{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,27]],"date-time":"2026-07-27T19:14:15Z","timestamp":1785179655507,"version":"3.55.0"},"reference-count":15,"publisher":"BMJ","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Gut"],"accepted":{"date-parts":[[1997,10,31]]},"published-print":{"date-parts":[[1998,5,1]]},"abstract":"<jats:p>In a newborn girl with a history of connatal liver damage, histological examination of a liver biopsy sample taken during the seventh week of life revealed incipient destruction of bile ducts. Very high titres of antimitochondrial antibodies were later detected in the plasma. As the hepatic injury tended towards fibrosis, the histological diagnosis became primary biliary cirrhosis. Autoantibodies against E<jats:sub>1\u03b1<\/jats:sub>, E<jats:sub>2<\/jats:sub>, and E<jats:sub>3<\/jats:sub> subunits and protein X component of pyruvate dehydrogenase complex, and against citrate synthase were detected on western immunoblotting in a 1 in 1000 dilution of the patient\u2019s serum. The patient died of her illness at 11 years of age. In liver specimens obtained at autopsy human immunoglobulin deposition was detected on the surface of almost all hepatic cells by immunohistology. As there is a physical and functional interaction between pyruvate dehydrogenase and citrate synthase within the mitochondria, the presence of autoantibodies against certain proteins in the patient suggests that in this form of the disease the molecular recognition and then the autoimmunisation process could be directed against a mitochondrial enzyme cluster containing both pyruvate dehydrogenase and citrate synthase.<\/jats:p>","DOI":"10.1136\/gut.42.5.753","type":"journal-article","created":{"date-parts":[[2010,6,30]],"date-time":"2010-06-30T12:46:12Z","timestamp":1277901972000},"page":"753-756","source":"Crossref","is-referenced-by-count":13,"title":["Autoantibodies against subunits of pyruvate dehydrogenase and citrate synthase in a case of paediatric biliary cirrhosis"],"prefix":"10.1136","volume":"42","author":[{"given":"B","family":"Melegh","sequence":"first","affiliation":[{"name":"aDepartment of Pediatrics, MTA-POTE Clinical Genetics Research Group, Hungarian Academy of Sciences, University Medical School, P\u00e9cs, Hungary, \n\nbDepartment of Pathology, \n\ncDepartment of Biochemistry, University Medical School, P\u00e9cs, Hungary, \n\ndDepartment of Pathology, Baranya County Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G","family":"Skuta","sequence":"additional","affiliation":[{"name":"aDepartment of Pediatrics, MTA-POTE Clinical Genetics Research Group, Hungarian Academy of Sciences, University Medical School, P\u00e9cs, Hungary, \n\nbDepartment of Pathology, \n\ncDepartment of Biochemistry, University Medical School, P\u00e9cs, Hungary, \n\ndDepartment of Pathology, Baranya County Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L","family":"Pajor","sequence":"additional","affiliation":[{"name":"aDepartment of Pediatrics, MTA-POTE Clinical Genetics Research Group, Hungarian Academy of Sciences, University Medical School, P\u00e9cs, Hungary, \n\nbDepartment of Pathology, \n\ncDepartment of 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