Cu Zn superoxide dismutase

Summary

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

Top Publications

  1. 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
  2. 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
  3. 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
  4. 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
  5. 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
  6. 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
  7. 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
  8. 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
  9. 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
  10. 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

Research Grants

  1. Glutathione in mitochondrial dysfunction and disease progression in ALS-models
    Marcelo R Vargas; Fiscal Year: 2011
  2. ALVARO G ESTEVEZ; Fiscal Year: 2014
  3. Marcelo R Vargas; Fiscal Year: 2014
  4. RODNEY JON SCHLOSSER; Fiscal Year: 2014
  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
  7. Mitochondrial SOD as a Target for Diabetic Neuropathy
    EVA LUCILLE FELDMAN; Fiscal Year: 2010
  8. Salman Azhar; Fiscal Year: 2016
  9. Extracellular Matrix Components, Oxidants and Antioxidants in Pulmonary Fibrosis
    CORRINE R KLIMENT; Fiscal Year: 2010
  10. Mitochondrial Oxidative Stress In Angiotensis II Induced Endothelial Dysfunction
    Sergey Dikalov; Fiscal Year: 2013

Detail Information

Publications416 found, 100 shown here

  1. 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...
  2. 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...
  3. 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...
  4. 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...
  5. 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...
  6. 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 ..
  7. 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 ..
  8. 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).
  9. 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 ..
  10. 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
    ....
  11. 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...
  12. 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...
  13. doi Familial amyotrophic lateral sclerosis in Alberta, Canada
    Ted Pfister
    Department of Clinical Neurosciences, Hotchkiss Brain Institute, University of Calgary, Calgary, Canada
    Amyotroph Lateral Scler Frontotemporal Degener 14:273-7. 2013
    Systematic review data demonstrate that 5% of ALS cases are familial (FALS). Causative superoxide dismutase-1 (SOD1) mutations are identified in 10-20% of FALS. Few reports of FALS epidemiology exist in Canada...
  14. ncbi Structure and mechanism of copper, zinc superoxide dismutase
    J A Tainer
    Nature 306:284-7. 1983
    Copper, zinc superoxide dismutase (SOD) catalyses the very rapid two-step dismutation of the toxic superoxide radical (O-2) to molecular oxygen and hydrogen peroxide through the alternate reduction and oxidation of the active-site copper...
  15. ncbi Dynamic properties of the G93A mutant of copper-zinc superoxide dismutase as detected by NMR spectroscopy: implications for the pathology of familial amyotrophic lateral sclerosis
    Eric L Shipp
    Magnetic Resonance Center CERM, University of Florence, Via Luigi Sacconi 6, 50019 Sesto Fiorentino, Italy
    Biochemistry 42:1890-9. 2003
    ..15)N R(1), R(2), and R(1)(rho) and (15)N-(1)H NOE experiments were then carried out at 600 MHz on G93A Cu(2)Zn(2)SOD and the values compared to the dynamics data for the "wild-type" protein...
  16. 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)...
  17. 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...
  18. 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...
  19. 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...
  20. 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 ..
  21. 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...
  22. 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...
  23. 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...
  24. 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...
  25. 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...
  26. 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...
  27. 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...
  28. 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...
  29. 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...
  30. 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
    ....
  31. 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...
  32. 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
    ....
  33. 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...
  34. 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.
  35. 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.
  36. 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...
  37. 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...
  38. 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, ..
  39. 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...
  40. 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
    ....
  41. 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...
  42. 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...
  43. 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.)...
  44. 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 ..
  45. 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...
  46. pmc A novel variant of human superoxide dismutase 1 harboring amyotrophic lateral sclerosis-associated and experimental mutations in metal-binding residues and free cysteines lacks toxicity in vivo
    Mercedes Prudencio
    Department of Neuroscience, Center for Translational Research in Neurodegenerative Disease, McKnight Brain Institute, University of Florida, Gainesville, Florida, USA
    J Neurochem 121:475-85. 2012
    ..In cell culture models, we determine that the combined mutation of C6 and C111 to G and S, respectively, dramatically reduces the aggregation propensity of SODMD and may account for the lack of toxicity for this mutant...
  47. pmc The effect of SOD1 mutation on cellular bioenergetic profile and viability in response to oxidative stress and influence of mutation-type
    Katie Richardson
    Sheffield Institute for Translational Neuroscience, University of Sheffield, Sheffield, United Kingdom
    PLoS ONE 8:e68256. 2013
    Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the progressive degeneration of motor neurons...
  48. doi Recurrent K3E mutation in Cu/Zn superoxide dismutase gene associated with amyotrophic lateral sclerosis
    Magdalena Kuzma-Kozakiewicz
    Department of Neurology, Medical University of Warsaw
    Amyotroph Lateral Scler Frontotemporal Degener 14:608-14. 2013
    ..dismutase (SOD1) gene mutations are the most frequently reported genetic causes of amyotrophic lateral sclerosis (ALS)...
  49. doi Novel SOD1 mutation p.V31A identified with a slowly progressive form of amyotrophic lateral sclerosis
    Audrey Dangoumau
    UMR Inserm U930, Universite Francois Rabelais, Tours, PRES Centre Val de Loire Université, France
    Neurobiol Aging 35:266.e1-4. 2014
    ..superoxide dismutase 1 (SOD1) protein is mutated in approximately 15% of familial amyotrophic lateral sclerosis (ALS) and 3% of sporadic ALS...
  50. pmc SOD2 to SOD1 switch in breast cancer
    Luena Papa
    From the the Department of Medicine, Division of Hematology Oncology, Tisch Cancer Institute, Mount Sinai School of Medicine, New York, New York 10029 and
    J Biol Chem 289:5412-6. 2014
    ..We propose that by analogy to the cadherin switch during epithelial-mesenchymal transition, cancer cells also undergo a SOD switch during transformation.
  51. pmc Dysregulation of the proteasome increases the toxicity of ALS-linked mutant SOD1
    Akira Kitamura
    Laboratory of Molecular Cell Dynamics, Faculty of Advanced Life Science, Hokkaido University, N21W11, Kita ku, Sapporo, 001 0021, Japan Department of Molecular and Cellular Biology, Institute for Frontier Medical Sciences, Kyoto University, 53 Shogoin Kawahara cho, Sakyo ku, Kyoto, 606 8397, Japan
    Genes Cells 19:209-24. 2014
    ..In this study, we examined the biophysical properties of amyotrophic lateral sclerosis (ALS)-linked mutations of Cu/Zn superoxide dismutase 1 (SOD1) expressed in human tissue culture cells...
  52. doi Computing stability effects of mutations in human superoxide dismutase 1
    Kasper P Kepp
    DTU Chemistry, Technical University of Denmark, DK 2800 Kongens Lyngby, Denmark
    J Phys Chem B 118:1799-812. 2014
    ..Finally, mutations involving glycine were most difficult to model, of relevance to future method improvement. This could be due to structure changes (glycine has a low structural propensity) or water colocalization with glycine...
  53. ncbi A caspase-3-cleaved fragment of the glial glutamate transporter EAAT2 is sumoylated and targeted to promyelocytic leukemia nuclear bodies in mutant SOD1-linked amyotrophic lateral sclerosis
    Stuart L Gibb
    Farber Institute for Neurosciences, Weinberg Unit for ALS Research, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, and Cecil B Day Laboratory for Neuromuscular Research, Massachusetts General Hospital, Charlestown 02129, USA
    J Biol Chem 282:32480-90. 2007
    ..During the course of human amyotrophic lateral sclerosis (ALS) and in a transgenic mutant SOD1 mouse model of the disease, expression and activity of EAAT2 is remarkably reduced...
  54. 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...
  55. 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 ..
  56. 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...
  57. 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...
  58. 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)...
  59. 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 ..
  60. 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...
  61. 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%...
  62. 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)...
  63. 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...
  64. 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...
  65. 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...
  66. 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...
  67. 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...
  68. 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...
  69. 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)...
  70. 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...
  71. 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 ..
  72. 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
  73. 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...
  74. 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...
  75. 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...
  76. 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 ..
  77. 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)...
  78. 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)...
  79. pmc 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 ..
  80. ncbi Superoxide dismutase isoenzymes in the normal and diseased human cornea
    A Behndig
    Department of Clinical Sciences Ophthalmology, Umea University Hospital, Sweden
    Invest Ophthalmol Vis Sci 42:2293-6. 2001
    The human cornea, a tissue much exposed to oxidative stress, is rich in extracellular superoxide dismutase (SOD)...
  81. ncbi Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis
    Bradley 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 ..
  82. ncbi Induction of the unfolded protein response in familial amyotrophic lateral sclerosis and association of protein-disulfide isomerase with superoxide dismutase 1
    Julie 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...
  83. ncbi Degradation of amyotrophic lateral sclerosis-linked mutant Cu,Zn-superoxide dismutase proteins by macroautophagy and the proteasome
    Tomohiro Kabuta
    Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo 187 8502, Japan
    J Biol Chem 281:30524-33. 2006
    ..These findings may provide further insights into the mechanisms of pathogenesis of fALS...
  84. doi Non-cell-autonomous effect of human SOD1 G37R astrocytes on motor neurons derived from human embryonic stem cells
    Maria 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. 2008
    Amyotrophic 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)...
  85. pmc An examination of wild-type SOD1 in modulating the toxicity and aggregation of ALS-associated mutant SOD1
    Mercedes 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...
  86. ncbi 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 mellitus
    O 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 ..
  87. 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...
  88. 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...
  89. pmc Mutant copper-zinc superoxide dismutase associated with amyotrophic lateral sclerosis binds to adenine/uridine-rich stability elements in the vascular endothelial growth factor 3'-untranslated region
    Xuelin Li
    Department of Neurology, University of Alabama at Birmingham, Alabama, USA
    J Neurochem 108:1032-44. 2009
    ..Loss of this factor produces a phenotype similar to amyotrophic lateral sclerosis (ALS)...
  90. pmc Disulfide-reduced ALS variants of Cu, Zn superoxide dismutase exhibit increased populations of unfolded species
    Can Kayatekin
    Department of Biochemistry and Molecular Pharmacology, University of Massachusetts Medical School, Worcester, MA 01605, USA
    J Mol Biol 398:320-31. 2010
    ..Mutations at dozens of sites in SOD1 induce amyotrophic lateral sclerosis (ALS), a fatal gain-of-function neurodegenerative disease whose molecular basis is unknown...
  91. 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...
  92. ncbi The unusually stable quaternary structure of human Cu,Zn-superoxide dismutase 1 is controlled by both metal occupancy and disulfide status
    Fabio Arnesano
    Magnetic Resonance Center CERM and Department of Chemistry, University of Florence, Via Luigi Sacconi 6, 50019 Sesto Fiorentino, Florence, Italy
    J Biol Chem 279:47998-8003. 2004
    ..interplay of conserved disulfide bond and metal-site occupancy in human copper,zinc superoxide dismutase (hSOD1) is of increasing interest as these post-translational modifications are known to dramatically alter the catalytic ..
  93. pmc Structural characterization of zinc-deficient human superoxide dismutase and implications for ALS
    Blaine R Roberts
    Department of Biochemistry and Biophysics, Oregon State University, Corvallis, OR 97331, USA
    J Mol Biol 373:877-90. 2007
    Over 130 mutations to copper, zinc superoxide dismutase (SOD) are implicated in the selective death of motor neurons found in 25% of patients with familial amyotrophic lateral sclerosis (ALS)...
  94. 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
    ....
  95. pmc Initiation and elongation in fibrillation of ALS-linked superoxide dismutase
    Madhuri Chattopadhyay
    Department of Chemistry and Biochemistry, University of California, Los Angeles, CA 90095, USA
    Proc Natl Acad Sci U S A 105:18663-8. 2008
    ..Our findings provide a rare glimpse into the specific changes in a protein that can lead to nucleation and into the ability of amyloid nuclei to recruit diverse forms of the same protein into fibrils...
  96. ncbi SOD1 mutations in amyotrophic lateral sclerosis. Results from a multicenter Italian study
    Stefania Battistini
    Dept of Neuroscience, University of Siena, Siena, Italy
    J Neurol 252:782-8. 2005
    Amyotrophic Lateral Sclerosis (ALS), the most common form among motoneuron diseases, is characterized by a progressive neurodegenerative process involving motor neurons in the motor cortex, brain stem and spinal cord...
  97. pmc Destabilization of apoprotein is insufficient to explain Cu,Zn-superoxide dismutase-linked ALS pathogenesis
    Jorge A Rodriguez
    Department of Chemistry and Biochemistry, University of California, Los Angeles, CA 90095, USA
    Proc Natl Acad Sci U S A 102:10516-21. 2005
    The relative stabilities and structural properties of a representative set of 20 ALS-mutant Cu,Zn-superoxide dismutase apoproteins were examined by using differential scanning calorimetry and hydrogen-deuterium (H/D) exchange followed by ..
  98. pmc Retrograde axonal transport and motor neuron disease
    Anna Lena Strom
    Department of Molecular and Cellular Biochemistry, College of Medicine, University of Kentucky, Lexington, Kentucky, USA
    J Neurochem 106:495-505. 2008
    ..contribute to the pathogenesis of multiple neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS)...
  99. pmc Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria
    Hibiki Kawamata
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY 10065, USA
    Hum Mol Genet 17:3303-17. 2008
    ..We find that the physiological regulation of mitochondrial localization is either inefficient or absent in SOD1 pathogenic mutants. We propose misfolding and aggregation of these mutants that trap them inside mitochondria...
  100. pmc Destabilizing protein polymorphisms in the genetic background direct phenotypic expression of mutant SOD1 toxicity
    Tali Gidalevitz
    Department of Biochemistry, Molecular Biology, and Cell Biology, Rice Institute for Biomedical Research, Northwestern University, Evanston, Illinois, United States of America
    PLoS Genet 5:e1000399. 2009
    ..Mutations in SOD1 in a subset of familial amyotrophic lateral sclerosis (ALS) cases confer dominant but clinically variable toxicity, thought to be mediated by misfolding and aggregation of ..
  101. ncbi Recessive amyotrophic lateral sclerosis families with the D90A SOD1 mutation share a common founder: evidence for a linked protective factor
    A Al-Chalabi
    Departments of Neuroscience and Clinical Neurosciences, Institute of Psychiatry and King s College School of Medicine and Dentistry, De Crespigny Park, Denmark Hill, London SE5 8AF, UK
    Hum Mol Genet 7:2045-50. 1998
    Amyotrophic lateral sclerosis (ALS) is a progressive motor neurodegeneration resulting in paralysis and death from respiratory failure within 3-5 years...

Research Grants89

  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. ALVARO G ESTEVEZ; Fiscal Year: 2014
    ..to SOD can increase oxidative stress and cause the death of motor neurons in amyotrophic lateral sclerosis (ALS)...
  3. Marcelo R Vargas; Fiscal Year: 2014
    ..with aggravated mitochondrial pathology commonly observed in transgenic mice overexpressing mutated forms of hSOD1 that retain superoxide dismutase activity...
  4. RODNEY JON SCHLOSSER; Fiscal Year: 2014
    ..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. Salman Azhar; Fiscal Year: 2016
    ..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. Jingsong Zhou; Fiscal Year: 2014
    DESCRIPTION (provided by applicant): Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disorder involving degeneration of motor neurons and atrophy of skeletal muscle...
  13. Wendong Huang; Fiscal Year: 2015
    ..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) ..
  14. 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...
  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. Robert F Foronjy; Fiscal Year: 2014
    ..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. Paolo Fanti; Fiscal Year: 2014
    ..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. Neil W Kowall; Fiscal Year: 2014
    DESCRIPTION (provided by applicant): Amyotrophic Lateral Sclerosis (ALS) is a fatal disease characterized by progressive paralysis due to motor neuron degeneration...
  21. 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...
  22. EVA NOZIK GRAYCK; Fiscal Year: 2016
    ..is the antioxidant extracellular superoxide dismutase (EC-SOD or SOD3)...
  23. 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...
  24. 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)...
  25. Arlan G Richardson; Fiscal Year: 2014
    ....
  26. VINOD D LABHASETWAR; Fiscal Year: 2014
    ..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 ..
  27. Lee J Martin; Fiscal Year: 2014
    ..Many different SOD1 mutations occur, but the mechanisms of human SOD1 (hSOD1) toxicity to MNs are unresolved...
  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. ANN NARDULLI; Fiscal Year: 2014
    ..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. Ines Batinic-Haberle; Fiscal Year: 2014
    ..The potency of these compounds approaches that of SOD enzymes...
  33. 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...
  34. Tzung K Hsiai; Fiscal Year: 2015
    ..production via an up-regulation in Mn-SOD activities. In contrast, atherogenic hemodynamics and oxidized LDL induced mitochondrial O2...
  35. 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 ..
  36. Valeria C Culotta; Fiscal Year: 2014
    ..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
    Abstract ALS is a fatal progressive paralysis affecting 34,000 Americans and killing 8,000 per year...
  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. 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)...
  42. Navdeep S Chandel; Fiscal Year: 2015
    ....
  43. 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...
  44. 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 ..
  45. 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. ..
  46. Peter A Campochiaro; Fiscal Year: 2014
    ..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...
  47. 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 ..
  48. 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...
  49. 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...
  50. 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...
  51. 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)...
  52. 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...
  53. 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...
  54. 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...
  55. Joan Selverstone Valentine; Fiscal Year: 2014
    ..Finally, ALS tissue will be used as a source to validate the findings and test new hypotheses...
  56. 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...
  57. Peter H King; Fiscal Year: 2015
    ..Mutations of Cu, Zn superoxide dismutase (SOD)1 that are associated with familial ALS gain a high RNA binding affinity for U- and AU-rich elements present in 5&..
  58. 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)...
  59. Robert H Brown; Fiscal Year: 2015
    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. Jiou Wang; Fiscal Year: 2016
    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. NIKOLAY DOKHOLYAN; Fiscal Year: 2015
    ..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. JADA M LEWIS; Fiscal Year: 2014
    ....
  77. Julian P Whitelegge; Fiscal Year: 2014
    ..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. 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...
  85. 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)...