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Gene Review

VWF  -  von Willebrand factor

Sus scrofa

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Disease relevance of VWF


High impact information on VWF


Chemical compound and disease context of VWF

  • A similar inhibition in thrombus formation due to vWF deficiency was observed in both heparinized and native blood; however, thrombus formation was significantly more reduced (p less than 0.05) in the presence of heparin, presumably due to the lack of stability of the accumulated platelets in the absence of fibrin formation [2].
  • von Willebrand disease (vWD), one of the most common bleeding disorders in humans, is manifested as a quantitative or qualitative defect in von Willebrand factor (vWF), an adhesive glycoprotein (GP) with critical hemostatic functions [8].
  • Intimal hyperplasia (IH) in carotid arteries was prominent in C57BL/6J mice, absent in RIIIS/J mice, and moderate in RIIIS/J treated with DDAVP (intima-media ratio, 71% +/- 18%, 0, and 32% +/- 12%, respectively; P <.01). vWF deposition occurred in all hyperplastic intima subjacent to intact endothelium [9].
  • These findings suggest that exposure to low levels of carbon monoxide may increase the incidence of myocardial infarction and that the absence of von Willebrand factor may be protective [10].
  • 1. The antithrombotic effect of aurintricarboxylic acid (ATA) which inhibits binding of von Willebrand factor (vWF) to platelet glycoprotein lb (GPlb) receptor was evaluated in photochemically-induced thrombosis models in the femoral artery of rats and guinea-pigs [11].

Biological context of VWF


Anatomical context of VWF


Associations of VWF with chemical compounds

  • Significant differences are found in two discontinuous segments thought to be involved in the binding of vWF to platelet glycoprotein Ib [13].
  • Porcine vWF lacks four contiguous residues in the first segment and has two positively charged arginine residues in the second [13].
  • A well-demarcated homogeneous layer of VWF was observed on the luminal surface from 30 minutes to day 7, but there was a progressive shift of positive staining from the lumen to the outer media from days 1 to 7 [14].
  • Variations in the method of inhibiting coagulation (none, heparin, citrate, hirudin, and EDTA) and of perfusing blood (in vitro v ex vivo) were compared by determining the influence of wall shear rate and vWF on the deposition of 111In-labeled platelets on subendothelium [15].
  • Porcine factor VIII:C prepared by affinity interaction with von Willebrand factor and heterologous antibodies: sodium dodecyl sulfate polyacrylamide gel analysis [16].

Regulatory relationships of VWF


Other interactions of VWF


Analytical, diagnostic and therapeutic context of VWF


  1. Differential aspects of the glycoprotein Ib-von Willebrand factor axis in human and pig species. Zurbano, M.J., Escolar, G., Heras, M., Ordinas, A., Castillo, R. Haematologica (2000) [Pubmed]
  2. Platelet thrombus formation on collagen type I. A model of deep vessel injury. Influence of blood rheology, von Willebrand factor, and blood coagulation. Badimon, L., Badimon, J.J., Turitto, V.T., Vallabhajosula, S., Fuster, V. Circulation (1988) [Pubmed]
  3. Platelet adhesion to damaged coronary arteries: Comparison in normal and von Willebrand disease swine. Reddick, R.L., Griggs, T.R., Lamb, M.A., Brinkhous, K.M. Proc. Natl. Acad. Sci. U.S.A. (1982) [Pubmed]
  4. Role of von Willebrand factor in arterial thrombosis. Studies in normal and von Willebrand disease pigs. Nichols, T.C., Bellinger, D.A., Reddick, R.L., Read, M.S., Koch, G.G., Brinkhous, K.M., Griggs, T.R. Circulation (1991) [Pubmed]
  5. von Willebrand's disease prevents occlusive thrombosis in stenosed and injured porcine coronary arteries. Nichols, T.C., Bellinger, D.A., Johnson, T.A., Lamb, M.A., Griggs, T.R. Circ. Res. (1986) [Pubmed]
  6. Transplantation of normal bone marrow into a pig with severe von Willebrand's disease. Bowie, E.J., Solberg, L.A., Fass, D.N., Johnson, C.M., Knutson, G.J., Stewart, M.L., Zoecklein, L.J. J. Clin. Invest. (1986) [Pubmed]
  7. Function of von Willebrand factor after crossed bone marrow transplantation between normal and von Willebrand disease pigs: effect on arterial thrombosis in chimeras. Nichols, T.C., Samama, C.M., Bellinger, D.A., Roussi, J., Reddick, R.L., Bonneau, M., Read, M.S., Bailliart, O., Koch, G.G., Vaiman, M. Proc. Natl. Acad. Sci. U.S.A. (1995) [Pubmed]
  8. Molecular genetic analysis of porcine von Willebrand disease: tight linkage to the von Willebrand factor locus. Bahou, W.F., Bowie, E.J., Fass, D.N., Ginsburg, D. Blood (1988) [Pubmed]
  9. Overexpression of von Willebrand factor is an independent risk factor for pathogenesis of intimal hyperplasia: preliminary studies. Qin, F., Impeduglia, T., Schaffer, P., Dardik, H. J. Vasc. Surg. (2003) [Pubmed]
  10. Effect of carbon monoxide on atherogenesis in normal pigs and pigs with von Willebrand's disease. Sultzer, D.L., Brinkhous, K.M., Reddick, R.L., Griggs, T.R. Atherosclerosis (1982) [Pubmed]
  11. A comparative study of the antithrombotic effect of aurintricarboxylic acid on arterial thrombosis in rats and guinea pigs. Takiguchi, Y., Shimazawa, M., Nakashima, M. Br. J. Pharmacol. (1996) [Pubmed]
  12. O-linked glycosylation and functional incompatibility of porcine von Willebrand factor for human platelet GPIb receptors. Schulte Am Esch, J., Robson, S.C., Knoefel, W.T., Hosch, S.B., Rogiers, X. Xenotransplantation (2005) [Pubmed]
  13. Comparison of the primary structure of the functional domains of human and porcine von Willebrand factor that mediate platelet adhesion. Bahnak, B.R., Lavergne, J.M., Ferreira, V., Kerbiriou-Nabias, D., Meyer, D. Biochem. Biophys. Res. Commun. (1992) [Pubmed]
  14. Redistribution of von Willebrand factor in porcine carotid arteries after balloon angioplasty. Giddings, J.C., Banning, A.P., Ralis, H., Lewis, M.J. Arterioscler. Thromb. Vasc. Biol. (1997) [Pubmed]
  15. Role of von Willebrand factor in mediating platelet-vessel wall interaction at low shear rate; the importance of perfusion conditions. Badimon, L., Badimon, J.J., Turitto, V.T., Fuster, V. Blood (1989) [Pubmed]
  16. Porcine factor VIII:C prepared by affinity interaction with von Willebrand factor and heterologous antibodies: sodium dodecyl sulfate polyacrylamide gel analysis. Knutson, G.J., Fass, D.N. Blood (1982) [Pubmed]
  17. P-selectin mRNA is expressed at a later phase of megakaryocyte maturation than mRNAs for von Willebrand factor and glycoprotein Ib-alpha. Schick, P.K., Konkle, B.A., He, X., Thornton, R.D. J. Lab. Clin. Med. (1993) [Pubmed]
  18. A newly established porcine aortic endothelial cell line: characterization and application to the study of human-to-swine graft rejection. Malassagne, B., Taboit, F., Conti, F., Batteux, F., Atia, N., Chéreau, C., Conjeaud, H., Théron, M.C., Attal, J., Braet, F., Houdebine, L.M., Calmus, Y., Houssin, D., Weill, B. Exp. Cell Res. (1998) [Pubmed]
  19. Antithrombotic effect of a recombinant von Willebrand factor, VCL, on nitrogen laser-induced thrombus formation in guinea pig mesenteric arteries. Azzam, K., Garfinkel, L.I., Bal dit Sollier, C., Cisse Thiam, M., Drouet, L. Thromb. Haemost. (1995) [Pubmed]
  20. Canine preprorelaxin: nucleic acid sequence and localization within the canine placenta. Klonisch, T., Hombach-Klonisch, S., Froehlich, C., Kauffold, J., Steger, K., Steinetz, B.G., Fischer, B. Biol. Reprod. (1999) [Pubmed]
  21. Effects of tempol, a free radical scavenger, on long-term hyperdynamic porcine bacteremia. Matejovic, M., Krouzecky, A., Martinkova, V., Rokyta, R., Radej, J., Kralova, H., Treska, V., Radermacher, P., Novak, I. Crit. Care Med. (2005) [Pubmed]
  22. Optimization of immunohistochemical techniques to detect extracellular matrix proteins in fixed skin specimens. Folkvord, J.M., Viders, D., Coleman-Smith, A., Clark, R.A. J. Histochem. Cytochem. (1989) [Pubmed]
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