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 …

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 …

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

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

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 …

James M. Wilson, Editor ductance, and by analogy with the sweat duct (11, 12) this allows
salt levels in the ASL to remain at levels similar to those in plasma. The high salt in the ASL …

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 pathogenesis of cystic fibrosis (CF) airways infection is unknown. Two hypotheses,"
hypotonic [low salt]/defensin" and" isotonic volume transport/mucus clearance," attempt to …

Cigarette smoke (CS) exposure induces mucus obstruction and the development of chronic
bronchitis (CB). While many of these responses are determined genetically, little is known …

The human airway has two aqueous layers that are vital for normal lung function. A mucous
layer traps inhaled bacteria and foreign particles. A thin layer of airway surface fluid (ASL) …