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Murine Models of Huntington’s Disease for Evaluating Therapeutics

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Huntington’s Disease

Part of the book series: Methods in Molecular Biology ((MIMB,volume 1780))

Abstract

Huntington’s disease (HD) is an autosomal dominant progressive neurological disorder characterized by motor, cognitive, and psychiatric symptoms that typically present later on in life, although juvenile cases do exist. The identification of the disease-causing mutation, a CAG triplet repeat expansion in the HTT gene, in 1993 generated numerous investigations into the cellular and molecular pathways underlying the disorder. HD mouse models have played a prominent role in these studies, and the use of these mouse models of HD in the development and evaluation of novel therapeutic strategies is reviewed in this chapter. As new interventions and therapeutic approaches are evaluated and implemented, genetic mouse models will continue to be used with the hope of developing effective treatments for HD.

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Authors and Affiliations

  1. Centre for Molecular Medicine and Therapeutics, and Department of Medical Genetics, BC Children’s Hospital Research Institute, University of British Columbia, Vancouver, BC, Canada

    Natalia Kosior & Blair R. Leavitt

Authors
  1. Natalia Kosior
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  2. Blair R. Leavitt
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Correspondence to Blair R. Leavitt .

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Editors and Affiliations

  1. Brain Repair Group, Cardiff University, Cardiff, United Kingdom

    Sophie V. Precious

  2. Brain Repair Group, Cardiff University, Cardiff, United Kingdom

    Anne E. Rosser

  3. Brain Repair Group, Cardiff University, Cardiff, United Kingdom

    Stephen B. Dunnett

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Kosior, N., Leavitt, B.R. (2018). Murine Models of Huntington’s Disease for Evaluating Therapeutics. In: Precious, S., Rosser, A., Dunnett, S. (eds) Huntington’s Disease. Methods in Molecular Biology, vol 1780. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-7825-0_10

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  • DOI: https://doi.org/10.1007/978-1-4939-7825-0_10

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  • Publisher Name: Humana Press, New York, NY

  • Print ISBN: 978-1-4939-7824-3

  • Online ISBN: 978-1-4939-7825-0

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