Berg Christoph P, Kannan Thirumalai R, Klein Reinhild, Gregor Michael, Baseman Joel B, Wesselborg Sebastian, Lauber Kirsten, Stein Gerburg M
Department of Internal Medicine I, Medical Clinic, Eberhard-Karls-University of Tübingen, Tübingen, Germany.
Liver Int. 2009 Jul;29(6):797-809. doi: 10.1111/j.1478-3231.2008.01942.x.
In primary biliary cirrhosis (PBC), autoreactivity mainly targets members of the pyruvate dehydrogenase complex (PDC). Because PDC subunits are expressed on the surface of mycoplasma and molecular mimicry may be one aetiological factor, we analysed the presence of mammalian and mycoplasma PDC-specific antibodies in PBC patients.
Antibodies to porcine PDC and Mycoplasma pneumoniae (mp) antigens mpPDH-C (to be designated mpPDC-E2 chain), mpPDH-B (to be designated mpPDC-E1beta chain), mpCARDS TX and mpP1 were investigated in sera from 43 PBC patients, 19 patients with autoimmune hepatitis and 11 healthy controls by an enzyme-linked immunosorbent assay and Western blotting. To study the rate of acute mycoplasma infection, an adhesin P1-specific polymerase chain reaction (PCR) was performed.
Immune reactivity to the mpPDC-E2 antigen was significantly enhanced in PBC patients (83.7%) as compared with controls (overall frequency of 36.7%), while antibodies to the porcine PDC-E2 chain were found only in PBC patients (88%) excluding a simple cross-reactivity of PDC-related antibodies. This observation was confirmed by inhibition studies demonstrating that porcine PDC did not inhibit mycoplasma PDC-specific antibodies and vice versa. The occurrence of antibodies to mpPDC seems to precede the occurrence of antibodies to porcine PDC. Infection with mycoplasma was equally distributed in the groups as evidenced by an antibody frequency comparable to CARDS TX and P1 and PCR reactivity.
Because PBC patients show a significantly enhanced frequency of mpPDC-E2-related antibodies, besides other factors, molecular mimicry between surface molecules of mycoplasma and epitopes of the autoantigen may play a central role in the aetiopathology of PBC.
在原发性胆汁性肝硬化(PBC)中,自身反应性主要针对丙酮酸脱氢酶复合体(PDC)的成员。由于PDC亚基在支原体表面表达,分子模拟可能是一个病因学因素,我们分析了PBC患者中哺乳动物和支原体PDC特异性抗体的存在情况。
通过酶联免疫吸附测定和蛋白质印迹法,对43例PBC患者、19例自身免疫性肝炎患者和11名健康对照者血清中的抗猪PDC抗体以及肺炎支原体(mp)抗原mpPDH-C(将被命名为mpPDC-E2链)、mpPDH-B(将被命名为mpPDC-E1β链)、mpCARDS TX和mpP1进行了研究。为了研究急性支原体感染率,进行了黏附素P1特异性聚合酶链反应(PCR)。
与对照组(总频率为36.7%)相比,PBC患者中对mpPDC-E2抗原的免疫反应性显著增强(83.7%),而抗猪PDC-E2链抗体仅在PBC患者中发现(88%),排除了PDC相关抗体的简单交叉反应性。抑制研究证实了这一观察结果,表明猪PDC不能抑制支原体PDC特异性抗体,反之亦然。mpPDC抗体的出现似乎先于猪PDC抗体的出现。如与CARDS TX和P1的抗体频率及PCR反应性相当所示,支原体感染在各组中分布相同。
由于PBC患者中mpPDC-E2相关抗体的频率显著增加,除其他因素外,支原体表面分子与自身抗原表位之间的分子模拟可能在PBC的发病机制中起核心作用。