Hepatocanalicular transport defects

PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Hepatocanalicular transport defects: pathophysiologic mechanisms of rare diseases” (Gastroenterology. 2006 Mar;130(3):908-25 ). Author are Elferink RP, Paulusma CC and Groen AK from the AMC Liver Center, Academic Medical Center, Amsterdam, The Netherlands. The apical membrane of the hepatocyte fulfils a unique function in the formation of primary bile. For all important biliary constituents a primary active transporter is present that extrudes or translocates its substrate toward the canalicular lumen. Two types of transporters can be recognized: those having endogenous metabolites as substrates (which could be referred to as “physiologic” transporters) and those involved in the elimination of drugs, toxins, and waste products. The importance of the canalicular transporters has been recognized by the pathologic consequence of their genetic defects. For each of the physiologic transporter genes an inherited disease has now been identified and most of these diseases have a quite serious clinical phenotype. To access the full abstract of the article, click here.