| 别名 | F8VWF |
| 基因名称 | VWF |
| 蛋白名称 | von Willebrand factor (vWF) [Cleaved into: von Willebrand antigen 2 (von Willebrand antigen II)] |
| 推荐应用 | |
| 反应物种 | Human,Mouse,Rat |
| 存储缓冲液 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Human Gene Link | http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=7450 |
| Human Swissprot No. | P04275 |
| Human Swissprot Link | https://www.uniprot.org/uniprotkb/P04275/entry |
| Mouse Swissprot No. | Q8CIZ8 |
| Mouse Swissprot Link | http://www.uniprot.org/uniprot/Q8CIZ8 |
| Rat Swissprot No. | Q62935 |
| Rat Swissprot Link | http://www.uniprot.org/uniprot/O54941Q62935 |
| 稀释度 | IHC-P 1:1000-1:4000, WB 1:2000-1:10000, IF-P/IF-F/IF-ICC 1:200-1:1000, ELISA 1:5000-1:20000, IP 1:50-1:200 |
| 参考分子量 | 280kDa |
| 预测分子量 | 309kDa |
| 运输及保存条件 | Biological ice bag transportation. Store at -20°C for at least 12 months(Do not lower than -25°C). Avoid freeze/thaw cycles |
| 宿主 | Rabbit |
| 同种型 | Monoclonal, rabbit, IgG, Kappa |
| 背景介绍 | This gene encodes a glycoprotein involved in hemostasis. The encoded preproprotein is proteolytically processed following assembly into large multimeric complexes. These complexes function in the adhesion of platelets to sites of vascular injury and the transport of various proteins in the blood. Mutations in this gene result in von Willebrand disease, an inherited bleeding disorder. An unprocessed pseudogene has been found on chromosome 22. [provided by RefSeq, Oct 2015], |
| 信号通路 | Focal adhesion;ECM-receptor interaction;Complement and coagulation cascades; |
| 功能 | disease:Defects in VWF are associated with various forms of von Willebrand disease (VWD) [MIM:193400, 277480]. VWD is characterized by frequent bleeding (gingival, minor skin quantitative lacerations, menorrhagia, etc.). Type I VWD is associated with a deficiency of VWF; type II by normal to decreased plasma level of VWF; type III by a virtual absence of VWF. There are subtypes (A to H) of type II VWD; for example: type IIA is characterized by the absence of VWF high molecular weight multimers in plasma.,domain:The von Willebrand antigen 2 is required for multimerization of vWF and for its targeting to storage granules.,function: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.,online information:von Willebrand factor (vWF) mutation db,online information:Von Willebrand factor entry,PTM:All cysteine residues are involved in intrachain or interchain disulfide bonds.,similarity:Contains 1 CTCK (C-terminal cystine knot-like) domain.,similarity:Contains 3 VWFA domains.,similarity:Contains 3 VWFC domains.,similarity:Contains 4 TIL (trypsin inhibitory-like) domains.,similarity:Contains 4 VWFD domains.,subcellular location:Localized to storage granules.,subunit:Multimeric. Interacts with F8.,tissue specificity:Plasma., |
| 纯化 | Protein A |
| Clonality | Monoclonal |
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