Mollnes T E, Lea T, Rogde S, Tedesco F
Scand J Immunol. 1986 Sep;24(3):307-12. doi: 10.1111/j.1365-3083.1986.tb02098.x.
The terminal complement complex (TCC) was quantified in sera from patients with a genetic deficiency of C8 alpha-gamma or C8 beta. The individual sera contained only trace amounts of TCC compared with a normal serum pool. The content of TCC increased after mixing the two sera, which was consistent with reconstitution of C8 activity. Only a moderate increase in TCC was obtained after zymosan activation of the individual sera, whereas activation of the mixture resulted in high amounts of TCC. C8 was demonstrated in the TCC of both deficient sera. These results may indicate that functional C8 is present in trace amounts despite the genetic deficiency, and that the terminal pathway may function to some extent although not enough to be detectable in less sensitive assays.
对患有C8α-γ或C8β基因缺陷的患者血清中的终末补体复合物（TCC）进行了定量分析。与正常血清库相比，个体血清中仅含有微量的TCC。将两种血清混合后，TCC含量增加，这与C8活性的重建一致。单独激活个体血清后，TCC仅适度增加，而混合物的激活则产生大量TCC。在两种缺陷血清的TCC中均检测到C8。这些结果可能表明，尽管存在基因缺陷，但仍存在微量的功能性C8，并且终末途径可能在一定程度上发挥作用，尽管在不太敏感的检测中不足以检测到。
