Abstract
Von Willebrand factor (vWF) is a multimeric plasma glycoprotein with Mr of the multimers varying in size from ~1 to 20 x 106. Congenital and acquired vWF deficiency are associated with bleeding tendencies and prolonged bleeding times. (Ruggeri and Zimmerman, 1980 & 1981; Weiss et al, 1983; Levine, 1987; Sixma, 1987). Thus, vWF plays a crucial role in hemostatic plug formation as the adhesion of platelets to the subendothelium subsequent to vascular injury is absolutely dependent on the participation of vWF (Hovig et al, 1974; Jorgenson and Borchgreven, 1964; Hoyer, 1982; Kinoshita et al, 1984). Two platelet receptors for vWF (GpIb-IX and GpIIb-IIIa mediate the interactions between platelets and vWF. GpIb is missing in the Bernard-Soulier syndrome while GpIIb-IIIa is missing in thrombasthenia (George et al 1984). One must stress that prior platelet activation is not required for the interaction between vWF and platelets to occur. Non-stimulated platelets bind vWF coupled to a surface via platelet GpIb (Olson et al, 1989). Thrombin-stimulated or ADP-stimulated platelets bind vWF in solution (Fujimoto & Hawiger, 1982; Fujimoto et al, 1982). Platelet GpIb-vWF interactions are irreversible and increase with increasing shear-rates (Stel et al, 1980; Moake et al, 1986; Olson et al, 1989). In contrast, interactions between GpIIb-IIIa and vWF are readily reversed by vWF and fibrinogen among other competitive proteins.
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Weinstein MJ, Chute LE (1984) Two distinct forms of factor VIII coagulant protein in human plasma. Cleavage by thrombin, and differences in coagulant activity and association with von Willebrand factor. J Clin Invest 73: 307–316
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Weiss HJ, Sussman II, Hoyer LW (1977) Studies on post-transfusion and dissociated factor VIII and in patients with von Willebrand’s disease. J Clin Invest 60: 390–404
Weiss HJ, Pietu G, Rabinowitz R, Girma JP, Rogers J, Meyer D (1983) Heterogenous 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 Willebrands disease. J Lab Clin Med 101: 411–425
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Zelechowska MG, Van Mourik JA, Brodniewicz T (1985) Ultrastructure localization of factor VIII procoagulant antigen in human liver hepatocytes. Nature 317: 29–730
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Ofosu, F.A. (1990). Functions of von Willebrand Factor in Plasma. In: Harenberg, J., Heene, D.L., Stehle, G., Schettler, G. (eds) New Trends in Haemostasis. Veröffentlichungen aus der Geomedizinischen Forschungsstelle der Heidelberger Akademie der Wissenschaften, vol 1990 / 1990/3. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-84318-1_4
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DOI: https://doi.org/10.1007/978-3-642-84318-1_4
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