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merck millipore,默克密理博,MAB3440,Anti-Factor VIII Antibody, 83 kDa light chain, clone 24-2-C7
产品名称：Anti-Factor VIII Antibody, 83 kDa light chain, clone 24-2-C7
产品型号：MAB3440
This Anti-Factor VIII Antibody, 83 kDa light chain, clone 24-2-C7 is validated for use in ELISA, IF, IH(P) for the detection of Factor VIII.
merck millipore,默克密理博,MAB3440,Anti-Factor VIII Antibody, 83 kDa light chain, clone 24-2-C7
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H ELISA M Purified Monoclonal Antibody 描述 产品目录编号 MAB3440 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Factor VIII Antibody, 83 kDa light chain, clone 24-2-C7 Alternate Names - AHF
- Coagulant Component
- Antihemophilic Factor
产品信息 格式 Purified 演示 The antibody is supplied in 0.5ml phosphate buffered saline, pH 7.4 containing 0.1% sodium azide and 50% (v/v) glycerol. The characteristics of each lot are tested by electrophoresis and specific ELISA.
BIOCHEMISTRY: pI = 5.8应用 应用 This Anti-Factor VIII Antibody, 83 kDa light chain, clone 24-2-C7 is validated for use in ELISA, IF, IH(P) for the detection of Factor VIII. 主要应用 - ELISA
应用说明 This antibody is an instant inhibiter of coagulation. In conjunction with von Willebrand Factor monoclonal antibody (Catalog Number MAB3442), it can be used to distinguish haemophilia from von Willebrand's disease. Factor VIIICAg antigen in plasma can be quantified using a sandwich radioimmunoassay, with this antibody coated onto the solid phase (Fancis, 1985, 1986; Exner, 1984). The antigen may be visualised in the cytoplasm of vascular endothelial cells by either immuno-fluorescence or immunoperoxidase staining on formalin-fixed, paraffin-embedded and frozen tissue sections.
SUGGESTED USAGE
Indirect immunoperoxidase staining - the final dilution will depend on the assay conditions and detection system employed. However, a dilution of at least 1:25 will be applicable to most commonly used systems.生物信息 免疫原品种 purified Factor VIII complex 表位 83 kDa light chain 克隆 24-2-C7 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 特异性 This antibody reacts with the coagulant component of the Factor VIII complex which is defective or absent in human patients with classical haemophilia (Haemophilia A). Clone 24-2-C7 reacts with the 83 kDa light chain of Factor VIII protein and prolongs clotting time in coagulant assays in vitro (Francis, 1985). 同种型 IgG1 品种反应性 Human 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_019863.2
- NM_000132.2
Entrez基因汇总 This gene encodes coagulation factor VIII, which participates in the intrinsic pathway of blood coagulation; factor VIII is a cofactor for factor IXa which, in the presence of Ca+2 and phospholipids, converts factor X to the activated form Xa. This gene produces two alternatively spliced transcripts. Transcript variant 1 encodes a large glycoprotein, isoform a, which circulates in plasma and associates with von Willebrand factor in a noncovalent complex. This protein undergoes multiple cleavage events. Transcript variant 2 encodes a putative small protein, isoform b, which consists primarily of the phospholipid binding domain of factor VIIIc. This binding domain is essential for coagulant activity. Defects in this gene results in hemophilia A, a common recessive X-linked coagulation disorder. 基因符号 - F8B
- F8
- OTTHUMP00000061446
- FVIII
- F8C
- HEMA
- AHF
- DXS1253E
UniProt编号 - P00451
UniProt汇总 FUNCTION: SwissProt: P00451 # 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.
SIZE: 2351 amino acids; 267009 Da
SUBUNIT: Interacts with VWF. VWF binding is essential for the stabilization of F8 in circulation.
SUBCELLULAR LOCATION: Secreted, extracellular space.
DOMAIN: SwissProt: P00451 Domain F5/8 type C 2 is responsible for phospholipid- binding and essential for factor VIII activity.
DISEASE: SwissProt: P00451 # Defects in F8 are the cause of hemophilia A (HEMA) [MIM:306700]. HEMA is a common recessive X-linked coagulation disorder. The frequency of hemophilia A is 1-2 in 10,000 male births in all ethnic groups. About 50% of patients have severe hemophilia A with F8C activity less than 1% of normal; they have frequent spontaneous bleeding into joints, muscles and internal organs. Moderately severe hemophilia A occurs in about 10% of patients; F8C activity is 2-5% of normal, and there is bleeding after minor trauma. Mild hemophilia A, which occurs in 30-40% of patients, is associated with F8C activity of 5-30% and bleeding occurs only after significant trauma or surgery. Of particular interest for the understanding of the function of F8C is the category of CRM (cross-reacting material) positive patients (approximately 5%) that have considerable amount of F8C in their plasma (at least 30% of normal), but the protein is nonfunctional; i.e., the F8C 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.
SIMILARITY: 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.产品使用声明 使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Store at 2° to 8°C, for up to 6 months. For prolonged periods, store at -15° to -20°C. At this temperature the glycerol solution will not freeze.
WARNING: The monoclonal reagent solution contains 0.1% sodium azide as a preservative. Due to potential hazards arising from the build up of this material in pipes, spent reagent should be disposed of with liberal volumes of water.包装信息 数量 500 µL
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