Endeavor BioMedicines Launches with $62 Million to Attack Idiopathic Pulmonary Fibrosis

Rare Daily Staff

Endeavor BioMedicines has raised $62 million in a series A financing to develop new treatments targeting the underlying causes of pulmonary fibrosis.

Omega Funds, Longitude Capital and Endeavor’s management led the financing, proceeds of which will be used to advance taladegib, an inhibitor of the Hedgehog pathway, into two phase 2 clinical studies for the treatment of idiopathic pulmonary fibrosis (IPF) in 2021.

IPF is a serious, life-limiting lung disease characterized by fibrosis in the lungs, and this scar tissue accumulates until it reaches deadly levels. Replacement of normal lung tissue by fibrosis results in restriction in the ability to fill the lungs with air and decreased transfer of oxygen from inhaled air into the bloodstream resulting in lower oxygen delivery to the brain and other organs. Patients with IPF most often suffer from progressive shortness of breath, particularly with exertion; chronic cough; fatigue and weakness; and chest discomfort.

Current treatments help to slow progression, but there are none available that treat the underlying mechanisms of disease progression. There are approximately 132,000 people affected with IPF in the United States alone.

“IPF is a devastating condition with an estimated mean survival of two to five years from time of diagnosis, and currently there are no available therapies that stop the progression of fibrosis or treat the underlying causes of the disease,” said John Hood, co-founder, CEO and chairman of Endeavor. “Emerging preclinical and clinical evidence shows the Hedgehog signaling pathway, which is implicated in chronic wound healing, plays a critical role in IPF disease pathology. With taladegib’s impressive potency and safety profile, there is the potential to introduce an entirely new class of medicine that may be able to stop or reverse the course of this deadly disease.”

Taladegib is a small-molecule inhibitor of the Hedgehog signaling pathway. Hedgehog inhibitors have been approved for the treatment of cancers such as basal cell carcinoma and acute myelogenous leukemia, but they have yet to be applied toward pulmonary diseases.

The Hedgehog signaling pathway is a key modulator for disease progression in IPF. Myofibroblasts – the repair cells activated by the Hedgehog pathway – become dysregulated, relentlessly remodeling lung tissue, forming fibrotic scars and contracting the lung. This tissue remodeling disorder impairs lung function in IPF patients by making the lung inelastic, smaller and with compromised tissue structure. Selectively inhibiting this pathway in lung tissue causes the myofibroblasts responsible for the disorder to become inactivated and undergo apoptosis, thereby eliminating the key cellular driver of IPF and potentially stopping or reversing the disease.

Studied in 176 subjects to date, taladegib has been shown to safely and effectively inhibit the Hedgehog pathway. Endeavor intends to initiate a phase 2 study of taladegib as a monotherapy in patients with IPF in the second quarter of 2021. Pending results from the monotherapy study, Endeavor anticipates initiating a second phase 2 study in combination with standard of care by 2022.

“In developing a best-in-class Hedgehog inhibitor therapy and applying it in an emerging area of science, Endeavor has the potential to completely change the trajectory of IPF,” said Bernard Davitian, partner at Omega Funds.

Hood, who previously founded and served as CEO of Impact Biomedicines until its acquisition by Celgene in 2018, co-founded Endeavor BioMedicines specifically to meet the immense demand in the IPF patient community. His co-founder Miguel de los Rios, chief scientific officer at Endeavor, previously served as CEO of Rift Biotherapeutics.