Article
Aberrant CFTR-dependent HCO3- transport in mutations associated with cystic fibrosis.
Nature - 1 Mar 2001
Choi J Y, Muallem D, Kiselyov K, Lee M G, Thomas P J, Muallem S
Abstract excerpt
Cystic fibrosis (CF) is a disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). Initially, Cl- conductance in the sweat duct was discovered to be impaired in CF, a finding that has been extended to all CFTR-expressing cells. Subsequent cloning of the gene showed that CFTR functions as a cyclic-AMP-regulated Cl- channel; and some CF-causing mutations inhibit CFTR Cl-...
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