Nakanuma Y, Yasoshima M, Morita T, Gershwin M E
Second Department of Pathology, Kanazawa University School of Medicine.
Nihon Rinsho. 1995 May;53(5):1287-96.
Primary biliary cirrhosis (PBC) is characterized by nonsuppurative destructive cholangitis (CNSDC) of interlobular bile ducts. The ducts with CNSDC are positive for HLA-A, B, C and -DR and also infiltrated and surrounded by immunocompetent cells, suggesting the participation of autoimmune mechanism. Target antigens of antimitochondrial antibodies (AMAs), characteristically and very frequently detected in PBC patients, are closely related to pyruvate dehydrogenase complex-E2 (PDC-E2) which has been recently shown to be expressed abnormally on the luminal side of interlobular bile ducts of PBC. AMAs can inhibit the enzyme activities of PDC-E2. T cells reactive with this enzyme have been recently cloned from the livers with PBC. Although unusual expression of PDC-E2 on the bile ducts, particularly on their luminal side, is observed, it is unclear whether this phenomenon is related primarily or secondarily to the cause of bile duct damages in PBC.
原发性胆汁性肝硬化（PBC）的特征是小叶间胆管的非化脓性破坏性胆管炎（CNSDC）。患有CNSDC的胆管HLA-A、B、C和-DR呈阳性，并且也被免疫活性细胞浸润和包围，提示自身免疫机制的参与。抗线粒体抗体（AMA）的靶抗原在PBC患者中特征性且非常频繁地被检测到，其与丙酮酸脱氢酶复合物-E2（PDC-E2）密切相关，最近研究表明PDC-E2在PBC的小叶间胆管腔侧异常表达。AMA可抑制PDC-E2的酶活性。最近已从患有PBC的肝脏中克隆出与该酶反应的T细胞。尽管观察到PDC-E2在胆管上异常表达，特别是在其腔侧，但尚不清楚这种现象是主要还是次要地与PBC中胆管损伤的原因相关。
