Sod1

Summary

Gene Symbol: Sod1
Description: superoxide dismutase 1, soluble
Alias: B430204E11Rik, Cu/Zn-SOD, CuZnSOD, Ipo-1, Ipo1, SODC, Sod-1, superoxide dismutase [Cu-Zn], Cu(2+)-Zn2+ superoxide dismutase
Species: mouse

Top Publications

  1. pmc ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration
    Zhihui Zhong
    Center for Neurodegenerative and Vascular Brain Disorders and Department of Neurosurgery, University of Rochester Medical Center, Kornberg Medical Research Bldg, 601 Elmwood Avenue, Box 670, Rochester, New York 14642, USA
    Nat Neurosci 11:420-2. 2008
  2. pmc Cu/Zn superoxide dismutase and age-related hearing loss
    Elizabeth M Keithley
    Division of Otolaryngology Head and Neck Surgery, University of California, San Diego, La Jolla, 92093 0666, USA
    Hear Res 209:76-85. 2005
  3. ncbi Trends in oxidative aging theories
    Florian L Muller
    Department of Cellular and Structural Biology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229 3900, USA
    Free Radic Biol Med 43:477-503. 2007
  4. pmc The in vivo gene expression signature of oxidative stress
    Eun Soo Han
    Department of Biological Science, University of Tulsa, Tulsa, Oklahoma, USA
    Physiol Genomics 34:112-26. 2008
  5. doi Skin atrophy in cytoplasmic SOD-deficient mice and its complete recovery using a vitamin C derivative
    Kazuma Murakami
    Research Team for Molecular Biomarkers, Tokyo Metropolitan Institute of Gerontology, Tokyo, Japan
    Biochem Biophys Res Commun 382:457-61. 2009
  6. 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
  7. 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
  8. doi Superoxide dismutase 1 regulates caspase-1 and endotoxic shock
    Felix Meissner
    Department of Cellular Microbiology, Max Planck Institute for Infection Biology, 10117 Berlin, Germany
    Nat Immunol 9:866-72. 2008
  9. ncbi Ovarian function in superoxide dismutase 1 and 2 knockout mice
    M M Matzuk
    Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
    Endocrinology 139:4008-11. 1998
  10. ncbi Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury
    A G Reaume
    Department of Molecular Biology, Cephalon Inc, West Chester Pennsylvania 19380, USA
    Nat Genet 13:43-7. 1996

Research Grants

  1. INNER EAR IMMUNE RESPONSES
    Elizabeth Keithley; Fiscal Year: 2002
  2. DNA Damage/Repair and Cell Death
    Lee Martin; Fiscal Year: 2009
  3. OXIDATION OF MACROMOLECULES IN SPINAL CORD INJURY
    Danxia Liu; Fiscal Year: 2001
  4. MECHANISMS OF NEURONAL APOPTOSIS IN VIVO
    Lee Martin; Fiscal Year: 2009
  5. SYMPOSIA FOR ASSOCIATION FOR RESEARCH IN OTOLARYNGOLOGY
    Elizabeth Keithley; Fiscal Year: 2008
  6. Regulation of Key Processes in Oocyte Biology
    Martin Matzuk; Fiscal Year: 2007
  7. RNA-mediated motor neuron degeneration
    WILLIAM SCHLAEPFER; Fiscal Year: 2007
  8. Mechanisms of Motor Neuron Death
    Lee Martin; Fiscal Year: 2006
  9. Endothelial Function in the Cerebral Circulation
    Frank Faraci; Fiscal Year: 2009
  10. The Mouse as an Instrument for Ear Research II
    Kenneth Johnson; Fiscal Year: 2005

Detail Information

Publications152 found, 100 shown here

  1. pmc ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration
    Zhihui Zhong
    Center for Neurodegenerative and Vascular Brain Disorders and Department of Neurosurgery, University of Rochester Medical Center, Kornberg Medical Research Bldg, 601 Elmwood Avenue, Box 670, Rochester, New York 14642, USA
    Nat Neurosci 11:420-2. 2008
    We report here that amyotrophic lateral sclerosis-linked superoxide dismutase 1 (SOD1) mutants with different biochemical characteristics disrupted the blood-spinal cord barrier in mice by reducing the levels of the tight junction ..
  2. pmc Cu/Zn superoxide dismutase and age-related hearing loss
    Elizabeth M Keithley
    Division of Otolaryngology Head and Neck Surgery, University of California, San Diego, La Jolla, 92093 0666, USA
    Hear Res 209:76-85. 2005
    ..Antioxidant enzymes such as Cu/Zn superoxide dismutase (SOD1) protect cells from toxic, reactive oxygen species and may be involved in age-related degeneration...
  3. ncbi Trends in oxidative aging theories
    Florian L Muller
    Department of Cellular and Structural Biology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229 3900, USA
    Free Radic Biol Med 43:477-503. 2007
    ..Studies in mice argue for a role of oxidative stress in age-related disease, especially cancer; however, with regard to aging per se, the data either do not support or remain inconclusive on whether oxidative stress determines life span...
  4. pmc The in vivo gene expression signature of oxidative stress
    Eun Soo Han
    Department of Biological Science, University of Tulsa, Tulsa, Oklahoma, USA
    Physiol Genomics 34:112-26. 2008
    ..induced in vivo by diquat or by genetic ablation of the major antioxidant enzymes CuZn-superoxide dismutase (Sod1) and glutathione peroxidase-1 (Gpx1)...
  5. doi Skin atrophy in cytoplasmic SOD-deficient mice and its complete recovery using a vitamin C derivative
    Kazuma Murakami
    Research Team for Molecular Biomarkers, Tokyo Metropolitan Institute of Gerontology, Tokyo, Japan
    Biochem Biophys Res Commun 382:457-61. 2009
    ..The CuZn-SOD-deficient mice might be applicable to evaluation of therapeutic medicines against skin ageing...
  6. 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
    ..report tight genetic linkage between FALS and a gene that encodes a cytosolic, Cu/Zn-binding superoxide dismutase (SOD1), a homodimeric metalloenzyme that catalyzes the dismutation of the toxic superoxide anion O2.- to O2 and H2O2 (ref...
  7. 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
    Mutations 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...
  8. doi Superoxide dismutase 1 regulates caspase-1 and endotoxic shock
    Felix Meissner
    Department of Cellular Microbiology, Max Planck Institute for Infection Biology, 10117 Berlin, Germany
    Nat Immunol 9:866-72. 2008
    ..We show here that superoxide dismutase 1 (SOD1) regulates caspase-1 activation...
  9. ncbi Ovarian function in superoxide dismutase 1 and 2 knockout mice
    M M Matzuk
    Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
    Endocrinology 139:4008-11. 1998
    Copper/zinc superoxide dismutase (SOD1) and manganese superoxide dismutase (SOD2) are the two major intracellular enzymes which inactivate superoxide radicals...
  10. ncbi Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury
    A G Reaume
    Department of Molecular Biology, Cephalon Inc, West Chester Pennsylvania 19380, USA
    Nat Genet 13:43-7. 1996
    ..amyotrophic lateral sclerosis (FALS) are associated with mutations in the gene encoding Cu/Zn superoxide dismutase (SOD1) has focused much attention on the function of SOD1 as related to motor neuron survival...
  11. pmc Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice
    Koji Yamanaka
    Ludwig Institute for Cancer Research and Department of Medicine and Neuroscience, University of California at San Diego, La Jolla, CA 92093, USA
    Proc Natl Acad Sci U S A 105:7594-9. 2008
    Dominant mutations in ubiquitously expressed superoxide dismutase (SOD1) cause familial ALS by provoking premature death of adult motor neurons...
  12. ncbi Beneficial effect of ginseng root in SOD-1 (G93A) transgenic mice
    F Jiang
    McMaster University Medical Centre, Rm 4U7, 1200 Main St W, Hamilton, Ontario, Canada L8N 3Z5
    J Neurol Sci 180:52-4. 2000
    ..be beneficial in ALS as well, we studied the effect of ginseng (Panax quinquefolium), 40 and 80 mg/Kg, in B6SJL-TgN(SOD1-G93A)1Gur transgenic mice. The ginseng was given in drinking water, from age 30d onwards...
  13. ncbi Early decrease of redox factor-1 in spinal motor neurons of presymptomatic transgenic mice with a mutant SOD1 gene
    Y Manabe
    Department of Neurology, Graduate School of Medicine and Dentistry, Okayama University, 2 5 1 Shikata cho, Okayama 700 8558, Japan
    Brain Res 915:104-7. 2001
    ..protein was examined in the spinal cord of transgenic mice with an ALS-linked mutant Cu/Zn superoxide dismutase (SOD1) gene...
  14. ncbi Transgenic SOD1 G93A mice develop reduced GLT-1 in spinal cord without alterations in cerebrospinal fluid glutamate levels
    C Bendotti
    Department of Neuroscience Istituto di Ricerche Farmacologiche Mario Negri, Milano, Italy
    J Neurochem 79:737-46. 2001
    ..The lack of changes in GLT-1 at the presymptomatic stage suggests that glial glutamate transporter reduction is not a primary event leading to motor neurone loss...
  15. pmc Corneal endothelial integrity in aging mice lacking superoxide dismutase-1 and/or superoxide dismutase-3
    Anders Behndig
    Department of Clinical Science Ophthalmology, Umea University Hospital, Umea, Sweden
    Mol Vis 14:2025-30. 2008
    ..To evaluate the age-induced changes in corneal endothelial morphology in mice lacking the cytosolic copper-zinc superoxide dismutase (SOD-1), the interstitial extracellular superoxide dismutase (SOD-3), or both of these SOD isoenzymes...
  16. 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
    ..We report that ALS-linked mutant superoxide dismutase 1 (SOD1) activates caspase-1 and IL-1beta in microglia...
  17. ncbi Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons
    T L Williamson
    Ludwig Institute for Cancer Research, University of California, San Diego, La Jolla 92093, USA
    Nat Neurosci 2:50-6. 1999
    Mutations in copper/zinc superoxide dismutase 1 (SOD1), primary causes of human amyotrophic lateral sclerosis (ALS), provoke motor neuron death through an unidentified toxic property...
  18. ncbi Mutations in dynein link motor neuron degeneration to defects in retrograde transport
    Majid Hafezparast
    Department of Neurodegenerative Disease, Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK
    Science 300:808-12. 2003
    ..These mutations exclusively perturb neuron-specific functions of dynein...
  19. ncbi Motor neuron disease in mice expressing the wild type-like D90A mutant superoxide dismutase-1
    P Andreas Jonsson
    Department of Medical Biosciences, Umea University Hospital, SE 901 85 Umea, Sweden
    J Neuropathol Exp Neurol 65:1126-36. 2006
    ..01). Thus, wild-type hSOD1 shows a significant neurotoxicity in the spinal cord, that is less than equal but more than half as large as that of D90A mutant enzyme...
  20. ncbi Age-related cochlear hair cell loss is enhanced in mice lacking copper/zinc superoxide dismutase
    S L McFadden
    Center for Hearing and Deafness, State University of New York at Buffalo, 14214, USA
    Neurobiol Aging 20:1-8. 1999
    ..wild type (WT) mice with normal levels of Cu/Zn SOD and mutant knockout (KO) mice with a targeted deletion of Sod1, the gene that codes for Cu/Zn SOD. WT and KO mice exhibited similar patterns of hair cell loss with age, i.e...
  21. ncbi Immune reactivity in a mouse model of familial ALS correlates with disease progression
    M E Alexianu
    Department of Neurology, Baylor College of Medicine, Houston, TX 77030, USA
    Neurology 57:1282-9. 2001
    ..This study aims to define the potential involvement of nonneuronal immune-inflammatory factors in the destruction of motor neurons in mutant superoxide dismutase-1 (SOD1) transgenic mice as a model of ALS.
  22. ncbi Lack of CuZnSOD activity: a pointer to the mechanisms underlying age-related loss of muscle function, a commentary on "absence of CuZn superoxide dismutase leads to elevated oxidative stress and acceleration of age-dependent skeletal muscle atrophy&q
    Malcolm J Jackson
    Division of Metabolic and Cellular Medicine, School of Clinical Sciences, University of Liverpool, Liverpool L69 3GA, UK
    Free Radic Biol Med 40:1900-2. 2006
  23. ncbi Denervation-induced skeletal muscle atrophy is associated with increased mitochondrial ROS production
    Florian L Muller
    Department of Cellular and Structural Biology, University of Texas Health Science Center at San Antonio, Barshop Institute for Longevity and Aging Studies, 15355 Lambda Drive, San Antonio, TX 78245 3207, USA
    Am J Physiol Regul Integr Comp Physiol 293:R1159-68. 2007
    ..ROS generation in three conditions associated with muscle atrophy: in aging, in mice lacking CuZn-SOD (Sod1(-/-)), and in the neurodegenerative disease, amyotrophic lateral sclerosis (ALS)...
  24. pmc Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS
    Jiou Wang
    Howard Hughes Medical Institute, Department of Genetics, Section of Comparative Medicine, and Department of Neurosurgery, Yale University School of Medicine, New Haven, CT 06510, USA
    Proc Natl Acad Sci U S A 106:1392-7. 2009
    Recent studies suggest that superoxide dismutase 1 (SOD1)-linked amyotrophic lateral sclerosis results from destabilization and misfolding of mutant forms of this abundant cytosolic enzyme...
  25. pmc The age-related failure of adaptive responses to contractile activity in skeletal muscle is mimicked in young mice by deletion of Cu,Zn superoxide dismutase
    Aphrodite Vasilaki
    School of Clinical Sciences, University of Liverpool, Liverpool L69 3GA, UK
    Aging Cell 9:979-90. 2010
    ..Mice lacking the Cu,Zn superoxide dismutase (Cu,ZnSOD, SOD1) show an accelerated, age-related loss of muscle mass and function...
  26. ncbi Delaying caspase activation by Bcl-2: A clue to disease retardation in a transgenic mouse model of amyotrophic lateral sclerosis
    S Vukosavic
    Departments of Neurology and Pathology, Columbia University, New York, New York 10032, USA
    J Neurosci 20:9119-25. 2000
    ....
  27. ncbi Neuron-specific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment
    A Pramatarova
    Centre for Research in Neuroscience, McGill University, and the Montreal General Hospital Research Institute, Montreal, Quebec, H3G 1A4, Canada
    J Neurosci 21:3369-74. 2001
    Mutations were identified in the Cu/Zn superoxide dismutase gene (SOD1) in approximately 15% of patients with familial amyotrophic lateral sclerosis. Transgenic animals expressing mutant SOD1 in all tissues develop an ALS-like phenotype...
  28. ncbi Cytokine upregulation in a murine model of familial amyotrophic lateral sclerosis
    J L Elliott
    Department of Neurology, University of Texas, Southwestern Medical Center, Dallas, TX 75390, USA
    Brain Res Mol Brain Res 95:172-8. 2001
    ..various cytokines in the spinal cords of transgenic mice expressing a Cu-Zn superoxide dismutase mutation (Tgn G93A SOD1) by using semi-quantitative RT-PCR...
  29. ncbi GLT-1 glutamate transporter levels are unchanged in mice expressing G93A human mutant SOD1
    Jeffrey S Deitch
    Department of Neurology, MCP Hahnemann University, MS 423, 245 North 15th Street, Philadelphia, PA 19102 1192, USA
    J Neurol Sci 193:117-26. 2002
    ..We examined protein levels of the glutamate transporters GLT-1, GLAST and EAAC1 in the G93A (SOD1) transgenic mouse model of ALS. GLT-1 was detected in two bands (72 and 150 kD)...
  30. ncbi Paradoxical actions of hydrogen peroxide on long-term potentiation in transgenic superoxide dismutase-1 mice
    Ariel Kamsler
    Department of Neurobiology, The Weizmann Institute, Rehovot 76100, Israel
    J Neurosci 23:10359-67. 2003
    ..These data promoted a dual role for H2O2 in the regulation of LTP, and proposed that it is mediated by the protein phosphatase calcineurin...
  31. ncbi Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria
    Jian Liu
    Ludwig Institute for Cancer Research, Department of Neurosciences, Medicine, and Cellular and Molecular Medicine, University of California, San Diego, 9500 Gilman Drive, La Jolla, CA 92093, USA
    Neuron 43:5-17. 2004
    ..of amyotrophic lateral sclerosis (ALS) is mutation in ubiquitously expressed copper/zinc superoxide dismutase (SOD1), but the mechanism of toxicity to motor neurons is unknown...
  32. ncbi Unsaturated fatty acids induce cytotoxic aggregate formation of amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutants
    Yeon Jeong Kim
    Laboratory for Motor System Neurodegeneration, RIKEN Brain Science Institute, Saitama 351 0198, Japan
    J Biol Chem 280:21515-21. 2005
    ..Superoxide dismutase 1 (SOD1) immunoreactive inclusions have been found in the spinal cord of ALS animal models and patients, implicating the ..
  33. ncbi Mutant superoxide dismutase disrupts cytoplasmic dynein in motor neurons
    Lee A Ligon
    Department of Physiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
    Neuroreport 16:533-6. 2005
    ..Thus, inhibition of dynein/dynactin function may have a role in motor neuron degeneration in amyotrophic lateral sclerosis...
  34. ncbi Neuroprotective effect of oxidized galectin-1 in a transgenic mouse model of amyotrophic lateral sclerosis
    Ren Chang-Hong
    Department of Neurology, Hematology, Metabolism, Endocrinology and Diabetology, Yamagata University School of Medicine, 2 2 2 Iida nishi, Yamagata 990 9585, Japan
    Exp Neurol 194:203-11. 2005
    ..the left gastrocnemius muscle of the transgenic mice over-expressing a mutant copper/zinc superoxide dismutase (SOD1) with a substitution of histidine to arginine at position 46 (H46R SOD1)...
  35. ncbi Phenotypes of mice lacking extracellular superoxide dismutase and copper- and zinc-containing superoxide dismutase
    Marie Louise Sentman
    Department of Medical Biosciences, Clinical Chemistry, Umea University Hospital, SE 901 85 Umea, Sweden
    J Biol Chem 281:6904-9. 2006
    ..In conclusion, apart from the increases in the global urinary and plasma oxidant stress markers, our phenotype studies revealed no other evidence that the copper- and zinc-containing SOD isoenzymes have overlapping roles...
  36. ncbi Effect of the reduction of superoxide dismutase 1 and 2 or treatment with alpha-tocopherol on tumorigenesis in Atm-deficient mice
    Laura Erker
    Departments of Pediatrics and Medicine, Center for Human Genetics and Genomics, UCSD School of Medicine, La Jolla, CA 92093, USA
    Free Radic Biol Med 41:590-600. 2006
    ..we attempted to genetically increase oxidative stress in Atm-deficient mice by either removing cytosolic Sod1 or reducing mitochondrial Sod2, or we attempted to decrease oxidative stress by treatment of Atm-deficient mice ..
  37. pmc Disease-associated mutations at copper ligand histidine residues of superoxide dismutase 1 diminish the binding of copper and compromise dimer stability
    Jiou Wang
    Department of Pathology, Johns Hopkins University, Baltimore, Maryland 21205, USA
    J Biol Chem 282:345-52. 2007
    A subset of superoxide dismutase 1 (Cu/Zn-SOD1) mutants that cause familial amyotrophic lateral sclerosis (FALS) have heightened reactivity with (-)ONOO and H(2)O(2) in vitro...
  38. ncbi Oxidative modification to cysteine sulfonic acid of Cys111 in human copper-zinc superoxide dismutase
    Noriko Fujiwara
    Department of Biochemistry, Hyogo College of Medicine, Nishinomiya, Hyogo, 663 8501, Japan
    J Biol Chem 282:35933-44. 2007
    Copper-zinc superoxide dismutase (SOD1) plays a protective role against oxidative stress...
  39. pmc Detergent-insoluble aggregates associated with amyotrophic lateral sclerosis in transgenic mice contain primarily full-length, unmodified superoxide dismutase-1
    Bryan F Shaw
    Department of Chemistry and Biochemistry, David Geffen School of Medicine, UCLA, Los Angeles, CA 90095, USA
    J Biol Chem 283:8340-50. 2008
    Determining the composition of aggregated superoxide dismutase 1 (SOD1) species associated with amyotrophic lateral sclerosis (ALS), especially with respect to co-aggregated proteins and post-translational modifications, could identify ..
  40. pmc Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria
    Christine Vande Velde
    Ludwig Institute and Departments of Medicine and Neuroscience, University of California at San Diego, 9500 Gilman Drive, La Jolla, CA 92093 0670, USA
    Proc Natl Acad Sci U S A 105:4022-7. 2008
    Mutations in copper/zinc superoxide dismutase (SOD1) are causative for dominantly inherited amyotrophic lateral sclerosis (ALS)...
  41. pmc The Psi(m) depolarization that accompanies mitochondrial Ca2+ uptake is greater in mutant SOD1 than in wild-type mouse motor terminals
    Khanh T Nguyen
    Neuroscience Program, University of Miami, Miller School of Medicine, Miami, FL 33136, USA
    Proc Natl Acad Sci U S A 106:2007-11. 2009
    ..5 times larger in motor terminals from presymptomatic mice expressing mutations of human superoxide dismutase I (SOD1) that cause familial amyotrophic lateral sclerosis (SOD1-G85R, which lacks dismutase activity; SOD1-G93A, which ..
  42. ncbi Variants of the heavy neurofilament subunit are associated with the development of amyotrophic lateral sclerosis
    D A Figlewicz
    Centre for Research in Neuroscience, McGill University, Montreal, Quebec, Canada
    Hum Mol Genet 3:1757-61. 1994
    ..We propose that changes in the KSP-repeat domain may affect the cross-linking properties of the heavy neurofilament subunit and perhaps contribute to the development of neurofilamentous swellings in motor neurons, a hallmark of ALS...
  43. ncbi Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1
    L I Bruijn
    Ludwig Institute for Cancer Research and Departments of Medicine and Neuroscience, University of California, La Jolla, CA 92093, USA
    Science 281:1851-4. 1998
    ..mice expressing familial amyotrophic lateral sclerosis (ALS)-linked mutations in the enzyme superoxide dismutase (SOD1) have shown that motor neuron death arises from a mutant-mediated toxic property or properties...
  44. ncbi Neurodegeneration in striatum induced by the mitochondrial toxin 3-nitropropionic acid: role of matrix metalloproteinase-9 in early blood-brain barrier disruption?
    Gyung W Kim
    Department of Neurosurgery, Department of Neurology and Neurological Sciences, and Program in Neurosciences, Stanford University School of Medicine, Stanford, California 94305 5487, USA
    J Neurosci 23:8733-42. 2003
    ..Furthermore, transgenic mice that overexpress copper/zinc-superoxide dismutase (SOD1) show decreased lesion size and edema along with decreased immunoreactivity for MMP-9, compared with wild-type ..
  45. ncbi Complex III releases superoxide to both sides of the inner mitochondrial membrane
    Florian L Muller
    Department of Cellular Biology, University of Texas Health Science Center, San Antonio, TX 78284 7762, USA
    J Biol Chem 279:49064-73. 2004
    ..This explains extramitochondrial release of superoxide but raises the question of how superoxide could reach the matrix. We discuss two models explaining this result...
  46. 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
    Mutations in the intracellular metalloenzyme superoxide dismutase 1 (SOD1) are linked to neurotoxicity in familial amyotrophic lateral sclerosis (ALS) by an unclear mechanism...
  47. ncbi Absence of CuZn superoxide dismutase leads to elevated oxidative stress and acceleration of age-dependent skeletal muscle atrophy
    Florian L Muller
    Department of Cellular and Structural Biology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229 3900, USA
    Free Radic Biol Med 40:1993-2004. 2006
    ..Thus, the absence of CuZnSOD imposes elevated oxidative stress, loss of muscle mass, and physiological consequences that resemble an ..
  48. pmc ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1
    Hideki Nishitoh
    Core Research for Evolutional Science and Technology CREST, Japan Science and Technology Corporation, Bunkyo ku, Tokyo 113 0033, Japan
    Genes Dev 22:1451-64. 2008
    Mutation in Cu/Zn-superoxide dismutase (SOD1) is a cause of familial amyotrophic lateral sclerosis (ALS)...
  49. pmc Lysyl-tRNA synthetase is a target for mutant SOD1 toxicity in mitochondria
    Hibiki Kawamata
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, New York 10065, USA
    J Biol Chem 283:28321-8. 2008
    ..The majority of familial forms of ALS are caused by mutations in the Cu,Zn-superoxide dismutase (SOD1)...
  50. doi Recruitment of mitochondria into apoptotic signaling correlates with the presence of inclusions formed by amyotrophic lateral sclerosis-associated SOD1 mutations
    Kai Y Soo
    Department of Biochemistry and Molecular Biology, Monash University, Victoria, Australia
    J Neurochem 108:578-90. 2009
    Mutations in Cu, Zn-superoxide dismutase 1 (SOD1) are associated with degeneration of motor neurons in the disease, familial amyotrophic lateral sclerosis...
  51. doi Skeletal muscle is a primary target of SOD1G93A-mediated toxicity
    Gabriella Dobrowolny
    Institute Pasteur Cenci Bolognetti, Department of Histology and Medical Embryology, CE BEMM and IIM, Sapienza University of Rome, Via A Scarpa, 14 Rome 00161, Italy
    Cell Metab 8:425-36. 2008
    The antioxidant enzyme superoxide dismutase 1 (SOD1) is a critical player of the antioxidative defense whose activity is altered in several chronic diseases, including amyotrophic lateral sclerosis...
  52. doi Haemolytic anaemia and alterations in hepatic iron metabolism in aged mice lacking Cu,Zn-superoxide dismutase
    Rafał R Starzyński
    Department of Molecular Biology, Institute of Genetics and Animal Breeding, Polish Academy of Sciences, Jastrzebiec, Wolka Kosowska, Poland
    Biochem J 420:383-90. 2009
    ..observations indicate that the recently described chronic, but moderate regenerative, haemolytic anaemia of aged SOD1 (superoxide dismutase 1)-knockout mice is associated with red blood cell modifications and sensitivity to both ..
  53. ncbi An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
    P C Wong
    Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Neuron 14:1105-16. 1995
    Mutations in Cu/Zn superoxide dismutase (SOD1) cause a subset of cases of familial amyotrophic lateral sclerosis. Four lines of mice accumulating one of these mutant proteins (G37R) develop severe, progressive motor neuron disease...
  54. ncbi Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation
    M E Gurney
    Department of Cell and Molecular Biology, Northwestern University Medical School, Chicago, IL 60611
    Science 264:1772-5. 1994
    ..The results show that dominant, gain-of-function mutations in SOD contribute to the pathogenesis of familial ALS...
  55. ncbi Novel mutations in an otherwise strictly conserved domain of CuZn superoxide dismutase
    R M Luche
    Department of Pediatrics, University of California, San Francisco 94143 0748, USA
    Mol Cell Biochem 168:191-4. 1997
    All mutations in the human gene for CuZn superoxide dismutase (CuZnSOD) reported to date are associated with the disease amyotrophic lateral sclerosis (ALS)...
  56. ncbi Reduced fertility in female mice lacking copper-zinc superoxide dismutase
    Y S Ho
    Institute of Chemical Toxicology and Department of Biochemistry and Molecular Biology, Wayne State University, Detroit, Michigan 48201, USA
    J Biol Chem 273:7765-9. 1998
    ..These data, for the first time, suggest a role of oxygen free radicals in causing abnormality of female reproduction in mammals...
  57. ncbi Cu/Zn SOD deficiency potentiates hearing loss and cochlear pathology in aged 129,CD-1 mice
    S L McFadden
    Center for Hearing and Deafness, State University of New York, Buffalo, New York 14214, USA
    J Comp Neurol 413:101-12. 1999
    ..The results indicate that Cu/Zn SOD deficiencies increase the vulnerability of the cochlea to damage associated with normal aging, presumably through metabolic pathways involving the superoxide radical...
  58. ncbi Mice lacking cytosolic copper/zinc superoxide dismutase display a distinctive motor axonopathy
    J M Shefner
    Department of Neurology, State University of New York Health Science Center at Syracuse, 13210, USA
    Neurology 53:1239-46. 1999
    ..To characterize the motor neuron dysfunction in two models by performing physiologic and morphometric studies...
  59. ncbi Targeted disruption of the mouse Sod I gene makes the hearts vulnerable to ischemic reperfusion injury
    T Yoshida
    Cardiovascular Research Center, Department of Surgery, University of Connecticut School of Medicine, Farmington, CT 06030 1110, USA
    Circ Res 86:264-9. 2000
    ....
  60. ncbi In vitro photochemical cataract in mice lacking copper-zinc superoxide dismutase
    A Behndig
    Department of Clinical Science Ophthalmology, Umea University Hospital, Umea, Sweden
    Free Radic Biol Med 31:738-44. 2001
    ..We conclude that CuZn-SOD is an important superoxide scavenger in the lens, and that it may have a protective role against cataract formation...
  61. ncbi Postischemic recovery of contractile function is impaired in SOD2(+/-) but not SOD1(+/-) mouse hearts
    Gregory K Asimakis
    Department of Surgery, Division of Cardiothoracic Surgery, The University of Texas Medical Branch, Galveston, Tex 77555 0828, USA
    Circulation 105:981-6. 2002
    ..Superoxide dismutase (SOD) is a major defense mechanism against ROS. The purpose of this study was to evaluate the contributions of cytosolic (SOD1) and mitochondrial (SOD2) isoforms to protect against myocardial stunning.
  62. pmc Amyotrophic lateral sclerosis: a proposed mechanism
    Ayako Okado-Matsumoto
    Department of Biochemistry, Duke University Medical Center, Durham, NC 27710, USA
    Proc Natl Acad Sci U S A 99:9010-4. 2002
    Missense mutations in Cu,Zn-superoxide dismutase (SOD1) account for approximately 20% of familial amyotrophic lateral sclerosis (FALS) through some, as yet undefined, toxic gain of function that leads to gradual death of motor neurons...
  63. 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
    ..b>sod1, or cuzn-sod (ec 1.15.1...
  64. 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
    Accumulating evidence indicates that abnormal conformation of mutant superoxide dismutase 1 (SOD1) is an essential feature underlying the pathogenesis of mutant SOD1-linked familial amyotrophic lateral sclerosis (ALS)...
  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 CuZnSOD deficiency leads to persistent and widespread oxidative damage and hepatocarcinogenesis later in life
    Sailaja Elchuri
    Department of Neurology and Neurological Sciences, Stanford University, Stanford, CA 94305, USA
    Oncogene 24:367-80. 2005
    Mice deficient in CuZn superoxide dismutase (CuZnSOD) showed no overt abnormalities during development and early adulthood, but had a reduced lifespan and increased incidence of neoplastic changes in the liver...
  67. ncbi A Rac1/phosphatidylinositol 3-kinase/Akt3 anti-apoptotic pathway, triggered by AlsinLF, the product of the ALS2 gene, antagonizes Cu/Zn-superoxide dismutase (SOD1) mutant-induced motoneuronal cell death
    Kohsuke Kanekura
    Department of Pharmacology and Neurosciences, Keio University School of Medicine, 35 Shinanomachi, Shinjuku ku, Tokyo 160 8582, Japan
    J Biol Chem 280:4532-43. 2005
    AlsinLF, the product of the ALS2 gene, inhibits Cu/Zn-superoxide dismutase (SOD1) mutant-induced neurotoxicity via its Rho guanine nucleotide-exchanging factor domain...
  68. ncbi Mouse motor neuron disease caused by truncated SOD1 with or without C-terminal modification
    Yasuhiro Watanabe
    Department of Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Nishi cho 36 1, Yonago 683 8504, Japan
    Brain Res Mol Brain Res 135:12-20. 2005
    Mutation of Cu/Zn superoxide dismutase (SOD1) contributes to a portion of the cases of familial amyotrophic lateral sclerosis (FALS)...
  69. pmc Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model
    Hitoshi Kikuchi
    Department of Neurology, Columbia University, New York, NY 10032, USA
    Proc Natl Acad Sci U S A 103:6025-30. 2006
    Mutation in superoxide dismutase-1 (SOD1), which is a cause of ALS, alters the folding patterns of this protein. Accumulation of misfolded mutant SOD1 might activate endoplasmic reticulum (ER) stress pathways...
  70. pmc Disulfide cross-linked protein represents a significant fraction of ALS-associated Cu, Zn-superoxide dismutase aggregates in spinal cords of model mice
    Yoshiaki Furukawa
    Department of Chemistry, Northwestern University, 2145 Sheridan Road, Evanston, IL 60208, USA
    Proc Natl Acad Sci U S A 103:7148-53. 2006
    Point mutations in Cu, Zn-superoxide dismutase (SOD1) cause a familial form of the neurodegenerative disease amyotrophic lateral sclerosis (ALS)...
  71. pmc Drusen, choroidal neovascularization, and retinal pigment epithelium dysfunction in SOD1-deficient mice: a model of age-related macular degeneration
    Yutaka Imamura
    Department of Ophthalmology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku ku, Tokyo 160 8582, Japan
    Proc Natl Acad Sci U S A 103:11282-7. 2006
    ..We show that mice deficient in Cu, Zn-superoxide dismutase (SOD1) have features typical of age-related macular degeneration in humans...
  72. ncbi CuZn-SOD deficiency causes ApoB degradation and induces hepatic lipid accumulation by impaired lipoprotein secretion in mice
    Satoshi Uchiyama
    Research Team for Molecular Biomarkers, Tokyo Metropolitan Institute of Gerontology, Tokyo 173 0015, Japan
    J Biol Chem 281:31713-9. 2006
    ..and compared the hepatic lipid metabolisms of liver-specific Sod2 (superoxide dismutase 2) knock-out (Sod2 KO), Sod1 knock-out (Sod1 KO), and Sod1/liver-specific Sod2 double knock-out mice (double KO)...
  73. ncbi Heterozygous CuZn superoxide dismutase deficiency produces a vascular phenotype with aging
    Sean P Didion
    Department of Internal Medicine, Cardiovascular Center, University of Iowa Carver College of Medicine, Iowa City 52242, USA
    Hypertension 48:1072-9. 2006
    ..of this study was to test the hypothesis that loss of a single copy of the gene for CuZn superoxide dismutase (CuZnSOD) increases vascular superoxide levels and produces vascular dysfunction with aging...
  74. pmc Elevated oxidative stress in erythrocytes due to a SOD1 deficiency causes anaemia and triggers autoantibody production
    Yoshihito Iuchi
    Department of Biomolecular Function, Graduate School of Medical Science, Yamagata University, Yamagata, Japan
    Biochem J 402:219-27. 2007
    ..Despite the important role of Cu/Zn SOD (superoxide dismutase) encoded by SOD1, SOD1-/- mice appear to grow normally under conventional breeding conditions...
  75. ncbi Inhibition of p38 mitogen activated protein kinase activation and mutant SOD1(G93A)-induced motor neuron death
    Maarten Dewil
    Laboratory for Neurobiology, Experimental Neurology, University of Leuven, Campus Gasthuisberg, Herestraat 49, B 3000 Leuven, Belgium
    Neurobiol Dis 26:332-41. 2007
    ..We studied the relevance of p38 MAPK for motor neuron degeneration in the mutant SOD1 mouse...
  76. 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
    ..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 motor neurons...
  77. ncbi 17Beta-estradiol reduces nitrotyrosine immunoreactivity and increases SOD1 and SOD2 immunoreactivity in nigral neurons in male mice following MPTP insult
    Wanida Tripanichkul
    Department of Anatomy, Faculty of Medicine, Srinakharinwirot University, Bangkok, 10110, Thailand
    Brain Res 1164:24-31. 2007
    ..nigral neurons and glia for nitrotyrosine (NT, a stable marker for oxidative stress), Cu/Zn superoxide dismutase (SOD1) and Mn superoxide dismutase (SOD2) in the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) mouse model...
  78. 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
    One of the mechanisms by which mutations in superoxide dismutase 1 (SOD1) cause familial amyotrophic lateral sclerosis (fALS) is proposed to involve the accumulation of detergent-insoluble, disulfide-cross-linked, mutant protein...
  79. pmc Retinal dysfunction and progressive retinal cell death in SOD1-deficient mice
    Kouhei Hashizume
    Department of Ophthalmology, Inaida Laboratory, Keio University School of Medicine, 35 Shinanomachi, Shinjuku ku, Tokyo 160 8582, Japan
    Am J Pathol 172:1325-31. 2008
    The superoxide dismutase (SOD) family is a major antioxidant system, and deficiency of Cu,Zn-superoxide dismutase (SOD1) in mice leads to many different phenotypes that resemble accelerated aging...
  80. 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
    ..We used mutant SOD1(G93A) transgenic mice, a commonly used animal model for ALS...
  81. pmc Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death
    Hristelina S Ilieva
    Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, CA 92093, USA
    Proc Natl Acad Sci U S A 105:12599-604. 2008
    ..In mouse models of inherited ALS caused by mutations in superoxide dismutase (SOD1), mutant dynein modestly prolonged survival in the one mouse model with the most extensive motor neuron loss (SOD(..
  82. doi The effects of superoxide dismutase knockout on the oxidative stress parameters and survival of mouse erythrocytes
    Agnieszka Grzelak
    Department of Molecular Biophysics, University of Łódź, Banacha 12 16, 90 237 Łódź, Poland
    Cell Mol Biol Lett 14:23-34. 2009
    The erythrocytes of 12-month old Sod1 (-/-) mice showed an increased level of reactive oxygen species (ROS), as estimated by the degree of dihydroethidine and dihydrorhodamine oxidation, and the increased level of Heinz bodies...
  83. pmc Sequestosome 1/p62 links familial ALS mutant SOD1 to LC3 via an ubiquitin-independent mechanism
    Jozsef Gal
    Department of Molecular and Cellular Biochemistry, University of Kentucky, Lexington, Kentucky 40536, USA
    J Neurochem 111:1062-73. 2009
    ..a critical pathway for degrading misfolded and/or damaged proteins, including the copper-zinc superoxide dismutase (SOD1) mutants linked to familial ALS...
  84. pmc Increased superoxide in vivo accelerates age-associated muscle atrophy through mitochondrial dysfunction and neuromuscular junction degeneration
    Youngmok C Jang
    Department of Cellular and Structural Biology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78245, USA
    FASEB J 24:1376-90. 2010
    ..examine the role of chronic oxidative stress in vivo, we used a mouse model that lacks the antioxidant enzyme CuZnSOD (Sod1). Sod1(-/-) mice are characterized by high levels of oxidative damage and an acceleration of sarcopenia...
  85. pmc Effects of ALS-related SOD1 mutants on dynein- and KIF5-mediated retrograde and anterograde axonal transport
    Ping Shi
    Department of Molecular and Cellular Biochemistry, College of Medicine, University of Kentucky, Lexington, KY 40536, USA
    Biochim Biophys Acta 1802:707-16. 2010
    ..We have previously shown that several familial ALS-linked copper-zinc superoxide dismutase (SOD1) mutants (A4V, G85R, and G93A) interacted and colocalized with the retrograde dynein-dynactin motor complex in ..
  86. doi Essential role of copper-zinc superoxide dismutase for ischemia-induced neovascularization via modulation of bone marrow-derived endothelial progenitor cells
    Jessika Groleau
    Department of Cardiovascular Research, Centre Hospitalier de l Universite de Montreal, 1560 Sherbrooke E, Montreal, QC H2L 4M1, Canada
    Arterioscler Thromb Vasc Biol 30:2173-81. 2010
    ..To investigate the effect of oxidative stress on ischemia-induced neovascularization in copper-zinc (CuZn) superoxide dismutase (SOD)-deficient mice...
  87. pmc SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse
    Lindsey R Fischer
    Department of Neurology, Emory University School of Medicine, Atlanta, GA 30322, USA
    Brain 134:196-209. 2011
    ..oxidative stress-mediated axonal degeneration in mice lacking the antioxidant enzyme, Cu,Zn superoxide dismutase (SOD1)...
  88. ncbi Rapamycin treatment augments motor neuron degeneration in SOD1(G93A) mouse model of amyotrophic lateral sclerosis
    XiaoJie Zhang
    The Key Laboratory of Stem Cell Biology, Institute of Health Sciences, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences and Shanghai Jiao Tong University School of Medicine, Shanghai, China
    Autophagy 7:412-25. 2011
    ..is accumulated progressively in the spinal cord, indicating that the possibility of impaired autophagic flux in the SOD1(G93A) mice...
  89. pmc Hydrogen-deuterium exchange in vivo to measure turnover of an ALS-associated mutant SOD1 protein in spinal cord of mice
    George W Farr
    Howard Hughes Medical Institute, Yale University School of Medicine, Boyer Center, New Haven, Connecticut 06510, USA
    Protein Sci 20:1692-6. 2011
    Mutations of cytosolic Cu/Zn superoxide dismutase 1 (SOD1) in humans and overexpression of mutant human SOD1 genes in transgenic mice are associated with the motor neuron degenerative condition known as amyotrophic lateral sclerosis (ALS; ..
  90. 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
    ....
  91. doi Cytoplasmic superoxide causes bone fragility owing to low-turnover osteoporosis and impaired collagen cross-linking
    Hidetoshi Nojiri
    Molecular Gerontology, Tokyo Metropolitan Institute of Gerontology, Itabashi ku, Tokyo, Japan
    J Bone Miner Res 26:2682-94. 2011
    ..the bone tissue of mice deficient in cytoplasmic copper/zinc superoxide dismutase (CuZn-SOD, encoded by the Sod1 gene; Sod1(-/-))...
  92. pmc SOD1 (copper/zinc superoxide dismutase) deficiency drives amyloid β protein oligomerization and memory loss in mouse model of Alzheimer disease
    Kazuma Murakami
    Molecular Gerontology, Tokyo Metropolitan Institute of Gerontology, Itabashi ku, Tokyo 173 0015, Japan
    J Biol Chem 286:44557-68. 2011
    ..Our previous study demonstrated that mice deficient in cytoplasmic copper/zinc superoxide dismutase (CuZn-SOD, SOD1) have features of drusen formation, a hallmark of age-related macular degeneration (Imamura, Y., Noda, S...
  93. doi Amyloid precursor protein (APP) contributes to pathology in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis
    J Barney Bryson
    Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK
    Hum Mol Genet 21:3871-82. 2012
    ..A4) precursor protein (APP) in muscle fibres coincides with symptom onset in both sporadic ALS patients and the SOD1(G93A) mouse model of familial ALS...
  94. pmc CuZnSOD gene deletion targeted to skeletal muscle leads to loss of contractile force but does not cause muscle atrophy in adult mice
    Yiqiang Zhang
    Department of Physiology, University of Texas Health Science Center, San Antonio, Texas 78249, USA
    FASEB J 27:3536-48. 2013
    We have previously shown that deletion of CuZnSOD in mice (Sod1(-/-) mice) leads to accelerated loss of muscle mass and contractile force during aging...
  95. pmc Elevated protein carbonylation, and misfolding in sciatic nerve from db/db and Sod1(-/-) mice: plausible link between oxidative stress and demyelination
    Ryan T Hamilton
    Department of Cellular and Structural Biology, University of Texas Health Science Center, San Antonio, Texas, USA
    PLoS ONE 8:e65725. 2013
    ..the leptin receptor deficient mouse (dbdb) model of diabetic polyneuropathy and 2) superoxide dismutase 1 knockout (Sod1(-/-) ) mouse model of in vivo oxidative stress...
  96. ncbi A low expressor line of transgenic mice carrying a mutant human Cu,Zn superoxide dismutase (SOD1) gene develops pathological changes that most closely resemble those in human amyotrophic lateral sclerosis
    M C Dal Canto
    Department of Pathology Neuropathology, Northwestern University Medical School, Chicago, IL 60611, USA
    Acta Neuropathol 93:537-50. 1997
    ..lateral sclerosis (ALS) carry one of several missense mutations in the gene for Cu,Zn superoxide dismutase (SOD1)...
  97. ncbi Ubiquitous overexpression of CuZn superoxide dismutase does not extend life span in mice
    T T Huang
    Department of Pediatrics, University of California, San Francisco 94143 0546, USA
    J Gerontol A Biol Sci Med Sci 55:B5-9. 2000
    ..the life spans of transgenic mice on a CD-1 background expressing increased levels of CuZn superoxide dismutase (CuZnSOD), the enzyme that metabolizes superoxide radicals, were determined...
  98. ncbi Increased superoxide and vascular dysfunction in CuZnSOD-deficient mice
    Sean P Didion
    Department of Internal Medicine, Cardiovascular Center, The University of Iowa College of Medicine, Iowa City, Iowa 52242 1081, USA
    Circ Res 91:938-44. 2002
    ..We tested the hypothesis that selective loss of CuZnSOD results in increased superoxide and altered vascular responsiveness in CuZnSOD-deficient (CuZnSOD(-/-)) mice ..
  99. ncbi Developmental changes in murine brain antioxidant enzymes
    Janine Y Khan
    Department of Pediatrics and Molecular Pharmacology, Northwestern University, Chicago, IL 60611, USA
    Pediatr Res 54:77-82. 2003
    ..These data will be important in our future understanding of the mechanisms by which hypoxia mediates injury in the immature and the mature brain...
  100. ncbi The spatio-temporal expression pattern of cytoplasmic Cu/Zn superoxide dismutase (SOD1) mRNA during mouse embryogenesis
    Jung Min Yon
    College of Veterinary Medicine and Research Institute of Veterinary Medicine, Chungbuk National University, Cheongju, 361 763, Korea
    J Mol Histol 39:95-103. 2008
    The cytoplasmic Cu/Zn-superoxide dismutase (SOD1) represents along with catalase and glutathione peroxidase at the first defense line against reactive oxygen species in all aerobic organisms, but little is known about its distribution in ..
  101. pmc SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model
    Maged M Harraz
    Department of Anatomy and Cell Biology, University of Iowa Carver College of Medicine, Iowa City, Iowa 52242, USA
    J Clin Invest 118:659-70. 2008
    ..sclerosis (ALS) is associated with enhanced redox stress caused by dominant mutations in superoxide dismutase-1 (SOD1). SOD1 is a cytosolic enzyme that facilitates the conversion of superoxide (O(2)(*-)) to H(2)O(2)...

Research Grants23

  1. INNER EAR IMMUNE RESPONSES
    Elizabeth Keithley; Fiscal Year: 2002
    ..Such knowledge will enable clinicians to improve the treatments administered to patients who experience the unfortunate and potentially devastating trauma of rapidly progressing sensorineural hearing loss. ..
  2. DNA Damage/Repair and Cell Death
    Lee Martin; Fiscal Year: 2009
    ....
  3. OXIDATION OF MACROMOLECULES IN SPINAL CORD INJURY
    Danxia Liu; Fiscal Year: 2001
    ..This will directly test whether free radicals damage neurons by inducing oxidation of major cell components and will provide strategies for therapy. ..
  4. MECHANISMS OF NEURONAL APOPTOSIS IN VIVO
    Lee Martin; Fiscal Year: 2009
    ..This work can define a new mitochondrial mechanism for target deprivation-induced neurodegeneration and can improve the understanding of the cellular and molecular mechanisms of neuronal apoptosis in vivo ..
  5. SYMPOSIA FOR ASSOCIATION FOR RESEARCH IN OTOLARYNGOLOGY
    Elizabeth Keithley; Fiscal Year: 2008
    ..abstract_text> ..
  6. Regulation of Key Processes in Oocyte Biology
    Martin Matzuk; Fiscal Year: 2007
    ..Compromised fertility in knockout models lacking these oocyte-specific genes would demonstrate that these proteins are potential contraceptive targets and possibly mutated in infertile women. ..
  7. RNA-mediated motor neuron degeneration
    WILLIAM SCHLAEPFER; Fiscal Year: 2007
    ..the synergistic effects of RNA-mediated phenomena on mSOD-1-mediated motor neuron degeneration and for testing the modulating effects of trans-acting factors on RNA-mediated and mSOD-1 mediated motor neuron degeneration ..
  8. Mechanisms of Motor Neuron Death
    Lee Martin; Fiscal Year: 2006
    ..abstract_text> ..
  9. Endothelial Function in the Cerebral Circulation
    Frank Faraci; Fiscal Year: 2009
    ..These studies should provide new insight into mechanisms of cerebral vascular protection during hypertension. ..
  10. The Mouse as an Instrument for Ear Research II
    Kenneth Johnson; Fiscal Year: 2005
    ..Ultimately, this meeting will bring students and established investigators, with varying expertise and experience, together to discuss their research findings, identify areas of common research interest and develop future directions. ..
  11. Cord Blood Cells in ALS: Replacement of Protection?
    Svitlana Garbuzova Davis; Fiscal Year: 2004
    ..The results of this study may provide the basis for developing a novel, non-invasive therapy, easily accessed by ALS patients. ..
  12. INTRAOCULAR TRANSPORT AND METABOLISM
    Venkat Reddy; Fiscal Year: 2004
    ..changes in lens transparency in other transgenic animal models, which lack the enzyme CuZn superoxide dismutase (CuZnSOD)...
  13. NEUROFILAMENT EXPRESSION AND MOTOR NEURON DEGENERATION
    WILLIAM SCHLAEPFER; Fiscal Year: 2004
    ..The studies involve pathogenetic mechanisms causing motor neuron degeneration and have far-reaching implications about the diagnosis, treatment and cure of motor neuron diseases. ..
  14. Defining the Mammalian Intercellular Bridge Interactome
    Martin M Matzuk; Fiscal Year: 2010
    ..Our proposed Specific Aims will also help us to develop additional high throughput screening assays for the identification of small molecule disruptors of the intercellular bridge. ..
  15. MOUSE MODELS TO STUDY GONADAL TUMOR DEVELOPMENT
    Martin M Matzuk; Fiscal Year: 2010
    ..Characterization of these mice will lead to important insights into the development of gonadal cancers in men and women and the roles of several proteins in reproductive physiology. ..
  16. REGULATION AND FUNCTION OF LUNG SPECIFIC FREAC GENES
    Ye Shih Ho; Fiscal Year: 2002
    ..abstract_text> ..
  17. Neuroprotection by a metalloporphyrin in spinal injury
    Danxia Liu; Fiscal Year: 2009
    ..The immunohistochemical stained cells will be counted to determine the effect of MnTBAP on cell death. The therapeutic potential of MnTBAP will be assessed by behavioral tests. ..
  18. NUTRITIONAL COPPER STATUS AND THE NERVOUS SYSTEM
    JOSEPH ROBERT PROHASKA; Fiscal Year: 2010
    ..AIM 4: We will test the hypothesis that limitation in Cu-dependent ferroxidases lead to lower brain iron and are responsible by comparing rats reared on an iron-fortified diet to restore brain iron. ..
  19. The Mouse as an Instrument in Hearing Research III
    Kenneth Johnson; Fiscal Year: 2008
    ..Promoting the responsible use of appropriate models is critical to the development of effective new therapeutic approaches to treating human disease. [unreadable] [unreadable] [unreadable] [unreadable]..
  20. MORPHOLOGY AND FUNCTION OF BRAIN VESSELS
    GARY BAUMBACH; Fiscal Year: 2006
    ..These studies should provide important new insight into the mechanisms of vascular remodeling. [unreadable] [unreadable] [unreadable]..
  21. CNS Stem Cells for Alzheimer's Disease Therapy
    George Carlson; Fiscal Year: 2005
    ..The objectives of this grant are to test human neural stern cells as candidate therapeutics for the treatment of Alzheimer's disease. ..
  22. The Mouse as a Tool for Ear Research
    Kenneth Johnson; Fiscal Year: 2003
    ..abstract_text> ..
  23. 24-Capillary Reveal Mutation Discovery System
    George Carlson; Fiscal Year: 2003
    ..The Spectrumedix Reveal 2410 will not only accelerate current research at McLaughlin Research Institute, but help in recruiting new biomedical researchers in the field of neurogenetics. ..