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Autoantibodies in primary biliary cirrhosis:Recent progress in research on the pathogenetic and clinical significance

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World Journal of Gastroenterology 2014年第10期20卷 2606-2612页

作者： Satoshi Yamagiwa Hiroteru Kamimura Masaaki Takamura Yutaka Aoyagi Division of Gastroenterology and Hepatology Niigata University Graduate School of Medical and Dental Sciences Niigata 951-8510 Japan

Primary biliary cirrhosis(PBC)is a chronic progressive cholestatic liver disease characterized by immunemediated destruction of the small-and medium-sized intrahepatic bile ducts and the presence of antimitochondrial antibodies(AMA)in the *** are detected in over 90%of patients with PBC,whereas their prevalence in the general population is extremely low,varying from 0.16%to 1%.Previous studies have shown that the unique characteristics of biliary epithelial cells undergoing apoptosis may result in a highly direct and very specific immune response to mitochondrial ***,recent studies have demonstrated that serum from AMA-positive PBC patients is reactive with a number of xenobiotic modified E2 subunits of the pyruvate dehydrogenase complex,which is not observed in the serum of normal *** findings indicate that chemicals originating from the environment may be associated with a breakdown in the tolerance to mitochondrial *** it is currently generally accepted that AMA are the most specific serological markers of PBC,more than 60 au-toantibodies have been investigated in patients with PBC,and some have previously been considered specific to other autoimmune *** review covers the recent progress in research on the pathogenetic and clinical significance of important autoantibodies in *** the pathogenic role of those autoantibodies in PBC remains a priority of basic and clinical research.

关键词： Primary biliary cirrhosis Autoantibodies anti-mitochondrial antibodies anticentromere antibodies anti-gp210 antibodies

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