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MeSH Review

Jaundice, Chronic Idiopathic

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Disease relevance of Jaundice, Chronic Idiopathic


High impact information on Jaundice, Chronic Idiopathic


Chemical compound and disease context of Jaundice, Chronic Idiopathic


Biological context of Jaundice, Chronic Idiopathic

  • The elucidation of the selective absence of an isoform of MRP and from the canalicular membrane domain in conjunction with the defined substrate specificity of the MRP and cMRP gene-encoded conjugate export pumps contributes to the molecular definition of the transport defect in Dubin-Johnson syndrome [16].

Anatomical context of Jaundice, Chronic Idiopathic


Gene context of Jaundice, Chronic Idiopathic

  • MRP1 is a ubiquitously expressed efflux pump for the products of phase II of xenobiotic detoxification, while MRP2, whose hereditary deficiency results in Dubin-Johnson syndrome, functions to extrude organic anions into the bile [18].
  • Moreover, Mrp2 (Abcc2) deficient animals display mild conjugated hyperbilirubinemia, corresponding to a human condition known as Dubin-Johnson syndrome (DJS) [19].
  • Mutations in the canilicular multispecific organic anion transporter (cMOAT) gene, a novel ABC transporter, in patients with hyperbilirubinemia II/Dubin-Johnson syndrome [20].
  • The absence of this canalicular Mrp in the mutants is analogous to the defect in the human Dubin-Johnson syndrome which is characterized by an impaired excretion of conjugated anions across the canalicular membrane [21].
  • MRP2 is not expressed in the human Dubin-Johnson syndrome, which is therefore associated with an inherited deficiency in the secretion of amphiphilic anionic conjugates into the bile [22].

Analytical, diagnostic and therapeutic context of Jaundice, Chronic Idiopathic


  1. Radixin deficiency causes conjugated hyperbilirubinemia with loss of Mrp2 from bile canalicular membranes. Kikuchi, S., Hata, M., Fukumoto, K., Yamane, Y., Matsui, T., Tamura, A., Yonemura, S., Yamagishi, H., Keppler, D., Tsukita, S., Tsukita, S. Nat. Genet. (2002) [Pubmed]
  2. A family of drug transporters: the multidrug resistance-associated proteins. Borst, P., Evers, R., Kool, M., Wijnholds, J. J. Natl. Cancer Inst. (2000) [Pubmed]
  3. The nicotinic acid test in constitutional conjugated hyperbilirubinemias and effects of corticosteroid. Ohkubo, H., Okuda, K. Hepatology (1984) [Pubmed]
  4. Hepatocellular carcinoma in a case of Dubin-Johnson syndrome treated successfully with a central bilateral segmentectomy. Shikada, Y., Matsumata, T., Suehiro, T., Sugimachi, K. Hepatogastroenterology (2004) [Pubmed]
  5. Neonatal Dubin-Johnson syndrome with severe cholestasis: effective phenobarbital therapy. Kimura, A., Ushijima, K., Kage, M., Mahara, R., Tohma, M., Inokuchi, T., Shibao, K., Tanaka, N., Fujisawa, T., Ono, E. Acta paediatrica Scandinavica. (1991) [Pubmed]
  6. Two distinct mechanisms for bilirubin glucuronide transport by rat bile canalicular membrane vesicles. Demonstration of defective ATP-dependent transport in rats (TR-) with inherited conjugated hyperbilirubinemia. Nishida, T., Gatmaitan, Z., Roy-Chowdhry, J., Arias, I.M. J. Clin. Invest. (1992) [Pubmed]
  7. Exon-intron organization of the human multidrug-resistance protein 2 (MRP2) gene mutated in Dubin-Johnson syndrome. Tsujii, H., König, J., Rost, D., Stöckel, B., Leuschner, U., Keppler, D. Gastroenterology (1999) [Pubmed]
  8. Ursodeoxycholic acid oral tolerance test in patients with constitutional hyperbilirubinemias and effect of phenobarbital. Ohkubo, H., Okuda, K., Iida, S., Makino, I. Gastroenterology (1981) [Pubmed]
  9. Bile acid excretion in Dubin-Johnson syndrome. Javitt, N.B., Kondo, T., Kuchiba, K. Gastroenterology (1978) [Pubmed]
  10. Defective ATP-dependent bile canalicular transport of organic anions in mutant (TR-) rats with conjugated hyperbilirubinemia. Kitamura, T., Jansen, P., Hardenbrook, C., Kamimoto, Y., Gatmaitan, Z., Arias, I.M. Proc. Natl. Acad. Sci. U.S.A. (1990) [Pubmed]
  11. Impaired protein maturation of the conjugate export pump multidrug resistance protein 2 as a consequence of a deletion mutation in Dubin-Johnson syndrome. Keitel, V., Kartenbeck, J., Nies, A.T., Spring, H., Brom, M., Keppler, D. Hepatology (2000) [Pubmed]
  12. Bile salt transport in the Dubin-Johnson syndrome. Douglas, J.G., Beckett, G.J., Percy-Robb, I.W., Finlayson, N.D. Gut (1980) [Pubmed]
  13. A new diagnostic approach to the Dubin-Johnson syndrome. Pinós, T., Constansa, J.M., Palacin, A., Figueras, C. Am. J. Gastroenterol. (1990) [Pubmed]
  14. A common Dubin-Johnson syndrome mutation impairs protein maturation and transport activity of MRP2 (ABCC2). Keitel, V., Nies, A.T., Brom, M., Hummel-Eisenbeiss, J., Spring, H., Keppler, D. Am. J. Physiol. Gastrointest. Liver Physiol. (2003) [Pubmed]
  15. The effect of phenobarbital on patients with Dubin-Johnson syndrome. Merdler, C., Burke, M., Shani, M., Felner, S., Lurie, I. Digestion (1976) [Pubmed]
  16. Absence of the canalicular isoform of the MRP gene-encoded conjugate export pump from the hepatocytes in Dubin-Johnson syndrome. Kartenbeck, J., Leuschner, U., Mayer, R., Keppler, D. Hepatology (1996) [Pubmed]
  17. Characterization of the human multidrug resistance protein isoform MRP3 localized to the basolateral hepatocyte membrane. König, J., Rost, D., Cui, Y., Keppler, D. Hepatology (1999) [Pubmed]
  18. MRP subfamily transporters and resistance to anticancer agents. Kruh, G.D., Zeng, H., Rea, P.A., Liu, G., Chen, Z.S., Lee, K., Belinsky, M.G. J. Bioenerg. Biomembr. (2001) [Pubmed]
  19. Polymorphisms of MRP1 (ABCC1) and related ATP-dependent drug transporters. Conseil, G., Deeley, R.G., Cole, S.P. Pharmacogenet. Genomics (2005) [Pubmed]
  20. Mutations in the canilicular multispecific organic anion transporter (cMOAT) gene, a novel ABC transporter, in patients with hyperbilirubinemia II/Dubin-Johnson syndrome. Wada, M., Toh, S., Taniguchi, K., Nakamura, T., Uchiumi, T., Kohno, K., Yoshida, I., Kimura, A., Sakisaka, S., Adachi, Y., Kuwano, M. Hum. Mol. Genet. (1998) [Pubmed]
  21. The function of the multidrug resistance proteins (MRP and cMRP) in drug conjugate transport and hepatobiliary excretion. Keppler, D., Leier, I., Jedlitschky, G., Mayer, R., Büchler, M. Adv. Enzyme Regul. (1996) [Pubmed]
  22. Hepatic canalicular membrane 5: Expression and localization of the conjugate export pump encoded by the MRP2 (cMRP/cMOAT) gene in liver. Keppler, D., Konig, J. FASEB J. (1997) [Pubmed]
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