VWF
GeneName
VWF
Summary
VWF, or von Willebrand factor, is a large glycoprotein with a mass of 309 kDa, predominantly expressed in endothelial cells and platelets. It plays a critical role in haemostasis by mediating platelet adhesion to the site of vascular injury and stabilising factor VIII in circulation. VWF is found in various cellular compartments, including the Weibel-Palade bodies of endothelial cells and platelet alpha granules, and is secreted into the extracellular space. Its functions are mediated through interactions with collagen, integrins, and other proteins, facilitating processes such as blood coagulation and cell adhesion.
Importance
VWF is relevant to: - Disorders of bleeding, such as von Willebrand disease, due to its essential role in platelet function and coagulation - Cardiovascular research, as it is involved in thrombus formation and vascular integrity - Wound healing processes, where it contributes to cell-substrate adhesion and response to injury - The study of endothelial function and dysfunction, particularly in relation to atherosclerosis and other vascular diseases
Top Products
For researchers investigating VWF, we recommend two excellent primary antibodies. The first is the well-cited polyclonal antibody, Anti-Von Willebrand Factor antibody (ab6994), which has garnered 594 citations, highlighting its reliability in immunohistochemistry (IHC). This antibody is a trusted choice for those focusing on VWF detection in tissue samples. Additionally, we offer the recombinant antibody, Anti-Von Willebrand Factor antibody [EPSISR15] (ab154193). This monoclonal antibody has been validated for use in immunocytochemistry (ICC), western blotting (WB), and flow cytometry (FC), making it a versatile option for various experimental needs. With 25 citations, it is gaining recognition in the research community for its consistent performance. Together, these antibodies provide robust tools for studying VWF in different contexts. "The Human Von Willebrand Factor ELISA Kit (ab223864), backed by 11 citations, is an excellent option for researchers looking to accurately measure VWF levels in their samples."
Abcam Product Citation Summary
The data indicates a significant focus on the use of VWF antibodies in various contexts related to endothelial cells and vascular health. Studies have been conducted on human, rat, and mouse models, highlighting the role of VWF in conditions such as pulmonary arterial hypertension, gastric cancer, and endothelial cell behaviour. The applications of these antibodies include Western blotting, immunohistochemistry, and immunofluorescence, demonstrating their versatility in research.
Abcam Product Citation Table
Domain
The von Willebrand antigen 2 is required for multimerization of vWF and for its targeting to storage granules.
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.
Involvement in disease
von Willebrand disease 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.
None
The disease is caused by variants affecting the gene represented in this entry.
von Willebrand disease 2
VWD2
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.
None
The disease is caused by variants affecting the gene represented in this entry.
von Willebrand disease 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.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
All cysteine residues are involved in intrachain or interchain disulfide bonds.
N- and O-glycosylated.
Tissue Specificity
Plasma.
Cellular localization
- Secreted
- Secreted
- Extracellular space
- Extracellular matrix
- Localized to storage granules.
Alternative names
F8VWF, VWF, von Willebrand factor, vWF
Database links
swissprot:P04275 entrezGene:7450 omim:613160
Other research areas
- Oncology