Heparin cofactor II

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Protein SERPIND1 PDB 1jmj.png
Available structures
PDB Ortholog search: PDBe RCSB
Aliases SERPIND1, D22S673, HC2, HCF2, HCII, HLS2, LS2, THPH10, serpin family D member 1
External IDs OMIM: 142360 MGI: 96051 HomoloGene: 36018 GeneCards: SERPIND1
Gene location (Human)
Chromosome 22 (human)
Chr. Chromosome 22 (human)[1]
Chromosome 22 (human)
Genomic location for SERPIND1
Genomic location for SERPIND1
Band 22q11.21 Start 20,774,113 bp[1]
End 20,787,720 bp[1]
RNA expression pattern
PBB GE SERPIND1 205576 at fs.png
More reference expression data
Species Human Mouse
RefSeq (mRNA)



RefSeq (protein)



Location (UCSC) Chr 22: 20.77 – 20.79 Mb Chr 16: 17.33 – 17.34 Mb
PubMed search [3] [4]
View/Edit Human View/Edit Mouse

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").[5]

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 and other members of the alpha 1-antitrypsin superfamily. Mutations in this gene are associated with heparin cofactor II deficiency.[5]

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


  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000099937 - Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000022766 - Ensembl, May 2017
  3. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. ^ a b "Entrez Gene: SERPIND1 serpin peptidase inhibitor, clade D (heparin cofactor), member 1".

Further reading

External links

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