A. Durr, M. Cossee, Y. Agid, V. Campuzano, C. Mignard et al., Clinical and Genetic Abnormalities in Patients with Friedreich's Ataxia, New England Journal of Medicine, vol.335, issue.16, pp.1169-1175, 1996.
DOI : 10.1056/NEJM199610173351601

A. Harding, FRIEDREICH'S ATAXIA: A CLINICAL AND GENETIC STUDY OF 90 FAMILIES WITH AN ANALYSIS OF EARLY DIAGNOSTIC CRITERIA AND INTRAFAMILIAL CLUSTERING OF CLINICAL FEATURES, Brain, vol.104, issue.3, pp.589-620, 1981.
DOI : 10.1093/brain/104.3.589

V. Campuzano, L. Montermini, Y. Lutz, L. Cova, C. Hindelang et al., Frataxin is Reduced in Friedreich Ataxia Patients and is Associated with Mitochondrial Membranes, Human Molecular Genetics, vol.6, issue.11, pp.1771-1780, 1997.
DOI : 10.1093/hmg/6.11.1771

M. Cossee, V. Campuzano, H. Koutnikova, K. Fischbeck, J. Mandel et al., Frataxin fracas, Nature Genetics, vol.56, issue.4, pp.337-338, 1997.
DOI : 10.1038/ng0996-33

E. Grabczyk, M. Mancuso, and M. Sammarco, A persistent RNA{middle dot}DNA hybrid formed by transcription of the Friedreich ataxia triplet repeat in live bacteria, and by T7 RNAP in vitro, Nucleic Acids Research, vol.35, issue.16, pp.5351-5359, 2007.
DOI : 10.1093/nar/gkm589

N. Sakamoto, K. Ohshima, L. Montermini, M. Pandolfo, and R. Wells, Sticky DNA, a Self-associated Complex Formed at Long GAA{middle dot}TTC Repeats in Intron 1 of the Frataxin Gene, Inhibits Transcription, Journal of Biological Chemistry, vol.276, issue.29, pp.27171-27177, 2001.
DOI : 10.1074/jbc.M101879200

A. Saveliev, C. Everett, T. Sharpe, Z. Webster, and R. Festenstein, DNA triplet repeats mediate heterochromatin-protein-1-sensitive variegated gene silencing, Nature, vol.422, issue.6934, pp.909-913, 2003.
DOI : 10.1038/nature01596

J. Lamarche, M. Cote, and B. Lemieux, SUMMARY:, Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques, vol.3, issue.04, pp.389-396, 1980.
DOI : 10.1002/path.1700580414

M. Babcock, D. De-silva, R. Oaks, S. Davis-kaplan, S. Jiralerspong et al., Regulation of Mitochondrial Iron Accumulation by Yfh1p, a Putative Homolog of Frataxin, Science, vol.276, issue.5319, pp.1709-1712, 1997.
DOI : 10.1126/science.276.5319.1709

J. Bradley, S. Homayoun, P. Hart, A. Schapira, and J. Cooper, Role of Oxidative Damage in Friedreich's Ataxia, Neurochemical Research, vol.29, issue.3, pp.561-567, 2004.
DOI : 10.1023/B:NERE.0000014826.00881.c3

M. Emond, G. Lepage, M. Vanasse, and M. Pandolfo, Increased levels of plasma malondialdehyde in Friedreich ataxia, Neurology, vol.55, issue.11, pp.1752-1753, 2000.
DOI : 10.1212/WNL.55.11.1752

F. Piemonte, A. Pastore, G. Tozzi, D. Tagliacozzi, F. Santorelli et al., Glutathione in blood of patients with Friedreich's ataxia, European Journal of Clinical Investigation, vol.221, issue.11, pp.1007-1011, 2001.
DOI : 10.1006/bbrc.1997.7812

J. Schulz, T. Dehmer, L. Schols, H. Mende, C. Hardt et al., Oxidative stress in patients with Friedreich ataxia, Neurology, vol.55, issue.11, pp.1719-1721, 2000.
DOI : 10.1212/WNL.55.11.1719

K. Chantrel-groussard, V. Geromel, H. Puccio, M. Koenig, A. Munnich et al., Disabled early recruitment of antioxidant defenses in Friedreich's ataxia, Human Molecular Genetics, vol.10, issue.19, pp.2061-2067, 2001.
DOI : 10.1093/hmg/10.19.2061

S. Jiralerspong, B. Ge, T. Hudson, and M. Pandolfo, Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells, FEBS Letters, vol.276, issue.1, pp.101-105, 2001.
DOI : 10.1016/S0014-5793(01)03140-4

M. Santos, K. Ohshima, and M. Pandolfo, Frataxin deficiency enhances apoptosis in cells differentiating into neuroectoderm, Human Molecular Genetics, vol.10, issue.18, pp.1935-1944, 2001.
DOI : 10.1093/hmg/10.18.1935

A. Wong, J. Yang, P. Cavadini, C. Gellera, B. Lonnerdal et al., The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis, Human Molecular Genetics, vol.8, issue.3, pp.425-430, 1999.
DOI : 10.1093/hmg/8.3.425

B. Sturm, U. Bistrich, M. Schranzhofer, J. Sarsero, U. Rauen et al., Friedreich's Ataxia, No Changes in Mitochondrial Labile Iron in Human Lymphoblasts and Fibroblasts: A DECREASE IN ANTIOXIDATIVE CAPACITY?, Journal of Biological Chemistry, vol.280, issue.8, pp.6701-6708, 2005.
DOI : 10.1074/jbc.M408717200

P. Arnold, O. Boulat, R. Maire, and T. Kuntzer, Expanding view of phenotype and oxidative stress in Friedreich's ataxia patients with and without idebenone. Schweizer archiv für neurologie und psychiatrie, pp.169-176, 2006.

D. Prospero, N. Baker, A. Jeffries, N. Fischbeck, and K. , Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a randomised, placebo-controlled trial, The Lancet Neurology, vol.6, issue.10, pp.878-886, 2007.
DOI : 10.1016/S1474-4422(07)70220-X

L. Myers, D. Lynch, J. Farmer, L. Friedman, J. Lawson et al., Urinary isoprostanes in Friedreich ataxia: Lack of correlation with disease features, Movement Disorders, vol.85, issue.13, pp.1920-1922, 2008.
DOI : 10.1002/mds.22038

H. Seznec, D. Simon, C. Bouton, L. Reutenauer, A. Hertzog et al., Friedreich ataxia: the oxidative stress paradox, Human Molecular Genetics, vol.14, issue.4, pp.463-474, 2005.
DOI : 10.1093/hmg/ddi042

URL : https://hal.archives-ouvertes.fr/hal-00187760

H. Puccio, D. Simon, M. Cossee, P. Criqui-filipe, F. Tiziano et al., Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits, Nature Genetics, vol.27, issue.2, pp.181-186, 2001.
DOI : 10.1038/84818

S. Al-mahdawi, R. Pinto, D. Varshney, L. Lawrence, M. Lowrie et al., GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology, Genomics, vol.88, issue.5, pp.580-590, 2006.
DOI : 10.1016/j.ygeno.2006.06.015

C. Lu and G. Cortopassi, Frataxin knockdown causes loss of cytoplasmic iron???sulfur cluster functions, redox alterations and induction of heme transcripts, Archives of Biochemistry and Biophysics, vol.457, issue.1, pp.111-122, 2007.
DOI : 10.1016/j.abb.2006.09.010

H. Seznec, D. Simon, L. Monassier, P. Criqui-filipe, A. Gansmuller et al., Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia, Human Molecular Genetics, vol.13, issue.10, pp.1017-1024, 2004.
DOI : 10.1093/hmg/ddh114

G. Duby, F. Foury, A. Ramazzotti, J. Herrmann, and T. Lutz, A non-essential function for yeast frataxin in iron-sulfur cluster assembly, Human Molecular Genetics, vol.11, issue.21, pp.2635-2643, 2002.
DOI : 10.1093/hmg/11.21.2635

A. Martelli, M. Wattenhofer-donze, S. Schmucker, S. Bouvet, L. Reutenauer et al., Frataxin is essential for extramitochondrial Fe S cluster proteins in mammalian tissues, Human Molecular Genetics, vol.16, issue.22, pp.2651-2658, 2007.
DOI : 10.1093/hmg/ddm163

URL : https://hal.archives-ouvertes.fr/hal-00187819

U. Muhlenhoff, N. Richhardt, M. Ristow, G. Kispal, and R. Lill, The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S proteins, Human Molecular Genetics, vol.11, issue.17, pp.2025-2036, 2002.
DOI : 10.1093/hmg/11.17.2025

A. Rotig, P. De-lonlay, D. Chretien, F. Foury, M. Koenig et al., Aconitase and mitochondrial iron???sulphur protein deficiency in Friedreich ataxia, Nature Genetics, vol.12, issue.2, pp.215-217, 1997.
DOI : 10.1016/0009-8981(94)90055-8

F. Foury, A. Pastore, and M. Trincal, Acidic residues of yeast frataxin have an essential role in Fe???S cluster assembly, EMBO reports, vol.125, issue.2, pp.194-199, 2007.
DOI : 10.1038/sj.embor.7400881

J. Gerber, U. Muhlenhoff, and R. Lill, An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1, EMBO reports, vol.4, issue.9, pp.906-911, 2003.
DOI : 10.1038/sj.embor.embor918

G. Layer, S. Ollagnier-de-choudens, Y. Sanakis, and M. Fontecave, Iron-Sulfur Cluster Biosynthesis: CHARACTERIZATION OF ESCHERICHIA COLI CYaY AS AN IRON DONOR FOR THE ASSEMBLY OF [2Fe-2S] CLUSTERS IN THE SCAFFOLD IscU, Journal of Biological Chemistry, vol.281, issue.24, pp.16256-16263, 2006.
DOI : 10.1074/jbc.M513569200

URL : https://hal.archives-ouvertes.fr/hal-00374594

Y. Shan, E. Napoli, and G. Cortopassi, Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones, Human Molecular Genetics, vol.16, issue.8, pp.929-941, 2007.
DOI : 10.1093/hmg/ddm038

M. Pandolfo, Drug Insight: antioxidant therapy in inherited ataxias, Nature Clinical Practice Neurology, vol.60, issue.2, pp.86-96, 2008.
DOI : 10.1038/ncpneuro0704

N. Boddaert, L. Q. Sang, K. Rotig, A. Leroy-willig, A. Gallet et al., Selective iron chelation in Friedreich ataxia: biologic and clinical implications, Blood, vol.110, issue.1, pp.401-408, 2007.
DOI : 10.1182/blood-2006-12-065433

URL : https://hal.archives-ouvertes.fr/hal-00687082

D. Richardson, Friedreich???s ataxia: iron chelators that target the mitochondrion as a therapeutic strategy?, Expert Opinion on Investigational Drugs, vol.94, issue.2, pp.235-245, 2003.
DOI : 10.1042/bj3510477

O. Kakhlon, H. Manning, W. Breuer, N. Melamed-book, C. Lu et al., Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation, Blood, vol.112, issue.13, pp.5219-5227, 2008.
DOI : 10.1182/blood-2008-06-161919

S. Boesch, B. Sturm, S. Hering, H. Goldenberg, and W. Poewe, Friedreich's ataxia: clinical pilot trial with recombinant human erythropoietin, Annals of Neurology, vol.55, issue.5, pp.521-524, 2007.
DOI : 10.1002/ana.21177

M. Rai, E. Soragni, K. Jenssen, R. Burnett, D. Herman et al., HDAC Inhibitors Correct Frataxin Deficiency in a Friedreich Ataxia Mouse Model, PLoS ONE, vol.92, issue.4, p.1958, 2008.
DOI : 10.1371/journal.pone.0001958.s003

M. Jauslin, T. Wirth, T. Meier, and F. Schoumacher, A cellular model for Friedreich Ataxia reveals small-molecule glutathione peroxidase mimetics as novel treatment strategy, Human Molecular Genetics, vol.11, issue.24, pp.3055-3063, 2002.
DOI : 10.1093/hmg/11.24.3055

E. Napoli, D. Morin, R. Bernhardt, A. Buckpitt, and G. Cortopassi, Hemin rescues adrenodoxin, heme a and cytochrome oxidase activity in frataxin-deficient oligodendroglioma cells, Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, vol.1772, issue.7, pp.773-780, 2007.
DOI : 10.1016/j.bbadis.2007.04.001

R. Schoenfeld, E. Napoli, A. Wong, S. Zhan, L. Reutenauer et al., Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian cells, Human Molecular Genetics, vol.14, issue.24, pp.3787-3799, 2005.
DOI : 10.1093/hmg/ddi393

URL : https://hal.archives-ouvertes.fr/hal-00187745

O. Stehling, H. Elsasser, B. Bruckel, U. Muhlenhoff, and R. Lill, Iron-sulfur protein maturation in human cells: evidence for a function of frataxin, Human Molecular Genetics, vol.13, issue.23, pp.3007-3015, 2004.
DOI : 10.1093/hmg/ddh324

G. Tan, E. Napoli, F. Taroni, and G. Cortopassi, Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells, Human Molecular Genetics, vol.12, issue.14, pp.1699-1711, 2003.
DOI : 10.1093/hmg/ddg187

I. Zanella, M. Derosas, M. Corrado, E. Cocco, P. Cavadini et al., The effects of frataxin silencing in HeLa cells are rescued by the expression of human mitochondrial ferritin, Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, vol.1782, issue.2, pp.178290-178298, 2008.
DOI : 10.1016/j.bbadis.2007.11.006

URL : https://hal.archives-ouvertes.fr/hal-00501557

M. Kilpatrick, L. Phylactou, M. Godfrey, C. Wu, G. Wu et al., Delivery of a hammerhead ribozyme specifically down-regulates the production of fibrillin-1 by cultured dermal fibroblasts, Human Molecular Genetics, vol.5, issue.12, pp.1939-1944, 1996.
DOI : 10.1093/hmg/5.12.1939

R. Montgomery and H. Dietz, Inhibition of fibrillin 1 expression using U1 snRNA as a vehicle for the presentation of antisense targeting sequence, Human Molecular Genetics, vol.6, issue.4, pp.519-525, 1997.
DOI : 10.1093/hmg/6.4.519

R. Freshney, Culture of animal cells: a manual of basic technique 4th edition, 2000.

L. Daya-grosjean, M. James, C. Drougard, and A. Sarasin, An immortalized xeroderma pigmentosum, group C, cell line which replicates SV40 shuttle vectors, Mutation Research/DNA Repair Reports, vol.183, issue.2, pp.185-196, 1987.
DOI : 10.1016/0167-8817(87)90061-7

D. Metzger and P. Chambon, Site- and Time-Specific Gene Targeting in the Mouse, Methods, vol.24, issue.1, pp.71-80, 2001.
DOI : 10.1006/meth.2001.1159

P. Rustin, D. Chretien, T. Bourgeron, B. Gerard, A. Rotig et al., Biochemical and molecular investigations in respiratory chain deficiencies, Clinica Chimica Acta, vol.228, issue.1, pp.35-51, 1994.
DOI : 10.1016/0009-8981(94)90055-8

P. Anderson, K. Kirby, A. Hilliker, and J. Phillips, RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila, Human Molecular Genetics, vol.14, issue.22, pp.3397-3405, 2005.
DOI : 10.1093/hmg/ddi367

J. Zhang, T. Chung, and K. Oldenburg, A Simple Statistical Parameter for Use in Evaluation and Validation of High Throughput Screening Assays, Journal of Biomolecular Screening, vol.4, issue.2, pp.67-73, 1999.
DOI : 10.1177/108705719900400206

M. Cossee, H. Puccio, A. Gansmuller, H. Koutnikova, A. Dierich et al., Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation, Human Molecular Genetics, vol.9, issue.8, pp.1219-1226, 2000.
DOI : 10.1093/hmg/9.8.1219

C. Wermuth, Selective optimization of side activities: the SOSA approach, Drug Discovery Today, vol.11, issue.3-4, pp.160-164, 2006.
DOI : 10.1016/S1359-6446(05)03686-X