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Anti-Factor VIII, clone GMA-012

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号:05-871

牌:Millipore 密理博

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Replacement Information

重要规格表

Species Reactivity Key Applications Host Format Antibody Type
H ELISA, WB M Purified Monoclonal Antibody
Description
Catalogue Number 05-871
Brand Family Upstate
Trade Name
  • Upstate
Description Anti-Factor VIII Antibody, clone GMA-012
References
Product Information
Format Purified
Applications
Application Detect Factor VIII using this Anti-Factor VIII Antibody, clone GMA-012 validated for use in ELISA & WB.
Key Applications
  • ELISA
  • Western Blotting
Biological Information
Immunogen Purified human Factor VIII
Clone clone GMA-012
Host Mouse
Specificity Factor VIII
Isotype IgG1
Species Reactivity
  • Human
Antibody Type Monoclonal Antibody
Entrez Gene Number
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.
Gene Symbol
  • F8
  • F8C
  • OTTHUMP00000061446
  • FVIII
  • HEMA
  • AHF
  • DXS1253E
  • F8B
Purification Method Protein G purified
UniProt Number
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.
Molecular Weight Mr 280kDa
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Quality Assurance routinely 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 Conditions stable 2 years at -20°C from date of shipment
Packaging Information
Material Size 100 µg
Transport Information
Supplemental Information
Specifications

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