Names & Taxonomy

Uniprot ID:
P05155
Entry Name:
IC1_HUMAN
Status:
reviewed
Protein Names:
Plasma protease C1 inhibitor (C1 Inh) (C1Inh) (C1 esterase inhibitor) (C1-inhibiting factor) (Serpin G1)
Gene Names:
SERPING1 C1IN C1NH
Gene Names Primary:
SERPING1
Organism:
Homo sapiens (Human)

Structure

Length:
500
Sequence:
MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Secreted.

Function

Function:
Activation of the C1 complex is under control of the C1-inhibitor. It forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play a potentially crucial role in regulating important physiological pathways including complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein.
Cross Reference Drug Bank:
DB06404
Gene Ontology Go:
blood microparticle
extracellular exosome
extracellular region
extracellular space
platelet alpha granule lumen
serine-type endopeptidase inhibitor activity
aging
blood circulation
blood coagulation
blood coagulation, intrinsic pathway
complement activation, classical pathway
fibrinolysis
innate immune response
negative regulation of complement activation, lectin pathway
negative regulation of endopeptidase activity
platelet activation
platelet degranulation
Gene Ontology Biological Process:
aging
blood circulation
blood coagulation
blood coagulation, intrinsic pathway
complement activation, classical pathway
fibrinolysis
innate immune response
negative regulation of complement activation, lectin pathway
negative regulation of endopeptidase activity
platelet activation
platelet degranulation
Gene Ontology Molecular Function:
serine-type endopeptidase inhibitor activity
Gene Ontology Cellular Component:
blood microparticle
extracellular exosome
extracellular region
extracellular space
platelet alpha granule lumen
Keywords:
3D-structure
Alternative splicing
Blood coagulation
Complement pathway
Complete proteome
Direct protein sequencing
Disease mutation
Disulfide bond
Fibrinolysis
Glycoprotein
Hemostasis
Immunity
Innate immunity
Polymorphism
Protease inhibitor
Reference proteome
Repeat
Secreted
Serine protease inhibitor
Signal

Publication

PubMed ID:
3488058 3756141 3267220 2026152 14702039 16554811 15489334 3393514 12773530 6416294 2154751 3089875 8618290 3458172 7814636 8495195 10946292 12123444 12754519 15096536 14760718 16040958 16335952 19159218 19139490 19838169 22171320 23234360 24275569 17488724 7749926 3178731 2365061 2296585 2118657 1451784 1363816 8172583 7883978 8529136 8755917 14635117 16409206 22994404 24456027