Abstract is: 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 but also as the major regulator of the contact system. 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.
| P267 | ATC code | B06AC01 |
| P705 | Ensembl protein ID | ENSP00000278407 |
| ENSP00000341861 | ||
| ENSP00000367574 | ||
| ENSP00000367575 | ||
| ENSP00000384420 | ||
| ENSP00000384561 | ||
| ENSP00000399746 | ||
| ENSP00000431226 | ||
| ENSP00000432554 | ||
| ENSP00000435431 | ||
| ENSP00000478572 | ||
| P2888 | exact match | http://purl.uniprot.org/uniprot/P05155 |
| P646 | Freebase ID | /m/05668y |
| P486 | MeSH descriptor ID | D050718 |
| P672 | MeSH tree code | D12.644.861.140.500 |
| D12.776.124.486.274.920.250.500 | ||
| D12.776.395.320 | ||
| D12.776.872.140.500 | ||
| D09.400.430.780 | ||
| P6366 | Microsoft Academic ID | 2779931649 |
| P10283 | OpenAlex ID | C2779931649 |
| P638 | PDB structure ID | 2OAY |
| P637 | RefSeq protein ID | NP_000053 |
| NP_001027466 | ||
| P2892 | UMLS CUI | C0540301 |
| P352 | UniProt protein ID | P05155 |
| P11143 | WikiProjectMed ID | C1-inhibitor |
| P682 | biological process | human ageing | Q332154 |
| innate immune response | Q428253 | ||
| blood coagulation | Q179217 | ||
| hemostasis | Q487937 | ||
| fibrinolysis | Q1410794 | ||
| complement activation, classical pathway | Q5128320 | ||
| platelet degranulation | Q14819456 | ||
| blood coagulation, intrinsic pathway | Q14820992 | ||
| regulation of blood coagulation | Q14820997 | ||
| blood circulation | Q14852003 | ||
| negative regulation of endopeptidase activity | Q14859614 | ||
| negative regulation of peptidase activity | Q14861064 | ||
| regulation of complement activation | Q14864528 | ||
| negative regulation of complement activation, lectin pathway | Q14865076 | ||
| negative regulation of complement activation | Q14905311 | ||
| immune system process | Q14916317 | ||
| P681 | cell component | extracellular exosome | Q903634 |
| extracellular space | Q1385443 | ||
| extracellular region | Q14645596 | ||
| platelet alpha granule lumen | Q14819447 | ||
| blood microparticle | Q21095089 | ||
| collagen-containing extracellular matrix | Q54810453 | ||
| P702 | encoded by | SERPING1 | Q14865077 |
| P703 | found in taxon | Homo sapiens | Q15978631 |
| P527 | has part(s) | Serpin, conserved site | Q24722422 |
| Serpin domain | Q24770809 | ||
| P680 | molecular function | protein binding | Q167149 |
| serine-type endopeptidase inhibitor activity | Q14859581 | ||
| peptidase inhibitor activity | Q14861063 | ||
| P361 | part of | Plasma protease C1 inhibitor | Q32849772 |
| Serpin superfamily | Q41797311 | ||
| Serpin domain, protein family | Q83140026 | ||
| Serpin, conserved site, protein family | Q95352347 |
FileName: PBB GE SERPING1 200986 at fs.png
Description: Gene expression pattern of the SERPING1 gene.
Artist: AndrewGNF at English Wikipedia
Work is copyrighted.
License: CC BY-SA 3.0
Attribution is required.
| Q99979017 | SERPING1 A456E [extracellular region] |
| Q99979013 | SERPING1 A458T [extracellular region] |
| Q99979014 | SERPING1 A458V [extracellular region] |
| Q99979002 | SERPING1 F236S [endoplasmic reticulum lumen] |
| Q99978995 | SERPING1 G184R [endoplasmic reticulum lumen] |
| Q99978999 | SERPING1 G184_P228del [endoplasmic reticulum lumen] |
| Q99979016 | SERPING1 I462S [extracellular region] |
| Q99979003 | SERPING1 L177P [endoplasmic reticulum lumen] |
| Q99979006 | SERPING1 N291H [endoplasmic reticulum lumen] |
| Q99978997 | SERPING1 P476L [endoplasmic reticulum lumen] |
| Q99979008 | SERPING1 P489R [endoplasmic reticulum lumen] |
| Q99979015 | SERPING1 P498S [extracellular region] |
| Q99979007 | SERPING1 R400C [endoplasmic reticulum lumen] |
| Q99979009 | SERPING1 R466C [extracellular region] |
| Q99979010 | SERPING1 R466H [extracellular region] |
| Q99979011 | SERPING1 R466L [extracellular region] |
| Q99979012 | SERPING1 R466S [extracellular region] |
| Q99979005 | SERPING1 S170P [endoplasmic reticulum lumen] |
| Q99979000 | SERPING1 S280_P282del [endoplasmic reticulum lumen] |
| Q99978996 | SERPING1 T189N [endoplasmic reticulum lumen] |
| Q99979004 | SERPING1 V288E [endoplasmic reticulum lumen] |
| Q99978998 | SERPING1 V473M [endoplasmic reticulum lumen] |
| Q113897126 | Berinert (C1INH) vs Placebo for DGF/IRI |
| Q86249432 | C1-Esteraseremmer-N for the Treatment of Hereditary (and Acquired) Angioedema (2006-02-28) |
| Q98604366 | Complement Inhibition: Attacking the Overshooting Inflammation @Fter Traumatic Brain Injury |
| Q96179005 | Conestat Alfa in the Prevention of Severe SARS-CoV-2 Infection in Hospitalized Patients With COVID-19 |
| Q113936165 | Efficacy and Safety of GNR-038 vs Berinert® in Patients With Hereditary Angioedema |
| Q113923809 | Evaluation of the Effects of Bradykinin Antagonists on Pulmonary Manifestations of COVID-19 Infections (AntagoBrad-Cov Study). |
| Q113919825 | Prevention of Acute Kidney Injury in Patients With NSTEMI |
| Q113889517 | Prevention of Severe SARS-CoV-2 Infection in Hospitalized Patients With COVID-19 |
| Q86246623 | Recombinant Human C1 Inhibitor for the Treatment of Acute Attacks in Patients With Hereditary Angioedema (2003-06-30) |
| Q86246571 | Recombinant Human C1 Inhibitor for the Treatment of Acute Attacks in Patients With Hereditary Angioedema (2004-04-30) |
| Q86247303 | Recombinant Human C1 Inhibitor for the Treatment of Acute Attacks in Patients With Hereditary Angioedema (2005-07-31) |
| Q113920021 | Study to Evaluate the Benefit of RUCONEST in Improving Neurological Symptoms in Post COVID-19 Infection |
| Q50261414 | Activated C1R:SERPING1 [extracellular region] |
| Q50261417 | Activated C1S:SERPING1 [extracellular region] |
| Q50261409 | Antigen:IgG:C1Q:2xActivated C1R:2xActivated C1S [plasma membrane] |
| Q50291936 | C1-Inh binds Antigen: antibody: C1 complex activated C1r, C1s |
| Q50259219 | Platelet alpha granule contents [extracellular region] |
| Q50259202 | Platelet alpha granule contents [platelet alpha granule lumen] |
| Q100154416 | SERPING1 is secreted |
| Q101003010 | complement C1 inactivator proteins |
| Q50291205 | factor XIIa binds SERPING1 |
| Q50259315 | factor XIIa:C1Inh [extracellular region] |
| Q50291202 | kallikrein binds SERPING1 |
| Q50259309 | kallikrein:C1Inh [extracellular region] |
| Q35567084 | Bench-to-bedside review: the role of C1-esterase inhibitor in sepsis and other critical illnesses |
| Q47436596 | Binding of human complement C1 sterase inhibitor to Leptospira spp. |
| Q24316891 | CpG mutations in the reactive site of human C1 inhibitor |
| Q92297975 | Hereditary Angioedema due to C1-Inhibitor Deficiency in Pediatric Patients in Croatia – First National Study, Diagnostic and Prophylactic Challenges |
| Q36166388 | Hereditary angioedema (HAE) in children and adolescents--a consensus on therapeutic strategies |
| Q24339566 | Identification of a new P1 residue mutation (444Arg----Ser) in a dysfunctional C1 inhibitor protein contained in a type II hereditary angioedema plasma |
| Q38645385 | Potential Roles for C1 Inhibitor in Transplantation |
| Q24290100 | Proteolytic activities of two types of mannose-binding lectin-associated serine protease |
| Q28087102 | Recombinant human C1 esterase inhibitor in the management of hereditary angioedema |
| Q24291623 | Substrate specificities of recombinant mannan-binding lectin-associated serine proteases-1 and -2 |
| Q14865077 | SERPING1 | encodes | P688 |
| Q18553584 | C1 inhibitor deficiency | afflicts | P689 |
| Arabic (ar / Q13955) | مثبط سي1 | wikipedia |
| cy | SERPING1 | wikipedia |
| C1-Esterase-Inhibitor | wikipedia | |
| C1-inhibitor | wikipedia | |
| Inhibidor C1 | wikipedia | |
| Persian (fa / Q9168) | بازدارنده C1 | wikipedia |
| C1-Inhibiteur | wikipedia | |
| C1-inibitore | wikipedia | |
| Inhibitor C1 | wikipedia | |
| SERPING1 | wikipedia |
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