Bile acid transport.
Bile acids undergo a unique enterohepatic circulation, which allows them to be efficiently reused with minimal loss. With the cloning of key bile acid transporter genes in the liver and intestine, clinicians now have a detailed understanding of how the different components in the enterohepatic circulation operate. These advances in basic knowledge of this process have directly led to a rapid and highly detailed understanding of rare genetic disorders of bile acid transport, which usually present as pediatric cholestatic disorders. Mutations in specific bile acid or lipid transporters have been identified within specific cholestatic disorders, which allows for genetic tests to be established for specific diseases and provides a unique opportunity to understand how these genes operate together. These same transporters may also prove useful for development of novel drug delivery systems, which can either enhance intestinal absorption of drugs or be used to target delivery to the liver or biliary system. Knowledge gained from these transporters will provide new therapeutic modalities to treat cholestatic disorders caused by common diseases.
Bahar
R J
RJ
Department of Pediatrics, University of California-Los Angeles School of Medicine, USA.
Stolz
A
A
D000818:Animals; D001647:Bile Acids and Salts / Q000378:metabolism*; D001692:Biological Transport; D002352:Carrier Proteins / Q000235:genetics / Q000378:metabolism; D002780:Cholestasis, Intrahepatic / Q000235:genetics / Q000378:metabolism; D006801:Humans; D006913:Hydroxysteroid Dehydrogenases; D007413:Intestinal Mucosa / Q000378:metabolism; D008099:Liver / Q000378:metabolism; D008562:Membrane Glycoproteins; D012964:Sodium / Q000378:metabolism; D045002:3-alpha-Hydroxysteroid Dehydrogenase (B-Specific)
D001647:Bile Acids and Salts; D002352:Carrier Proteins; D006913:Hydroxysteroid Dehydrogenases; D008562:Membrane Glycoproteins; D012964:Sodium; C037643:bile acid binding proteins; C435496:AKR1C2 protein, human; D045002:3-alpha-Hydroxysteroid Dehydrogenase (B-Specific)