Wakamiya Nobutaka
Department of Microbiology and Immunochemistry, Asahikawa Medical University, Asahikawa 078-8510, Japan.
Rinsho Byori. 2012 Aug;60(8):779-85.
The complement has been identified as a complementation factor to compensate for the function of an antibody. The complement consists of C1-C9, a complement-related molecule, and its regulating molecules. Three major biological roles of the complement have been classified: First: opsonization following phagocytosis and the elimination of microbes; second: direct destruction of bacteria due to membrane attack complex (MAC); third: complement activation following the induction of anaphylactoid factors and local recruitment and activation of neutrophilic leukocytes. In this review, the basic findings and recent treatments of paroxysmal nocturnal hemoglobinuria (PNH) and hereditary angioedema (HAE) are summarized. Finally, there is a short review of a rare autosomal recessive disorder of 3MC syndrome and new biological functions of complement factors except for that of innate immunity are proposed.
补体已被鉴定为一种补体因子，用于补偿抗体的功能。补体由C1 - C9、一种补体相关分子及其调节分子组成。补体的三大主要生物学作用已被分类：第一：吞噬作用后的调理作用及微生物的清除；第二：由于膜攻击复合物（MAC）导致细菌的直接破坏；第三：类过敏因子诱导后补体激活以及嗜中性白细胞的局部募集和激活。在本综述中，总结了阵发性夜间血红蛋白尿（PNH）和遗传性血管性水肿（HAE）的基本研究结果和最新治疗方法。最后，对一种罕见的常染色体隐性3MC综合征进行了简短综述，并提出了补体因子除先天免疫功能外的新生物学功能。
