货号：05-871

品牌：Merck Millipore

规格：100Ug

目录价：￥6138.00

市场价格：￥5217.30

会员价格：￥4910.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Factor VIII Antibody, clone GMA-012 | 05-871 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Upstate (Millipore) View All» Specificity: Factor VIII View All» Molecular Weight: Mr 280kDa View All» Immunogen: Purified human Factor VIII View All» Isotype: IgG1 View All» Species Reactivity: Human View All» Quality Assurance: routinely evaluated by immunoblot on human Factor VIII View All» Purification Method: DEAE purified View All» Storage Conditions: stable 2 years at -20°C from date of shipment View All» UniProt Number: P00451 View All» Entrez Gene Number: NM_000132 / NM_019863 View All» Gene Symbol:

• F8

• F8C

• OTTHUMP00000061446

• FVIII

• HEMA

• AHF

• DXS1253E

• F8B
  View All» Usage Statement: 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. View All» Key Applications:
  • ELISA

  • Western Blotting
    View All» Entrez Gene Summary: 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. View All» UniProt Summary: 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. View All» Brand Family: Upstate View All» Product Name: Anti-Factor VIII Antibody, clone GMA-012 | 05-871 View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 µg View All» Format: Purified View All» Host: Mouse View All»
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