Native人Von Willebrand Factor蛋白(factor VIII free) (ab88555)
Key features and details
- Expression system: Native
- Purity: > 95% SDS-PAGE
- Suitable for: SDS-PAGE
描述
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产品名称
Native人Von Willebrand Factor蛋白(factor VIII free)
参阅全部 Von Willebrand Factor 蛋白酶 -
纯度
> 95 % SDS-PAGE.
Purified VWF has been treated to dissociate from FVIII and subsequently separated from FVIII using size exclusion chromatography. -
表达系统
Native -
蛋白长度
Full length protein -
无动物成分
No -
性质
Native -
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种属
Human -
额外的序列信息
Amino acid sequence is not determined.
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描述
Native人Von Willebrand Factor蛋白
相关产品
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Related Products
技术指标
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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应用
SDS-PAGE
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形式
Liquid -
补充说明
Factor VIII Free. -
Concentration information loading...
制备和贮存
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稳定性和存储
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
常规信息
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别名
- Coagulation factor VIII
- Coagulation factor VIII VWF
- F8VWF
see all -
功能
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. -
组织特异性
Plasma. -
疾病相关
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. -
序列相似性
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. -
结构域
The von Willebrand antigen 2 is required for multimerization of vWF and for its targeting to storage granules. -
翻译后修饰
All cysteine residues are involved in intrachain or interchain disulfide bonds.
N- and O-glycosylated. -
细胞定位
Secreted. Secreted > extracellular space > extracellular matrix. Localized to storage granules. - Information by UniProt
实验方案
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.
数据表及文件
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SDS download
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Datasheet download
文献 (0)
ab88555 尚未被引用在任何文献中。