SERPIND1
Domain
The N-terminal acidic repeat region mediates, in part, the glycosaminoglycan-accelerated thrombin inhibition.
Function
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.
Isoform 2
Shows negligible inhibition, in vitro, of thrombin and tPA and no inhibition of factor Xa, in vitro.
Involvement in disease
Thrombophilia due to heparin cofactor 2 deficiency
THPH10
A hemostatic disorder characterized by a tendency to recurrent thrombosis.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
Phosphorylated by FAM20C in the extracellular medium.
Sequence Similarities
Belongs to the serpin family.
Tissue Specificity
Expressed predominantly in liver. Also present in plasma.
Isoform 2
Expressed in plasma (at protein level) (PubMed:32827448). Expressed in liver (PubMed:32827448).
Alternative names
HCF2, SERPIND1, Heparin cofactor 2, Heparin cofactor II, Protease inhibitor leuserpin-2, Serpin D1, HC-II, HLS2