Motor Neuron Disease and Model Systems: Aetiologies, Mechanisms and Therapies

  1. Gregory R. Bock Organizer and
  2. Jamie A. Goode
  1. Donald L. Price1,2,3,7,
  2. Vassilis E. Koliatsos1,2,3,7,
  3. Philip C.-Y. Wong2,7,
  4. Carlos A. Pardo2,7,
  5. David R. Borchelt2,7,
  6. Michael K. Lee2,4,7,
  7. Don W. Cleveland1,4,†,
  8. John W. Griffin1,3,
  9. Paul N. Hoffman3,5,
  10. Linda C. Cork3,6,‡ and
  11. Sangram S. Sisodia2,7

Published Online: 28 SEP 2007

DOI: 10.1002/9780470514863.ch2

Ciba Foundation Symposium 196 - Growth Factors as Drugs for Neurological and Sensory Disorders

Ciba Foundation Symposium 196 - Growth Factors as Drugs for Neurological and Sensory Disorders

How to Cite

Price, D. L., Koliatsos, V. E., Wong, P. C.-Y., Pardo, C. A., Borchelt, D. R., Lee, M. K., Cleveland, D. W., Griffin, J. W., Hoffman, P. N., Cork, L. C. and Sisodia, S. S. (2007) Motor Neuron Disease and Model Systems: Aetiologies, Mechanisms and Therapies, in Ciba Foundation Symposium 196 - Growth Factors as Drugs for Neurological and Sensory Disorders (eds G. R. Bock and J. A. Goode), John Wiley & Sons, Ltd., Chichester, UK. doi: 10.1002/9780470514863.ch2

Author Information

  1. 1

    Department of Neuroscience, Johns Hopkins University School of Medicine, 720 Rutland Avenue, Baltimore, MD 21205–2196, USA

  2. 2

    Department of Pathology, Johns Hopkins University School of Medicine, 720 Rutland Avenue, Baltimore, MD 21205–2196, USA

  3. 3

    Department of Neurology, Johns Hopkins University School of Medicine, 720 Rutland Avenue, Baltimore, MD 21205–2196, USA

  4. 4

    Department of Biological Chemistry, Johns Hopkins University School of Medicine, 720 Rutland Avenue, Baltimore, MD 21205–2196, USA

  5. 5

    Department of Ophthalmology, Johns Hopkins University School of Medicine, 720 Rutland Avenue, Baltimore, MD 21205–2196, USA

  6. 6

    Department of Comparative Medicine, Johns Hopkins University School of Medicine, 720 Rutland Avenue, Baltimore, MD 21205–2196, USA

  7. 7

    Department of Neuropathology, Johns Hopkins University School of Medicine, 720 Rutland Avenue, Baltimore, MD 21205–2196, USA

  1. Ludwig Institute, University of California at San Diego, La Jolla, CA, USA.

  2. Departments of Comparative Medicine and Pathology, Stanford Medical Center, Stanford, CA

Publication History

  1. Published Online: 28 SEP 2007

ISBN Information

Print ISBN: 9780471957218

Online ISBN: 9780470514863

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Keywords:

  • motor neuron disease;
  • phenotypes;
  • alzheimer's disease;
  • human superoxide dismutase 1;
  • Parkinson's disease

Summary

The phenotypes of many neurological diseases, including motor neuron disease (amyotrophic lateral sclerosis; ALS) and Alzheimer's disease (AD), are determined by the vulnerabilities of populations of nerve cells and the character/evolution of cellular abnormalities. Because different cell types respond selectively to individual trophic factors, these factors may be useful in ameliorating pathology in cells that express their cognate receptors. To test therapies for ALS and AD, investigators require model systems. Although there are a variety of models of ALS, two models are particularly attractive: transgenic mice that express human superoxide dismutase 1 (SOD-1) mutations linked to familial ALS develop paralysis associated with a gain of adverse property of the mutant SOD; and axotomy of facial axons in neonatal rats, a manipulation that causes retrograde cell degeneration, which can be ameliorated by several trophic factors.