Features of idebenone and related short-chain quinones that rescue ATP levels under conditions of impaired mitochondrial complex I

PLoS ONE

Volumn 7, Issue 4, 2012, Pages

  Erb, Michael ^a   Hoffmann Enger, Barbara ^a   Deppe, Holger ^a   Soeberdt, Michael ^a   Haefeli, Roman H ^a,b   Rummey, Christian ^a   Feurer, Achim ^a   Gueven, Nuri ^a  

^a Santhera Pharmaceuticals   (Switzerland)

^b UNIVERSITY OF BASEL   (Switzerland)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE TRIPHOSPHATE; ALPHA TOCOTRIENOL QUINONE; DECYLUBIQUINONE; IDEBENONE; QUINONE DERIVATIVE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); UNCLASSIFIED DRUG; ANTIOXIDANT; DRUG DERIVATIVE; UBIQUINONE;

ANIMAL CELL; ARTICLE; CELL ENERGY; CONTROLLED STUDY; DRUG ACTIVITY; DRUG STRUCTURE; HUMAN; HUMAN CELL; LIPID PEROXIDATION; NONHUMAN; PHYSICAL CHEMISTRY; RAT; ANIMAL; CELL LINE; CHEMISTRY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; DRUG EFFECT; METABOLISM; MITOCHONDRIAL COMPLEX I DEFICIENCY;

ADENOSINE TRIPHOSPHATE; ANIMALS; ANTIOXIDANTS; CELL LINE; ELECTRON TRANSPORT COMPLEX I; HUMANS; LIPID PEROXIDATION; MITOCHONDRIAL DISEASES; PHYSICOCHEMICAL PROCESSES; RATS; UBIQUINONE;

EID: 84860477515     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0036153     Document Type: Article

References (36)

1. O'Brien PJ, (1991) Molecular mechanisms of quinone cytotoxicity. Chem Biol Interactions 80: 1-41.
2. Monks TJ, Hanzlik RP, Cohen GM, Ross D, Graham DG, (1992) Contemporary issues in toxicology. Toxicol Appl Pharmacol 112: 2-16.
3. Long DJ II, Jaiswal AK, (2000) NRH:quinone oxidoreductase2 (NQO2). Chem Biol Interact 129: 99-112.
4. Colucci MA, Moody CJ, Gouch GD, (2008) Natural and synthetic quinones and their reduction by the quinone reductase enzyme NQO1: from synthetic organic chemistry to compounds with anticancer potential. Org Biomol Chem 6 (4): 637-56.
5. Boutin, JA Chatelain-Egger F, Vella F, Delagrangea P, Ferry G, (2005) Quinone reductase 2 substrate specificity and inhibition pharmacology. Chem Biol Interact 151: 213-228.
6. Chan TS, Teng S, Wilson JX, Galati G, Khan S, et al. (2002) Coenzyme Q cytoprotective mechanisms for mitochondrial complex I cytopathies involves NAD(P)H: quinone oxidoreductase 1(NQO1). Free Radic Res 36: 421-7.
7. Dragan M, Dixon SJ, Jaworski E, Chan TS, O'Brien PJ, et al. (2006) Coenzyme Q(1) depletes NAD(P)H and impairs recycling of ascorbate in astrocytes. Brain Res 1078: 9-18.
8. Suno M, Akinobu N, (1984) Inhibition of lipid peroxidation by a novel compound (CV-2619) in brain mitochondria and mode of action of the inhibition. Biochem Biophys Res Comm 125: 1046-1052.
9. Sugiyama Y, Fujita T, Matsumoto M, Okamoto K, Imada I, (1985) Effects of idebenone (CV-2619) and its metabolites on respiratory activity and lipid peroxidation in brain mitochondria from rats and dogs. J Pharmacobio-Dyn 8: 1006-1017.
10. Suno M, Akinobu N, (1989) Inhibition of lipid peroxidation by idebenone in brain mitochondria in the presence of succinate. Arch Gerontol Geriatr 8: 291-297.
11. Rauchovà H, Vrbacky M, Bergamini C, Fato R, Lenaz G, et al. (2006) Inhibition of glycerophosphate-dependent H2O2 generation in brown fat mitochondria by idebenone. Biochem Biophys Res Comm 339: 362-366.
12. Ranganathan S, Harmison GG, Meyertholen K, Pennuto M, Burnett BG, et al. (2009) Mitochondrial abnormalities in spinal and bulbar muscular atrophy. Hum Mol Genet 18: 27-42.
13. Jauslin ML, Wirth T, Meier T, Schoumacher F, (2002) A cellular model for Friedreich Ataxia reveals small-molecule glutathione peroxidase mimetics as novel treatment strategy. Human Mol. Genet 11: 3055.
14. Becker C, Bray-French K, Drewe J, (2010) Pharmacokinetic evaluation of idebenone. Expert Opin Drug Metab Toxicol. 6 (11): 1437-44.
15. Haginoya K, Miyabayashi S, Kikuchi M, Kojima A, Yamamoto K, et al. (2009) Efficacy of idebenone for respiratory failure in a patient with Leigh syndrome: a long-term follow-up study. J Neurol Sci 278: 112-4.
16. Ikejiri Y, Mori E, Ishii K, Nishimoto K, Yasuda M, et al. (1996) Idebenone improves cerebral mitochondrial oxidative metabolism in a patient with MELAS. Neurology 47: 583-5.
17. Napolitano A, Salvetti S, Vista M, et al. (2000) Long-term treatment with idebenone and riboflavin in a patient with MELAS. Neurol Sci 21: 981-2.
18. Klopstock T, Yu-Wai-Man P, Dimitriadis K, Rouleau J, Heck S, et al. (2011) A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy. Brain 134(Pt 9): 2677-86.
19. Carelli V, La Morgia C, Valentino ML, Rizzo G, Carbonelli M, et al. (2011) Idebenone treatment in Leber's hereditary optic neuropathy. Brain 134(Pt 9): e188.
20. Tsuruo Y, Ishimura K, Tamura M, Kagawa S, Morita K, (1994) Biochemical and histochemical studies of the effects of cerebral metabolism-improving drugs on NADPH diaphorase activity in mouse brain. Jpn J Pharmacol. 65 (3): 285-8.
21. Haefeli RH, Erb M, Gemperli AC, Robay D, Courdier Fruh I, et al. (2011) NQO1-dependent redox cycling of idebenone: effects on cellular redox potential and energy levels. PLoS One. 2011 Mar 31; 6 (3): e17963.
22. Giorgio V, Petronilli V, Ghelli A, Carelli V, Rugolo M, et al. (2011) The effects of idebenone on mitochondrial bioenergetics. Biochim Biophys Acta. 1817 (2): 363-9.
23. King MS, Sharpley MS, Hirst J, (2009) Reduction of hydrophilic ubiquinones by the flavin in mitochondrial NADH:ubiquinone oxidoreductase (Complex I) and production of reactive oxygen species. Biochemistry. 48 (9): 2053-62.
24. Genova ML, Pich MM, Biondi A, Bernacchia A, Falasca A, et al. (2003) Mitochondrial production of oxygen radical species and the role of Coenzyme Q as an antioxidant. Exp Biol Med (Maywood). 228 (5): 506-13.
25. Ross D, Kepa JK, Winski SL, Beall HD, Anwar A, et al. (2000) NAD(P)H:quinone oxidoreductase 1 (NQO1): chemoprotection, bioactivation, gene regulation and genetic polymorphisms. Chem Biol Interact. 129: 77-97.
26. Pink JJ, Planchon SM, Tagliarino C, Varnes ME, Siegel D, et al. (2000) NAD(P)H:Quinone oxidoreductase activity is the principal determinant of β-lapachone cytotoxicity. J Biol Chem 275: 5416-5424.
27. Adikesavan AK, Barrios R, Jaiswal AK, (2007) In vivo role of NAD(P)H:Quinone oxidoreductase 1 in metabolic activation of mitomycin C and bone marrow cytotoxicity. Cancer Res 67: 7966-7971.
28. Mordente A, Martorana GE, Minotti G, Giardina B, (1998) Antioxidant properties of 2,3-dimethoxy-5-methyl-6-(10-hydroxydecyl)-1,4-benzoquinone (idebenone). Chem Res Toxicol. 11 (1): 54-63.
29. Degli Esposti M, Ngo A, Ghelli A, Benelli B, Carelli B, et al. (1996) The interaction of Q analogs, particularly hydroxydecyl-benzoquinone (idebenone), with the respiratory complexes of heart mitochondria. Arch Biochem Biophys 330: 395-400.
30. Fato R, Bergamini C, Bortolus M, Maniero AL, Leoni S, et al. (2008) Differential effects of mitochondrial Complex I inhibitors on production of reactive oxygen species. Biochim Biophys Acta 1787: 384-92.
31. Rauchová H, Drahota Z, Bergamini Z, Fato R, Lenaz G, (2008) Modification of respiratory-chain enzyme activities in brown adipose tissue mitochondria by idebenone (hydroxydecyl-ubiquinone). J Bioenerg Biomembr 40: 85-93.
32. King MS, Sharpley MS, Hirst J, (2009) Reduction of hydrophilic ubiquinones by the flavin in mitochondrial NADH:ubiquinone oxidoreductase (Complex I) and production of reactive oxygen species. Biochemistry. 48 (9): 2053-62.
33. Feurer A, Gueven N, Hoffmann-Enger B, Erb M, Deppe H, et al. (2012) Benzoquinone Derivatives as Modulators of Mitochondrial Function. WO/2012/ 022468.
34. Feurer A, Hoffmann-Enger B, Deppe H, Soeberdt M, Gueven N, et al. (2012) Novel Benzoquinone Derivatives and Use thereof as Modulators of Mitochondrial Function WO/2012/ 022467.
35. Courdier-Fruh I, Briguet A, (2006) Utrophin is a calpain substrate in muscle cells. Muscle Nerve; 33 (6): 753-9.
36. Ernster L, (1967) DT-diaphorase. Methods Enzymol 10: 309-317.