AMAs及其靶抗原在原发性胆汁性肝硬化发病中的作用

原发性胆汁性肝硬化（PBC）是一种以慢性进行性胆汁淤积为特点的自身免疫性肝病,血清中抗线粒体抗体（AMAs）,尤其AMA-M2升高是该病的特异性表现.AMAs在PBC发病过程中的作用是研究重点之一.丙酮酸脱氢酶复合物（PDC）的E2亚单位（PDC-E2）是AMAs的主要抗原.诱发AMAs靶抗原暴露和打破免疫耐受的主要诱因是感染及化学因素.后者或与靶抗原交叉反应,或通过对靶抗原的化学修饰,或是使凋亡后抗原的清除发生障碍,从而促进AMAs的产生.针对PDC-E2的细胞免疫主要参与PBC的发病.AMAs在PBC发病中的作用存在争议,更多研究认为其不参与PBC的发病.

The Effects of AMAs and Its Target Antigen in Primary Biliary Cirrhosis

Primary biliary cirrhosis （PBC） is a slowly progressive cholestatic liver disease of autoimmune etiology. High- titer serum anti-mitochondrial antibodies （AMAs） are pathognomonic for PBC, especially AMA-M2. The effection of AMAs in the pathogenesis of Pt3C remains hotspot of research all the time. AMAs specifically target E2-protein components of the pyruvate dehydrogenase complex （PDC）. Several environmental factors--mainly infectious and chemical--are also thought to contribute to the generation of the AMAs, largely through molecular mimicry, modification of auto-antigens or inappropriate post-apoptotic degradation. In addition, cellular immunity to the E2 component of PDC are important immunopathogenesis of PBC. Moreover, more researches failed support that AMAs were associated with the pathogenesis of PBC.