Natural Human Von Willebrand Factor protein (ab88533)

製品の概要

製品の詳細

  • 由来Natural
  • 由来Native
  • アミノ酸配列
    • 生物種Human

特性

Our Abpromise guarantee covers the use of ab88533 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • アプリケーション

    SDS-PAGE

  • 精製度> 95 % SDS-PAGE.

  • 製品の状態Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    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

関連情報

  • 別名
    • 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
  • 機能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.
  • 組織特異性Plasma.
  • 関連疾患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.
  • 配列類似性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.
  • ドメインThe von Willebrand antigen 2 is required for multimerization of vWF and for its targeting to storage granules.
  • 翻訳後修飾All cysteine residues are involved in intrachain or interchain disulfide bonds.
    N- and O-glycosylated.
  • 細胞内局在Secreted. Secreted > extracellular space > extracellular matrix. Localized to storage granules.
  • Information by UniProt

Natural Human Von Willebrand Factor protein 画像

  • 4-12% SDS-PAGE analysis of Reduced and Non-reduced samples of ab88533 (1µg).
    Molecular weight markers: Myosin (191 kDa), Phosphorylase B (97 kDa), BSA (64 kDa), Glutamic Dehydrogenase (51 kDa), Alcohol Dehydrogenase (39 kDa), Carbonic Anhydrase (28 kDa), Myoglobin Red (19 kDa), Lysozyme (14 kDa)

Natural Human Von Willebrand Factor protein (ab88533) 使用論文

ab88533 has not yet been referenced specifically in any publications.

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