C1-inhibitor

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; OMA:SERPING1 - orthologs
Gene location (Human)
Chr.	Chromosome 11 (human)
End	57,619,171 bp
Gene location (Mouse)
Chr.	Chromosome 2 (mouse)
End	84,605,788 bp
RNA expression pattern
	Top expressed in
	right lobe of liver; ; right lung; ; right coronary artery; ; gastric mucosa; ; Descending thoracic aorta; ; ascending aorta; ; popliteal artery; ; tibial arteries; ; left uterine tube; ; right ovary;
	Top expressed in
	left lobe of liver; ; gastrula; ; carotid body; ; decidua; ; ankle; ; right lung; ; right lung lobe; ; efferent ductule; ; white adipose tissue; ; uterus;
	More reference expression data
	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; collagen-containing extracellular matrix;
Biological process	hemostasis; blood coagulation, intrinsic pathway; negative regulation of peptidase activity; fibrinolysis; immune system process; ageing; 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
	710
	12258
	ENSG00000149131
	ENSMUSG00000023224
	P05155
	P97290
	NM_001032295; NM_000062
	NM_009776
	NP_000053; NP_001027466
	NP_033906
	Wikidata
View/Edit Human	View/Edit Mouse

C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily.^[5] Its main function is the inhibition of the complement system (C1r, C1s) to prevent spontaneous activation but also as the major regulator of the contact system (PK, FXIIa, and FXIa).^[6]^[7]^[8]

^ ^a ^b ^c GRCh38: Ensembl release 89: ENSG00000149131 – Ensembl, May 2017
^ ^a ^b ^c GRCm38: Ensembl release 89: ENSMUSG00000023224 – Ensembl, May 2017
^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ Law RH, Zhang Q, McGowan S, Buckle AM, Silverman GA, Wong W, et al. (2006). "An overview of the serpin superfamily". Genome Biology. 7 (5): 216. doi:10.1186/gb-2006-7-5-216. PMC 1779521. PMID 16737556.{{cite journal}}: CS1 maint: overridden setting (link)
^ Davis AE (September 2004). "Biological effects of C1 inhibitor". Drug News & Perspectives. 17 (7): 439–46. doi:10.1358/dnp.2004.17.7.863703. PMID 15514703.
^ Cicardi M, Zingale L, Zanichelli A, Pappalardo E, Cicardi B (November 2005). "C1 inhibitor: molecular and clinical aspects". Springer Seminars in Immunopathology. 27 (3): 286–98. doi:10.1007/s00281-005-0001-4. PMID 16267649. S2CID 24583403.
^ Long AT, Kenne E, Jung R, Fuchs TA, Renné T (March 2016). "Contact system revisited: an interface between inflammation, coagulation, and innate immunity". Journal of Thrombosis and Haemostasis. 14 (3): 427–437. doi:10.1111/jth.13235. PMID 26707513.

[refGRCh38Ensembl-1] GRCh38: Ensembl release 89: ENSG00000149131 – Ensembl, May 2017

[refGRCm38Ensembl-2] GRCm38: Ensembl release 89: ENSMUSG00000023224 – Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[5] Law RH, Zhang Q, McGowan S, Buckle AM, Silverman GA, Wong W, et al. (2006). "An overview of the serpin superfamily". Genome Biology. 7 (5): 216. doi:10.1186/gb-2006-7-5-216. PMC 1779521. PMID 16737556.{{cite journal}}: CS1 maint: overridden setting (link)

[pmid15514703-6] Davis AE (September 2004). "Biological effects of C1 inhibitor". Drug News & Perspectives. 17 (7): 439–46. doi:10.1358/dnp.2004.17.7.863703. PMID 15514703.

[pmid16267649-7] Cicardi M, Zingale L, Zanichelli A, Pappalardo E, Cicardi B (November 2005). "C1 inhibitor: molecular and clinical aspects". Springer Seminars in Immunopathology. 27 (3): 286–98. doi:10.1007/s00281-005-0001-4. PMID 16267649. S2CID 24583403.

[Long2016-8] Long AT, Kenne E, Jung R, Fuchs TA, Renné T (March 2016). "Contact system revisited: an interface between inflammation, coagulation, and innate immunity". Journal of Thrombosis and Haemostasis. 14 (3): 427–437. doi:10.1111/jth.13235. PMID 26707513.

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