Serping1

Summary

Gene Symbol: Serping1
Description: serpin family G member 1
Alias: plasma protease C1 inhibitor, C1 Inh, C1 esterase inhibitor, C1-inhibiting factor, C1Inh, serine (or cysteine) peptidase inhibitor, clade G, member 1, serine (or cysteine) proteinase inhibitor, clade G (C1 inhibitor), member 1, (angioedema, hereditary), serpin G1, serpin peptidase inhibitor, clade G (C1 inhibitor), member 1, serpin peptidase inhibitor, clade G, member 1
Species: rat

Top Publications

  1. pmc Common variation in the SERPING1 gene is not associated with age-related macular degeneration in two independent groups of subjects
    Kyu Hyung Park
    Department of Ophthalmology, Mayo Clinic, Rochester, MN 55905, USA
    Mol Vis 15:200-7. 2009
  2. pmc The effect of genetic variants in SERPING1 on the risk of neovascular age-related macular degeneration
    A Y Lee
    Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Br J Ophthalmol 94:915-7. 2010
  3. ncbi Anti-apoptotic role for C1 inhibitor in ischemia/reperfusion-induced myocardial cell injury
    Jinrong Fu
    Department of Cardiology, Renmin Hospital, Wuhan University School of Medicine, Wuhan, Hubei, PR China
    Biochem Biophys Res Commun 349:504-12. 2006
  4. pmc Inhibition of classical complement activation attenuates liver ischaemia and reperfusion injury in a rat model
    B H M Heijnen
    Department of Surgery, Surgical Laboratory, Academic Medical Centre, Amsterdam, The Netherlands
    Clin Exp Immunol 143:15-23. 2006
  5. ncbi Detection of C1 inhibitor (SERPING1/C1NH) mutations in exon 8 in patients with hereditary angioedema: evidence for 10 novel mutations
    Alvaro Blanch
    Unidad de Inmunologia, Hospital Universitario La Paz, Paseo de la Castellana, 261, 28046 Madrid, Spain
    Hum Mutat 20:405-6. 2002
  6. doi Variation in complement component C1 inhibitor in age-related macular degeneration
    J Gibson
    Genetic Epidemiology and Bioinformatics Group, Human Genetics Division, School of Medicine, University of Southampton, Southampton General Hospital, Southampton, UK
    Immunobiology 217:251-5. 2012
  7. doi Determination of a gene and environment risk model for age-related macular degeneration
    Jane Gibson
    Genetic Epidemiology and Bioinformatics Group, University of Southampton, Human Genetics Division, Southampton General Hospital, Southampton, UK
    Br J Ophthalmol 94:1382-7. 2010
  8. pmc Association between the SERPING1 gene and age-related macular degeneration and polypoidal choroidal vasculopathy in Japanese
    Isao Nakata
    Department of Ophthalmology and Visual Sciences, Kyoto University Graduate School of Medicine, Kyoto, Japan
    PLoS ONE 6:e19108. 2011
  9. pmc Age-related alterations in retinal neurovascular and inflammatory transcripts
    Colleen A Van Kirk
    Department of Pharmacology, Penn State College of Medicine, University Drive, Hershey, PA 17033, USA
    Mol Vis 17:1261-74. 2011
  10. pmc Mutant prourokinase with adjunctive C1-inhibitor is an effective and safer alternative to tPA in rat stroke
    Simone Tomasi
    Neuroscience Institute Cavalieri Ottolenghi, San Luigi Gonzaga Hospital, Orbassano, Turin, Italy
    PLoS ONE 6:e21999. 2011

Research Grants

Scientific Experts

  • L A Norris
  • Bruce L Zuraw
  • Petra J G Zwijnenburg
  • Simone Tomasi
  • Reindert W Emmens
  • Jurgen Birnbaum
  • P Kruse
  • Ineke G A Bos
  • Stephanie A Smith
  • Z Su
  • Claudia Duehrkop
  • Mark P V Begieneman
  • J Gibson
  • Colleen A Van Kirk
  • Isao Nakata
  • Jane Gibson
  • A Y Lee
  • Kyu Hyung Park
  • B H M Heijnen
  • Jinrong Fu
  • Marie Eve Moreau
  • Emanuela Pappalardo
  • Richard J Travers
  • James H Morrissey
  • C Pan
  • Alvaro Blanch
  • A L Serra
  • R P Beyer
  • J Zhang
  • J Zhou
  • C Mei
  • X Wang
  • X Gao
  • H Hu
  • M Shi
  • R P Wuthrich
  • Y Liu
  • Steven H Sacks
  • Sylvia Miescher
  • Rolf Spirig
  • Martin Spycher
  • Richard A G Smith
  • Yara Banz
  • Robert Rieben
  • Marc W Nolte
  • B P Morgan
  • Miranda Tempelaars
  • A J Lotery
  • S Ennis
  • C L Harris
  • Yvette Stumpf-Stolker
  • A Collins
  • Hans W M Niessen
  • S Hakobyan
  • B Vangerow
  • Marieke S van Ham
  • Magda M W Ulrich
  • A J Cree
  • Esther Middelkoop
  • Nynke E Hahn
  • Paul A J Krijnen
  • Bela Kubat
  • Sacha Zeerleder
  • Diana Wouters
  • Hisako Hayashi
  • Heather D VanGuilder
  • Akio Oishi
  • Willard M Freeman
  • Keitaro Matsuo
  • William E Sonntag
  • Norimoto Gotoh
  • Fumihiko Matsuda
  • Megan Young
  • Hideo Nakanishi
  • Nagahisa Yoshimura
  • Ryo Yamada
  • Kenji Yamashiro
  • Tomohiro Iida
  • Akitaka Tsujikawa
  • Julie A Farley
  • Atsushi Otani
  • Masaaki Saito
  • Kazuo Tajima
  • Andrew Collins
  • Andrew Lotery
  • Angela Cree
  • S Edelstein
  • Sarah Ennis
  • M A Brantley
  • M P Osborn

Detail Information

Publications29

  1. pmc Common variation in the SERPING1 gene is not associated with age-related macular degeneration in two independent groups of subjects
    Kyu Hyung Park
    Department of Ophthalmology, Mayo Clinic, Rochester, MN 55905, USA
    Mol Vis 15:200-7. 2009
    Common genetic variation in the complement component 1 inhibitor gene (SERPING1) was recently reported to increase the risk of developing age-related macular degeneration (AMD)...
  2. pmc The effect of genetic variants in SERPING1 on the risk of neovascular age-related macular degeneration
    A Y Lee
    Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Br J Ophthalmol 94:915-7. 2010
    ..Previous studies investigating the potential association between all AMD subtypes and the SERPING1 gene, which encodes a key regulator of the classic complement pathway, have yielded conflicting results...
  3. ncbi Anti-apoptotic role for C1 inhibitor in ischemia/reperfusion-induced myocardial cell injury
    Jinrong Fu
    Department of Cardiology, Renmin Hospital, Wuhan University School of Medicine, Wuhan, Hubei, PR China
    Biochem Biophys Res Commun 349:504-12. 2006
    ..injury and apoptosis during ischemia/reperfusion (I/R), whereas complement system inhibition with C1 inhibitor (C1INH), a serine protease inhibitor, exerts markedly cardioprotective effects...
  4. pmc Inhibition of classical complement activation attenuates liver ischaemia and reperfusion injury in a rat model
    B H M Heijnen
    Department of Surgery, Surgical Laboratory, Academic Medical Centre, Amsterdam, The Netherlands
    Clin Exp Immunol 143:15-23. 2006
    ..Pre-ischaemic administration of C1-inh is an effective pharmacological intervention to protect against liver I/R injury...
  5. ncbi Detection of C1 inhibitor (SERPING1/C1NH) mutations in exon 8 in patients with hereditary angioedema: evidence for 10 novel mutations
    Alvaro Blanch
    Unidad de Inmunologia, Hospital Universitario La Paz, Paseo de la Castellana, 261, 28046 Madrid, Spain
    Hum Mutat 20:405-6. 2002
    Hereditary angioedema (HAE) is caused by mutations in the C1 inhibitor gene (SERPING1, C1NH) and the result is C1 inhibitor deficiency, either in levels or function...
  6. doi Variation in complement component C1 inhibitor in age-related macular degeneration
    J Gibson
    Genetic Epidemiology and Bioinformatics Group, Human Genetics Division, School of Medicine, University of Southampton, Southampton General Hospital, Southampton, UK
    Immunobiology 217:251-5. 2012
    ..levels were analysed for relationships with age, gender, smoking, AMD disease status and genetic variation in the SERPING1 gene, which encodes C1inh, using a multivariate analysis...
  7. doi Determination of a gene and environment risk model for age-related macular degeneration
    Jane Gibson
    Genetic Epidemiology and Bioinformatics Group, University of Southampton, Human Genetics Division, Southampton General Hospital, Southampton, UK
    Br J Ophthalmol 94:1382-7. 2010
    ..age-related macular degeneration (AMD) and genetic variants in the serpin peptidase inhibitor, clade G, member 1 (SERPING1) gene...
  8. pmc Association between the SERPING1 gene and age-related macular degeneration and polypoidal choroidal vasculopathy in Japanese
    Isao Nakata
    Department of Ophthalmology and Visual Sciences, Kyoto University Graduate School of Medicine, Kyoto, Japan
    PLoS ONE 6:e19108. 2011
    Recently, a complement component 1 inhibitor (SERPING1) gene polymorphism was identified as a novel risk factor for age-related macular degeneration (AMD) in Caucasians...
  9. pmc Age-related alterations in retinal neurovascular and inflammatory transcripts
    Colleen A Van Kirk
    Department of Pharmacology, Penn State College of Medicine, University Drive, Hershey, PA 17033, USA
    Mol Vis 17:1261-74. 2011
    ..The goal of this study was to investigate the effects of aging on retinal mRNA expression of neurovascular and inflammatory transcripts previously demonstrated to be regulated with diabetes...
  10. pmc Mutant prourokinase with adjunctive C1-inhibitor is an effective and safer alternative to tPA in rat stroke
    Simone Tomasi
    Neuroscience Institute Cavalieri Ottolenghi, San Luigi Gonzaga Hospital, Orbassano, Turin, Italy
    PLoS ONE 6:e21999. 2011
    ..The C1I did not compromise fibrinolysis by M5 in contrast with tPA, consistent with previous in vitro findings...
  11. ncbi C1 inhibitor treatment improves host defense in pneumococcal meningitis in rats and mice
    Petra J G Zwijnenburg
    Department of Pediatrics, Vrije Universiteit Medical Center, Amsterdam, The Netherlands
    J Infect Dis 196:115-23. 2007
    ..C1-inh treatment increased bacterial clearance, possibly through an up-regulation of CR3. Hence, C1-inh may be a useful agent in the treatment of pneumococcal meningitis...
  12. doi Prolonged C1 inhibitor administration improves local healing of burn wounds and reduces myocardial inflammation in a rat burn wound model
    Mark P V Begieneman
    Department of Pathology, VU Medical Center, Amsterdam, The Netherlands
    J Burn Care Res 33:544-51. 2012
    ..Here, the effects of C1 esterase inhibitor (C1inh), an inhibitor of complement activation, on burn wound progression and the heart were analyzed in ..
  13. doi Safety and efficacy of prophylactic nanofiltered C1-inhibitor in hereditary angioedema
    Bruce L Zuraw
    University of California, San Diego, La Jolla, USA
    Am J Med 125:938.e1-7. 2012
    Nanofiltered C1-inhibitor (C1INH-nf) is approved for prophylactic treatment of hereditary angioedema. This study assessed the efficacy and safety of C1INH-nf as prophylactic therapy in a large cohort of patients with hereditary angioedema.
  14. pmc C1 esterase inhibitor reduces lower extremity ischemia/reperfusion injury and associated lung damage
    Claudia Duehrkop
    Department of Clinical Research, University of Bern, Bern, Switzerland Graduate School for Cellular and Biomedical Sciences, University of Bern, Bern, Switzerland
    PLoS ONE 8:e72059. 2013
    ..Upon reperfusion inflammatory cascades are activated causing tissue injury. We therefore tested preoperative treatment for prevention of reperfusion injury by using C1 esterase inhibitor (C1 INH).
  15. doi Excessive activation of the alternative complement pathway in autosomal dominant polycystic kidney disease
    Z Su
    Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, China
    J Intern Med 276:470-85. 2014
    ..The complement system is involved in many immune complex-mediated kidney diseases, yet its role in the pathogenesis of autosomal dominant polycystic kidney disease (ADPKD) has not been examined in detail...
  16. pmc How it all starts: Initiation of the clotting cascade
    Stephanie A Smith
    a Department of Biochemistry, University of Illinois at Urbana Champaign, Urbana, IL, USA
    Crit Rev Biochem Mol Biol 50:326-36. 2015
    ..This review focuses on how the initiation phase of the blood clotting cascade is regulated in both pathways, with a discussion of the contributions of these pathways to hemostasis versus thrombosis. ..
  17. doi Endogenous C1-inhibitor production and expression in the heart after acute myocardial infarction
    Reindert W Emmens
    Department of Pathology, VU University Medical Center, De Boelelaan 1117, 1081 HV, Amsterdam, The Netherlands Institute for Cardiovascular Research, VU University Medical Center, De Boelelaan 1117, 1081 HV, Amsterdam, The Netherlands Department of Immunopathology, Sanquin Research, Postbus 9892, 1006 AN, Amsterdam, The Netherlands Electronic address
    Cardiovasc Pathol 25:33-9. 2016
    ..In this study, we investigated whether endogenous C1-inhibitor, a well-known inhibitor of complement activation, is expressed in the heart after acute myocardial infarction...
  18. ncbi Impact of combined C1 esterase inhibitor/coagulation factor XIII or N-acetylcysteine/tirilazad mesylate administration on leucocyte adherence and cytokine release in experimental endotoxaemia
    J Birnbaum
    Klinik fur Anaesthesiologie und operative Intensivmedizin, Charite Universitatsmedizin Berlin, Campus Charité Mitte and Campus Virchow Klinikum, Berlin, Germany
    J Int Med Res 36:748-59. 2008
    We determined the effects of combinations of C1 esterase inhibitor (C1-INH) with factor XIII and of N-acetylcysteine (NAC) with tirilazad mesylate (TM) during lipo-polysaccharide (LPS)-induced endotoxaemia in rats...
  19. ncbi Fat-storing cells of the rat liver synthesize and secrete C1-esterase inhibitor; modulation by cytokines
    S Schwögler
    Department of Internal Medicine, University of Mainz, Germany
    Hepatology 16:794-802. 1992
    ....
  20. ncbi C1 inhibitor gene expression in patients with hereditary angioedema: quantitative evaluation by means of real-time RT-PCR
    Emanuela Pappalardo
    Department of Internal Medicine, University of Milan, Ospedale S Giuseppe, 20123 Milan, Italy
    J Allergy Clin Immunol 114:638-44. 2004
    Hereditary angioedema (HAE) is caused by heterozygous defects in the C1 inhibitor (C1-INH) gene (SERPING1/C1NH)...
  21. ncbi Blood coagulation
    Lucy A Norris
    Coagulation Research Laboratory, Department of Obstetrics and Gynaecology, Trinity College Centre for Health Sciences, St James s Hospital, 8, Dublin, Ireland
    Best Pract Res Clin Obstet Gynaecol 17:369-83. 2003
    ..Both genetic and environmental factors can influence the activation of coagulation and may predispose affected individuals to thrombosis...
  22. ncbi Effects of C1 inhibitor and r-SP-C surfactant on oxygenation and histology in rats with lavage-induced acute lung injury
    B Vangerow
    Department of Anaesthesia, Hannover Medical School, 30625 Hannover, Germany
    Intensive Care Med 27:1526-31. 2001
    ..To assess the effects of C1 inhibitor (INH) administration and r-SP-C surfactant application on oxygenation and lung histology in an acute respiratory distress syndrome model...
  23. ncbi C1-esterase inhibitor reduces infarct volume after cortical vein occlusion
    A Heimann
    Institute for Neurosurgical Pathophysiology, Johannes Gutenberg University Mainz, 55101, Mainz, Germany
    Brain Res 838:210-3. 1999
    ..Thus, the neuroprotective effect cannot be explained by an improvement of cerebral perfusion but rather by protection of the parenchyma in the penumbra...
  24. ncbi Proteases and protease inhibitors in taurocholate-induced acute pancreatitis in rats
    P Kruse
    Department of Surgical Gastroenterology D, Copenhagen University Hospital, Glostrup, Denmark
    Int J Pancreatol 25:113-21. 1999
    ..Protease activation and protease inhibitor consumption occur consistent with a two-stage development, and contact-phase activation is a possible primary event in this model...
  25. ncbi Beneficial effects of C1 esterase inhibitor in murine traumatic shock
    L Kochilas
    Department of Physiology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania 19107 6799, USA
    Shock 8:165-9. 1997
    ..We investigated the effects of early complement blockade induced by a C1 esterase inhibitor (C1 INH) in a rat model of traumatic shock...
  26. pmc Combined treatment with C1 esterase inhibitor and antithrombin III improves survival in severe acute experimental pancreatitis
    H Yamaguchi
    Department of Internal Medicine, University of Ulm, Germany
    Gut 40:531-5. 1997
    ..Patients with severe acute pancreatitis die of complications closely related to the systemic activation of protease cascades...
  27. ncbi Influence of C1-inhibitor on inflammation, edema and shock
    G Dickneite
    Research Laboratories, Behringwerke AG, Marburg, Germany
    Behring Inst Mitt . 1993
    C1-Inhibitor (Berinert, C1 INH), a 104 kDa protein, inhibits complement components (C1 esterase) as well as enzymes of the contact phase of coagulation (Factor XII, Factor XI) and kallikrein, thus regulating kinin generation...
  28. ncbi The kallikrein-kinin system: current and future pharmacological targets
    Marie Eve Moreau
    Faculty of Pharmacy, University of Montreal, Canada
    J Pharmacol Sci 99:6-38. 2005
    ....
  29. ncbi The functional integrity of the serpin domain of C1-inhibitor depends on the unique N-terminal domain, as revealed by a pathological mutant
    Ineke G A Bos
    Department of Immunopathology, Sanquin Research at CLB, and Landsteiner Laboratory, Academic Medical Center, University of Amsterdam, 1066 CX Amsterdam, The Netherlands
    J Biol Chem 278:29463-70. 2003
    ....

Research Grants2

  1. RHINOVIRUS MEDIATED TRANSCRIPTION IN AIRWAY INFLAMMATION
    Bruce Zuraw; Fiscal Year: 2002
    ..These results will provide a necessary foundation for additional investigations into downstream inflammatory consequences of rhinovirus infection as well as potential targets for new intervention strategies. ..
  2. GILZ Controls TLR-Induced NF-kB Activity In Acute Asthma
    Bruce Zuraw; Fiscal Year: 2008
    ..We anticipate that this proposal will provide novel and clinically relevant data regarding the mechanisms of acute asthma exacerbations. ..