Partial deficiency of manganese superoxide dismutase exacerbates a transgenic mouse model of amyotrophic lateral sclerosis

Annals of Neurology

Volumn 47, Issue 4, 2000, Pages 447-455

  Andreassen, Ole A ^a   Ferrante, Robert J ^b,c   Klivenyi, Peter ^a   Klein, Autumn M ^b,c   Shinobu, Leslie A ^a   Epstein, Charles J ^d   Beal, M Flint ^a,e  

^a MASSACHUSETTS GENERAL HOSPITAL   (United States)

^b BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^c DEPARTMENT OF VETERANS AFFAIRS   (United States)

^d UNIVERSITY OF CALIFORNIA   (United States)

^e WEILL CORNELL MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

MANGANESE SUPEROXIDE DISMUTASE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; DOPAMINERGIC NERVE CELL; ENZYME DEFICIENCY; GENE MUTATION; LUMBAR SPINAL CORD; MOTOR NEURON DISEASE; MOUSE; NEUROPATHOLOGY; NONHUMAN; PRIORITY JOURNAL; TRANSGENIC MOUSE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; ANTERIOR HORN CELLS; CELL DEATH; DISEASE MODELS, ANIMAL; FEMALE; HUMANS; MALE; MICE; MICE, MUTANT STRAINS; MICE, TRANSGENIC; MOTOR NEURONS; OXIDATIVE STRESS; PHENOTYPE; POINT MUTATION; SUBSTANTIA NIGRA; SUPEROXIDE DISMUTASE;

EID: 0034113695     PISSN: 03645134     EISSN: None     Source Type: Journal    
DOI: 10.1002/1531-8249(200004)47:4<447::AID-ANA7>3.0.CO;2-R     Document Type: Article

References (54)

1. Brown RH Jr. Amyotrophic lateral sclerosis: recent insights from genetics and transgenic mice. Cell 1995;80:687-692
2. Rosen DR, Siddique T, Patterson D, et al. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 1993;362:59-62
3. Gurney ME, Pu H, Chiu AY, et al. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. Science 1994;264:1772-1775
4. Reaume AG, Elliott JL, Hoffman EK, et al. Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury. Nat Genet 1996;13:43-47
5. Ripps ME, Huntley GW, Hof PR, et al. Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis. Proc Natl Acad Sci USA 1995;92:689-693
6. Wong PC, Pardo CA, Borchelt DR, et al. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 1995;14:1105-1116
7. Corson LB, Strain JJ, Culotta VC, Cleveland DW. Chaperone-facilitated copper binding is a property common to several classes of familial amyotrophic lateral sclerosis-linked superoxide dismutase mutants. Proc Natl Acad Sci USA 1998;26:6361-6366
8. Crow JP, Sampson JB, Zhuang Y, et al. Decreased zinc affinity of amyotrophic lateral sclerosis-associated superoxide dismutase mutants leads to enhanced catalysis of tyrosine nitration by peroxynitrite. J Neurochem 1997;69:1936-1944
9. Lyons TJ, Liu H, Goto JJ, et al. Mutations in copper-zinc superoxide dismutase that cause amyotrophic lateral sclerosis alter the zinc binding site and the redox behavior of the protein. Proc Natl Acad Sci USA 1996;93:12240-12244
10. Hodgson EK, Fridovich I. The interaction of bovine erythrocyte superoxide dismutase with hydrogen peroxide: chemiluminescence and peroxidation. Biochemistry 1975;14:5299-5303
11. Wiedau-Pazos M, Goto JJ, Rabizadeh S, et al. Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis. Science 1996;271:515-518
12. m for hydrogen peroxide. Proc Natl Acad Sci USA 1996;93:5709-5714
13. Beckman JS, Carson M, Smith CD, Koppenol WH. ALS, SOD and peroxynitrite. Nature 1993;364:584 (Letter)
14. Lebovitz RM, Zhang H, Vogel H, et al. Neurodegeneration, myocardial injury, and perinatal death in mitochondrial superoxide dismutase-deficient mice. Proc Natl Acad Sci USA 1996; 93:9782-9787
15. Li Y, Huang T-T, Carlson EJ, et al. Dilated cardiomyopathy and neonatal lethality in mutant mice lacking manganese superoxide dismutase. Nat Genet 1995;11:376-381
16. Williams MD, Van Remmen H, Conrad CC, et al. Increased oxidative damage is correlated to altered mitochondrial function in heterozygous manganese superoxide dismutase knockout mice. J Biol Chem 1998;273:28510-28515
17. Murakami K, Kondo T, Kawase M, et al. Mitochondrial susceptibility to oxidative stress exacerbates cerebral infarction that follows permanent focal cerebral ischemia in mutant mice with manganese superoxide dismutase deficiency. J Neurosci 1998; 18:205-213
18. Ferrante RJ, Browne SE, Shinobu LA, et al. Evidence of increased oxidative damage in both sporadic and familial amyotrophic lateral sclerosis. J Neurochem 1997;69:2064-2074
19. Blanchard V, Raisman-Vozari R, Vyas S, et al. Differential expression of tyrosine hydroxylase and membrane dopamine transporter genes in subpopulations of dopaminergic neurons of rat mesencephalon. Mol Brain Res 1994;22:29-40
20. Franklin KBJ, Paxinos G. The mouse brain in stereotaxic coordinates. New York: Academic Press, 1997
21. Sterio DC. The unbiased estimation of number and sizes of arbitrary particles using the dissector. J Microsc 1984;134:127-136
22. West MJ. Stereological methods for estimating the total number of neurons and synapses: issues of precision and bias. Trends Neurosci 1999;22:51-61
23. Deng H-X, Hentati A, Tainer JA, et al. Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase. Science 1993;261:1047-1051
24. Bruijn LI, Becher MW, Lee MK, et al. ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease and SOD1-containing inclusions. Neuron 1997;18:327-338
25. Ferrante RJ, Shinobu LA, Schulz JB, et al. Increased 3-nitrotyrosine and oxidative damage in mice with a human Cu,Zn superoxide dismutase mutation. Ann Neurol 1997;42: 326-334
26. Beal MF, Ferrante RJ, Browne SE, et al. Increased 3-nitrotyrosine in both sporadic and familial amyotrophic lateral sclerosis, Ann Neurol 1997;42:646-654
27. m for hydrogen peroxide. J Biol Chem 1997;272:8861-8863
28. Ghadge GD, Lee JP, Bindokas VP, et al. Mutant superoxide dismutase-1-linked familial amyotrophic lateral sclerosis: molecular mechanisms of neuronal death and protection. J Neurosci 1997;17:8756-8766
29. Pasinelli P, Borchelt DR, Houseweart MK, et al. Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase. Proc Natl Acad Sci USA 1998;95:15763-15768
30. Bogdanov MB, Ramos LE, Xu X, Beal MF. Elevated "hydroxyl radical" generation in vivo in an animal model of amyotrophic lateral sclerosis. J Neurochem 1998;71:1321-1324
31. Andrus PK, Fleck TJ, Gurney ME, Hall ED. Protein oxidative damage in a transgenic mouse model of familial amyotrophic lateral sclerosis. J Neurochem 1998;71:2041-2048
32. Liu R, Althaus JS, Ellerbrock BR, et al. Enhanced oxygen radical production in a transgenic mouse model of familial amyotrophic lateral sclerosis. Ann Neurol 1998;44:763-770
33. DiFiglia M, Sapp E, Chase KO, et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 1997;277:1990-1993
34. Ross CA. Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases? Neuron 1997;19:1147-1150
35. Durham HD, Roy J, Dong L, Figlewicz DA. Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS. J Neuropathol Exp Neurol 1997;56:523-530
36. Bruening W, Roy J, Giasson B, et al. Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis. J Neurochem 1999;72:693-699
37. Shibata N, Hirano A, Kobayashi M, et al. Intense superoxide dismutase-1 immunoreactivity in intracytoplasmic hyaline inclusions of familial amyotrophic lateral sclerosis with posterior column involvement. J Neuropathol Exp Neurol 1996;55:481-490
38. Bruijn LI, Houseweart MK, Kato S, et al. Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. Science 1998;281:1851-1854
39. Gonzalez-Zulueta M, Ensz LM, Mukhina G, et al. Manganese superoxide dismutase protects nNOS neurons from NMDA and nitric oxide-mediated neurotoxicity. J Neurosci 1998;18: 2040-2055
40. Kostic V, Gurney ME, Deng H-X, et al. Midbrain dopaminergic neuronal degeneration in a transgenic mouse model of familial amyotrophic lateral sclerosis. Ann Neurol 1997;41:497-504
41. Klivenyi P, Ferrante RJ, Matthews RT, et al. Neuroprotective effects of creatine in a transgenic animal model of ALS. Nat Med 1999;5:347-350
42. Beal MF. Oxidative damage in neurodegenerative diseases. Neuroscientist 1997;3:21-27
43. Zhang Y, Marcillat O, Giulivi C, et al. The oxidative inactivation of mitochondrial electron transport chain components and ATPase. J Biol Chem 1996;265:16330-16336
44. Kong J, Xu Z. Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1. J Neurosci 1998;18:3241-3250
45. Siklos L, Engelhardt JI, Alexianu ME, et al. Intracellular calcium parallels motoneuron degeneration in SOD-1 mutant mice. J Neuropathol Exp Neurol 1998;57:571-587
46. Borchelt DR, Wong PC, Becher MW, et al. Axonal transport of mutant superoxide dismutase 1 and focal axonal abnormalities in the proximal axons of transgenic mice. Neurobiol Dis 1998;5:27-35
47. Benov L, Chang LY, Day B, Fridovich I. Copper, zinc superoxide dismutase in Escherichia coli periplasmic localization. J Biochem Biophys 1995;319:508-511
48. Weisiger RA, Fridovich I. Mitochondrial superoxide dismutase: site of synthesis and intramitochondrial localisation. J Biol Chem 1973;248:4793-4796
49. Ghafourifar P, Richter C. Nitric oxide synthase activity in mitochondria. FEBS Lett 1997;418:291-296
50. Giulivi C, Poderoso JJ, Boveris A. Production of nitric oxide by mitochondria. J Biol Chem 1998;273:11038-11043
51. Tatoyan A, Giulivi C. Purification and characterization of a nitric-oxide synthase from rat liver mitochondria. J Biol Chem 1998;273:11044-11048
52. Konorev EA, Hogg N, Kalyanaraman B. Rapid and irreversible inhibition of creatine kinase by peroxynitrite. FEBS Lett 1998; 427:171-174
53. MacMillan-Crow LA, Crow JP, Kerby JD, et al. Nitration and inactivation of manganese superoxide dismutase in chronic rejection of human renal allografts. Proc Natl Acad Sci USA 1996;93:11853-11858
54. Yamakura F, Taka H, Fujimura T, Murayama K. Inactivation of human manganese-superoxide dismutase by peroxynitrite is caused by exclusive nitration of tyrosine 34 to 3-nitrotyrosine. J Biol Chem 1998;273:14085-14089