Cystic fibrosis (CF) is a genetic disease hallmarked by aberrant ion transport that results in
delayed mucus clearance, chronic infection, and progressive lung function decline. Several …

Cystic fibrosis, the most common inherited lethal disease in Caucasians, is caused by
mutations in the CF gene. The gene product, the CF transmembrane conductance regulator …

In humans, cystic fibrosis (CF) lung disease is characterised by chronic infection,
inflammation, airway remodelling, and mucus obstruction. A lack of pulmonary …

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) result in
defective ion transport, leading to thick mucus, impaired mucociliary clearance and …

RECURRENT INFECTION AND DETERIORATION of lung function are the major causes of
morbidity and mortality in cystic fibrosis. Although the genetic defect in cystic fibrosis …

Patients with cystic fibrosis (CF) suffer from the consequences of deficient CFTR (CF
transmembrane conductance regulator gene) activity (1). CFTR controls epithelial chloride …

Animal models have been highly informative for understanding the pathogenesis and
progression of cystic fibrosis (CF) lung disease. In particular, the CF rat models recently …

Cystic fibrosis (CF) results from mutations that disrupt CF transmembrane conductance
regulator (CFTR), an anion channel found mainly in apical membranes of epithelial cells. CF …

Airway disease currently causes most of the morbidity and mortality in patients with cystic
fibrosis (CF). However, understanding the pathogenesis of CF lung disease and developing …

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 …