Abstract
Background. Primary biliary cirrhosis (PBC) is characterized by antimitochondrial antibodies (AMA), directed to the E2 component of the pyruvate dehydrogenase complex (PDC-E2). A vital question in the pathogenesis of PBC is why biliary epithelial cells are the primary target of pathology despite the presence of mitochondria in virtually all nucleated cells; the reasons for this tissue specificity remain unknown. It has been demonstrated that, following apoptosis of biliary epithelial cells (BEC), the autoantigenic PDC-E2 remains immunologically intact and still recognizable as such by AMA.
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