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antithrombin-III (ATIII, heparin cofactor, SERPINC1, AT3, PRO0309)
Function:
- most important serine protease inhibitor in plasma
- regulates the blood coagulation cascade
- inhibits thrombin, factors IXa, Xa & XIa
- inhibitory activity is greatly enhanced in the presence of heparin (heparin cofactor)
- anti-inflammatory properties [2]
Structure: belongs to the serpin family
Compartment: secreted, extracellular space
Expression: found in plasma
Pathology:
- defects in SERPINC1 are the cause of
- antithrombin III deficiency
- plasma activity may be reduced as a result of acute thrombosis
- anticoagulation does NOT lower activity of antithrombin III in plasma
Pharmacology: see recombinant antithrombin-III
Interactions
molecular events
Related
antithrombin 3 in plasma (antithrombin 3 assay)
SERPINC1 gene mutation
Specific
Antithrombin Iii Parenteral
recombinant antithrombin-III
General
antithrombin
beta globulin
glycoprotein
Properties
SIZE: entity length = 464 aa
MW = 53 kD
COMPARTMENT: plasma
MOTIF: signal sequence {1-32}
cysteine residue {C40}
MODIFICATION: cysteine residue {C160}
cysteine residue {C53}
MODIFICATION: cysteine residue {C127}
binding site
SITE: 81-81
FOR-BINDING-OF: Heparin
cysteine residue {C127}
MODIFICATION: cysteine residue {C53}
N-glycosylation site {N128}
cysteine residue {C160}
MODIFICATION: cysteine residue {C40}
binding site
SITE: 161-161
FOR-BINDING-OF: Heparin
N-glycosylation site {N167}
binding site
SITE: 177-177
FOR-BINDING-OF: Heparin
N-glycosylation site {N187}
N-glycosylation site {N224}
cysteine residue {C279}
MODIFICATION: cysteine residue {C462}
Reactive bond {425-426}
cysteine residue {C462}
MODIFICATION: cysteine residue {C279}
INHIBITS: thrombin
Database Correlations
OMIM correlations
MORBIDMAP 107300
UniProt P01008
Pfam PF00079
References
- UniProt :accession P01008
- antithrombin mutation db
http://www.med.ic.ac.uk/divisions/7/antithrombin/
- GeneReviews
http://www.genetests.org/query?gene=SERPINC1
- SHMPD;The Singapore human mutation andcpolymorphism database
http://shmpd.bii.a-star.edu.sg/gene.php?genestart=A&genename=SERPINC1
- Wikipedia; antithrombin entry
http://en.wikipedia.org/wiki/antithrombin