XBP1S Regulates MUC5B in a Promoter Variant–Dependent Pathway in Idiopathic Pulmonary Fibrosis Airway Epithelia

摘要

The goal was to connect elements of idiopathic pulmonary fibrosis (IPF) pathogenesis, including chronic endoplasmic reticulum stress in respiratory epithelia associated with injury/inflammation and remodeling, distal airway mucus obstruction and honeycomb cyst formation with accumulation of MUC5B (mucin 5B), and associations between IPF risk and polymorphisms in the promoter.