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Oxidative damage and metabolic dysfunction in Huntington's disease: Selective vulnerability of the basal ganglia
Abstract
The etiology of the selective neuronal death that occurs in Huntington's disease (HD) is unknown. Several lines of evidence implicate the involvement of energetic defects and oxidative damage in the disease process, including a recent study that demonstrated an interaction between huntingtin protein and the glycolytic enzyme glyceraldehyde‐3‐phosphate dehydrogenase (GAPDH). Using spectrophotometric assays in postmortem brain tissue, we found evidence of impaired oxidative phosphorylation enzyme activities restricted to the basal ganglia in HD brain, while enzyme activities were unaltered in three regions relatively spared by HD pathology (frontal cortex, parietal cortex, and cerebellum). Citrate synthase‐corrected complex II‐III activity was markedly reduced in both HD caudate (−29%) and putamen (−67%), and complex IV activity was reduced in HD putamen (−62%). Complex I and GAPDH activities were unaltered in all regions examined. We also measured levels of the oxidative damage product 8‐hydroxydeoxyguanosine (OH 8 dG) in nuclear DNA, and superoxide dismutase (SOD) activity. OH 8 dG levels were significantly increased in HD caudate. Cytosolic SOD activity was slightly reduced in HD parietal cortex and cerebellum, whereas particulate SOD activity was unaltered in these regions. These results further support a role for metabolic dysfunction and oxidative damage in the pathogenesis of HD.
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“Biochemical studies revealed reduced activity of several key components of oxidative phosphorylation and the TCA cycle, including complexes II-III and aconitase, in advanced stage HD patients, while complex-I activity was unaltered40-43.”
From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [42]
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Metadata
- Authors
- Browne, S. E., Bowling, A. C., Macgarvey, U., Baik, M. J., Berger, S. C., Muquit, M. M. K., Bird, E. D., & Beal, M. F.
- Affiliations
- Harvard University (US)
- McLean Hospital (US)
- Corresponding author
- Beal, M. Flint
- Venue
- Annals of Neurology
- Publisher
- Wiley
- Published
- 1997-05
- Volume
- 41
- Issue
- 5
- Pages
- 646-653
- ISSN
- 0364-5134 (ISSN-L)
- 1531-8249
- OpenAlex
- W2102430316
- Cited by
- 885
- References
- 41
- Citations by year
- 2026: 14 · 2025: 12 · 2024: 18 · 2023: 17 · 2022: 25 · 2021: 33 · 2020: 22 · 2019: 18 · 2018: 31 · 2017: 13 · 2016: 47 · 2015: 31 · 2014: 41 · 2013: 37 · 2012: 46
- FWCI
- 10.37
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Annals of Neurology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Keywords
- Huntington's disease, oxidative damage, metabolic dysfunction, basal ganglia, selective neuronal vulnerability, oxidative phosphorylation, complex IV activity, complex I, glyceraldehyde-3-phosphate dehydrogenase, huntingtin, 8-hydroxy-2'-deoxyguanosine, superoxide dismutase, caudate nucleus, putamen, postmortem brain
- MeSH terms
- Basal Ganglia, Cerebellum, Cerebral Cortex, DNA, Glyceraldehyde-3-Phosphate Dehydrogenases, Humans, Huntington Disease, Mitochondria, Oxidation-Reduction, Reference Values, Spectrophotometry, Stress, Physiological, Superoxide Dismutase, NAD(P)H Dehydrogenase (Quinone), In Vitro Techniques
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