Cu Zn superoxide dismutase

Summary

Gene Symbol: Cu Zn superoxide dismutase
Description: superoxide dismutase 1, soluble
Alias: ALS, ALS1, HEL-S-44, IPOA, SOD, hSod1, homodimer, Cu/Zn superoxide dismutase, SOD, soluble, epididymis secretory protein Li 44, indophenoloxidase A, superoxide dismutase, cystolic
Species: human

Top Publications

  1. ncbi Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
    D R Rosen
    Day Neuromuscular Research Laboratory, Massachusetts General Hospital, Charlestown 02129
    Nature 362:59-62. 1993
  2. doi Spinal inhibitory interneuron pathology follows motor neuron degeneration independent of glial mutant superoxide dismutase 1 expression in SOD1-ALS mice
    Mehdi Hossaini
    Department of Neuroscience, Erasmus Medical Centre, Rotterdam, The Netherlands
    J Neuropathol Exp Neurol 70:662-77. 2011
  3. pmc Astrocytes from familial and sporadic ALS patients are toxic to motor neurons
    Amanda M Haidet-Phillips
    The Research Institute at Nationwide Children s Hospital, Columbus, Ohio, USA
    Nat Biotechnol 29:824-8. 2011
  4. ncbi Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice
    A M Clement
    Ludwig Institute for Cancer Research, University of California, 9500 Gilman Drive, La Jolla, CA 92093 0670, USA
    Science 302:113-7. 2003
  5. ncbi Onset and progression in inherited ALS determined by motor neurons and microglia
    Severine Boillee
    Ludwig Institute for Cancer Research and Departments of Medicine and Neuroscience, University of California at San Diego, 9500 Gilman Drive, La Jolla, CA 92093, USA
    Science 312:1389-92. 2006
  6. pmc Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease
    Mercedes Prudencio
    Department of Neuroscience, McKnight Brain Institute, University of Florida, Gainesville, FL, USA
    Hum Mol Genet 18:3217-26. 2009
  7. ncbi Monomeric Cu,Zn-superoxide dismutase is a common misfolding intermediate in the oxidation models of sporadic and familial amyotrophic lateral sclerosis
    Rishi Rakhit
    Departments of Medical Biophysics and Biochemistry, University of Toronto, Toronto, Ontario M5G 2M9, Canada
    J Biol Chem 279:15499-504. 2004
  8. pmc Mutant superoxide dismutase 1-induced IL-1beta accelerates ALS pathogenesis
    Felix Meissner
    Department of Cellular Microbiology, Max Planck Institute for Infection Biology, 10117 Berlin, Germany
    Proc Natl Acad Sci U S A 107:13046-50. 2010
  9. doi Spinal cord mRNA profile in patients with ALS: comparison with transgenic mice expressing the human SOD-1 mutant
    Daniel 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
  10. doi Localization of a gene for keratoconus to a 5.6-Mb interval on 13q32
    Marzena 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

Detail Information

Publications351 found, 100 shown here

  1. ncbi Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
    D R Rosen
    Day Neuromuscular Research Laboratory, Massachusetts General Hospital, Charlestown 02129
    Nature 362:59-62. 1993
    Amyotrophic 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...
  2. doi Spinal inhibitory interneuron pathology follows motor neuron degeneration independent of glial mutant superoxide dismutase 1 expression in SOD1-ALS mice
    Mehdi Hossaini
    Department of Neuroscience, Erasmus Medical Centre, Rotterdam, The Netherlands
    J Neuropathol Exp Neurol 70:662-77. 2011
    Motor 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...
  3. pmc Astrocytes from familial and sporadic ALS patients are toxic to motor neurons
    Amanda M Haidet-Phillips
    The Research Institute at Nationwide Children s Hospital, Columbus, Ohio, USA
    Nat Biotechnol 29:824-8. 2011
    Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease, with astrocytes implicated as contributing substantially to motor neuron death in familial (F)ALS...
  4. ncbi Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice
    A M Clement
    Ludwig Institute for Cancer Research, University of California, 9500 Gilman Drive, La Jolla, CA 92093 0670, USA
    Science 302:113-7. 2003
    The 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)...
  5. ncbi Onset and progression in inherited ALS determined by motor neurons and microglia
    Severine Boillee
    Ludwig Institute for Cancer Research and Departments of Medicine and Neuroscience, University of California at San Diego, 9500 Gilman Drive, La Jolla, CA 92093, USA
    Science 312:1389-92. 2006
    Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), a progressive paralytic disease characterized by loss of motor neurons...
  6. pmc Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease
    Mercedes 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...
  7. ncbi Monomeric Cu,Zn-superoxide dismutase is a common misfolding intermediate in the oxidation models of sporadic and familial amyotrophic lateral sclerosis
    Rishi Rakhit
    Departments of Medical Biophysics and Biochemistry, University of Toronto, Toronto, Ontario M5G 2M9, Canada
    J Biol Chem 279:15499-504. 2004
    ..aggregates in motor neurons are a key feature of both sporadic and familial amyotrophic lateral sclerosis (ALS)...
  8. pmc Mutant superoxide dismutase 1-induced IL-1beta accelerates ALS pathogenesis
    Felix Meissner
    Department of Cellular Microbiology, Max Planck Institute for Infection Biology, 10117 Berlin, Germany
    Proc Natl Acad Sci U S A 107:13046-50. 2010
    b>ALS is a fatal motor neuron disease of adult onset. Neuroinflammation contributes to ALS disease progression; however, the inflammatory trigger remains unclear...
  9. doi Spinal cord mRNA profile in patients with ALS: comparison with transgenic mice expressing the human SOD-1 mutant
    Daniel 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 ..
  10. doi Localization of a gene for keratoconus to a 5.6-Mb interval on 13q32
    Marzena 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...
  11. pmc Intermolecular transmission of superoxide dismutase 1 misfolding in living cells
    Leslie 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 ..
  12. pmc Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS
    Terrell 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 ..
  13. ncbi Variation in the biochemical/biophysical properties of mutant superoxide dismutase 1 enzymes and the rate of disease progression in familial amyotrophic lateral sclerosis kindreds
    T 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...
  14. doi Retinal ganglion cell loss in superoxide dismutase 1 deficiency
    Kenya Yuki
    Laboratory of Retinal Cell Biology, Keio University School of Medicine, Tokyo, Japan
    Invest Ophthalmol Vis Sci 52:4143-50. 2011
    To investigate the influence of deficiency in superoxide dismutase (SOD) 1, a major antioxidative enzyme, on retinal ganglion cells (RGCs).
  15. ncbi SOD1: a candidate gene for keratoconus
    Nitin 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)...
  16. pmc Superoxide dismutase 1 encoding mutations linked to ALS adopts a spectrum of misfolded states
    Mercedes Prudencio
    Department of Neuroscience, McKnight Brain Institute, University of Florida, Gainesville, FL 32610, USA
    Mol Neurodegener 6:77. 2011
    ....
  17. ncbi Proteasomal inhibition by misfolded mutant superoxide dismutase 1 induces selective motor neuron death in familial amyotrophic lateral sclerosis
    Makoto Urushitani
    Laboratory for Motor System Neurodegeneration, Brain Science Institute BSI, RIKEN, Saitama, Japan
    J Neurochem 83:1030-42. 2002
    ..Furthermore, the treatment of hSOD1-expressing cells with hydrogen peroxide promoted the oligomerization, and detergent-insolubility of mutant SOD1 ..
  18. ncbi Superoxide dismutase multigene family: a comparison of the CuZn-SOD (SOD1), Mn-SOD (SOD2), and EC-SOD (SOD3) gene structures, evolution, and expression
    Igor N Zelko
    Division of Pulmonary and Critical Care, Department of Medicine, Duke University Medical Center, Durham, NC 27710, USA
    Free Radic Biol Med 33:337-49. 2002
    ..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...
  19. doi 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
    ..implicated in progressive motor neuron death and paralysis in one form of inherited amyotrophic lateral sclerosis (ALS)...
  20. ncbi CHIP promotes proteasomal degradation of familial ALS-linked mutant SOD1 by ubiquitinating Hsp/Hsc70
    Makoto Urushitani
    Laboratory for Motor System Neurodegeneration, RIKEN Brain Science Institute, Wako, Saitama, Japan
    J Neurochem 90:231-44. 2004
    ..CHIP was predominantly expressed in spinal neurons, and ubiquitinated inclusions in the spinal motor neurons of hSOD1(G93A) transgenic mice were CHIP-immunoreactive...
  21. pmc Atomic structures of wild-type and thermostable mutant recombinant human Cu,Zn superoxide dismutase
    H E Parge
    Department of Molecular Biology, Scripps Research Institute, La Jolla, CA 92037
    Proc Natl Acad Sci U S A 89:6109-13. 1992
    ....
  22. ncbi Magnetic resonance imaging reveals neuronal degeneration in the brainstem of the superoxide dismutase 1 transgenic mouse model of amyotrophic lateral sclerosis
    Da Wei Zang
    Howard Florey Institute, The University of Melbourne, Parkville, Victoria 3010, Australia
    Eur J Neurosci 20:1745-51. 2004
    ..MRI) is becoming the preferred neuroimaging modality for the diagnosis of human amyotrophic lateral sclerosis (ALS)...
  23. pmc Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis
    Edward Pokrishevsky
    Brain Research Centre, University of British Columbia, Vancouver, Canada
    PLoS ONE 7:e35050. 2012
    Amyotrophic lateral sclerosis (ALS) is incurable and characterized by progressive paralysis of the muscles of the limbs, speech and swallowing, and respiration due to the progressive degeneration of voluntary motor neurons...
  24. pmc Aggregation modulating elements in mutant human superoxide dismutase 1
    Celeste M Karch
    Department of Neuroscience, University of Florida, McKnight Brain Institute, Gainesville, 32610, USA
    Arch Biochem Biophys 503:175-82. 2010
    ..Our data suggest that some type of intermolecular interaction between these elements may occur in promoting mutant SOD1 aggregation...
  25. pmc Hydrogen peroxide induce modifications of human extracellular superoxide dismutase that results in enzyme inhibition
    Randi H Gottfredsen
    Department of Biomedicine, Aarhus University, DK 8000 Aarhus, Denmark
    Redox Biol 1:24-31. 2013
    Superoxide dismutase (EC-SOD) controls the level of superoxide in the extracellular space by catalyzing the dismutation of superoxide into hydrogen peroxide and molecular oxygen...
  26. doi Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mouse model of amyotrophic lateral sclerosis
    Jyothsna Krishnan
    Laboratory of Neurobiology, Campus Gasthuisberg, Leuven, Belgium
    J Neurochem 106:2170-83. 2008
    Amyotrophic lateral sclerosis (ALS) is a chronic, adult-onset neurodegenerative disorder characterized by the selective loss of upper and lower motor neurons, resulting in severe atrophy of muscles and death...
  27. pmc Disulfide scrambling describes the oligomer formation of superoxide dismutase (SOD1) proteins in the familial form of amyotrophic lateral sclerosis
    Keisuke Toichi
    Laboratory for Mechanistic Chemistry of Biomolecules, Department of Chemistry, Keio University, Yokohama, Kanagawa 223 8522, Japan
    J Biol Chem 288:4970-80. 2013
    ..Based upon our results, therefore, scrambling of the conserved disulfide bond will be a key event to cause the pathological changes in disease-associated mutant SOD1 proteins...
  28. pmc Copper,zinc superoxide dismutase is primarily a cytosolic protein in human cells
    J D Crapo
    Department of Medicine, Duke University Medical Center, Durham, NC 27710
    Proc Natl Acad Sci U S A 89:10405-9. 1992
    The intracellular localization of human copper,zinc superoxide dismutase (Cu,Zn-SOD; superoxide:superoxide oxidoreductase, EC 1.15.1.1) was evaluated by using EM immunocytochemistry and both isolated human cell lines and human tissues...
  29. pmc Structural changes to monomeric CuZn superoxide dismutase caused by the familial amyotrophic lateral sclerosis-associated mutation A4V
    Tom Schmidlin
    Department of Biochemistry, University of Washington, Seattle, Washington, USA
    Biophys J 97:1709-18. 2009
    Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron degenerative disease, and the inherited form, familial ALS (fALS), has been linked to over 100 different point mutations scattered throughout the Cu-Zn superoxide ..
  30. doi DJ-1 changes in G93A-SOD1 transgenic mice: implications for oxidative stress in ALS
    Nirit 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. 2009
    Amyotrophic lateral sclerosis (ALS) is a progressive, lethal, neurodegenerative disorder. The causes of ALS are still obscure...
  31. ncbi Overexpression of superoxide dismutase 1 protects against beta-amyloid peptide toxicity: effect of estrogen and copper chelators
    Fulvio 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...
  32. doi DJ-1 forms complexes with mutant SOD1 and ameliorates its toxicity
    Satoshi Yamashita
    Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Honjo, Kumamoto, Japan
    J Neurochem 113:860-70. 2010
    Mutations 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...
  33. ncbi Modification of cysteine 111 in human Cu,Zn-superoxide dismutase
    Ayako Okado-Matsumoto
    Department of Biochemistry, Duke University Medical Center, Durham, NC 27710, USA
    Free Radic Biol Med 41:1837-46. 2006
    Human 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...
  34. pmc Strategies for stabilizing superoxide dismutase (SOD1), the protein destabilized in the most common form of familial amyotrophic lateral sclerosis
    Jared 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. 2010
    Amyotrophic 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...
  35. doi 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 sclerosis
    Wendy 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...
  36. ncbi Enhanced transduction of Cu,Zn-superoxide dismutase with HIV-1 Tat protein transduction domains at both termini
    Won 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...
  37. doi HIV-1 Tat neurotoxicity: a model of acute and chronic exposure, and neuroprotection by gene delivery of antioxidant enzymes
    Lokesh Agrawal
    Department of Pathology, Anatomy and Cell Biology, Thomas Jefferson University, Philadelphia, PA 19107, USA
    Neurobiol Dis 45:657-70. 2012
    ....
  38. pmc Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis
    Karin Forsberg
    Department of Medical Biosciences, Pathology, Umea University, SE 901 85, Umea, Sweden
    Acta Neuropathol 121:623-34. 2011
    The most common cause of amyotrophic lateral sclerosis (ALS) is mutations in superoxide dismutase-1 (SOD1)...
  39. pmc Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo
    Sophia 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...
  40. ncbi Copper(2+) binding to the surface residue cysteine 111 of His46Arg human copper-zinc superoxide dismutase, a familial amyotrophic lateral sclerosis mutant
    H 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...
  41. pmc Presymptomatic biochemical changes in hindlimb muscle of G93A human Cu/Zn superoxide dismutase 1 transgenic mouse model of amyotrophic lateral sclerosis
    Kevin 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...
  42. pmc Compartmentalization of superoxide dismutase 1 (SOD1G93A) aggregates determines their toxicity
    Sarah J Weisberg
    Department of Biological Chemistry, Weizmann Institute of Science, Rehovot 76100, Israel
    Proc Natl Acad Sci U S A 109:15811-6. 2012
    ..Preventing the accumulation of SOD1G93A in the JUNQ by enhancing its sequestration in an insoluble inclusion reduces the harmful effects of aggregation on cell viability...
  43. ncbi NEDL1, a novel ubiquitin-protein isopeptide ligase for dishevelled-1, targets mutant superoxide dismutase-1
    Kou Miyazaki
    Division of Biochemistry, Chiba Cancer Center Research Institute, Chiba 260 8717, Japan
    J Biol Chem 279:11327-35. 2004
    ....
  44. doi SOD1 haplotypes in familial keratoconus
    Nitin 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...
  45. ncbi Morphine modulates HIV-1 gp160-induced murine macrophage and human monocyte apoptosis by disparate ways
    Aditi 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.
  46. doi Diffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosis
    Biba 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. 2009
    The basis of heterogeneity in the clinical presentation and rate of progression of amyotrophic lateral sclerosis (ALS) is poorly understood.
  47. pmc Functional alterations of the ubiquitin-proteasome system in motor neurons of a mouse model of familial amyotrophic lateral sclerosis
    Cristina Cheroni
    Laboratory of Molecular Neurobiology, Department of Neuroscience, Mario Negri Institute for Pharmacological Research, Via La Masa, 19, 20156 Milan, Italy
    Hum Mol Genet 18:82-96. 2009
    In familial and sporadic amyotrophic lateral sclerosis (ALS) and in rodent models of the disease, alterations in the ubiquitin-proteasome system (UPS) may be responsible for the accumulation of potentially harmful ubiquitinated proteins, ..
  48. pmc Dysregulation of astrocyte-motoneuron cross-talk in mutant superoxide dismutase 1-related amyotrophic lateral sclerosis
    Laura 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
    ....
  49. ncbi Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity
    Jun ichi Niwa
    Department of Neurology, Nagoya University Graduate School of Medicine, Showa Ku, Nagoya 466 8550, Japan
    J Biol Chem 277:36793-8. 2002
    Amyotrophic lateral sclerosis (ALS) is a progressive paralytic disorder resulting from the degeneration of motor neurons in the cerebral cortex, brainstem, and spinal cord...
  50. ncbi Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations
    Ian R A Mackenzie
    Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada
    Ann Neurol 61:427-34. 2007
    Amyotrophic lateral sclerosis (ALS) is a common, fatal motor neuron disorder with no effective treatment...
  51. pmc Decreased stability and increased formation of soluble aggregates by immature superoxide dismutase do not account for disease severity in ALS
    Kenrick A Vassall
    Department of Chemistry, Guelph Waterloo Centre for Graduate Studies in Chemistry and Biochemistry, Waterloo, ON, Canada N2L 3G1
    Proc Natl Acad Sci U S A 108:2210-5. 2011
    Protein aggregation is a hallmark of many diseases, including amyotrophic lateral sclerosis (ALS), where aggregation of Cu/Zn superoxide dismutase (SOD1) is implicated in causing neurodegeneration...
  52. pmc A limited role for disulfide cross-linking in the aggregation of mutant SOD1 linked to familial amyotrophic lateral sclerosis
    Celeste M Karch
    Department of Neuroscience, McKnight Brain Institute, SantaFe HealthCare Alzheimer s Disease Research Center, University of Florida, Gainesville, Florida 32611, USA
    J Biol Chem 283:13528-37. 2008
    ..Instead we suggest that these residues participate in other features of the protein that, in some manner, modulate aggregation...
  53. pmc Zinc binding modulates the entire folding free energy surface of human Cu,Zn superoxide dismutase
    Can Kayatekin
    Department of Biochemistry and Molecular Pharmacology, University of Massachusetts Medical School, Worcester, MA 01605, USA
    J Mol Biol 384:540-55. 2008
    Over 100 amino acid replacements in human Cu,Zn superoxide dismutase (SOD) are known to cause amyotrophic lateral sclerosis, a gain-of-function neurodegenerative disease that destroys motor neurons...
  54. ncbi Oxidative stress and metal content in blood and cerebrospinal fluid of amyotrophic lateral sclerosis patients with and without a Cu, Zn-superoxide dismutase mutation
    Yuetsu 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. 2005
    Hydroxyl 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 ..
  55. ncbi Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1
    Jun 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. 2007
    Mutations 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...
  56. ncbi Tumor necrosis factor-alpha down-regulates human Cu/Zn superoxide dismutase 1 promoter via JNK/AP-1 signaling pathway
    Valéry 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...
  57. pmc Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS
    Daryl A Bosco
    Department of Neurology, University of Massachusetts Medical Center, Worcester, Massachusetts, USA
    Nat Neurosci 13:1396-403. 2010
    ..In a subset of human sporadic ALS (SALS) cases, motor neurons in the lumbosacral spinal cord were markedly C4F6 immunoreactive, indicating that an ..
  58. pmc Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria
    Han 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. 2006
    Twenty 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...
  59. doi Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form
    Aaron 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. 2010
    Amyotrophic 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...
  60. pmc Cysteine 111 affects aggregation and cytotoxicity of mutant Cu,Zn-superoxide dismutase associated with familial amyotrophic lateral sclerosis
    Mauro 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...
  61. pmc Superoxide dismutase 1 (SOD1) is essential for H2O2-mediated oxidation and inactivation of phosphatases in growth factor signaling
    Jose 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...
  62. ncbi Bicarbonate-dependent peroxidase activity of human Cu,Zn-superoxide dismutase induces covalent aggregation of protein: intermediacy of tryptophan-derived oxidation products
    Hao 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.)...
  63. ncbi 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 stress
    Massimo 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...
  64. ncbi 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 pathways
    Shing 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 ..
  65. pmc Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse
    Lijun Wang
    Department of Neurology, The University of Chicago Pritzker School of Medicine, IL 60637, USA
    Hum Mol Genet 18:1642-51. 2009
    Approximately 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)...
  66. ncbi Antioxidant enzyme gene delivery to protect from HIV-1 gp120-induced neuronal apoptosis
    L 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...
  67. pmc Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neurons
    Christian 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. 2007
    Global, 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 ..
  68. pmc Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis
    Koji Yamanaka
    Ludwig Institute for Cancer Research and Department of Medicine and Neuroscience, University of California at San Diego, 9500 Gilman Drive, La Jolla, California 92093 0670, USA
    Nat Neurosci 11:251-3. 2008
    Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease that is characterized by the loss of motor neurons...
  69. pmc Identification of new mutations in the Cu/Zn superoxide dismutase gene of patients with familial amyotrophic lateral sclerosis
    A Pramatarova
    Centre for Research in Neuroscience, McGill University, Montreal
    Am J Hum Genet 56:592-6. 1995
    Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder affecting motor neurons. Although most cases of ALS are sporadic, approximately 10% are inherited as an autosomal dominant trait...
  70. ncbi Calorimetric analysis of thermodynamic stability and aggregation for apo and holo amyotrophic lateral sclerosis-associated Gly-93 mutants of superoxide dismutase
    Peter B Stathopulos
    Department of Chemistry, University of Waterloo, Waterloo, Ontario N2L 3G1
    J Biol Chem 281:6184-93. 2006
    ..in thermodynamic stability and aggregation for glycine 93 mutants of human copper, zinc-superoxide dismutase (SOD)...
  71. pmc Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme
    Mercedes Prudencio
    Department of Neuroscience, McKnight Brain Institute, University of Florida, Gainesville, USA
    J Neurochem 108:1009-18. 2009
    ..Mice over-expressing wild-type human SOD1 (WT hSOD1) do not develop amyotrophic lateral sclerosis-like disease, but co-expression of WT enzyme at high levels with ..
  72. pmc Proteins that bind to misfolded mutant superoxide dismutase-1 in spinal cords from transgenic amyotrophic lateral sclerosis (ALS) model mice
    Per Zetterström
    Department of Medical Biosciences, Clinical Chemistry, Umea University, SE 901 85 Umea, Sweden
    J Biol Chem 286:20130-6. 2011
    Mutant superoxide dismutase-1 (SOD1) has an unidentified toxic property that provokes ALS. Several ALS-linked SOD1 mutations cause long C-terminal truncations, which suggests that common cytotoxic SOD1 conformational species should be ..
  73. ncbi Coexistence of dominant and recessive familial amyotrophic lateral sclerosis with the D90A Cu,Zn superoxide dismutase mutation within the same country
    J Khoris
    Department of Neurology B, INSERM EPI 99 30, University Hospital of Montpellier, France
    Eur J Neurol 7:207-11. 2000
    The Cu,Zn superoxide dismutase (Cu,Zn SOD) mutations described in amyotrophic lateral sclerosis (ALS) have, for the most part, a dominant influence...
  74. pmc An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1
    Stefania Guareschi
    Weinberg Unit for ALS Research, Department of Neuroscience, Farber Institute for Neurosciences, Thomas Jefferson University, Philadelphia, PA 19107, USA
    Proc Natl Acad Sci U S A 109:5074-9. 2012
    ..Cu/Zn superoxide dismutase (SOD1) could be pathogenic in both familial and sporadic amyotrophic lateral sclerosis (ALS) through either inheritable or nonheritable modifications...
  75. pmc Different human copper-zinc superoxide dismutase mutants, SOD1G93A and SOD1H46R, exert distinct harmful effects on gross phenotype in mice
    Lei Pan
    Department of Molecular Life Sciences, Tokai University School of Medicine, Isehara, Kanagawa, Japan
    PLoS ONE 7:e33409. 2012
    Amyotrophic lateral sclerosis (ALS) is a heterogeneous group of fatal neurodegenerative diseases characterized by a selective loss of motor neurons in the brain and spinal cord...
  76. pmc Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model
    Luc 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. 2004
    Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease characterized by selective loss of motor neurons and progressive muscle wasting...
  77. doi 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. 2010
    Several neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), are characterized by the presence of misfolded proteins, thought to trigger neurotoxicity...
  78. pmc Disulfide bond as a switch for copper-zinc superoxide dismutase activity in asthma
    Sudakshina Ghosh
    Department of Pathobiology, Lerner Research Institute, Cleveland, OH 44195, USA
    Antioxid Redox Signal 18:412-23. 2013
    Loss of superoxide dismutase (SOD) activity is a defining biochemical feature of asthma. However, mechanisms for the reduced activity are unknown...
  79. ncbi [Superoxyde dismutase 1 gene abnormalities in familial amyotrophic lateral sclerosis: phenotype/genotype correlations. The French experience and review of the literature]
    H F Jafari-Schluep
    Service d Explorations Neurologiques et Epileptologie, Hopital Gui de Chauliac, CHU de Montpellier, Montpellier
    Rev Neurol (Paris) 160:44-50. 2004
    ..SOD1 mutations, the variability of the transmission mode, and the non-exceptional absence of proven causality for ALS. Specific cases where a frequent SOD1 mutation with a recognized causal effect is recognized (no more than 15 out ..
  80. ncbi Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis
    Makoto 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...
  81. pmc Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis
    Timothy M Miller
    Ludwig Institute for Cancer Research, La Jolla, CA 92093, USA
    Proc Natl Acad Sci U S A 103:19546-51. 2006
    Amyotrophic 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)...
  82. ncbi 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 SOD1
    Lucia 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...
  83. ncbi 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 apoptosis
    Rika 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%...
  84. ncbi Familial amyotrophic lateral sclerosis (ALS) in Japan associated with H46R mutation in Cu/Zn superoxide dismutase gene: a possible new subtype of familial ALS
    M Aoki
    Department of Neurology, Tohoku, University School of Medicine, Sendai, Japan
    J Neurol Sci 126:77-83. 1994
    Amyotrophic lateral sclerosis (ALS) is a progressive and fatal neurological disorder that results in relentless damage to the motor neuron system...
  85. ncbi Novel mutations that enhance or repress the aggregation potential of SOD1
    Uma 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...
  86. doi Derlin-1 overexpression ameliorates mutant SOD1-induced endoplasmic reticulum stress by reducing mutant SOD1 accumulation
    Akira 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...
  87. doi DJ-1 modulates the expression of Cu/Zn-superoxide dismutase-1 through the Erk1/2-Elk1 pathway in neuroprotection
    Zhiquan 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...
  88. ncbi Human SOD1 before harboring the catalytic metal: solution structure of copper-depleted, disulfide-reduced form
    Lucia 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...
  89. pmc Colocalization of 14-3-3 proteins with SOD1 in Lewy body-like hyaline inclusions in familial amyotrophic lateral sclerosis cases and the animal model
    Yoko 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)...
  90. doi Superoxide dismutase 1 mutants related to amyotrophic lateral sclerosis induce endoplasmic stress in neuro2a cells
    Yeo 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...
  91. ncbi Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria
    Piera 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. 2004
    Familial amyotrophic lateral sclerosis (ALS)-linked mutations in the copper-zinc superoxide dismutase (SOD1) gene cause motor neuron death in about 3% of ALS cases...
  92. pmc Differential corticospinal tract degeneration in homozygous 'D90A' SOD-1 ALS and sporadic ALS
    C R V Blain
    Department of Clinical Neuroscience, King s College London, Institute of Psychiatry, London, UK
    J Neurol Neurosurg Psychiatry 82:843-9. 2011
    The 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 ..
  93. ncbi Overexpression of mutant superoxide dismutase 1 causes a motor axonopathy in the zebrafish
    Robin 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 ..
  94. ncbi Aberrant neuronal and mitochondrial proteins in hippocampus of transgenic mice overexpressing human Cu/Zn superoxide dismutase 1
    Joo 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...
  95. ncbi "True" sporadic ALS associated with a novel SOD-1 mutation
    Michael D Alexander
    Department of Neurology, Beaumont Hospital, Beaumont Road, Dublin 9, Ireland
    Ann Neurol 52:680-3. 2002
    ..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...
  96. doi The human G93A-superoxide dismutase-1 mutation, mitochondrial glutathione and apoptotic cell death
    H 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)...
  97. doi Evidence from computer simulations for alterations in the membrane biophysical properties and dendritic processing of synaptic inputs in mutant superoxide dismutase-1 motoneurons
    Sherif M Elbasiouny
    Department of Physiology, Northwestern University, Chicago, Illinois 60611, USA
    J Neurosci 30:5544-58. 2010
    A 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 ..
  98. ncbi The copper chaperone CCS directly interacts with copper/zinc superoxide dismutase
    R 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...
  99. pmc ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2
    Steve 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. 2010
    In mutant superoxide dismutase (SOD1)-linked amyotrophic lateral sclerosis (ALS), accumulation of misfolded mutant SOD1 in spinal cord mitochondria is thought to cause mitochondrial dysfunction...
  100. ncbi Novel G16S (GGC-AGC) mutation in the SOD-1 gene in a patient with apparently sporadic young-onset amyotrophic lateral sclerosis
    J Kawamata
    Department of Neurology, Faculty of Medicine, Kyoto University, Japan
    Hum Mutat 9:356-8. 1997
  101. pmc 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 factor
    Liang 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)...

Research Grants85

  1. Glutathione in mitochondrial dysfunction and disease progression in ALS-models
    Marcelo R Vargas; Fiscal Year: 2011
    ..with aggravated mitochondrial pathology commonly observed in transgenic mice overexpressing mutated forms of hSOD1 that retain superoxide dismutase activity...
  2. Peroxynitrite, nitrotyrosine and HSP90 in neuronal death
    ALVARO G ESTEVEZ; Fiscal Year: 2013
    Our long-term goal is to understand how mutations to SOD can increase oxidative stress and cause the death of motor neurons in amyotrophic lateral sclerosis (ALS)...
  3. Glutathione in mitochondrial dysfunction and disease progression in ALS-models
    Marcelo R Vargas; Fiscal Year: 2013
    ..with aggravated mitochondrial pathology commonly observed in transgenic mice overexpressing mutated forms of hSOD1 that retain superoxide dismutase activity...
  4. Nanoparticle Coupled Antioxidants for Respiratory Illness in Veterans
    RODNEY JON SCHLOSSER; Fiscal Year: 2013
    ..These antioxidants include superoxide dismutase (SOD), which converts superoxide, one of the most toxic ROS, to much less reactive peroxide, and catalase (CAT), which ..
  5. Role of Reactive Oxygen Species in B Lymphoma Fate
    Gregory B Carey; Fiscal Year: 2012
    ....
  6. Mitochondrial Diabetes & Manganic Porphyrin Treatment
    Douglas C Wallace; Fiscal Year: 2010
    ..These same cell lines with then be treated with metallopophyrin superoxide dismutase (SOD) mimetics to determine with these catalytic antioxidants can reduce ameliorate the biochemical defect caused by the ..
  7. Mitochondrial SOD as a Target for Diabetic Neuropathy
    EVA LUCILLE FELDMAN; Fiscal Year: 2010
    ..Our studies will identify cellular targets for treatment of DN and have the potential to benefit all patients with diabetes. ..
  8. Role of Cholesterol in Age-related Decline in Steroidogenesis
    Salman Azhar; Fiscal Year: 2013
    ..to mitochondria in adrenal (adrenocortical cells) and Leydig cells isolated from oxidative stress-prone A/T-Mn-SOD-/- (or Mn-SOD+/-), GPX1-/- and Cu,Zn-SOD-/- mice...
  9. Extracellular Matrix Components, Oxidants and Antioxidants in Pulmonary Fibrosis
    CORRINE R KLIMENT; Fiscal Year: 2010
    ..Extracellular superoxide dismutase (EC-SOD) is antioxidant enzyme highly expressed in the lung and has been shown to protect the lung from oxidant-mediated ..
  10. Mitochondrial Oxidative Stress In Angiotensis II Induced Endothelial Dysfunction
    Sergey Dikalov; Fiscal Year: 2013
    ..Indeed, treatment of endothelial cells with mitochondria- targeted SOD mimetic mitoTEMPO or inhibition of mitoKATP channels reduced mitochondrial oxidative stress, improved mitochondrial ..
  11. A Novel Murine Model of Chronic Inflammatory Periodontitus
    Helieh S Oz; Fiscal Year: 2010
    ..Specific aims for this proposal will examine targeted antioxidant therapies using mouse models of oral disease as a potential strategy for adjunctive management of human periodontitis. ..
  12. Ca Signaling in Progression of Amyotrophic Lateral Sclerosis in Skeletal Muscle
    Jingsong Zhou; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disorder involving degeneration of motor neurons and atrophy of skeletal muscle...
  13. Bifunctional Nitric Oxide Donor Refractory to Nitrate Tolerance
    GARRY JOHN SOUTHAN; Fiscal Year: 2012
    ..nitroxide domain that acts as a catalyst of reactive oxygen species degradation: a superoxide dismutase (SOD) mimetic, a catalase mimic that detoxifies hydrogen peroxide, and a peroxynitrite decomposition catalyst...
  14. INVESTIGATION OF THE ROLES OF NUCLEAR RECEPTOR FXR IN HEPATOCELLULAR
    Wendong Huang; Fiscal Year: 2013
    ..Specifically, FXR regulates the expression of a superoxide dismutase (EC-SOD, SOD3) and other anti-oxidative stress genes that suppress the deleterious effect of reactive oxygen species (ROS) ..
  15. White Matter Protection in Acute Ischemic Stroke
    Chia Yi Kuan; Fiscal Year: 2012
    ..By quantitative analysis and outcome comparison among various treatment-groups, these experiments will suggest strategies of WM protection and shed new insights into the mechanisms of WM injury in acute ischemic stroke. ..
  16. The Effects of PP2A on TNF Signaling and Smoke-Induced Lung Injury
    Robert F Foronjy; Fiscal Year: 2013
    ..Importantly, our findings will have important implications for other diseases where TNF signaling and inflammation play a central role. ..
  17. The Nrf2-ARE Pathway in Amyotrophic Lateral Sclerosis
    Jeffrey A Johnson; Fiscal Year: 2013
    ..Some of the inherited forms of ALS are associated with mutations in human Cu/Zn superoxide dismutase 1 (hSOD1)...
  18. Correction of glutathione deficiency for treatment of diabetic nephropathy
    Paolo Fanti; Fiscal Year: 2013
    ..including GSH-reductase (GSH-R), GSH-peroxidase (GSH-Px), GSH-S-transferases (GST) and superoxide dismutase (SOD)...
  19. N-Acetylcysteine and Milk Thistle for Treatment of Diabetic Nephropathy
    Paolo Fanti; Fiscal Year: 2011
    ..including GSH-reductase (GSH-R), GSH-peroxidase (GSH-Px), GSH-S-transferases (GST) and superoxide dismutase (SOD)...
  20. Epigenetic mechanisms relevant to the pathogenesis of ALS
    Neil W Kowall; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Amyotrophic Lateral Sclerosis (ALS) is a fatal disease characterized by progressive paralysis due to motor neuron degeneration...
  21. Does overexpressing Cu/Zn superoxide dismutase retard aging in rodents
    Arlan G Richardson; Fiscal Year: 2013
    ....
  22. Reactive Oxygen Species Signaling in the Hippocampus
    Eric Klann; Fiscal Year: 2012
    ..have found that scavenging mitochondrial superoxide by overexpression of mitochondrial superoxide dismutase (SOD-2) can prevent the aforementioned abnormalities...
  23. Stroke Therapy
    VINOD D LABHASETWAR; Fiscal Year: 2013
    ..Our preliminary studies show that nanoparticle-mediated delivery of the antioxidant enzyme superoxide dismutase (SOD) to the brain mitigates the effects of ROS, protects the BBB, and significantly reduces reperfusion injury in ..
  24. Impact of Insufficient Vascular EC-SOD in Pulmonary Hypertension
    EVA NOZIK GRAYCK; Fiscal Year: 2013
    ..is the antioxidant extracellular superoxide dismutase (EC-SOD or SOD3)...
  25. Radiation and Oxidative Stress: Effects on Neurogenesis
    John R Fike; Fiscal Year: 2012
    ..The superoxide dismutase (SOD) isoforms mitigate the physiological and pathological effects of ROS...
  26. Skeletal Muscle Mechanisms of Disease in ALS
    Lee J Martin; Fiscal Year: 2013
    ..Many different SOD1 mutations occur, but the mechanisms of human SOD1 (hSOD1) toxicity to MNs are unresolved...
  27. Interaction Between DNA Repair and Antioxidant Genes During Aging
    Carlos A Torres Ramos; Fiscal Year: 2010
    ..of defense is represented by the antioxidant defense system, which includes among others, the superoxide dismutase (SOD) enzymes. The second line of defense is DNA repair, particularly base excision repair (BER)...
  28. Activation Mechanism of Cu/Zn Superoxide Dimutase
    JEFFREY LEITCH; Fiscal Year: 2010
    ..However, dominate, toxic gain of function mutations in SOD1 can lead to amyotrophic lateral sclerosis (ALS), or Lou Gehrigs disease...
  29. INTERACTION OF THE ESTROGEN RECEPTOR WITH DNA
    ANN NARDULLI; Fiscal Year: 2013
    ..Our studies will provide critical new information about the role of a variety of estrogens and progestins in the brain and aid in the design of more effective hormonal treatments for women of all ages. ..
  30. Antenatal betamethasone for persistent pulmonary hypertension of newborn
    Girija G Konduri; Fiscal Year: 2010
    ..A decrease in anti-oxidant signals such as superoxide dismutase (SOD) and nitric oxide (NO) and an increase in pro-oxidant, endothelin peptide (ET-1) occur in pulmonary arteries in ..
  31. HIGH SALT DIET, ANGIOTENSIN II, AND MICROVESSEL DILATION
    Julian H Lombard; Fiscal Year: 2012
    ..stress resulting from down regulation of antioxidant enzymes such as copper/zinc superoxide dismutase (Cu/Zn SOD), and possibly upregulation of pro- oxidant enzymes...
  32. Redox control of CD8 T cell proliferation, differentiation and death
    JASON MITCHELL GRAYSON; Fiscal Year: 2012
    ..P14 TCR transgenic mice that overexpress both catalase and Cu superoxide dismutase (CAT/SOD) or lack peroxiredoxin II (Prx II -/-) will be used...
  33. Genetic Studies of Optic Atrophy
    Taosheng Huang; Fiscal Year: 2012
    ..Moreover, we also show that Superoxide dismutase 1 (SOD1), Vitamin E, and genetically overexpressed human SOD1 (hSOD1) is able to reverse the glossy eye phenotype of dOPA1 mutant large clones, further suggesting that ROS play an ..
  34. Fe porphyrin-based Redox Modulator in Experimental Stroke Outcome
    Ines Batinic-Haberle; Fiscal Year: 2013
    ..The potency of these compounds approaches that of SOD enzymes...
  35. Linking Spatial Variations in Shear Stress with Oxidation Stress
    Tzung K Hsiai; Fiscal Year: 2013
    ..production via an up-regulation in Mn-SOD activities. In contrast, atherogenic hemodynamics and oxidized LDL induced mitochondrial O2...
  36. GENETIC DETERMINANTS OF OXYGEN TOXICITY
    Valeria C Culotta; Fiscal Year: 2013
    ..We identified the SODl disulfide as the target for redox control of SOD], but how the disulfide is oxidized under hypoxic conditions without CCS is not clear...
  37. The role of ECSODF in lung repair after silica-induces injury
    Cheryl L Fattman; Fiscal Year: 2012
    ..ECSOD overexpression or treatment with SOD-mimetic drugs can prevent this injury...
  38. Superoxide Dismutase, Peroxynitrite and ALS
    Joseph S Beckman; Fiscal Year: 2012
    ..The discovery in 1993 of dominant mutations to Cu,Zn-superoxide dismutase (Cu,Zn- SOD) linked to 2% of ALS cases led to hopes that new treatments for ALS based on antioxidants might be forthcoming...
  39. Ca2+ mishandling and ischemia-vulnerability in fALS model motor terminals
    Gavriel David; Fiscal Year: 2012
    ..Treatments to preserve remaining motor nerve terminals should slow the progression of paralysis, and thus may become an important new component of therapies for treating ALS.
  40. DNA methylation of extracellular superoxide dismutase in pulmonary hypertension
    EVA NOZIK GRAYCK; Fiscal Year: 2012
    ..One critically important antioxidant in the vessel wall is extracellular superoxide dismutase (EC-SOD), the sole enzymatic defense against extracellular O2-...
  41. Enhance Production of Functional Recombinant Human Protein C Variant In Mammalian
    Nienwen Chow; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): Amyotrophic lateral sclerosis (ALS) is a motor neuron disorder characterized by the degeneration and death of motor neurons. Currently there is no cure for ALS. Most ALS cases are sporadic...
  42. Brain Delivery of Nanoformulated Superoxide Dismutase
    Erin G Rosenbaugh; Fiscal Year: 2013
    ..glycol (PEI-PEG) polymer to form a stable polyion complex, so-called CuZnSOD nanozyme (SOD-nano)...
  43. Role of p62 in Protein Aggregation and Neurodegeneration in ALS
    Haining Zhu; Fiscal Year: 2009
    ..supplement to our NIH grant R21AG032567 entitled "Role of p62 in Protein Aggregation and Neurodegeneration in ALS." Protein aggregates containing mutant copper-zinc superoxide dismutase (SOD1) are a hallmark of familial ..
  44. Pre-clinical testing of necrostatin as a potential small molecule for the treatme
    SERGE E PRZEDBORSKI; Fiscal Year: 2012
    ..necrostatin-1 (Nec-1) as a potential neuroprotective agent for the treatment of amyotrophic lateral sclerosis (ALS), an incurable fatal paralytic disorder...
  45. Defining a clinically relevant time point for astrocyte targeted therapy in ALS
    Brian K Kaspar; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Amyotrophic lateral sclerosis (ALS) is one of the most common neuromuscular diseases worldwide...
  46. Transgenic Study of ALS-Linked CCS Mutations
    Han Xiang Deng; Fiscal Year: 2011
    DESCRIPTION (provided by applicant): Amyotrophic lateral sclerosis (ALS) is a progressive paralytic disorder caused by degeneration of motor neurons in the brain and spinal cord...
  47. Mitochondrial Metabolism and ROS Regulate Lung Cancer
    Navdeep S Chandel; Fiscal Year: 2013
    ....
  48. Proinflammatory enzymes in amytrophic lateral sclerosis
    SERGE E PRZEDBORSKI; Fiscal Year: 2012
    ..provided by applicant): Mutations in superoxide dismutase-1 (SOD1) cause familial amyotrophic lateral sclerosis (ALS)...
  49. Therapeutic Development for Amyotrophic Lateral Sclerosis in a Canine Model
    JOAN RIPLEY COATES; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Amyotrophic lateral sclerosis (ALS) is characterized by loss of motor neurons resulting in stiffness, slowing of movement, and severe muscle wasting and weakness...
  50. Novel RNAi therapy for ALS and other Neurodegenerative Disorders
    Lyn Libertine; Fiscal Year: 2011
    DESCRIPTION (provided by applicant): Project Summary / Abstract Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons in the central nervous system...
  51. Local Circuit Properties of Mouse Corticospinal Neurons
    Patrick L Sheets; Fiscal Year: 2011
    ..including paralysis/paraplegia, epilepsy, and movement disorders including amyotrophic lateral sclerosis (ALS), a disease affecting upper motor neurons...
  52. Molecular Mechanisms of SOD1-linked ALS (P01)
    Joan Selverstone Valentine; Fiscal Year: 2013
    ..Finally, ALS tissue will be used as a source to validate the findings and test new hypotheses...
  53. Phase II/III Study of Arimoclomol in the Treatment of SOD1 Positive Familial?.
    Merit E Cudkowicz; Fiscal Year: 2013
    ..progressive forms of familial amyotrophic lateral sclerosis (ALS) due to mutations in the superoxide dismutase-1 (SOD-1) gene...
  54. RNA-Targeted Dysregulation of Survival Factors in ALS: HuR to the Rescue
    Peter H King; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Amyotrophic lateral sclerosis (ALS) is a degenerative disease of motor neurons that inexorably leads to progressive weakness and death...
  55. Redox Determinants of Severe Asthma
    Serpil C Erzurum; Fiscal Year: 2010
    ..1) impaired regulation of airway acidity, (2) increased oxidation and loss of antioxidant superoxide dismutase (SOD) activity, and (3) alterations of nitric oxide (NO) metabolism with loss of beneficial S-nitrosothiols and ..
  56. Regulation of caspase-1 by Sod2 in the heart
    Kumuda C Das; Fiscal Year: 2010
    ..The outcome of these studies may help in the therapeutic use of MnSOD or substances that induce MnSOD in the protection of myocardial injury in heart failure, endothelial dysfunction or other cardiovascular diseases. ..
  57. The Role of the Intrinsic Apoptotic Pathway in Familial ALS
    Nichole Reyes; Fiscal Year: 2010
    ..affecting our aging population, including Alzheimer disease, Parkinson disease, and Amyotrophic Lateral Sclerosis (ALS)...
  58. Oxidative damage and cone cell death in RP
    Peter A Campochiaro; Fiscal Year: 2013
    ..damage and promote cone function and survival, but only if there is co- expression of a superoxide dismutase (SOD) and a H2O2-detoxifying enzyme in mitochondria or in the cytosol...
  59. High Throughput Screening for Compounds to Mitigate Toxicity of FUS/TLS &SOD1
    Robert H Brown; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Amyotrophic lateral sclerosis (ALS) is an untreatable, paralytic neurodegenerative disorder that is uniformly lethal, usually within 3-5 years...
  60. Molecular library screen for suppressors of FUS proteinopathy
    Frank P Shewmaker; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Amyotrophic lateral sclerosis (ALS, motor neuron or Lou Gehrig's disease) and frontotemporal lobar degeneration (FTLD) are fatal neurodegenerative disorders that are associated with the neural ..
  61. Dysregulation of VEGF RNA Stability in Amyotrophic Lateral Sclerosis
    Liang Lu; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Project Summary: Amyotrophic lateral sclerosis (ALS) is a devastating disease...
  62. Cytoprotective mechanism against misfolded SOD1-induced toxicity
    CHERYL HO YUNG; Fiscal Year: 2013
    Amyotrophic lateral sclerosis (ALS) is a progressive neurological disease characterized by selective motor neuron death and accumulation of insoluble proteinaceous deposits in surviving motor neurons...
  63. UNDERSTANDING THE ROLE OF MITOCHONDRIAL IMPORT IN AMYOTROPHIC LATERAL SCLEROSIS
    Matthew Crisp; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the loss of motor neurons, ultimately leading to paralysis and death in three to five years after onset...
  64. Investigation of post-translational modifications in WT SOD1 in sporadic ALS
    DARYL ANGELA BOSCO; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is the most common motor neuron disease...
  65. Mechanisms of SOD1 toxicity in ALS
    Jeffrey L Elliott; Fiscal Year: 2012
    ..of the proposed aims in this grant will provide insights into the cellular and molecular mechanisms underlying one form of familial amyotrophic lateral sclerosis (ALS) related to mutations in the copper, zinc superoxide dismutase gene.
  66. The impact of post-translational modification on SOD1 aggregation in ALS
    RACHEL REDLER; Fiscal Year: 2013
    ..The first specific aim will focus on the first step in oligomer formation, dissociation of the native SOD1 homodimer. Crystal structures of modified SOD1 will be solved and assessed for any perturbations in the dimeric structure...
  67. Neurodegeneration and Proteotoxicity Dissected in C. elegans and Mammals
    Jiou Wang; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by the degeneration of motor neurons...
  68. Nascent SOD1 in Amyotrophic Lateral Sclerosis
    PETER JOHN HART; Fiscal Year: 2013
    ..dismutase (SOD1) cause an inherited form of the fatal neurodegenerative disease amyotrophic lateral sclerosis (ALS, Lou Gehrig's disease, motor neuron disease)...
  69. X-RAY STUDIES OF SOD IN AMYOTROPHIC LATERAL SCLEROSIS
    PETER JOHN HART; Fiscal Year: 2010
    ..single site mutations in human SOD1 have been linked to an inherited form of amyotrophic lateral sclerosis (ALS, Lou Gehrig's disease, motor neuron disease)...
  70. SUPEROXIDE DISMUTASE STRUCTURES AND LOU GEHRIGS DISEASE
    John A Tainer; Fiscal Year: 2013
    ..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...
  71. Folding and Aggregation Mechanisms of Human Cu,Zn Superoxide Dismutase
    C Robert Matthews; Fiscal Year: 2013
    ..Tragically, mutations at dozens of positions in SOD1 cause amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease...
  72. RNAi-Based Therapy of ALS
    Tariq M Rana; Fiscal Year: 2010
    ..diseases are neurodegenerative diseases including Huntingtqn's, a subset of amyotrophic lateral sclerosis (ALS), Alzheimer's and Parkinson's diseases...
  73. Putting the pieces together: The role of CCS in intracellular copper distribution
    CHRISTOPHER RYAN POPE; Fiscal Year: 2012
    ..The work also will yield the first direct structural data on any complex between an intact copper transporter and a chaperone, which will make a critical contribution to understanding the structural basis of cellular copper homeostasis. ..
  74. Protein misfolding and aggregation
    NIKOLAY DOKHOLYAN; Fiscal Year: 2013
    ..cytotoxic agents in several such disorders, including Alzheimer's disease and amyotrophic lateral sclerosis (ALS)...
  75. Structural Consequences of ALS-related Modifications of SOD1
    JEFFREY NEIL AGAR; Fiscal Year: 2013
    ..abstract_text> ..
  76. Proteostasis in Neurodegenerative Disease
    JADA M LEWIS; Fiscal Year: 2013
    ....
  77. Microfluidics for High-Throughput HDX-MS
    Julian P Whitelegge; Fiscal Year: 2013
    ..Whitelegge and C. K.-F. Shen) uniquely positioned to translate new technologies into ongoing scientific discovery at UCLA. ..
  78. NITRIC OXIDE/SUPEROXIDE IN LIPID VASCULAR DISEASE
    TIMOTHY O BRIEN; Fiscal Year: 2000
    ..Superoxide anions are scavenged by SOD. This project was designed to determine the role of NO and superoxide in cholesterol-induced vasomotor dysfunction ..
  79. The novel family of superoxide dismutase enzymes in Candida albicans
    Valeria C Culotta; Fiscal Year: 2013
    ..One set of virulence factors for C. albicans includes a family of copper containing superoxide dismutase (SOD) enzymes that scavenge toxic superoxide free radicals...
  80. REGULATION--COPPER-ZINC SUPEROXIDE DISMUTASE EXPRESSION
    Jeffrey Whitsett; Fiscal Year: 1990
    ..Two hypotheses will be tested: 1) CuZn SOD is temporally and hormonally regulated during the perinatal period: 2) Oxygen increases pulmonary CuZn SOD activity ..
  81. CHONDROPROTECTIVE EFFECTS OF SUPEROXIDE DISMUTASE
    Constance 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 ..
  82. ZINC DEFICIENT SOD AND NEUROFILAMENT DYSFUNCTION IN ALS
    John Crow; Fiscal Year: 2001
    ..lateral sclerosis (FALS) patients possessing any one of 35 known mutations to Cu,Zn superoxide dismutase (SOD) stand a >85% chance of developing this fatal neurodegenerative disease...
  83. REGULATION OF EXTRACELLULAR SUPEROXIDE DISMUTASE BY PROT
    Russell 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...
  84. SUPPRESSORS OF SUPEROXIDE DISMUTASE FUNCTION IN YEAST
    Valeria Culotta; Fiscal Year: 1992
    ..which organisms are thought to combat oxygen toxicity involves the 02- scavenging activity of superoxide dismutase (SOD)...
  85. HYDROXYNONENAL MODIFICATION OF SUPEROXIDE DISMUTASE
    Kenneth 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...