Rationale: Animal models have been highly informative for understanding the
characteristics, onset, and progression of cystic fibrosis (CF) lung disease. In particular, the …

Cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated chloride
channel expressed in a wide variety of epithelial cells, mutations of which are responsible …

Previous studies demonstrated that ACh-induced liquid secretion by porcine bronchi is
driven by active Cl− and H CO 3− secretion. The present study was undertaken to determine …

Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis
transmembrane conductance regulator (CFTR) anion channel. Airway disease is the major …

Pancreatic duct epithelium secretes a HCO3−-rich fluid by a mechanism dependent on
cystic fibrosis transmembrane conductance regulator (CFTR) in the apical membrane …

A central function of cystic fibrosis transmembrane conductance regulator (CFTR)-
expressing tissues is the secretion of fluid containing 100–140 mmHCO 3−. High levels of …

Background Disrupted HCO 3–transport and reduced airway surface liquid (ASL) pH in
cystic fibrosis (CF) may initiate airway disease. We hypothesized that ASL pH is reduced in …

BACKGROUND: CFTR contributes to HCO3− transport in epithelial cells both directly (by
HCO3− permeation through the channel) and indirectly (by regulating Cl−/HCO3− exchange …

The role of the cystic fibrosis transmembrane conductance regulator (CFTR) in cAMP-
stimulated HCO 3− secretion across the murine duodenum was investigated. Serosal-to …

The tracheobronchial submucosal glands secrete liquid that is important for hydrating airway
surfaces, supporting mucociliary transport, and serving as a fluid matrix for numerous …