功能: disease:Defects in SERPIND1 are the cause of heparin cofactor 2 deficiency (HCF2D) [MIM:612356]. HCF2D is an important risk factor for hereditary thrombophilia, a hemostatic disorder characterized by a tendency to recurrent thrombosis.,domain:The N-terminal acidic repeat region mediates, in part, the glycosaminoglycan-accelerated thrombin inhibition.,function:Peptides at the N-terminal of HC-II have chemotactic activity for both monocytes and neutrophils.,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.,similarity:Belongs to the serpin family.,tissue specificity:Expressed predominantly in liver.,
功能: disease:Defects in SERPIND1 are the cause of heparin cofactor 2 deficiency (HCF2D) [MIM:612356]. HCF2D is an important risk factor for hereditary thrombophilia, a hemostatic disorder characterized by a tendency to recurrent thrombosis.,domain:The N-terminal acidic repeat region mediates, in part, the glycosaminoglycan-accelerated thrombin inhibition.,function:Peptides at the N-terminal of HC-II have chemotactic activity for both monocytes and neutrophils.,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.,similarity:Belongs to the serpin family.,tissue specificity:Expressed predominantly in liver.,