Crigler-Najjar syndrome type I (CN-I) is caused by an inherited absence of UDP-glucuronosyltransferase
activity toward bilirubin (B-UGT), resulting in severe non-hemolytic …

The canalicular (apical) membrane of the hepatocyte contains an ATP-dependent transport
system for organic anions, known as the multispecific organic anion transporter (cMOAT). …

Disruption of the mdr2 gene in mice leads to a complete absence of phospholipid from bile (Smit,
JJM, et al. 1993. Cell. 75:451-462). We have investigated the control of both mdr2 P-…

The human multidrug resistance-associated protein MRP confers resistance to various
cytotoxic drugs by lowering the intracellular drug concentration. Recent evidence indicates that …

Hepatocellular secretion of bile salts into the biliary space induces phospholipid and
cholesterol secretion, but the mechanism for integrated lipid secretion is poorly understood. …

Crigler-Najjar (CN) disease is classified into two subtypes, type I and II. The molecular basis
for the difference between these types is not well understood. Several mutations in the …

Mice homozygous for a disruption in the Mdr2 gene (Mdr2 (−/−) mice) lack the Mdr2 P-glycoprotein
(P-gp) in the canalicular membrane of the hepatocyte and are unable to excrete …

We have studied regulation of the multidrug resistance protein 2 (mrp2) during bile duct
ligation (BDL) in the rat. In hepatocytes isolated after 16, 48, and 72 hours of BDL, mrp2-…

TR- mutant rats have an autosomal recessive mutation that is expressed as a severely
impaired hepatobiliary secretion of organic anions like bilirubin-(di)glucuronide and …

Mice in which the gene for mdr2 P-glycoprotein has been disrupted have a severe deficiency
in biliary phospholipid and cholesterol secretion. We studied the relation between mdr2 …