Literature DB >> 9585233

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

S Strand1, W J Hofmann, A Grambihler, H Hug, M Volkmann, G Otto, H Wesch, S M Mariani, V Hack, W Stremmel, P H Krammer, P R Galle.   

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 CD95 system and induction of apoptosis which is operative during the course of hepatic failure in acute Wilson's disease.

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Year:  1998        PMID: 9585233     DOI: 10.1038/nm0598-588

Source DB:  PubMed          Journal:  Nat Med        ISSN: 1078-8956            Impact factor:   53.440


  44 in total

1.  Overexpression of M68/DcR3 in human gastrointestinal tract tumors independent of gene amplification and its location in a four-gene cluster.

Authors:  C Bai; B Connolly; M L Metzker; C A Hilliard; X Liu; V Sandig; A Soderman; S M Galloway; Q Liu; C P Austin; C T Caskey
Journal:  Proc Natl Acad Sci U S A       Date:  2000-02-01       Impact factor: 11.205

Review 2.  Apoptosis: a mechanism of acute and chronic liver injury.

Authors:  M E Guicciardi; G J Gores
Journal:  Gut       Date:  2005-07       Impact factor: 23.059

3.  Bone demineralisation in a large cohort of Wilson disease patients.

Authors:  Karl Heinz Weiss; Mart Van de Moortele; Daniel Nils Gotthardt; Jan Pfeiffenberger; Jessica Seessle; Elena Ullrich; Evelien Gielen; Herman Borghs; Els Adriaens; Wolfgang Stremmel; Wouter Meersseman; Steven Boonen; David Cassiman
Journal:  J Inherit Metab Dis       Date:  2015-02-07       Impact factor: 4.982

4.  Suppression of Akt signaling induces Fas ligand expression: involvement of caspase and Jun kinase activation in Akt-mediated Fas ligand regulation.

Authors:  Toshimitsu Suhara; Hyo-Soo Kim; Lorrie A Kirshenbaum; Kenneth Walsh
Journal:  Mol Cell Biol       Date:  2002-01       Impact factor: 4.272

Review 5.  Clinical presentations of Wilson disease.

Authors:  Samuel Shribman; Thomas T Warner; James S Dooley
Journal:  Ann Transl Med       Date:  2019-04

Review 6.  Clinical features of Wilson disease.

Authors:  Wolfgang Stremmel; Uta Merle; Ralf Weiskirchen
Journal:  Ann Transl Med       Date:  2019-04

7.  Inhibition of drug-induced Fas ligand transcription and apoptosis by Bcl-XL.

Authors:  R S Biswas; H J Cha; J M Hardwick; R K Srivastava
Journal:  Mol Cell Biochem       Date:  2001-09       Impact factor: 3.396

Review 8.  Insights into the management of Wilson's disease.

Authors:  Mohmadshakil Kathawala; Gideon M Hirschfield
Journal:  Therap Adv Gastroenterol       Date:  2017-10-03       Impact factor: 4.409

9.  Copper Induces Apoptosis of Neuroblastoma Cells Via Post-translational Regulation of the Expression of Bcl-2-family Proteins and the tx Mouse is a Better Model of Hepatic than Brain Cu Toxicity.

Authors:  Hsien W Chan; Tianbing Liu; Giuseppe Verdile; Glenda Bishop; Ryan J Haasl; Mark A Smith; George Perry; Ralph N Martins; Craig S Atwood
Journal:  Int J Clin Exp Med       Date:  2008-01-20

10.  Development of an accurate index for predicting outcomes of patients with acute liver failure.

Authors:  Anna Rutherford; Lindsay Y King; Linda S Hynan; Chetan Vedvyas; Wenyu Lin; William M Lee; Raymond T Chung
Journal:  Gastroenterology       Date:  2012-08-08       Impact factor: 22.682

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