C1 esterase

SERPING1
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 2OAY
Identifiers
Aliases	SERPING1, C1IN, C1INH, C1NH, HAE1, HAE2, serpin family G member 1
External IDs	OMIM: 606860 MGI: 894696 HomoloGene: 44 GeneCards: SERPING1
Gene location (Human) Chr. Chromosome 11 (human) Band No data available Start 57,597,387 bp End 57,614,853 bp
Gene location (Mouse) Chr. Chromosome 2 (mouse) Band No data available Start 84,765,387 bp End 84,775,444 bp
RNA expression pattern More reference expression data
Gene ontology Molecular function • peptidase inhibitor activity • protein binding • serine-type endopeptidase inhibitor activity Cellular component • blood microparticle • extracellular region • extracellular exosome • platelet alpha granule lumen • extracellular space Biological process • hemostasis • blood coagulation, intrinsic pathway • negative regulation of peptidase activity • fibrinolysis • immune system process • aging • platelet degranulation • blood circulation • negative regulation of complement activation, lectin pathway • complement activation, classical pathway • innate immune response • negative regulation of endopeptidase activity • blood coagulation • regulation of complement activation • regulation of blood coagulation • negative regulation of complement activation Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	710	12258
Ensembl	ENSG00000149131	ENSMUSG00000023224
UniProt	P05155	P97290
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 11: 84.77 – 84.78 Mb
PubMed search

2OAY

710

12258

ENSG00000149131

ENSMUSG00000023224

P05155

P97290

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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Wikipedia