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Energy metabolism defects in Huntington's disease and effects of coenzyme Q10
Abstract
We investigated whether the Huntington's desease (HD) gene mutation may produce either primary or secondary effects on energy metalbolism. 31 P magnetic resonance spectroscopy demonstrated a significant decrease in the phosphocreatine to inorganic phosphate ratio in resting muscle of 8 patients as compared with 8 control subjects. The cerebrospinal fluid lactate‐pyruvate ratio was significantly increased in 15 patients as compared with 13 control subjects. Lactate concentrations assessed using 1 H magnetic resonance spectroscopy are increased in Huntington's disease cerebral cortex. Treatment with coenzyme Q 10, an essential cofactor of the electron transport chain, resulted in significant decreases in cortical lactate concentrations in 18 patients, which reversed following withdrawal of therapy. These findings provide evidence for a generalized energy defect in Huntington's disease, and suggest a possible therapy.
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“We observed that CoQ10 treatment produced a 37% reduction in occipital cortex lactate concentrations, which was reversed following withdrawal of therapy39.”
From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [39]
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Metadata
- Authors
- Koroshetz, W. J., Jenkins, B. G., Rosen, B. R., & Beal, M. F.
- Affiliations
- Harvard University (US)
- Corresponding author
- Beal, M. Flint
- Venue
- Annals of Neurology
- Publisher
- Wiley
- Published
- 1997-02
- Volume
- 41
- Issue
- 2
- Pages
- 160-165
- ISSN
- 0364-5134 (ISSN-L)
- 1531-8249
- OpenAlex
- W2111856493
- Cited by
- 405
- References
- 38
- Citations by year
- 2025: 4 · 2024: 3 · 2023: 7 · 2022: 8 · 2021: 12 · 2020: 11 · 2019: 3 · 2018: 13 · 2017: 5 · 2016: 12 · 2015: 9 · 2014: 13 · 2013: 9 · 2012: 15
- FWCI
- 8.61
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Annals of Neurology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (5r01ns032365-03)
- National Institute of Neurological Disorders and Stroke (NS32365)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Coenzyme Q10 studies and effects
- Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
- Keywords
- Huntington's disease, energy metabolism, coenzyme Q10, phosphocreatine to inorganic phosphate ratio, muscle energy metabolism, cerebral lactate, electron transport chain, 31P NMR, 1H magnetic resonance spectroscopy, treatment withdrawal
- MeSH terms
- Aged, Coenzymes, Energy Metabolism, Humans, Huntington Disease, Middle Aged, Prognosis, Ubiquinone
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Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
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