{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,24]],"date-time":"2026-01-24T18:10:23Z","timestamp":1769278223037,"version":"3.49.0"},"reference-count":0,"publisher":"Wiley","issue":"2-4","license":[{"start":{"date-parts":[[2000,1,1]],"date-time":"2000-01-01T00:00:00Z","timestamp":946684800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["SFB 244-C11"],"award-info":[{"award-number":["SFB 244-C11"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Journal of Immunology Research"],"published-print":{"date-parts":[[2003,1]]},"abstract":"<jats:p><jats:italic>Background and aims<\/jats:italic>: Antimitochondrial antibodies (AMA) which recognize pyruvate acetyltransferase (PDC\u2010E2) represent a highly diagnostic feature of primary biliary cirrhosis (PBC). The analysis of immunofluorescence (IF)\u2010AMA\u2010positive sera in PBC patients indicates a conformational epitope located within the lipoyl binding domain of bovine branched\u2010chain acyltransferase (BCKADC\u2010E2) alone or in combination with AMA directed against PDC\u2010E2 the significance of which is presently unclear. In the present study, immunoreactivities and disease associations of AMA against BCKADC\u2010E2 were analyzed. B\u2010cell autoepitopes on BCKADC\u2010E2 were mapped by immunoprecipitation assay.<\/jats:p><jats:p><jats:italic>Methods<\/jats:italic>: Sera of 96 IF\u2010AMA\u2010positive patients with serological evidence of anti\u2010BCKADC\u2010E2 alone (<jats:italic>n<\/jats:italic>=26), anti\u2010PDC\u2010E2 alone (<jats:italic>n<\/jats:italic>=15), and both anti\u2010BCKADC\u2010E2 and anti\u2010PDC\u2010E2 (<jats:italic>n<\/jats:italic>=55) were analyzed by Western blot and ELISA in addition to an analysis of B cell autoepitopes on BCKADC\u2010E2 by immunoprecipitation using <jats:italic>in vitro<\/jats:italic> translated, unmodified human proteins. Ninety\u2010four patients without IF\u2010AMA [blood donors (<jats:italic>n<\/jats:italic>=30), rheumatoid arthritis (<jats:italic>n<\/jats:italic>=40), autoimmune hepatitis (AIH) (<jats:italic>n<\/jats:italic>=10) and primary sclerosing cholangitis (PSC) (<jats:italic>n<\/jats:italic>=14] served as controls.<\/jats:p><jats:p><jats:italic>Results<\/jats:italic>: Eighty of 81 (99%) sera positive for BCKADC\u2010E2 recognized the full length, mature protein, while only 2\/10 AIH sera and none of the other controls showed reactivity. Of the 68 PBC sera 58 (85%) recognized the N\u2010terminus consisting of aa 1\u2010144 representing the lipoyl domain. Surprisingly, C\u2010terminal sequences (aa 143\u2010421) were recognized by 46 out of 68 sera (68%). Three PBC sera reacted with the C\u2010terminus only. Only 1\/7 serum from patients with an \u201coverlap syndrome of PBC and AIH\u201d was reactive with C\u2010terminal sequences.<\/jats:p><jats:p><jats:italic>Conclusions<\/jats:italic>: Our analysis of BCKADC\u2010E2\u2010positive PBC sera identified a novel B cell epitope on the C\u2010terminal part of the human protein. Our data indicate that a distinct subset of AMA recognize sequence(s) on BCKADC\u2010E2 which located outside of the lipoyl binding domain. The absence of immunoreactivity against C\u2010terminal sequences may serve as a marker differentiating patients with PBC and overlap syndrome of PBC with AIH.<\/jats:p>","DOI":"10.1080\/10446670310001642159","type":"journal-article","created":{"date-parts":[[2003,12,30]],"date-time":"2003-12-30T17:02:39Z","timestamp":1072803759000},"page":"173-181","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Characterization of a Lipoyl Domain\u2010Independent B\u2010Cell Autoepitope on the Human Branched\u2010Chain Acyltransferase in Primary Biliary Cirrhosis and Overlap Syndrome with Autoimmune Hepatitis"],"prefix":"10.1155","volume":"10","author":[{"given":"Antal","family":"Csepregi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Petra","family":"Obermayer-Straub","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susanne","family":"Kneip","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne","family":"Kayser","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephanie","family":"Loges","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eleonore","family":"Schmidt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elem\u00e9r","family":"Nemes\u00e1nszky","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ferenc","family":"Szalay","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael P.","family":"Manns","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian P.","family":"Strassburg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2003,1]]},"container-title":["Journal of Immunology Research"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/downloads.hindawi.com\/journals\/jir\/2003\/207213.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1080\/10446670310001642159","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,8,8]],"date-time":"2024-08-08T11:30:54Z","timestamp":1723116654000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1080\/10446670310001642159"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2003,1]]},"references-count":0,"journal-issue":{"issue":"2-4","published-print":{"date-parts":[[2003,1]]}},"alternative-id":["10.1080\/10446670310001642159"],"URL":"https:\/\/doi.org\/10.1080\/10446670310001642159","archive":["Portico"],"relation":{},"ISSN":["2314-8861","2314-7156"],"issn-type":[{"value":"2314-8861","type":"print"},{"value":"2314-7156","type":"electronic"}],"subject":[],"published":{"date-parts":[[2003,1]]},"assertion":[{"value":"2003-01-01","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}