Skip to main content
PMC home page
Proceedings of the National Academy of Sciences of the United States of America logoLink to Proceedings of the National Academy of Sciences of the United States of America
. 1984 Dec;81(24):7890–7892. doi: 10.1073/pnas.81.24.7890

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.

S Rodriguez de Cordoba, T R Dykman, F Ginsberg-Fellner, G Ercilla, M Aqua, J P Atkinson, P Rubinstein
PMCID: PMC392258  PMID: 6240062

Abstract

Three pedigrees informative for the segregation of genetic variants of the binding protein for the fourth component of complement (C4BP) and C3b/C4b receptor (C3bR) have been identified. There were 10 informative meioses with no recombinants, indicating a close linkage between the loci encoding C4BP and C3bR, C4BP and C3bR [maximum lod (logarithm of odds of linkage) score: 2.4 at recombinant fraction = 0.0]. In addition, in the four unrelated individuals who were doubly heterozygous (C4BP*1, C4BP*2, C3bR*A, C3bR*B), the infrequent allele C4BP*2 segregated together with the uncommon allele C3bR*B, supporting the hypothesis of linkage between C4BP and C3bR and suggesting that linkage disequilibrium exists between these particular alleles. We conclude that the loci encoding C3bR and C4BP, two functionally related molecules, are linked.

Full text

PDF
7891

Images in this article

7891Image
on p.7891
7891Image
on p.7891

Selected References

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

  1. Allen F. H., Jr Linkage of HL-A and GBG. Vox Sang. 1974;27(4):382–384. doi: 10.1111/j.1423-0410.1974.tb02433.x. [DOI] [PubMed] [Google Scholar]
  2. Alper C. A. Inherited structural polymorphism in human C2: evidence for genetic linkage between C2 and Bf. J Exp Med. 1976 Oct 1;144(4):1111–1115. doi: 10.1084/jem.144.4.1111. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Dixit R., Schneider R., Law S. K., Kulczycki A., Jr, Atkinson J. P. Ligand binding specificity of a rabbit alveolar macrophage receptor for C3b. J Biol Chem. 1982 Feb 25;257(4):1595–1597. [PubMed] [Google Scholar]
  4. 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]
  5. 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]
  6. 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]
  7. Fujita T., Gigli I., Nussenzweig V. Human C4-binding protein. II. Role in proteolysis of C4b by C3b-inactivator. J Exp Med. 1978 Oct 1;148(4):1044–1051. doi: 10.1084/jem.148.4.1044. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Fujita T., Nussenzweig V. The role of C4-binding protein and beta 1H in proteolysis of C4b and C3b. J Exp Med. 1979 Aug 1;150(2):267–276. doi: 10.1084/jem.150.2.267. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Gigli I., Fujita T., Nussenzweig V. Modulation of the classical pathway C3 convertase by plasma proteins C4 binding protein and C3b inactivator. Proc Natl Acad Sci U S A. 1979 Dec;76(12):6596–6600. doi: 10.1073/pnas.76.12.6596. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Hatch J. A., Atkinson J. P., Suarez B. K., Dykman T. R. Evaluation of linkage of the human C3b/C4b receptor to HLA. J Immunol. 1984 May;132(5):2168–2169. [PubMed] [Google Scholar]
  11. Hobart M. J., Joysey V., Lachmann P. J. Inherited structural variation and linkage relationships of C7. J Immunogenet. 1978 Jun;5(3):157–163. doi: 10.1111/j.1744-313x.1978.tb00641.x. [DOI] [PubMed] [Google Scholar]
  12. 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]
  13. Lachmann P. J., Hobart M. J., Woo P. Combined genetic deficiency of C6 and C7 in man. Clin Exp Immunol. 1978 Aug;33(2):193–203. [PMC free article] [PubMed] [Google Scholar]
  14. Nicholson-Weller A., Burge J., Austen K. F. Purification from guinea pig erythrocyte stroma of a decay-accelerating factor for the classical c3 convertase, C4b,2a. J Immunol. 1981 Nov;127(5):2035–2039. [PubMed] [Google Scholar]
  15. Nicholson-Weller A., Burge J., Fearon D. T., Weller P. F., Austen K. F. Isolation of a human erythrocyte membrane glycoprotein with decay-accelerating activity for C3 convertases of the complement system. J Immunol. 1982 Jul;129(1):184–189. [PubMed] [Google Scholar]
  16. Pangburn M. K., Schreiber R. D., Müller-Eberhard H. J. Human complement C3b inactivator: isolation, characterization, and demonstration of an absolute requirement for the serum protein beta1H for cleavage of C3b and C4b in solution. J Exp Med. 1977 Jul 1;146(1):257–270. doi: 10.1084/jem.146.1.257. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Rodriguez de Córdoba S., Rubinstein P., Ferreira A. High resolution isoelectric focusing of immunoprecipitated proteins under denaturing conditions. A simple analytical method applied to the study of complement component polymorphisms. J Immunol Methods. 1984 Apr 27;69(2):165–172. doi: 10.1016/0022-1759(84)90314-4. [DOI] [PubMed] [Google Scholar]
  18. Rodríguez de Córdoba S., Rubinstein P. Genetic polymorphism of human factor H (beta 1H). J Immunol. 1984 Apr;132(4):1906–1908. [PubMed] [Google Scholar]
  19. Scharfstein J., Ferreira A., Gigli I., Nussenzweig V. Human C4-binding protein. I. Isolation and characterization. J Exp Med. 1978 Jul 1;148(1):207–222. doi: 10.1084/jem.148.1.207. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Schneider R. J., Kulczycki A., Jr, Law S. K., Atkinson J. P. Isolation of a biologically active macrophage receptor for the third component of complement. Nature. 1981 Apr 30;290(5809):789–792. doi: 10.1038/290789a0. [DOI] [PubMed] [Google Scholar]
  21. Teisberg P., Akesson I., Olaisen B., Gedde-Dahl T., Jr, Thorsby E. Genetic polymorphism of C4 in man and localisation of a structural C4 locus to the HLA gene complex of chromosome 6. Nature. 1976 Nov 18;264(5583):253–254. doi: 10.1038/264253a0. [DOI] [PubMed] [Google Scholar]
  22. Whaley K., Ruddy S. Modulation of the alternative complement pathways by beta 1 H globulin. J Exp Med. 1976 Nov 2;144(5):1147–1163. doi: 10.1084/jem.144.5.1147. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. 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]
  24. de Cordoba S. R., Ferreira A., Nussenzweig V., Rubinstein P. Genetic polymorphism of human C4-binding protein. J Immunol. 1983 Sep;131(3):1565–1569. [PubMed] [Google Scholar]

Articles from Proceedings of the National Academy of Sciences of the United States of America are provided here courtesy of National Academy of Sciences

RESOURCES