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Protein C is a medication used to treat protein C deficiency leading to purpura fulminans or coumarin-induced skin necrosis.
Protein C is a medication used to treat protein C deficiency leading to purpura fulminans or coumarin-induced skin necrosis.
Protein C is an endogenously occurring plasma protein that plays a key role within the coagulation cascade. Also known as blood coagulation factor XIV, Protein C is a zymogen, or enzyme precursor, of a vitamin K-dependent anticoagulant glycoprotein (serine protease) that is synthesized in the liver. It is converted by the thrombin/thrombomodulin-complex on the endothelial cell surface to Activated Protein C (APC). Once in its activated form, APC functions as a serine protease with potent anticoagulant effects, especially in the presence of its cofactor protein S. APC exerts its effect by inactivating essential components of the coagulation cascade (specifically factors V and VIII), which leads to a decrease in thrombin formation, and therefore a reduction in clot formation.
Protein C concentrate is indicated for pediatric and adult patients with severe congenital protein C deficiency for the prevention and treatment of venous thrombosis and purpura fulminans. It is also found as a component of some prothrombin complex concentrate (i.e. Factor IX Complex (Human)) formulations, such as Kcentra.
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