[CFTR and transepithelial ionic transport abnormalities in cystic fibrosis].
The genetic disease cystic fibrosis (CF) is caused by mutations of the CF gene and generates defective Cl- transport across the affected epithelium. Recent progress have been made to understand CFTR activity and regulation in epithelia and its role in the muco-ciliary clearance of airway. This revue-overviews the mechanisms of transepithelial ion transport, the role of CFTR in that process and the consequences for CF of CFTR mutations.
Becq
F
F
LBSC, UMR 6558 CNRS, université de Poitiers, 40, avenue du Recteur-Pineau, 86022 Poitiers, France. frederic.becq@univ-poitiers.fr
D003550:Cystic Fibrosis / Q000235:genetics* / Q000503:physiopathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics* / Q000502:physiology*; D006801:Humans; D017136:Ion Transport; D009079:Mucociliary Clearance / Q000502:physiology*; D009154:Mutation
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator