F8 | Coagulation factor VIII
IMS01-015-308 | Clonality: Polyclonal | Host: Hen | Reactivity: Human
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purified full length native human Coagulation Factor VIII-VWF-F VIII:c complex
IgG concentration is 22.2 µg/µl
Immunohistochemistry: antibody was tested on bonin-fixed rat blood vessel.
The factor VIII complex, with a molecular weight in excess of 1 million, has 2 components: (1) factor VIII P00451 (molecular weight of 267 kDa) called factor VIII C, when measured by procoagulant activity or factor VIII Ag, when measured immunologically; (2) factor VIII R P04275 (the von Willebrand factor or vWF) has a molecular weight of 225 kDa. Factor VIII, along with calcium and phospholipids, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa. Domain F5/8 type C 2 Is responsible for phospholipid binding and essential for factor VIII activity. Defects in F8 are the cause of hemophilia A (HEMA). HEMA is a common recessive X-linked coagulation disorder.
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