Multimeric Structure of von Willebrand Factor: Each vWF monomer (Mr = 260kD) contains a Factor VIII binding site near the NH2-terminal end of the molecule. The monomers are joined end-to-end (NH2 to NH2 and COOH to COOH) by disulfide bonds to form large multimers. The mature multimers can bind one Factor VIII molecule per monomeric subunit. Von Willebrand Factor (vWF) is a multimeric plasma glycoprotein that is required for normal hemostatic platelet plug formation (1-8). The mature plasma protein is composed of apparently identical subunits (Mr = 260kD) which are held together by disulfide bonds. The circulating vWF molecule ranges in size from dimers (Mr = 520kD) to extremely large multimers (Mr > 10,000,000). During normal hemostasis, the larger multimers of vWF are responsible for facilitating platelet plug formation by forming a bridge between platelet glycoprotein IB and exposed collagen in the subendothelium (9-14). Either a lack of vWF protein or the presence of abnormalities which result in decreased polymerization may cause a loss of biological activity which is characteristic of von Willebrand's Disease.
In addition to its role in platelet plug formation, vWF is also responsible for the binding and transport of Factor VIII (Antihemophilic Aactor) 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.
Localization:Plasma and subendothelium
Mode of Action:Facilitates platelet plug formation by forming a bridge between platelet glycoprotein IB and exposed collagen in the subendothelium. Also binds and transports Factor VIII.
Molecular Weight:Monomer: 260kDMultimers: 1-20 x 1000kD (multimers)
Structure:Multimeric protein composed of identical 250kD molecular weight subunits.
Storage and Stability:Store at -70°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note:Immediately after removal from dry ice (-80°C). Thaw in 37°C water bath for several minutes to prevent a precipitation of vWF. Do not agitate or vortex.
Related Product:V2650: Von Willebrand Factor, Human
仕様
Size：100ug
Source Antigen：Human plasma
Grade：Highly Purified
Purity：≥95% by SDS-PAGE
Form：Supplied as a liquid in 25mM sodium citrate, 100mM glycine, 100mM sodium chloride, pH 6.8.
