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The gene for Factor VIII is located on the X chromosome (Xq28).
FVIII is a glycoprotein synthesized and released into the bloodstream by the liver. In the circulating blood, it is mainly bound to von Willebrand factor (vWF, also known as Factor VIII-related antigen) to form a stable complex. Upon activation by thrombin, it dissociates from the complex to interact with other factors of the coagulation cascade. It is a cofactor to Factor IX in the activiation of Factor X , which in turn activates more thrombin. Thrombin leads to fibrin formation and the development of a clot.
No longer protected by vWF, activated FVIII is proteolytically inactivated in the process (most prominently by activated Protein C ) and quickly degraded.
FVIII harvested from donated blood or Recombinant FVIII can be given to hemophiliacs to restore hemostasis. Thus, FVIII is also known as Anti-Hemophilic Factor.
| Cardiovascular system - Blood |
| Red blood cells - White blood cells - Platelets - Blood plasma |
| Granulocytes ( Neutrophil granulocytes, Eosinophil granulocytes, Basophil granulocytes) - Lymphocytes - Monocytes |
| Coagulation factors: - Fibrin - Thrombin - FVII - FVIII - FXII - HMWK - vWF |
| Inhibitors: Antithrombin - Protein C - Protein S - TFPI |
| Fibrinolysis: Plasmin - tPA/uPA - PAI-1/2 - α2-AP |