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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.
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“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]
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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
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Apoptosis and Caspases in Neurodegenerative Diseases
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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
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
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