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Semin Liver Dis 1997; 17(2): 97-103
DOI: 10.1055/s-2007-1007187
ORIGINAL ARTICLE

© 1997 by Thieme Medical Publishers, Inc.

Ductular Expression of Autoantigens in Primary Biliary Cirrhosis

Ruth Joplin1 , M. Eric Gershwin2
  • 1Liver Research Laboratories, University Hospital, Birmingham, England
  • 2Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, Davis, California
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Publication History

Publication Date:
17 March 2008 (online)

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ABSTRACT

The role of biliary epithelial cell (BEC) antigens in immune recognition and damage of biliary epithelium in primary biliary cirrhosis (PBC) is unknown. The major autoantigen in PBC (the mitochondrial enzyme pyru-vate dehydrogenase dihydmlipoamide acetyItransferase [PDC-E2]) is abnormally distributed in the biliary epithelium of patients with PBC relative to controls. The antigen is not only present in mitochondria but also associated with the BEC plasma membrane. This atypical distribution ofPDC-E2 is present both in early (stages I-II) and advanced (stages III-IV) disease, suggesting a role for the antigen in progression and/or etiology of PBC. The identity of the plasma membrane antigen remains unknown, but there is evidence to suggest that it is an antigen that cross reacts with antibodies to PDC-E2. Use of BEC purified from human liver may help in deciphering the possible importance of BEC plasma membrane antigens in immune recognition and toxicity toward BEC in PBC.

KEY WORDS

biliary epithelium - pyruvate dehydrogenase complex - mitochondria

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