Investigating Origins of Idiopathic Pulmonary Fibrosis Development
Idiopathic pulmonary fibrosis (IPF) results in 40,000 deaths in the U.S. annually. Patients with IPF have a median survival of 4 years. The only definitive treatment is lung transplantation. New interventions are urgently needed. IPF is thought to originate from alveolar epithelial type II (ATII) lung cells, which secrete a substance called pulmonary surfactant and prevent collapse of the lung. Mutations found in IPF patients impede ATII cell functions, including surfactant production and promote deterioration of lung activity. This research will investigate the origins of IPF development by introducing mutations to generate IPF cells. We will study mechanisms that lead to dysfunctional ATII cells. This research could lead to new drug targets for IPF.