Natural人Von Willebrand Factor protein (factor VIII free) (ab88555)

概述

描述

  • 性质Natural
  • 来源Native
  • 氨基酸序列
    • 种属Human

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.

  • 应用

    SDS-PAGE

  • 纯度> 95 % SDS-PAGE.

  • 形式Liquid
  • 补充说明Factor VIII Free.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    Preservative: None
    Constituents: 25mM Sodium citrate, 100mM Sodium chloride, 100mM Glycine, pH 6.8

常规信息

  • 别名
    • Coagulation factor VIII
    • Coagulation factor VIII VWF
    • F8VWF
    • Factor VIII related antigen
    • von Willebrand antigen 2
    • von Willebrand antigen II
    • Von Willebrand disease
    • VWD
    • vWF
    • VWF_HUMAN
    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

Natural Human Von Willebrand Factor protein (factor VIII free) (ab88555)参考文献

ab88555 has not yet been referenced specifically in any publications.

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