Ipsen to Acquire Clementia Pharmaceuticals to Significantly Boost Rare Disease Portfolio

Regulatory News:

Ipsen (Euronext: IPN; ADR: IPSEY) and Clementia Pharmaceuticals (NASDAQ:
CMTA) today announced that they have entered into an agreement for Ipsen
to acquire Clementia Pharmaceuticals, including its key late-stage
clinical asset palovarotene, an investigational retinoic acid receptor
gamma (RARγ) selective agonist, for the treatment of fibrodysplasia
ossificans progressiva (FOP), multiple osteochondromas (MO) and other
diseases. The acquisition will proceed by way of a court-approved plan
of arrangement pursuant to the Canada Business Corporations Act.

Continuing the transformation of Ipsen:

• Accelerating a global Rare Disease organization with the mission to
  bring treatment options for ultra-orphan diseases to patients worldwide
• Executing on a key strategic objective to increase the value of the
  pipeline with innovative first-in-class or best-in-class assets
• Acquiring a near-term launch opportunity of a largely de-risked asset
  with limited competition which enhances sustainable growth of the
  company with significant upside potential from additional indications

Palovarotene inhibits excess bone morphogenetic protein (BMP) signaling
which is linked to the progression of FOP and MO, two
well-characterized, ultra-rare/rare and severely-disabling bone
disorders for which there are currently no treatment options available.

A New Drug Application (NDA) for palovarotene for episodic flare-up
treatment of FOP is expected to be submitted to the U.S. Food and Drug
Administration (FDA) in the second half of 2019, and subject to FDA
approval, a first commercial launch is expected in mid-2020. A Phase 3
registrational trial evaluating a chronic dosing regimen for FOP, a
Phase 2 trial for MO, and a Phase 1 trial for dry eye disease are also
ongoing. Palovarotene has received Orphan Drug designation for FOP and
MO from the FDA and the European Medicines Agency (EMA), and Fast Track,
Breakthrough Therapy and Rare Pediatric Disease designations for FOP
from the FDA.

David Meek, Chief Executive Officer of Ipsen, commented, “The
acquisition of Clementia Pharmaceuticals accelerates the ongoing
transformation of Ipsen as we are successfully executing on our external
innovation strategy to identify and acquire innovative medicines to
serve patients with unmet medical needs. Through this transaction, we
will gain scientific expertise, exceptional talent, and a cornerstone
ultra-rare disease drug candidate with rare pediatric disease and
breakthrough therapy designations, potential U.S. approval in 2020 and
additional indications to follow. We look forward to working closely
with Clementia to successfully integrate two companies that share a
similar patient-centric culture and the ambition to deliver new
treatments to patients with unmet medical needs.”

Dr. Clarissa Desjardins, Chief Executive Officer of Clementia,
commented, “I am proud of the entire Clementia team, whose tireless
efforts have rapidly advanced palovarotene towards a planned NDA
submission, and we are all grateful for the dedication of the patient
community and our clinical trial investigators who have supported us
along the way. Ipsen’s global commercial presence and capabilities will
expedite our shared vision of bringing palovarotene to patients around
the world as quickly as possible. We anticipate a smooth transition of
our operations into the Ipsen organization that will continue
Clementia’s vision of delivering palovarotene to patients worldwide.”

Under the terms of the agreement, Ipsen will pay US$25.00 per share in
cash upfront on completion of the transaction, for an initial aggregate
consideration of US$1.04 billion, plus deferred payments on the
achievement of a future regulatory milestone in the form of a contingent
value right (CVR) of US$6.00 per share upon FDA acceptance of the NDA
filing for palovarotene for the treatment of MO, representing an
additional potential payment of US$263 million. The initial cash
consideration represents a premium of 77% to Clementia’s 30-day
volume-weighted average stock price.

The transaction will be fully financed by Ipsen’s existing cash and
lines of credit and significantly increase the level of net debt. It is
expected to have a limited dilutive impact on Ipsen’s core operating
margin for 2019 and 2020 given the costs of the ongoing clinical trials
and preparation for the commercial launch of palovarotene. Consequently,
Ipsen is updating its 2019 financial objectives and now expects:

• Sales growth of greater than 13.0% at current exchange rates
  (unchanged)
• Core operating margin of around 30.0% of net sales (previous guidance
  of around 31.0% of net sales), excluding other potential investments
  in pipeline expansion initiatives

The transaction will also be dilutive at the Consolidated Net Income
level.

The Boards of Directors of both companies have approved the transaction.
Completion of the transaction is anticipated to occur in the second
quarter of 2019, subject to satisfaction of all closing conditions. The
acquisition will proceed by way of a court-approved plan of arrangement
pursuant to the Canada Business Corporations Act and will require, at
the special meeting of Clementia shareholders expected to be held on or
about April 9, 2019, the approval of at least 66 2/3% of the votes cast
by Clementia's shareholders present in person or represented by proxy as
well as the approval of a majority of the votes cast by Clementia's
disinterested shareholders present in person or represented by proxy. A
proxy circular relating to the special meeting of shareholders of
Clementia and containing further details regarding the Arrangement and
the agreement will be mailed to Clementia’s shareholders and made
available on SEDAR and EDGAR.

The Board of Directors of Clementia, acting on the unanimous
recommendation of the transaction committee comprised of independent
directors and after having received an opinion from its financial
advisor to the effect that the consideration to be received by Clementia
shareholders pursuant to the plan of arrangement is fair from a
financial point of view, has unanimously approved the arrangement.
OrbiMed Private Investments IV, LP, Clementia’s largest shareholder with
approximately 27.5% of Clementia’s total shares outstanding (on a
non-diluted basis) as of the date hereof, has entered into a support and
voting agreement with Ipsen pursuant to which it has agreed to vote its
Clementia shares in favor of the transaction. In addition, directors and
officers of Clementia holding an aggregate of approximately 3.2% of the
Clementia shares (on a non-diluted basis) as of the date hereof have
entered into support and voting agreements with Ipsen.

In addition to shareholders’ and court approval, the arrangement is also
subject to other customary conditions. The arrangement agreement is
subject to customary “fiduciary out” provisions, and a right in favor of
Ipsen to match any superior proposal. A termination fee is payable to
Ipsen in certain specified circumstances, including if it fails to
exercise its right to match in the context of a superior proposal
supported by Clementia.

Centerview Partners is acting as exclusive financial advisor to Ipsen
and Goodwin Procter LLP and Davies Ward Phillips & Vineberg LLP are
acting as U.S. and Canadian legal counsel to Ipsen, respectively.

Morgan Stanley & Co. LLC is acting as exclusive financial advisor to
Clementia and Skadden, Arps, Slate, Meagher & Flom LLP and Stikeman
Elliott LLP are acting as U.S. and Canadian legal counsel to Clementia,
respectively.

Conference Call

Ipsen will host a conference call and web conference (available at www.ipsen.com)
today to discuss this announcement. Participants should dial in
approximately 5 to 10 minutes prior to the start. No reservation is
required to participate in the conference call.

A replay will be available on Ipsen’s website: www.ipsen.com

About Fibrodysplasia Ossificans Progressiva (FOP)

FOP is an ultra-rare, severely disabling disorder characterized by
heterotopic ossification (HO), or bone that forms outside the normal
skeleton, in muscles, tendons or soft tissue. In FOP, HO progressively
restricts movement by locking joints, leading to a cumulative loss of
function, progressive disability, and increased risk of early death. FOP
is caused by a mutation in the ACVR1 gene, resulting in excess signaling
in the bone morphogenetic pathway, a key pathway controlling bone growth
and development, by way of both ligand-dependent and independent
mechanisms. The prevalence of FOP is approximately 1.3 individuals per
million lives, or approximately 9,000 patients globally. There are
currently no approved treatments for FOP.

About Multiple Osteochondromas (MO)

MO, also called multiple hereditary exostoses (MHE) is a rare, severely
disabling, progressive, chronic disease in which multiple benign bone
tumors, also known as osteochondromas (OCs) or osteocartilaginous
exostoses, develop on bones. MO is typically diagnosed in early
childhood when OCs become visible with a median age at diagnosis of four
years. Because of their development around joints, children develop limb
deformity and restricted movement as they grow. Today, the only
available treatments for MO are surgery and palliative care, and many
patients will undergo surgery, some as many as 30 surgeries, before
adulthood. MO is estimated to affect 20 individuals per million lives,
or approximately 150,000 globally. MO is among the most common inherited
bone disorders with multiple family members in multiple generations
affected.

About Palovarotene

Palovarotene is an RARγ agonist being developed as a treatment for
patients with ultra-rare/rare and debilitating bone diseases, including
fibrodysplasia ossificans progressiva (FOP) and multiple osteochondromas
(MO), as well as other diseases. Palovarotene was in-licensed from Roche
Pharmaceuticals, where it was previously evaluated in more than 800
subjects, including 450 patients treated for up to two years.
Palovarotene has received Orphan Drug status for FOP and MO from the
U.S. Food and Drug Administration (FDA) and the European Medicines
Agency (EMA). In addition, palovarotene has been granted Fast Track,
Breakthrough Therapy and Rare Pediatric Disease designations for FOP
from the FDA.

About Ipsen

Ipsen is a global specialty-driven biopharmaceutical group focused on
innovation and specialty care. The group develops and commercializes
innovative medicines in three key therapeutic areas - Oncology,
Neuroscience and Rare Diseases. Its commitment to Oncology is
exemplified through its growing portfolio of key therapies for prostate
cancer, neuroendocrine tumors, renal cell carcinoma and pancreatic
cancer. Ipsen also has a well-established Consumer Healthcare business.
With total sales over €2.2 billion in 2018, Ipsen sells more than 20
drugs in over 115 countries, with a direct commercial presence in more
than 30 countries. Ipsen's R&D is focused on its innovative and
differentiated technological platforms located in the heart of the
leading biotechnological and life sciences hubs (Paris-Saclay, France;
Oxford, UK; Cambridge, US). The Group has about 5,700 employees
worldwide. Ipsen is listed in Paris (Euronext: IPN) and in the United
States through a Sponsored Level I American Depositary Receipt program
(ADR: IPSEY). For more information on Ipsen, visit www.ipsen.com.

About Clementia Pharmaceuticals Inc.

Clementia is a clinical-stage company innovating treatments for people
with ultra-rare bone disorders and other diseases with high medical
need. The company is preparing for a 2019 NDA submission to the FDA to
seek approval of its lead product candidate, palovarotene, a novel RARγ
agonist, for the prevention of heterotopic ossification (HO) associated
with flare up symptoms in adults and children with fibrodysplasia
ossificans progressiva (FOP). The ongoing Phase 3 MOVE Trial is
evaluating an additional dosing regimen of palovarotene for the
treatment of FOP. Palovarotene is also in a Phase 2 trial, the MO-Ped
Trial, for the treatment of multiple osteochondromas (MO, also known as
multiple hereditary exostoses, or MHE). In addition, Clementia has
commenced a Phase 1 trial for an eye drop formulation of palovarotene
for the potential treatment of dry eye disease and is also investigating
other conditions that may benefit from RARγ therapy. For more
information, please visit www.clementiapharma.com
and connect with us on Twitter @ClementiaPharma.

Forward Looking Statement

This press release may include “forward-looking statements” within the
meaning of the applicable securities laws, including with respect to the
timing and completion of the arrangement, the proposed timing of filings
and submissions with the FDA for palovarotene and the impact of the
proposed transaction on Ipsen and Clementia, the operations of Ipsen and
Clementia post-transaction and the amounts potentially payable under the
CVRs. Each forward-looking statement contained in this press release is
subject to known and unknown risks and uncertainties and other unknown
factors that could cause actual results to differ materially from
historical results and those expressed or implied by such statement. In
addition to statements which explicitly describe such risks and
uncertainties, readers are urged to consider statements labeled with the
terms “believes,” “belief,” “expects,” “intends,” “anticipates,” “will,”
or “plans” to be uncertain and forward-looking. Applicable risks and
uncertainties include, among others, the risk that a condition to
closing of the arrangement may not be satisfied, the risk that any
required shareholder, court or applicable regulatory approvals for the
arrangement may not be obtained or be obtained subject to conditions
that are not anticipated, the outcome of the FDA approval of
palovarotene product candidate for the treatment of multiple
osteochondromas (MO), Clementia’s ability to successfully complete in a
timely manner the studies required to be completed in order to submit
the NDA, Clementia’s ability to generate revenue and become profitable,
the risks related to its heavy reliance on palovarotene, its only
current product candidate, the risks associated with the development of
palovarotene and any future product candidate, including the
demonstration of efficacy and safety, Ipsen's and Clementia’s dependence
on licensed intellectual property, including the ability to source and
maintain licenses from third-party owners; as well as the risks
identified in Ipsen's registration documents filed with the French Autorité
des Marchés Financiers and Clementia’s public filings with the SEC
and the Québec Autorité des Marchés Financiers. Ipsen and
Clementia caution investors not to rely on the forward-looking
statements contained in this press release when making an investment
decision in their securities. Investors are encouraged to read Ipsen's
filings available on its website (www.ipsen.com)
as well as Clementia’s filings with the SEC or on SEDAR, available at www.sec.gov
or www.sedar.com,
for a discussion of these and other risks and uncertainties. The
forward-looking statements in this press release speak only as of the
date of this press release, and Ipsen and Clementia undertake no
obligation to update or revise any of these statements, whether as a
result of new information, future events or otherwise, except as
required by law.

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