Article
The cystic fibrosis transmembrane conductance regulator: an intriguing protein with pleiotropic functions.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Mar 2002
Vankeerberghen Anne, Cuppens Harry, Cassiman Jean-Jacques
Abstract excerpt
Cystic fibrosis is a frequent autosomal recessive disorder that is caused by the malfunctioning of a small chloride channel, the cystic fibrosis transmembrane conductance regulator. The protein is found in the apical membrane of epithelial cells lining exocrine glands. Absence of this channel results in imbalance of ion concentrations across the cell membrane. As a result, fluids secreted through these glands...
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