The terminal complement complex in sera deficient in the eighth component of complement (C8).
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.
D000911:Antibodies, Monoclonal; D003185:Complement C8 / Q000172:deficiency*; D003186:Complement C9 / Q000276:immunology; D015938:Complement Membrane Attack Complex; D003165:Complement System Proteins / Q000502:physiology*; D006801:Humans; D046911:Macromolecular Substances; D015054:Zymosan / Q000276:immunology
D000911:Antibodies, Monoclonal; D003185:Complement C8; D003186:Complement C9; D015938:Complement Membrane Attack Complex; D046911:Macromolecular Substances; D003165:Complement System Proteins; D015054:Zymosan