CFTR ion transport deficiency primes the epithelium for partial epithelial-mesenchymal transition in cystic fibrosis.

ABSTRACT:

Introduction

Cystic fibrosis (CF) is a monogenic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which encodes a Cl^-/HCO₃ ^- ion channel located at the apical plasma membrane (PM) of epithelial cells. CFTR dysfunction disrupts epithelial barrier integrity, drives progressive airway remodelling and has been associated with epithelial-to-mesenchymal transition (EMT), a process in which cells lose epithelial properties and acquire mesenchymal characteristics. We previously demonstrated that mutant CFTR directly drives partial EMT, independently of secondary events such as bacterial infection or inflammation.

Methods

Here, we investigated whether PM localisation of CFTR alone is sufficient to pre

SUBMITTER: Rodrigues CS 

PROVIDER: S-EPMC12405223 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature