Serpin Family D Member 1, Cysteine Proteinase Inhibitor Clade D Member 1, Serpin Peptidase Inhibitor Clade D Member 1, Protease Inhibitor Leuserpin-2, Serpin D1, HCF2, HLS2, Leuserpin 2, D22S673, THPH10, HC-II, HCII, HC2, LS2.
Serpin Peptidase Inhibitor, Clade D Member 1 (SERPIND1), also known as heparin cofactor II, is a protein that in humans is encoded by the SERPIND1 gene. This gene belongs to the serpin superfamily whose members are the most important inhibitors of serine proteases in human plasma. SERPIND1 is a potent inhibitor of thrombin, a key enzyme in the coagulation cascade. The inhibitory activity of SERPIND1 is significantly enhanced in the presence of glycosaminoglycans such as dermatan sulfate and heparin. SERPIND1 is primarily synthesized in the liver and circulates in the bloodstream. Mutations in this gene are associated with SERPIND1 deficiency, a rare bleeding disorder characterized by a reduced ability to control blood clotting.
Serpin Family D Member 1, Cysteine Proteinase Inhibitor Clade D Member 1, Serpin Peptidase Inhibitor Clade D Member 1, Protease Inhibitor Leuserpin-2, Serpin D1, HCF2, HLS2, Leuserpin 2, D22S673, THPH10, HC-II, HCII, HC2, LS2.
The SERPIND1 gene encodes a plasma serine protease that functions primarily as a thrombin and chymotrypsin inhibitor . The protein is activated by heparin, dermatan sulfate, and glycosaminoglycans . Members of the serpin family have highly conserved secondary structures with a reactive center loop that interacts with the protease active site to inhibit protease activity .
SERPIND1 is known for its role as a thrombin inhibitor. In the presence of glycosaminoglycans like heparin or dermatan sulfate, Heparin Cofactor II (HC-II), which is encoded by the SERPIND1 gene, becomes the predominant thrombin inhibitor, replacing antithrombin III (AT-III) . This mechanism is essential for regulating blood coagulation and preventing excessive clot formation .
Human recombinant SERPIND1 is used in various research applications to study its role in blood coagulation and its potential therapeutic uses. Understanding the function and regulation of this protein can provide insights into developing treatments for coagulation disorders and other related conditions .
For more detailed information, you can refer to resources like GeneCards and the Early Detection Research Network.