heparin cofactor 2; heparin cofactor II; HC-II; protease inhibitor leuserpin-2; HLS2; Serpin D1 (SERPIND1, HCF2)
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Function
- cofactor for heparin & dermatan sulfate
- inhibits thrombin in the presence of heparin or dermatan sulfate
- in the presence of the dermatan sulfate, SERPIND1 becomes the predominant thrombin inhibitor in place of antithrombin III (AT-III)
- also inhibits chymotrypsin (glycosaminoglycan-independent)
- peptides at the N-terminal of SERPIND1 have chemotactic activity for both monocytes & neutrophils
- phosphorylation sites are present in the extracellular medium
Structure
- the N-terminal acidic repeat region mediates, in part, the glycosaminoglycan-accelerated thrombin inhibition
- belongs to the serpin family
Expression
- expressed predominantly in liver
- present in plasma
Pathology
- defects in SERPIND1 are the cause of heparin cofactor 2 deficiency
Laboratory
More general terms
References
- ↑ UniProt http://www.uniprot.org/uniprot/P05546.html
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 11, American College of Physicians, Philadelphia 1998
Database
- Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=3053
- Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:3053
- OMIM: https://mirror.omim.org/entry/142360
- OMIM: https://mirror.omim.org/entry/188050
- OMIM: https://mirror.omim.org/entry/612356
- UniProt: http://www.uniprot.org/uniprot/P05546.html