title: The anticoagulant protein C pathway.
abstract: The anticoagulant protein C system regulates the activity of coagulation factors VIIIa and Va, cofactors in the activation of factor X and prothrombin, respectively. Protein C is activated on endothelium by the thrombin-thrombomodulin-EPCR (endothelial protein C receptor) complex. Activated protein C (APC)-mediated cleavages of factors VIIIa and Va occur on negatively charged phospholipid membranes and involve protein cofactors, protein S and factor V. APC also has anti-inflammatory and anti-apoptotic activities that involve binding of APC to EPCR and cleavage of PAR-1 (protease-activated receptor-1). Genetic defects affecting the protein C system are the most common risk factors of venous thrombosis. The protein C system contains multi-domain proteins, the molecular recognition of which will be reviewed.
authors.affiliation: Department of Laboratory Medicine, Clinical Chemistry, Lund University, The Wallenberg laboratory, University Hospital, Malmö, SE-205 02 Malmö, Sweden. bjorn.dahlback@klkemi.mas.lu.se
authors.forename: Björn
authors.initials: B
authors.lastname: Dahlbäck
authors.forename: Bruno O
authors.initials: BO
authors.lastname: Villoutreix
mesh_terms: D000818:Animals; D000925:Anticoagulants / Q000737:chemistry / Q000378:metabolism*; D001777:Blood Coagulation; D004727:Endothelium / Q000166:cytology / Q000187:drug effects / Q000201:enzymology; D006801:Humans; D011486:Protein C / Q000737:chemistry / Q000378:metabolism*; D017293:Protein S / Q000378:metabolism; D014812:Vitamin K / Q000378:metabolism / Q000494:pharmacology
chemical_list: D000925:Anticoagulants; D011486:Protein C; D017293:Protein S; D014812:Vitamin K