Article
Hepatobiliary complications of cystic fibrosis.
Current gastroenterology reports - 1 Jun 2004
Feranchak Andrew P
Abstract excerpt
Cystic fibrosis (CF) is the most common potentially lethal genetic disease in the Caucasian population. The disease results from mutations in the gene for the cystic fibrosis transmembrane conductance regulator (CFTR), a cAMP-activated Cl(-) channel in the apical membrane of most secretory epithelia. In the liver, CFTR is located in biliary epithelial cells or cholangiocytes and gallbladder epithelia, where it...
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