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鉴定血小板糖蛋白IIb/IIIa复合物为原发性胆汁性肝硬化相关自身免疫性血小板减少症的靶抗原。有证据表明血小板反应性自身抗体也可与线粒体抗原M2结合。

Identification of the platelet glycoprotein IIb/IIIa complex as a target antigen in primary biliary cirrhosis-associated autoimmune thrombocytopenia. Evidence that platelet-reactive autoantibodies can also bind to the mitochondrial antigen M2.

作者信息

Panzer S, Penner E, Nelson P J, Prochazka E, Benda H, Saurugger P N

机构信息

First Internal Medical Clinic, University of Vienna, Austria.

出版信息

J Autoimmun. 1990 Aug;3(4):473-83. doi: 10.1016/s0896-8411(05)80014-9.

Abstract

A 67-year-old woman with a 4-year history of primary biliary cirrhosis (PBC) unexpectedly developed autoimmune thrombocytopenia. The platelet-bound IgG antibody was eluted from the patient's platelets to determine the platelet target antigen. The autoantibodies were found to precipitate the platelet glycoprotein complex IIb/IIIa of autologous and allogeneic platelets. A further precipitate of 70 kDa was detectable under reducing conditions. In addition, platelet-reactive antibodies bound to the 70 kDa mitochondrial antigen M2. No cross-absorption studies were performed to confirm that a single antibody reacted with both antigens. Computer analysis of published peptide sequences of the mitochondrial protein and the platelet GPIIb/IIIa complex showed partial amino acid sequence homology suggesting the possibility of a common antibody binding site. These findings suggest a relationship between the development of autoimmune thrombocytopenia in PBC and the underlying liver disease.

摘要

一名患有原发性胆汁性肝硬化(PBC)4年的67岁女性意外出现自身免疫性血小板减少症。从患者血小板中洗脱结合血小板的IgG抗体,以确定血小板靶抗原。发现自身抗体能沉淀自体和异体血小板的血小板糖蛋白复合物IIb/IIIa。在还原条件下可检测到70 kDa的进一步沉淀。此外,血小板反应性抗体与70 kDa线粒体抗原M2结合。未进行交叉吸收研究以确认单一抗体与两种抗原反应。对线粒体蛋白和血小板GPIIb/IIIa复合物已发表的肽序列进行计算机分析,显示部分氨基酸序列同源,提示可能存在共同的抗体结合位点。这些发现提示PBC中自身免疫性血小板减少症的发生与潜在肝脏疾病之间存在关联。

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