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

The mechanisms underlying the development and natural progression of the airway mucus
defect in cystic fibrosis (CF) remain largely unclear. New animal models of CF, coupled with …

Cystic fibrosis (CF) lung disease involves chronic bacterial infection of retained airway
secretions (mucus). Recent data suggest that CF lung disease pathogenesis reflects the …

The current pathophysiological model of cystic fibrosis lung disease assumes that defective
expression, trafficking, or function of the cystic fibrosis transmembrane regulator (CFTR) …

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

Lung disease in people with cystic fibrosis (CF) is initiated by defective host defense that
predisposes airways to bacterial infection. Advanced CF is characterized by a deficit in …

Cystic fibrosis (CF) is a recessive genetic disease that is characterised by airway mucus
plugging and reduced mucus clearance. There are currently alternative hypotheses that …

The pathogenesis of mucoinfective lung disease in cystic fibrosis (CF) patients likely
involves poor mucus clearance. A recent model of mucus clearance predicts that mucus flow …

Cystic fibrosis (CF) is a life-limiting disease characterised by recurrent respiratory infections,
inflammation and lung damage. The volume and composition of the airway surface liquid …

Cystic fibrosis (CF) lung disease reflects persistent bacterial infection of airway lumens.
Several hypotheses have been advanced to link mutations in the CFTR gene to the failure of …