Native Human Von Willebrand Factor protein (ab88533)
Key features and details
- Expression system: Native
- Purity: > 95% SDS-PAGE
- Suitable for: SDS-PAGE
製品の詳細
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製品名
Native Human Von Willebrand Factor protein
Von Willebrand Factor タンパク質・ペプチド 製品一覧 -
精製度
> 95 % SDS-PAGE. -
発現系
Native -
アクセッション番号
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タンパク質長
Full length protein -
Animal free
No -
由来
Native -
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生物種
Human -
配列の追加情報
Amino acid sequence is not determined.
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関連製品
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Related Products
特性
Our Abpromise guarantee covers the use of ab88533 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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アプリケーション
SDS-PAGE
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製品の状態
Liquid -
Concentration information loading...
前処理および保存
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保存方法および安定性
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 6.80
Constituents: 0.735% Sodium citrate, 0.75% Glycine, 0.58% Sodium chloride
The percentages are based on the constituent weight/volume
関連情報
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別名
- Coagulation factor VIII
- Coagulation factor VIII VWF
- F8VWF
see all -
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. -
組織特異性
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
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プロトコール
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (3)
ab88533 は 3 報の論文で使用されています。
- Bao J et al. Von Willebrand Factor Facilitates Intravascular Dissemination of Microsporidia Encephalitozoon hellem. Front Cell Infect Microbiol 11:694957 (2021). PubMed: 34095003
- Jalan AA et al. Chain alignment of collagen I deciphered using computationally designed heterotrimers. Nat Chem Biol 16:423-429 (2020). PubMed: 31907373
- Howes JM et al. Cleavage by MMP-13 renders VWF unable to bind to collagen but increases its platelet reactivity. J Thromb Haemost 18:942-954 (2020). PubMed: 31894636