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Altered Neural Synchronies Underlying Cognitive Deficits in a Transgenic Mouse Model of Huntington’s Disease

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The Neurobiological Basis of Memory

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Abstract

Huntington’s disease (HD) is an inherited and progressive neurodegenerative disease associated with the pathology of basal ganglia mediating memory for motor response and attributes. Hallmark chorea and motor disturbances are often preceded by cognitive and psychiatric symptoms associated with neuronal dysfunction, rather than cell death, of the vulnerable neural circuits. The exact nature of the neural functions altered in the disease, in particular, the information coding process mediating cognitive and behavioral disturbances, is unknown.

A few recent studies have attempted to elucidate this issue by performing electrophysiological recordings of single-unit and population neural activity in awake and behaving transgenic mice used to model HD. These investigations revealed dramatic and unique alterations in electrophysiological activity in basal ganglia circuitry over different brain activity states, i.e., task acquisition, exploration of environment, and sleep. These alterations took the form of a reduced recruitment of striatal projection neurons during behaviors as well as aberrant oscillatory activities in a wide range of frequencies in the cortico-striatal circuit. The aberrant rhythmic activities in θ, β, and γ frequencies were differently expressed according to brain activation levels and vigilance states. These neural functional modifications may collectively contribute to the cognitive and behavioral abnormities observed in HD transgenic mice.

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Acknowledgment

The works summarized here were supported by the Hereditary Disease Foundation, the University of Bordeaux 1, the HD Society of America, and the Agence Nationale de la Recherche (ANR-08-MNPS-019-01).

The authors wish to thank Vietminh Paz, Sebastien Delcasso, Sebastien Cayzac, Susanna Pietropaolo, Pauline Delage, Fanny Lebreton, Michele Pignatelli, Xavier Leinekugel, Fares Bassil, Nicole Mons, Huowei Du, Maurice Garret, Magali Cabanas for their fruitful collaborations.

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  1. Institut de Neurosciences Cognitives et Intégratives d’Aquitaine, University of Bordeaux, Avenue des Facultes, 33405, Talence, Cedex, France

    Yoon H. Cho PhD & Yannick Jeantet PhD

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  1. Yoon H. Cho PhD
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  2. Yannick Jeantet PhD
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Correspondence to Yoon H. Cho PhD .

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Editors and Affiliations

  1. Department of Psychology, Radford University, Radford, Virginia, USA

    Pamela A. Jackson

  2. Department of Cognitive Science, University of California-San Diego, La Jolla, California, USA

    Andrea A. Chiba

  3. Department of Neurological Surgery and Center for Neuroscience, University of California-Davis, Davis, California, USA

    Robert F. Berman

  4. Department of Psychology, University of Illinois at Chicago, Chicago, Illinois, USA

    Michael E. Ragozzino

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Cho, Y., Jeantet, Y. (2016). Altered Neural Synchronies Underlying Cognitive Deficits in a Transgenic Mouse Model of Huntington’s Disease. In: Jackson, P., Chiba, A., Berman, R., Ragozzino, M. (eds) The Neurobiological Basis of Memory. Springer, Cham. https://doi.org/10.1007/978-3-319-15759-7_14

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