Orphazyme completes offering of 7,032,937 shares in a directed issue and private placement and raises approximately DKK 745,000,000 (EUR 100 million/USD 110 million)

Copenhagen, Denmark, February 7, 2020 - Orphazyme A/S (ORPHA.CO), a biopharmaceutical company pioneering Heat-Shock Protein response for the treatment of neurodegenerative orphan diseases, today announces the completion of an offering of 7,032,937 new shares and existing shares in a directed issue and private placement raising gross proceeds of approximately DKK 745,000,000.

Kim Stratton, Chief Executive Officer, said: "I would like to thank our investors, both existing and new, for their confidence in Orphazyme as the company enters a very exciting phase in its development. This funding provides a significant cash runway for Orphazyme as we progress our lead product arimoclomol towards market, and is expected to cover our ambitious clinical development plans well into 2021. In particular, the financing will support our imminent US and European filings of arimoclomol for Niemann-Pick disease Type C (NPC) and preparations for commercial launch."

Orphazyme also intends to use the proceeds to cover completion of arimoclomol's clinical development in sporadic Inclusion Body Myositis (sIBM) and Amyotrophic Lateral Sclerosis (ALS) and to prepare regulatory filings in Europe and potentially the US in these indications, as well as for general corporate purposes.

Based on the positive data package announced in 2019, Orphazyme currently expects to submit an application for approval of arimoclomol for the treatment of NPC in H1 2020 in the US and H2 2020 in the EU. Orphazyme expects to release results from the ongoing phase 2 trial for Gaucher Disease in H1 2020 and continue its ongoing phase 3 trial in ALS and phase 2/3 trial in sIBM. Orphazyme has been granted Fast Track and Breakthrough Therapy designation from the US Food and Drug Administration (FDA) for the development of arimoclomol in NPC, and Fast Track designation for the development of arimoclomol in sIBM.

The directed issue and private placement of 3,961,264 new shares of a nominal value of DKK 1 each (the "New Shares") and private placement of 3,071,673 existing shares of a nominal value of DKK 1 each (the "Existing Shares" and together with the New Shares, the "Offer Shares") (the "Offering") in Orphazyme A/S (the "Company") has been successfully executed. Reference is made to company announcement no. 08/2020.

The Offer Shares were subscribed for and sold with significant demand from both domestic and international investors in the EU and the US.

The offering of Existing Shares was facilitated by a share loan from Novo Holdings A/S and Orpha Pooling B.V. (the "Lending Shareholders") to the Company pursuant to a stock lending and subscription agreement with an obligation for the Company to redeliver new shares of an equivalent number as the Existing Shares borrowed by the Company from each of the Lending Shareholders (the "Replacement Shares"). The Lending Shareholders do not participate in the Offering, and are only facilitating the loan of the Lending Shares for purposes of the Company's offering of Existing Shares in the Offering.

The investors have subscribed for or purchased 3,961,264 New Shares and 3,071,673 Existing Shares at an offer price of DKK 106 per Offer Share, as determined by the Board of Directors through a book-building process and accordingly 3,071,673 Replacement Shares will be subscribed for at an offer price of DKK 106 per Replacement Share, raising aggregate gross proceeds for Orphazyme of approximately DKK 745,000,000 (EUR 100 million/USD 110 million).

As the Offering was oversubscribed by Danish and international institutional and professional investors, the shares have been allocated on an individual basis.

Capital increases
Subject to settlement of the capital increases, the share capital of the Company will consist of 27,038,386 shares with a nominal value of DKK 1 each.

The nominal value of the New Shares and Replacement Shares represents approximately 35.16% of the Company's registered share capital of DKK 20,005,449 before the capital increases and will account for approximately 26.01% of the Company's registered share capital of DKK 27,038,386 upon completion of the capital increases.

Expected timetable

Date Event
February 11, 2020 Settlement and payment for the Offer Shares and Replacement Shares
February 11, 2020 Registration of the capital increases with the Danish Business Authority
February 12, 2020 New Shares to be admitted to trading and official listing on Nasdaq Copenhagen A/S

The New Shares will be issued in the temporary ISIN code DK0061274289, the Existing Shares will be purchased in the permanent ISIN code DK0060910917, and the Replacement Shares for the Lending Shareholders will be issued in the temporary ISIN code DK0061274362.

Settlement for investors, who have subscribed for New Shares will take place in the temporary ISIN code DK0061274289, settlement for investors, who have purchased Existing Shares will take place in the permanent ISIN code DK0060910917, and settlement of the Replacement Shares will take place in the temporary ISIN code DK0061274362.

The admission to trading and official listing of the New Shares and the later admission to trading and official listing of the Replacement Shares on Nasdaq Copenhagen A/S is subject to the Offering not being withdrawn prior to the settlement of the Offering and the Company making an announcement to that effect, and in respect of the Replacement Shares only (which will only be delivered to the Lending Shareholders and not be sold as part of the Offering), the publication of a listing prospectus to be approved by the Danish Financial Supervisory Authority, which is expected to occur no later than by the end of March 2020.

Managers
Guggenheim Securities, LLC and Danske Bank A/S are acting as Joint Global Coordinators in connection with the Offering.

ISIN codes
Permanent ISIN code DK0060910917.

Temporary ISIN code for New Shares DK0061274289.

Temporary ISIN code for Replacement Shares DK0061274362.

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 statements
This company announcement may contain certain forward-looking statements, including with respect to the timing of the Company's clinical trials and the expected submission of application for approval of arimoclomol for the treatment of NPC in the U.S and the EU. 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.

----ooOoo----

This announcement is not for publication or distribution, directly or indirectly, in or into the United States of America. This announcement and the information it contains do not constitute an offer of securities for sale nor a solicitation of any offer to buy any securities issued by Orphazyme A/S in the United States or any other jurisdiction where such offer or sale would be unlawful and the announcement and the information contained herein are nor distribution or release, directly or indirectly, in or into such jurisdictions. The securities referred to herein have not been and will not be registered under the U.S. Securities Act of 1933, as amended (the "U.S. Securities Act"), and may not be offered or sold in the United States, except pursuant to registration or an applicable exemption from registration under the U.S. Securities Act and applicable state securities law. No public offering of securities is being made in the United States under the U.S. Securities Act.

With respect to the member states of the European Economic Area subject to Regulation 2017/1129/EU (together with any applicable implementing measures, the "Prospectus Regulation") (each a "Relevant Member State"), no action has been undertaken or will be undertaken to make an offer to the public of the securities referred to herein requiring the publication of a prospectus in any Relevant Member State. As a result, these securities may only be offered or sold in any Relevant Member State pursuant to an exemption under the Prospectus Regulation.

This announcement is only addressed to, and directed at, persons in Relevant Member States of the European Economic Area who are "qualified investors" within the meaning of Article 2(e) of the Prospectus Regulation ("Qualified Investors").

In the United Kingdom, this announcement is directed only at Qualified Investors (i) who are "investment professionals" falling within Article 19(5) of the Financial Services and Markets Act 2000 (Financial Promotion) Order 2005, as amended (the "Order"), or (ii) persons falling within Article 49(2)(a)-(d) ("high net worth companies, unincorporated associations etc") of the Order all such persons together being referred to as ("Relevant Persons"). Under no circumstances should persons who are not Relevant Persons rely or act upon the contents of this announcement. Any investment or investment activity to which this announcement relates in the United Kingdom is available only to, and will be engaged only with, Relevant Persons.

Any investment decision in connection with the Offering must be made on the basis of all publicly available information relating to Orphazyme A/S and Orphazyme A/S' shares. Such information has not been independently verified by Guggenheim Securities, LLC and Danske Bank A/S. The information contained in this announcement is for background purposes only and does not purport to be full or complete. No reliance may be placed for any purpose on the information contained in this announcement or its accuracy or completeness. This announcement does not purport to identify or suggest the risks (direct or indirect) which may be associated with an investment in Orphazyme A/S and Orphazyme A/S' shares.

Guggenheim Securities, LLC and Danske Bank A/S are acting for Orphazyme A/S only in connection with the Offering and no one else, and will not be responsible to anyone other than Orphazyme A/S for providing the protections offered to clients of Guggenheim Securities, LLC and Danske Bank A/S, nor for providing advice in relation to the Orphazyme A/S' shares or the Offering.

In connection with any offering of the Offer Shares, Guggenheim Securities, LLC and Danske Bank A/S and any of their respective affiliates acting as an investor for their own account may take up as a proprietary position any Offer Shares and in that capacity may retain, purchase or sell for their own account such Offer Shares. In addition certain of Guggenheim Securities, LLC and Danske Bank A/S or their affiliates may enter into financing arrangements and swaps with investors in connection with which Guggenheim Securities, LLC and Danske Bank A/S (or their affiliates) may from time to time acquire, hold or dispose of Orphazyme A/S' shares. Guggenheim Securities, LLC and Danske Bank A/S do not intend to disclose the extent of any such investment or transactions otherwise than in accordance with any legal or regulatory obligation to do so.

To read more Press Release articles, click here.