Comparative Study on 3-Nitropropionic Acid Neurotoxicity

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Book Chapter

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Quinolinic Acid, Choreic Movement, General Locomotor Activity, Basal Ganglion Neuron, Nonprimate Animal

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The complex etiologies and mechanisms of cell death associated with, as well as protective/reparative techniques for several neurodegenerative disorders, have been examined using animal models. We have witnessed during the last 5 yr advances in gene knockout animal models and how these animal systems can pave the way for better understanding of human diseases. For example, the discovery of the gene underlying Huntington’s disease (HD) opens the possibility that genetic therapy may be the next logical step toward finding a cure for this disease. HD is a progressive debilitating disorder associated with severe degeneration of basal ganglia neurons, especially the intrinsic neurons of the striatum, and characterized by involuntary abnormal choreiform movements and progressive dementia. In this chapter, cross-species models of HD are discussed, which recently received critical attention from researchers interested in the involvement of impaired energy metabolism in the evolution of the disease. The preceding chapters dealt largely with the utility of rodent model of 3-nitropropionic acid (3-NPA), which has been demonstrated as closely resembling the neurobiological and clinical symptoms of the disease.

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Citation / Publisher Attribution

Comparative Study on 3-Nitropropionic Acid Neurotoxicity, in P. R. Sanberg, H. Nishino, & C. V. Borlongan (Eds.), Mitochondrial Inhibitors and Neurodegenerative Disorders, Springer, p. 93-106