Serpin Peptidase Inhibitor, Clade D Member 1 Protein

Background

SERPIND1 encodes Heparin Cofactor II (HCII), a serine protease inhibitor (serpin) that plays a critical role in the regulation of coagulation. It shares functional similarities with antithrombin (SERPINC1), particularly in inhibiting thrombin, a key enzyme in the coagulation cascade. However, HCII exhibits a more specialized function as its activity is highly dependent on heparin-like glycosaminoglycans (GAGs), particularly dermatan sulfate, and to a lesser extent, heparin. HCII's involvement in thrombin inhibition and its activation through GAGs have made it an essential component in controlling clot formation and preventing thrombosis, particularly in vascular tissue. HCII deficiency has been linked to a predisposition for thromboembolic disorders and cardiovascular diseases.