05-871 | Anti-Factor VIII Antibody, clone GMA-012

05-871
100 µg  
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      Overview

      Replacement Information

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HELISA, WBMPurifiedMonoclonal Antibody
      Description
      Catalogue Number05-871
      Brand Family Upstate
      Trade Name
      • Upstate
      DescriptionAnti-Factor VIII Antibody, clone GMA-012
      References
      Product Information
      FormatPurified
      Applications
      ApplicationDetect Factor VIII using this Anti-Factor VIII Antibody, clone GMA-012 validated for use in ELISA & WB.
      Key Applications
      • ELISA
      • Western Blotting
      Biological Information
      ImmunogenPurified human Factor VIII
      Cloneclone GMA-012
      HostMouse
      SpecificityFactor VIII
      IsotypeIgG1
      Species Reactivity
      • Human
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThis 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.
      Gene Symbol
      • F8
      • F8C
      • OTTHUMP00000061446
      • FVIII
      • HEMA
      • AHF
      • DXS1253E
      • F8B
      Purification MethodProtein G purified
      UniProt Number
      UniProt SummaryFUNCTION: 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.
      Molecular WeightMr 280kDa
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality Assuranceroutinely evaluated by immunoblot on human Factor VIII
      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.
      Storage and Shipping Information
      Storage Conditionsstable 2 years at -20°C from date of shipment
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      SDS

      Title

      Safety Data Sheet (SDS) 

      Certificates of Analysis

      TitleLot Number
      Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) - 2154081 2154081
      Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) - 2367792 2367792
      Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) 2971807
      Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) - 2047262 2047262
      Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) - 2074053 2074053
      Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) - 2095342 2095342
      Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) - 2102018 2102018
      Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) - 2189722 2189722
      Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) - 2224419 2224419
      Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) - 2299529 2299529

      References

      Reference overviewPub Med ID
      Recombinant factor VIIa: review of efficacy, dosing regimens and safety in patients with congenital and acquired factor VIII or IX inhibitors.
      Abshire, T and Kenet, G
      J. Thromb. Haemost., 2: 899-909 (2004)  2004

      15140125 15140125
      Blood coagulation factor VIII: An overview.
      Bhopale, G M and Nanda, R K
      J. Biosci., 28: 783-9 (2003)  2003

      14660878 14660878
      Coagulation factor VIII: structure and stability.
      Wang, Wei, et al.
      International journal of pharmaceutics, 259: 1-15 (2003)  2003

      Show Abstract
      12787631 12787631

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      Categories

      Life Science Research > Antibodies and Assays > Primary Antibodies