The DOI matches its Crossref record; 7 of the 7 references it cites were found in a registry or an index.
Impaired Nuclear Nrf2 Translocation Undermines the Oxidative Stress Response in Friedreich Ataxia
Open accessAbstract
BACKGROUND: Friedreich ataxia originates from a decrease in mitochondrial frataxin, which causes the death of a subset of neurons. The biochemical hallmarks of the disease include low activity of the iron sulfur cluster-containing proteins (ISP) and impairment of antioxidant defense mechanisms that may play a major role in disease progression. METHODOLOGY/PRINCIPAL FINDINGS: We thus investigated signaling pathways involved in antioxidant defense mechanisms. We showed that cultured fibroblasts from patients with Friedreich ataxia exhibited hypersensitivity to oxidative insults because of an impairment in the Nrf2 signaling pathway, which led to faulty induction of antioxidant enzymes. This impairment originated from previously reported actin remodeling by hydrogen peroxide. CONCLUSIONS/SIGNIFICANCE: Thus, the defective machinery for ISP synthesis by causing mitochondrial iron dysmetabolism increases hydrogen peroxide production that accounts for the increased susceptibility to oxidative stress.
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Friedreich's Ataxia: Autosomal Recessive Disease Caused by an Intronic GAA Triplet Repeat Expansion
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PGAM5 tethers a ternary complex containing Keap1 and Nrf2 to mitochondria
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Friedreich ataxia: the oxidative stress paradox
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Sykiotis, G. P., & Bohmann, D. (2010) · Science Signaling · doi:10.1126/scisignal.3112re3 · Cited by 776
Lane, D. J. R., Merlot, A. M., Huang, M. L.-H., Bae, D.-H., Jansson, P. J., Sahni, S., Kalinowski, D. S., & Richardson, D. R. (2015) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2015.01.021 · Cited by 399
The multifaceted role of Nrf2 in mitochondrial function
Holmström, K. M., Kostov, R. V., & Dinkova-Kostova, A. T. (2016) · Current Opinion in Toxicology · doi:10.1016/j.cotox.2016.10.002 · Cited by 368
The role of Nrf2 signaling in counteracting neurodegenerative diseases
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Metadata
- Authors
- Paupe, V., Dassa, E. P., Goncalves, S., Auchère, F., Lönn, M., Holmgren, A., & Rustin, P.
- Venue
- PLoS ONE
- Publisher
- Public Library of Science (PLoS)
- Published
- 2009-01-22
- Volume
- 4
- Issue
- 1
- Pages
- e4253
- ISSN
- 1932-6203 (ISSN-L)
- OpenAlex
- W2071849674
- Cited by
- 218
- References
- 7
- FWCI
- 5.02
- Type
- journal article
- Language
- en
- Open access
- gold
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- Paupe, V., Dassa, E. P., Goncalves, S., Auchère, F., Lönn, M., Holmgren, A., & Rustin, P. (2009). Impaired Nuclear Nrf2 Translocation Undermines the Oxidative Stress Response in Friedreich Ataxia. PLoS ONE. https://doi.org/10.1371/journal.pone.0004253
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