Abstract
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.
Full text
PDF






Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Alper C. A., Balavitch D. Cobra venom factor: evidence for its being altered cobra C3 (the third component of complement). Science. 1976 Mar 26;191(4233):1275–1276. doi: 10.1126/science.56780. [DOI] [PubMed] [Google Scholar]
- Arroyave C. M., Vallota E. H., Müller-Eberhard H. J. Lysis of human erythrocytes due to activation of the alternate complement pathway by nephritic factor (C3NeF). J Immunol. 1974 Sep;113(3):764–768. [PubMed] [Google Scholar]
- Bhakdi S., Tranum-Jensen J. Membrane damage by complement. Biochim Biophys Acta. 1983 Aug 11;737(3-4):343–372. doi: 10.1016/0304-4157(83)90006-0. [DOI] [PubMed] [Google Scholar]
- Daha M. R., Deelder A. M., Van Es L. A. Stabilization of the amplification convertase of complement by monoclonal antibodies directed against human factor B. J Immunol. 1984 May;132(5):2538–2542. [PubMed] [Google Scholar]
- Daha M. R., Kok D. J., Van Es L. A. Regulation of the C3 nephritic factor stabilized C3/C5 convertase of complement by purified human erythrocyte C3b receptor. Clin Exp Immunol. 1982 Oct;50(1):209–214. [PMC free article] [PubMed] [Google Scholar]
- Groggel G. C., Adler S., Rennke H. G., Couser W. G., Salant D. J. Role of the terminal complement pathway in experimental membranous nephropathy in the rabbit. J Clin Invest. 1983 Dec;72(6):1948–1957. doi: 10.1172/JCI111159. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Iida K., Nussenzweig V. Complement receptor is an inhibitor of the complement cascade. J Exp Med. 1981 May 1;153(5):1138–1150. doi: 10.1084/jem.153.5.1138. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Mollnes T. E. Early- and late-phase activation of complement evaluated by plasma levels of C3d,g and the terminal complement complex. Complement. 1985;2(2-3):156–164. doi: 10.1159/000467856. [DOI] [PubMed] [Google Scholar]
- Mollnes T. E., Lea T., Frøland S. S., Harboe M. Quantification of the terminal complement complex in human plasma by an enzyme-linked immunosorbent assay based on monoclonal antibodies against a neoantigen of the complex. Scand J Immunol. 1985 Aug;22(2):197–202. doi: 10.1111/j.1365-3083.1985.tb01871.x. [DOI] [PubMed] [Google Scholar]
- Mollnes T. E., Lea T., Harboe M. Detection and quantification of the terminal C5b-9 complex of human complement by a sensitive enzyme-linked immunosorbent assay. Scand J Immunol. 1984 Aug;20(2):157–166. doi: 10.1111/j.1365-3083.1984.tb00989.x. [DOI] [PubMed] [Google Scholar]
- Mollnes T. E. Quantification of the C3d split products of human complement by a sensitive enzyme-linked immunosorbent assay. Scand J Immunol. 1985 Jun;21(6):607–613. doi: 10.1111/j.1365-3083.1985.tb01851.x. [DOI] [PubMed] [Google Scholar]
- Müller-Eberhard H. J., Schreiber R. D. Molecular biology and chemistry of the alternative pathway of complement. Adv Immunol. 1980;29:1–53. doi: 10.1016/s0065-2776(08)60042-5. [DOI] [PubMed] [Google Scholar]
- Peters D. K., Martin A., Weinstein A., Cameron J. S., Barratt T. M., Ogg C. S., Lachmann P. J. Complement studies in membrano-proliferative glomerulonephritis. Clin Exp Immunol. 1972 Jul;11(3):311–320. [PMC free article] [PubMed] [Google Scholar]
- Podack E. R., Kolb W. P., Müller-Eberhard H. J. The SC5b-7 complex: formation, isolation, properties, and subunit composition. J Immunol. 1977 Dec;119(6):2024–2029. [PubMed] [Google Scholar]
- Podack E. R., Müller-Eberhard H. J. SC5b-9 complex of complement: formation of the dimeric membrane attack complex by removal of S-protein. J Immunol. 1980 Apr;124(4):1779–1783. [PubMed] [Google Scholar]
- Salama A., Bhakdi S., Mueller-Eckhardt C., Kayser W. Deposition of the terminal C5b-9 complement complex on erythrocytes by human red cell autoantibodies. Br J Haematol. 1983 Sep;55(1):161–169. doi: 10.1111/j.1365-2141.1983.tb01234.x. [DOI] [PubMed] [Google Scholar]
- Scott D. M., Amos N., Sissons J. G., Lachmann P. J., Peters D. K. The immunogloblin nature of nephritic factor (NeF). Clin Exp Immunol. 1978 Apr;32(1):12–24. [PMC free article] [PubMed] [Google Scholar]
- Sissons J. G., Liebowitch J., Amos N., Peters D. K. Metabolism of the fifth component of complement, and its relation to metabolism of the third component, in patients with complement activation. J Clin Invest. 1977 Apr;59(4):704–715. doi: 10.1172/JCI108689. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Thompson R. A. C3 inactivating factor in the serum of a patient with chronic hypocomplementaemic proliferative glomerulo-nephritis. Immunology. 1972 Jan;22(1):147–158. [PMC free article] [PubMed] [Google Scholar]
- Tranum-Jensen J., Bhakdi S., Bhakdi-Lehnen B., Bjerrum O. J., Speth V. Complement lysis: the ultrastructure and orientation of the C5b-9 complex on target sheep erythrocyte membranes. Scand J Immunol. 1978;7(1):45–46. doi: 10.1111/j.1365-3083.1978.tb00425.x. [DOI] [PubMed] [Google Scholar]
- Williams D. G., Peters D. K., Fallows J., Petrie A., Kourilsky O., Morel-Maroger L., Cameron J. S. Studies of serum complement in the hypocomplementaemic nephritides. Clin Exp Immunol. 1974 Nov;18(3):391–405. [PMC free article] [PubMed] [Google Scholar]