P05546
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
heparin cofactor II
NCBI Official Synonym Full Names
serpin peptidase inhibitor, clade D (heparin cofactor), member 1
NCBI Official Synonym Symbols
HC2; LS2; HCF2; HCII; HLS2; D22S673 [Similar Products]
NCBI Protein Information
heparin cofactor 2; serpin D1; leuserpin 2; OTTHUMP00000198450; OTTHUMP00000198451; 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
UniProt Synonym Gene Names
UniProt Entry Name
HEP2_HUMAN
NCBI Summary for SERPIND1
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]
UniProt Comments for SERPIND1
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: extracellular space; extracellular region
Molecular Function: serine-type endopeptidase inhibitor activity; heparin binding; endopeptidase inhibitor activity
Biological Process: chemotaxis; blood coagulation
Disease: Heparin Cofactor Ii Deficiency
Research Articles on SERPIND1
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Pathways associated with anti-SERPIND1 antibody
Diseases associated with anti-SERPIND1 antibody
Organs/Tissues associated with anti-SERPIND1 antibody
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