Cystic fibrosis (CF) lung disease is the major cause of morbidity and mortality in people with
CF. Abnormal mucociliary transport has been the leading hypothesis for the underlying …

Aspergillus fumigatus (Af) is a mold frequently detected in airway samples from people with
cystic fibrosis (pwCF). Abnormal airway mucus may allow Af to germinate, resulting in airway …

In cystic fibrosis (CF), loss of CF transmembrane conductance regulator (CFTR) anion
channel activity causes airway surface liquid (ASL) pH to become acidic, which impairs …

BACKGROUND. Disruption of cystic fibrosis transmembrane conductance regulator (CFTR)
anion channel function causes cystic fibrosis (CF), and lung disease produces most of the …

Individuals with cystic fibrosis (CF) suffer progressive airway inflammation, infection and
lung damage. Airway inflammation and infection are present from early in life, often before …

In cystic fibrosis patients, electrolytic and osmolality imbalance secondary to cystic fibrosis
transmembrane conductance regulator mutations may impact on mucoid secretion …

Key points Cl− and HCO3− had similar paracellular permeabilities in human airway
epithelia. PCl/PNa of airway epithelia was unaltered by pH 7.4 vs. pH 6.0 solutions. Under …

Key points The epithelial Na+ channel (ENaC) is hyperactive in cystic fibrosis (CF) airway
epithelia, and contributes to excessive Na+ absorption and dehydration of the airway …

Introduction: Cystic fibrosis (CF) is a genetic disorder that results in a multi-organ disease
with progressive respiratory decline that ultimately leads to premature death. CF is caused …

Cystic fibrosis (CF) is an autosomal recessive disease caused by CFTR mutations. It is
characterized by high NaCl concentration in sweat and the production of a thick and sticky …