Cited by 522Genetic Neurodegenerative DiseasesParkinson's Disease Mechanisms and TreatmentsAmyotrophic Lateral Sclerosis Research

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

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Ferrante, Robert J., Andreassen, Ole A., Jenkins, Bruce G., Dedeoglu, Alpaslan, Kuemmerle, Stefan, Kubilus, James K., Kaddurah-Daouk, Rima, Hersch, Steven M., Beal, M. Flint·2000·The Journal of Neuroscience

DOI 10.1523/jneurosci.20-12-04389.2000PMC PMC6772461

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Abstract

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.

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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
Self-citation
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
Self-citation
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
Self-citation

Show all 62 references

Research works printed but not linked: 1

Gutekunst, 1996, Mitochondrial calcium homeostasis in Huntington's disease fibroblasts., Soc Neurosci Abstr, 22, 227

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Ducray, A., Schlaeppi, J.-A., Qualls, R., Andres, R. H., Seiler, R. W., Schlattner, U., Wallimann, T., & Widmer, H. R. (2007) · Journal of Neuroscience Research · doi:10.1002/jnr.21337 · Cited by 31
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Self-citation

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)
ORCID: Andreassen, Ole A.
Dedeoglu, Alpaslan
Kaddurah-Daouk, Rima
Hersch, Steven M.
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
DOI: 10.1523/jneurosci.20-12-04389.2000
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: cc-by-nc-sa
creativecommons.org/licenses/by-nc-sa/4.0/ (vor, 183 d)
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

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

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