Coenzyme Q10 as a therapy for mitochondrial disease

International Journal of Biochemistry and Cell Biology

Volumn 49, Issue 1, 2014, Pages 105-111

  Hargreaves, I P ^a  

^a National Hosptal   (United Kingdom)

Author keywords

Coenzyme Q10; EPI 743; Idebenone; Mitochondrial respiratory chain; Oxidative stress

Indexed keywords

UBIDECARENONE; ALPHA-TOCOTRIENOL QUINONE; IDEBENONE; UBIQUINONE;

ARTICLE; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ELECTRON TRANSPORT; ENCEPHALOMYOPATHY; ENZYME DEFICIENCY; ENZYME SYNTHESIS; HUMAN; NONHUMAN; OXIDATIVE STRESS; RESPIRATORY CHAIN; ANALOGS AND DERIVATIVES; ANIMAL; ATAXIA; CHEMICAL STRUCTURE; CHEMISTRY; DEFICIENCY; MITOCHONDRIAL DISEASES; MUSCLE WEAKNESS; TREATMENT OUTCOME;

ANIMALS; ATAXIA; HUMANS; MITOCHONDRIAL DISEASES; MOLECULAR STRUCTURE; MUSCLE WEAKNESS; TREATMENT OUTCOME; UBIQUINONE;

EID: 84894492310     PISSN: 13572725     EISSN: 18785875     Source Type: Journal    
DOI: 10.1016/j.biocel.2014.01.020     Document Type: Short Survey

References (87)

1. 10 in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): evaluation by noninvasive tissue oximetry J Neurol Sci 162 1999 65 68 (Pubitemid 29064120)
2. F. Aberg, E.L. Applelvist, G. Dallner, and L. Ernster Distribution and redox state of ubiquinones in rat and human tissues Arch Biochem Biophys 295 1992 230 234
3. Z. Argov, W.J. Bank, J. Maris, S. Eleff, N.G. Kennaway, and R.E. Olson et al. Treatment of mitochondrial myopathy due to complex III deficiency with vitamins KE3 and C: a 31P NMR follow-up study Ann Neurol 19 1986 598 602 (Pubitemid 16085197)
4. C. Asencio, J.C. Rodriguez-Aguilera, Ruiz-Ferrer, J. Vela, and P. Navas Silencing of ubiquinone biosynthesis genes extends life span in Caenorhabditis elegans FASEB J 17 2003 1135 1137
5. 10 deficiency Neurology 63 2004 727 729 (Pubitemid 39100837)
6. B. Barbiroli, S. Lotti, and R. Lodi Improved brain and muscle mitochondrial respiration with CoQ. An in vivo study by 31P-MR spectroscopy in patients with mitochondrial cytopathies Biofactors 9 1999 253 260 (Pubitemid 29289004)
7. G. Benard, B. Faustin, E. Passerieux, A. Galinier, C. Rocher, and N. Bellance Physiological diversity of mitochondrial oxidative phosphorylation Am J Physiol Cell Physiol 29 2006 1172 1182
8. D. Bendahan, C. Desnuelle, D. Vanuxem, S. Confort-Gouny, D. Figarella-Branger, and J.F. Pellissier et al. 31P NMR spectroscopy and ergometer exercise test as evidence for muscle oxidative performance improvement with coenzyme Q in mitochondrial myopathies Neurology 42 1992 1203 1208
9. M. Bentinger, K. Brismar, and G. Dallner The antioxidant role of coenzyme Q Mitochondrion 7S 2007 S41 S51
10. 10 response to oral ingestión of coenzyme Q10 formulations Mitochondrion 7S 2007 S78 S88
11. M. Bjornstedt, T. Nordman, and J.M. Olsson Extramitochondrial reduction by flavoenzymes Methods Enzymol 378 2004 131 138
12. M. Bodmer, P. Vankan, M. Dreier, K.W. Kutz, and J. Drewe Pharmacokinetics and metabolism of idebenone in healthy male subjects Eur J Clin Pharmacol 65 2009 493 501
13. J.-J. Briere, D. Schlemmer, D. Chretien, and P. Rustin Quinone analogues regulate mitochondrial substrate competitive oxidation Biochem Biophys Res Commun 316 2004 1138 1142 (Pubitemid 38369071)
14. 10 Neurology 38 1988 892 899
15. N. Bresolin, C. Doriguzzi, C. Ponzetto, C. Angelini, I. Moroni, and E. Castelli et al. Ubidecarenone in the treatment of mitochondrial myopathies: a multicentre double-blind trial J Neurol Sci 10 1990 70 78
16. 10 therapy J Neurol 245 1998 681 685 (Pubitemid 28439035)
17. S. Dimauro, M. Mancuso, and A. Naini Mitochondrial encephalomyopathies: therapeutic approach Ann N Y Acad Sci 1011 2004 232 245 (Pubitemid 38608408)
18. 10 status in blood mononuclear cells, skeletal muscle and plasma by HPLC with a di-propoxy-coenzyme Q10 as an internal standard Clin Chem 51 2005 2380 2382 (Pubitemid 41692590)
19. 10 deficiency results in a global loss of mitochondrial respiratory chain activity, increased mitochondrial oxidative stress and a reversal of ATP synthase activity: implications for the pathogenesis and treatment Inherit Metab Dis 36 2013 63 73
20. 10 deficiency: patient study and literature review Arch Neurol 69 2012 979 983
21. J. Engelsen, J.D. Nielsen, and K.F. Hansen Effect of coenzyme Q10 and Ginkgo biloba on warfarin dosage in patients on long-term warfarin treatment. A randomized, double-blind, placebo-controlled cross-over trial Ugeskr Laeger 165 2003 1868 1871 (Pubitemid 38094624)
22. G.M. Enns, S.L. Kinsma, S.L. Perlman, K.M. Spicer, J.E. Abdenur, and B.H. Cohen et al. Initial experience in the treatment of inherited mitochondrial disease with EPI-743 Molec Genet Metab 105 2012 91 102
23. L. Ernster, and G. Dallner Biochemical, physiological and medical aspects of ubiquinone function Biochim Biophys Acta 1271 1995 195 204
24. L. Ernster, and G. Dallner Biochemical, physiological and medical aspects of ubiquinone function Biochim Biophys Acta 1271 1995 5 204
25. L. Ernster, and P. Forsmark-Andree Ubiquinol: an endogenous antioxidant in aerobic organisms Clin Invest 40 1993 380 390
26. R. Fadic, and D.R. Johns Clinical spectrum of mitochondrial diseases Semin Neurol 16 1996 11 20 (Pubitemid 126447960)
27. B.D. Fink, J.A. Herlein, M.A. Yorek, A.M. Fenner, R.J. Kerns, and W.I. Sivitz Bioenergetic effects of mitochondrial-targeted coenzyme Q analogs in endothelial cells JPET 342 2012 709 712
28. 10) and idebenone in the therapy of respiratory chain diseases: rationale and comparative benefits Mol Genet Metab 77 2002 21 30 (Pubitemid 35178511)
29. V. Giorgio, V. Petronilli, A. Ghelli, V. Carelli, M. Rugolo, and G. Lenaz et al. The effects of idebenone on mitocondrial bioenergetics Biochim Biophys Acta 1817 2012 363 369
30. 10 in mitochondrial disorders Muscle Nerve 2010 739 748
31. 10 in a patient with mitochondrial encephalomyopathy J Neurol 234 1987 62 63
32. R.H. Haas, S. Parikh, M.J. Falk, R.P. Saneto, N.I. Wolf, and N. Darin et al. Mitochondrial disease: a practical approach for primary care physicians Pediatrics 120 2007 1326 1333 (Pubitemid 350223685)
33. R.H. Haefeli, M. Erb, A.C. Gemperli, D. Robay, I. Courdier Fruh, and C. Anklin et al. NQO1-dependent redox cycling of idebenone: effects on cellular redox potencial and energy levels PLoS ONE 6 2011 e17963
34. K. Haginoya, S. Miyabayashi, M. Kikuchi, A. Kojima, K. Yamamoto, and K. Omura et al. Efficacy of idebenone for respiratory failure in a patient with Leigh syndrome: a long-term follow-up study J Neurol Sci 278 2009 112 114
35. I.P. Hargreaves Ubiquinone: cholesterol's reclusive cousin Ann Clin Biochem 40 2003 207 218 (Pubitemid 36560991)
36. 10 Drug Saf 28 2005 659 676
37. I.P. Hargreaves, Y. Sheena, J.M. Land, and S.J.R. Heales Glutathione deficiency in patients with mitochondrial disease: implications for pathogenesis and treatment J Inherit Metab 28 2005 81 88 (Pubitemid 40253986)
38. A. Hassani, R. Horvath, and P.F. Chinnery Mitochondrial myopathies: developments in treatment Curr Opin Neurol 23 2010 459 465
39. S.F. Heeringa, G. Chernin, M. Chaki, W. Zhou, A.J. Sloan, and Z. Ji et al. COQ6 mutations cause nephrotic syndrome with sensorineural deafness in humans, concurrent with increased apoptosis J Clin Invest 121 2011 2013 2024
40. T. Hidaka, K. Fujii, I. Funahashi, N. Fukutomi, and K. Hosoe Safety assessment of coenzyme Q10 (CoQ10) Biofactors 32 2008 199 208
41. Y. Ikejiri, E. Mori, K. Ishii, K. Nishimoto, M. Yasuda, and M. Sasaki Idebenone improves cerebral mitocondrial oxidative metabolismo in a patient with MELAS Neurology 47 1996 583 585 (Pubitemid 26324069)
42. A.M. James, H.M. Cocheme, and M.P. Murphy Mitochondria-targeted redox robes as tools in the study of oxidative damage and aging Mech Ageing Dev 126 2005 982 986 (Pubitemid 41033179)
43. D.S. Kerr Review of clinical trials for mitochondrial disorder: 1997-2012 Neurotherapeutics 10 2013 307 319
44. M.A. Kirkman, A. Korsten, M. Leonhardt, K. Dimitriadis, I.F. De Coo, and T. Klopstock et al. Quality of life in patients with leber hereditary optic neuropathy Invest Ophthalmol Vis Sci 50 2009 3112 3115
45. T. Klopstock, P. Yu-Wai-Man, K. Dimitriadis, J. Rouleau, S. Heck, and M. Bailie et al. A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy Brain 134 2011 2677 2686
46. 10 administration on its tissue concentrations, mitochondrial oxidant generation, and oxidative stress in the rat Free Radic Biol Med 33 2002 627 638 (Pubitemid 35245835)
47. 10 deficiency Neurochem Res 32 2007 837 843 (Pubitemid 46481312)
48. J.M. Land, J.A. Morgan-Hughes, I. Hargreaves, and S.J.R. Heales Mitochondrial disease: a historical, biochemical, and London perspective Neurochem Res 29 2004 488 491
49. C. Landbo, and T.P. Almdal Interaction between warfarin and coenzyme Q10 Ugwskr Laeger 160 1998 3226 3227
50. 10 in cardiovascular disease with special emphasis on heart failure and myocardial ischemia Asia Pacific Heart J 7 1998 160 168 (Pubitemid 29154691)
51. E. Lapuente-Brun, R. Moreno-Loshuertos, R. Acin-Perez, A. Latorre-Pellicer, C. Colás, and E. Balsa et al. Supercomplex assembly determines electron flux in the mitocondrial electron transport chain Science 340 2013 1567 1570
52. A. Lass, and R.S. Sohal Comparisons of coenzymevQ bound to mitochondrial membrane proteins among different mammalian species Free Radic Biol Med 27 1999 220 226 (Pubitemid 29353103)
53. 10 deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effects PLoS ONE 5 2010 e11897
54. J.M. Lopez-Martin, L. Salviati, E. Trevisson, G. Montini, S. DiMauro, and C. Quinzii et al. Missense mutation of the CoQ2 gene causes defects of bioenergetics and defects of bioenergetics and de novo pyrimidine synthesis Hum Mol Genet 16 2007 1091 1097 (Pubitemid 47062729)
55. D.J. Mahoney, G. Parise, and M.A. Tarnopolsky Nutritional and exercise-based therapies in the treatment of mitochondrial disease Curr Opin Clin Nutr Metabol Care 5 2002 619 629 (Pubitemid 40684628)
56. 10 administration increases brain mitochondrial concentrations and exerts neuroprotective effects Proc Natl Acad Sci USA 95 1998 8892 8897
57. D. Martinelli, M. Catteruccia, F. Piemonte, A. Pastore, G. Tozzi, and C. Dionisi-Vici et al. EPI-743 reverses the progrssion of the pediatric mitochondrial disease Mol Genet Metab 107 2012 383 388
58. 10 in mitocondrial encephalomyopathies Neuromuscul Disord 1 1991 443 447
59. 10 with multiple vitamins is generally ineffective in treatment of mitochondrial disease Neurology 43 1993 884 890 (Pubitemid 23150556)
60. M.V. Miles, L. Miles, L.M. Tang, P.S. Horn, P.E. Steele, and A.J. DeGrauw et al. Systemic evaluation of muscle coenzyme Q10 content in children with mitochondrial respiratory chain enzyme deficiencies Mitochondrion 8 2008 170 180
61. G. Montini, C. Malaventura, and L. Salviati Early supplementation in primary coenzyme Q10 deficiency N Engl J Med 358 2008 2849 2850 (Pubitemid 351930884)
62. 10 concentrations in patients with a probable and definitive diagnosis of respiratory chain disorders Biofactors 25 2005 109 115 (Pubitemid 44070838)
63. 10 deficiency syndromes Clin Biochem. 41 2008 697 700
64. R. Montero, M. Grazina, E. Lopez-Gallardo, J. Montoya, P. Briones, and A. Navarro-Sastre et al. Coenzyme Q10 deficiency in mitocondrial DNA depletion síndromes Mitochondrion 13 2013 337 341
65. 10 supplementation in primary coenzyme Q10 deficiency N Engl J Med 358 2008 2849 2850 (Pubitemid 351930884)
66. 10 therapy for a mitochondrial encephalomyopathy with cytochrome c oxidase deficiency: a 31P NMR study Neurology 39 1989 399 403 (Pubitemid 19094734)
67. S. Ogasahara, A.G. Engel, D. Frens, and D. Mack Muscle coenzymee Q deficiency in familial mitocondrial encephalomyopathy Proc Natl Acad Sci USA 86 1989 2379 2382 (Pubitemid 19102512)
68. 10 Neurology 36 1986 45 53 (Pubitemid 16186045)
69. 10 in Kearns-Sayre syndrome Neurology 35 1985 372 377 (Pubitemid 15100678)
70. 10 on total serum calcium concentration in two patients with Kearns-Sayre syndrome and hypoparathyroidism Neuromuscul Disord 6 1996 49 53
71. H. Päivä, K.M. Thelen, R. Van Coster, J. Smet, B. De Paepe, and K.M. Mattila et al. High dose statins and skeletal muscle metabolism in humans: a randomized controlled trial Clin Pharm Ther 78 2005 60 68 (Pubitemid 40956932)
72. B.A. Parker, S.M. Gregory, L. Lorson, D. Polk, C.M. White, and P.D. Thompson A randomized trial of coenzyme Q10 in patients with statin myopathy: rationale and study design J Clin Lipidol 7 2013 187 193
73. J.L. Quiles, J.J. Ochoa, M. Battino, P. Gutierrez-Rios, E.A. Nepomuceno, and M.L. Frías et al. Life-long supplementation with a low dosage of coenzyme Q10 in the rat: effects on antioxidant status and DNA damage Biofactors 25 2005 73 86 (Pubitemid 44070835)
74. 10 deficiency Neurochem Res 32 2007 723 727 (Pubitemid 46481301)
75. 10 deficiency Neuromuscul Disord 22 2012 76 86
76. S. Rahman, and M.G. Hanna Mitochondrial diseases disorders: diagnosis and new treatments in mitochondrial disease J Neurol Neurosurg Psychiatry 80 2009 943 953
77. G. Rudolph, K. Dimitriadis, B. Büchner, S. Heck, J. Al-Tamami, and F. Seidensticker et al. Effects of idebenone on color vision in patients with Leber hereditary optic neuropathy J Neuro-Ophthalmol 33 2013 30 36
78. R.A.J. Smith, and M.P. Murphy Animal and human studies with the mitochondria-targeted antioxidant mitoQ Ann N Y Acad Sci 1201 2010 96 103
79. A. Rotig, J. Mollet, M. Rio, and Munnich Infantile and pediatric quinone deficiency diseases Mitochondrion 7S 2007 S112 S121
80. 10 is frequently reduced in muscle of patients with mitocondrial myopathy Neuromuscular Disorders 20 2010 44 48
81. A.A. Sadun, C.F. Chicani, F.N. Ross-Cisneros, P. Barboni, M. Thoolen, and W.D. Shrader et al. Effect of EPI-743 on the clinical course of the mitochondrial disease Leber hereditary optic neuropathy Arch Neurol 69 2012 331 338
82. J.M. Shoffner, M.T. Lott, A.S. Voljavec, S.A. Soueidan, D.A. Costigan, and D.C. Wallace Spontaneous Kearns-Sayre/chronic external ophthalmoplegia plus syndrome associated with a mitochondrial DNA deletion: a slip-replication model and metabolic therapy Proc Natl Acad Sci USA 86 1989 7952 7956 (Pubitemid 19265298)
83. 10 formulations in Huntington's disease Biochim Biophys Acta 1762 2006 616 626 (Pubitemid 43903119)
84. R.S. Sohal, and M.J. Forster Coenzyme Q, oxidative stress and aging Mitochondrion 7S 2007 S103 S111
85. 10 in children with primary mitochondrial disease Mitochondrion 12 2012 623 629
86. M. Turunen, J. Olsson, and G. Dallner Metabolism and function of coenzyme Q Biochem Biophys Acta 1660 2004 171 199 (Pubitemid 38125244)
87. S. Zierz, G. Jahns, and F. Jerusalem Coenzyme Q in serum and muscle of 5 patients with Kearns-Sayre syndrome and 12 patients with ophthalmoplegia plus J Neurol 236 1989 97 101 (Pubitemid 19110225)