Product nameAnti-Von Willebrand Factor antibody [EPR18569-105]
See all Von Willebrand Factor primary antibodies
DescriptionRabbit monoclonal [EPR18569-105] to Von Willebrand Factor
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Mouse, Human
Recombinant fragment within Mouse Von Willebrand Factor aa 1-250. The exact sequence is proprietary.
Database link: Q8CIZ8
- WB: Human and mouse plasma and liver lysates; Hepa1-6 whole cell lysate.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.01% Sodium azide
Constituents: PBS, 40% Glycerol, 0.05% BSA
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab189500 in the following tested applications.
|WB||1/1000. Detects a band of approximately 90,309 kDa (predicted molecular weight: 309 kDa).|
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.
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.
modificationsAll 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
- Coagulation factor VIII antibody
- Coagulation factor VIII VWF antibody
- F8VWF antibody
All lanes : Anti-Von Willebrand Factor antibody [EPR18569-105] (ab189500) at 1/1000 dilution
Lane 1 : Human liver lysate
Lane 2 : Human plasma
Lane 3 : Hep1-6 (mouse hepatoma epithelial cell line) whole cell lysate
Lane 4 : Mouse plasma
Lane 5 : Mouse liver lysate
Lysates/proteins at 20 µg per lane.
All lanes : Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/100000 dilution
Developed using the ECL technique.
Predicted band size: 309 kDa
Observed band size: 309,90 kDa why is the actual band size different from the predicted?
Exposure times: Lanes 1,2: 3 minutes; Lane 3: 1 minute; Lane 4,5: 15 seconds.
Blocking/Dilution buffer: 5% NFDM/TBST.
The molecular masses are consistent with what has been described in the literature (PMID: 8204880; PMID: 18344423).
ab189500 has not yet been referenced specifically in any publications.