Factor VIII and von Willebrand factor

Thrombosis and Haemostasis

Volumn 79, Issue 3, 1998, Pages 456-465

  Vlot, André J ^a   Koppelman, Stefan J ^a   Bouma, Bonno N ^a   Sixma, Jan J ^a  

^a UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

BLOOD CLOTTING FACTOR 8; BLOOD CLOTTING FACTOR 8A; VON WILLEBRAND FACTOR;

BINDING SITE; BLOOD CLOTTING; CARBOXY TERMINAL SEQUENCE; CHROMOSOME XQ; HEMOPHILIA A; HEMOSTASIS; HUMAN; MOLECULAR WEIGHT; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN DOMAIN; PROTEIN PROTEIN INTERACTION; PROTEIN STRUCTURE; PROTEIN SYNTHESIS; REVIEW; STOICHIOMETRY; VON WILLEBRAND DISEASE;

EID: 0031932313     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-0037-1614927     Document Type: Review

References (177)

1. Hoyer LW. The factor VIII complex: structure and function. Blood 1981; 58: 1-13.
2. Wion KL, Kelly D, Summerfield JA, Tuddenham EG, Lawn RM. Distribution of factor VIII mRNA and antigen in human liver and other tissues. Nature 1985; 317: 726-9.
3. 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: 3433-42.
4. Jaffe EA, Hoyer LW, Nachman R. Synthesis of antihemophilic factor antigen by human endothelial cells. J Clin Invest 1973; 52: 2757-64.
5. Wagner DD, Marder VJ. Biosynthesis of von Willebrand protein by human endothelial cells. J Biol Chem 1983; 258: 2065-7.
6. Nachman R, Levine R, Jaffe EA. Synthesis of factor VIII antigen by cultured guinea pig megakaryocytes. J Clin Invest 1977; 60: 914-21.
7. Weiss HJ, Baumgartner HR, Tschopp TB, Turitto VT, Cohen D. Correction by factor VIII of the impaired platelet adhesion to subendothelium in von Willebrand disease. Blood 1978; 51: 267-79.
8. Sakariassen KS, Bolhuis PA, Sixma JJ. Human blood platelet adhesion to artery subendothelium is mediated by factor VIII-Von Willebrand factor bound to the subendothelium. Nature 1979; 279: 636-8.
9. Zimmerman TS, Ruggeri ZM. Von Willebrand's disease. Prog Hemost Thromb 1982; 6: 203-36.
10. Kaufman RJ. Biological regulation of factor VIII activity. Annu Rev Med 1992; 43: 325-39.
11. Leyte A, Mertens K, Distel B, Evers RF, de Keyzer-Nellen MJ, Groenen-van Dooren MM, De Bruin J, Pannekoek H, van Mourik JA, Verbeet MP. Inhibition of human coagulation factor VIII by monoclonal antibodies. Mapping of functional epitopes with the use of recombinant factor VIII fragments. Biochem J 1989; 263: 187-94.
12. Leyte A, Van Schijndel HB, Niehrs C, Huttner WB, Verbeet MP, Mertens K, van Mourik JA. Sulfation of Tyr1680 of human blood coagulation factor VIII is essential for the interaction of factor VIII with von Willebrand factor. J Biol Chem 1991; 266: 740-6.
13. Foster PA, Fulcher CA, Houghten RA, Zimmerman TS. A synthetic factor VIII peptide of eight amino acid residues (1677-1684) contains the binding region of an anti-factor VIII antibody which inhibits the binding of factor VIII to von Willebrand factor. Thromb Haemost 1990; 63: 403-6.
14. Fressinaud E, Baruch D, Rothschild C, Baumgartner HR, Meyer D. Platelet von Willebrand factor: evidence for its involvement in platelet adhesion to collagen. Blood 1987; 70: 1214-17.
15. Sixma JJ, Schiphorst ME, Verweij CL, Pannekoek H. Effect of deletion of the A1 domain of von Willebrand factor on its binding to heparin, collagen and platelets in the presence of ristocetin. Eur J Biochem 1991; 196: 369-75.
16. Koppelman SJ, Hoeij van M, Vink T, Lankhof H, Schiphorst ME, Damas C, Vlot AJ, Wise RJ, Bouma BN, Sixma JJ. Requirements of von Willebrand factor to protect factor VIII against degradation by activated protein C. Blood 1996; 87: 2292-300.
17. Weiss HJ, Sussman II, Hoyer LW. Stabilization of factor VIII in plasma by the von Willebrand factor. Studies on posttransfusion and dissociated factor VIII and in patients with von Willebrand's disease. J Clin Invest 1977; 60: 390-404.
18. Over J, Sixma JJ, Bouma BN, Bolhuis PA, Vlooswijk RA, Beeser-Visser NH. Survival of iodine-125-labeled factor VIII in patients with von Willebrand's disease. J Lab Clin Med 1981; 97: 332-44.
19. Koedam JA, Meijers JC, Sixma JJ, Bouma BN. 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; 82: 1236-43.
20. Koedam JA, Hamer RJ, Beeser-Visser NH, Bouma BN, Sixma JJ. The effect of von Willebrand factor on activation of factor VIII by factor Xa. Eur J Biochem 1990; 189: 229-34.
21. Foster PA, Fulcher CA, Marti T, Titani K, Zimmerman TS. A major factor VIII binding domain resides within the amino-terminal 272 amino acid residues of von Willebrand factor. J Biol Chem 1987; 262: 8443-6.
22. Lollar P, Hill-Eubanks DC, Parker CG. Association of the factor VIII light chain with von Willebrand factor. J Biol Chem 1988; 263: 10451-5.
23. Fay PJ, Coumans JV, Walker FJ. von Willebrand factor mediates protection of factor VIII from activated protein C-catalyzed inactivation. J Biol Chem 1991; 266: 2172-7.
24. Layet S, Girma JP, Obert B, Peynaud-Debayle E, Bihoreau N, Meyer D. Evidence that a secondary binding and protecting site for factor VIII on von Willebrand factor is highly unlikely. Biochem J 1992; 282: 129-37.
25. Nishino M, Girma JP, Rothschild C, Fressinaud E, Meyer D. New variant of von Willebrand disease with defective binding to factor VIII. Blood 1989; 74: 1591-9.
26. Sadler JE. A revised classification of von Willebrand disease. Thromb Haemost 1994; 71: 520-5.
27. Mazurier C. von Willebrand disease masquerading as haemophilia A. Thromb Haemost 1992; 67: 391-6.
28. Hamer RJ, Koedam JA, Beeser-Visser NH, Bertina RM, van Mourik JA, Sixma JJ. Factor VIII binds to von Willebrand factor via its Mr-80,000 light chain. Eur J Biochem 1987; 166: 37-43.
29. Foster PA, Fulcher CA, Houghten RA, Zimmerman TS. An immunogenic region within residues Val1670-Glu1684 of the factor VIII light chain induces antibodies which inhibit binding of factor VIII to von Willebrand factor. J Biol Chem 1988; 263: 5230-4.
30. Leyte A, Verbeet MP, Brodniewicz-Proba T, van Mourik JA, Mertens K. The interaction between human blood-coagulation factor VIII and von Willebrand factor. Characterization of a high-affinity binding site on factor VIII. Biochem J 1989; 257: 679-83.
31. Saenko EL, Shima M, Rajalakshmi KJ, Scandella D. A role for the C2 domain of factor VIII in binding to von Willebrand factor. J Biol Chem 1994; 269: 11601-5.
32. Saenko EL, Scandella D. A mechanism for inhibition of factor VIII binding to phospholipid by von Willebrand factor. J Biol Chem 1995; 270: 13826-33.
33. Ginsburg D, Handin RI, Bonthron DT, Donlon TA, Bruns GA, Latt SA, Orkin SH. Human von Willebrand factor (vWF): isolation of complementary DNA (cDNA) clones and chromosomal localization. Science 1985; 228: 1401-6.
34. Verweij CL, de Vries CJ, Distel B, van Zonneveld AJ, van Kessel AG, van Mourik JA, Pannekoek H. Construction of cDNA coding for human von Willebrand factor using antibody probes for colony-screening and mapping of the chromosomal gene. Nucleic Acids Res 1985; 13: 4699-17.
35. Mancuso DJ, Tuley EA, Westfield LA, Worrall NK, Shelton Inloes BB, Sorace JM, Alevy YG, Sadler JE. Structure of the gene for human von Willebrand factor. J Biol Chem 1989; 264: 19514-27.
36. Fay PJ, Kawai Y, Wagner DD, Ginsburg D, Bonthron D, Ohlsson-Wilhelm BM, Chavin SI, Abraham GN, Handin RI, Orkin SH. Propolypeptide of von Willebrand factor circulates in blood and is identical to von Willebrand antigen II. Science 1986; 232: 995-8.
37. Marti T, Rosselet SJ, Titani K, Walsh KA. Identification of disulfide-bridged substructures within human von Willebrand factor. Biochemistry 1987; 26: 8099-9.
38. Wise RJ, Pittman DD, Handin RI, Kaufman RJ, Orkin SH. The propeptide of von Willebrand factor independently mediates the assembly of von Willebrand multimers. Cell 1988; 52: 229-36.
39. van de Ven WJM, Voorberg J, Fontijn R, Pannekoek H, van den Ouwenland AMW, van Duinhoven HLP, Roebroek AJM, Siezen RJ. Furin is a subtilisin like proprotein processing enzyme in higher eukaryotes. Mol Biol Rep 1990; 14: 265-75.
40. Wise RJ, Barr PJ, Wong PA, Kiefer MC, Brake AJ, Kaufman RJ. Expression of a human proprotein processing enzyme. Correct cleavage of von Willebrand factor precursor at a paired basic amino acid site. Proc Natl Acad Sci USA 1990; 87: 9378-82.
41. Dent JA, Galbusera M, Rugged ZM. Heterogeneity of plasma von Willebrand factor multimers resulting from proteolysis of the constituent subunit. J Clin Invest 1991; 88: 774-82.
42. Tsai HM, Sussman II, Nagel RL. Shear stress enhances the proteolysis of von Willebrand factor in normal plasma. Blood 1994; 83: 2171-9.
43. Furlan M, Robles R, Lamie B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood 1996; 87: 4223-34.
44. Lankhof H, Damas C, Schiphorst ME, Useldijk MJW, Bracke M, Furlan M, Tsai HM, de Groot PG, Sixma JJ, Vink T. Von Willebrand factor without the A2 domain is resistent to proteolysis. Thromb Haemost 1997; 77: 1008-13.
45. Verweij CL, Diergaarde PJ, Hart M, Pannekoek H. Full-length von Willebrand factor (vWF) cDNA encodes a highly repetitive protein considerably larger than the mature vWF subunit. EMBO J 1986; 5: 1839-47.
46. Bonthron DT, Handin RI, Kaufman RJ, Wasley LC, Orr EC, Mitsock LM, Ewenstein B, Loscalzo J, Ginsburg D, Orkin SH. Structure of pre-pro-von Willebrand factor and its expression in heterologous cells. Nature 1986; 324: 270-3.
47. Beumer S, IJseldijk MJW, de Groot PG, Sixma JJ. Platelet adhesion to fibronectin in flow: dependence on surface concentration and shear rate, role of platelet membrane glycoproteins GP IIb/IIIa and VLA-5, and inhibition by heparin. Blood 1994; 84: 3724-33.
48. ++ ions, shear rate, and platelet membrane glycoproteins. Blood 1992; 79: 928-35.
49. Bolhuis PA, Sakariassen KS, Sander HJ, Bouma BN, Sixma JJ. Binding of factor VIII-von Willebrand factor to human arterial subendothelium precedes increased platelet adhesion and enhances platelet spreading. J Lab Clin Med 1981; 97: 568-76.
50. Turitto VT, Weiss HJ, Baumgartner HR. Decreased platelet adhesion on vessel segments in von Willebrand's disease: a defect in initial platelet attachment. J Lab Clin Med 1983; 102: 551-64.
51. Stel HV, Sakariassen KS, de-Groot PG, van Mourik JA, Sixma JJ. Von Willebrand factor in the vessel wall mediates platelet adherence. Blood 1985; 65: 85-90.
52. Sakariassen KS, Banga JD, de Groot PG, Sixma JJ. Comparison of platelet interaction with subendothelium of human renal and umbilical arteries and the extracellular matrix produced by human venous endothelial cells. Thromb Haemost 1984; 52: 60-5.
53. Endenburg SJ, Hantgan RR, Lindeboom-Blokzijl L, Lankhof H, Jerome WG, Lewis JC, Sixma JJ, de Groot PG. On the role of vWF in promoting platelet adhesion to fibrin in flowing blood. Blood 1995; 86: 4258-65.
54. Sadler JE. von Willebrand factor. J Biol Chem 1991; 266: 22777-80.
55. Sixma JJ, de Groot PG. von Willebrand factor and the blood vessel wall. Mayo Clin Proc 1991; 66: 628-33.
56. Andersson LO, Brown JE. Interaction of factor VIII-von Willebrand Factor with phospholipid vesicles. Biochem J 1981; 200: 161-7.
57. Marchioro TL, Hougie C, Ragde H, Epstein RB, Thomas ED. Hemophilia: role of organ homografts. Science 1969; 163: 188-90.
58. Lewis JH, Bontempo FA, Spero JA, Ragni MV, Starzl TE. Liver transplantation in a hemophiliac. N Engl J Med 1985; 312: 1189-90.
59. Bontempo FA, Lewis JH, Gorenc TJ, Spero JA, Ragni MV, Scott JP, Starzl TE. Liver transplantation in hemophilia A. Blood 1987; 69: 1721-4.
60. Fass DN, Knutson GJ, Katzmann JA. Monoclonal antibodies to porcine factor VIII coagulant and their use in the isolation of active coagulant protein. Blood 1982; 59: 594-600.
61. Fulcher CA, Zimmerman TS. Characterization of the human factor VIII procoagulant protein with a heterologous precipitating antibody. Proc Natl Acad Sci USA 1982; 79: 1648-52.
62. Rotblat F, O'Brien DP, O'Brien FJ, Goodall AH, Tuddenham EG. Purification of human factor VIII:C and its characterization by Western blotting using monoclonal antibodies. Biochemistry 1985; 24: 4294-300.
63. Purrello M, Alhadeff B, Esposito D, Szabo P, Rocchi M, Truett M, Masiarz F, Siniscalco M. The human genes for hemophilia A and hemophilia B flank the X chromosome fragile site at Xq27.3. EMBO J 1985; 4: 725-9.
64. Gitschier J, Wood WI, Goralka TM, Wion KL, Chen EY, Eaton DH, Vehar GA, Capon DJ, Lawn RM. Characterization of the human factor VIII gene. Nature 1984; 312: 326-30.
65. Vehar GA, Keyt B, Eaton D, Rodriguez H, O'Brien DP, Rotblat F, Oppermann H, Keck R, Wood WI, Harkins RN, Tuddenham EG, Lawn RM, Capon DJ. Structure of human factor VIII. Nature 1984; 312: 337-42.
66. Toole JJ, Knopf JL, Wozney JM, Sultzman LA, Buecker 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: 342-7.
67. Fass DN, Hewick RM, Knutson GJ, Nesheim ME, Mann KG. Internal duplication and sequence homology in factors V and VIII. Proc Natl Acad Sci USA 1985; 82: 1688-91.
68. 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: 6934-7.
69. Poole S, Firtel RA, Lamar E, Rowekamp W. Sequence and expression of the Discoidin I gene family in Dictyostelium discoideum. J Mol Biol 1981; 153: 273-89.
70. Toole JJ, Pittman DD, Orr EC, Murtha P, Wasley LC, Kaufman RJ. A large region (approximately equal to 95 kDa) of human factor VIII is dispensable for in vitro procoagulant activity. Proc Natl Acad Sci USA 1986; 83: 5939-42.
71. Burke RL, Pachl C, Quiroga M, Rosenberg S, Haigwood N, Nordfang O, Ezban M. The functional domains of coagulation factor VIII:C. J Biol Chem 1986; 261: 12574-8.
72. Krishnan S, Kolbe HV, Lepage P, Faure T, Sauerwald R, de la Salle H, Muller C, Bihoreau N, Paolantonacci P, Roitsch C, Meulien P, Pavirani A. Thrombin cleavage analysis of a novel antihaemophilic factor variant, factor VIII delta II. Eur J Biochem 1991; 195: 637-44.
73. Pittman DD, Alderman EM, Tomkinson KN, Wang JH, Giles AR, Kaufman RJ. Biochemical, immunological, and in vivo functional characterization of B-domain-deleted factor VIII. Blood 1993; 81: 2925-35.
74. Mertens K, Donath MJ, van Leen RW, de Keyzer-Nellen MJ, Verbeet MP, Klaasse-Bos JM, Leyte A, van Mourik JA. Biological activity of recombinant factor VIII variants lacking the central B-domain and the heavy-chain sequence Lys713-Arg740: discordant in vitro and in vivo activity. Br J Haematol 1993; 85: 133-42.
75. Eaton DL, Wood WI, Eaton D, Hass PE, Hollingshead P, Wion K, Mather J, Lawn RM, Vehar GA, Gorman C. Construction and characterization of an active factor VIII variant lacking the central one-third of the molecule. Biochemistry 1986; 25: 8343-7.
76. Pittman DD, Wang JH, Kaufman RJ. Identification and functional importance of tyrosine sulfate residues within recombinant factor VIII. Biochemistry 1992; 31: 3315-25.
77. Fulcher CA, Roberts JR, Zimmerman TS. Thrombin proteolysis of purified factor VIII procoagulant protein: correlation of activation with generation of a specific polypeptide. Blood 1983; 61: 807-11.
78. Fulcher CA, de Graaf-Mahoney S, Roberts JR, Kasper CK, Zimmerman TS. Localization of human factor FVIII inhibitor epitopes to two polypeptide fragments. Proc Natl Acad Sci USA 1985; 82: 7728-32.
79. Andersson LO, Forsman N, Huang K, Larsen K, Lundin A, Pavlu B, Sandberg H, Sewerin K, Smart J. Isolation and characterization of human factor VIII: molecular forms in commercial factor VIII concentrate, cryoprecipitate, and plasma. Proc Natl Acad Sci USA 1986; 83: 2979-83.
80. Nordfang O, Ezban M. Generation of active coagulation factor VIII from isolated subunits. J Biol Chem 1988; 263: 1115-8.
81. Bihoreau N, Fontaine-Aupart MP, Lehegarat A, Desmadril M, Yon JM. First determination of the secondary structure of purified factor VIII light chain. Biochem J 1992; 288: 35-40.
82. Mosesson MW, Fass DN, Lollar P, DiOrio JP, Parker CG, Knutson GJ, Hainfeld JF, Wall JS. Structural model of porcine factor VIII and factor VIIIa molecules based on scanning transmission electron microscope (STEM) images and STEM mass analysis. J Clin Invest 1990; 85: 1983-90.
83. Heijnen HF, Koedam JA, Sandberg H, Beeser-Visser NH, Slot JW, Sixma JJ. Characterization of human factor VIII and interaction with von Willebrand factor. An electron microscopic study. Eur J Biochem 1990; 194: 491-8.
84. Fay PJ, Smudzin TM. Intersubunit fluorescence energy transfer in human factor VIII. J Biol Chem 1989; 264: 14005-10.
85. Davie EW, Fujikawa K, Kisiel W. The coagulation cascade: initiation, maintenance, and regulation. Biochemistry 1991; 30: 10363-70.
86. von dem Borne PA, Meijers JC, Bouma BN. Feedback activation of factor XI by thrombin in plasma results in additional formation of thrombin that protects fibrin clots from fibrinolysis. Blood 1995; 86: 3035-42.
87. Repke D, Gemmell CH, Guha A, Turitto VT, Broze GJ, Nemerson Y. Hemophilia as a defect of the tissue factor pathway of blood coagulation: effect of factors VIII and IX on factor X activation in a continuous-flow reactor. Proc Natl Acad Sci USA 1990; 87: 7623-7.
88. 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: 654-60.
89. Muntean W, Rothwangl HJ. Dissociation of the Factor-VIII complex during clotting: role of thrombin and phospholipids. Eur J Clin Invest 1984; 14: 462-8.
90. Hill-Eubanks DC, Parker CG, Lollar P. Differential proteolytic activation of factor VIII-von Willebrand factor complex by thrombin. Proc Natl Acad Sci USA 1989; 86: 6508-12.
91. Atkins JS, Ganz PR. The association of human coagulation factors VIII, IXa and X with phospholipid vesicles involves both electrostatic and hydrophobic interactions. Mol Cell Biochem 1992; 112: 61-71.
92. Kemball-Cook G, Barrowcliffe TW. Interaction of factor VIII with phospholipids: role of composition and negative charge. Thromb Res 1992; 67: 57-71.
93. Brinkman HJ, Mertens K, Holthuis J, Zwart-Huinink LA, Grijm K, van Mourik JA. The activation of human blood coagulation factor X on the surface of endothelial cells: a comparison with various vascular cells, platelets and monocytes. Br J Haematol 1994; 87: 332-42.
94. Nesheim ME, Pittman DD, Wang JH, Slonosky D, Giles AR, Kaufman RJ. The binding of 35S-labeled recombinant factor VIII to activated and unactivated human platelets. J Biol Chem 1988; 263: 16467-70.
95. Gilbert GE, Drinkwater D, Barter S, Clouse SB. Specificity of phosphatidylserine-containing membrane binding sites for factor VIII. Studies with model membranes supported by glass microspheres (lipospheres). J Biol Chem 1992; 267: 15861-8.
96. Bloom JW. The interaction of rDNA factor VIII, factor VIIIdes-797-1562 and factor VIIIdes-797-1562-derived peptides with phospholipid. Thromb Res 1987; 48: 439-48.
97. Kemball-Cook G, Edwards SJ, Sewerin K, Andersson LO, Barrowcliffe TW. Factor VIII procoagulant protein interacts with phospholipid vesicles via its 80 kDa light chain. Thromb Haemost 1988; 60: 442-6.
98. 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 phosphatidylserine. Blood 1990; 75: 1999-2004.
99. Shima M, Scandella D, Yoshioka A, Nakai H, Tanaka I, Kamisue S, Terada S, Fukui H. A factor VIII neutralizing monoclonal antibody and a human inhibitor alloantibody recognizing epitopes in the C2 domain inhibit factor VIII binding to von Willebrand factor and to phosphatidylserine. Thromb Haemost 1993; 69: 240-6.
100. Eaton D, Rodriguez H, Vehar GA. 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; 25: 505-12.
101. Lollar P, Knutson GJ, Fass DN. Activation of porcine factor VIII:C by thrombin and factor Xa. Biochemistry 1985; 24: 8056-64.
102. Hill-Eubanks DC, Lollar P. von Willebrand factor is a cofactor for thrombin-catalyzed cleavage of the factor VIII light chain. J Biol Chem 1990; 265: 17854-8.
103. Neuenschwander P, Jesty J. A comparison of phospholipid and platelets in the activation of human factor VIII by thrombin and factor Xa, and in the activation of factor X. Blood 1988; 72: 1761-70.
104. Pittman DD, Kaufman RJ. Proteolytic requirements for thrombin activation of anti-hemophilic factor (factor VIII). Proc Natl Acad Sci USA 1988; 85: 2429-33.
105. Pittman DD, Millenson M, Marquette K, Bauer K, Kaufman RJ. A2 domain of human recombinant-derived factor VIII is required for procoagulant activity but not for thrombin cleavage. Blood 1992; 79: 389-97.
106. Aly AM, Hoyer LW. Factor VIII-East Hartford (arginine 1689 to cysteine) has procoagulant activity when separated from von Willebrand factor. J Clin Invest 1992; 89: 1382-7.
107. O'Brien DP, Tuddenham EG. Purification and characterization of factor VIII 1,689-Cys: a nonfunctional cofactor occurring in a patient with severe hemophilia A. Blood 1989; 73: 2117-22.
108. Lollar P, Parker CG. Subunit structure of thrombin-activated porcine factor VIII. Biochemistry 1989; 28: 666-74.
109. Fay PJ, Haidaris PJ, Smudzin TM. Human factor VIIIa subunit structure. Reconstruction of factor VIIIa from the isolated A1/A3-C1-C2 dimer and A2 subunit. J Biol Chem 1991; 266: 8957-62.
110. Comp PC, Esmon CT. Generation of fibrinolytic activity by infusion of activated protein C into dogs. J Clin Invest 1981; 68: 1221-8.
111. Kisiel W, Canfield WM, Ericsson LH, Davie EW. Anticoagulant properties of bovine plasma protein C following activation by thrombin. Biochemistry 1977; 16: 5824-31.
112. Marlar RA, Kleiss AJ, Griffin JH. Mechanism of action of human activated protein C, a thrombin-dependent anticoagulant enzyme. Blood 1982; 59: 1067-72.
113. Suzuki K, Stenflo J. Dahlbäck B, Teodorsson B. Inactivation of human coagulation factor V by activated protein C. J Biol Chem 1983; 258: 1914-20.
114. Fay PJ, Walker FJ. Inactivation of human factor VIII by activated protein C: evidence that the factor VIII light chain contains the activated protein C binding site. Biochim Biophys Acta 1989; 994: 142-8.
115. Walker FJ, Scandella D, Fay PJ. Identification of the binding site for activated protein C on the light chain of factors V and VIII. J Biol Chem 1990; 265: 1484-9.
116. Fulcher CA, Gardiner JE, Griffin JH, Zimmerman TS. Proteolytic inactivation of human factor VIII procoagulant protein by activated human protein C and its analogy with factor V. Blood 1984; 63: 486-9.
117. Walker FJ, Chavin SI, Fay PJ. Inactivation of factor VIII by activated protein C and protein S. Arch Biochem Biophys 1987; 252: 322-8.
118. Fay PJ, Smudzin TM, Walker FJ. Activated protein C-catalyzed inactivation of human factor VIII and factor VIIIa. Identification of cleavage sites and correlation of proteolysis with cofactor activity. J Biol Chem 1991; 266: 20139-45.
119. Shen L, Dahlbäck B. Factor V and protein S as synergistic cofactors to activated protein C in degradation of factor VIIIa. J Biol Chem 1994; 269: 18735-8.
120. Heeb MJ, Rosing J, Bakker HM, Fernandez JA, Tans G, Griffin JH. Protein S binds to and inhibits factor Xa. Proc Natl Acad Sci USA 1994; 91: 2728-32.
121. Hackeng TM, van 't Veer C, Meijers JC, Bouma BN. Human protein S inhibits prothrombinase complex activity on endothelial cells and platelets via a direct interaction of protein S with factors Va and Xa. J Biol Chem 1994; 269: 21051-8.
122. Koppelman SJ, Hackeng TM, Sixma JJ, Bouma BN. Inhibition of the intrinsic factor X activating complex by protein S: evidence for a specific binding of protein S to factor VIII. Blood 1995; 86: 1062-71.
123. Heeb MJ, Mesters RM, Tans G, Rosing J, Griffin JH. Binding of protein S to factor Va associated with inhibition of prothrombinase that is independent of activated protein C. J Biol Chem 1993; 268: 2872-7.
124. Hessing M, Kanters D, Hackeng TM, Bouma BN. Identification of different forms of human C4b-binding protein lacking the beta-chain and protein S binding ability. Thromb Haemost 1990; 64: 245-50.
125. Koppelman SJ, van't Veer C, Sixma JJ, Bouma BN. Synergistic inhibition of the intrinsic factor X activation by protein S and C4b-binding protein. Blood 1995; 86: 2653-60.
126. O'Brien DP, Johnson D, Byfield P, Tuddenham EG. Inactivation of factor VIII by factor IXa. Biochemistry 1992; 31: 2805-12.
127. Lamphear BJ, Fay PJ. Proteolytic interactions of factor IXa with human factor VIII and factor VIIIa. Blood 1992; 80: 3120-6.
128. Lollar P, Knutson GJ, Fass DN. Stabilization of thrombin-activated porcine factor VIII:C by factor IXa phospholipid. Blood 1984; 63: 1303-8.
129. Neuenschwander PF, Jesty J. Thrombin-activated and factor Xa-activated human factor VIII: differences in cofactor activity and decay rate. Arch Biochem Biophys 1992; 296: 426-34.
130. Curtis JE, Helgerson SL, Parker ET, Lollar P. Isolation and characterization of thrombin-activated human factor VIII. J Biol Chem 1994; 269: 6246-51.
131. Bertina RM, Cupers R, van Wijngaarden A. Factor IXa protects activated factor VIII against inactivation by activated protein C. Biochem Biophys Res Commun 1984; 125: 177-83.
132. Lollar P, Parker ET, Fay PJ. Coagulant properties of hybrid human/porcine factor VIII molecules. J Biol Chem 1992; 267: 23652-7.
133. 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: 3725-30.
134. Lenting PJ, Donath MJ, 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: 7150-5.
135. Lenting PJ, van de Loo JW, Donath MJ, van Mourik JA, Mertens K. The sequence Glu 1811-Lys 1818 of human blood coagulation factor VIII comprises a binding site for activated factor IX. J Biol Chem 1996: 271: 1935-40.
136. 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: 20522-7.
137. Precup JW, Kline BC, Fass DN. A monoclonal antibody to factor VIII inhibits von Willebrand factor binding and thrombin cleavage. Blood 1991; 77: 1929-36.
138. Pittman DD, Kaufman RJ. Structure-function relationships of factor VIII elucidated through recombinant DNA technology. Thromb Haemost 1989; 61: 161-5.
139. Hamer RJ, Koedam JA, Beeser-Visser NH, Sixma JJ. The effect of thrombin on the complex between factor VIII and von Willebrand factor. Eur J Biochem 1987; 167: 253-9.
140. Fay PJ, Smudzin TM. Topography of the human factor VIII-von Willebrand factor complex. J Biol Chem 1990; 265: 6197-6202.
141. Fay PJ. Reconstitution of human factor VIII from isolated subunits. Arch Biochem Biophys 1988; 262: 525-31.
142. Kaufman RJ, Wasley LC, Dorner AJ. Synthesis, processing, and secretion of recombinant human factor VIII expressed in mammalian cells. J Biol Chem 1988; 263: 6352-62.
143. Jorieux S, Magallon T, Mazurier C. Evidence that NH2-terminal but not COOH-terminal moiety of plasma von Willebrand factor binds to factor VIII. Thromb Res 1987; 48: 205-10.
144. Takahashi Y, Kalafatis M, Girma JP, Sewerin K, Andersson LO, Meyer D. Localization of a factor VIII binding domain on a 34 kilodalton fragment of the N-terminal portion of von Willebrand factor. Blood 1987; 70: 1679-82.
145. Bahou WF, Ginsburg D, Sikkink R, Litwiller R, Fass DN. A monoclonal antibody to von Willebrand factor (vWF) inhibits factor VIII binding. Localization of its antigenic determinant to a nonadecapeptide at the amino terminus of the mature vWF polypeptide. J Clin Invest 1989; 84: 56-61.
146. Jorieux S, Gaucher C, Cherel G, Meyer D, Mazurier C. Fine epitope mapping of monoclonal antibodies to the NH2-terminal part of von Willebrand factor (vWF) by using recombinant and synthetic peptides: interest for the localization of the factor VIII binding domain. Br J Haematol 1994; 87: 113-8.
147. Ganz PR, Atkins JS, Palmer DS, Dudani AK, Hashemi S, Luison F. Definition of the affinity of binding between human von Willebrand factor and coagulation factor VIII. Biochem Biophys Res Commun 1991; 180: 231-7.
148. Vlot AJ, Koppelman SJ, van den Berg MH, Bouma BN, Sixma JJ. The affinity and stoichiometry of binding of human factor VIII to von Willebrand Factor. Blood 1995; 85: 3150-7.
149. Lopez-Fernandez MF, Blanco-Lopez MJ, Castineira MP, Batlle J. Further evidence for recessive inheritance of von Willebrand disease with abnormal binding of von Willebrand factor to factor VIII. Am J Hematol 1992; 40: 20-7.
150. Morris JA, Kaufman RJ. Von Willebrand factor (vWF) multimerization increases the binding affinity and a free mature amino-terminus on vWF is required for factor VIII interaction. Blood 1995; 86: 449 (Abstract).
151. Leyte A, Voorberg J, van Schijndel HB, Duim B, Pannekoek H, van Mourik JA. The pro-polypeptide of von Willebrand factor is required for the formation of a functional factor VIII-binding site on mature von Willebrand factor. Biochem J 1991; 274: 257-61.
152. Wise RJ, Dorner AJ, Krane M, Pittman DD, Kaufman RJ. The role of von Willebrand factor multimers and propeptide cleavage in binding and stabilization of factor VIII. J Biol Chem 1991; 266: 21948-55.
153. Tuddenham EG, Lane RS, Rotblat F, Johnson AJ, Snape TJ, Middleton S, Kernoff PB. Response to infusions of polyelectrolyte fractionated human factor VIII concentrate in human haemophilia A and von Willebrand's disease. Br J Haematol 1982; 52: 259-67.
154. Brinkhous KM, Sandberg H, Garris JB, Mattsson C, Palm M, Griggs T, Read MS. Purified human factor VIII procoagulant protein: comparative hemostatic response after infusions into hemophilic and von Willebrand disease dogs. Proc Natl Acad Sci USA 1985; 82: 8752-6.
155. Morfini M, Mannucci PM, Teneoni PM, Longo G, Mazzucconi MG, Rodeghiero F, Ciavarella N, De Rosa V, Arter A. Pharmacokinetics of monoclonally-purified and recombinant factor VIII in patients with severe von Willebrand disease. Thromb Haemost 1993; 70: 270-2.
156. Sadler JE. A revised classification of von Willebrand disease. Thromb Haemost 1994; 71(4): 520-5.
157. Mazurier C, Dieval J, Jorieux S, Delohel J, Goudemand M. A new von Willebrand factor (vWF) defect in a patient with factor VIII (FVIII) deficiency but with normal levels and multimeric patterns of both plasma and platelet vWF. Characterization of abnormal vWF/FVIII interaction. Blood 1990; 75: 20-6.
158. Mazurier C, Gaucher C, Jorieux S, Parquet-Gernez A, Goudemand M. Evidence for a von Willebrand factor defect in factor VIII binding in three members of a family previously misdiagnosed mild haemophilia A and haemophilia A carriers: consequences for therapy and genetic counselling. Br J Haematol 1990; 76: 372-9.
159. Kroner PA, Friedman KD, Fahs SA, Scott JP, Montgomery RR. Abnormal binding of factor VIII is linked with the substitution of glutamine for arginine 91 in von Willebrand factor in a variant form of von Willebrand disease. J Biol Chem 1991; 266: 19146-9.
160. Cacheris PM, Nichols WC, Ginsburg D. Molecular characterization of a unique von Willebrand disease variant. A novel mutation affecting von Willebrand factor/factor VIII interaction. J Biol Chem 1991; 266: 13499-502.
161. Gaucher C, Mercier B, Jorieux S, Oufkir D, Mazurier C. Identification of two point mutations in the von Willebrand factor gene of three families with the 'Normandy' variant of von Willebrand disease. Br J Haematol 1991; 78: 506-14.
162. Tuley EA, Gaucher C, Jorieux S, Worrall NK, Sadler JE, Mazurier C. Expression of von Willebrand factor "Normandy": an autosomal mutation that mimics hemophilia A. Proc Natl Acad Sci USA 1991; 88: 6377-81.
163. Jorieux S, Tuley EA, Gaucher C, Mazurier C, Sadler JE. The mutation Arg (53)→Trp causes von Willebrand disease Normandy by abolishing binding to factor VIII. Studies with recombinant von Willebrand factor. Blood 1992; 79: 563-7.
164. Peerlinck K, Eikenboom JC, Ploos van Amstel HK, Sangtawesin W, Arnout J, Reitsma PH, Vermylen J, Briet E. A patient with von Willebrand's disease characterized by a compound heterozygosity for a substitution of Arg854 by Gln in the putative factor-VIII-binding domain of von Willebrand factor (vWF) on one allele and very low levels of mRNA from the second vWF allele. Br J Haematol 1992; 80: 358-63.
165. Nesheim M, Pittman DD, Giles AR, Fass DN, Wang JH, Slonosky D, Kaufman RJ. The effect of plasma von Willebrand factor on the binding of human factor VIII to thrombin-activated human platelets. J Biol Chem 1991; 266: 17815-20.
166. Over J, Sixma JJ, Bruine MH, Trieschnigg MC, Vlooswijk RA, Beeser-Visser NH, Bouma BN. Survival of 125iodine-labeled Factor VIII in normals and patients with classic hemophilia. Observations on the heterogeneity of human Factor VIII. J Clin Invest 1978; 62: 223-34.
167. Stoddart JH, Andersen J, Lynch DC. Clearance of normal and type 2A von Willebrand factor in the rat. Blood 1996; 88: 1692-9.
168. Irving MG, Roll FJ, Huang S, Bissell DM. Characterization and culture of sinusoidal endothelium from normal rat liver: lipoprotein uptake and collagen
169. Horn T, Lyon H, Christoffersen P. The blood hepatocytic barrier: a light microscopical, transmission- and scanning electron microscopic study. Liver 1986; 6: 233-45.
170. Wisse E. An electron microscopic study of the fenestrated endothelial lining of rat liver sinusoids. J Ultrastruct Res 1970; 31: 125-50.
171. Fay PJ, Anderson MT, Chavin SI, Marder VJ. The size of human factor VIII heterodimers and the effects produced by thrombin. Biochim Biophys Acta 1986; 871: 268-78.
172. Zucker MB, Soberano ME, Johnson AJ, Fulton AJ, Kowalski S, Adler M. The in vitro association of antihemophilic factor and von Willebrand factor. Thromb Haemost 1983; 49: 37-41.
173. Lollar P, Parker CG. Stoichiometry of the porcine factor VIII-von Willebrand factor association. J Biol Chem 1987; 262: 17572-6.
174. Nesheim M, Pittman DD, Giles AR, Fass DN, Wang JH, Slonosky D, Kaufman RJ. The effect of plasma von Willebrand factor on the binding of human factor VIII to thrombin-activated human platelets. J Biol Chem 1991; 266: 17815-20.
175. Vlot AJ, Koppelman SJ, Meijers JC, Damas C, van den Berg HM, Bouma BN, Sixma JJ, Willems GM. Kinetics of factor VIII-von Willebrand factor association. Blood 1996; 87: 1809-16.
176. Gilbert GE, Furie BC, Furie B. Binding of human factor VIII to phospholipid vesicles. J Biol Chem 1990; 265: 815-22.
177. Koppelman SJ, Koedam JA, van Wijnen M, Stern DM, Nawroth PP, Sixma JJ, Bouma BN. von Willebrand factor as a regulator of intrinsic factor X activation. J Lab Clin Med 1994; 123: 585-93.