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

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...

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Main Authors: Strand, Susanne (Author) , Hofmann, Walter J. (Author) , Grambihler, Annette (Author) , Hug, Hubert (Author) , Volkmann, Martin (Author) , Otto, Gerd (Author) , Wesch, Horst (Author) , Mariani, Sara M. (Author) , Hack, Volker (Author) , Stremmel, Wolfgang (Author) , Krammer, Peter H. (Author) , Galle, Peter R. (Author)
Format: Article (Journal)
Language:English
Published: 01 May 1998
In: Nature medicine
Year: 1998, Volume: 4, Issue: 5, Pages: 588-593
ISSN:1546-170X
DOI:10.1038/nm0598-588
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1038/nm0598-588
Verlag, lizenzpflichtig, Volltext: https://www.nature.com/articles/nm0598-588
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Author Notes:Susanne Strand, Walter J. Hofmann, Annette Grambihler, Hubert Hug, Martin Volkmann, Gerd Otto, Horst Wesch, Sara M. Mariani, Volker Hack, Wolfgang Stremmel, Peter H. Krammer, Peter R. Galle
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Summary: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.
Item Description:Gesehen am 10.03.2025
Physical Description:Online Resource
ISSN:1546-170X
DOI:10.1038/nm0598-588