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The immunology of primary biliary cirrhosis: The end of the beginning?

Lookup NU author(s): Dr Jeremy Palmer, Emeritus Professor John Kirby, Professor David Jones


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The chronic liver disease primary biliary cirrhosis (PBC) is characterised by autoreactive B-cell and T-cell responses directed against mitochondrial antigens. In recent years these responses have been extensively characterised and the principal PBC associated autoantigen identified as pyruvate dehydrogenase complex (PDC). The identification of anti-PDC responses (present in over 95% of PDC patients) has given rise to important questions pertinent to our understanding of the pathogenesis of PBC. What specific role to anti-PDC responses play in target cell damage? How and why does immune tolerance break down to as highly conserved and ubiquitously expressed self-antigen as PDC? Why does breakdown in tolerance to an antigen present in all nucleated cells result in damage restricted to the intra-hepatic bile ducts? In attempting to answer these key questions we have, in this review, proposed a unifying hypothesis for the pathogenesis of PBC.

Publication metadata

Author(s): Palmer JM, Kirby JA, Jones DEJ

Publication type: Review

Publication status: Published

Journal: Clinical and Experimental Immunology

Year: 2002

Volume: 129

Issue: 2

Pages: 191-197

Print publication date: 01/01/2002

ISSN (print): 0009-9104

ISSN (electronic): 1365-2249


DOI: 10.1046/j.1365-2249.2002.01948.x

PubMed id: 12165073