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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Oral administration of 360 mg/day CoQ10 produced a 37% reduction in occipital cortex lactate concentrations in 18 patients with HD, which was reversed following withdrawal of therapy.
“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]
Cites
MACDONALD, M. (1993) · Cell · doi:10.1016/0092-8674(93)90585-e · Cited by 8,366 · see citing works
Beal, M. F. (1992) · Annals of Neurology · doi:10.1002/ana.410310202 · Cited by 956 · see citing works
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 · see citing works
Alternative excitotoxic hypotheses
Albin, R. L., & Greenamyre, J. T. (1992) · Neurology · doi:10.1212/wnl.42.4.733 · Cited by 574 · see citing works
Brouillet, E., Hantraye, P., Ferrante, R. J., Dolan, R., Leroy‐Willig, A., Kowall, N. W., & Beal, M. F. (1995) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.92.15.7105 · Cited by 485 · see citing works
Self-citationMarbach, E. P., & Weil, M. H. (1967) · PubMed · Cited by 480 · see citing works
Jenkins, B. G., Koroshetz, W. J., Beal, M. F., & Rosen, B. R. (1993) · Neurology · doi:10.1212/wnl.43.12.2689 · Cited by 479 · see citing works
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 · see citing works
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 · see citing works
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 · see citing works
An analysis of the role of coenzyme Q in free radical generation and as an antioxidant
Beyer, R. E. (1992) · Biochemistry and Cell Biology · doi:10.1139/o92-061 · Cited by 250 · see citing works
Factors Associated With Slow Progression in Huntington's Disease
Myers, R. H., Sax, D. S., Koroshetz, W. J., Mastromauro, C. A., Cupples, L. A., Kiely, D. K., Pettengill, F. K., & Bird, E. D. (1991) · Archives of Neurology · doi:10.1001/archneur.1991.00530200036015 · Cited by 208 · see citing works
Self-citationBrouillet, M. (1995) · Medical Entomology and Zoology · Cited by 190 · see citing works
Beal, M. F., Henshaw, D. R., Jenkins, B. G., Rosen, B. R., & Schulz, J. B. (1994) · Annals of Neurology · doi:10.1002/ana.410360613 · Cited by 189 · see citing works
Self-citationTreatment of Kearns‐Sayre syndrome with coenzyme Q 10
Ogasahara, S., Nishikawa, Y., Yorifuji, S., Soga, F., Nakamura, Y., Takahashi, M., Hashimoto, S., Kôno, N., & Tarui, S. (1986) · Neurology · doi:10.1212/wnl.36.1.45 · Cited by 158 · see citing works
Coenzyme Q 10 with multiple vitamins is generally ineffective in treatment of mitochondrial disease
Matthews, P. M., Ford, B., Dandurand, R. J., Eidelman, D. H., O'Connor, D., Sherwin, A. L., Karpati, G., Andermann, F., & Arnold, D. L. (1993) · Neurology · doi:10.1212/wnl.43.5.884 · Cited by 125 · see citing works
Matthews, P. M., Allaire, C., Shoubridge, E. A., Karpati, G., Carpenter, S., & Arnold, D. L. (1991) · Neurology · doi:10.1212/wnl.41.1.114 · Cited by 121 · see citing works
Schneider, H., Lemasters, J. J., & Hackenbrock, C. R. (1982) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)33893-6 · Cited by 110 · see citing works
Assessment of coenzyme q10 tolerability in huntington's disease
Feigin, A., Kieburtz, K. D., Como, P. G., Hickey, C., Abwender, D. A., Zimmerman, C., Steinberg, K., & Shoulson, I. (1996) · Movement Disorders · doi:10.1002/mds.870110317 · Cited by 108 · see citing works
Clinical and biochemical correlations in mitochondrial myopathies treated with coenzyme Q 10
Bresolin, N., Bet, L., Binda, A., Moggio, M. G., Comi, G. P., Nador, F., Ferrante, C., Carenzi, A., & Scarlato, G. (1988) · Neurology · doi:10.1212/wnl.38.6.892 · Cited by 107 · see citing works
Phosphorus magnetic resonance spectroscopy (31P MRS) in neuromuscular disorders
Argov, Z., & Bank, W. (1991) · Annals of Neurology · doi:10.1002/ana.410300116 · Cited by 105 · see citing works
Bendahan, D., Desnuelle, C., Vanuxem, D., Confort-Gouny, S., Figarella-Branger, D., Pellissier, J. F., Kozak-Ribbens, G., Pouget, J., Serratrice, G., & Cozzone, P. J. (1992) · Neurology · doi:10.1212/wnl.42.6.1203 · Cited by 104 · see citing works
Robinson, B. H., Glerum, D. M., Chow, W., Petrova-Benedict, R., Lightowlers, R. N., & Capaldi, R. (1990) · Pediatric Research · doi:10.1203/00006450-199011000-00027 · Cited by 98 · see citing works
Ubiquinone Protects Cultured Neurons against Spontaneous and Excitotoxin-Induced Degeneration
Favit, A., Nicoletti, F., Scapagnini, U., & Canonico, P. L. (1992) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1038/jcbfm.1992.88 · Cited by 98 · see citing works
Role of mobility of redox components in the inner mitochondrial membrane
Lenaz, G. (1988) · The Journal of Membrane Biology · doi:10.1007/bf01872322 · Cited by 84 · see citing works
Penn, A. M. W., Roberts, T., Hodder, J., Allen, P. S., Zhu, G., & Martin, W. R. W. (1995) · Neurology · doi:10.1212/wnl.45.11.2097 · Cited by 80 · see citing works
Ellison, D. W., Beal, M. F., & Martin, J. B. (1987) · Journal of Neuroscience Methods · doi:10.1016/0165-0270(87)90073-2 · Cited by 79 · see citing works
Self-citationAbe, K., Fujimura, H., Nishikawa, Y., Yorifuji, S., Mezaki, T., Hirono, N., Nishitani, N., & Kameyama, M. (1991) · Acta Neurologica Scandinavica · doi:10.1111/j.1600-0404.1991.tb03962.x · Cited by 76 · see citing works
Goda, S., Hamada, T., Ishimoto, S., Kobayashi, T., Goto, I., & Kuroiwa, Y. (1987) · Journal of Neurology · doi:10.1007/bf00314013 · Cited by 64 · see citing works
Taylor, D. J., Krige, D., Barnes, P. R. J., Kemp, G. J., Carroll, M. T., Mann, V. M., Cooper, J. A. D., MARSDEN, C. D., & Schapira, A. H. V. (1994) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(94)90245-3 · Cited by 64 · see citing works
Yamamoto, M., SATO, T. A., Anno, M., Ujike, H., & Takemoto, M. (1987) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.50.11.1475 · Cited by 57 · see citing works
El-Hazmi, M. A. F., Al-Swailem, A. R., Al-Faleh, F. Z., & Warsy, A. S. (1986) · Human Heredity · doi:10.1159/000153599 · Cited by 47 · see citing works
Elevated cerebrospinal fluid lactate/pyruvate ratio in Machado-Joseph disease
Matsuishi, T., Sakai, T., Naito, E., Nagamitsu, S., Kuroda, Y., Iwashita, H., & Kato, H. (2009) · Acta Neurologica Scandinavica · doi:10.1111/j.1600-0404.1996.tb00174.x · Cited by 19 · see citing works
Ueber veränderungen der zusammensetzung des liquor cerebrospinalis beim choreatischen syndrom
Brück, J., Gerstenbrand, F., Gnad, H., Gründig, E., & Prosenz, P. (1967) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(67)90134-7 · Cited by 14 · see citing works
Ethanolamine Kinase Activity in Purified Myelin of Rat Brain
Kunishita, T., Vaswani, K. K., Morrow, C. R., Novak, G., & Ledeen, R. W. (1987) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1987.tb13119.x · Cited by 12 · see citing works
Borud, O., Torbergsen, T., & Mathiesen, E. B. (1987) · Journal of Inherited Metabolic Disease · doi:10.1007/bf01799985 · Cited by 7 · see citing works
Increased alanine aminotransferase activity in the Huntington's disease putamen
Carter, C. J. (1984) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(84)90138-2 · Cited by 7 · see citing works
STA-MCA bypass surgery for chronic cerebral ischemia
Schmiedek, P., Piepgras, A., Leinsinger, G., & Gückel, F. (1996) · Acta Neurologica Scandinavica · doi:10.1111/j.1600-0404.1996.tb00553.x · Cited by 5 · see citing works
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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 · see citing works
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 · see citing works
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 · see citing works
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 · see citing works
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Johri, A., & Beal, M. F. (2012) · The Journal of Pharmacology and Experimental Therapeutics · doi:10.1124/jpet.112.192138 · Cited by 806 · see citing works
Self-citationMitochondrial dysfunction in neurodegenerative diseases
Beal, M. F. (1998) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/s0005-2728(98)00114-5 · Cited by 608 · see citing works
Self-citationMatthews, R. T., Yang, L., Browne, S., Baik, M., & Beal, M. F. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.15.8892 · Cited by 557 · see citing works
Self-citationSawa, A., Wiegand, G. W., Cooper, J. K., Margolis, R. L., Sharp, A. H., Lawler, J. F., Greenamyre, J. T., Snyder, S. H., & Ross, C. A. (1999) · Nature Medicine · doi:10.1038/13518 · Cited by 552 · see citing works
Neuroprotective Effects of Creatine in a Transgenic Mouse Model of Huntington's Disease
Ferrante, R. J., Andreassen, O. A., Jenkins, B. G., Dedeoglu, A., Kuemmerle, S., Kubilus, J. K., Kaddurah-Daouk, R., Hersch, S. M., & Beal, M. F. (2000) · The Journal of Neuroscience · doi:10.1523/jneurosci.20-12-04389.2000 · Cited by 522 · see citing works
Self-citationOxidative stress in Huntington’s disease
Browne, S. E., Ferrante, R. J., & Beal, M. F. (1999) · Brain Pathology · doi:10.1111/j.1750-3639.1999.tb00216.x · Cited by 508 · see citing works
Self-citationCha, J.-H. J., Kosinski, C. M., Kerner, J. A., Alsdorf, S. A., Mangiarini, L., Davies, S. W., Penney, J. B., Bates, G. P., & Young, A. B. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.11.6480 · Cited by 504 · see citing works
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Gil‐Mohapel, J., & Rego, A. C. (2008) · European Journal of Neuroscience · doi:10.1111/j.1460-9568.2008.06310.x · Cited by 480 · see citing works
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Matthews, R. T., Yang, L., Jenkins, B. G., Ferrante, R. J., Rosen, B. R., Kaddurah-Daouk, R. F., & Beal, M. F. (1998) · Journal of Neuroscience · doi:10.1523/jneurosci.18-01-00156.1998 · Cited by 431 · see citing works
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Replicating Huntington's disease phenotype in experimental animals
Brouillet, E. (1999) · Progress in Neurobiology · doi:10.1016/s0301-0082(99)00005-2 · Cited by 413 · see citing works
A Randomized Clinical Trial of High-Dosage Coenzyme Q10 in Early Parkinson Disease
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Self-citationNeuronal degeneration and mitochondrial dysfunction
Schon, E. A., & Manfredi, G. (2003) · Journal of Clinical Investigation · doi:10.1172/jci17741 · Cited by 393 · see citing works
Ferrante, R. J., Andreassen, O. A., Dedeoglu, A., Ferrante, K. L., Jenkins, B. G., Hersch, S. M., & Beal, M. F. (2002) · The Journal of Neuroscience · doi:10.1523/jneurosci.22-05-01592.2002 · Cited by 391 · see citing works
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Andres, R. H., Ducray, A., Schlattner, U., Wallimann, T., & Widmer, H. R. (2008) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2008.02.035 · Cited by 372 · see citing works
Brouillet, E., Jacquard, C., Bizat, N., & Blum, D. L. (2005) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2005.03515.x · Cited by 361 · see citing works
Mitochondria in Neurodegeneration: Acute Ischemia and Chronic Neurodegenerative Diseases
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Self-citationCreatine: Endogenous Metabolite, Dietary, and Therapeutic Supplement
Brosnan, J. T., & Brosnan, M. E. (2007) · Annual Review of Nutrition · doi:10.1146/annurev.nutr.27.061406.093621 · Cited by 347 · see citing works
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The Huntington Study Group (2001) · Neurology · doi:10.1212/wnl.57.3.397 · Cited by 312 · see citing works
Oxidative Damage in Huntington's Disease Pathogenesis
Browne, S. E., & Beal, M. F. (2006) · Antioxidants and Redox Signaling · doi:10.1089/ars.2006.8.2061 · Cited by 309 · see citing works
Self-citationKeene, C. D., Rodrigues, C. M. P., Eich, T., Chhabra, M. S., Steer, C. J., & Low, W. C. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.162362299 · Cited by 306 · see citing works
Mitochondrial dysfunction and oxidative stress in aging and neurodegenerative disease
Albers, D. S., & Beal, M. F. (2000) · Springer eBooks · doi:10.1007/978-3-7091-6781-6_16 · Cited by 297 · see citing works
Self-citationNeuroprotective strategies for basal ganglia degeneration: Parkinson's and Huntington's diseases
Alexi, T. (2000) · Progress in Neurobiology · doi:10.1016/s0301-0082(99)00032-5 · Cited by 290 · see citing works
Mitochondrial Approaches for Neuroprotection
Chaturvedi, R. K., & Beal, M. F. (2008) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1427.027 · Cited by 294 · see citing works
Self-citationHD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism
Seong, I. S., Ivanova, E. V., Lee, J., Choo, Y. S., Fossale, E., Anderson, M., Gusella, J. F., Laramie, J. M., Myers, R. H., Lesort, M. J., et al. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi319 · Cited by 291 · see citing works
Andreassen, O. A., Dedeoglu, A., Ferrante, R. J., Jenkins, B. G., Ferrante, K. L., Thomas, M., Friedlich, A., Browne, S. E., Schilling, G., Borchelt, D. R., et al. (2001) · Neurobiology of Disease · doi:10.1006/nbdi.2001.0406 · Cited by 293 · see citing works
Self-citationOxidative Stress in Neurodegeneration
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Mitochondria in Huntington's disease
Damiano, M. G., Galvan, L., Déglon, N., & Brouillet, E. (2009) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2009.07.012 · Cited by 282 · see citing works
Mitochondria and degenerative disorders
Orth, M., & Schapira, A. H. V. (2001) · American Journal of Medical Genetics · doi:10.1002/ajmg.1425 · Cited by 278 · see citing works
Simmons, D. A., Casale, M., Alcon, B., Pham, N., Narayan, N., & LYNCH, G. S. (2007) · Glia · doi:10.1002/glia.20526 · Cited by 277 · see citing works
Beal, M. F. (2004) · Journal of Bioenergetics and Biomembranes · doi:10.1023/b:jobb.0000041772.74810.92 · Cited by 276 · see citing works
Self-citationRegional Specificity of Brain Atrophy in Huntington's Disease
Halliday, G. M., McRitchie, D. A., Macdonald, V., Double, K. L., Trent, R. J., & McCusker, E. A. (1998) · Experimental Neurology · doi:10.1006/exnr.1998.6919 · Cited by 271 · see citing works
Transcriptional signatures in Huntington's disease
Cha, J.-H. J. (2007) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2007.03.004 · Cited by 267 · see citing works
Antoine, J.-C. G., Cinotti, L., Tilikete, C., Bouhour, F., Camdessanch�, J. P., Confavreux, C., Vighetto, A., Renault‐Mannel, V., Michel, D., & Honnorat, J. (2000) · Annals of Neurology · doi:10.1002/1531-8249(200007)48:1 · Cited by 86 · see citing works
Antioxidants as treatment for neurodegenerative disorders
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Benchoua, A., Trioulier, Y., Zala, D., Gaillard, M., Lefort, N., Dufour, N., Saudou, F., Elalouf, J., Hirsch, É. C., Hantraye, P., et al. (2006) · Molecular Biology of the Cell · doi:10.1091/mbc.e05-07-0607 · Cited by 248 · see citing works
Tarnopolsky, M. A., & Beal, M. F. (2001) · Annals of Neurology · doi:10.1002/ana.1028 · Cited by 244 · see citing works
Self-citationThe Energetics of Huntington's Disease
Browne, S. E., & Beal, M. F. (2004) · Neurochemical Research · doi:10.1023/b:nere.0000014824.04728.dd · Cited by 240 · see citing works
Self-citationBioenergetic approaches for neuroprotection in Parkinson's disease
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Self-citationDayalu, P., & Albin, R. L. (2014) · Neurologic Clinics · doi:10.1016/j.ncl.2014.09.003 · Cited by 230 · see citing works
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Experimental therapeutics in transgenic mouse models of Huntington's disease
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Li, J. Y., Popović, N., & Brundin, P. (2005) · NeuroRx · doi:10.1602/neurorx.2.3.447 · Cited by 211 · see citing works
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Guidetti, P., Charles, V., Chen, E., Reddy, P. H., Kordower, J. H., Whetsell, W. O., Schwarcz, R. M., & Tagle, D. A. (2001) · Experimental Neurology · doi:10.1006/exnr.2000.7626 · Cited by 197 · see citing works
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Beal, M. F. (2011) · Amino Acids · doi:10.1007/s00726-011-0851-0 · Cited by 191 · see citing works
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Carsten Saft, C., Zange, J., Andrich, J., Müller, K., Lindenberg, K. S., Landwehrmeyer, G. B., Vorgerd, M., Kraus, P. H., Przuntek, H., & Schöls, L. J. (2005) · Movement Disorders · doi:10.1002/mds.20373 · Cited by 184 · see citing works
The role of mitochondria in inherited neurodegenerative diseases
Kwong, J. Q., Beal, M. F., & Manfredi, G. (2006) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2006.03990.x · Cited by 180 · see citing works
Self-citationAdhihetty, P. J., & Beal, M. F. (2008) · NeuroMolecular Medicine · doi:10.1007/s12017-008-8053-y · Cited by 184 · see citing works
Self-citationMitochondria and Huntington's Disease Pathogenesis: Insight from Genetic and Chemical Models
Browne, S. E. (2008) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1427.018 · Cited by 176 · see citing works
Huntington's disease: The current state of research with peripheral tissues
Sassone, J., Colciago, C., Cislaghi, G., Silani, V., & Ciammola, A. (2009) · Experimental Neurology · doi:10.1016/j.expneurol.2009.05.012 · Cited by 175 · see citing works
Open Label Trial of Coenzyme Q10 as A Migraine Preventive
Rozen, T., Oshinsky, M. L., Gebeline, C., Bradley, K., Young, W., Shechter, A. L., & Silberstein, S. (2002) · Cephalalgia · doi:10.1046/j.1468-2982.2002.00335.x · Cited by 174 · see citing works
Complex I Defect in muscle from patients with Huntington's disease
Arenas, J., Campos, Y., Ribacoba, R., Martín, M. A., Rubio‐Terres, C., Ablanedo, P., & Cabello, A. (1998) · Annals of Neurology · doi:10.1002/ana.410430321 · Cited by 174 · see citing works
Stack, C., Ho, D. J., Wille, E. J., Calingasan, N. Y., Williams, C. R., Liby, K. T., Sporn, M. B., Dumont, M., & Beal, M. F. (2010) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2010.03.017 · Cited by 171 · see citing works
Self-citationDedeoglu, A., Kubilus, J. K., Yang, L., Ferrante, K. L., Hersch, S. M., Beal, M. F., & Ferrante, A. R. J. (2003) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2003.01706.x · Cited by 169 · see citing works
Self-citationEvidence of altered energy metabolism in autistic children
Chugani, D. C., Sundram, B. S., Behen, M. E., Lee, M.-L., & Moore, G. J. (1999) · Progress in Neuro-Psychopharmacology and Biological Psychiatry · doi:10.1016/s0278-5846(99)00022-6 · Cited by 169 · see citing works
Evidence of Oxidant Damage in Huntington's Disease: Translational Strategies Using Antioxidants
Stack, E. C., Matson, W. R., & Ferrante, R. J. (2008) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1427.008 · Cited by 167 · see citing works
Selective Neuronal Degeneration in Huntington's Disease
Cowan, C. M., & Raymond, L. A. (2006) · Current topics in developmental biology/Current Topics in Developmental Biology · doi:10.1016/s0070-2153(06)75002-5 · Cited by 163 · see citing works
Creatine supplementation in Huntington’s disease
Verbessem, P., Lemiere, J., Eijnde, B. O., Swinnen, S. P., Vanhees, L., Leemputte, M. V., Hespel, P., & Dom, R. (2003) · Neurology · doi:10.1212/01.wnl.0000090629.40891.4b · Cited by 163 · see citing works
Huntington disease: A single-gene degenerative disorder of the striatum
Nopoulos, P. C. (2016) · Dialogues in Clinical Neuroscience · doi:10.31887/dcns.2016.18.1/pnopoulos · Cited by 161 · see citing works
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Beal, M. F. (2002) · Free Radical Research · doi:10.1080/10715760290021315 · Cited by 160 · see citing works
Self-citationSwarnakar, N. K., Jain, A., Singh, R. P., Godugu, C., Das, M., & Jain, S. (2011) · Biomaterials · doi:10.1016/j.biomaterials.2011.05.079 · Cited by 155 · see citing works
Meningeal cells can communicate with astrocytes by calcium signaling
Grafstein, B., Liu, S., Cotrina, M. L., Goldman, S. A., & Nedergaard, M. (2000) · Annals of Neurology · doi:10.1002/1531-8249(200001)47:1 · Cited by 50 · see citing works
Huntington's disease: molecular basis of neurodegeneration
Rubinsztein, D. C., & Carmichael, J. (2003) · Expert Reviews in Molecular Medicine · doi:10.1017/s1462399403006549 · Cited by 154 · see citing works
Role of oxidative DNA damage in mitochondrial dysfunction and Huntington’s disease pathogenesis
Ayala‐Peña, S. (2013) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2013.04.017 · Cited by 142 · see citing works
Redox Mechanisms in Neurodegeneration: From Disease Outcomes to Therapeutic Opportunities
Sbodio, J. I., Snyder, S. H., & Paul, B. D. (2018) · Antioxidants and Redox Signaling · doi:10.1089/ars.2017.7321 · Cited by 143 · see citing works
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McGarry, A. J., McDermott, M., Kieburtz, K. D., de Blieck, E. A., Beal, F. M., Marder, K. S., Ross, C. A., Shoulson, I., Gilbert, P. R., Mallonee, W. M., et al. (2016) · Neurology · doi:10.1212/wnl.0000000000003478 · Cited by 139 · see citing works
Mitochondria and Neurodegeneration
Petrozzi, L., Ricci, G., Giglioli, N. J., Siciliano, G., & Mancuso, M. (2007) · Bioscience Reports · doi:10.1007/s10540-007-9038-z · Cited by 139 · see citing works
Mitochondria targeted therapeutic approaches in Parkinson's and Huntington's diseases
Chaturvedi, R. K., & Beal, M. F. (2012) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2012.11.011 · Cited by 139 · see citing works
Self-citationA randomized trial of coenzyme Q 10 in mitochondrial disorders
Glover, E. I., Martin, J. C., Maher, A. C., Thornhill, R. E., Moran, G. R., & Tarnopolsky, M. A. (2010) · Muscle & Nerve · doi:10.1002/mus.21758 · Cited by 137 · see citing works
Therapeutic use of coenzyme Q 10 and coenzyme Q 10 -related compounds and formulations
Villalba, J. M., Parrado‐Fernández, C., González, M. S., & Alcain, F. J. (2010) · Expert Opinion on Investigational Drugs · doi:10.1517/13543781003727495 · Cited by 134 · see citing works
Cardiac dysfunction in the R6/2 mouse model of Huntington’s disease
Mihm, M. J., Amann, D. M., Schanbacher, B. L., Altschuld, R. A., Bauer, J. A., & Hoyt, K. R. (2006) · Neurobiology of Disease · doi:10.1016/j.nbd.2006.09.016 · Cited by 136 · see citing works
Tarnopolsky (1999) · doi:10.1002/(sici)1097-4598(199909)22:9
Chaturvedi, R. K., Calingasan, N. Y., Yang, L., Hennessey, T., Johri, A., & Beal, M. F. (2010) · Human Molecular Genetics · doi:10.1093/hmg/ddq229 · Cited by 135 · see citing works
Self-citationMunsie, L., Caron, N., Atwal, R. S., Marsden, I., Wild, E. J., Bamburg, J. R., Tabrizi, S. J., & Truant, R. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddr075 · Cited by 133 · see citing works
Neuropsychiatric Correlates and Treatment of Lenticulostriatal Diseases
Lauterbach, E. C., Cummings, J. L., Duffy, J., Coffey, C. E., Kaufer, D., Lovell, M. R., Malloy, P. F., Reeve, A., Royall, D. R., Rummans, T. A., et al. (1998) · Journal of Neuropsychiatry · doi:10.1176/jnp.10.3.249 · Cited by 132 · see citing works
Keene, C. D., Rodrigues, C. M. P., Eich, T., Linehan-Stieers, C., Abt, A., Kren, B. T., Steer, C. J., & Low, W. C. (2001) · Experimental Neurology · doi:10.1006/exnr.2001.7755 · Cited by 132 · see citing works
Proton MR spectroscopy in clinical routine
Björkman‐Burtscher, I. M., & Holtås, S. (2001) · Journal of Magnetic Resonance Imaging · doi:10.1002/jmri.1079 · Cited by 132 · see citing works
Fatigue in patients with Parkinson's disease
Karlsen, K., Larsen, J. O., Tandberg, E., & J�rgensen, K. (1999) · Movement Disorders · doi:10.1002/1531-8257(199903)14:2 · Cited by 235 · see citing works
Self-citationAntonini, A., Leenders, K. L., & Eidelberg, D. (1998) · Annals of Neurology · doi:10.1002/ana.410430216 · Cited by 127 · see citing works
de Barcelos, I. P., & Haas, R. (2019) · Biology · doi:10.3390/biology8020028 · Cited by 126 · see citing works
Ubiquinone: cholesterol's reclusive cousin
Hargreaves, I. P. (2003) · Annals of Clinical Biochemistry International Journal of Laboratory Medicine · doi:10.1258/000456303321610493 · Cited by 117 · see citing works
- 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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