Zevra Refinances Existing Debt with Up to $100 Million Under New Credit Facility

Rare Daily Staff

Rare diseases therapeutics company Zevra Therapeutics said it has entered into a new credit facility provided by leading biotech investors that provides up to $100 million in committed capital.

Led by Perceptive Advisors and Healthcare Royalty, the new credit facility comes in three tranches: an initial draw of $60 million at closing, a second tranche of up to $20 million available until October 5, 2025, and a third tranche of up to $20 million, which becomes available upon approval of arimoclomol, the company’s product candidate for the treatment of Niemann Pick disease Type C (NPC), in each case subject to certain terms and conditions. As part of this transaction, Zevra has retired its combined existing debt of approximately $43.1 million.

“Entry into this new credit facility is another step in Zevra’s execution of our strategic vision to become a leading rare disease company,” said R. LaDuane Clifton, Zevra’s chief financial officer, secretary, and treasurer.

The company says the restructuring of its debt will allow it to focus on its strategic priorities and extend its cash runway into 2026. These include successfully launching Olpruva, an approved treatment for urea cycle disorders that it acquired through a reverse merger with Acer Therapeutics, and preparing for the launch of arimoclomol, if approved.

Arimoclomol, Zevra’s orally delivered, first-in-class investigational product candidate for the treatment NPC type C, is under review by the U.S. Food and Drug Administration with the agency expected to act by September 21, 2024.

NPC is an ultra-rare, progressive, neurodegenerative lysosomal storage disorder characterized by an inability of the body to transport cholesterol and other lipids within the cell, leading to an accumulation of these substances in various tissue areas, including brain tissue. The disease is caused by mutations in the NPC1 or NPC2 genes, which are responsible for making lysosomal proteins. Both children and adults can be affected by NPC with varying clinical presentations. Those living with NPC lose independence due to physical and cognitive limitations, with key neurological impairments presenting in speech, cognition, swallowing, ambulation, and fine motor skills. Disease progression is irreversible and can be fatal within months or take years to be diagnosed and advance in severity.

Arimoclomol has been granted Orphan Drug, Fast Track, Breakthrough Therapy, and Rare Pediatric Disease designations by the FDA, and Orphan Medicinal Product designation for the treatment of NPC by the European Medicines Agency.