Submucosal glands line the cartilaginous airways and produce most of the antimicrobial
mucus that keeps the airways sterile. The glands are defective in cystic fibrosis (CF), but how …

Chronic lung disease remains the major cause of morbidity and mortality of cystic fibrosis
(CF) patients. Cftr mutant mice developed severe intestinal obstruction, but did not exhibit …

Question Cystic fibrosis (CF) is characterised by the accumulation of viscous adherent
mucus in the lungs. While several hypotheses invoke a direct relationship with cystic fibrosis …

The pathogenesis of cystic fibrosis (CF) lung disease is reviewed, focusing on an overview
of the physiologic mechanisms that regulate mucus transport. A major emphasis is placed …

Key points• Lung hydration and mucus clearance rates are set by a balance between CFTR‐
mediated Cl− secretion and ENaC‐led Na+ absorption. In CF airways, CFTR is diminished …

Rationale: The mechanisms underlying cystic fibrosis (CF) lung disease pathogenesis are
unknown. Objectives: To establish mechanisms linking anion transport with the functional …

Airway disease in cystic fibrosis (CF) is characterised by a continuous cycle of chronic
infection and inflammation dominated by a neutrophilic infiltrate. This inflammation is …

Cystic fibrosis (CF), which is caused by mutations in CFTR, affects many tissues, including
the lung. Submucosal gland serous acinar cells are primary sites of fluid secretion and …

Background: The loss of cystic fibrosis transmembrane conductance regulator (CFTR)
mediated chloride conductance does not fully explain the diverse pathologies evident in …

Cystic Fibrosis (CF), an autosomal recessive genetic disease, is caused by a mutation in the
gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). This …