Factor VIII-factor IX interactions: Molecular sites involved in enzyme-cofactor complex assembly

Thrombosis and Haemostasis

Volumn 82, Issue 2, 1999, Pages 209-217

  Mertens, Koen ^a   Celie, Patrick H N ^a   Kolkman, Joost A ^a   Lenting, Peter J ^a  

^a CENTRAL LABORATORY   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

ACTIVATED PROTEIN C; BLOOD CLOTTING FACTOR 10; BLOOD CLOTTING FACTOR 10A; BLOOD CLOTTING FACTOR 8; BLOOD CLOTTING FACTOR 9; BLOOD CLOTTING FACTOR 9A;

AMINO ACID SEQUENCE; AMINO ACID SUBSTITUTION; BINDING SITE; BLOOD CLOTTING; CONFERENCE PAPER; ENZYME BINDING; HEMOPHILIA A; HUMAN; MOLECULAR MODEL; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN DOMAIN; PROTEIN PROTEIN INTERACTION;

EID: 0032722436     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-0037-1615835     Document Type: Conference Paper

References (71)

1. Mann KG, Nesheim ME, Church WR, Haley P, Krishnaswamy S. Surface dependent reactions of the vitamin K-dependent enzyme complexes. Stood. 1990;76:1-16.
2. Bode W, Brandstetter H, Mather T, Stubbs MT. Comparative analysis of haemostatic proteinases: structural aspects of thrombin, factor Xa, factor IXa and protein C. Thromb Haemost. 1997;78:501-511.
3. Lenting PJ, Van Mourik JA, Mertens K. The life cycle of coagulation factor VIII in view of its structure and function. Blood. 1998;92:3983-3996.
4. Harlos K, Martin DMA, O'Brien DP, Jones EY, Stuart DI, Polikarpov I, Miller A, Tuddenham EGD, Boys CWG. Crystal structure of the extracellular region of human tissue factor. Nature. 1994;370:662-666.
5. Banner DW, D'Arcy A, Chène C, Winkler FK, Guha A, Konigsberg WH, Nemerson Y, Kirchhofer D. The crystal structure of the complex of blood coagulation factor Vila with soluble tissue factor. Nature. 1996;380:41-46.
6. Edgington TS, Dickinson CD, Ruf W. The structural basis of function of the TF:VIIa complex in the cellular initiation of coagulation. Thromb Haemost. 1997;78:401-405.
7. Pan Y, DeFay T, Gitschier J, Cohen FE. Proposed structure of the A domains of factor VIII by homology modelling. Nat Struct Biol. 1995;2:740-744.
8. Pemberton S, Lindley P, Zaitsev V, Card G, Tuddenham EGD, Kemball-Cook G. A molecular model for the triplicated A domains of human factor VIII based on the crystal structure of human ceruloplasmin. Blood. 1997;89:2413-2421.
9. Pellequer J-L, Gale AJ, Griffin JH, Getzoff ED. Homology models of the C domains of blood coagulation factors V and VIII: a proposed membrane binding mode for FV and FVIII C2 domains. Blood Cells Mol Dis. 1998;24:448-461.
10. Macfarlane RG. An enzyme cascade in the blood clotting mechanism and its function as a biological amplifier. Nature. 1964;202:498-499.
11. Davie EW, Ratnoff OD. Waterfall sequence for intrinsic blood clotting. Science. 1964;145:1310-1312.
12. Van Dieijen G, Tans G, Rosing J, Hemker HC. The role of phospholipid and factor VIIIa in the activation of bovine factor X. J Biol Chem. 1981;256:3433-3442.
13. 2+ and phospholipids to the activation of human blood coagulation factor X by activated factor IX. Biochem J. 1984;223:607-615.
14. Mertens K, Van Wijngaarden A, Bertina RM. The role of factor VIII in the activation of human blood coagulation factor X by activated factor IX. Thromb Haemost. 1985;54:654-660.
15. Furie B, Furie BC. The molecular basis of blood coagulation. Cell. 1988;53:505-518.
16. Brandstetter H, Bauer M, Huber R, Lollar P, Bode W. X-ray structure of clotting factor IXa: active site and module structure related to Xase activity and hemophilia B. Proc Natl Acad Sci USA. 1995;92:9796-9800.
17. Wood WI, Capon DJ, Simonson CC, Eaton DL, Gitschier J, Keyt B, Seeburg PH, Smith DH, Hollingshead P, Wion KL, Delwart E, Tuddenham EGD, Vehar GA, Lawn RM. Expression of active human factor VIII from recombinant DNA clones. Nature. 1984;312:330-337
18. Vehar GA, Keyt B, Eaton D, Rodriguez H, O'Brien DP, Rotblat F, Oppermann H, Keck R, Wood WI, Harkins RN, Tuddenham EGD, Lawn RM, Capon DJ. Structure of human factor VIII. Nature. 1984;312:337-342.
19. Toole JJ, Knopf JL, Wozney JM, Sultzman LA, Bueker JL, Pittman DD, Kaufman RJ, Brown E, Shoemaker C, Orr EC, Amphlett GW, Foster WB, Coe ML, Knutson GJ, Fass DN, Hewick RM. Molecular cloning of a cDNA encoding human antihaemophilic factor. Nature. 1984;312:342-347.
20. Church WR, Jernigan RL, Toole J, Hewick RM, Knopf J, Knutson GJ, Nesheim ME, Mann KG, Fass DN. Coagulation factors V and VIII and ceruloplasmin constitute a family of structurally related proteins. Proc Natl Acad Sci USA. 1984;81:6934-6937.
21. Kaufman RJ. Post-translational modifications required for coagulation factor secretion and function. Thromb Haemost. 1998;79:1068-1079.
22. Mertens K, Cupers R, Van Wijngaarden A, Bertina RM. Binding of human blood coagulation factors IXa and X to phospholipid membranes. Biochem J. 1984;223:599-605.
23. Freedman SJ, Blostein MD, Baleja JD, Jacobs M, Furie BC, Furie B. Identification of the phospholipid binding site in the vitamin K-dependent blood coagulation protein factor IX. J Biol Chem. 1996;271:16227-16236.
24. Ahmad SS, Wong MY, Rawala R, Jameson BA, Walsh PN. Coagulation factor IX residues G4-Q11 mediate its interaction with a shared factor IX/IXa binding site on activated platelets but not the assembly of the functional factor X activating complex. Biochemistry. 1998;37:1671-1679.
25. Cheung WF, Van den Born J, Kuhn K, Kjellen L, Hudson BG, Stafford DW. Identification of the endothelial cell binding site for factor IX. Proc Natl Acad Sci USA. 1996;93:11068-11073.
26. Mutucumarana VP, Duffy EJ, Lollar P, Johnson AE. The active site of factor IXa is located far above the membrane surface and its conformation is altered upon association with factor VIIIa. J Biol Chem. 1992;267:17012-17021.
27. Stoylova S, Gray E, Barrowcliffe TW, Kemball-Cook G, Holzenburg A. Structural determination of lipid-bound human blood coagulation factor IX. Biochim Biophys Acta. 1998;1383:175-178.
28. Gilbert GE, Drinkwater D, Barter S, Clouse SB. Specificity of phosphatidylserine-containing membrane binding sites for factor VIII. J Biol Chem. 1992;267:15861-15868.
29. Gilbert GE, Furie BC, Furie B. Binding of human factor VIII to phospholipids. J Biol Chem. 1990;265:815-822.
30. Gilbert GE, Drinkwater D. Specific membrane binding of factor VIII is mediated by O-phospho-L-serine, a moiety of phosphatidylserine. Biochemistry. 1993;32:9577-9585.
31. Spaargaren J, Giessen PLA, Janssen MP, Voorberg J, Willems GM, van Mourik JA. Binding of blood coagulation factor VIII and its light chain to phosphatidylserine/phosphatidylcholine bilayers as measured by ellipsometry. Biochem J. 1995;310:539-545.
32. Foster PA, Fulcher CA, Houghten RA, Zimmerman TS. Synthetic factor VIII peptides with amino acid sequences contained within the C2 domain of factor VIII inhibit factor VIII binding to phosphatidyl serine. Blood. 1990;75:1999-2004.
33. Lenting PJ, Donath MJSH, van Mourik JA, Mertens K. Identification of a binding site for blood coagulation factor IXa on the light chain of human factor VIII. J Biol Chem. 1994;269:7150-7155.
34. O'Brien LM, Medved LV, Fay PJ. Localization of factor IXa and factor VIIIa interactive sites. J Biol Chem. 1995;270:27087-27092.
35. Fay PJ, Koshibu K. The A2 subunit of factor VIIIa modulates the active site of factor IXa. J Biol Chem. 1998;273:19049-19054.
36. Lapan KA, Fay PJ. Localization of a factor X interactive site in the A1 subunit of factor VIIIa. J Biol Chem. 1997;272:2082-2088.
37. Lapan KA, Fay PJ. Interaction of the A1 subunit of factor VIIIa and the serine protease domain of factor X identified by zero-length cross-linking. Thromb Haemost. 1998;80:418-422.
38. Lenting PJ, ter Maat H, Clijsters PPFM, Donath MJSH, van Mourik JA, Mertens K. Cleavage at arginine 145 in human blood coagulation factor IX converts the zymogen into a factor VIII binding enzyme. J Biol Chem. 1995;270:14884-14890.
39. Lollar P, Hill-Eubanks DC, Parker CG. Association of the factor VIII light chain with von Willebrand factor. J Biol Chem. 1988;263:10451-10455.
40. Leyte A, Verbeet MP, Brodniewicz-Proba T, van Mourik JA, Mertens K. The interaction between human blood coagulation factor VIII and von Willebrand factor. Biochem J. 1989;257:679-683.
41. Saenko EL, Scandella D. The acidic region of the factor VIII light chain and the C2 domain together form the high affinity binding site for von Willebrand factor. J Biol Chem. 1997;272,18007-18014.
42. Andersson LO, Brown JE. Interaction of factor VIII-von Willebrand factor with phospholipid vesicles. Biochem J. 1981;200:161-167.
43. Laimanovich A, Hudry-Clergeon G, Freyssinet J-M, Marguerie G. Human factor VIII procoagulant activity and phospholipid interaction. Biochim Biophys Acta. 1981;678:132-136.
44. Saenko EL, Scandella D. A mechanism for inhibition of factor VIII binding to phospholipid by von Willebrand factor. J Biol Chem. 1995;270:13826-13833.
45. Saenko EL, Scandella D, Yakhyaev AV, Greco NJ. Activation of factor VIII by thrombin increases its affinity for binding to synthetic phospholipid membranes and activated platelets. J Biol Chem. 1998;273:27918-27926.
46. Duffy EJ, Parker ET, Mutucumarana VP, Johnson AE, Lollar P. Binding of factor VIIIa and factor VIII to factor IXa on phospholipid vesicles. J Biol Chem. 1992;267:17006-17011.
47. Lamphear BJ, Fay PJ. Factor IXa enhances reconstitution of factor VIIIa from isolated A2 subunit and A1/A3-C1-C2 dimer. J Biol Chem. 1992;267:3725-3730.
48. Fay PJ, Beattie T, Huggins CF, Regan LM. Factor VIIIa A2 subunit residues 558-565 represent a factor IXa interactive site. J Biol Chem. 1994;269:20522-20527.
49. Gilbert GE, Arena AA. Activation of the factor VIIIa-factor IXa enzyme complex of blood coagulation by membranes containing phosphatidyl-L-serine. J Biol Chem. 1996;271:11120-11125.
50. Mathur A, Zhong D, Sabharwal AK, Smith KJ, Bajaj SP. Interaction of factor IXa with factor VIIIa. J Biol Chem. 1997;272:23418-23426.
51. Jorquera JI, McClintock RA, Roberts JR, MacDonald MJ, Houghten RA, Fulcher CA. Synthetic peptides derived from residues 698 to 710 of factor VIII inhibit factor IXa activity. Circulation. 1992;86:685a.
52. 531 substitution variants of factor VIII associated with mild haemophilia. Thromb Haemost. 1997;77(Suppl):570a.
53. Kemball-Cook G, Tuddenham EGD. The factor VIII mutation database on the world wide web: the haemophilia A mutation, search, test and resource site. Hamsters update (version 3.0, http://europium.mrc.rpms.ac.uk). Nucl Acid Res. 1997;25:128-132.
54. Amano K, Sarkar R, Pemberton S, Kemball-Cook G, Kazazian HH, Kaufman RJ. The molecular basis for cross-reacting material-positive hemophilia A due to missense mutations within the A2-domain of factor VIII. Blood. 1998;91:538-548.
55. Liles DK, Monroe DM, Roberts HR. The factor VIII peptide consisting of amino acids 698 to 712 enhances factor IXa cleavage of factor X. Blood. 1997;90(Suppl 1):463a.
56. Mertens K, van Wijngaarden A, Bertina RM, Veltkamp JJ. The functional defect of factor VIII Leiden, a genetic variant of coagulation factor VIII. Thromb Haemost. 1985;54:650-653.
57. Pieneman WC, Fay PJ, Briët E, Reitsma PH, Bertina RM. Partial reconstitution of factor VIII activity from a mild CRM+ hemophilia A patient by replacement of the defective A2 domain. Thromb Haemost. 1998;79:943-948.
58. Kolkman JA, Lenting PJ, Mertens K. Regions 301-303 and 333-339 in the catalytic domain of blood coagulation factor IX are factor-VIII interactive sites involved in stimulation of enzyme activity. Biochem J. 1999;339:217-221.
59. Chang J, Jin J, Lollar P, Bode W, Brandstetter H, Hamaguchi N, Straight DL, Stafford DW. Changing residue 338 in human factor IX from Arginine to Alanine causes an increase in catalytic activity. J Biol Chem. 1998;273:12089-12094.
60. Mathur A, Zhong D, Smith KJ, Bajaj SP. Protease and EGF1 domains of factor IXa play distinct roles in binding to factor VIIIa. Blood. 1998;92;252a-253a.
61. Chang JL, Lin JP, Straight DL, Stafford DW. Residues 330-338 in catalytic domain of factor IX represent an interactive site for both factor VIIIa and factor X. Blood. 1998;92:184a.
62. 1818 of human blood coagulation factor VIII comprises a binding site for activated factor IX. J Biol Chem. 1996;271:1935-1940.
63. Fijnvandraat K, Celie PHN, Turenhout E, ten Cate JW, van Mourik JA, Mertens K, Peters M, Voorberg J. A human alloantibody interferes with binding of factor IXa to the factor VIII light chain. Blood. 1998;91:2347-2352.
64. Zhong D, Saenko EL, Shima M, Felch M, Scandella D. Some human inhibitor antibodies interfere with factor VIII binding to factor IX. Blood. 1998;92:136-142.
65. 2+ binding to the first epidermal growth factor-like domain of human blood coagulation factor IX promotes enzyme activity and factor VIII light chain binding. J Biol Chem. 1996;271:25332-25337.
66. 94 provide a link between both epidermal growth factor-like domains that is crucial in the interaction with factor VIII light chain. J Biol Chem. 1998;273:222-227.
67. Lin SW, Smith KJ, Welsch D, Stafford DW. Expression and characterization of human factor IX and factor IX-factor X chimeras in mouse C127 cells. J Biol Chem. 1990;265:144-150.
68. Chang JY, Monroe DM, Stafford DW, Brinkhous KM, Roberts HR. Replacing the first epidermal growth factor-like domain of factor IX with that of factor VII enhances activity in vitro and in canine hemophilia B. J Clin Invest. 1997;100:886-892.
69. Larson PJ, Stanfield-Oakly S, van Dusen WJ, Kasper CK, Smith KJ, Monroe DM, High KA. Structural integrity of the gamma-carboxyglutamic acid domain of human blood coagulation factor IXa is required for its binding to cofactor VIIIa. J Biol Chem. 1996;271:3869-3876.
70. Hughes PE, Morgan G, Rooney EK, Brownlee GG, Handford PA. Tyrosine 69 of the first epidermal growth factor-like domain of human factor IX is essential for clotting activity. J Biol Chem. 1993;268:17727-17773.
71. 92 by His in the second epidermal growth factor-like domain results in defective interaction with factors VIIIa/X. J Biol Chem. 1993;268:24041-24046.