National Heart, Lung, and Blood Institute 5P01HL170952-03

Patient-specific iPSCs to model and treat the inception of pulmonary fibrosis

This NIH grant funds research using patient-specific iPSC-derived AT2 cells to study mitochondrial dysfunction and environmental factors in pulmonary fibrosis and to test AMPK activators as potential treatments.

Description

Idiopathic pulmonary fibrosis (IPF) is characterized by progressive fibrosis leading to disruption of the gas exchange unit and death within an average of four years from diagnosis. A poor understanding of IPF pathogenesis, partly due to a lack of reliable human disease models, has hindered the development of effective therapies. Dysfunction of alveolar epithelial type 2 (AT2) cells has been implicated as a potential proximal disease driver. Using patient-specific induced pluripotent stem cell (iPSC)-derived AT2 cells (iAT2s), the research aims to understand mitochondrial dysfunction, environmental insults like cigarette smoke, and test potential therapeutics such as AMPK activators.

View original record