Abstract
Primary biliary cirrhosis (PBC) is considered to be an autoimmune disease selectively targeted for interlobular bile ducts. While anti-mitochondrial antibodies are specifically detected in more than 90% of PBC patients, anti-nuclear envelope-gp210 antibodies are also specifically detected in 20-30% of PBC patients. In this review, we present 1, T cells specific for mitochondrial major epitope, PDC-E2 163-176, cross-react with peptides derived from nuclear envelope-gp210 protein, 2, PBC patients who have sustained high antibody titers to gp210 are at high risk for the progression to end-stage hepatic failure. These evidences may be very important for the epitope spreading of autoantigens from PDC-E2 to nuclear antigens and for the identification of target antigens on biliary epithelial cells which are recognized by cytotoxic T cells in PBC.
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