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| Cu Zn superoxide dismutaseSummaryGene Symbol: Cu Zn superoxide dismutase Description: superoxide dismutase 1, soluble Alias: ALS, ALS1, IPOA, SOD, hSod1, homodimer, Cu/Zn superoxide dismutase, SOD, soluble, indophenoloxidase A, superoxide dismutase [Cu-Zn], superoxide dismutase, cystolic Species: human Top Publications
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Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosisD R Rosen
Day Neuromuscular Research Laboratory, Massachusetts General Hospital, Charlestown 02129
Nature 362:59-62. 1993Amyotrophic lateral sclerosis (ALS) is a degenerative disorder of motor neurons in the cortex, brainstem and spinal cord. Its cause is unknown and it is uniformly fatal, typically within five years...- Onset and progression in inherited ALS determined by motor neurons and microgliaSeverine 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. 2006Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), a progressive paralytic disease characterized by loss of motor neurons... 
Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosisKoji 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. 2008Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease that is characterized by the loss of motor neurons...- Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALSDaryl A Bosco
Department of Neurology, University of Massachusetts Medical Center, Worcester, Massachusetts, USA
Nat Neurosci 13:1396-403. 2010..In a subset of human sporadic ALS (SALS) cases, motor neurons in the lumbosacral spinal cord were markedly C4F6 immunoreactive, indicating that an .. - Astrocytes from familial and sporadic ALS patients are toxic to motor neuronsAmanda M Haidet-Phillips
The Research Institute at Nationwide Children s Hospital, Columbus, Ohio, USA
Nat Biotechnol 29:824-8. 2011Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease, with astrocytes implicated as contributing substantially to motor neuron death in familial (F)ALS... - Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosisD R Rosen
Nature 364:362. 1993 
Spinal inhibitory interneuron pathology follows motor neuron degeneration independent of glial mutant superoxide dismutase 1 expression in SOD1-ALS miceMehdi Hossaini
Department of Neuroscience, Erasmus Medical Centre, Rotterdam, The Netherlands
J Neuropathol Exp Neurol 70:662-77. 2011Motor neuron degeneration and skeletal muscle denervation are hallmarks of amyotrophic lateral sclerosis (ALS), but other neuron populations and glial cells are also involved in ALS pathogenesis...- Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALSBradley 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..implicated in progressive motor neuron death and paralysis in one form of inherited amyotrophic lateral sclerosis (ALS)... - Non-cell-autonomous effect of human SOD1 G37R astrocytes on motor neurons derived from human embryonic stem cellsMaria C N Marchetto
Laboratory of Genetics, The Salk Institute for Biological Studies, 10010 North Torrey Pines Road, La Jolla, CA 92037, USA
Cell Stem Cell 3:649-57. 2008Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by motor neuron death. ALS can be induced by mutations in the superoxide dismutase 1 gene (SOD1)... - Intermolecular transmission of superoxide dismutase 1 misfolding in living cellsLeslie I Grad
Brain Research Centre, University of British Columbia, Vancouver, BC, Canada V6T 2B5
Proc Natl Acad Sci U S A 108:16398-403. 2011..Expression of the enzymatically inactive, natural familial ALS SOD1 mutations G127X and G85R in human mesenchymal and neural cell lines induces misfolding of wild-type natively .. - Novel antibodies reveal inclusions containing non-native SOD1 in sporadic ALS patientsKarin Forsberg
Department of Medical Biosciences, Umea University, Umea, Sweden
PLoS ONE 5:e11552. 2010Mutations in CuZn-superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS) and are found in 6% of ALS patients. Non-native and aggregation-prone forms of mutant SOD1s are thought to trigger the disease... - The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS)Valeria Crippa
Dipartimento di Endocrinologia, Fisiopatologia e Biologia Applicata Center of Excellence on Neurodegenerative Diseases, CEND, Universita degli Studi di Milano, Milano, Italy
Hum Mol Genet 19:3440-56. 2010Several neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), are characterized by the presence of misfolded proteins, thought to trigger neurotoxicity... - Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosisMakoto Urushitani
Department of Anatomy and Physiology, Laval University, Centre de Recherche du Centre Hospitalier de l Universite Laval, 2705 Boulevard Laurier, Sainte Foy, Quebec G1V 4G2, Canada
Nat Neurosci 9:108-18. 2006..interact with mutant forms of superoxide dismutase (SOD1) that are linked to amyotrophic lateral sclerosis (ALS), but not with wild-type SOD1... - Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS miceA M Clement
Ludwig Institute for Cancer Research, University of California, 9500 Gilman Drive, La Jolla, CA 92093 0670, USA
Science 302:113-7. 2003The most common inherited [correct] form of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease affecting adult motor neurons, is caused by dominant mutations in the ubiquitously expressed Cu-Zn superoxide dismutase (SOD1)... - Mutant superoxide dismutase 1-induced IL-1beta accelerates ALS pathogenesisFelix Meissner
Department of Cellular Microbiology, Max Planck Institute for Infection Biology, 10117 Berlin, Germany
Proc Natl Acad Sci U S A 107:13046-50. 2010b>ALS is a fatal motor neuron disease of adult onset. Neuroinflammation contributes to ALS disease progression; however, the inflammatory trigger remains unclear... - Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human diseaseMercedes Prudencio
Department of Neuroscience, McKnight Brain Institute, University of Florida, Gainesville, FL, USA
Hum Mol Genet 18:3217-26. 2009..in superoxide dismutase 1 (SOD1) have been identified in patients with familial amyotrophic lateral sclerosis (ALS). The mean age of disease onset in patients inheriting mutations in SOD1 is 45-47 years of age... - Monomeric Cu,Zn-superoxide dismutase is a common misfolding intermediate in the oxidation models of sporadic and familial amyotrophic lateral sclerosisRishi Rakhit
Departments of Medical Biophysics and Biochemistry, University of Toronto, Toronto, Ontario M5G 2M9, Canada
J Biol Chem 279:15499-504. 2004..Here, we show that oxidative damage of wild type SOD at physiological concentrations ( approximately 40 microm) results in destabilization and aggregation in vitro... - Absence of pathogenic mutations in VSX1 and SOD1 genes in patients with keratoconusMirna Stabuc-Silih
Eye Clinic, University Medical Centre, Ljubljana, Slovenia
Cornea 29:172-6. 2010..In recent years, several candidate genes, including VSX1 and SOD1, have been proposed and some disease-causing mutations have been identified... - Induction of the unfolded protein response in familial amyotrophic lateral sclerosis and association of protein-disulfide isomerase with superoxide dismutase 1Julie D Atkin
Brain Injury and Repair Group, Howard Florey Institute, University of Melbourne, Parkville, Victoria 3010, USA
J Biol Chem 281:30152-65. 2006..in Cu/Zn superoxide dismutase (SOD1) are linked to motor neuron death in familial amyotrophic lateral sclerosis (ALS) by an unclear mechanism, although misfolded SOD1 aggregates are commonly associated with disease... - Spinal cord mRNA profile in patients with ALS: comparison with transgenic mice expressing the human SOD-1 mutantDaniel Offen
Felsenstein Medical Research Center, Department of Neurology, Rabin Medical Center, Sackler School of Medicine, Tel Aviv University, Petah Tikva, 49100, Israel
J Mol Neurosci 38:85-93. 2009..Further analysis of these genes in hSOD1-G93A transgenic mice revealed increase in the expression in parallel with the deterioration of motor functions .. - Localization of a gene for keratoconus to a 5.6-Mb interval on 13q32Marzena Gajecka
Basic Medical Sciences Program, Washington, Wyoming, Alaska, Montana, and Idaho, Washington State University, Spokane, Washington 99210 1495, USA
Invest Ophthalmol Vis Sci 50:1531-9. 2009..There is also evidence that VSX1 and SOD1 may be involved in the etiology of KTCN. The purpose of this study was to verify the available data and to identify a new keratoconus susceptibility locus... - Superoxide dismutase multigene family: a comparison of the CuZn-SOD (SOD1), Mn-SOD (SOD2), and EC-SOD (SOD3) gene structures, evolution, and expressionIgor 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..SOD1, or CuZn-SOD (EC 1.15.1.1), was the first enzyme to be characterized and is a copper and zinc-containing homodimer that is found almost exclusively in intracellular cytoplasmic spaces. SOD2, or Mn-SOD (EC 1.15.1... - Retinal ganglion cell loss in superoxide dismutase 1 deficiencyKenya Yuki
Laboratory of Retinal Cell Biology, Keio University School of Medicine, Tokyo, Japan
Invest Ophthalmol Vis Sci 52:4143-50. 2011To investigate the influence of deficiency in superoxide dismutase (SOD) 1, a major antioxidative enzyme, on retinal ganglion cells (RGCs). - Variation in the biochemical/biophysical properties of mutant superoxide dismutase 1 enzymes and the rate of disease progression in familial amyotrophic lateral sclerosis kindredsT Ratovitski
Department of Pathology, Johns Hopkins School of Medicine, 558 Ross Building, 720 Rutland Avenue, Baltimore, MD 21205, USA
Hum Mol Genet 8:1451-60. 1999..We conclude that the basis for the different life expectancies of patients in different kindreds of sod1-linked FALS may result from an as yet unidentified property of these mutant enzymes... - Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALSTerrell E Brotherton
Department of Neurology, Emory University, Atlanta, GA 30322, USA
Proc Natl Acad Sci U S A 109:5505-10. 2012..In spinal cords from patients with sporadic ALS, other neurodegenerative diseases, and normal controls, C4F6-immunoreactive inclusions were not detected, but the .. - Dysregulation of astrocyte-motoneuron cross-talk in mutant superoxide dismutase 1-related amyotrophic lateral sclerosisLaura Ferraiuolo
Sheffield Institute for Translational Neuroscience, Department of Neuroscience, Faculty of Medicine, Dentistry and Health University of Sheffield 385A Glossop Road, Sheffield S10 2HQ, UK
Brain 134:2627-41. 2011.... - SOD1: a candidate gene for keratoconusNitin Udar
Jules Stein Eye Institute, University of California Los Angeles, 92868, USA
Invest Ophthalmol Vis Sci 47:3345-51. 2006..To screen superoxide dismutase 1 (SOD1) on chromosome 21 as a possible candidate gene for familial keratoconus (KC)... - Restricted expression of mutant SOD1 in spinal motor neurons and interneurons induces motor neuron pathologyLijun Wang
Department of Neurology MC2030, The University of Chicago Pritzker School of Medicine, 5841 S Maryland Avenue, Chicago, IL 60637, USA
Neurobiol Dis 29:400-8. 2008Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the selective loss of motor neurons (MNs)... - Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial formAaron Kerman
Department of Medical Biophysics, Ontario Cancer Institute, University of Toronto, and Department of Laboratory Medicine and Pathobiology, Toronto General Hospital, TMDT 4 305, 101 College Street, Toronto, ON, M5G 1L7, Canada
Acta Neuropathol 119:335-44. 2010Amyotrophic lateral sclerosis (ALS) is a conformational disease in which misfolding and aggregation of proteins such as SOD1 (familial ALS) and TDP-43 (sporadic ALS) are central features... - Vascular protection: superoxide dismutase isoforms in the vessel wallFrank M Faraci
Department of Internal Medicine, Cardiovascular Center, University of Iowa, Carver College of Medicine, Iowa City, IA 52242 1081, USA
Arterioscler Thromb Vasc Biol 24:1367-73. 2004Blood vessels express 3 isoforms of superoxide dismutase (SOD): cytosolic or copper-zinc SOD (CuZn-SOD), manganese SOD (Mn-SOD) localized in mitochondria, and an extracellular form of CuZn-SOD (EC-SOD)... - Mutation of SOD1 in ALS: a gain of a loss of functionDaniela Sau
Institute of Endocrinology, Center of Excellence on Neurodegenerative Diseases, Division of Human Nutrition, University of Milan, Milan, Italy
Hum Mol Genet 16:1604-18. 2007Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease caused by motoneuron loss... - Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse modelLuc Dupuis
Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Faculte de Medecine, EA3433, Universite Louis Pasteur, 11 rue Humann, 67085 Strasbourg Cedex, France
Proc Natl Acad Sci U S A 101:11159-64. 2004Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease characterized by selective loss of motor neurons and progressive muscle wasting... - An examination of wild-type SOD1 in modulating the toxicity and aggregation of ALS-associated mutant SOD1Mercedes Prudencio
Department of Neuroscience, McKnight Brain Institute, University of Florida, Gainesville, Florida 32610, USA
Hum Mol Genet 19:4774-89. 2010..we demonstrate that the effects of WT SOD1 on the age at which transgenic mice expressing mutant human SOD1 (hSOD1) develop paralysis are influenced by the nature of the ALS mutation and the expression levels of WT hSOD1... - Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALSAdrian Israelson
Ludwig Institute for Cancer Research and Department of Cellular and Molecular Medicine, University of California at San Diego, La Jolla, CA 92093 0670, USA
Neuron 67:575-87. 2010Mutations in superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by loss of motor neurons... - Atomic structures of wild-type and thermostable mutant recombinant human Cu,Zn superoxide dismutaseH E Parge
Department of Molecular Biology, Scripps Research Institute, La Jolla, CA 92037
Proc Natl Acad Sci U S A 89:6109-13. 1992.... - Enhanced transduction of Cu,Zn-superoxide dismutase with HIV-1 Tat protein transduction domains at both terminiWon Sik Eum
Department of Genetic Engineering and Research Institute for Bioscience and Biotechnology, Hallym University, Chunchon 200 702, Korea
Mol Cells 19:191-7. 2005..In a previous study, we showed that Tat-Cu,Zn-superoxide dismutase (Tat-SOD) can be directly transduced into mammalian cells across the lipid membrane barrier... - 50bp deletion in the promoter for superoxide dismutase 1 (SOD1) reduces SOD1 expression in vitro and may correlate with increased age of onset of sporadic amyotrophic lateral sclerosisWendy J Broom
Day Neuromuscular Research Laboratory, Massachusetts General Hospital, Charlestown, Massachusetts 02129, USA
Amyotroph Lateral Scler 9:229-37. 2008..Our findings suggest the hypothesis that this deletion reduces expression of the SOD1 gene and that levels of the SOD1 protein may modify the phenotype of SALS within selected populations... - DJ-1 changes in G93A-SOD1 transgenic mice: implications for oxidative stress in ALSNirit Lev
Laboratory of Neurosciences, Felsenstein Medical Research Center and Department of Neurology, Rabin Medical Center Beilinson Campus, The Sackler School of Medicine, Tel Aviv University, Petah Tikva, 49100, Israel
J Mol Neurosci 38:94-102. 2009Amyotrophic lateral sclerosis (ALS) is a progressive, lethal, neurodegenerative disorder. The causes of ALS are still obscure... - Presymptomatic biochemical changes in hindlimb muscle of G93A human Cu/Zn superoxide dismutase 1 transgenic mouse model of amyotrophic lateral sclerosisKevin H J Park
Department of Paediatrics and Centre for Molecular Medicine and Therapeutics, Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, V5Z 4H4 Canada
Biochim Biophys Acta 1782:462-8. 2008..Here we provide evidence of biochemical changes in the hindlimb muscle of young, presymptomatic G93A hSOD1 transgenic mice... - Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosisBradley J Turner
Motor Neuron Disease Research Laboratory, Brain Injury and Repair Group, Howard Florey Institute of Experimental Physiology and Medicine, University of Melbourne, Victoria 3010, Australia
J Neurosci 25:108-17. 2005..Expression of enhanced green fluorescent protein-tagged mutant human SOD1 (hSOD1-EGFP) in NSC-34 cells induced frequent cytoplasmic inclusions and protein insolubility that correlated with .. - Compartmentalization of superoxide dismutase 1 (SOD1G93A) aggregates determines their toxicitySarah 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... - Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivoSophia T Papadeas
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Proc Natl Acad Sci U S A 108:17803-8. 2011..The SOD1(G93A) astrocyte-induced MN death seemed in part mediated by host microglial activation. These findings show that mSOD1 astrocytes alone can induce WT MN death and associated pathological changes in vivo... - Overexpression of superoxide dismutase 1 protects against beta-amyloid peptide toxicity: effect of estrogen and copper chelatorsFulvio Celsi
Fondazione Santa Lucia IRCCS, Rome, Italy
Neurochem Int 44:25-33. 2004..in Abeta-induced apoptosis in this system; (3) estrogen protects both lines, without significantly affecting SOD activity; and (4) copper chelators prevent Abeta-induced toxicity... - Modification of cysteine 111 in human Cu,Zn-superoxide dismutaseAyako Okado-Matsumoto
Department of Biochemistry, Duke University Medical Center, Durham, NC 27710, USA
Free Radic Biol Med 41:1837-46. 2006Human Cu,Zn-superoxide dismutase (hSOD1) has 4 cysteines per subunit. Cys57 and Cys148 are involved in an intrasubunit disulfide bond, while Cys6 and Cys111 are free... - Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosisKarin Forsberg
Department of Medical Biosciences, Pathology, Umea University, SE 901 85, Umea, Sweden
Acta Neuropathol 121:623-34. 2011The most common cause of amyotrophic lateral sclerosis (ALS) is mutations in superoxide dismutase-1 (SOD1)... - Misfolded superoxide dismutase-1 in CSF from amyotrophic lateral sclerosis patientsPer Zetterström
Department of Medical Biosciences, Clinical Chemistry, Umea University, Umea, Sweden
J Neurochem 117:91-9. 2011Several of the superoxide dismutase-1 (SOD1) mutations linked to amyotrophic lateral sclerosis (ALS) lead to synthesis of structurally defective molecules, suggesting that any cytotoxic conformational species common for all mutations .. - Strategies for stabilizing superoxide dismutase (SOD1), the protein destabilized in the most common form of familial amyotrophic lateral sclerosisJared R Auclair
Department of Chemistry and Rosenstiel Basic Medical Sciences Research Center, Brandeis University, 415 South Street, Waltham, MA 02454, USA
Proc Natl Acad Sci U S A 107:21394-9. 2010Amyotrophic lateral sclerosis (ALS) is a disorder characterized by the death of both upper and lower motor neurons and by 3- to 5-yr median survival postdiagnosis... - Identification of new mutations in the Cu/Zn superoxide dismutase gene of patients with familial amyotrophic lateral sclerosisA Pramatarova
Centre for Research in Neuroscience, McGill University, Montreal
Am J Hum Genet 56:592-6. 1995..Mutations in the Cu/Zn superoxide dismutase gene (SOD 1) are responsible for a fraction of familial ALS (FALS)... - SOD1, ANG, VAPB, TARDBP, and FUS mutations in familial amyotrophic lateral sclerosis: genotype-phenotype correlationsStephanie Millecamps
Centre de Recherche de l Institut du Cerveau et de la Moelle épinière INSERM UMR_S975, CNRS UMR7225, Universite Pierre et Marie Curie Paris 6, Hopital Pitie Salpetriere, Paris, France
J Med Genet 47:554-60. 2010Mutations in SOD1, ANG, VAPB, TARDBP and FUS genes have been identified in amyotrophic lateral sclerosis (ALS). - Copper(2+) binding to the surface residue cysteine 111 of His46Arg human copper-zinc superoxide dismutase, a familial amyotrophic lateral sclerosis mutantH Liu
Department of Chemistry and Biochemistry, Pasarow Mass Spectrometry Laboratory, The Neuropsychiatric Institute, University of California, Los Angeles, CA, USA
Biochemistry 39:8125-32. 2000..Furthermore, we propose that the two cysteine 111 residues, found on opposing subunits of the same dimeric enzyme, may provide a docking location for initial metal insertion during biosynthesis of wild-type CuZnSOD in vivo... - DJ-1 forms complexes with mutant SOD1 and ameliorates its toxicitySatoshi Yamashita
Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Honjo, Kumamoto, Japan
J Neurochem 113:860-70. 2010Mutations in Cu/Zn superoxide dismutase (SOD1) gene cause familial amyotrophic lateral sclerosis (ALS), which could be attributed to the toxic properties of the misfolded protein, oxidative stress, and mitochondrial dysfunction... - Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutaseH X Deng
Department of Neurology, Northwestern University Medical School, Chicago, IL 60611
Science 261:1047-51. 1993Single-site mutants in the Cu,Zn superoxide dismutase (SOD) gene (SOD1) occur in patients with the fatal neurodegenerative disorder familial amyotrophic lateral sclerosis (FALS)... - HIV-1 Tat neurotoxicity: a model of acute and chronic exposure, and neuroprotection by gene delivery of antioxidant enzymesLokesh Agrawal
Department of Pathology, Anatomy and Cell Biology, Thomas Jefferson University, Philadelphia, PA 19107, USA
Neurobiol Dis 45:657-70. 2012.... - NEDL1, a novel ubiquitin-protein isopeptide ligase for dishevelled-1, targets mutant superoxide dismutase-1Kou Miyazaki
Division of Biochemistry, Chiba Cancer Center Research Institute, Chiba 260 8717, Japan
J Biol Chem 279:11327-35. 2004.... - SOD1 haplotypes in familial keratoconusNitin Udar
Department of Ophthalmology, The Eye Institute, University of California Irvine, Orange, CA, USA
Cornea 28:902-7. 2009..The goal of this study was to determine if the 2 families (families A and H) shared a common haplotype by identifying closely linked new microsatellite markers flanking the SOD1 gene... - Morphine modulates HIV-1 gp160-induced murine macrophage and human monocyte apoptosis by disparate waysAditi A Kapasi
Immunology and Inflammation Center, North Shore LIJ Research Institute, The Division of Kidney Disease and Hypertension, Long Island Jewish Medical Center, New Hyde Park, NY 11040, USA
J Neuroimmunol 148:86-96. 2004..On the other hand, free radical scavengers such as superoxide dismutase (SOD), dimethylthiourea (DMTU) and catalase attenuated morphine and gp160-induced human monocyte apoptosis. - Diffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosisBiba R Stanton
Medical Research Council Centre for Neurodegeneration Research and Department of Clinical Neuroscience, Institute of Psychiatry, King s College London, London SE5 8AF, England
Arch Neurol 66:109-15. 2009The basis of heterogeneity in the clinical presentation and rate of progression of amyotrophic lateral sclerosis (ALS) is poorly understood. - Human embryonic stem cell-derived motor neurons expressing SOD1 mutants exhibit typical signs of motor neuron degeneration linked to ALSSaravanan Karumbayaram
Department of Neurology, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA
Dis Model Mech 2:189-95. 2009..the expression of a mutant gene, superoxide dismutase 1(SOD1), linked to familial amyotrophic lateral sclerosis (ALS) in hESC-derived motor neurons... - Structural and dynamic aspects related to oligomerization of apo SOD1 and its mutantsLucia Banci
Magnetic Resonance Center and Department of Chemistry, Fiorgen Foundation, University of Florence, Via Luigi Sacconi 6, 50019 Sesto Fiorentino, Italy
Proc Natl Acad Sci U S A 106:6980-5. 2009..The present results suggest that the investigation of the solution state coupled with that of the crystal state can provide major insights into SOD1 pathway toward oligomerization in relation to fALS... - Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALSCeleste M Karch
Department of Neuroscience, McKnight Brain Institute, University of Florida, Gainesville, FL 32610 0235, USA
Proc Natl Acad Sci U S A 106:7774-9. 2009Transgenic mice that model familial (f)ALS, caused by mutations in superoxide dismutase (SOD)1, develop paralysis with pathology that includes the accumulation of aggregated forms of the mutant protein... - Oxidative stress and metal content in blood and cerebrospinal fluid of amyotrophic lateral sclerosis patients with and without a Cu, Zn-superoxide dismutase mutationYuetsu Ihara
Clinical Research Institute and Department of Neurology, National Hospital Organization Minami Okayama Medical Center, 4066 Hayashima Cho, Tsukubo Gun, Okayama 701 0304, Japan
Neurol Res 27:105-8. 2005Hydroxyl radical, ascorbate free radical, superoxide dismutase (SOD) activities, Cu,Zn-SOD protein, Mn-SOD protein, 8-hydroxy-2' -deoxyguanosine (8-OHdG) and metals were compared in red blood cells (RBC), plasma and/or cerebrospinal .. - Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1Jun ichi Niwa
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai Cho, Showa Ku, Nagoya 466 8500, Japan
J Biol Chem 282:28087-95. 2007Mutations in the Cu/Zn-superoxide dismutase (SOD1) gene cause familial amyotrophic lateral sclerosis (ALS) through the gain of a toxic function; however, the nature of this toxic function remains largely unknown... - Tumor necrosis factor-alpha down-regulates human Cu/Zn superoxide dismutase 1 promoter via JNK/AP-1 signaling pathwayValéry Afonso
INSERM U606, Lariboisiere Hospital, Paris, France
Free Radic Biol Med 41:709-21. 2006..A greater understanding of the mechanisms of TNF-alpha-induced SOD1 repression could facilitate the design and development of novel therapeutic drugs for inflammatory conditions... - Bicarbonate-dependent peroxidase activity of human Cu,Zn-superoxide dismutase induces covalent aggregation of protein: intermediacy of tryptophan-derived oxidation productsHao Zhang
Biophysics Research Institute and Free Radical Research Center, Medical College of Wisconsin, Milwaukee, Wisconsin 53226, USA
J Biol Chem 278:24078-89. 2003..HCO3--dependent peroxidase activity and covalent aggregation of hSOD1WT were mimicked by UV photolysis of hSOD1-WT in the presence of a [Co(NH3)5CO3]+ complex that generates the carbonate radical anion (CO3.)... - Expression of SOD1 G93A or wild-type SOD1 in primary cultures of astrocytes down-regulates the glutamate transporter GLT-1: lack of involvement of oxidative stressMassimo Tortarolo
Biochemical Neuropharmacology Group, GKT Centre for Neuroscience Research, King s College London, Guy s Hospital Campus, London, UK
J Neurochem 88:481-93. 2004..We transfected primary mouse astrocytes to investigate the effect of the FALS-linked mutant hSOD1(G93A) and wild-type SOD1 (hSOD1wt) on the glutamate uptake system... - The endoplasmic reticulum-Golgi pathway is a target for translocation and aggregation of mutant superoxide dismutase linked to ALSMakoto Urushitani
Research Centre of Centre Hospitallier Université de Québec, Department of Anatomy and Physiology, Laval University, Laurier, Quebec, QC, G1V4G2, Canada
FASEB J 22:2476-87. 2008Mutations in superoxide dismutase 1 (SOD1) are responsible for 20% cases of familial amyotrophic lateral sclerosis (ALS). However, the mechanism of motor neuron degeneration caused by ALS-linked SOD1 mutants is not fully understood... - Superoxide dismutase 1 (SOD1) is essential for H2O2-mediated oxidation and inactivation of phosphatases in growth factor signalingJose C Juarez
Attenuon, LLC, San Diego, CA 92121, USA
Proc Natl Acad Sci U S A 105:7147-52. 2008..These results identify SOD1 as a master regulator of GF signaling and as a therapeutic target for the inhibition of angiogenesis and tumor growth... - Superoxide dismutase inhibits the expression of vascular cell adhesion molecule-1 and intracellular cell adhesion molecule-1 induced by tumor necrosis factor-alpha in human endothelial cells through the JNK/p38 pathwaysShing Jong Lin
Institute of Clinical Medicine, Cardiovascular Research Center, National Yang Ming University, Taiwan, Republic of China
Arterioscler Thromb Vasc Biol 25:334-40. 2005..We tried to study possible effects of Cu/Zn superoxide dismutase (SOD) on adhesion molecule expression and its underlying mechanism in the prevention and treatment of cardiovascular .. - Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic miceMarina Mattiazzi
Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, New York 10021, USA
J Biol Chem 277:29626-33. 2002..in the pathogenesis of ALS, we studied mitochondria from transgenic mice expressing wild type and G93A mutated hSOD1. We found that a significant proportion of enzymatically active SOD1 was localized in the intermembrane space of .. - Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neuronsChristian S Lobsiger
Ludwig Institute and Department of Medicine, University of California at San Diego, 9500 Gilman Drive, La Jolla, CA 92093, USA
Proc Natl Acad Sci U S A 104:7319-26. 2007Global, age-dependent changes in gene expression from rodent models of inherited ALS caused by dominant mutations in superoxide-dismutase 1 (SOD1) were identified by using gene arrays and RNAs isolated from purified embryonic and adult .. - Antioxidant enzyme gene delivery to protect from HIV-1 gp120-induced neuronal apoptosisL Agrawal
Department of Pathology, Anatomy, and Cell Biology, Thomas Jefferson University, 1020 Locust Street, Philadelphia, PA 19107, USA
Gene Ther 13:1645-56. 2006..Thus, SOD1 and GPx1 can be delivered by SV40 vectors in vitro or in vivo. This approach may merit consideration for therapies in HIV-1-induced encephalopathy... - Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutationsIan R A Mackenzie
Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada
Ann Neurol 61:427-34. 2007Amyotrophic lateral sclerosis (ALS) is a common, fatal motor neuron disorder with no effective treatment... - Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouseLijun Wang
Department of Neurology, The University of Chicago Pritzker School of Medicine, IL 60637, USA
Hum Mol Genet 18:1642-51. 2009Approximately 10% of amyotrophic lateral sclerosis (ALS) cases are familial (FALS), and approximately 25% of FALS cases are caused by mutations in Cu/Zn superoxide dismutase type 1 (SOD1)... - 9,10-Phenanthraquinone in diesel exhaust particles downregulates Cu,Zn-SOD and HO-1 in human pulmonary epithelial cells: intracellular iron scavenger 1,10-phenanthroline affords protection against apoptosisRika Sugimoto
Majors of Medical Sciences, Graduate School of Comprehensive Human Sciences, University of Tsukuba, 1 1 1 Tennodai, Tsukuba, Ibaraki 305 8575, Japan
Free Radic Biol Med 38:388-95. 2005..cells with 10-20 microM PQ for 12 h specifically down-regulated protein levels of Cu,Zn-superoxide dismutase (Cu,Zn-SOD) and heme oxygenase-1 (HO-1) by more than 50%... - Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosisTimothy M Miller
Ludwig Institute for Cancer Research, La Jolla, CA 92093, USA
Proc Natl Acad Sci U S A 103:19546-51. 2006Amyotrophic lateral sclerosis (ALS) is a fatal, progressive paralysis arising from the premature death of motor neurons. An inherited form is caused by a dominant mutation in the ubiquitously expressed superoxide dismutase (SOD1)... - Metalation of the amyotrophic lateral sclerosis mutant glycine 37 to arginine superoxide dismutase (SOD1) apoprotein restores its structural and dynamical properties in solution to those of metalated wild-type SOD1Lucia Banci
Magnetic Resonance Center, University of Florence, Via Luigi Sacconi 6, 50019 Sesto Fiorentino, Italy
Biochemistry 46:9953-62. 2007..These results suggest further that it is the metal-free apo forms of the mutant SOD1 protein that are the agents of its toxicity... - Familial amyotrophic lateral sclerosis (ALS) in Japan associated with H46R mutation in Cu/Zn superoxide dismutase gene: a possible new subtype of familial ALSM Aoki
Department of Neurology, Tohoku, University School of Medicine, Sendai, Japan
J Neurol Sci 126:77-83. 1994..We identified a novel point mutation A to G in exon 2 of the Cu/Zn SOD gene, resulting in an amino acid substitution of histidine46 by arginine (H46R), in two Japanese familial ALS (FALS).. - Novel mutations that enhance or repress the aggregation potential of SOD1Uma Krishnan
Department of Neurology, University of Texas, Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, Texas, 75390, USA
Mol Cell Biochem 287:201-11. 2006..These results demonstrate that various mutations have different effects on SOD1 aggregation potential and that the H80G mutation appears to uniquely act as a dominant inhibitor of SOD1 aggregation... - Colocalization of 14-3-3 proteins with SOD1 in Lewy body-like hyaline inclusions in familial amyotrophic lateral sclerosis cases and the animal modelYoko Okamoto
Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, Japan
PLoS ONE 6:e20427. 2011..inclusions inside the anterior horn cells of spinal cords with sporadic amyotrophic lateral sclerosis (ALS)... - Human SOD1 before harboring the catalytic metal: solution structure of copper-depleted, disulfide-reduced formLucia Banci
Magnetic Resonance Center, University of Florence, Via Luigi Sacconi 6, 50019 Sesto Fiorentino, Florence, Italy
J Biol Chem 281:2333-7. 2006..The structure allows us to further discuss the copper loading mechanism in SOD1... - Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondriaPiera Pasinelli
Day Laboratory for Neuromuscular Research, Department of Neurology, Harvard Medical School, Mass General Institute for Neurodegenerative Disease, Massachusetts General Hospital, Charlestown, MA 02129, USA
Neuron 43:19-30. 2004Familial amyotrophic lateral sclerosis (ALS)-linked mutations in the copper-zinc superoxide dismutase (SOD1) gene cause motor neuron death in about 3% of ALS cases... - DJ-1 modulates the expression of Cu/Zn-superoxide dismutase-1 through the Erk1/2-Elk1 pathway in neuroprotectionZhiquan Wang
Laboratory of Neurodegenerative Diseases, Institute of Health Science, Shanghai Institutes for Biological Sciences, Chinese Academy of Science and Shanghai Jiao Tong University School of Medicine, Shanghai, China
Ann Neurol 70:591-9. 2011..However, the mechanisms underlying dopaminergic neuron loss related to DJ-1 mutation remain undefined. Therefore, it is important to find the new mechanisms underlying the antioxidative functions of DJ-1... - Superoxide dismutase 1 mutants related to amyotrophic lateral sclerosis induce endoplasmic stress in neuro2a cellsYeo Kyoung Oh
Department of Molecular Biology, Sejong University, Seoul, Korea
J Neurochem 104:993-1005. 2008..In amyotrophic lateral sclerosis (ALS) of both sporadic and familial forms, protein aggregates are found in the affected spinal cords... - Derlin-1 overexpression ameliorates mutant SOD1-induced endoplasmic reticulum stress by reducing mutant SOD1 accumulationAkira Mori
Department of Neurology, Faculty of Life Sciences, Kumamoto University, 1 1 1 Honjo, Kumamoto 860 8556, Japan
Neurochem Int 58:344-53. 2011..sensor kinases, chaperones, and apoptotic mediators, are involved in the familial amyotrophic lateral sclerosis (ALS) model related to mutant Cu/Zn superoxide dismutase (SOD1) and sporadic ALS... - Differential corticospinal tract degeneration in homozygous 'D90A' SOD-1 ALS and sporadic ALSC R V Blain
Department of Clinical Neuroscience, King s College London, Institute of Psychiatry, London, UK
J Neurol Neurosurg Psychiatry 82:843-9. 2011The homogeneous genotype and stereotyped phenotype of a unique familial form of amyotrophic lateral sclerosis (ALS) (patients homozygous for aspartate-to-alanine mutations in codon 90 (homD90A) superoxide dismutase 1) provides an ideal .. - Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondriaHan Xiang Deng
Davee Department of Neurology and Clinical Neurosciences, Northwestern University Feinberg School of Medicine, Tarry Building, Room 13 715, 303 East Chicago Avenue, Chicago, IL 60611, USA
Proc Natl Acad Sci U S A 103:7142-7. 2006Twenty percent of the familial form of amyotrophic lateral sclerosis (ALS) is caused by mutations in the Cu, Zn-superoxide dismutase gene (SOD1) through the gain of a toxic function... - Aberrant neuronal and mitochondrial proteins in hippocampus of transgenic mice overexpressing human Cu/Zn superoxide dismutase 1Joo Ho Shin
Department of Pediatrics, University of Vienna, A 1090, Vienna, Austria
Free Radic Biol Med 37:643-53. 2004..This observation is of relevance to understanding brain deficits in Down syndrome, as SOD1 is encoded on chromosome 21... - ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2Steve Pedrini
Frances and Joseph Weinberg Unit for ALS Research, Farber Institute for Neurosciences, Department of Neuroscience, Thomas Jefferson University, Philadelphia, PA 19107, USA
Hum Mol Genet 19:2974-86. 2010In mutant superoxide dismutase (SOD1)-linked amyotrophic lateral sclerosis (ALS), accumulation of misfolded mutant SOD1 in spinal cord mitochondria is thought to cause mitochondrial dysfunction... - The human G93A-superoxide dismutase-1 mutation, mitochondrial glutathione and apoptotic cell deathH Muyderman
Centre for Neuroscience and Department of Medical Biochemistry, School of Medicine, Flinders University, GPO Box 2100, Adelaide, SA 5001, Australia
Neurochem Res 34:1847-56. 2009..dismutase are a cause of motor neuron death in about 20% of cases of familial amyotrophic lateral sclerosis (ALS)... - "True" sporadic ALS associated with a novel SOD-1 mutationMichael D Alexander
Department of Neurology, Beaumont Hospital, Beaumont Road, Dublin 9, Ireland
Ann Neurol 52:680-3. 2002Mutations in the Cu/Zn superoxide dismutase gene (SOD-1) are reported in 20% of familial amyotrophic lateral sclerosis (ALS) cases, but no definite report of a mutation in a "truly" sporadic case of ALS has been proved... - Evidence from computer simulations for alterations in the membrane biophysical properties and dendritic processing of synaptic inputs in mutant superoxide dismutase-1 motoneuronsSherif M Elbasiouny
Department of Physiology, Northwestern University, Chicago, Illinois 60611, USA
J Neurosci 30:5544-58. 2010A critical step in improving our understanding of the development of amyotrophic lateral sclerosis (ALS) is to identify the factors contributing to the alterations in the excitability of motoneurons and assess their individual .. - Amyotrophic lateral sclerosis-linked mutant SOD1 sequesters Hu antigen R (HuR) and TIA-1-related protein (TIAR): implications for impaired post-transcriptional regulation of vascular endothelial growth factorLiang Lu
Department of Neurology, University of Alabama at Birmingham and the Birmingham Veterans Affairs Medical Center, Birmingham, Alabama 35294, USA
J Biol Chem 284:33989-98. 2009..mouse leads to progressive and selective degeneration of motor neurons similar to amyotrophic lateral sclerosis (ALS)... - The copper chaperone CCS directly interacts with copper/zinc superoxide dismutaseR L Casareno
Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St Louis, Missouri 63110, USA
J Biol Chem 273:23625-8. 1998..Our findings therefore reveal a common mechanism whereby different SOD1 FALS mutants may result in neuronal injury and suggest a novel therapeutic approach in patients affected by this fatal disease... - Novel G16S (GGC-AGC) mutation in the SOD-1 gene in a patient with apparently sporadic young-onset amyotrophic lateral sclerosisJ Kawamata
Department of Neurology, Faculty of Medicine, Kyoto University, Japan
Hum Mutat 9:356-8. 1997 - Overexpression of mutant superoxide dismutase 1 causes a motor axonopathy in the zebrafishRobin Lemmens
Department of Neurology, University Hospital Gasthuisberg, K U Leuven, Belgium
Hum Mol Genet 16:2359-65. 2007..We have investigated the zebrafish as a model to study amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder characterized by the selective loss of motor neurons, caused by mutations in .. - Lack of association between polymorphisms of catalase, copper-zinc superoxide dismutase (SOD), extracellular SOD and endothelial nitric oxide synthase genes and macroangiopathy in patients with type 2 diabetes mellitusO Ukkola
Department of Internal Medicine and Biocenter Oulu, University of Oulu, Finland
J Intern Med 249:451-9. 2001..the polymorphisms of three different antioxidative enzyme - catalase, copper-zinc superoxide dismutase (Cu/ Zn-SOD) and extracellular superoxide dismutase (EC-SOD) - genes and macroangiopathy was examined in patients with type 2 .. - Identification of two novel mutations in the Cu/Zn superoxide dismutase gene with familial amyotrophic lateral sclerosis: mass spectrometric and genomic analysesTakako Sato
Department of Neurology D4, Osaka University Graduate School of Medicine, 2 2 Yamada oka, Suita, Osaka 565 0871, Japan
J Neurol Sci 218:79-83. 2004Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting motor neurons. The majority of patients are sporadic cases, while 5-10% of the patients have a family history of ALS (fALS)... - Cysteine 111 affects aggregation and cytotoxicity of mutant Cu,Zn-superoxide dismutase associated with familial amyotrophic lateral sclerosisMauro Cozzolino
Laboratory of Neurochemistry, Fondazione S Lucia Istituto di Ricovero e Cura a Carattere Scientifico, Rome 00179, Italy
J Biol Chem 283:866-74. 2008..These data are consistent with the view that the redox environment influences the oligomerization/aggregation pathway of mutSOD1 and point to Cys-111 as a key mediator of this process... - Clinical characteristics of familial amyotrophic lateral sclerosis with a Phe20Cys mutation in the SOD1 gene in a Korean familyHyun Young Kim
Department of Neurology, College of Medicine, Hanyang University, Seoul
Amyotroph Lateral Scler 8:73-8. 2007..Five exons of the Cu/Zn SOD gene were analyzed by polymerase chain reaction... - Truncated wild-type SOD1 and FALS-linked mutant SOD1 cause neural cell death in the chick embryo spinal cordGhanashyam D Ghadge
Department of Neurology MC2030, The University of Chicago Pritzker School of Medicine, 5841 S Maryland Avenue, Chicago, IL 60637, USA
Neurobiol Dis 21:194-205. 2006Approximately 10% of amyotrophic lateral sclerosis (ALS) cases are familial (FALS), and approximately 25% of FALS cases are caused by mutations in superoxide dismutase-1 (SOD1)... - A rare Cu/Zn superoxide dismutase mutation causing familial amyotrophic lateral sclerosis with variable age of onset, incomplete penetrance and a sensory neuropathyKourosh Rezania
Department of Neurology, The University of Chicago, 5841 S Maryland Ave MC2030, Chicago, IL 60637, USA
Amyotroph Lateral Scler Other Motor Neuron Disord 4:162-6. 2003..The incomplete disease penetrance seen with this mutation (and others reported in the literature) emphasizes the potential value for obtaining an SOD1 genotype in patients with ALS, even if there is no apparent family history.
Research Grants
- Ca Signaling in Progression of Amyotrophic Lateral Sclerosis in Skeletal MuscleJingsong Zhou; Fiscal Year: 2010Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disorder involving degeneration of motor neurons and atrophy of skeletal muscle...
- SUPEROXIDE DISMUTASE STRUCTURES AND LOU GEHRIGS DISEASEJohn Tainer; Fiscal Year: 2001DESCRIPTION: (applicant's abstract) The metalloenzyme Cu,Zn superoxide dismutase (SOD) is a master eukaryotic regulator of reactive oxygen species within cells by dismutating superoxide anions into peroxide and oxygen...
- SUPEROXIDE DISMUTASE STRUCTURES AND LOU GEHRIGS DISEASEJOHN A contact TAINER; Fiscal Year: 2010Cu,Zn superoxide dismutase (SOD) mutations can cause Familial Amyotrophic Lateral Sclerosis (FALS), an inherited variety of ALS (or Lou Gehrig's disease), the most common human motor neuron disease...
- The Effects of PP2A on TNF Signaling and Smoke-Induced Lung InjuryRobert F Foronjy; Fiscal Year: 2010..This proposal will advance public health by determining how PP2A, the primary eukaryotic serine/threonine phosphatase, alters the injurious responses to cigarette smoke exposure in the lung. ..
- NITRIC OXIDE/SUPEROXIDE IN LIPID VASCULAR DISEASEZVONIMIR KATUSIC; Fiscal Year: 2001..Superoxide anions are scavenged by SOD. This project was designed to determine the role of NO and superoxide in cholesterol-induced vasomotor dysfunction ..
- EXTRACELLULAR SUPEROXIDE DISMUTASE IN PULMONARY FIBROSISTim Oury; Fiscal Year: 2003..The antioxidant enzyme extracellular superoxide dismutase (EC-SOD) is found in high concentrations in association with type I collagen in the lung...
- CHONDROPROTECTIVE EFFECTS OF SUPEROXIDE DISMUTASEConstance Chu; Fiscal Year: 2002..Exploratory studies using extracelluar superoxide dismutase (EC-SOD) knock-out and transgenic mice originally developed for evaluating the pathogenesis of pulmonary fibrosis to ..
- Reactive Oxygen Species Signaling in the HippocampusEric Klann; Fiscal Year: 2010..have found that scavenging mitochondrial superoxide by overexpression of mitochondrial superoxide dismutase (SOD-2) can prevent the aforementioned abnormalities...
- Superoxide Dismutase, Peroxynitrite and ALSJoseph S Beckman; Fiscal Year: 2010..The discovery in 1993 of dominant mutations to Cu,Zn-superoxide dismutase (Cu,Zn-SOD) linked to 2-7% of ALS cases led to hopes that new treatments for ALS based on antioxidants might be forthcoming...
- STRUCTURE AND REDESIGN OF CU-ZN SUPEROXIDE DISMUTASEJohn Tainer; Fiscal Year: 1993..for folding, stability, metal binding, and reactivity of proteins and general and of Cu,Zn superoxide dismutase (SOD) specifically, we propose the renewal of our integrated program of SOD redesign, biochemical characterization, ..
- Folding and Aggregation Mechanisms of Human Cu,Zn Superoxide DismutaseC Robert Matthews; Fiscal Year: 2010..Tragically, mutations at dozens of positions in SOD1 cause amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease...
- SUPEROXIDE DISMUTASE--A NOVEL TARGET FOR CANCER THERAPYPeng Huang; Fiscal Year: 2004DESCRIPTION: Superoxide dismutases (SOD) are a class of essential enzymes responsible for the elimination of the toxic free radical, superoxide (O2-), and thus protect cells from oxidative stress...
- HYDROXYNONENAL MODIFICATION OF SUPEROXIDE DISMUTASEKenneth Hensley; Fiscal Year: 2002..of decreased distance between a key pair of lysine residues (Lys-9) in the interfacial region of the SOD1 homodimer, and that this reaction leads to protein aggregation and metal release from the enzyme...
- REGULATION OF EXTRACELLULAR SUPEROXIDE DISMUTASE BY PROTRussell Bowler; Fiscal Year: 2004..Extracellular superoxide dismutase (EC-SOD) is the primary extracellular antioxidant enzyme; it is uniquely abundant in lung tissue and in vessel walls...