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Review Article

Design, power, and interpretation of studies in the standard murine model of ALS

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Pages 4-15 | Received 27 Sep 2007, Accepted 11 Dec 2007, Published online: 10 Jul 2009

References

  • Rosen D. R., Siddique T., Patterson D., Figlewicz D. A., Sapp P., Hentati A., et al. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 1993; 362: 59–62
  • Tu P. H., Raju P., Robinson K. A., Gurney M. E., Trojanowski J. Q., Lee V. M. 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 1996; 93: 3155–60
  • Gurney M. E. The use of transgenic mouse models of amyotrophic lateral sclerosis in preclinical drug studies. J Neurol Sci 1997; 152((Suppl 1))S67–73
  • Nirmalananthan N., Greensmith L. Amyotrophic lateral sclerosis: recent advances and future therapies. Curr Opin Neurol 2005; 18: 712–9
  • Rothstein J. D. Preclinical studies: how much can we rely on?. Amyotroph Lateral Scler Other Motor Neuron Disord 2004; 5((Suppl 1))22–5
  • Miller RG, Mitchell JD, Lyon M, Moore DH. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). John Wiley & Sons, Ltd. Available: http://www.cochrane.org/reviews/en/ab001447.html
  • Gurney M. E., Pu H., Chiu A. Y., Dal Canto M. C., Polchow C. Y., Alexander D. D., et al. Motor neuron degeneration in mice that express a human Cu/Zn superoxide dismutase mutation. Science 1994; 264: 1772–5
  • Alexander G. M., Erwin K. L., Byers N., Deitch J. S., Augelli B. J., Blankenhorn E. P., Heiman‐Patterson T. D. Effect of transgene copy number on survival in the G93A SOD1 transgenic mouse model of ALS. Brain Res Mol Brain Res 2004; 130: 7–15
  • Dal Canto M. C., Gurney M. E. 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 (Berl) 1997; 93: 537–50
  • Chiu A. Y., Zhai P., Dal Canto M. C., Peters T. M., Kwon Y. W., Prattis S. M., Gurney M. E. Age‐dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis. Mol Cell Neurosci 1995; 6: 349–62
  • Trieu V. N., Uckun F. M. Genistein is neuroprotective in murine models of familial amyotrophic lateral sclerosis and stroke. Biochem Biophys Res Commun 1999; 258: 685–8
  • Cudkowicz M. E., Pastusza K. A., Sapp P. C., Mathews R. K., Leahy J., Pasinelli P., et al. Survival in transgenic ALS mice does not vary with CNS glutathione peroxidase activity. Neurology 2002; 59: 729–34
  • Heiman‐Patterson T. D., Deitch J. S., Blankenhorn E. P., Erwin K. L., Perreault M. J., Alexander B. K., et al. Background and gender effects on survival in the TgN(SOD1‐G93A)1Gur mouse model of ALS. J Neurol Sci 2005; 236: 1–7
  • Miana‐Mena F. J., Munoz M. J., Yague G., Mendez M., Moreno M., Ciriza J., et al. Optimal methods to characterize the G93A mouse model of ALS. Amyotroph Lateral Scler Other Motor Neuron Disord 2005; 6: 55–62
  • Snow R. J., Turnbull J., da S. S., Jiang F., Tarnopolsky M. A. Creatine supplementation and riluzole treatment provide similar beneficial effects in copper, zinc superoxide dismutase (G93A) transgenic mice. Neuroscience 2003; 119: 661–7
  • Ende N., Weinstein F., Chen R., Ende M. Human umbilical cord blood effect on SOD mice (amyotrophic lateral sclerosis). Life Sci 2000; 67: 53–9
  • Chen R., Ende N. The potential for the use of mononuclear cells from human umbilical cord blood in the treatment of amyotrophic lateral sclerosis in SOD1 mice. J Med 2000; 31: 21–30
  • Bruce K. M., Narayan K., Kong H. C., Larmour I., Lopes E. C., Turner B. J., et al. Chemotherapy delays progression of motor neuron disease in the SOD1 G93A transgenic mouse. Chemotherapy 2004; 50: 138–42
  • Gurney M. E., Cutting F. B., Zhai P., Doble A., Taylor C. P., Andrus P. K., Hall E. D. Benefit of vitamin E, riluzole, and gabapentin in a transgenic model of familial amyotrophic lateral sclerosis. Ann Neurol 1996; 39: 147–57
  • Gurney M. E., Fleck T. J., Himes C. S., Hall E. D. Riluzole preserves motor function in a transgenic model of familial amyotrophic lateral sclerosis. Neurology 1998; 50: 62–6
  • Drachman D. B., Frank K., Dykes‐Hoberg M., Teismann P., Almer G., Przedborski S., Rothstein J. D. Cyclooxygenase 2 inhibition protects motor neurons and prolongs survival in a transgenic mouse model of ALS. Ann Neurol 2002; 52: 771–8
  • Acsadi G., Anguelov R. A., Yang H., Toth G., Thomas R., Jani A., et al. Increased survival and function of SOD1 mice after glial cell‐derived neurotrophic factor gene therapy. Hum Gene Ther 2002; 13: 1047–59
  • Crow J. P., Calingasan N. Y., Chen J., Hill J. L., Beal M. F. Manganese porphyrin given at symptom onset markedly extends survival of ALS mice. Ann Neurol 2005; 58: 258–65
  • Ioannidis J. P. Why most published research findings are false. PloS 2005; 2: 124
  • Sterne J. A., Davey S. G. Sifting the evidence – what's wrong with significance tests?. Br Med J 2001; 322: 226–31
  • Andries M., van D. P., Robberecht W., van den B. L. Ivermectin inhibits AMPA receptor‐mediated excitotoxicity in cultured motor neurons and extends the lifespan of a transgenic mouse model of amyotrophic lateral sclerosis. Neurobiol Dis. 2006
  • Trieu V. N., Liu R., Liu X. P., Uckun F. M. A specific inhibitor of janus kinase‐3 increases survival in a transgenic mouse model of amyotrophic lateral sclerosis. Biochem Biophys Res Commun 2000; 267: 22–5
  • Klivenyi P., Kiaei M., Gardian G., Calingasan N. Y., Beal M. F. Additive neuroprotective effects of creatine and cyclooxygenase 2 inhibitors in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurochem 2004; 88: 576–82
  • Klivenyi P., Ferrante R. J., Matthews R. T., Bogdanov M. B., Klein A. M., Andreassen O. A., et al. Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis. Nat Med 1999; 5: 347–50
  • Andreassen O. A., Jenkins B. G., Dedeoglu A., Ferrante K. L., Bogdanov M. B., Kaddurah‐Daouk R., Beal M. F. Increases in cortical glutamate concentrations in transgenic amyotrophic lateral sclerosis mice are attenuated by creatine supplementation. J Neurochem 2001; 77: 383–90
  • Zhang W., Narayanan M., Friedlander R. M. Additive neuroprotective effects of minocycline with creatine in a mouse model of ALS. Ann Neurol 2003; 53: 267–70
  • Derave W., van den B. L., Lemmens G., Eijnde B. O., Robberecht W., Hespel P. Skeletal muscle properties in a transgenic mouse model for amyotrophic lateral sclerosis: effects of creatine treatment. Neurobiol Dis 2003; 13: 264–72
  • Van den Bosch L., Tilkin P., Lemmens G., Robberecht W. Minocycline delays disease onset and mortality in a transgenic model of ALS. Neuroreport 2002; 13: 1067–70
  • Zhu S., Stavrovskaya I. G., Drozda M., Kim B. Y., Ona V., Li M., et al. Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice. Nature 2002; 417: 74–8
  • Kriz J., Nguyen M. D., Julien J. P. Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis. Neurobiol Dis 2002; 10: 268–78
  • Rothstein J. D., Patel S., Regan M. R., Haenggeli C., Huang Y. H., Bergles D. E., et al. Beta‐lactam antibiotics offer neuroprotection by increasing glutamate transporter expression. Nature 2005; 433: 73–7
  • Ryu H., Smith K., Camelo S. I., Carreras I., Lee J., Iglesias A. H., et al. Sodium phenylbutyrate prolongs survival and regulates expression of anti‐apoptotic genes in transgenic amyotrophic lateral sclerosis mice. J Neurochem 2005; 93: 1087–98
  • Kiaei M., Petri S., Kipiani K., Gardian G., Choi D. K., Chen J., et al. Thalidomide and lenalidomide extend survival in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurosci 2006; 26: 2467–73
  • Ramesh T. M., Buradagunta S., Thompson K., et al. Analysis of critical parameters for preclinical drug screening in the SOD1 G93A mouse model for amyotrophic lateral sclerosis., Abstract at the 13th International Symposium on ALS/MND; November, 2002; Melbourne

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