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VWF/Von Willebrand Factor  Protein, Antibody, ELISA Kit, cDNA Clone

表達宿主: CHO Stable Cells  
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10973-H08C-50
10973-H08C-100
50 µg 
100 µg 
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VWF/Von Willebrand Factor 相關信號通路

    VWF/Von Willebrand Factor 概述&蛋白信息

    VWF/Von Willebrand Factor 研究背景

    基因概述: 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
    亞單位結構: Multimeric. Interacts with F8.
    結構域: The von Willebrand antigen 2 is required for multimerization of vWF and for its targeting to storage granules.
    亞細胞定位: Secreted. Secreted, extracellular space, extracellular matrix. Note=Localized to storage granules.
    組織特異性: Plasma.
    翻譯後修飾: All cysteine residues are involved in intrachain or interchain disulfide bonds.
    N- and O-glycosylated.
    相關疾病 : 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.
    相似的序列: 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/Von Willebrand Factor 別稱

    VWD,F8VWF, [homo-sapiens]
    F8VWF,VWD,VWF, [human]
    6820430P06Rik,AI551257,B130011O06Rik,C630030D09,F8VWF,VWD,Vwf, [mouse]
    VWD,F8VWF,AI551257,C630030D09,6820430P06Rik,B130011O06Rik, [mus-musculus]

    VWF/Von Willebrand Factor 相關文獻

  • 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.
  • 請注意:所有產品都是“僅用於科研,而不能用於診斷或治療用途”