Effect of nephritic factor on C3 and on the terminal pathway of complement in vivo and in vitro.
Plasma samples from patients with nephritic factor (NeF) were examined for their C3 converting activity. C3, C3dg, C5 and the fluid phase terminal complement complex (TCC) were quantified. All patients had evidence of C3 activation with low plasma C3 and high C3dg. Some patients had normal C5 and normal TCC levels, and thus no evidence of terminal pathway activation in vivo; others, with slower C3 conversion in vitro, had low C5 levels with TCC either elevated or in the upper normal range, suggesting in vivo activation of the terminal pathway. These observations were confirmed by in vitro experiments using purified NeFs. It is concluded that considerable activation of C3 may occur in vivo without a simultaneous activation of the terminal pathway, and that NeF is heterogeneous with regard to its ability to activate complement.
D000328:Adult; D002648:Child; D003166:Complement Activating Enzymes / Q000378:metabolism*; D003167:Complement Activation; D003176:Complement C3 / Q000032:analysis; D003178:Complement C3 Nephritic Factor / Q000378:metabolism*; D050577:Complement C3-C5 Convertases / Q000378:metabolism*; D003182:Complement C5 / Q000032:analysis; D003169:Complement Inactivator Proteins / Q000378:metabolism*; D015938:Complement Membrane Attack Complex; D003165:Complement System Proteins / Q000378:metabolism*; D005260:Female; D005921:Glomerulonephritis / Q000276:immunology; D006801:Humans; D066298:In Vitro Techniques; D008060:Lipodystrophy / Q000276:immunology; D008297:Male; D008875:Middle Aged
D003176:Complement C3; D003178:Complement C3 Nephritic Factor; D003182:Complement C5; D003169:Complement Inactivator Proteins; D015938:Complement Membrane Attack Complex; D003165:Complement System Proteins; D003166:Complement Activating Enzymes; D050577:Complement C3-C5 Convertases