Key features and details
- Rabbit polyclonal to Von Willebrand Factor
- Suitable for: IHC-P
- Reacts with: Human
- Isotype: IgG
- 倫理基準に準拠 - アニマル・フリーの生産
製品名Anti-Von Willebrand Factor antibody
Von Willebrand Factor 一次抗体 製品一覧
製品の詳細Rabbit polyclonal to Von Willebrand Factor
アプリケーション適用あり: IHC-Pmore details
交差が予測される動物種: Rat, Sheep, Horse, Guinea pig, Cow, Dog非交差種: Chicken
Full length native protein (purified) corresponding to Human Von Willebrand Factor. Purified from plasma.
- IHC-P: Human kidney tissue.
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保存方法Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Preservative: 0.097% Sodium azide
Concentration information loading...
特記事項（精製）Whole antiserum is fractionated and then further purified by ion exchange chromatography to provide the IgG fraction of antiserum. This fraction is essentially free of other rabbit serum proteins.
Our Abpromise guarantee covers the use of ab6994 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/200 - 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.|
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
ab6994 は 308 報の論文で使用されています。
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