Award
NIH Reporter #5R01HL145408-07
Alveolar Epithelial Cell Dysfunction in Pulmonary Fibrosis: Leveraging SFTPC Mutations for Discovery of Molecular and Cellular Targets
Recipient
University of Pennsylvania
Award Amount
$856,402.00
Ceiling
$856,402.00
Awarded
July 23, 2026
Identifier
5R01HL145408-07
Research grant awarded to University of Pennsylvania to study alveolar epithelial cell dysfunction in pulmonary fibrosis related to SFTPC mutations, aiming to discover molecular and cellular therapeutic targets.
Description
This project focuses on the role of alveolar epithelial type 2 (AT2) cells and their genetic variants, particularly mutations in the Surfactant Protein C (SFTPC) gene, which are associated with human lung fibrosis. The research aims to understand how disruptions in cell quality control and unfolded protein response (UPR) signaling contribute to epithelial cell dysfunction and fibrosis. Using novel mouse models expressing mutant SFTPC, the study will investigate mechanisms of proteostasis disruption, metabolic reprogramming, and mitochondrial dynamics in AT2 cells, with the goal of identifying new therapeutic targets.