Genetic defects in hepatocanalicular transport.
Genetic defects in hepatocanalicular transport.
复制标题
肝小管运输的遗传缺陷。
DOI：
10.1055/s-2000-9384
复制
发表时间：
2000
影响因子：
4.2
通讯作者：
P. Jansen
中科院分区：
文献类型：
--
作者：
R. Thompson;P. Jansen
胆汁是胆汁成分主动进入胆道的结果。这种转运大部分发生在小管膜上，还有来自胆管细胞的作用。水被动地进入胆汁。从肝细胞转运出来的主要底物是胆汁酸、磷脂、胆固醇和胆红素。除了胆固醇，这些主要的底物现在都被认为有自己的运输机制。在胆汁酸和磷脂的情况下，转运蛋白似乎是特定的，尽管胆红素转运蛋白是多特异性的。这三种转运蛋白中孤立的常染色体隐性缺陷现已被鉴定，并有助于确认这些蛋白的生理作用。此外，胆汁酸转运的继发性缺陷似乎是由于细胞膜氨基磷脂分布异常所致。
Bile is made as the result of active transport of its constituents into the biliary space. Most of this transport occurs across the canalicular membrane, with a further contribution from cholangiocytes. Water moves passively into bile. The major substrates that are transported out of hepatocytes are bile acids, phospholipids, cholesterol, and bilirubin. With the exception of cholesterol, each of these major substrates is now recognized to have its own transport mechanism. In the case of bile acids and phospholipids, the transporters appear to be specific, though the bilirubin transporter is multispecific. Isolated autosomal recessive defects in all three of these transporters have now been identified and have helped to confirm the physiologic role of these proteins. In addition, a secondary defect in bile acid transport has been identified that appears to be due to an abnormality in plasma membrane aminophospholipid distribution.