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merck millipore,默克密理博,MAB038,Anti-Factor VIII Antibody, 83 kDa light chain
产品名称:Anti-Factor VIII Antibody, 83 kDa light chain
产品型号:MAB038
Anti-Factor VIII Antibody, 83 kDa light chain is an antibody against Factor VIII for use in ELISA & WB.
merck millipore,默克密理博,MAB038,Anti-Factor VIII Antibody, 83 kDa light chain
- 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 WB, ELISA M Ascites Monoclonal Antibody 描述 产品目录编号 MAB038 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Factor VIII Antibody, 83 kDa light chain Alternate Names - AHF
- Coagulant Component
- Antihemophilic Factor
产品信息 格式 Ascites 演示 Liquid. Contains no preservative. 应用 应用 Anti-Factor VIII Antibody, 83 kDa light chain is an antibody against Factor VIII for use in ELISA & WB. 主要应用 - Western Blotting
- ELISA
应用说明 ELISA (indirect): 1:16,000.
Western blotting: Factor VIII makes up only about 1% of the ciruculating FactorVIII/vWF complex with the bulk of the complex being vWF (99% by mass, see Blood 58:1-13, 1981). Thus it is recommended that the FactorVII/vWF be precipitated out of plasma prior to detection of Factor VII with MAB038. Also, when blotting plasma preparations, background, non-specific bands, caused by the serum proteins are common. More complex blocking solutions or higher salt concentrations can help remove non-specific bands. Rotblat, et al (1985) Biochemistry 24(16):4294-4300. Should be consulted for additional tips with regard to detecting Factor VII via westerns.
Optimal working dilutions must be determined by the end user.生物信息 免疫原品种 Purified Human Factor VIII. 表位 83 kDa light chain 宿主 Mouse 特异性 By Western blot the antibody recognizes the 83 kDa light chain of human Factor VIII. No cross reactivity with von Willebrand factor. 同种型 IgG2a 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
- F8C
- HEMA
- AHF
- DXS1253E
- FVIII
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 Maintain at -20°C in undiluted aliquots for up to 12 months from date of receipt. Avoid repeated freeze/thaw cycles. 包装信息 数量 500 µL
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