Protein C is a vitamin K-dependent plasma glycoprotein that is cleaved to its activated form by the thrombin-thrombomodulin complex. Protein C in its activated form contains a serine protease domain and functions in degradation of the activated forms of coagulation factors V and VIII. Protein C is a vitamin K-dependent serine protease produced in the liver and made up of 2 polypeptide chains. The 62 kDa proenzyme is activated by thrombin bound to an endothelial surface receptor and the active enzyme cleaves factor Va and VIIIa and thus inhibits blood coagulation. The molecular weight of the active enzyme is 55 kDa and the normal concentrations in human plasma is approximately 1-3 ng/mL because of the very fast turnover, the proenzyme concentration is approximately 3 µg/mL. Mutations in the Protein C gene have been associated with thrombophilia due to protein C deficiency, neonatal purpura fulminans, and recurrent venous thrombosis.
蛋白别名: Activation peptide; Anticoagulant protein C; Autoprothrombin IIA; Blood coagulation factor XIV; EDQVDPRLIDGK; inactivator of coagulation factors Va, VIII; prepro-protein C; protein C (inactivator of coagulation factors Va and VIIIa); type I protein C; Vitamin K-dependent protein C; Vitamin K-dependent protein C heavy chain; Vitamin K-dependent protein C light chain
基因别名: APC; PC; PROC; PROC1; THPH3; THPH4
分子生物学功能: serine protease