FA8A (light chain; Cleaved-Glu1668) rabbit pAb
Produktgrößen
50UL
£ POA
ES19999-50UL
100UL
£ POA
ES19999-100UL
Über dieses Produkt
- SKU:
- ES19999
- Zusätzliche Namen:
- Coagulation factor VIII (Antihemophilic factor;AHF;Procoagulant component) [Cleaved into: Factor VIIIa heavy chain; 200 kDa isoform; Factor VIIIa heavy chain; 92 kDa isoform; Factor VIII B chain; Factor VIIIa light chain]
- Anwendung:
- Western Blot, ELISA
- Klonalität:
- Polyclonal
- Konzentration:
- 1 mg/ml
- Weitere Details:
- disease: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 non-functional; 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.;domain:Domain F5/8 type C 2 is responsible for phospholipid-binding and essential for factor VIII activity.;function: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.;mass spectrometry:Disulfated PubMed:10368977;mass spectrometry:Monosulfated PubMed:10368977;mass spectrometry:Nonsulfated PubMed:10368977;mass spectrometry:Sulfated PubMed:10368977;mass spectrometry:Trisulfated PubMed:10368977;online information:Factor VIII entry;online information:Factor VIII mutation db;pharmaceutical:Available under the names Kogenate (Bayer) and Recombinate (Baxter and American Home Products). Used to treat hemophilia A.;PTM:Sulfation on Tyr-1699 is essential for binding vWF.;similarity:Belongs to the multicopper oxidase family.;similarity:Contains 1 F5/8 type C domain.;similarity:Contains 2 F5/8 type C domains.;similarity:Contains 3 F5/8 type A domains.;similarity:Contains 6 plastocyanin-like domains.;subunit:Interacts with vWF. vWF binding is essential for the stabilization of F8 in circulation.;
- Immunogen:
- Synthesized peptide derived from human FA8A (light chain; Cleaved-Glu1668)
- Isotyp:
- IgG
- Reaktivitäten:
- Human, Mouse
- Versandbedingungen:
- Blue Ice
- Quelle:
- Rabbit
- Lagerbedingungen:
- -20[o]C
- Hersteller:
- ELK Biotechnology
- Typ:
- Antibodies: Polyclonal Antibody