A randomized, placebo-controlled trial of coenzyme Q 10 and remacemide in Huntington’s disease
Abstract
OBJECTIVES: To determine whether chronic treatment with coenzyme Q10 or remacemide hydrochloride slows the functional decline of early Huntington's disease (HD). METHODS: The authors conducted a multicenter, parallel group, double-blind, 2 x 2 factorial, randomized clinical trial. Research participants with early HD (n = 347) were randomized to receive coenzyme Q10 300 mg twice daily, remacemide hydrochloride 200 mg three times daily, both, or neither treatment, and were evaluated every 4 to 5 months for a total of 30 months on assigned treatment. The prespecified primary measure of efficacy was the change in total functional capacity (TFC) between baseline and 30 months. Safety measures included the frequency of clinical adverse events. RESULTS: Neither intervention significantly altered the decline in TFC. Patients treated with coenzyme Q10 showed a trend toward slowing in TFC decline (13%) over 30 months (2.40- versus 2.74-point decline, p = 0.15), as well as beneficial trends in some secondary measures. There was increased frequency of nausea, vomiting, and dizziness with remacemide and increased frequency of stomach upset with coenzyme Q10. CONCLUSIONS: Neither remacemide nor coenzyme Q10, at the dosages studied, produced significant slowing in functional decline in early HD.
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In the CARE-HD trial, administration of CoQ10 resulted in a 14% decrease in disease progression in HD patients.
“Administration of CoQ10 resulted in a 14% decrease in disease progression.”
From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [155]
In the CARE-HD trial, remacemide demonstrated no efficacy.
“Remacemide demonstrated no efficacy.”
From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [155]
The CARE-HD trial was powered to determine a 40% attenuation of total functional capacity decline with 80% power.
“This trial was powered to be able to determine a 40% attenuation of total functional capacity decline with 80% power.”
From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [155]
Cites
Unified Huntington's disease rating scale: Reliability and consistency
Kremer, H. P. H. (1996) · Movement Disorders · doi:10.1002/mds.870110204 · Cited by 2,109 · see citing works
The hopkins verbal learning test: Development of a new memory test with six equivalent forms
Brandt, J. (1991) · Clinical Neuropsychologist · doi:10.1080/13854049108403297 · Cited by 1,317 · see citing works
Browne, S. E., Bowling, A. C., Macgarvey, U., Baik, M. J., Berger, S. C., Muquit, M. M. K., Bird, E. D., & Beal, M. F. (1997) · Annals of Neurology · doi:10.1002/ana.410410514 · Cited by 885 · see citing works
Huntington disease: Clinical care and evaluation
Shoulson, I., & Fahn, S. (1979) · Neurology · doi:10.1212/wnl.29.1.1 · Cited by 746 · see citing works
Energy metabolism defects in Huntington's disease and effects of coenzyme Q<sub>10</sub>
Koroshetz, W. J., Jenkins, B. G., Rosen, B. R., & Beal, M. F. (1997) · Annals of Neurology · doi:10.1002/ana.410410206 · Cited by 405 · see citing works
Rate of functional decline in Huntington’s disease
Marder, K., Zhao, H., Myers, R. H., Cudkowicz, M., Kayson, E., Kieburtz, K., Orme, C., Paulsen, J., Penney, J. B., Siemers, E., et al. (2000) · Neurology · doi:10.1212/wnl.54.2.452 · Cited by 362 · see citing works
Self-citationFatigue 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
Huntington's disease in venezuela: 7 years of follow‐up on symptomatic and asymptomatic individuals
Penney, J. B., Young, A. B., Shoulson, I., Starosta‐Rubinstein, S., Snodgrass, S. R., Sanchez‐Ramos, J., Ramos‐Arroyo, M. A., Gomez, F., Penchaszadeh, G. K., Alvir, J., et al. (1990) · Movement Disorders · doi:10.1002/mds.870050202 · Cited by 218 · 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
Influence of lamotrigine on progression of early Huntington disease
Kremer, B. P. H., Clark, C. M., Almqvist, E. W., Raymond, L. A., Graf, P., Jacova, C., Mezei, M., Hardy, M., Snow, B. J., Martin, W. R. W., et al. (1999) · Neurology · doi:10.1212/wnl.53.5.1000 · Cited by 147 · see citing works
Trial of d-alpha-tocopherol in Huntington's disease
Peyser, C. E., Folstein, M. F., Chase, G. A., STARKSTEIN, S. E., Brandt, J., Cockrell, J. R., Bylsma, F., Coyle, J. T., McHugh, P. R., & Folstein, S. E. (1995) · American Journal of Psychiatry · doi:10.1176/ajp.152.12.1771 · Cited by 139 · see citing works
Petrides, M. (1990) · Neuropsychologia · doi:10.1016/0028-3932(90)90096-7 · Cited by 119 · 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
A controlled clinical trial of baclofen as protective therapy in early huntington's disease
Shoulson, I., Odoroff, C. L., Oakes, D., Behr, J., Goldblatt, D., Caine, E. D., Kennedy, J., Miller, C., Bamford, K. A., Rubin, A., et al. (1989) · Annals of Neurology · doi:10.1002/ana.410250308 · Cited by 106 · see citing works
A controlled trial of remacemide hydrochloride in Huntington's disease
Kieburtz, K. D., Feigin, A., McDermott, M., Como, P. G., Abwender, D. A., Zimmerman, C. F., Hickey, C., Orme, C., Claude, K., Sotack, J., et al. (1996) · Movement Disorders · doi:10.1002/mds.870110310 · Cited by 104 · see citing works
A controlled trial of idebenone in Huntington's disease
Ranen, N. G., Peyser, C. E., Coyle, J. T., Bylsma, F. W., Sherr, M., Day, L., Folstein, M. F., Brandt, J., Ross, C. A., & Folstein, S. E. (1996) · Movement Disorders · doi:10.1002/mds.870110510 · Cited by 97 · see citing works
Dure, L. S., Young, A. B., & Penney, J. B. (1991) · Annals of Neurology · doi:10.1002/ana.410300607 · Cited by 88 · 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
Validation of the brief test of attention in patients with huntington's disease and amnesia
Schretlen, D. J., Brandt, J., & Bobholz, J. H. (1996) · The Clinical Neuropsychologist · doi:10.1080/13854049608406667 · Cited by 34 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
Walker, F. O. (2007) · The Lancet · doi:10.1016/s0140-6736(07)60111-1 · Cited by 2,623 · see citing works
Huntington's disease: from molecular pathogenesis to clinical treatment
Ross, C. A., & Tabrizi, S. J. (2010) · The Lancet Neurology · doi:10.1016/s1474-4422(10)70245-3 · Cited by 1,751 · 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
Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target
Barber, S. C., & Shaw, P. J. (2009) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2009.11.018 · Cited by 617 · see citing works
Ferroptosis and its potential role in the physiopathology of Parkinson’s Disease
Mahoney‐Sanchez, L., Bouchaoui, H., Ayton, S., Devos, D., Duce, J. A., & Devedjian, J.-C. (2020) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2020.101890 · Cited by 558 · see citing works
Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities
Tabrizi, S. J., Flower, M. D., Ross, C. A., & Wild, E. J. (2020) · Nature Reviews Neurology · doi:10.1038/s41582-020-0389-4 · Cited by 544 · see citing works
Huntington’s Disease: Mechanisms of Pathogenesis and Therapeutic StrategiesPreprint
Jiménez-Sánchez, M., Licitra, F., Underwood, B. R., & Rubinsztein, D. C. (2016) · Cold Spring Harbor Perspectives in Medicine · doi:10.1101/cshperspect.a024240 · Cited by 512 · see citing works
Schapira, A. H. V. (2012) · The Lancet · doi:10.1016/s0140-6736(11)61305-6 · Cited by 499 · see citing works
Mitochondrial Diseases of the Brain
Chaturvedi, R. K., & Beal, M. F. (2013) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2013.03.018 · Cited by 416 · see citing works
Huntington's disease: from pathology and genetics to potential therapies
Imarisio, S., Carmichael, J., Korolchuk, V., Chen, C.-W., Saiki, S., Rose, C., Krishna, G., Davies, J. E., Ttofi, E., Underwood, B. R., et al. (2008) · Biochemical Journal · doi:10.1042/bj20071619 · Cited by 409 · 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
Bachoud‐Lévi, A., Gaura-Schmidt, V., Brugières, P., Lefaucheur, J., Boissé, M., Maison, P., Baudic, S., Ribeiro, M. J., Bourdet, C., Rémy, P., et al. (2006) · The Lancet Neurology · doi:10.1016/s1474-4422(06)70381-7 · Cited by 357 · 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
Delivery of peptide and protein drugs over the blood–brain barrier
Brasnjevic, I., Steinbusch, H. W. M., Schmitz, C., & Martinez-Martinez, P. (2009) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2008.12.002 · Cited by 279 · 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
Antioxidants as treatment for neurodegenerative disorders
Moosmann, B., & Behl, C. (2002) · Expert Opinion on Investigational Drugs · doi:10.1517/13543784.11.10.1407 · Cited by 253 · see citing works
Stack, E. C., Kubilus, J. K., Smith, K., Cormier, K., Signore, S. J. D., Guélin, E., Ryu, H., Hersch, S. M., & Ferrante, R. J. (2005) · The Journal of Comparative Neurology · doi:10.1002/cne.20680 · Cited by 246 · see citing works
Plasma coenzyme Q10 response to oral ingestion of coenzyme Q10 formulations
Bhagavan, H. N., & Chopra, R. K. (2007) · Mitochondrion · doi:10.1016/j.mito.2007.03.003 · Cited by 243 · see citing works
The 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
Antioxidants in Central Nervous System Diseases: Preclinical Promise and Translational Challenges
Kamat, C. D., Gadal, S., Mhatre, M., Williamson, K. S., Pye, Q. N., & Hensley, K. (2008) · Journal of Alzheimer s Disease · doi:10.3233/jad-2008-15314 · Cited by 237 · see citing works
Oxidative Stress and Huntington’s Disease: The Good, the Bad, and the Ugly
Kumar, A., & Ratan, R. R. (2016) · Journal of Huntington s Disease · doi:10.3233/jhd-160205 · Cited by 236 · see citing works
Mitochondrial Dysfunction and Chronic Disease: Treatment With Natural Supplements.
Nicolson, G. L. (2014) · PubMed Central · Cited by 235 · see citing works
Yang, L., Calingasan, N. Y., Wille, E. J., Cormier, K., Smith, K., Ferrante, R. J., & Beal, M. F. (2009) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2009.06074.x · Cited by 232 · see citing works
Huntington's Disease: Can Mice Lead the Way to Treatment?
Crook, Z. R., & Housman, D. E. (2011) · Neuron · doi:10.1016/j.neuron.2010.12.035 · Cited by 229 · see citing works
Metformin therapy in a transgenic mouse model of Huntington's disease
Ma, T. C., Buescher, J. L., Oatis, B., Funk, J. A., Nash, A. J., Carrier, R. L., & Hoyt, K. R. (2006) · Neuroscience Letters · doi:10.1016/j.neulet.2006.10.039 · Cited by 220 · see citing works
Experimental therapeutics in transgenic mouse models of Huntington's disease
Beal, M. F., & Ferrante, R. J. (2004) · Nature reviews. Neuroscience · doi:10.1038/nrn1386 · Cited by 224 · see citing works
Energy deficit in Huntington disease: why it matters
Mochel, F., & Haller, R. G. (2011) · Journal of Clinical Investigation · doi:10.1172/jci45691 · Cited by 220 · see citing works
Paulson, H. L. (2009) · Journal of Neuro-Ophthalmology · doi:10.1097/wno0b013e3181b416de · Cited by 220 · see citing works
Li, J. Y., Popović, N., & Brundin, P. (2005) · NeuroRx · doi:10.1602/neurorx.2.3.447 · Cited by 211 · see citing works
The molecular genetics of Huntington disease — a history
Bates, G. P. (2005) · Nature Reviews Genetics · doi:10.1038/nrg1686 · Cited by 195 · see citing works
Dopamine and Glutamate in Huntington's Disease: A Balancing Act
André, V. M., Cepeda, C., & Levine, M. S. (2010) · CNS Neuroscience & Therapeutics · doi:10.1111/j.1755-5949.2010.00134.x · Cited by 189 · see citing works
Safety assessment of coenzyme Q 10 (CoQ 10 )
Hidaka, T., Fujii, K., Funahashi, I., Fukutomi, N., & Hosoe, K. (2008) · BioFactors · doi:10.1002/biof.5520320124 · Cited by 187 · see citing works
Mitochondrial impairment in patients and asymptomatic mutation carriers of Huntington's disease
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
Kipps, C. (2005) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2004.047993 · Cited by 182 · see citing works
The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies
Bauer, P. O., & Nukina, N. (2009) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2009.06302.x · Cited by 178 · see citing works
Riluzole in Huntington's disease: a 3‐year, randomized controlled study
Landwehrmeyer, G. B., Dubois, B., de Yébenes, J. G., Kremer, B. P. H., Gaus, W., Kraus, P. H., Przuntek, H., Dib, M., Doble, A., Fischer, W., et al. (2007) · Annals of Neurology · doi:10.1002/ana.21181 · Cited by 176 · see citing works
Frank, S. (2009) · BMC Neurology · doi:10.1186/1471-2377-9-62 · Cited by 174 · see citing works
Minocycline and doxycycline are not beneficial in a model of Huntington's disease
Smith, D. L., Woodman, B., Mahal, A., Sathasivam, K., Ghazi‐Noori, S., Lowden, P. A. S., Bates, G. P., & Hockly, E. (2003) · Annals of Neurology · doi:10.1002/ana.10614 · Cited by 173 · 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
de Yébenes, J. G., Landwehrmeyer, G. B., Squitieri, F., Reilmann, R., Rosser, A., Barker, R. A., Carsten Saft, C., Magnet, M. K., Sword, A., Rembratt, Å., et al. (2011) · The Lancet Neurology · doi:10.1016/s1474-4422(11)70233-2 · Cited by 171 · see citing works
Zeron, M. M., Fernandes, H. B., Krebs, C., Shehadeh, J., Wellington, C. L., Leavitt, B. R., Baimbridge, K. G., Hayden, M. R., & Raymond, L. A. (2004) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2003.11.014 · 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
Therapeutic approaches to Huntington disease: from the bench to the clinic
Caron, N. S., Dorsey, E. R., & Hayden, M. R. (2018) · Nature Reviews Drug Discovery · doi:10.1038/nrd.2018.133 · Cited by 161 · see citing works
The first frontier: digital biomarkers for neurodegenerative disorders
Dorsey, E. R., Papapetropoulos, S., Xiong, M., & Kieburtz, K. (2017) · Digital Biomarkers · doi:10.1159/000477383 · Cited by 162 · see citing works
From Pathogenesis to Therapeutics: A Review of 150 Years of Huntington’s Disease Research
Jiang, A., Handley, R. R., Lehnert, K., & Snell, R. G. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms241613021 · Cited by 168 · see citing works
Therapeutic effects of coenzyme Q 10 (CoQ 10 ) and reduced CoQ 10 in the MPTP model of Parkinsonism
Cléren, C., Yang, L., Lorenzo, B. J., Calingasan, N. Y., Schomer, A. C., Sireci, A. N., Wille, E. J., & Beal, M. F. (2007) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2007.05097.x · Cited by 158 · see citing works
Motor, cognitive, and functional declines contribute to a single progressive factor in early HD
Schobel, S., Palermo, G., Auinger, P., Long, J. D., Ma, S., Khwaja, O., Trundell, D., Cudkowicz, M., Hersch, S. M., Sampaio, C., et al. (2017) · Neurology · doi:10.1212/wnl.0000000000004743 · Cited by 157 · see citing works
The current clinical management of Huntington's disease
Phillips, W., Shannon, K. M., & Barker, R. A. (2008) · Movement Disorders · doi:10.1002/mds.21971 · Cited by 156 · see citing works
Alterations in synaptic function and plasticity in Huntington disease
Smith-Dijak, A., Sepers, M. D., & Raymond, L. A. (2019) · Journal of Neurochemistry · doi:10.1111/jnc.14723 · Cited by 153 · see citing works
Therapeutics development for triplet repeat expansion diseases
Prospero, N. A. D., & Fischbeck, K. H. (2005) · Nature Reviews Genetics · doi:10.1038/nrg1690 · Cited by 149 · see citing works
Schilling, G., Coonfield, M. L., Ross, C. A., & Borchelt, D. R. (2001) · Neuroscience Letters · doi:10.1016/s0304-3940(01)02326-6 · Cited by 151 · see citing works
Rodent genetic models of Huntington disease
Heng, M. Y., Detloff, P. J., & Albin, R. L. (2008) · Neurobiology of Disease · doi:10.1016/j.nbd.2008.06.005 · Cited by 149 · see citing works
The Clinical and Genetic Features of Huntington Disease
Sturrock, A., & Leavitt, B. R. (2010) · Journal of Geriatric Psychiatry and Neurology · doi:10.1177/0891988710383573 · Cited by 142 · see citing works
A randomized, double-blind, placebo-controlled trial of coenzyme Q10 in Huntington disease
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 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
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
Dose ranging and efficacy study of high-dose coenzyme Q10 formulations in Huntington's disease mice
Smith, K. M., Matson, S., Matson, W. R., Cormier, K., Del Signore, S. J., Hagerty, S. W., Stack, E. C., Ryu, H., & Ferrante, R. J. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2006.03.004 · Cited by 127 · see citing works
The uptake and distribution of coenzyme Q(10)
Miles, M. V. (2007) · Mitochondrion · doi:10.1016/j.mito.2007.02.012 · Cited by 126 · see citing works
Shehadeh, J., Fernandes, H. B., Mullins, M. Z., Graham, R. K., Leavitt, B. R., Hayden, M. R., & Raymond, L. A. (2005) · Neurobiology of Disease · doi:10.1016/j.nbd.2005.08.001 · Cited by 120 · see citing works
Symptomatic Treatment of Huntington Disease
Adam, O. R., & Jankovic, J. J. (2008) · Neurotherapeutics · doi:10.1016/j.nurt.2008.01.008 · Cited by 119 · see citing works
Huntingtin in health and disease
Young, A. B. (2003) · Journal of Clinical Investigation · doi:10.1172/jci17742 · Cited by 118 · see citing works
Combination therapy using minocycline and coenzyme Q10 in R6/2 transgenic Huntington's disease mice
Stack, E. C., Smith, K. M., Ryu, H., Cormier, K., Chen, M., Hagerty, S. W., Del Signore, S. J., Cudkowicz, M. E., Friedlander, R. M., & Ferrante, R. J. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2005.11.002 · Cited by 114 · see citing works
Risk assessment for coenzyme Q10 (Ubiquinone)
Hathcock, J. N., & Shao, A. (2006) · Regulatory Toxicology and Pharmacology · doi:10.1016/j.yrtph.2006.05.006 · Cited by 114 · see citing works
Nelson, L. M., Tanner, C. M., Eeden, S. K. V. D., & McGuire, V. M. (2004) · Neuroepidemiology · doi:10.1093/acprof:oso/9780195133790.003.03 · Cited by 111 · see citing works
Bhidayasiri, R., & Truong, D. D. (2004) · Postgraduate Medical Journal · doi:10.1136/pgmj.2004.019356 · Cited by 110 · see citing works
Mo, C., Hannan, A. J., & Renoir, T. (2015) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2015.03.003 · Cited by 103 · see citing works
Combined riluzole and sodium phenylbutyrate therapy in transgenic amyotrophic lateral sclerosis mice
Signore, S. J. D., Amante, D. J., Kim, J.-H., Stack, E. C., Goodrich, S., Cormier, K., Smith, K., Cudkowicz, M., & Ferrante, R. J. (2008) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960802226148 · Cited by 105 · see citing works
Huntington Disease and the Huntingtin Protein
Zheng, Z., & Diamond, M. I. (2012) · Progress in molecular biology and translational science · doi:10.1016/b978-0-12-385883-2.00010-2 · Cited by 104 · see citing works
Ikematsu, H., Nakamura, K., Harashima, S., Fujii, K., & Fukutomi, N. (2006) · Regulatory Toxicology and Pharmacology · doi:10.1016/j.yrtph.2005.12.002 · Cited by 101 · see citing works
Mouse models of Huntington disease: variations on a theme
Ehrnhoefer, D. E., Butland, S. L., Pouladi, M. A., & Hayden, M. R. (2009) · Disease Models & Mechanisms · doi:10.1242/dmm.002451 · Cited by 100 · see citing works
Therapeutic interventions for disease progression in Huntington's disease
Mestre, T., Ferreira, J. J., Coelho, M. M., Rosa, M. M., & Sampaio, C. (2009) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd006455.pub2 · Cited by 100 · see citing works
MacDonald, M. E., Ginés, S., Gusella, J. F., & Wheeler, V. C. (2003) · NeuroMolecular Medicine · doi:10.1385/nmm:4:1-2:7 · Cited by 100 · see citing works
The relationship between CAG repeat length and clinical progression in Huntington's disease
Ravina, B. M., Romer, M., Constantinescu, R., Biglan, K., Brocht, A. F. D., Kieburtz, K. D., Shoulson, I., & McDermott, M. (2008) · Movement Disorders · doi:10.1002/mds.21988 · Cited by 99 · see citing works
Therapy development in Huntington disease: From current strategies to emerging opportunities
Dickey, A. S., & Spada, A. R. L. (2017) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.38494 · Cited by 98 · see citing works
Clinical correlates of mitochondrial function in Huntington's disease muscle
Turner, C., Cooper, J. M., & Schapira, A. H. V. (2007) · Movement Disorders · doi:10.1002/mds.21540 · Cited by 97 · see citing works
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Cudkowicz, M., & The Huntington Study Group DOMINO Investigators (2010) · Movement Disorders · doi:10.1002/mds.23236 · Cited by 97 · see citing works
Modelling brain diseases in mice: the challenges of design and analysis
Watase, K., & Zoghbi, H. Y. (2003) · Nature Reviews Genetics · doi:10.1038/nrg1045 · Cited by 94 · see citing works
Cellular Consequences of Coenzyme Q10 Deficiency in Neurodegeneration of the Retina and Brain
Manzar, H., Abdulhussein, D., Yap, T. E., & Cordeiro, M. F. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21239299 · Cited by 94 · see citing works
The evidence basis for coenzyme Q therapy in oxidative phosphorylation disease
Haas, R. H. (2007) · Mitochondrion · doi:10.1016/j.mito.2007.03.008 · Cited by 92 · see citing works
Safety and tolerability of high‐dosage coenzyme Q10 in Huntington's disease and healthy subjects
The Huntington Study Group Pre2CARE Investigators (2010) · Movement Disorders · doi:10.1002/mds.22408 · Cited by 91 · see citing works
The relationship between uric acid levels and Huntington's disease progression
Auinger, P., Kieburtz, K. D., & McDermott, M. (2010) · Movement Disorders · doi:10.1002/mds.22907 · Cited by 90 · see citing works
Pharmacologic approaches to the treatment of Huntington's disease
Venuto, C. S., McGarry, A. J., Ma, Q., & Kieburtz, K. D. (2011) · Movement Disorders · doi:10.1002/mds.23953 · Cited by 86 · see citing works
Environmental, pharmacological, and genetic modulation of the HD phenotype in transgenic mice
Schilling, G., Savonenko, A. V., Coonfield, M. L., Morton, J. L., Vorovich, E. B., Gale, A., Neslon, C., Chan, N., Eaton, M., Fromholt, D., et al. (2004) · Experimental Neurology · doi:10.1016/j.expneurol.2004.01.003 · Cited by 85 · see citing works
Alternative designs for clinical trials in rare diseases
Abrahamyan, L., Feldman, B. M., TOMLINSON, G. A., Faughnan, M. E., Johnson, S. R., Diamond, I. R., & Gupta, S. (2016) · American Journal of Medical Genetics Part C Seminars in Medical Genetics · doi:10.1002/ajmg.c.31533 · Cited by 85 · see citing works
Bergamini, C., Moruzzi, N., Sblendido, A., Lenaz, G., & Fato, R. (2012) · PLoS ONE · doi:10.1371/journal.pone.0033712 · Cited by 84 · see citing works
Coenzyme Q10: Clinical Applications beyond Cardiovascular Diseases
Testai, L., Martelli, A., Flori, L., Cicero, A. F. G., & Colletti, A. (2021) · Nutrients · doi:10.3390/nu13051697 · Cited by 82 · see citing works
Bioequivalence of coenzyme Q10 from over-the-counter supplements
Miles, M. V., Horn, P. S., Miles, L., Tang, P., Steele, P. E., & deGrauw, T. J. (2002) · Nutrition Research · doi:10.1016/s0271-5317(02)00402-5 · Cited by 81 · see citing works
The spinocerebellar ataxias: Order emerges from chaos
Margolis, R. L. (2002) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-002-0072-8 · Cited by 81 · see citing works
Animal models of Huntington's disease for translation to the clinic: Best practices
Menalled, L., & Brunner, D. (2014) · Movement Disorders · doi:10.1002/mds.26006 · Cited by 79 · see citing works
Interrater agreement in the assessment of motor manifestations of Huntington's disease
Hogarth, P., Kayson, E., Kieburtz, K. D., Marder, K. S., Oakes, D., Rosas, H. D., Shoulson, I., Wexler, N. S., Young, A. B., Zhao, H., et al. (2004) · Movement Disorders · doi:10.1002/mds.20332 · Cited by 78 · see citing works
Self-citationTreatment of Parkinson???s Disease
Wu, S., & Frucht, S. J. (2005) · CNS Drugs · doi:10.2165/00023210-200519090-00001 · Cited by 78 · see citing works
Ryu, J. K., Choi, H. B., & McLarnon, J. (2006) · Neuroscience · doi:10.1016/j.neuroscience.2006.05.043 · Cited by 83 · see citing works
Coenzyme Q treatment of neurodegenerative diseases of aging
Galpern, W. R., & Cudkowicz, M. (2007) · Mitochondrion · doi:10.1016/j.mito.2007.01.004 · Cited by 78 · see citing works
Coenzyme Q10 a mitochondrial restorer for various brain disorders
Pradhan, N., Singh, C., & Singh, A. (2021) · Naunyn-Schmiedeberg s Archives of Pharmacology · doi:10.1007/s00210-021-02161-8 · Cited by 77 · see citing works
Miles, M. V., Horn, P. S., Morrison, J. A., Tang, P., deGrauw, T. J., & Pesce, A. J. (2003) · Clinica Chimica Acta · doi:10.1016/s0009-8981(03)00137-2 · Cited by 77 · see citing works
Therapeutic role of coenzyme Q10 in Parkinson's disease
Shults, C. W. (2005) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2005.02.002 · Cited by 76 · see citing works
Lesser, G. J., Case, D., Stark, N. N., Williford, S., Giguere, J. K., Garino, L. A., Naughton, M. J., Vitolins, M. Z., Lively, M. O., & Shaw, E. G. (2012) · The Journal of Supportive Oncology · doi:10.1016/j.suponc.2012.03.003 · Cited by 75 · see citing works
Microencapsulation of ubiquinone-10 in carbohydrate matrices for improved stability
Bule, M. V., Singhal, R. S., & Kennedy, J. F. (2010) · Carbohydrate Polymers · doi:10.1016/j.carbpol.2010.07.012 · Cited by 75 · see citing works
Translating therapies for Huntington’s disease from genetic animal models to clinical trials
Hersch, S. M., & Ferrante, R. J. (2004) · NeuroRx · doi:10.1602/neurorx.1.3.298 · Cited by 75 · see citing works
- Authors
- The Huntington Study Group
- Corresponding author
- The Huntington Study Group
- Venue
- Neurology
- Published
- 2001-08-14
- Volume
- 57
- Issue
- 3
- Pages
- 397-404
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- OpenAlex
- W4245847463
- Cited by
- 312
- References
- 19
- Citations by year
- 2026: 2 · 2025: 8 · 2024: 4 · 2023: 5 · 2022: 8 · 2021: 10 · 2020: 8 · 2019: 9 · 2018: 12 · 2017: 11 · 2016: 11 · 2015: 3 · 2014: 9 · 2013: 5 · 2012: 16
- FWCI
- 7.24
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Neurology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institute of Neurological Disorders and Stroke (NS R01-35284, R01NS035284)
- National Center for Research Resources (RR-00044, RR 01066, RR 07122, M01RR001066, M01RR000044, RR00042, RR 00645, M01RR000042, M01RR000645)
- 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, coenzyme Q10, remacemide, functional decline, total functional capacity, disease-modifying therapy, early-stage Huntington's disease, adverse events, nausea and vomiting
- MeSH terms
- Acetamides, Adult, Coenzymes, Double-Blind Method, Female, Humans, Huntington Disease, Male, Middle Aged, Prognosis, Time Factors, Ubiquinone
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