NIH Reporter #11184155

Surfactant Protein C Mutations and Interstitial Lung Disease

This award funds research on idiopathic pulmonary fibrosis using novel mouse models with mutations in the Surfactant Protein C gene to study lung epithelial cell dysfunction and identify new therapeutic targets.

Description

Idiopathic Pulmonary Fibrosis (IPF) is a devastating lung disease of older veterans marked by progressive respiratory failure for which there remains a significant unmet need for effective medical therapies. Lung epithelial cell dysfunction has emerged as an important causal contributor to the development of fibrotic lung scarring. To improve understanding of IPF, two novel mouse models expressing IPF-associated mutations in the lung epithelial cell restricted Surfactant Protein C gene (SFTPC) have been generated. This preclinical platform will be used to characterize mechanisms underlying lung epithelial dysfunction, including abnormal cellular quality control responses such as endoplasmic reticulum stress, autophagy, mitophagy, and metabolic reprogramming. The results aim to facilitate rapid identification of new cell populations, signaling pathways, and molecular targets, thereby accelerating the discovery of new IPF treatments.

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