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merck millipore,默克密理博,05-871,Anti-Factor VIII Antibody, clone GMA-012
产品名称：Anti-Factor VIII Antibody, clone GMA-012
产品型号：05-871
Detect Factor VIII using this Anti-Factor VIII Antibody, clone GMA-012 validated for use in ELISA & WB.
merck millipore,默克密理博,05-871,Anti-Factor VIII Antibody, clone GMA-012
- 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 Purified Monoclonal Antibody 描述 产品目录编号 05-871 品牌系列 Upstate
商名 - Upstate
描述 Anti-Factor VIII Antibody, clone GMA-012 产品信息 格式 Purified 应用 应用 Detect Factor VIII using this Anti-Factor VIII Antibody, clone GMA-012 validated for use in ELISA & WB. 主要应用 - Western Blotting
- ELISA
生物信息 免疫原品种 Purified human Factor VIII 克隆 clone GMA-012 宿主 Mouse 特异性 Factor VIII 同种型 IgG1 品种反应性 Human 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_000132 / NM_019863
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
- F8C
- OTTHUMP00000061446
- FVIII
- HEMA
- AHF
- DXS1253E
纯化方法 DEAE purified 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 质量保证 routinely evaluated by immunoblot on human Factor VIII 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 stable 2 years at -20°C from date of shipment 包装信息 数量 100 µg
上一件merck millipore产品：merck millipore,默克密理博,PIHP01250,Millicell 插入式细胞培养皿, 12 mm, 聚碳酸酯, 0.4 µm
下一件merck millipore产品：merck millipore,默克密理博,23-024M,p27 Protein complex, activated, 250 µg
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