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Nonhuman Primate Models of Huntington’s Disease and Their Application in Translational Research

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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 a monogenic, autosomal dominant inherited fatal disease that affects 1 in 10,000 people worldwide. Given its unique genetic characteristics, HD would appear as one of the most straightforward neurodegenerative diseases to replicate in animal models. Indeed, mutations in the HTT gene have been used to generate a variety of animal models that display differential pathologies and have significantly increased our understanding of the pathological mechanisms of HD. However, decades of efforts have also shown the complexity of recapitulating the human condition in other species. Here we describe the three different types of models that have been generated in nonhuman primate species, stating their advantages and limitations and attempt to give a critical perspective of their translational value to test the efficacy of novel therapeutic strategies. Obtaining construct, phenotypic, and predictive validity has proven to be challenging in most animal models of human diseases. In HD in particular, it is hard to assess the predictive validity of a new therapeutic strategy when no effective “benchmark” treatment is available in the clinic. In this light, only phenotypic/face validity and construct validity are discussed.

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Acknowledgments

The authors acknowledge that the genetic model of HD in primates was jointly developed with Dr. Nicole Déglon and Dr. Marta Ruiz.

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Authors and Affiliations

  1. Commissariat à l’Energie Atomique (CEA), Molecular Imaging Research Center (MIRCen), Fontenay-aux-Roses, France

    Romina Aron Badin

  2. Centre National de la Recherche Scientifique (CNRS), Université Paris-Sud, Université Paris-Saclay, UMR 9199, Neurodegenerative Diseases Laboratory, Fontenay-aux-Roses, France

    Romina Aron Badin

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  1. Romina Aron Badin
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Correspondence to Romina Aron Badin .

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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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Aron Badin, R. (2018). Nonhuman Primate Models of Huntington’s Disease and Their Application in Translational Research. 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_14

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  • DOI: https://doi.org/10.1007/978-1-4939-7825-0_14

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