Platelet—von Willebrand Factor Interactions

  • Barry S. Coller

Abstract

Clinical observations indicate that the interaction between von Willebrand factor (VWF)* and platelets plays a central role in platelet physiology. The most convincing evidence is that patients with quantitative and/or qualitative abnormalities of their VWF protein have a hemorrhagic diathesis characterized by abnormal platelet function (Zimmerman and Ruggeri, 1983; Coller, 1984a). Additional support for the importance of this interaction comes from observations of patients whose platelets cannot bind VWF to their surface as a result of abnormalities in the platelet membrane receptor mechanisms; however, conclusions from these patients must be tempered by an appreciation of the complexity of the disorders. Thus, patients with the Bernard-Soulier syndrome, whose platelets do not bind human VWF in the presence of ristocetin (Zucker et al., 1977; Moake et al., 1980) and cannot be aggregated by bovine VWF (Bithell et al., 1972; Howard et al., 1973), have a serious bleeding disorder (George et al., 1984; Coller, 1984b). As discussed in Chapter 16, the platelets from these patients lack normal glycoprotein (GP) lb (Nurden and Caen, 1975), the glycoprotein that several lines of evidence indicate is the receptor for both human VWF (when platelets are incubated with ristocetin) and bovine VWF (Kirby, 1977; Phillips, 1980; Coller et al., 1983). In addition to the GP Ib abnormality, platelets from these patients also appear to lack GP V and a glycoprotein of M r 17-22,000 (Clemetson et al., 1982; Berndt et al., 1983; Nurden et al., 1983) that may be noncovalently associated with GP Ib (Coller et al, 1983; Bemdt et al., 1983; Chong et al., 1983; see Chapter 4). Patients with Glanzmann’s thrombasthenia, whose washed platelets fail to bind VWF when stimulated with either thrombin (Ruggeri et al., 1982a) or ADP (Gralnick et al., 1984), also suffer from a severe bleeding disorder (George et al., 1984; Coller, 1984b).

Keywords

Cholesterol Sulfide Leukemia Agarose Citrate 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Aihara, M., Cooper, H. A., and Wagner, R. H., 1984, Platelet-collagen interactions: Increase in rate of adhesion of fixed washed platelets by factor VIII-related antigen, Blood 63:495–501.PubMedGoogle Scholar
  2. Bamhart, M. I., and Chen, S-T., 1978, Vessel wall models for studying interaction capabilities with blood platelets, Semin. Thromb. Hemost. 5:112–155.Google Scholar
  3. Baumgartner, H. R., 1973, The role of blood flow in platelet adhesion, fibrin deposition and formation of mural thrombi, Microvasc. Res. 5:167–179.PubMedCrossRefGoogle Scholar
  4. Baumgartner, H. R., Turitto, V. T., and Weiss, H. J., 1980, Effect of shear rate on platelet interaction with subendothelium in citrated and native blood. II. Relationships among platelet adhesion, thrombus dimensions, and fibrin formation, J. Lab. Clin. Med. 95:208–221.PubMedGoogle Scholar
  5. Bemdt, M. C., Gregory, C., Castaldi, P. A., and Zola, H., 1983, Purification of human platelet membrane lb complex using a monoclonal antibody,Thromb. Haemostasis 50:361.Google Scholar
  6. Bithell, T. C., Parekh, S. J., and Strong, R. R., 1972, Platelet-function studies in the Bemard-Soulier syndrome, Ann. N.Y. Acad. Sci. 201:145–160.PubMedCrossRefGoogle Scholar
  7. Boda, A., Solum, N. O., Sztaricska, R., and Kaiman, R., 1979, Study of platelet agglutination induced by the antibiotics of the vancomycin group: Ristocetin, ristomycin, actinoidin and vancomycin, Thromb. Haemostasis 42:1164–1180.Google Scholar
  8. Bolhuis, P. A., Sakariassen, K. S., Sander, H. J., Bouma, B. N., and Sixma, J. J., 1981, Binding of factor VIII-von Willebrand factor to human arterial subendothelium precedes increased adhesion and enhances platelet spreading, J. Lab. Clin. Med. 97:568–576.PubMedGoogle Scholar
  9. Bottecchia, D., and Veraiylen, J., 1976, Factor VIII and human platelet aggregation. I. Evidence that the platelet aggregating activity of bovine factor VIII is a property of its “carrier protein” subunit, Br. J. Haematol 34:303–320.PubMedCrossRefGoogle Scholar
  10. Bouma, B. N., Wiegemich, Y., and Sixma, J. J., 1972, Immunological characterization of purified antihaemophilic factor (factor VIII) which corrects abnormal platelet retention in von Willebrand’s disease, Nature (New Biol.) 236:104–106.Google Scholar
  11. Bowie, E. J. W., and Fuster, V., 1980, Resistance to atherosclerosis in pigs with von Willebrand’s disease, Acta Med. Scand. (Suppl.) 642:121–130.Google Scholar
  12. Bowie, E. J. W., Fass, D. N. and Katzman, J. A., 1983, Functional studies of Willebrand factor using monoclonal antibodies. Blood 62:146–151.PubMedGoogle Scholar
  13. Brinkhous, K. M., Read, M. S., Fricke, W. A., and Wagner, R. H., 1983, Botrocetin (venom coagglutinin): Reaction with a broad spectrum of multimeric forms of factor VIII macromolecular complex, Proc. Natl. Acad. Sci. U.S.A. 80:1463–1466.PubMedCrossRefGoogle Scholar
  14. Brooks, D E., Millar, J. S., Seaman, G. V. F., and Vassar, P. S., 1967, Some physicochemical factors relevant to cellular interactions, J. Cell Physiol. 69:155–168.PubMedCrossRefGoogle Scholar
  15. Caen, J. P., Castaldi, P. A., Leclerc, J. C., Inceman, S., Larrieu, M-J., Probst, M., and Bernard, J., 1966, Congenital bleeding disorders with long bleeding time and normal platelet count. I. Glanzmann’s thrombasthenia (report of fifteen patients), Am. J. Med. 41:4–26.CrossRefGoogle Scholar
  16. Chong, B. H., Bemdt, M. C., Koutts, J., Castaldi, P. A., 1983, Quinidine-induced thrombocytopenia and leukopenia: Demonstration and characterization of the quinidine-dependent antiplatelet and antileukocyte antibodies,Blood 62:1218–1223.PubMedGoogle Scholar
  17. Clemetson, K. J., McGregor, J. L., James, E., Dechavanne, M., and Luscher, E. F., 1982, Characterization of the platelet membrane glycoprotein abnormalities in Bemard-Soulier syndrome and comparison with normal by surface-labeling techniques and high-resolution two-dimensional gel electrophoresis, J. Clin. Invest. 70:304–311.PubMedCrossRefGoogle Scholar
  18. Coller, B. W., 1978, The effects of ristocetin and von Willebrand factor on platelet electrophoretic mobility, J. Clin. Invest. 61:1168–1175.PubMedCrossRefGoogle Scholar
  19. Coller, B. S., 1980, Polybrene-induced platelet agglutination and reduction in electrophoretic mobility: Enhancement by von Willebrand factor and inhibition by vancomycin. Blood 55:276–281.PubMedGoogle Scholar
  20. Coller, B. S., 1981, Inhibition of von Willebrand factor-dependent platelet function by increased platelet cyclic AMP and its prevention by cytoskeleton disrupting agents. Blood 57:846–855.PubMedGoogle Scholar
  21. Coller, B. S., 1982, Effects of tertiary amine local anesthetics on von Willebrand factor-dependent platelet function: Alteration of membrane reactivity and degradation of GP lb by a calcium-dependent protease(s),Blood 60:731–743.PubMedGoogle Scholar
  22. Coller, B. S., 1983, Biochemical and electrostatic considerations in primary platelet aggregation, Ann. N.Y. Acad. Sci. 416:693–708.PubMedCrossRefGoogle Scholar
  23. Coller, B. S., 1984a, Von Willebrand’s disease, in: Disorders of Hemostasis (O. D. Ratnoff and C. D. Forbes, eds.), Grune and Stratton, New York, pp. 241–269.Google Scholar
  24. Coller, B. S., 1984b, Platelet disorders, in: Disorders of Hemostasis (O. D. Ratnoff and C. D. Forbes, eds.), Grune and Stratton, New York, pp. 73–176.Google Scholar
  25. Coller, B. S., and Gralnick, H. R., 1977, Studies on the mechanism of ristocetin-induced platelet agglutination: Effects of structural modification of ristocetin and vancomycin, J. Clin. Invest. 60:302–312.PubMedCrossRefGoogle Scholar
  26. Coller, B. S., Hirschman, R. J., and Gralnick, H. R., 1975, Studies on the factor VIII/von Willebrand factor antigen on the platelet surface, Thromb. Res. 6:469–480.PubMedCrossRefGoogle Scholar
  27. Coller, B. S., Franza, B. R., Jr., and Gralnick, H. R., 1976, The pH dependence of quantitative ristocetininduced platelet aggregation: Theoretical and practical implications. A new device for maintenance of platelet-rich plasma pH, Blood 47:841–854.PubMedGoogle Scholar
  28. Coller, B. S., Frank, R. N., Milton, R. C., and Gralnick, H. R., 1978, Plasma cofactors of platelet function: Correlation with diabetic retinopathy and hemoglobial Aiac. Studies in diabetic patients and normal persons, Ann. Intern. Med. 88:311–316.PubMedGoogle Scholar
  29. Coller, B. S., Peerschke, E. I., Scudder, L. E., and Sullivan, C. A., 1983, Studies with a murine nomoclonal antibody that abolishes ristocetin-induced binding of von Willebrand factor to platelets: Additional evidence in support of GP Ib as a platelet receptor for von Willebrand factor, Blood 61:99–110PubMedGoogle Scholar
  30. Counts, R. B., Paskell, S. L., and Elgee, S. K., 1978, Disulfide bonds and the quaternary structure of factor VIII/von Willebrand factor, J. Clin. Invest. 62:702–709.PubMedCrossRefGoogle Scholar
  31. De Marco, L., and Shapiro, S. S., 1981, Properties of human asialo-factor VIII. A ristocetin-independent platelet-aggregating agent, J. Clin. Invest. 68:321–328.PubMedCrossRefGoogle Scholar
  32. Degos, L., Tobelem, G., Lethielleaux, P., Levy-Toledano, S., Caen, J., and Colombani, J., 1977, Molecular defect in platelets from patients with Bemard-Soulier syndrome, Blood 50:899–903.PubMedGoogle Scholar
  33. Doolittle, R. F., Humkapiller, M. W., Hood, L. E., Devare, S. G., Robbins, K. C., Aaronson, S. A., and Antoniades, H. N., 1983, Simian sarcoma virus one gene, v-sis, is derived from the gene (or genes) encoding a platelet-derived growth factor, Science 221:275–211.PubMedCrossRefGoogle Scholar
  34. Doucet-de Bruine, M. H. M., Sixma, J. J., Over, J., and Beeser-Visser, N. H., 1978, Heterogeneity of human factor VIII. II. Characterization of forms of factor VIII binding to platelets in the presence of ristocetin, J. Lab. Clin. Med. 92:96–107.Google Scholar
  35. Enayat, S. M., and Hill, F. G. H., 1983, Subclassification of Type IA von Willebrand’s disease patients using multimeric analysis, Thromb. Haemostasis 50:32.Google Scholar
  36. Evensen, S. A., Solum, N. O., Grottum, K. A., and Hovig, T., 1974, Familial bleeding disorder with moderate thrombocytopenia and giant blood platelet, Scand. J. Haematol. 13:203–214.PubMedCrossRefGoogle Scholar
  37. Fass, D. N., Knutson, G. E., and Bowie, E. J. W., 1978, Porcine Willebrand factor: A population of multimers, J. Lab. Clin. Med. 91:307–320.PubMedGoogle Scholar
  38. Fauvel, F., Grant, M. E., Legrand, Y. J., Souchon, H., Tobelem, G., Jackson, D. S., and Caen, J. P., 1983, Interaction of blood platelets with a microfibriller extract from adult bovine aorta: Requirement for von Willebrand factor, Proc. Natl. Acad. Sci. U.S.A. 80:551–554.PubMedCrossRefGoogle Scholar
  39. Federici, A. B., Elder, J. H., Ruggeri, Z. M., and Zimmerman, T. S., 1983, Role of carbohydrate in von Willebrand factor structure and function: Studies with a new specific endoglycosidase, Thromb. Haemostasis 50:318.Google Scholar
  40. Fernanda Lopez Fernandez, M., Ginsberg, M. H., Ruggeri, Z. M., Battlle, F. J., and Zimmerman, T. S., 1982, Multimeric structure of platelet factor VIII/von Willebrand factor: The presence of larger multimers and their reassociation with thrombin-stimulated platelets, Blood 60:1132–1138.PubMedGoogle Scholar
  41. Forbes, C. D., Ban, R. D., McNicol, G. P., and Douglas, A. S., 1972, Aggregation of human platelets by commercial preparations of bovine and porcine antihaemophilic globulin, J. Clin. Pathol. 25:210–217.PubMedCrossRefGoogle Scholar
  42. Fujimoto, T., and Hawiger, J., 1982, Adenosine diphosphate induces binding of von Willebrand Factor to human platelets, Nature (London) 297:154–156.CrossRefGoogle Scholar
  43. Fujimoto, T., Ohara, S., and Hawiger, J., 1982, Thrombin-induced exposure and prostacyclin inhibition of the receptor for factor VIII/von Willebrand Factor on human platelets, J. Clin. Invest. 69:1212–1222.PubMedCrossRefGoogle Scholar
  44. Furlan, M., Perret, B. S., and Beck, E. A., 1979, Besteht ein Zusammenhang zwischen Zuckergehalt, plattchen aggregierender akrivitat und molekulgrosse von factor VIII? Schweiz. Med. Wschr. 109:1369–1370.PubMedGoogle Scholar
  45. Fuster, V., Bowie, E. J. W., Lewis, J. C., Fass, D. N., Owen, C. A., Jr., and Brown, A. L., Jr., 1978, Resistance to artherosclerosis in pigs with von Willebrand’s disease: Spontaneous and high cholesterol diet-induced artherosclerosis, J. Clin. Invest. 61:722–730.PubMedCrossRefGoogle Scholar
  46. George, J. N., 1972, Direct assessment of platelet adhesion to glass: A study of the forces of interaction and the effects of plasma and serum factors, platelet function, and modification of the glass surface, Blood 40:862–874.PubMedGoogle Scholar
  47. George, J. N., and Onofre, A. R., 1982, Human platelet surface binding of endogenous secreted factor VIII-von Willebrand factor and platelet factor 4, Blood 59:194–197.PubMedGoogle Scholar
  48. George, J. N., Nurden, A. T., and Phillips, D. R., 1984, Molecular defects that cause abnormalities of platelet-vessel wall interactions, N. Engl. J. Med. 311:1084–1098.PubMedCrossRefGoogle Scholar
  49. Ginsberg, M. H., Wolff, R., Marguerie, G., Coller, B., McEver, R., and Plow, E. F., 1984, Thrombospondin binding to thrombin-stimulated platelets: Evidence for a conmion adhesive protein binding mechanism,Clin Res. 32:308A.Google Scholar
  50. Goudemand, J., Samor, B., Mazurier, C., and Goudemand, M., 1983, The role of the carbohydrate moiety of factor VIII/VWF on binding to human platelets, Thromb. Haemostasis 50:191.Google Scholar
  51. Gralnick, H. R., 1978, Factor VIII/von Willebrand factor protein. Galactose, a cryptic determinant of von Willebrand factor activity, J. Clin. Invest. 62:496–499.PubMedCrossRefGoogle Scholar
  52. Gralnick, H. R., Coller, B. S., and Sultan, Y., 1976, Carbohydrate deficiency of the factor VIII/von Willebrand factor protein in von Willebrand’s disease variants, Science 192:56–59.PubMedCrossRefGoogle Scholar
  53. Gralnick, H. R., Sultan, Y., and Coller, B. S., 1977, Von Willebrand’s disease: Combined qualitative and quantitative abnormalities, N. Engl. J. Med. 296:1024–1030.PubMedCrossRefGoogle Scholar
  54. Gralnick, H. R., Williams, S. B., and Morisato, D. K., 1981, Effect of the multimeric structure of the factor VIII/von Willebrand factor protein on binding to platelets. Blood 58:387–397.PubMedGoogle Scholar
  55. Gralnick, H. R., Williams, S. B., Shafer, B. C., and Corash, L., 1982, Factor VIII/von Willebrand factor binding to von Willebrand’s disease platelets. Blood 60:328–332.PubMedGoogle Scholar
  56. Gralnick, H. R., Williams, S., and Rick, M., 1983, The role of carbohydrate in the maintenance of the multimeric structure of the factor VIII/VWF protein, Thromb. Haemostasis 50:318.Google Scholar
  57. Gralnick, H. R., Williams, S. B., and Coller, B. S., 1984, Fibrinogen competes with von Willebrand factor for binding to the glycoprotein IIb/IIIa complex when platelets are stimulated with thrombin. Blood 64:797–800.PubMedGoogle Scholar
  58. Gralnick, H. R., Williams, S. B., and Coller, B. S., 1985, Asialo von Willebrand Factor interactions with platelets: Interdependence of glycoproteins Ib and Ib/IIIa for binding and aggregation, J. Clin. Invest. (in press).Google Scholar
  59. Grant, R. A., Zucker, M. B., and McPherson, J., 1976, ADP-induced inhibition of von Willebrand factor-mediated platelet agglutination. Am. J. Physiol. 230:1406–1410.PubMedGoogle Scholar
  60. Griggs, T. R., Cooper, H. A., Webster, W. P., Wagner, R. H., and Brinkhous, K. M., 1973, Plasma aggregating factor (bovine) for human platelets: A marker for study of antihemophilic and von Willebrand factors, Proc. Natl. Acad. Sci. U.S.A. 70:2814–2818.PubMedCrossRefGoogle Scholar
  61. Griggs, T. R., Reddick, R. L., Saltzer, D., and Brinkhous, K. M., 1981, Susceptibility to atherosclerosis in aortas and coronary arteries of swine with von Willebrand’s disease, Am. J. Pathol. 102:137–145.PubMedGoogle Scholar
  62. Guisasola, J. A., Cockbum, C. G., and Hardisty, R. M., 1978, Plasmin digestion of factor VIII: Characterization of the breakdown products with respect to antigenicity and von Willebrand activity, Thromb. Haemostasis 40:302–315.Google Scholar
  63. Holmberg, L., and Nilsson, I. M., 1979, AHF related protein in clinical praxis, Scand. J. Haematol. 12:221–231CrossRefGoogle Scholar
  64. Holmberg, L., Nilsson, I. M., Borge, L., Gunnarsson, M., and Sjörin, E., 1983, Platelet aggregation induced by DDAVP in type IIB von Willebrand’s disease, N. Engl. J. Med. 309:816–821.PubMedCrossRefGoogle Scholar
  65. Holmberg, L., Nilsson, I. M., and Lamme, S., 1984, Letter to the editor, N. Engl. J. Med. 310:723.CrossRefGoogle Scholar
  66. Howard, M. A., 1975, Inhibition and reversal of ristocetin-induced platelet aggregation, Thromb. Res. 6:489–499.PubMedCrossRefGoogle Scholar
  67. Howard, M. A., and Firkin, B. G., 1971, Ristocetin—A new tool in the investigation of platelet aggregation, Thromb. Diath. Haemorrh. 26:362–369.PubMedGoogle Scholar
  68. Howard, M. A., Hutton, R. A., and Hardisty, R. M., 1973, Hereditary giant platelet syndrome: A disorder of a new aspect of platelet function,Br. Med. J. 4:586–588.CrossRefGoogle Scholar
  69. Howard, M. A., Montgomery, D. C., and Hardisty, R. M., 1974, Factor-VIII-related antigen in platelets, Thromb. Res. 4:617–624.PubMedCrossRefGoogle Scholar
  70. Howard, M. A., Hendrix, L., and Firkin, B. G., 1979, Further studies on the factor VIII of a patient with a variant form of von Willebrand’s disease, Thromb. Res. 14:609–619.PubMedCrossRefGoogle Scholar
  71. Howard, M. A., Salem, H. H., Thomas, K. B., Hau, L., Perkin, J., Coghlan, M., and Firkin, B., 1982, Variant von Willebrand’s disease type B-revisited, Blood 60:1420–1428.PubMedGoogle Scholar
  72. Howard, M. A., Perkin, J., Salem, H. H., and Firkin, B. S., 1984, The agglutination of human platelets by botrocetin: Evidence that botrocetin and ristocetin act at different sites on the factor VIII molecule and platelet membrane, Br. J. Haematol. 57:25–35.PubMedGoogle Scholar
  73. Hoyer, L. W., and Shainoff, J. R., 1980, Factor VIII related protein circulates in normal human plasma as high molecular weight multimers. Blood 55: 1056–1059.PubMedGoogle Scholar
  74. Jaffe, E. A., Hoyer, L. W., and Nachman, R. L., 1974, Synthesis of von Willebrand factor by cultured human endothelial cells, Proc. Natl. Acad. Sci. U.S.A. 71:1906–1909.PubMedCrossRefGoogle Scholar
  75. Kao, K. J., Pizzo, S.V., and McKee, P. A., 1979, Demonstration and characterization of specific binding sites for factor VIII/von Willebrand Factor on human platelets, J. Clin. Invest. 63:656–664.PubMedCrossRefGoogle Scholar
  76. Konoshita, S., Harrison, J., Lazerson, J., and Abildgaard, C., 1983, A new variant of dominant Type II von Willebrand disease with aberrant multimeric structure of factor VIII-related antigen, Thromb. Haemostasis 50:78.Google Scholar
  77. Kirby, E. P., 1977, Factor VIII-associated platelet aggregation, Thromb. Haemostasis 38:1054–1072.Google Scholar
  78. Kirby, E. P., 1981, Characterization of a form in bovine factor VIII which does not aggregate human platelets, Thromb. Haemostasis 46:479–484.Google Scholar
  79. Kirby, E. P., 1982, The agglutination of human platelets by bovine factor VIII,R. J. Lab. Clin. Med. 100:963–976.Google Scholar
  80. Kirby, E. P., and Mills, D. B., 1975, The interaction of bovine factor VIII with human platelets, J. Clin. Invest. 56:491–502.PubMedCrossRefGoogle Scholar
  81. Kirby, E. P., Mills, D. C. B., Holmsen, H., and Russo, M., 1982, Factor VIII-induced superaggregation of human platelets. Blood 60:1359–1369.PubMedGoogle Scholar
  82. Koutts, J., Walsh, P. N., Plow, E. F., Fenton, J. W., Bouma, B. N., and Zimmerman, T. S., 1978, Active release of human platelet factor VIII related antigen by adenosine diphosphate, collagen and thrombin, J. Clin. Invest. 62:1255–1263.PubMedCrossRefGoogle Scholar
  83. Legaz, M. E., Schmer, G., Counts, R. B., and Davie, E. W., 1973, Isolation and characterization of human factor VIII (antihemophilic factor), J. Biol. Chem. 248:3946–3955.PubMedGoogle Scholar
  84. Legrand, Y. J., Rodriguez-Zeballos, A., Kartalis, G., Fauvel, F., and Caen, J. P., 1978, Adsorption of factor VIII antigen-activity complex by collagen, Thromb. Res. 13:909–911.PubMedCrossRefGoogle Scholar
  85. Legrand, Y. J., Fauvel, F., Gutman, N., Muh, J. P., Tobelem, G., Souchon, H., Kamiguian, A., and Caen, J. P., 1980, Microfibrils platelet interaction: Requirement for von Willebrand factor, Thromb. Res. 19:737–739.PubMedCrossRefGoogle Scholar
  86. Loscalzo, J., Slayter, H., and Handin, I., 1983, The native conformation of human von Willebrand protein: Electron microscopy and quasielastic light scattering analyses. Blood 62(Suppl. l):289a.Google Scholar
  87. Lynch, D. C., Williams, R., Zimmerman, T. S., Kirby, E. P., and Livingston, D. M., 1983, Biosynthesis of the subunits of factor VIIIR by bovine aortic endothelial cells,Proc. Natl. Acad. Sci. U.S.A., 80:2738–2742.PubMedCrossRefGoogle Scholar
  88. Macfarlane, R. G., Biggs, R., and Bidwell, E., 1954, Bovine anti-haemophilic globulin in the treatment of hemophilia. Lancet 1:1316–1319.CrossRefGoogle Scholar
  89. Mannucci, P. M., Lombardi, R., Pareti, F. I., Solinas, S., Mazzaconi, G., and Mariani, G., 1983, A variant of von Willebrand’s disease characterized by recessive inheritance and missing triplet structure of von Willebrand factor multimers, Thromb. Haemostasis 50:78.Google Scholar
  90. Marchesi, S. L., Shulman, N. R., and Gralnick, H. R., 1972, Studies on the purification and characterization of human factor VIII, J. Clin. Invest. 51:2151–2161.PubMedCrossRefGoogle Scholar
  91. Martin, S. W., Marder, V. J., Francis, C. W., Loftus, L. S., and Barlow, G. H., 1980, Enzymatic degradation of the factor VIII-von Willebrand protein: A unique tryptic fragment with ristocetin cofactor activity. Blood 55:847–858.Google Scholar
  92. McMichael, A. J., Rust, N. A., Pilch, J. R., Sochynsky, R., Morton, J., Mason, D. Y., Ruan, C., Tobelem, G., and Caen, J., 1981, Monoclonal antibody to human platelet glycoprotein I. I. Immunoglobulin studies,Br. J. Haematol. 49:501–509.PubMedCrossRefGoogle Scholar
  93. McPherson, J., and Zucker, M. B., 1976, Platelet retention in glass bead columns: Adhesion to glass and subsequent platelet-platelet interactions, Blood 47:55–67.PubMedGoogle Scholar
  94. Meyer, D., Obert, B., Peitu, G., Lavergne, J. M., and Zimmerman, T. S., 1980, Multimeric structure of factor VIII/von Willebrand factor in von Willebrand’s disease, J. Lab. Clin. Med. 95:590–602.PubMedGoogle Scholar
  95. Meyer, D., Lavergne, J. M., Baumgartner, H. R., Tobelem, G., Pietu, G., and Edgington, T. S., 1983, Monoclonal antibodies to human von Willebrand factor: Role of intramolecular loci in mediation of platelet adhesion to the subendothelium, Thromb. Haemostasis 50:191.Google Scholar
  96. Miller, J. L., and Castella, A., 1982, Platelet-type von Willebrand’s disease: Characterization of a new bleeding disorder. Blood 60:790–794.PubMedGoogle Scholar
  97. Miller, J. L., Kupinski, J. M., Castella, A., and Ruggeri, Z. M., 1983, von Willebrand factor binds to platelets and induces aggregation in platelet-type but not type IIB von Willebrand’s disease, J. Clin. Invest. 72:1532–1542.Google Scholar
  98. Miller, J. L., Boselli, B. D., and Kupinski, J. M., 1984, In vivo interaction of von Willebrand factor with platelets following cryoprecipitate transfusion of platelet-type von Willebrand’s disease. Blood 63:226–230.PubMedGoogle Scholar
  99. Moake, J. L., Cimo, P. L., Peterson, D. M., Roper, P., and Natelson, E. A., 1977, Inhibition of ristocetin-induced platelet agglutination by vancomycin,Blood 50:397–401.PubMedGoogle Scholar
  100. Moake, J. L., Olson, J. D., Troll, J. H., Tang, S. S., Funicella, T., and Peterson, D. M., 1980, Binding of radioiodinated human von Willebrand factor to Bemard-Soulier, thrombasthenic and von Willebrand’s disease platelets,Thromb. Res. 19:21–27.PubMedCrossRefGoogle Scholar
  101. Moake, J. L., Tang, S. S., Olson, J. D., Troll, J. H., Cimo, P. L., and Davies, P. J. A., 1981, PGI2 and AET inhibit F.VIII/VWF-mediated platelet cohesion, Am. J. Physiol. 241:454–459.Google Scholar
  102. Morisato, D. K., and Gralnick, H. R., 1980, Selective binding of the factor VIII/von Willebrand factor protein to human platelets, Blood 55:9–15.PubMedGoogle Scholar
  103. Muraki, H., 1977, Changes in electrokinetic properties of human platelets during their aggregation by ristocetin, J. Keio. Med. Soc. 54:87–96.Google Scholar
  104. Nachman, R. I., and Jaffe, E. A., 1975, Subcellular platelet factor VIII antigen and von Willebrand factor, J. Exp. Med. 141:1101–1113.PubMedCrossRefGoogle Scholar
  105. Nachman, R. L., and Leung, L. L. K., 1982, Complex formation of platelet membrane glycoproteins lib and Ilia with fibrinogen, J. Clin. Invest. 69:263–269.PubMedCrossRefGoogle Scholar
  106. Nachman, R. L., Jaffe, E. A., and Weksler, B. B.,1977a, Immunoinhibition of ristocetin-induced platelet aggregation, J. Clin. Invest. 59:143–148.PubMedCrossRefGoogle Scholar
  107. Nachman, R., Levine, R., and Jaffe, E. A., 1977b, Synthesis of factor VIII antigen by cultured guinea pig megakaryocytes, J. Clin. Invest. 60:914–921.CrossRefGoogle Scholar
  108. Nachman, R. L., Jaffe, E. A., Ferris, B., 1980, Peptide map analysis of normal plasma and platelet factor VIII antigen, Biochem. Biophys. Res. Commun. 92:1208–1214.PubMedCrossRefGoogle Scholar
  109. Nurden, A. T., and Caen, J. P., 1975, Specific roles for platelet surface glycoproteins in platelet function. Nature (London) 255:720–722.CrossRefGoogle Scholar
  110. Nurden, A. T., Didry, D., and Rosa, J. P., 1983, Molecular defects of platelets in Bemard-Soulier syndrome, Blood Cells 9:333–353.PubMedGoogle Scholar
  111. Nyman, D., 1977, Interaction of collagen with the factor VIII antigen-activity-von Willebrand factor complex, Thromb. Res. 11:433–438.PubMedCrossRefGoogle Scholar
  112. O’Brien, J., and Heywood, J. B., 1967, Some interactions between human platelets and glass: Von Willebrand’s disease compared with normal, J. Clin. Pathol. 20:56–64.PubMedCrossRefGoogle Scholar
  113. Ohmori, K., Fretto, L. J., Harrison, R. L., Switzer, M. E. P., Erickson, H. P., and McKee, P. A., 1982, Electron microscopy of human factor VIII/von Willebrand glycoprotein: Effect of reducing reagents on structure and function, J. Cell. Biol. 95:632–640.PubMedCrossRefGoogle Scholar
  114. Olson, J. D., Moake, J. L., Collins, M. F., and Michael, B. S., 1983, Adhesion of human platelets to purified solid-phase von Willebrand factor: Studies of normal and Bemard-Soulier platelets,Thromb. Res. 32:115–122.PubMedCrossRefGoogle Scholar
  115. Over, J., Bouma, B. N., and van Mourik, J. A., 1978a, Heterogeneity of human factor VIII. I. Characterization of factor VIII present in the supernatant of cryoprecipitate, J. Lab. Clin. Med. 91:32–46.Google Scholar
  116. Over, J., Sixma, J. J., Doucet-de Bruine, M. H. M., Trieschnigg, A. M. C., Vlooswijk, R. A. A., Beeser-Visser, N. H., and Bouma, B. N., 1978b, Survival of 125iodine-labelled factor VIII in normals and patients with classic hemophilia. Observations on the heterogeneity of human factor VIII, J. Clin. Invest. 62:223–234.CrossRefGoogle Scholar
  117. Peake, I. R., and Bloom, A. L., 1977, Abnormal factor VIII related antigen in von Willebrand’s disease: Decreased precipitation by concanavalin A,Thromb. Haemostasis 37:361–362.Google Scholar
  118. Perret, B. A., Furian, M., and Beck, E. A., 1979, Studies in factor VIII-related protein. II. Estimation of molecular size differences between factor VIII oligomers, Biochim. Biophys. Acta 578:164–174.PubMedGoogle Scholar
  119. Pethica, B. A., 1961, The physical chemistry of cell adhesion, Exp. Cell Res. 8:123–140.PubMedCrossRefGoogle Scholar
  120. Phillips, D. R., 1980, An evaluation of membrane glycoproteins in platelet adhesion and aggregation. Prog. Hemostas. Thromb. 5:81–109.Google Scholar
  121. Plow, E. F., Srouji, A. H., Meyer, D., Marguerie, G., and Ginsberg, M. H., 1984, Evidence that three adhesive proteins interact with a common recognition site on activated platelets, J. Biol. Chem. 259:5388–5391.PubMedGoogle Scholar
  122. Rand, J. H., Sussman, I.I., Gordon, S. V., Chu, S. V., and Solomon, V., 1980, Localization of factor VIII related antigen in human vascular subendothelium, Blood 55:752–756.PubMedGoogle Scholar
  123. Reddick, R. L., Griggs, T. R., Lamb, M. A., and Brinkhous, K. M., 1982, Platelet adhesion to damaged coronary arteries: Comparison in normal and von Willebrand disease swine, Proc. Natl. Acad. Sci. U.S.A. 79:5076–5079.PubMedCrossRefGoogle Scholar
  124. Rosborough, T. K., 1980, Von Willebrand factor, polycations, and platelet agglutination, Thromb. Res. 17:481–490.PubMedCrossRefGoogle Scholar
  125. Rosborough, T. K., and Swaim, W. R., 1978, Abnormal polybrene-induced platelet agglutination in von Willebrand’s disease, Thromb. Res. 12:937–942.PubMedCrossRefGoogle Scholar
  126. Ross, R., Glomset, J., Kaviya, N., and Marker, L, 1974, A platelet-dependent serum factor that stimulates the proliferation of arterial smooth muscle cells in vitro, Proc. Natl. Acad. Sci. U.S.A. 71:1207–1210.PubMedCrossRefGoogle Scholar
  127. Ruan, C., Tobelem, G., McMichael, A. J., Drouet, L., Legrand, Y., Degos, L., Kieffer, N., Lee, H., and Caen, J, P., 1981, Monoclonal antibody to human platelet glycoprotein I. II. Effects on human platelet function, Br. J. Haematol. 49:511–519.PubMedCrossRefGoogle Scholar
  128. Ruggeri, Z. M., and Zimmerman, T. S., 1980, Variant von Willebrand’s disease. Characterization of two subtypes by analysis of multimeric composition of factor VIII/von Willbrand factor in plasma and platelets, J. Clin. Invest. 65:1318–1325.PubMedCrossRefGoogle Scholar
  129. Ruggeri, A. M., and Zimmerman, T. A., 1981, The complex multimeric composition of factor VIII/von Willebrand factor, Blood 57:1140–1143.PubMedGoogle Scholar
  130. Ruggeri, Z. M., Bader, R., and DeMarco, L., 1982a, Glanzmann thrombasthenia: Deficient binding of von Willebrand factor to thrombin-stimulated platelets, Proc. Natl. Acad. Sci. U.S.A. 79:6038–6041.CrossRefGoogle Scholar
  131. Ruggeri, Z. M., Mannucci, P. M., Lombardi, R., Federici, A. B., and Zimmerman, T. S., 1982b, Multimeric composition of Factor VIII/von Willebrand factor following administration of DD A VP: Implications for pathophysiology and therapy of von Willebrand’s disease subtypes. Blood 59:1272–1278.PubMedGoogle Scholar
  132. Ruggeri, Z. M., Nilsson, L M., Lombardi, R., Holmberg, L., and Zimmerman, T. S., 1982c, Aberrant multimeric structure of von Willebrand factor in a new variant of von Willebrand’s disease (Type lie), J. Clin. Invest. 70:1124–1127.CrossRefGoogle Scholar
  133. Ruggeri, Z. M., Bader, R., Pareti, F. L, Mannucci, L., and Zimmerman, T. S., 1983a, High affinity interaction of platelet von Willebrand factor with distinct platelet membrane sites, Thromb. Res. 50:35.Google Scholar
  134. Ruggeri, Z. M., DeMarco, L., Montgomery, R. R., 1983b, Platelets have more than one binding site for von Willebrand factor, J. Clin. Invest. 72:1–12.CrossRefGoogle Scholar
  135. Sakariassen, K. S., Bolhuis, P. A., and Sixma, J. J., 1979, Human blood platelet adhesion to artery subendothelium is mediated by factor VIII-von Willebrand factor bound to the subendothelium, Nature (London) 279:636–638.CrossRefGoogle Scholar
  136. Sakariassen, K. S., Nieuwenhuis, H. K., A Ottenhof-Rovers, and Sixma, J. J., 1983, Thrombocytopenia with large platelets and increased platelet binding of factor VIII-von Willebrand factor without aggregation with exogenous fVIII-VWF: A new entity? Thromb. Haemostasis 50:79.Google Scholar
  137. Salzman, E. W., 1963, Measurement of platelet adhesiveness. A simple in vitro technique demonstrating an abnormality in von Willebrand’s disease,J. Lab. Clin. Med. 62:724–735.PubMedGoogle Scholar
  138. Santoro, S. A., 1983, Preferential binding of high molecular weight forms of von Willebrand factor to fibrillar collagen. 1. Biochim. Biophys. Acta 756:123–126.PubMedCrossRefGoogle Scholar
  139. Santoro, S. A., and Cowan, J. F., 1982, Adsorption of von Willebrand factor by fibrillar collagen — Implications concerning the adhesion of platelets to collagen, Collagen Relat. Res. 2:31–43.Google Scholar
  140. Schmer, G., Kirby, E. P., Teller, D. C., and Davie, E. W., 1972, The isolation and characterization of bovine factor VIII (antihemophilic factor), J. Biol. Chem. 247:2512–2521.PubMedGoogle Scholar
  141. Schullek, J., Jordan, J., and Montgomery, R. R., 1984, Interaction of von Willebrand factor with platelets in a plasma milieu, J. Clin. Invest. 73:421–428.PubMedCrossRefGoogle Scholar
  142. Seaman, G. V. F., 1976, Electrochemical features of platelet interactions, Thromb. Res. (Suppl. II) 8:235–246.PubMedCrossRefGoogle Scholar
  143. Senogles, S. E., and Nelsestuen, G. L., 1983, von Willebrand factor: A protein which binds at the cell surface interface between platelets,J. Biol. Chem. 258:12327–12333.PubMedGoogle Scholar
  144. Shapiro, G. A., Anderson, J. C., Pizzo, S. V., and McKee, P. A., 1973, The subunit structure of normal and hemophilic factor VIII, J. Clin. Invest. 52:2198–2210.PubMedCrossRefGoogle Scholar
  145. Sharp, A. A., and Bidwell, E., 1957, The toxicity and fate of injected animal antihaemophilic globulin. Lancet 2:359–362.CrossRefGoogle Scholar
  146. Sixma, J. J., Sakariassen, K. S., Beeser-Visser, N. H., Ottenhof-Rovers, M., and Bolhuis, P. A., 1984, Adhesion of platelets to human artery subendothelium. Effect of factor VIII-von Willebrand factor of various multimeric composition, Blood 63:128–139.PubMedGoogle Scholar
  147. Sodetz, J. M., Pizzo, S. V., and McKee, P. A., 1977, Relationship of sialic-acid to function and in vivo survival of human factor VIII-von Willebrand factor protein, J. Biol. Chem. 252:5538–5546.PubMedGoogle Scholar
  148. Sodetz, J. M., Paulson, M. C., Pizzo, S. V., and McKee, P. A., 1978, Carbohydrate on human factor VIII/von Willebrand factor. Impairment of function by removal of specific galactose residues,J. Biol. Chem. 253:7202–7206.PubMedGoogle Scholar
  149. Solum, N. O., 1968, Aggregation of human platelets by bovine platelet fibrinogen, Scand, J. Haematol. 5:474–485.CrossRefGoogle Scholar
  150. Solum, N. O., Olsen, T. M., Gogstad, G. O., Hagen, L, and Brosstad, F., 1983, Demonstration of a new glycoprotein Ib-related component in platelet extracts prepared in the presence of leupeptin, Biochim. Biophys. Acta 729:53–61.PubMedCrossRefGoogle Scholar
  151. Sussman, I. L, and Rand, J. H., 1982, Subendothelial deposition of von Willebrand’s factor requires the presence of endothelial cells, J. Lab. Clin. Med. 100:526–532.PubMedGoogle Scholar
  152. Takahashi, H., 1980, Studies on the pathophysiology and treatment of von Willebrand’s disease. IV. Mechanism of increased ristocetin-induced platelet aggregation in von Willebrand’s disease, Thromb. Res. 19:857–867.PubMedCrossRefGoogle Scholar
  153. Takahashi, H., Nagayama, R., Hattori, A., Shibata, A., 1984, Platelet aggregation induced DD A VP in platelet-type von Willebrand’s disease, N. Engl. J. Med. 310:722.PubMedGoogle Scholar
  154. Tobelem, G., Levy-Toledano, S., Bredoux, R., Michel, H., Nurden, A., Degos, L., and Caen, J., 1976, New approach to determination of specific functions of platelet membrane sites.Nature (London) 263:427–429.CrossRefGoogle Scholar
  155. Tschopp, T. B., Weiss, H. J., and Baumgartner, H. R., 1974, Decreased adhesion of platelets to subendothelium in von Willebrand’s disease, J. Lab. Clin. Med. 83:296–300.PubMedGoogle Scholar
  156. Turitto, V., Weiss, H., Sussman, I., and Zimmerman, T., 1981, Factor VIII in vessel wall influences platelet interaction with subendothelium,Thromb. Haemostasis 46:199.Google Scholar
  157. Vermylen, J., Bottecchia, D., and Szpilman, H., 1976, Factor VIII and human platelet aggregation. III. Further studies on aggregation of human platelets by neuraminidase-treated human factor VIII, Br. J. Haematol. 34:321–330.PubMedCrossRefGoogle Scholar
  158. Wagner, D. D., and Marder, V. J., 1983, Biosynthesis of von Willebrand protein by human endothelial cells. Identification of a large precursor polypeptide chain,J. Biol. Chem. 258:2065–2067.PubMedGoogle Scholar
  159. Wagner, D. D., Olmstead, J. B., and Marder, V. J., 1982, Immunolocalization of von Willebrand protein in Weibel-Palade bodies of human endothelial cells, J. Cell. Biol. 95:355–360.PubMedCrossRefGoogle Scholar
  160. Waterfield, M. D., Scrace, G. T., Whittlen, N., Stroobant, P., Johnsson, A., Wasteson, A., Westermark, B., Heldin, C-H., Huang, J. S., and Deuel, T. F., 1983, Platelet-derived growth factor is structurally related to the putative transforming protein of simian sarcoma vims, Nature (London) 304:35–39.CrossRefGoogle Scholar
  161. Weinstein, M., and Deykin, D., 1979, Comparison of factor VIII-related von Willebrand factor proteins prepared from human cryoprecipitate and FVIII concentrate, Blood 53:1095–1105.PubMedGoogle Scholar
  162. Weiss, H. J., and Rogers, J., 1972, Correction of the platelet abnormality in von Willebrand’s disease by cryoprecipitate. Am. J. Med. 53:734–738.PubMedCrossRefGoogle Scholar
  163. Weiss, H. J., Turitto, V. T., and Baumgartner, H. R., 1978, Effect of shear rate on platelet interaction with subendothelium in citrated and native blood. I. Shear dependent decrease of adhesion in von Willebrand’s disease and the Bemard-Soulier syndrome, J. Lab. Clin. Med. 92:750–764.PubMedGoogle Scholar
  164. Weiss, H. J., Turitto, V. T., and Baumgartner, H. R., 1981, Platelet-fibrin deposition on subendothelium in congential bleeding disorders, Thromb. Haemostasis 46:249.Google Scholar
  165. Weiss, H. J., Meyer, D., Rabinowitz, R., Pietu, G., Girma, J-P., Vicic, W. J., and Rogers, J., 1982, Pseudo-von Willebrand’s disease. An intrinsic platelet defect with aggregation by unmodified human factor VIII/von Willebrand factor and enhanced adsorption of its high-molecular-weight multimers,N. Engl. J. Med. 306:326–333.PubMedCrossRefGoogle Scholar
  166. Weiss, H. J., Pietu, G., Rabinowitz, R., Girma, J-P., Rogers, J., and Meyer, D., 1983, Heterogeneous abnormalities in the multimeric structure, antigenic properties, and plasma-platelet content of factor VIII/von Willebrand factor in subtypes of classic (type I) and variant (type IIA) von Willebrand’s disease, J. Lab. Clin. Med. 101:411–415.PubMedGoogle Scholar
  167. Yoshida, N., Weksler, B., and Nachman, R. L., 1983, Purification of human platelet calcium activated protease: Effect on platelet and endothelial cell function, J. Biol. Chem. 258:7168–7174.PubMedGoogle Scholar
  168. Zimmerman, T. S., and Ruggeri, Z. M., 1983, Von Willebrand’s disease, Clin. Haematol. 12:175–200.PubMedGoogle Scholar
  169. Zimmerman, T. S., Roberts, J., and Edgington, T. S., 1975, Factor-VIII-related antigen: Multiple molecular forms in human plasma, Proc. Natl. Acad. Sci. U.S.A. 72:5121–5125.PubMedCrossRefGoogle Scholar
  170. Zimmerman, T. S., Voss, R., and Edgington, T. S., 1979, Carbohydrate of factor VIII/von Willebrand factor in von Willebrand’s disease, J. Clin. Invest. 64:1298–1302.PubMedCrossRefGoogle Scholar
  171. Zimmerman, T. S., Dent, J. A., Federici, A. B., Ruggeri, Z. M., Nilsson, I. M., Holmberg, L., Abildgaard, C. A., and Nannini, L. H., 1984, High resolution Na Dod SO 4 — agarose electrophoresis identifies new molecular abnormalities in von Willebrand’s disease, Clin. Res. 32:501A.Google Scholar
  172. Zucker, M. B., 1963, In vitro abnormality of the blood in von Willebrand’s disease correctable by normal plasma, Nature (London) 197:601–602.CrossRefGoogle Scholar
  173. Zucker, M. B., Kim, S. J., McPherson, J., and Grant, R. A., 1977, Binding of factor VIII to platelets in the presence of ristocetin, Br. J. Haematol. 35:535–549.PubMedCrossRefGoogle Scholar
  174. Zucker, M. B., Broehman, F. J., and Kaplan, K. L., 1979, Factor VIII related antigen in human blood platelets. Localization and release by thrombin and collagen, J. Lab. Clin. Med. 94:675–682.PubMedGoogle Scholar

Copyright information

© Plenum Press, New York 1985

Authors and Affiliations

  • Barry S. Coller
    • 1
  1. 1.Division of Hematology, Departments of Medicine and PathologyState University of New York at Stony BrookStony BrookUSA

Personalised recommendations