- Bayer, R.E. (1992) "An analysis of the role of coenzyme Q in free radical generation and as an antioxidant", Biochem. Cell. Biol. 70, 390 - 403
- Dallner, G. and Sindelar, P.J. (2000) "Regulation of ubiquinone metabolism", Free Radic. Biol. Med. 29, 285 - 294
- Noack, H., Kube, U. and Augustin, W. (1994) "Relations between tocopherol depletion and coenzyme Q during lipid peroxidation in rat liver mitochondria", Free Radic. Res. 20, 375 - 386
- Lass, A. and Sohal, R.S. (1998) "Electron transport-linked ubiquinone-dependent recycling of a-tocopherol inhibits autooxidation of mitochondrial membranes", Arch. Biochem. Biophys. 352, 229 - 236
- Ogasahara, S., Engel, A.G., Frens, D. and Mack, D. (1989) "Muscle coenzyme Q deficiency in familial mitochondrial encephalomyopathy", Proc. Natl. Acad. Sci. USA 86, 2379 - 2382
- Sobereira, C., Hirano, M., Shanske, S., Keller, R.K., Haller, R.G., Davidson, E., Santorelli, F.M., Miranda, A.F., Bonilla, E., Mojon, D.S., Barreira, A.A., King, M.P. and Dimauro, S. (1997) "Mitochondrial encephalomyopathy with coenzyme Q 10 deficiency", Neurology 48, 1238 - 1243
- Boieter, E., Degoul, F., Desguerre, I., Charpentier, C., Francois, D., Ponsot, G., Diry, M., Rustin, P. and Marsac, C. (1998) "A case of mitochondrial encephalomyopathy associated with a muscle coenzyme Q 10 deficiency", J. Neurol. Sci. 156, 41 - 46
- Rotig, A., Appelkvist, E.L., Geromel, V., Chretien, D., Kadhom, N., Edery, P., Lebideau, M., Dallner, G., Munnich, A., Ernster, L. and Rustin, P. (2000) "Quinone-responsive multiple respiratory-chain dysfunction due to wide spread coenzyme Q 10 deficiency", Lancet 356, 391 - 395
- Abe, K., Fujimura, H., Nishikawa, Y., Yorifuki, S., Mezaki, T., Hirono, N., Nishitani, N. and Kameyama, M. (1991) "Marked reduction in CSF lactate and pyruvate levels after CoQ therapy in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS)", Acta Neurol. Scand. 83, 356 - 359
- Bresolin, N., Bet, L., Binda, A., Moggio, M., Comi, G., Nador, F., Ferrante, C., Carenzi, A. and Scarlato, G. (1988) "Clinical and biochemical correlations in mitochondrial myopathies treated with coenzyme Q 10", Neurology 38, 892 - 899
- Ihara, Y., Namba, R., Kuroda, S., Sato, T. and Shirabe, T. (1989) "Mitochondrial encephalomyopathy (MELAS): pathological study and successful therapy with coenzyme Q 10 and idebenone", J. Neurol. Sci. 90, 263 - 271
- Nishikawa, Y., Takahashi, M., Yorifuji, S., Nakamura, Y., Ueno, S., Tarui, S., Kozuka, T. and Nishimura, T. (1989) "Long-term coenzyme Q 10 therapy for a mitochondrial encephalomyopathy with cytochrome c oxidase deficiency: a 31 P NMR study", Neurology 39, 399 - 403
- Chan, A., Reichmann, H., Kogel, A., Beck, A. and Gold, R. (1998) "Metabolic changes in patients with mitochondrial myopathies and effects of coenzyme Q 10 therapy", J. Neurol. 245, 681 - 685
- Barbiroli, B., Frassineti, C., Martinelli, P., Lotti, S., Lodi, R., Cortelli, P. and Montagna, P. (1997) "Coenzyme Q 10 improves mitochondrial respiration in patients with mitochondrial cytopathies. An in vivo study on brain and skeletal muscle by phosphorus magnetic resonance spectroscopy", Cell. Mol. Biol. 43, 741 - 749
- Abe, K., Matsuo, Y., Kadekawa, J., Inoue, S. and Yanagihara, T. (1999) "Effect of coenzyme Q 10 in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): evaluation by noninvasive tissue oximetry", J. Neurol. Sci. 162, 65 - 68
- Chariot, P., Brugieres, P., Eliezer-Vanerot, M.C., Geny, C., Binaghi, M. and Cesaro, P. (1999) "Choreic movements and MRI abnormalities in the subthalamic nuclei reversible after administration of coenzyme Q 10 and multiple vitamins in a patient with bilateral optic neuropathy", Mov. Disorders 14, 855 - 859
- Bruno, V., Battaglia, G., Copani, A., Sortino, M.A., Canonico, P.L. and Nicoletti, F. (1994) "Protective action of idebenone against excitotoxic degeneration in cultured cortical neurons", Neurosci. Lett. 178, 193 - 196
- Ikejiri, Y., Mori, E., Ishii, K., Nishimoto, K., Yasuda, M. and Sasaki, M. (1996) "Idebenone improves cerebral mitochondrial oxidative metabolism in a patient with MELAS", Neurology 47, 583 - 585
- Cortelli, P., Montagna, P., Pierangeli, G., Lodi, R., Barboni, P., Liguori, R., Carelli, V., Lotti, S., Zaniol, P., Lugaresi, E. and Barbiroli, B. (1997) "Clinical and brain bioenergetics improvement with idebenone in a patient with Leber's hereditary optic neuropathy: a clinical and 31 P-MRS study", J. Neurol. Sci. 148, 25 - 31
- Rustin, P., Von Kleist-Retzow, J.C., Chantrel-Groussard, K., Sidi, D., Munnich, A. and Rotig, A. (1999) "Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study", Lancet 354, 477 - 479
- Landi, L., Fiorentini, D., Galli, M.C., Segura-Aguilar, J. and Beyer, R.E. (1997) "DT-diaphorase maintains the reduced state of ubiquinones in lipid vesicles thereby promoting their antioxidant function", Free Radic. Biol. Med. 22, 329 - 335
- Edlund, C., Holmberg, K., Dallner, G., Norrby, E. and Kristensson, K. (1994) "Ubiquinone-10 protects neurons from virus-induced degeneration", J. Neurochem. 63, 634 - 639
- Favit, A., Nicoletti, F., Scapagnini, U. and Canonico, P.L. (1992) "Ubiquinone Protects cultured neurons against spontaneous and excitotoxin-induced degeneration", J. Cereb. Blood Flow Metab. 12, 638 - 645
- Beal, M.F., Henshaw, R., Jenkins, B.G., Rosen, B.R. and Schulz, J.B. (1994) "Coenzyme Q 10 and nicotinamide block striatal lesions produced by the mitochondrial toxin malonate", Annals Neurol. 36, 882 - 888
- Beal, M.F. and Matthews, R.T. (1997) "Coenzyme Q 10 in the central nervous system and its potential usefulness in the treatment of neurodegenerative diseases", Mol. Asp. Med. 18, s169 - s179
- Brouillet, E., Henshaw, D.R., Schulz, J.B. and Beal, M.F. (1994) "Aminooxyacetic acid striatal lesions attenuated by 1,3-butanediol and coenzyme Q 10", Neurosci. Lett. 177, 58 - 62
- Matthews, R.T., Yang, S., Browne, S., Baik, M. and Beal, M.F. (1998) "Coenzyme Q 10 administration increases brain mitochondrial concentrations and exerts neuroprotective effects", Proc. Natl. Acad. Sci. USA 95, 8892 - 8897
- Ostrowski, R.P. (2000) "Effect of coenzyme Q 10 on biochemical and morphological changes in experimental ischemia in the rat brain", Brain Res. Bull. 53, 399 - 407
- Reahal, S. and Wrigglesworth, J. (1992) "Tissue concentrations of coenzyme Q 10 in the rat following its oral and intraperitoneal administration", Drug Metab. Dispos. 20, 423 - 427
- Zhang, Y., Turunen, M. and Appelkvist, E.-L. (1996) "Restricted uptake of dietary coenzyme Q is in contrast to the unrestricted uptake of α-tocopherol into rat organs and cells", J. Nutr. 126, 2089 - 2097
- Lass, A., Forster, M.J. and Sohal, R.S. (1999) "Effects of coenzyme Q 10 and α-tocopherol administration on their tissue levels in the mouse: elevation of mitochondrial alpha-tocopherol by coenzyme Q 10", Free Radic. Biol. Med. 26, 1375 - 1382
- Ibrahim, W.H., Bhagavan, H.N., Chopra, R.K. and Chow, C.K. (2000) "Dietary coenzyme Q 10 and vitamin E alter the status of these compounds in rat tissues and mitochondria", J. Nutr. 130, 2343 - 2348
- Battino, M., Gorini, A., Villa, R.F., Genova, M.L., Bovina, C., Sassi, S., Littarru, G.P. and Lenaz, G. (1995) "Coenzyme Q content in synaptic and non-synaptic mitochondria from different brain regions in the ageing rat", Mech. Ageing Dev. 78, 173 - 187
- Beyer, R.E., Burnett, B.-A., Cartwright, K.J., Edington, D.E., Falzon, M.J., Kreitman, K.R., Kuhn, T.W., Ramp, B.J., Rhee, S.Y.S., Rosenwasser, M.J., Stein, M. and An, L.C.-I. (1985) "Tissue coenzyme Q (ubiquinone) and protein concentrations over the life span of the laboratory rat", Mech. Ageing Dev. 32, 267 - 281
- Kalen, A., Appelkvist, E.-L. and Daliner, G. (1989) "Age-related changes in the lipid compositions of rat and human tissues", Lipids 24, 579 - 584
- Kagan, V.E., Serbinova, E.A., Koynova, G.M., Kitanova, S.A., Tyurin, V.A., Stoytchev, T.S., Quinn, P.J. and Packer, L. (1990) "Antioxidant action of ubiquinol homologues with different isoprenoid chain length in biomembranes", Free Radic. Biol. Med. 9, 117 - 126
- Poderoso, J.J., Carreras, M.C., Schopfer, F., Lisdero, C.L., Riobo, N.A., Giulivi, C., Boveris, A.D., Boveris, A. and Cadenas, E. (1999) "The reaction of nitric oxide with ubiquinol: kinetic properties and biological significance", Free Radic. Biol. Med. 26, 925 - 935
- Kagan, V., Serbinova, E. and Packer, L. (1990) "Antioxidant effects of ubiquinones in microsomes and mitochondria are mediated by tocopherol recycling", Biochem. Biophys. Res. Commun. 169, 851 - 857
- Maguire, J.J., Kagan, V., Ackrell, B.A., Serbinova, E. and Packer, L. (1992) "Succinate-ubiquinone reductase linked recycling of α-tocopherol in reconstituted systems and mitochondria: requirement of reduced ubiquinone", Arch. Biochem. 292, 47 - 53
- Mukai, K., Morimoto, H., Kikuchi, S. and Nagaoka, S. (1993) "Kinetic study of free-radical-scavenging action of biological hydroquinones (reduced forms of ubiquinone, vitamin K and tocopherol quinone) in solution", Biochim. Biopys. Acta 1157, 313 - 317
- Shi, H., Noguchi, N. and Niki, E. (1999) "Comparative study on dynamics of antioxidative action of α-tocopheryl hydroquinone, ubiquinol, and α-tocopherol against lipid peroxidation", Free Radic. Biol. Med. 27, 334 - 346
- Kozlov, A.V., Gille, L., Staniek, K. and Nohl, H. (1999) "Dihydrolipoic acid maintains ubiquinone in the antioxidant active form by two-electron reduction of ubiquinone and one-electron reduction of ubisemiquinone", Arch. Biochem. Biophys. 363, 148 - 154
- Gotz, M.E., Dirr, A., Burger, R., Janetzky, B., Weinmuller, M., Chan, W.W., Chen, S.C., Reichmann, H., Rausch, W.D. and Riederer, P. (1994) "Effect of lipoic acid on redox state of coenzyme Q in mice treated with 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine and diethyldithiocarbamate", Eur. J. Pharmacol. 266, 291 - 300
- Echtay, K.S., Winkler, E. and Klingenberg, M. (2000) "Coenzyme Q is an obligatory cofactor for uncoupling protein function", Nature 408, 609 - 613
- Echtay, K., Winkler, E., Frischmuth, K. and Klingenberg, M. (2001) "Uncoupling protein 2 and 3 are highly active H+ transporters and highly nucleotide sensitive when activated by coenzyme Q (ubiquinone)", Proc. Natl. Acad. Sci. USA 98, 1416 - 1421
- Ricquier, D. and Bouillaud, F. (2000) "The uncoupling protein homologues: UCP1, UCP2, UCP3, StUCP and AtUCP", Biochem. J. 345, 161 - 179
- Tomasetti, M., Littarru, G.P., Stocker, R. and Alleva, R. (1999) "Coenzyme Q 10 enrichment decreases oxidative DNA damage in human lymphocytes", Free Radic. Biol. Med. 27, 1027 - 1032
- Schulz, J.B., Matthews, R.T., Henshaw, D.R. and Beal, M.F. (1996) "Neuroprotective strategies for treatment of lesions produced by mitochondrial toxins: implications for neurodegenerative diseases", Neuroscience 71, 1043 - 1048
- Beal, M.F., Brouillet, E., Jenkins, B.G., Ferrante, R.J., Kowall, N.W., Miller, J.M., Storey, E., Srivastava, R., Rosen, B.R. and Hyman, B.T. (1993) "Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid", J. Neurosci. 13, 4181 - 4192
- Brouillet, E., Hantraye, P., Ferrante, R.J., Dolan, R., Leroy-Willig, A., Kowall, N.W. and Beal, M.F. (1995) "Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates", Proc. Natl. Acad. Sci. USA 92, 7105 - 7109
- Gurney, M.E., Pu, H., Chiu, A.Y., Dal Canto, M.C., Polchow, C.Y., Alexander, D.D., Caliendo, J., Hentati, A., Kwon, Y.W., Deng, H.-X., Chen, W., Zhai, P., Sufit, R.L. and Siddique, T. (1994) "Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation", Science 264, 1772 - 1775
- Kong, J. and Xu, Z. (1998) "Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1", J. Neurosci. 18, 3241 - 3250
- Mangiarini, L., Sathasivam, K., Seller, M., Cozens, B., Harper, A., Hetherington, C., Lawton, M., Trottier, Y., Lehrach, H., Davies, S.W. and Bates, G.P. (1996) "Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice", Cell 87, 493 - 506
- Ferrange, R.J., Andreassen, O.A., Jenkins, B.G., Dedeoglu, A., Kuemmerle, S., Kubilus, J.K., Kaddurah-Daouk, R., Hersch, S.M. and Beal, M.F. (2000) "Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease", J. Neurosci. 20, 4389 - 4397
- Shults, C.W., Haas, R.H., Passov, D. and Beal, M.F. (1997) "Coenzyme Q 10 levels correlate with the activities of complexes I and II/III in mitochondria from parkinsonian and nonparkinsonian subjects", Annals Neurol. 42, 261 - 264
- Koroshetz, W.J., Jenkins, B.G., Rosen, B.R. and Beal, M.F. (1997) "Energy metabolism defects in Huntington's disease and effects of coenzyme Q 10", Annals Neurol. 41, 160 - 165
- Feigin, A., Kieburtz, K., Como, P., Hickey, C., Claude, K., Abwendere, D., Zimmerman, C., Steinberg, K. and Shoulson, I. (1996) "Assessment of coenzyme Q 10 tolerability in Huntington's disease", Mov. Disorders 11, 321 - 323
- de Bustos, F., Molina, J.A., Jimenez-Jimenez, F.J., Garcia-Redondo, A., Gomez-Escalonilla, C., Porta-Etessam, J., Berbel, A., Zurdo, M., Barcenilla, B., Parrilla, G., Enriquez-de-Salamanca, R. and Arenas, J. (2000) "Serum levels of coenzyme Q 10 in patients with Alzheimer's disease", J. Neural Transm. 107, 233 - 239
- Molina, J.A., de Bustos, F., Jimenez-Jimenez, F.J., Gomez-Escalonilla, C., Garcia-Redondo, A., Esteban, J., Guerrero-Sola, A., Del Hoyo, P., Martinez-Salio, A., Ramirez-Ramos, C., Indurain, G.R. and Arenas, J. (2000) "Serum levels of coenzyme Q 10 in patients with amyotrophic lateral sclerosis", J. Neural Transm. 107, 1021 - 1026
- Gotz, M.E., Gerstner, A., Harth, R., Dirr, A., Janetzky, B., Kuhn, W., Riederer, P. and Gerlach, M. (2000) "Altered redox state of platelet coenzyme Q 10 in Parkinson's disease", J. Neural Transm. 107, 41 - 48
- Jimenez-Jimenez, F.J., Molina, J.A., de Bustos, F., Garcia-Redondo, A., Gomez-Escalonilla, C., Martinez-Salio, A., Berbel, A., Camacho, A., Zurdo, M., Barcenilla, B., Enriquez de Salamanca, R. and Arenas, J. (2000) "Serum levels of coenzyme Q 10 in patients with Parkinson's disease", J. Neural Transm. 107, 177 - 181
Coenzyme Q 10 as a Possible Treatment for Neurodegenerative Diseases
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