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Factor VIII

From Academic Kids

Factor VIII (FVIII) is an essential clotting factor. The lack of normal FVIII causes Hemophilia A, an inherited bleeding disorder.

Contents

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 or factor Xa, it dissociates from the complex to interact with other factors of the coagulation cascade. It is a cofactor to Factor IX in the activation 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


Cardiovascular system - Blood
Red blood cells - White blood cells - Platelets - Blood plasma
White blood cells
Granulocytes (Neutrophil granulocytes, Eosinophil granulocytes, Basophil granulocytes) - Lymphocytes - Monocytes
Coagulation
Coagulation factors: - Fibrin (I) - (Pro)thrombin (II) - FV - FVII - FVIII - FIX - FX - FXI - FXII - FXIII - HMWK - vWF - Tissue factor
Inhibitors: Antithrombin - Protein C - Protein S - Protein Z - ZPI - TFPI
Fibrinolysis: Plasmin - tPA/urokinase - PAI-1/2 - α2-AP - TAFI
pt:Fator VIII
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