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人类补体第八成分缺乏。血清淋病奈瑟菌杀菌活性对C8的需求。

Human deficiency of the eighth component of complement. The requirement of C8 for serum Neisseria gonorrhoeae bactericidal activity.

作者信息

Petersen B H, Graham J A, Brooks G F

出版信息

J Clin Invest. 1976 Feb;57(2):283-90. doi: 10.1172/JCI108279.

Abstract

The serum of a 23-yr-old woman with prolonged disseminated gonococcal infection syndrome failed to normally promote hemolysis of sensitized sheep red blood cells (RBC). The patient's serum was deficient in the eight component of complement (C8) as determined by functional assays, immunoelectrophoresis, and quantitative immunoprecipitation. Functional titers of each of her other complement components were normal. No serum inhibitors of C8 were detected. The patient's serum supported activation of both the classical and alternate complement pathways. Her fresh serum lacked any bactericidal activity against Neisseria gonorrhoeae, but addition of purified C8 or complement donor serum restored bactericidal activity as well as RBC hemolytic activity. Her serum gave normal opsonization of yeast particles and staphylococci and had normal capacity to coat sensitized RBC with C8 and C4 and to generate chemotactic activity. No defects were observed in the patient's blood coagulation mechanisms. Complement-mediated bacterial lysis may be important in human defense against bacteremic Neisseria infections.

摘要

一名患有持续性播散性淋球菌感染综合征的23岁女性的血清未能正常促进致敏绵羊红细胞(RBC)的溶血。通过功能测定、免疫电泳和定量免疫沉淀确定,该患者血清中补体(C8)的第八成分缺乏。她其他补体成分的功能滴度均正常。未检测到C8的血清抑制剂。患者的血清支持经典和替代补体途径的激活。她的新鲜血清对淋病奈瑟菌没有任何杀菌活性,但添加纯化的C8或补体供体血清可恢复杀菌活性以及RBC溶血活性。她的血清对酵母颗粒和葡萄球菌具有正常的调理作用,并且具有用C8和C4包被致敏RBC以及产生趋化活性的正常能力。在患者的凝血机制中未观察到缺陷。补体介导的细菌溶解可能在人类抵御奈瑟菌血症感染中起重要作用。

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