National Heart, Lung, and Blood Institute 3R01HL145408-07S1

Alveolar Epithelial Cell Dysfunction in Pulmonary Fibrosis: Leveraging SFTPC Mutations for Discovery of Molecular and Cellular Targets

NIH grant awarded to University of Pennsylvania to study the molecular and cellular mechanisms by which SFTPC mutations cause alveolar epithelial cell dysfunction leading to pulmonary fibrosis, aiming to discover new therapeutic targets for IPF.

Description

This project focuses on the role of alveolar epithelial type 2 (AT2) cells, particularly mutations in the Surfactant Protein C (SFTPC) gene, in the pathogenesis of Idiopathic Pulmonary Fibrosis (IPF). The research aims to understand how disruptions in cell quality control and mitochondrial function contribute to epithelial cell dysfunction and fibrosis, using novel mouse models expressing human-relevant SFTPC mutations to investigate molecular mechanisms driving epithelial cell reprogramming and fibrotic remodeling, with the goal of identifying new therapeutic targets.

View original record