Jansen P L
Semin Liver Dis. 2000;20(3):245-50.
Uptake of drugs and metabolites from the circulation into the liver is facilitated by transporter proteins in the basolateral membrane of the hepatocyte. Among these proteins are the sodium taurocholate cotransporting protein, various multispecific transporters for organic anions and cations, transporters for glucose, amino acids, and prostaglandins. The canalicular membrane contains a number of ATP-dependent transporters belonging to the families of P-glycoproteins and multidrug resistance-associated proteins. Transport across the canalicular membrane represents the rate-determining step in the secretion of compounds from blood to bile. Mutations of genes encoding these canalicular transporters are associated with liver diseases such as progressive familial intrahepatic cholestasis and Dubin-Johnson syndrome. Wilson's disease appears to be due to a defect of a copper-transporting P-type ATPase. Also, bile ductuli contribute to bile formation. Mutations in the CFTR gene, encoding a chloride channel in bile duct epithelial cells, leads to the hepatic component of cystic fibrosis.
肝细胞基底外侧膜上的转运蛋白有助于将循环中的药物和代谢产物摄取到肝脏中。这些蛋白质包括牛磺胆酸钠共转运蛋白、多种有机阴离子和阳离子的多特异性转运蛋白、葡萄糖、氨基酸和前列腺素的转运蛋白。胆小管膜含有一些属于P-糖蛋白家族和多药耐药相关蛋白家族的ATP依赖性转运蛋白。跨胆小管膜的转运是化合物从血液分泌到胆汁中的限速步骤。编码这些胆小管转运蛋白的基因突变与进行性家族性肝内胆汁淤积症和杜宾-约翰逊综合征等肝脏疾病有关。威尔逊病似乎是由于一种铜转运P型ATP酶的缺陷所致。此外，胆小管也参与胆汁形成。编码胆管上皮细胞中氯离子通道的CFTR基因突变会导致囊性纤维化的肝脏病变。
