benefits
of Rule 144 promulgated under the Act and any other rule or regulation of the SEC that may
at any time permit a Holder to sell securities of the Company to the public without registration or pursuant to a registration on Form F-3, the Company agrees to: 

        (a)   make
and keep public information available, as those terms are understood and defined in SEC Rule 144, at all times after ninety (90) days after the
effective date of the first registration statement filed by the Company for the offering of its securities to the general public; 

        (b)   take
such action, including the voluntary registration of its Common Stock under Section 12 of the 1934 Act, as is necessary to enable the Holders to utilize
Form F-3 for the sale of their Registrable Securities, such action to be taken as soon as practicable after the end of the fiscal year in which the first registration statement
filed by the Company for the offering of its securities to the general public is declared effective; 

        (c)   file
with the SEC in a timely manner all reports and other documents required of the Company under the Act and the 1934 Act; and 

        (d)   furnish
to any Holder, so long as the Holder owns any Registrable Securities, forthwith upon request (i) a written statement by the Company that it has complied
with the reporting requirements of SEC Rule 144 (at any time after one (1) year after the effective date of the first registration statement filed by the Company), the Act and the 1934
Act (at any time after it has become subject to such reporting requirements), or that it qualifies as a registrant whose securities may be resold pursuant to Form F-3 (at any time
after it so qualifies), (ii) a copy of the most recent annual report of the Company and such other reports and documents so filed by the Company, and (iii) such other information as may
be reasonably requested in availing any Holder of any rule or regulation of the SEC which permits the selling of any such securities without registration or pursuant to such form. 

        1.12.    Form F-3 Registration.    In case the Company shall receive from any Holder or Holders a
written request or requests that the Company effect a registration on Form F-3 and any related qualification or compliance with respect to all or a part of the Registrable
Securities owned by such Holder or Holders, the Company will: 

        (a)   promptly
give written notice of the proposed registration, and any related qualification or compliance, to all other Holders; and 

        (b)   as
soon as practicable, effect such registration and all such qualifications and compliances as may be so requested and as would permit or facilitate the sale and
distribution of all or such portion of such Holder's or Holders' Registrable Securities as are specified in 

8

 

such
request, together with all or such portion of the Registrable Securities of any other Holder or Holders joining in such request as are specified in a written request given within 15 days
after receipt of such written notice from the Company; provided, however, that the Company shall not be obligated to effect any such registration, qualification or compliance, pursuant to this
section 1.12: (1) if Form F-3 is not available for such offering by the Holders; (2) if the Holders propose to sell Registrable Securities at an aggregate price
to the public (net of any underwriters' discounts or commissions) of less than one million dollars ($1,000,000); (3) if the Company shall furnish to the Holders a certificate signed by the
President of the Company stating that in the good faith judgment of the Board of Directors of the Company, it would be seriously detrimental to the Company and its shareholders for such
Form F-3 Registration to be effected at such time, in which event the Company shall have the right to defer the filing of the Form F-3 registration statement for
a period of not more than one hundred twenty (120) days after receipt of the request of the Holder or Holders under this Section 1.12; provided, however, that the Company shall not
utilize this right more than once in any twelve month period; (4) if the Company has, within the twelve (12) month period preceding the date of such request, already effected two
registrations on Form F-3 for the Holders pursuant to this Section 1.12; or (5) in any particular jurisdiction in which the Company would be required to qualify to do
business or to execute a general consent to service of process in effecting such registration, qualification or compliance. 

        (c)   Subject
to the foregoing, the Company shall file a registration statement covering the Registrable Securities and other securities so requested to be registered
as soon as practicable after receipt of the request or requests of the Holders. All expenses incurred in connection with a registration requested pursuant to Section 1.12, including (without
limitation) all registration, filing, qualification, printer's and accounting fees and the reasonable fees and disbursements of counsel for the selling Holder or Holders and counsel for the Company,
but excluding any underwriters' discounts or commissions associated with Registrable Securities, shall be borne by the Company. Registrations effected pursuant to this Section 1.12 shall not be
counted as demands for registration or registrations effected pursuant to Sections 1.2 or 1.3, respectively. 

        1.13.    Assignment of Registration Rights.    The rights to cause the Company to register Registrable Securities
pursuant to this Section 1 may be assigned (but only with all related obligations) by a Holder to a transferee or assignee of such securities who, after such assignment or transfer, holds at
least 100,000 shares of Registrable Securities (subject to appropriate adjustment for stock splits, stock dividends, combinations and other recapitalizations), provided: (a) the Company is,
within a reasonable time after such transfer, furnished with written notice of the name and address of such transferee or assignee and the securities with respect to which such registration rights are
being assigned; (b) such transferee or assignee agrees in writing to be bound by and subject to the terms and conditions of this Agreement, including without limitation the provisions of
Section 1.14 below; and (c) such assignment shall be effective only if immediately following such transfer the further disposition of such securities by the transferee or assignee is
restricted under the Act. For the purposes of determining the number of shares of Registrable Securities held by a transferee or assignee, the holdings of transferees and assignees of a partnership
who are partners or retired partners of such partnership (including spouses and ancestors, lineal descendants and siblings of such partners or spouses who acquire Registrable Securities by gift, will
or intestate succession) shall be aggregated together and with the partnership; provided that all assignees and transferees who would not qualify individually for assignment of registration rights
shall have a single attorney-in-fact for the purpose of exercising any rights, receiving notices or taking any action under this Section 1. 

9

 

        1.14.    "Market Stand-Off" Agreement.    Each Investor hereby agrees that, during the period of duration
specified by the Company and an underwriter of common stock or other securities of the Company, following the effective date of a registration statement of the Company filed under the Act, it shall
not, to the extent requested by the Company and such underwriter, directly or indirectly sell, offer to sell, contract to sell (including, without limitation, any short sale), grant any option to
purchase or otherwise transfer or dispose of (other than to donees who agree to be similarly bound) any securities of the Company held by it at any time during such period except common stock included
in such registration; provided, however, that: 

        (a)   all
officers and directors of the Company and all other persons with registration rights (whether or not pursuant to this Agreement) enter into similar agreements; and 

        (b)   such
market stand-off time period shall not exceed 360 days for an initial public offering of the Company's securities and for ninety (90) days
for a follow-on offering of the Company's securities. 

        In
order to enforce the foregoing covenant, the Company may impose stop-transfer instructions with respect to the Registrable Securities of each Investor (and the shares or
securities of every other person subject to the foregoing restriction) until the end of such period. 

        Notwithstanding
the foregoing, the obligations described in this Section 1.14 shall not apply to a registration relating solely to employee benefit plans on
Form F-l or Form S-8 or similar forms which may be promulgated in the future, or a registration relating solely to a Commission Rule 145 transaction on
Form S-4 or similar forms which may be promulgated in the future. 

        1.15.    Termination of Registration Rights.    

        (a)   No
Holder shall be entitled to exercise any right provided for in this Section 1 after three (3) years following the consummation of the sale of securities
pursuant to a registration statement filed by the Company under the Act in connection with the initial firm commitment underwritten offering of its securities to the general public. 

        (b)   In
addition, the right of any Holder to request registration or inclusion in any registration pursuant to Section 1.3 shall terminate on the closing of the first
Company-initiated registered public offering of Common Stock of the Company if all shares of Registrable Securities held or entitled to be held upon conversion by such Holder may immediately be sold
under Rule 144 during any 90-day period, or on such date after the closing of the first Company-initiated registered public offering of Common Stock of the Company as all shares of
Registrable Securities held or entitled to be held upon conversion by such Holder may immediately be sold under Rule 144 during any 90-day period; provided, however, that the
provisions of this subsection 1.15(b) shall not apply to any Holder who owns more than two percent (2%) of the Company's outstanding stock until such time as such Holder owns less than two percent
(2%) of the outstanding stock of the Company. 

        2.    Covenants of the Company.    

        2.1.    Delivery of Financial Statements.    The Company shall deliver to each Investor for so long as such Investor
holds outstanding Notes: 

        (a)   as
soon as practicable, but in any event within ninety (90) days after the end of each fiscal year of the Company, an income statement for such fiscal year, a
balance sheet of the Company and statement of shareholder's equity as of the end of such year, and a schedule as to the sources and applications of funds for such year, such year-end
financial reports to be in reasonable detail, prepared in accordance with Italian generally accepted accounting principles ("GAAP"), and audited and certified by independent public accountants of
nationally recognized standing selected by the Company; 

10

 

        (b)   as
soon as practicable, but in any event within forty-five (45) days after the end of each of the first three (3) quarters of each fiscal year
of the Company, an unaudited profit or loss statement, schedule as to the sources and application of funds for such fiscal quarter and an unaudited balance sheet as of the end of such fiscal quarter. 

        (c)   within
thirty (30) days of the end of each month, an unaudited income statement and schedule as to the sources and application of funds and balance sheet for and
as of the end of such month, in reasonable detail; 

        (d)   as
soon as practicable, but in any event thirty (30) days prior to the end of each fiscal year, a budget and business plan for the next fiscal year, prepared on a
monthly basis, including balance sheets and sources and applications of funds statements for such months and, as soon as prepared, any other budgets or revised budgets prepared by the Company; 

        (e)   with
respect to the financial statements called for in subsections (b) and (c) of this Section 2.1, an instrument executed by the Chief Financial
Officer or President of the Company and certifying that such financials were prepared in accordance with GAAP consistently applied with prior practice for earlier periods (with the exception of
footnotes that may be required by GAAP) and fairly present the financial condition of the Company and its results of operation for the period specified, subject to year-end audit
adjustment; 

        (f)    such
other information relating to the financial condition, business, prospects or corporate affairs of the Company as the Investor or any assignee of the Investor may
from time to time request, provided, however, that the Company shall not be obligated under this subsection (f) or any other subsection of Section 2.1 to provide information which it
deems in good faith to be a trade secret or similar confidential information. 

        2.2.    Inspection.    The Company shall permit each Investor, at such Investor's expense, to visit and inspect the
Company's properties, to examine its books of account and records and to discuss the Company's affairs, finances and accounts with its officers, all at such reasonable times as may be requested by the
Investor; provided, however, that the Company shall not be obligated pursuant to this Section 2.2 to provide access to any information which it reasonably considers to be a trade secret or
similar confidential information. 

        2.3.    Termination of Information and Inspection Covenants.    The covenants set forth in subsections 2.1(c),
(d) and (f) and Section 2.2 shall terminate as to Investors and be of no further force or effect when the sale of securities pursuant to a registration statement filed by the
Company under the Act in connection with the firm commitment underwritten offering of its securities to the general public is consummated or when the Company first becomes subject to the periodic
reporting requirements of Sections 12(g) or 15(d) of the 1934 Act, whichever event shall first occur. 

        2.4.    Right of First Refusal on Dispositions.    

        (a)   If
at any time any Investor desires to sell all or any part of such Investor's Securities to a third party (the "Proposed Transferee"), such Investor ("Selling
Investor") shall first submit to the Majority Shareholder and the Company (i) a bona fide written offer from the Proposed Transferee to purchase such Securities (which, upon acceptance by such
Investor, would constitute a legally binding obligation) and (ii) a written offer from the Selling Investor (the "Offer") to sell such Securities (the "Offered Securities") to the Majority
Shareholder and the Company on terms and conditions, including price, not less favorable than those on which the Selling Investor proposes to sell such Offered Securities to the Proposed Transferee.
The Offer shall disclose the identity of the Proposed Transferee, the Offered Securities proposed to be sold, the total number of Securities owned by the Selling Investor, the terms and conditions,
including price, of the proposed sale, and any other material facts relating to 

11

 

the
proposed sale and shall include a copy of the Offer to purchase from the Proposed Transferee. The Offer shall further state that the Majority Shareholder and the Company may acquire, in accordance
with the provisions of this Agreement, all, but not less than all, of the Offered Securities for the price and upon the other terms and conditions, including deferred payment (if applicable), set
forth therein. 

        (b)   If
the Majority Shareholder and/or the Company desires to purchase all, but not less than all, of the Offered Securities, the Majority Shareholder and/or the Company, as
applicable, shall communicate in writing its election to purchase to the Selling Investor within fifteen (15) days of the date the Offer was made to the Majority Shareholder and the Company.
Such communications shall, when taken in conjunction with the Offer, be deemed to constitute valid, legally binding and enforceable agreement(s) for the sale and purchase of such Offered Securities. 

        (c)   If
the Majority Shareholder and/or the Company elects to purchase the Offered Securities, the sales of such Offered Securities to the Majority Shareholder and/or the
Company, as applicable, pursuant to this Section 2.4 shall be made at the offices of the Company or counsel to the Company on the 30th day following the date the Offer was made pursuant to
subsection (a) above (or if such day is not a business day, then on the next succeeding business day). Such sale shall be effected by the Selling Investor's delivery to the Majority Shareholder
and/or the Company, as applicable, of certificates or other instruments evidencing the Offered Securities to be purchased by it, duly endorsed for transfer, against payment to the Selling Investor of
the purchase price therefor by the Majority Shareholder and/or the Company, as applicable. 

        (d)   If
neither the Majority Shareholder nor the Company elects to purchase all of the Offered Securities, the Offered Securities may be sold by the Selling Investor at any
time within 30 days after the date the Offer was accepted, rejected or lapsed pursuant to subsection (b) above. Any such sale shall be to the Proposed Transferee and at not less than the
price and upon other terms and conditions, if any, not more favorable to the Proposed Transferee than those specified in the Offer. Any
Offered Securities not sold within such 30-day period shall continue to be subject to the requirements of a prior offer pursuant to this Section 2.4. 

        (e)   The
Majority Shareholder's and the Company's right of first refusal provided in this Section 2.4 shall not apply (i) in conjunction with the sale of the
Company to an unaffiliated third party whether by merger, consolidation or sale of stock in a transaction in which the Majority Shareholder's shares are also sold or transferred; or (ii) in
conjunction with a public offering pursuant to an effective registration statement under the Act. 

        (f)    If
an Investor is subject to a transfer of his, her or its Securities by any bankruptcy or insolvency law or proceeding, any divorce proceeding or otherwise by operation
of law (other than by death), or if any transfer of Securities is made or attempted contrary to the provisions of this Agreement, the Majority Shareholder and the Company will have the right to
purchase any or all of such Securities from the Investor, his, her or its legal representative or transferees at any time before or after the transfer, at the price, if any, paid for or proposed to be
paid for such Securities or for fair market value as determined under subsection (g), whichever is less. 

        (g)   The
fair market value of the Securities subject to purchase pursuant to subsection (f) will be jointly determined by the seller and the Majority Shareholder and
the Company, as applicable, who elect to purchase, or if they are unable to agree, by such other appraiser as the seller and the Majority Shareholder and the Company, as applicable, may jointly
choose. The seller shall bear all of the fees and expenses arising out of the appraisal. 

12

 

        (h)   The
agreements set forth in this Section 2.4 shall terminate and be of no further force or effect when the sale of Securities pursuant to a registration statement
filed by the Company under the Act in connection with the firm commitment underwritten offering of its securities to the general public is consummated or when the Company first becomes subject to the
periodic reporting requirements of Sections 12(g) or 15(d) of the 1934 Act, whichever event shall first occur. 

        2.5.    Drag-Along Rights.    At any time prior to an initial public offering of securities of the
Company, in the event that (i) the Company receives a bona fide offer from any party unaffiliated with any party to this Agreement (such party a "Third Party Offeror" and such an offer the
"Third Party Offer") to purchase all or substantially all of the Company's issued and outstanding shares of capital stock in a transaction (including a merger), (ii) such transaction is
approved by the Board of Directors of the Company, and (iii) the holders (the "Proposing Shareholders") of shares representing a majority of the votes represented by all then outstanding shares
of capital stock of the Company approve or otherwise consent in writing to such transaction, then (x) all parties to this Agreement (the "Drag-Along Shareholders") will be required,
if so demanded by the Proposing Shareholders, to vote such Drag-Along Shareholder's shares of capital stock in favor
thereof, and otherwise consent to and raise no objection to such transaction, and waive any dissenters' rights, appraisal rights or similar rights that such Drag-Along Shareholder may have
in connection therewith, and take all necessary and desirable actions as directed by the Company's Board of Directors and the Proposing Shareholders in connection with the consummation of such
transactions, including, to the extent applicable, executing a purchase agreement and selling, exchanging or otherwise transferring all of the shares of the Company's capital stock (or notes, warrants
or other rights to subscribe for or purchase capital stock) held by such Drag-Along Shareholders to such Third Party Offeror at the same price and upon the same terms and conditions as the
Third Party Offer; or (y) the holders of Notes shall be entitled to sell their Notes pursuant to, and upon the same terms as, the Third Party Offer on an
as-converted-to-Common Stock basis. If a Drag-Along Shareholder fails or refuses to vote his, her or its shares of capital stock of the Company as
required by the terms of this Section 2.5, the President of the Company shall be deemed to be granted by such Drag-Along Shareholder an irrevocable proxy, coupled with an interest,
to vote such Drag-Along Shareholder's shares of capital stock in accordance with this Section 2.5. 

        3.    Miscellaneous.    

        3.1.    Successors and Assigns.    Except as otherwise provided herein, the terms and conditions of this Agreement
shall inure to the benefit of and be binding upon the respective successors and assigns of the parties (including transferees of any shares of Registrable Securities). Subject to Sections 2.4 and 2.5,
if applicable, nothing in this Agreement, express or implied, is intended to confer upon any party other than the parties hereto or their respective successors and assigns any rights, remedies,
obligations, or liabilities under or by reason of this Agreement, except as expressly provided in this Agreement. 

        3.2.    Governing Law.    This Agreement shall be construed in accordance with and governed by the laws of the State
of New York without regard to principles of conflicts of laws. In the event that a judicial proceeding is necessary, the exclusive forums for resolving disputes arising out of or relating to this
Agreement are either the Supreme Court of the State of New York in and for the County of New York or the federal courts for such State and County, and all related appellate courts, the parties hereby
irrevocably consent to the jurisdiction of such courts and agree to said venue. 

13

 

        3.3.    Counterparts.    This Agreement may be executed in two or more counterparts, each of which shall be deemed an
original, but all of which together shall constitute one and the same instrument. 

        3.4.    Titles and Subtitles.    The titles and subtitles used in this Agreement are used for convenience only and are
not to be considered in construing or interpreting this Agreement. 

        3.5.    Notices.    Except as otherwise expressly specified herein, all notices, requests and other communications
required or permitted hereunder shall be in writing and shall be sent by an internationally recognized overnight courier service; by certified or registered mail, return receipt requested (or in the
case of a notice sent to an address in Italy, by international express mail, return receipt requested); by facsimile transmission or by hand delivery. 

        The
address for such notices and communications shall be as follows: 

If
to the Company:

Gentium S.p.A.

Piazza XX Settembre, 2

22079 Villa Guardia Como

Italy

Attention: Dott. Sauro Carsana

Fax: +39 031 385333 

If
to an Investor: 

To
the address set forth in the signature page to his or its respective Subscription Agreement or such other address as provided by the Investor to Issuer in writing. 

        Any
party may designate a different notice address, contact person, telephone number or facsimile number with respect to such party by providing a notice describing such changes to the
other party hereto in accordance with the provisions of this Section 3.5. Any notice sent by internationally recognized overnight mail courier service shall be deemed to be delivered to the
address shown on the mailing receipt on the expected date of delivery upon proper evidence of mailing for purposes of this Section 3.5. Any notice sent by certified or registered mail, return
receipt requested (or, in the case of a notice sent to an address in Italy, by international express mail, return receipt requested), shall be deemed to be delivered five business days after mailing.
Any notice sent by facsimile transmission shall be deemed delivered as of the open of business on the business day following the date on which sent provided the sender receives written confirmation of
transmission and provided that within 24 hours such notice is also sent by regular mail or by an internationally-recognized overnight mail courier service to the appropriate address specified
above. Any notice sent by hand delivery shall be deemed delivered as of the date of delivery. 

        3.6.    Expenses.    If any action at law or in equity is necessary to enforce or interpret the terms of this
Agreement, the prevailing party shall be entitled to reasonable attorneys' fees, costs and necessary disbursements in addition to any other relief to which such party may be entitled. 

        3.7.    Amendments and Waivers.    Any term of this Agreement may be amended and the observance of any term of this
Agreement may be waived (either generally or in a particular instance and either retroactively or prospectively), only with the written consent of the Company and the holders of a
majority-in-interest of the Registrable Securities then outstanding which holders shall include Generation Capital Associates. Any amendment or waiver effected in accordance
with this paragraph shall be binding upon each holder of any Registrable Securities then outstanding, each future holder of all such Registrable Securities, and the Company. 

        3.8.    Severability.    The holding of any provision of this Agreement to be invalid or unenforceable by a court of
competent jurisdiction shall not affect any other provision of this 

14

 

Agreement,
which shall remain in full force and effect. If any provision of this Agreement shall be declared by a court of competent jurisdiction to be invalid, illegal or incapable of being enforced
in whole or in part, such provision shall be interpreted so as to remain enforceable to the maximum extent permissible consistent with applicable law and the remaining conditions and provisions or
portions thereof shall nevertheless remain in full force and effect and enforceable to the extent that they are valid, legal and enforceable, and no provisions shall be deemed dependent upon any other
covenant or provision unless so expressed herein. 

        3.9.    Entire Agreement.    This Agreement sets forth the entire agreement and understanding between the parties as
to the subject matter hereof and merges and supersedes all prior discussions, agreements and understandings of any and every nature among them. 

        [Remainder
of page intentionally left blank] 

15

 

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

	 	 	 	 	GENTIUM S.P.A.
	

 	
 	

 	
 	

By:	
 	

	 	 	 	 	 	 	, President
	

 	
 	

Address:	
 	

	

 	
 	

 	
 	

	

 	
 	

 	
 	
INVESTOR:
	

 	
 	

 	
 	

By:	
 	

	

 	
 	

Address:	
 	

	

 	
 	

 	
 	

16

QuickLinks

Exhibit 4.2.4

INVESTORS' RIGHTS AGREEMENT

Table of Contents

INVESTORS' RIGHTS AGREEMENT

RECITALS

Source: [{"source": "alea-institute/alea-institute/kl3m-data-edgar-agreements/train-00077-of-00352.parquet"}, [{"source": "alea-institute/alea-institute/kl3m-data-edgar-agreements/train-00077-of-00352.parquet"}]]