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.
Application:Suitable for use in ELISA, Immunoelectrophoresis and Neutralization.
Other applications not tested.
Recommended Dilutions:Neutralization: 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 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：1mg
Host：sheep
Source Antibody：human
Grade：Purified
Purity：Purified by caprylic acid and ammonium sulfate precipitations, and solid-phase adsorption to remove unwanted crossreactivity.
Form：Supplied as a liquid in 10mM HEPES, pH 7.4, 0.15M sodium chloride, 50% glycerol.
Specificity：Recognizes human F.VIII as demonstrated by immunodiffusion and ELISA. No reactivity was observed with von Willebrand Factor.
Isotype：IgG
Calc Applications Abbrev：E N
Calc Crossreactivity：Hu
Immunogen：Human Factor VIIIC (F.VIII) purified from concentrate
