Native Human Von Willebrand Factor protein (factor VIII free) (ab88555)
Key features and details
- Expression system: Native
- Purity: > 95% SDS-PAGE
- Suitable for: SDS-PAGE
Description
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Product name
Native Human Von Willebrand Factor protein (factor VIII free)
See all Von Willebrand Factor proteins and peptides -
Purity
> 95 % SDS-PAGE.
Purified VWF has been treated to dissociate from FVIII and subsequently separated from FVIII using size exclusion chromatography. -
Expression system
Native -
Protein length
Full length protein -
Animal free
No -
Nature
Native -
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Species
Human -
Additional sequence information
Amino acid sequence is not determined.
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Description
Native Human Von Willebrand Factor protein
Associated products
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Related Products
Specifications
Our Abpromise guarantee covers the use of ab88555 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Applications
SDS-PAGE
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Form
Liquid -
Additional notes
Factor VIII Free. -
Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 6.80
Constituents: 0.8% Sodium citrate, 0.8% Glycine, 0.7% Sodium chloride, 1% Human von Willibrand Factor
General Info
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Alternative names
- Coagulation factor VIII
- Coagulation factor VIII VWF
- F8VWF
see all -
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. -
Tissue specificity
Plasma. -
Involvement in disease
Defects in VWF are the cause of von Willebrand disease (VWD) [MIM:277480]. 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. -
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. -
Domain
The von Willebrand antigen 2 is required for multimerization of vWF and for its targeting to storage granules. -
Post-translational
modificationsAll cysteine residues are involved in intrachain or interchain disulfide bonds.
N- and O-glycosylated. -
Cellular localization
Secreted. Secreted > extracellular space > extracellular matrix. Localized to storage granules. - Information by UniProt
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
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SDS download
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Datasheet download
References (0)
ab88555 has not yet been referenced specifically in any publications.