Protein C is a vitamin K-dependent serine protease that regulates blood coagulation by inactivating factors Va and VIIIa in the presence of calcium ions and phospholipids. Defects in PROC are the cause of thrombophilia due to protein C deficiency, autosomal dominant (THPH3). A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. However, many adults with heterozygous disease may be asymptomatic. Individuals with decreased amounts of protein C are classically referred to as having type I protein C deficiency and those with normal amounts of a functionally defective protein as having type II deficiency. Defects in PROC are the cause of thrombophilia due to protein C deficiency, autosomal recessive (THPH4). A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. It results in a thrombotic condition that can manifest as a severe neonatal disorder or as a milder disorder with late-onset thrombophilia. The severe form leads to neonatal death through massive neonatal venous thrombosis. Often associated with ecchymotic skin lesions which can turn necrotic called purpura fulminans, this disorder is very rare. Belongs to the peptidase S1 family. Note: This description may include information from UniProtKB.
Molecular Function: protein binding; serine-type endopeptidase activity
Biological Process: blood coagulation; ER to Golgi vesicle-mediated transport; leukocyte migration; negative regulation of apoptosis; negative regulation of blood coagulation; negative regulation of coagulation; negative regulation of inflammatory response; peptidyl-glutamic acid carboxylation; signal peptide processing
Alt. Names/Synonyms: Activation peptide; Anticoagulant protein C; APC; Autoprothrombin IIA; Blood coagulation factor XIV; PC; PROC; PROC1; protein C; protein C (inactivator of coagulation factors Va and VIIIa); Vitamin K-dependent protein C; Vitamin K-dependent protein C heavy chain; Vitamin K-dependent protein C light chain