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

February 1, 2021 2:10 PM EST

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Orphazyme A/SCompany announcement                                                                              No. 03/2021                                                                                                  Company Registration No. 32266355                                                                                       

Copenhagen, Denmark, February 1, 2021 – 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:

Details of the Reporting Person / Closely Associated Persona) NameAnders Vadsholt

Reason for the notification a) Position/statusChief Financial Officer b) Initial notification/AmendmentInitial notification

Details of the Company, emission allowance market participant, auction platform, auctioneer or auction monitor a) NameOrphazyme A/Sb) LEI code54930025OZD2GGSQ7L42

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 codeSharesISIN DK0060910917

b) Nature of the transactionVesting of Matching Sharesc) Price(s) and volume(s)


d) Aggregated informationAggregated volume: 17,561 sharesAggregated price: DKK 17,561.00Price per share (volume weighted average): DKK 1e) Date of the transactionFebruary 1, 2021f) Place of the transactionOutside a trading venue

For additional information, please contact

Orphazyme A/S

Anders Vadsholt, CFO                             +45 28 98 90 55

About Orphazyme A/S Orphazyme is a late-stage biopharmaceutical company pioneering the Heat-Shock Protein response for the treatment of neurodegenerative orphan diseases. Orphazyme is harnessing amplification of Heat-Shock Proteins (or HSPs) in order to develop and commercialize novel therapeutics for diseases caused by protein misfolding, protein aggregation, and lysosomal dysfunction, including lysosomal storage diseases and neuromuscular degenerative diseases. Arimoclomol, the Orphazyme’s lead candidate, is in clinical development for four orphan diseases: Niemann-Pick disease Type C (NPC), Amyotrophic Lateral Sclerosis (ALS), sporadic Inclusion Body Myositis (sIBM) and Gaucher disease. Orphazyme is headquartered in Denmark and has operations in the U.S. and Switzerland. Orphazyme’s shares are listed on Nasdaq U.S. (ORPH) and Nasdaq Copenhagen (ORPHA).

About arimoclomol Arimoclomol is an investigational drug candidate that amplifies the production of Heat-Shock Proteins (HSPs). HSPs can rescue defective misfolded proteins, clear protein aggregates, and improve the function of lysosomes. Arimoclomol is administered orally and has now been studied in seven phase 1, four phase 2 and one pivotal phase 2/3 trial. Arimoclomol is in clinical development for NPC, Gaucher Disease, sIBM, and ALS. Arimoclomol has received orphan drug designation (ODD) for NPC, sIBM, and ALS in the US and EU. Arimoclomol has received fast-track designation (FTD) from the U.S. Food and Drug Administration (FDA) for NPC, sIBM and ALS. In addition, arimoclomol has received breakthrough therapy designation (BTD) and rare-pediatric disease designation (RPDD) from the FDA for NPC.

Forward-looking statements 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.



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