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. 1991 Aug 1;277(Pt 3):581–592. doi: 10.1042/bj2770581

The interaction between complement component C4b-binding protein and the vitamin K-dependent protein S forms a link between blood coagulation and the complement system.

M Hessing 1
PMCID: PMC1151280  PMID: 1831349

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Selected References

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

  1. Baker M. E., French F. S., Joseph D. R. Vitamin K-dependent protein S is similar to rat androgen-binding protein. Biochem J. 1987 Apr 1;243(1):293–296. doi: 10.1042/bj2430293. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Barnum S. R., Dahlbäck B. C4b-binding protein, a regulatory component of the classical pathway of complement, is an acute-phase protein and is elevated in systemic lupus erythematosus. Complement Inflamm. 1990;7(2):71–77. doi: 10.1159/000463131. [DOI] [PubMed] [Google Scholar]
  3. Barnum S. R., Kristensen T., Chaplin D. D., Seldin M. F., Tack B. F. Molecular analysis of the murine C4b-binding protein gene. Chromosome assignment and partial gene organization. Biochemistry. 1989 Oct 17;28(21):8312–8317. doi: 10.1021/bi00447a008. [DOI] [PubMed] [Google Scholar]
  4. Belt K. T., Carroll M. C., Porter R. R. The structural basis of the multiple forms of human complement component C4. Cell. 1984 Apr;36(4):907–914. doi: 10.1016/0092-8674(84)90040-0. [DOI] [PubMed] [Google Scholar]
  5. Bentley D. R. Primary structure of human complement component C2. Homology to two unrelated protein families. Biochem J. 1986 Oct 15;239(2):339–345. doi: 10.1042/bj2390339. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Bertina R. M., Broekmans A. W., van der Linden I. K., Mertens K. Protein C deficiency in a Dutch family with thrombotic disease. Thromb Haemost. 1982 Aug 24;48(1):1–5. [PubMed] [Google Scholar]
  7. Bertina R. M. Hereditary protein S deficiency. Haemostasis. 1985;15(4):241–246. doi: 10.1159/000215155. [DOI] [PubMed] [Google Scholar]
  8. Bertina R. M., van Wijngaarden A., Reinalda-Poot J., Poort S. R., Bom V. J. Determination of plasma protein S--the protein cofactor of activated protein C. Thromb Haemost. 1985 Apr 22;53(2):268–272. [PubMed] [Google Scholar]
  9. Bevilacqua M. P., Stengelin S., Gimbrone M. A., Jr, Seed B. Endothelial leukocyte adhesion molecule 1: an inducible receptor for neutrophils related to complement regulatory proteins and lectins. Science. 1989 Mar 3;243(4895):1160–1165. doi: 10.1126/science.2466335. [DOI] [PubMed] [Google Scholar]
  10. Boerger L. M., Morris P. C., Thurnau G. R., Esmon C. T., Comp P. C. Oral contraceptives and gender affect protein S status. Blood. 1987 Feb;69(2):692–694. [PubMed] [Google Scholar]
  11. Bora N. S., Lublin D. M., Kumar B. V., Hockett R. D., Holers V. M., Atkinson J. P. Structural gene for human membrane cofactor protein (MCP) of complement maps to within 100 kb of the 3' end of the C3b/C4b receptor gene. J Exp Med. 1989 Feb 1;169(2):597–602. doi: 10.1084/jem.169.2.597. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Brett J. G., Steinberg S. F., deGroot P. G., Nawroth P. P., Stern D. M. Norepinephrine down-regulates the activity of protein S on endothelial cells. J Cell Biol. 1988 Jun;106(6):2109–2118. doi: 10.1083/jcb.106.6.2109. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Broekmans A. W., Bertina R. M., Reinalda-Poot J., Engesser L., Muller H. P., Leeuw J. A., Michiels J. J., Brommer E. J., Briët E. Hereditary protein S deficiency and venous thrombo-embolism. A study in three Dutch families. Thromb Haemost. 1985 Apr 22;53(2):273–277. [PubMed] [Google Scholar]
  14. Broekmans A. W. Hereditary protein C deficiency. Haemostasis. 1985;15(4):233–240. doi: 10.1159/000215154. [DOI] [PubMed] [Google Scholar]
  15. Carroll M. C., Alicot E. M., Katzman P. J., Klickstein L. B., Smith J. A., Fearon D. T. Organization of the genes encoding complement receptors type 1 and 2, decay-accelerating factor, and C4-binding protein in the RCA locus on human chromosome 1. J Exp Med. 1988 Apr 1;167(4):1271–1280. doi: 10.1084/jem.167.4.1271. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. 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]
  17. Chung L. P., Gagnon J., Reid K. B. Amino acid sequence studies of human C4b-binding protein: N-terminal sequence analysis and alignment of the fragments produced by limited proteolysis with chymotrypsin and the peptides produced by cyanogen bromide treatment. Mol Immunol. 1985 Apr;22(4):427–435. doi: 10.1016/0161-5890(85)90127-0. [DOI] [PubMed] [Google Scholar]
  18. Chung L. P., Reid K. B. Structural and functional studies on C4b-binding protein, a regulatory component of the human complement system. Biosci Rep. 1985 Oct-Nov;5(10-11):855–865. doi: 10.1007/BF01119897. [DOI] [PubMed] [Google Scholar]
  19. Comp P. C., Esmon C. T. Activated protein C inhibits platelet prothrombin-converting activity. Blood. 1979 Dec;54(6):1272–1281. [PubMed] [Google Scholar]
  20. Comp P. C., Esmon C. T. Generation of fibrinolytic activity by infusion of activated protein C into dogs. J Clin Invest. 1981 Nov;68(5):1221–1228. doi: 10.1172/JCI110368. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Comp P. C., Esmon C. T. Recurrent venous thromboembolism in patients with a partial deficiency of protein S. N Engl J Med. 1984 Dec 13;311(24):1525–1528. doi: 10.1056/NEJM198412133112401. [DOI] [PubMed] [Google Scholar]
  22. Comp P. C., Nixon R. R., Esmon C. T. Determination of functional levels of protein C, an antithrombotic protein, using thrombin-thrombomodulin complex. Blood. 1984 Jan;63(1):15–21. [PubMed] [Google Scholar]
  23. Comp P. C., Thurnau G. R., Welsh J., Esmon C. T. Functional and immunologic protein S levels are decreased during pregnancy. Blood. 1986 Oct;68(4):881–885. [PubMed] [Google Scholar]
  24. D'Angelo A., Vigano-D'Angelo S., Esmon C. T., Comp P. C. Acquired deficiencies of protein S. Protein S activity during oral anticoagulation, in liver disease, and in disseminated intravascular coagulation. J Clin Invest. 1988 May;81(5):1445–1454. doi: 10.1172/JCI113475. [DOI] [PMC free article] [PubMed] [Google Scholar]
  25. Daha M. R., van Es L. A. Relative resistance of the F-42-stabilized classical pathway C3 convertase to inactivation by C4-binding protein. J Immunol. 1980 Nov;125(5):2051–2054. [PubMed] [Google Scholar]
  26. Dahlbäck B., Frohm B., Nelsestuen G. High affinity interaction between C4b-binding protein and vitamin K-dependent protein S in the presence of calcium. Suggestion of a third component in blood regulating the interaction. J Biol Chem. 1990 Sep 25;265(27):16082–16087. [PubMed] [Google Scholar]
  27. Dahlbäck B., Hildebrand B. Degradation of human complement component C4b in the presence of the C4b-binding protein-protein S complex. Biochem J. 1983 Mar 1;209(3):857–863. doi: 10.1042/bj2090857. [DOI] [PMC free article] [PubMed] [Google Scholar]
  28. Dahlbäck B., Hildebrand B., Malm J. Characterization of functionally important domains in human vitamin K-dependent protein S using monoclonal antibodies. J Biol Chem. 1990 May 15;265(14):8127–8135. [PubMed] [Google Scholar]
  29. Dahlbäck B. Inhibition of protein Ca cofactor function of human and bovine protein S by C4b-binding protein. J Biol Chem. 1986 Sep 15;261(26):12022–12027. [PubMed] [Google Scholar]
  30. Dahlbäck B. Interaction between vitamin K-dependent protein S and the complement protein, C4b-binding protein. A link between coagulation and the complement system. Semin Thromb Hemost. 1984 Apr;10(2):139–148. doi: 10.1055/s-2007-1004416. [DOI] [PubMed] [Google Scholar]
  31. Dahlbäck B., Lundwall A., Stenflo J. Localization of thrombin cleavage sites in the amino-terminal region of bovine protein S. J Biol Chem. 1986 Apr 15;261(11):5111–5115. [PubMed] [Google Scholar]
  32. Dahlbäck B., Lundwall A., Stenflo J. Primary structure of bovine vitamin K-dependent protein S. Proc Natl Acad Sci U S A. 1986 Jun;83(12):4199–4203. doi: 10.1073/pnas.83.12.4199. [DOI] [PMC free article] [PubMed] [Google Scholar]
  33. Dahlbäck B., Müller-Eberhard H. J. Ultrastructure of C4b-binding protein fragments formed by limited proteolysis using chymotrypsin. J Biol Chem. 1984 Oct 10;259(19):11631–11634. [PubMed] [Google Scholar]
  34. Dahlbäck B. Purification of human C4b-binding protein and formation of its complex with vitamin K-dependent protein S. Biochem J. 1983 Mar 1;209(3):847–856. doi: 10.1042/bj2090847. [DOI] [PMC free article] [PubMed] [Google Scholar]
  35. Dahlbäck B. Purification of human vitamin K-dependent protein S and its limited proteolysis by thrombin. Biochem J. 1983 Mar 1;209(3):837–846. doi: 10.1042/bj2090837. [DOI] [PMC free article] [PubMed] [Google Scholar]
  36. Dahlbäck B., Smith C. A., Müller-Eberhard H. J. Visualization of human C4b-binding protein and its complexes with vitamin K-dependent protein S and complement protein C4b. Proc Natl Acad Sci U S A. 1983 Jun;80(11):3461–3465. doi: 10.1073/pnas.80.11.3461. [DOI] [PMC free article] [PubMed] [Google Scholar]
  37. Dahlbäck B., Stenflo J. High molecular weight complex in human plasma between vitamin K-dependent protein S and complement component C4b-binding protein. Proc Natl Acad Sci U S A. 1981 Apr;78(4):2512–2516. doi: 10.1073/pnas.78.4.2512. [DOI] [PMC free article] [PubMed] [Google Scholar]
  38. Dahlbäck B., Stenflo J. Inhibitory effect of activated protein C on activation of prothrombin by platelet-bound factor Xa. Eur J Biochem. 1980 Jun;107(2):331–335. doi: 10.1111/j.1432-1033.1980.tb06033.x. [DOI] [PubMed] [Google Scholar]
  39. Di Scipio R. G., Hermodson M. A., Yates S. G., Davie E. W. A comparison of human prothrombin, factor IX (Christmas factor), factor X (Stuart factor), and protein S. Biochemistry. 1977 Feb 22;16(4):698–706. doi: 10.1021/bi00623a022. [DOI] [PubMed] [Google Scholar]
  40. DiScipio R. G., Chakravarti D. N., Muller-Eberhard H. J., Fey G. H. The structure of human complement component C7 and the C5b-7 complex. J Biol Chem. 1988 Jan 5;263(1):549–560. [PubMed] [Google Scholar]
  41. DiScipio R. G., Davie E. W. Characterization of protein S, a gamma-carboxyglutamic acid containing protein from bovine and human plasma. Biochemistry. 1979 Mar 6;18(5):899–904. doi: 10.1021/bi00572a026. [DOI] [PubMed] [Google Scholar]
  42. DiScipio R. G., Hugli T. E. The molecular architecture of human complement component C6. J Biol Chem. 1989 Sep 25;264(27):16197–16206. [PubMed] [Google Scholar]
  43. Doege K., Fernandez P., Hassell J. R., Sasaki M., Yamada Y. Partial cDNA sequence encoding a globular domain at the C terminus of the rat cartilage proteoglycan. J Biol Chem. 1986 Jun 25;261(18):8108–8111. [PubMed] [Google Scholar]
  44. Doolittle R. F., Feng D. F., Johnson M. S. Computer-based characterization of epidermal growth factor precursor. Nature. 1984 Feb 9;307(5951):558–560. doi: 10.1038/307558a0. [DOI] [PubMed] [Google Scholar]
  45. Drakenberg T., Fernlund P., Roepstorff P., Stenflo J. beta-Hydroxyaspartic acid in vitamin K-dependent protein C. Proc Natl Acad Sci U S A. 1983 Apr;80(7):1802–1806. doi: 10.1073/pnas.80.7.1802. [DOI] [PMC free article] [PubMed] [Google Scholar]
  46. Eaton D., Rodriguez H., Vehar G. A. Proteolytic processing of human factor VIII. Correlation of specific cleavages by thrombin, factor Xa, and activated protein C with activation and inactivation of factor VIII coagulant activity. Biochemistry. 1986 Jan 28;25(2):505–512. doi: 10.1021/bi00350a035. [DOI] [PubMed] [Google Scholar]
  47. Edenbrandt C. M., Lundwall A., Wydro R., Stenflo J. Molecular analysis of the gene for vitamin K dependent protein S and its pseudogene. Cloning and partial gene organization. Biochemistry. 1990 Aug 28;29(34):7861–7868. doi: 10.1021/bi00486a012. [DOI] [PubMed] [Google Scholar]
  48. Esmon C. T., Esmon N. L. Protein C activation. Semin Thromb Hemost. 1984 Apr;10(2):122–130. doi: 10.1055/s-2007-1004414. [DOI] [PubMed] [Google Scholar]
  49. Esmon C. T., Owen W. G. Identification of an endothelial cell cofactor for thrombin-catalyzed activation of protein C. Proc Natl Acad Sci U S A. 1981 Apr;78(4):2249–2252. doi: 10.1073/pnas.78.4.2249. [DOI] [PMC free article] [PubMed] [Google Scholar]
  50. Esmon N. L., Owen W. G., Esmon C. T. Isolation of a membrane-bound cofactor for thrombin-catalyzed activation of protein C. J Biol Chem. 1982 Jan 25;257(2):859–864. [PubMed] [Google Scholar]
  51. Fair D. S., Marlar R. A. Biosynthesis and secretion of factor VII, protein C, protein S, and the Protein C inhibitor from a human hepatoma cell line. Blood. 1986 Jan;67(1):64–70. [PubMed] [Google Scholar]
  52. Fair D. S., Marlar R. A., Levin E. G. Human endothelial cells synthesize protein S. Blood. 1986 Apr;67(4):1168–1171. [PubMed] [Google Scholar]
  53. Fielder A. H., Walport M. J., Batchelor J. R., Rynes R. I., Black C. M., Dodi I. A., Hughes G. R. Family study of the major histocompatibility complex in patients with systemic lupus erythematosus: importance of null alleles of C4A and C4B in determining disease susceptibility. Br Med J (Clin Res Ed) 1983 Feb 5;286(6363):425–428. doi: 10.1136/bmj.286.6363.425. [DOI] [PMC free article] [PubMed] [Google Scholar]
  54. Francis R. B., Jr Protein S deficiency in sickle cell anemia. J Lab Clin Med. 1988 May;111(5):571–576. [PubMed] [Google Scholar]
  55. 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]
  56. Fujita T., Kamato T., Tamura N. Characterization of functional properties of C4-binding protein by monoclonal antibodies. J Immunol. 1985 May;134(5):3320–3324. [PubMed] [Google Scholar]
  57. 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]
  58. Fujita T., Tamura N. Interaction of C4-binding protein with cell-bound C4b. A quantitative analysis of binding and the role of C4-binding protein in proteolysis of cell-bound C4b. J Exp Med. 1983 Apr 1;157(4):1239–1251. doi: 10.1084/jem.157.4.1239. [DOI] [PMC free article] [PubMed] [Google Scholar]
  59. Funakoshi M., Sasaki J., Arakawa K. Proline-rich protein is a glycoprotein and an acute phase reactant. Biochim Biophys Acta. 1988 Nov 4;963(1):98–108. doi: 10.1016/0005-2760(88)90342-6. [DOI] [PubMed] [Google Scholar]
  60. Furie B., Furie B. C. The molecular basis of blood coagulation. Cell. 1988 May 20;53(4):505–518. doi: 10.1016/0092-8674(88)90567-3. [DOI] [PubMed] [Google Scholar]
  61. Gershagen S., Fernlund P., Lundwall A. A cDNA coding for human sex hormone binding globulin. Homology to vitamin K-dependent protein S. FEBS Lett. 1987 Aug 10;220(1):129–135. doi: 10.1016/0014-5793(87)80890-6. [DOI] [PubMed] [Google Scholar]
  62. 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]
  63. Goldberger G., Colten H. R. Precursor complement protein (pro-C4) is converted in vitro to native C4 by plasmin. Nature. 1980 Jul 31;286(5772):514–516. doi: 10.1038/286514a0. [DOI] [PubMed] [Google Scholar]
  64. Griffin J. H., Evatt B., Zimmerman T. S., Kleiss A. J., Wideman C. Deficiency of protein C in congenital thrombotic disease. J Clin Invest. 1981 Nov;68(5):1370–1373. doi: 10.1172/JCI110385. [DOI] [PMC free article] [PubMed] [Google Scholar]
  65. Harris K. W., Esmon C. T. Protein S is required for bovine platelets to support activated protein C binding and activity. J Biol Chem. 1985 Feb 25;260(4):2007–2010. [PubMed] [Google Scholar]
  66. Hauptmann G., Goetz J., Uring-Lambert B., Grosshans E. Component deficiencies. 2. The fourth component. Prog Allergy. 1986;39:232–249. [PubMed] [Google Scholar]
  67. Hessing M., Kanters D., Hackeng T. M., Bouma B. N. Identification of different forms of human C4b-binding protein lacking beta-chain and protein S binding ability. Thromb Haemost. 1990 Oct 22;64(2):245–250. [PubMed] [Google Scholar]
  68. Hessing M., Vlooswijk R. A., Hackeng T. M., Kanters D., Bouma B. N. The localization of heparin-binding fragments on human C4b-binding protein. J Immunol. 1990 Jan 1;144(1):204–208. [PubMed] [Google Scholar]
  69. Hessing M., van 'T Veer C., Bouma B. N. The binding site of human C4b-binding protein on complement C4 is localized in the alpha'-chain. J Immunol. 1990 Apr 1;144(7):2632–2637. [PubMed] [Google Scholar]
  70. Hillarp A., Dahlbäck B. Cloning of cDNA coding for the beta chain of human complement component C4b-binding protein: sequence homology with the alpha chain. Proc Natl Acad Sci U S A. 1990 Feb;87(3):1183–1187. doi: 10.1073/pnas.87.3.1183. [DOI] [PMC free article] [PubMed] [Google Scholar]
  71. Hillarp A., Dahlbäck B. Novel subunit in C4b-binding protein required for protein S binding. J Biol Chem. 1988 Sep 5;263(25):12759–12764. [PubMed] [Google Scholar]
  72. Hillarp A., Dahlbäck B. The protein S-binding site localized to the central core of C4b-binding protein. J Biol Chem. 1987 Aug 15;262(23):11300–11307. [PubMed] [Google Scholar]
  73. Hillarp A., Hessing M., Dahlbäck B. Protein S binding in relation to the subunit composition of human C4b-binding protein. FEBS Lett. 1989 Dec 18;259(1):53–56. doi: 10.1016/0014-5793(89)81492-9. [DOI] [PubMed] [Google Scholar]
  74. Hing S., Day A. J., Linton S. J., Ripoche J., Sim R. B., Reid K. B., Solomon E. Assignment of complement components C4 binding protein (C4BP) and factor H (FH) to human chromosome 1q, using cDNA probes. Ann Hum Genet. 1988 May;52(Pt 2):117–122. doi: 10.1111/j.1469-1809.1988.tb01086.x. [DOI] [PubMed] [Google Scholar]
  75. Hoskins J., Norman D. K., Beckmann R. J., Long G. L. Cloning and characterization of human liver cDNA encoding a protein S precursor. Proc Natl Acad Sci U S A. 1987 Jan;84(2):349–353. doi: 10.1073/pnas.84.2.349. [DOI] [PMC free article] [PubMed] [Google Scholar]
  76. Hourcade D., Holers V. M., Atkinson J. P. The regulators of complement activation (RCA) gene cluster. Adv Immunol. 1989;45:381–416. doi: 10.1016/s0065-2776(08)60697-5. [DOI] [PubMed] [Google Scholar]
  77. Howard P. F., Hochberg M. C., Bias W. B., Arnett F. C., Jr, McLean R. H. Relationship between C4 null genes, HLA-D region antigens, and genetic susceptibility to systemic lupus erythematosus in Caucasian and black Americans. Am J Med. 1986 Aug;81(2):187–193. doi: 10.1016/0002-9343(86)90250-0. [DOI] [PubMed] [Google Scholar]
  78. Huisveld I. A., Hospers J. E., Meijers J. C., Starkenburg A. E., Erich W. B., Bouma B. N. Oral contraceptives reduce total protein S, but not free protein S. Thromb Res. 1987 Jan 1;45(1):109–114. doi: 10.1016/0049-3848(87)90262-3. [DOI] [PubMed] [Google Scholar]
  79. Ichinose A., McMullen B. A., Fujikawa K., Davie E. W. Amino acid sequence of the b subunit of human factor XIII, a protein composed of ten repetitive segments. Biochemistry. 1986 Aug 12;25(16):4633–4638. doi: 10.1021/bi00364a027. [DOI] [PubMed] [Google Scholar]
  80. Isenman D. E., Young J. R. The molecular basis for the difference in immune hemolysis activity of the Chido and Rodgers isotypes of human complement component C4. J Immunol. 1984 Jun;132(6):3019–3027. [PubMed] [Google Scholar]
  81. Janatova J., Reid K. B., Willis A. C. Disulfide bonds are localized within the short consensus repeat units of complement regulatory proteins: C4b-binding protein. Biochemistry. 1989 May 30;28(11):4754–4761. doi: 10.1021/bi00437a036. [DOI] [PubMed] [Google Scholar]
  82. Johnston G. I., Cook R. G., McEver R. P. Cloning of GMP-140, a granule membrane protein of platelets and endothelium: sequence similarity to proteins involved in cell adhesion and inflammation. Cell. 1989 Mar 24;56(6):1033–1044. doi: 10.1016/0092-8674(89)90636-3. [DOI] [PubMed] [Google Scholar]
  83. Journet A., Tosi M. Cloning and sequencing of full-length cDNA encoding the precursor of human complement component C1r. Biochem J. 1986 Dec 15;240(3):783–787. doi: 10.1042/bj2400783. [DOI] [PMC free article] [PubMed] [Google Scholar]
  84. Kamiya T., Sugihara T., Ogata K., Saito H., Suzuki K., Nishioka J., Hashimoto S., Yamagata K. Inherited deficiency of protein S in a Japanese family with recurrent venous thrombosis: a study of three generations. Blood. 1986 Feb;67(2):406–410. [PubMed] [Google Scholar]
  85. Karp D. R. Post-translational modification of the fourth component of complement. Effect of tunicamycin and amino acid analogs on the formation of the internal thiol ester and disulfide bonds. J Biol Chem. 1983 Dec 10;258(23):14490–14495. [PubMed] [Google Scholar]
  86. Karp D. R. Post-translational modification of the fourth component of complement. Sulfation of the alpha-chain. J Biol Chem. 1983 Nov 10;258(21):12745–12748. [PubMed] [Google Scholar]
  87. Kerr M. A. The human complement system: assembly of the classical pathway C3 convertase. Biochem J. 1980 Jul 1;189(1):173–181. doi: 10.1042/bj1890173. [DOI] [PMC free article] [PubMed] [Google Scholar]
  88. Kimura S., Hong Y. S., Kotani T., Ohtaki S., Kikkawa F. Structure of the human thyroid peroxidase gene: comparison and relationship to the human myeloperoxidase gene. Biochemistry. 1989 May 16;28(10):4481–4489. doi: 10.1021/bi00436a054. [DOI] [PubMed] [Google Scholar]
  89. Kisiel W., Canfield W. M., Ericsson L. H., Davie E. W. Anticoagulant properties of bovine plasma protein C following activation by thrombin. Biochemistry. 1977 Dec 27;16(26):5824–5831. doi: 10.1021/bi00645a029. [DOI] [PubMed] [Google Scholar]
  90. Klickstein L. B., Bartow T. J., Miletic V., Rabson L. D., Smith J. A., Fearon D. T. Identification of distinct C3b and C4b recognition sites in the human C3b/C4b receptor (CR1, CD35) by deletion mutagenesis. J Exp Med. 1988 Nov 1;168(5):1699–1717. doi: 10.1084/jem.168.5.1699. [DOI] [PMC free article] [PubMed] [Google Scholar]
  91. Koedam J. A., Meijers J. C., Sixma J. J., Bouma B. N. Inactivation of human factor VIII by activated protein C. Cofactor activity of protein S and protective effect of von Willebrand factor. J Clin Invest. 1988 Oct;82(4):1236–1243. doi: 10.1172/JCI113721. [DOI] [PMC free article] [PubMed] [Google Scholar]
  92. Kotwal G. J., Moss B. Vaccinia virus encodes a secretory polypeptide structurally related to complement control proteins. Nature. 1988 Sep 8;335(6186):176–178. doi: 10.1038/335176a0. [DOI] [PubMed] [Google Scholar]
  93. 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]
  94. 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]
  95. Krusius T., Gehlsen K. R., Ruoslahti E. A fibroblast chondroitin sulfate proteoglycan core protein contains lectin-like and growth factor-like sequences. J Biol Chem. 1987 Sep 25;262(27):13120–13125. [PubMed] [Google Scholar]
  96. Kurosky A., Barnett D. R., Lee T. H., Touchstone B., Hay R. E., Arnott M. S., Bowman B. H., Fitch W. M. Covalent structure of human haptoglobin: a serine protease homolog. Proc Natl Acad Sci U S A. 1980 Jun;77(6):3388–3392. doi: 10.1073/pnas.77.6.3388. [DOI] [PMC free article] [PubMed] [Google Scholar]
  97. Kusunoki Y., Itami N., Tochimaru H., Takekoshi Y., Nagasawa S., Yoshiki T. Glomerular deposition of C4 cleavage fragment (C4d) and C4-binding protein in idiopathic membranous glomerulonephritis. Nephron. 1989;51(1):17–19. doi: 10.1159/000185234. [DOI] [PubMed] [Google Scholar]
  98. Lappin D. F., Whaley K. Interferon-induced transcriptional and post-transcriptional modulation of factor H and C4 binding-protein synthesis in human monocytes. Biochem J. 1990 Nov 1;271(3):767–772. doi: 10.1042/bj2710767. [DOI] [PMC free article] [PubMed] [Google Scholar]
  99. Lasky L. A., Singer M. S., Yednock T. A., Dowbenko D., Fennie C., Rodriguez H., Nguyen T., Stachel S., Rosen S. D. Cloning of a lymphocyte homing receptor reveals a lectin domain. Cell. 1989 Mar 24;56(6):1045–1055. doi: 10.1016/0092-8674(89)90637-5. [DOI] [PubMed] [Google Scholar]
  100. Law S. K., Dodds A. W., Porter R. R. A comparison of the properties of two classes, C4A and C4B, of the human complement component C4. EMBO J. 1984 Aug;3(8):1819–1823. doi: 10.1002/j.1460-2075.1984.tb02052.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  101. Leonard W. J., Depper J. M., Kanehisa M., Krönke M., Peffer N. J., Svetlik P. B., Sullivan M., Greene W. C. Structure of the human interleukin-2 receptor gene. Science. 1985 Nov 8;230(4726):633–639. doi: 10.1126/science.2996141. [DOI] [PubMed] [Google Scholar]
  102. Lintin S. J., Lewin A. R., Reid K. B. Derivation of the sequence of the signal peptide in human C4b-binding protein and interspecies cross-hybridisation of the C4bp cDNA sequence. FEBS Lett. 1988 May 23;232(2):328–332. doi: 10.1016/0014-5793(88)80763-4. [DOI] [PubMed] [Google Scholar]
  103. Lintin S. J., Reid K. B. Studies on the structure of the human C4b-binding protein gene. FEBS Lett. 1986 Aug 11;204(1):77–81. doi: 10.1016/0014-5793(86)81390-4. [DOI] [PubMed] [Google Scholar]
  104. Long G. L., Marshall A., Gardner J. C., Naylor S. L. Genes for human vitamin K-dependent plasma proteins C and S are located on chromosomes 2 and 3, respectively. Somat Cell Mol Genet. 1988 Jan;14(1):93–98. doi: 10.1007/BF01535052. [DOI] [PubMed] [Google Scholar]
  105. Lozier J., Takahashi N., Putnam F. W. Complete amino acid sequence of human plasma beta 2-glycoprotein I. Proc Natl Acad Sci U S A. 1984 Jun;81(12):3640–3644. doi: 10.1073/pnas.81.12.3640. [DOI] [PMC free article] [PubMed] [Google Scholar]
  106. 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]
  107. Lublin D. M., Liszewski M. K., Post T. W., Arce M. A., Le Beau M. M., Rebentisch M. B., Lemons L. S., Seya T., Atkinson J. P. Molecular cloning and chromosomal localization of human membrane cofactor protein (MCP). Evidence for inclusion in the multigene family of complement-regulatory proteins. J Exp Med. 1988 Jul 1;168(1):181–194. doi: 10.1084/jem.168.1.181. [DOI] [PMC free article] [PubMed] [Google Scholar]
  108. Lundwall A., Dackowski W., Cohen E., Shaffer M., Mahr A., Dahlbäck B., Stenflo J., Wydro R. Isolation and sequence of the cDNA for human protein S, a regulator of blood coagulation. Proc Natl Acad Sci U S A. 1986 Sep;83(18):6716–6720. doi: 10.1073/pnas.83.18.6716. [DOI] [PMC free article] [PubMed] [Google Scholar]
  109. Lundwall A., Malmheden I., Stålenheim G., Sjöquist J. Isolation of component C4 of human complement and its polypeptide chains. Eur J Biochem. 1981 Jun;117(1):141–146. doi: 10.1111/j.1432-1033.1981.tb06312.x. [DOI] [PubMed] [Google Scholar]
  110. MUELLER-EBERHARD H. J., LEPOW I. H. C'1 ESTERASE EFFECT ON ACTIVITY AND PHYSICOCHEMICAL PROPERTIES OF THE FOURTH COMPONENT OF COMPLEMENT. J Exp Med. 1965 May 1;121:819–833. doi: 10.1084/jem.121.5.819. [DOI] [PMC free article] [PubMed] [Google Scholar]
  111. Malm J., Bennhagen R., Holmberg L., Dahlbäck B. Plasma concentrations of C4b-binding protein and vitamin K-dependent protein S in term and preterm infants: low levels of protein S-C4b-binding protein complexes. Br J Haematol. 1988 Apr;68(4):445–449. doi: 10.1111/j.1365-2141.1988.tb04233.x. [DOI] [PubMed] [Google Scholar]
  112. Malm J., Cohen E., Dackowski W., Dahlbäck B., Wydro R. Expression of completely gamma-carboxylated and beta-hydroxylated recombinant human vitamin-K-dependent protein S with full biological activity. Eur J Biochem. 1990 Feb 14;187(3):737–743. doi: 10.1111/j.1432-1033.1990.tb15361.x. [DOI] [PubMed] [Google Scholar]
  113. Malm J., Laurell M., Dahlbäck B. Changes in the plasma levels of vitamin K-dependent proteins C and S and of C4b-binding protein during pregnancy and oral contraception. Br J Haematol. 1988 Apr;68(4):437–443. doi: 10.1111/j.1365-2141.1988.tb04232.x. [DOI] [PubMed] [Google Scholar]
  114. Malm J., Persson U., Dahlbäck B. Inhibition of human vitamin-K-dependent protein-S-cofactor activity by a monoclonal antibody specific for a Ca2+-dependent epitope. Eur J Biochem. 1987 May 15;165(1):39–45. doi: 10.1111/j.1432-1033.1987.tb11191.x. [DOI] [PubMed] [Google Scholar]
  115. Mann K. G., Jenny R. J., Krishnaswamy S. Cofactor proteins in the assembly and expression of blood clotting enzyme complexes. Annu Rev Biochem. 1988;57:915–956. doi: 10.1146/annurev.bi.57.070188.004411. [DOI] [PubMed] [Google Scholar]
  116. Mannucci P. M., Tripodi A., Bertina R. M. Protein S deficiency associated with "juvenile" arterial and venous thromboses. Thromb Haemost. 1986 Jun 30;55(3):440–440. [PubMed] [Google Scholar]
  117. Marlar R. A., Kleiss A. J., Griffin J. H. Mechanism of action of human activated protein C, a thrombin-dependent anticoagulant enzyme. Blood. 1982 May;59(5):1067–1072. [PubMed] [Google Scholar]
  118. Matsuguchi T., Okamura S., Aso T., Sata T., Niho Y. Molecular cloning of the cDNA coding for proline-rich protein (PRP): identity of PRP as C4b-binding protein. Biochem Biophys Res Commun. 1989 Nov 30;165(1):138–144. doi: 10.1016/0006-291x(89)91045-0. [DOI] [PubMed] [Google Scholar]
  119. Matthews W. J., Jr, Goldberger G., Marino J. T., Jr, Einstein L. P., Gash D. J., Colten H. R. Complement proteins C2, C4 and factor B. Effect of glycosylation on their secretion and catabolism. Biochem J. 1982 Jun 15;204(3):839–846. doi: 10.1042/bj2040839. [DOI] [PMC free article] [PubMed] [Google Scholar]
  120. Mauff G., Alper C. A., Awdeh Z., Batchelor J. R., Bertrams J., Bruun-Petersen G., Dawkins R. L., Démant P., Edwards J., Grosse-Wilde H. Statement on the nomenclature of human C4 allotypes. Immunobiology. 1983 Mar;164(2):184–191. doi: 10.1016/s0171-2985(83)80009-6. [DOI] [PubMed] [Google Scholar]
  121. McMullen B. A., Fujikawa K., Kisiel W., Sasagawa T., Howald W. N., Kwa E. Y., Weinstein B. Complete amino acid sequence of the light chain of human blood coagulation factor X: evidence for identification of residue 63 as beta-hydroxyaspartic acid. Biochemistry. 1983 Jun 7;22(12):2875–2884. doi: 10.1021/bi00281a016. [DOI] [PubMed] [Google Scholar]
  122. McMullen B. A., Fujikawa K., Kisiel W. The occurrence of beta-hydroxyaspartic acid in the vitamin K-dependent blood coagulation zymogens. Biochem Biophys Res Commun. 1983 Aug 30;115(1):8–14. doi: 10.1016/0006-291x(83)90961-0. [DOI] [PubMed] [Google Scholar]
  123. Medof M. E., Lublin D. M., Holers V. M., Ayers D. J., Getty R. R., Leykam J. F., Atkinson J. P., Tykocinski M. L. Cloning and characterization of cDNAs encoding the complete sequence of decay-accelerating factor of human complement. Proc Natl Acad Sci U S A. 1987 Apr;84(7):2007–2011. doi: 10.1073/pnas.84.7.2007. [DOI] [PMC free article] [PubMed] [Google Scholar]
  124. Meijers J. C., Kanters D. H., Vlooswijk R. A., van Erp H. E., Hessing M., Bouma B. N. Inactivation of human plasma kallikrein and factor XIa by protein C inhibitor. Biochemistry. 1988 Jun 14;27(12):4231–4237. doi: 10.1021/bi00412a005. [DOI] [PubMed] [Google Scholar]
  125. Meijers J. C., Vlooswijk R. A., Kanters D. H., Hessing M., Bouma B. N. Identification of monoclonal antibodies that inhibit the function of protein C inhibitor. Evidence for heparin-independent inhibition of activated protein C in plasma. Blood. 1988 Oct;72(4):1401–1403. [PubMed] [Google Scholar]
  126. Melissari E., Kakkar V. V. The effects of oestrogen administration on the plasma free protein S and C4b-binding protein. Thromb Res. 1988 Mar 1;49(5):489–495. doi: 10.1016/s0049-3848(98)90006-8. [DOI] [PubMed] [Google Scholar]
  127. Melissari E., Nicolaides K. H., Scully M. F., Kakkar V. V. Protein S and C4b-binding protein in fetal and neonatal blood. Br J Haematol. 1988 Oct;70(2):199–203. doi: 10.1111/j.1365-2141.1988.tb02464.x. [DOI] [PubMed] [Google Scholar]
  128. Mitchell C. A., Hau L., Salem H. H. Control of thrombin mediated cleavage of protein S. Thromb Haemost. 1986 Oct 21;56(2):151–154. [PubMed] [Google Scholar]
  129. Moalic P., Gruel Y., Body G., Foloppe P., Delahousse B., Leroy J. Levels and plasma distribution of free and C4b-BP-bound protein S in human fetuses and full-term newborns. Thromb Res. 1988 Mar 1;49(5):471–480. doi: 10.1016/s0049-3848(98)90004-4. [DOI] [PubMed] [Google Scholar]
  130. Morita T., Mizuguchi J., Kawabata S., Iwanaga S. Proteolytic cleavage of vitamin K-dependent bovine plasma protein S by alpha-thrombin and plasma serine protease. J Biochem. 1986 Feb;99(2):561–568. doi: 10.1093/oxfordjournals.jbchem.a135512. [DOI] [PubMed] [Google Scholar]
  131. Morley B. J., Campbell R. D. Internal homologies of the Ba fragment from human complement component Factor B, a class III MHC antigen. EMBO J. 1984 Jan;3(1):153–157. doi: 10.1002/j.1460-2075.1984.tb01776.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  132. Nagasawa S., Ichihara C., Stroud R. M. Cleavage of C4b by C3b inactivator: production of a nicked form of C4b, C4b', as an intermediate cleavage product of C4b by C3b inactivator. J Immunol. 1980 Aug;125(2):578–582. [PubMed] [Google Scholar]
  133. Nagasawa S., Mizuguchi K., Ichihara C., Koyama J. Limited chymotryptic cleavage of human C4-binding protein: isolation of a carbohydrate-containing core domain and an active fragment. J Biochem. 1982 Oct;92(4):1329–1332. doi: 10.1093/oxfordjournals.jbchem.a134052. [DOI] [PubMed] [Google Scholar]
  134. Nagasawa S., Stroud R. M. Purification and characterization of a macromolecular weight cofactor for C3b-inactivator, C4bC3bINA-cofactor, of human plasma. Mol Immunol. 1980 Nov;17(11):1365–1372. doi: 10.1016/0161-5890(80)90005-x. [DOI] [PubMed] [Google Scholar]
  135. Nagasawa S., Unno H., Ichihara C., Koyama J., Koide T. Human C4b-binding protein, C4bp. Chymotryptic cleavage and location of the 48 kDa active fragment within C4bp. FEBS Lett. 1983 Nov 28;164(1):135–138. doi: 10.1016/0014-5793(83)80036-2. [DOI] [PubMed] [Google Scholar]
  136. Nelsestuen G. L., Kisiel W., Di Scipio R. G. Interaction of vitamin K dependent proteins with membranes. Biochemistry. 1978 May 30;17(11):2134–2138. doi: 10.1021/bi00604a017. [DOI] [PubMed] [Google Scholar]
  137. Nesheim M. E., Canfield W. M., Kisiel W., Mann K. G. Studies of the capacity of factor Xa to protect factor Va from inactivation by activated protein C. J Biol Chem. 1982 Feb 10;257(3):1443–1447. [PubMed] [Google Scholar]
  138. Nishioka J., Suzuki K. Inhibition of cofactor activity of protein S by a complex of protein S and C4b-binding protein. Evidence for inactive ternary complex formation between protein S, C4b-binding protein, and activated protein C. J Biol Chem. 1990 Jun 5;265(16):9072–9076. [PubMed] [Google Scholar]
  139. Nukatsuka M., Nagasawa S. Characterization of the interaction between human protein S and C4b-binding protein (C4bp). J Biochem. 1987 Sep;102(3):599–605. doi: 10.1093/oxfordjournals.jbchem.a122093. [DOI] [PubMed] [Google Scholar]
  140. Ogura M., Tanabe N., Nishioka J., Suzuki K., Saito H. Biosynthesis and secretion of functional protein S by a human megakaryoblastic cell line (MEG-01). Blood. 1987 Jul;70(1):301–306. [PubMed] [Google Scholar]
  141. Owen W. G., Esmon C. T. Functional properties of an endothelial cell cofactor for thrombin-catalyzed activation of protein C. J Biol Chem. 1981 Jun 10;256(11):5532–5535. [PubMed] [Google Scholar]
  142. Pan E. Y., Gomperts E. D., Millen R., Gilsanz V. Bone mineral density and its association with inherited protein S deficiency. Thromb Res. 1990 May 1;58(3):221–231. doi: 10.1016/0049-3848(90)90092-q. [DOI] [PubMed] [Google Scholar]
  143. Pardo-Manuel F., Rey-Campos J., Hillarp A., Dahlbäck B., Rodriguez de Cordoba S. Human genes for the alpha and beta chains of complement C4b-binding protein are closely linked in a head-to-tail arrangement. Proc Natl Acad Sci U S A. 1990 Jun;87(12):4529–4532. doi: 10.1073/pnas.87.12.4529. [DOI] [PMC free article] [PubMed] [Google Scholar]
  144. Park C. H., Tulinsky A. Three-dimensional structure of the kringle sequence: structure of prothrombin fragment 1. Biochemistry. 1986 Jul 15;25(14):3977–3982. doi: 10.1021/bi00362a001. [DOI] [PubMed] [Google Scholar]
  145. Paul M. S., Aegerter M., O'Brien S. E., Kurtz C. B., Weis J. H. The murine complement receptor gene family. Analysis of mCRY gene products and their homology to human CR1. J Immunol. 1989 Jan 15;142(2):582–589. [PubMed] [Google Scholar]
  146. Perkins S. J., Chung L. P., Reid K. B. Unusual ultrastructure of complement-component-C4b-binding protein of human complement by synchrotron X-ray scattering and hydrodynamic analysis. Biochem J. 1986 Feb 1;233(3):799–807. doi: 10.1042/bj2330799. [DOI] [PMC free article] [PubMed] [Google Scholar]
  147. Perkins S. J., Haris P. I., Sim R. B., Chapman D. A study of the structure of human complement component factor H by Fourier transform infrared spectroscopy and secondary structure averaging methods. Biochemistry. 1988 May 31;27(11):4004–4012. doi: 10.1021/bi00411a017. [DOI] [PubMed] [Google Scholar]
  148. Ploos van Amstel H. K., Reitsma P. H., Bertina R. M. The human protein S locus: identification of the PS alpha gene as a site of liver protein S messenger RNA synthesis. Biochem Biophys Res Commun. 1988 Dec 30;157(3):1033–1038. doi: 10.1016/s0006-291x(88)80978-1. [DOI] [PubMed] [Google Scholar]
  149. Ploos van Amstel H. K., Reitsma P. H., Hamulyák K., de Die-Smulders C. E., Mannucci P. M., Bertina R. M. A mutation in the protein S pseudogene is linked to protein S deficiency in a thrombophilic family. Thromb Haemost. 1989 Nov 24;62(3):897–901. [PubMed] [Google Scholar]
  150. Ploos van Amstel H. K., Reitsma P. H., van der Logt C. P., Bertina R. M. Intron-exon organization of the active human protein S gene PS alpha and its pseudogene PS beta: duplication and silencing during primate evolution. Biochemistry. 1990 Aug 28;29(34):7853–7861. doi: 10.1021/bi00486a011. [DOI] [PubMed] [Google Scholar]
  151. Ploos van Amstel H. K., van der Zanden A. L., Reitsma P. H., Bertina R. M. Human protein S cDNA encodes Phe-16 and Tyr 222 in consensus sequences for the post-translational processing. FEBS Lett. 1987 Sep 28;222(1):186–190. doi: 10.1016/0014-5793(87)80217-x. [DOI] [PubMed] [Google Scholar]
  152. Ploos van Amstel J. K., van der Zanden A. L., Bakker E., Reitsma P. H., Bertina R. M. Two genes homologous with human protein S cDNA are located on chromosome 3. Thromb Haemost. 1987 Dec 18;58(4):982–987. [PubMed] [Google Scholar]
  153. Porter R. R., Reid K. B. Activation of the complement system by antibody-antigen complexes: the classical pathway. Adv Protein Chem. 1979;33:1–71. doi: 10.1016/s0065-3233(08)60458-1. [DOI] [PubMed] [Google Scholar]
  154. Porter R. R., Reid K. B. The biochemistry of complement. Nature. 1978 Oct 26;275(5682):699–704. doi: 10.1038/275699a0. [DOI] [PubMed] [Google Scholar]
  155. Rees D. J., Jones I. M., Handford P. A., Walter S. J., Esnouf M. P., Smith K. J., Brownlee G. G. The role of beta-hydroxyaspartate and adjacent carboxylate residues in the first EGF domain of human factor IX. EMBO J. 1988 Jul;7(7):2053–2061. doi: 10.1002/j.1460-2075.1988.tb03045.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  156. Reid K. B., Gagnon J. Human C4-binding protein: N-terminal amino acid sequence analysis and limited proteolysis by trypsin. FEBS Lett. 1982 Jan 11;137(1):75–79. doi: 10.1016/0014-5793(82)80318-9. [DOI] [PubMed] [Google Scholar]
  157. Reveille J. D., Arnett F. C., Wilson R. W., Bias W. B., McLean R. H. Null alleles of the fourth component of complement and HLA haplotypes in familial systemic lupus erythematosus. Immunogenetics. 1985;21(4):299–311. doi: 10.1007/BF00430796. [DOI] [PubMed] [Google Scholar]
  158. Ripoche J., Day A. J., Harris T. J., Sim R. B. The complete amino acid sequence of human complement factor H. Biochem J. 1988 Jan 15;249(2):593–602. doi: 10.1042/bj2490593. [DOI] [PMC free article] [PubMed] [Google Scholar]
  159. Rittner C., Meier E. M., Stradmann B., Giles C. M., Köchling R., Mollenhauer E., Kreth H. W. Partial C4 deficiency in subacute sclerosing panencephalitis. Immunogenetics. 1984;20(4):407–415. doi: 10.1007/BF00345615. [DOI] [PubMed] [Google Scholar]
  160. 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]
  161. 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]
  162. Rogers J. S., 2nd, Murgo A. J., Fontana J. A., Raich P. C. Chemotherapy for breast cancer decreases plasma protein C and protein S. J Clin Oncol. 1988 Feb;6(2):276–281. doi: 10.1200/JCO.1988.6.2.276. [DOI] [PubMed] [Google Scholar]
  163. Rose P. G., Essig G. F., Vaccaro P. S., Brandt J. T. Protein S deficiency in pregnancy. Am J Obstet Gynecol. 1986 Jul;155(1):140–141. doi: 10.1016/0002-9378(86)90097-9. [DOI] [PubMed] [Google Scholar]
  164. Saeki T., Hirose S., Nukatsuka M., Kusunoki Y., Nagasawa S. Evidence that C4b-binding protein is an acute phase protein. Biochem Biophys Res Commun. 1989 Nov 15;164(3):1446–1451. doi: 10.1016/0006-291x(89)91832-9. [DOI] [PubMed] [Google Scholar]
  165. Sakata Y., Curriden S., Lawrence D., Griffin J. H., Loskutoff D. J. Activated protein C stimulates the fibrinolytic activity of cultured endothelial cells and decreases antiactivator activity. Proc Natl Acad Sci U S A. 1985 Feb;82(4):1121–1125. doi: 10.1073/pnas.82.4.1121. [DOI] [PMC free article] [PubMed] [Google Scholar]
  166. Sata T., Havel R. J., Kotite L., Kane J. P. New protein in human blood plasma, rich in proline, with lipid-binding properties. Proc Natl Acad Sci U S A. 1976 Apr;73(4):1063–1067. doi: 10.1073/pnas.73.4.1063. [DOI] [PMC free article] [PubMed] [Google Scholar]
  167. 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]
  168. Schmidel D. K., Tatro A. V., Phelps L. G., Tomczak J. A., Long G. L. Organization of the human protein S genes. Biochemistry. 1990 Aug 28;29(34):7845–7852. doi: 10.1021/bi00486a010. [DOI] [PubMed] [Google Scholar]
  169. Schwalbe R., Dahlbäck B., Hillarp A., Nelsestuen G. Assembly of protein S and C4b-binding protein on membranes. J Biol Chem. 1990 Sep 25;265(27):16074–16081. [PubMed] [Google Scholar]
  170. Schwarz H. P., Fischer M., Hopmeier P., Batard M. A., Griffin J. H. Plasma protein S deficiency in familial thrombotic disease. Blood. 1984 Dec;64(6):1297–1300. [PubMed] [Google Scholar]
  171. Schwarz H. P., Heeb M. J., Lottenberg R., Roberts H., Griffin J. H. Familial protein S deficiency with a variant protein S molecule in plasma and platelets. Blood. 1989 Jul;74(1):213–221. [PubMed] [Google Scholar]
  172. Schwarz H. P., Heeb M. J., Lämmle B., Berrettini M., Griffin J. H. Quantitative immunoblotting of plasma and platelet protein S. Thromb Haemost. 1986 Dec 15;56(3):382–386. [PubMed] [Google Scholar]
  173. Schwarz H. P., Heeb M. J., Wencel-Drake J. D., Griffin J. H. Identification and quantitation of protein S in human platelets. Blood. 1985 Dec;66(6):1452–1455. [PubMed] [Google Scholar]
  174. Schwarz H. P., Muntean W., Watzke H., Richter B., Griffin J. H. Low total protein S antigen but high protein S activity due to decreased C4b-binding protein in neonates. Blood. 1988 Mar;71(3):562–565. [PubMed] [Google Scholar]
  175. Stenflo J., Jönsson M. Protein S, a new vitamin K-dependent protein from bovine plasma. FEBS Lett. 1979 May 15;101(2):377–381. doi: 10.1016/0014-5793(79)81048-0. [DOI] [PubMed] [Google Scholar]
  176. Stenflo J., Lundwall A., Dahlbäck B. beta-Hydroxyasparagine in domains homologous to the epidermal growth factor precursor in vitamin K-dependent protein S. Proc Natl Acad Sci U S A. 1987 Jan;84(2):368–372. doi: 10.1073/pnas.84.2.368. [DOI] [PMC free article] [PubMed] [Google Scholar]
  177. Stern D. M., Nawroth P. P., Harris K., Esmon C. T. Cultured bovine aortic endothelial cells promote activated protein C-protein S-mediated inactivation of factor Va. J Biol Chem. 1986 Jan 15;261(2):713–718. [PubMed] [Google Scholar]
  178. Stern D., Brett J., Harris K., Nawroth P. Participation of endothelial cells in the protein C-protein S anticoagulant pathway: the synthesis and release of protein S. J Cell Biol. 1986 May;102(5):1971–1978. doi: 10.1083/jcb.102.5.1971. [DOI] [PMC free article] [PubMed] [Google Scholar]
  179. Stoolman L. M. Adhesion molecules controlling lymphocyte migration. Cell. 1989 Mar 24;56(6):907–910. doi: 10.1016/0092-8674(89)90620-x. [DOI] [PubMed] [Google Scholar]
  180. Sugo T., Dahlbäck B., Holmgren A., Stenflo J. Calcium binding of bovine protein S. Effect of thrombin cleavage and removal of the gamma-carboxyglutamic acid-containing region. J Biol Chem. 1986 Apr 15;261(11):5116–5120. [PubMed] [Google Scholar]
  181. Suzuki K., Nishioka J. Binding site for vitamin K-dependent protein S on complement C4b-binding protein. J Biol Chem. 1988 Nov 15;263(32):17034–17039. [PubMed] [Google Scholar]
  182. Suzuki K., Nishioka J., Hashimoto S. Protein C inhibitor. Purification from human plasma and characterization. J Biol Chem. 1983 Jan 10;258(1):163–168. [PubMed] [Google Scholar]
  183. Suzuki K., Nishioka J., Hashimoto S. Regulation of activated protein C by thrombin-modified protein S. J Biochem. 1983 Sep;94(3):699–705. doi: 10.1093/oxfordjournals.jbchem.a134409. [DOI] [PubMed] [Google Scholar]
  184. Suzuki K., Nishioka J., Matsuda M., Murayama H., Hashimoto S. Protein S is essential for the activated protein C-catalyzed inactivation of platelet-associated factor Va. J Biochem. 1984 Aug;96(2):455–460. doi: 10.1093/oxfordjournals.jbchem.a134857. [DOI] [PubMed] [Google Scholar]
  185. Suzuki K., Stenflo J., Dahlbäck B., Teodorsson B. Inactivation of human coagulation factor V by activated protein C. J Biol Chem. 1983 Feb 10;258(3):1914–1920. [PubMed] [Google Scholar]
  186. Tokunaga F., Miyata T., Nakamura T., Morita T., Kuma K., Miyata T., Iwanaga S. Lipopolysaccharide-sensitive serine-protease zymogen (factor C) of horseshoe crab hemocytes. Identification and alignment of proteolytic fragments produced during the activation show that it is a novel type of serine protease. Eur J Biochem. 1987 Sep 15;167(3):405–416. doi: 10.1111/j.1432-1033.1987.tb13352.x. [DOI] [PubMed] [Google Scholar]
  187. Tomonaga M., Yoshida Y., Tagawa M., Jinnai I., Kuriyama K., Amenomori T., Yoshioka A., Matsuo T., Nonaka H., Ichimaru M. Cytochemistry of acute promyelocytic leukemia (M3): leukemic promyelocytes exhibit heterogeneous patterns in cellular differentiation. Blood. 1985 Aug;66(2):350–357. [PubMed] [Google Scholar]
  188. Tosi M., Duponchel C., Meo T., Julier C. Complete cDNA sequence of human complement Cls and close physical linkage of the homologous genes Cls and Clr. Biochemistry. 1987 Dec 29;26(26):8516–8524. doi: 10.1021/bi00400a004. [DOI] [PubMed] [Google Scholar]
  189. Van de Waart P., Bruls H., Hemker H. C., Lindhout T. Functional properties of factor Va subunits after proteolytic alterations by activated protein C. Biochim Biophys Acta. 1984 May 25;799(1):38–44. doi: 10.1016/0304-4165(84)90324-6. [DOI] [PubMed] [Google Scholar]
  190. Vehar G. A., Davie E. W. Preparation and properties of bovine factor VIII (antihemophilic factor). Biochemistry. 1980 Feb 5;19(3):401–410. doi: 10.1021/bi00544a001. [DOI] [PubMed] [Google Scholar]
  191. Vigano-D'Angelo S., D'Angelo A., Kaufman C. E., Jr, Sholer C., Esmon C. T., Comp P. C. Protein S deficiency occurs in the nephrotic syndrome. Ann Intern Med. 1987 Jul;107(1):42–47. doi: 10.7326/0003-4819-107-1-42. [DOI] [PubMed] [Google Scholar]
  192. Villiers M. B., Reboul A., Thielens N. M., Colomb M. G. Purification and characterization of C4-binding protein from human serum. FEBS Lett. 1981 Sep 14;132(1):49–54. doi: 10.1016/0014-5793(81)80425-5. [DOI] [PubMed] [Google Scholar]
  193. Walker F. J. Characterization of a synthetic peptide that inhibits the interaction between protein S and C4b-binding protein. J Biol Chem. 1989 Oct 25;264(30):17645–17648. [PubMed] [Google Scholar]
  194. Walker F. J., Chavin S. I., Fay P. J. Inactivation of factor VIII by activated protein C and protein S. Arch Biochem Biophys. 1987 Jan;252(1):322–328. doi: 10.1016/0003-9861(87)90037-3. [DOI] [PubMed] [Google Scholar]
  195. Walker F. J. Interactions of protein S with membranes. Semin Thromb Hemost. 1988 Apr;14(2):216–221. doi: 10.1055/s-2007-1002780. [DOI] [PubMed] [Google Scholar]
  196. Walker F. J. Protein S and the regulation of activated protein C. Semin Thromb Hemost. 1984 Apr;10(2):131–138. doi: 10.1055/s-2007-1004415. [DOI] [PubMed] [Google Scholar]
  197. Walker F. J. Regulation of activated protein C by a new protein. A possible function for bovine protein S. J Biol Chem. 1980 Jun 25;255(12):5521–5524. [PubMed] [Google Scholar]
  198. Walker F. J. Regulation of activated protein C by protein S. The role of phospholipid in factor Va inactivation. J Biol Chem. 1981 Nov 10;256(21):11128–11131. [PubMed] [Google Scholar]
  199. Walker F. J. Regulation of bovine activated protein C by protein S: the role of the cofactor protein in species specificity. Thromb Res. 1981 May 1;22(3):321–327. doi: 10.1016/0049-3848(81)90125-0. [DOI] [PubMed] [Google Scholar]
  200. Walker F. J. Regulation of vitamin K-dependent protein S. Inactivation by thrombin. J Biol Chem. 1984 Aug 25;259(16):10335–10339. [PubMed] [Google Scholar]
  201. Walker F. J., Sexton P. W., Esmon C. T. The inhibition of blood coagulation by activated Protein C through the selective inactivation of activated Factor V. Biochim Biophys Acta. 1979 Dec 7;571(2):333–342. doi: 10.1016/0005-2744(79)90103-7. [DOI] [PubMed] [Google Scholar]
  202. Watkins P. C., Eddy R., Fukushima Y., Byers M. G., Cohen E. H., Dackowski W. R., Wydro R. M., Shows T. B. The gene for protein S maps near the centromere of human chromosome 3. Blood. 1988 Jan;71(1):238–241. [PubMed] [Google Scholar]
  203. 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]
  204. Weis J. J., Toothaker L. E., Smith J. A., Weis J. H., Fearon D. T. Structure of the human B lymphocyte receptor for C3d and the Epstein-Barr virus and relatedness to other members of the family of C3/C4 binding proteins. J Exp Med. 1988 Mar 1;167(3):1047–1066. doi: 10.1084/jem.167.3.1047. [DOI] [PMC free article] [PubMed] [Google Scholar]
  205. Weiss P., Soff G. A., Halkin H., Seligsohn U. Decline of proteins C and S and factors II, VII, IX and X during the initiation of warfarin therapy. Thromb Res. 1987 Mar 15;45(6):783–790. doi: 10.1016/0049-3848(87)90088-0. [DOI] [PubMed] [Google Scholar]
  206. Ziccardi R. J., Dahlback B., Müller-Eberhard H. J. Characterization of the interaction of human C4b-binding protein with physiological ligands. J Biol Chem. 1984 Nov 25;259(22):13674–13679. [PubMed] [Google Scholar]
  207. Zimmermann D. R., Ruoslahti E. Multiple domains of the large fibroblast proteoglycan, versican. EMBO J. 1989 Oct;8(10):2975–2981. doi: 10.1002/j.1460-2075.1989.tb08447.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  208. van Hinsbergh V. W., Bertina R. M., van Wijngaarden A., van Tilburg N. H., Emeis J. J., Haverkate F. Activated protein C decreases plasminogen activator-inhibitor activity in endothelial cell-conditioned medium. Blood. 1985 Feb;65(2):444–451. [PubMed] [Google Scholar]
  209. von Zabern I., Bloom E. L., Chu V., Gigli I. The fourth component of human complement treated with amines or chaotropes or frozen-thawed (C4b-like C4): interaction with C4 binding protein and cleavage by C3b/C4b inactivator. J Immunol. 1982 Mar;128(3):1433–1438. [PubMed] [Google Scholar]
  210. von Zabern I., Gigli I. Conformational changes in complement component C4 induced by activation, treatment with amines, chaotropes, or freezing-thawing, detectable by radioiodination using lactoperoxidase. J Immunol. 1982 Mar;128(3):1439–1442. [PubMed] [Google Scholar]

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