Targeting NRF2 for the Treatment of Friedreich’s Ataxia: A Comparison among Drugs
Abstract
:1. Introduction
2. Results
2.1. The Pathway of NRF2 Is Down-Regulated in Fibroblasts of FRDA Patients
2.2. The NRF2 Expression Is Modulated by Redox-Active Drugs
2.3. Redox-Active Drugs Promote Differential Patterns of NRF2 Induction in FRDA Fibroblasts
2.4. NRF2 Inducers Increase the Expression of FXN Gene in FRDA Fibroblasts
2.5. The Drug-Mediated NRF2 Induction Is Time Dependent
2.6. The NRF2 Deficiency is Mediated by “Keap-1/DJ-1/p62 Axis” in FRDA Fibroblasts
3. Discussion
4. Materials and Methods
4.1. Fibroblasts Cultures
4.2. Quantitative Real-Time PCR (qRT-PCR)
4.3. GSH Assay
4.4. Western Blot Analysis
4.5. Statistical Analysis
5. Conclusions
Author Contributions
Funding
Acknowledgments
Conflicts of Interest
References
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Patient | Age (Yrs) | Sex | GAA Repeats | Cardiomyopathy | Diabetes |
---|---|---|---|---|---|
#1 | 19 | F | 680/350 | No | No |
#2 | 14 | M | 848/848 | Yes | No |
#3 | 8 | M | 448/848 | Yes | No |
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Petrillo, S.; D’Amico, J.; La Rosa, P.; Bertini, E.S.; Piemonte, F. Targeting NRF2 for the Treatment of Friedreich’s Ataxia: A Comparison among Drugs. Int. J. Mol. Sci. 2019, 20, 5211. https://doi.org/10.3390/ijms20205211
Petrillo S, D’Amico J, La Rosa P, Bertini ES, Piemonte F. Targeting NRF2 for the Treatment of Friedreich’s Ataxia: A Comparison among Drugs. International Journal of Molecular Sciences. 2019; 20(20):5211. https://doi.org/10.3390/ijms20205211
Chicago/Turabian StylePetrillo, Sara, Jessica D’Amico, Piergiorgio La Rosa, Enrico Silvio Bertini, and Fiorella Piemonte. 2019. "Targeting NRF2 for the Treatment of Friedreich’s Ataxia: A Comparison among Drugs" International Journal of Molecular Sciences 20, no. 20: 5211. https://doi.org/10.3390/ijms20205211