Therapeutic use of coenzyme Q₁₀ and coenzyme Q₁₀-related compounds and formulations

Bibliography

• Crane FL, Hatefi Y, Lester RL, Widmer C. Isolation of a quinone from beef heart mitochondria. Biochim Biophys Acta 1957;25:220-1
   PubMed Web of Science ®Google Scholar
• Wolf DE, HoVman CH, Trenner NR, Coenzyme Q. Structure studies on the coenzyme Q group. J Am Chem Soc 1958;80:4750-2
   Google Scholar
• Crane FL. Biochemical functions of Coenzyme Q10. J Am Coll Nutr 2001;20:591-8
   PubMed Web of Science ®Google Scholar
• Villalba JM, Navarro F, Cordoba F, Coenzyme Q reductase from liver plasma membrane: purification and role in trans-plasma-membrane electron transport. Proc Natl Acad Sci USA 1995;92:4887-91
   PubMed Web of Science ®Google Scholar
• Navarro F, Villalba JM, Crane FL, A phospholipid-dependent NADH-coenzyme Q reductase from liver plasma membrane. Biochem Biophys Res Commun 1995;212:138-43
   PubMed Web of Science ®Google Scholar
• Stocker R, Bowry VW, Frei B. Ubiquinol-10 protects human low density lipoprotein more efficiently against lipid peroxidation than does alpha-tocopherol. Proc Natl Acad Sci USA 1991;88:1646-50
   PubMed Web of Science ®Google Scholar
• Walter L, Nogueira V, Leverve X, Three classes of ubiquinone analogs regulate the mitochondrial permeability transition pore through a common site. J Biol Chem 2000;275:29521-7
   PubMed Web of Science ®Google Scholar
• Martin SF, Navarro F, Forthoffer N, Neutral magnesium-dependent sphingomyelinase from liver plasma membrane: purification and inhibition by ubiquinol. J Bioenerg Biomembr 2001;33:143-53
   PubMed Web of Science ®Google Scholar
• Kaikkonen J, Nyyssönen K, Porkkala-Sarataho E, Effect of oral coenzyme Q10 supplementation on the oxidation resistance of human VLDL+LDL fraction: absorption and antioxidative properties of oil and granule-based preparations. Free Radic Biol Med 1997;22:1195-202
   PubMed Web of Science ®Google Scholar
• Santos-González M, Gómez-Díaz C, Navas P, Villalba JM. Modifications of plasma proteome in long-lived rats fed on a coenzyme Q10-supplemented diet. Exp Gerontol 2007;42:798-806
   PubMed Web of Science ®Google Scholar
• Groneberg DA, Kindermann B, Althammer M, Coenzyme Q10 affects expression of genes involved in cell signalling, metabolism and transport in human CaCo-2 cells. Int J Biochem Cell Biol 2005;37:1208-18
   PubMed Web of Science ®Google Scholar
• Schmelzer C, Kitano M, Rimbach G, Effects of ubiquinol-10 on microRNA-146a expression in vitro and in vivo. Mediators Inflamm 2009;2009:415437
   PubMedGoogle Scholar
• Turunen M, Olsson J, Dallner G. Metabolism and function of coenzyme Q. Biochim Biophys Acta 2004;1660:171-99
   PubMed Web of Science ®Google Scholar
• Quinzii CM, DiMauro S, Hirano M. Human coenzyme Q deficiencies. Neurochem Res 2007;32:723-7
   PubMed Web of Science ®Google Scholar
• Forsgren M, Attersand A, Lake S, Isolation and functional expression of human COQ2, a gene encoding a polyprenyl transferase involved in the synthesis of CoQ. Biochem J 2004;382:519-26
   PubMed Web of Science ®Google Scholar
• Casarin A, Jimenez-Ortega JC, Trevisson E, Functional characterization of human COQ4, a gene required for Coenzyme Q10 biosynthesis. Biochem Biophys Res Commun 2008;372:35-9
   PubMed Web of Science ®Google Scholar
• Tran UT, Clarke CF. Endogenous synthesis of coenzyme Q in eukaryotes. Mitochondrion 2007;7S:S62-71
   Google Scholar
• Hermán MD, Alcaín FJ, Burón MI, Subcellular distribution and biosynthesis of ubiquinone in rat liver. Biol Cell 2001;93:342
   Google Scholar
• Fernández-Ayala DJ, Brea-Calvo G, López-Lluch G, Navas P. Coenzyme Q distribution in HL-60 human cells depends on the endomembrane system. Biochim Biophys Acta 2005;1713:129-37
   PubMed Web of Science ®Google Scholar
• Åberg F, Appelkvist EL, Dallner G, Ernster L. Distribution and redox state of ubiquinones in rat and human tissues. Arch Biochem Biophys 1992;295:230-4
   PubMed Web of Science ®Google Scholar
• Runquist M, Parmryd I, Thelin A, Distribution of branch-point prenyltransferases in regions of bovine brain. J Neurochem 1995;65:2299-306
   PubMed Web of Science ®Google Scholar
• Naini A, Lewis VJ, Hirano M, DiMauro S. Primary coenzyme Q10 deficiency and the brain. Biofactors 2003;18:145-52
   PubMed Web of Science ®Google Scholar
• Elmberger PG, Kalen A, Appelkvist EL, Dallner G. In vitro and in vivo synthesis of dolichol and other main mevalonate products in various organs of the rat. Eur J Biochem 1987;168:1-11
   PubMedGoogle Scholar
• Molyneux SL, Florkowski CM, Lever M, George PM. Biological variation of coenzyme Q10. Clin Chem 2005;51:455-7
   PubMed Web of Science ®Google Scholar
• Dallner G, Sindelar PJ. Regulation of ubiquinone metabolism. Free Radic Biol Med 2000;29:285-94
   PubMed Web of Science ®Google Scholar
• Aberg F, Zhang Y, Teclebrhan H, Increases in tissue levels of ubiquinone in association with peroxisome proliferation. Chem Biol Interact 1996;99:205-18
   PubMed Web of Science ®Google Scholar
• Brea-Calvo G, Rodríguez-Hernández A, Fernández-Ayala DJ, Chemotherapy induces an increase in coenzyme Q10 levels in cancer cell lines. Free Radic Biol Med 2006;40:1293-302
   PubMed Web of Science ®Google Scholar
• Brea-Calvo G, Siendones E, Sánchez-Alcázar JA, Cell survival from chemotherapy depends on NF-kappaB transcriptional up-regulation of coenzyme Q biosynthesis. PLoS One 2009;4:e5301
   PubMed Web of Science ®Google Scholar
• Bhagavan HN, Chopra RK. Coenzyme Q10: absorption, tissue uptake, metabolism and pharmacokinetics. Free Radic Res 2006;40:445-53
   PubMed Web of Science ®Google Scholar
• Beal MF. Coenzyme Q administration and its potential for treatment of neurodegenerative diseases. Biofactors 1999;9:261-6
   PubMed Web of Science ®Google Scholar
• Weber C, Bysted A, Hølmer G. Coenzyme Q10 in the diet – daily intake and relative bioavailability. Mol Aspects Med 1997;18:S251-4
   PubMed Web of Science ®Google Scholar
• Zhang Y, Aberg F, Appelkvist EL, Uptake of dietary coenzyme Q supplement is limited in rats. J Nutr 1995;125:446-53
   PubMed Web of Science ®Google Scholar
• Bhagavan HN, Chopra RK, Craft NE, Assessment of coenzyme Q10 absorption using an in vitro digestion-Caco-2 cell model. Int J Pharm 2007;333:112-7
   PubMed Web of Science ®Google Scholar
• Ochiai A, Itagaki S, Kurokawa T, Improvement in intestinal coenzyme Q10 absorption by food intake. Yakugaku Zasshi 2007;127:1251-4
   PubMed Web of Science ®Google Scholar
• Kwong LK, Kamzalov S, Rebrin I, Effects of coenzyme Q10 administration on its tissue concentrations, mitochondrial oxidant generation, and oxidative stress in the rat. Free Radic Biol Med 2002;33:627-38
   PubMed Web of Science ®Google Scholar
• Hosoe K, Kitano M, Kishida H, Study on safety and bioavailability of ubiquinol (Kaneka QH) after single and 4-week multiple oral administration to healthy volunteers. Regul Toxicol Pharmacol 2007;47:19-28
   PubMed Web of Science ®Google Scholar
• Weis M, Mortensen SA, Rassing MR, Bioavailability of four oral coenzyme Q10. formulations in healthy volunteers. Mol Aspects Med 1994;15:s273-80
   PubMed Web of Science ®Google Scholar
• Liu ZX, Artmann C. Relative bioavailability comparison of different coenzyme Q10 formulations with a novel delivery system. Altern Ther Health Med 2009;15:42-6
   PubMed Web of Science ®Google Scholar
• Žmitek J, Šmidovnik A, Fir M, Relative bioavailability of two forms of a novel water soluble CoQ10. Ann Nutr Metab 2008;52:281-7
   PubMed Web of Science ®Google Scholar
• Kemijski Inst [SI]. Beta-cyclodextrin, process of preparing, and use thereof. EP1755683; 2007
   Google Scholar
• Nukui K, Yamagishi T, Miyawaki H, Comparison of uptake between PureSorb-Q40 and regular hydrophobic coenzyme Q10 in rats and humans after single oral intake. J Nutr Sci Vitaminol 2007;53:187-90
   PubMed Web of Science ®Google Scholar
• Ikematsu H, Nakamura K, Harashima S, Safety assessment of coenzyme Q10 (Kaneka Q10) in healthy subjects: a double-blind, randomized, placebo-controlled trial. Regul Toxicol Pharmacol 2006;44:212-8
   PubMed Web of Science ®Google Scholar
• Nakamura T, Ohno T, Hamamura K, Sato T. Metabolism of coenzyme Q10: biliary and urinary excretion study in guinea pigs. Biofactors 1999;9:111-9
   PubMed Web of Science ®Google Scholar
• Palamakula A, Khan MA. Evaluation of cytotoxicity of oils used in coenzyme Q10 Self-Emulsifying Drug Delivery Systems (SEDDS). Int J Pharm 2004;273:63-73
   PubMed Web of Science ®Google Scholar
• Sigma Tau Healthscience Spa. Ubiquinone-containing composition suitable for promoting enhanced intramitochondrial transportation of ubiquinones and methods of using same. WO0023069; 2000
   Google Scholar
• Miles MV, Horn P, Miles L, Bioequivalence of coenzyme Q10 from over-the-counter supplements. Nutr Res 2002;22:919-29
   Web of Science ®Google Scholar
• Ingredient Innovations Interna. Stable aqueous dispersion of nutrients. US6455072; 2002
   Google Scholar
• Chopra RK. Reduced form of Coenzyme Q in high bioavailability stable oral dosage form. US6740338; 2004
   Google Scholar
• Biosytes USA, Inc. Method for enhancing dissolution properties of relatively insoluble dietary supplements and product incorporating same. US6056971; 2000
   Google Scholar
• Molyneux S, Florkowski C, Lever M, George P. The bioavailability of coenzyme Q10 supplements available in New Zealand differs markedly. NZ Med J 2004;117:U1108
   PubMedGoogle Scholar
• Nisshin Pharma Inc. Coenzyme Q10-containing water-soluble compositions. WO2007080787; 2007
   Google Scholar
• Vesifact AG. Conzyme Q10 containing microemulsion preconcentrates and microemulsions. HK1068806; 2007
   Google Scholar
• Wajda R, Zirkel J, Schaffer T. Increase of bioavailability of coenzyme Q10 and vitamin E. J Med Food 2007;10:731-4
   PubMed Web of Science ®Google Scholar
• Lipoid Gmbh Emulsive water-soluble concentrates. KR20050096919; 2005
   Google Scholar
• Constantinescu R, McDermott MP, Dicenzo R, A randomized study of the bioavailability of different formulations of coenzyme Q10 (ubiquinone). J Clin Pharmacol 2007;47:1580-6
   PubMed Web of Science ®Google Scholar
• Hidaka T, Fujii K, Funahashi I, Safety assesment of coenzyme Q10 (CoQ). Biofactors 2008;32:199-208
   PubMed Web of Science ®Google Scholar
• Kitano M, Mizuhashi F, Kubo H, Evaluation of the mutagenic and genotoxic potential of ubiquinol. Int J Toxicol 2007;26:533-44
   PubMed Web of Science ®Google Scholar
• Sumien N, Heinrich KR, Shetty RA, Prolonged intake of coenzyme Q10 impairs cognitive functions in mice. J Nutr 2009;139:1926-32
   PubMed Web of Science ®Google Scholar
• Boitier E, Degoul F, Desguerre I, A case of mitochondrial encephalomyopathy associated with a muscle coenzyme Q10 deficiency. J Neurol Sci 1998;156:41-6
   PubMed Web of Science ®Google Scholar
• Rötig A, Appelkvist EL, Geromel V, Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency. Lancet 2000;356:391-5
   PubMed Web of Science ®Google Scholar
• Lamperti C, Naine A, Hirano M, Cerebellar ataxia and coenzyme Q10 deficiency. Neurology 2003;60:1206-8
   PubMed Web of Science ®Google Scholar
• Ogasahara S, Engel AG, Frens D, Mack D. Muscle coenzyme Q deficiency in familial mitochondrial encephalomyopathy. Proc Natl Acad Sci USA 1989;86:2379-82
   PubMed Web of Science ®Google Scholar
• DiMauro S, Quinzii CM, Hirano M. Mutations in coenzyme Q10 biosynthetic genes. J Clin Invest 2007;117:587-9
   PubMed Web of Science ®Google Scholar
• Quinzii C, Naini A, Salviati L, A mutation in para-hydroxybenzoate-polyprenyl transferase (COQ2) causes primary coenzyme Q10 deficiency. Am J Hum Genet 2006;78:345-9
   PubMed Web of Science ®Google Scholar
• López LC, Schuelke M, Quinzii CM, Leigh syndrome with nephropathy and CoQ10 deficiency due to decaprenyl diphosphate synthase subunit 2 (PDSS2) mutations. Am J Hum Genet 2006;79:1125-9
   PubMed Web of Science ®Google Scholar
• Mollet J, Giurgea I, Schlemmer D, Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders. J Clin Invest 2007;117:765-72
   PubMed Web of Science ®Google Scholar
• Lagier-Tourenne C, Tazir M, López LC, ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q10 deficiency. Am J Hum Genet 2008;82:661-72
   PubMed Web of Science ®Google Scholar
• Mollet J, Delahodde A, Serre V, CABC1 gene mutations cause ubiquinone deficiency with cerebellar ataxia and seizures. Am J Hum Genet 2008;82:623-30
   PubMed Web of Science ®Google Scholar
• Duncan AJ, Bitner-Glindzicz M, Meunier B, A nonsense mutation in COQ9 causes autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency: a potentially treatable form of mitochondrial disease. Am J Hum Genet 2009;84:558-66
   PubMed Web of Science ®Google Scholar
• Quinzii CM, López LC, Von-Moltke J, Respiratory chain dysfunction and oxidative stress correlate with severity of primary CoQ10 deficiency. FASEB J 2008;22:1874-85
   PubMed Web of Science ®Google Scholar
• Lopez-Martin JM, Salviati L, Trevisson E, Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis. Hum Mol Genet 2007;16:1091-7
   PubMed Web of Science ®Google Scholar
• Quinzii CM, Kattah AG, Naini A, Coenzyme Q deficiency and cerebellar ataxia associated with an aprataxin mutation. Neurology 2005;64:539-41
   PubMed Web of Science ®Google Scholar
• Gempel K, Topaloglu H, Talim B, The myopathic form of coenzyme Q10 deficiency is caused by mutations in the electron-transferring flavoprotein dehydrogenase (ETFDH) gene. Brain 2007;130:2037-44
   PubMed Web of Science ®Google Scholar
• Cooper JM, Korlipara LV, Hart PE, Coenzyme Q10 and vitamin E deficiency in Friedreich's ataxia: predictor of efficacy of vitamin E and coenzyme Q10 therapy. Eur J Neurol 2008;15:1371-9
   PubMed Web of Science ®Google Scholar
• Marcoff L, Thompson PD. The role of coenzyme Q10 in statin-associated myopathy. J Am Coll Cardiol 2007;49:2231-7
   PubMed Web of Science ®Google Scholar
• Ghirlanda G, Oradei A, Manto A, Evidence of plasma CoQ10-lowering effect by HMG-CoA reductase inhibitors: a double-blind, placebo-controlled study. J Clin Pharmacol 1993;33:226-9
   PubMed Web of Science ®Google Scholar
• Berthold HK, Naini A, Di Mauro S, Effect of ezetimibe and/or simvastatin on coenzyme Q10 levels in plasma: a randomised trial. Drug Saf 2006;29:703-12
   PubMed Web of Science ®Google Scholar
• Kalén A, Appelkvist EL, Dallner G. Age-related changes in the lipid compositions of rat and human tissues. Lipids 1989;24:579-84
   PubMed Web of Science ®Google Scholar
• Miles MV, Horn PS, Tang PH, Age-related changes in plasma coenzyme Q10 concentrations and redox state in apparently healthy children and adults. Clin Chim Acta 2004;347:139-44
   PubMed Web of Science ®Google Scholar
• Wada H, Goto H, Hagiwara S, Yamamoto Y. Redox status of coenzyme Q10 is associated with chronological age. J Am Geriatr Soc 2007;55:1141-2
   PubMed Web of Science ®Google Scholar
• Sohal RJ, Forster MJ. Coenzyme Q, oxidative stress and aging. Mitochondrion 2007;7S:S103-11
   Google Scholar
• Folkers K, Osterborg A, Nylander M, Activities of vitamin Q10 in animal models and a serious deficiency in patients with cancer. Biochem Biophys Res Commun 1997;234:296-9
   PubMed Web of Science ®Google Scholar
• Mano T, Iwase K, Hayashi R, Vitamin E and coenzyme Q concentrations in the thyroid tissues of patients with various thyroid disorders. Am J Med Sci 1998;315:230-2
   PubMed Web of Science ®Google Scholar
• Portakal O, Ozkaya O, Erden Inal M, Coenzyme Q10 concentrations and antioxidant status in tissues of breast cancer patients. Clin Biochem 2000;33:279-84
   PubMed Web of Science ®Google Scholar
• Jolliet P, Simon N, Barré J, Plasma coenzyme Q10 concentrations in breast cancer: prognosis and therapeutic consequences. Int J Clin Pharmacol Ther 1998;36:506-9
   PubMed Web of Science ®Google Scholar
• Rusciani L, Proietti I, Paradisi A, Recombinant interferon alpha-2b and coenzyme Q10 as a postsurgical adjuvant therapy for melanoma: a 3-year trial with recombinant interferon-alpha and 5-year follow-up. Melanoma Res 2007;17:177-83
   PubMed Web of Science ®Google Scholar
• Yamamoto Y, Yamashita S, Fujisawa A, Oxidative stress in patients with hepatitis, cirrhosis, and hepatoma evaluated by plasma antioxidants. Biochem Biophys Res Commun 1998;247:166-70
   PubMed Web of Science ®Google Scholar
• Salviati L, Sacconi S, Murer L, Infantile encephalomyopathy and nephropathy with CoQ10 deficiency: a CoQ10-responsive condition. Neurology 2005;65:606-8
   PubMed Web of Science ®Google Scholar
• Artuch R, Brea-Calvo G, Briones P, Cerebellar ataxia with coenzyme Q10 deficiency: diagnosis and follow-up after coenzyme Q10 supplementation. J Neurol Sci 2006;246:153-8
   PubMed Web of Science ®Google Scholar
• Di Giovanni S, Mirabella M, Spinazzola A, Coenzyme Q10 reverses pathological phenotype and reduces apoptosis in familial CoQ10 deficiency. Neurology 2001;57:375-6
   PubMedGoogle Scholar
• Mancuso M, Orsucci D, Volpi L, Coenzyme Q10 in neuromuscular and neurodegenerative disorders. Curr Drug Targets 2010;11:111-21
   PubMed Web of Science ®Google Scholar
• Young AJ, Johnson S, Steffens DC, Coenzyme Q10: a review of its promise as a neuroprotectant. CNS Spectr 2007;12:62-8
   PubMed Web of Science ®Google Scholar
• Laxdale Ltd. Coenzyme Q and eicosapentaenoic acid (EPA). NZ529339; 2004
   Google Scholar
• US Dept Veterans Affairs, Method of ameliorating or abrogating the effects of a neurodegenerative disorder, such as Huntington's disease, by using coenzyme Q10. US2006078549; 2006
   Google Scholar
• US Dept of Veterans Affairs, Method of ameliorating or abrogating the effects of a neurodegenerative disorder, such as Huntington's disease, by sodium butyrate chemotherapy. US2006069157; 2006
   Google Scholar
• Kanegafuuchi Chem Ind. agent for improving nervous system cell functions. EP2005951; 2008
   Google Scholar
• Kunming Xianghao Tec Co [CN]. Lycopene and coenzyme Q10 containing formulation and its manufacturing method. CN1634075; 2005
   Google Scholar
• Delanty N, Dichter MA. Oxidative injury in the nervous system. Acta Neurol Scand 1998;98:145-53
   PubMed Web of Science ®Google Scholar
• Feigin A, Kieburtz K, Como P, Assessment of coenzyme Q10 tolerability in Huntington's disease. Mov Disord 1996;11:321-3
   PubMed Web of Science ®Google Scholar
• Koroshetz WJ, Jenkins BG, Rosen BR, Beal MF. Energy metabolism defects in Huntington's disease and effects of coenzyme Q10. Ann Neurol 1997;41:160-5
   PubMed Web of Science ®Google Scholar
• Ferrante RJ, Andreassen OA, Dedeoglu A, Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease. J Neurosci 2002;22:1592-9
   PubMed Web of Science ®Google Scholar
• Yang L, Calingasan NY, Wille EJ, Combination therapy with coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson's and Huntington's diseases. J Neurochem 2009;109:1427-39
   PubMed Web of Science ®Google Scholar
• Huntington Study Group. A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease. Neurology 2001;57:397-404
   PubMed Web of Science ®Google Scholar
• Aylward EH, Rosenblatt A, Field K, Caudate volume as an outcome measure in clinical trials for Huntington's disease: a pilot study. Brain Res Bull 2003;62:137-41
   PubMed Web of Science ®Google Scholar
• Shults CW. Therapeutic role of coenzyme Q10 in Parkinson's disease. Pharmacol Ther 2005;107:120-30
   PubMed Web of Science ®Google Scholar
• Sohmiya M, Tanaka M, Tak NW, Redox status of plasma coenzyme Q10 indicates elevated systemic oxidative stress in Parkinson's disease. J Neurol Sci 2004;223:161-6
   PubMed Web of Science ®Google Scholar
• Götz ME, Gerstner A, Harth R, Altered redox state of platelet coenzyme Q10 in Parkinson's disease. J Neural Transm 2000;107:41-8
   PubMed Web of Science ®Google Scholar
• Samii A, Carleton BC, Etminan M. Statin use and the risk of Parkinson disease: a nested case control study. J Clin Neurosci 2008;15:1272-3
   PubMed Web of Science ®Google Scholar
• Cleren C, Yang L, Lorenzo B, Therapeutic effects of coenzyme Q10 (CoQ10) and reduced CoQ10 in the MPTP model of Parkinsonism. J Neurochem 2008;104:1613-21
   PubMed Web of Science ®Google Scholar
• Shults CW, Oakes D, Kieburtz K, Effects of coenzyme Q10 in early Parkinson disease: evidence of slowing of the functional decline. Arch Neurol 2002;59:1541-50
   PubMed Web of Science ®Google Scholar
• Müller T, Büttner T, Gholipour AF, Kuhn W. Coenzyme Q10 supplementation provides mild symptomatic benefit in patients with Parkinson's disease. Neurosci Lett 2003;341:201-4
   PubMed Web of Science ®Google Scholar
• Shults CW, Flint Beal M, Song D, Fontaine D. Pilot trial of high dosages of coenzyme Q10 in patients with Parkinson's disease. Exp Neurol 2004;188:491-4
   PubMed Web of Science ®Google Scholar
• MSE Pharmazeutica Gmb. Particles with modified physicochemical properties, their preparation and uses. EP0711151; 1996
   Google Scholar
• Storch A, Jost WH, Vieregge P, Randomized, double-blind, placebo-controlled trial on symptomatic effects of coenzyme Q10 in Parkinson disease. Arch Neurol 2007;64:938-44
   PubMed Web of Science ®Google Scholar
• Stamelou M, Reuss A, Pilatus U, Short-term effects of coenzyme Q10 in progressive supranuclear palsy: a randomized, placebo-controlled trial. Mov Disord 2008;23:942-9
   PubMed Web of Science ®Google Scholar
• Wadsworth TL, Bishop JA, Pappu AS, Evaluation of coenzyme Q as an antioxidant strategy for Alzheimer's disease. J Alzheimers Dis 2008;14:225-34
   PubMed Web of Science ®Google Scholar
• Molina JA, de Bustos F, Jiménez-Jiménez FJ, Serum levels of coenzyme Q10 in patients with amyotrophic lateral sclerosis. J Neural Transm 2000;107:1021-6
   PubMed Web of Science ®Google Scholar
• Sohmiya M, Tanaka M, Suzuki Y, An increase of oxidized coenzyme Q10 occurs in the plasma of sporadic ALS patients. J Neurol Sci 2005;228:49-53
   PubMed Web of Science ®Google Scholar
• Ferrante KL, Shefner J, Zhang H, Tolerance of high-dose (3,000 mg/day) coenzyme Q10 in ALS. Neurology 2005;65:1834-6
   PubMed Web of Science ®Google Scholar
• Traynor BJ, Bruijn L, Conwit R, Neuroprotective agents for clinical trials in ALS: a systematic assessment. Neurology 2006;67:20-7
   PubMed Web of Science ®Google Scholar
• Kaufmann P, Thompson JL, Levy G, Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III. Ann Neurol 2009;66:235-44
   PubMed Web of Science ®Google Scholar
• Siddiqi SA, Thompson PD. How do you treat patients with myalgia who take statins? Curr Atheroscler Rep 2009;11:9-14
   PubMed Web of Science ®Google Scholar
• Lamperti C, Naini AB, Lucchini V, Muscle coenzyme Q10 level in statin-related myopathy. Arch Neurol 2005;62:1709-12
   PubMed Web of Science ®Google Scholar
• Päivä H, Thelen KM, Van Coster R, High-dose statins and skeletal muscle metabolism in humans: a randomized, controlled trial. Clin Pharmacol Ther 2005;78:60-8
   PubMed Web of Science ®Google Scholar
• Schick BA, Laaksonen R, Frohlich JJ, Decreased skeletal muscle mitochondrial DNA in patients treated with high-dose simvastatin. Clin Pharmacol Ther 2007;81:650-3
   PubMed Web of Science ®Google Scholar
• Kucharská J, Gvozdjáková A, Simko F. Simvastatin decreased coenzyme Q in the left ventricle and skeletal muscle but not in the brain and liver in L-NAME-induced hypertension. Physiol Res 2007;56:S49-54
   PubMed Web of Science ®Google Scholar
• Merck & Co Inc. Coenzyme Q10 with HMG-CoA reductase inhibitors. US4929437; 1990
   Google Scholar
• Merck & Co Inc. Coenzyme Q10 with HMG-CoA reductase inhibitors. US4933165; 1990
   Google Scholar
• Young JM, Florkowski CM, Molyneux SL, Effect of coenzyme Q10 supplementation on simvastatin-induced myalgia. Am J Cardiol 2007;100:1400-3
   PubMed Web of Science ®Google Scholar
• Reidenberg MM. Statins, lack of energy and ubiquinone. Br J Clin Pharmacol 2005;59:606-7
   PubMed Web of Science ®Google Scholar
• Kanegafushi Chem Ind. Anti-fatigue composition. EP1897539; 2008
   Google Scholar
• Folkers K, Simonsen R. Two successful double-blind trials with coenzyme Q10 (vitamin Q10) on muscular dystrophies and neurogenic atrophies. Biochim Biophys Acta 1995;1271:281-6
   PubMed Web of Science ®Google Scholar
• Tedeschi D, Lombardi V, Mancuso M, Potential involvement of ubiquinone in myotonic dystrophy pathophysiology: new diagnostic approaches for new rationale therapeutics. Neurol Sci 2000;21:S979-80
   PubMed Web of Science ®Google Scholar
• Miles L, Miles MV, Tang PH, Muscle coenzyme Q: a potential test for mitochondrial activity and redox status. Pediatr Neurol 2005;32:318-24
   PubMed Web of Science ®Google Scholar
• Nisshin Pharma Inc (JP). Therapeutics for muscular dystrophy and therapeutic foods therefor. WO2004075908; 2004
   Google Scholar
• Folkers K, Vadhanavikit S, Mortensen SA. Biochemical rationale and myocardial tissue data on the effective therapy of cardiomyopathy with coenzyme Q10. Proc Natl Acad Sci USA 1985;82:901-4
   PubMed Web of Science ®Google Scholar
• Langsjoen PH, Langsjoen A, Willis R, Folkers K. Treatment of hypertrophic cardiomyopathy with coenzyme Q10. Mol Aspects Med 1997;18:S145-51
   PubMed Web of Science ®Google Scholar
• Univ Texas. Restoration of impaired cardiac function of patients with diverse muscular dystrophies by therapy with coenzyme Q10. US4885167; 1989
   Google Scholar
• Lifepharma Srl. Pharmaceutical or dietary composition having vigorous antioxidant activity in cardiovascular and/or liver diseases. IT1255230; 1995
   Google Scholar
• Molyneux SL, Florkowski CM, George, Coenzyme Q10: an independent predictor of mortality in chronic heart failure. J Am Coll Cardiol 2008;52:1435-41
   PubMed Web of Science ®Google Scholar
• Pepe S, Marasco SF, Haas SJ, Coenzyme Q10 in cardiovascular disease. Mitochondrion 2007:S154-67
   Google Scholar
• Kumar A, Kaur H, Devi P, Mohan V. Role of coenzyme Q10 (CoQ10) in cardiac disease, hypertension and Meniere-like syndrome. Pharmacol Ther 2009;124(3):259-68
   PubMed Web of Science ®Google Scholar
• Langsjoen PH, Langsjoen AM. Supplemental ubiquinol in patients with advanced congestive heart failure. Biofactors 2008;32:119-28
   PubMed Web of Science ®Google Scholar
• Hadj A, Esmore D, Rowland M, Pre-operative preparation for cardiac surgery utilising a combination of metabolic, physical and mental therapy. Heart Lung Circ 2006;15:172-81
   PubMedGoogle Scholar
• Pepe S, Leong JY, Van der Merwe J, Targeting oxidative stress in surgery: effects of ageing and therapy. Exp Gerontol 2008;43:653-7
   PubMed Web of Science ®Google Scholar
• Makhija N, Sendasgupta C, Kiran U, The role of oral coenzyme Q10 in patients undergoing coronary artery bypass graft surgery. J Cardiothorac Vasc Anesth 2008;22:832-9
   PubMed Web of Science ®Google Scholar
• Hodgson JM, Watts GF, Playford DA, Coenzyme Q10 improves blood pressure and glycaemic control: a controlled trial in subjects with type 2 diabetes. Eur J Clin Nutr 2002;56:1137-42
   PubMed Web of Science ®Google Scholar
• Playford DA, Watts GF, Croft KD, Burke V. Combined effect of coenzyme Q10 and fenofibrate on forearm microcirculatory function in type 2 diabetes. Atherosclerosis 2003;168:169-79
   PubMed Web of Science ®Google Scholar
• Watts GF, Playford DA, Croft KD, Coenzyme Q(10) improves endothelial dysfunction of the brachial artery in type II diabetes mellitus. Diabetologia 2002;45:420-6
   PubMed Web of Science ®Google Scholar
• Chew GT, Watts GF, Davis TM, Hemodynamic effects of fenofibrate and coenzyme Q10 in type 2 diabetic subjects with left ventricular diastolic dysfunction. Diabetes Care 2008;31:1502-9
   PubMed Web of Science ®Google Scholar
• Hamilton SJ, Chew GT, Watts GF. Coenzyme Q10 improves endothelial dysfunction in statin-treated type 2 diabetic patients. Diabetes Care 2009;32:810-2
   PubMed Web of Science ®Google Scholar
• Tiano L, Belardinelli R, Carnevali P, Effect of coenzyme Q10 administration on endothelial function and extracellular superoxide dismutase in patients with ischaemic heart disease: a double-blind, randomized controlled study. Eur Heart J 2007;28:2249-55
   PubMed Web of Science ®Google Scholar
• Belardinelli R, Tiano L, Littarru GP. Oxidative stress, endothelial function and coenzyme Q10. Biofactors 2008;32:129-33
   PubMed Web of Science ®Google Scholar
• Seuref AG. Pharmaceutical compositions having vasodilating and antianoxic activities. US4778798; 1988
   Google Scholar
• Univ Leland Stanford Junior. Enhancement of vascular function by modulation of endogenous nitric oxide production or activity. US2006009407; 2006
   Google Scholar
• Tsuneki H, Sekizaki N, Suzuki T, Coenzyme Q10 prevents high glucose-induced oxidative stress in human umbilical vein endothelial cells. Eur J Pharmacol 2007;566:1-10
   PubMed Web of Science ®Google Scholar
• Roffe L, Schmidt K, Ernst E. Efficacy of coenzyme Q10 for improved tolerability of cancer treatments: a systematic review. J Clin Oncol 2004;22:4418-24
   PubMed Web of Science ®Google Scholar
• Premkumar VG, Yuvaraj S, Vijayasarathy K, Effect of coenzyme Q10, riboflavin and niacin on serum CEA and CA 15-3 levels in breast cancer patients undergoing tamoxifen therapy. Biol Pharm Bull 2007;30:367-70
   PubMed Web of Science ®Google Scholar
• Premkumar VG, Yuvaraj S, Vijayasarathy K, Serum cytokine levels of interleukin-1beta, -6, -8, tumour necrosis factor-alpha and vascular endothelial growth factor in breast cancer patients treated with tamoxifen and supplemented with co-enzyme Q10, riboflavin and niacin. Basic Clin Pharmacol Toxicol 2007;100:387-91
   PubMed Web of Science ®Google Scholar
• Premkumar VG, Yuvaraj S, Shanthi P, Co-enzyme Q10, riboflavin and niacin supplementation on alteration of DNA repair enzyme and DNA methylation in breast cancer patients undergoing tamoxifen therapy. Br J Nutr 2008;100:1179-82
   PubMed Web of Science ®Google Scholar
• Premkumar VG, Yuvaraj S, Sathish S, Anti-angiogenic potential of coenzymeQ10, riboflavin and niacin in breast cancer patients undergoing tamoxifen therapy. Vascul Pharmacol 2008;48:191-201
   PubMed Web of Science ®Google Scholar
• Hoenjet KM, Dagnelie PC, Delaere KP, Effect of a nutritional supplement containing vitamin E, selenium, vitamin C and coenzyme Q10 on serum PSA in patients with hormonally untreated carcinoma of the prostate: a randomised placebo-controlled study. Eur Urol 2005;47:43343-9
   Google Scholar
• Singh RP, Kumar R, Kapur N. Molecular regulation of cholesterol biosynthesis: implications in carcinogenesis. J Environ Pathol Toxicol Oncol 2003;22:75-92
   PubMedGoogle Scholar
• Kaneka Corp (JP), Japan Government (JP). Composition for prevention of cancer. EP1908459; 2008
   Google Scholar
• Kaneka Corp (JP). Method for cancer treatment, carcinogenesis suppression or mitigation of adverse reactions of anticancer agents. US2008138326; 2008
   Google Scholar
• Kucharská J, Braunová Z, Ulicná O, Deficit of coenzyme Q in heart and liver mitochondria of rats with streptozotocin-induced diabetes. Physiol Res 2000;49:411-8
   PubMed Web of Science ®Google Scholar
• Menke T, Niklowitz P, Wiesel T, Andler W. Antioxidant level and redox status of coenzyme Q10 in the plasma and blood cells of children with diabetes mellitus type 1. Pediatr Diabetes 2008;9:540-5
   PubMed Web of Science ®Google Scholar
• Miles MV, Morrison JA, Horn PS, Coenzyme Q10 changes are associated with metabolic syndrome. Clin Chim Acta 2004;344:173-9
   PubMed Web of Science ®Google Scholar
• Fujii K, Kawabe T, Hosoe K, Compositions for diabetes. US2005113459; 2005
   Google Scholar
• Huaibei Huike Pharmaceutical. The compound preparation for treating diabetes mellitus. WO2006102804; 2006
   Google Scholar
• Response Scient Inc. Medical food or nutritional supplement, method of manufacturing same, and method of managing diabetes. US2009053303; 2009
   Google Scholar
• Mancini A, Conte B, De Marinis L, Coenzyme Q10 levels in human seminal fluid: diagnostic and clinical implications. Mol Aspects Med 1994;15:s249-55
   PubMed Web of Science ®Google Scholar
• Balercia G, Arnaldi G, Fazioli F, Coenzyme Q10 levels in idiopathic and varicocele-associated asthenozoospermia. Andrologia 2002;34:107-11
   PubMed Web of Science ®Google Scholar
• Balercia G, Buldreghini E, Vignini A, Coenzyme Q10 treatment in infertile men with idiopathic asthenozoospermia: a placebo-controlled, double-blind randomized trial. Fertil Steril 2009;91:1785-92
   PubMed Web of Science ®Google Scholar
• Lewin A, Lavon H. The effect of coenzyme Q10 on sperm motility and function. Mol Aspects Med 1997;18:S213-9
   PubMed Web of Science ®Google Scholar
• Littarru G, Balercia G. Method for treating and preventing male infertility. US2006154993; 2006
   Google Scholar
• Miles MV, Patterson BJ, Chalfonte-Evans ML, Coenzyme Q10 (ubiquinol-10) supplementation improves oxidative imbalance in children with trisomy 21. Pediatr Neurol 2007;37:398-403
   PubMed Web of Science ®Google Scholar
• Tiano L, Padella L, Carnevali P, Coenzyme Q10 and oxidative imbalance in Down syndrome: biochemical and clinical aspects. Biofactors 2008;32:161-7
   PubMed Web of Science ®Google Scholar
• Takeda Chem Ind Ltd. Cerebral circulatory metabolism-improving agent. JP1221316; 1989
   Google Scholar
• Geromel V, Darin N, Chrétien D, Coenzyme Q10 and idebenone in the therapy of respiratory chain diseases: rationale and comparative benefits. Mol Genet Metab 2002;77:21-30
   PubMed Web of Science ®Google Scholar
• Bodmer M, Vankan P, Dreier M. Pharmacokinetics and metabolism of idebenone in healthy male subjects. Eur J Clin Pharmacol 2009;65:493-501
   PubMed Web of Science ®Google Scholar
• Kutz K, Drewe J, Vankan P. Pharmacokinetic properties and metabolism of idebenone. J Neurol 2009;256:31-5
   PubMed Web of Science ®Google Scholar
• Torii H, Yoshida K, Kobayashi T. Disposition of idebenone (CV-2619), a new cerebral metabolism improving agent, in rats and dogs. J Pharmacobiodyn 1985;8:457-67
   PubMedGoogle Scholar
• Haginoya K, Miyabayashi S, Kikuchi M, Efficacy of idebenone for respiratory failure in a patient with Leigh syndrome: a long-term follow-up study. J Neurol Sci 2009;278:112-4
   PubMed Web of Science ®Google Scholar
• Ikejiri Y, Mori E, Ishii K, Idebenone improves cerebral mitochondrial oxidative metabolism in a patient with MELAS. Neurology 1996;47:583-5
   PubMed Web of Science ®Google Scholar
• Napolitano A, Salvetti S, Vista M, Long-term treatment with idebenone and riboflavin in a patient with MELAS. Neurol Sci 2000;21:S981-2
   PubMed Web of Science ®Google Scholar
• Mashima Y, Kigasawa K, Wakakura M, Oguchi Y. Do idebenone and vitamin therapy shorten the time to achieve visual recovery in Leber hereditary optic neuropathy? J Neuroophthalmol 2000;20:166-70
   PubMed Web of Science ®Google Scholar
• Carelli V, Valentino ML, Liguori R, Leber's hereditary optic neuropathy (LHON/11778) with myoclonus: report of two cases. J Neurol Neurosurg Psychiatry 2001;71:813-6
   PubMed Web of Science ®Google Scholar
• Barnils N, Mesa E, Muñoz S, Response to idebenone and multivitamin therapy in Leber's hereditary optic neuropathy. Arch Soc Esp Oftalmol 2007;82:377-80
   PubMedGoogle Scholar
• Hirai K, Hayako H, Kato K, Miyamoto M. Idebenone protects hippocampal neurons against amyloid beta-peptide-induced neurotoxicity in rat primary cultures. Naunyn Schmiedebergs Arch Pharmacol 1998;358:582-5
   PubMed Web of Science ®Google Scholar
• Takeda Chem Ind. Use of idebenone and analogues against beta amyloid induced cytotoxicity. US6271266; 2001
   Google Scholar
• Takeda Chem Ind. Treating and preventing agent for Alzheimer's disease. JP10259126; 1998
   Google Scholar
• Takeda Chem Ind. Idebenone compositions for treating Alzheimer's disease. EP0629400; 2000
   Google Scholar
• Weyer G, Babej-Dölle RM, Hadler D, A controlled study of 2 doses of idebenone in the treatment of Alzheimer's disease. Neuropsychobiology 1997;36:73-82
   PubMed Web of Science ®Google Scholar
• Gutzmann H, Hadler D. Sustained efficacy and safety of idebenone in the treatment of Alzheimer's disease: update on a 2-year double-blind multicentre study. J Neural Transm Suppl 1998;54:301-10
   PubMedGoogle Scholar
• Thal LJ, Grundman M, Berg J, Idebenone treatment fails to slow cognitive decline in Alzheimer's disease. Neurology 2003;61:1498-502
   PubMed Web of Science ®Google Scholar
• Tonon C, Lodi R. Idebenone in Friedreich's ataxia. Expert Opin Pharmacother 2008;9:2327-37
   PubMed Web of Science ®Google Scholar
• Dürr A, Cossee M, Agid Y. Clinical and genetic abnormalities in patients with Friedreich's ataxia. N Engl J Med 1996;335:1169-75
   PubMed Web of Science ®Google Scholar
• Rötig A, de Lonlay P, Chretien D. Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia. Nat Genet 1997;17:215-17
   PubMed Web of Science ®Google Scholar
• Meier T, Buyse G. Idebenone: an emerging therapy for Friedreich ataxia. J Neurol 2009;256:25-30
   PubMed Web of Science ®Google Scholar
• Inst. Nat. Sante Rech Med. (FR). Quinone derivatives for treating or preventing diseases associated with iron overload. US6133322; 2000
   Google Scholar
• Myocontract Ltd. A screening method and compounds for treating Friedreich ataxia. WO2004003565; 2004
   Google Scholar
• Di Prospero NA, Baker A, Jeffries N, Fischbeck KH. Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a randomised, placebo-controlled trial. Lancet Neurol 2007;6:878-86
   PubMed Web of Science ®Google Scholar
• Pineda M, Arpa J, Montero R, Idebenone treatment in paediatric and adult patients with Friedreich ataxia: long-term follow-up. Eur J Paediatr Neurol 2008;12:470-5
   PubMed Web of Science ®Google Scholar
• Rinaldi C, Tucci T, Maione S, Low-dose idebenone treatment in Friedreich's ataxia with and without cardiac hypertrophy. J Neurol 2009;256:1434-7
   PubMed Web of Science ®Google Scholar
• Jauslin ML, Meier T, Smith RA, Murphy MP. Mitochondria-targeted antioxidants protect Friedreich Ataxia fibroblasts from endogenous oxidative stress more effectively than untargeted antioxidants. FASEB J 2003;17:1972-84
   PubMed Web of Science ®Google Scholar
• Murphy MP, Smith RA. Targeting antioxidants to mitochondria by conjugation to lipophilic cations. Annu Rev Pharmacol Toxicol 2007;47:629-56
   PubMed Web of Science ®Google Scholar
• Univ Otago, New Zeeland. Mitochondrially targeted antioxidants. US2004106579; 2004
   Google Scholar
• Cochemé HM, Kelso GF, James AM, Mitochondrial targeting of quinones: therapeutic implications. Mitochondrion 2007;7S:S94-102
   Google Scholar
• Tauskela JS. MitoQ – a mitochondria-targeted antioxidant. IDrugs 2007;10:399-412
   PubMed Web of Science ®Google Scholar
• Snow BJ, Rolfe FL, Murphy MP. Phase II study of the mitochondrial antioxidant mitoquinone for Parkinson's disease. Proceedings of 60th American Academy of Neurology Annual Meeting; 12 – 19 April 2008; Chicago
   Google Scholar