Search
heparin cofactor 2; heparin cofactor II; HC-II; protease inhibitor leuserpin-2; HLS2; Serpin D1 (SERPIND1, HCF2)
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:
- heparin cofactor II Ag in plasma
Interactions
molecular events
General
antithrombin
glycoprotein
phosphoprotein
Properties
SIZE: entity length = 499 aa
MW = 57 kD
COMPARTMENT: plasma
MOTIF: signal sequence {1-19}
Ser phosphorylation site {S37}
N-glycosylation site {N49}
Chemotactic activity {68-79}
2 X 11 AA approximate repeats, Asp/Glu- rich (acidic) (hirudin-like) {73-97}
MOTIF: consensus repeat {73-83}
consensus repeat {87-97}
N-glycosylation site {N188}
binding site
SITE: 192-212
FOR-BINDING-OF: glycosaminoglycan
N-glycosylation site {N387}
peptide motif {463-464}
INHIBITS: thrombin
Database Correlations
OMIM correlations
UniProt P05546
Pfam PF00079
Entrez Gene 3053
Kegg hsa:3053
References
- UniProt :accession P05546
- Medical Knowledge Self Assessment Program (MKSAP) 11, American
College of Physicians, Philadelphia 1998
Component-of
molecular complex