PMID: 9659176Jul 11, 1998Paper

Autoantibodies against subunits of pyruvate dehydrogenase and citrate synthase in a case of paediatric biliary cirrhosis

Gut
B MeleghB Sumegi

Abstract

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 E1 alpha, E2, and E3 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's 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.

Citations

Jul 10, 2003·Autoimmunity Reviews·S Alice LongM Eric Gershwin
Dec 22, 2009·Integrative Biology : Quantitative Biosciences From Nano to Macro·Bénédicte CauweGhislain Opdenakker
Mar 23, 2000·Postgraduate Medical Journal·M I Prince, D E Jones
Dec 18, 2012·Case Reports in Pediatrics·Ivana KiticDragan Prokic
Dec 6, 2011·Immunity & Ageing : I & a·Daniel S SmykDimitrios P Bogdanos
May 1, 2011·Auto- Immunity Highlights·Daniel SmykDimitrios P Bogdanos

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