Other clinical studyAutoantibodies to BCOADC-E2 in patients with primary biliary cirrhosis recognize a conformational epitope☆
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2013, Journal of AutoimmunityCitation Excerpt :AMA is present in over 95% of patients with PBC and is the most specific diagnostic antibody marker of PBC [43]. The autoantigens of AMA have been identified as the E2 subunits of the 2-oxo-acid dehydrogenase complexes (2OADC-E2), including the E2 subunits of the pyruvate dehydrogenase complex (PDC-E2), branched chain 2-oxo acid dehydrogenase complex (BCOADC-E2) 2-oxo-glutarate dehydrogenase complex (OGDC-E2) [44–47] and the E3 binding protein of dihydrolipoamide dehydrogenase [48]. The AMA target antigens are all localized within the inner mitochondrial matrix and catalyze the oxidative decarboxylation of 2-oxo-acid acid substrates [49].
Xenobiotics and autoimmunity: Does acetaminophen cause primary biliary cirrhosis?
2012, Trends in Molecular MedicineCitation Excerpt :AMAs are present in over 95% of patients with PBC and are the most specific diagnostic antibody marker of PBC [24,25]. The autoantigens of AMAs have been identified as the E2 subunits of the 2-oxo-acid dehydrogenase complexes (2OADC-E2), including the E2 subunits of the pyruvate dehydrogenase complex (PDC-E2), branched chain 2-oxo acid dehydrogenase complex (BCOADC-E2), and 2-oxo-glutarate dehydrogenase complex (OGDC-E2) [26–30]. The AMA target antigens are all localized to the inner mitochondrial matrix and catalyze the oxidative decarboxylation of 2-oxo-acid acid substrates [31].
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This study was supported by NIH grants DK 39588 and AI 31585 and the Liver Tissue Procurement and Distribution System Grant 1-DK-6-2274.
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Dr Cha's current address is the Department of Biology and Technology, Kangweon National University, Choonchan, South Korea.