{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T18:51:21Z","timestamp":1773427881531,"version":"3.50.1"},"reference-count":35,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"6","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":9223,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1990,6]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>Sera from patients with primary biliary cirrhosis contain autoantibodies that recognize mitochondrial proteins. Five of the target autoantigens have now been identified as enzymes of three related multienzyme complexes: the pyruvate dehydrogenase complex, the branched chain \u03b1\u2013ketoacid dehydrogenase complex and the \u03b1\u2013ketoglutarate dehydrogenase complex. Each complex consists of component enzymes designated E1, E2 and E3. In this report, we confirm that primary biliary cirrhosis sera react with dihydrolipoamide succinyltransferase, the E2 component of \u03b1\u2013ketoglutarate dehydrogenase complex. Seventy\u2013three of 188 (39%) primary biliary cirrhosis sera reacted with \u03b1\u2013ketoglutarate dehydrogenase complex\u2013E2 when immunoblotted against purified \u03b1\u2013ketoglutarate dehydrogenase complex; one of these sera also reacted with the E1 component. In addition, primary biliary cirrhosis sera possessing \u03b1\u2013ketoglutarate dehydrogenase complex\u2013E2 reactivity specifically inhibited enzyme function of \u03b1\u2013ketoglutarate dehydrogenase complex. Enzyme activity was not affected by primary biliary cirrhosis sera that contained autoantibodies to pyruvate dehydrogenase complex\u2013E2 and\/or branched chain \u03b1\u2013ketoacid dehydrogenase complex\u2013E2, which lacked \u03b1\u2013ketoglutarate dehydrogenase complex\u2013E2 reactivity. Furthermore, affinity\u2013purified primary biliary cirrhosis sera against \u03b1\u2013ketoglutarate dehydrogenase complex\u2013E2 inhibited only \u03b1\u2013ketoglutarate dehydrogenase complex activity but did not alter enzyme activity of either pyruvate dehydrogenase complex or branched chain \u03b1\u2013ketoacid dehydrogenase complex. Finally, \u03b1\u2013ketoglutarate dehydrogenase complex\u2013E2 specific affinity\u2013purified antisera did not react on immunoblot with any component enzymes of pyruvate dehydrogenase complex or branched chain \u03b1\u2013ketoacid dehydrogenase complex. These data demonstrate that the E2 component of \u03b1\u2013ketoglutarate dehydrogenase complex is recognized by a distinct population of autoantibodies separate from autoantibodies that recognize pyruvate dehydrogenase complex\u2013E2 or branched chain \u03b1\u2013ketoacid dehydrogenase complex\u2013E2. Our data further suggest that these autoantibodies are directed toward a functional domain of this enzyme.(HEPATOLOGY 1990;11:975\u2013981.).<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/hep.1840110611","type":"journal-article","created":{"date-parts":[[2007,3,3]],"date-time":"2007-03-03T23:35:56Z","timestamp":1172964956000},"page":"975-981","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":51,"title":["Inhibition of \u03b1\u2013Ketoglutarate Dehydrogenase Activity by A Distinct Population of Autoantibodies Recognizing Dihydrolipoamide Succinyltransferase in Primary Biliary Cirrhosis"],"prefix":"10.1097","volume":"11","author":[{"given":"David R.","family":"Fregeau","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Prindiville","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ross 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