The DOI matches its Crossref record; 54 of the 54 references it cites were found in a registry or an index.
Creatine Increases Survival and Delays Motor Symptoms in a Transgenic Animal Model of Huntington's Disease
Open accessAbstract
There is substantial evidence for bioenergetic defects in Huntington's disease (HD). Creatine administration increases brain phosphocreatine levels and it stabilizes the mitochondrial permeability transition. We examined the effects of creatine administration in a transgenic mouse model of HD produced by 82 polyglutamine repeats in a 171 amino acid N-terminal fragment of huntingtin (N171-82Q). Dietary supplementation of 2% creatine significantly improved survival, slowed the development of motor symptoms, and delayed the onset of weight loss. Creatine lessened brain atrophy and the formation of intranuclear inclusions, attenuated reductions in striatal N-acetylaspartate as assessed by NMR spectroscopy, and delayed the development of hyperglycemia. These results are similar to those observed using dietary creatine supplementation in the R6/2 transgenic mouse model of HD and provide further evidence that creatine may exert therapeutic effects in HD.
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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 [118]
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
mouse brain in stereotaxic coordinates
Franklin, K. B. J., & Paxinos, G. (1997) · Cited by 3,258
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
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
Wallimann, T., Wyss, M., Brdiczka, D., Nicolay, K., & Eppenberger, H. M. (1992) · Biochemical Journal · doi:10.1042/bj2810021 · Cited by 2,011
Andrew, S. E., Goldberg, Y. P., Kremer, B. P. H., Telenius, H., Theilmann, J., Adam, S., Starr, E. M., Squitieri, F., Lin, B., Kalchman, M. A., et al. (1993) · Nature Genetics · doi:10.1038/ng0893-398 · Cited by 1,135
Trinucleotide repeat length instability and age of onset in Huntington's disease
Duyao, M., Ambrose, C., Myers, R., Novelletto, A., Persichetti, F., Frontali, M., Folstein, S., Ross, C., Franz, M., Abbott, M., et al. (1993) · Nature Genetics · doi:10.1038/ng0893-387 · Cited by 1,089
Self-citationChen, M., Ona, V. O., Li, M., Ferrante, R. J., Fink, K., Zhu, S., Bian, J., Guo, L., Farrell, L. A., Hersch, S. M., et al. (2000) · Nature Medicine · doi:10.1038/77528 · Cited by 1,060
Self-citationNuclear 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
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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
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
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
Harper, S. Q., Staber, P. D., He, X., Eliason, S. L., Martins, I. H., Mao, Q., Yang, L., Kotin, R. M., Paulson, H. L., & Davidson, B. L. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0501507102 · Cited by 682
Mitochondrial creatine kinase in human health and disease
Schlattner, U., Tokarska-Schlattner, M., & Wallimann, T. (2005) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2005.09.004 · Cited by 641
Metadata
- Authors
- 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., Hersch, S. M., Ross, C. A., & Beal, M. F.
- Affiliations
- Harvard University (US)
- Boston University (US)
- NewYork–Presbyterian Hospital (US)
- Johns Hopkins University (US)
- Emory University (US)
- ORCID
- Venue
- Neurobiology of Disease
- Publisher
- Elsevier BV
- Published
- 2001-06
- Volume
- 8
- Issue
- 3
- Pages
- 479-491
- ISSN
- 0969-9961 (ISSN-L)
- OpenAlex
- W1997883336
- Cited by
- 293
- References
- 54
- Citations by year
- 2026: 3 · 2025: 7 · 2024: 7 · 2023: 13 · 2022: 4 · 2021: 3 · 2020: 1 · 2019: 6 · 2018: 6 · 2017: 5 · 2016: 7 · 2015: 4 · 2014: 17 · 2013: 12 · 2012: 7
- FWCI
- 8.01
- Type
- journal article
- Language
- en
- Open access
- green
- doaj.org
- Licence
- Locations
- Neurobiology of Disease (publishedVersion)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- National Institutes of Health (5r29ns037102-02)
- National Institute on Aging (AG 12992, AG 13846, P30AG013846, P01AG012992)
- National Institute of Neurological Disorders and Stroke (NS37102, NS16375, NS 35255, NS 38144, NS38180, R01NS035255, R01NS038144)
- National Center for Complementary and Integrative Health (AT00613)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- creatine supplementation, Huntington's disease, transgenic mouse model, polyglutamine expansion, N-terminal huntingtin fragments, motor symptoms, survival, brain atrophy, intranuclear inclusions, mitochondrial permeability transition, hyperglycemia, dietary supplementation
- MeSH terms
- Huntingtin Protein, Animals, Aspartic Acid, Blood Glucose, Brain Chemistry, Cell Survival, Creatinine, Disease Models, Animal, Energy Metabolism, Female, Huntington Disease, Hyperglycemia, Insulin, Male, Mice, Transgenic, Motor Activity, Nerve Tissue Proteins, Neurons, Magnetic Resonance Spectroscopy, Nuclear Proteins, Organ Size, Survival Rate, Neostriatum, Mice
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