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Anti -Factor VIIIc (Antihaemophilic Factor)
Anti -Factor VIIIc (Antihaemophilic Factor)
Pricing
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| Clone |
Host |
Grade |
Applications |
| Polyclonal |
Sheep |
Purified |
E N |
|
| Factor VIII (antihemophilic globulin and Factor VIII:C) is a large glycoprotein (320kD) that circulates in plasma at ~200ng/ml. Synthesized in the liver, the majority of Factor VIII is cleaved during expression, resulting in a heterogeneous mixture of partially cleaved forms of F.VIII ranging in size from 200-280kD. The F.VIII is stabilized by association with von Willebrand Factor to form a F.VIII-vWF complex required for the normal survival of F.VIII in vivo (t1/2 of 8-12 hours). F.VIII is a pro-cofactor that is activated through limited proteolysis by thrombin. In this process F.VIIIa dissociates from vWF to combine with activated Factor IX, calcium and a phospholipid surface where it is an essential cofactor in the assembly of the Factor X activator complex. Once dissociated from vWF, F.VIIIa is susceptible to inactivation by activated Protein C and by non-enzymatic decay. | | | Hemophilia A is a congenital bleeding disorder resulting from an X-chromosome-linked deficiency of F.VIII. The severity of the deficiency generally correlates with the severity of the disease. Some Hemophiliacs (~10%) produce a F.VIII protein that is partially or totally inactive. The production of neutralizing antibodies to F.VIII also occurs in 5-20% of Hemophiliacs (1-3). | | | Catalog # | F0016-30 | | Application | Suitable for use in ELISA, Immunoelectrophoresis and Neutralization. Other applications not tested. | | Recommended Dilution | Neutralization: 1175 Bethesda Units/ml IgG against normal plasma (4). One Bethesda unit/ml is defined as the amount of inhibitor that resulted in 50% residual F.VIII activity after 2 hours at 37ºC. | | Optimal dilutions to be determined by the researcher. | | Storage and Stability | May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. | | CAS Number | n/a | | Clone Type | Polyclonal | | Isotype | IgG | | Host | Sheep | | Source | Human | | Concentration | ~10mg/ml | | Form | Supplied as a liquid in 10mM HEPES, pH 7.4, 0.15M sodium chloride, 50% glycerol. | | Purity | Purified by caprylic acid and ammonium sulfate precipitations, and solid -phase adsorption to remove unwanted crossreactivity. | | Immunogen | Human Factor VIIIC (F.VIII) purified from concentrate | | Specificity | Recognizes human F.VIII as demonstrated by immunodiffusion and ELISA. No reactivity was observed with von Willebrand Factor. | | | Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological. |
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