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.
journal_name
Scand J Immunoljournal_title
Scandinavian journal of immunologyauthors
Høgåsen K,Mollnes TE,Nürnberger W,Pausa M,Fukumori Y,Tedesco Fdoi
10.1046/j.1365-3083.1998.00390.xsubject
Has Abstractpub_date
1998-09-01 00:00:00pages
261-8issue
3eissn
0300-9475issn
1365-3083journal_volume
48pub_type
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