- 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)...
- 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...
- 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...
- Reactive oxygen species promote TNFalpha-induced death and sustained JNK activation by inhibiting MAP kinase phosphatases
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 ..
- Posttranscriptional induction of two Cu/Zn superoxide dismutase genes in Arabidopsis is mediated by downregulation of miR398 and important for oxidative stress tolerance
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...
- Onset and progression in inherited ALS determined by motor neurons and microglia
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...
- Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
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 ..
- Reactive oxygen species have a causal role in multiple forms of insulin resistance
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...
- Calorie restriction reduces oxidative stress by SIRT3-mediated SOD2 activation
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...
- ALS: a disease of motor neurons and their nonneuronal neighbors
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)...
- Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis
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...
- Superoxide dismutases: role in redox signaling, vascular function, and diseases
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...
- Transformation from a neuroprotective to a neurotoxic microglial phenotype in a mouse model of ALS
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)...
- Topology of superoxide production from different sites in the mitochondrial electron transport chain
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 ..
- Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators
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...
- Dilated cardiomyopathy and neonatal lethality in mutant mice lacking manganese superoxide dismutase
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 ..
- 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)...
- Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons
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...
- 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...
- Sirt3-mediated deacetylation of evolutionarily conserved lysine 122 regulates MnSOD activity in response to stress
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...
- An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1
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...
- Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis
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 ..
- 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)...
- 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
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...
- 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...
- 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...
- 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...
- 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...
- 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...
- 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 ..
- Coupled degradation of a small regulatory RNA and its mRNA targets in Escherichia coli
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...
- 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...
- Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells
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 ..
- 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...
- 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...
- 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...
- The daf-2 gene network for longevity regulates oxidative stress resistance and Mn-superoxide dismutase gene expression in Caenorhabditis elegans
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...
- SOD1 (copper/zinc superoxide dismutase) deficiency drives amyloid β protein oligomerization and memory loss in mouse model of Alzheimer disease
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,..
- Induction of manganese superoxide dismutase by nuclear translocation and activation of SIRT1 promotes cell survival in chronic heart failure
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 ..
- Antioxidant defense and aging in C. elegans: is the oxidative damage theory of aging wrong?
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?..
- 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 ..
- Tumour suppressor SIRT3 deacetylates and activates manganese superoxide dismutase to scavenge ROS
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...
- 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)...
- 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
- 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...
- 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...
- 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...
- 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...
- 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...
- Age-related dysfunction of the lacrimal gland and oxidative stress: evidence from the Cu,Zn-superoxide dismutase-1 (Sod1) knockout mice
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 ..
- 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...
- 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 ..
- 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...
- 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...
- Mutations of optineurin in amyotrophic lateral sclerosis
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 (..
- 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...
- 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 ..
- 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...
- 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
- Skeletal muscle is a primary target of SOD1G93A-mediated toxicity
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...
- Epigenetic changes in mitochondrial superoxide dismutase in the retina and the development of diabetic retinopathy
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 ..
- 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...
- A comprehensive assessment of the SOD1G93A low-copy transgenic mouse, which models human amyotrophic lateral sclerosis
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...
- Drusen, choroidal neovascularization, and retinal pigment epithelium dysfunction in SOD1-deficient mice: a model of age-related macular degeneration
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...
- 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...
- 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...
- 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...
- 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...
- 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)...
- Manganese superoxide dismutase, MnSOD and its mimics
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...
- XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy
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)...
- Effect of RyhB small RNA on global iron use in Escherichia coli
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...
- Muscle expression of a local Igf-1 isoform protects motor neurons in an ALS mouse model
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...
- Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosis
Prince of Wales Medical Research Institute, University of New SouthWales, Sydney, NSW 2031, Australia
Brain 131:1540-50. 2008
- Extracellular superoxide dismutase protects against pulmonary emphysema by attenuating oxidative fragmentation of ECM
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)(*-)...
- 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...
- microRNA-directed cleavage and translational repression of the copper chaperone for superoxide dismutase mRNA in Arabidopsis
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 ..
- Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases
Friedrich Miescher Institute, CH 4058 Basel, Switzerland
J Neurosci 20:2534-42. 2000
- Tanshinone IIA protects cardiac myocytes against oxidative stress-triggered damage and apoptosis
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...
- Mitochondrial superoxide production and nuclear factor erythroid 2-related factor 2 activation in p75 neurotrophin receptor-induced motor neuron apoptosis
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...
- Overexpression of SOD in retina: need for increase in H2O2-detoxifying enzyme in same cellular compartment
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) ..
- Spinal inhibitory interneuron pathology follows motor neuron degeneration independent of glial mutant superoxide dismutase 1 expression in SOD1-ALS mice
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; ..
- Evidence for secretion of Cu,Zn superoxide dismutase via exosomes from a cell model of amyotrophic lateral sclerosis
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...
- 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)..
- Inactivation of human manganese-superoxide dismutase by peroxynitrite is caused by exclusive nitration of tyrosine 34 to 3-nitrotyrosine
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...
- Moderate exercise attenuates caspase-3 activity, oxidative stress, and inhibits progression of diabetic renal disease in db/db mice
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...
- Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
D R Rosen
Nature 364:362. 1993
- 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)...
- 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)...
- Potential markers of oxidative stress in stroke
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...
- 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...
- Subcellular distribution of superoxide dismutases (SOD) in rat liver: Cu,Zn-SOD in mitochondria
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 ..
- CuZnSOD deficiency leads to persistent and widespread oxidative damage and hepatocarcinogenesis later in life
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...
- 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...
- 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...
- 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 ..
- 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...
- 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), ..
- 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
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)...
- Extracellular mutant SOD1 induces microglial-mediated motoneuron injury
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)...
- 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.