The evidence basis for coenzyme Q therapy in oxidative phosphorylation disease
Abstract
The evidence supporting a treatment benefit for coenzyme Q10 (CoQ10) in primary mitochondrial disease (mitochondrial disease) whilst positive is limited. Mitochondrial disease in this context is defined as genetic disease causing an impairment in mitochondrial oxidative phosphorylation (OXPHOS). There are no treatment trials achieving the highest Level I evidence designation. Reasons for this include the relative rarity of mitochondrial disease, the heterogeneity of mitochondrial disease, the natural cofactor status and easy 'over the counter availability' of CoQ10 all of which make funding for the necessary large blinded clinical trials unlikely. At this time the best evidence for efficacy comes from controlled trials in common cardiovascular and neurodegenerative diseases with mitochondrial and OXPHOS dysfunction the etiology of which is most likely multifactorial with environmental factors playing on a background of genetic predisposition. There remain questions about dosing, bioavailability, tissue penetration and intracellular distribution of orally administered CoQ10, a compound which is endogenously produced within the mitochondria of all cells. In some mitochondrial diseases and other commoner disorders such as cardiac disease and Parkinson's disease low mitochondrial or tissue levels of CoQ10 have been demonstrated providing an obvious rationale for supplementation. This paper discusses the current state of the evidence supporting the use of CoQ10 in mitochondrial disease.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Evidence suggests a net benefit of CoQ supplements to humans with neurodegenerative disease or genetic defects in mitochondrial function.
“Although human studies are limited, evidence suggests a net benefit of CoQ supplements to humans with neurodegenerative disease or genetic defects in mitochondrial function (126).”
From Mitochondrial Dysfunction in Diabetes: From Molecular Mechanisms to Functional Significance and Therapeutic Opportunities (2010) · cites this paper as [126]
Cites
Guyatt, G. H. (1992) · JAMA · doi:10.1001/jama.1992.03490170092032 · Cited by 4,447 · 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
Mitochondria take center stage in aging and neurodegeneration
Beal, M. F. (2005) · Annals of Neurology · doi:10.1002/ana.20624 · Cited by 957 · see citing works
Mitochondrial DNA deletions in human brain: regional variability and increase with advanced age
Corral-Debrinski, M., Horton, T., Lott, M. T., Shoffner, J. M., Flint Beal, M., & Wallace, D. C. (1992) · Nature Genetics · doi:10.1038/ng1292-324 · Cited by 890 · see citing works
Oxidative damage to mitochondrial DNA shows marked age-dependent increases in human brain
Mecocci, P., MacGarvey, U., Kaufman, A. E., Koontz, D., Shoffner, J. M., Wallace, D. C., & Beal, M. F. (1993) · Annals of Neurology · doi:10.1002/ana.410340416 · Cited by 757 · see citing works
Schapira, A. H. V. (2006) · The Lancet · doi:10.1016/s0140-6736(06)68970-8 · Cited by 594 · see citing works
Diagnostic criteria for respiratory chain disorders in adults and children
Bernier, F. P., Boneh, A., Dennett, X., Chow, C. W., Cleary, M., & Thorburn, D. R. (2002) · Neurology · doi:10.1212/01.wnl.0000033795.17156.00 · Cited by 574 · see citing works
Treatment for mitochondrial disorders
Pfeffer, G., Majamaa, K., Turnbull, D. M., Thorburn, D. R., & Chinnery, P. F. (2012) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd004426.pub3 · Cited by 501 · see citing works
Minimum birth prevalence of mitochondrial respiratory chain disorders in children
Skladal, D. (2003) · Brain · doi:10.1093/brain/awg170 · Cited by 487 · 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
Muscle coenzyme Q deficiency in familial mitochondrial encephalomyopathy.
Ogasahara, S., Engel, A. G., Frens, D. B., & Mack, D. (1989) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.86.7.2379 · Cited by 365 · see citing works
Dichloroacetate causes toxic neuropathy in MELAS
Kaufmann, P., Engelstad, K., Wei, Y., Jhung, S., Sano, M. C., Shungu, D. C., Millar, W. S., Hong, X., Gooch, C. L., Mao, X., et al. (2006) · Neurology · doi:10.1212/01.wnl.0000196641.05913.27 · Cited by 365 · see citing works
Association of mitochondrial DNA damage with aging and coronary atherosclerotic heart disease
Corral‐Debrinski, M., Shoffner, J. M., Lott, M. T., & Wallace, D. C. (1992) · Mutation Research/DNAging · doi:10.1016/0921-8734(92)90021-g · Cited by 360 · see citing works
Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency
Rötig, A., Appelkvist, E., Géromel, V., Chrétien, D., Kadhom, N., Edery, P., Bideau, M. L., Dallner, G., Münnich, A., Ernster, L., et al. (2000) · The Lancet · doi:10.1016/s0140-6736(00)02531-9 · Cited by 354 · see citing works
The Huntington Study Group (2001) · Neurology · doi:10.1212/wnl.57.3.397 · Cited by 312 · see citing works
Beneficial effects of creatine, CoQ<sub>10</sub>, and lipoic acid in mitochondrial disorders
Rodriguez, M. C., MacDonald, J. R., Mahoney, D. J., Parise, G., Beal, M. F., & Tarnopolsky, M. A. (2007) · Muscle & Nerve · doi:10.1002/mus.20688 · Cited by 255 · see citing works
The incidence of inherited metabolic disorders in the West Midlands, UK
Sanderson, S. C., Green, A., Preece, M. A., & Burton, H. (2006) · Archives of Disease in Childhood · doi:10.1136/adc.2005.091637 · Cited by 253 · see citing works
Familial cerebellar ataxia with muscle coenzyme Q10 deficiency
Musumeci, O., Naini, A., Slonim, A. E., Skavin, N., Hadjigeorgiou, G. L., Krawiecki, N., Weissman, B. M., Tsao, C., Mendell, J. R., Shanske, S., et al. (2001) · Neurology · doi:10.1212/wnl.56.7.849 · Cited by 252 · see citing works
Rundek, T., Naini, A., Sacco, R. L., Coates, K., & DiMauro, S. (2004) · Archives of Neurology · doi:10.1001/archneur.61.6.889 · Cited by 229 · see citing works
Mitochondrial DNA mutations in human degenerative diseases and aging
Wallace, D. C., Shoffner, J. M., Trounce, I. A., Brown, M. D., Ballinger, S. W., Corral‐Debrinski, M., Horton, T. M., Jun, A. S., & Lott, M. T. (1995) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/0925-4439(95)00021-u · Cited by 225 · see citing works
Antioxidant Treatment of Patients With Friedreich Ataxia
Hart, P. E., Lodi, R., Rajagopalan, B., Bradley, J. L., Crilley, J. G., Turner, C., Blamire, A. M., Manners, D. N., Styles, P., Schapira, A. H. V., et al. (2005) · Archives of Neurology · doi:10.1001/archneur.62.4.621 · Cited by 224 · see citing works
Pilot trial of high dosages of coenzyme Q10 in patients with Parkinson's disease
Shults, C. W., Flint Beal, M., Song, D., & Fontaine, D. (2004) · Experimental Neurology · doi:10.1016/j.expneurol.2004.05.003 · Cited by 223 · see citing works
Kwong, L. K., Kamzalov, S., Rebrin, I., Bayne, A.-C. V., Jana, C. K., Morris, P., Forster, M. J., & Sohal, R. S. (2002) · Free Radical Biology and Medicine · doi:10.1016/s0891-5849(02)00916-4 · Cited by 198 · see citing works
Infantile encephalomyopathy and nephropathy with CoQ10 deficiency: A CoQ10-responsive condition
Salviati, L., Sacconi, S., Murer, L., Zacchello, G., Franceschini, L., Laverda, A. M., Basso, G., Quinzii, C. M., Angelini, C. I., Hirano, M., et al. (2005) · Neurology · doi:10.1212/01.wnl.0000172859.55579.a7 · Cited by 196 · see citing works
Uptake of Dietary Coenzyme Q Supplement is Limited in Rats
Zhang, Y., Åberg, F. O., Appelkvist, E., Dallner, G., & Ernster, L. (1995) · Journal of Nutrition · doi:10.1093/jn/125.3.446 · Cited by 192 · see citing works
Quinzii, C. M., DiMauro, S., & Hirano, M. (2006) · Neurochemical Research · doi:10.1007/s11064-006-9190-z · Cited by 188 · see citing works
Muscle coenzyme Q10 level in statin-related myopathy
Lamperti, C., Naini, A. B., Lucchini, V., Prelle, A., Bresolin, N., Moggio, M., Sciacco, M., Kaufmann, P., & DiMauro, S. (2005) · Archives of Neurology · doi:10.1001/archneur.62.11.1709 · Cited by 178 · see citing works
Coenzyme Q deficiency and cerebellar ataxia associated with an aprataxin mutation
Quinzii, C. M., Kattah, A. G., Naini, A., Akman, H. O., Mootha, V. K., DiMauro, S., & Hirano, M. (2005) · Neurology · doi:10.1212/01.wnl.0000150588.75281.58 · Cited by 171 · see citing works
Coenzyme Q10 in cardiovascular disease
Pepe, S., Marasco, S., Haas, S. J., Sheeran, F. L., Krum, H., & Rosenfeldt, F. (2007) · Mitochondrion · doi:10.1016/j.mito.2007.02.005 · Cited by 165 · see citing works
Coenzyme Q 10 reverses pathological phenotype and reduces apoptosis in familial CoQ 10 deficiency
Giovanni, S. D., Massimiliano Mirabella, A., Spinazzola, A., Crociani, P., Silvestri, G., Broccolini, A., Tonali, P., Mauro, S. D., & Servidei, S. (2001) · Neurology · doi:10.1212/wnl.57.3.515 · Cited by 160 · see citing works
The Impact of Coenzyme Q10 on Systolic Function in Patients With Chronic Heart Failure
Sander, S. D., Coleman, C. I., Patel, A., Kluger, J., & White, C. M. (2006) · Journal of Cardiac Failure · doi:10.1016/j.cardfail.2006.03.007 · Cited by 159 · see citing works
Treatment 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
Wei, Y., Lu, C. Y., Wei, C. Y., Ma, Y. S., & Lee, H. (2001) · PubMed · Cited by 158 · see citing works
Tolerance of high‐dose (3,000 mg/day) coenzyme Q10 in ALS
Ferrante, K. L., Shefner, J., Zhang, H., Betensky, R., O'Brien, M., Yu, H., Fantasia, M., Taft, J., Beal, M. F., Traynor, B., et al. (2005) · Neurology · doi:10.1212/01.wnl.0000187070.35365.d7 · Cited by 154 · see citing works
Lass, A., Forster, M. J., & Sohal, R. S. (1999) · Free Radical Biology and Medicine · doi:10.1016/s0891-5849(98)00330-x · Cited by 148 · see citing works
Distribution and breakdown of labeled coenzyme Q10 in rat
Bentinger, M., Dallner, G., Chojnacki, T., & Świeżewska, E. (2003) · Free Radical Biology and Medicine · doi:10.1016/s0891-5849(02)01357-6 · Cited by 142 · see citing works
Rules of evidence and clinical recommendations for the management of patients.
Sackett, D. L. (1993) · PubMed · Cited by 134 · see citing works
Rosenfeldt, F., Marasco, S., Lyon, W. J., Wowk, M., Sheeran, F. L., Bailey, M., Esmore, D. S., Davis, B. B., Pick, A., Rabinov, M., et al. (2004) · Journal of Thoracic and Cardiovascular Surgery · doi:10.1016/j.jtcvs.2004.03.034 · Cited by 131 · see citing works
Treatment for mitochondrial disorders
Chinnery, P. F., Majamaa, K., Turnbull, D. M., & Thorburn, D. R. (2006) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd004426.pub2 · Cited by 129 · see citing works
A case of mitochondrial encephalomyopathy associated with a muscle coenzyme Q10 deficiency
Boitier, E., Degoul, F., Desguerre, I., Charpentier, C., François, D., Ponsot, G., Diry, M., Rustin, P., & Marsac, C. (1998) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(98)00006-9 · Cited by 127 · see citing works
Isolated Mitochondrial Myopathy Associated With Muscle Coenzyme Q10 Deficiency
Lalani, S. R., Vladutiu, G. D., Plunkett, K., Lotze, T. E., Adesina, A. M., & Scaglia, F. (2005) · Archives of Neurology · doi:10.1001/archneur.62.2.317 · Cited by 127 · 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
Suzuki, S., Hinokio, Y., Ohtomo, M., Hirai, M., Hirai, A. M., Chiba, M., Kasuga, S., Sato, Y., Akai, H., & Toyota, T. (1998) · Diabetologia · doi:10.1007/s001250050950 · Cited by 116 · see citing works
Ubidecarenone in the treatment of mitochondrial myopathies: a multi-center double-blind trial
Bresolin, N., Doriguzzi, C., Ponzetto, C., Angelini, C. I., Moroni, I., Castelli, E., Cossutta, E., Binda, A., Gallanti, A., Gabellini, S., et al. (1990) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(90)90015-f · Cited by 109 · see citing works
Coenzyme Q10 Treatment in Mitochondrial Encephalomyopathies
Lang, C. J. G., Sittl, H., & Erbguth, F. (2008) · European Neurology · doi:10.1159/000117445 · Cited by 107 · 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
Mitochondrial Disease: Mutations and Mechanisms
McKenzie, M., Liolitsa, D., & Hanna, M. G. (2004) · Neurochemical Research · doi:10.1023/b:nere.0000014829.42364.dd · 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
Artuch, R., Brea‐Calvo, G., Briones, P., Aracil, A., Galván, M. F., Espinós, C., Corral, J., Volpini, V., Ribes, A., Andreu, A. L., et al. (2006) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2006.01.021 · Cited by 100 · see citing works
Folkers, K., & Simonsen, R. (1995) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/0925-4439(95)00040-b · Cited by 91 · see citing works
Kamzalov, S., Sohal, R. S., Sumien, N., & Forster, M. J. (2003) · Journal of Nutrition · doi:10.1093/jn/133.10.3175 · Cited by 89 · see citing works
Evidence-based to value-based medicine
Brown, M. M., Brown, G. C., & Sharma, S. (2005) · Medical Entomology and Zoology · Cited by 80 · see citing works
Zierz, S., Jahns, G., & Jerusalem, F. (1989) · Journal of Neurology · doi:10.1007/bf00314404 · Cited by 74 · see citing works
Abe, K., Matsuo, Y., Kadekawa, J., Inoue, S., & Yanagihara, T. (1999) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(98)00296-2 · Cited by 67 · see citing works
Chronic treatment of mitochondrial disease patients with dichloroacetate
Barshop, B. A., Naviaux, R. K., McGowan, K. A., Levine, F., Nyhan, W. L., Loupis-Geller, A., & Haas, R. (2004) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2004.06.009 · Cited by 67 · see citing works
Self-citation(1997) · PubMed · Cited by 65 · see citing works
The treatment of mitochondrial myopathies and encephalomyopathies
Peterson, P. L. (1995) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/0925-4439(95)00039-7 · Cited by 64 · see citing works
Clinical trials of coenzyme Q10 in neurological disorders
Shults, C. W., & Haas, R. (2005) · BioFactors · doi:10.1002/biof.5520250113 · Cited by 62 · see citing works
Self-citationMetabolic changes in patients with mitochondrial myopathies and effects of coenzyme Q 10 therapy
Chan, A., Reichmann, H., Kögel, A., Beck, A., & Gold, R. (1998) · Journal of Neurology · doi:10.1007/s004150050267 · Cited by 62 · see citing works
Quiles, J. L., Ochoa, J. J., Battino, M. A., Gutierrez-Ríos, P., Araujo-Nepomuceno, E., López‐Frías, M., Huertas, J. R., & Mataix, J. (2005) · BioFactors · doi:10.1002/biof.5520250109 · Cited by 51 · see citing works
YUZURIHA, T., TAKADA, M., & Katayama, K. (1983) · Biochimica et Biophysica Acta (BBA) - General Subjects · doi:10.1016/0304-4165(83)90325-2 · Cited by 43 · see citing works
Effects of mevinolin treatment on tissue dolichol and ubiquinone levels in the rat
Lőw, P., Andersson, M., Edlund, C., & Dallner, G. (1992) · Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism · doi:10.1016/0005-2760(92)90081-6 · Cited by 42 · see citing works
Muscle coenzyme Q10 in mitochondrial encephalomyopathies
Matsuoka, T., Maeda, H., Goto, Y., & Nonaka, I. (1991) · Neuromuscular Disorders · doi:10.1016/0960-8966(91)90007-f · Cited by 37 · see citing works
Exercise intolerance in mitochondrial myopathy is not related to lactic acidosis
Vissing, J., Gansted, U., & Quistorff, B. (2001) · Annals of Neurology · doi:10.1002/ana.1026 · Cited by 35 · see citing works
Gold, R., Seibel, P., Reinelt, G., Schindler, R., Landwehr, P., Beck, A., & Reichmann, H. (2008) · European Neurology · doi:10.1159/000117246 · Cited by 34 · see citing works
Montero, R., Artuch, R., Briones, P., Nascimento, A., García‐Cazorla, À., Vilaseca, M. A., Śnchez‐Alćzar, J. A., Navas, P., Montoya, J., & Pineda, M. (2005) · BioFactors · doi:10.1002/biof.5520250112 · Cited by 33 · see citing works
Evidence-Based to Value-Based Medicine
Parker, M. J. (2006) · ACP Journal Club · doi:10.7326/acpjc-2006-144-3-a10 · Cited by 27 · see citing works
Muscle Coenzyme Q: A Potential Test for Mitochondrial Activity and Redox Status
Miles, L., Miles, M. V., Tang, P., Horn, P. S., Wong, B. L. Y., deGrauw, T. J., Morehart, P. J., & Bove, K. E. (2005) · Pediatric Neurology · doi:10.1016/j.pediatrneurol.2005.01.009 · Cited by 25 · see citing works
Desnuelle, C., Pellissier, J., Serratrice, G., Pouget, J., & Turnbull, D. M. (1989) · PubMed · Cited by 18 · see citing works
Coenzyme Q10 in dilated cardiomyopathy.
Manzoli, U. C., Rossi, E. M. C., Littarru, G. P., Frustaci, A., Lippa, S., ORADEI, A., & Aureli, V. (1990) · PubMed · Cited by 17 · see citing works
Scalori, V., Alessandri, M., Giovannini, L., & Bertelli, A. (1990) · PubMed · Cited by 12 · see citing works
Tissue Bioavailability and Detection of Coenzyme Q
Rebrin, I., Kamzalov, S., & Sohal, R. S. (2004) · Methods in enzymology on CD-ROM/Methods in enzymology · doi:10.1016/s0076-6879(04)78009-x · Cited by 5 · see citing works
Flagged first, then by citations
3 of the 75 references couldn’t be matched to a known paper.
Cited by
Sorted by citations
Oxidative stress, mitochondrial damage and neurodegenerative diseases.
Guo, C., Sun, L., Chen, X., & Zhang, D. (2013) · PubMed Central · doi:10.3969/j.issn.1673-5374.2013.21.009 · Cited by 1,308 · see citing works
Sivitz, W. I., & Yorek, M. A. (2010) · Antioxidants & Redox Signaling · doi:10.1089/ars.2009.2531 · Cited by 742 · see citing works
Reactive Oxygen Species in the Regulation of Synaptic Plasticity and Memory
Massaad, C. A., & Klann, E. (2011) · Antioxidants & Redox Signaling · doi:10.1089/ars.2010.3208 · Cited by 635 · see citing works
Parikh, S., Goldstein, A., Koenig, M. K., Scaglia, F., Enns, G. M., Saneto, R., Anselm, I., Cohen, B. H., Falk, M. J., Greene, C., et al. (2015) · Genetics in Medicine · doi:10.1038/gim.2014.177 · Cited by 607 · see citing works
Self-citationThe Effect of Coenzyme Q 10 on Morbidity and Mortality in Chronic Heart Failure
Mortensen, S. A., Rosenfeldt, F., Kumar, A., Dolliner, P., Filipiak, K. J., Pella, D., Alehagen, U., Steurer, G., & Littarru, G. P. (2014) · JACC: Heart Failure · doi:10.1016/j.jchf.2014.06.008 · Cited by 488 · see citing works
Mitochondrial dysfunction in neurodegenerative disorders
Klemmensen, M. M., Borrowman, S. H., Pearce, C., Pyles, B., & Chandra, B. (2024) · Neurotherapeutics · doi:10.1016/j.neurot.2023.10.002 · Cited by 396 · see citing works
The in-depth evaluation of suspected mitochondrial disease
Haas, R., Parikh, S., Falk, M. J., Saneto, R. P., Wolf, N. I., Darín, N., Wong, L.-J. C., Cohen, B. H., & Naviaux, R. K. (2008) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2007.11.018 · Cited by 363 · see citing works
Self-citationJin, H., Kanthasamy, A., Ghosh, A., Anantharam, V., Kalyanaraman, B., & Kanthasamy, A. G. (2013) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2013.09.007 · Cited by 362 · see citing works
A modern approach to the treatment of mitochondrial disease
The Mitochondrial Medicine Society, Parikh, S., Saneto, R. P., Falk, M. J., Anselm, I. A., Cohen, B. H., & Haas, R. (2009) · Current Treatment Options in Neurology · doi:10.1007/s11940-009-0046-0 · Cited by 349 · see citing works
Self-citationWang, Y., & Hekimi, S. (2016) · Trends in Cell Biology · doi:10.1016/j.tcb.2015.12.007 · Cited by 256 · see citing works
Magen, D., Georgopoulos, C. P., Bross, P., Ang, D., Segev, Y., Goldsher, D., Nemirovski, A., Shahar, E. M., Ravid, S., LUDER, A. S., et al. (2008) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2008.05.016 · Cited by 212 · see citing works
Scaini, G., Rezin, G. T., Carvalho, A. F., STRECK, E. L., Berk, M., & Quevedo, J. (2016) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2016.06.040 · Cited by 184 · see citing works
Clinical applications of coenzyme Q₁₀
Garrido-Maraver, J. (2014) · Frontiers in Bioscience · doi:10.2741/4231 · Cited by 178 · see citing works
Tarnopolsky, M. A. (2008) · Advanced Drug Delivery Reviews · doi:10.1016/j.addr.2008.05.001 · Cited by 158 · see citing works
Garrido‐Maraver, J., Cordero, M. D., Oropesa-Ávila, M., Fernández-Vega, A., de la Mata, M., Pavón, A. D., de Miguel, M., Calero, C. P., Paz, M. V., Cotán, D., et al. (2014) · Molecular Syndromology · doi:10.1159/000360101 · Cited by 154 · see citing works
Noh, Y. H., Kim, K., Shim, M. S., Choi, S.-H., Choi, S., Ellisman, M. H., Weinreb, R. N., Perkins, G. A., & Ju, W.-K. (2013) · Cell Death and Disease · doi:10.1038/cddis.2013.341 · Cited by 146 · see citing works
A 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
Kucharczyk, R., Zick, M., Bietenhader, M., Rak, M., Couplan, E., Blondel, M., Duvezin‐Caubet, S., & di Rago, J. (2008) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2008.06.012 · Cited by 121 · 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
Mitochondrial disease patient motivations and barriers to participate in clinical trials
Zolkipli‐Cunningham, Z., Xiao, R., Stoddart, A., McCormick, E. M., Holberts, A., Burrill, N., McCormack, S. E., Williams, L., Wang, X., Thompson, J. L. P., et al. (2018) · PLoS ONE · doi:10.1371/journal.pone.0197513 · Cited by 81 · see citing works
Wang, Y., Lilienfeldt, N., & Hekimi, S. (2024) · Physiological Reviews · doi:10.1152/physrev.00040.2023 · Cited by 83 · see citing works
Polyák, E., Ostrovsky, J., Peng, M., Dingley, S. D., Tsukikawa, M., Kwon, Y. J., McCormack, S. E., Bennett, M. J., Xiao, R., Seiler, C., et al. (2018) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2018.02.013 · Cited by 69 · see citing works
Bömer, N., Pávez, M., Beverborg, N. G., Cleland, J. G. F., van Veldhuisen, D. J., & van der Meer, P. (2022) · Journal of Internal Medicine · doi:10.1111/joim.13456 · Cited by 64 · see citing works
Limongelli, G., Masarone, D., D‘Alessandro, R., & Elliot, P. M. (2011) · Future Cardiology · doi:10.2217/fca.11.79 · Cited by 65 · see citing works
CoQ10Function and Role in Heart Failure and Ischemic Heart Disease
Ayer, A., Macdonald, P. S., & Stocker, R. (2015) · Annual Review of Nutrition · doi:10.1146/annurev-nutr-071714-034258 · Cited by 64 · see citing works
Tragni, V., Primiano, G., Tummolo, A., Beltrame, L. C., Piana, G. L., Sgobba, M. N., Cavalluzzi, M. M., Paterno, G., Gorgoglione, R., Volpicella, M., et al. (2022) · Molecules · doi:10.3390/molecules27113494 · Cited by 55 · see citing works
Fan, H., Lee, H.-F., Yue, C.-T., & Chi, C. (2021) · Life · doi:10.3390/life11111111 · Cited by 51 · see citing works
Probucol ameliorates renal and metabolic sequelae of primary CoQ deficiency in Pdss2 mutant mice
Falk, M. J., Polyák, E., Zhang, Z., Peng, M., King, R., Maltzman, J. S., Okwuego, E. Y., Horyn, O., Nakamaru‐Ogiso, E., Ostrovsky, J., et al. (2011) · EMBO Molecular Medicine · doi:10.1002/emmm.201100149 · Cited by 48 · see citing works
New Insights into Mitochondria in Health and Diseases
Li, Y., Zhang, H., Yu, C., Dong, X., Yang, F., Wang, M., Wen, Z., Su, M., Li, B., & Yang, L. (2024) · International Journal of Molecular Sciences · doi:10.3390/ijms25189975 · Cited by 46 · see citing works
Targeting Oxidative Stress Component in the Therapeutics of Epilepsy
Azam, F., Prasad, M. V. V., & Thangavel, N. (2012) · Current Topics in Medicinal Chemistry · doi:10.2174/156802612800229224 · Cited by 40 · see citing works
Emerging therapeutic approaches to mitochondrial diseases
Wenz, T., Williams, S. L., Bacman, S. R., & Moraes, C. T. (2010) · Developmental Disabilities Research Reviews · doi:10.1002/ddrr.109 · Cited by 40 · see citing works
Treatment of Mitochondrial Disorders
Enns, G. M. (2014) · Journal of Child Neurology · doi:10.1177/0883073814538509 · Cited by 39 · see citing works
Symptoms of somatization as a rapid screening tool for mitochondrial dysfunction in depression
Gardner, A., & Boles, R. G. (2008) · BioPsychoSocial Medicine · doi:10.1186/1751-0759-2-7 · Cited by 38 · see citing works
Effect of coenzyme Q10 supplementation on fatigue: A systematic review of interventional studies
Mehrabani, S., Askari, G., Miraghajani, M. S., Tavakoly, R., & Arab, A. (2019) · Complementary Therapies in Medicine · doi:10.1016/j.ctim.2019.01.022 · Cited by 37 · see citing works
How Can We Treat Mitochondrial Encephalomyopathies? Approaches to Therapy
Horváth, R., Gorman, G. S., & Chinnery, P. F. (2008) · Neurotherapeutics · doi:10.1016/j.nurt.2008.07.002 · Cited by 36 · see citing works
Coenzyme Q10 oral bioavailability: effect of formulation type
Barakat, A., Shegokar, R., Dittgen, M., & Müller, R. H. (2013) · Journal of Pharmaceutical Investigation · doi:10.1007/s40005-013-0101-4 · Cited by 35 · see citing works
Nutritional Interventions for Mitochondrial OXPHOS Deficiencies: Mechanisms and Model Systems
Kuszak, A. J., Espey, M. G., Falk, M. J., Holmbeck, M. A., Manfredi, G., Shadel, G. S., Vernon, H. J., & Zolkipli‐Cunningham, Z. (2017) · Annual Review of Pathology Mechanisms of Disease · doi:10.1146/annurev-pathol-020117-043644 · Cited by 33 · see citing works
Molecular diagnosis of coenzyme Q10 deficiency: an update
Yubero, D., Montero, R., Santos‐Ocaña, C., Salviati, L., Navas, P., & Artuch, R. (2018) · Expert Review of Molecular Diagnostics · doi:10.1080/14737159.2018.1478290 · Cited by 29 · see citing works
Redox State of Coenzyme Q10 Determines Its Membrane Localization
Ausili, A., Torrecillas, A., Aranda, F. J., de Godos, A. M., Sánchez‐Bautista, S., Corbalán‐García, S., & Gómez-Fernández, J. C. (2008) · The Journal of Physical Chemistry B · doi:10.1021/jp802215s · Cited by 28 · see citing works
Involvement of Coenzyme Q10 in Various Neurodegenerative and Psychiatric Diseases
Ebrahimi, A., Kamyab, A., Hosseini, S., Ebrahimi, S., & Ashkani‐Esfahani, S. (2023) · Biochemistry Research International · doi:10.1155/2023/5510874 · Cited by 29 · see citing works
Stacpoole, P. W., deGrauw, T. J., Feigenbaum, A. S. J., Hoppel, C. L., Kerr, D. S., McCandless, S. E., Miles, M. V., Robinson, B. H., & Tang, P. (2012) · Mitochondrion · doi:10.1016/j.mito.2012.09.005 · Cited by 25 · see citing works
Why are there no proven therapies for genetic mitochondrial diseases?
Stacpoole, P. W. (2011) · Mitochondrion · doi:10.1016/j.mito.2011.05.002 · Cited by 24 · see citing works
Wojtala, A., Karkucińska‐Więckowska, A., Sardão, V. A., Szczepanowska, J., Kowalski, P., Pronicki, M., Duszyński, J., & Wieckowski, M. R. (2017) · Mitochondrion · doi:10.1016/j.mito.2017.07.002 · Cited by 23 · see citing works
Payne, R. M., Pride, P. M., & Babbey, C. M. (2011) · Pediatric Cardiology · doi:10.1007/s00246-011-9943-6 · Cited by 21 · see citing works
Alcocer‐Gómez, E., Cano‐García, F. J., & Cordero, M. D. (2013) · Nutrition · doi:10.1016/j.nut.2013.05.005 · Cited by 21 · see citing works
Functional Properties of Meat in Athletes’ Performance and Recovery
di Corcia, M., Tartaglia, N., Polito, R., Ambrosi, A., Messina, G., Francavilla, V. C., Cincione, R. I., della Malva, A., Ciliberti, M. G., Sevi, A., et al. (2022) · International Journal of Environmental Research and Public Health · doi:10.3390/ijerph19095145 · Cited by 20 · see citing works
Ovchinnikov, A. N., Paoli, A., Seleznev, V. V., & Deryugina, A. V. (2022) · Journal of the International Society of Sports Nutrition · doi:10.1080/15502783.2022.2086015 · Cited by 18 · see citing works
Yen, H., Chen, F. F., Chen, S., Huang, Y., Chen, Y., & Chen, C. (2011) · Journal of Biochemical and Molecular Toxicology · doi:10.1002/jbt.20387 · Cited by 17 · see citing works
Therapeutical Management and Drug Safety in Mitochondrial Diseases—Update 2020
Gruosso, F., Montano, V., Simoncini, C., Siciliano, G., & Mancuso, M. (2020) · Journal of Clinical Medicine · doi:10.3390/jcm10010094 · Cited by 16 · see citing works
Haddad, H. W., Mallepalli, N. R., Scheinuk, J. E., Bhargava, P., Cornett, E. M., Urits, I., & Kaye, A. D. (2021) · Pain and Therapy · doi:10.1007/s40122-021-00266-9 · Cited by 17 · see citing works
Coenzyme Q10 in the Treatment of Corneal Edema in Kearns-Sayre
Kim, J. T., Medsinge, A., Chauhan, B. K., Wiest, C., Scanga, H. L., Monaghan, R., Moore, W. H., & Nischal, K. K. (2016) · Cornea · doi:10.1097/ico.0000000000000927 · Cited by 16 · see citing works
Cusimano, E. M., Knight, A. R., Slusser, J. G., Clancy, R. L., & Pierce, J. D. (2009) · Advanced Emergency Nursing Journal · doi:10.1097/tme.0b013e3181956200 · Cited by 15 · see citing works
Vyas, P. M., & Payne, R. M. (2008) · Molecular Therapy · doi:10.1038/mt.2008.24 · Cited by 15 · see citing works
Heart Transplantation for a Patient With Kearns-Sayre Syndrome and End-Stage Heart Failure
Homan, D. J., Niyazov, D. M., Fisher, P. W., Mandras, S. A., Patel, H. M., Bates, M. J., Parrino, G., & Ventura, H. O. (2011) · Congestive Heart Failure · doi:10.1111/j.1751-7133.2011.00211.x · Cited by 15 · see citing works
Mitochondrial myopathy presenting as fibromyalgia: a case report
Abdullah, M., Vishwanath, S., Elbalkhi, A., & Ambrus, J. L. (2012) · Journal of Medical Case Reports · doi:10.1186/1752-1947-6-55 · Cited by 14 · see citing works
Secondary coenzyme Q deficiency in neurological disorders
Guéguen, N., Baris, O. R., Lenaers, G., Reynier, P., & Spinazzi, M. (2021) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2021.01.017 · Cited by 14 · see citing works
Hawi, A. A., Heald, S. L., & Sciascia, T. R. (2011) · The Journal of Clinical Pharmacology · doi:10.1177/0091270010390807 · Cited by 11 · see citing works
Ovchinnikov, A. N., Deryugina, A. V., & Paoli, A. (2022) · Frontiers in Nutrition · doi:10.3389/fnut.2022.893515 · Cited by 11 · see citing works
Cordero, M. D., Santos-Garcia, R., Bermejo-Jover, D., Sánchez-Domínguez, B., Jaramillo-Santos, M. R., & Bullón, P. (2012) · Clinical Biochemistry · doi:10.1016/j.clinbiochem.2012.02.001 · Cited by 9 · see citing works
Nyariki, J. N., Kimani, N. M., Kibet, P. S., Kinuthia, G. K., & Isaac, A. O. (2023) · Molecular and Biochemical Parasitology · doi:10.1016/j.molbiopara.2023.111579 · Cited by 8 · see citing works
Zheng, S., Wang, J., Sun, M., Wang, P., Shi, W., Zhang, Z., Wang, Z., & Zhang, H. (2024) · Clinical Case Reports · doi:10.1002/ccr3.8458 · Cited by 8 · see citing works
The current landscape for the treatment of mitochondrial disorders
Slone, J. D., Gui, B., & Huang, T. (2018) · Journal of genetics and genomics/Journal of Genetics and Genomics · doi:10.1016/j.jgg.2017.11.008 · Cited by 8 · see citing works
Neyrinck, A. M., Catry, É., Sohet, F. M., Cani, P. D., Pachikian, B. D., Bindels, L. B., & Delzenne, N. M. (2015) · Clinical Nutrition Experimental · doi:10.1016/j.yclnex.2015.07.002 · Cited by 7 · see citing works
Saeed, U., Piracha, Z. Z., Tariq, M. N., Gilani, S. S., Rauf, M. A., Razaq, L., Iftikhar, M. K., Maqsood, A., & Ahsan, S. M. (2025) · Frontiers in Neurology · doi:10.3389/fneur.2025.1422707 · Cited by 6 · see citing works
H-FABP Levels and Psycho-Emotional Improvement of CABG Patients during Cardiac Rehabilitation
Namat, R. A., Namat, D. A., Ciocoiu, M., Hînganu, M. V., Șorodoc, L., Șorodoc, V., Foia, L. G., Florea, L., Vlad, C.-E., Tănasă, A., et al. (2022) · Journal of Cardiovascular Development and Disease · doi:10.3390/jcdd9080242 · Cited by 6 · see citing works
Reiner, G., & Panyard-Davis, J. L. (2012) · Nursing · doi:10.1097/01.nurse.0000413616.59485.cf · Cited by 6 · see citing works
Muscle Physiology in Healthy Men and Women and Those with Metabolic Myopathies
Devries, M. C., & Tarnopolsky, M. A. (2008) · Neurologic Clinics · doi:10.1016/j.ncl.2007.11.010 · Cited by 6 · see citing works
Metabolic myopathy presenting with polyarteritis nodosa: a case report
Vishwanath, S., Abdullah, M., Elbalkhi, A., & Ambrus, J. L. (2011) · Journal of Medical Case Reports · doi:10.1186/1752-1947-5-262 · Cited by 5 · see citing works
Investigating Fatigue and Exercise Intolerance in a University Immunology Clinic
Ambrus, J. L. (2020) · Archives of Rheumatology & Arthritis Research · doi:10.33552/arar.2020.01.000505 · Cited by 4 · see citing works
Madden, R. F., Lalonde-Bester, S., Parnell, J. A., Trudeau, M. S., Martin, J. M., Khan, A., & Shearer, J. (2022) · Clinical Nutrition ESPEN · doi:10.1016/j.clnesp.2022.06.016 · Cited by 4 · see citing works
Mitochondrial Dysfunction in Neurodegenerative Diseases
Chen, C., Zhao, Y., Wang, J., Pan, D., Wang, X., Xu, Y., Yan, J., Wang, L., Yang, X., Lu, M., et al. (2025) · MedComm · doi:10.1002/mco2.70326 · Cited by 3 · see citing works
Sarkar, M., Bag, P., Kumar, B., & Chawla, P. A. (2021) · Indian Journal of Pharmaceutical Sciences · doi:10.36468/pharmaceutical-sciences.875 · Cited by 3 · see citing works
Murray, K. E., Ratliff, W. A., Delic, V., & Citron, B. A. (2023) · Brain Research · doi:10.1016/j.brainres.2023.148682 · Cited by 3 · see citing works
Gvozdjáková, A., Mravec, B., Kucharská, J., Lackovicova, L., Ondičová, K., Tkačov, M., & Singh, R. B. (2012) · General Physiology and Biophysics · doi:10.4149/gpb_2012_052 · Cited by 3 · see citing works
Recovering Mitochondrial Function in Patients’ Fibroblasts
Wieckowski, M. R., Danese, A., Morciano, G., Pronicki, M., Duszyński, J., Pinton, P., & Karkucińska‐Więckowska, A. (2018) · Springer eBooks · doi:10.1007/978-3-319-73344-9_17 · Cited by 3 · see citing works
Muscle Physiology in Healthy Men and Women and Those with Metabolic Myopathies
Devries, M. C., & Tarnopolsky, M. A. (2008) · Physical Medicine and Rehabilitation Clinics of North America · doi:10.1016/j.pmr.2008.10.011 · Cited by 2 · see citing works
Le, P.-Q., Kim, J. H., Park, K., & Lee, B. (2026) · International Journal of Pharmaceutics · doi:10.1016/j.ijpharm.2026.127496 · Cited by 1 · see citing works
Supporting Children With Mitochondrial Disorders
Haas, R., Hoy, L., & Berenhaut, E. (2014) · ICAN Infant Child & Adolescent Nutrition · doi:10.1177/1941406414531929 · Cited by 1 · see citing works
Self-citationMitochondrial diseases: past, present, and future
Akl, A. I., Afsah, I., & Saad, R. A. (2022) · Urology & Nephrology Open Access Journal · doi:10.15406/unoaj.2022.10.00316 · Cited by 1 · see citing works
Enfermedades mitocondriales: problemas en la planta de producción de energía
Reiner, G., & Panyard-Davis, J. L. (2012) · Nursing (Ed española) · doi:10.1016/s0212-5382(12)70114-0
KATERE SO PRILAGODITVENE REAKCIJE NA TELESNO
Eiken, O. (2008)
Potentiel thérapeutique du coenzyme Q10
Pincemail, J., & Defraigne, J.-O. (2008) · ORBi (University of Liège)
MITOCHONDRIAL CYTOPATHIES IN CHILDREN AND ADULTS
Tarnopolsky, M. A. (2009) · CONTINUUM Lifelong Learning in Neurology · doi:10.1212/01.con.0000348880.16694.08
(2008) · Current Opinion in Neurology · doi:10.1097/wco.0b013e32830fb782
Nischal, K. K., Scanga, H. L., & Kim, J. T. (2016) · Cornea · doi:10.1097/ico.0000000000001041
Zheng, S., Wang, J., Sun, M., Wang, P., Shi, W. M., Zhang, Z., Wang, Z., & Zhang, H. (2023) · doi:10.22541/au.169459029.98052841/v1
Dilaver, E., & Yılmaz, S. E. (2024) · Toros University Journal of Food Nutrition and Gastronomy · doi:10.58625/jfng-2671
Rath, D., Roberts, V., Waghmare, S., Hassan, H., Harris, M., Ramallo, M., Kuo, P., Zinabu, S., Bisrat, M., & Michael, M. B. (2025) · medtigo Journal of Medicine · doi:10.63096/medtigo3092224
Chang, J. S. (2022) · Digital Archive @ GSU · doi:10.57709/1955407
- Authors
- Haas, R. H.
- Affiliations
- University of California San Diego (US)
- Corresponding author
- Haas, Richard H.
- ORCID
- Haas, Richard H.
- Venue
- Mitochondrion
- Publisher
- Elsevier BV
- Published
- 2007-06
- Volume
- 7
- Pages
- S136-S145
- ISSN
- 1567-7249 (ISSN-L)
- OpenAlex
- W2015731982
- Cited by
- 92
- References
- 75
- Citations by year
- 2026: 1 · 2025: 3 · 2024: 4 · 2023: 5 · 2022: 9 · 2021: 5 · 2020: 4 · 2019: 1 · 2018: 5 · 2017: 2 · 2016: 4 · 2015: 2 · 2014: 6 · 2013: 5 · 2012: 8
- FWCI
- 2.65
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Mitochondrion (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Wright Family Foundation
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Coenzyme Q10 studies and effects
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Keywords
- coenzyme Q10, mitochondrial disease, oxidative phosphorylation, primary mitochondrial disease, coenzyme Q10 supplementation, evidence base, clinical trial feasibility, Parkinson's disease, heart disease, neurodegenerative diseases
- MeSH terms
- Animals, Antioxidants, Cardiovascular Diseases, Coenzymes, Guinea Pigs, Humans, Mitochondria, Models, Biological, Oxidative Phosphorylation, Oxygen, Phosphorylation, Ubiquinone, Evidence-Based Medicine, Mitochondrial Diseases, Rats
About these checks
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.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review