Alper C A, Goodkofsky I, Lepow I H
J Exp Med. 1973 Feb 1;137(2):424-37. doi: 10.1084/jem.137.2.424.
Factor B activity of the properdin system was found to be identical with purified glycine-rich beta-glycoprotein (GBG) but was distinct from the normal human serum protein capable of forming a C3-inactivating complex with a protein from cobra venom (CoF). Factor B activity coincided with electrophoretically separated GBG genetic variants, whereas the CoF-binding protein did not. GBGase destroyed factor B as it cleaved GBG but did not destroy the C3-inactivating activity of the CoF-binding protein. During incubation of serum with CoF, GBG did not change in molecular size, nor was there any coincidence in the immunoelectrophoretic mobilities of CoF and GBG. It was not possible to precipitate labeled CoF incubated with serum by anti-GBG, nor labeled GBG from serum incubated with CoF by anti-CoF. The CoF-binding capacity of serum was 2 mg/100 ml or less or under 6.5% of the serum concentration of GBG. When labeled CoF was added to serum below the binding capacity, complete complexation of the CoF was demonstrated, whereas CoF was largely uncomplexed when CoF was added in amounts equimolar to GBG.
发现备解素系统的B因子活性与纯化的富含甘氨酸的β-糖蛋白(GBG)相同,但与能够与眼镜蛇毒蛋白形成C3灭活复合物的正常人血清蛋白(CoF)不同。B因子活性与经电泳分离的GBG基因变体一致,而结合CoF的蛋白则不然。GBG酶在裂解GBG时会破坏B因子,但不会破坏结合CoF的蛋白的C3灭活活性。血清与CoF孵育期间,GBG的分子大小不变,CoF和GBG在免疫电泳迁移率上也没有任何重合。用抗GBG无法沉淀与血清孵育的标记CoF,用抗CoF也无法沉淀与CoF孵育的血清中的标记GBG。血清的CoF结合能力为2mg/100ml或更低,即低于血清中GBG浓度的6.5%。当将标记的CoF以低于结合能力的量加入血清时,CoF会完全形成复合物,而当加入与GBG等摩尔量的CoF时,CoF大部分未形成复合物。