The DOI matches its Crossref record; 38 of the 38 references it cites were found in a registry or an index.
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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Cites
MACDONALD, M. (1993) · Cell · doi:10.1016/0092-8674(93)90585-e · Cited by 8,366
Beal, M. F. (1992) · Annals of Neurology · doi:10.1002/ana.410310202 · Cited by 962
Self-citationMitochondrial defect in Huntington's disease caudate nucleus
Gu, M., Gash, M. T., Mann, V. M., Javoy‐Agid, F., Cooper, J. M., & Schapira, A. H. V. (1996) · Annals of Neurology · doi:10.1002/ana.410390317 · Cited by 758
Alternative excitotoxic hypotheses
Albin, R. L., & Greenamyre, J. T. (1992) · Neurology · doi:10.1212/wnl.42.4.733 · Cited by 574
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Self-citationMarbach, E. P., & Weil, M. H. (1967) · PubMed · Cited by 480
Jenkins, B. G., Koroshetz, W. J., Beal, M. F., & Rosen, B. R. (1993) · Neurology · doi:10.1212/wnl.43.12.2689 · Cited by 479
Self-citationHuntingtin and DRPLA proteins selectively interact with the enzyme GAPDH
Burke, J. R., Enghild, J. J., Martin, M. E., Jou, Y., Myers, R., Roses, A. D., Vance, J. M., & Strittmatter, W. J. (1996) · Nature Medicine · doi:10.1038/nm0396-347 · Cited by 430
Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues
Strong, T. V., Tagle, D. A., Valdes, J. M., Elmer, L. W., Boehm, K., Swaroop, M., Kaatz, K. W., Collins, F. S., & Albin, R. L. (1993) · Nature Genetics · doi:10.1038/ng1193-259 · Cited by 417
Rapid Enzymatic Measurement of Blood Lactate and Pyruvate
Marbach, E. P., & Weil, M. H. (1967) · Clinical Chemistry · doi:10.1093/clinchem/13.4.314 · Cited by 396
Research works printed but not linked: 1
- O'Brien CF, 1990, Extraneural metabolism in early Huntington's disease, Ann Neurol, 28, 300
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Metabolism and function of coenzyme Q
Turunen, M., Olsson, J., & Dallner, G. (2004) · Biochimica et Biophysica Acta (BBA) - Biomembranes · doi:10.1016/j.bbamem.2003.11.012 · Cited by 1,026
Cui, L., Jeong, H., Borovecki, F., Parkhurst, C. N., Tanese, N., & Krainc, D. (2006) · Cell · doi:10.1016/j.cell.2006.09.015 · Cited by 1,012
Effects of Coenzyme Q10 in Early Parkinson Disease: Evidence of Slowing of the Functional Decline
Shults, C. W. (2002) · Archives of Neurology · doi:10.1001/archneur.59.10.1541 · Cited by 984
Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease
Zuccato, C., Valenza, M., & Cattaneo, E. (2010) · Physiological Reviews · doi:10.1152/physrev.00041.2009 · Cited by 848
Mitochondrial Dysfunction in Neurodegenerative Diseases
Johri, A., & Beal, M. F. (2012) · The Journal of Pharmacology and Experimental Therapeutics · doi:10.1124/jpet.112.192138 · Cited by 806
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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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