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VWF Protein, Antibody, ELISA Kit, cDNA Clone

VWF Related Areas

VWF Related Pathways

VWF Related Product

    VWF Summary & Protein Information

    VWF Background

    Gene Summary: The glycoprotein encoded by VWF gene functions as both an antihemophilic factor carrier and a platelet-vessel wall mediator in the blood coagulation system. It is crucial to the hemostasis process. Mutations in this gene or deficiencies in this protein result in von Willebrand's disease. An unprocessed pseudogene has been found on chromosome 22. [provided by RefSeq, Jul 2008]
    General information above from NCBI
    Subunit structure: Multimeric. Interacts with F8.
    Domain: The von Willebrand antigen 2 is required for multimerization of vWF and for its targeting to storage granules.
    Subcellular location: Secreted. Secreted, extracellular space, extracellular matrix. Note=Localized to storage granules.
    Tissue specificity: Plasma.
    Post-translational: All cysteine residues are involved in intrachain or interchain disulfide bonds.
    N- and O-glycosylated.
    Involvement in disease: von Willebrand disease 1 (VWD1) [MIM:193400]: A common hemorrhagic disorder due to defects in von Willebrand factor protein and resulting in impaired platelet aggregation. Von Willebrand disease type 1 is characterized by partial quantitative deficiency of circulating von Willebrand factor, that is otherwise structurally and functionally normal. Clinical manifestations are mucocutaneous bleeding, such as epistaxis and menorrhagia, and prolonged bleeding after surgery or trauma. Note=The disease is caused by mutations affecting the gene represented in this entry.
    von Willebrand disease 2 (VWD2) [MIM:613554]: A hemorrhagic disorder due to defects in von Willebrand factor protein and resulting in altered platelet aggregation. Von Willebrand disease type 2 is characterized by qualitative deficiency and functional anomalies of von Willebrand factor. It is divided in different subtypes including 2A, 2B, 2M and 2N (Normandy variant). The mutant VWF protein in types 2A, 2B and 2M are defective in their platelet-dependent function, whereas the mutant protein in type 2N is defective in its ability to bind factor VIII. Clinical manifestations are mucocutaneous bleeding, such as epistaxis and menorrhagia, and prolonged bleeding after surgery or trauma. Note=The disease is caused by mutations affecting the gene represented in this entry.
    von Willebrand disease 3 (VWD3) [MIM:277480]: A severe hemorrhagic disorder due to a total or near total absence of von Willebrand factor in the plasma and cellular compartments, also leading to a profound deficiency of plasmatic factor VIII. Bleeding usually starts in infancy and can include epistaxis, recurrent mucocutaneous bleeding, excessive bleeding after minor trauma, and hemarthroses. Note=The disease is caused by mutations affecting the gene represented in this entry.
    Sequence similarity: Contains 1 CTCK (C-terminal cystine knot-like) domain.
    Contains 4 TIL (trypsin inhibitory-like) domains.
    Contains 3 VWFA domains.
    Contains 3 VWFC domains.
    Contains 4 VWFD domains.
    General information above from UniProt

    Von Willebrand Factor (VWF) is a multimeric glycoprotein involved in hemostasis in blood, binds receptors on the surface of platelets and in connective tissue, thereby mediating the adhesion of platelets to sites of vascular injury. From studies it appears that VWF protein uncoils under these circumstances, decelerating passing platelets. VWF protein is deficient or defective in von Willebrand disease (VWD) and is involved in a large number of other diseases, including thrombosis, thrombotic thrombocytopenic purpura, Stroke, Heyde's syndrome, possibly hemolytic-uremic syndrome and so on.

    VWF Alternative Name

    F8VWF,VWD,VWF, [human]
    6820430P06Rik,AI551257,B130011O06Rik,C630030D09,F8VWF,VWD,Vwf, [mouse]

    VWF Related Studies

  • Sadler JE. (1998) Biochemistry and genetics of von Willebrand factor. Annu Rev Biochem, 67: 395-424.
  • Batlle J, et al. (2009) Von Willebrand factor/factor VIII concentrates in the treatment of von Willebrand disease. Blood Coagul Fibrinolysis. 20(2):89-100.
  • Sadler JE. (2009) von Willebrand factor assembly and secretion. J Thromb Haemost. 7 Suppl 1:24-7.
  • Auton M, et al. (2010) The mechanism of VWF-mediated platelet GPIbalpha binding. Biophys J. 99(4):1192-201.
  • Bowen DJ. (2010) Sugar targets VWF for the chop. Blood. 115(13):2565.
  • Lpez JA, et al. (2010) VWF self-association: more bands for the buck. Blood. 116(19):3693-4.
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