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

Free Radicals in Biology and Medicine

Halliwell, B., & Gutteridge, J. M. C. (2015) · doi:10.1093/acprof:oso/9780198717478.001.0001 · Cited by 8,990 · see citing works

How mitochondria produce reactive oxygen species

Murphy, M. P. (2009) · Biochemical Journal · doi:10.1042/bj20081386 · Cited by 8,207 · see citing works

Hydroperoxide metabolism in mammalian organs.

Chance, B., Sies, H., & Boveris, A. (1979) · Physiological Reviews · doi:10.1152/physrev.1979.59.3.527 · Cited by 5,827 · see citing works

Mitochondrial formation of reactive oxygen species

Turrens, J. F. (2003) · The Journal of Physiology · doi:10.1113/jphysiol.2003.049478 · Cited by 5,084 · see citing works

Three-dimensional tracking of axonal projections in the brain by magnetic resonance imaging

Mori, S., Crain, B. J., Chacko, V. P., & van Zijl, P. C. M. (1999) · Annals of Neurology · doi:10.1002/1531-8249(199902)45:2 · Cited by 3,479 · see citing works

Mitochondrial free radical generation, oxidative stress, and aging

Cadenas, E., & Davies, K. J. A. (2000) · Free Radical Biology and Medicine · doi:10.1016/s0891-5849(00)00317-8 · Cited by 3,010 · see citing works

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 · see citing works

Dilated cardiomyopathy and neonatal lethality in mutant mice lacking manganese superoxide dismutase

Li, Y., Huang, T.-T., Carlson, E. J., Melov, S., Ursell, P. C., Olson, J. L., Noble, L. J., Yoshimura, M. P., Berger, C., Chan, P. H., et al. (1995) · Nature Genetics · doi:10.1038/ng1295-376 · Cited by 1,789 · see citing works

Generation of superoxide anion by the NADH dehydrogenase of bovine heart mitochondria

Turrens, J. F., & Boveris, A. (1980) · Biochemical Journal · doi:10.1042/bj1910421 · Cited by 1,601 · see citing works

Oxidative Phosphorylation at the fin de siècle

Saraste, M. (1999) · Science · doi:10.1126/science.283.5407.1488 · Cited by 1,318 · see citing works

The measurement and mechanism of lipid peroxidation in biological systems

Gutteridge, J. M. C., & Halliwell, B. B. (1990) · Trends in Biochemical Sciences · doi:10.1016/0968-0004(90)90206-q · Cited by 1,292 · see citing works

Ubisemiquinone is the electron donor for superoxide formation by complex III of heart mitochondria

Turrens, J. F., Alexandre, A., & Lehninger, A. L. (1985) · Archives of Biochemistry and Biophysics · doi:10.1016/0003-9861(85)90293-0 · Cited by 1,214 · see citing works

Aconitase and mitochondrial iron–sulphur protein deficiency in Friedreich ataxia

Rötig, A., de Lonlay, P., Chretien, D., Foury, F., Koenig, M., Sidi, D., Munnich, A., & Rustin, P. (1997) · Nature Genetics · doi:10.1038/ng1097-215 · Cited by 991 · see citing works

Architecture of succinate dehydrogenase and reactive oxygen species generation

Yankovskaya, V., Horsefield, R., Törnroth, S., Luna-Chavez, C., Miyoshi, H., Léger, C., Byrne, B., Cecchini, G., & Iwata, S. (2003) · Science · doi:10.1126/science.1079605 · Cited by 927 · see citing works

NRF2 and KEAP1 mutations: permanent activation of an adaptive response in cancer

Hayes, J. D., & McMahon, M. J. (2009) · Trends in Biochemical Sciences · doi:10.1016/j.tibs.2008.12.008 · Cited by 870 · see citing works

Superoxide in Apoptosis

Cai, J., & Jones, D. P. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.19.11401 · Cited by 765 · see citing works

Lipid peroxidation of membrane phospholipids generates hydroxy-alkenals and oxidized phospholipids active in physiological and/or pathological conditions

Catalá, A. (2009) · Chemistry and Physics of Lipids · doi:10.1016/j.chemphyslip.2008.09.004 · Cited by 745 · see citing works

Frataxin is Reduced in Friedreich Ataxia Patients and is Associated with Mitochondrial Membranes

Campuzano, V., Montermini, L., Lutz, Y., Cova, L., Hindelang, C., Jiralerspong, S., Trottier, Y., Kish, S. J., Faucheux, B. A., Trouillas, P., et al. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.11.1771 · Cited by 739 · see citing works

Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits

Puccio, H., Simon, D., Cossée, M., Criqui-Filipe, P., Tiziano, F., Melki, J., Hindelang, C., Matyas, R., Rustin, P., & Koenig, M. (2001) · Nature Genetics · doi:10.1038/84818 · Cited by 738 · see citing works

Voltage-dependent anion channels control the release of the superoxide anion from mitochondria to cytosol

Han, D., Antunes, F., Canali, R., Rettori, D., & Cadenas, E. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m210269200 · Cited by 711 · see citing works

Complex I-mediated reactive oxygen species generation: modulation by cytochrome c and NAD(P)+ oxidation–reduction state

Kushnareva, Y., Murphy, A. N., & Andreyev, A. Y. (2002) · Biochemical Journal · doi:10.1042/bj20021121 · Cited by 677 · see citing works

Cysteine desulfurase activity indicates a role for NIFS in metallocluster biosynthesis.

Zheng, L., White, R. H., Cash, V. L., Jack, R. F., & Dean, D. R. (1993) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.90.7.2754 · Cited by 597 · see citing works

The protonmotive Q cycle. Energy transduction by coupling of proton translocation to electron transfer by the cytochrome bc1 complex.

Trumpower, B. L. (1990) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)38410-8 · Cited by 596 · see citing works

Superoxide sensitivity of the Escherichia coli aconitase.

Gardner, P. R., & Fridovich, I. (1991) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)55001-8 · Cited by 514 · see citing works

Role of glutathione depletion and reactive oxygen species generation in apoptotic signaling in a human B lymphoma cell line

Armstrong, J. S., Steinauer, K. K., Hornung, B., Irish, J. M., Lecane, P. S., Birrell, G. W., Peehl, D. M., & Knox, S. J. (2002) · Cell Death and Differentiation · doi:10.1038/sj.cdd.4400959 · Cited by 507 · see citing works

S-glutathionylation in protein redox regulation

Dalle‐Donne, I., Rossi, R., Giustarini, D., Colombo, R., & Milzani, A. (2007) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2007.06.014 · Cited by 498 · see citing works

Mitochondrial Oxygen Radical Generation and Leak: Sites of Production in States 4 and 3, Organ Specificity, and Relation to Aging and Longevity

Barja, G. (1999) · Journal of Bioenergetics and Biomembranes · doi:10.1023/a:1005427919188 · Cited by 494 · see citing works

Redox control and oxidative stress in yeast cells

Herrero, E., Ros, J., Bellı́, G., & Cabiscol, E. (2008) · Biochimica et Biophysica Acta (BBA) - General Subjects · doi:10.1016/j.bbagen.2007.12.004 · Cited by 488 · see citing works

Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin

Koutníková, H., Campuzano, V., Foury, F., Dollé, P., Cazzalini, O., & Koenig, M. (1997) · Nature Genetics · doi:10.1038/ng0897-345 · Cited by 473 · see citing works

Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia

Herman, D., Jenssen, K., Burnett, R., Soragni, E., Perlman, S. L., & Gottesfeld, J. M. (2006) · Nature Chemical Biology · doi:10.1038/nchembio815 · Cited by 442 · see citing works

The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis

Wong, A. (1999) · Human Molecular Genetics · doi:10.1093/hmg/8.3.425 · Cited by 374 · see citing works

Iron−Sulfur Cluster Biosynthesis. Characterization of Frataxin as an Iron Donor for Assembly of [2Fe-2S] Clusters in ISU-Type Proteins

Yoon, T., & Cowan, J. A. (2003) · Journal of the American Chemical Society · doi:10.1021/ja027967i · Cited by 353 · see citing works

Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia

Lodi, R., Cooper, J. A. D., Bradley, J. L., Manners, D. N., Styles, P., Taylor, D. J., & Schapira, A. H. V. (1999) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.96.20.11492 · Cited by 352 · see citing works

Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue

Wilson, R. B., & Roof, D. M. (1997) · Nature Genetics · doi:10.1038/ng0897-352 · Cited by 345 · see citing works

Oxidative stress in patients with Friedreich ataxia

Schulz, J. B., Dehmer, T., Schöls, L. J., Mende, H. E., Hardt, C., Vorgerd, M., Bürk, K., Matson, W. R., DICHGANS, J., Beal, M. F., et al. (2000) · Neurology · doi:10.1212/wnl.55.11.1719 · Cited by 305 · see citing works

Structures and Proton-Pumping Strategies of Mitochondrial Respiratory Enzymes

Schultz, B. E., & Chan, S. I. (2001) · Annual Review of Biophysics and Biomolecular Structure · doi:10.1146/annurev.biophys.30.1.23 · Cited by 299 · see citing works

The site of production of superoxide radical in mitochondrial Complex I is not a bound ubisemiquinone but presumably iron–sulfur cluster N2

Genova, M. L., Ventura, B., Giuliano, G., Bovina, C., Formiggini, G., Castelli, G. P., & Lenaz, G. (2001) · FEBS Letters · doi:10.1016/s0014-5793(01)02850-2 · Cited by 297 · see citing works

High-affinity transport of glutathione is part of a multicomponent system essential for mitochondrial function.

Mårtensson, J., Lai, J. C. K., & Meister, A. (1990) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.87.18.7185 · Cited by 282 · see citing works

The Yeast Frataxin Homologue Mediates Mitochondrial Iron Efflux

Radisky, D. C., Babcock, M. C., & Kaplan, J. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.8.4497 · Cited by 277 · see citing works

Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS

Adinolfi, S., Iannuzzi, C., Prischi, F., Pastore, C., Iametti, S., Martin, S. R., Bonomi, F., & Pastore, A. (2009) · Nature Structural & Molecular Biology · doi:10.1038/nsmb.1579 · Cited by 256 · see citing works

Iron-Dependent Self-Assembly of Recombinant Yeast Frataxin: Implications for Friedreich Ataxia

Adamec, J., Rusnak, F., Owen, W. G., Naylor, S., Benson, L. M., Gacy, A. M., & Isaya, G. (2000) · The American Journal of Human Genetics · doi:10.1086/303056 · Cited by 255 · see citing works

Intracellular generation of reactive oxygen species by mitochondria

Nohl, H., Gille, L., & Staniek, K. (2005) · Biochemical Pharmacology · doi:10.1016/j.bcp.2004.12.002 · Cited by 251 · see citing works

The pathogenesis of Friedreich ataxia and the structure and function of frataxin

Pandolfo, M., & Pastore, A. (2009) · Journal of Neurology · doi:10.1007/s00415-009-1003-2 · Cited by 242 · see citing works

Frataxin activates mitochondrial energy conversion and oxidative phosphorylation

Ristow, M., Pfister, M. F., Yee, A. J., Schubert, M., Michael, L., Zhang, C., Ueki, K., Michael, M. D., Lowell, B. B., & Kahn, C. R. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.220403797 · Cited by 221 · see citing works

Friedreich ataxia: the oxidative stress paradox

Seznec, H., Simon, D., Bouton, C., Reutenauer, L., Hertzog, A., Golik, P., Procaccio, V., Patel, M., Drapier, J.-C., Koenig, M., et al. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi042 · Cited by 219 · see citing works

Impaired Nuclear Nrf2 Translocation Undermines the Oxidative Stress Response in Friedreich Ataxia

Paupe, V., Dassa, E. P., Gonçalves, S., Auchère, F., Lönn, M. E., Holmgren, A., & Rustin, P. (2009) · PLoS ONE · doi:10.1371/journal.pone.0004253 · Cited by 218 · see citing works

HDAC Inhibitors Correct Frataxin Deficiency in a Friedreich Ataxia Mouse Model

Rai, M., Soragni, E., Jenssen, K., Burnett, R., Herman, D., Coppola, G., Geschwind, D. H., Gottesfeld, J. M., & Pandolfo, M. (2008) · PLoS ONE · doi:10.1371/journal.pone.0001958 · Cited by 216 · see citing works

Effect of Glutathione Depletion on Sites and Topology of Superoxide and Hydrogen Peroxide Production in Mitochondria

Han, D., Canali, R., Rettori, D., & Kaplowitz, N. (2003) · Molecular Pharmacology · doi:10.1124/mol.64.5.1136 · Cited by 212 · see citing works

Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant

Huang, M. L.-H., Becker, E. M., Whitnall, M., Rahmanto, Y. S., Ponka, P., & Richardson, D. R. (2009) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0906784106 · Cited by 212 · see citing works

Localization at Complex I and Mechanism of the Higher Free Radical Production of Brain Nonsynaptic Mitochondria in the Short-Lived Rat Than in the Longevous Pigeon

Barja, G., & Herrero, A. (1998) · Journal of Bioenergetics and Biomembranes · doi:10.1023/a:1020592719405 · Cited by 203 · see citing works

Iron–sulfur cluster biosynthesis in bacteria: Mechanisms of cluster assembly and transfer

Fontecave, M., & de Choudens, S. O. (2008) · Archives of Biochemistry and Biophysics · doi:10.1016/j.abb.2007.12.014 · Cited by 202 · see citing works

Autosomal recessive cerebellar ataxias

Palau, F., & Espinós, C. (2006) · Orphanet Journal of Rare Diseases · doi:10.1186/1750-1172-1-47 · Cited by 198 · see citing works

Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia

Cavadini, P. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.3.217 · Cited by 197 · see citing works

Disabled early recruitment of antioxidant defenses in Friedreich's ataxia

Chantrel-Groussard, K. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.19.2061 · Cited by 189 · see citing works

Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity

Gakh, O., Park, S., Liu, G., Macomber, L., Imlay, J. A., Ferreira, G. C., & Isaya, G. (2006) · Human Molecular Genetics · doi:10.1093/hmg/ddi461 · Cited by 184 · see citing works

Iron–sulfur protein maturation in human cells: evidence for a function of frataxin

Stehling, O., Elsässer, H.-P., Brückel, B., Mühlenhoff, U., & Lill, R. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddh324 · Cited by 176 · see citing works

Coenzyme Q 10 and vitamin E deficiency in Friedreich’s ataxia: predictor of efficacy of vitamin E and coenzyme Q 10 therapy

Cooper, J. A. D., Korlipara, L. V. P., Hart, P. E., Bradley, J. L., & Schapira, A. H. V. (2008) · European Journal of Neurology · doi:10.1111/j.1468-1331.2008.02318.x · Cited by 163 · see citing works

Mitochondrial ferritin limits oxidative damage regulating mitochondrial iron availability: hypothesis for a protective role in Friedreich ataxia

Campanella, A., Rovelli, E., Santambrogio, P., Cozzi, A., Taroni, F., & Levi, S. (2008) · Human Molecular Genetics · doi:10.1093/hmg/ddn308 · Cited by 162 · see citing works

Actin Glutathionylation Increases in Fibroblasts of Patients with Friedreich's Ataxia

Pastore, A., Tozzi, G., Gaeta, L. M., Bertini, E., Serafini, V., Cesare, S. D., Bonetto, V., Casoni, F., Carrozzo, R., Federici, G., et al. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m301872200 · Cited by 161 · see citing works

Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones

Shan, Y., Napoli, E., & Cortopassi, G. A. (2007) · Human Molecular Genetics · doi:10.1093/hmg/ddm038 · Cited by 152 · see citing works

Mitochondrial Production of Hydrogen Peroxide Regulation by Nitric Oxide and the Role of Ubisemiquinone

Boveris, A., & Cadenas, E. (2000) · IUBMB Life · doi:10.1080/713803732 · Cited by 148 · see citing works

Frataxin interacts functionally with mitochondrial electron transport chain proteins

González-Cabo, P., Vázquez-Manrique, R. P., García-Gimeno, M. A., Sanz, P., & Palau, F. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi214 · Cited by 140 · see citing works

Production of Superoxide Anions by Keratinocytes Initiates P. acnes-Induced Inflammation of the Skin

Grange, P., Chéreau, C., Raingeaud, J., Nicco, C., Weill, B. J., Dupin, N., & Batteux, F. (2009) · PLoS Pathogens · doi:10.1371/journal.ppat.1000527 · Cited by 140 · see citing works

Iron Binding and Oxidation Kinetics in Frataxin CyaY of Escherichia coli

Bou‐Abdallah, F., Adinolfi, S., Pastore, A., Laue, T. M., & Chasteen, N. D. (2004) · Journal of Molecular Biology · doi:10.1016/j.jmb.2004.05.072 · Cited by 129 · see citing works

Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation

Kakhlon, O., Manning, H., Breuer, W. V., Melamed‐Book, N., Lu, C., Cortopassi, G. A., Münnich, A., & Cabantchik, Z. I. (2008) · Blood · doi:10.1182/blood-2008-06-161919 · Cited by 128 · see citing works

The MCK mouse heart model of Friedreich's ataxia: Alterations in iron-regulated proteins and cardiac hypertrophy are limited by iron chelation

Whitnall, M., Rahmanto, Y. S., Šuťák, R., Xu, X., Becker, E., Mikhael, M., Ponka, P., & Richardson, D. R. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0804261105 · Cited by 122 · see citing works

Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase

Koutníková, H. (1998) · Human Molecular Genetics · doi:10.1093/hmg/7.9.1485 · Cited by 122 · see citing works

Permeability of bilayer lipid membranes for superoxide (O2−) radicals

Gus'kova, R. A., Ivanov, I. I., Koltover, V. K., Akhobadze, V. V., & Rubin, A. (1984) · Biochimica et Biophysica Acta (BBA) - Biomembranes · doi:10.1016/0005-2736(84)90409-7 · Cited by 112 · see citing works

Hydrogen peroxide scavenging rescues frataxin deficiency in a <i>Drosophila</i> model of Friedreich's ataxia

Anderson, P. R., Kirby, K., Orr, W. C., Hilliker, A. J., & Phillips, J. P. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0709691105 · Cited by 105 · see citing works

Hypersensitivity to oxygen and shortened lifespan in a <i>Drosophila</i> mitochondrial complex II mutant

Walker, D. W., Hájek, P., Muffat, J., Knoepfle, D., Cornelison, S., Attardi, G., & Benzer, S. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0607918103 · Cited by 105 · see citing works

Recombinant human erythropoietin: effects on frataxin expression in vitro

Sturm, B., Stupphann, D., Kaun, C., Boesch, S., Schranzhofer, M., Wojta, J., Goldenberg, H., & Scheiber‐Mojdehkar, B. (2005) · European Journal of Clinical Investigation · doi:10.1111/j.1365-2362.2005.01568.x · Cited by 103 · see citing works

The Use of Transgenic and Mutant Mice to Study Oxygen Free Radical Metabolism

Huang, T., Carlson, E. J., Raineri, I., Gillespie, A. M., Kozy, H. M., & Epstein, C. J. (1999) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.1999.tb07820.x · Cited by 101 · see citing works

Assembly of Human Frataxin Is a Mechanism for Detoxifying Redox-Active Iron

O’Neill, H. A., Gakh, O., Park, S., Cui, J., Mooney, S. M., Sampson, M., Ferreira, G. C., & Isaya, G. (2004) · Biochemistry · doi:10.1021/bi048459j · Cited by 98 · see citing works

Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant

Vázquez‐Manrique, R. P., González‐Cabo, P., Ros, S., Aziz, H., Baylis, H. A., & Palau, F. (2005) · The FASEB Journal · doi:10.1096/fj.05-4212fje · Cited by 98 · see citing works

Frataxin, Iron–Sulfur Clusters, Heme, ROS, and Aging

Napoli, E., Taroni, F., & Cortopassi, G. A. (2006) · Antioxidants and Redox Signaling · doi:10.1089/ars.2006.8.506 · Cited by 96 · see citing works

Unique Iron Binding and Oxidation Properties of Human Mitochondrial Ferritin: A Comparative Analysis with Human H-chain Ferritin

Bou‐Abdallah, F., Santambrogio, P., Levi, S., Arosio, P., & Chasteen, N. D. (2005) · Journal of Molecular Biology · doi:10.1016/j.jmb.2005.01.007 · Cited by 96 · see citing works

Reduction of Hydrophilic Ubiquinones by the Flavin in Mitochondrial NADH:Ubiquinone Oxidoreductase (Complex I) and Production of Reactive Oxygen Species

King, M. S., Sharpley, M. S., & Hirst, J. (2009) · Biochemistry · doi:10.1021/bi802282h · Cited by 95 · see citing works

Frataxin knockdown causes loss of cytoplasmic iron–sulfur cluster functions, redox alterations and induction of heme transcripts

Lu, C., & Cortopassi, G. (2007) · Archives of Biochemistry and Biophysics · doi:10.1016/j.abb.2006.09.010 · Cited by 94 · see citing works

Binding of Yeast Frataxin to the Scaffold for Fe-S Cluster Biogenesis, Isu

Wang, T., & Craig, E. A. (2008) · Journal of Biological Chemistry · doi:10.1074/jbc.m800399200 · Cited by 94 · see citing works

The Ferroxidase Activity of Yeast Frataxin

Park, S., Gakh, O., Mooney, S. M., & Isaya, G. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m206711200 · Cited by 88 · see citing works

Glutathione-dependent redox status of frataxin-deficient cells in a yeast model of Friedreich's ataxia

Auchère, F., Santos, R., Planamente, S., Lesuisse, E., & Camadro, J. (2008) · Human Molecular Genetics · doi:10.1093/hmg/ddn178 · Cited by 87 · see citing works

Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells

Tan, G. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg187 · Cited by 86 · see citing works

Oxidative stress and protease dysfunction in the yeast model of Friedreich ataxia

Bulteau, A., Dancis, A., Gareil, M., Montagne, J.-J., Camadro, J., & Lesuisse, E. (2007) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2007.02.014 · Cited by 84 · see citing works

Frataxin expression rescues mitochondrial dysfunctions in FRDA cells

Tan, G. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.19.2099 · Cited by 82 · see citing works

Friedreich's Ataxia: From Disease Mechanisms to Therapeutic Interventions

Lodi, R., Tonon, C., Calabrese, V., & Schapira, A. H. V. (2006) · Antioxidants and Redox Signaling · doi:10.1089/ars.2006.8.438 · Cited by 79 · see citing works

Frataxin deficiency enhances apoptosis in cells differentiating into neuroectoderm

Santos, M. M. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.18.1935 · Cited by 79 · see citing works

Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells

Jiralerspong, S., Ge, B., Hudson, T. J., & Pandolfo, M. (2001) · FEBS Letters · doi:10.1016/s0014-5793(01)03140-4 · Cited by 78 · see citing works

Friedreich's Ataxia, No Changes in Mitochondrial Labile Iron in Human Lymphoblasts and Fibroblasts

Sturm, B., Bistrich, U., Schranzhofer, M., Sarsero, J. P., Rauen, U., Scheiber-Mojdehkar, B., de Groot, H., Ioannou, P., & Petrat, F. (2005) · Journal of Biological Chemistry · doi:10.1074/jbc.m408717200 · Cited by 73 · see citing works

Structure of Frataxin Iron Cores: An X-ray Absorption Spectroscopic Study

Nichol, H., Gakh, O., O’Neill, H. A., Pickering, I. J., Isaya, G., & George, G. N. (2003) · Biochemistry · doi:10.1021/bi027021l · Cited by 68 · see citing works

The effects of frataxin silencing in HeLa cells are rescued by the expression of human mitochondrial ferritin

Zanella, I., Derosas, M., Corrado, M., Cocco, E., Cavadini, P., Biasiotto, G., Poli, M., Verardi, R., & Arosio, P. (2007) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2007.11.006 · Cited by 60 · see citing works

Quality of potent Mn porphyrin-based SOD mimics and peroxynitrite scavengers for pre-clinical mechanistic/therapeutic purposes

Reboucas, J. S., Spasojević, I., & Batinic̈‐Haberle, I. (2008) · Journal of Pharmaceutical and Biomedical Analysis · doi:10.1016/j.jpba.2008.08.005 · Cited by 59 · see citing works

Impaired respiration is positively correlated with decreased life span in Caenorhabditis elegans models of Friedreich Ataxia

Zarse, K., Schulz, T. J., Birringer, M., & Ristow, M. (2007) · The FASEB Journal · doi:10.1096/fj.06-6994com · Cited by 58 · see citing works

Multicellular models of Friedreich ataxia

Puccio, H. M. (2009) · Journal of Neurology · doi:10.1007/s00415-009-1004-1 · Cited by 54 · see citing works

N-terminal iron-mediated self-cleavage of human frataxin: regulation of iron binding and complex formation with target proteins

Yoon, T., Dizin, E., & Cowan, J. A. (2007) · JBIC Journal of Biological Inorganic Chemistry · doi:10.1007/s00775-007-0205-2 · Cited by 41 · see citing works

Partial Correction of Sensitivity to Oxidant Stress in Friedreich Ataxia Patient Fibroblasts by Frataxin-Encoding Adeno-Associated Virus and Lentivirus Vectors

Fleming, J., Spinoulas, A., Zheng, M., Cunningham, S. C., Ginn, S. L., McQuilty, R. C., Rowe, P. B., & Alexander, I. E. (2005) · Human Gene Therapy · doi:10.1089/hum.2005.16.947 · Cited by 40 · see citing works

Frataxin deficiency and mitochondrial dysfunction

Pandolfo, M. (2002) · Mitochondrion · doi:10.1016/s1567-7249(02)00039-9 · Cited by 39 · see citing works

Intreactions between superoxide and nitric oxide Implications in DNA damage and mutagenesis

Grisham, M. B. (1997) · Frontiers in bioscience · doi:10.2741/a182 · Cited by 39 · see citing works

Functional studies of frataxin

Isaya, G., O'Neill, H., Gakh, O., Park, S., Mantcheva, R., & Mooney, S. (2004) · Acta Paediatrica · doi:10.1111/j.1651-2227.2004.tb03061.x · Cited by 37 · see citing works

The chemical form of mitochondrial iron in Friedreich’s ataxia

Popescu, B. F. G., Pickering, I. J., George, G. N., & Nichol, H. (2007) · Journal of Inorganic Biochemistry · doi:10.1016/j.jinorgbio.2007.03.004 · Cited by 36 · see citing works

Frataxin and frataxin deficiency in Friedreich's ataxia

Wilson, R. B. (2003) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(02)00432-x · Cited by 32 · see citing works

Mitochondrial superoxide anion production and release into intermembrane space

Han, D., Antunes, F., Daneri, F., & Cadenas, E. (2002) · Methods in enzymology on CD-ROM/Methods in enzymology · doi:10.1016/s0076-6879(02)49341-x · Cited by 28 · see citing works

The Role of Oxidative Stress and Hypoxia in Pancreatic Beta-Cell Dysfunction in Diabetes Mellitus

Gerber, P. A., & Rutter, G. A. (2017) · Antioxidants &amp; Redox Signaling · doi:10.1089/ars.2016.6755 · Cited by 654 · see citing works

Redox regulation of mitochondrial function

Handy, D. E., & Loscalzo, J. (2012) · Antioxidants &amp; Redox Signaling · doi:10.1089/ars.2011.4123 · Cited by 588 · see citing works

Ferroptosis and Its Role in Diverse Brain Diseases

Weiland, A., Wang, Y., Wu, W., Lan, X., Han, X., Li, Q., & Wang, J. (2018) · Molecular Neurobiology · doi:10.1007/s12035-018-1403-3 · Cited by 469 · see citing works

Dysregulation of Glutathione Homeostasis in Neurodegenerative Diseases

Johnson, W. M., Wilson‐Delfosse, A. L., & Mieyal, J. J. (2012) · Nutrients · doi:10.3390/nu4101399 · Cited by 354 · see citing works

Mild dehydration impairs cognitive performance and mood of men

Ganio, M. S., Armstrong, L. E., Casa, D. J., McDermott, B. P., Lee, E. C., Yamamoto, L. M., Marzano, S., Lopez, R. M., Jimenez, L., Le Bellego, L., et al. (2011) · British Journal of Nutrition · doi:10.1017/s0007114511002005 · Cited by 316 · see citing works

Mitochondrial iron–sulfur protein biogenesis and human disease

Stehling, O., Wilbrecht, C., & Lill, R. (2014) · Biochimie · doi:10.1016/j.biochi.2014.01.010 · Cited by 272 · see citing works

The Neuroprotective Effect of Klotho is Mediated via Regulation of Members of the Redox System

Zeldich, E., Chen, C.-D., Colvin, T. A., Bove‐Fenderson, E., Liang, J. J., Zhou, T. B. T., Harris, D. A., & Abraham, C. R. (2014) · Journal of Biological Chemistry · doi:10.1074/jbc.m114.567321 · Cited by 225 · see citing works

Iron –sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster delivery

Maio, N., & Rouault, T. A. (2014) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2014.09.009 · Cited by 215 · see citing works

Friedreich ataxia- pathogenesis and implications for therapies

Delatycki, M. B., & Bidichandani, S. I. (2019) · Neurobiology of Disease · doi:10.1016/j.nbd.2019.104606 · Cited by 160 · see citing works

The Nrf2 induction prevents ferroptosis in Friedreich's Ataxia

Rosa, P. L., Petrillo, S., Turchi, R., Berardinelli, F., Schirinzi, T., Vasco, G., Lettieri‐Barbato, D., Fiorenza, M. T., Bertini, E., Aquilano, K., et al. (2020) · Redox Biology · doi:10.1016/j.redox.2020.101791 · Cited by 149 · see citing works

Deferiprone in F riedreich ataxia: A 6‐Month randomized controlled trial

Pandolfo, M., Arpa, J., Delatycki, M. B., Sang, K. L. Q., Mariotti, C., Münnich, A., Sanz‐Gallego, I., Tai, G., Tarnopolsky, M. A., Taroni, F., et al. (2014) · Annals of Neurology · doi:10.1002/ana.24248 · Cited by 138 · see citing works

Ferroptosis as a Novel Therapeutic Target for Friedreich’s Ataxia

Cotticelli, M. G., Xia, S. J., Lin, D., Lee, T., Terrab, L., Wipf, P., Huryn, D. M., & Wilson, R. B. (2019) · Journal of Pharmacology and Experimental Therapeutics · doi:10.1124/jpet.118.252759 · Cited by 137 · see citing works

Antioxidants halt axonal degeneration in a mouse model of X‐adrenoleukodystrophy

López‐Erauskin, J., Fourcade, S., Galino, J., Ruíz, M., Schlüter, A., Naudí, A., Jové, M., Portero‐Otín, M., Pamplona, R., Ferrer, I., et al. (2011) · Annals of Neurology · doi:10.1002/ana.22363 · Cited by 135 · see citing works

NADPH oxidases as therapeutic targets in ischemic stroke

Kahles, T., & Brandes, R. P. (2012) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-012-1011-8 · Cited by 133 · see citing works

Mitochondrial iron metabolism and neurodegenerative diseases

Cheng, R., Dhorajia, V. V., Kim, J., & Kim, Y. (2021) · NeuroToxicology · doi:10.1016/j.neuro.2021.11.003 · Cited by 120 · see citing works

Glutathione as a Redox Biomarker in Mitochondrial Disease—Implications for Therapy

Enns, G. M., & Cowan, T. M. (2017) · Journal of Clinical Medicine · doi:10.3390/jcm6050050 · Cited by 94 · see citing works

Diabetes in Friedreich Ataxia

Cnop, M., Mulder, H., & Igoillo‐Esteve, M. (2013) · Journal of Neurochemistry · doi:10.1111/jnc.12216 · Cited by 93 · see citing works

The optic nerve: A “mito-window” on mitochondrial neurodegeneration

Maresca, A., Morgia, C. L., Caporali, L., Valentino, M. L., & Carelli, V. (2012) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2012.08.004 · Cited by 92 · see citing works

Insights into the role of oxidative stress in the pathology of Friedreich ataxia using peroxidation resistant polyunsaturated fatty acids

Cotticelli, M. G., Crabbe, A. M., Wilson, R. B., & Shchepinov, M. S. (2013) · Redox Biology · doi:10.1016/j.redox.2013.06.004 · Cited by 90 · see citing works

Neurodegeneration in Friedreich’s Ataxia: From Defective Frataxin to Oxidative Stress

Gomes, C. M., & Santos, R. (2013) · Oxidative Medicine and Cellular Longevity · doi:10.1155/2013/487534 · Cited by 78 · see citing works

Regulation of Lifespan by the Mitochondrial Electron Transport Chain: Reactive Oxygen Species-Dependent and Reactive Oxygen Species-Independent Mechanisms

Scialò, F., Mallikarjun, V., Stefanatos, R. K. A., & Sanz, A. (2012) · Antioxidants and Redox Signaling · doi:10.1089/ars.2012.4900 · Cited by 70 · see citing works

Friedreich's ataxia: the vicious circle hypothesis revisited

Bayot, A., Santos, R., Camadro, J., & Rustin, P. (2011) · BMC Medicine · doi:10.1186/1741-7015-9-112 · Cited by 69 · see citing works

Unveiling a common mechanism of apoptosis in β-cells and neurons in Friedreich's ataxia

Igoillo‐Esteve, M., Gurgul-Convey, E., Hu, A., Santos, L. R. B., Abdulkarim, B., Chintawar, S., Marselli, L., Marchetti, P., Jonas, J., Eizirik, D. L., et al. (2014) · Human Molecular Genetics · doi:10.1093/hmg/ddu745 · Cited by 67 · see citing works

Oxidative stress and altered lipid metabolism in Friedreich ataxia

Tamarit, J., Òbis, È., & Ros, J. (2016) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2016.06.007 · Cited by 65 · see citing works

Oxidative stress-dependent frataxin inhibition mediated alcoholic hepatocytotoxicity through ferroptosis

Liu, J., He, H., Wang, J., Guo, X., Lin, H., Chen, H., Jiang, C., Chen, L., Yao, P., & Tang, Y. (2020) · Toxicology · doi:10.1016/j.tox.2020.152584 · Cited by 62 · see citing works

Role of frataxin protein deficiency and metabolic dysfunction in Friedreich ataxia, an autosomal recessive mitochondrial disease

Clark, E. M., Johnson, J., Dong, Y., Mercado‐Ayón, E., Warren, N., Zhai, M., McMillan, E., Salovin, A., Lin, H., & Lynch, D. R. (2018) · Neuronal Signaling · doi:10.1042/ns20180060 · Cited by 59 · see citing works

Frataxin and the molecular mechanism of mitochondrial iron-loading in Friedreich's ataxia

Chiang, S., Kovačević, Ž., Sahni, S., Lane, D. J. R., Merlot, A. M., Kalinowski, D. S., Huang, M. L. H., & Richardson, D. R. (2016) · Clinical Science · doi:10.1042/cs20160072 · Cited by 58 · see citing works

Role of Mesenchymal Stem Cells in Counteracting Oxidative Stress—Related Neurodegeneration

Angeloni, C., Gatti, M., Prata, C., Hrelia, S., & Maraldi, T. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21093299 · Cited by 57 · see citing works

Frataxin participates to the hypoxia-induced response in tumors

Guccini, I., Serio, D., Condò, I., Rufini, A., Tomassini, B., Mangiola, A., Maira, G., Anile, C., Fina, D., Pallone, F., et al. (2011) · Cell Death and Disease · doi:10.1038/cddis.2011.5 · Cited by 56 · see citing works

Fixing frataxin: ‘ironing out’ the metabolic defect in F riedreich's ataxia

Anzovino, A., Lane, D. J. R., Huang, M. L. H., & Richardson, D. R. (2013) · British Journal of Pharmacology · doi:10.1111/bph.12470 · Cited by 55 · see citing works

Silencing of frataxin gene expression triggers p53-dependent apoptosis in human neuron-like cells

Palomo, G. M., Cerrato, T., Gargini, R., & Díaz‐Nido, J. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddr187 · Cited by 54 · see citing works

Misdistribution of iron and oxidative stress in chronic kidney disease

Nakanishi, T., Kuragano, T., Nanami, M., Nagasawa, Y., & Hasuike, Y. (2018) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2018.06.025 · Cited by 54 · see citing works

Deferiprone and idebenone rescue frataxin depletion phenotypes in a Drosophila model of Friedreich's ataxia

Soriano, S., Llorens, J. V., Blanco-Sobero, L., Gutiérrez, L., Calap-Quintana, P., Morales, M. P., Moltó, M. D., & Martínez-Sebastián, M. J. (2013) · Gene · doi:10.1016/j.gene.2013.02.049 · Cited by 51 · see citing works

Mitochondrial dysfunction in the neuro-degenerative and cardio-degenerative disease, Friedreich's ataxia

Chiang, S., Kalinowski, D. S., Jansson, P. J., Richardson, D. R., & Huang, M. L. H. (2017) · Neurochemistry International · doi:10.1016/j.neuint.2017.08.002 · Cited by 48 · see citing works

Changes in mitochondrial glutathione levels and protein thiol oxidation in ∆yfh1 yeast cells and the lymphoblasts of patients with Friedreich's ataxia

Bulteau, A., Planamente, S., Jornéa, L., Dürr, A., Lesuisse, E., Camadro, J., & Auchère, F. (2011) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2011.11.003 · Cited by 47 · see citing works

Ferroptosis in Friedreich’s Ataxia: A Metal-Induced Neurodegenerative Disease

Rosa, P. L., Petrillo, S., Fiorenza, M. T., Bertini, E., & Piemonte, F. (2020) · Biomolecules · doi:10.3390/biom10111551 · Cited by 47 · see citing works

Progress in the treatment of Friedreich ataxia

Tai, G., Corben, L. A., Yiu, E. M., Milne, S., & Delatycki, M. B. (2018) · Neurologia i Neurochirurgia Polska · doi:10.1016/j.pjnns.2018.02.003 · Cited by 43 · see citing works

Redox Processes in Neurodegenerative Disease Involving Reactive Oxygen Species

Kovacic, P., & Somanathan, R. (2012) · Current Neuropharmacology · doi:10.2174/157015912804499429 · Cited by 41 · see citing works

Redox Processes in Neurodegenerative Disease Involving Reactive Oxygen Species

Kovacic, P., & Somanathan, R. (2012) · Current Neuropharmacology · doi:10.2174/157015912804143487 · Cited by 38 · see citing works

Iron in Friedreich Ataxia: A Central Role in the Pathophysiology or an Epiphenomenon?

Alsina, D., Purroy, R., Ros, J., & Tamarit, J. (2018) · Pharmaceuticals · doi:10.3390/ph11030089 · Cited by 38 · see citing works

Therapeutic Developments in Friedreich Ataxia

Wilson, R. B. (2012) · Journal of Child Neurology · doi:10.1177/0883073812449691 · Cited by 34 · see citing works

Small molecules preventing GAPDH aggregation are therapeutically applicable in cell and rat models of oxidative stress

Lazarev, V. F., Nikotina, A. D., Semenyuk, P. I., Evstafeva, D., Mikhaylova, E. R., Muronetz, V. I., Shevtsov, M. A., Tolkacheva, A. V., Добродумов, А. В., Shavarda, A. L., et al. (2015) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2015.12.025 · Cited by 34 · see citing works

A Review of Friedreich Ataxia Clinical Trial Results

Perlman, S. (2012) · Journal of Child Neurology · doi:10.1177/0883073812453872 · Cited by 31 · see citing works

Friedreich ataxia: metal dysmetabolism in dorsal root ganglia

Koeppen, A. H., Kuntzsch, E. C., Bjork, S. T., Ramirez, R. L., Mazurkiewicz, J. E., & Feustel, P. J. (2013) · Acta Neuropathologica Communications · doi:10.1186/2051-5960-1-26 · Cited by 30 · see citing works

Human Mesenchymal Stem Cells Increase Anti-oxidant Defences in Cells Derived from Patients with Friedreich’s Ataxia

Dey, R., Kemp, K. C., Gray, E., Rice, C. M., Scolding, N. J., & Wilkins, A. (2012) · The Cerebellum · doi:10.1007/s12311-012-0406-2 · Cited by 29 · see citing works

Antiferroptotic Activity of Phenothiazine Analogues: A Novel Therapeutic Strategy for Oxidative Stress Related Disease

Liu, J., Bandyopadhyay, I., Zheng, L., Khdour, O. M., & Hecht, S. M. (2020) · ACS Medicinal Chemistry Letters · doi:10.1021/acsmedchemlett.0c00293 · Cited by 28 · see citing works

Sweet and Sour - Oxidative and Carbonyl Stress in Neurological Disorders

Harris, R. A., & Amor, S. (2011) · CNS & Neurological Disorders - Drug Targets · doi:10.2174/187152711794488656 · Cited by 27 · see citing works

Antioxidant defense mechanisms and its dysfunctional regulation in the mitochondrial disease, Friedreich's ataxia

Chiang, S., Huang, M. L. H., Park, K. C., & Richardson, D. R. (2020) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2020.07.019 · Cited by 27 · see citing works

A single strand that links multiple neuropathologies in human disease

Reynolds, J. J., & Stewart, G. S. (2013) · Brain · doi:10.1093/brain/aws310 · Cited by 27 · see citing works

Friedreich Ataxia

Pandolfo, M. (2012) · Journal of Child Neurology · doi:10.1177/0883073812448534 · Cited by 26 · see citing works

Frataxin Silencing Inactivates Mitochondrial Complex I in NSC34 Motoneuronal Cells and Alters Glutathione Homeostasis

Carletti, B., Piermarini, E., Tozzi, G., Travaglini, L., Torraco, A., Pastore, A., Sparaco, M., Petrillo, S., Carrozzo, R., Bertini, E., et al. (2014) · International Journal of Molecular Sciences · doi:10.3390/ijms15045789 · Cited by 25 · see citing works

Quantitative proteomics in Friedreich's ataxia B-lymphocytes: A valuable approach to decipher the biochemical events responsible for pathogenesis

Télot, L., Rousseau, E., Lesuisse, E., Garcia, C., Morlet, B., Léger, T., Camadro, J., & Serre, V. (2018) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2018.01.010 · Cited by 25 · see citing works

A Strategy for Suppressing Redox Stress within Mitochondria

Arce, P. M., Khdour, O. M., Goldschmidt, R., Armstrong, J. S., & Hecht, S. M. (2011) · ACS Medicinal Chemistry Letters · doi:10.1021/ml200095w · Cited by 24 · see citing works

Base Excision Repair of Chemotherapeutically-Induced Alkylated DNA Damage Predominantly Causes Contractions of Expanded GAA Repeats Associated with Friedreich's Ataxia

Lai, Y.-H., Beaver, J. M., Lorente, K., Melo, J., Ramjagsingh, S., Agoulnik, I., Zhang, Z.-Z., & Liu, Y. (2014) · PLoS ONE · doi:10.1371/journal.pone.0093464 · Cited by 20 · see citing works

Co-precipitation of Phosphate and Iron Limits Mitochondrial Phosphate Availability in Saccharomyces cerevisiae Lacking the Yeast Frataxin Homologue (YFH1)

Seguin, A., Santos, R., Pain, D., Dancis, A., Camadro, J., & Lesuisse, E. (2010) · Journal of Biological Chemistry · doi:10.1074/jbc.m110.163253 · Cited by 24 · see citing works

The yeast metacaspase is implicated in oxidative stress response in frataxin‐deficient cells

Lefevre, S. D., Sliwa, D., Auchère, F., Brossas, C., Ruckenstuhl, C., Boggetto, N., Lesuisse, E., Madeo, F., Camadro, J., & Santos, R. (2011) · FEBS Letters · doi:10.1016/j.febslet.2011.12.002 · Cited by 20 · see citing works

Quercetin supplementation does not enhance cerebellar mitochondrial biogenesis and oxidative status in exercised rats

Casuso, R. A., Martínez‐Amat, A., Hita‐Contreras, F., Camiletti‐Moirón, D., Aranda, P., & López, E. J. M. (2015) · Nutrition Research · doi:10.1016/j.nutres.2015.05.007 · Cited by 20 · see citing works

Effects of alkyl side chain modification of coenzyme Q10 on mitochondrial respiratory chain function and cytoprotection

Fash, D. M., Khdour, O. M., Sahdeo, S., Goldschmidt, R., Jaruvangsanti, J., Dey, S., Arce, P. M., Collin, V. C., Cortopassi, G. A., & Hecht, S. M. (2013) · Bioorganic & Medicinal Chemistry · doi:10.1016/j.bmc.2013.01.075 · Cited by 19 · see citing works

Cells Lacking Pfh1, a Fission Yeast Homolog of Mammalian Frataxin Protein, Display Constitutive Activation of the Iron Starvation Response

Gabrielli, N., Ayté, J., & Hidalgo, E. (2012) · Journal of Biological Chemistry · doi:10.1074/jbc.m112.421735 · Cited by 19 · see citing works

Treatment of dilated cardiomyopathy in a mouse model of Friedreich’s ataxia using N-acetylcysteine and identification of alterations in microRNA expression that could be involved in its pathogenesis

Chiang, S., Huang, M. L. H., & Richardson, D. R. (2020) · Pharmacological Research · doi:10.1016/j.phrs.2020.104994 · Cited by 19 · see citing works

Effects of tocotrienol supplementation in Friedreich’s ataxia: A model of oxidative stress pathology

Bolotta, A., Pini, A., Abruzzo, P. M., Ghezzo, A., Modesti, A., Gamberi, T., Ferreri, C., Bugamelli, F., Fortuna, F., Vertuani, S., et al. (2019) · Experimental Biology and Medicine · doi:10.1177/1535370219890873 · Cited by 18 · see citing works

Double blind trial of a deuterated form of linoleic acid (RT001) in Friedreich ataxia

Lynch, D. R., Mathews, K. D., Perlman, S., Zesiewicz, T. A., Subramony, S. H., Omidvar, O., Vogel, A. P., Krtolica, A., Litterman, N. K., der Ploeg, L. H. T. V., et al. (2022) · Journal of Neurology · doi:10.1007/s00415-022-11501-4 · Cited by 17 · see citing works

Frataxin mRNA Isoforms in FRDA Patients and Normal Subjects: Effect of Tocotrienol Supplementation

Abruzzo, P. M., Marini, M., Bolotta, A., Malisardi, G., Manfredini, S., Ghezzo, A., Pini, A., Tasco, G., & Casadio, R. (2013) · BioMed Research International · doi:10.1155/2013/276808 · Cited by 17 · see citing works

Common data elements for clinical research in Friedreich's ataxia

Lynch, D. R., Pandolfo, M., Schulz, J. B., Perlman, S., Delatycki, M. B., Payne, R. M., Shaddy, R. E., Fischbeck, K. H., Farmer, J. M., Kantor, P. F., et al. (2012) · Movement Disorders · doi:10.1002/mds.25201 · Cited by 16 · see citing works

Analysis of the structural and mechanistic factors in antioxidants that preserve mitochondrial function and confer cytoprotection

Arce, P. M., Goldschmidt, R., Khdour, O. M., Madathil, M. M., Jaruvangsanti, J., Dey, S., Fash, D. M., Armstrong, J. S., & Hecht, S. M. (2012) · Bioorganic & Medicinal Chemistry · doi:10.1016/j.bmc.2012.07.005 · Cited by 16 · see citing works

Design, synthesis, and evaluation of an α-tocopherol analogue as a mitochondrial antioxidant

Lu, J., Khdour, O. M., Armstrong, J. S., & Hecht, S. M. (2010) · Bioorganic & Medicinal Chemistry · doi:10.1016/j.bmc.2010.08.030 · Cited by 15 · see citing works

Cytoprotective pyridinol antioxidants as potential therapeutic agents for neurodegenerative and mitochondrial diseases

Alam, M. P., Khdour, O. M., Arce, P. M., Chen, Y., Roy, B., Johnson, W. G., Dey, S., & Hecht, S. M. (2014) · Bioorganic & Medicinal Chemistry · doi:10.1016/j.bmc.2014.06.040 · Cited by 14 · see citing works

An Acetate Prodrug of a Pyridinol-Based Vitamin E Analogue

Khdour, O. M., Lu, J., & Hecht, S. M. (2011) · Pharmaceutical Research · doi:10.1007/s11095-011-0491-9 · Cited by 14 · see citing works

Perturbation of cellular proteostasis networks identifies pathways that modulate precursor and intermediate but not mature levels of frataxin

Nabhan, J. F., Gooch, R. L., Chekler, E. L. P., Pierce, B. S., & Bulawa, C. (2015) · Scientific Reports · doi:10.1038/srep18251 · Cited by 14 · see citing works

Diabetes mellitus in Friedreich Ataxia: A case series of 19 patients from the German-Austrian diabetes mellitus registry

Pappa, A., Häusler, M., Veigel, A., Tzamouranis, K., Pfeifer, M., Schmidt, A., Bökamp, M., Haberland, H., Wagner, S., Brückel, J., et al. (2018) · Diabetes Research and Clinical Practice · doi:10.1016/j.diabres.2018.05.008 · Cited by 14 · see citing works

Increased iron supplied through Fet3p results in replicative life span extension of Saccharomyces cerevisiae under conditions requiring respiratory metabolism

Botta, G., Turn, C. S., Quintyne, N. J., & Kirchman, P. A. (2011) · Experimental Gerontology · doi:10.1016/j.exger.2011.07.002 · Cited by 14 · see citing works

Drosophila melanogasterModels of Friedreich’s Ataxia

Calap-Quintana, P., Navarro, J. A., González-Fernández, J., Martínez-Sebastián, M. J., Moltó, M. D., & Llorens, J. V. (2018) · BioMed Research International · doi:10.1155/2018/5065190 · Cited by 13 · see citing works

Lipophilic methylene blue analogues enhance mitochondrial function and increase frataxin levels in a cellular model of Friedreich’s ataxia

Khdour, O. M., Bandyopadhyay, I., Chowdhury, S. R., Visavadiya, N. P., & Hecht, S. M. (2018) · Bioorganic & Medicinal Chemistry · doi:10.1016/j.bmc.2018.05.005 · Cited by 13 · see citing works

An Optimized Pyrimidinol Multifunctional Radical Quencher

Khdour, O. M., Arce, P. M., Roy, B., & Hecht, S. M. (2013) · ACS Medicinal Chemistry Letters · doi:10.1021/ml400130z · Cited by 12 · see citing works

DNA repair pathways are altered in neural cell models of frataxin deficiency

Moreno-Lorite, J., Pérez-Luz, S., Katsu-Jiménez, Y., Oberdoerfer, D., & Díaz‐Nido, J. (2021) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2020.103587 · Cited by 11 · see citing works

Phenothiazine antioxidants increase mitochondrial biogenesis and frataxin levels in Friedreich's ataxia cells

Khdour, O. M., Bandyopadhyay, I., Visavadiya, N. P., Chowdhury, S. R., & Hecht, S. M. (2018) · MedChemComm · doi:10.1039/c8md00274f · Cited by 10 · see citing works

Apn1 AP-endonuclease is essential for the repair of oxidatively damaged DNA bases in yeast frataxin-deficient cells

Lefevre, S. D., Brossas, C., Auchère, F., Boggetto, N., Camadro, J., & Santos, R. (2012) · Human Molecular Genetics · doi:10.1093/hmg/dds230 · Cited by 9 · see citing works

Adenosine Improves Mitochondrial Function and Biogenesis in Friedreich’s Ataxia Fibroblasts Following L-Buthionine Sulfoximine-Induced Oxidative Stress

Lew, S. Y., Hisam, N. S. M., Phang, M. W. L., Rahman, S. N. S. A., Poh, R. Y. Y., Lim, S., Kamaruzzaman, M. A., Chau, S. C., Tsui, K. C., Lim, L. W., et al. (2023) · Biology · doi:10.3390/biology12040559 · Cited by 9 · see citing works

Nuclear Factor Erythroid 2-Related Factor 2 Activation Might Mitigate Clinical Symptoms in Friedreich’s Ataxia: Clues of an “Out-Brain Origin” of the Disease From a Family Study

Petrillo, S., Santoro, M., Rosa, P. L., Perna, A., Gallo, M. G., Bertini, E., Silvestri, G., & Piemonte, F. (2021) · Frontiers in Neuroscience · doi:10.3389/fnins.2021.638810 · Cited by 9 · see citing works

Optimization of pyrimidinol antioxidants as mitochondrial protective agents: ATP production and metabolic stability

Chevalier, A., Alam, M. P., Khdour, O. M., Schmierer, M., Arce, P. M., Cripe, C. D., & Hecht, S. M. (2016) · Bioorganic & Medicinal Chemistry · doi:10.1016/j.bmc.2016.08.039 · Cited by 8 · see citing works

Ataxia and coenzyme Q10: an overview

Mantle, D., & Hargreaves, I. P. (2018) · British Journal of Neuroscience Nursing · doi:10.12968/bjnn.2018.14.3.108 · Cited by 8 · see citing works

Treatment of Friedreich's ataxia

Pandolfo, M. (2013) · Expert Opinion on Orphan Drugs · doi:10.1517/21678707.2013.771578 · Cited by 7 · see citing works

Synthesis and Biological Activities of N-(3-Carboxylpropyl)-5-amino-2-hydroxy-3-tridecyl-1,4-benzoquinone and Analogues

Madathil, M. M., Khdour, O. M., Jaruvangsanti, J., & Hecht, S. M. (2012) · Journal of Natural Products · doi:10.1021/np3007099 · Cited by 7 · see citing works

Mitochondrial targeting of potent nanoparticulated drugs in combating diseases

Mandal, A. K. (2022) · Journal of Biomaterials Applications · doi:10.1177/08853282221111656 · Cited by 6 · see citing works

Influence of substituent heteroatoms on the cytoprotective properties of pyrimidinol antioxidants

Chevalier, A., Khdour, O. M., Schmierer, M., Bandyopadhyay, I., & Hecht, S. M. (2017) · Bioorganic & Medicinal Chemistry · doi:10.1016/j.bmc.2017.01.030 · Cited by 6 · see citing works

Lipophilic methylene violet analogues as modulators of mitochondrial function and dysfunction

Chowdhury, S. R., Khdour, O. M., Bandyopadhyay, I., & Hecht, S. M. (2017) · Bioorganic & Medicinal Chemistry · doi:10.1016/j.bmc.2017.08.021 · Cited by 6 · see citing works

In Vitro interaction between yeast frataxin and superoxide dismutases: Influence of mitochondrial metals

Han, T.-H.-L., Camadro, J., Barbault, F., Santos, R., Chahine, J. E. H., & Duong, N.-T. H. (2019) · Biochimica et Biophysica Acta (BBA) - General Subjects · doi:10.1016/j.bbagen.2019.02.011 · Cited by 5 · see citing works

Interferon Gamma Enhances Cytoprotective Pathways via Nrf2 and MnSOD Induction in Friedreich’s Ataxia Cells

Luffarelli, R., Panarello, L., Quatrana, A., Tiano, F., Fortuni, S., Rufini, A., Malisan, F., Testi, R., & Condò, I. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms241612687 · Cited by 5 · see citing works

A Structurally Simplified Analogue of Geldanamycin Exhibits Neuroprotective Activity

Madathil, M. M., Khdour, O. M., Jaruvangsanti, J., & Hecht, S. M. (2013) · ACS Medicinal Chemistry Letters · doi:10.1021/ml400207m · Cited by 4 · see citing works

ROLE OF REDOX AND CERULOPLASMIN IN IRON DEPOSITION IN GLIAL CELLS: IMPLICATION IN NEURODEGENERATIVE DAMAGES

Mukhopadhyay, C. K., Dev, S., Tapryal, N., Mukherjee, R., & Mukhopadhyay, C. (2011) · Cited by 4 · see citing works

Oxidative Stress and its Different Roles in Neurodegenerative Diseases

Rajabi, S., Noori, S., Zal, F., & Jahan‐Abad, A. J. (2017) · The Neuroscience Journal of Shefaye Khatam · doi:10.18869/acadpub.shefa.5.1.73 · Cited by 4 · see citing works

Friedreich ataxia

Pandolfo, M. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-19176-3.00041-8 · Cited by 3 · see citing works

Anti-Oxidants, the Aging Brain and Age-Related Brain Disorders

Baudry, M., & Malfroy-Camine, B. (2016) · Oxidative stress in applied basic research and clinical practice · doi:10.1007/978-3-319-33486-8_13 · Cited by 3 · see citing works

Multifunctional Radical Quenchers as Potential Therapeutic Agents for the Treatment of Mitochondrial Dysfunction

Ji, X., Khdour, O. M., & Hecht, S. M. (2019) · Future Medicinal Chemistry · doi:10.4155/fmc-2018-0481 · Cited by 3 · see citing works

Bioinorganic Neurochemistry

Folk, D. S., Kielar, F., & Franz, K. J. (2013) · Elsevier eBooks · doi:10.1016/b978-0-08-097774-4.00315-6 · Cited by 3 · see citing works

Redox Processes in Neurodegenerative Disease Involving Reactive Oxygen Species

Kovacic, P., & Somanathan, R. (2012) · Current Neuropharmacology · doi:10.2174/1570159x11209040289 · Cited by 2 · see citing works

Animal Models of Friedreich Ataxia

Pandolfo, M. (2014) · Elsevier eBooks · doi:10.1016/b978-0-12-405195-9.00065-2 · Cited by 2 · see citing works

Structure Activity Studies of Quinones and Analogues

Raghav, N. (2011)

A protective role of autophagy in a Drosophila model of Friedreich's ataxia (FRDA)

Wang, L. (2015) · Human Biology

The Involvement of Anti-Oxidative Response and Mitochondrial Dynamics in the Pathogenesis of Friedreich’s Ataxia: Relevance to the Development of Future Therapeutics

Chiang, S. (2019) · The Sydney eScholarship Repository (The University of Sydney)

Friedreich ataxia: the oxidative stress paradox

Seznec, H., Simon, D., Bouton, C., Reutenauer, L., Hertzog, A., Golik, P., Procaccio, V., Patel, M., Drapier, J.-C., Koenig, M., et al. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi042 · Cited by 219 · see citing works

Friedreich ataxia: a paradigm for mitochondrial diseases

Puccio, H. M., & Koenig, M. (2002) · Current Opinion in Genetics & Development · doi:10.1016/s0959-437x(02)00298-8 · Cited by 111 · see citing works

The role of oxidative stress in Friedreich's ataxia

Lupoli, F., Vannocci, T., Longo, G., Niccolai, N., & Pastore, A. (2017) · FEBS Letters · doi:10.1002/1873-3468.12928 · Cited by 102 · see citing works

Emerging antioxidant therapies in Friedreich’s ataxia

Edzeamey, F. J., Ramchunder, Z., Pourzand, C., & Virmouni, S. A. (2024) · Frontiers in Pharmacology · doi:10.3389/fphar.2024.1359618 · Cited by 23 · see citing works

Oxidative Stress and Antioxidant Therapies in Friedreich’s Ataxia

Jiménez‐Jiménez, F. J., Alonso‐Navarro, H., García‐Martín, E., Cárcamo-Fonfría, A., Martín-Gómez, M. Á., & Agúndez, J. A. G. (2025) · Cells · doi:10.3390/cells14181406 · Cited by 6 · see citing works