Impaired protein maturation of the conjugate export pump multidrug resistance protein 2 as a consequence of a deletion mutation in Dubin–Johnson syndrome
V Keitel, J Kartenbeck, AT Nies, H Spring, M Brom… - Hepatology, 2000 - journals.lww.com
Abstract The Dubin–Johnson syndrome is an inherited disorder characterized by conjugated
hyperbilirubinemia. The deficient hepatobiliary transport of anionic conjugates is caused by
the absence of a functional multidrug–resistance protein 2 (MRP2, symbol ABCC2) from the
apical (canalicular) membrane of hepatocytes. Mechanisms underlying this deficiency may
include rapid degradation of mutated MRP2 messenger RNA (mRNA) or impaired MRP2
protein maturation and trafficking. We investigated the consequences of the mutation …
hyperbilirubinemia. The deficient hepatobiliary transport of anionic conjugates is caused by
the absence of a functional multidrug–resistance protein 2 (MRP2, symbol ABCC2) from the
apical (canalicular) membrane of hepatocytes. Mechanisms underlying this deficiency may
include rapid degradation of mutated MRP2 messenger RNA (mRNA) or impaired MRP2
protein maturation and trafficking. We investigated the consequences of the mutation …
