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Inozyme Pharma Raises $67 Million in Series A2 Financing to Develop Therapies for Rare Mineralization Disorders

– Financing to Advance Lead Development Program, INZ-701, through
Clinical Proof of Concept in Two Indications –

Company Expands Pipeline with New Program for a Rare
Mineralization Disorder –

BOSTON–(BUSINESS WIRE)–Inozyme
Pharma, Inc.
, a biotechnology company developing novel medicines to
treat rare, debilitating, and life-threatening mineralization disorders,
today announced that the Company raised $67 million in a Series A2
financing led by Pivotal bioVenture Partners and Sofinnova Investments.
Cross-over investors, including RA Capital Management, Cowen Healthcare
Investments, and Rock Springs Capital, as well as the Company’s previous
investors, Longitude Capital, NEA, Novo Holdings, and Sanofi Ventures,
also joined this round.

Launched in 2017, Inozyme has to date raised $116 million in venture
capital. The Company will use the proceeds from the Series A2 financing
to advance its lead enzyme replacement therapy, INZ-701, into clinical
development for severe disorders of calcification associated with
deficiencies in the enzyme ENPP1, including Generalized Arterial
Calcification of Infancy (GACI) and Autosomal Recessive Hypophosphatemic
Rickets Type 2 (ARHR2).

Inozyme also announced that it is expanding the INZ-701 development
program by adding ABCC6 Deficiency as a second indication. Similar to
mutated ENPP1, mutations in the ABCC6 gene are linked to
calcification disorders resulting from low pyrophosphate (PPi) and can
lead to a condition known as pseudoxanthoma elasticum (PXE).
Furthermore, the Company has broadened its pipeline by adding a second
research program targeting an undisclosed rare bone disease.

“Since our inception, we have advanced our lead candidate INZ-701
through preclinical development. Our goal is to develop first-in-class
therapies for patients with rare mineralization disorders,” said Axel
Bolte, chief executive officer and co-founder of Inozyme Pharma. “With
the addition of a second indication for INZ-701 and a second rare bone
disease program, we are expanding our footprint in these underserved
disorders. This latest round of financing enables us to broaden our
development pipeline and to bring INZ-701 through clinical proof of
concept. We are excited to evolve our investor base by adding five
highly regarded new investors who are committed to helping us achieve
our goals, and we appreciate the continued support of our existing
investors as Inozyme continues to grow. We will continue to evaluate
additional assets that are highly innovative and have the potential to
make a meaningful impact for patients with mineralization disorders.”

The Company has already demonstrated preclinical proof of concept for
INZ-701 in GACI and ARHR2. Both of these disorders result from mutations
in the ENPP1 gene, leading to ENPP1 Deficiency.

The Company is currently completing the IND-enabling studies for INZ-701
and expects to initiate clinical trials in 2020.

“Pivotal bioVenture Partners is delighted to join the Inozyme Pharma
team in its quest to develop therapeutic solutions for patients with
rare, debilitating, and life-threatening mineralization disorders,”
commented Rob Hopfner, RPh, Ph.D., MBA, managing partner of Pivotal
bioVenture Partners. “Inozyme has assembled a world class team of drug
hunters and drug developers to advance first-in-class therapies for rare
orphan diseases. We have been highly impressed by their solid science,
well-defined regulatory strategy, and deep understanding of the biology
of rare mineralization disorders.”

Inozyme also announced the addition of Dr. Hopfner to its Board of
Directors, along with Sarah Bhagat, Ph.D., principal at Sofinnova
Investments.

About INZ-701

INZ-701 is an enzyme replacement therapy in development for the
treatment of mineralization disorders of the circulatory system, bones,
and kidneys. In preclinical studies, the experimental therapy has shown
potential to generate plasma pyrophosphate (PPi) and to restore it to
appropriate physiological levels, thereby preventing calcification in
the vasculature and kidneys, while at the same time normalizing bone
mineralization. Inozyme is developing INZ-701 for certain rare,
life-threatening, and devastating genetic disorders such as ENPP1
Deficiency (GACI and ARHR2) and ABCC6 Deficiency (PXE) in which PPi
levels are below the normal physiological levels. For more information
about INZ-701, please visit: http://www.inozyme.com/our-science/.

About Inozyme Pharma, Inc.

Inozyme Pharma is a biotechnology company committed to developing novel
medicines for the treatment of rare mineralization disorders. Inozyme
Pharma was founded in 2017 by Joseph Schlessinger Ph.D., Demetrios
Braddock, M.D., Ph.D., and Axel Bolte, MSc., MBA. The Company licensed
technology from Yale University, developed in the laboratory of Dr.
Braddock.

Contacts

Inozyme Pharma, Inc.
Axel Bolte, President and CEO
(857)
330-4340
axel.bolte@inozyme.com

SmithSolve
Alex
Van Rees
(973) 442-1555 ext. 111
alex.vanrees@smithsolve.com

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