CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity.

ABSTRACT:

Background

The primary underlying defect in cystic fibrosis (CF) is disrupted ion transport in epithelia throughout the body. It is unclear if symptoms such as airway hyperreactivity (AHR) and increased airway smooth muscle (ASM) volume in people with CF are due to inherent abnormalities in smooth muscle or are secondary to epithelial dysfunction. Transforming Growth Factor beta 1 (TGFβ) is an established genetic modifier of CF lung disease and a known driver of abnormal ASM function. Prior studies have demonstrated that CF mice develop greater AHR, goblet cell hyperplasia, and ASM hypertrophy after pulmonary TGFβ exposure. However, the mechanism driving these abnormalities in CF lung disease, specifically the contribution of CFTR loss in ASM, was unknown.

Methods

In this st

SUBMITTER: Kramer EL 

PROVIDER: S-EPMC10416378 | biostudies-literature | 2023 Aug

REPOSITORIES: biostudies-literature