The DOI matches its Crossref record; 101 of the 101 references it cites were found in a registry or an index.
Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question
Abstract
Friedreich's ataxia (FRDA) is a hereditary neurodegenerative disease that frequently culminates in cardiac failure at an early age. FRDA is believed to arise from reduced synthesis of the mitochondrial iron chaperone frataxin due to impaired gene transcription, which leads to mitochondrial iron accumulation, dysfunction of mitochondrial Fe‐S containing enzymes, and increased Fenton‐mediated free radical production. Recent reports have challenged this generally accepted hypothesis, by suggesting that the oxidative stress component in FRDA is minimal and thereby questioning the benefit of antioxidant therapeutic strategies. We suggest that this apparent paradox results from the radically divergent chemistries of the participating reactive oxygen species (ROS), the major cellular subcompartments involved and the overall cellular responses to ROS. In this review, we consider these factors and conclude that oxidative stress does constitute a major contributing factor to FRDA pathology. This reaffirms the idea that the rational design of specific small molecule multifunctional antioxidants will benefit FRDA patients.—Armstrong, J. S., Khdour, O., Hecht, S. M. Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question. FASEBJ. 24, 2152–2163 (2010). www.fasebj.org
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“In addition, the hypoxia-induced suppression of Fe-S protein activity can be contrasted with the suppression of Fe-S protein activity found in Friedreich's ataxia, a hereditary neurogenerative disease that results from decreased production/function of the frataxin protein (17, 220).”
From Redox regulation of mitochondrial function (2012) · cites this paper as [17]
In the citing paper
“Increased production of mitochondrial oxidants was found to correlate with a decrease in both total GSH as well as a decrease in the GSH/GSSG ratio in cells with frataxin deficiency; these changes may lead to the increased accumulation of protein S-glutathiolation in this disease (17).”
From Redox regulation of mitochondrial function (2012) · cites this paper as [17]
In the citing paper
“Decreased expression of frataxin also increases the Fenton reaction due to excess free iron (17, 220).”
From Redox regulation of mitochondrial function (2012) · cites this paper as [17]
Citations
Cites
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Friedreich's Ataxia: Autosomal Recessive Disease Caused by an Intronic GAA Triplet Repeat Expansion
Campuzano, V., Montermini, L., Moltò, M. D., Pianese, L., Cossée, M., Cavalcanti, F., Monros, E., Rodius, F., Duclos, F., Monticelli, A., et al. (1996) · Science · doi:10.1126/science.271.5254.1423 · Cited by 2,786
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Oxidative Phosphorylation at the fin de siècle
Saraste, M. (1999) · Science · doi:10.1126/science.283.5407.1488 · Cited by 1,345
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Manto, M. U., & Hampe, C. S. (2016) · Springer eBooks · doi:10.1007/978-3-319-24551-5_81
Pandolfo, M. (2020) · Elsevier eBooks · doi:10.1016/b978-0-12-813866-3.00006-0
Manto, M. U., & Hampe, C. S. (2023) · Springer eBooks · doi:10.1007/978-3-031-15070-8_91
Manto, M. U. (2023) · Springer eBooks · doi:10.1007/978-3-031-15070-8_92
Squillaci, G., Cotticelli, M. G., Carbone, V., Westfall, A. O., Wilson, R. B., & Morana, A. (2025) · Molecules · doi:10.3390/molecules31010070
Oxidative Stress and Friedreich’s Ataxia
Bolotta, A. (2014) · AMS Dottorato Institutional Doctoral Theses Repository (University of Bologna) · doi:10.6092/unibo/amsdottorato/6282
Metadata
- Authors
- Armstrong, J. S., Khdour, O., & Hecht, S. M.
- Affiliations
- Arizona State University (US)
- Corresponding author
- Armstrong, Jeffrey S.
- Venue
- The FASEB Journal
- Publisher
- Wiley
- Published
- 2010-03-10
- Volume
- 24
- Issue
- 7
- Pages
- 2152-2163
- ISSN
- 0892-6638 (ISSN-L)
- 1530-6860
- OpenAlex
- W2032253254
- Cited by
- 107
- References
- 101
- Citations by year
- 2025: 1 · 2024: 1 · 2023: 4 · 2022: 2 · 2021: 3 · 2020: 8 · 2019: 6 · 2018: 11 · 2017: 5 · 2016: 6 · 2015: 4 · 2014: 11 · 2013: 13 · 2012: 16
- FWCI
- 1.30
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The FASEB Journal (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Redox biology and oxidative stress
- Keywords
- Friedreich's ataxia, oxidative stress, frataxin, mitochondrial iron accumulation, reactive oxygen species, Fenton reaction, antioxidant therapy, neurodegeneration, heart failure, gene transcription
- MeSH terms
- Frataxin, Energy Metabolism, Friedreich Ataxia, Humans, Iron-Sulfur Proteins, Oxidation-Reduction, Oxidative Stress, Iron-Binding Proteins
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