Orphazyme Raises $19.6 Million in a Series A Financing Round

Copenhagen, Denmark - September 7, 2011; Orphazyme ApS, a biopharmaceutical company which develops innovative treatments for a family of genetic disorders called lysosomal storage diseases, today announced the successful completion of a €14 million Series A financing round. Participants were new investor Aescap Venture, as well as existing investors Novo A/S, and Sunstone Capital. In conjunction with the closing of the financing round, Dr. Kim Dueholm and Dr. Peter Moldt, both Partners at Novo Ventures, and Dr. Hakan Goker, Principal at Aescap Venture, will join Orphazyme’s Board of Directors.

Orphazyme was founded on the pioneering discovery related to the molecular chaperone, heat-shock protein 70, which reverts the pathology in cell extracts from patients suffering from various lysosomal storage diseases. Currently, only few treatment options exist for this family of serious genetic disorders, of which most are currently untreatable and often fatal, thus representing a significant unmet medical need. Orphazyme has applied its discovery capabilities to building a proprietary platform used for a novel and paradigm-changing approach to developing new treatments for these serious diseases.

Hakan Goker, Principal at Aescap Venture, said: “Lysosomal storage disorders, a group of debilitating diseases with high unmet medical need, still lack new and effective treatments. Our efforts in this space have identified Orphazyme as one of the best biotechs globally with potentially the most broadly applicable technology that can revolutionize the treatment of these diseases. We are proud to be able to support the strong leadership and science of Orphazyme, and be a part of this exciting development with an excellent syndicate of investors.”

Kim Dueholm, Partner at Novo Ventures, said: "Novo A/S, through Novo Seeds, provided grant funding prior to becoming a shareholder and was a founding investor in Orphazyme. Novo Ventures is pleased to continue the funding provided by Novo A/S to Orphazyme. Novo A/S believed in Orphazyme from the beginning and we still find that Orphazyme has one the most promising approaches to finding a breakthrough treatment for several lysosomal storage diseases giving hope to patients suffering from these rare, but very serious disorders."

Sten Verland, existing member of the Board of Directors for Sunstone Capital, said: “Sunstone Capital has supported Orphazyme from the early stages of development and we are very pleased that the unique technology and the progress of Orphazyme’s lead preclinical development program have made it possible to attract a dedicated and experienced new investor to the syndicate.”

Anders Hinsby, CEO of Orphazyme, said: ”We are very pleased to have completed this €14 million Series A financing round and the commitment of such high quality investors. We are now well positioned to progress our lead program, Orph-001, into clinical development.”

For further information, please contact:

Anders Hinsby, CEO of Orphazyme:

E-mail: amh@orphazyme.com

About Orphazyme

Orphazyme ApS is a Danish biopharmaceutical company, which develops paradigm-changing medicines for the treatment of a family of genetic disorders called lysosomal storage diseases. This family of genetic disorders consists of more than 45 diseases, most of which are currently untreatable and often fatal. Lysosomal storage diseases affect mostly children, who often die at a young age, many within a few years from birth. Other patients live longer, but ultimately die following years of suffering from defects in various organs such as the kidneys, liver, heart and CNS. Orphazyme has two projects in preclinical development, headed by its lead program, Orph-001, a recombinant version of human HSP70 being developed for the treatment of lysosomal storage diseases. Orphazyme is based on the pioneering discoveries emerging from the academic laboratory of its scientific founders: Professor Marja Jäättela and Dr. Thomas Kirkegaard Jensen from the Danish Cancer Society (Kræftens Bekæmpelse). The company is based in Copenhagen, Denmark, and has established collaborations with top-tier academic institutions in Europe and the USA. Orphazyme has a strong investor base which includes Novo Ventures, Sunstone Capital and Aescap Venture. For more information, please visit www.orphazyme.com

About Novo Ventures

Novo A/S, the holding company in the Novo Group, was established prior to the demerger of Novo Nordisk A/S in 2000. Novo A/S is a private limited liability company fully owned by the Novo Nordisk Foundation. Besides being the majority shareholder in Novo Nordisk A/S and Novozymes A/S, Novo A/S provides seed capital (through Novo Seeds), venture capital (through Novo Ventures), and growth capital (through Novo Growth Equity) to development stage companies within life science and biotechnology. Novo A/S also manages a broad portfolio of financial assets. For further information visit www.novo.dk

About Sunstone Capital

Sunstone Capital is an independent venture capital investor founded in 2007 by an international team of industry experts with combined entrepreneurial, operational and financial experience. Sunstone Capital focuses on developing and expanding early-stage Life Science and Technology companies with strong potential to achieve global success in their markets. Within life science, Sunstone Capital has invested in more than 35 companies in the areas of pharmaceuticals, medical technologies and diagnostics, and has completed several successful exits and IPOs. With total funds of 500 million Euro under management, Sunstone Capital is one of the largest Nordic-based venture capital investors. For further information, please visit www.sunstonecapital.com

About Aescap Venture

Aescap Venture is a venture capital company investing in private medical companies in Europe. Aescap Venture has a long-term investment horizon and invests in high-potential companies. This involves investments in all phases of development with an emphasis on earlier stages. Aescap Venture’s added value is based on a multi-disciplinary team of experienced investment partners with proven track records of success and the skills to coach entrepreneurs in accelerating the growth of their companies. For further information, please visit www.aescap.com

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