Increased vulnerability to 3-nitropropionic acid in an animal model of Huntington's disease.
There is substantial evidence for both metabolic dysfunction and oxidative damage in Huntington's disease ( HD). In the present study, we used in vivo microdialysis to measure the conversion of 4-hydroxybenzoic acid to 3,4-dihydroxybenzoic acid (3,4-DHBA) as a measure of hydroxyl radical production in a transgenic mouse model of HD, as well as in littermate controls. The conversion of 4-hydroxybenzoic acid to 3,4-DHBA was unchanged in the striatum of transgenic HD mice at baseline. Following administration of the mitochondrial toxin 3-nitropropionic acid (3-NP), there were significant increases in 3,4-DHBA generation in both control and transgenic HD mice, and the increases in the transgenic HD mice were significantly greater than those in controls. Furthermore, administration of 3-NP produced significantly larger striatal lesions in transgenic HD mice than in littermate controls. The present results show increased sensitivity to the mitochondrial toxin 3-NP in transgenic HD mice, which suggests metabolic dysfunction in this mouse model of HD.[1]References
- Increased vulnerability to 3-nitropropionic acid in an animal model of Huntington's disease. Bogdanov, M.B., Ferrante, R.J., Kuemmerle, S., Klivenyi, P., Beal, M.F. J. Neurochem. (1998) [Pubmed]
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