The DOI matches its Crossref record; 62 of the 62 references it cites were found in a registry or an index.
Neuroprotective Effects of Creatine in a Transgenic Mouse Model of Huntington's Disease
Open accessAbstract
Huntington's disease (HD) is a progressive neurodegenerative illness for which there is no effective therapy. We examined whether creatine, which may exert neuroprotective effects by increasing phosphocreatine levels or by stabilizing the mitochondrial permeability transition, has beneficial effects in a transgenic mouse model of HD (line 6/2). Dietary creatine supplementation significantly improved survival, slowed the development of brain atrophy, and delayed atrophy of striatal neurons and the formation of huntingtin-positive aggregates in R6/2 mice. Body weight and motor performance on the rotarod test were significantly improved in creatine-supplemented R6/2 mice, whereas the onset of diabetes was markedly delayed. Nuclear magnetic resonance spectroscopy showed that creatine supplementation significantly increased brain creatine concentrations and delayed decreases in N-acetylaspartate concentrations. These results support a role of metabolic dysfunction in a transgenic mouse model of HD and suggest a novel therapeutic strategy to slow the pathological process.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Creatine significantly improves survival, improves motor performance, slows the development of brain atrophy, increases brain ATP levels, delays atrophy of striatal neurons and the formation of Htt-positive aggregates in the R6/2 and N-171-82Q transgenic mouse models of HD.
“We found that creatine significantly improves survival, improves motor performance, slows the development of brain atrophy, increases brain ATP levels, delays atrophy of striatal neurons and the formation of Htt-positive aggregates in the R6/2 and N-171-82Q transgenic mouse models of HD118-120.”
From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [120]
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
Cites
The Mouse Brain in Stereotaxic Coordinates
Paxinos, G. (2001) · Cited by 12,859
MACDONALD, M. (1993) · Cell · doi:10.1016/0092-8674(93)90585-e · Cited by 8,366
Mangiarini, L., Sathasivam, K., Seller, M., Cozens, B., Harper, A., Hetherington, C., Lawton, M., Trottier, Y., Lehrach, H., Davies, S. W., et al. (1996) · Cell · doi:10.1016/s0092-8674(00)81369-0 · Cited by 3,073
Shah, M. A., Datson, N. A., Srinidhi, L., Stanton, V. P., MacDonald, M. E., Allard, M. W., Youngman, S., Am, F., Mott, R., Draths, K., et al. (1993) · Cell · Cited by 3,055
Self-citationAggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain
DiFiglia, M., Sapp, E., Chase, K. O., Davies, S. W., Bates, G. P., Vonsattel, J. P., & Aronin, N. (1997) · Science · doi:10.1126/science.277.5334.1990 · Cited by 2,439
Neuropathological Classification of Huntingtonʼs Disease
VONSATTEL, J.-P., MYERS, R. H., STEVENS, T. J., FERRANTE, R. J., BIRD, E. D., & RICHARDSON, E. P. (1985) · Journal of Neuropathology and Experimental Neurology · doi:10.1097/00005072-198511000-00003 · Cited by 2,227
Self-citationDavies, S. W., Turmaine, M., Cozens, B. A., DiFiglia, M., Sharp, A. H., Ross, C. A., Scherzinger, E., Wanker, E. E., Mangiarini, L., & Bates, G. P. (1997) · Cell · doi:10.1016/s0092-8674(00)80513-9 · Cited by 2,188
Wallimann, T., Wyss, M., Brdiczka, D., Nicolay, K., & Eppenberger, H. M. (1992) · Biochemical Journal · doi:10.1042/bj2810021 · Cited by 2,011
Spatial Localization in NMR Spectroscopy in Vivo
Bottomley, P. A. (1987) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.1987.tb32915.x · Cited by 1,382
Nuclear and Neuropil Aggregates in Huntington’s Disease: Relationship to Neuropathology
Gutekunst, C.-A., Li, S.-H., Yi, H., Mulroy, J. S., Kuemmerle, S., Jones, R., Rye, D., Ferrante, R. J., Hersch, S. M., & Li, X.-J. (1999) · The Journal of Neuroscience · doi:10.1523/jneurosci.19-07-02522.1999 · Cited by 914
Self-citationBrowne, S. E., Bowling, A. C., Macgarvey, U., Baik, M. J., Berger, S. C., Muquit, M. M. K., Bird, E. D., & Beal, M. F. (1997) · Annals of Neurology · doi:10.1002/ana.410410514 · Cited by 885
Self-citationHodgson, J., Agopyan, N., Gutekunst, C., Leavitt, B. R., LePiane, F. G., Singaraja, R. R., Smith, D. J., Bissada, N., McCutcheon, K. M., Nasir, J., et al. (1999) · Neuron · doi:10.1016/s0896-6273(00)80764-3 · Cited by 851
Self-citationMitochondrial defect in Huntington's disease caudate nucleus
Gu, M., Gash, M. T., Mann, V. M., Javoy‐Agid, F., Cooper, J. M., & Schapira, A. H. V. (1996) · Annals of Neurology · doi:10.1002/ana.410390317 · Cited by 758
Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis
Klivenyi, P., Ferrante, R. J., Matthews, R. T., Bogdanov, M. B., Klein, A. M., Andreassen, O. A., Mueller, G., Wermer, M., Kaddurah-Daouk, R., & Beal, M. F. (1999) · Nature Medicine · doi:10.1038/6568 · Cited by 682
Self-citationInhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
Ona, V. O., Li, M., Vonsattel, J. P., Andrews, L. J., Khan, S. Q., Chung, W. M., Frey, A. S., Menon, A., Li, X., Stieg, P. E., et al. (1999) · Nature · doi:10.1038/20446 · Cited by 640
Sawa, A., Wiegand, G. W., Cooper, J. K., Margolis, R. L., Sharp, A. H., Lawler, J. F., Greenamyre, J. T., Snyder, S. H., & Ross, C. A. (1999) · Nature Medicine · doi:10.1038/13518 · Cited by 557
Widespread expression of Huntington's disease gene (IT15) protein product
Sharp, A. H., Loev, S. J., Schilling, G., Li, S.-H., Li, X.-J., Bao, J., Wagster, M. V., Kotzuk, J. A., Steiner, J. P., Lo, A., et al. (1995) · Neuron · doi:10.1016/0896-6273(95)90345-3 · Cited by 516
Cha, J.-H. J., Kosinski, C. M., Kerner, J. A., Alsdorf, S. A., Mangiarini, L., Davies, S. W., Penney, J. B., Bates, G. P., & Young, A. B. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.11.6480 · Cited by 504
Brouillet, E., Hantraye, P., Ferrante, R. J., Dolan, R., Leroy‐Willig, A., Kowall, N. W., & Beal, M. F. (1995) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.92.15.7105 · Cited by 485
Self-citationJenkins, B. G., Koroshetz, W. J., Beal, M. F., & Rosen, B. R. (1993) · Neurology · doi:10.1212/wnl.43.12.2689 · Cited by 479
Self-citationNeuroprotective Effects of Creatine and Cyclocreatine in Animal Models of Huntington’s Disease
Matthews, R. T., Yang, L., Jenkins, B. G., Ferrante, R. J., Rosen, B. R., Kaddurah-Daouk, R. F., & Beal, M. F. (1998) · Journal of Neuroscience · doi:10.1523/jneurosci.18-01-00156.1998 · Cited by 431
Self-citationInhibition of N-acetylaspartate production: implications for 1H MRS studies in vivo.
Bates, T. E., Strangward, M., Keelan, J., Davey, G. P., Munro, P., & Clark, J. B. (1996) · PubMed · Cited by 422
Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues
Strong, T. V., Tagle, D. A., Valdes, J. M., Elmer, L. W., Boehm, K., Swaroop, M., Kaatz, K. W., Collins, F. S., & Albin, R. L. (1993) · Nature Genetics · doi:10.1038/ng1193-259 · Cited by 417
Creatine deficiency in the brain: A new, treatable inborn error of metabolism
Stöckler, S., Holzbach, U., Hanefeld, F., Marquardt, I., Helms, G., Requart, M., Hänicke, W., & Frahm, J. (1994) · Pediatric Research · doi:10.1203/00006450-199409000-00023 · Cited by 417
Energy metabolism defects in Huntington's disease and effects of coenzyme Q10
Koroshetz, W. J., Jenkins, B. G., Rosen, B. R., & Beal, M. F. (1997) · Annals of Neurology · doi:10.1002/ana.410410206 · Cited by 405
Self-citationCreatine in humans with special reference to creatine supplementation
Balsom, P. D., Söderlund, K., & Ekblom, B. (1994) · Sports Medicine · doi:10.2165/00007256-199418040-00005 · Cited by 404 · cited 1×
“Creatine administration is well tolerated in man, results in increased PCr levels, and may have benefits in pathological conditions (Balsom et al., 1994; Dawson et al., 1995; Greenhaff, 1997).”
BackgroundReddy, P. H., Williams, M., Charles, V., Garrett, L. J., Pike-Buchanan, L., Whetsell, W. O., Miller, G., & Tagle, D. A. (1998) · Nature Genetics · doi:10.1038/2510 · Cited by 399
Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse
Wheeler, V. C. (1999) · Human Molecular Genetics · doi:10.1093/hmg/8.1.115 · Cited by 395
Creatine and Cyclocreatine Attenuate MPTP Neurotoxicity
Matthews, R. T., Ferrante, R. J., Klivènyi, P., Yang, L., Klein, A., Mueller, G., Kaddurah-Daouk, R. F., & Beal, M. F. (1999) · Experimental Neurology · doi:10.1006/exnr.1999.7049 · Cited by 374
Self-citationInhibition of N-acetylaspartate production
Bates, T. E., Strangward, M., Keelan, J., Davey, G. P., Munro, P., & Clark, J. B. (1996) · Neuroreport · doi:10.1097/00001756-199605310-00014 · Cited by 365
Huntington aggregates may not predict neuronal death in Huntington's disease.
Kuemmerle, S., Gutekunst, C., Klein, A., Li, X. J., Li, S. H., Beal, M. F., Hersch, S. M., & Ferrante, R. J. (1999) · PubMed · Cited by 362
Self-citationApoptosis, Excitotoxicity, and Neuropathology
Leist, M., & Nicotera, P. (1998) · Experimental Cell Research · doi:10.1006/excr.1997.4026 · Cited by 287
Steeghs, K., Benders, A. A. G. M., Oerlemans, F. T. J. J., HAAN, A. D., Heerschap, A., Ruitenbeek, W., Jost, C. R., van Deursen, J., Perryman, B., Pette, D., et al. (1997) · Cell · doi:10.1016/s0092-8674(00)80186-5 · Cited by 252
A randomized, controlled trial of creatine monohydrate in patients with mitochondrial cytopathies
Tarnopolsky, M. A., Roy, B. D., & MacDonald, J. R. (1997) · Muscle & Nerve · doi:10.1002/(sici)1097-4598(199712)20:12 · Cited by 246
The role of creatine kinase in inhibition of mitochondrial permeability transition
O’Gorman, E., Beutner, G., Dolder, M., Koretsky, A. P., Brdiczka, D. G., & Wallimann, T. (1997) · FEBS Letters · doi:10.1016/s0014-5793(97)01045-4 · Cited by 243
1 H NMR spectroscopy studies of Huntington's disease
Jenkins, B. G., Rosas, H. D., Chen, Y. I., Makabe, T., Myers, R. H., MacDonald, M. E., Rosen, B. R., Beal, M. F., & Koroshetz, W. J. (1998) · Neurology · doi:10.1212/wnl.50.5.1357 · Cited by 241
Self-citationStöckler, S., Hanefeld, F., & Frahm, J. (1996) · The Lancet · doi:10.1016/s0140-6736(96)04116-5 · Cited by 240
Landwehrmeyer, G. B., McNeil, S. M., Dure, L. S., Ge, P., Aizawa, H., Huang, Q. M., Ambrose, C. M., Duyao, M. P., Bird, E. D., Bonilla, E., et al. (1995) · Annals of Neurology · doi:10.1002/ana.410370213 · Cited by 222
Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes.
Hurlbert, M. S., Zhou, W., Wasmeier, C., Kaddis, F. G., Hutton, J. C., & Freed, C. R. (1999) · Diabetes · doi:10.2337/diabetes.48.3.649 · Cited by 218
Creatine monohydrate increases strength in patients with neuromuscular disease
Tarnopolsky, M. A., & Martin, J. C. (1999) · Neurology · doi:10.1212/wnl.52.4.854 · Cited by 209
Brouillet, M. (1995) · Medical Entomology and Zoology · Cited by 190
Stachowiak, O., Dolder, M., Wallimann, T., & Richter, C. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.27.16694 · Cited by 189
Supplementary creatine as a treatment for gyrate atrophy of the choroid and retina
Sipilä, I., Rapola, J., Simell, O., & Vannas, A. (1981) · New England Journal of Medicine · doi:10.1056/nejm198104093041503 · Cited by 181 · cited 2×
“Long-term administration to patients with gyrate atrophy of the choroid in the retina prevented visual field constriction and resulted in improvement of muscle biopsy findings ( Sipila et al., 1981 ).”
BackgroundJenkins, B. G., Klivènyi, P., Küstermann, E., Andreassen, O. A., Ferrante, R. J., Rosen, B. R., & Beal, M. F. (2000) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2000.0742108.x · Cited by 170
Self-citationFunctional Aspects of Creatine Kinase in Brain
Hemmer, W., & Wallimann, T. (1994) · Developmental Neuroscience · doi:10.1159/000111342 · Cited by 166
Ferrante, R. J., Gutekunst, C., Persichetti, F., McNeil, S. M., Kowall, N. W., Gusella, J. F., MacDonald, M. E., Beal, M. F., & Hersch, S. M. (1997) · Journal of Neuroscience · doi:10.1523/jneurosci.17-09-03052.1997 · Cited by 162
Self-citationBrdiczka, D. G., Beutner, G., Rück, A., Dolder, M., & Wallimann, T. (1998) · BioFactors · doi:10.1002/biof.5520080311 · Cited by 138
O’Gorman, E., Beutner, G., Wallimann, T., & Brdiczka, D. G. (1996) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/0005-2728(96)00074-6 · Cited by 135
The nutritional biochemistry of creatine
Greenhaff, P. L. (1997) · The Journal of Nutritional Biochemistry · doi:10.1016/s0955-2863(97)00116-2 · Cited by 121
Sauter, A., & Rudin, M. (1993) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)38633-8 · Cited by 110
Phosphocreatine-dependent Glutamate Uptake by Synaptic Vesicles
Xu, C. J., Klunk, W. E., Kanfer, J. N., Xiong, Q. P., Miller, G., & Pettegrew, J. W. (1996) · Journal of Biological Chemistry · doi:10.1074/jbc.271.23.13435 · Cited by 95
Increased Vulnerability to 3‐Nitropropionic Acid in an Animal Model of Huntington's Disease
Bogdanov, M. B., Ferrante, R. J., Kuemmerle, S., Klivènyi, P., & Beal, M. F. (1998) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.1998.71062642.x · Cited by 90
Self-citationCarter, A. J., MULLER, R. E., Pschorn, U., & Stransky, W. (1995) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.1995.64062691.x · Cited by 83
KIAA0369, doublecortin-like kinase, is expressed during brain development
Burgess, H. A., Martı́nez, S., & Reiner, O. (1999) · Journal of Neuroscience Research · doi:10.1002/(sici)1097-4547(19991115)58:4 · Cited by 79
Deshpande, S. B., Fukuda, A., & Nishino, H. (1997) · Experimental Neurology · doi:10.1006/exnr.1997.6457 · Cited by 69
Neuroprotective effects of gangliosides may involve inhibition of nitric oxide synthase
Dawson, T. M., Hung, K. E., Dawson, V. L., Steiner, J. P., & Snyder, S. H. (1995) · Annals of Neurology · doi:10.1002/ana.410370122 · Cited by 69
Octamer-dimer Transitions of Mitochondrial Creatine Kinase in Heart Disease
Soboll, S., Brdiczka, D. G., Jahnke, D., Schmidt, A., Schlattner, U., Wendt, S., Wyss, M., & Wallimann, T. (1999) · Journal of Molecular and Cellular Cardiology · doi:10.1006/jmcc.1998.0925 · Cited by 55
Dunant, Y., Loctin, F., Marsal, J., Müller, D., Párducz, Á., & Rabasseda, X. (1988) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1988.tb02930.x · Cited by 39
Perspectives on the mitochondrial permeability transition
Bernardi, P., Basso, E., Colonna, R., Costantini, P., Lisa, F. D., Eriksson, O., Fontaine, E. M., Forte, M. A., Ichas, F., Massari, S., et al. (1998) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/s0005-2728(98)00069-3 · Cited by 37
Corbett, R. J. T., & Laptook, A. R. (1994) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1038/jcbfm.1994.140 · Cited by 25
■ REVIEW : Excitotoxicity, Free Radicals, Necrosis, and Apoptosis
Lipton, S. A., & Nicotera, P. (1998) · The Neuroscientist · doi:10.1177/107385849800400516 · Cited by 11
- Self-citation
Flagged first, then by citations
Research works printed but not linked: 1
- Gutekunst, 1996, Mitochondrial calcium homeostasis in Huntington's disease fibroblasts., Soc Neurosci Abstr, 22, 227
Cited by
Sorted by citations
N-Acetylaspartate in the CNS: From neurodiagnostics to neurobiology
MOFFETT, J., Ross, B., Arun, P., Madhavarao, C. N., & Namboodiri, A. M. (2007) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2006.12.003 · Cited by 1,443
Cui, L., Jeong, H., Borovecki, F., Parkhurst, C. N., Tanese, N., & Krainc, D. (2006) · Cell · doi:10.1016/j.cell.2006.09.015 · Cited by 1,012
Apoptosis and Caspases in Neurodegenerative Diseases
Friedlander, R. M. (2003) · New England Journal of Medicine · doi:10.1056/nejmra022366 · Cited by 873
Kreider, R. B., Kalman, D. S., Antonio, J., Ziegenfuss, T. N., Wildman, R., Collins, R., Candow, D. G., Kleiner, S. M., Almada, A. L., & Lopez, H. L. (2017) · Journal of the International Society of Sports Nutrition · doi:10.1186/s12970-017-0173-z · Cited by 1,057
Hockly, E., Richon, V. M., Woodman, B., Smith, D. L., Zhou, X., Rosa, E., Sathasivam, K., Ghazi‐Noori, S., Mahal, A., Lowden, P. A. S., et al. (2003) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0437870100 · Cited by 847
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease
Slow, E. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg169 · Cited by 821
Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease
Tanaka, M., Machida, Y., Niu, S., Ikeda, T., Jana, N. R., Doi, H., Kurosawa, M., Nekooki, M., & Nukina, N. (2004) · Nature Medicine · doi:10.1038/nm985 · Cited by 777
Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease
Zuccato, C., Valenza, M., & Cattaneo, E. (2010) · Physiological Reviews · doi:10.1152/physrev.00041.2009 · Cited by 848
Ferrante, R. J., Kubilus, J. K., Lee, J.-H., Ryu, H., Beesen, A. A., Zucker, B., Smith, K., Kowall, N. W., Ratan, R. R., Luthi‐Carter, R., et al. (2003) · Journal of Neuroscience · doi:10.1523/jneurosci.23-28-09418.2003 · Cited by 705
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
Self-citationStork, C., & Renshaw, P. F. (2005) · Molecular Psychiatry · doi:10.1038/sj.mp.4001711 · Cited by 489
Energetics in the pathogenesis of neurodegenerative diseases
Beal, M. F. (2000) · Trends in Neurosciences · doi:10.1016/s0166-2236(00)01584-8 · Cited by 475
Self-citationThe Role of Autophagy in Parkinson's DiseasePreprint
Lynch-Day, M. A., Mao, K., Wang, K., Zhao, M., & Klionsky, D. J. (2012) · Cold Spring Harbor Perspectives in Medicine · doi:10.1101/cshperspect.a009357 · Cited by 477
The NINDS NET-PD Investigators (2006) · Neurology · doi:10.1212/01.wnl.0000201252.57661.e1 · Cited by 453
Jana, N. R. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.10.1049 · Cited by 453
Oxidative stress and mitochondrial dysfunction in neurodegenerative diseases
Trushina, E. V., & McMurray, C. T. (2007) · Neuroscience · doi:10.1016/j.neuroscience.2006.10.056 · Cited by 451
de Vries, H. E., Witte, M. E., Hondius, D., Rozemüller, A. J. M., Drukarch, B., Hoozemans, J. J. M., & van Horssen, J. (2008) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2008.09.001 · Cited by 436
Mitochondrial Diseases of the Brain
Chaturvedi, R. K., & Beal, M. F. (2013) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2013.03.018 · Cited by 426
Self-citationHuntington's disease: from pathology and genetics to potential therapies
Imarisio, S., Carmichael, J., Korolchuk, V., Chen, C.-W., Saiki, S., Rose, C., Krishna, G., Davies, J. E., Ttofi, E., Underwood, B. R., et al. (2008) · Biochemical Journal · doi:10.1042/bj20071619 · Cited by 409
Molecular genetics: Unmasking polyglutamine triggers in neurodegenerative disease
Gusella, J. F., & MacDonald, M. E. (2000) · Nature reviews. Neuroscience · doi:10.1038/35039051 · Cited by 404
Therapeutic Effects of Coenzyme Q10and Remacemide in Transgenic Mouse Models of Huntington's Disease
Ferrante, 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
Self-citationKarpuj, M., Becher, M., Springer, J. E., Chabas, D., Youssef, S., Pedotti, R., Mitchell, D., & Steinman, L. (2002) · Nature Medicine · doi:10.1038/nm0202-143 · Cited by 365
Functions and effects of creatine in the central nervous system
Andres, R. H., Ducray, A., Schlattner, U., Wallimann, T., & Widmer, H. R. (2008) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2008.02.035 · Cited by 389
Creatine: Endogenous Metabolite, Dietary, and Therapeutic Supplement
Brosnan, J. T., & Brosnan, M. E. (2007) · Annual Review of Nutrition · doi:10.1146/annurev.nutr.27.061406.093621 · Cited by 347
International Society of Sports Nutrition position stand: creatine supplementation and exercise
Buford, T. W., Kreider, R. B., Stout, J. R., Greenwood, M., Campbell, B., Spano, M., Ziegenfuss, T., Lopez, H., Landis, J., & Antonio, J. (2007) · Journal of the International Society of Sports Nutrition · doi:10.1186/1550-2783-4-6 · Cited by 341
Clinical Pharmacology of the Dietary Supplement Creatine Monohydrate
Persky, A. M., & Brazeau, G. A. (2001) · Pharmacological Reviews · doi:10.1016/s0031-6997(24)01490-x · Cited by 333
Mouse models of Huntington's disease
Menalled, L., & Chesselet, M. (2002) · Trends in Pharmacological Sciences · doi:10.1016/s0165-6147(00)01884-8 · Cited by 311
Therapeutic Effects of Cystamine in a Murine Model of Huntington's Disease
Dedeoglu, A., Kubilus, J. K., Jeitner, T. M., Matson, S. A., Bogdanov, M., Kowall, N. W., Matson, W. R., Cooper, A. J. L., Ratan, R. R., Beal, M. F., et al. (2002) · Journal of Neuroscience · doi:10.1523/jneurosci.22-20-08942.2002 · Cited by 317
Self-citationCreatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2′dG
Hersch, S. M., Gevorkian, S., Marder, K., Moskowitz, C., Feigin, A., Cox, M., Como, P., Zimmerman, C., Lin, M., Zhang, L., et al. (2006) · Neurology · doi:10.1212/01.wnl.0000194318.74946.b6 · Cited by 313
Self-citationKeene, C. D., Rodrigues, C. M. P., Eich, T., Chhabra, M. S., Steer, C. J., & Low, W. C. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.162362299 · Cited by 306
Mitochondrial Approaches for Neuroprotection
Chaturvedi, R. K., & Beal, M. F. (2008) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1427.027 · Cited by 294
Self-citationAndreassen, O. A., Dedeoglu, A., Ferrante, R. J., Jenkins, B. G., Ferrante, K. L., Thomas, M., Friedlich, A., Browne, S. E., Schilling, G., Borchelt, D. R., et al. (2001) · Neurobiology of Disease · doi:10.1006/nbdi.2001.0406 · Cited by 293
Self-citationSingh, S., Vrishni, S., Singh, B. K., Rahman, I., & Kakkar, P. (2010) · Free Radical Research · doi:10.3109/10715762.2010.507670 · Cited by 305
Mitochondria in Huntington's disease
Damiano, M. G., Galvan, L., Déglon, N., & Brouillet, E. (2009) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2009.07.012 · Cited by 295
Weight loss in early stage of Huntington’s disease
Djoussé, L., Knowlton, B. A., Cupples, L. A., Marder, K. S., Shoulson, I., & Myers, R. H. (2002) · Neurology · doi:10.1212/01.wnl.0000031791.10922.cf · Cited by 284
Lessons from animal models of Huntington's disease
Rubinsztein, D. C. (2002) · Trends in Genetics · doi:10.1016/s0168-9525(01)02625-7 · Cited by 281
Heikkinen, T., Lehtimäki, K. K., Vartiainen, N. E., Puoliväli, J. T., Hendricks, S. J., Glaser, J. R., Bradaïa, A., Wadel, K., Touller, C., Kontkanen, O., et al. (2012) · PLoS ONE · doi:10.1371/journal.pone.0050717 · Cited by 265
NMDA receptor function in mouse models of Huntington disease
Cepeda, C., Ariano, M. A., Calvert, C. R., Flores‐Hernández, J., Chandler, S. H., Leavitt, B. R., Hayden, M. R., & Levine, M. S. (2001) · Journal of Neuroscience Research · doi:10.1002/jnr.1244 · Cited by 275
Antioxidants as treatment for neurodegenerative disorders
Moosmann, B., & Behl, C. (2002) · Expert Opinion on Investigational Drugs · doi:10.1517/13543784.11.10.1407 · Cited by 255
Calkins, M. J., Jakel, R. J., Johnson, D. A., Chan, K., Kan, Y. W., & Johnson, J. A. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0408487101 · Cited by 247
Delivery of peptide and protein drugs over the blood–brain barrier
Brasnjevic, I., Steinbusch, H. W. M., Schmitz, C., & Martinez-Martinez, P. (2009) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2008.12.002 · Cited by 279
Stack, E. C., Kubilus, J. K., Smith, K., Cormier, K., Signore, S. J. D., Guélin, E., Ryu, H., Hersch, S. M., & Ferrante, R. J. (2005) · The Journal of Comparative Neurology · doi:10.1002/cne.20680 · Cited by 255
Self-citationTarnopolsky, M. A., & Beal, M. F. (2001) · Annals of Neurology · doi:10.1002/ana.1028 · Cited by 244
Self-citationThe Energetics of Huntington's Disease
Browne, S. E., & Beal, M. F. (2004) · Neurochemical Research · doi:10.1023/b:nere.0000014824.04728.dd · Cited by 240
Self-citationBioenergetic approaches for neuroprotection in Parkinson's disease
Beal, M. F. (2003) · Annals of Neurology · doi:10.1002/ana.10479 · Cited by 236
Self-citationMismatch repair gene Msh2 modifies the timing of early disease in HdhQ111 striatum
Wheeler, V. C. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg056 · Cited by 232
Yang, 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 237
Self-citationHuntington's Disease: Can Mice Lead the Way to Treatment?
Crook, Z. R., & Housman, D. E. (2011) · Neuron · doi:10.1016/j.neuron.2010.12.035 · Cited by 236
Creatine in health and disease
Kreider, R. B., & Stout, J. R. (2021) · Nutrients · doi:10.3390/nu13020447 · Cited by 227
Effects of overexpression of Huntingtin proteins on mitochondrial integrity
Wang, H., Lim, P. J., Karbowski, M., & Monteiro, M. J. (2008) · Human Molecular Genetics · doi:10.1093/hmg/ddn404 · Cited by 222
Metformin therapy in a transgenic mouse model of Huntington's disease
Ma, T. C., Buescher, J. L., Oatis, B., Funk, J. A., Nash, A. J., Carrier, R. L., & Hoyt, K. R. (2006) · Neuroscience Letters · doi:10.1016/j.neulet.2006.10.039 · Cited by 224
Experimental 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 227
Self-citationEnergy deficit in Huntington disease: why it matters
Mochel, F., & Haller, R. G. (2011) · Journal of Clinical Investigation · doi:10.1172/jci45691 · Cited by 220
Neuronal Life-and-Death Signaling, Apoptosis, and Neurodegenerative Disorders
Mattson, M. P. (2006) · Antioxidants and Redox Signaling · doi:10.1089/ars.2006.8.1997 · Cited by 211
Li, J. Y., Popović, N., & Brundin, P. (2005) · NeuroRx · doi:10.1602/neurorx.2.3.447 · Cited by 221
Wyss, M., & Schulze, A. (2002) · Neuroscience · doi:10.1016/s0306-4522(02)00088-x · Cited by 210
Petersén, Å. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.12.1243 · Cited by 210
Wasser, C. I., Mercieca, E.-C., Kong, G., Hannan, A. J., McKeown, S. J., Glikmann-Johnston, Y., & Stout, J. C. (2020) · Brain Communications · doi:10.1093/braincomms/fcaa110 · Cited by 198
Delaying Brain Mitochondrial Decay and Aging with Mitochondrial Antioxidants and Metabolites
Liu, J., Atamna, H., Kuratsune, H., & Ames, B. N. (2002) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.2002.tb02090.x · Cited by 203
The biological basis of injury and neuroprotection in the fetal and neonatal brain
Rees, S. M., Harding, R., & Walker, D. W. (2011) · International Journal of Developmental Neuroscience · doi:10.1016/j.ijdevneu.2011.04.004 · Cited by 207
Creatine and the creatine transporter: A review
Snow, R. J., & Murphy, R. M. (2001) · Molecular and Cellular Biochemistry · doi:10.1023/a:1011908606819 · Cited by 197
Synthesis and transport of creatine in the CNS: Importance for cerebral functions
Béard, E., & Braissant, O. (2010) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2010.06935.x · Cited by 192
Reddy, P. H., & Shirendeb, U. P. (2011) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2011.10.016 · Cited by 188
Neuroprotective effects of creatine
Beal, M. F. (2011) · Amino Acids · doi:10.1007/s00726-011-0851-0 · Cited by 196
Self-citationCreatine metabolism and psychiatric disorders: Does creatine supplementation have therapeutic value?
Allen, P. J. (2012) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2012.03.005 · Cited by 200
Additive neuroprotective effects of minocycline with creatine in a mouse model of ALS
Zhang, W., Narayanan, M. V., & Friedlander, R. M. (2003) · Annals of Neurology · doi:10.1002/ana.10476 · Cited by 185
Cecil, K. M., Salomons, G. S., Ball, W. S., Wong, B. L. Y., Chuck, G., Verhoeven‐Duif, N. M., Jakobs, C., & deGrauw, T. J. (2001) · Annals of Neurology · doi:10.1002/ana.79 · Cited by 177
Adhihetty, P. J., & Beal, M. F. (2008) · NeuroMolecular Medicine · doi:10.1007/s12017-008-8053-y · Cited by 190
Self-citationScaini, G., Rezin, G. T., Carvalho, A. F., STRECK, E. L., Berk, M., & Quevedo, J. (2016) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2016.06.040 · Cited by 187
Cystamine Inhibits Caspase Activity
Lesort, M. J., Lee, M., Tucholski, J., & Johnson, G. V. W. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m205812200 · Cited by 177
Mitochondrial dysfunction in epilepsy
Folbergrová, J., & Kunz, W. S. (2011) · Mitochondrion · doi:10.1016/j.mito.2011.04.004 · Cited by 175
CGS21680 attenuates symptoms of Huntington's disease in a transgenic mouse model
Chou, S., Lee, Y., Chen, H., Chiang, M., Lai, H., Chang, H., Wu, Y., Sun, C., Chien, C., Lin, Y., et al. (2005) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2005.03029.x · Cited by 179
Phosphorylation of profilin by ROCK1 regulates polyglutamine aggregation
Shao, J., Welch, W. J., DiProspero, N. A., & Diamond, M. I. (2008) · Molecular and Cellular Biology · doi:10.1128/mcb.00079-08 · Cited by 171
Kazantsev, A. G., Walker, H. A., Slepko, N., Bear, J. E., Preisinger, E., Steffan, J. S., Zhu, Y.-Z., Gertler, F. B., Housman, D. E., Marsh, J., et al. (2002) · Nature Genetics · doi:10.1038/ng864 · Cited by 173
Minocycline and doxycycline are not beneficial in a model of Huntington's disease
Smith, D. L., Woodman, B., Mahal, A., Sathasivam, K., Ghazi‐Noori, S., Lowden, P. A. S., Bates, G. P., & Hockly, E. (2003) · Annals of Neurology · doi:10.1002/ana.10614 · Cited by 173
Dedeoglu, A., Kubilus, J. K., Yang, L., Ferrante, K. L., Hersch, S. M., Beal, M. F., & Ferrante, A. R. J. (2003) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2003.01706.x · Cited by 169
Self-citationThe mitochondrial permeability transition in neurologic disease
Norenberg, M. D., & RAO, K. (2007) · Neurochemistry International · doi:10.1016/j.neuint.2007.02.008 · Cited by 168
The genetics of primary dystonias and related disorders
Nemeth, A. H. (2002) · Brain · doi:10.1093/brain/awf090 · Cited by 168
Evidence of Oxidant Damage in Huntington's Disease: Translational Strategies Using Antioxidants
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 169
Self-citationCreatine for treating muscle disorders
Kley, R. A., Tarnopolsky, M. A., & Vorgerd, M. (2013) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd004760.pub4 · Cited by 169
Effect of Oral Creatine Supplementation on Human Muscle GLUT4 Protein Content After Immobilization
Eijnde, B. O. '., Ursø, B., Richter, E. A., Greenhaff, P. L., & Hespel, P. (2001) · Diabetes · doi:10.2337/diabetes.50.1.18 · Cited by 162
Mitochondrial structural and functional dynamics in Huntington's disease
Reddy, P. H., Mao, P., & Mańczak, M. (2009) · Brain Research Reviews · doi:10.1016/j.brainresrev.2009.04.001 · Cited by 164
Creatine supplementation in Huntington’s disease
Verbessem, P., Lemiere, J., Eijnde, B. O., Swinnen, S. P., Vanhees, L., Leemputte, M. V., Hespel, P., & Dom, R. (2003) · Neurology · doi:10.1212/01.wnl.0000090629.40891.4b · Cited by 163
Antioxidants 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 163
Self-citationExploring the therapeutic role of creatine supplementation
Gualano, B., Artioli, G. G., Poortmans, J. R., & Lancha Junior, A. H. (2010) · Amino Acids · doi:10.1007/s00726-009-0263-6 · Cited by 156
Tarnopolsky, M. A. (2008) · Advanced Drug Delivery Reviews · doi:10.1016/j.addr.2008.05.001 · Cited by 161
From Pathogenesis to Therapeutics: A Review of 150 Years of Huntington’s Disease Research
Jiang, A., Handley, R. R., Lehnert, K., & Snell, R. G. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms241613021 · Cited by 174
Jost, C. R., der Zee, C. E. E. M. V., Zandt, R. I. ‘., Oerlemans, F. T. J. J., Verheij, M. M. M., Streijger, F., Fransen, J. A. M., Heerschap, A., Cools, A. R., & Wieringa, B. (2002) · European Journal of Neuroscience · doi:10.1046/j.1460-9568.2002.02001.x · Cited by 161
Coenzyme Q 10 as a Possible Treatment for Neurodegenerative Diseases
Beal, M. F. (2002) · Free Radical Research · doi:10.1080/10715760290021315 · Cited by 160
Self-citationThe pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies
Bauer, P. O., & Nukina, N. (2009) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2009.06302.x · Cited by 178
Hansson, O., Guatteo, E., Mercuri, N. B., Bernardi, G., Li, X., Castilho, R. F., & Brundin, P. (2001) · European Journal of Neuroscience · doi:10.1046/j.0953-816x.2001.01767.x · Cited by 154
Schilling, G., Coonfield, M. L., Ross, C. A., & Borchelt, D. R. (2001) · Neuroscience Letters · doi:10.1016/s0304-3940(01)02326-6 · Cited by 151
Huntington's disease: molecular basis of neurodegeneration
Rubinsztein, D. C., & Carmichael, J. (2003) · Expert Reviews in Molecular Medicine · doi:10.1017/s1462399403006549 · Cited by 155
Safdar, A., Yardley, N. J., Snow, R., Melov, S., & Tarnopolsky, M. A. (2008) · Physiological Genomics · doi:10.1152/physiolgenomics.00157.2007 · Cited by 149
Resveratrol protects against peripheral deficits in a mouse model of Huntington's disease
Ho, D. J., Calingasan, N. Y., Wille, E. J., Dumont, M., & Beal, M. F. (2010) · Experimental Neurology · doi:10.1016/j.expneurol.2010.05.006 · Cited by 148
Self-citationThe role of mitochondria in mood disorders: from physiology to pathophysiology and to treatment
Giménez-Palomo, A., Dodd, S., Anmella, G., Carvalho, A. F., Scaini, G., Quevedo, J., Pacchiarotti, I., Vieta, E., & Berk, M. (2021) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2021.546801 · Cited by 140
Role of oxidative DNA damage in mitochondrial dysfunction and Huntington’s disease pathogenesis
Ayala‐Peña, S. (2013) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2013.04.017 · Cited by 147
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
Prophylactic Creatine Administration Mediates Neuroprotection in Cerebral Ischemia in Mice
Zhu, S., Li, M., Figueroa, B. E., Liu, A., Stavrovskaya, I. G., Pasinelli, P., Beal, M. F., Brown, R. H., Kristal, B. S., Ferrante, R. J., et al. (2004) · The Journal of Neuroscience · doi:10.1523/jneurosci.1278-04.2004 · Cited by 138
Self-citationMitochondria targeted therapeutic approaches in Parkinson's and Huntington's diseases
Chaturvedi, R. K., & Beal, M. F. (2012) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2012.11.011 · Cited by 139
Self-citation
Metadata
- Authors
- Ferrante, R. J., Andreassen, O. A., Jenkins, B. G., Dedeoglu, A., Kuemmerle, S., Kubilus, J. K., Kaddurah-Daouk, R., Hersch, S. M., & Beal, M. F.
- Affiliations
- Boston University (US)
- Harvard University (US)
- Emory University (US)
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 2000-06-15
- Volume
- 20
- Issue
- 12
- Pages
- 4389-4397
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- PMC
- PMC6772461
- OpenAlex
- W1750171938
- Cited by
- 522
- References
- 62
- Citations by year
- 2026: 1 · 2025: 6 · 2024: 7 · 2023: 8 · 2022: 7 · 2021: 10 · 2020: 6 · 2019: 12 · 2018: 10 · 2017: 9 · 2016: 23 · 2015: 7 · 2014: 23 · 2013: 14 · 2012: 21
- FWCI
- 16.55
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Neuroscience (publishedVersion, cc-by-nc-sa)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Hereditary Disease Foundation
- National Institutes of Health (5r29ns037102-02, AG13846)
- National Institute on Aging (P30AG013846, P01AG012992)
- National Institute of Neurological Disorders and Stroke (NS37102, NS 35255, NS38180, R01NS035255)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Health Sciences › Medicine › Clinical Neurology › Parkinson's Disease Mechanisms and Treatments
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- Huntington's disease, creatine supplementation, neuroprotection, R6/2 transgenic mice, phosphocreatine, mitochondrial permeability transition, brain atrophy, striatal neurons, huntingtin aggregates, N-acetylaspartate, diabetes onset, motor performance
- MeSH terms
- Huntingtin Protein, Animals, Aspartic Acid, Atrophy, Brain, Corpus Striatum, Creatine, Crosses, Genetic, Female, Humans, Huntington Disease, Magnetic Resonance Imaging, Male, Mice, Inbred Strains, Motor Activity, Nerve Tissue Proteins, Neurons, Nuclear Proteins, Neuroprotective Agents, Dietary Supplements, Animals, Genetically Modified, Mice
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
