Factor VIII

Factor VIII (formerly referred to as antihemophilic globulin and Factor VIIIc) is a large glycoprotein (320kD) that circulates in plasma at approximately 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 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 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. F0016-10A is an instant inhibitor of coagulation. In conjunction with von Willebrand Factor monoclonal antibody (Catalog Number V2700-08), it can be used to distinguish haemophilia from von Willebrand's disease. Factor VIIICAg antigen in plasma can be quantified using a sandwich radioimmunoassay, with this antibody coated onto the solid phase (Francis, 1985, 1986; Exner, 1984). The antigen may be visualised in the cytoplasm of vascular endothelial cells by either immuno-fluorescence or immunoperoxidase staining on formalin-fixed, paraffin-embedded and frozen tissue sections.\n\nApplications: \nSuitable for use in ELISA, Immunofluorescence and Immunohistochemistry. Other applications not tested. \n\nRecommended Dilution:\nImmunohistochemistry (Formalin fixed, paraffin and frozen): 1:25\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

F0016-10A

Size

500ul

Applications

ELISA, IF, IHC

Hosts

Mouse

Reactivities

Hum

Form

Supplied as a liquid in PBS, pH 7.4, 0.1% sodium azide, 50% glycerol.

P Type

Mab

Purity

Purified

Isotype

IgG1

References

1. Francis S.E. et al., Pathology 17: 579-585 (1985). 2.Francis S.E. et al., Abstracts from the 21st International Congress of Haematology. Sydney (1986). 3. Exner T. et al., Pathology 16: 356 (1984).

Additional Info

Recognizes the coagulant component of the Factor VIII complex which is defective or absent in human patients with classical haemophilia (Haemophilia A). The characteristics of each lot are tested by electrophoresis and specific ELISA. Epitope: 83kD light chain.