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Factor VIII

Order number Description Quantity Delivery time CE
SAF8C-APBIO Sheep anti-human FVIII:C, Aff. Pur. Biotinylated 0,1 mg 7-10 days

Sheep anti-human FVIII:C, Aff. Pur. Biotinylated

Factor VIII (formerly referred to as antihemophilic globulin and Factor VIII:C) is a large glycoprotein (320 kDa) that circulates in plasma at approximately 200 ng/mL. Synthesized in the liver, the majority of Factor VIII is cleaved during expression, resulting in a heterogeneous mixture of partially cleaved forms of FVIII ranging in size from 200-280 kDa. The FVIII is
stabilized by association with von Willebrand Factor to form a FVIII-vWF complex required for the normal survival of FVIII in vivo (t1/2 of 8-12 hours).
FVIII is a pro-cofactor that is activated through limited proteolysis by thrombin. In this process FVIIIa 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, FVIIIa 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 FVIII. The severity of the deficiency generally correlates with the severity of the disease. Some Hemophiliacs (~10%) produce a FVIII protein that is partially or totally inactive. The production of neutralizing antibodies to FVIII also occurs in 5-20% of Hemophiliacs.



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