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Hepatic failure and liver cell damage in acute Wilson's disease involve CD95 (APO-1 /Fas) Mediated apoptosis

Nature Medicine volume 4, pages 588–593 (1998)Cite this article

Abstract

Wilson's disease can result in fulminant liver failure due to hepatic copper overload. The CD95 system mediates apoptosis and has been demonstrated to be involved in liver disease. In this study CD95 mediated apoptosis was investigated in patients with fulminant hepatic failure in the course of Wilson's disease and in an in vitro model of copper treated human hepatoma cells. In patients, hepatic expression of CD95 and CD95L mRNA and apoptosis were detected. Copper overload in vitro resulted in hepatocytic apoptosis which could be reduced with a neutralizing anti-CD95L antibody. Copper treatment of hepatocytes results in activation of the CD9S system and induction of apoptosis which is operative during the course of hepatic failure in acute Wilson's disease.

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Author notes

  1. Peter R. Galle: Correspondence should be addressed to P.R.G.

Authors and Affiliations

  1. University Hospital, Deptartment of Gastroenterology, Bergheimerstr. 58, 69115, Heidelberg, FRG

    Susanne Strand, Annette Grambihler, Martin Volkmann, Wolfgang Stremmel & Peter R. Galle

  2. Institute of Pathology, Im Neuenheimer Feld 220, 69120, Heidelberg, FRG

    Walter J. Hofmann

  3. Center of Molecular Biology (ZMBH), Im Neuenheimer Feld 282, 69120, Heidelberg, FRG

    Hubert Hug

  4. Deptpartment of Surgery, Im Neuenheimer Feld 110, 69120, Heidelberg, FRG

    Gerd Otto

  5. German Cancer Research Center, Tumorimmunology Program (SMM, PHK), Radiology Program (HW), Division of lmmunochemistry (VH), Im Neuenheimer Feld 280, 69120, Heidelberg, FRG

    Horst Wesch, Sara M. Mariani, Volker Hack & Peter H. Krammer

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  1. Susanne Strand
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Strand, S., Hofmann, W., Grambihler, A. et al. Hepatic failure and liver cell damage in acute Wilson's disease involve CD95 (APO-1 /Fas) Mediated apoptosis. Nat Med 4, 588–593 (1998). https://doi.org/10.1038/nm0598-588

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