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C1 esterase inhibitor

SERPING1
C1-inhibitor.png
Available structures
PDB Ortholog search: PDBe RCSB
Identifiers
Aliases SERPING1, C1IN, C1INH, C1NH, HAE1, HAE2, serpin family G member 1
External IDs OMIM: 606860 MGI: 894696 HomoloGene: 44 GeneCards: SERPING1
RNA expression pattern
PBB GE SERPING1 200986 at fs.png
More reference expression data
Orthologs
Species Human Mouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_001032295
NM_000062

NM_009776

RefSeq (protein)

NP_000053
NP_001027466

NP_033906

Location (UCSC) Chr 11: 57.6 – 57.61 Mb Chr 2: 84.77 – 84.78 Mb
PubMed search

2OAY

NM_001032295
NM_000062

NM_009776

NP_000053
NP_001027466

NP_033906

C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily. Its main function is the inhibition of the complement system to prevent spontaneous activation. C1-inhibitor is an acute-phase protein that circulates in blood at levels of around 0.25 g/L. The levels rise ~2-fold during inflammation. C1-inhibitor irreversibly binds to and inactivates C1r and C1s proteases in the C1 complex of classical pathway of complement. MASP-1 and MASP-2 proteases in MBL complexes of the lectin pathway are also inactivated. This way, C1-inhibitor prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. Although named after its complement inhibitory activity, C1-inhibitor also inhibits proteases of the fibrinolytic, clotting, and kinin pathways. Note that C1-inhibitor is the most important physiological inhibitor of plasma kallikrein, fXIa, and fXIIa.


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