Key features and details
- Mouse monoclonal [27.4] to Factor VIII
- Suitable for: ELISA, WB
- Reacts with: Human
- Isotype: IgG2a
参阅全部 Factor VIII 一抗
描述小鼠单克隆抗体[27.4] to Factor VIII
特异性ab41188 does not cross react with the von Willebrand factor.
经测试应用适用于: ELISA, WBmore details
Full length native protein (purified) corresponding to Human Factor VIII (N terminal).
表位ab41188 recognises an epitope in the N-terminal region of the 83kD light chain of Factor VIII.
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.
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存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Constituent: 99% PBS
Concentration information loading...
纯度Protein G purified
纯化说明Purified from TCS
The Abpromise guarantee
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.
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
- AHF antibody
- Antihemophilic factor antibody
- Coagulation factor VIII antibody
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