Protein C in thromboembolic disease.
Human protein C is the central protein of an important regulatory mechanism, as shown by the high incidence of thromboembolic complications in congenital deficiencies of protein C. Both abnormal molecules and half-normal levels of protein C antigen and activity have been found in patients with familial thrombotic complications. Newborn infants with congenital homozygous protein C deficiency develop catastrophic thrombosis (purpura fulminans) and will not survive beyond the neonatal period without protein C replacement. Individuals with systemic thrombosis have significantly decreased levels of protein C concomitant with the severity of the DIC. These studies suggest that protein C is important not only in the congenital deficiencies, but also in acquired deficiencies, such as during DIC or possibly the postsurgical hypercoagulable state.
D000818:Animals; D001779:Blood Coagulation Factors / Q000378:metabolism*; D002417:Cattle; D006023:Glycoproteins / Q000172:deficiency / Q000235:genetics / Q000378:metabolism*; D006720:Homozygote; D006801:Humans; D011486:Protein C; D012016:Reference Values; D013923:Thromboembolism / Q000097:blood*
D001779:Blood Coagulation Factors; D006023:Glycoproteins; D011486:Protein C