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Anti-Von-Willebrand-Factor Antibody

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产品名称: Anti-Von-Willebrand-Factor Antibody
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Anti-Von-Willebrand-Factor Antibody


Anti-Von-Willebrand-Factor Antibody  的详细介绍
Name: Anti-Von-Willebrand-Factor Antibody
See all Von Willebrand Factor primary antibodies
Description: Mouse monoclonal (F8/86) antibody to Von-Willebrand-Factor.
Specificity: Human von-Willebrand-Factor, Factor VIII related Antigen.
Applications: IHC
Reactivity: Human
Immunogen: Purified von-Willebrand-Factor isolated from human plasma.
Host: Mouse
Clonality: Monoclonal
Clone: F8/86
Isotype: IgG1
Conjugate: Unconjugated
Product Form: Liquid
Formulation: Supplied in Phosphate Buffered Saline, pH 7.3, with 0.09% Sodium Azide.
Storage: Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
General Notes: This antibody detects normal endothelia, megakaryocytes and platelets, but also tumours derived from these cells. It is a good marker for vascularisation of tissues and re-vascularisation after injury. This antibody may also be used for the detection of tumour invasion into blood vessels.
Function: Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex GPIb-IX-V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma.
Tissue Specificity: Plasma.
Involvement in Disease: von Willebrand disease 1: 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.

von Willebrand disease 2: 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.

von Willebrand disease 3: 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.
Post-Translational Modification: All cysteine residues are involved in intrachain or interchain disulfide bonds.
Cellular Location: Secreted. Secreted > Extracellular space > Extracellular matrix.

Localized to storage granules.
Database Links:
  • Entrez Gene: 7450?Human
  • Omim: 613160?Human
  • SwissProt: P04275?Human
  • Unigene: 440848?Human
    		•
    Synonyms:
  • Coagulation factor VIII Antibody
  • Coagulation factor VIII VWF Antibody
  • F8VWF Antibody
  • Factor VIII related antigen Antibody
  • von Willebrand antigen 2 Antibody
  • von Willebrand antigen II Antibody
  • Von Willebrand disease Antibody
  • Von Willebrand Factor Antibody
  • VWD Antibody
  • vWF Antibody
  • VWF_HUMAN Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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