Mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator
undermine many host defense systems by inhibiting the function of airway-surface liquid …

The airway surface liquid (ASL) is a thin sheet of fluid that covers the luminal aspect of the
airway epithelium. The ASL is a site of several first-line host defenses, and its composition is …

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 …

Cystic fibrosis (CF) is caused by mutations in the gene coding for the CF transmembrane
conductance regulator (CFTR). From human normal tracheal submucosal gland cells in …

The development of effective therapies for cystic fibrosis (CF) requires animal models that
can appropriately reproduce the human disease phenotype. CF mouse models have …

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 …

The link between the genetic defect in cystic fibrosis (CF) and the recently described breach
in pulmonary host defense has focused on the role of salt and water metabolism in the …

Airway submucosal glands have been proposed as a primary site for initiating and
sustaining airway disease in cystic fibrosis (CF). However, it has been difficult to define the …

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

Cystic fibrosis patients exhibit lung disease consistent with a failure of innate airway defense
mechanisms. The link between abnormal ion transport and disease initiation and …