Skip to main content
PMC home page
The Journal of Experimental Medicine logoLink to The Journal of Experimental Medicine
. 1988 Feb 1;167(2):664–669. doi: 10.1084/jem.167.2.664

A physical map of the human regulator of complement activation gene cluster linking the complement genes CR1, CR2, DAF, and C4BP

PMCID: PMC2188826  PMID: 2450163

Abstract

We report the organization of the human genes encoding the complement components C4-binding protein (C4BP), C3b/C4b receptor (CR1), decay accelerating factor (DAF), and C3dg receptor (CR2) within the regulator of complement activation (RCA) gene cluster. Using pulsed field gel electrophoresis analysis these genes have been physically linked and aligned as CR1-CR2-DAF-C4BP in an 800-kb DNA segment. The very tight linkage between the CR1 and the C4BP loci, contrasted with the relative long DNA distance between these genes, suggests the existence of mechanisms interfering with recombination within the RCA gene cluster.

Full Text

The Full Text of this article is available as a PDF (439.7 KB).

Selected References

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

  1. Caras I. W., Davitz M. A., Rhee L., Weddell G., Martin D. W., Jr, Nussenzweig V. Cloning of decay-accelerating factor suggests novel use of splicing to generate two proteins. Nature. 1987 Feb 5;325(6104):545–549. doi: 10.1038/325545a0. [DOI] [PubMed] [Google Scholar]
  2. Carroll M. C., Belt K. T., Palsdottir A., Yu Y. Molecular genetics of the fourth component of human complement and steroid 21-hydroxylase. Immunol Rev. 1985 Oct;87:39–60. doi: 10.1111/j.1600-065x.1985.tb01144.x. [DOI] [PubMed] [Google Scholar]
  3. Chu G., Vollrath D., Davis R. W. Separation of large DNA molecules by contour-clamped homogeneous electric fields. Science. 1986 Dec 19;234(4783):1582–1585. doi: 10.1126/science.3538420. [DOI] [PubMed] [Google Scholar]
  4. Chung L. P., Bentley D. R., Reid K. B. Molecular cloning and characterization of the cDNA coding for C4b-binding protein, a regulatory protein of the classical pathway of the human complement system. Biochem J. 1985 Aug 15;230(1):133–141. doi: 10.1042/bj2300133. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Feinberg A. P., Vogelstein B. A technique for radiolabeling DNA restriction endonuclease fragments to high specific activity. Anal Biochem. 1983 Jul 1;132(1):6–13. doi: 10.1016/0003-2697(83)90418-9. [DOI] [PubMed] [Google Scholar]
  6. Kenwrick S., Patterson M., Speer A., Fischbeck K., Davies K. Molecular analysis of the Duchenne muscular dystrophy region using pulsed field gel electrophoresis. Cell. 1987 Jan 30;48(2):351–357. doi: 10.1016/0092-8674(87)90438-7. [DOI] [PubMed] [Google Scholar]
  7. Kristensen T., D'Eustachio P., Ogata R. T., Chung L. P., Reid K. B., Tack B. F. The superfamily of C3b/C4b-binding proteins. Fed Proc. 1987 May 15;46(7):2463–2469. [PubMed] [Google Scholar]
  8. Kristensen T., Ogata R. T., Chung L. P., Reid K. B., Tack B. F. cDNA structure of murine C4b-binding protein, a regulatory component of the serum complement system. Biochemistry. 1987 Jul 28;26(15):4668–4674. doi: 10.1021/bi00389a012. [DOI] [PubMed] [Google Scholar]
  9. Kristensen T., Wetsel R. A., Tack B. F. Structural analysis of human complement protein H: homology with C4b binding protein, beta 2-glycoprotein I, and the Ba fragment of B2. J Immunol. 1986 May 1;136(9):3407–3411. [PubMed] [Google Scholar]
  10. Lublin D. M., Lemons R. S., Le Beau M. M., Holers V. M., Tykocinski M. L., Medof M. E., Atkinson J. P. The gene encoding decay-accelerating factor (DAF) is located in the complement-regulatory locus on the long arm of chromosome 1. J Exp Med. 1987 Jun 1;165(6):1731–1736. doi: 10.1084/jem.165.6.1731. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Porter R. R. The complement components coded in the major histocompatibility complexes and their biological activities. Immunol Rev. 1985 Oct;87:7–17. doi: 10.1111/j.1600-065x.1985.tb01142.x. [DOI] [PubMed] [Google Scholar]
  12. Rey-Campos J., Rubinstein P., Rodriguez de Cordoba S. Decay-accelerating factor. Genetic polymorphism and linkage to the RCA (regulator of complement activation) gene cluster in humans. J Exp Med. 1987 Jul 1;166(1):246–252. doi: 10.1084/jem.166.1.246. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Rodriguez de Cordoba S., Dykman T. R., Ginsberg-Fellner F., Ercilla G., Aqua M., Atkinson J. P., Rubinstein P. Evidence for linkage between the loci coding for the binding protein for the fourth component of human complement (C4BP) and for the C3b/C4b receptor. Proc Natl Acad Sci U S A. 1984 Dec;81(24):7890–7892. doi: 10.1073/pnas.81.24.7890. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Rodriguez de Cordoba S., Lublin D. M., Rubinstein P., Atkinson J. P. Human genes for three complement components that regulate the activation of C3 are tightly linked. J Exp Med. 1985 May 1;161(5):1189–1195. doi: 10.1084/jem.161.5.1189. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Rodriguez de Cordoba S., Rubinstein P. New alleles of C4-binding protein and factor H and further linkage data in the regulator of complement activation (RCA) gene cluster in man. Immunogenetics. 1987;25(4):267–268. doi: 10.1007/BF00404698. [DOI] [PubMed] [Google Scholar]
  16. Weis J. H., Morton C. C., Bruns G. A., Weis J. J., Klickstein L. B., Wong W. W., Fearon D. T. A complement receptor locus: genes encoding C3b/C4b receptor and C3d/Epstein-Barr virus receptor map to 1q32. J Immunol. 1987 Jan 1;138(1):312–315. [PubMed] [Google Scholar]
  17. Weis J. J., Fearon D. T., Klickstein L. B., Wong W. W., Richards S. A., de Bruyn Kops A., Smith J. A., Weis J. H. Identification of a partial cDNA clone for the C3d/Epstein-Barr virus receptor of human B lymphocytes: homology with the receptor for fragments C3b and C4b of the third and fourth components of complement. Proc Natl Acad Sci U S A. 1986 Aug;83(15):5639–5643. doi: 10.1073/pnas.83.15.5639. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Wong W. W., Kennedy C. A., Bonaccio E. T., Wilson J. G., Klickstein L. B., Weis J. H., Fearon D. T. Analysis of multiple restriction fragment length polymorphisms of the gene for the human complement receptor type I. Duplication of genomic sequences occurs in association with a high molecular mass receptor allotype. J Exp Med. 1986 Nov 1;164(5):1531–1546. doi: 10.1084/jem.164.5.1531. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Wong W. W., Klickstein L. B., Smith J. A., Weis J. H., Fearon D. T. Identification of a partial cDNA clone for the human receptor for complement fragments C3b/C4b. Proc Natl Acad Sci U S A. 1985 Nov;82(22):7711–7715. doi: 10.1073/pnas.82.22.7711. [DOI] [PMC free article] [PubMed] [Google Scholar]

Articles from The Journal of Experimental Medicine are provided here courtesy of The Rockefeller University Press

RESOURCES