Cholesterol metabolism in Huntington disease
Abstract
The CNS is rich in cholesterol, which is essential for neuronal development and survival, synapse maturation, and optimal synaptic activity. Alterations in brain cholesterol homeostasis are linked to neurodegeneration. Studies have demonstrated that Huntington disease (HD), a progressive and fatal neurodegenerative disorder resulting from polyglutamine expansion in the huntingtin protein, is associated with changes in cellular cholesterol metabolism. Emerging evidence from human and animal studies indicates that attenuated brain sterol synthesis and accumulation of cholesterol in neuronal membranes represent two distinct mechanisms occurring in the presence of mutant huntingtin that influence neuronal survival. Increased knowledge of how changes in intraneuronal cholesterol metabolism influence the pathogenesis of HD will provide insights into the potential application of brain cholesterol regulation as a therapeutic strategy for this devastating disease.
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Altered cholesterol, HDL and LDL metabolism, gene variants of APOA1, APOB, and APOE4, and decreased ApoA1 levels have been implicated in other neurologic disorders.
“Altered cholesterol, HDL and LDL metabolism, gene variants of APOA1, APOB, and APOE4, and decreased ApoA1 levels have been implicated in other neurologic disorders [21–35].”
From Sterol and lipid analyses identifies hypolipidemia and apolipoprotein disorders in autism associated with adaptive functioning deficits (2021) · cites this paper as [23]
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References 126 · Cited by 154
- Authors
- Karasinska, J. M., & Hayden, M. R.
- Affiliations
- University of British Columbia (CA)
- Corresponding author
- Hayden, Michael R.
- Venue
- Nature Reviews Neurology
- Published
- 2011-09-06
- Volume
- 7
- Issue
- 10
- Pages
- 561-572
- ISSN
- 1759-4758 (ISSN-L)
- 1759-4766
- OpenAlex
- W2010404670
- Cited by
- 154
- References
- 126
- Citations by year
- 2026: 3 · 2025: 8 · 2024: 11 · 2023: 13 · 2022: 13 · 2021: 15 · 2020: 8 · 2019: 11 · 2018: 7 · 2017: 6 · 2016: 7 · 2015: 15 · 2014: 11 · 2013: 14 · 2012: 11
- FWCI
- 1.12
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature Reviews Neurology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Canadian Institutes of Health Research (MOP106684, MOP-84438)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
- Health Sciences › Medicine › Surgery › Cholesterol and Lipid Metabolism
- Keywords
- Huntington's disease, cholesterol metabolism, brain cholesterol homeostasis, sterol biosynthesis, mutant huntingtin, neurodegeneration, neuronal survival, therapeutic target
- MeSH terms
- Animals, Brain, Cholesterol, Humans, Huntington Disease, Nerve Degeneration
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