NIH R01 · 2025
Role of the ER stress transcription factor XBP1S in the development of idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis (IPF) is a progressive and end-stage lung disease of unknown etiology and no cure. It is likely that genetic changes increase a person's risk of developing IPF, and then exposure to certain environmental factors and/or aging trigger the onset of the disease. The MUC5B promoter variant rs35705950 is present in ∼50% of individuals with IPF and is recognized as the strongest known risk factor (genetic and otherwise) for the development of IPF. This variant leads to overexpression of MUC5B mRNA and protein in both distal airway epithelia and honeycomb cysts in the peripheral lung. These observations raise the question of why excessive MUC5B expression in distal…
From the public funding record at NIH RePORTER. Describes the funded project, not the reviews below.