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

05-871
100 µg  
Purchase on Sigma-Aldrich

特卖

概述

Replacement Information

重要规格表

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
Quality LevelMQ100
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
Global Trade Item Number
产品目录编号 GTIN
05-871 04053252327599

Documentation

Anti-Factor VIII Antibody, clone GMA-012 MSDS

职位

物料安全数据表 (MSDS) 

Anti-Factor VIII Antibody, clone GMA-012 分析证书

标题批号
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 - 3204811 3204811
Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) 3018219
Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) 3018220
Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) 2971807
Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) 3025865
Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) 3048494
Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) - 2047262 2047262
Anti-Factor VIII, clone GMA-012 (mouse monoclonal IgG1) - 2074053 2074053

参考

参考概述公共医疗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
Coagulation factor VIII: structure and stability.
Wang, Wei, et al.
International journal of pharmaceutics, 259: 1-15 (2003)  2003

显示摘要
12787631 12787631
Blood coagulation factor VIII: An overview.
Bhopale, G M and Nanda, R K
J. Biosci., 28: 783-9 (2003)  2003

14660878 14660878