Hu V W, Nicholson-Weller A
Proc Natl Acad Sci U S A. 1985 Aug;82(16):5520-4. doi: 10.1073/pnas.82.16.5520.
The interaction of terminal complement proteins (C5-C9) with normal erythrocytes and type III paroxysmal nocturnal hemoglobinuria erythrocytes (PNH-E) has been compared in terms of binding of the C5-9 complex, C9 polymerization, and C9 insertion into membranes. Complement components C5, C7, and C8 bind equally well to both types of erythrocytes, whereas the binding of C9 to PNH-E is 5-6 times greater than that to normal erythrocytes. The kinetics of C9 binding was compared with the kinetics of lysis for both types of cells under conditions leading to 100% lysis. There was a noticeable lag time between C9 binding and lysis of normal erythrocytes, but the lysis of PNH-E proceeded without a lag and the kinetics of lysis more closely paralleled C9 binding. The efficiency of C9 insertion was similar for both types of cells, but C9 polymerization was significantly enhanced on PNH-E. These data indicate that the enhanced susceptibility of type III PNH-E toward lysis by C5-9 can be correlated with abnormally high C9 binding and increased formation of poly(C9).
已从C5-9复合物的结合、C9聚合以及C9插入膜等方面，对终末补体蛋白（C5-C9）与正常红细胞及III型阵发性睡眠性血红蛋白尿红细胞（PNH-E）之间的相互作用进行了比较。补体成分C5、C7和C8与这两种类型的红细胞结合情况相同，而C9与PNH-E的结合比与正常红细胞的结合大5至6倍。在导致100%溶血的条件下，比较了两种细胞的C9结合动力学和溶血动力学。正常红细胞的C9结合与溶血之间存在明显的延迟时间，但PNH-E的溶血无延迟进行，且溶血动力学与C9结合更为密切平行。两种类型细胞的C9插入效率相似，但PNH-E上的C9聚合显著增强。这些数据表明，III型PNH-E对C5-9介导的溶血敏感性增强，可能与异常高的C9结合及多聚（C9）形成增加有关。
