Cited by 107Genetic Neurodegenerative DiseasesMitochondrial Function and PathologyRedox biology and oxidative stress

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Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question

Armstrong, Jeffrey S., Khdour, Omar, Hecht, Sidney M.·2010·The FASEB Journal

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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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Authors: Armstrong, J. S., Khdour, O., & Hecht, S. M.
Affiliations: Arizona State University (US)
Corresponding author: Armstrong, Jeffrey S.
ORCID: Khdour, Omar
Hecht, Sidney M.
Venue: The FASEB Journal
Publisher: Wiley
Published: 2010-03-10
Volume: 24
Issue: 7
Pages: 2152-2163
ISSN: 0892-6638 (ISSN-L)
1530-6860
DOI: 10.1096/fj.09-143222
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
Licence: onlinelibrary.wiley.com/termsAndConditions#vor (vor)
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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Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question

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