title: The endothelial cell protein C receptor: its role in thrombosis.
abstract: The protein C anticoagulant pathway plays a crucial role as a regulator of the blood clotting cascade. Protein C is activated on the vascular endothelial cell membrane by the thrombin-thrombomodulin complex. The endothelial protein C receptor binds protein C and further enhances protein C activation. Once formed, activated protein C down-regulates thrombin formation by inactivating factors Va and VIIIa and exerts cytoprotective effects through endothelial protein C receptor binding. An adequate generation of activated protein C depends on the precise assembly, on the surface of the endothelial cells, of thrombin, thrombomodulin, protein C, and endothelial protein C receptor. Therefore, any change in the efficiency of this assembly may cause a reduction or increase in activated protein C generation and modulate the risk of thrombosis. This review highlights the role of the endothelial protein C receptor in disease and discusses the association of its mutations with the risk of thrombosis.
authors: Hemostasis and Thrombosis Unit, Research Center, La Fe University Hospital, Valencia, Spain.
authors: Silvia
authors: S
authors: Navarro
authors: Elena
authors: E
authors: Bonet
authors: Amparo
authors: A
authors: Estellés
authors: Ramón
authors: R
authors: Montes
authors: José
authors: J
authors: Hermida
authors: Laura
authors: L
authors: Martos
authors: Francisco
authors: F
authors: España
authors: Pilar
authors: P
authors: Medina
mesh_terms: D001779:Blood Coagulation Factors / Q000737:chemistry / Q000235:genetics* / Q000378:metabolism*; D006801:Humans; D009154:Mutation; D011956:Receptors, Cell Surface / Q000737:chemistry / Q000235:genetics* / Q000378:metabolism*; D013927:Thrombosis / Q000209:etiology*
chemical_list: D001779:Blood Coagulation Factors; D011956:Receptors, Cell Surface; C091963:activated protein C receptor