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Pyruvate dehydrogenase complex as an autoantigen in primary biliary cirrhosis

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Alpha-Keto Acid Dehydrogenase Complexes

Part of the book series: MCBU Molecular and Cell Biology Updates ((MCBU))

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Abstract

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease which is characterised by progressive inflammatory obliteration of the intrahepatic bile ducts, leading to fibrosis, liver cell damage and ultimately liver failure (Sherlock and Dooley, 1993). A variety of experimental evidence has indicated an autoimmune basis to this disease. Over 30 years ago it was recognised by Walker et al. (1965) that PBC-specific autoantibodies are present in the sera of patients and that these autoantibodies can be detected by indirect immunofluorescence. Subsequently the autoantigens involved were shown to be associated with the inner mitochondrial membrane (Berg et al., 1967). A variety of other conditions are also associated with the presence of autoantibodies to mitochondrial antigens but a subset of these antigens, termed M2, have been defined as specific to PBC (Berg et al., 1982). Furthermore a variety of other autoantibodies have been reported to be associated with PBC but they are not as disease–specific or are found in a lower proportion of patients (Berg and Klein, 1989).

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Authors and Affiliations

  1. Departments of Biochemistry and Genetics, The Medical School, University of Newcastle upon Tyne, Newcastle upon Tyne, NE2 4HH, UK

    S. J. Yeaman

  2. Departments of Immunology, The Medical School, University of Newcastle upon Tyne, Newcastle upon Tyne, NE2 4HH, UK

    A. G. Diamond

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  1. S. J. Yeaman
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Editors and Affiliations

  1. Department of Biochemistry School of Medicine and Biomedical Sciences, State University of New York, 140 Farber Hall 3435 Main Street, 14214, Buffalo, New York, USA

    Mulchand S. Patel

  2. Department of Biochemistry, Kansas State University, 104 Willard Hall, 66506, Manhattan, KS, USA

    Thomas E. Roche

  3. Department of Biochemistry and Molecular Biology, Indiana University School of Medicine, 635 Barnhill Drive, 46202, Indianapolis, IN, USA

    Robert A. Harris

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© 1996 Birkhäuser Verlag Basel/Switzerland

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Yeaman, S.J., Diamond, A.G. (1996). Pyruvate dehydrogenase complex as an autoantigen in primary biliary cirrhosis. In: Patel, M.S., Roche, T.E., Harris, R.A. (eds) Alpha-Keto Acid Dehydrogenase Complexes. MCBU Molecular and Cell Biology Updates. Birkhäuser Basel. https://doi.org/10.1007/978-3-0348-8981-0_21

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  • DOI: https://doi.org/10.1007/978-3-0348-8981-0_21

  • Publisher Name: Birkhäuser Basel

  • Print ISBN: 978-3-0348-9853-9

  • Online ISBN: 978-3-0348-8981-0

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