Cited by 293Genetic Neurodegenerative DiseasesMetabolism and Genetic DisordersAmyotrophic Lateral Sclerosis Research

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

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Andreassen, Ole A., Dedeoglu, Alpaslan, Ferrante, Robert J., Jenkins, Bruce G., Ferrante, Kimberly L., Thomas, Melissa, Friedlich, Avi, Browne, Susan E., Schilling, Gabriele, Borchelt, David R., Hersch, Steven M., Ross, Christopher A., Beal, M.Flint·2001·Neurobiology of Disease

DOI 10.1006/nbdi.2001.0406

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Abstract

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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Self-citation
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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: Andreassen, Ole A.
Dedeoglu, Alpaslan
Ferrante, Kimberly L.
Thomas, Melissa
Browne, Susan E.
Borchelt, David R.
Hersch, Steven M.
Ross, Christopher A.
Beal, M.Flint
Venue: Neurobiology of Disease
Publisher: Elsevier BV
Published: 2001-06
Volume: 8
Issue: 3
Pages: 479-491
ISSN: 0969-9961 (ISSN-L)
DOI: 10.1006/nbdi.2001.0406
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: cc-by-sa
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
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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Creatine Increases Survival and Delays Motor Symptoms in a Transgenic Animal Model of Huntington's Disease

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