Heparin cofactor II

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Serpin peptidase inhibitor, clade D (heparin cofactor), member 1
Protein SERPIND1 PDB 1jmj.png
PDB rendering based on 1jmj.
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols SERPIND1 ; D22S673; HC2; HCF2; HCII; HLS2; LS2; THPH10
External IDs OMIM142360 MGI96051 HomoloGene36018 GeneCards: SERPIND1 Gene
RNA expression pattern
PBB GE SERPIND1 205576 at tn.png
More reference expression data
Species Human Mouse
Entrez 3053 15160
Ensembl ENSG00000099937 ENSMUSG00000022766
UniProt P05546 P49182
RefSeq (mRNA) NM_000185 NM_008223
RefSeq (protein) NP_000176 NP_032249
Location (UCSC) Chr 22:
21.13 – 21.14 Mb
Chr 16:
17.33 – 17.34 Mb
PubMed search [1] [2]

Heparin cofactor II (HCII), a protein encoded by the SERPIND1 gene, is a coagulation factor that inhibits IIa, and is a cofactor for heparin and dermatan sulfate ("minor antithrombin"). [1]

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.[1] Heparin Cofactor II deficiency can lead to increased thrombin generation and a hypercoagulable state.


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