Skip to main content
PMC home page
Clinical and Experimental Immunology logoLink to Clinical and Experimental Immunology
. 1980 Dec;42(3):495–505.

Evaluation of alternative pathway and factor B haemolytic activities in patients with systemic lupus erythematosus: correlations with the alternative pathway regulatory proteins.

M T Aguado, L H Perrin, R Ramirez, P A Miescher, P H Lambert
PMCID: PMC1537163  PMID: 6163579

Abstract

The haemolytic activities of the complement alternative pathway and factor B were studied using a 51Cr-release assay in 77 sera from patients with systemic lupus erythematosus (SLE). These values were compared to those of several complement components and of the regulatory proteins beta H and C3b INA. There was a significant decrease in the alternative pathway activity, which correlated with a decrease in the haemolytic activity of factor B and with the activity of the classical pathway. There was a significant decrease in the level of one regulatory protein beta 1H, while the level of the other regulatory protein, C3b INA, was increased; however, there was a positive correlation between these two parameters. The results obtained suggest that the decreased activity of the alternative pathway observed in SLE reflects a consumption due to a triggering of the C3b amplification loop through the activation of the classical pathway. In one patient, an acquired deficiency of the capacity to activate the alternative pathway in the presence of inulin was observed.

Full text

PDF
495

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Brai M., Osler A. G. Studies of the C3 shunt activation in cobra venom induced lysis of unsensitized erythrocytes. Proc Soc Exp Biol Med. 1972 Jul;140(3):1116–1121. doi: 10.3181/00379727-140-36623. [DOI] [PubMed] [Google Scholar]
  2. Charlesworth J. A., Scott D. M., Pussell B. A., Peters D. K. Metabolism of human beta 1H: studies in man and experimental animals. Clin Exp Immunol. 1979 Dec;38(3):397–404. [PMC free article] [PubMed] [Google Scholar]
  3. Charlesworth J. A., Williams D. G., Sherington E., Lachmann P. J., Peters D. K. Metabolic studies of the third component of complement and the glycine-rich beta glycoprotein in patients with hypocomplementemia. J Clin Invest. 1974 Jun;53(6):1578–1587. doi: 10.1172/JCI107708. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Fearon D. T., Daha M. R., Strom T. B., Weiler J. M., Carpenter C. B., Austen K. F. Pathways of complement activation in membranoproliferative glomerulonephritis and allograft rejection. Transplant Proc. 1977 Mar;9(1):729–739. [PubMed] [Google Scholar]
  5. Fong J. S., Renaud L. A hemolytic plate method for alternative-pathway complement activity assay. Am J Clin Pathol. 1978 Feb;69(2):156–160. doi: 10.1093/ajcp/69.2.156. [DOI] [PubMed] [Google Scholar]
  6. Gewurz H., Pickering R. J., Naff G., Snyderman R., Mergenhagen S. E., Good R. A. Decreased properdin activity in acute glomerulonephritis. Int Arch Allergy Appl Immunol. 1969;36(6):592–598. doi: 10.1159/000230780. [DOI] [PubMed] [Google Scholar]
  7. Hunsicker L. G., Ruddy S., Carpenter C. B., Schur P. H., Merrill J. P., Müller-Eberhard H. J., Austen K. F. Metabolism of third complement component (C3) in nephritis. Involvement of the classic and alternate (properdin) pathways for complement activation. N Engl J Med. 1972 Oct 26;287(17):835–840. doi: 10.1056/NEJM197210262871701. [DOI] [PubMed] [Google Scholar]
  8. Jones B. M. The quantitation of alternative pathway complement function by timed lysis assay. J Immunol Methods. 1979;29(3):287–292. doi: 10.1016/0022-1759(79)90317-x. [DOI] [PubMed] [Google Scholar]
  9. Koffler D., Agnello V., Carr R. I., Kunkel H. G. Variable patterns of immunoglobulin and complement deposition in the kidneys of patients with systemic lupus erythematosus. Am J Pathol. 1969 Sep;56(3):305–316. [PMC free article] [PubMed] [Google Scholar]
  10. Martin A., Lachmann P. J., Halbwachs L., Hobart M. J. Haemolytic diffusion plate assays for factors B and D of the alternative pathway of complement activation. Immunochemistry. 1976 Apr;13(4):317–324. doi: 10.1016/0019-2791(76)90341-4. [DOI] [PubMed] [Google Scholar]
  11. McLean R. H., Michael A. F. Properdin anc C3 proactivator: alternate pathway components in human glomerulonephritis. J Clin Invest. 1973 Mar;52(3):634–644. doi: 10.1172/JCI107225. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Miller G. W., Nussenzweig V. A new complement function: solubilization of antigen-antibody aggregates. Proc Natl Acad Sci U S A. 1975 Feb;72(2):418–422. doi: 10.1073/pnas.72.2.418. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Nicol P. A., Lachmann P. J. The alternate pathway of complement activation. The role of C3 and its inactivator (KAF). Immunology. 1973 Feb;24(2):259–275. [PMC free article] [PubMed] [Google Scholar]
  14. Nydegger U. E., Achermann L. M., Lambert P. H., Miescher P. A. A simple automated method for complement estimation in a continuous flow system. J Immunol. 1972 Oct;109(4):910–913. [PubMed] [Google Scholar]
  15. Nydegger U. E., Fearon D. T., Austen K. F. The modulation of the alternative pathway of complement in C2-deficient human serum by changes in concentration of the component and control proteins. J Immunol. 1978 Apr;120(4):1404–1408. [PubMed] [Google Scholar]
  16. Perrin L. H., Joseph B. S., Cooper N. R., Oldstone M. B. Mechanism of injury of virus-infected cells by antiviral antibody and complement: participation of IgG, F(ab')2, and the alternative complement pathway. J Exp Med. 1976 May 1;143(5):1027–1041. doi: 10.1084/jem.143.5.1027. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. 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]
  18. Perrin L. H., Lambert P. H., Miescher P. A. Properdin levels in systemic lupus erythematosus and membranoproleferative glomerulonephritis. Clin Exp Immunol. 1974 Apr;16(4):575–581. [PMC free article] [PubMed] [Google Scholar]
  19. Perrin L. H., Lambert P. H., Nydegger U. E., Miescher P. A. Quantitation of C3PA (properdin factor B) and other complement components in diseases associated with a low C3 level. Clin Immunol Immunopathol. 1973 Nov;2(1):16–27. doi: 10.1016/0090-1229(73)90032-9. [DOI] [PubMed] [Google Scholar]
  20. Platts-Mills T. A., Ishizaka K. Activation of the alternate pathway of human complements by rabbit cells. J Immunol. 1974 Jul;113(1):348–358. [PubMed] [Google Scholar]
  21. Polhill R. B., Jr, Pruitt K. M., Johnston R. B., Jr Kinetic assessment of alternative complement pathway activity in a hemolytic system. I. Experimental and mathematical analyses. J Immunol. 1978 Jul;121(1):363–370. [PubMed] [Google Scholar]
  22. Takada A., Imamura Y., Takada Y. Relationships between the haemolytic activities of the human complement system and complement components. Clin Exp Immunol. 1979 Feb;35(2):324–328. [PMC free article] [PubMed] [Google Scholar]
  23. Whaley K., Schur P. H., Ruddy S. C3b inactivator in the rheumatic diseases. Measurement by radial immunodiffusion and by inhibition of formation of properdin pathway C3 convertase. J Clin Invest. 1976 Jun;57(6):1554–1563. doi: 10.1172/JCI108426. [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Whaley K., Schur P. H., Ruddy S. Relative importance of C3b inactivator and beta 1H globulin in the modulation of the properdin amplification loop in systemic lupus erythematosus. Clin Exp Immunol. 1979 Jun;36(3):408–414. [PMC free article] [PubMed] [Google Scholar]
  25. Whaley K., Thompson R. A. Requirements for beta1H globulin and C3b inactivator in the control of the alternative complement pathway in human serum. Immunology. 1978 Dec;35(6):1045–1049. [PMC free article] [PubMed] [Google Scholar]
  26. 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]
  27. Ziegler J. B., Rosen F. S., Alper C. A., Grupe W., Lepow I. H. Metabolism of properdin in normal subjects and patients with renal disease. J Clin Invest. 1975 Sep;56(3):761–767. doi: 10.1172/JCI108147. [DOI] [PMC free article] [PubMed] [Google Scholar]
  28. Zubler R. H., Lange G., Lambert P. H., Miescher P. A. Detection of immune complexes in unheated sera by modified 125I-Clq binding test. Effect of heating on the binding of Clq by immune complexes and application of the test to systemic lupus erythematosus. J Immunol. 1976 Jan;116(1):232–235. [PubMed] [Google Scholar]

Articles from Clinical and Experimental Immunology are provided here courtesy of British Society for Immunology

RESOURCES