The product specifically reacts with the cytoplasm of human endothelial cells from normal and neoplastic blood and from lymphatic vessels. It also reacts with human endocardium, platelets and megakaryocytes.
Historically, this antibody worked well in mouse (see references), however more recent batches have shown inconsistent results in mouse. Therefore, we do not recommend this antibody for use in this species.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/200. (see Abreview)
1/200 - 1/400. for IF and 1/1000-1/2000 for ABC methods with HRP conjugates. Perform enzymatic antigen retrieval with 0.1% pronase for 10 min at 35 °C before commencing with IHC protocol.
Indirect Immunofluorescence: minimum working dilution of 1:200 was determined using FFPE sections of human tongue with FITC-conjugated secondary.
Indirect Immunoperoxidase Labeling: minimum working dilution of 1:800 was determined using FFPE sections of human tongue with biotinylated secondary and signal amplification.
Use a concentration of 5 µg/ml.
ab171870 - Rabbit polyclonal IgG, is suitable for use as an isotype control with this antibody.
Use at an assay dependent concentration. PubMed: 19622235
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.
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.
Immunocytochemistry/ Immunofluorescence - Anti-Von Willebrand Factor antibody (ab6994)This image is courtesy of Adam Puche
Confocal extended focus photograph of von Willebrand factor staining (red) and non-specific nuclear counterstain (green) in the adult mouse olfactory bulb. This picture shows a single major vessel within the glomerular layer of the olfactory bulb. For more details see the review of this antibody by Adam Puche.
Immunocytochemical analysis labeling Von Willebrand Factor with ab6994 at 1/100 dilution.
The nuclear counterstain is DAPI(blue)
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Von Willebrand Factor antibody (ab6994)This image is courtesy of an anonymous Abreview
ab6994 staining Von Willebrand Factor in pig skin tissue sections by Immunohistochemistry (IHC-P - paraformaldehyde-fixed, paraffin-embedded sections). Tissue was fixed with formaldehyde and blocked with 3% serum for 30 minutes at 20°C; antigen retrieval was enzymatic using pronase, 1mg/ml. Samples were incubated with primary antibody (1/500 in PBS) for 12 hours at 4°C. A Biotin-conjugated goat anti-rabbit IgG polyclonal (1/200) was used as the secondary antibody.
Xu L et al. Umbilical cord-derived mesenchymal stem cells on scaffolds facilitate collagen degradation via upregulation of MMP-9 in rat uterine scars. Stem Cell Res Ther8:84 (2017).
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Montali M et al. Human adult mesangiogenic progenitor cells reveal an early angiogenic potential, which is lost after mesengenic differentiation. Stem Cell Res Ther8:106 (2017).
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