Cystic Fibrosis (CF) is the most common, severe, autosomal recessive disease in the
Caucasian population, and is caused by mutations in the cystic fibrosis transmembrane …

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause a
characteristic defect in epithelial ion transport that plays a central role in the pathogenesis of …

Introduction Cystic fibrosis (CF), a potentially fatal genetic disease, is caused by loss-of-
function mutations in the gene encoding for the CFTR chloride/bicarbonate channel …

The concept that increasing airway hydration leads to improvements in mucus clearance
and lung function in cystic fibrosis has been clinically validated with osmotic agents such as …

Lung health relies on effective mucociliary clearance and innate immune defence
mechanisms. In cystic fibrosis (CF), an imbalance in ion transport due to an absence of …

Cystic Fibrosis (CF) is a life-shortening genetic disorder caused by mutations in the CF
Transmembrane Conductance Regulator (CFTR) gene, which encodes a cAMP-regulated …

Cystic fibrosis (CF) is a life-shortening monogenic disease caused by mutations in the gene
encoding the CF transmembrane conductance regulator (CFTR) protein, an anion channel …

With knowledge of the molecular behaviour of the cystic fibrosis transmembrane
conductance regulator (CFTR), its physiological role and dysfunction in cystic fibrosis (CF) …

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause
the life-limiting hereditary disease, cystic fibrosis (CF). Decreased or absent functional CFTR …