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Central Nervous System Diseases pp 301–332Cite as

Systemic Administration of 3-Nitropropionic Acid

A New Model of Huntington’s Disease in Rat

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Part of the book series: Contemporary Neuroscience ((CNEURO))

Abstract

Huntington’s disease (HD) is a neurodegenerative disorder characterized by dyskinetic abnormal movements and cognitive decline associated with progressive atrophy of the striatum (1). Generally onset of symptoms occurs in adults and the disease evolves over 10–15 yr toward a fatal outcome. The gene responsible for HD has been identified, and molecular studies of the corresponding encoded protein named huntingtin have made considerable progress (2). However, there are no appropriate phenotypic animal models of the disease based on transgenesis, and the mechanism underlying cell death in HD remains largely unknown.

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  1. Emmanuel Brouillet
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  2. Philippe Hantraye
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  3. M. Flint Beal
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  1. Alkermes, Inc., Cambridge, MA, USA

    Dwaine F. Emerich PhD (Director of Neuroscience) (Director of Neuroscience)

  2. Department of Neuroscience, Alkermes, Inc., Cambridge, MA, USA

    Reginald L. Dean III MS

  3. University of South Florida College of Medicine, Tampa, FL, USA

    Paul R. Sanberg PhD, DSc

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Brouillet, E., Hantraye, P., Beal, M.F. (2000). Systemic Administration of 3-Nitropropionic Acid. In: Emerich, D.F., Dean, R.L., Sanberg, P.R. (eds) Central Nervous System Diseases. Contemporary Neuroscience. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-691-1_16

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