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
Products:     Sod1

Top Publications

  1. Zhong Z, Deane R, Ali Z, Parisi M, Shapovalov Y, O Banion M, et al. ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat Neurosci. 2008;11:420-2 pubmed publisher
    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. Muller F, Lustgarten M, Jang Y, Richardson A, Van Remmen H. Trends in oxidative aging theories. Free Radic Biol Med. 2007;43:477-503 pubmed
  3. Han E, Muller F, Perez V, Qi W, Liang H, Xi L, et al. The in vivo gene expression signature of oxidative stress. Physiol Genomics. 2008;34:112-26 pubmed publisher
    ..induced in vivo by diquat or by genetic ablation of the major antioxidant enzymes CuZn-superoxide dismutase (Sod1) and glutathione peroxidase-1 (Gpx1)...
  4. Murakami K, Inagaki J, Saito M, Ikeda Y, Tsuda C, Noda Y, et al. Skin atrophy in cytoplasmic SOD-deficient mice and its complete recovery using a vitamin C derivative. Biochem Biophys Res Commun. 2009;382:457-61 pubmed publisher
    ..The CuZn-SOD-deficient mice might be applicable to evaluation of therapeutic medicines against skin ageing. ..
  5. Yonashiro R, Sugiura A, Miyachi M, Fukuda T, Matsushita N, Inatome R, et al. Mitochondrial ubiquitin ligase MITOL ubiquitinates mutant SOD1 and attenuates mutant SOD1-induced reactive oxygen species generation. Mol Biol Cell. 2009;20:4524-30 pubmed publisher
    ..We found that in the mitochondria, MITOL interacted with and ubiquitinated mSOD1 but not wild-type SOD1. In vitro ubiquitination assay revealed that MITOL directly ubiquitinates mSOD1...
  6. Zhang Y, Davis C, Sakellariou G, Shi Y, Kayani A, Pulliam D, et al. CuZnSOD gene deletion targeted to skeletal muscle leads to loss of contractile force but does not cause muscle atrophy in adult mice. FASEB J. 2013;27:3536-48 pubmed publisher
    We have previously shown that deletion of CuZnSOD in mice (Sod1(-/-) mice) leads to accelerated loss of muscle mass and contractile force during aging...
  7. Vasilaki A, van der Meulen J, Larkin L, Harrison D, Pearson T, Van Remmen H, et al. 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. Aging Cell. 2010;9:979-90 pubmed publisher
    ..Mice lacking the Cu,Zn superoxide dismutase (Cu,ZnSOD, SOD1) show an accelerated, age-related loss of muscle mass and function...
  8. Turner B, Atkin J, Farg M, Zang D, Rembach A, Lopes E, et al. Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis. J Neurosci. 2005;25:108-17 pubmed
    Mutations in the intracellular metalloenzyme superoxide dismutase 1 (SOD1) are linked to neurotoxicity in familial amyotrophic lateral sclerosis (ALS) by an unclear mechanism...
  9. Israelson A, Arbel N, Da Cruz S, Ilieva H, Yamanaka K, Shoshan Barmatz V, et al. Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS. Neuron. 2010;67:575-87 pubmed publisher
    Mutations in superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by loss of motor neurons...
  10. Sakellariou G, Davis C, Shi Y, Ivannikov M, Zhang Y, Vasilaki A, et al. Neuron-specific expression of CuZnSOD prevents the loss of muscle mass and function that occurs in homozygous CuZnSOD-knockout mice. FASEB J. 2014;28:1666-81 pubmed publisher
    Deletion of copper-zinc superoxide dismutase (CuZnSOD) in Sod1(-/-) mice leads to accelerated loss of muscle mass and force during aging, but the losses do not occur with muscle-specific deletion of CuZnSOD...

Detail Information

Publications85

  1. Zhong Z, Deane R, Ali Z, Parisi M, Shapovalov Y, O Banion M, et al. ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat Neurosci. 2008;11:420-2 pubmed publisher
    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. Muller F, Lustgarten M, Jang Y, Richardson A, Van Remmen H. Trends in oxidative aging theories. Free Radic Biol Med. 2007;43:477-503 pubmed
  3. Han E, Muller F, Perez V, Qi W, Liang H, Xi L, et al. The in vivo gene expression signature of oxidative stress. Physiol Genomics. 2008;34:112-26 pubmed publisher
    ..induced in vivo by diquat or by genetic ablation of the major antioxidant enzymes CuZn-superoxide dismutase (Sod1) and glutathione peroxidase-1 (Gpx1)...
  4. Murakami K, Inagaki J, Saito M, Ikeda Y, Tsuda C, Noda Y, et al. Skin atrophy in cytoplasmic SOD-deficient mice and its complete recovery using a vitamin C derivative. Biochem Biophys Res Commun. 2009;382:457-61 pubmed publisher
    ..The CuZn-SOD-deficient mice might be applicable to evaluation of therapeutic medicines against skin ageing. ..
  5. Yonashiro R, Sugiura A, Miyachi M, Fukuda T, Matsushita N, Inatome R, et al. Mitochondrial ubiquitin ligase MITOL ubiquitinates mutant SOD1 and attenuates mutant SOD1-induced reactive oxygen species generation. Mol Biol Cell. 2009;20:4524-30 pubmed publisher
    ..We found that in the mitochondria, MITOL interacted with and ubiquitinated mSOD1 but not wild-type SOD1. In vitro ubiquitination assay revealed that MITOL directly ubiquitinates mSOD1...
  6. Zhang Y, Davis C, Sakellariou G, Shi Y, Kayani A, Pulliam D, et al. CuZnSOD gene deletion targeted to skeletal muscle leads to loss of contractile force but does not cause muscle atrophy in adult mice. FASEB J. 2013;27:3536-48 pubmed publisher
    We have previously shown that deletion of CuZnSOD in mice (Sod1(-/-) mice) leads to accelerated loss of muscle mass and contractile force during aging...
  7. Vasilaki A, van der Meulen J, Larkin L, Harrison D, Pearson T, Van Remmen H, et al. 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. Aging Cell. 2010;9:979-90 pubmed publisher
    ..Mice lacking the Cu,Zn superoxide dismutase (Cu,ZnSOD, SOD1) show an accelerated, age-related loss of muscle mass and function...
  8. Turner B, Atkin J, Farg M, Zang D, Rembach A, Lopes E, et al. Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis. J Neurosci. 2005;25:108-17 pubmed
    Mutations in the intracellular metalloenzyme superoxide dismutase 1 (SOD1) are linked to neurotoxicity in familial amyotrophic lateral sclerosis (ALS) by an unclear mechanism...
  9. Israelson A, Arbel N, Da Cruz S, Ilieva H, Yamanaka K, Shoshan Barmatz V, et al. Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS. Neuron. 2010;67:575-87 pubmed publisher
    Mutations in superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by loss of motor neurons...
  10. Sakellariou G, Davis C, Shi Y, Ivannikov M, Zhang Y, Vasilaki A, et al. Neuron-specific expression of CuZnSOD prevents the loss of muscle mass and function that occurs in homozygous CuZnSOD-knockout mice. FASEB J. 2014;28:1666-81 pubmed publisher
    Deletion of copper-zinc superoxide dismutase (CuZnSOD) in Sod1(-/-) mice leads to accelerated loss of muscle mass and force during aging, but the losses do not occur with muscle-specific deletion of CuZnSOD...
  11. Noda Y, Ota K, Shirasawa T, Shimizu T. Copper/zinc superoxide dismutase insufficiency impairs progesterone secretion and fertility in female mice. Biol Reprod. 2012;86:1-8 pubmed publisher
    Copper/zinc superoxide dismutase (CuZn-SOD, SOD1) is one of the major antioxidant enzymes, and is localized in the cytoplasm to scavenge superoxide...
  12. Didion S, Kinzenbaw D, Schrader L, Faraci F. Heterozygous CuZn superoxide dismutase deficiency produces a vascular phenotype with aging. Hypertension. 2006;48:1072-9 pubmed
    ..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...
  13. Alexianu M, Kozovska M, Appel S. Immune reactivity in a mouse model of familial ALS correlates with disease progression. Neurology. 2001;57:1282-9 pubmed
    ..of nonneuronal immune-inflammatory factors in the destruction of motor neurons in mutant superoxide dismutase-1 (SOD1) transgenic mice as a model of ALS...
  14. Yoshida T, Maulik N, Engelman R, Ho Y, Das D. Targeted disruption of the mouse Sod I gene makes the hearts vulnerable to ischemic reperfusion injury. Circ Res. 2000;86:264-9 pubmed
  15. Lu L, Wang S, Zheng L, Li X, Suswam E, Zhang X, et al. 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. J Biol Chem. 2009;284:33989-98 pubmed publisher
    ..In mice expressing ALS-associated mutant superoxide dismutase 1 (SOD1), VEGF mRNA expression in the spinal cord declines significantly prior to the onset of clinical manifestations...
  16. Nguyen K, García Chacón L, Barrett J, Barrett E, David G. The Psi(m) depolarization that accompanies mitochondrial Ca2+ uptake is greater in mutant SOD1 than in wild-type mouse motor terminals. Proc Natl Acad Sci U S A. 2009;106:2007-11 pubmed publisher
    ..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 ..
  17. Muller F, Song W, Liu Y, Chaudhuri A, Pieke Dahl S, Strong R, et al. Absence of CuZn superoxide dismutase leads to elevated oxidative stress and acceleration of age-dependent skeletal muscle atrophy. Free Radic Biol Med. 2006;40:1993-2004 pubmed
    ..Thus, the absence of CuZnSOD imposes elevated oxidative stress, loss of muscle mass, and physiological consequences that resemble an ..
  18. Behndig A, Karlsson K, Reaume A, Sentman M, Marklund S. In vitro photochemical cataract in mice lacking copper-zinc superoxide dismutase. Free Radic Biol Med. 2001;31:738-44 pubmed
    ..We conclude that CuZn-SOD is an important superoxide scavenger in the lens, and that it may have a protective role against cataract formation. ..
  19. Jang Y, Lustgarten M, Liu Y, Muller F, Bhattacharya A, Liang H, et al. Increased superoxide in vivo accelerates age-associated muscle atrophy through mitochondrial dysfunction and neuromuscular junction degeneration. FASEB J. 2010;24:1376-90 pubmed publisher
    ..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...
  20. Nimchinsky E, Young W, Yeung G, Shah R, Gordon J, Bloom F, et al. Differential vulnerability of oculomotor, facial, and hypoglossal nuclei in G86R superoxide dismutase transgenic mice. J Comp Neurol. 2000;416:112-25 pubmed
    ..However, these patterns of cellular vulnerability may provide clues for understanding the differential susceptibility of neural structures in ALS and other neurodegenerative diseases. ..
  21. Gal J, Strom A, Kwinter D, Kilty R, Zhang J, Shi P, et al. Sequestosome 1/p62 links familial ALS mutant SOD1 to LC3 via an ubiquitin-independent mechanism. J Neurochem. 2009;111:1062-73 pubmed publisher
    ..a critical pathway for degrading misfolded and/or damaged proteins, including the copper-zinc superoxide dismutase (SOD1) mutants linked to familial ALS...
  22. Nishitoh H, Kadowaki H, Nagai A, Maruyama T, Yokota T, Fukutomi H, et al. ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1. Genes Dev. 2008;22:1451-64 pubmed publisher
    Mutation in Cu/Zn-superoxide dismutase (SOD1) is a cause of familial amyotrophic lateral sclerosis (ALS)...
  23. Lev N, Ickowicz D, Barhum Y, Melamed E, Offen D. DJ-1 changes in G93A-SOD1 transgenic mice: implications for oxidative stress in ALS. J Mol Neurosci. 2009;38:94-102 pubmed publisher
    ..We used mutant SOD1(G93A) transgenic mice, a commonly used animal model for ALS...
  24. Dobrowolny G, Aucello M, Rizzuto E, Beccafico S, Mammucari C, Boncompagni S, et al. Skeletal muscle is a primary target of SOD1G93A-mediated toxicity. Cell Metab. 2008;8:425-36 pubmed publisher
    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...
  25. Zhang X, Li L, Chen S, Yang D, Wang Y, Zhang X, et al. Rapamycin treatment augments motor neuron degeneration in SOD1(G93A) mouse model of amyotrophic lateral sclerosis. Autophagy. 2011;7:412-25 pubmed
    ..is accumulated progressively in the spinal cord, indicating that the possibility of impaired autophagic flux in the SOD1(G93A) mice...
  26. Kostrominova T. Advanced age-related denervation and fiber-type grouping in skeletal muscle of SOD1 knockout mice. Free Radic Biol Med. 2010;49:1582-93 pubmed publisher
    In this study skeletal muscles from 1.5- and 10-month-old Cu/Zn superoxide dismutase (SOD1) homozygous knockout (JLSod1(-/-)) mice obtained from The Jackson Laboratory (C57Bl6/129SvEv background) were compared with muscles from age- and ..
  27. Zelko I, Mariani T, Folz R. Superoxide dismutase multigene family: a comparison of the CuZn-SOD (SOD1), Mn-SOD (SOD2), and EC-SOD (SOD3) gene structures, evolution, and expression. Free Radic Biol Med. 2002;33:337-49 pubmed
    ..b>sod1, or cuzn-sod (ec 1.15.1...
  28. Fischer L, Igoudjil A, Magrane J, Li Y, Hansen J, Manfredi G, et al. SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse. Brain. 2011;134:196-209 pubmed publisher
    ..oxidative stress-mediated axonal degeneration in mice lacking the antioxidant enzyme, Cu,Zn superoxide dismutase (SOD1)...
  29. Li Q, Spencer N, Pantazis N, Engelhardt J. Alsin and SOD1(G93A) proteins regulate endosomal reactive oxygen species production by glial cells and proinflammatory pathways responsible for neurotoxicity. J Biol Chem. 2011;286:40151-62 pubmed publisher
    ..In this context, ALS mutant forms of SOD1 enhance Rac1 activation, leading to increased Nox2-dependent microglial ROS production and neuron cell death in ..
  30. Iuchi Y, Okada F, Onuma K, Onoda T, Asao H, Kobayashi M, et al. Elevated oxidative stress in erythrocytes due to a SOD1 deficiency causes anaemia and triggers autoantibody production. Biochem J. 2007;402:219-27 pubmed
    ..Despite the important role of Cu/Zn SOD (superoxide dismutase) encoded by SOD1, SOD1-/- mice appear to grow normally under conventional breeding conditions...
  31. Pasinelli P, Belford M, Lennon N, Bacskai B, Hyman B, Trotti D, et al. Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria. Neuron. 2004;43:19-30 pubmed
    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...
  32. Kim G, Gasche Y, Grzeschik S, Copin J, Maier C, Chan P. Neurodegeneration in striatum induced by the mitochondrial toxin 3-nitropropionic acid: role of matrix metalloproteinase-9 in early blood-brain barrier disruption?. J Neurosci. 2003;23:8733-42 pubmed
    ..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 ..
  33. McFadden S, Ding D, Burkard R, Jiang H, Reaume A, Flood D, et al. Cu/Zn SOD deficiency potentiates hearing loss and cochlear pathology in aged 129,CD-1 mice. J Comp Neurol. 1999;413:101-12 pubmed
    ..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. ..
  34. Starzyński R, Canonne Hergaux F, Willemetz A, Gralak M, Woliński J, Styś A, et al. Haemolytic anaemia and alterations in hepatic iron metabolism in aged mice lacking Cu,Zn-superoxide dismutase. Biochem J. 2009;420:383-90 pubmed publisher
    ..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 ..
  35. Shi P, Strom A, Gal J, Zhu H. Effects of ALS-related SOD1 mutants on dynein- and KIF5-mediated retrograde and anterograde axonal transport. Biochim Biophys Acta. 2010;1802:707-16 pubmed publisher
    ..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 ..
  36. McFadden S, Ding D, Reaume A, Flood D, Salvi R. Age-related cochlear hair cell loss is enhanced in mice lacking copper/zinc superoxide dismutase. Neurobiol Aging. 1999;20:1-8 pubmed
    ..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...
  37. Grad L, Guest W, Yanai A, Pokrishevsky E, O Neill M, Gibbs E, et al. Intermolecular transmission of superoxide dismutase 1 misfolding in living cells. Proc Natl Acad Sci U S A. 2011;108:16398-403 pubmed publisher
    Human wild-type superoxide dismutase-1 (wtSOD1) is known to coaggregate with mutant SOD1 in familial amyotrophic lateral sclerosis (FALS), in double transgenic models of FALS, and in cell culture systems, but the structural determinants ..
  38. Kanekura K, Hashimoto Y, Kita Y, Sasabe J, Aiso S, Nishimoto I, et al. 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. J Biol Chem. 2005;280:4532-43 pubmed
    AlsinLF, the product of the ALS2 gene, inhibits Cu/Zn-superoxide dismutase (SOD1) mutant-induced neurotoxicity via its Rho guanine nucleotide-exchanging factor domain...
  39. Vukosavic S, Stefanis L, Jackson Lewis V, Guegan C, Romero N, Chen C, et al. Delaying caspase activation by Bcl-2: A clue to disease retardation in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurosci. 2000;20:9119-25 pubmed
  40. Yon J, Baek I, Lee S, Jin Y, Kim M, Nahm S, et al. The spatio-temporal expression pattern of cytoplasmic Cu/Zn superoxide dismutase (SOD1) mRNA during mouse embryogenesis. J Mol Histol. 2008;39:95-103 pubmed
    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 ..
  41. Hafezparast M, Klocke R, Ruhrberg C, Marquardt A, Ahmad Annuar A, Bowen S, et al. Mutations in dynein link motor neuron degeneration to defects in retrograde transport. Science. 2003;300:808-12 pubmed
    ..These mutations exclusively perturb neuron-specific functions of dynein. ..
  42. Hashizume K, Hirasawa M, Imamura Y, Noda S, Shimizu T, Shinoda K, et al. Retinal dysfunction and progressive retinal cell death in SOD1-deficient mice. Am J Pathol. 2008;172:1325-31 pubmed publisher
    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...
  43. Urushitani M, Kurisu J, Tsukita K, Takahashi R. Proteasomal inhibition by misfolded mutant superoxide dismutase 1 induces selective motor neuron death in familial amyotrophic lateral sclerosis. J Neurochem. 2002;83:1030-42 pubmed
    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)...
  44. West E, Prohaska J. Cu,Zn-superoxide dismutase is lower and copper chaperone CCS is higher in erythrocytes of copper-deficient rats and mice. Exp Biol Med (Maywood). 2004;229:756-64 pubmed
    ..In most cases, the ratio of CCS:SOD was several-fold higher in red blood cell extracts from copper-deficient compared with copper-adequate rodents. Determination of red cell CCS:SOD may be useful for assessing copper status of humans. ..
  45. Ligon L, LaMonte B, Wallace K, Weber N, Kalb R, Holzbaur E. Mutant superoxide dismutase disrupts cytoplasmic dynein in motor neurons. Neuroreport. 2005;16:533-6 pubmed
    ..Thus, inhibition of dynein/dynactin function may have a role in motor neuron degeneration in amyotrophic lateral sclerosis. ..
  46. Okado Matsumoto A, Fridovich I. Amyotrophic lateral sclerosis: a proposed mechanism. Proc Natl Acad Sci U S A. 2002;99:9010-4 pubmed
    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...
  47. Fischer L, Li Y, Asress S, Jones D, Glass J. Absence of SOD1 leads to oxidative stress in peripheral nerve and causes a progressive distal motor axonopathy. Exp Neurol. 2012;233:163-71 pubmed publisher
    ..denervation at the neuromuscular junction in mice lacking the antioxidant enzyme, Cu,Zn-superoxide dismutase (SOD1) (Fischer et al., 2011)...
  48. Ilieva H, Yamanaka K, Malkmus S, Kakinohana O, Yaksh T, Marsala M, et al. Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death. Proc Natl Acad Sci U S A. 2008;105:12599-604 pubmed publisher
    ..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(..
  49. Lobsiger C, Boillee S, Cleveland D. Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neurons. Proc Natl Acad Sci U S A. 2007;104:7319-26 pubmed
    ..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...
  50. Muller F, Liu Y, Van Remmen H. Complex III releases superoxide to both sides of the inner mitochondrial membrane. J Biol Chem. 2004;279:49064-73 pubmed
    ..This explains extramitochondrial release of superoxide but raises the question of how superoxide could reach the matrix. We discuss two models explaining this result...
  51. Urushitani M, Sik A, Sakurai T, Nukina N, Takahashi R, Julien J. Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis. Nat Neurosci. 2006;9:108-18 pubmed
    ..that chromogranins, components of neurosecretory vesicles, interact with mutant forms of superoxide dismutase (SOD1) that are linked to amyotrophic lateral sclerosis (ALS), but not with wild-type SOD1...
  52. Elliott J. Cytokine upregulation in a murine model of familial amyotrophic lateral sclerosis. Brain Res Mol Brain Res. 2001;95:172-8 pubmed
    ..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...
  53. Bendotti C, Tortarolo M, Suchak S, Calvaresi N, Carvelli L, Bastone A, et al. Transgenic SOD1 G93A mice develop reduced GLT-1 in spinal cord without alterations in cerebrospinal fluid glutamate levels. J Neurochem. 2001;79:737-46 pubmed
    ..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. ..
  54. Yamanaka K, Boillee S, Roberts E, Garcia M, McAlonis Downes M, Mikse O, et al. Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice. Proc Natl Acad Sci U S A. 2008;105:7594-9 pubmed publisher
    Dominant mutations in ubiquitously expressed superoxide dismutase (SOD1) cause familial ALS by provoking premature death of adult motor neurons...
  55. Hegedus J, Putman C, Tyreman N, Gordon T. Preferential motor unit loss in the SOD1 G93A transgenic mouse model of amyotrophic lateral sclerosis. J Physiol. 2008;586:3337-51 pubmed publisher
    ..The fast-twitch tibialis anterior (TA) and medial gastrocnemius (MG) muscles of transgenic SOD1(G93A) and SOD1(WT) mice were studied during the presymptomatic phase of disease progression at 60 days of age...
  56. Murakami K, Murata N, Noda Y, Tahara S, Kaneko T, Kinoshita N, et al. SOD1 (copper/zinc superoxide dismutase) deficiency drives amyloid ? protein oligomerization and memory loss in mouse model of Alzheimer disease. J Biol Chem. 2011;286:44557-68 pubmed publisher
    ..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...
  57. Huang T, Carlson E, Gillespie A, Shi Y, Epstein C. Ubiquitous overexpression of CuZn superoxide dismutase does not extend life span in mice. J Gerontol A Biol Sci Med Sci. 2000;55:B5-9 pubmed
    ..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...
  58. Saxena S, Roselli F, Singh K, Leptien K, Julien J, Gros Louis F, et al. Neuroprotection through excitability and mTOR required in ALS motoneurons to delay disease and extend survival. Neuron. 2013;80:80-96 pubmed publisher
    ..Here, we show that in mouse models of familial motoneuron (MN) disease, SOD1 mutants specifically render vulnerable MNs dependent on endogenous neuroprotection signaling involving excitability ..
  59. Karch C, Borchelt D. A limited role for disulfide cross-linking in the aggregation of mutant SOD1 linked to familial amyotrophic lateral sclerosis. J Biol Chem. 2008;283:13528-37 pubmed publisher
    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...
  60. Soo K, Atkin J, Horne M, Nagley P. Recruitment of mitochondria into apoptotic signaling correlates with the presence of inclusions formed by amyotrophic lateral sclerosis-associated SOD1 mutations. J Neurochem. 2009;108:578-90 pubmed publisher
    Mutations in Cu, Zn-superoxide dismutase 1 (SOD1) are associated with degeneration of motor neurons in the disease, familial amyotrophic lateral sclerosis...
  61. Jonsson P, Graffmo K, Brannstrom T, Nilsson P, Andersen P, Marklund S. Motor neuron disease in mice expressing the wild type-like D90A mutant superoxide dismutase-1. J Neuropathol Exp Neurol. 2006;65:1126-36 pubmed
    ..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. ..
  62. Nojiri H, Saita Y, Morikawa D, Kobayashi K, Tsuda C, Miyazaki T, et al. Cytoplasmic superoxide causes bone fragility owing to low-turnover osteoporosis and impaired collagen cross-linking. J Bone Miner Res. 2011;26:2682-94 pubmed publisher
    ..the bone tissue of mice deficient in cytoplasmic copper/zinc superoxide dismutase (CuZn-SOD, encoded by the Sod1 gene; Sod1(-/-))...
  63. Imamura Y, Noda S, Hashizume K, Shinoda K, Yamaguchi M, Uchiyama S, et al. Drusen, choroidal neovascularization, and retinal pigment epithelium dysfunction in SOD1-deficient mice: a model of age-related macular degeneration. Proc Natl Acad Sci U S A. 2006;103:11282-7 pubmed
    ..We show that mice deficient in Cu, Zn-superoxide dismutase (SOD1) have features typical of age-related macular degeneration in humans...
  64. Elchuri S, Oberley T, Qi W, Eisenstein R, Jackson Roberts L, Van Remmen H, et al. CuZnSOD deficiency leads to persistent and widespread oxidative damage and hepatocarcinogenesis later in life. Oncogene. 2005;24:367-80 pubmed
    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...
  65. Fujiwara N, Nakano M, Kato S, Yoshihara D, Ookawara T, Eguchi H, et al. Oxidative modification to cysteine sulfonic acid of Cys111 in human copper-zinc superoxide dismutase. J Biol Chem. 2007;282:35933-44 pubmed
    Copper-zinc superoxide dismutase (SOD1) plays a protective role against oxidative stress...
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    ..Mutations in the gene encoding cytosolic superoxide dismutase 1 (SOD1) account for 25% of familial ALS (FALS)...
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    ..These data will be important in our future understanding of the mechanisms by which hypoxia mediates injury in the immature and the mature brain. ..
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    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...
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    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...
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    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...