Heparin Cofactor II (HCII) Antibody – Rabbit Polyclonal

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]

SERPIND1: 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. Defects in SERPIND1 are the cause of thrombophilia due to heparin cofactor 2 deficiency (THPH10). A hemostatic disorder characterized by a tendency to recurrent thrombosis. Belongs to the serpin family.

Chromosomal Location of Human Ortholog: 22q11.21

Cellular Component: endoplasmic reticulum lumen; extracellular region; extracellular space

Molecular Function: endopeptidase inhibitor activity; heparin binding; serine-type endopeptidase inhibitor activity

Biological Process: bioluminescence; blood coagulation; cellular protein metabolic process; chemotaxis; post-translational protein modification

Disease: Heparin Cofactor Ii Deficiency