Abstract
Serological and immunopathological studies of human glomerulonephritis have suggested that alternate pathways of activation of the third component of complement may be important in some forms of glomerulonephritis. We have investigated the role of two alternate pathway proteins, properdin and C3 proactivator, in 22 patients with chronic membranoproliferative glomerulonephritis, 21 patients with systemic lupus erythematosus, 20 patients with acute poststreptococcal glomerulonephritis, and 19 patients with other forms of renal disease. C3 (measured at β1A), properdin, and C3 proactivator were assayed by single radial immunodiffusion.
In sera with low β1A (< 2 SD), mean properdin was most significantly decreased in patients with acute poststreptococcal glomerulonephritis but was also significantly decreased in chronic membranoproliferative glomerulonephritis and in untreated systemic lupus erythematosus. Properdin levels in other renal disease, acute glomerulonephritis, and chronic membranoproliferative glomerulonephritis with normal β1A levels were not significantly different from normal. A positive correlation between β1A and properdin levels in individual sera was present in all diseases except systemic lupus erythematosus.
Serum C3 proactivator was markedly decreased in active systemic lupus erythematosus and there was a positive correlation between β1A and C3 proactivator levels in systemic lupus erythematosus and other renal diseases but not acute poststreptococcal glomerulonephritis.
Properdin in fresh sera from four patients with systemic lupus erythematosus and five with chronic membranoproliferative glomerulonephritis showed increased migration toward the cathode on immunoelectrophoresis, suggesting in vivo change of the properdin molecule.
The observation of reduced serum levels of properdin and C3 proactivator and altered electrophoretic migration of properdin in some patients with glomerulonephritis provide new evidence for participation of these alternate pathway proteins in glomerulonephritis.
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Selected References
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- Alper C. A., Boenisch T., Watson L. Genetic polymorphism in human glycine-rich beta-glycoprotein. J Exp Med. 1972 Jan;135(1):68–80. doi: 10.1084/jem.135.1.68. [DOI] [PMC free article] [PubMed] [Google Scholar]
- BLUM L., PILLEMER L., LEPOW I. H. The properdin system and immunity. XIII. Assay and properties of a heat-labile serum factor (factor B) in the properdin system. Z Immun exp ther. 1959 Oct-Nov;118:349–357. [PubMed] [Google Scholar]
- Ensky J., Hinz C. F., Jr, Todd E. W., Wedgwood R. J., Boyer J. T., Lepow I. H. Properties of highly purified human properdin. J Immunol. 1968 Jan;100(1):142–158. [PubMed] [Google Scholar]
- Fish A. J., Herdman R. C., Michael A. F., Pickering R. J., Good R. A. Epidemic acute glomerulonephritis associated with type 49 streptococcal pyoderma. II. Correlative study of light, immunofluorescent and electron microscopic findings. Am J Med. 1970 Jan;48(1):28–39. doi: 10.1016/0002-9343(70)90095-1. [DOI] [PubMed] [Google Scholar]
- 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]
- Götze O., Müller-Eberhard H. J. Paroxysmal nocturnal hemoglobinuria. Hemolysis initiated by the C3 activator system. N Engl J Med. 1972 Jan 27;286(4):180–184. doi: 10.1056/NEJM197201272860403. [DOI] [PubMed] [Google Scholar]
- Kohler P. F., Ten Bensel R. Serial complement component alterations in acute glomerulonephritis and systemic lupus erythematosus. Clin Exp Immunol. 1969 Feb;4(2):191–202. [PMC free article] [PubMed] [Google Scholar]
- LANGE K., GRAIG F., OBERMAN J., SLOBODY L., OGUR G., LoCASTO F. Changes in serum complement during the course and treatment of glomerulonephritis. AMA Arch Intern Med. 1951 Oct;88(4):433–445. doi: 10.1001/archinte.1951.03810100017002. [DOI] [PubMed] [Google Scholar]
- Lewis E. J., Carpenter C. B., Schur P. H. Serum complement component levels in human glomerulonephritis. Ann Intern Med. 1971 Oct;75(4):555–560. doi: 10.7326/0003-4819-75-4-555. [DOI] [PubMed] [Google Scholar]
- MULLER-EBERHARD H. J., NILSSON U., ARONSSON T. Isolation and characterization of two beta1-glycoproteins of human serum. J Exp Med. 1960 Feb 1;111:201–215. doi: 10.1084/jem.111.2.201. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Mancini G., Carbonara A. O., Heremans J. F. Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry. 1965 Sep;2(3):235–254. doi: 10.1016/0019-2791(65)90004-2. [DOI] [PubMed] [Google Scholar]
- McLean R. H., Michael A. F. The immunoelectrophoretic pattern of properdin in fresh and aged human serum. Proc Soc Exp Biol Med. 1972 Nov;141(2):403–407. doi: 10.3181/00379727-141-36786. [DOI] [PubMed] [Google Scholar]
- Müller-Eberhard H. J., Fjellström K. E. Isolation of the anticomplementary protein from cobra venom and its mode of action on C3. J Immunol. 1971 Dec;107(6):1666–1672. [PubMed] [Google Scholar]
- Müller-Eberhard H. J., Götze O. C3 proactivator convertase and its mode of action. J Exp Med. 1972 Apr 1;135(4):1003–1008. doi: 10.1084/jem.135.4.1003. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Oliveira B., Osler A. G., Siraganian R. P., Sandberg A. L. The biologic activities of guinea pig antibodies. I. Separation of gamma1 and gamma2 immunoglobulins and their participation in allergic reactions of the immediate type. J Immunol. 1970 Feb;104(2):320–328. [PubMed] [Google Scholar]
- PENSKY J., WURZ L., PILLEMER L., LEPOW I. H. The properdin system and immunity. XII. Assay, properties and partial purification of a hydrazine-sensitive serum factor (factor A) in the properdin system. Z Immun exp ther. 1959 Oct-Nov;118:329–348. [PubMed] [Google Scholar]
- PILLEMER L., BLUM L., LEPOW I. H., ROSS O. A., TODD E. W., WARDLAW A. C. The properdin system and immunity. I. Demonstration and isolation of a new serum protein, properdin, and its role in immune phenomena. Science. 1954 Aug 20;120(3112):279–285. doi: 10.1126/science.120.3112.279. [DOI] [PubMed] [Google Scholar]
- SOULIER J. P., MENACHE D. Variations pathologiques du complément et de la properdine chez l'homme. Acta Haematol. 1958 Jul-Oct;20(1-4):260–274. doi: 10.1159/000205492. [DOI] [PubMed] [Google Scholar]
- Sandberg A. L., Götze O., Müller-Eberhard H. J., Osler A. G. Complement utilization by guinea pig 1 and 2-immunoglobulins through the C3 activator system. J Immunol. 1971 Sep;107(3):920–923. [PubMed] [Google Scholar]
- Sandberg A. L., Osler A. G., Shin H. S., Oliveira B. The biologic activities of guinea pig antibodies. II. Modes of complement interaction with gamma 1 and gamma 2-immunoglobulins. J Immunol. 1970 Feb;104(2):329–334. [PubMed] [Google Scholar]
- Vallota E. H., Forristal J., Spitzer R. E., Davis N. C., West C. D. Characteristics of a non-complement-dependent C3-reactive complex formed form factors in nephritic and normal serum. J Exp Med. 1970 Jun 1;131(6):1306–1324. doi: 10.1084/jem.131.6.1306. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Westberg N. G., Naff G. B., Boyer J. T., Michael A. F. Glomerular deposition of properdin in acute and chronic glomerulonephritis with hypocomplementemia. J Clin Invest. 1971 Mar;50(3):642–649. doi: 10.1172/JCI106534. [DOI] [PMC free article] [PubMed] [Google Scholar]