Putting bicarbonate on the spot: pharmacological insights for CFTR correction in the airway epithelium.

ABSTRACT: Introduction: Cystic fibrosis (CF) is caused by defective Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) proteins. CFTR controls chloride (Cl^-) and bicarbonate (HCO₃ ^-) transport into the Airway Surface Liquid (ASL). We investigated the impact of F508del-CFTR correction on HCO₃ ^- secretion by studying transepithelial HCO₃ ^- fluxes. Methods: HCO₃ ^- secretion was measured by pH-stat technique in primary human respiratory epithelial cells from healthy subjects (WT) and people with CF (pwCF) carrying at least one F508del variant. Its changes after CFTR modulation by the triple combination VX445/661/770 and in the context of TNF-α+IL-17 induced inflammation were correlate

SUBMITTER: Zajac M 

PROVIDER: S-EPMC10750368 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature