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The 3-Nitropropionic Acid Model of Huntington’s Disease

Do Alterations in the Expression of Metabolic mRNAs Predict the Development of Striatal Pathology?

  • Chapter
Mitochondrial Inhibitors and Neurodegenerative Disorders

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

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Abstract

Metabolic compromise has been found to precede both neuronal loss and the appearance of motoric and cognitive deficits in Huntington’s disease (HD) patients. Studies using both magnetic resonance imaging (MRI) and positron emission tomography (PET) to assess the metabolic status of the central nervous system (CNS) have demonstrated anatomically restricted deficits in energy metabolism in the caudate putamen and frontal cortex of both confirmed HD patients and also those genetically at risk of developing the disease (1–6). These findings have been confirmed biochemically in regional assessments of oxidative damage and metabolic dysfunctions in the HD brain (7). Taken together, these findings strongly suggest that a preclinical determinant of HD is the specific inhibition of energy metabolism in those brain regions most affected during the course of the pathology. Precisely how metabolic inhibition leads to neurodegeneration in the HD brain is currently not fully understood. However, analysis of the consequences of metabolic dysfunction in the CNS has opened avenues for researchers interested in establishing the pathological basis for not only Huntington’ s disease but also Alzheimer’s and Parkinson’s diseases and schizophrenia (8,9).

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Authors
  1. Keith J. Page
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  2. Alicia Meldrum
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  3. Stephen B. Dunnett
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Editor information

Editors and Affiliations

  1. Department of Neurosurgery, and Program in Neuroscience, University of South Florida College of Medicine, Tampa, FL, USA

    Paul R. Sanberg PhD, DSc

  2. Department of Physiology, Nagoya City University Medical School, Mizuho-cho, Mizuho-ku, Nagoya, Japan

    Hitoo Nishino MD, PhD

  3. Cellular Neurophysiology, Intramural Research Program, National Institute on Drug Abuse, National Institutes of Health, Baltimore, MD, USA

    Cesario V. Borlongan PhD

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© 2000 Humana Press Inc., Totowa, NJ

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Page, K.J., Meldrum, A., Dunnett, S.B. (2000). The 3-Nitropropionic Acid Model of Huntington’s Disease. In: Sanberg, P.R., Nishino, H., Borlongan, C.V. (eds) Mitochondrial Inhibitors and Neurodegenerative Disorders. Contemporary Neuroscience. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-692-8_10

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  • DOI: https://doi.org/10.1007/978-1-59259-692-8_10

  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-4684-9821-9

  • Online ISBN: 978-1-59259-692-8

  • eBook Packages: Springer Book Archive

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