Overview

  • Product name

    Anti-Von Willebrand Factor antibody [VWF/1465]
    See all Von Willebrand Factor primary antibodies
  • Description

    Mouse monoclonal [VWF/1465] to Von Willebrand Factor
  • Host species

    Mouse
  • Tested applications

    Suitable for: WB, IHC-Pmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Recombinant fragment within Human Von Willebrand Factor aa 1815-1939. The exact sequence is proprietary.
    Sequence:

    ARSNRV TVFPIGIGDR YDAAQLRILA GPAGDSNVVK LQRIEDLPTM VTLGNSFLHK LCSGFVRICM DEDGNEKRPG DVWTLPDQCH TVTCQPDGQT LLKSHRVNCD RGLRPSCPNS QSPVKVEET


    Database link: P04275

  • Positive control

    • Human lung lysate; Human tonsil tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab218333 in the following tested applications.

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

Application Abreviews Notes
WB Use a concentration of 0.5 - 1 µg/ml. Predicted molecular weight: 309 kDa.
IHC-P Use a concentration of 1 - 2 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Target

  • 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
    modifications

    All 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
  • Database links

  • Alternative names

    • Coagulation factor VIII antibody
    • Coagulation factor VIII VWF antibody
    • F8VWF antibody
    • Factor VIII related antigen antibody
    • von Willebrand antigen 2 antibody
    • von Willebrand antigen II antibody
    • Von Willebrand disease antibody
    • Von Willebrand factor precursor antibody
    • VWD antibody
    • vWF antibody
    • VWF_HUMAN antibody
    see all

Images

  • All lanes : Anti-Von Willebrand Factor antibody [VWF/1465] (ab218333) at 1 µg/ml

    Lane 1 : Recombinant protein
    Lane 2 : Human lung lysate

    Predicted band size: 309 kDa

  • Immunohistochemical analysis of Formalin-fixed, paraffin-embedded Human tonsil tissue labeling Von Willebrand Factor with ab218333 at 2µg/ml.

References

ab218333 has not yet been referenced specifically in any publications.

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