Abstract
Fluid phase C3 conversion by C3 nephritic factor (C3NeF) is usually easily detectable and forms the basis of the C3NeF screening test. We have described a group of patients with membranoproliferative glomerulonephritis or partial lipodystrophy and hypocomplementaemia who have an unusual C3NeF which stabilizes cell-bound C3 convertase of the alternative pathway (C3bBb) but causes such weak fluid phase C3 conversion that a C3NeF screening test is negative. These patients have low concentrations of C5 in serum.
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Selected References
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- Cameron J. S., Turner D. R., Heaton J., Williams D. G., Ogg C. S., Chantler C., Haycock G. B., Hicks J. Idiopathic mesangiocapillary glomerulonephritis. Comparison of types I and II in children and adults and long-term prognosis. Am J Med. 1983 Feb;74(2):175–192. doi: 10.1016/0002-9343(83)90606-x. [DOI] [PubMed] [Google Scholar]
- Curtis J. J., Wyatt R. J., Bhathena D., Lucas B. A., Holland N. H., Luke R. G. Renal transplantation for patients with type I and type II membranoproliferative glomerulonephritis: serial complement and nephritic factor measurements and the problem of recurrence of disease. Am J Med. 1979 Feb;66(2):216–225. doi: 10.1016/0002-9343(79)90530-8. [DOI] [PubMed] [Google Scholar]
- Daha M. R., Austen K. F., Fearon D. T. Heterogeneity, polypeptide chain composition and antigenic reactivity of C3 nephritic factor. J Immunol. 1978 Apr;120(4):1389–1394. [PubMed] [Google Scholar]
- Daha M. R., Fearon D. T., Austen K. F. C3 nephritic factor (C3NeF): stabilization of fluid phase and cell-bound alternative pathway convertase. J Immunol. 1976 Jan;116(1):1–7. [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]
- Davis A. E., 3rd, Ziegler J. B., Gelfand E. W., Rosen F. S., Alper C. A. Heterogeneity of nephritic factor and its identification as an immunoglobulin. Proc Natl Acad Sci U S A. 1977 Sep;74(9):3980–3983. doi: 10.1073/pnas.74.9.3980. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Isenman D. E., Podack E. R., Cooper N. R. The interaction of C5 with C3b in free solution: a sufficient condition for cleavage by a fluid phase C3/C5 convertase. J Immunol. 1980 Jan;124(1):326–331. [PubMed] [Google Scholar]
- Perrin L. H., Lambert P. H., Miescher P. A. Complement breakdown products in plasma from patients with systemic lupus erythematosus and patients with membranoproliferative or other glomerulonephritis. J Clin Invest. 1975 Jul;56(1):165–176. doi: 10.1172/JCI108065. [DOI] [PMC free article] [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]
- Schena F. P., Pertosa G., Stanziale P., Vox E., Pecoraro C., Andreucci V. E. Biological significance of the C3 nephritic factor in membranoproliferative glomerulonephritis. Clin Nephrol. 1982 Nov;18(5):240–246. [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]
- Sissons J. G., West R. J., Fallows J., Williams D. G., Boucher B. J., Amos N., Peters D. K. The complement abnormalities of lipodystrophy. N Engl J Med. 1976 Feb 26;294(9):461–465. doi: 10.1056/NEJM197602262940902. [DOI] [PubMed] [Google Scholar]
- Spitzer R. E., Vallota E. H., Forristal J., Sudora E., Stitzel A., Davis N. C., West C. D. Serum C'3 lytic system in patients with glomerulonephritis. Science. 1969 Apr 25;164(3878):436–437. doi: 10.1126/science.164.3878.436. [DOI] [PubMed] [Google Scholar]
- Vallota E. H., Forristal J., Davis N. C., West C. D. The C3 nephritic factor and membranoproliferative nephritis: correlation of serum levels of the nephritic factor with C3 levels, with therapy, and with progression of the disease. J Pediatr. 1972 Jun;80(6):947–959. doi: 10.1016/s0022-3476(72)80006-4. [DOI] [PubMed] [Google Scholar]
- Vogt W., Schmidt G., Von Buttlar B., Dieminger L. A new function of the activated third component of complement: binding to C5, an essential step for C5 activation. Immunology. 1978 Jan;34(1):29–40. [PMC free article] [PubMed] [Google Scholar]