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Animal models of amyotrophic lateral sclerosis and Huntington’s disease

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Abstract

Amyotrophic lateral sclerosis (ALS) and Huntington’s disease (HD) are debilitating neurodegenerative conditions for which there is no effective cure. Genetic determinants of both diseases have been identified, providing insight into neuropathological mechanisms and opportunities for therapeutic intervention. Aggregation of mutant proteins is the most prominent phenotype of these neurodegenerative diseases as is the case in Alzheimer’s disease and Parkinson’s disease. Here we review transgenic animal models of ALS and HD in mouse, zebrafish, C. elegans, and Drosophila that have been developed to study different aspects of disease progression. We also cover some large mammal transgenic models that have been recently developed. To effectively tackle these conditions will likely require effective use of several of these animal models, as each offers distinct advantages and insights into disease pathology.

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Acknowledgments

This work was supported by the Basic Science Research Program by the National Research Foundation of Korea (NRF) (2011-0021845), the “Leaders Industry-University Cooperation” Project by the Ministry of Education, and 2011 Research Grant from Kangwon National University to YK.

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Authors and Affiliations

  1. Department of Systems Immunology, College of Biomedical Science, Kangwon National University, Chuncheon, 200-701, Gangwondo, Republic of Korea

    Abu M. T. Islam, Jina Kwak, Yoo Jung Jung & Yun Kee

  2. Institute of Bioscience and Biotechnology, Kangwon National University, Chuncheon, 200-701, Republic of Korea

    Yun Kee

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  1. Abu M. T. Islam
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  2. Jina Kwak
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  3. Yoo Jung Jung
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  4. Yun Kee
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Correspondence to Yun Kee.

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Islam, A.M.T., Kwak, J., Jung, Y.J. et al. Animal models of amyotrophic lateral sclerosis and Huntington’s disease. Genes Genom 36, 399–413 (2014). https://doi.org/10.1007/s13258-014-0188-7

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  • DOI: https://doi.org/10.1007/s13258-014-0188-7

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