 |
||||
|
||||
|
||||
|
||||
Previous Article | Next Article
The Journal of Neuroscience, May 15, 2001, 21(10):3369-3374
Neuron-Specific Expression of Mutant Superoxide Dismutase 1 in Transgenic Mice Does Not Lead to Motor Impairment
Albéna Pramatarova, Janet Laganière, Julie Roussel, Katéri Brisebois, and Guy A. Rouleau Centre for Research in Neuroscience, McGill University, and the Montreal General Hospital Research Institute, Montreal, Quebec, H3G 1A4, Canada
Mutations were identified in the Cu/Zn superoxide dismutase gene (SOD1) in ~15% of patients with familial amyotrophic lateral sclerosis. Transgenic animals expressing mutant SOD1 in all tissues develop an ALS-like phenotype. To determine whether neuron-specific expression of mutant SOD1 is sufficient to produce such a phenotype, we generated transgenic animals carrying the G37R mutation that is associated with the familial form of ALS (FALS), which is driven by the neurofilament light chain promoter. The transgenic animals express high levels of the human SOD1 protein in neuronal tissues, especially in the large motor neurons of the spinal cord, but they show no apparent motor deficit at up to 1.5 years of age. Our animal model suggests that neuron-specific expression of ALS-associated mutant human SOD1 may not be sufficient for the development of the disease in mice.
Key words: amyotrophic lateral sclerosis; superoxide dismutase; mutant SOD1; transgenic mice; neuronal expression; cell death; neurofilament promoter
Copyright © 2001 Society for Neuroscience 0270-6474/01/21103369-06$05.00/0
This article has been cited by other articles:
G. Gowing, T. Philips, B. Van Wijmeersch, J.-N. Audet, M. Dewil, L. Van Den Bosch, A. D. Billiau, W. Robberecht, and J.-P. Julien
Ablation of Proliferating Microglia Does Not Affect Motor Neuron Degeneration in Amyotrophic Lateral Sclerosis Caused by Mutant Superoxide Dismutase
J. Neurosci., October 8, 2008; 28(41): 10234 - 10244.
[Abstract] [Full Text] [PDF]
M. R. Watson, R. D. Lagow, K. Xu, B. Zhang, and N. M. Bonini
A Drosophila Model for Amyotrophic Lateral Sclerosis Reveals Motor Neuron Damage by Human SOD1
J. Biol. Chem., September 5, 2008; 283(36): 24972 - 24981.
[Abstract] [Full Text] [PDF]
H. Nishitoh, H. Kadowaki, A. Nagai, T. Maruyama, T. Yokota, H. Fukutomi, T. Noguchi, A. Matsuzawa, K. Takeda, and H. Ichijo
ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1
Genes & Dev., June 1, 2008; 22(11): 1451 - 1464.
[Abstract] [Full Text] [PDF]
K. Yamanaka, S. Boillee, E. A. Roberts, M. L. Garcia, M. McAlonis-Downes, O. R. Mikse, D. W. Cleveland, and L. S. B. Goldstein
Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice
PNAS, May 27, 2008; 105(21): 7594 - 7599.
[Abstract] [Full Text] [PDF]
Y. Chang, M. P. Stockinger, H. Tashiro, and C.-l. G. Lin
A novel noncoding RNA rescues mutant SOD1-mediated cell death
FASEB J, March 1, 2008; 22(3): 691 - 702.
[Abstract] [Full Text] [PDF]
D. Jaarsma, E. Teuling, E. D. Haasdijk, C. I. De Zeeuw, and C. C. Hoogenraad
Neuron-Specific Expression of Mutant Superoxide Dismutase Is Sufficient to Induce Amyotrophic Lateral Sclerosis in Transgenic Mice
J. Neurosci., February 27, 2008; 28(9): 2075 - 2088.
[Abstract] [Full Text] [PDF]
J.-L. Gonzalez de Aguilar, C. Niederhauser-Wiederkehr, B. Halter, M. De Tapia, F. Di Scala, P. Demougin, L. Dupuis, M. Primig, V. Meininger, and J.-P. Loeffler
Gene profiling of skeletal muscle in an amyotrophic lateral sclerosis mouse model
Physiol Genomics, January 17, 2008; 32(2): 207 - 218.
[Abstract] [Full Text] [PDF]
J. Kang and S. Rivest
MyD88-deficient bone marrow cells accelerate onset and reduce survival in a mouse model of amyotrophic lateral sclerosis
J. Cell Biol., December 17, 2007; 179(6): 1219 - 1230.
[Abstract] [Full Text] [PDF]
L. Ferraiuolo, P. R. Heath, H. Holden, P. Kasher, J. Kirby, and P. J. Shaw
Microarray Analysis of the Cellular Pathways Involved in the Adaptation to and Progression of Motor Neuron Injury in the SOD1 G93A Mouse Model of Familial ALS
J. Neurosci., August 22, 2007; 27(34): 9201 - 9219.
[Abstract] [Full Text] [PDF]
M. Urushitani, S. A. Ezzi, and J.-P. Julien
Therapeutic effects of immunization with mutant superoxide dismutase in mice models of amyotrophic lateral sclerosis
PNAS, February 13, 2007; 104(7): 2495 - 2500.
[Abstract] [Full Text] [PDF]
T. Ahtoniemi, G. Goldsteins, V. Keksa-Goldsteine, T. Malm, K. Kanninen, A. Salminen, and J. Koistinaho
Pyrrolidine Dithiocarbamate Inhibits Induction of Immunoproteasome and Decreases Survival in a Rat Model of Amyotrophic Lateral Sclerosis
Mol. Pharmacol., January 1, 2007; 71(1): 30 - 37.
[Abstract] [Full Text] [PDF]
D. R. Beers, J. S. Henkel, Q. Xiao, W. Zhao, J. Wang, A. A. Yen, L. Siklos, S. R. McKercher, and S. H. Appel
Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosis
PNAS, October 24, 2006; 103(43): 16021 - 16026.
[Abstract] [Full Text] [PDF]
T. Kabuta, Y. Suzuki, and K. Wada
Degradation of Amyotrophic Lateral Sclerosis-linked Mutant Cu,Zn-Superoxide Dismutase Proteins by Macroautophagy and the Proteasome
J. Biol. Chem., October 13, 2006; 281(41): 30524 - 30533.
[Abstract] [Full Text] [PDF]
S. Kalra, C. C. Hanstock, W. R. W. Martin, P. S. Allen, and W. S. Johnston
Detection of Cerebral Degeneration in Amyotrophic Lateral Sclerosis Using High-Field Magnetic Resonance Spectroscopy.
Arch Neurol, August 1, 2006; 63(8): 1144 - 1148.
[Abstract] [Full Text] [PDF]
S. Boillee, K. Yamanaka, C. S. Lobsiger, N. G. Copeland, N. A. Jenkins, G. Kassiotis, G. Kollias, and D. W. Cleveland
Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia.
Science, June 2, 2006; 312(5778): 1389 - 1392.
[Abstract] [Full Text] [PDF]
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.
S-nitrosothiol depletion in amyotrophic lateral sclerosis
PNAS, February 14, 2006; 103(7): 2404 - 2409.
[Abstract] [Full Text] [PDF]
P. A. Jonsson, K. S. Graffmo, P. M. Andersen, T. Brannstrom, M. Lindberg, M. Oliveberg, and S. L. Marklund
Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models
Brain, February 1, 2006; 129(2): 451 - 464.
[Abstract] [Full Text] [PDF]
F. E. Perrin, G. Boisset, M. Docquier, O. Schaad, P. Descombes, and A. C. Kato
No widespread induction of cell death genes occurs in pure motoneurons in an amyotrophic lateral sclerosis mouse model
Hum. Mol. Genet., November 1, 2005; 14(21): 3309 - 3320.
[Abstract] [Full Text] [PDF]
J. Wang, G. Xu, H. Li, V. Gonzales, D. Fromholt, C. Karch, N. G. Copeland, N. A. Jenkins, and D. R. Borchelt
Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: {alpha}B-crystallin modulates aggregation
Hum. Mol. Genet., August 15, 2005; 14(16): 2335 - 2347.
[Abstract] [Full Text] [PDF]
P J Shaw
Molecular and cellular pathways of neurodegeneration in motor neurone disease
J. Neurol. Neurosurg. Psychiatry, August 1, 2005; 76(8): 1046 - 1057.
[Abstract] [Full Text] [PDF]
J. Kirby, E. Halligan, M. J. Baptista, S. Allen, P. R. Heath, H. Holden, S. C. Barber, C. A. Loynes, C. A. Wood-Allum, J. Lunec, et al.
Mutant SOD1 alters the motor neuronal transcriptome: implications for familial ALS
Brain, July 1, 2005; 128(7): 1686 - 1706.
[Abstract] [Full Text] [PDF]
C. W. Strey, D. Spellman, A. Stieber, J. O. Gonatas, X. Wang, J. D. Lambris, and N. K. Gonatas
Dysregulation of Stathmin, a Microtubule-Destabilizing Protein, and Up-Regulation of Hsp25, Hsp27, and the Antioxidant Peroxiredoxin 6 in a Mouse Model of Familial Amyotrophic Lateral Sclerosis
Am. J. Pathol., November 1, 2004; 165(5): 1701 - 1718.
[Abstract] [Full Text] [PDF]
C. Vanoni, S. Massari, M. Losa, P. Carrega, C. Perego, L. Conforti, and G. Pietrini
Increased internalisation and degradation of GLT-1 glial glutamate transporter in a cell model for familial amyotrophic lateral sclerosis (ALS)
J. Cell Sci., October 15, 2004; 117(22): 5417 - 5426.
[Abstract] [Full Text] [PDF]
L. Dupuis, H. Oudart, F. Rene, J.-L. G. de Aguilar, and J.-P. Loeffler
Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: Benefit of a high-energy diet in a transgenic mouse model
PNAS, July 27, 2004; 101(30): 11159 - 11164.
[Abstract] [Full Text] [PDF]
S. P. Koushika, A. M. Schaefer, R. Vincent, J. H. Willis, B. Bowerman, and M. L. Nonet
Mutations in Caenorhabditis elegans Cytoplasmic Dynein Components Reveal Specificity of Neuronal Retrograde Cargo
J. Neurosci., April 21, 2004; 24(16): 3907 - 3916.
[Abstract] [Full Text] [PDF]
M. D. Nguyen, T. D'Aigle, G. Gowing, J.-P. Julien, and S. Rivest
Exacerbation of Motor Neuron Disease by Chronic Stimulation of Innate Immunity in a Mouse Model of Amyotrophic Lateral Sclerosis
J. Neurosci., February 11, 2004; 24(6): 1340 - 1349.
[Abstract] [Full Text] [PDF]
P. A. Jonsson, K. Ernhill, P. M. Andersen, D. Bergemalm, T. Brannstrom, O. Gredal, P. Nilsson, and S. L. Marklund
Minute quantities of misfolded mutant superoxide dismutase-1 cause amyotrophic lateral sclerosis
Brain, January 1, 2004; 127(1): 73 - 88.
[Abstract] [Full Text] [PDF]
A. M. Clement, M. D. Nguyen, E. A. Roberts, M. L. Garcia, S. Boillee, M. Rule, A. P. McMahon, W. Doucette, D. Siwek, R. J. Ferrante, et al.
Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice
Science, October 3, 2003; 302(5642): 113 - 117.
[Abstract] [Full Text] [PDF]
S. D. Rao, H. Z. Yin, and J. H. Weiss
Disruption of Glial Glutamate Transport by Reactive Oxygen Species Produced in Motor Neurons
J. Neurosci., April 1, 2003; 23(7): 2627 - 2633.
[Abstract] [Full Text] [PDF]
R. J. Mockett, S. N. Radyuk, J. J. Benes, W. C. Orr, and R. S. Sohal
Phenotypic effects of familial amyotrophic lateral sclerosis mutant Sod alleles in transgenic Drosophila
PNAS, January 7, 2003; 100(1): 301 - 306.
[Abstract] [Full Text] [PDF]
D. Z. Ellis, J. Rabe, and K. J. Sweadner
Global Loss of Na,K-ATPase and Its Nitric Oxide-Mediated Regulation in a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis
J. Neurosci., January 1, 2003; 23(1): 43 - 51.
[Abstract] [Full Text] [PDF]
K. Puttaparthi, W. L. Gitomer, U. Krishnan, M. Son, B. Rajendran, and J. L. Elliott
Disease Progression in a Transgenic Model of Familial Amyotrophic Lateral Sclerosis Is Dependent on Both Neuronal and Non-Neuronal Zinc Binding Proteins
J. Neurosci., October 15, 2002; 22(20): 8790 - 8796.
[Abstract] [Full Text] [PDF]
W. Sun, H. Funakoshi, and T. Nakamura
Overexpression of HGF Retards Disease Progression and Prolongs Life Span in a Transgenic Mouse Model of ALS
J. Neurosci., August 1, 2002; 22(15): 6537 - 6548.
[Abstract] [Full Text] [PDF]
D. S. Howland, J. Liu, Y. She, B. Goad, N. J. Maragakis, B. Kim, J. Erickson, J. Kulik, L. DeVito, G. Psaltis, et al.
Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)
PNAS, January 24, 2002; (2002) 32539299.
[Abstract] [Full Text] [PDF]
D. S. Howland, J. Liu, Y. She, B. Goad, N. J. Maragakis, B. Kim, J. Erickson, J. Kulik, L. DeVito, G. Psaltis, et al.
Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)
PNAS, February 5, 2002; 99(3): 1604 - 1609.
[Abstract] [Full Text] [PDF]
|
|
|
|
 |
|