Anti-Factor VIII抗体[27.4] (ab41188)
Key features and details
- Mouse monoclonal [27.4] to Factor VIII
- Suitable for: ELISA, WB
- Reacts with: Human
- Isotype: IgG2a
概述
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产品名称
Anti-Factor VIII抗体[27.4]
参阅全部 Factor VIII 一抗 -
描述
小鼠单克隆抗体[27.4] to Factor VIII -
宿主
Mouse -
特异性
ab41188 does not cross react with the von Willebrand factor. -
经测试应用
适用于: ELISA, WBmore details -
种属反应性
与反应: Human -
免疫原
Full length native protein (purified) corresponding to Human Factor VIII (N terminal).
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表位
ab41188 recognises an epitope in the N-terminal region of the 83kD light chain of Factor VIII. -
常规说明
This product was changed from ascites to tissue culture supernatant on 19/12/2018. Please note that the dilutions may need to be adjusted accordingly. If you have any questions please do not hesitate to contact our scientific support team.
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles. -
存储溶液
pH: 7.40
Constituent: 99% PBS -
Concentration information loading...
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纯度
Protein G purified -
纯化说明
Purified from TCS -
克隆
单克隆 -
克隆编号
27.4 -
同种型
IgG2a -
研究领域
相关产品
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Compatible Secondaries
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab41188于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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ELISA | ||
WB |
说明 |
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WB: Use at an assay dependent dilution. Predicted molecular weight: 267 kDa.
Dilute in PBS or medium which is identical to that used in the assay system.
Can inhibit Factor VIII activity in coagulation assays.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
靶标
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功能
Factor VIII, along with calcium and phospholipid, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa. -
疾病相关
Defects in F8 are the cause of hemophilia A (HEMA) [MIM:306700]. A disorder of blood coagulation characterized by a permanent tendency to hemorrhage. About 50% of patients have severe hemophilia resulting in frequent spontaneous bleeding into joints, muscles and internal organs. Less severe forms are characterized by bleeding after trauma or surgery. Note=Of particular interest for the understanding of the function of F8 is the category of CRM (cross-reacting material) positive patients (approximately 5%) that have considerable amount of F8 in their plasma (at least 30% of normal), but the protein is non-functional; i.e., the F8 activity is much less than the plasma protein level. CRM-reduced is another category of patients in which the F8C antigen and activity are reduced to approximately the same level. Most mutations are CRM negative, and probably affect the folding and stability of the protein. -
序列相似性
Belongs to the multicopper oxidase family.
Contains 3 F5/8 type A domains.
Contains 2 F5/8 type C domains.
Contains 6 plastocyanin-like domains. -
结构域
Domain F5/8 type C 2 is responsible for phospholipid-binding and essential for factor VIII activity. -
翻译后修饰
Sulfation on Tyr-1699 is essential for binding vWF. -
细胞定位
Secreted > extracellular space. - Information by UniProt
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数据库链接
- Entrez Gene: 2157 Human
- Omim: 300841 Human
- SwissProt: P00451 Human
- Unigene: 654450 Human
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别名
- AHF antibody
- Antihemophilic factor antibody
- Coagulation factor VIII antibody
see all
数据表及文件
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SDS download
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Datasheet download
文献 (4)
ab41188 被引用在 4 文献中.
- Gage BK et al. Therapeutic correction of hemophilia A by transplantation of hPSC-derived liver sinusoidal endothelial cell progenitors. Cell Rep 39:110621 (2022). PubMed: 35385743
- Gage BK et al. Generation of Functional Liver Sinusoidal Endothelial Cells from Human Pluripotent Stem-Cell-Derived Venous Angioblasts. Cell Stem Cell 27:254-269.e9 (2020). IHC-P ; Human . PubMed: 32640183
- Jankowska KI et al. Further Evidence That MicroRNAs Can Play a Role in Hemophilia A Disease Manifestation: F8 Gene Downregulation by miR-19b-3p and miR-186-5p. Front Cell Dev Biol 8:669 (2020). PubMed: 32850803
- Pandey GS et al. Detection of intracellular Factor VIII protein in peripheral blood mononuclear cells by flow cytometry. Biomed Res Int 2013:793502 (2013). PubMed: 23555096