The role of protein C in congenital and acquired thrombotic disorders.
Control of the coagulation pathway requires mechanisms that limit and localize the clotting process. The vascular endothelium has been shown to play an active role in preventing blood clot formation in vivo. The endothelial cell surface contains a thrombin-binding protein, thrombomodulin, which, when complexed with thrombin, activates vitamin K-dependent protein C, an endogenous anticoagulant. Protein C and its cofactor, protein S, inhibit the clotting process by inactivating factors Va and VIIIa and, additionally, enhance the lysis of fibrin. It has been demonstrated by the detection of heterozygous and homozygous protein C-deficient patients with severe thrombotic complications that protein C is a major regulatory protein of hemostasis and thrombosis. This endogenous anticoagulant pathway is providing a better understanding of both congenital and acquired thrombotic disorders.
Housholder
G T
GT
Department of Pharmacology, University of Texas Health Science Center, Houston.
D001777:Blood Coagulation; D004730:Endothelium, Vascular / Q000378:metabolism*; D006023:Glycoproteins / Q000378:metabolism; D006801:Humans; D011486:Protein C / Q000378:metabolism*; D017293:Protein S; D011956:Receptors, Cell Surface / Q000378:metabolism; D018179:Receptors, Thrombin; D013917:Thrombin / Q000378:metabolism*; D013927:Thrombosis / Q000151:congenital / Q000378:metabolism*
D006023:Glycoproteins; D011486:Protein C; D017293:Protein S; D011956:Receptors, Cell Surface; D018179:Receptors, Thrombin; D013917:Thrombin