Until 4 years ago, PBC could only be classified as an autoimmune liver disease characterized by biliary obstruction and the presence of high titer of AMA. Using the tools of molecular biology, the molecular structures of the autoantigens as well as the B-cell epitopes have been defined within a relatively short time. However, the mechanism of how these subcellular enzymes become autoantigens is still unclear. Is the antibody response antigen driven or the result of molecular mimicry? With the rapid accumulation of information as well as unique reagents such as cDNA clones, recombinant proteins, monoclonal antibodies, it is possible to address questions on the possible mechanisms by which these mitochondrial proteins can become the target of immune response and how these responses are related to the pathogenesis of PBC. The attainment of these challenging goals and directions will be of substantial value in understanding the role of subcellular molecular interactions in the development of autoimmunity.
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