Strategic Partners A/S — Director's Dealing 2020

Sep 1, 2020

ORPHAZYME

Company announcement

No. 47/2020

Orphazyme A/S

Ole Maaloes Vej 3

DK-2200 Copenhagen N

www.orphazyme.com

Company Registration No. 32266355

Reporting of transactions in Orphazyme's shares made by persons discharging managerial responsibilities

Copenhagen, Denmark, September 1, 2020 – Pursuant to the Market Abuse Regulation article 19, Orphazyme A/S, CVR no. 32266355 ("Orphazyme"), hereby notifies receipt of information of the following transactions in Orphazyme's shares by persons discharging managerial responsibilities in Orphazyme:

1	Details of the Reporting Person / Closely Associated Person
a)	Name	Sten Verland
2	Reason for the notification
a)	Position/status	Members of the Board of Directors
b)	Initial notification/Amendment	Initial notification
3	Details of the Company, emission allowance market participant, auction platform, auctioneer or auction monitor
a)	Name	Orphazyme A/S
b)	LEI code	54930025OZD2GGSQ7L42
4	Details of the transaction(s): section to be repeated for (i) each type of instrument; (ii) each type of transaction; (iii) each date; and (iv) each place where transactions have been conducted
a)	Description of the financial instrument, type of instrument and identification code	Restricted Share Units for shares in Orphazyme A/S
ISIN DK0060910917
b)	Nature of the transaction	Exercise of Restricted Share Units
c)	Price(s) and volume(s)		Price (s)		Volume(s)
			DKK	61.1895	1,927
d)	Aggregated information	Aggregated volume: 1,927
Aggregated price: DKK 117,912.17
Price per share (volume weighted average): DKK 61.1895
e)	Date of the transaction	1 September 2020, 5 p.m. CET
f)	Place of the transaction	Outside a trading venue
4	Details of the transaction(s): section to be repeated for (i) each type of instrument; (ii) each type of transaction; (iii) each date; and (iv) each place where transactions have been conducted

---

ORPHAZYME

| a) | Description of the financial instrument, type of instrument and identification code | Shares acquired by exercise of Restricted Share Units
ISIN DK0060910917 | | |
| --- | --- | --- | --- | --- |
| b) | Nature of the transaction | Sale of shares to Sunstone Life Science Ventures Fund II K/S | | |
| c) | Price(s) and volume(s) | Price (s) | | Volume(s) |
| | | DKK | 88.30 | 1,927 |
| d) | Aggregated information | Aggregated volume: 1,927
Aggregated price: DKK 170,154.10
Price per share (volume weighted average): DKK 88.30 | | |
| e) | Date of the transaction | 1 September 2020, 5 p.m. CET | | |
| f) | Place of the transaction | Outside a trading venue | | |
| | | | | |
| 1 | Details of the Reporting Person / Closely Associated Person | | | |
| a) | Name | Sunstone Life Science Ventures Fund II K/S | | |
| 2 | Reason for the notification | | | |
| a) | Position/status | Closely associated to Sten Verland, member of the Board of Directors | | |
| b) | Initial notification/Amendment | Initial notification | | |
| 3 | Details of the Company, emission allowance market participant, auction platform, auctioneer or auction monitor | | | |
| a) | Name | Orphazyme A/S | | |
| b) | LEI code | 54930025OZD2GGSQ7L42 | | |
| 4 | Details of the transaction(s): section to be repeated for (i) each type of instrument; (ii) each type of transaction; (iii) each date; and (iv) each place where transactions have been conducted | | | |
| a) | Description of the financial instrument, type of instrument and identification code | Shares acquired by exercise of Restricted Share Units
ISIN DK0060910917 | | |
| b) | Nature of the transaction | Purchase of shares from Sten Verland | | |
| c) | Price(s) and volume(s) | Price (s) | | Volume(s) |
| | | DKK | 88.30 | 1,927 |
| d) | Aggregated information | Aggregated volume: 1,927
Aggregated price: DKK 170,154.10
Price per share (volume weighted average): DKK 88.30 | | |
| e) | Date of the transaction | 1 September 2020, 5 p.m. CET | | |
| f) | Place of the transaction | Outside a trading venue | | |

Page 2 of 3

---

ORPHAZYME

For additional information, please contact

Orphazyme A/S

Anders Vadsholt, CFO
+45 28 98 90 55

About Orphazyme A/S

Orphazyme is a biopharmaceutical company focused on bringing novel treatments to patients living with life-threatening or debilitating rare diseases. Our research focuses on developing therapies for diseases caused by misfolding of proteins, including lysosomal storage diseases. Arimoclomol, the company's lead candidate, is in clinical development for four orphan diseases: Niemann-Pick disease Type C, Gaucher disease, sporadic Inclusion Body Myositis, and Amyotrophic Lateral Sclerosis. The Denmark-based company is listed on Nasdaq Copenhagen (ORPHA.CO). For more information, please visit www.orphazyme.com.

Forward-looking statement

This company announcement may contain certain forward-looking statements. Although the Company believes its expectations are based on reasonable assumptions, all statements other than statements of historical fact included in this company announcement about future events are subject to (i) change without notice and (ii) factors beyond the Company's control. These statements may include, without limitation, any statements preceded by, followed by, or including words such as "target," "believe," "expect," "aim," "intend," "may," "anticipate," "estimate," "plan," "project," "will," "can have," "likely," "should," "would," "could", and other words and terms of similar meaning or the negative thereof. Forward-looking statements are subject to inherent risks and uncertainties beyond the Company's control that could cause the Company's actual results, performance, or achievements to be materially different from the expected results, performance, or achievements expressed or implied by such forward-looking statements. Except as required by law, the Company assumes no obligation to update these forward-looking statements publicly, or to update the reasons actual results could differ materially from those anticipated in the forward-looking statements, even if new information becomes available in the future.

Page 3 of 3