Skip to main content
PMC home page
Clinical and Experimental Immunology logoLink to Clinical and Experimental Immunology
. 1985 Mar;59(3):547–554.

Family studies of erythrocyte complement receptor type 1 levels: reduced levels in patients with SLE are acquired, not inherited.

M J Walport, G D Ross, C Mackworth-Young, J V Watson, N Hogg, P J Lachmann
PMCID: PMC1576941  PMID: 3987091

Abstract

It has been claimed that patients with systemic lupus erythematosus (SLE) have an inherited deficiency of erythrocyte complement receptor type 1 (CR1, with ligand binding specificity for C3b, iC3b and C4b). CR1 functions as the only cofactor for factor I-mediated cleavage of iC3b to C3c and C3dg. The activity of this receptor on red cells may be an important mechanism for handling immune complexes which have bound C3b or iC3b. Radioligand binding studies were performed using a monoclonal antibody to CR1, E11, to enumerate these receptors accurately. The results confirmed that patients with SLE have a reduced number of CR1 molecules per red cell, but showed no reduction in CR1 levels amongst their consanguineous relatives. Study of 13 normal families suggested the presence of heritable factors controlling the numbers of erythrocyte CR1 molecules; in particular there was a correlation between mean parental CR1 numbers and CR1 numbers in their children. However, amongst 17 families of 19 patients with SLE, four families were identified in which genotypically 'high CR1' SLE patients had persistently low phenotypes. This is not compatible with the hypothesis that the reduction in erythrocyte CR1 numbers in these patients is inherited.

Full text

PDF
547

Selected References

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

  1. 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]
  2. Dykman T. R., Cole J. L., Iida K., Atkinson J. P. Polymorphism of human erythrocyte C3b/C4b receptor. Proc Natl Acad Sci U S A. 1983 Mar;80(6):1698–1702. doi: 10.1073/pnas.80.6.1698. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Dykman T. R., Hatch J. A., Atkinson J. P. Polymorphism of the human C3b/C4b receptor. Identification of a third allele and analysis of receptor phenotypes in families and patients with systemic lupus erythematosus. J Exp Med. 1984 Mar 1;159(3):691–703. doi: 10.1084/jem.159.3.691. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Fraker P. J., Speck J. C., Jr Protein and cell membrane iodinations with a sparingly soluble chloroamide, 1,3,4,6-tetrachloro-3a,6a-diphrenylglycoluril. Biochem Biophys Res Commun. 1978 Feb 28;80(4):849–857. doi: 10.1016/0006-291x(78)91322-0. [DOI] [PubMed] [Google Scholar]
  5. Frank M. M., Lawley T. J., Hamburger M. I., Brown E. J. NIH Conference: Immunoglobulin G Fc receptor-mediated clearance in autoimmune diseases. Ann Intern Med. 1983 Feb;98(2):206–218. doi: 10.7326/0003-4819-98-2-218. [DOI] [PubMed] [Google Scholar]
  6. Hogg N., Ross G. D., Jones D. B., Slusarenko M., Walport M. J., Lachmann P. J. Identification of an anti-monocyte monoclonal antibody that is specific for membrane complement receptor type one (CR1). Eur J Immunol. 1984 Mar;14(3):236–243. doi: 10.1002/eji.1830140307. [DOI] [PubMed] [Google Scholar]
  7. Iida K., Mornaghi R., Nussenzweig V. Complement receptor (CR1) deficiency in erythrocytes from patients with systemic lupus erythematosus. J Exp Med. 1982 May 1;155(5):1427–1438. doi: 10.1084/jem.155.5.1427. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Inada Y., Kamiyama M., Kanemitsu T., Hyman C. L., Clark W. S. Studies on immune adherence (C3b) receptor activity of human erythrocytes: relationship between receptor activity and presence of immune complexes in serum. Clin Exp Immunol. 1982 Oct;50(1):189–197. [PMC free article] [PubMed] [Google Scholar]
  9. Kazatchkine M. D., Fearon D. T., Appay M. D., Mandet C., Bariety J. Immunohistochemical study of the human glomerular C3b receptor in normal kidney and in seventy-five cases of renal diseases: loss of C3b receptor antigen in focal hyalinosis and in proliferative nephritis of systemic lupus erythematosus. J Clin Invest. 1982 Apr;69(4):900–912. doi: 10.1172/JCI110529. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Lachmann P. J. Inherited complement deficiencies. Philos Trans R Soc Lond B Biol Sci. 1984 Sep 6;306(1129):419–430. doi: 10.1098/rstb.1984.0102. [DOI] [PubMed] [Google Scholar]
  11. 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]
  12. Schifferli J. A., Woo P., Peters D. K. Complement-mediated inhibition of immune precipitation. I. Role of the classical and alternative pathways. Clin Exp Immunol. 1982 Mar;47(3):555–562. [PMC free article] [PubMed] [Google Scholar]
  13. Steinbuch M., Audran R. The isolation of IgG from mammalian sera with the aid of caprylic acid. Arch Biochem Biophys. 1969 Nov;134(2):279–284. doi: 10.1016/0003-9861(69)90285-9. [DOI] [PubMed] [Google Scholar]
  14. Tan E. M., Cohen A. S., Fries J. F., Masi A. T., McShane D. J., Rothfield N. F., Schaller J. G., Talal N., Winchester R. J. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982 Nov;25(11):1271–1277. doi: 10.1002/art.1780251101. [DOI] [PubMed] [Google Scholar]
  15. Watson J. V. Enzyme kinetic studies in cell populations using fluorogenic substrates and flow cytometric techniques. Cytometry. 1980 Sep;1(2):143–151. doi: 10.1002/cyto.990010209. [DOI] [PubMed] [Google Scholar]
  16. Wilson J. G., Wong W. W., Schur P. H., Fearon D. T. Mode of inheritance of decreased C3b receptors on erythrocytes of patients with systemic lupus erythematosus. N Engl J Med. 1982 Oct 14;307(16):981–986. doi: 10.1056/NEJM198210143071604. [DOI] [PubMed] [Google Scholar]
  17. Wong W. W., Wilson J. G., Fearon D. T. Genetic regulation of a structural polymorphism of human C3b receptor. J Clin Invest. 1983 Aug;72(2):685–693. doi: 10.1172/JCI111018. [DOI] [PMC free article] [PubMed] [Google Scholar]

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

RESOURCES