SERPIND1

This gene belongs to the serpin gene superfamily. Serpins play roles in many processes including inflammation, blood clotting, and cancer metastasis. Members of this family have highly conserved secondary structures with a reactive center loop that interacts with the protease active site to inhibit protease activity. This gene encodes a plasma serine protease that functions as a thrombin and chymotrypsin inhibitor. The protein is activated by heparin, dermatan sulfate, and glycosaminoglycans. Allelic variations in this gene are associated with heparin cofactor II deficiency. [provided by RefSeq, Jul 2015]

Serpin Family D Member 1

Thrombin inhibitor activated by the glycosaminoglycans, heparin or dermatan sulfate. In the presence of the latter, HC-II becomes the predominant thrombin inhibitor in place of antithrombin III (AT-III). Also inhibits chymotrypsin, but in a glycosaminoglycan-independent manner.
Peptides at the N-terminal of HC-II have chemotactic activity for both monocytes and neutrophils.

Biological Process blood coagulationIEA:UniProtKB-KW
Biological Process chemotaxisIEA:UniProtKB-KW
Biological Process negative regulation of endopeptidase activityManual Assertion Based On ExperimentIBA:GO_Central

endoplasmic reticulum lumen
extracellular exosome
extracellular region
extracellular space

Thrombophilia due to heparin cofactor 2 deficiency (THPH10):
A hemostatic disorder characterized by a tendency to recurrent thrombosis.

Phosphorylated by FAM20C in the extracellular medium.