Article
Correction of CFTR malfunction and stimulation of Ca-activated Cl channels restore HCO3- secretion in cystic fibrosis bile ductular cells.
Hepatology (Baltimore, Md.) - 1 Jan 2002
Zsembery Akos, Jessner Wolfgang, Sitter Gerlinde, Spirlí Carlo, Strazzabosco Mario, Graf Jürg
Abstract excerpt
In view of the occurrence of hepatobiliary disorders in cystic fibrosis (CF) this study addresses the role of the cystic fibrosis transmembrane conductance regulator (CFTR) and of Ca(2+)-activated Cl(-) channels in promoting HCO3- secretion in bile ductular cells. Human cholangiocytes were isolated from control livers and from 1 patient with CF (DeltaF508/G542X mutations). Single channel and whole cell currents...
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