Capital Increase of 11,380 Shares (Equivalent to Approximately 0.06% of the Existing Shares) in Orphazyme A/S as a Result of an Issue of Bonus Shares to KLSDC and UCLB

Copenhagen, Denmark, January 29, 2018 – Orphazyme, a Danish biotech company listed on Nasdaq Copenhagen (TICKER: ORPHA.CO), with a late-stage, orphan-drug pipeline, today decided on a capital increase as a result of a directed issue of bonus shares to Kansas Life Sciences Development Company, Inc. (“KLSDC”) and UCL Business PLC (“UCLB”). The capital increase was decided pursuant to the Board of Directors’ authorization laid down in Article 3.6 of the Articles of Association. 

According to the license agreement entered into on October 31, 2017 between among others Orphazyme A/S, KLSDC, and UCLB, Orphazyme A/S shall, following the end of each calendar year, issue or otherwise deliver to KLSDC and UCLB a total number of shares equal to the aggregate amount of cash funding that KLSDC and UCLB have reported to have spent under the grant from the US Food and Drug Administration received with respect to Orphazyme A/S’ on-going Phase II/III clinical trial on arimoclomol for the treatment of sIBM. On the basis of a financial report of expenditures provided by KLSDC and UCLB, Orphazyme A/S will issue 11,380 new shares each with a nominal value of DKK 1, equal to approximately 0.06% of its total share capital, in favor of KLSDC and UCLB. Accordingly, Orphazyme A/S’ share capital will be increased by nominally DKK 11,380 by way of an issue of bonus shares at par value by transfer of DKK 11,380 from Orphazyme’s distributable reserves. The number of shares to be delivered to KLSDC and UCLB has been calculated based on the average closing price of Orphazyme’s shares on Nasdaq Copenhagen during a 30-day period preceding the date of issuance. Reference is made to the prospectus published by Orphazyme A/S on November 6, 2017 for a further description of the license agreement.

The new shares are negotiable instruments and will in every respect carry the same rights as the existing shares from the time of registration of the capital increase with the Danish Business Authority (which is expected to occur today). The new shares are expected to be admitted to trading and official listing on Nasdaq Copenhagen on or around February 1, 2018.

Following the share capital increase, the total nominal share capital will be DKK 19,939,564, divided into 19,939,564 shares each with a nominal value of DKK 1. Each share carries one vote at Orphazyme's general meetings. Updated Articles of Association can be found at www.orphazyme.com.

For additional information, please contact

Orphazyme A/S

Anders Vadsholt, CFO                         +45 28 98 90 55

About Orphazyme A/S
Orphazyme is a Danish biotech company listed on Nasdaq Copenhagen (ticker: ORPHA.CO) with a late-stage 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. The company is headquartered in Copenhagen and currently has 35 employees. 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. For more information, please visit www.orphazyme.com.