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24 Oct 2017

Orphazyme announces intention to launch an Initial Public Offering

Orphazyme, a Danish biotech company with a late stage orphan drug pipeline, announces its
intention to launch an Initial Public Offering (“IPO” or “Offering”) of its shares and to apply for
admission to trading and official listing on Nasdaq Copenhagen.

The intended IPO is expected to consist of an issue of new shares to raise gross proceeds of
approximately DKK 600 million to support the Company’s strategy by primarily funding on-going
and planned clinical trials within four orphan diseases. The total offer size will be announced in
connection with the publication of a prospectus by the Company.

Orphazyme is currently owned by Novo Holdings A/S, Coöperative Aescap Venture I U.A.,
Sunstone Life Science Ventures Fund II K/S, Orpha Pooling B.V. (a joint venture between LSP V
Coöperatieve U.A. and ALS Invest 2 B.V.), certain funds managed by Idinvest and Kurma Biofund
II and other minority shareholders including members of senior management and employees.

Prior to publication of the prospectus, the Company’s existing share classes will be merged as
part of a reorganisation of the current capital structure. The exact ownership stakes of the current
shareholders after the reorganisation of the capital structure will depend on the final offer price in
the IPO. None of the current shareholders have expressed an intention to sell shares in
connection with the IPO and will continue as shareholders in Orphazyme in addition to
undertaking lock-up obligations in connection with the IPO.

Anders Hinsby, CEO of Orphazyme, said:
“Since the foundation in 2009, we have been able to establish a Danish biotech company with a
product candidate for the treatment of four severe orphan diseases. We have an extensive news
flow coming up and expect to have completed three phase II/III trials by the end of 2020 with the
first potential marketing authorisation in 2020. We have, for a longer period of time, had many
meetings with domestic and international investors and we are very pleased with the feedback
received.”

Georges Gemayel, Chairman of Orphazyme, said:
“Today’s announcement marks an important step in Orphazyme’s strategic journey. We look
forward to inviting new shareholders to take part in the future ownership and to join us in our
journey to assist patients with a significant unmet medical need.”

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