Strategic Partners A/S - Major Shareholding Notification 2017

Orphazyme A/S: Major shareholder announcement

Orphazyme A/S: Major shareholder announcement


Company announcement	Orphazyme A/S
No. 10/2017	Ole Maaløes Vej 3
	DK-2200 Copenhagen N

	www.orphazyme.com
	Company Registration No. 32266355

	16 November 2017

Major shareholder announcement

With
reference to company announcement no. 5/2017 dated 16 November 2017 regarding
the result of the Initial Public Offering (the "Offering") of Orphazyme A/S
("Orphazyme"), Orphazyme hereby announces that it has received the following
notifications pursuant to section 29 of the Danish Securities Trading Act
regarding the existing major shareholders' direct and indirect respective holdings
of shares in Orphazyme.

Prior
to the completion of the Offering, the major shareholders of Orphazyme were the
Novo Nordisk Foundation through Novo Holdings A/S (33.1%); Aescap Venture
Management B.V. through Coöperative Aescap Venture I U.A. (15.2%); Sunstone
Life Science Ventures A/S through Sunstone LSV General Partner II ApS and Sunstone
Life Science Ventures Fund II K/S (15.6%); LSP Management Group B.V. through LSP
V Management B.V., LSP V Coöperatieve U.A. and Orpha Pooling B.V. (21.0%);
Idinvest Partners through FCPI Idinvest Patrimoine n°3, FCPI Idinvest
Patrimoine n°4, FCPI Objectif Innovation Patrimoine n°6 and FCPI Objectif
Innovation Patrimoine n°7 (7.2%); and Kurma Partners through Kurma Biofund II (6.6%).

Novo
Holdings A/S has reduced its shareholding in Orphazyme as a consequence of (i) the
dilution resulting from the issue of 7,500,000 new shares in Orphazyme of a
nominal value of DKK 1 each in connection with the Offering; and (ii) the
lending of 1,125,000 shares in Orphazyme of a nominal value of DKK 1 each by
Novo Holdings A/S to Joint Global Coordinators for the purposes of delivery of
shares to investors as a result of the overallotment option in connection with
the Offering (the "Overallotment Option"). Regardless of whether the
Overallotment Option is exercised, Novo Holdings A/S will have an equivalent
number of shares redelivered, either by way of existing shares or new shares
issued by Orphazyme.

Aescap
Venture Management B.V., Sunstone LSV General Partner II ApS, LSP Management
Group B.V., Idinvest Partners and Kurma Partners have reduced their respective
indirect shareholdings in Orphazyme as a consequence of the dilution resulting
from the issue of 7,500,000 new shares in Orphazyme of a nominal value of DKK 1
each in connection with the Offering.

Moreover,
Kurma Biofund II and LSP V Coöperatieve U.A. have increased their respective direct
shareholdings in Orphazyme by having subscribed for an additional number of
shares in Orphazyme in connection with the Offering.

Subsequent
to the Offering, Orphazyme has received the following major shareholder
announcements:

Novo
Holdings A/S

Following completion and settlement of the above
transactions in connection with the Offering, Novo Holdings A/S' holding of
shares in Orphazyme will be a total of 2,705,832 shares (i.e. excluding lending
shares pursuant to the Overallotment Option) of a nominal value of DKK 1 each,
corresponding to 13.6% of the total share capital and voting rights.

Novo
Holdings A/S is a limited liability company organized under the laws of Denmark
under CVR no. 24257630 with its registered office at Tuborg Havnevej 19, 2900
Hellerup, Denmark. Novo Holdings A/S is wholly-owned by the Novo Nordisk
Foundation.

The
Novo Nordisk Foundation is a foundation organised under the laws of Denmark
under CVR no. 10582989 with its registered office at Tuborg Havnevej 19,
DK-2900 Hellerup, Denmark.

Aescap
Venture

Following
completion and settlement of the Offering, Aescap Venture Management B.V.'s indirect
holding of shares in Orphazyme through Coöperative Aescap Venture I U.A. will
be a total of 1,765,605 shares of a nominal value of DKK 1 each, corresponding
to 8.9% of the total share capital and voting rights.

Coöperative
Aescap Venture I U.A. is a limited liability cooperative association organised
under the laws of the Netherlands, registered under registration number
34257886 with its registered address at Science Park 406, 1098 XH Amsterdam,
the Netherlands. Aescap Venture I U.A. is managed by Aescap Venture Management
B.V.

Aescap
Venture Management B.V. is a limited liability company organised under the laws
of the Netherlands, registered under registration number 32108991 with its
registered address at Science Park 406, 1098 XH Amsterdam, the Netherlands.

Sunstone
Life Science Ventures

Following
completion and settlement of the Offering, Sunstone Life Science Ventures A/S' indirect
holding of shares in Orphazyme through Sunstone LSV General Partner II ApS and
Sunstone Life Science Ventures Fund II K/S will be a total of 1,804,405 shares
of a nominal value of DKK 1 each, corresponding to 9.1% of the total share
capital and voting rights.

Sunstone
Life Science Ventures Fund II K/S is organised under the laws of Denmark under
CVR no. 30582268 with its registered address at Lautrupsgade 7, 5., 2100
Copenhagen Ø, Denmark. Sunstone Life Science Venture Fund II K/S is managed by
Sunstone LSV General Partner II ApS.

Sunstone
LSV General Partner II ApS is organised under the laws of Denmark under CVR no.
30575245 with its registered address at Lautrupsgade 7, 5., 2100 Copenhagen Ø,
Denmark. Sun-stone LSV General Partner II ApS is a subsidiary of Sunstone Life
Science Ventures A/S.

Sunstone
Life Science Ventures A/S is organised under the laws of Denmark under CVR no.
33859198 with its registered address at Lautrupsgade 7, 5., 2100 Copenhagen Ø,
Denmark.

LSP

Following
completion and settlement of the Offering, (i) LSP V Coöperatieve U.A.'s direct
holding of 279,157 shares of a nominal value of DKK 1 each in Orphazyme will
correspond to 1.4% of the total share capital and voting rights; and (ii) Orpha
Pooling B.V.'s direct holding of 2,431,672 shares of a nominal value of DKK 1
each in Orphazyme will correspond to 12.2% of the total share capital and
voting rights.

Orpha
Pooling B.V. is a private limited liability company and has its official seat
in Amsterdam, the Netherlands, with registered office address at Johannes Vermeer,
Plein 9, 1071 DV Amsterdam, the Netherlands, and is registered with the Dutch
trade register under number 67827055. The shareholders of Orpha Pooling B.V.
are (i) LSP V Coöperatieve U.A. (88.24% of the total share capital and voting
rights), and (ii) ALS Invest 2 B.V. (11.76% of the total share capital and
voting rights), a private limited liability company, having its official seat
in Amsterdam, the Netherlands, and its registered office address at Eerste
Weteringdwarsstraat 54 E, 1017TP Amsterdam, the Netherlands, registered with
the Dutch trade register under number 67804187.

LSP V
Coöperatieve U.A. is a cooperative with excluded liability and has its official
seat in Amsterdam, the Netherlands, with registered office address at Johannes
Vermeer, Plein 9, 1071 DV Amsterdam, the Netherlands, and is registered with
the Dutch trade register under number 61888575. LSP V Coöperatieve U.A. is a
closed end investment fund managed by LSP V Management B.V.

LSP V
Management B.V. is a private limited liability company and has its official
seat in Amsterdam, the Netherlands, with registered office address at Johannes
Vermeer, Plein 9, 1071 DV Amsterdam, the Netherlands, and is registered with
the Dutch trade register under number 60800542. LSP V Management B.V. is a
subsidiary of LSP Management Group B.V.

LSP
Management Group B.V. is a limited liability company has its official seat in
Amsterdam, the Netherlands, with registered office address at Johannes Vermeer,
Plein 9, 1071 DV Amsterdam, the Netherlands, and is registered with the Dutch
trade register under number 34288020.

Idinvest
Partners

Following
completion and settlement of the Offering, Idinvest Partners' indirect holding
of shares in Orphazyme through the FCPI Idinvest Patrimoine n°3, FCPI Idinvest
Patrimoine n°4, FCPI Objectif Innovation Patrimoine n°6 and FCPI Objectif
Innovation Patrimoine n°7 will be below 5% of the total share capital and
voting rights. Consequently, Idinvest Partners will no longer be a major
shareholder of Orphazyme pursuant to section 29 of the Danish Securities
Trading Act.

Idinvest
Partners manages its investment in Orphazyme through FCPI Idinvest Patrimoine
n°3, FCPI Idinvest Patrimoine n°4, FCPI Objectif Innovation Patrimoine n°6 and
FCPI Objectif Innovation Patrimoine n°7, which are French Fonds Communs de
Placement dans l'Innovation, governed by the laws of France, having their
registered office at 117 Avenue des Champs-Elysées, 75 008 Paris, France,
registered with the Registry of Trade and Companies of Paris under number 414
735 175.

Idinvest
Partners is a French société anonyme governed by the laws of France,
having its registered office at 117 Avenue des Champs-Elysées, 75 008 Paris,
France, registered with the Registry of Trade and Companies of Paris under
number 414 735 175.

Kurma
Partners

Following
completion and settlement of the Offering, Kurma Partners' indirect holding of
shares in Orphazyme through Kurma Biofund II will be below 5% of the total
share capital and voting rights. Consequently, Kurma Partners and Kurma Biofund
II will no longer be a major shareholder of Orphazyme pursuant to section 29 of
the Danish Securities Trading Act.

Kurma
Biofund II, a professional private equity fund represented by its management
company Kurma Partners, a French société anonyme (corporation), with its
registered office at 24 rue Royale - 75008 Paris, registered with the Paris
Trade and Companies Registry under number 510 043 136.

For additional information, please contact

Orphazyme

Anders Vadsholt, CFO +45
28 98 90 55

About Orphazyme

Orphazyme is a Danish biotech company with a
late stage orphan drug pipeline, developing new treatment options for orphan
protein misfolding diseases. The Company was founded in 2009 based on early
scientific discovery in heat shock proteins ("HSPs"). Since inception, the
Company has translated scientific discovery into a late stage clinical
development programme. The Company is headquartered in Copenhagen and currently
has 30 employees.

The Company focuses on severe and mostly fatal
diseases with a high unmet need, and with a particularly strong commitment to
neuromuscular diseases and a group of severe genetic diseases called lysosomal
storage diseases. The Company plans to pursue development of its lead candidate
through to registration in the EU and the United States after which launch and
commercialisation is expected to be undertaken by the Company.

The
lead candidate arimoclomol is in development as a potential treatment for four
orphan diseases; two neuromuscular diseases, sporadic Inclusion Body Myositis
("sIBM") and Amyotrophic Lateral Sclerosis ("ALS"), and two lysosomal storage
diseases, Niemann Pick type C ("NPC") and Gaucher disease.

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