Cat#:PA-2949F;Product Name:Sheep Anti-Human Factor VIII Antibody, Biotin-Conjugated;Synonym:antihemophilic globulin; Factor VIII:C; coagulation factor VIII, procoagulant component; F8B; DXS1253E; OTTHUMP00000024269; FVIII; OTTHUMP00000196174; HEMA; coagulation factor VIII; Antihemophilic factor; coagulation factor VIIIc; AHF; factor VIII F8B; F8C; Procoagulant component; F8; Factor VIIIF8B; hemophilia A; Short name=AHF; Factor VIIIa heavy chain, 200 kDa isoform; Factor VIIIa heavy chain, 92 kDa isoform; Factor VIII B chain; Factor VIIIa light chain; anti-hemophilic factor (AHF); Factor VIII; F.VIII;Background: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.;Description:Sheep Anti-Human Factor VIII Polyclonal Antibody, Biotin-Conjugated;Host Species:Sheep;Species Reactivity:human;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;
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.
Description:
Sheep Anti-Human Factor VIII Polyclonal Antibody, Biotin-Conjugated
Host Species:
Sheep
Species Reactivity:
human
Isotype:
IgG
Application:
IEP, ELISA
Usage:
For Lab Research Use Only
Storage:
Store antibody products at 2-8°C. For long term storage, aliquot and freeze at -20°C. Avoid repeated freeze/thaw cycles
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