Factor VIII
Factor VIII (FVIII) is an essential clotting factor. The lack of normal FVIII causes Hemophilia A, an inherited bleeding disorder.
Genetics
The gene for Factor VIII is located on the X chromosome (Xq28).
Physiology
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.
Therapeutic use
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.
External link
- A review on Recombinant Factor VIII (http://www.niper.nic.in/uc.pdf)
| 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 |