Molecular prosthetics and CFTR modulators additively increase secretory HCO₃ ^- flux in cystic fibrosis airway epithelia.

ABSTRACT: Cystic Fibrosis (CF) is caused by loss-of-function mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR), an anion channel predominantly expressed on the apical membrane of epithelial cells. Reduced Cl^- and HCO₃ ^- secretion due to dysfunctional CFTR results in a decrease in lung function and is the leading cause of morbidity in individuals with CF. Recent therapies, known as highly effective CFTR modulator therapy (HEMT), help improve the lung function in individuals with specific CF-causing mutations by enhancing the folding, trafficking, and gating of CFTR. However, variability in HEMT responsiveness leads to sub-optimal clinical outcomes in some people with CF undergoing modulator therapy. A potential strategy is t

SUBMITTER: Celis N 

PROVIDER: S-EPMC12262498 | biostudies-literature | 2025 Jun

REPOSITORIES: biostudies-literature