Key features and details
- Mouse monoclonal [F8/86] to Von Willebrand Factor
- Suitable for: IHC-P, IHC-Fr
- Reacts with: Human
- Isotype: IgG1
保存方法Shipped at 4°C. Store at +4°C short term (1-2 weeks). Store at -20°C or -80°C. Avoid freeze / thaw cycle.
バッファーPreservative: 0.05% Sodium azide
Constituents: Tissue culture supernatant, 1% BSA
Concentration information loading...
精製度Tissue culture supernatant
Our Abpromise guarantee covers the use of ab778 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/15 - 1/30. Perform enzymatic antigen retrieval before commencing with IHC staining protocol.|
|IHC-Fr||1/15 - 1/30. ABC method. We suggest an incubation period of 60 minutes at room temperature.|
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.
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
- Coagulation factor VIII antibody
- Coagulation factor VIII VWF antibody
- F8VWF antibody
ab778 は 9 報の論文で使用されています。
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