Coenzyme Q10 and nicotinamide block striatal lesions produced by the mitochondrial toxin malonate
Abstract
A potential mechanism of neuronal injury in neurodegenerative diseases is a defect in energy metabolism that may lead to slow excitotoxic neuronal death. Consistent with this possibility, we showed that specific inhibitors of the electron transport chain produce excitotoxic lesions in vivo. In the present study we examined whether agents that improve energy metabolism can block lesions produced by the mitochondrial toxin malonate. Striatal lesions produced by the complex II inhibitor malonate were blocked in a dose‐dependent manner by oral pretreatment with coenzyme Q 10. Administration of nicotinamide by Alzet pump for 1 week attenuated malonate‐induced lesions, but riboflavin had no effect. Administration of nicotinamide intraperitoneally just prior to and following induction of the lesions produced dose‐dependent neuroprotection. A combination of coenzyme Q 10 with nicotinamide was more effective than either compound alone, as shown by both lesion size and magnetic resonance imaging in vivo. Both coenzyme Q 10 and nicotinamide blocked adenosine triphosphate depletions and lactate increases. These results confirm that mitochondrial toxins produce striatal excitotoxic lesions by a mechanism involving energy depletion in vivo. Furthermore, they suggest novel neuroprotective strategies that may be useful in the treatment of both mitochondrial encephalopathies and neurodegenerative diseases.
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Oral administration of CoQ10 exerts significant neuroprotective effects against striatal lesions in rats produced by aminoxyacetic acid, malonate and 3-NP.
“Oral administration of CoQ10 exerts significant neuroprotective effects against striatal lesions in rats produced by aminoxyacetic acid, and the mitochondrial toxins malonate and 3-NP148-150.”
From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [148]
Oral CoQ10 produced dose-dependent neuroprotective effects against malonate-induced striatal lesions, depletion of ATP and increases in lactate concentration.
“Oral administration of CoQ10 produced dose-dependent neuroprotective effects against malonate induced striatal lesions, depletion of ATP and increases in lactate concentration148.”
From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [148]
Cites
Morris, R. G. M., Anderson, E., Lynch, G. S., & Baudry, M. (1986) · Nature · doi:10.1038/319774a0 · Cited by 3,640 · see citing works
Fluorometric assay of proteins in the nanogram range
Böhlen, P., Stein, S., Dairman, W. M., & Udenfriend, S. (1973) · Archives of Biochemistry and Biophysics · doi:10.1016/s0003-9861(73)80023-2 · Cited by 1,632 · see citing works
Nitric Oxide Activation of Poly(ADP-Ribose) Synthetase in Neurotoxicity
Zhang, J., Dawson, V. L., Dawson, T. M., & Snyder, S. H. (1994) · Science · doi:10.1126/science.8080500 · Cited by 1,118 · see citing works
Beal, M. F., Brouillet, E., Jenkins, B. G., Ferrante, R. J., Kowall, N. W., Miller, J., Storey, E., Srivastava, R., Rosen, B. R., & Hyman, B. T. (1993) · Journal of Neuroscience · doi:10.1523/jneurosci.13-10-04181.1993 · Cited by 1,026 · see citing works
Self-citationNovelli, A., Reilly, J. A., Lysko, P. G., & Henneberry, R. C. (1988) · Brain Research · doi:10.1016/0006-8993(88)90765-2 · Cited by 961 · see citing works
Beal, M. F. (1992) · Annals of Neurology · doi:10.1002/ana.410310202 · Cited by 956 · see citing works
Self-citationUbiquinol-10 is an effective lipid-soluble antioxidant at physiological concentrations.
Frei, B. B., Kim, M. C., & Ames, B. N. (1990) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.87.12.4879 · Cited by 623 · see citing works
Alternative excitotoxic hypotheses
Albin, R. L., & Greenamyre, J. T. (1992) · Neurology · doi:10.1212/wnl.42.4.733 · Cited by 574 · see citing works
Frahm, J., Bruhn, H., Gyngell, M. L., Merboldt, K. D., Hänicke, W., & Sauter, R. (1989) · Magnetic Resonance in Medicine · doi:10.1002/mrm.1910110105 · Cited by 522 · see citing works
Shoffner, J. M., Lott, M. T., Voljavec, A. S., Soueidan, S. A., Costigan, D. A., & Wallace, D. C. (1989) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.86.20.7952 · Cited by 505 · see citing works
Schraufstätter, I. U., Hyslop, P. A., Hinshaw, D. B., Spragg, R. G., Sklar, L. A., & Cochrane, C. G. (1986) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.83.13.4908 · Cited by 459 · see citing works
Age-dependent vulnerability of the striatum to the mitochondrial toxin 3-nitropropionic acid
Brouillet, E., Jenkins, B. G., Hyman, B. T., Ferrante, R. J., Kowall, N. W., Srivastava, R., Roy, D. S., Rosen, B. R., & Beal, M. F. (1993) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1993.tb05859.x · Cited by 367 · see citing works
Self-citationInvolvement of Free Radicals in Excitotoxicity In Vivo
Schulz, J. B., Henshaw, D. R., Siwek, D. F., Jenkins, B. G., Ferrante, R. J., Cipolloni, P. B., Kowall, N. W., Rosen, B. R., & Beal, M. F. (1995) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.1995.64052239.x · Cited by 322 · see citing works
Self-citationBeal, M. F., Brouillet, E., Jenkins, B. G., Henshaw, R., Rosen, B. R., & Hyman, B. T. (1993) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1993.tb03633.x · Cited by 297 · see citing works
Self-citationGreene, J. G., Porter, R. H. P., Eller, R. V., & Greenamyre, J. T. (1993) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1993.tb03634.x · Cited by 208 · see citing works
Koek, W., & Colpaert, F. C. (1990) · Journal of Pharmacology and Experimental Therapeutics · doi:10.1016/s0022-3565(25)13353-3 · Cited by 202 · see citing works
Storey, E., Hyman, B. T., Jenkins, B. G., Brouillet, E., Miller, J. M., Rosen, B. R., & Beal, M. F. (1992) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1992.tb10080.x · Cited by 164 · see citing works
Self-citationThe enzymatic measurement of adenine nucleotides and P-creatine in picomole amounts
Lust, W. D., Feussner, G. K., Barbehenn, E. K., & Passonneau, J. V. (1981) · Analytical Biochemistry · doi:10.1016/0003-2697(81)90144-5 · Cited by 163 · see citing works
Ihara, Y., Namba, R., Kuroda, S., SATO, T. A., & Shirabe, T. (1989) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(89)90112-3 · Cited by 161 · 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
MELAS syndrome with mitochondrial tRNA Leu[UUR] mutation
Penn, A. M. W., Lee, J. W. K., Thuillier, P., Wagner, M., Maclure, K. M., Menard, M. R., Hall, L. D., & Kennaway, N. G. (1992) · Neurology · doi:10.1212/wnl.42.11.2147 · Cited by 124 · see citing works
Aminooxyacetic Acid Results in Excitotoxin Lesions by a Novel Indirect Mechanism
Beal, M. F., Swartz, K. J., Hyman, B. T., Storey, E., Finn, S. F., & Koroshetz, W. J. (1991) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1991.tb08258.x · Cited by 118 · see citing works
Self-citationSchneider, H., Lemasters, J. J., & Hackenbrock, C. R. (1982) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)33893-6 · Cited by 110 · 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
Vatassery, G. T., Brin, M. F., Fahn, S., Kayden, H. J., & Traber, M. G. (1988) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1988.tb01083.x · Cited by 106 · see citing works
Therapy of mitochondrial disorders
Przyrembel, H. (1987) · Journal of Inherited Metabolic Disease · doi:10.1007/bf01812853 · Cited by 102 · see citing works
Nishikawa, Y., Takahashi, M. M., Yorifuji, S., Nakamura, Y., Ueno, S., Tarui, S., Kozuka, T., & Nishimura, T. (1989) · Neurology · doi:10.1212/wnl.39.3.399 · Cited by 100 · see citing works
Ubiquinone Protects Cultured Neurons against Spontaneous and Excitotoxin-Induced Degeneration
Favit, A., Nicoletti, F., Scapagnini, U., & Canonico, P. L. (1992) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1038/jcbfm.1992.88 · Cited by 98 · see citing works
Treatment of complex I deficiency with riboflavin
Bernsen, P. L. J. A., Gabreëls, F. J. M., Ruitenbeek, W., & Hamburger, H. (1993) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(93)90108-b · Cited by 97 · see citing works
Malonate produces striatal lesions by indirect NMDA receptor activation
Henshaw, R., Jenkins, B. G., Schulz, J. B., Ferrante, R. J., Kowall, N. W., Rosen, B. R., & Beal, M. F. (1994) · Brain Research · doi:10.1016/0006-8993(94)91412-5 · Cited by 92 · see citing works
Self-citationAbe, K., Fujimura, H., Nishikawa, Y., Yorifuji, S., Mezaki, T., Hirono, N., Nishitani, N., & Kameyama, M. (1991) · Acta Neurologica Scandinavica · doi:10.1111/j.1600-0404.1991.tb03962.x · Cited by 76 · see citing works
A protective effect of coenzyme Q10 on ischemia and reperfusion of the isolated perfused rat heart
Ohhara, H. (1981) · Journal of Molecular and Cellular Cardiology · doi:10.1016/0022-2828(81)90229-7 · Cited by 74 · see citing works
Reahal, S., & Wrigglesworth, J. M. (1992) · Drug Metabolism and Disposition · doi:10.1016/s0090-9556(25)08606-4 · Cited by 57 · see citing works
Shew, R. L., Robinson, M. F., Olansky, L., Yee, J. A., Titishov, N., Mechoulam, R., Zimmerman, A. M., Raghoebar, M., Tiemessen, H. L. G. M., van den Berg, W. B., et al. (2008) · Pharmacology · doi:10.1159/000138630 · Cited by 18 · see citing works
Invalidity of the Rao Map Function for Three Loci
Weeks, D. E. (1994) · Human Heredity · doi:10.1159/000154213 · Cited by 12 · see citing works
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Aging, energy, and oxidative stress in neurodegenerative diseases
Beal, M. F. (1995) · Annals of Neurology · doi:10.1002/ana.410380304 · Cited by 1,434 · see citing works
Self-citationGilgun‐Sherki, Y., Melamed, E., & Offen, D. (2001) · Neuropharmacology · doi:10.1016/s0028-3908(01)00019-3 · Cited by 839 · see citing works
Statin Adverse Effects: A Review of the Literature and Evidence for a Mitochondrial Mechanism
Golomb, B. A., & Evans, M. A. (2008) · American Journal of Cardiovascular Drugs · doi:10.2165/0129784-200808060-00004 · Cited by 677 · see citing works
Matthews, R. T., Yang, L., Browne, S., Baik, M., & Beal, M. F. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.15.8892 · Cited by 557 · see citing works
Self-citationMitochondria, Oxidative Damage, and Inflammation in Parkinson's Disease
Beal, M. F. (2003) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.2003.tb07470.x · Cited by 523 · see citing works
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Browne, S. E., Ferrante, R. J., & Beal, M. F. (1999) · Brain Pathology · doi:10.1111/j.1750-3639.1999.tb00216.x · Cited by 508 · see citing works
Self-citationEnergetics in the pathogenesis of neurodegenerative diseases
Beal, M. F. (2000) · Trends in Neurosciences · doi:10.1016/s0166-2236(00)01584-8 · Cited by 472 · see citing works
Self-citationModification of Brain Aging and Neurodegenerative Disorders by Genes, Diet, and Behavior
Mattson, M. P., Chan, S. L., & Duan, W. (2002) · Physiological Reviews · doi:10.1152/physrev.00004.2002 · Cited by 438 · 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
Self-citationFerrante, R. J., Andreassen, O. A., Dedeoglu, A., Ferrante, K. L., Jenkins, B. G., Hersch, S. M., & Beal, M. F. (2002) · The Journal of Neuroscience · doi:10.1523/jneurosci.22-05-01592.2002 · Cited by 391 · see citing works
Self-citationMitochondria in Neurodegeneration: Acute Ischemia and Chronic Neurodegenerative Diseases
Fiskum, G. M., Murphy, A. N., & Beal, M. F. (1999) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1097/00004647-199904000-00001 · Cited by 351 · see citing works
Self-citationRole of Oxidative Stress and Antioxidants in Neurodegenerative Diseases
Rao, A. V., & Balachandran, B. (2002) · Nutritional Neuroscience · doi:10.1080/1028415021000033767 · Cited by 337 · see citing works
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Alexi, T. (2000) · Progress in Neurobiology · doi:10.1016/s0301-0082(99)00032-5 · Cited by 290 · see citing works
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Self-citationPalfi, S., Ferrante, R. J., Brouillet, E., Beal, M. F., Dolan, R., Guyot, M., Peschanski, M., & Hantraye, P. (1996) · Journal of Neuroscience · doi:10.1523/jneurosci.16-09-03019.1996 · Cited by 259 · see citing works
Self-citationTarnopolsky, M. A., & Beal, M. F. (2001) · Annals of Neurology · doi:10.1002/ana.1028 · Cited by 244 · see citing works
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Self-citationYang, L., Calingasan, N. Y., Wille, E. J., Cormier, K., Smith, K., Ferrante, R. J., & Beal, M. F. (2009) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2009.06074.x · Cited by 232 · see citing works
Self-citationExperimental therapeutics in transgenic mouse models of Huntington's disease
Beal, M. F., & Ferrante, R. J. (2004) · Nature reviews. Neuroscience · doi:10.1038/nrn1386 · Cited by 224 · see citing works
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Fricker, R. A., Green, E. L., Jenkins, S. I., & Griffin, S. M. (2018) · International Journal of Tryptophan Research · doi:10.1177/1178646918776658 · Cited by 200 · see citing works
Ishrat, T., Khan, M. B., Hoda, M. N., Yousuf, S., Ahmad, M., Ansari, M. A., Ahmad, A. S., & Islam, F. (2006) · Behavioural Brain Research · doi:10.1016/j.bbr.2006.03.009 · Cited by 190 · see citing works
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Stack, E. C., Matson, W. R., & Ferrante, R. J. (2008) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1427.008 · Cited by 167 · see citing works
Coenzyme Q 10 as a Possible Treatment for Neurodegenerative Diseases
Beal, M. F. (2002) · Free Radical Research · doi:10.1080/10715760290021315 · Cited by 160 · see citing works
Self-citationAntioxidants in Huntington's disease
Johri, A., & Beal, M. F. (2011) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2011.11.014 · Cited by 158 · see citing works
Self-citationTherapeutic effects of coenzyme Q 10 (CoQ 10 ) and reduced CoQ 10 in the MPTP model of Parkinsonism
Cléren, C., Yang, L., Lorenzo, B. J., Calingasan, N. Y., Schomer, A. C., Sireci, A. N., Wille, E. J., & Beal, M. F. (2007) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2007.05097.x · Cited by 158 · see citing works
Self-citationSchulz, J. B., Matthews, R. T., Henshaw, D. R., & Beal, M. F. (1996) · Neuroscience · doi:10.1016/0306-4522(95)00527-7 · Cited by 155 · see citing works
Self-citationLibri, V., Yandım, C., Athanasopoulos, S., Loyse, N., Natisvili, T., Law, P. P., Chan, P. K., Mohammad, T., Mauri, M., Tam, K. T., et al. (2014) · The Lancet · doi:10.1016/s0140-6736(14)60382-2 · Cited by 152 · see citing works
Faust, K., Gehrke, S., Yang, Y., Yang, L., Beal, M. F., & Lu, B. (2009) · BMC Neuroscience · doi:10.1186/1471-2202-10-109 · Cited by 151 · see citing works
Self-citationMitochondrial Disorders: Clinical and Genetic Features
Simon, D. K., & Johns, D. R. (1999) · Annual Review of Medicine · doi:10.1146/annurev.med.50.1.111 · Cited by 150 · see citing works
Walter, L., Nogueira, V., Leverve, X. M., Heitz, M., Bernardi, P., & Fontaine, E. M. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.m004128200 · Cited by 146 · see citing works
Fontaine, E. M., & Bernardi, P. (1999) · Journal of Bioenergetics and Biomembranes · doi:10.1023/a:1005475802350 · Cited by 146 · see citing works
A randomized, double-blind, placebo-controlled trial of coenzyme Q10 in Huntington disease
McGarry, A. J., McDermott, M., Kieburtz, K. D., de Blieck, E. A., Beal, F. M., Marder, K. S., Ross, C. A., Shoulson, I., Gilbert, P. R., Mallonee, W. M., et al. (2016) · Neurology · doi:10.1212/wnl.0000000000003478 · Cited by 139 · see citing works
Oxidative Stress and Cognitive Decline: The Neuroprotective Role of Natural Antioxidants
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Gómez-Lázaro, M., Galindo, M. F., de Mera, R. M. M.-F., Fernández-Gómez, F.-J., Concannon, C. G., Segura, M. F., Comella, J. X., Prehn, J. H. M., & Jordán, J. (2006) · Molecular Pharmacology · doi:10.1124/mol.106.030718 · Cited by 136 · see citing works
Mokudai, T., Ayoub, I. A., Sakakibara, Y., Lee, E.-J., Ogilvy, C. S., & Maynard, K. I. (2000) · Stroke · doi:10.1161/01.str.31.7.1679 · Cited by 131 · see citing works
Topical Review: Glutamate in Neurologic Diseases
Bittigau, P., & Ikonomidou, C. (1997) · Journal of Child Neurology · doi:10.1177/088307389701200802 · Cited by 131 · see citing works
Application of MRS to mouse models of neurodegenerative illness
Choi, J., Dedeoglu, A., & Jenkins, B. G. (2007) · NMR in Biomedicine · doi:10.1002/nbm.1145 · Cited by 126 · see citing works
Self-citationMitochondria as targets for chemotherapy
Gogvadze, V. G., Orrenius, S., & Zhivotovsky, B. (2009) · APOPTOSIS · doi:10.1007/s10495-009-0323-0 · Cited by 125 · see citing works
Coenzyme Q10 Combined With Mild Hypothermia After Cardiac Arrest
Damian, M. S., Ellenberg, D., Gildemeister, R., Lauermann, J., Simonis, G., Sauter, W., & Georgi, C. (2004) · Circulation · doi:10.1161/01.cir.0000146894.45533.c2 · Cited by 113 · see citing works
Martinelli, C., Pucci, C., Battaglini, M., Marino, A., & Ciofani, G. (2019) · Advanced Healthcare Materials · doi:10.1002/adhm.201901589 · Cited by 111 · see citing works
Coenzyme Q10administration and its potential for treatment of neurodegenerative diseases
Beal, M. F. (1999) · BioFactors · doi:10.1002/biof.5520090222 · Cited by 111 · see citing works
Self-citationAyoub, I. A., Lee, E. J., Ogilvy, C. S., Beal, M. F., & Maynard, K. I. (1999) · Neuroscience Letters · doi:10.1016/s0304-3940(98)00881-7 · Cited by 107 · see citing works
Self-citationMethylmalonate toxicity in primary neuronal cultures
McLaughlin, B., NELSON, D. S., Silver, I. A., Erecińska, M., & Chesselet, M. ‐. (1998) · Neuroscience · doi:10.1016/s0306-4522(97)00594-0 · Cited by 107 · see citing works
McBride, J. L., Ramaswamy, S., Gasmi, M., Bartus, R. T., Herzog, C. D., Brandon, E. P., Zhou, L., Pitzer, M. R., Berry‐Kravis, E., & Kordower, J. H. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0508875103 · Cited by 106 · see citing works
Abdelgawad, H. M., & Khalifa, A. E. (2001) · Pharmacological Research · doi:10.1006/phrs.2000.0781 · Cited by 104 · see citing works
Coenzyme Q10 and nicotinamide and a free radical spin trap protect against MPTP neurotoxicity
Schulz, J. B., Ross Henshaw, D., Matthews, R. T., & Flint Beal, M. (1995) · Experimental Neurology · doi:10.1016/0014-4886(95)90033-0 · Cited by 103 · see citing works
Self-citationThe Role of Mitochondria in the Pathogenesis of Neurodegenerative Diseases
Manfredim, G., & Beal, M. F. (2000) · Brain Pathology · doi:10.1111/j.1750-3639.2000.tb00278.x · Cited by 101 · see citing works
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Ross, C. A., Margolis, R. L., Rosenblatt, A., Ranen, N. G., Bêcher, M. W., & Aylward, E. (1997) · Medicine · doi:10.1097/00005792-199709000-00001 · Cited by 101 · see citing works
Bioenergetics in Huntington's Disease
Grünewald, T. G. P., & Beal, M. F. (1999) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.1999.tb07827.x · Cited by 100 · see citing works
Self-citationOlivieri, G., Baysang, G., Meier, F., Müller-Spahn, F., Stähelin, H. B., Brockhaus, M., & Brack, C. (2001) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2001.00090.x · Cited by 98 · see citing works
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Kandhare, A. D., Ghosh, P., Ghule, A. E., & Bodhankar, S. L. (2012) · Fundamental and Clinical Pharmacology · doi:10.1111/fcp.12003 · Cited by 91 · see citing works
Nicotinamide therapy protects against both necrosis and apoptosis in a stroke model
Yang, J., Klaidman, L. K., Chang, M. L., Kem, S., Sugawara, T., Chan, P. H., & Adams, J. D., Jr. (2002) · Pharmacology Biochemistry and Behavior · doi:10.1016/s0091-3057(02)00939-5 · Cited by 90 · see citing works
Maciel, E. N., Kowaltowski, A. J., Schwalm, F. D., Rodrigues, J. M., Souza, D. O., Vercesi, A. E., Wajner, M., & Castilho, R. F. (2004) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2004.02565.x · Cited by 82 · see citing works
Menke, T., Niklowitz, P., Adam, S., Weber, M. A., Schlüter, B., & Andler, W. (2000) · Analytical Biochemistry · doi:10.1006/abio.2000.4579 · Cited by 79 · see citing works
Matthews, R. T., Yang, L., & Beal, M. F. (1997) · Experimental Neurology · doi:10.1006/exnr.1996.6406 · Cited by 79 · see citing works
Self-citationCoenzyme Q treatment of neurodegenerative diseases of aging
Galpern, W. R., & Cudkowicz, M. (2007) · Mitochondrion · doi:10.1016/j.mito.2007.01.004 · Cited by 78 · see citing works
Coenzyme Q10 a mitochondrial restorer for various brain disorders
Pradhan, N., Singh, C., & Singh, A. (2021) · Naunyn-Schmiedeberg s Archives of Pharmacology · doi:10.1007/s00210-021-02161-8 · Cited by 77 · see citing works
Sakakibara, Y., Mitha, A. P., Ogilvy, C. S., & Maynard, K. I. (2000) · Neuroscience Letters · doi:10.1016/s0304-3940(00)00854-5 · Cited by 76 · see citing works
Neuroprotective effects of creatine administration against NMDA and malonate toxicity
Malcon, C., Kaddurah-Daouk, R. F., & Beal, M. F. (2000) · Brain Research · doi:10.1016/s0006-8993(00)02038-2 · Cited by 76 · see citing works
Self-citationChan, P. K., Torres, R., Yandım, C., Law, P. P., Khadayate, S. P., Mauri, M., Groşan, C., Chapman‐Rothe, N., Giunti, P., Pook, M. A., et al. (2013) · Human Molecular Genetics · doi:10.1093/hmg/ddt115 · Cited by 75 · see citing works
Blum, D. L., Gall, D., Galas, M., d’Alcantara, P., Bantubungi, K., & Schiffmann, S. N. (2002) · Journal of Neuroscience · doi:10.1523/jneurosci.22-20-09122.2002 · Cited by 73 · see citing works
Tavares, R. G., dos Santos, C. E. S., Tasca, C. I., Wajner, M., Souza, D. O., & Dutra‐Filho, C. S. (2000) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(00)00402-0 · Cited by 72 · see citing works
Lee, W., & Chang, C. (2004) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2004.02.002 · Cited by 67 · see citing works
Sadanaga, F., Yao, H., Tanuma, S., Nakahara, T., Hong, J. S., Ibayashi, S., Uchimura, H., & Fujishima, M. (2003) · Neurochemical Research · doi:10.1023/a:1024236614015 · Cited by 65 · see citing works
El-Abhar, H. S. (2010) · European Journal of Pharmacology · doi:10.1016/j.ejphar.2010.09.012 · Cited by 63 · see citing works
Huntington's Disease and Dentatorubral‐Pallidoluysian Atrophy: Proteins, Pathogenesis and Pathology
Ross, C. A., Bêcher, M. W., Colomer, V., Engelender, S., Wood, J. D., & Sham, A. H. (1997) · Brain Pathology · doi:10.1111/j.1750-3639.1997.tb00898.x · Cited by 63 · 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
Sax, D. S., Powsner, R. A., Kim, A., Tilak, S., Bhatia, R. G., Cupples, L. A., & Myers, R. H. (1996) · Movement Disorders · doi:10.1002/mds.870110612 · Cited by 61 · see citing works
Protective Effect of L-Carnitine and Coenzyme Q10 on CCl4-Induced Liver Injury in Rats
Ali, S. A. (2010) · Scientia Pharmaceutica · doi:10.3797/scipharm.1006-02 · Cited by 59 · see citing works
Effects of Coenzyme Q10 in Huntington's disease and early Parkinson's disease
Beal, M. F., & Shults, C. W. (2003) · BioFactors · doi:10.1002/biof.5520180218 · Cited by 58 · see citing works
Self-citationBeal, M. F., & Matthews, R. T. (1997) · Molecular Aspects of Medicine · doi:10.1016/s0098-2997(97)00024-1 · Cited by 58 · see citing works
Self-citationZhang, Y. P., Song, C. Y., Yuan, Y., Eber, A. E., Rodriguez, Y. F., Levitt, R. C., Takács, P., Yang, Z., Goldberg, R. B., & Candiotti, K. A. (2013) · Neurobiology of Disease · doi:10.1016/j.nbd.2013.05.003 · Cited by 57 · see citing works
Burrows, K. B., Nixdorf, W. L., & Yamamoto, B. K. (2000) · Journal of Pharmacology and Experimental Therapeutics · doi:10.1016/s0022-3565(24)35363-7 · Cited by 57 · see citing works
MPP+Produces Progressive Neuronal Degeneration Which Is Mediated by Oxidative Stress
Fallon, J., Matthews, R. T., Hyman, B. T., & Beal, M. F. (1997) · Experimental Neurology · doi:10.1006/exnr.1997.6416 · Cited by 57 · see citing works
Self-citationKONES, R. J. (2010) · Nutrition in Clinical Practice · doi:10.1177/0884533610373932 · Cited by 55 · see citing works
Mendelsohn, B. A., Bennett, N. K., Darch, M. A., Yu, K., Nguyen, M. K., Pucciarelli, D., Nelson, M. R., Horlbeck, M. A., Gilbert, L. A., Hyun, W. C., et al. (2018) · PLoS Biology · doi:10.1371/journal.pbio.2004624 · Cited by 55 · see citing works
Mitochondrial Toxin 3-Nitropropionic Acid Induces Cardiac and Neurotoxicity Differentially in Mice
Gabrielson, K. L., Hogue, B. A., Bohr, V. A., Cardounel, A. J., Nakajima, W., Kofler, J., Zweier, J. L., Rodríguez, E. R., Martin, L. J., de Souza-Pinto, N. C., et al. (2001) · American Journal Of Pathology · doi:10.1016/s0002-9440(10)62536-9 · Cited by 54 · see citing works
Shen, C., Huang, H., Ou, H., Chen, H., Chen, W., & Jeng, K.-C. G. (2004) · Journal of Biomedical Science · doi:10.1007/bf02256096 · Cited by 54 · see citing works
Therapeutic Effects of Coenzyme Q10 in Neurodegenerative Diseases
Beal, M. F. (2004) · Methods in enzymology on CD-ROM/Methods in enzymology · doi:10.1016/s0076-6879(04)82026-3 · Cited by 54 · see citing works
Self-citationFighera, M. R., Bonini, J. S., de Oliveira, T. G., Frussa‐Filho, R., da Rocha, J. B. T., Dutra‐Filho, C. S., Rubin, M. A., & Mello, C. F. (2003) · The International Journal of Biochemistry & Cell Biology · doi:10.1016/s1357-2725(02)00275-3 · Cited by 53 · see citing works
Lloret, A., & Beal, M. F. (2019) · Neurochemical Research · doi:10.1007/s11064-019-02809-1 · Cited by 52 · see citing works
Self-citationNarasimhan, P., Sklar, R., Murrell, M. T., Swanson, R. A., & Sharp, F. R. (1996) · Journal of Neuroscience · doi:10.1523/jneurosci.16-22-07336.1996 · Cited by 51 · see citing works
Fredriksson, A., Eriksson, P., & Archer, T. (1997) · Journal of Neural Transmission · doi:10.1007/bf01291877 · Cited by 50 · see citing works
Mitochondrial encephalomyopathies: what next?
DiMauro, S. (1996) · Journal of Inherited Metabolic Disease · doi:10.1007/bf01799110 · Cited by 50 · see citing works
Attenuating effects of coenzyme Q10 and amlodipine in ulcerative colitis model in rats
Morsy, E. M. E., Kamel, R., & Ahmed, M. A. E. (2015) · Immunopharmacology and Immunotoxicology · doi:10.3109/08923973.2015.1021357 · Cited by 49 · see citing works
The failure of mitochondria leads to neurodegeneration: Do mitochondria need a jump start?☆
Lee, J.-H., Boo, J. H., & Ryu, H. (2009) · Advanced Drug Delivery Reviews · doi:10.1016/j.addr.2009.07.016 · Cited by 49 · see citing works
Malfatti, C. R. M., Royes, L. F. F., Francescato, L. N., Sanabria, E., Rubin, M. A., Cavalheiro, É. A., & Mello, C. F. (2003) · Epilepsia · doi:10.1046/j.1528-1157.2003.42902.x · Cited by 49 · see citing works
Sawa, A. (2001) · Journal of Molecular Medicine · doi:10.1007/s001090100223 · Cited by 49 · see citing works
Neuroprotective Effects of Thymoquinone on the Hippocampus in a Rat Model of Traumatic Brain Injury
Gülşen, İ., Ak, H., Çölçimen, N., Hakan, H., Akyol, M. E., Demır, İ., Atalay, T., Balahroğlu, R., & Rağbetli̇, M. Ç. (2015) · World Neurosurgery · doi:10.1016/j.wneu.2015.09.052 · Cited by 48 · see citing works
Shetty, R. A., Forster, M. J., & Sumien, N. (2013) · AGE · doi:10.1007/s11357-012-9484-9 · Cited by 47 · see citing works
Q10 therapy in patients with idiopathic Parkinson's disease
Strijks, E., Kremer, H. P. H., & Horstink, M. W. I. M. (1997) · Molecular Aspects of Medicine · doi:10.1016/s0098-2997(97)00008-3 · Cited by 45 · see citing works
Mitochondrial matters of the brain: the role in Huntington’s disease
Turner, C., & Schapira, A. H. V. (2010) · Journal of Bioenergetics and Biomembranes · doi:10.1007/s10863-010-9290-y · Cited by 44 · see citing works
Evidence for a role of energy dysregulation in the MDMA-induced depletion of brain 5-HT
Darvesh, A. S., & Gudelsky, G. A. (2005) · Brain Research · doi:10.1016/j.brainres.2005.07.009 · Cited by 44 · see citing works
Antiglutamate therapies in Huntington’s disease
Kieburtz, K. D. (1999) · Springer eBooks · doi:10.1007/978-3-7091-6369-6_9 · Cited by 44 · see citing works
- Authors
- Beal, M. F., Henshaw, D. R., Jenkins, B. G., Rosen, B. R., & Schulz, J. B.
- Affiliations
- Massachusetts General Hospital (US)
- Harvard University (US)
- Corresponding author
- Beal, M. Flint
- ORCID
- Rosen, Bruce R.
- Venue
- Annals of Neurology
- Publisher
- Wiley
- Published
- 1994-12
- Volume
- 36
- Issue
- 6
- Pages
- 882-888
- ISSN
- 0364-5134 (ISSN-L)
- 1531-8249
- OpenAlex
- W1965876571
- Cited by
- 189
- References
- 35
- Citations by year
- 2026: 1 · 2024: 5 · 2022: 1 · 2021: 4 · 2020: 3 · 2019: 5 · 2018: 2 · 2017: 1 · 2016: 3 · 2015: 3 · 2014: 2 · 2013: 2 · 2012: 6
- FWCI
- 2.78
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Annals of Neurology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (5p01ns031579-05)
- U.S. Public Health Service (16367)
- National Institute of Neurological Disorders and Stroke (NS10828, NS31579)
- 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 › Neuroscience › Neurology › Neurological Disorders and Treatments
- Keywords
- coenzyme Q10, nicotinamide, malonate, mitochondrial toxins, striatal lesions, excitotoxicity, neuroprotection, mitochondrial bioenergetics, electron transport inhibition, complex II inhibition, ATP depletion, lactate, mitochondrial encephalopathy, neurodegenerative diseases
- MeSH terms
- Animals, Coenzymes, Corpus Striatum, Dose-Response Relationship, Drug, Magnetic Resonance Imaging, Male, Malonates, Mitochondria, Niacinamide, Ubiquinone, Rats, Sprague-Dawley, Rats
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