Mutimer D J, Fussey S P, Yeaman S J, Kelly P J, James O F, Bassendine M F
Department of Medicine, Medical School, University of Newcastle Upon Tyne, United Kingdom.
Hepatology. 1989 Oct;10(4):403-7. doi: 10.1002/hep.1840100402.
We have previously identified four of the M2 antigens in primary biliary cirrhosis as the E2 components (dihydrolipoamide acyltransferases) of pyruvate dehydrogenase complex, branched-chain 2-oxo acid dehydrogenase complex and 2-oxoglutarate dehydrogenase complex and the protein X component of pyruvate dehydrogenase complex (approximate molecular masses: 74, 50, 50 and 52 kD, respectively). In the present study, we have examined by immunoblotting the frequency of IgG and IgM autoantibodies to these four proteins in 129 patients with primary biliary cirrhosis (36 histological Stage I, 42 Stage II/III, 51 Stage IV) and 77 controls (49 non-primary biliary cirrhosis chronic liver disease, 16 primary Sjögren's syndrome, 12 healthy normal women). One hundred twenty-seven of 129 (98%) primary biliary cirrhosis patients had antibodies against at least one of the four M2 polypeptides, compared to 2/77 controls (both had autoimmune chronic active hepatitis and were antimitochondrial antibody positive by indirect immunofluorescence). One hundred twenty-one of 129 (94%) primary biliary cirrhosis sera reacted with the E2 component and protein X of pyruvate dehydrogenase complex, 69/129 (53%) primary biliary cirrhosis sera reacted with E2 of branched-chain 2-oxo acid dehydrogenase complex and 113/129 (88%) reacted with E2 of 2-oxoglutarate dehydrogenase complex. Primary biliary cirrhosis patients with histological Stage I disease had a lower incidence of autoantibodies to each M2 protein, compared to more advanced disease (IgG, p less than 0.05) but only 2/36 Stage I patients had no anti-M2 antibodies. There was no correlation between the presence of IgG or IgM antibodies to the M2 polypeptides and established prognostic markers in primary biliary cirrhosis (serum bilirubin and albumin levels).(ABSTRACT TRUNCATED AT 250 WORDS)
我们之前已确定，原发性胆汁性肝硬化中的四种M2抗原是丙酮酸脱氢酶复合体、支链2-氧代酸脱氢酶复合体和2-氧代戊二酸脱氢酶复合体的E2成分（二氢硫辛酰胺酰基转移酶）以及丙酮酸脱氢酶复合体的蛋白X成分（近似分子量分别为74、50、50和52 kD）。在本研究中，我们通过免疫印迹法检测了129例原发性胆汁性肝硬化患者（36例组织学I期、42例II/III期、51例IV期）和77例对照者（49例非原发性胆汁性肝硬化慢性肝病、16例原发性干燥综合征、12例健康正常女性）中针对这四种蛋白的IgG和IgM自身抗体的频率。129例原发性胆汁性肝硬化患者中有127例（98%）针对四种M2多肽中的至少一种有抗体，相比之下，77例对照者中有2例（均患有自身免疫性慢性活动性肝炎且间接免疫荧光法检测抗线粒体抗体呈阳性）。129例原发性胆汁性肝硬化血清中有121例（94%）与丙酮酸脱氢酶复合体的E2成分和蛋白X发生反应，69/129例（53%）原发性胆汁性肝硬化血清与支链2-氧代酸脱氢酶复合体的E2发生反应，113/129例（88%）与2-氧代戊二酸脱氢酶复合体的E2发生反应。与病情更严重的患者相比，组织学I期疾病的原发性胆汁性肝硬化患者针对每种M2蛋白的自身抗体发生率较低（IgG，p<0.05），但I期患者中只有2/36没有抗M2抗体。原发性胆汁性肝硬化中针对M2多肽的IgG或IgM抗体的存在与既定的预后标志物（血清胆红素和白蛋白水平）之间没有相关性。（摘要截选至250词）
