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Review
Fishing for causes and cures of motor neuron disorders
Shunmoogum A. Patten, Gary A. B. Armstrong, Alexandra Lissouba, Edor Kabashi, J. Alex Parker, Pierre Drapeau
Disease Models & Mechanisms 2014 7: 799-809; doi: 10.1242/dmm.015719
Shunmoogum A. Patten
Department of Neuroscience, FRQS Groupe de Recherche sur le Système Nerveux Central and CRCHUM, University of Montréal, Montréal, QC H3A 2B4, Canada.
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Gary A. B. Armstrong
Department of Neuroscience, FRQS Groupe de Recherche sur le Système Nerveux Central and CRCHUM, University of Montréal, Montréal, QC H3A 2B4, Canada.
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Alexandra Lissouba
Department of Neuroscience, FRQS Groupe de Recherche sur le Système Nerveux Central and CRCHUM, University of Montréal, Montréal, QC H3A 2B4, Canada.
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Edor Kabashi
Institut du Cerveau et de la Moelle Épinière, Centre de Recherche, CHU Pitié-Salpétrière, 75013 Paris, France.
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J. Alex Parker
Department of Neuroscience, FRQS Groupe de Recherche sur le Système Nerveux Central and CRCHUM, University of Montréal, Montréal, QC H3A 2B4, Canada.
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Pierre Drapeau
Department of Neuroscience, FRQS Groupe de Recherche sur le Système Nerveux Central and CRCHUM, University of Montréal, Montréal, QC H3A 2B4, Canada.
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  • For correspondence: p.drapeau@umontreal.ca
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Abstract

Motor neuron disorders (MNDs) are a clinically heterogeneous group of neurological diseases characterized by progressive degeneration of motor neurons, and share some common pathological pathways. Despite remarkable advances in our understanding of these diseases, no curative treatment for MNDs exists. To better understand the pathogenesis of MNDs and to help develop new treatments, the establishment of animal models that can be studied efficiently and thoroughly is paramount. The zebrafish (Danio rerio) is increasingly becoming a valuable model for studying human diseases and in screening for potential therapeutics. In this Review, we highlight recent progress in using zebrafish to study the pathology of the most common MNDs: spinal muscular atrophy (SMA), amyotrophic lateral sclerosis (ALS) and hereditary spastic paraplegia (HSP). These studies indicate the power of zebrafish as a model to study the consequences of disease-related genes, because zebrafish homologues of human genes have conserved functions with respect to the aetiology of MNDs. Zebrafish also complement other animal models for the study of pathological mechanisms of MNDs and are particularly advantageous for the screening of compounds with therapeutic potential. We present an overview of their potential usefulness in MND drug discovery, which is just beginning and holds much promise for future therapeutic development.

Footnotes

  • This article is part of a Special Issue, Spotlight on Zebrafish: Translational Impact. See all the articles in the issue at http://dmm.biologists.org/content/7/7.toc.

  • Competing interests

    The authors declare no competing financial interests.

  • Funding

    This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

  • © 2014. Published by The Company of Biologists Ltd

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attributed.

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Keywords

  • ALS
  • HSP
  • SMA
  • Zebrafish
  • Drug discovery
  • Motor neuron disorders

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Review
Fishing for causes and cures of motor neuron disorders
Shunmoogum A. Patten, Gary A. B. Armstrong, Alexandra Lissouba, Edor Kabashi, J. Alex Parker, Pierre Drapeau
Disease Models & Mechanisms 2014 7: 799-809; doi: 10.1242/dmm.015719
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Review
Fishing for causes and cures of motor neuron disorders
Shunmoogum A. Patten, Gary A. B. Armstrong, Alexandra Lissouba, Edor Kabashi, J. Alex Parker, Pierre Drapeau
Disease Models & Mechanisms 2014 7: 799-809; doi: 10.1242/dmm.015719

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Article navigation

  • Top
  • Article
    • Abstract
    • Introduction
    • Common pathological pathways between the different MNDs
    • The zebrafish toolbox: how useful is it for studying MNDs?
    • Zebrafish as a tool for drug screening
    • Spinal muscular atrophy (SMA)
    • Amyotrophic lateral sclerosis (ALS)
    • Hereditary spastic paraplegia (HSP)
    • Chemical screens in zebrafish models for other neural diseases
    • Concluding remarks and perspectives
    • Footnotes
    • References
  • Figures & tables
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