Von Willebrand Factor Protein | VWF protein
Von Willebrand Factor, Human (vWF, Factor VIII Related Antigen)
95% by SDS-PAGE. vWF is prepared from citrated human plasma using a combination of the procedures described by Thorell (19), and Lollar (20). A factor VIII free vWF preparation, further purified to ensure removal of factor VIII procoagu
95% by SDS-PAGE. vWF is prepared from citrated human plasma using a combination of the procedures described by Thorell (19), and Lollar (20). A factor VIII free vWF preparation, further purified to ensure removal of factor VIII procoagu
In addition to its role in platelet plug formation, vWF is also responsible for the binding and transport of factor VIII (antihemophilic factor) in plasma (15). It appears that this latter event is responsible for both the stability and effective delivery of functional factor VIII. Studies indicate that factor VIII binds to the NH2-terminal portion of the mature vWF subunit with a stoichiometry of one factor VIII molecule per vWF monomer (16,17).
The single chain vWF monomer contains a large number of cysteine residues at both the NH2-terminal and COOH-terminal ends, which are involved in the multimer formation. Carbohydrate analyses indicate that nearly 15% of the mass of vWF is contributed by carbohydrate (18). It appears that the carbohydrate serves to protect vWF from proteolysis, but is not necessary for functional activity or multimer formation.
NCBI and Uniprot Product Information
NCBI Description
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, Jul 2008]
Uniprot Description
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; Motility/polarity/chemotaxis; Secreted, signal peptide; Cell adhesion; 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; protein N-terminus binding; immunoglobulin 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; protein homooligomerization; cell-substrate adhesion; placenta development
Disease: Von Willebrand Disease, Type 3; Von Willebrand Disease, Type 1; Von Willebrand Disease, Type 2
Research Articles on VWF
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Product Notes
The VWF vwf (Catalog #AAA634277) is a Protein produced from Human plasma and is intended for research purposes only. The product is available for immediate purchase. It is sometimes possible for the material contained within the vial of "Von Willebrand Factor, Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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