superoxide dismutase

Summary

Summary: An oxidoreductase that catalyzes the reaction between superoxide anions and hydrogen to yield molecular oxygen and hydrogen peroxide. The enzyme protects the cell against dangerous levels of superoxide. EC 1.15.1.1.

Top Publications

  1. ncbi Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation
    M E Gurney
    Department of Cell and Molecular Biology, Northwestern University Medical School, Chicago, IL 60611
    Science 264:1772-5. 1994
  2. ncbi Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
    D R Rosen
    Day Neuromuscular Research Laboratory, Massachusetts General Hospital, Charlestown 02129
    Nature 362:59-62. 1993
  3. ncbi Forkhead transcription factor FOXO3a protects quiescent cells from oxidative stress
    Geert J P L Kops
    Department of Physiological Chemistry, University Medical Center Utrecht and Center for Biomedical Genetics, 3584 CG Utrecht, The Netherlands
    Nature 419:316-21. 2002
  4. ncbi Reactive oxygen species promote TNFalpha-induced death and sustained JNK activation by inhibiting MAP kinase phosphatases
    Hideaki Kamata
    Laboratory of Gene Regulation and Signal Transduction, School of Medicine, University of California, San Diego, 9500 Gilman Drive, La Jolla, CA 92093, USA
    Cell 120:649-61. 2005
  5. pmc Posttranscriptional induction of two Cu/Zn superoxide dismutase genes in Arabidopsis is mediated by downregulation of miR398 and important for oxidative stress tolerance
    Ramanjulu Sunkar
    Institute for Integrative Genome Biology and Department of Botany and Plant Sciences, University of California, Riverside, California 92521, USA
    Plant Cell 18:2051-65. 2006
  6. ncbi Onset and progression in inherited ALS determined by motor neurons and microglia
    Severine Boillee
    Ludwig Institute for Cancer Research and Departments of Medicine and Neuroscience, University of California at San Diego, 9500 Gilman Drive, La Jolla, CA 92093, USA
    Science 312:1389-92. 2006
  7. pmc Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
    Hristelina Ilieva
    Ludwig Institute for Cancer Research and Department of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA
    J Cell Biol 187:761-72. 2009
  8. ncbi Reactive oxygen species have a causal role in multiple forms of insulin resistance
    Nicholas Houstis
    Broad Institute of MIT and Harvard, Cambridge, Massachusetts 02141, USA
    Nature 440:944-8. 2006
  9. ncbi Calorie restriction reduces oxidative stress by SIRT3-mediated SOD2 activation
    Xiaolei Qiu
    Department of Nutritional Science and Toxicology, University of California, Berkeley, CA 94720, USA
    Cell Metab 12:662-7. 2010
  10. ncbi ALS: a disease of motor neurons and their nonneuronal neighbors
    Severine Boillee
    Ludwig Institute for Cancer Research and Departments of Medicine and Neuroscience, University of California, San Diego, La Jolla, California 92093, USA
    Neuron 52:39-59. 2006

Detail Information

Publications275 found, 100 shown here

  1. ncbi Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation
    M E Gurney
    Department of Cell and Molecular Biology, Northwestern University Medical School, Chicago, IL 60611
    Science 264:1772-5. 1994
    Mutations of human Cu,Zn superoxide dismutase (SOD) are found in about 20 percent of patients with familial amyotrophic lateral sclerosis (ALS)...
  2. ncbi Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
    D R Rosen
    Day Neuromuscular Research Laboratory, Massachusetts General Hospital, Charlestown 02129
    Nature 362:59-62. 1993
    ..Here we report tight genetic linkage between FALS and a gene that encodes a cytosolic, Cu/Zn-binding superoxide dismutase (SOD1), a homodimeric metalloenzyme that catalyzes the dismutation of the toxic superoxide anion O2...
  3. ncbi Forkhead transcription factor FOXO3a protects quiescent cells from oxidative stress
    Geert J P L Kops
    Department of Physiological Chemistry, University Medical Center Utrecht and Center for Biomedical Genetics, 3584 CG Utrecht, The Netherlands
    Nature 419:316-21. 2002
    ..protects quiescent cells from oxidative stress by directly increasing their quantities of manganese superoxide dismutase (MnSOD) messenger RNA and protein...
  4. ncbi Reactive oxygen species promote TNFalpha-induced death and sustained JNK activation by inhibiting MAP kinase phosphatases
    Hideaki Kamata
    Laboratory of Gene Regulation and Signal Transduction, School of Medicine, University of California, San Diego, 9500 Gilman Drive, La Jolla, CA 92093, USA
    Cell 120:649-61. 2005
    ..We now show that TNFalpha-induced ROS, whose accumulation is suppressed by mitochondrial superoxide dismutase, cause oxidation and inhibition of JNK-inactivating phosphatases by converting their catalytic cysteine to ..
  5. pmc Posttranscriptional induction of two Cu/Zn superoxide dismutase genes in Arabidopsis is mediated by downregulation of miR398 and important for oxidative stress tolerance
    Ramanjulu Sunkar
    Institute for Integrative Genome Biology and Department of Botany and Plant Sciences, University of California, Riverside, California 92521, USA
    Plant Cell 18:2051-65. 2006
    ..Thus, relieving miR398-guided suppression of CSD2 in transgenic plants is an effective new approach to improving plant productivity under oxidative stress conditions...
  6. ncbi Onset and progression in inherited ALS determined by motor neurons and microglia
    Severine Boillee
    Ludwig Institute for Cancer Research and Departments of Medicine and Neuroscience, University of California at San Diego, 9500 Gilman Drive, La Jolla, CA 92093, USA
    Science 312:1389-92. 2006
    Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), a progressive paralytic disease characterized by loss of motor neurons...
  7. pmc Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
    Hristelina Ilieva
    Ludwig Institute for Cancer Research and Department of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA
    J Cell Biol 187:761-72. 2009
    ..Focusing on amyotrophic lateral sclerosis (ALS), especially disease caused by dominant mutations in Cu/Zn superoxide dismutase (SOD1), we review here the evidence that it is the convergence of damage developed within multiple cell ..
  8. ncbi Reactive oxygen species have a causal role in multiple forms of insulin resistance
    Nicholas Houstis
    Broad Institute of MIT and Harvard, Cambridge, Massachusetts 02141, USA
    Nature 440:944-8. 2006
    ..Together, our findings suggest that increased ROS levels are an important trigger for insulin resistance in numerous settings...
  9. ncbi Calorie restriction reduces oxidative stress by SIRT3-mediated SOD2 activation
    Xiaolei Qiu
    Department of Nutritional Science and Toxicology, University of California, Berkeley, CA 94720, USA
    Cell Metab 12:662-7. 2010
    ..SIRT3 reduces cellular ROS levels dependent on superoxide dismutase 2 (SOD2), a major mitochondrial antioxidant enzyme...
  10. ncbi ALS: a disease of motor neurons and their nonneuronal neighbors
    Severine Boillee
    Ludwig Institute for Cancer Research and Departments of Medicine and Neuroscience, University of California, San Diego, La Jolla, California 92093, USA
    Neuron 52:39-59. 2006
    ..The etiology of most ALS cases remains unknown, but 2% of instances are due to mutations in Cu/Zn superoxide dismutase (SOD1)...
  11. pmc Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis
    Koji Yamanaka
    Ludwig Institute for Cancer Research and Department of Medicine and Neuroscience, University of California at San Diego, 9500 Gilman Drive, La Jolla, California 92093 0670, USA
    Nat Neurosci 11:251-3. 2008
    Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease that is characterized by the loss of motor neurons...
  12. pmc Superoxide dismutases: role in redox signaling, vascular function, and diseases
    Tohru Fukai
    Section of Cardiology, Department of Medicine, University of Illinois at Chicago, 835 S Wolcott, Chicago, IL 60612, USA
    Antioxid Redox Signal 15:1583-606. 2011
    ..Because of the importance of metal cofactors in the activity of SODs, we will also discuss how each SOD obtains catalytic metal in the active sites. Finally, we will discuss the development of future SOD-dependent therapeutic strategies...
  13. pmc Transformation from a neuroprotective to a neurotoxic microglial phenotype in a mouse model of ALS
    Bing Liao
    Department of Neurology, Xiangya Hospital, Central South University, Changsha, 410008, China
    Exp Neurol 237:147-52. 2012
    ..demonstrated a phenotypic and functional transformation of adult ALS mice microglia that overexpress mutant superoxide dismutase (mSOD1)...
  14. ncbi Topology of superoxide production from different sites in the mitochondrial electron transport chain
    Julie St-Pierre
    MRC Medical Research Council Dunn Human Nutrition Unit, Hills Road, Cambridge CB2 2XY, England
    J Biol Chem 277:44784-90. 2002
    ..By measuring hydrogen peroxide production in the absence and presence of exogenous superoxide dismutase, we established the topology of superoxide production (on which side of the mitochondrial inner membrane ..
  15. ncbi Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators
    Julie St-Pierre
    Dana Farber Cancer Institute and Department of Cell Biology, Harvard Medical School, Boston, MA 02115, USA
    Cell 127:397-408. 2006
    ..These studies reveal that PGC-1alpha is a broad and powerful regulator of ROS metabolism, providing a potential target for the therapeutic manipulation of these important endogenous toxins...
  16. ncbi Dilated cardiomyopathy and neonatal lethality in mutant mice lacking manganese superoxide dismutase
    Y Li
    Department of Neurosurgery, University of California, San Francisco 94143 0748, USA
    Nat Genet 11:376-81. 1995
    The Sod2 gene for Mn-superoxide dismutase (MnSOD), an intramitochondrial free radical scavenging enzyme that is the first line of defense against superoxide produced as a byproduct of oxidative phosphorylation, was inactivated by ..
  17. pmc Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS
    Daryl A Bosco
    Department of Neurology, University of Massachusetts Medical Center, Worcester, Massachusetts, USA
    Nat Neurosci 13:1396-403. 2010
    Many mutations confer one or more toxic function(s) on copper/zinc superoxide dismutase 1 (SOD1) that impair motor neuron viability and cause familial amyotrophic lateral sclerosis (FALS)...
  18. pmc Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons
    Makiko Nagai
    Department of Neurology, Columbia University, 710 West 168th Street, New York, New York 10032, USA
    Nat Neurosci 10:615-22. 2007
    Mutations in superoxide dismutase-1 (SOD1) cause a form of the fatal paralytic disorder amyotrophic lateral sclerosis (ALS), presumably by a combination of cell-autonomous and non-cell-autonomous processes...
  19. pmc The microglial-motoneuron dialogue in ALS
    S H Appel
    Methodist Neurological Institute, Methodist Hospital Research Institute, Houston, TX, USA
    Acta Myol 30:4-8. 2011
    ..Evidence from transgenic models expressing mutant superoxide dismutase 1 (SOD) suggests that the dialogue between motor neurons and microglia initially protects motor neurons...
  20. pmc Sirt3-mediated deacetylation of evolutionarily conserved lysine 122 regulates MnSOD activity in response to stress
    Randa Tao
    Howard Hughes Medical Institute and Molecular Radiation Oncology, Radiation Oncology Branch, Center for Cancer Research, NCI, NIH, Bethesda, MD 20892, USA
    Mol Cell 40:893-904. 2010
    ..Finally, IR was unable to induce MnSOD deacetylation or activity in Sirt3⁻/⁻ livers, and these irradiated livers displayed significant IR-induced cell damage and microvacuolization in their hepatocytes...
  21. pmc An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1
    Stefania Guareschi
    Weinberg Unit for ALS Research, Department of Neuroscience, Farber Institute for Neurosciences, Thomas Jefferson University, Philadelphia, PA 19107, USA
    Proc Natl Acad Sci U S A 109:5074-9. 2012
    Recent studies suggest that Cu/Zn superoxide dismutase (SOD1) could be pathogenic in both familial and sporadic amyotrophic lateral sclerosis (ALS) through either inheritable or nonheritable modifications...
  22. pmc Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis
    Edward Pokrishevsky
    Brain Research Centre, University of British Columbia, Vancouver, Canada
    PLoS ONE 7:e35050. 2012
    ..Clinically indistinguishable ALS can be caused by genetic mutations of Cu/Zn superoxide dismutase (SOD1), TAR-DNA binding protein 43 (TDP43), or fused in sarcoma/translocated in liposarcoma (FUS/TLS), or ..
  23. ncbi Manganese superoxide dismutase regulation and cancer
    Sanjit Kumar Dhar
    Graduate Center for Toxicology, University of Kentucky, Lexington, KY 40536, USA
    Free Radic Biol Med 52:2209-22. 2012
    ..The major antioxidant enzyme that scavenges superoxide anion radical in mitochondria is manganese superoxide dismutase (MnSOD)...
  24. pmc Against the oxidative damage theory of aging: superoxide dismutases protect against oxidative stress but have little or no effect on life span in Caenorhabditis elegans
    Ryan Doonan
    Institute of Healthy Ageing and Research Department of Genetics, Evolution and Environment, University College London, London WC1E 6BT, United Kingdom
    Genes Dev 22:3236-41. 2008
    ..O(2)(-) in cytosolic, extracellular, and mitochondrial pools is detoxified by dedicated superoxide dismutase (SOD) isoforms...
  25. ncbi FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis
    Han Xiang Deng
    Division of Neuromuscular Medicine, Davee Department of Neurology and Clinical Neurosciences, Chicago, IL 60611, USA
    Ann Neurol 67:739-48. 2010
    ..The pathogenic mechanism of the mutant FUS-mediated ALS and potential roles of FUS in non-FUS ALS remain to be investigated...
  26. ncbi Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man
    Lindsey R Fischer
    Department of Neurology, Emory University School of Medicine, Atlanta, GA 30322, USA
    Exp Neurol 185:232-40. 2004
    ..We conclude that in this widely studied animal model of human ALS, and in this single human case, motor neuron pathology begins at the distal axon and proceeds in a "dying back" pattern...
  27. ncbi Role of superoxide dismutases (SODs) in controlling oxidative stress in plants
    Ruth Grene Alscher
    Department of Plant Pathology, Physiology, and Weed Science, Virginia Tech, Blacksburg, VA 24061, USA
    J Exp Bot 53:1331-41. 2002
    ..SODs are clearly among the most important of those defences, when coupled with the necessary downstream events for full detoxification of ROS...
  28. pmc Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model
    Francesco Paolo Di Giorgio
    The Stowers Medical Institute, the Harvard Stem Cell Institute Harvard University, 7 Divinity Ave, Cambridge, Massachusetts 02138, USA
    Nat Neurosci 10:608-14. 2007
    ..These phenotypes displayed in culture could provide cell-based assays for the identification of new ALS drugs...
  29. ncbi Oxidative stress in ALS: key role in motor neuron injury and therapeutic target
    Sian C Barber
    Academic Neurology Unit and Sheffield Care and Research Centre for Motor Neuron Disorders, Department of Neuroscience, University of Sheffield, Sheffield S10 2RX, UK
    Free Radic Biol Med 48:629-41. 2010
    ..oxidative damage in ALS patient spinal cord and cerebrospinal fluid and mutations in the antioxidant enzyme superoxide dismutase 1 (SOD1) causing approximately 20% of familial ALS cases...
  30. ncbi Antioxidant enzymes and human diseases
    J M Mates
    Department of Molecular Biology and Biochemistry, Faculty of Sciences, University of Malaga, Spain
    Clin Biochem 32:595-603. 1999
    To describe the importance of the antioxidant enzymes superoxide dismutase, glutathione peroxidase, and catalase working together in human cells against toxic reactive oxygen species, their relationship with several pathophysiologic ..
  31. pmc Coupled degradation of a small regulatory RNA and its mRNA targets in Escherichia coli
    Eric Massé
    Laboratory of Molecular Biology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    Genes Dev 17:2374-83. 2003
    ..Thus, this large class of regulatory RNAs share an unexpected intrinsic mechanism for shutting off their action...
  32. ncbi Extension of murine life span by overexpression of catalase targeted to mitochondria
    Samuel E Schriner
    Department of Genome Sciences, University of Washington, Seattle, WA 91895, USA
    Science 308:1909-11. 2005
    ..These results support the free radical theory of aging and reinforce the importance of mitochondria as a source of these radicals...
  33. pmc Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells
    Christian Münch
    Medical Research Council Laboratory of Molecular Biology, Hills Road, Cambridge CB2 0QH, United Kingdom
    Proc Natl Acad Sci U S A 108:3548-53. 2011
    ..Misfolding of the normally globular mutant superoxide dismutase-1 (SOD1) is a central, early, but poorly understood event in the pathogenic cascade leading to familial ..
  34. ncbi Current hypotheses for the underlying biology of amyotrophic lateral sclerosis
    Jeffrey D Rothstein
    Department of Neurology and Neuroscience, Brain Science Institute, Johns Hopkins University, Baltimore, MD 21287, USA
    Ann Neurol 65:S3-9. 2009
    ..Although most cases have no known cause, mutations in the gene encoding Cu/Zn superoxide dismutase (SOD1) have been implicated in a fraction of familial cases of the disease...
  35. ncbi Pathways of oxidative damage
    James A Imlay
    Department of Microbiology, University of Illinois, Urbana, Illinois 61801, USA
    Annu Rev Microbiol 57:395-418. 2003
    ..The vulnerability of cells to oxidation lies at the root of obligate anaerobiosis, spontaneous mutagenesis, and the use of oxidative stress as a biological weapon...
  36. pmc T lymphocytes potentiate endogenous neuroprotective inflammation in a mouse model of ALS
    Isaac M Chiu
    Department of Pathology, Immune Disease Institute, Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 105:17913-8. 2008
    ..Innate and adaptive immunity were investigated in the CNS of the Superoxide Dismutase 1 (SOD1)(G93A) transgenic mouse model of ALS...
  37. ncbi The daf-2 gene network for longevity regulates oxidative stress resistance and Mn-superoxide dismutase gene expression in Caenorhabditis elegans
    Y Honda
    Tokyo Metropolitan Institute of Gerontology, Tokyo 173 0015, Japan
    FASEB J 13:1385-93. 1999
    ..We found that the mRNA level of the sod-3 gene, which encodes Mn-superoxide dismutase (SOD), was much higher in daf-2 mutants than in the wild type...
  38. pmc SOD1 (copper/zinc superoxide dismutase) deficiency drives amyloid β protein oligomerization and memory loss in mouse model of Alzheimer disease
    Kazuma Murakami
    Molecular Gerontology, Tokyo Metropolitan Institute of Gerontology, Itabashi ku, Tokyo 173 0015, Japan
    J Biol Chem 286:44557-68. 2011
    ..Our previous study demonstrated that mice deficient in cytoplasmic copper/zinc superoxide dismutase (CuZn-SOD, SOD1) have features of drusen formation, a hallmark of age-related macular degeneration (Imamura,..
  39. pmc Induction of manganese superoxide dismutase by nuclear translocation and activation of SIRT1 promotes cell survival in chronic heart failure
    Masaya Tanno
    Department of Pharmacology, Sapporo Medical University, Sapporo 060 8556, Japan
    J Biol Chem 285:8375-82. 2010
    ..Nuclear but not cytoplasmic SIRT1-induced manganese superoxide dismutase (Mn-SOD), which was further enhanced by resveratrol, and increased the resistance of C2C12 myoblasts to ..
  40. ncbi Antioxidant defense and aging in C. elegans: is the oxidative damage theory of aging wrong?
    David Gems
    Institute of Healthy Ageing and Research Department of Genetics, Evolution and Environment, University College London, London, UK
    Cell Cycle 8:1681-7. 2009
    ..New challenges to the theory from work using nematode worms seem set to bring them down to peck at its bones. But is the theory really dead, or does it just need to be modified?..
  41. ncbi Complex III releases superoxide to both sides of the inner mitochondrial membrane
    Florian L Muller
    Department of Cellular Biology, University of Texas Health Science Center, San Antonio, TX 78284 7762, USA
    J Biol Chem 279:49064-73. 2004
    ..this could only account for approximately 50% of the total electron leak even in mitochondria lacking CuZn-superoxide dismutase. We posit that the remaining approximately 50% of the electron leak must be due to superoxide released to ..
  42. pmc Tumour suppressor SIRT3 deacetylates and activates manganese superoxide dismutase to scavenge ROS
    Yaohui Chen
    State Key Laboratory of Genetic Engineering, School of Life Science, Novartis Fudan Joint Research Laboratory, Institutes of Biomedical Sciences, Fudan University, Shanghai, China
    EMBO Rep 12:534-41. 2011
    Mitochondria manganese superoxide dismutase (SOD2) is an important antioxidant enzyme, deficiency of which is associated with various human diseases. The known primary regulation of SOD2 is through transcriptional activation...
  43. pmc Hyperglycemia inhibits endothelial nitric oxide synthase activity by posttranslational modification at the Akt site
    X L Du
    Diabetes Research Center, Albert Einstein College of Medicine, Bronx, New York 10461, USA
    J Clin Invest 108:1341-8. 2001
    ..or by blocking mitochondrial superoxide overproduction with uncoupling protein-1 (UCP-1) or manganese superoxide dismutase (MnSOD)...
  44. pmc Is the oxidative stress theory of aging dead?
    Viviana I Perez
    Barshop Institute for Longevity and Aging Studies, Department of Cellular and Structural Biology, The University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA
    Biochim Biophys Acta 1790:1005-14. 2009
    ....
  45. ncbi Life-long reduction in MnSOD activity results in increased DNA damage and higher incidence of cancer but does not accelerate aging
    Holly Van Remmen
    Department of Cellular and Structural Biology at the University of Texas Health Science Center at San Antonio, San Antonio 78229 3900, USA
    Physiol Genomics 16:29-37. 2003
    ..heterozygous for the Sod2 gene (Sod2+/- mice) have been used to study the phenotype of life-long reduced Mn-superoxide dismutase (MnSOD) activity. The Sod2+/- mice have reduced MnSOD activity (50%) in all tissues throughout life...
  46. ncbi Superoxide dismutase multigene family: a comparison of the CuZn-SOD (SOD1), Mn-SOD (SOD2), and EC-SOD (SOD3) gene structures, evolution, and expression
    Igor N Zelko
    Division of Pulmonary and Critical Care, Department of Medicine, Duke University Medical Center, Durham, NC 27710, USA
    Free Radic Biol Med 33:337-49. 2002
    ..A single amino acid mutation in human SOD3 is associated with 10 to 30-fold increases in serum SOD3 levels. As more information is obtained, further insights will be gained...
  47. pmc Deletion of the mitochondrial superoxide dismutase sod-2 extends lifespan in Caenorhabditis elegans
    Jeremy M Van Raamsdonk
    Department of Biology, McGill University, Montreal, Quebec, Canada
    PLoS Genet 5:e1000361. 2009
    ..Overall, we show that increased oxidative stress caused by deletion of sod genes does not result in decreased lifespan in C. elegans and that deletion of sod-2 extends worm lifespan by altering mitochondrial function...
  48. ncbi Absence of CuZn superoxide dismutase leads to elevated oxidative stress and acceleration of age-dependent skeletal muscle atrophy
    Florian L Muller
    Department of Cellular and Structural Biology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229 3900, USA
    Free Radic Biol Med 40:1993-2004. 2006
    We describe a novel phenotype in mice lacking the major antioxidant enzyme, CuZn-superoxide dismutase (Sod1(-/-) mice), namely a dramatic acceleration of age-related loss of skeletal muscle mass...
  49. pmc Neurodegeneration, myocardial injury, and perinatal death in mitochondrial superoxide dismutase-deficient mice
    R M Lebovitz
    Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
    Proc Natl Acad Sci U S A 93:9782-7. 1996
    Manganese superoxide dismutase (SOD2) converts superoxide to oxygen plus hydrogen peroxide and serves as the primary defense against mitochondrial superoxide...
  50. ncbi Age-related dysfunction of the lacrimal gland and oxidative stress: evidence from the Cu,Zn-superoxide dismutase-1 (Sod1) knockout mice
    Takashi Kojima
    Department of Ocular Surface and Visual Optics, Keio University School of Medicine, Tokyo, Japan
    Am J Pathol 180:1879-96. 2012
    ..The superoxide dismutase (SOD) family is a major antioxidant system, and deficiency of Cu,Zn-superoxide dismutase-1 (Sod1) in mice ..
  51. pmc Astrocytes from familial and sporadic ALS patients are toxic to motor neurons
    Amanda M Haidet-Phillips
    The Research Institute at Nationwide Children s Hospital, Columbus, Ohio, USA
    Nat Biotechnol 29:824-8. 2011
    ..in the pathology of ALS derives in part from rodent models of FALS based upon dominant mutations within the superoxide dismutase 1 (SOD1) gene, which account for <2% of all ALS cases...
  52. pmc CD4+ T cells support glial neuroprotection, slow disease progression, and modify glial morphology in an animal model of inherited ALS
    David R Beers
    Department of Neurology, Methodist Neurological Institute, Houston, TX, USA
    Proc Natl Acad Sci U S A 105:15558-63. 2008
    ..Recent evidence derived from transgenic mice ubiquitously overexpressing mutant Cu(2+)/Zn(2+) superoxide dismutase (mSOD1), a chronic neurodegenerative model of inherited amyotrophic lateral sclerosis (ALS), indicates that ..
  53. ncbi Induced pluripotent stem cells generated from patients with ALS can be differentiated into motor neurons
    John T Dimos
    Harvard Stem Cell Institute, Stowers Medical Institute, Department of Stem Cell and Regenerative Biology, Harvard University, Cambridge, MA 02138, USA
    Science 321:1218-21. 2008
    ..These patient-specific iPS cells possess properties of embryonic stem cells and were successfully directed to differentiate into motor neurons, the cell type destroyed in ALS...
  54. pmc Optimised and rapid pre-clinical screening in the SOD1(G93A) transgenic mouse model of amyotrophic lateral sclerosis (ALS)
    Richard J Mead
    Sheffield Institute for Translational Neuroscience, Department of Neuroscience, School of Medicine and Biomedical Sciences, University of Sheffield, Sheffield, United Kingdom
    PLoS ONE 6:e23244. 2011
    ..Such an approach will increase throughput for reduced costs, whilst reducing the severity of the experimental procedures involved...
  55. ncbi Mutations of optineurin in amyotrophic lateral sclerosis
    Hirofumi Maruyama
    Department of Epidemiology, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima 734 8553, Japan
    Nature 465:223-6. 2010
    ..Genes known to cause classic familial ALS (FALS) are superoxide dismutase 1 (SOD1), ANG encoding angiogenin, TARDP encoding transactive response (TAR) DNA-binding protein TDP-43 (..
  56. ncbi Human embryonic stem cell-derived motor neurons are sensitive to the toxic effect of glial cells carrying an ALS-causing mutation
    Francesco Paolo Di Giorgio
    The Harvard Stem Cell Institute, The Stowers Medical Institute, Department of Stem Cell and Regenerative Biology, Cambridge, MA 02138, USA
    Cell Stem Cell 3:637-48. 2008
    ..Our findings demonstrate the relevance of these non-cell-autonomous effects to human motor neurons and more broadly demonstrate the utility of human embryonic stem cells for studying disease and identifying potential therapeutics...
  57. pmc Nrf2 activation in astrocytes protects against neurodegeneration in mouse models of familial amyotrophic lateral sclerosis
    Marcelo R Vargas
    Division of Pharmaceutical Sciences, University of Wisconsin Madison, Madison, Wisconsin 53705 2222, USA
    J Neurosci 28:13574-81. 2008
    ..Dominant mutations in Cu/Zn-superoxide dismutase (SOD1) cause familial forms of amyotrophic lateral sclerosis (ALS), a fatal disorder characterized by the ..
  58. ncbi Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations
    Ian R A Mackenzie
    Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada
    Ann Neurol 61:427-34. 2007
    ..Approximately 10% of cases are familial ALS (FALS), and the most common genetic abnormality is superoxide dismutase-1 (SOD1) mutations...
  59. pmc Increased superoxide in vivo accelerates age-associated muscle atrophy through mitochondrial dysfunction and neuromuscular junction degeneration
    Youngmok C Jang
    Department of Cellular and Structural Biology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78245, USA
    FASEB J 24:1376-90. 2010
    ....
  60. ncbi Skeletal muscle is a primary target of SOD1G93A-mediated toxicity
    Gabriella Dobrowolny
    Institute Pasteur Cenci Bolognetti, Department of Histology and Medical Embryology, CE BEMM and IIM, Sapienza University of Rome, Via A Scarpa, 14 Rome 00161, Italy
    Cell Metab 8:425-36. 2008
    The antioxidant enzyme superoxide dismutase 1 (SOD1) is a critical player of the antioxidative defense whose activity is altered in several chronic diseases, including amyotrophic lateral sclerosis...
  61. pmc Epigenetic changes in mitochondrial superoxide dismutase in the retina and the development of diabetic retinopathy
    Qing Zhong
    Kresge Eye Institute, Wayne State University, Detroit, Michigan, USA
    Diabetes 60:1304-13. 2011
    To investigate the role of epigenetic regulation of the manganese superoxide dismutase gene (sod2) in the development of diabetic retinopathy and the metabolic memory phenomenon associated with its continued progression after ..
  62. pmc Superoxide dismutase in redox biology: the roles of superoxide and hydrogen peroxide
    Garry R Buettner
    Free Radical and Radiation Biology, The University of Iowa, Iowa City, 52242 1181, USA
    Anticancer Agents Med Chem 11:341-6. 2011
    ..It is now realized that superoxide and hydrogen peroxide are essential for normal cellular and organism function. MnSOD appears to be a central player in the redox biology of cells and tissues...
  63. pmc A comprehensive assessment of the SOD1G93A low-copy transgenic mouse, which models human amyotrophic lateral sclerosis
    Abraham Acevedo-Arozena
    MRC Mammalian Genetics Unit, Harwell, OX11 0RD, UK
    Dis Model Mech 4:686-700. 2011
    ..Several causative genes are known and, of these, mutant superoxide dismutase 1 (SOD1) is by far the most frequently found, accounting for up to 20% of familial ALS...
  64. pmc Drusen, choroidal neovascularization, and retinal pigment epithelium dysfunction in SOD1-deficient mice: a model of age-related macular degeneration
    Yutaka Imamura
    Department of Ophthalmology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku ku, Tokyo 160 8582, Japan
    Proc Natl Acad Sci U S A 103:11282-7. 2006
    ..We show that mice deficient in Cu, Zn-superoxide dismutase (SOD1) have features typical of age-related macular degeneration in humans...
  65. pmc Superoxide dismutase is dispensable for normal animal lifespan
    Jeremy Michael Van Raamsdonk
    Department of Biology, McGill University, Montreal, QC, Canada H3A 1B1
    Proc Natl Acad Sci U S A 109:5785-90. 2012
    ..The antioxidant enzyme superoxide dismutase (SOD) is the only eukaryotic enzyme capable of detoxifying superoxide, one type of ROS...
  66. pmc Compartmentalization of superoxide dismutase 1 (SOD1G93A) aggregates determines their toxicity
    Sarah J Weisberg
    Department of Biological Chemistry, Weizmann Institute of Science, Rehovot 76100, Israel
    Proc Natl Acad Sci U S A 109:15811-6. 2012
    ..Preventing the accumulation of SOD1G93A in the JUNQ by enhancing its sequestration in an insoluble inclusion reduces the harmful effects of aggregation on cell viability...
  67. pmc Deficits in axonal transport precede ALS symptoms in vivo
    Lynsey G Bilsland
    Molecular Neuropathobiology Laboratory, Cancer Research UK London Research Institute, London WC2A 3LY, United Kingdom
    Proc Natl Acad Sci U S A 107:20523-8. 2010
    ..Mutations in superoxide dismutase 1 (SOD1) are responsible for a subset of familial cases of ALS...
  68. pmc Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)
    David S Howland
    Department of Molecular Genetics, Wyeth Research, CN8000, Princeton, NJ 08543, USA
    Proc Natl Acad Sci U S A 99:1604-9. 2002
    Transgenic overexpression of Cu(+2)/Zn(+2) superoxide dismutase 1 (SOD1) harboring an amyotrophic lateral sclerosis (ALS)-linked familial genetic mutation (SOD1(G93A)) in a Sprague-Dawley rat results in ALS-like motor neuron disease...
  69. ncbi Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS
    Bradley J Turner
    MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX1 3QX, UK
    Prog Neurobiol 85:94-134. 2008
    Gain-of-function mutations in the Cu,Zn-superoxide dismutase (SOD1) gene are implicated in progressive motor neuron death and paralysis in one form of inherited amyotrophic lateral sclerosis (ALS)...
  70. pmc Manganese superoxide dismutase, MnSOD and its mimics
    Sumitra Miriyala
    Graduate Center for Toxicology, University of Kentucky, Lexington, KY, 40536, USA
    Biochim Biophys Acta 1822:794-814. 2012
    ..This article is part of a Special Issue entitled: Antioxidants and Antioxidant Treatment in Disease...
  71. pmc XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy
    Claudio Hetz
    Institute of Biomedical Sciences, The FONDAP Center for Molecular Studies of the Cell CEMC and the Millennium Nucleus for Neural Morphogenesis NEMO, University of Chile, Santiago, Chile
    Genes Dev 23:2294-306. 2009
    Mutations in superoxide dismutase-1 (SOD1) cause familial amyotrophic lateral sclerosis (fALS)...
  72. pmc Effect of RyhB small RNA on global iron use in Escherichia coli
    Eric Massé
    Laboratory of Molecular Biology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
    J Bacteriol 187:6962-71. 2005
    ..Our results demonstrate the broad effects of a single noncoding RNA on iron homeostasis...
  73. pmc Muscle expression of a local Igf-1 isoform protects motor neurons in an ALS mouse model
    Gabriella Dobrowolny
    Department of Histology and Medical Embryology, CE BEMM and Interuniversity Institute of Myology, University of Rome La Sapienza, 14 00161 Rome, Italy
    J Cell Biol 168:193-9. 2005
    ..These studies establish skeletal muscle as a primary target for the dominant action of inherited SOD1 mutation and suggest that muscle fibers provide appropriate factors, such as mIgf-1, for neuron survival...
  74. ncbi Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosis
    Steve Vucic
    Prince of Wales Medical Research Institute, University of New SouthWales, Sydney, NSW 2031, Australia
    Brain 131:1540-50. 2008
    ....
  75. pmc Extracellular superoxide dismutase protects against pulmonary emphysema by attenuating oxidative fragmentation of ECM
    Hongwei Yao
    Department of Environmental Medicine, Lung Biology and Disease Program, University of Rochester Medical Center, Rochester, NY 14642, USA
    Proc Natl Acad Sci U S A 107:15571-6. 2010
    Extracellular superoxide dismutase (ECSOD or SOD3) is highly expressed in lungs and functions as a scavenger of O(2)(*-)...
  76. ncbi The role of periplasmic antioxidant enzymes (superoxide dismutase and thiol peroxidase) of the Shiga toxin-producing Escherichia coli O157:H7 in the formation of biofilms
    Young Hoon Kim
    Division of Food Science, Korea University, Seoul, South Korea
    Proteomics 6:6181-93. 2006
    This study examined the role of the periplasmic oxidative defense proteins, copper, zinc superoxide dismutase (SodC), and thiol peroxidase (Tpx), from the Shiga toxin-producing Escherichia coli O157:H7 (STEC) in the formation of biofilms...
  77. ncbi microRNA-directed cleavage and translational repression of the copper chaperone for superoxide dismutase mRNA in Arabidopsis
    Linda Beauclair
    Institut Jean Pierre Bourgin, UMR1318 INRA AgroParisTech, INRA Centre de Versailles Grignon, Route de St Cyr, F 78026 Versailles, France
    Plant J 62:454-62. 2010
    ..of mutants impaired in miR398 production, and found that the mRNAs encoding the copper chaperone for superoxide dismutase (CCS1), which delivers copper to CSD1 and CSD2 apoproteins in different cellular compartments, are ..
  78. ncbi Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases
    D Frey
    Friedrich Miescher Institute, CH 4058 Basel, Switzerland
    J Neurosci 20:2534-42. 2000
    ....
  79. ncbi Tanshinone IIA protects cardiac myocytes against oxidative stress-triggered damage and apoptosis
    Jiajia Fu
    Department of Pharmacology and Toxicology, Sun Yat Sen University, Guangzhou, 510080, PR China
    Eur J Pharmacol 568:213-21. 2007
    ..were associated with an increased ratio of Bcl-2 to Bax protein in cardiomyocytes, an elevation of serum superoxide dismutase (SOD) activity and a decrease in serum malondialdehyde (MDA) level...
  80. ncbi Mitochondrial superoxide production and nuclear factor erythroid 2-related factor 2 activation in p75 neurotrophin receptor-induced motor neuron apoptosis
    Mariana Pehar
    Departamento de Neurobiologia Celular y Molecular, Instituto de Investigaciones Biologicas Clemente Estable, Montevideo 11600, Uruguay
    J Neurosci 27:7777-85. 2007
    ..In motor neurons overexpressing the amyotrophic lateral sclerosis (ALS)-linked superoxide dismutase 1(G93A) (SOD1(G93A)) mutation, NGF induced apoptosis even in the absence of an external source of *NO...
  81. pmc Overexpression of SOD in retina: need for increase in H2O2-detoxifying enzyme in same cellular compartment
    Shinichi Usui
    Department of Ophthalmology and Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA
    Free Radic Biol Med 51:1347-54. 2011
    ..rd10(+/+) mice deficient in superoxide dismutase 1 (SOD1) showed increased levels of superoxide radicals and carbonyl adducts (a marker of oxidative damage) ..
  82. ncbi Spinal inhibitory interneuron pathology follows motor neuron degeneration independent of glial mutant superoxide dismutase 1 expression in SOD1-ALS mice
    Mehdi Hossaini
    Department of Neuroscience, Erasmus Medical Centre, Rotterdam, The Netherlands
    J Neuropathol Exp Neurol 70:662-77. 2011
    ..Because mutant superoxide dismutase 1 (SOD1) in glia might contribute to the pathogenesis, we also examined neuron-specific G93A-SOD1 mice; ..
  83. ncbi Evidence for secretion of Cu,Zn superoxide dismutase via exosomes from a cell model of amyotrophic lateral sclerosis
    Catarina Gomes
    Instituto de Tecnologia Quimica e Biologica, Apartado 127, 2781 901, Oeiras, Portugal
    Neurosci Lett 428:43-6. 2007
    ..disease amyotrophic lateral sclerosis (ALS), is caused by dominant mutations in the cytosolic Cu,Zn superoxide dismutase (SOD1). There has been evidence for secretion of SOD1, by an unknown mechanism...
  84. ncbi Undetectable intracellular free copper: the requirement of a copper chaperone for superoxide dismutase
    T D Rae
    Department of Chemistry and Department of Biochemistry, Molecular Biology and Cell Biology, Northwestern University, Evanston, IL 60208, USA
    Science 284:805-8. 1999
    The copper chaperone for the superoxide dismutase (CCS) gene is necessary for expression of an active, copper-bound form of superoxide dismutase (SOD1) in vivo in spite of the high affinity of SOD1 for copper (dissociation constant = 6 fM)..
  85. ncbi Inactivation of human manganese-superoxide dismutase by peroxynitrite is caused by exclusive nitration of tyrosine 34 to 3-nitrotyrosine
    F Yamakura
    Department of Chemistry, Juntendo University School of Medicine, Inba, Chiba 270 16, Japan
    J Biol Chem 273:14085-9. 1998
    ..of a specific tyrosine residue is responsible for inactivation of recombinant human mitochondrial manganese-superoxide dismutase (Mn-SOD) by peroxynitrite...
  86. pmc Moderate exercise attenuates caspase-3 activity, oxidative stress, and inhibits progression of diabetic renal disease in db/db mice
    S Ghosh
    Dept of Anaesthesiology, Pharmacology, and Therapeutics, Faculty of Medicine, Univ of British Columbia, Vancouver, British Columbia, Canada V6T 1Z3
    Am J Physiol Renal Physiol 296:F700-8. 2009
    ..produced higher levels of superoxides and had greater oxidative damage concurrent with downregulation of superoxide dismutase (SOD) 1 and 3...
  87. ncbi Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
    D R Rosen
    Nature 364:362. 1993
  88. pmc Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosis
    David R Beers
    Department of Neurology, Methodist Neurological Institute, Houston, TX 77030, USA
    Proc Natl Acad Sci U S A 103:16021-6. 2006
    ..affecting adult motoneurons, is caused by dominant mutations in the ubiquitously expressed Cu(2+)/Zn(2+) superoxide dismutase (SOD1)...
  89. ncbi Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice
    A M Clement
    Ludwig Institute for Cancer Research, University of California, 9500 Gilman Drive, La Jolla, CA 92093 0670, USA
    Science 302:113-7. 2003
    ..disease affecting adult motor neurons, is caused by dominant mutations in the ubiquitously expressed Cu-Zn superoxide dismutase (SOD1)...
  90. ncbi Potential markers of oxidative stress in stroke
    Antonio Cherubini
    Institute of Gerontology and Geriatrics, University of Perugia Medical School, Policlinico Monteluce Pad E, Via Brunamonti 51, 06122 Perugia, Italy
    Free Radic Biol Med 39:841-52. 2005
    ..Among them ascorbic acid, alpha-tocopherol, uric acid, and superoxide dismutase are related to brain damage and clinical outcome...
  91. ncbi Status of myocardial antioxidants in ischemia-reperfusion injury
    N S Dhalla
    Institute of Cardiovascular Sciences, St Boniface General Hospital Research Centre, 351 Tache Ave, Manitoba, R2H 2A6, Winnipeg, Canada
    Cardiovasc Res 47:446-56. 2000
    ..These changes are considered to be the consequence of imbalance between the formation of oxidants and the availability of endogenous antioxidants in the heart...
  92. ncbi Subcellular distribution of superoxide dismutases (SOD) in rat liver: Cu,Zn-SOD in mitochondria
    A Okado-Matsumoto
    Department of Biochemistry, Duke University Medical Center, Durham, North Carolina 27710, USA
    J Biol Chem 276:38388-93. 2001
    ..Fractions were assayed for both Cu,Zn-superoxide dismutase (SOD) and Mn-SOD by exploiting the cyanide sensitivity of the former activity and by the use of specific ..
  93. ncbi CuZnSOD deficiency leads to persistent and widespread oxidative damage and hepatocarcinogenesis later in life
    Sailaja Elchuri
    Department of Neurology and Neurological Sciences, Stanford University, Stanford, CA 94305, USA
    Oncogene 24:367-80. 2005
    Mice deficient in CuZn superoxide dismutase (CuZnSOD) showed no overt abnormalities during development and early adulthood, but had a reduced lifespan and increased incidence of neoplastic changes in the liver...
  94. ncbi Oxidative stress-related parameters in the liver of non-alcoholic fatty liver disease patients
    Luis A Videla
    Molecular and Clinical Pharmacology Program, Institute of Biomedical Sciences, Faculty of Medicine, University of Chile, Casilla 70058, Santiago 7, Chile
    Clin Sci (Lond) 106:261-8. 2004
    ..by 403% in patients with steatosis (n=15) compared with control values (n=12), whereas glutathione content, superoxide dismutase (SOD) activity and the ferric reducing ability of plasma (FRAP) were decreased by 57%, 48% and 21% (P<0...
  95. ncbi From transcriptome analysis to therapeutic anti-CD40L treatment in the SOD1 model of amyotrophic lateral sclerosis
    John M Lincecum
    ALS Therapy Development Institute, Cambridge, Massachusetts, USA
    Nat Genet 42:392-9. 2010
    ..This work demonstrates that unbiased transcript profiling can identify cellular pathways responsive to therapeutic intervention in a preclinical model of human disease...
  96. pmc Progressive changes in microglia and macrophages in spinal cord and peripheral nerve in the transgenic rat model of amyotrophic lateral sclerosis
    David J Graber
    Department of Pathology, Dartmouth Medical School, One Medical Center Drive, Lebanon, New Hampshire 03756, USA
    J Neuroinflammation 7:8. 2010
    ..The human mutant superoxide dismutase-1 (hmSOD1)-expressing murine transgenic model of ALS has provided some insight into changes in microglia ..
  97. ncbi Accumulation of manganese in Neisseria gonorrhoeae correlates with resistance to oxidative killing by superoxide anion and is independent of superoxide dismutase activity
    H J Tseng
    Department of Microbiology and Parasitology, The University of Queensland, Brisbane, QLD 4072, Australia
    Mol Microbiol 40:1175-86. 2001
    ..This protection was not associated with the superoxide dismutase enzymes of N. gonorrhoeae. In contrast to previous studies, which suggested that some strains of N...
  98. ncbi Survival motor neuron deficiency enhances progression in an amyotrophic lateral sclerosis mouse model
    Bradley J Turner
    MRC Functional Genomics Unit, University of Oxford, Department of Physiology, Anatomy and Genetics, Oxford, UK
    Neurobiol Dis 34:511-7. 2009
    Mutations in the ubiquitously expressed survival motor neuron 1 (SMN1) and superoxide dismutase 1 (SOD1) genes are selectively lethal to motor neurons in spinal muscular atrophy (SMA) and familial amyotrophic lateral sclerosis (ALS), ..
  99. pmc Mice deficient in both Mn superoxide dismutase and glutathione peroxidase-1 have increased oxidative damage and a greater incidence of pathology but no reduction in longevity
    Yiqiang Zhang
    Department of Cellular and Structural Biology, University of Texas Health Science Center at San Antonio, 15355 Lambda Drive, San Antonio, TX 78245, USA
    J Gerontol A Biol Sci Med Sci 64:1212-20. 2009
    ..we generated mice with a combined deficiency in two mitochondrial-localized antioxidant enzymes, Mn superoxide dismutase (MnSOD) and glutathione peroxidase-1 (Gpx-1)...
  100. pmc Extracellular mutant SOD1 induces microglial-mediated motoneuron injury
    Weihua Zhao
    Department of Neurology, Methodist Neurological Institute, The Methodist Research Institute, The Methodist Hospital, Houston, Texas, USA
    Glia 58:231-43. 2010
    Through undefined mechanisms, dominant mutations in (Cu/Zn) superoxide dismutase-1 (mSOD1) cause the non-cell-autonomous death of motoneurons in inherited amyotrophic lateral sclerosis (ALS)...
  101. pmc Deletion of the Trypanosoma brucei superoxide dismutase gene sodb1 increases sensitivity to nifurtimox and benznidazole
    S Radhika Prathalingham
    Department of Infectious and Tropical Diseases, London School of Hygiene and Tropical Medicine, London WC1E 7HT, United Kingdom
    Antimicrob Agents Chemother 51:755-8. 2007
    ..Here, we identify a superoxide dismutase required to protect Trypanosoma brucei from drug-generated superoxide.

Research Grants75

  1. Correction of glutathione deficiency for treatment of diabetic nephropathy
    Paolo Fanti; Fiscal Year: 2013
    ..in GSH metabolism, including GSH-reductase (GSH-R), GSH-peroxidase (GSH-Px), GSH-S-transferases (GST) and superoxide dismutase (SOD)...
  2. Peroxynitrite Decomposition Catalyst and Nitric Oxide Donor for Endotoxemia
    GARRY JOHN SOUTHAN; Fiscal Year: 2012
    ..2) a pyrrolidine nitroxide that acts as a trifunctional catalyst of reactive oxygen species degradation: a superoxide dismutase mimetic, a catalase mimic that detoxifies hydrogen peroxide, and a peroxynitrite decomposition catalyst...
  3. Epigenetic mechanisms relevant to the pathogenesis of ALS
    Neil W Kowall; Fiscal Year: 2013
    ..Many therapies have been tested but no cure or effective therapy is available. Mutations of superoxide dismutase-1 (SOD1) are a cause of familial ALS and it is widely held that oxidative injury is likely to be a major ..
  4. A Hybrid Katp Channel Opener to Prevent Radiocontrast-Induced Nephropathy
    Kanneganti Murthy; Fiscal Year: 2012
    ..of: 1) the non-selective K+-ATP channel opener pinacidil, and 2) a pyrrolidine nitroxide that acts as a superoxide dismutase mimetic, a catalase mimic, and a peroxynitrite decomposition catalyst...
  5. DNA methylation of extracellular superoxide dismutase in pulmonary hypertension
    EVA NOZIK GRAYCK; Fiscal Year: 2012
    ..One critically important antioxidant in the vessel wall is extracellular superoxide dismutase (EC-SOD), the sole enzymatic defense against extracellular O2-...
  6. Nanoparticle Coupled Antioxidants for Respiratory Illness in Veterans
    RODNEY JON SCHLOSSER; Fiscal Year: 2013
    ..These antioxidants include superoxide dismutase (SOD), which converts superoxide, one of the most toxic ROS, to much less reactive peroxide, and catalase (..
  7. Protein Nitration in the Placenta
    Leslie Myatt; Fiscal Year: 2012
    ..The involvement of the mtNOS, eNOS and iNOS isoforms, NOX enzymes and manganese superoxide dismutase in nitration and the role of hypoxia and hypoxia/reoxygenation on protein nitration and the apoptotic ..
  8. REGULATION OF PULMONARY CIRCULATION IN FETUS AND NEWBORN
    Girija G Konduri; Fiscal Year: 2013
    ..The expression of manganese superoxide dismutase (MnSOD) is also decreased in PPHN...
  9. Hybrid Katp Channel Opener and Redox Catalyst for Lung Transplantation
    Prakash Jagtap; Fiscal Year: 2013
    ..channel activating moiety derived from pinacidil, and 2) a pyrrolidine nitroxide domain that acts as superoxide dismutase and catalase mimetics and a peroxynitrite decomposition catalyst...
  10. Conference on Autonomic Regulation of Cardiovascular Function in Health and Disea
    Martin Frank; Fiscal Year: 2012
    ..J. Physiol. 296:R1-R8. 2009) that transfection of the subfornical organ with superoxide dismutase in mice with heart failure, not only reduces sympathetic nerve activity but actually improves cardiac ..
  11. Genetic Studies of Optic Atrophy
    Taosheng Huang; Fiscal Year: 2012
    ..Moreover, we also show that Superoxide dismutase 1 (SOD1), Vitamin E, and genetically overexpressed human SOD1 (hSOD1) is able to reverse the glossy eye ..
  12. A Novel Thioredoxin Mimetic Prodrug for Prevention of Bronchopulmonary Dysplasia
    Prakash Jagtap; Fiscal Year: 2013
    ..this increase in superoxide production is the developmental deficiency of anti-oxidant proteins, such as superoxide dismutase and Trx, that are present in insufficient quantity at an early gestational age...
  13. N-Acetylcysteine and Milk Thistle for Treatment of Diabetic Nephropathy
    Paolo Fanti; Fiscal Year: 2011
    ..in GSH metabolism, including GSH-reductase (GSH-R), GSH-peroxidase (GSH-Px), GSH-S-transferases (GST) and superoxide dismutase (SOD)...
  14. Roles of oxidation and inflammation in aortic stiffening
    David G Harrison; Fiscal Year: 2013
    ..We will accomplish this by using mice we have made that allow us to delete either the extracellular superoxide dismutase (SOD3) or the NADPH oxidase subunit p22phox...
  15. PARP inhibitor and Redox Catalyst Conjugate for Traumatic Brain Injury
    Kanneganti Murthy; Fiscal Year: 2012
    ..polymeras ("PARP") (IC50=20 nM), and 2) a dihydrolipoate ("DHL")-based redox catalyst that is a superoxide dismutase mimetic, a catalase mimetic, and a peroxynitrite decomposition catalyst...
  16. Ca Signaling in Progression of Amyotrophic Lateral Sclerosis in Skeletal Muscle
    Jingsong Zhou; Fiscal Year: 2013
    ..Mutations in the superoxide dismutase (SOD1) gene are linked to most cases of inherited ALS, resulting in impaired metabolic function of the ..
  17. Role of Potent Trophic Factors on Glia and Motor Neurons in ALS
    Brian K Kaspar; Fiscal Year: 2013
    ..studies have surprisingly demonstrated that astrocytes and microglia expressing a mutation in the enzyme superoxide dismutase can exacerbate motor neuron death, supporting earlier studies that ALS is a non-cell autonomous disease...
  18. Brain Delivery of Nanoformulated Superoxide Dismutase
    Erin G Rosenbaugh; Fiscal Year: 2013
    ..studies have shown that adenoviral-mediated overexpression of the O2O- scavenging enzyme copper/zinc superoxide dismutase (CuZnSOD) attenuates central AngII-dependent cardiovascular dysfunction...
  19. INTERACTION OF THE ESTROGEN RECEPTOR WITH DNA
    ANN NARDULLI; Fiscal Year: 2013
    ..we will (1) determine whether estrogen increases expression of the oxidative stress proteins Cu/Zn superoxide dismutase (SOD1), thioredoxin (Trx), thioredoxin reductase (TrxR), protein disulfide isomerase (PDI), and apurinic ..
  20. Mechanism of Irradiation Pulmonary Fibrosis
    Joel S Greenberger; Fiscal Year: 2012
    ..Both migrations are significantly reduced by intrapulmonary manganese superoxide dismutase-plasmid liposome (MnSOD-PL) gene therapy...
  21. The Effects of PP2A on TNF Signaling and Smoke-Induced Lung Injury
    Robert F Foronjy; Fiscal Year: 2013
    ..We have recently published that superoxide dismutase-1 (SOD1), a lung antioxidant, prevents cigarette smoke-induced inflammation and emphysema formation in mice...
  22. Novel pathways for fat burning that protects from high fat diet-induced metabolic
    Sihem Boudina; Fiscal Year: 2013
    ..this, we generated mice with increased superoxide production in adipocytes through deletion of manganese superoxide dismutase (MnSOD), the sole enzyme responsible for detoxifying superoxide to hydrogen peroxide in the mitochondrial ..
  23. Enhance Production of Functional Recombinant Human Protein C Variant In Mammalian
    Nienwen Chow; Fiscal Year: 2010
    ..About 20 percent of familial ALS were caused by genetic mutations in superoxide dismutase 1 (SOD1). Mice expressing SOD1 mutants have been used frequently as models of familial ALS...
  24. Evaluating Novel Multi-kinase Peptide Inhibitors as a Treatment for Rheumatoid Ar
    DAVID MEN HWEI TSAI; Fiscal Year: 2013
    ..In vivo, AX-3 exhibits anti-cancer activity, increases the level of antioxidant enzyme Superoxide Dismutase, and reduces pro-inflammatory factors TNF-alpha, MCP-1, and RANTES...
  25. Nitric Oxide in Pulmonary Hypertension
    Serpil C Erzurum; Fiscal Year: 2013
    ..The HIF-expression is mechanistically linked to the alterations in STAT3, NO and Mn superoxide dismutase (MnSOD)...
  26. Glutathione in mitochondrial dysfunction and disease progression in ALS-models
    Marcelo R Vargas; Fiscal Year: 2011
    ..Among the familial cases, approximately 20% are caused by dominantly inherited mutations in the Cu, Zn superoxide dismutase (SOD1). Rodents overexpressing human mutant SOD1 generally develop an ALS-like phenotype...
  27. Transplantation of Reduced-Size Fatty Livers
    Zhi Zhong; Fiscal Year: 2010
    ..The effects of antioxidants (Ad-superoxide dismutase, N-acetylcysteine and polyphenols) and antinitrative therapies (nitric oxide synthase inhibitors) on ..
  28. A Novel Murine Model of Chronic Inflammatory Periodontitus
    Helieh S Oz; Fiscal Year: 2010
    ..These studies will use wild-type mice (Aim 1) and mice with a deficiency in superoxide dismutase (SOD1) as a major intrinsic molecule that protects against ROS damage...
  29. Inhibitors of S. aureus bNOS for adjunctive therapy
    Donald T Moir; Fiscal Year: 2013
    ..NO in bacterial cells was shown to activate catalase, induce sodA (superoxide dismutase), suppress the Fenton reaction (production of reactive hydroxyl radicals from H2O2), and rescue cells from ..
  30. Reperfusion and Neurovascular Injury in Stroke
    Pak H Chan; Fiscal Year: 2013
    ..and activator of transcription 3 (STAT3) is a transcription activator of the promoter of manganese-superoxide dismutase (SOD2)...
  31. Antenatal betamethasone for persistent pulmonary hypertension of newborn
    Girija G Konduri; Fiscal Year: 2010
    ..A decrease in anti-oxidant signals such as superoxide dismutase (SOD) and nitric oxide (NO) and an increase in pro-oxidant, endothelin peptide (ET-1) occur in pulmonary ..
  32. ALS therapies and genomics for mutant TDP-43 and TLS/FUS
    Don W Cleveland; Fiscal Year: 2010
    ..over the last 15 years have focused on disease caused by mutation in the ubiquitously expressed superoxide dismutase (representing ~2% of all instances of ALS), during the past 14 months, a paradigm shift in understanding ..
  33. Bifunctional Redox Catalyst &Organic Nitrate for Limb Reperfusion
    ANDREW LURIE SALZMAN; Fiscal Year: 2013
    ..of an organic nitrovasodilator that releases nitric oxide, and a pyrrolidine nitroxide that acts as a superoxide dismutase mimetic, catalase mimic, and peroxynitrite decomposition catalyst...
  34. Mitochondrial Metabolism and ROS Regulate Lung Cancer
    Navdeep S Chandel; Fiscal Year: 2013
    ..is hydrogen peroxide (H2O2), which is generated by its conversion from superoxide (O2-) by copper-zinc superoxide dismutase (SOD1) in the cytosol...
  35. The Organ Specific Role of Superoxide Dismutase in Sepsis
    Alix Ashare; Fiscal Year: 2010
    ..b>Superoxide dismutase 2 (SOD2) is the most abundant mitochondrial antioxidant...
  36. Bifunctional Redox Agent for the Treatment of PPHN
    GARRY JOHN SOUTHAN; Fiscal Year: 2011
    ..an organic nitrovasodilator domain that releases NO, and 2) a pyrrolidine nitroxide domain that acts as a superoxide dismutase mimetic, catalase mimetic, and peroxynitrite decomposition catalyst...
  37. Molecular Imaging Probes for Reporting on Vascular Oxidative Response
    Alexei A Bogdanov; Fiscal Year: 2013
    ..We propose to synthesize and test in model systems paramagnetic superoxide dismutase (SOD) mimic-MPO substrates based on chelated paramagnetic Mn(II) and Gd(III), which supply additional ..
  38. Ca2+ mishandling and ischemia-vulnerability in fALS model motor terminals
    Gavriel David; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Mice that transgenically express mutated forms of human superoxide dismutase I (SOD1) that produce familial amyotrophic lateral sclerosis (fALS) develop progressive loss of motor nerve terminals that ..
  39. Role of p62 in Protein Aggregation and Neurodegeneration in ALS
    Haining Zhu; Fiscal Year: 2009
    ..Protein aggregates containing mutant copper-zinc superoxide dismutase (SOD1) are a hallmark of familial amyotrophic lateral sclerosis (ALS, Lou Gehrig's disease), an age-..
  40. Peroxynitrite, nitrotyrosine and HSP90 in neuronal death
    ALVARO G ESTEVEZ; Fiscal Year: 2013
    ..Both inhibitors of nitric oxide synthesis as well as Cu.Zn superoxide dismutase (SOD) delivered intracellularly with liposomes protect motor neurons from apoptosis...
  41. HIGH SALT DIET, ANGIOTENSIN II, AND MICROVESSEL DILATION
    Julian H Lombard; Fiscal Year: 2012
    ..in vascular oxidative stress resulting from down regulation of antioxidant enzymes such as copper/zinc superoxide dismutase (Cu/Zn SOD), and possibly upregulation of pro- oxidant enzymes...
  42. Redox Determinants of Severe Asthma
    Serpil C Erzurum; Fiscal Year: 2010
    ..abnormalities: (1) impaired regulation of airway acidity, (2) increased oxidation and loss of antioxidant superoxide dismutase (SOD) activity, and (3) alterations of nitric oxide (NO) metabolism with loss of beneficial S-nitrosothiols ..
  43. Regulation of caspase-1 by Sod2 in the heart
    Kumuda C Das; Fiscal Year: 2010
    ..Manganese superoxide dismutase (MnSOD) is an antioxidant enzyme located in the mitochondria that provides critical protection against ..
  44. Reactive Oxygen Species Signaling in the Hippocampus
    Eric Klann; Fiscal Year: 2012
    ..We recently have found that scavenging mitochondrial superoxide by overexpression of mitochondrial superoxide dismutase (SOD-2) can prevent the aforementioned abnormalities...
  45. Human Aging, Exercise &FMD: Translational Physiology
    Douglas R Seals; Fiscal Year: 2013
    ..and FMD;4) a reduction in vascular oxidative stress, related in part to an increase in extracellular superoxide dismutase (ecSOD), is an important mechanism by which regular aerobic exercise increases BH4 and NO bioavailability ..
  46. INVESTIGATION OF THE ROLES OF NUCLEAR RECEPTOR FXR IN HEPATOCELLULAR
    Wendong Huang; Fiscal Year: 2013
    ..Specifically, FXR regulates the expression of a superoxide dismutase (EC-SOD, SOD3) and other anti-oxidative stress genes that suppress the deleterious effect of reactive ..
  47. Diabetes Erectile dysfunction and apoptosis of cavernous tissues
    Rajvir Dahiya; Fiscal Year: 2010
    ..in diabetic rats and then analyze apoptotic index, anti-apoptotic genes, pro-apoptotic genes, cytochrome c, superoxide dismutase and smooth muscle differentiation markers...
  48. Defining a clinically relevant time point for astrocyte targeted therapy in ALS
    Brian K Kaspar; Fiscal Year: 2013
    ..Approximately 20% of FALS have mutations within the gene encoding superoxide dismutase 1 (SOD1)...
  49. Transgenic Study of ALS-Linked CCS Mutations
    Han Xiang Deng; Fiscal Year: 2011
    ..Among these genes, mutations in the Cu, Zn-superoxide dismutase gene (SOD1) account for approximately 20% of familial ALS, and represent the most prevalent known cause of ..
  50. Glutathione in mitochondrial dysfunction and disease progression in ALS-models
    Marcelo R Vargas; Fiscal Year: 2013
    ..Among the familial cases, approximately 20% are caused by dominantly inherited mutations in the Cu, Zn superoxide dismutase (SOD1). Rodents overexpressing human mutant SOD1 generally develop an ALS-like phenotype...
  51. Pre-clinical testing of necrostatin as a potential small molecule for the treatme
    SERGE E PRZEDBORSKI; Fiscal Year: 2012
    ..Mutations in superoxide dismutase-1 (SOD1) cause familial ALS and studies in chimeric or conditional mutant mice indicate that non-neuronal ..
  52. Mechanism of Type I and II Caused Diabetic Bladder Dysfunction
    Firouz Daneshgari; Fiscal Year: 2013
    ..of the bladder, and transgenic mice with conditional, smooth muscle-selective deletion of the manganese superoxide dismutase (MnSOD) gene], we will test our hypotheses in two Specific Aims: SA#1- To distinguish the roles of ..
  53. ADENOSINE AND ANTIOXIDANT ENZYMES
    Vickram Ramkumar; Fiscal Year: 1999
    ..antioxidant defense system provides protection of the cell from free radicals and comprises enzymes such as superoxide dismutase (SOD), catalase CAT), glutathione peroxidase (GSH...
  54. Rel B mediated-redox regulation of radiation therapy
    William H St Clair; Fiscal Year: 2013
    ..prostate cancers have high nuclear RelB, a member of the alternative pathway of NF-:B and manganese superoxide dismutase (MnSOD), a critical antioxidant enzyme levels;2) suppression of RelB in androgen independent-aggressive ..
  55. Oxidative damage and cone cell death in RP
    Peter A Campochiaro; Fiscal Year: 2013
    ..reduce oxidative damage and promote cone function and survival, but only if there is co- expression of a superoxide dismutase (SOD) and a H2O2-detoxifying enzyme in mitochondria or in the cytosol...
  56. Proinflammatory enzymes in amytrophic lateral sclerosis
    SERGE E PRZEDBORSKI; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Mutations in superoxide dismutase-1 (SOD1) cause familial amyotrophic lateral sclerosis (ALS)...
  57. Ah Receptor and Endothelin-Dependent Hypertension
    Mary K Walker; Fiscal Year: 2010
    ..Our results will define a novel regulatory pathway for suppression of hypoxia-induced ET- 1, which could be use to develop new therapies for ET-1-dependent cardiovascular diseases. ..
  58. Impact of Insufficient Vascular EC-SOD in Pulmonary Hypertension
    EVA NOZIK GRAYCK; Fiscal Year: 2013
    ..The sole extracellular enzymatic defense against superoxide (O2.-) is the antioxidant extracellular superoxide dismutase (EC-SOD or SOD3)...
  59. PPAR-gamma-mediated neuroprotection against HIV-1 and alcohol CNS injury
    Yuri Persidsky; Fiscal Year: 2009
    ..by decreasing monocyte migration across the BBB (via up-regulation of ROS scavenging enzyme, superoxide dismutase)? And 3) Can PPARgamma agonists diminish neuroinflammation, improve BBB dysfunction and restore impaired ..
  60. Therapeutic Development for Amyotrophic Lateral Sclerosis in a Canine Model
    JOAN RIPLEY COATES; Fiscal Year: 2013
    ..There are no effective therapies for ALS. The discovery of superoxide dismutase (SOD1) mutations as causative for a proportion of the inherited forms of ALS led to the generation of ..
  61. Mitochondria, Superoxide &MnSOD in a fetal hypoxic cerebral palsy model
    Matthew Derrick; Fiscal Year: 2012
    ..of the primary enzymes that removes superoxide one of the major sources of oxidative stress, is manganese superoxide dismutase which is found in the mitochondria The hypothesis is the level of mitochondrial dysfunction and superoxide ..
  62. Novel RNAi therapy for ALS and other Neurodegenerative Disorders
    Lyn Libertine; Fiscal Year: 2011
    ..In a subset of cases, ALS is caused by dominantly transmitted mutations in the gene encoding cytosolic superoxide dismutase (SOD1). Transgenic expression of mutant SOD1 causes ALS in mice...
  63. Role of Cholesterol in Age-related Decline in Steroidogenesis
    Salman Azhar; Fiscal Year: 2013
    ....
  64. LIPID PEROXIDATION IN COLORECTAL CANCER
    Manuela Gago Dominguez; Fiscal Year: 2009
    ..genes, including myeloperoxidase (MPO), endothelial cell nitric oxide synthase (ecNOS), manganese superoxide dismutase (MnSOD), selenium-dependent glutathione peroxidase (GPX1), catalase (CAT), glutathione S- transferase A1 (..
  65. Mitochondrial SOD & Breast Cancer Risk-Mechanism
    JAMES YAGER; Fiscal Year: 2003
    ..Val-9Ala polymorphism affects the secondary structure of the import sequence in mitochondrial manganese superoxide dismutase (MnSOD)...
  66. Nitric Oxide in Pulmonary Hypertension
    Serpil Erzurum; Fiscal Year: 2009
    ..The HIF-expression is mechanistically linked to the alterations in STAT3, NO and Mn superoxide dismutase (MnSOD)...
  67. TRANSGENIC MICE, AGING, CANCER, AND OXIDATIVE DAMAGE
    Arlan Richardson; Fiscal Year: 2001
    ..4. To measure parameters of mitochondrial function (e.g., mitochondrial respiration, hydrogen peroxide generation, and permeability transition) in tissues of wild type, Sod2+/-, and Sod2+/-XGpx1+/-F1 mice at 5, 14 and 26 moths of age. ..
  68. POSTTRANSCRIPTIONAL REGULATION OF ANTIOXIDANT ENZYMES
    LINDA CLERCH; Fiscal Year: 2002
    ..The experiments will test the hypothesis that catalase and manganese superoxide dismutase (MnSOD) RNA 3' and 5' untranslated regions (UTRs) and their corresponding binding proteins affect RNA ..
  69. NITRIC OXIDE/SUPEROXIDE IN LIPID VASCULAR DISEASE
    TIMOTHY O BRIEN; Fiscal Year: 2000
    ..This project is designed to determine the role of endothelial nitric oxide synthase (eNOS) and superoxide dismutase (SOD) in cholesterol-induced vasomotor dysfunction by overexpressing the genes for endothelial eNOS, copper ..
  70. REDUCTION OF MOLECULAR OXYGEN BY NEISSERIA GONORRHOEAE
    ERMINIA NORROD; Fiscal Year: 1991
    ..Aerobic organisms possess protective mechanisms, such as superoxide dismutase, catalase, and peroxidase, and intracellular reductants...
  71. Anti-oxidative gene therapy for liver transplantation
    Jian Wu; Fiscal Year: 2006
    ..To combat these ROS, free radical scavengers, such as superoxide dismutase (SOD) and catalase play crucial roles in the attenuation of hepatic I/R injury...
  72. GLIAL ACTIVATION IN AGED MEMORY IMPAIRED RATS
    Kiminobu Sugaya; Fiscal Year: 1999
    ..Subsequently, if we establish a relationship between memory impairment and glial activation in this animal model, it will be a good tool to develop noel therapeutics based on regulation of glial activity. ..
  73. SUPEROXIDE REGULATION OF FIBROSARCOMA CELL GROWTH
    JUAN MELENDEZ; Fiscal Year: 2001
    ..Preliminary studies conducted by the applicant demonstrate that manganese superoxide dismutase (MnSOD) overexpression, an enzyme which catalyzes the removal of superoxide from the mitochondria, is ..
  74. REGULATION--COPPER-ZINC SUPEROXIDE DISMUTASE EXPRESSION
    Jeffrey Whitsett; Fiscal Year: 1990
    ..This proposal is based on the premise that copper-zinc superoxide dismutase is an important antioxidant because it eliminates superoxide anion and prevents the generation of other ..
  75. SELENIUM AND LUNG CANCER RISK
    Henry Thompson; Fiscal Year: 2004
    ..if levels of oxidative damage in asbestos exposed individuals differ based on polymorphisms in manganese superoxide dismutase (Mn-SOD) and glutathione peroxidase (GPX) that have been associated with lung cancer risk, and assess ..