Heparin Cofactor II (SERPIND1) Antibody – Goat Polyclonal

The product encoded by this gene is a serine proteinase inhibitor which rapidly inhibits thrombin in the presence of dermatan sulfate or heparin. The gene contains five exons and four introns. This protein shares homology with antithrombin III and other members of the alpha 1-antitrypsin superfamily. Mutations in this gene are associated with heparin cofactor II deficiency. [provided by RefSeq]

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. Ref.11Peptides at the N-terminal of HC-II have chemotactic activity for both monocytes and neutrophils. Ref.11

Tissue specificity: Expressed predominantly in liver. Also present in plasma. Ref.15

Domain: The N-terminal acidic repeat region mediates, in part, the glycosaminoglycan-accelerated thrombin inhibition. Ref.11

Post-translational modification: Phosphorylation sites are present in the extracelllular medium.

Involvement in 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. Ref.16 Ref.17 Ref.19 Ref.20

Sequence similarities: Belongs to the serpin family.

Sequence caution: The sequence CAG30459.1 differs from that shown. Reason: Erroneous initiation.