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

Cystic fibrosis (CF) lung disease reflects the failure of airways defense against chronic
bacterial infection. Studies of CF cultures, transgenic mice, and CF patients suggest that the …

Introduction: Cystic fibrosis is an autosomal recessive disorder caused by mutations in the
cystic fibrosis transmembrane conductance regulator (CFTR) gene that codes for the CFTR …

Although there has been impressive progress in the elucidation of the genetic and molecular
basis of cystic fibrosis (CF), the pathogenesis of CF lung disease remains obscure. The …

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

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 …

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 …

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene result in
defective epithelial cAMP-dependent Cl− secretion and increased airway Na+ absorption …

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 …

Airway mucus obstruction is a key feature of cystic fibrosis (CF) and chronic obstructive
pulmonary disease (COPD). The thin layer of mucus that covers healthy airway surfaces has …