Semin Thromb Hemost 2002; 28(2): 133-138
DOI: 10.1055/s-2002-27815
Copyright © 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

The Role of von Willebrand Factor in Platelet Function

Philip G. de Groot
  • University Medical Center, Utrecht, The Netherlands
Further Information

Publication History

Publication Date:
03 May 2002 (online)

ABSTRACT

von Willebrand factor (vWF) has essential functions in the very first stage of hemostasis. At the site of vascular damage vWF binds immediately to exposed collagens, thereby facilitating the adhesion of platelets. After a first layer of platelets has been formed, vWF is also crucial as a link between platelets in the forming thrombus. In this article, the current knowledge on the role of vWF in primary hemostasis is reviewed.

REFERENCES

  • 1 Jaffe E A, Hoyer L W, Nachman R L. Synthesis of von Willebrand factor by cultured human endothelial cells.  Proc Natl Acad Sci USA . 1974;  71 1906-1909
  • 2 Nachman R, Levine R, Jaffe E A. Synthesis of factor VIII antigen by cultured guinea pig megakaryocytes.  J Clin Invest . 1977;  60 914-921
  • 3 Sadler J E. Biochemistry and genetics of von Willebrand factor.  Annu Rev Biochem . 1998;  67 395-424
  • 4 Ruggeri Z M. Structure and function of von Willebrand factor.  Thromb Haemost . 1999;  82 576-584
  • 5 Rohdeghiero F, Castaman G, Ruggeri Z M, Tosetto A. The bleeding time in normal subjects is mainly determined by platelet von Willebrand factor and is independent from blood group.  Thromb Res . 1992;  65 605-615
  • 6 Sarode R, Goldstein J, Sussman I I, Nagel R L, Tsai H M. Role of A and B blood group antigens in the expression of adhesive activity of von Willebrand factor.  Br J Haematol . 2000;  109 857-864
  • 7 Ewenstein B M. Von Willebrand's disease.  Annu Rev Med . 1997;  48 525-545
  • 8 Verweij C L, Diergaarde P J, Hart M, Pannekoek H. Full-length von Willebrand factor cDNA encodes a highly repetitive protein considerably larger than the mature vWF subunit.  EMBO J . 1986;  5 1839-1847
  • 9 Siedlecki C A, Lestini B J, Kottke-Marchant K. Shear dependent changes in the three dimensional structure of von Willebrand factor.  Blood . 1996;  88 2939-2950
  • 10 Sakariassen K S, Bolhuis P A, Sixma J J. Human blood platelets adhesion to artery subendothelium is mediated by factor VIII-von Willebrand factor bound to the subendothelium.  Nature . 1979;  279 636-638
  • 11 Celikel R, Ruggeri Z M, Varughese K I. Von Willebrand conformation and adhesive function is modulated by an internalized water molecule.  Nature . 2000;  7 881-884
  • 12 Read M S, Potter J Y, Brinkhous K M. Venom coagglutinin for detection of von Willebrand factor activity in animal plasmas.  J Lab Clin Med . 1983;  101 74-82
  • 13 Ruggeri Z M, Zimmerman T S. von Willebrand factor and von Willebrand disease.  Blood . 1987;  70 895-904
  • 14 Sadler J E, Matsushita T, Dong Z, Tuley E A, Westfield L A. Molecular mechanism and classification of von Willebrand disease.  Thromb Haemost . 1995;  74 161-166
  • 15 Lankhof H, van Hoeij M, Schiphorst M E. A3 domain is essential for interaction of von Willebrand factor with collagen type III.  Thromb Haemost . 1996;  75 950-958
  • 16 Clemetson K J, Clemetson J M. Platelet GPIb-V-IX complex.  Structure, function, physiology and pathology. Semin Thromb Hemost . 1995;  21 130-136
  • 17 Savage B, Saldivar E, Ruggeri Z M. Initiation of platelet adhesion by arrest onto fibrinogen or translocation on von Willebrand factor.  Cell . 1996;  84 289-297
  • 18 Miura S, Li C Q, Cao Z. Interaction of von Willebrand factor domain A1 with platelet glycoprotein Ib alpha-(1-289). Slow intrinsic binding kinetics mediate rapid platelet adhesion.  J Biol Chem . 2000;  275 7539-7546
  • 19 Nieuwenhuis H K, Sakariassen K S, Houdijk W PM, Nievelstein P E M F, Sixma J J. Deficiency of platelet membrane glycoprotein Ia is associated with a decreased platelet adhesion to subendothelium. A defect in platelet spreading.  Blood . 1986;  68 692-695
  • 20 Arai M, Yamamoto N, Moroi M. Platelets with 10% of the normal amount of glycoprotein VI have an impaired response to collagen that results in a mild bleeding tendency.  Br J Haematol . 1995;  89 124-130
  • 21 Kehrel B, Clementson K J, Anders O. Glycoprotein VI is a major collagen receptor for platelet activation: it recognizes the platelet-activating quarternary structure of collagen, whereas CD36, glycoprotein IIb/IIIa and von Willebrand factor do not.  Blood . 1998;  91 491-496
  • 22 Beumer S, IJsseldijk M JW, de Groot G Ph, Sixma J J. Platelet adhesion to fibronectin in flow: dependence on surface concentration and shear rate, role of platelet membrane glycoproteins GPIIb/IIIa and VLA5, and inhibition by heparin.  Blood . 1994;  84 3724-3733
  • 23 Weiss H J, Turitto V T, Baumgartner H R. Platelet adhesion and thrombus formation on subendothelium in platelets deficient in glycoproteins IIb-IIIa, Ib, and storage granules.  Blood . 1986;  67 322-330
  • 24 Weiss H J, Turitto V T, Baumgartner H R. Further evidence that glycoprotein IIb-IIIa mediates platelet spreading on subendothelium.  Thromb Haemost . 1991;  65 202-205
  • 25 Wu Y P, de Groot G Ph, Sixma J J. Shear stress-induced detachment of blood platelets adhered to various adhesive surfaces under flow.  Arterioscler Thromb Vasc Biol . 1997;  17 3202-3207
  • 26 Kulkarni S, Dopheide S M, Yap C L. A revised model of platelet aggregation.  J Clin Invest . 2000;  105 783-791
  • 27 Ruggeri Z M. Old concepts and new developments in the study of platelet aggregation.  J Clin Invest . 2000;  105 699-701
  • 28 Ruggeri Z M, Dent J A, Saldivar E. Contribution of distinct adhesive interactions to platelet aggregation in flowing blood.  Blood . 1999;  94 172-178
  • 29 Wu Y P, Vink T, Schiphorst M. Platelet thrombus formation on collagen at high shear rates is mediated by von Willebrand factor-GPIb interaction and inhibited by vWF-GPIIb:IIIa interaction.  Arterioscler Thromb Vasc Biol . 2000;  20 1661-1667
  • 30 Tsuji S, Sugimoto M, Miyata S. Real time analysis of mural thrombus formation in various platelet aggregation disorders: distinct shear-dependent roles of platelet receptors and adhesive proteins under flow.  Blood . 1999;  94 968-975
  • 31 Weiss H J, Hawiger J, Ruggeri Z M. Fibrinogen-independent platelet adhesion and thrombus formation on subendothelium mediated by glycoprotein IIb-IIIa complex at high shear rate.  J Clin Invest . 1989;  83 288-297
  • 32 Berliner S, Niija K, Roberts J R, Houghten R A, Ruggeri Z M. Generation and characterization of peptide-specific antibodies that inhibit von Willebrand factor binding to glycoprotein IIb-IIIa without interaction with other adhesive molecules.  J Biol Chem . 1988;  263 7500-7505
  • 33 Matsushita T, Meyer D, Sadler J E. Localization of von Willebrand factor-binding sites for platelet glycoprotein Ib and botrocetin by charged-to-alanine scanning mutagenesis.  J Biol Chem . 2000;  275 11044-11049
  • 34 Emsley J, Cruz M, Handin R, Liddington R. Crystal structure of the von Willebrand factor A1 domain and implications for the binding of platelet glycoprotein Ib.  J Biol Chem . 1998;  273 10396-10401
  • 35 Huizinga E G, Martijn van der Plas R, Kroon J, Sixma J J, Gros P. Crystal structure of the A3 domain of human von Willebrand factor: implications for collagen binding.  Structure . 1997;  5 1147-1156
  • 36 Bienkowska J, Cruz M, Atiemo A, Handin R, Liddington R. The von Willebrand factor A3 domain does not contain a metal ion-dependent adhesion site motif.  J Biol Chem . 1997;  272 25162-25167
  • 37 Romein R AP, Bouma B, Wuyster W. Identification of the collagen binding site of the von Willebrand A3-domain.  J Biol Chem . 2001;  276 9985-9991
  • 38 Sadler J E, Mannucci P M, Berntorp E. Impact, diagnosis and treatment of von Willebrand disease.  Thromb Haemost . 2000;  84 160-174
  • 39 Stel H V, Sakariassen K S, de Groot G Ph, van Mourik A J, Sixma J J. The von Willebrand factor in the vessel wall mediates platelet adherence.  Blood . 1985;  65 85-90
  • 40 Fressinaud E, Baruch D, Rothschild C, Baumgartner H R, Meyer D. Platelet von Willebrand factor: evidence for its involvement in platelet adhesion to collagen.  Blood . 1987;  70 1214-1217
  • 41 d'Allesio P, Zwaginga J J, de Boer C H. Platelet adhesion to collagen in subtypes of type I von Willebrand's disease is dependent on platelet von Willebrand factor.  Thromb Haemost . 1990;  64 227-231
  • 42 de Groot G Ph, Federici A B, de Boer H C, et al. Von Willebrand factor synthesized by endothelial cells from a patient with type IIB von Willebrand disease supports platelet adhesion normally, but has an increased affinity for platelets.  Proc Natl Acad Sci USA . 1989;  86 3793-3797
  • 43 Nichols W C, Ginsburg D. von Willebrand disease.  Medicine . 1997;  76 1-20
  • 44 Hillery C A, Mancuso D J, Sadler E J, et al. Type 2M von Willebrand disease: F606I and I662F mutations in the glycoprotein Ib binding domain selectively impair ristocetin- but not botrocetin-mediated binding of von Willebrand factor to platelets.  Blood . 1998;  91 1572-1581
  • 45 Weiss H J, Meyer D, Rabinowitz R, et al. Pseudo-von Willebrand's disease. <~>An intrinsic platelet defect with aggregation by unmodified human factor VIII/Willebrand factor and enhanced adsorption of its high-molecular-weight multimers.  N Engl J Med . 1982;  306 326-333
  • 46 Russell S D, Roth G J. Pseudo-von Willebrand disease: a mutation in the platelet glycoprotein Ib alpha gene associated with a hyperactive surface receptor.  Blood . 1993;  81 1787-1791
  • 47 Veyradier A, Jenkins S P, Fressinaud E, Meyer  D. Acquired von Willebrand syndrome: from pathophysiology to management.  Thromb Haemost . 2000;  84 175-182
  • 48 van Genderen J P, Vink T, Michiels J J. Acquired von Willebrand disease caused by an autoantibody selectively inhibiting the binding of von Willebrand factor to collagen.  Blood . 1994;  84 3378-3384