Journal of Clinical Immunology

, Volume 11, Issue 5, pp 239–245

Anti-pyruvate dehydrogenase autoantibodies in primary biliary cirrhosis

  • N. Zurgil
  • R. Bakimer
  • M. Kaplan
  • P. Youinou
  • Y. Shoenfeld
Original Articles

DOI: 10.1007/BF00918181

Cite this article as:
Zurgil, N., Bakimer, R., Kaplan, M. et al. J Clin Immunol (1991) 11: 239. doi:10.1007/BF00918181

Abstract

Antimitochondrial antibodies (AMA) may be detected in 95% of patients with primary biliary cirrhosis (PBC). The target autoantigens for the AMA were recently identified as four closely related metabolic enzymes located in the mitochondria. We have purified the pyruvate dehydrogenase (PDH) enzyme from bovine heart, showing that all PBC sera reacted with a 74-kd band. PDH was utilized to establish an ELISA assay for detecting the relevant antibodies. One hundred twelve of 120 sera from patients with PBC (95%) reacted with the PDH but none of the 201 control sera, including normal subjects and a panel of sera from other patients with liver diseases, showed similar reactivity. In 77% of the PBC sera the anti-PDH antibody isotype was identified as a combination of IgG and IgM, while in 18% only IgM was detected. In 5% of the sera the isotype was confined to IgG. PBC sera specifically inhibited the PDH enzyme activity. The enzyme inhibition correlated with the anti-PDH antibody titers. Thus, PDH seems to be one of the major target epitopes for AMA observed in sera of patients with PBC.

Key words

Antimitochondrial antibodies pyruvate dehydrogenase primary biliary cirrhosis autoimmunity autoantibodies 

Copyright information

© Plenum Publishing Corporation 1991

Authors and Affiliations

  • N. Zurgil
    • 1
    • 2
  • R. Bakimer
    • 1
  • M. Kaplan
    • 3
  • P. Youinou
    • 4
  • Y. Shoenfeld
    • 1
  1. 1.Research Unit of Autoimmune Diseases, Department of Medicine “B,”Sheba Medical CenterTel-HashomeIsrael
  2. 2.BioHytech (Israel) LtdRamat-GanIsrael
  3. 3.Department of Medicine, Gastroenterology DivisionTufts University School of Medicine and New England Medical Center HospitalBoston
  4. 4.Laboratoire D'Immunologic CentreHospitalier Regional et Universitaire de BrestBrestFrance

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