Rationale: Identification of the specific cell types expressing CFTR (cystic fibrosis [CF]
transmembrane conductance regulator) is required for precision medicine therapies for 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 …

Pilewski, Joseph M., and Raymond A. Frizzell. Role of CFTR in Airway Disease. Physiol.
Rev. 79, Suppl.: S215–S255, 1999.—Cystic fibrosis (CF) is caused by mutations in the gene …

Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis
transmembrane conductance regulator (CFTR) anion channel. Airway disease is the major …

Pulmonary disease is the major cause of morbidity and mortality in patients with cystic
fibrosis, a disease caused by mutations in the Cystic Fibrosis Transmembrane conductance …

Dysfunction of CFTR in cystic fibrosis (CF) airway epithelium perturbs the normal regulation
of ion transport, leading to a reduced volume of airway surface liquid (ASL), mucus …

Gene transfer of CFTR cDNA to airway epithelia is a promising approach to treat cystic
fibrosis (CF). Most gene transfer vectors use strong viral promoters even though the …

CFTR is a highly regulated apical chloride channel of epithelial cells that is mutated in cystic
fibrosis (CF). In this study, we characterized the apical stability and intracellular trafficking of …

Background Cigarette smoke and smoking-induced inflammation decrease cystic fibrosis
transmembrane conductance regulator (CFTR) activity and mucociliary transport in the nasal …

Cystic fibrosis (CF) has become a paradigm disorder for the clinical testing of gene therapies
in the treatment of inherited disease. In recent years, efforts directed at gene therapy of CF …