• 1
    Li Y, Togashi Y, Sato S, Emoto T, Rang JH, Takeichi N, Kobayshi H, et al. Spontaneous hepatic copper accumulation in Long-Evans Cinnamon rats with hereditary hepatitis. A model of Wilson's disease. J Clin Invest 1991; 87: 18581861.
  • 2
    Petrukhin K, Lutsenko S, Chernov I, Ross BM, Kaplan JH, Gilliam TC. Characterization of the Wilson disease gene encoding a P-type copper transporting ATPase: genomic organization, alternative splicing, and structure/function predictions. Hum Mol Genet 1994; 3: 16471656.
  • 3
    Wu J, Forbes JR, Chen HS, Cox DW. The LEG rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease gene. Nat Genet 1994; 7: 541545.
  • 4
    Sternlieb I, Quintana, N, Volenberg I, Schilsky ML. An array of mitochondrial alterations in the hepatocytes of Long-Evans Cinnamon rats. Hepatology 1995; 22: 17821787.
  • 5
    Sawaki M, Enomoto K, Hattori A, Tsuzuki N, Sugawara N, Mori M. Role of copper accumulation and metallothionein induction in spontaneous liver cancer development in LEG rats. Carcinogenesis 1994; 15: 18331837.
  • 6
    Sone K, Maeda M, Wakabayashi K, Takeichi N, Mori M, Sugimura T, Nagao. Inhibition of hereditary hepatitis and liver tumor development in Long-Evans Cinnamon rats by the copper-chelating agent trientine dihydrocholoride. Hepatology 1996; 23: 764770.
  • 7
    Schilsky ML, Scheinberg IH, Sternlieb I. Liver transplantation for Wilson's disease: indications and outcome. Hepatology 1994; 19: 583587.
  • 8
    Rakela J, Kurtz SB, McCarthy JT, Ludwig J, Ascher NL, Bloomer JR, Claus PL. Fulminant Wilson's disease treated with postdilution hemofiltration and orthotropic liver transplantation. Gastroenterology 1986; 90: 20042007.
  • 9
    Sarles J, Lefevre P, Picon P. Plasma exchange for fulminant Wilson disease. Eur J Pediatr 1992; 151: 310314.
  • 10
    O'Grady JG, Gimson AES, Obrien CJ, Pucknell A, Hughes RD, Williams R. Controlled trials of charcoal hemoperfusion and prognostic factors in fulminant hepatic failure. Gastroenterology 1988; 94: 11861192.
  • 11
    Stampfl DA, Muñoz SJ, Moritz MJ, Rubin R, Armenti VT, Jarrell BE, Maddrey WC. Heterotopic liver transplantation for fulminant Wilson's disease. Gastroenterology 1990; 99: 18341836.
  • 12
    Yoshida Y, Tokusashi Y, Lee G-H, Ogawa K. Intrahepatic transplantation of normal hepatocytes prevents Wilson's disease in Long-Evans Cinnamon rats. Gastroenterology 1996; 111: 16541660.
  • 13
    Stremmel W, Hofmann WJ, Otto G, Galle PR. Hepatic failure in Wilson's disease is accompanied by upregulated APO-I/FAS receptor levels [Abstract 26]. Z Gastroenterol 1995; 8: 479.
  • 14
    Galle PR, Strand S, Hofmann WJ. Grambihler A, Otto G, Krammer PH, Stremel W. The pathomechanism of hepatic failure in Wilson's disease–activation of the CD95 system [Abstract 494]. Hepatology 1996; 24: 250A.