丙酮酸脱氢酶复合体的E1α和β亚基是原发性胆汁性肝硬化中的M2'd'和M2'e'自身抗原。

The E1 alpha and beta subunits of the pyruvate dehydrogenase complex are M2'd' and M2'e' autoantigens in primary biliary cirrhosis.

Fussey S P, Bassendine M F, Fittes D, Turner I B, James O F, Yeaman S J

Department of Biochemistry and Genetics Medical School, University of Newcastle upon Tyne, U.K.

Clin Sci (Lond). 1989 Oct;77(4):365-8. doi: 10.1042/cs0770365.

Abstract

Sera from 76 patients with primary biliary cirrhosis (PBC) and 66 control subjects (53 with chronic liver disease and 13 healthy normal women) were immuno-blotted against purified E1 component of bovine pyruvate dehydrogenase complex (PDC) and bacterial PDC. 2. Thirty-one out of seventy-six (41%) sera from PBC patients showed a positive response to bovine E1 alpha, and five of these 31 (7% of total) reacted with bovine E1 beta. None of the control sera reacted with bovine E1 alpha or beta. 3. None of the PBC sera that recognized bovine E1 subunits reacted with bacterial PDC E1. 4. In the PBC patients there was no correlation between presence of antibodies to E1 alpha and beta subunits and histological stage of the disease. 5. Our data demonstrate that the E1 alpha and beta components of mammalian PDC are the M2'd' and 'e' mitochondrial autoantigens, respectively.

摘要

用纯化的牛丙酮酸脱氢酶复合体（PDC）的E1组分和细菌PDC对76例原发性胆汁性肝硬化（PBC）患者的血清以及66例对照者（53例慢性肝病患者和13例健康正常女性）的血清进行免疫印迹分析。2. 76例PBC患者血清中有31例（41%）对牛E1α呈阳性反应，这31例中有5例（占总数的7%）与牛E1β发生反应。对照血清中无一例与牛E1α或E1β发生反应。3. 识别牛E1亚基的PBC血清中无一例与细菌PDC E1发生反应。4. 在PBC患者中，针对E1α和β亚基的抗体的存在与疾病的组织学分期之间无相关性。5. 我们的数据表明，哺乳动物PDC的E1α和β组分分别是M2'd'和'e'线粒体自身抗原。