Anti-Von Willebrand Factor Antibody (RMAB-0251200)
Cat. No.: RMAB-0251200
Category: Primary Antibodies
INQUIRY
100 μL
Customer Size
Rabbit monoclonal to Von Willebrand Factor
Product Features
| Isotype | IgG |
|---|---|
| Clonality | Monoclonal |
| Host Species | Rabbit |
| Clone Number | EPR25069-131 |
| Form | Liquid |
| Purity | Protein A purified |
| Species Reactivity | Mouse, Rat |
| Immunogen | Recombinant fragment. |
| Applications | IHC-P, WB, IHC-Fr, Flow Cyt, ICC/IF |
| Key Features | Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply; Rabbit monoclonal to Von Willebrand Factor; Suitable for: IHC-P, WB, IHC-Fr, Flow Cyt, ICC/IF; Reacts with: Mouse, Rat |
Target Information
| Target Symbol | VWF |
|---|---|
| Target Name | von Willebrand factor |
| UniProt ID | P04275 |
| Cellular Localization | Secreted. Secreted > extracellular space > extracellular matrix. Localized 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 | Defects in VWF are the cause of von Willebrand disease (VWD). VWD defines a group of hemorrhagic disorders in which the von Willebrand factor is either quantitatively or qualitatively abnormal resulting in altered platelet function. Symptoms vary depending on severity and disease type but may include prolonged bleeding time, deficiency of factor VIII and impaired platelet adhesion. Type I von Willebrand disease is the most common form and is characterized by partial quantitative plasmatic deficiency of an otherwise structurally and functionally normal Willebrand factor; type II is associated with a qualitative deficiency and functional anomalies of the Willebrand factor; type III is the most severe form and is characterized by total or near-total absence of Willebrand factor in the plasma and cellular compartments, also leading to a profound deficiency of plasmatic factor VIII. |
| Post-translational Modifications | All cysteine residues are involved in intrachain or interchain disulfide bonds. N- and O-glycosylated. |
| Domain | The von Willebrand antigen 2 is required for multimerization of vWF and for its targeting to storage granules. |
| Sequence Similarities | 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. |
Storage & Shipping
| Storage Buffer | pH: 7.20; Preservative: 0.01% Sodium azide; Constituents: 59% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA |
|---|---|
| Storage & Shipping | Shipped at 4°C. Store at 4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. |
For research use only. Not for clinical use.
