| UniProt Protein Function: | 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. |
| UniProt Protein Details: | Chromosomal Location of Human Ortholog: 22q11.21 Cellular Component: extracellular space; extracellular region Molecular Function:heparin binding; serine-type endopeptidase inhibitor activity; endopeptidase inhibitor activity Biological Process: chemotaxis; blood coagulation Disease: Heparin Cofactor Ii Deficiency |
| NCBI Summary: | 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, Jul 2008] |
| UniProt Code: | P05546 |
| NCBI GenInfo Identifier: | 123055 |
| NCBI Gene ID: | 3053 |
| NCBI Accession: | P05546.3 |
| UniProt Secondary Accession: | P05546,Q6IBZ5, B2RAI1, D3DX34, |
| UniProt Related Accession: | P05546 |
| Molecular Weight: | 499 |
| NCBI Full Name: | Heparin cofactor 2 |
| NCBI Synonym Full Names: | serpin peptidase inhibitor, clade D (heparin cofactor), member 1 |
| NCBI Official Symbol: | SERPIND1 |
| NCBI Official Synonym Symbols: | HC2; LS2; HCF2; HCII; HLS2; THPH10; D22S673 |
| NCBI Protein Information: | heparin cofactor 2; serpin D1; leuserpin 2; protease inhibitor leuserpin-2; serine (or cysteine) proteinase inhibitor, clade D (heparin cofactor), member 1 |
| UniProt Protein Name: | Heparin cofactor 2 |
| UniProt Synonym Protein Names: | Heparin cofactor II; HC-II; Protease inhibitor leuserpin-2; HLS2; Serpin D1 |
| Protein Family: | Heparin cofactor |
| UniProt Gene Name: | SERPIND1 |
| UniProt Entry Name: | HEP2_HUMAN |
