NIH R01 · 2025
ABSTRACT Idiopathic Pulmonary fibrosis (IPF) is a devastating interstitial lung disease (ILD) characterized by disruption of distal lung architecture that results in scar formation, abnormal gas exchange, and respiratory failure. Barriers to better IPF outcomes have included an incomplete understanding of its pathophysiologic underpinnings and a dearth of translationally relevant preclinical models. However, identification of rare genetic variants in the alveolar epithelial type 2 (AT2) cell-restricted Surfactant Protein C (SP-C) gene (SFTPC) in subsets of PF patients has been part of a paradigm shift in which dysfunctional AT2 cells serve as a proximal driver of IPF. Coupled with the…
From the public funding record at NIH RePORTER. Describes the funded project, not the reviews below.