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Arch Pediatr. 2003 Sep;10 Suppl 2:325s-332s.

[CFTR and transepithelial ionic transport abnormalities in cystic fibrosis].

[Article in French]

Author information

  • LBSC, UMR 6558 CNRS, université de Poitiers, 40, avenue du Recteur-Pineau, 86022 Poitiers, France. frederic.becq@univ-poitiers.fr

Abstract

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.

PMID:
14671929
[PubMed - indexed for MEDLINE]
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