Cited by 405Genetic Neurodegenerative DiseasesCoenzyme Q10 studies and effectsNeurological diseases and metabolism

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Energy metabolism defects in Huntington's disease and effects of coenzyme Q10

Koroshetz, Walter J., Jenkins, Bruce G., Rosen, Bruce R., Beal, M. Flint·1997·Annals of Neurology

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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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Self-citation

Authors: Koroshetz, W. J., Jenkins, B. G., Rosen, B. R., & Beal, M. F.
Affiliations: Harvard University (US)
Corresponding author: Beal, M. Flint
ORCID: Koroshetz, Walter J.
Rosen, Bruce R.
Venue: Annals of Neurology
Publisher: Wiley
Published: 1997-02
Volume: 41
Issue: 2
Pages: 160-165
ISSN: 0364-5134 (ISSN-L)
1531-8249
DOI: 10.1002/ana.410410206
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
Licence: onlinelibrary.wiley.com/termsAndConditions#vor (vor, 2806 d)
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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Energy metabolism defects in Huntington's disease and effects of coenzyme Q10

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