Characterization of a novel SOD-1(G93A) transgenic mouse line with very decelerated disease development

PLoS ONE

Volumn 5, Issue 11, 2010, Pages

  Henriques, Alexandre ^a,b,c   Pitzer, Claudia ^a   Schneider, Armin ^a  

^a SYGNIS Bioscience   (Germany)

^b INSERM   (France)

^c UNIVERSITÉ DE STRASBOURG   (France)

Author keywords

[No Author keywords available]

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE; SOD1 G93A PROTEIN; SUPEROXIDE DISMUTASE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BREEDING LINE; CONTROLLED STUDY; DISEASE COURSE; GENE; GENE DOSAGE; INHERITANCE; MOUSE; NONHUMAN; ONSET AGE; SOD 1 GENE; SURVIVAL TIME; TRANSGENIC MOUSE; ANIMAL; C57BL MOUSE; DISEASE MODEL; FEMALE; GENETICS; HUMAN; MALE; MUSCLE WEAKNESS; PATHOLOGY; POINT MUTATION; SURVIVAL; TIME; WEIGHT REDUCTION;

ANIMALIA; MUS; MUS MUSCULUS;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; DISEASE MODELS, ANIMAL; DISEASE PROGRESSION; FEMALE; HUMANS; MALE; MICE; MICE, INBRED C57BL; MICE, TRANSGENIC; MUSCLE WEAKNESS; POINT MUTATION; SUPEROXIDE DISMUTASE; SURVIVAL ANALYSIS; TIME FACTORS; WEIGHT LOSS;

EID: 78649739214     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0015445     Document Type: Article

References (13)

1. Mitchell JD, Borasio GD (2007) Amyotrophic lateral sclerosis. Lancet 369: 2031-2041.
2. Fridovich I (1986) Superoxide dismutases. Adv Enzymol Relat Areas Mol Biol 58: 61-97.
3. Gurney ME, Pu H, Chiu AY, Dal Canto MC, Polchow CY, et al. (1994) Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. Science 264: 1772-1775.
4. Pitzer C, Kruger C, Plaas C, Kirsch F, Dittgen T, et al. (2008) Granulocyte-colony stimulating factor improves outcome in a mouse model of amyotrophic lateral sclerosis. Brain 131: 3335-3347.
5. Alexander GM, Erwin KL, Byers N, Deitch JS, Augelli BJ, et al. (2004) Effect of transgene copy number on survival in the G93A SOD1 transgenic mouse model of ALS. Brain Res Mol Brain Res 130: 7-15.
6. Tu PH, Raju P, Robinson KA, Gurney ME, Trojanowski JQ, et al. (1996) Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions. Proc Natl Acad Sci U S A 93: 3155-3160.
7. Dupuis L, Oudart H, Rene F, Gonzalez de Aguilar JL, Loeffler JP (2004) Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model. Proc Natl Acad Sci U S A 101: 11159-11164.
8. Dal Canto MC, Gurney ME (1997) 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. Acta Neuropathol 93: 537-550.
9. Stieber A, Chen Y, Wei S, Mourelatos Z, Gonatas J, et al. (1998) The fragmented neuronal Golgi apparatus in amyotrophic lateral sclerosis includes the trans-Golgi-network: functional implications. Acta Neuropathol 95: 245-253.
10. Pambo-Pambo A, Durand J, Gueritaud JP (2009) Early excitability changes in lumbar motoneurons of transgenic SOD1G85R and SOD1G(93A-Low) mice. J Neurophysiol 102: 3627-3642.
11. Feeney SJ, McKelvie PA, Austin L, Jean-Francois MJ, Kapsa R, et al. (2001) Presymptomatic motor neuron loss and reactive astrocytosis in the SOD1 mouse model of amyotrophic lateral sclerosis. Muscle Nerve 24: 1510-1519.
12. Henriques A, Pitzer C, Schneider A (2010) Neurotrophic growth factors for the treatment of amyotrophic lateral sclerosis: where do we stand? Frontiers in Neuropharmacology 4: 32.
13. Scott S, Kranz JE, Cole J, Lincecum JM, Thompson K, et al. (2008) Design, power, and interpretation of studies in the standard murine model of ALS. Amyotroph Lateral Scler 9: 4-15.