Hepatic transport systems.
The identification of the genes responsible for various genetic liver disorders lead to a better understanding of basic physiology of hepatic transport systems. In this review we focus on transport systems involved in the generation of bile and in the maintenance of copper homeostasis. Abnormal function of these transporters results in diseases like Wilson's disease, progressive familial cholestasis syndromes, Dubin-Johnson syndrome and cystic fibrosis. Beyond these well defined diseases, functional impairments of transport proteins may predispose to non-genetic diseases ranging from intrahepatic cholestasis of pregnancy to neurodegenerative disorders including Alzheimer's disease.
Ferenci
Peter
P
Department of Internal Medicine IV, Gastroenterology and Hepatology, University of Vienna, Austria. peter.ferenci@akh-wien.ac.at
Zollner
Gernot
G
Trauner
Michael
M
D000251:Adenosine Triphosphatases / Q000378:metabolism; D001646:Bile / Q000378:metabolism; D001692:Biological Transport; D027682:Cation Transport Proteins / Q000378:metabolism; D002780:Cholestasis, Intrahepatic / Q000235:genetics / Q000378:metabolism; D003300:Copper / Q000378:metabolism; D000073840:Copper-Transporting ATPases; D003550:Cystic Fibrosis / Q000235:genetics; D006801:Humans; D008099:Liver / Q000378:metabolism*
D027682:Cation Transport Proteins; D003300:Copper; D000251:Adenosine Triphosphatases; D000073840:Copper-Transporting ATPases