title: Characterization of soluble terminal complement complex assembled in C8beta-deficient plasma and serum.
abstract: Sera genetically deficient in either the alpha-gamma or the beta-subunit of complement component C8 virtually lack haemolytic activity. We have studied the formation and the structural organization of the soluble terminal complement complex (TCC) assembled in these sera following activation with cobra venom factor (CVF). The TCC concentration in the activated C8alpha-gamma and C8beta-deficient samples was 0.2% and 4%, respectively, when compared with zymosan-activated normal serum. TCC was purified from the activated C8beta-deficient samples by affinity chromatography and analysed by immunoblotting and enzyme immunoassay. No C8beta was detected in one TCC preparation, while 7% of the normal level was present in the other. The level of the other terminal components, including that of C8alpha-gamma, was normal. The ability of C8alpha-gamma to promote the assembly of TCC in the presence of a limited amount of C8beta or in the apparent absence of this subunit was confirmed using purified components, by mixing C5b6 and either of the purified C8 subunits together with C7 and C9. These data show that soluble TCC can be formed in C8beta-deficient sera that contain little or no C8beta.
authors.affiliation: Institute of Immunology and Rheumatology, University of Oslo, Norway.
authors.forename: K
authors.initials: K
authors.lastname: Høgåsen
authors.forename: T E
authors.initials: TE
authors.lastname: Mollnes
authors.forename: W
authors.initials: W
authors.lastname: Nürnberger
authors.forename: M
authors.initials: M
authors.lastname: Pausa
authors.forename: Y
authors.initials: Y
authors.lastname: Fukumori
authors.forename: F
authors.initials: F
authors.lastname: Tedesco
mesh_terms: D003182:Complement C5 / Q000032:analysis; D003183:Complement C6 / Q000032:analysis; D003184:Complement C7 / Q000032:analysis; D003185:Complement C8 / Q000032:analysis / Q000172:deficiency*; D003186:Complement C9 / Q000032:analysis; D015938:Complement Membrane Attack Complex / Q000737:chemistry* / Q000276:immunology / Q000302:isolation & purification; D006801:Humans; D007124:Immunoenzyme Techniques; D012995:Solubility; D013997:Time Factors
chemical_list: D003182:Complement C5; D003183:Complement C6; D003184:Complement C7; D003185:Complement C8; D003186:Complement C9; D015938:Complement Membrane Attack Complex