von Willebrand Factor

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 12; NC_000012.11 (6058040..6233836, complement). Location: 12p13.3

Monoclonal

VW92-3

Mouse

von Willebrand Factor [Mouse monoclonal antibody to von Willebrand Factor]

200 ug IgG in 10 mM PBS, pH 7.4, lyophilized.

Lyophilized antibody is stable at 4 degree C for 2 years. Store the reconstituted solution in aliquots at -20 degree C for one year or at 4 degree C for 6 months. Avoid repeated freeze-thaw cycles.

Small volumes of anti-VWF antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Coagulation Factor Monoclonal Antibodies

ELISA, Western Blot, Immunohistochemistry

309,265 Da[Similar Products]

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VWF_HUMAN

The glycoprotein encoded by this 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]

VWF: 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. Defects in VWF are the cause of von Willebrand disease type 1 (VWD1). 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. Defects in VWF are the cause of von Willebrand disease type 2 (VWD2). A hemorrhagic disorder due to defects in von Willebrand factor protein and resulting in impaired 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. Defects in VWF are the cause of von Willebrand disease type 3 (VWD3). 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.

Protein type: Secreted, signal peptide; Cell adhesion; Secreted; Motility/polarity/chemotaxis; Extracellular matrix

Chromosomal Location of Human Ortholog: 12p13.3

Cellular Component: extracellular matrix; proteinaceous extracellular matrix; endoplasmic reticulum; extracellular region; external side of plasma membrane

Molecular Function: integrin binding; collagen binding; identical protein binding; protein binding; protein homodimerization activity; protease binding; chaperone binding; immunoglobulin binding; protein N-terminus binding; glycoprotein binding

Biological Process: platelet activation; extracellular matrix organization and biogenesis; platelet degranulation; hemostasis; response to wounding; cell adhesion; blood coagulation; liver development; blood coagulation, intrinsic pathway; cell-substrate adhesion; protein homooligomerization; placenta development

Disease: Von Willebrand Disease, Type 3; Von Willebrand Disease, Type 1; Von Willebrand Disease, Type 2

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