Increased basal ganglia iron levels in Huntington disease
Abstract
OBJECTIVE: To quantify in vivo brain ferritin iron levels in patients with Huntington disease (HD) and normal control subjects. DESIGN AND SUBJECTS: A magnetic resonance imaging method that can quantify ferritin iron levels with specificity in vivo was employed to study 11 patients with HD and a matched group of 27 normal controls. Three basal ganglia structures (caudate, putamen, and globus pallidus) and 1 comparison region (frontal lobe white matter) were evaluated. RESULTS: Basal ganglia iron levels were significantly increased (P<.002) in patients with HD, and this increase occurred early in the disease process. This was not a generalized phenomenon, as white matter iron levels were lower in patients with HD. CONCLUSIONS: The data suggest that increased iron levels may be related to the pattern of neurotoxicity observed in HD. Reducing the oxidative stress associated with increased iron levels may offer novel ways to delay the rate of progression and possibly defer the onset of HD.
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Alterations in brain iron metabolism with increased iron accumulation were identified by MRI in the striata of patients with Huntington's disease and in particular the basal ganglia (caudate nucleus, putamen, and globus pallidus).
“Alterations in brain iron metabolism with increased iron accumulation were identified by MRI in the striata of patients with Huntington’s disease194 and in particular the basal ganglia (caudate nucleus, putamen, and globus pallidus).195,196”
From The role of iron in brain ageing and neurodegenerative disorders (2014) · cites this paper as [195]
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- Authors
- Bartzokis, G., Cummings, J., Perlman, S., Hance, D. B., & Mintz, J.
- Affiliations
- Central Arkansas Veterans Healthcare System (US)
- University of California, Los Angeles (US)
- Venue
- Archives of Neurology
- Publisher
- American Medical Association (AMA)
- Published
- 1999-05-01
- Volume
- 56
- Issue
- 5
- Pages
- 569
- ISSN
- 0003-9942 (ISSN-L)
- OpenAlex
- W2008362268
- Cited by
- 183
- References
- 49
- Citations by year
- 2026: 1 · 2025: 4 · 2024: 3 · 2023: 4 · 2022: 9 · 2021: 13 · 2020: 5 · 2019: 6 · 2018: 7 · 2017: 6 · 2016: 3 · 2015: 12 · 2014: 11 · 2013: 8 · 2012: 12
- FWCI
- 1.55
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Archives of Neurology (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 › Hematology › Iron Metabolism and Disorders
- Keywords
- Huntington's disease, basal ganglia, brain iron, ferritin, caudate nucleus, putamen, globus pallidus, oxidative stress, neurotoxicity, disease progression
- MeSH terms
- Adult, Aged, Basal Ganglia, Female, Ferritins, Humans, Huntington Disease, Male, Middle Aged, Neurotoxins, Oxidative Stress, Disease Progression
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