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Science 17 June 1994:
Vol. 264. no. 5166, pp. 1772 - 1775
DOI: 10.1126/science.8209258
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Articles

Science, Vol 264, Issue 5166, 1772-1775
Copyright © 1994 by American Association for the Advancement of Science

articles

Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation

ME Gurney, H Pu, AY Chiu, MC Dal Canto, CY Polchow, DD Alexander, J Caliendo, A Hentati, YW Kwon, HX Deng, and al. et

Department of Cell and Molecular Biology, Northwestern University Medical School, Chicago, IL 60611.

Mutations of human Cu,Zn superoxide dismutase (SOD) are found in about 20 percent of patients with familial amyotrophic lateral sclerosis (ALS). Expression of high levels of human SOD containing a substitution of glycine to alanine at position 93--a change that has little effect on enzyme activity--caused motor neuron disease in transgenic mice. The mice became paralyzed in one or more limbs as a result of motor neuron loss from the spinal cord and died by 5 to 6 months of age. The results show that dominant, gain-of-function mutations in SOD contribute to the pathogenesis of familial ALS.


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Nitric Oxide and Peroxynitrite in Health and Disease.
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Mol. Cell. Proteomics 5, 1233-1244
   Abstract »    Full Text »    PDF »
Impairment of mitochondrial anti-oxidant defence in SOD1-related motor neuron injury and amelioration by ebselen.
C. A. Wood-Allum, S. C. Barber, J. Kirby, P. Heath, H. Holden, R. Mead, A. Higginbottom, S. Allen, T. Beaujeux, S. E. Alexson, et al. (2006)
Brain 129, 1693-1709
   Abstract »    Full Text »    PDF »
Local Unfolding in a Destabilized, Pathogenic Variant of Superoxide Dismutase 1 Observed with H/D Exchange and Mass Spectrometry.
B. F. Shaw, A. Durazo, A. M. Nersissian, J. P. Whitelegge, K. F. Faull, and J. S. Valentine (2006)
J. Biol. Chem. 281, 18167-18176
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Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia..
S. Boillee, K. Yamanaka, C. S. Lobsiger, N. G. Copeland, N. A. Jenkins, G. Kassiotis, G. Kollias, and D. W. Cleveland (2006)
Science 312, 1389-1392
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Caspase-3 Cleaves and Inactivates the Glutamate Transporter EAAT2.
W. Boston-Howes, S. L. Gibb, E. O. Williams, P. Pasinelli, R. H. Brown Jr., and D. Trotti (2006)
J. Biol. Chem. 281, 14076-14084
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Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria.
H.-X. Deng, Y. Shi, Y. Furukawa, H. Zhai, R. Fu, E. Liu, G. H. Gorrie, M. S. Khan, W.-Y. Hung, E. H. Bigio, et al. (2006)
PNAS 103, 7142-7147
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Increasing cannabinoid levels by pharmacological and genetic manipulation delay disease progression in SOD1 mice.
L. G. Bilsland, J. R. T. Dick, G. Pryce, S. Petrosino, V. Di Marzo, D. Baker, and L. Greensmith (2006)
FASEB J 20, 1003-1005
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Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models..
D. Bergemalm, P. A. Jonsson, K. S. Graffmo, P. M. Andersen, T. Brannstrom, A. Rehnmark, and S. L. Marklund (2006)
J. Neurosci. 26, 4147-4154
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Chronic activation in presymptomatic amyotrophic lateral sclerosis (ALS) mice of a feedback loop involving Fas, Daxx, and FasL.
C. Raoul, E. Buhler, C. Sadeghi, A. Jacquier, P. Aebischer, B. Pettmann, C. E. Henderson, and G. Haase (2006)
PNAS 103, 6007-6012
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Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model.
H. Kikuchi, G. Almer, S. Yamashita, C. Guegan, M. Nagai, Z. Xu, A. A. Sosunov, G. M. McKhann II, and S. Przedborski (2006)
PNAS 103, 6025-6030
   Abstract »    Full Text »    PDF »
The Control of Neuromuscular Transmission in Health and Disease.
M. M. Rich (2006)
Neuroscientist 12, 134-142
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Familial amyotrophic lateral sclerosis with frontotemporal dementia is linked to a locus on chromosome 9p13.2-21.3.
C. Vance, A. Al-Chalabi, D. Ruddy, B. N. Smith, X. Hu, J. Sreedharan, T. Siddique, H. J. Schelhaas, B. Kusters, D. Troost, et al. (2006)
Brain 129, 868-876
   Abstract »    Full Text »    PDF »
Thalidomide and Lenalidomide Extend Survival in a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis.
M. Kiaei, S. Petri, K. Kipiani, G. Gardian, D.-K. Choi, J. Chen, N. Y. Calingasan, P. Schafer, G. W. Muller, C. Stewart, et al. (2006)
J. Neurosci. 26, 2467-2473
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Common dynamical signatures of familial amyotrophic lateral sclerosis-associated structurally diverse Cu, Zn superoxide dismutase mutants.
S. D. Khare and N. V. Dokholyan (2006)
PNAS 103, 3147-3152
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S-nitrosothiol depletion in amyotrophic lateral sclerosis.
C. M. Schonhoff, M. Matsuoka, H. Tummala, M. A. Johnson, A. G. Estevéz, R. Wu, A.és Kamaid, K. C. Ricart, Y. Hashimoto, B. Gaston, et al. (2006)
PNAS 103, 2404-2409
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Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models.
P. A. Jonsson, K. S. Graffmo, P. M. Andersen, T. Brannstrom, M. Lindberg, M. Oliveberg, and S. L. Marklund (2006)
Brain 129, 451-464
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Activation of RNA metabolism-related genes in mouse but not human tissues deficient in SMN.
R. Olaso, V. Joshi, J. Fernandez, N. Roblot, S. Courageot, J. P. Bonnefont, and J. Melki (2006)
Physiol Genomics 24, 97-104
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Proteasomal Degradation of Mutant Superoxide Dismutases Linked to Amyotrophic Lateral Sclerosis.
L. Di Noto, L. J. Whitson, X. Cao, P. J. Hart, and R. L. Levine (2005)
J. Biol. Chem. 280, 39907-39913
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Clinical Trials in Amyotrophic Lateral Sclerosis: The Tenuous Past and the Promising Future.
R. B. Choudry and M. E. Cudkowicz (2005)
J. Clin. Pharmacol. 45, 1334-1344
   Abstract »    Full Text »    PDF »


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