Overview on Dubin-Johnson Syndrome: A Benign Disorder

Another diagnostic bluster of DJS is that bilirubin diester conjugates are more numerous than monoester conjugates, differentiating DJS from other hepatobiliary disorders.

The molecular basis for Dubin-Johnson syndrome has been identified, and has been found to be an absence of a running(a) multidrug safeguard protein called, MRP2, with the symbol ABCC2, from the canalicular membrane of the hepatocytes (Keitel, et al G165). These researchers canvas a frequent DJS mutation which results in an exchange of two hydrophobic amino acids (isoleucine 1173 to phenylalanine) in a predicted extracellular iteration of MRP2. The protein was mainly core-glycosylated, and was retained mostly in the endoplasmic reticulum, where it was degraded by proteosomes. The mutant protein did not mediate ATP-dependent transport of leukotriene C4 into vesicles from plasma membranes and endoplasmic reticulum as the normal protein did. The mutant protein was locate to the apical membrane in only five percent of cells, whereas the normal protein localized to the apical membrane in 80 percent of cells. The researchers aim this may explain the deficient hepatobiliary elimination seen in DJS patients.

MRP2 is the major(ip) transporter for secreting bilirubin glucuronides into bile, and is also involved in the resistance to many drugs because it transports them out of cells (Borst, Evers, Kool and Wijnholds 1295).

Toh, Satoshi, Wada, Morimasa, Uchiuni, Takeshi, Inokuchi, Akihiko, Makimo, Yoshinari, Horie, Yutaka, Adachi, Yukihiko, Sakisaka, Shotaro, and Kuwano, Michihiko. " contractable structure of the canalicular multispecific organic anionic-transporter gene (MRP2/cMOAT) and mutations in the ATP-binding-cassette share in Dubin-Johnson syndrome." American diary of Human Genetics. 64(1999):739-746.

Keitel, Verena, Nies, Anne T., Brom, Manuela, and Hummel-Eisenbeiss, Johanna et al. "A plebeian Dubin-Johnson syndrome mutation impairs protein maturation and transport activity of MRP2 (ABCC2)." American Journal of Physiology: Gastroenterology and Live Physiology. 47(2003): G165.

Cai, Shi-Ying, Soroka, carol J., Ballatori, Nazzareno, and Boyer, James L. "Molecular characterization of a multidrug resistance-associated protein, Mrp2, from the little skate." The American Journal of Physiology. 28(2003:R125-R130.

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