The Energetics of Huntington's Disease

Browne, Susan E.; Beal, M. Flint

doi:10.1023/b:nere.0000014824.04728.dd

Cited by 240Genetic Neurodegenerative DiseasesNeurological diseases and metabolismAmyotrophic Lateral Sclerosis Research

The DOI matches its Crossref record; 144 of the 144 references it cites were found in a registry or an index.

The Energetics of Huntington's Disease

Browne, Susan E., Beal, M. Flint·2004·Neurochemical Research

DOI 10.1023/b:nere.0000014824.04728.dd

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Abstract

Huntington's disease (HD) is a hereditary neurodegenerative disorder that gradually robs sufferers of the ability to control movements and induces psychological and cognitive impairments. This devastating, lethal disease is one of several neurological disorders caused by trinucleotide expansions in affected genes, including spinocerebellar ataxias, dentatorubral-pallidoluysian atrophy, and spinal bulbar muscular atrophy. HD symptoms are associated with region-specific neuronal loss within the central nervous system, but to date the mechanism of this selective cell death remains unknown. Strong evidence from studies in humans and animal models suggests the involvement of energy metabolism defects, which may contribute to excitotoxic processes, oxidative dmage, and altered gene regulation. The development of transgenic mouse models expressing the human HD mutation has provided novel opportunities to explore events underlying selective neuronal death in HD, which has hitherto been impossible in humans. Here we discuss how animal models are redefining the role of energy metabolism in HD etiology.

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Biochemical studies revealed reduced activity of several key components of oxidative phosphorylation and the TCA cycle, including complexes II-III and aconitase, in advanced stage HD patients, while complex-I activity was unaltered.
“Biochemical studies revealed reduced activity of several key components of oxidative phosphorylation and the TCA cycle, including complexes II-III and aconitase, in advanced stage HD patients, while complex-I activity was unaltered40-43.”
From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [40]

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O'Brien, C. F., Miller, C., Goldblatt, D., Welle, S., Forbes, G., Lipinski, B., Panzik, J., Peck, R., Plumb, S., Oakes, D., Kurlan, R., and Shoulson, I. 1990. Extraneural metabolism in early Huntington's disease. Ann. Neurol. 28:300–301.
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Authors: Browne, S. E., & Beal, M. F.
Affiliations: Cornell University (US)
Corresponding author: Browne, Susan E.
ORCID: Browne, Susan E.
Venue: Neurochemical Research
Publisher: Springer Science and Business Media LLC
Published: 2004-03
Volume: 29
Issue: 3
Pages: 531-546
ISSN: 0364-3190 (ISSN-L)
1573-6903
DOI: 10.1023/b:nere.0000014824.04728.dd
OpenAlex: W2074304614
Cited by: 240
References: 144
Citations by year: 2026: 2 · 2025: 4 · 2024: 6 · 2023: 6 · 2022: 2 · 2021: 7 · 2020: 6 · 2019: 13 · 2018: 9 · 2017: 10 · 2016: 6 · 2015: 7 · 2014: 10 · 2013: 9 · 2012: 15
FWCI: 6.56
Type: journal article
Language: en
Open access: closed
Licence: www.springernature.com/gp/researchers/text-and-data-mining (tdm)
www.springernature.com/gp/researchers/text-and-data-mining (vor)
Locations: Neurochemical Research (publishedVersion)
PubMed (publishedVersion)
Topics: Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Keywords: Huntington's disease, energy metabolism, trinucleotide repeat expansion, selective neuronal death, excitotoxicity, oxidative damage, transgenic mouse model, neurodegeneration, spinocerebellar ataxia, dentatorubral-pallidoluysian atrophy, spinal and bulbar muscular atrophy
MeSH terms: Animals, Brain, Disease Models, Animal, Energy Metabolism, Glucose, Humans, Huntington Disease, Mitochondria, Models, Biological, Genetic Diseases, Inborn

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Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

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The Energetics of Huntington's Disease

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