protein C

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A vitamin K-dependent plasma glycoprotein (262 aa), the zymogen of a serine endopeptidase (EC that is a key component of the anticoagulation system. The thrombin–*thrombomodulin complex will remove a 42-aa pre-pro sequence and activate protein C that will, in conjunction with protein S, hydrolyse factors Va and VIIIa, inhibiting clotting. Heterozygous mutation in the gene leads to autosomal dominant hereditary thrombophilia; homozygous mutation causes the more severe autosomal recessive form. The latter is characterized by recurrent venous thrombosis whereas the former may be asymptomatic.

Subjects: Chemistry.

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