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Gene Review

VWF  -  von Willebrand factor

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Disease relevance of VWF


High impact information on VWF


Biological context of VWF


Anatomical context of VWF


Associations of VWF with chemical compounds

  • This probe codes for the major adhesion domain of vWF that includes the GPIb, collagen and heparin binding domains [16].
  • Cysteine residues #471, 474, 509 and 695, which form intrachain bonds in human vWF, are also present in the bovine vWF sequence [16].
  • VWF binding to formaldehyde-fixed platelets was dependent on the presence of a promoter such as ristocetin [11].
  • Polycations, such as poly(L-lysine) and Polybrene, also promoted the formation of platelet aggregates and facilitated the binding of VWF to platelets [11].
  • Inhibition of von Willebrand factor-dependent platelet function by increased platelet cyclic AMP and its prevention by cytoskeleton-disrupting agents [17].

Physical interactions of VWF

  • However, in endothelial cells, mutation of the VWF E4BP4 binding motif not only restores but also further elevates VWF promoter activity, suggesting that E4BP4 may be part of a coordinated binding complex [18].

Other interactions of VWF


Analytical, diagnostic and therapeutic context of VWF

  • To determine directly the location of the epitope for each mAb on the bovine pp-vWF molecule, we tested the reactivity of mAbs by immunoblotting toward peptide fragments obtained by digestion with lysylendopeptidase [24].
  • We have previously reported the identification of a region of the vWf gene that regulates its cell-type-specific expression in cell culture [19].
  • Physical changes associated with anti-vWF-induced uptake of vWF are not seen in platelets that are involved in hemostatic plug formation or clot retraction [25].
  • Secretion of vWF by BAECs under physiological glucose and high glucose conditions with or without thiamine (200 micromol/L) supplementation was studied with enzyme-linked immunosorbent assay [5].
  • Characterization of von Willebrand factor interaction with collagens in real time using surface plasmon resonance [26].


  1. Specific synergy of multiple substrate-receptor interactions in platelet thrombus formation under flow. Savage, B., Almus-Jacobs, F., Ruggeri, Z.M. Cell (1998) [Pubmed]
  2. Venom coagglutinin: an activator of platelet aggregation dependent on von Willebrand factor. Read, M.S., Shermer, R.W., Brinkhous, K.M. Proc. Natl. Acad. Sci. U.S.A. (1978) [Pubmed]
  3. Glanzmann thrombasthenia: deficient binding of von Willebrand factor to thrombin-stimulated platelets. Ruggeri, Z.M., Bader, R., de Marco, L. Proc. Natl. Acad. Sci. U.S.A. (1982) [Pubmed]
  4. Primary binding domain of bovine von Willebrand factor fragment expressed in E. coli. Bakhshi, M.R., Sinha, D., Vora, R.K., Budzynski, A.Z., Kirby, E.P. Thromb. Haemost. (1996) [Pubmed]
  5. Thiamine reverses hyperglycemia-induced dysfunction in cultured endothelial cells. Ascher, E., Gade, P.V., Hingorani, A., Puthukkeril, S., Kallakuri, S., Scheinman, M., Jacob, T. Surgery (2001) [Pubmed]
  6. Heparin inhibition of von Willebrand factor-dependent platelet function in vitro and in vivo. Sobel, M., McNeill, P.M., Carlson, P.L., Kermode, J.C., Adelman, B., Conroy, R., Marques, D. J. Clin. Invest. (1991) [Pubmed]
  7. Identification and isolation of a platelet GPIb-like protein in human umbilical vein endothelial cells and bovine aortic smooth muscle cells. Asch, A.S., Adelman, B., Fujimoto, M., Nachman, R.L. J. Clin. Invest. (1988) [Pubmed]
  8. A coagulation pathway on bovine aortic segments leading to generation of Factor Xa and thrombin. Stern, D.M., Nawroth, P.P., Kisiel, W., Handley, D., Drillings, M., Bartos, J. J. Clin. Invest. (1984) [Pubmed]
  9. Comparison of the 5'-flanking sequences of the human and bovine von Willebrand factor-encoding genes reveals alternation of highly homologous domains with species-specific Alu-type repeats. Janel, N., Schwachtgen, J.L., Bakhshi, M.R., Barek, L., Meyer, D., Kerbiriou-Nabias, D. Gene (1995) [Pubmed]
  10. Identification of factor-XIIIa-reactive glutaminyl residues in the propolypeptide of bovine von Willebrand factor. Takagi, J., Aoyama, T., Ueki, S., Ohba, H., Saito, Y., Lorand, L. Eur. J. Biochem. (1995) [Pubmed]
  11. von Willebrand factor. A protein which binds at the cell surface interface between platelets. Senogles, S.E., Nelsestuen, G.L. J. Biol. Chem. (1983) [Pubmed]
  12. Endocrine and cellular characteristics of corpora lutea from cows with a delayed post-ovulatory progesterone rise. Robinson, R.S., Hammond, A.J., Nicklin, L.T., Schams, D., Mann, G.E., Hunter, M.G. Domest. Anim. Endocrinol. (2006) [Pubmed]
  13. Interaction of blood platelets with a microfibrillar extract from adult bovine aorta: requirement for von Willebrand factor. Fauvel, F., Grant, M.E., Legrand, Y.J., Souchon, H., Tobelem, G., Jackson, D.S., Caen, J.P. Proc. Natl. Acad. Sci. U.S.A. (1983) [Pubmed]
  14. Vicinal cysteines in the prosequence play a role in von Willebrand factor multimer assembly. Mayadas, T.N., Wagner, D.D. Proc. Natl. Acad. Sci. U.S.A. (1992) [Pubmed]
  15. Ets transcription factors bind and transactivate the core promoter of the von Willebrand factor gene. Schwachtgen, J.L., Janel, N., Barek, L., Duterque-Coquillaud, M., Ghysdael, J., Meyer, D., Kerbiriou-Nabias, D. Oncogene (1997) [Pubmed]
  16. Sequencing of the primary adhesion domain of bovine von Willebrand factor. Bakhshi, M.R., Myers, J.C., Howard, P.S., Soprano, D.R., Kirby, E.P. Biochim. Biophys. Acta (1992) [Pubmed]
  17. Inhibition of von Willebrand factor-dependent platelet function by increased platelet cyclic AMP and its prevention by cytoskeleton-disrupting agents. Coller, B.S. Blood (1981) [Pubmed]
  18. Cell type-specific regulation of von Willebrand factor expression by the E4BP4 transcriptional repressor. Hough, C., Cuthbert, C.D., Notley, C., Brown, C., Hegadorn, C., Berber, E., Lillicrap, D. Blood (2005) [Pubmed]
  19. An NF1-like protein functions as a repressor of the von Willebrand factor promoter. Jahroudi, N., Ardekani, A.M., Greenberger, J.S. J. Biol. Chem. (1996) [Pubmed]
  20. Identification of a factor IX/IXa binding protein on the endothelial cell surface. Rimon, S., Melamed, R., Savion, N., Scott, T., Nawroth, P.P., Stern, D.M. J. Biol. Chem. (1987) [Pubmed]
  21. Platelet adhesion to collagen. Factors affecting Mg2(+)-dependent and bivalent-cation-independent adhesion. Zijenah, L.S., Morton, L.F., Barnes, M.J. Biochem. J. (1990) [Pubmed]
  22. Synergistic induction of t-PA by vascular endothelial growth factor and basic fibroblast growth factor and localization of t-PA to Weibel-Palade bodies in bovine microvascular endothelial cells. Pepper, M.S., Rosnoblet, C., Di Sanza, C., Kruithof, E.K. Thromb. Haemost. (2001) [Pubmed]
  23. SCO-spondin and RF-GlyI: two designations for the same glycoprotein secreted by the subcommissural organ. Didier, R., Creveaux, I., Meiniel, R., Herbet, A., Dastugue, B., Meiniel, A. J. Neurosci. Res. (2000) [Pubmed]
  24. Monoclonal antibodies that inhibit binding of propolypeptide of von Willebrand factor to collagen. Localization of epitopes. Fujisawa, T., Takagi, J., Sekiya, F., Goto, A., Miake, F., Saito, Y. Eur. J. Biochem. (1991) [Pubmed]
  25. Uptake of vWF-anti-vWF complexes by platelets in suspension. White, J.G., Krumwiede, M.D., Cocking-Johnson, D.J., Escolar, G. Arterioscler. Thromb. Vasc. Biol. (1996) [Pubmed]
  26. Characterization of von Willebrand factor interaction with collagens in real time using surface plasmon resonance. Li, F., Moake, J.L., McIntire, L.V. Annals of biomedical engineering. (2002) [Pubmed]
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