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MeSH Review

Hepatolenticular Degeneration

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Disease relevance of Hepatolenticular Degeneration


Psychiatry related information on Hepatolenticular Degeneration


High impact information on Hepatolenticular Degeneration


Chemical compound and disease context of Hepatolenticular Degeneration


Biological context of Hepatolenticular Degeneration


Anatomical context of Hepatolenticular Degeneration


Gene context of Hepatolenticular Degeneration


Analytical, diagnostic and therapeutic context of Hepatolenticular Degeneration


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  2. Hepatic failure and liver cell damage in acute Wilson's disease involve CD95 (APO-1/Fas) mediated apoptosis. Strand, S., Hofmann, W.J., Grambihler, A., Hug, H., Volkmann, M., Otto, G., Wesch, H., Mariani, S.M., Hack, V., Stremmel, W., Krammer, P.H., Galle, P.R. Nat. Med. (1998) [Pubmed]
  3. Acute lymphoblastic leukemia in a patient receiving penicillamine for Wilson's disease. Gilman, P.A., Holtzman, N.A. JAMA (1982) [Pubmed]
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  8. Anatomy and pathology of the basal ganglia. McGeer, P.L., McGeer, E.G., Itagaki, S., Mizukawa, K. The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques. (1987) [Pubmed]
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  12. The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene. Bull, P.C., Thomas, G.R., Rommens, J.M., Forbes, J.R., Cox, D.W. Nat. Genet. (1993) [Pubmed]
  13. The efficacy of oral zinc therapy as an alternative to penicillamine for Wilson's disease. Cossack, Z.T. N. Engl. J. Med. (1988) [Pubmed]
  14. The use of trientine in preventing the effects of interrupting penicillamine therapy in Wilson's disease. Scheinberg, I.H., Jaffe, M.E., Sternlieb, I. N. Engl. J. Med. (1987) [Pubmed]
  15. D-penicillamine-induced IgA deficiency in Wilson's disease. Proesmans, W., Jaeken, J., Eeckels, R. Lancet (1976) [Pubmed]
  16. Treatment of Wilson's disease with trientine (triethylene tetramine) dihydrochloride. Walshe, J.M. Lancet (1982) [Pubmed]
  17. Use of apomorphine to test for dopamine responsiveness in Wilson's disease. Frankel, J.P., Hughes, A., Lees, A.J., Stern, G.M., Walshe, J.M. Lancet (1989) [Pubmed]
  18. Clinical differentiation of fulminant Wilsonian hepatitis from other causes of hepatic failure. Berman, D.H., Leventhal, R.I., Gavaler, J.S., Cadoff, E.M., Van Thiel, D.H. Gastroenterology (1991) [Pubmed]
  19. Liver cell death and anemia in Wilson disease involve acid sphingomyelinase and ceramide. Lang, P.A., Schenck, M., Nicolay, J.P., Becker, J.U., Kempe, D.S., Lupescu, A., Koka, S., Eisele, K., Klarl, B.A., Rübben, H., Schmid, K.W., Mann, K., Hildenbrand, S., Hefter, H., Huber, S.M., Wieder, T., Erhardt, A., Häussinger, D., Gulbins, E., Lang, F. Nat. Med. (2007) [Pubmed]
  20. A 4.5-megabase yeast artificial chromosome contig from human chromosome 13q14.3 ordering 9 polymorphic microsatellites (22 sequence-tagged sites) tightly linked to the Wilson disease locus. White, A., Tomfohrde, J., Stewart, E., Barnes, R., Le Paslier, D., Weissenbach, J., Cavalli-Sforza, L., Farrer, L., Bowcock, A. Proc. Natl. Acad. Sci. U.S.A. (1993) [Pubmed]
  21. Functional characterization of missense mutations in ATP7B: Wilson disease mutation or normal variant? Forbes, J.R., Cox, D.W. Am. J. Hum. Genet. (1998) [Pubmed]
  22. Increased p53 mutation load in nontumorous human liver of wilson disease and hemochromatosis: oxyradical overload diseases. Hussain, S.P., Raja, K., Amstad, P.A., Sawyer, M., Trudel, L.J., Wogan, G.N., Hofseth, L.J., Shields, P.G., Billiar, T.R., Trautwein, C., Hohler, T., Galle, P.R., Phillips, D.H., Markin, R., Marrogi, A.J., Harris, C.C. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  23. The impact of apolipoprotein E genotypes on age at onset of symptoms and phenotypic expression in Wilson's disease. Schiefermeier, M., Kollegger, H., Madl, C., Polli, C., Oder, W., Kühn, H., Berr, F., Ferenci, P. Brain (2000) [Pubmed]
  24. Copper specifically regulates intracellular phosphorylation of the Wilson's disease protein, a human copper-transporting ATPase. Vanderwerf, S.M., Cooper, M.J., Stetsenko, I.V., Lutsenko, S. J. Biol. Chem. (2001) [Pubmed]
  25. A mutation of the Wilson disease protein, ATP7B, is degraded in the proteasomes and forms protein aggregates. Harada, M., Sakisaka, S., Terada, K., Kimura, R., Kawaguchi, T., Koga, H., Kim, M., Taniguchi, E., Hanada, S., Suganuma, T., Furuta, K., Sugiyama, T., Sata, M. Gastroenterology (2001) [Pubmed]
  26. A sialic acid-specific O-acetylesterase in human erythrocytes: possible identity with esterase D, the genetic marker of retinoblastomas and Wilson disease. Varki, A., Muchmore, E., Diaz, S. Proc. Natl. Acad. Sci. U.S.A. (1986) [Pubmed]
  27. Incidence of parenchymal liver diseases in Denmark, 1981 to 1985: analysis of hospitalization registry data. The Danish Association for the Study of the Liver. Almdal, T.P., Sørensen, T.I. Hepatology (1991) [Pubmed]
  28. Metal-induced hepatotoxicity. Britton, R.S. Semin. Liver Dis. (1996) [Pubmed]
  29. A homozygous nonsense mutation and a combination of two mutations of the Wilson disease gene in patients with different lysyl oxidase activities in cultured fibroblasts. Kemppainen, R., Palatsi, R., Kallioinen, M., Oikarinen, A. J. Invest. Dermatol. (1997) [Pubmed]
  30. Copper transporting P-type ATPases and human disease. Cox, D.W., Moore, S.D. J. Bioenerg. Biomembr. (2002) [Pubmed]
  31. Caenorhabditis elegans cDNA for a Menkes/Wilson disease gene homologue and its function in a yeast CCC2 gene deletion mutant. Sambongi, Y., Wakabayashi, T., Yoshimizu, T., Omote, H., Oka, T., Futai, M. J. Biochem. (1997) [Pubmed]
  32. Cloning, mapping and expression analysis of the sheep Wilson disease gene homologue. Lockhart, P.J., Wilcox, S.A., Dahl, H.M., Mercer, J.F. Biochim. Biophys. Acta (2000) [Pubmed]
  33. Metal ion chaperone function of the soluble Cu(I) receptor Atx1. Pufahl, R.A., Singer, C.P., Peariso, K.L., Lin, S.J., Schmidt, P.J., Fahrni, C.J., Culotta, V.C., Penner-Hahn, J.E., O'Halloran, T.V. Science (1997) [Pubmed]
  34. Involvement of chloride channels in hepatic copper metabolism: ClC-4 promotes copper incorporation into ceruloplasmin. Wang, T., Weinman, S.A. Gastroenterology (2004) [Pubmed]
  35. Effects of anticopper therapy on hepatocellular mitochondria in patients with Wilson's disease: an ultrastructural and stereological study. Sternlieb, I., Feldmann, G. Gastroenterology (1976) [Pubmed]
  36. Cofactors of mitochondrial enzymes attenuate copper-induced death in vitro and in vivo. Sheline, C.T., Choi, E.H., Kim-Han, J.S., Dugan, L.L., Choi, D.W. Ann. Neurol. (2002) [Pubmed]
  37. Decrease of D2 receptors indicated by 123I-iodobenzamide single-photon emission computed tomography relates to neurological deficit in treated Wilson's disease. Oertel, W.H., Tatsch, K., Schwarz, J., Kraft, E., Trenkwalder, C., Scherer, J., Weinzierl, M., Vogl, T., Kirsch, C.M. Ann. Neurol. (1992) [Pubmed]
  38. Wilson disease: genetic basis of copper toxicity and natural history. Schilsky, M.L. Semin. Liver Dis. (1996) [Pubmed]
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