Neuroprotective Effects of Creatine in a Transgenic Mouse Model of Huntington's Disease

The Mouse Brain in Stereotaxic Coordinates

Paxinos, G. (2001) · Cited by 12,859

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes

MACDONALD, M. (1993) · Cell · doi:10.1016/0092-8674(93)90585-e · Cited by 8,366

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

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

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group.

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

Aggregation 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

Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation

Davies, 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

Intracellular compartmentation, structure and function of creatine kinase isoenzymes in tissues with high and fluctuating energy demands: the “phosphocreatine circuit” for cellular energy homeostasis

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

Induction of neostriatal neurogenesis slows disease progression in a transgenic murine model of Huntington disease

Cho, S., Benraiss, A., Chmielnicki, E., Samdani, A. F., Economides, A. N., & Goldman, S. A. (2007) · Journal of Clinical Investigation · doi:10.1172/jci31778 · Cited by 138

Evaluation of R6/2 HD transgenic mice for therapeutic studies in Huntington's disease: behavioral testing and impact of diabetes mellitus

Lüesse, H. (2001) · Behavioural Brain Research · doi:10.1016/s0166-4328(01)00261-3 · Cited by 136

Cardiac dysfunction in the R6/2 mouse model of Huntington’s disease

Mihm, M. J., Amann, D. M., Schanbacher, B. L., Altschuld, R. A., Bauer, J. A., & Hoyt, K. R. (2006) · Neurobiology of Disease · doi:10.1016/j.nbd.2006.09.016 · Cited by 136

Pharmacokinetics of the Dietary Supplement Creatine

Persky, A. M., Brazeau, G. A., & Hochhaus, G. (2003) · Clinical Pharmacokinetics · doi:10.2165/00003088-200342060-00005 · Cited by 132

Cytochrome C and Caspase-9 Expression in Huntington 's Disease

Ferrante, R. J., Kiechle, T., Dedeoglu, A., Kubilus, J. K., Kowall, N. W., Beal, M. F., Friedlander, R. M., & Hersch, S. M. (2002) · NeuroMolecular Medicine · doi:10.1385/nmm:1:3:183 · Cited by 134

On the mechanisms of neuroprotection by creatine and phosphocreatine

Brustovetsky, N., Brustovetsky, T., & Dubinsky, J. M. (2001) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2001.00052.x · Cited by 135

A Bile Acid Protects against Motor and Cognitive Deficits and Reduces Striatal Degeneration in the 3-Nitropropionic Acid Model of Huntington's Disease

Keene, C. D., Rodrigues, C. M. P., Eich, T., Linehan-Stieers, C., Abt, A., Kren, B. T., Steer, C. J., & Low, W. C. (2001) · Experimental Neurology · doi:10.1006/exnr.2001.7755 · Cited by 132

Retinal energy metabolism in health and glaucoma

Casson, R. J., Chidlow, G., Crowston, J. G., Williams, P. A., & Wood, J. P. M. (2020) · Progress in Retinal and Eye Research · doi:10.1016/j.preteyeres.2020.100881 · Cited by 132

Dose ranging and efficacy study of high-dose coenzyme Q10 formulations in Huntington's disease mice

Smith, K. M., Matson, S., Matson, W. R., Cormier, K., Del Signore, S. J., Hagerty, S. W., Stack, E. C., Ryu, H., & Ferrante, R. J. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2006.03.004 · Cited by 127

Creatine in the central nervous system: From magnetic resonance spectroscopy to creatine deficiencies

Račkayová, V., Cudalbu, C., Pouwels, P. J. W., & Braissant, O. (2016) · Analytical Biochemistry · doi:10.1016/j.ab.2016.11.007 · Cited by 137

Creatine supplementation lowers brain glutamate levels in Huntington?s disease

Bender, A., Auer, D. P., Merl, T., Reilmann, R., Saemann, P., Yassouridis, A., Bender, J., Weindl, A., Dose, M., Gasser, T., et al. (2005) · Journal of Neurology · doi:10.1007/s00415-005-0595-4 · Cited by 129

Creatine supplementation reduces skeletal muscle degeneration and enhances mitochondrial function in mdx mice

Passaquin, A., Renard, M., Kay, L., Challet, C., Mokhtarian, A., Wallimann, T., & Rüegg, U. T. (2002) · Neuromuscular Disorders · doi:10.1016/s0960-8966(01)00273-5 · Cited by 127

Improved Reperfusion and Neuroprotection by Creatine in a Mouse Model of Stroke

Prass, K., Royl, G., Lindauer, U., Freyer, D., Megow, D., Dirnagl, U., Stöckler-Ipsiroglu, G., Wallimann, T., & Priller, J. (2007) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1038/sj.jcbfm.9600351 · Cited by 128

rAAV-mediated shRNA ameliorated neuropathology in Huntington disease model mouse

Machida, Y., Okada, T., Kurosawa, M., Oyama, F., Ozawa, K., & Nukina, N. (2006) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2006.02.141 · Cited by 127

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

Increases in cortical glutamate concentrations in transgenic amyotrophic lateral sclerosis mice are attenuated by creatine supplementation

Andreassen, O. A., Jenkins, B. G., Dedeoglu, A., Ferrante, K. L., Bogdanov, M. B., Kaddurah-Daouk, R. F., & Beal, M. F. (2001) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2001.00188.x · Cited by 123

The role of mitochondria in epileptogenesis

Kunz, W. S. (2002) · Current Opinion in Neurology · doi:10.1097/00019052-200204000-00009 · Cited by 123

Creatine supplementation increases glucose oxidation and AMPK phosphorylation and reduces lactate production in L6 rat skeletal muscle cells

Ceddia, R., & Sweeney, G. (2004) · Journal of Physiology · doi:10.1113/jphysiol.2003.056291 · Cited by 119

Modulation of nucleosome dynamics in Huntington's disease

Stack, E. C., Signore, S. J. D., Luthi‐Carter, R., Soh, B. Y., Goldstein, D. R., Matson, S. A., Goodrich, S., Markey, A., Cormier, K., Hagerty, S. W., et al. (2007) · Human Molecular Genetics · doi:10.1093/hmg/ddm064 · Cited by 119

Mitochondrial involvement in temporal lobe epilepsy

Kudin, A. P., Zsurka, G., Elger, C. E., & Kunz, W. S. (2009) · Experimental Neurology · doi:10.1016/j.expneurol.2009.02.014 · Cited by 122

Early Alterations of Brain Cellular Energy Homeostasis in Huntington Disease Models

Mochel, F., Durant, B. K., Meng, X., O'Callaghan, J. P., Yu, H., Brouillet, E., Wheeler, V. C., Humbert, S., Schiffmann, R., & Dürr, A. (2011) · Journal of Biological Chemistry · doi:10.1074/jbc.m111.309849 · Cited by 119

Mitochondria and reactive oxygen and nitrogen species in neurological disorders and stroke: Therapeutic implications☆

Bolaños, J. P., Moro, M. Á., Lizasoaín, I., & Almeida, Á. (2009) · Advanced Drug Delivery Reviews · doi:10.1016/j.addr.2009.05.009 · Cited by 120

Overexpression of heat shock protein 70 in R6/2 Huntington’s disease mice has only modest effects on disease progression

Hansson, O., Nylandsted, J., Castilho, R. F., Leist, M., Jäättelä, M., & Brundin, P. (2003) · Brain Research · doi:10.1016/s0006-8993(02)04275-0 · Cited by 119

Huntingtin in health and disease

Young, A. B. (2003) · Journal of Clinical Investigation · doi:10.1172/jci17742 · Cited by 118

Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease

Schiefer, J., Landwehrmeyer, G. B., Lüesse, H., Sprünken, A., Puls, C., Milkereit, A., Milkereit, E., & Kosinski, C. M. (2002) · Movement Disorders · doi:10.1002/mds.10229 · Cited by 117

Lipoic acid improves survival in transgenic mouse models of Huntington's disease

Andreassen, O. A., Ferrante, R. J., Dedeoglu, A., & Beal, M. F. (2001) · Neuroreport · doi:10.1097/00001756-200110290-00044 · Cited by 115

Identification of new kinase clusters required for neurite outgrowth and retraction by a loss-of-function RNA interference screen

Loh, S. H. Y., Francescut, L., Lingor, P., Bähr, M., & Nicotera, P. (2008) · Cell Death & Differentiation · doi:10.1038/sj.cdd.4402258 · Cited by 108

A small-molecule therapeutic lead for Huntington's disease: Preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse

Chopra, V., Fox, J. H., Lieberman, G., Dorsey, K., Matson, W. R., Waldmeier, P. C., Housman, D. E., Kazantsev, A. G., Young, A. B., & Hersch, S. M. (2007) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0707842104 · Cited by 108

The metabotropic glutamate receptor 5 antagonist MPEP and the mGluR2 agonist LY379268 modify disease progression in a transgenic mouse model of Huntington's disease

Schiefer, J., Sprünken, A., Puls, C., Lüesse, H., Milkereit, A., Milkereit, E., Johann, V., & Kosinski, C. M. (2004) · Brain Research · doi:10.1016/j.brainres.2004.06.005 · Cited by 107

Viral delivery of glial cell line-derived neurotrophic factor improves behavior and protects striatal neurons in a mouse model of Huntington’s disease

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

Huntington Disease and the Huntingtin Protein

Zheng, Z., & Diamond, M. I. (2012) · Progress in molecular biology and translational science · doi:10.1016/b978-0-12-385883-2.00010-2 · Cited by 118

Oral creatine supplementation and skeletal muscle metabolism in physical exercise

Mesa, J. L. M., Ruiz, J. R., Gonz??lez-Gross, M. M., Guti??rrez S??inz, ?., & Castillo Garz??n, M. J. (2002) · Sports Medicine · doi:10.2165/00007256-200232140-00003 · Cited by 103

Creatine in Type 2 Diabetes: A Randomized, Double-Blind, Placebo-Controlled Trial

GUALANO, B., DE SALLES PAINNELI, V., ROSCHEL, H., ARTIOLI, G. G., NEVES, M., DE SÁ PINTO, A. L., DA SILVA, M. E. R., CUNHA, M. R., OTADUY, M. C. G., DA COSTA LEITE, C., et al. (2011) · Medicine & Science in Sports & Exercise · doi:10.1249/mss.0b013e3181fcee7d · Cited by 99

The role of oxidative stress in Huntington’s disease: Are antioxidants good therapeutic candidates?

Gil-Mohapel, J., Brocardo, P., & Christie, B. (2014) · Current Drug Targets · doi:10.2174/1389450115666140115113734 · Cited by 101

Beyond muscles: The untapped potential of creatine

Riesberg, L. A., Weed, S. A., McDonald, T. L., Eckerson, J. M., & Drescher, K. M. (2016) · International Immunopharmacology · doi:10.1016/j.intimp.2015.12.034 · Cited by 95

The CREST-E study of creatine for Huntington disease

Hersch, S. M., Schifitto, G., Oakes, D., Bredlau, A., Meyers, C. M., Nahin, R. L., Rosas, H. D., & For the Huntington Study Group CREST-E Investigators and Coordinators (2017) · Neurology · doi:10.1212/wnl.0000000000004209 · Cited by 102

Creatine for neuroprotection in neurodegenerative disease: end of story?

Bender, A., & Klopstock, T. (2016) · Amino Acids · doi:10.1007/s00726-015-2165-0 · Cited by 96

Modulation of energy deficiency in Huntington's disease via activation of the peroxisome proliferator-activated receptor gamma

Chiang, M., Chen, C., Lee, M.-R., Chen, H.-W., Chen, H., Wu, Y.-S., Hung, C.-H., Kang, J.-J., Chang, C., Chang, C., et al. (2010) · Human Molecular Genetics · doi:10.1093/hmg/ddq322 · Cited by 98

Modelling brain diseases in mice: the challenges of design and analysis

Watase, K., & Zoghbi, H. Y. (2003) · Nature Reviews Genetics · doi:10.1038/nrg1045 · Cited by 94

Longitudinal characterization of brain atrophy of a Huntington's disease mouse model by automated morphological analyses of magnetic resonance images

Zhang, J., Peng, Q., Li, Q., Jahanshad, N., Hou, Z., Jiang, M., Masuda, N., Langbehn, D. R., Miller, M. I., Mori, S., et al. (2009) · NeuroImage · doi:10.1016/j.neuroimage.2009.10.027 · Cited by 94

High-dose creatine therapy for Huntington disease: A 2-year clinical and MRS study

Tabrizi, S. J., Blamire, A. M., Manners, D. N., Rajagopalan, B., Styles, P., Schapira, A. H. V., & Warner, T. T. (2005) · Neurology · doi:10.1212/01.wnl.0000160388.96242.77 · Cited by 93

Protein oxidation in Huntington disease affects energy production and vitamin B6 metabolism

Sorolla, M. A., Colman, M. J. R., Tamarit, J., Ortega, Z., Lucas, J. J., Ferrer, I., Ros, J., & Cabiscol, E. (2010) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2010.05.016 · Cited by 95

Mitochondrial dysfunction, metabolic deficits, and increased oxidative stress in Huntington's disease.

Chen, C. (2011) · PubMed · Cited by 89

Striatal neurochemical changes in transgenic models of Huntington's disease

Ariano, M. A., D, N. A. M., DiFiglia, M., Tagle, D. A., Sibley, D. R., Leavitt, B. R., Hayden, M. R., & Levine, M. S. (2002) · Journal of Neuroscience Research · doi:10.1002/jnr.10272 · Cited by 91

Motor behaviour deficits and their histopathological and functional correlates in the nigrostriatal system of dopamine transporter knockout mice

Fernagut, P., Chalon, S., Diguet, E., Guilloteau, D., Tison, F., & Jaber, M. (2003) · Neuroscience · doi:10.1016/s0306-4522(02)00778-9 · Cited by 91

Creatine therapy for Huntington’s disease: Clinical and MRS findings in a 1-year pilot study

Tabrizi, S. J., Blamire, A. M., Manners, D. N., Rajagopalan, B., Styles, P., Schapira, A. H. V., & Warner, T. T. (2003) · Neurology · doi:10.1212/01.wnl.0000070186.97463.a7 · Cited by 87

Pharmacologic approaches to the treatment of Huntington's disease

Venuto, C. S., McGarry, A. J., Ma, Q., & Kieburtz, K. D. (2011) · Movement Disorders · doi:10.1002/mds.23953 · Cited by 93

Of mice and men: Reconciling preclinical ALS mouse studies and human clinical trials

Rothstein, J. D. (2003) · Annals of Neurology · doi:10.1002/ana.10561 · Cited by 86

Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease

Jenkins, B. G., Andreassen, O. A., Dedeoglu, A., Leavitt, B. R., Hayden, M. R., Borchelt, D., Ross, C. A., Ferrante, R. J., & Beal, M. F. (2005) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2005.03411.x · Cited by 83

Nutritional therapy improves function and complements corticosteroid intervention in mdx mice

Payne, E. T., Yasuda, N., Bourgeois, J. M., Devries, M. C., Rodriguez, M. C., Yousuf, J., & Tarnopolsky, M. A. (2005) · Muscle & Nerve · doi:10.1002/mus.20436 · Cited by 83

Nitric oxide and nitric oxide synthase in Huntington's disease

Deckel, A. W. (2001) · Journal of Neuroscience Research · doi:10.1002/jnr.1057 · Cited by 83

A review of creatine supplementation in age-related diseases: more than a supplement for athletes

Smith, R. N., Agharkar, A. S., & Gonzales, E. B. (2014) · F1000Research · doi:10.12688/f1000research.5218.1 · Cited by 82

Using advances in neuroimaging to detect, understand, and monitor disease progression in Huntington’s disease

Rosas, H. D., Feigin, A., & Hersch, S. M. (2004) · Neurotherapeutics · doi:10.1602/neurorx.1.2.263 · Cited by 84

Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease

Andreassen, O. A., Ferrante, R. J., Huang, H., Dedeoglu, A., Park, L., Ferrante, K. L., Kwon, J. M., Borchelt, D., Ross, C. A., Gibson, G. E., et al. (2001) · Annals of Neurology · doi:10.1002/ana.1085 · Cited by 83

Mitochondrial Dysfunction in Parkinson's Disease: Pathogenesis and Neuroprotection

Mounsey, R. B., & Teismann, P. (2010) · Parkinson s Disease · doi:10.4061/2011/617472 · Cited by 86

The Phosphocreatine Circuit: Molecular and Cellular Physiology of Creatine Kinases, Sensitivity to Free Radicals, and Enhancement by Creatine Supplementation

Wallimann, T., Tokarska-Schlattner, M., Neumann, D., Epand, R. M., Epand, R. F., Andres, R. H., Widmer, H. R., Hornemann, T., Saks, V. A., Agarkova, I., et al. (2007) · Wiley eBooks · doi:10.1002/9783527621095.ch7 · Cited by 83

Animal models of Huntington's disease for translation to the clinic: Best practices

Menalled, L., & Brunner, D. (2014) · Movement Disorders · doi:10.1002/mds.26006 · Cited by 82

Mesenchymal stem cell transplantation and DMEM administration in a 3NP rat model of Huntington's disease: Morphological and behavioral outcomes

Rossignol, J., Boyer, C., Lévêque, X. T., Fink, K. D., Thinard, R., Blanchard, F., Dunbar, G. L., & Lescaudron, L. (2010) · Behavioural Brain Research · doi:10.1016/j.bbr.2010.11.006 · Cited by 80

A novel therapeutic strategy for polyglutamine diseases by stabilizing aggregation-prone proteins with small molecules

Tanaka, M., Machida, Y., & Nukina, N. (2005) · Journal of Molecular Medicine · doi:10.1007/s00109-004-0632-2 · Cited by 79

Effects of creatine treatment on the survival of dopaminergic neurons in cultured fetal ventral mesencephalic tissue

Andres, R. H., Huber, A. W., Schlattner, U., Pérez-Bouza, A., Krebs, S. H., Seiler, R. W., Wallimann, T., & Widmer, H. R. (2005) · Neuroscience · doi:10.1016/j.neuroscience.2005.03.004 · Cited by 80

Mitochondrial dysfunction as a critical event in the pathophysiology of bipolar disorder

Scaini, G., Andrews, T., Lima, C. N. D. C., Benevenuto, D., STRECK, E. L., & Quevedo, J. (2020) · Mitochondrion · doi:10.1016/j.mito.2020.12.002 · Cited by 77

A combination drug therapy improves cognition and reverses gene expression changes in a mouse model of Huntington's disease

Morton, A. J., Hunt, M. J., Hodges, A., Lewis, P. D., Redfern, A. J., Dunnett, S. B., & Jones, L. (2005) · European Journal of Neuroscience · doi:10.1111/j.1460-9568.2005.03895.x · Cited by 77

Huntington?s disease: pathomechanism and therapeutic perspectives

G�rdi�n, G., & V�csei, L. (2004) · Journal of Neural Transmission · doi:10.1007/s00702-004-0201-4 · Cited by 76

Brief ampakine treatments slow the progression of Huntington's disease phenotypes in R6/2 mice

Simmons, D. A., Mehta, R. A., Lauterborn, J. C., Gall, C. M., & LYNCH, G. S. (2010) · Neurobiology of Disease · doi:10.1016/j.nbd.2010.10.015 · Cited by 78

Translating therapies for Huntington’s disease from genetic animal models to clinical trials

Hersch, S. M., & Ferrante, R. J. (2004) · NeuroRx · doi:10.1602/neurorx.1.3.298 · Cited by 75

Therapeutic opportunities in polyglutamine disease

Hughes, R. E., & Olson, J. M. (2001) · Nature Medicine · doi:10.1038/86486 · Cited by 75

Probiotics for Neurodegenerative Diseases: A Systemic Review

Ojha, S., Patil, N., Jain, M., Kole, C., & Kaushik, P. (2023) · Microorganisms · doi:10.3390/microorganisms11041083 · Cited by 80

Natural history of disease in the YAC128 mouse reveals a discrete signature of pathology in Huntington disease

Carroll, J. B., Lerch, J. P., Franciosi, S., Spreeuw, A., Bissada, N., Henkelman, R. M., & Hayden, M. R. (2011) · Neurobiology of Disease · doi:10.1016/j.nbd.2011.03.018 · Cited by 73

Heterogeneity in 1H-MRS profiles of presymptomatic and early manifest Huntington's disease

Reynolds, N. C., Prost, R. W., & Mark, L. P. (2004) · Brain Research · doi:10.1016/j.brainres.2004.10.030 · Cited by 73

Early defect of transforming growth factor β1 formation in Huntington’s disease

Battaglia, G., Cannella, M., Riozzi, B., Orobello, S., Maat–Schieman, M. L. C., Aronica, E., Busceti, C. L., Ciarmiello, A., Alberti, S., Amico, E., et al. (2010) · Journal of Cellular and Molecular Medicine · doi:10.1111/j.1582-4934.2010.01011.x · Cited by 71

Differential Effects of Peroxynitrite on Human Mitochondrial Creatine Kinase Isoenzymes

Wendt, S., Schlattner, U., & Wallimann, T. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m208572200 · Cited by 72

From anatomy to the target: Contributions of magnetic resonance imaging to preclinical pharmaceutical research

Beckmann, N., Mueggler, T., ALLEGRINI, P. R., Laurent, D., & Rudin, M. (2001) · The Anatomical Record · doi:10.1002/ar.1059 · Cited by 72

Creatine Diet Supplement for Spinal Cord Injury: Influences on Functional Recovery and Tissue Sparing in Rats

Rabchevsky, A. G., Sullivan, P. G., Fugaccia, I., & Scheff, S. W. (2003) · Journal of Neurotrauma · doi:10.1089/089771503322144572 · Cited by 70

Milestones in Huntington disease

Shoulson, I., & Young, A. B. (2011) · Movement Disorders · doi:10.1002/mds.23685 · Cited by 73

The therapeutic role of creatine in Huntington's disease

Ryu, H., Rosas, H. D., Hersch, S. M., & Ferrante, R. J. (2005) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2005.04.008 · Cited by 69

Clinical neuroprotection in Parkinson's disease — Still waiting for the breakthrough

Löhle, M., & Reichmann, H. (2009) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2009.08.025 · Cited by 70

The paradigm of Huntington’s disease: Therapeutic opportunities in neurodegeneration

Leegwater‐Kim, J., & Cha, J.-H. J. (2004) · NeuroRx · doi:10.1602/neurorx.1.1.128 · Cited by 68

Genetic and pharmacological inactivation of the adenosine A2A receptor attenuates 3‐nitropropionic acid‐induced striatal damage

Fink, J. S., Kalda, A., Ryu, H., Stack, E. C., Schwarzschild, M. A., Chen, J., & Ferrante, R. J. (2003) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2003.02145.x · Cited by 68

Inhibition of Polyglutamine Aggregation in R6/2 HD Brain Slices—Complex Dose–Response Profiles

Smith, D. L., Portier, R. P., Woodman, B., Hockly, E., Mahal, A., Klunk, W. E., Li, X.-J., Wanker, E. E., Murray, K. D., & Bates, G. P. (2001) · Neurobiology of Disease · doi:10.1006/nbdi.2001.0438 · Cited by 69

Rhes Is Involved in Striatal Function

Spano, D., Branchi, I., Rosica, A., Pirro, M. T., Riccio, A., Mithbaokar, P., Affuso, A., Arra, C., Campolongo, P., Terracciano, D., et al. (2004) · Molecular and Cellular Biology · doi:10.1128/mcb.24.13.5788-5796.2004 · Cited by 68

Oxidative stress-mediated inhibition of brain creatine kinase activity by methylmercury

Glaser, V., Leipnitz, G., Straliotto, M. R., de Oliveira, J., dos Santos, V. V., Wannmacher, C. M. D., Bem, A. F. D., da Rocha, J. B. T., Farina, M., & Latini, A. (2010) · NeuroToxicology · doi:10.1016/j.neuro.2010.05.012 · Cited by 67

Caspases in Huntington’s Disease

Sanchez-Mejia, R. O., & Friedlander, R. M. (2001) · The Neuroscientist · doi:10.1177/107385840100700604 · Cited by 66

Magnetic resonance imaging and spectroscopy in assessing 3-nitropropionic acid-induced brain lesions: an animal model of Huntington’s disease

Lee, W., & Chang, C. (2004) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2004.02.002 · Cited by 67

Histological assessment of intermediate- and long-term creatine monohydrate supplementation in mice and rats

Tarnopolsky, M. A., Bourgeois, J. M., Snow, R. J., Keys, S., Roy, B. D., Kwiecień, J. M., & Turnbull, J. (2003) · American Journal of Physiology-Regulatory, Integrative and Comparative Physiology · doi:10.1152/ajpregu.00270.2003 · Cited by 67

Endogenous neuroprotection in chronic neurodegenerative disorders: with particular regard to the kynurenines

Zádori, D., Klivènyi, P., Plangár, I., Toldi, J., & Vécsei, L. (2010) · Journal of Cellular and Molecular Medicine · doi:10.1111/j.1582-4934.2010.01237.x · Cited by 68

Protective Effects of Antioxidants in Huntington’s Disease: an Extensive Review

Essa, M. M., Moghadas, M., Ba-Omar, T., Qoronfleh, M. W., Guillemin, G. J., Manivasagam, T., Justin‐Thenmozhi, A., Ray, B., Bhat, A., Chidambaram, S. B., et al. (2019) · Neurotoxicity Research · doi:10.1007/s12640-018-9989-9 · Cited by 68

Reduced creatine kinase activity in transgenic amyotrophic lateral sclerosis mice

Wendt, S., Dedeoglu, A., Speer, O., Wallimann, T., Beal, M. F., & Andreassen, O. A. (2002) · Free Radical Biology and Medicine · doi:10.1016/s0891-5849(02)00784-0 · Cited by 65

Kinetics of creatine in blood and brain after intraperitoneal injection in the rat

Perasso, L., Cupello, A., Lunardi, G. L., Principato, M. C., Gandolfo, C., & Balestrino, M. (2003) · Brain Research · doi:10.1016/s0006-8993(03)02547-2 · Cited by 65

The detection and measurement of locomotor deficits in a transgenic mouse model of Huntington's disease are task- and protocol-dependent: Influence of non-motor factors on locomotor function

Pallier, P. N., Drew, C. J. G., & Morton, A. J. (2008) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2008.10.007 · Cited by 64

Asialoerythropoetin is not effective in the R6/2 line of Huntington's disease mice

Gil‐Mohapel, J., Leist, M., Popović, N., Brundin, P., & Petersén, Å. (2004) · BMC Neuroscience · doi:10.1186/1471-2202-5-17 · Cited by 64

Cross linking of polyglutamine domains catalyzed by tissue transglutaminase is greatly favored with pathological-length repeats: does transglutaminase activity play a role in (CAG)n/Qn-expansion diseases?

Cooper, A. J. L., Jeitner, T. M., Gentile, V., & Blass, J. P. (2002) · Neurochemistry International · doi:10.1016/s0197-0186(01)00058-4 · Cited by 64

Effects of creatine supplementation on glucose tolerance and insulin sensitivity in sedentary healthy males undergoing aerobic training

Gualano, B., Novaes, R. B., Artioli, G. G., Freire, T. O., Df, C., Scagliusi, F. B., Rogeri, P. S., Roschel, H., Ugrinowitsch, C., & Lancha, A. H. (2007) · Amino Acids · doi:10.1007/s00726-007-0508-1 · Cited by 63

The R6 lines of transgenic mice: A model for screening new therapies for Huntington's disease

Gil‐Mohapel, J., & Rego, A. C. (2008) · Brain Research Reviews · doi:10.1016/j.brainresrev.2008.12.001 · Cited by 67

Creatine-Enhanced Diet Alters Levels of Lactate and Free Fatty Acids After Experimental Brain Injury

Scheff, S. W., & DHILLON, H. S. (2004) · Neurochemical Research · doi:10.1023/b:nere.0000013753.22615.59 · Cited by 63

Microglia density decreases with age in a mouse model of Huntington's disease

Ma, L., Morton, A. J., & Nicholson, L. F. B. (2003) · Glia · doi:10.1002/glia.10261 · Cited by 62

Increased survival and neuroprotective effects of BN82451 in a transgenic mouse model of Huntington's disease

Klivènyi, P., Ferrante, R. J., Gardian, G., Browne, S. E., Chabrier, P., & Beal, M. F. (2003) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2003.t01-1-01868.x · Cited by 62

Protective effects of oral creatine supplementation on spinal cord injury in rats

Hausmann, O., Fouad, K., Wallimann, T., & Schwab, M. (2002) · Spinal Cord · doi:10.1038/sj.sc.3101330 · Cited by 61

Potential of Creatine in Glucose Management and Diabetes

Solis, M. Y., Artioli, G. G., & Gualano, B. (2021) · Nutrients · doi:10.3390/nu13020570 · Cited by 68

Sex-specific antidepressant effects of dietary creatine with and without sub-acute fluoxetine in rats

Allen, P. J., D’Anci, K. E., Kanarek, R. B., & Renshaw, P. F. (2012) · Pharmacology Biochemistry and Behavior · doi:10.1016/j.pbb.2012.03.005 · Cited by 62

Creatine as a Neuroprotector: an Actor that Can Play Many Parts

Marques, E. P., & Wyse, Â. T. D. S. (2019) · Neurotoxicity Research · doi:10.1007/s12640-019-00053-7 · Cited by 62

Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice

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

Creatine Increases Survival and Delays Motor Symptoms in a Transgenic Animal Model of Huntington's Disease

Andreassen, 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

Creatine: prescription for bad genes and a hostile environment?

Mattson, M. P. (2000) · Trends in Neurosciences · doi:10.1016/s0166-2236(00)01697-0 · Cited by 6

Neuroprotective effects of creatine

Beal, M. F. (2011) · Amino Acids · doi:10.1007/s00726-011-0851-0 · Cited by 196

Neuroprotective 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