Cat#:PA-2951F;Product Name:Sheep Anti-Canine Factor VIII Antibody, HRP-Conjugated;Synonym:antihemophilic globulin; Factor VIII:C; hemophilia A; F8; coagulation factor VIII, procoagulant component; coagulation factor VIII; factor VIII; coagulation co-factor; procoagulant component; coagulation factor VIII, procoagulant component (hemophilia A); anti-hemophilic factor (AHF); FVIII;Background:Factor VIII is a large glycoprotein (320 kDa) synthesized in the liver. The majority of Factor VIII is cleaved during expression, resulting in a mixture of partially cleaved forms ranging in size from 200-280 kDa. The FVIII is stabilized in circulation through non-covalent association with von Willebrand Factor. The concentration of FVIII in normal human plasma is typically 200 ng/mL. In canine plasma, the FVIII activity is 5-7 fold higher relative to human plasma. 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. Hemophilia A is a congenital bleeding disorder resulting from an X-chromosome-linked deficiency of FVIII, occurring with a frequency of 1 in 4000 males. The defect can be caused by any one of hundreds of reported mutations but are most commonly due to inversions within intron 22 of the FVIII gene. Hemophilia A has also been reported in a variety of species including dog and mouse, with a clinical phenotype very similar to human. The genetic defect in one case of canine Hemophilia-A has been shown to also be due to a gene inversion similar to the human defect, possibly indicating a common instability of the FVIII gene in humans and dogs.;Description:Sheep Anti-Canine Factor VIII Polyclonal Antibody, HRP-Conjugated;Host Species:Sheep;Species Reactivity:canine;Isotype:IgG;Application:IEP, ELISA;Storage:Store antibody products at 2-8°C. For long term storage, aliquot and freeze at -20°C. Avoid repeated freeze/thaw cycles;Usage:For Lab Research Use Only;