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

Overview

Description

  • NatureNatural
  • SourceNative
  • Amino Acid Sequence
    • SpeciesHuman

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.

  • Applications

    SDS-PAGE

  • Purity> 95 % SDS-PAGE.

  • FormLiquid
  • Additional notesFactor VIII Free.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    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

General Info

  • Alternative names
    • 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
  • FunctionImportant 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 specificityPlasma.
  • Involvement in diseaseDefects 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 similaritiesContains 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.
  • DomainThe von Willebrand antigen 2 is required for multimerization of vWF and for its targeting to storage granules.
  • Post-translational
    modifications
    All cysteine residues are involved in intrachain or interchain disulfide bonds.
    N- and O-glycosylated.
  • Cellular localizationSecreted. Secreted > extracellular space > extracellular matrix. Localized to storage granules.
  • Information by UniProt

References for Natural Human Von Willebrand Factor protein (factor VIII free) (ab88555)

ab88555 has not yet been referenced specifically in any publications.

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