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. 1982 May 1;155(5):1427–1438. doi: 10.1084/jem.155.5.1427

Complement receptor (CR1) deficiency in erythrocytes from patients with systemic lupus erythematosus

PMCID: PMC2186668  PMID: 6978375

Abstract

This study reports quantitative information on the concentration of complement receptor for C3b and C4b (CR1) on erythrocytes from normal individuals and patients with immune complex disease. The measurements were performed by an immunoradiometric assay using monoclonal antibodies against CR1. The antibody specificity was confirmed by immunoprecipitation of CR1 from extracts of surface-labeled cells, by inhibition of rosette formation between B lymphocytes and the erythrocytes intermediate EAC14oxy23b, and by the characteristic distribution of the antigen among cells of human peripheral blood. The number of CR1 molecules in erythrocytes from 52 normal individuals was estimated as 1,410 +/- 620. No significant differences in CR1 levels were observed when individuals were grouped by sex, age, or blood groups. In patients with SLE and rheumatoid arthritis, the number of CR1 molecules per RBC was significantly lower, i.e., 600 +/- 307 and 903 +/- 417, respectively. CR1 levels were normal in asthmatics undergoing long-term treatment with prednisone. In SLE patients, significant correlations were found between CR1 levels, C4 hemolytic titers, and levels of circulating immune complexes. In two out of four patients with SLE, CR1 levels increased significantly during remission, showing that the deficiency is, at least in part, reversible. The deficiency in CR1 could be genetically controlled or could represent an epiphenomenon caused by the interaction of the receptor with a ligand present in the circulation of patients.

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Selected References

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  1. Arend W. P., Mannik M. Studies on antigen-antibody complexes. II. Quantification of tissue uptake of soluble complexes in normal and complement-depleted rabbits. J Immunol. 1971 Jul;107(1):63–75. [PubMed] [Google Scholar]
  2. Bianco C., Nussenzweig V. Complement receptors. Contemp Top Mol Immunol. 1977;6:145–176. doi: 10.1007/978-1-4684-2841-4_5. [DOI] [PubMed] [Google Scholar]
  3. Bolotin C., Morris S., Tack B., Prahl J. Purification and structural analysis of the fourth component of human complement. Biochemistry. 1977 May 3;16(9):2008–2015. doi: 10.1021/bi00628a039. [DOI] [PubMed] [Google Scholar]
  4. Cooper N. R. Immune adherence by the fourth component of complement. Science. 1969 Jul 25;165(3891):396–398. doi: 10.1126/science.165.3891.396. [DOI] [PubMed] [Google Scholar]
  5. Crossley L. G., Porter R. R. Purification of the human complement control protein C3b inactivator. Biochem J. 1980 Oct 1;191(1):173–182. doi: 10.1042/bj1910173. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Dobson N. J., Lambris J. D., Ross G. D. Characteristics of isolated erythrocyte complement receptor type one (CR1, C4b-C3b receptor) and CR1-specific antibodies. J Immunol. 1981 Feb;126(2):693–698. [PubMed] [Google Scholar]
  7. Fearon D. T. Identification of the membrane glycoprotein that is the C3b receptor of the human erythrocyte, polymorphonuclear leukocyte, B lymphocyte, and monocyte. J Exp Med. 1980 Jul 1;152(1):20–30. doi: 10.1084/jem.152.1.20. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Fearon D. T., Kaneko I., Thomson G. G. Membrane distribution and adsorptive endocytosis by C3b receptors on human polymorphonuclear leukocytes. J Exp Med. 1981 Jun 1;153(6):1615–1628. doi: 10.1084/jem.153.6.1615. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Fearon D. T. Regulation of the amplification C3 convertase of human complement by an inhibitory protein isolated from human erythrocyte membrane. Proc Natl Acad Sci U S A. 1979 Nov;76(11):5867–5871. doi: 10.1073/pnas.76.11.5867. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Frank M. M., Hamburger M. I., Lawley T. J., Kimberly R. P., Plotz P. H. Defective reticuloendothelial system Fc-receptor function in systemic lupus erythematosus. N Engl J Med. 1979 Mar 8;300(10):518–523. doi: 10.1056/NEJM197903083001002. [DOI] [PubMed] [Google Scholar]
  11. 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]
  12. Julius M. H., Simpson E., Herzenberg L. A. A rapid method for the isolation of functional thymus-derived murine lymphocytes. Eur J Immunol. 1973 Oct;3(10):645–649. doi: 10.1002/eji.1830031011. [DOI] [PubMed] [Google Scholar]
  13. Köhler G., Milstein C. Continuous cultures of fused cells secreting antibody of predefined specificity. Nature. 1975 Aug 7;256(5517):495–497. doi: 10.1038/256495a0. [DOI] [PubMed] [Google Scholar]
  14. Laemmli U. K. Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature. 1970 Aug 15;227(5259):680–685. doi: 10.1038/227680a0. [DOI] [PubMed] [Google Scholar]
  15. Miyakawa Y., Yamada A., Kosaka K., Tsuda F., Kosugi E., Mayumi M. Defective immune-adherence (C3b) receptor on erythrocytes from patients with systemic lupus erythematosus. Lancet. 1981 Sep 5;2(8245):493–497. doi: 10.1016/s0140-6736(81)90882-5. [DOI] [PubMed] [Google Scholar]
  16. NELSON R. A., Jr The immune-adherence phenomenon; a hypothetical role of erythrocytes in defence against bacteria and viruses. Proc R Soc Med. 1956 Jan;49(1):55–58. [PMC free article] [PubMed] [Google Scholar]
  17. NELSON R. A., Jr The immune-adherence phenomenon; an immunologically specific reaction between microorganisms and erythrocytes leading to enhanced phagocytosis. Science. 1953 Dec 18;118(3077):733–737. doi: 10.1126/science.118.3077.733. [DOI] [PubMed] [Google Scholar]
  18. NIH conference. Pathophysiology of immune hemolytic anemia. Ann Intern Med. 1977 Aug;87(2):210–222. doi: 10.7326/0003-4819-87-2-210. [DOI] [PubMed] [Google Scholar]
  19. Nagasawa S., Stroud R. M. Cleavage of C2 by C1s into the antigenically distinct fragments C2a and C2b: demonstration of binding of C2b to C4b. Proc Natl Acad Sci U S A. 1977 Jul;74(7):2998–3001. doi: 10.1073/pnas.74.7.2998. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Nelson R. A., Jr, Jensen J., Gigli I., Tamura N. Methods for the separation, purification and measurement of nine components of hemolytic complement in guinea-pig serum. Immunochemistry. 1966 Mar;3(2):111–135. doi: 10.1016/0019-2791(66)90292-8. [DOI] [PubMed] [Google Scholar]
  21. Okuda T., Tachibana T. Complement receptors on Raji cells. The presence of a new type of C3 receptor. Immunology. 1980 Sep;41(1):159–166. [PMC free article] [PubMed] [Google Scholar]
  22. PULVERTAFT J. V. A STUDY OF MALIGNANT TUMOURS IN NIGERIA BY SHORT-TERM TISSUE CULTURE. J Clin Pathol. 1965 May;18:261–273. doi: 10.1136/jcp.18.3.261. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. Potocnjak P., Yoshida N., Nussenzweig R. S., Nussenzweig V. Monovalent fragments (Fab) of monoclonal antibodies to a sporozoite surface antigen (Pb44) protect mice against malarial infection. J Exp Med. 1980 Jun 1;151(6):1504–1513. doi: 10.1084/jem.151.6.1504. [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Ross G. D., Polley M. J. Specificity of human lymphocyte complement receptors. J Exp Med. 1975 May 1;141(5):1163–1180. doi: 10.1084/jem.141.5.1163. [DOI] [PMC free article] [PubMed] [Google Scholar]
  25. Schmitt M., Mussel H. H., Dierich M. P. Qualitative and quantitative assessment of C3-receptor reactivities on lymphoid and phagocytic cells. J Immunol. 1981 May;126(5):2042–2047. [PubMed] [Google Scholar]
  26. Segal A. W., Fortunato A., Herd T. A rapid single centrifugation step method for the separation of erythrocytes, granulocytes and mononuclear cells on continuous density gradients of Percoll. J Immunol Methods. 1980;32(3):209–214. doi: 10.1016/0022-1759(80)90186-6. [DOI] [PubMed] [Google Scholar]
  27. Shulman M., Wilde C. D., Köhler G. A better cell line for making hybridomas secreting specific antibodies. Nature. 1978 Nov 16;276(5685):269–270. doi: 10.1038/276269a0. [DOI] [PubMed] [Google Scholar]
  28. Siegel I., Liu T. L., Gleicher N. The red-cell immune system. Lancet. 1981 Sep 12;2(8246):556–559. doi: 10.1016/s0140-6736(81)90941-7. [DOI] [PubMed] [Google Scholar]
  29. Sobel A. T., Bokisch V. A., Müller-Eberhard H. J. C1q deviation test for the detection of immune complexes, aggregates of IgG, and bacterial products in human serum. J Exp Med. 1975 Jul 1;142(1):139–150. doi: 10.1084/jem.142.1.139. [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. Tack B. D., Prahl J. W. Third component of human complement: purification from plasma and physicochemical characterization. Biochemistry. 1976 Oct 5;15(20):4513–4521. doi: 10.1021/bi00665a028. [DOI] [PubMed] [Google Scholar]
  31. Theofilopoulos A. N., Wilson C. B., Dixon F. J. The Raji cell radioimmune assay for detecting immune complexes in human sera. J Clin Invest. 1976 Jan;57(1):169–182. doi: 10.1172/JCI108257. [DOI] [PMC free article] [PubMed] [Google Scholar]
  32. Wysocki L. J., Sato V. L. "Panning" for lymphocytes: a method for cell selection. Proc Natl Acad Sci U S A. 1978 Jun;75(6):2844–2848. doi: 10.1073/pnas.75.6.2844. [DOI] [PMC free article] [PubMed] [Google Scholar]

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