
The coagulation system is carefully regulated by the anticoagulant protein C system. Thrombin bound to thrombomodulin losses its procoagulant functions and becomes a powerful activator of protein C. Activated protein C (APC) down-regulates the generation of thrombin thus preventing the propagation of the clot towards regions in which is not needed.

The activation of protein C by thrombin-thrombomodulin complexes increases ten-fold by the presence of the endothelial protein C receptor (EPCR), which is expressed on the surface of endothelial cells of arterial and venous vessels and on the placental throphoblast. The presence of autoantibodies against EPCR could induce the development of thrombosis. In case of blocking antibodies, by inhibiting the protein C systems, —in case of non-blocking ones, by injuring the vessel wall.
A system for the detection of autoantibodies anti-EPCR has been developed at the Cardiovascular Sciences Division of the Applied Medical Research Centre (CIMA) of the University of Navarra. Using this system we have detected, for the first time, the presence of these antibodies in thrombotic patients. We have demonstrated an association of autoantibodies against EPCR with venous thrombosis, myocardial infarction and obstetric complications like foetal death. We are currently exploring their possible association with cerebral infarction and autoimmune diseases.