Iron-Sulfur Clusters: Nature's Modular, Multipurpose Structures, Science, vol.277, issue.5326, pp.653-659, 1997. ,
DOI : 10.1126/science.277.5326.653
The Interface Between the Biological and Inorganic Worlds: Iron-Sulfur Metalloclusters, Science, vol.300, issue.5621, pp.929-931, 2003. ,
DOI : 10.1126/science.1083075
Ironesulphur clusters and the problem with oxygen, Mol. Microbiol, vol.59, 2006. ,
Biosynthesis of ironesulphur clusters is a complex and highly conserved process, Biochem. Soc. Trans, pp.30-680, 2002. ,
Evidence for a new type of iron containing electron carrier in mitochondria, Biochemical and Biophysical Research Communications, vol.5, issue.1, pp.40-45, 1961. ,
DOI : 10.1016/0006-291X(61)90077-8
Azotobacter vinelandii nifD- and nifE-encoded polypeptides share structural homology, Proceedings of the National Academy of Sciences, vol.82, issue.17, p.82, 1985. ,
DOI : 10.1073/pnas.82.17.5720
Assembly of ironesulfur clusters. Identification of an iscSUA-hscBA-fdx gene cluster from Azotobacter vinelandii, J. Biol. Chem, pp.273-13264, 1998. ,
Evidence for a conserved system for iron metabolism in the mitochondria of Saccharomyces cerevisiae, Proc. Natl ,
DOI : 10.1073/pnas.96.18.10206
The mitochondrial proteins Atm1p and Nfs1p are essential for biogenesis of cytosolic Fe/S proteins, The EMBO Journal, vol.18, issue.14, 1999. ,
DOI : 10.1093/emboj/18.14.3981
The role of Fe???S proteins in sensing and regulation in bacteria, Current Opinion in Microbiology, vol.6, issue.2, pp.181-185, 2003. ,
DOI : 10.1016/S1369-5274(03)00039-0
Iron-sulfur clusters: ever-expanding roles, Nature Chemical Biology, vol.12, issue.4, pp.171-174, 2006. ,
DOI : 10.1038/nchembio0406-171
Iron-sulfur proteins: ancient structures, still full of surprises, Journal of Biological Inorganic Chemistry, vol.5, issue.1, pp.2-15, 2000. ,
DOI : 10.1007/s007750050002
Structure of the Hydrophilic Domain of Respiratory Complex I from Thermus thermophilus, Science, vol.311, issue.5766, pp.1430-1436, 2006. ,
DOI : 10.1126/science.1123809
The structure of aconitase, Proteins: Structure, Function, and Genetics, vol.745, issue.4, 1989. ,
DOI : 10.1002/prot.340050406
Crystal structures of aconitase with isocitrate and nitroisocitrate bound, Biochemistry, vol.31, issue.10, pp.2735-2748, 1992. ,
DOI : 10.1021/bi00125a014
An iron-sulfur cluster plays a novel regulatory role in the iron-responsive element binding protein, BioMetals, vol.265, issue.3, pp.131-140, 1992. ,
DOI : 10.1007/BF01061319
Mechanistic investigations of lipoic acid biosynthesis in Escherichia coli: both sulfur atoms in lipoic acid are contributed by the same lipoyl synthase polypeptide, J. Am. Chem. Soc, vol.127, 2005. ,
Evidence that the fourth ligand to the [2Fee2S] cluster in animal ferrochelatase is a cysteine. Characterization of the enzyme from Drosophila melanogaster, J. Biol. Chem, pp.273-22311, 1998. ,
Disease-causing missense mutations in human DNA helicase disorders, Eukaryotic DNA polymerases require an ironesulfur cluster for the formation of active complexes, pp.125-132, 2012. ,
DOI : 10.1016/j.mrrev.2012.12.004
Building Fe???S proteins: bacterial strategies, Nature Reviews Microbiology, vol.28, issue.6, pp.436-446, 2010. ,
DOI : 10.1038/nrmicro2356
Mammalian Frataxin: An Essential Function for Cellular Viability through an Interaction with a Preformed ISCU/NFS1/ISD11 Iron-Sulfur Assembly Complex, PLoS ONE, vol.1, issue.1, p.16199, 2011. ,
DOI : 10.1371/journal.pone.0016199.s009
Frataxin is essential for extramitochondrial FeeS cluster proteins in mammalian tissues, Hum. Mol. Genet, p.16, 2007. ,
Biogenesis of FeeS cluster by the bacterial Suf system: SufS and SufE form a new type of cysteine desulfurase, J. Biol. Chem, pp.278-38352, 2003. ,
Essential role of Isd11 in mitochondrial iron???sulfur cluster synthesis on Isu scaffold proteins, The EMBO Journal, vol.33, issue.1, pp.184-195, 2006. ,
DOI : 10.1038/sj.emboj.7600906
URL : https://hal.archives-ouvertes.fr/hal-00136402
Dancis, D. Pain, Isd11p protein activates the mitochondrial cysteine desulfurase Nfs1p protein, J. Biol. Chem, pp.286-38242, 2011. ,
The Nfs1 interacting protein Isd11 has an essential role in Fe/S cluster biogenesis in mitochondria, The EMBO Journal, vol.90, issue.1, pp.174-183, 2006. ,
DOI : 10.1038/sj.emboj.7600905
Functional studies of frataxin, Acta Paediatrica, vol.278, 2004. ,
DOI : 10.1111/j.1651-2227.2004.tb03061.x
Solution Structure of the Bacterial Frataxin Ortholog, CyaY, Structure, vol.12, issue.11, 2004. ,
DOI : 10.1016/j.str.2004.08.012
Human Frataxin Is an Allosteric Switch That Activates the Fe???S Cluster Biosynthetic Complex, Biochemistry, vol.49, issue.43, pp.9132-9139, 2010. ,
DOI : 10.1021/bi1013062
Cluster Assembly, Journal of the American Chemical Society, vol.135, issue.2, pp.733-740, 2013. ,
DOI : 10.1021/ja308736e
URL : https://hal.archives-ouvertes.fr/hal-01054359
Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS, Nature Structural & Molecular Biology, vol.5, issue.4, pp.390-396, 2009. ,
DOI : 10.1038/nsmb.1579
Effector Role Reversal during Evolution: The Case of Frataxin in Fe???S Cluster Biosynthesis, Biochemistry, vol.51, issue.12, pp.2506-2514, 2012. ,
DOI : 10.1021/bi201628j
Both human ferredoxins 1 and 2 and ferredoxin reductase are important for iron-sulfur cluster biogenesis, Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, vol.1823, issue.2, pp.484-492, 2012. ,
DOI : 10.1016/j.bbamcr.2011.11.002
[2Fee2S]- Ferredoxin binds directly to cysteine desulfurase and supplies an electron for ironesulfur cluster assembly but is displaced by the scaffold protein or bacterial frataxin, J. Am. Chem. Soc, vol.135, issue.22, 2013. ,
Ferredoxin Competes with Bacterial Frataxin in Binding to the Desulfurase IscS, Journal of Biological Chemistry, vol.288, issue.34, pp.28834-24777, 2013. ,
DOI : 10.1074/jbc.M113.480327
Humans possess two mitochondrial ferredoxins, Fdx1 and Fdx2, with distinct roles in steroidogenesis, heme, and Fe/S cluster biosynthesis, Proceedings of the National Academy of Sciences, vol.107, issue.26, 2010. ,
DOI : 10.1073/pnas.1004250107
Deleterious mutation in FDX1L gene is associated with a novel mitochondrial muscle myopathy, European Journal of Human Genetics, vol.129, issue.7, 2013. ,
DOI : 10.1038/ejhg.2013.269
Molecular chaperones HscA/Ssq1 and HscB/ Jac1 and their roles in ironesulfur protein maturation, Crit. Rev. Biochem. Mol. Biol, pp.42-95, 2007. ,
Characterization of the human HSC20, an unusual DnaJ type III protein, involved in iron-sulfur cluster biogenesis, Human Molecular Genetics, vol.19, issue.19, pp.3816-3834, 2010. ,
DOI : 10.1093/hmg/ddq301
A specialized mitochondrial molecular chaperone system:??A role in formation of Fe/S centers, Cellular and Molecular Life Sciences, vol.59, issue.10, pp.1658-1665, 2002. ,
DOI : 10.1007/PL00012493
Sequence-specific interaction between mitochondrial FeeS scaffold protein Isu and Hsp70 Ssq1 is essential for their in vivo function, J. Biol. Chem, pp.279-29167, 2004. ,
Contributions of the LPPVK motif of the ironesulfur template protein IscU to interactions with the Hsc66eHsc20 chaperone system, J. Biol. Chem, pp.278-37582, 2003. ,
The mitochondrial Hsp70 chaperone Ssq1 facilitates Fe/S cluster transfer from Isu1 to Grx5 by complex formation, Molecular Biology of the Cell, vol.24, issue.12, pp.24-1830, 2013. ,
DOI : 10.1091/mbc.E12-09-0644
Glutaredoxin 5 deficiency causes sideroblastic anemia by specifically impairing heme biosynthesis and depleting cytosolic iron in human erythroblasts, Journal of Clinical Investigation, vol.120, issue.5, pp.1749-1761, 2010. ,
DOI : 10.1172/JCI40372DS1
Glutaredoxin systems, Biochimica et Biophysica Acta (BBA) - General Subjects, vol.1780, issue.11, pp.1304-1317, 2008. ,
DOI : 10.1016/j.bbagen.2008.06.003
Human ind1, an ironesulfur cluster assembly factor for respiratory complex I, Mol. Cell. Biol, pp.29-6059, 2009. ,
Mutations in Iron-Sulfur Cluster Scaffold Genes NFU1 and BOLA3 Cause a Fatal Deficiency of Multiple Respiratory Chain and 2-Oxoacid Dehydrogenase Enzymes, The American Journal of Human Genetics, vol.89, issue.4, pp.486-495, 2011. ,
DOI : 10.1016/j.ajhg.2011.08.011
A Fatal Mitochondrial Disease Is Associated with Defective NFU1 Function in the Maturation of a Subset of Mitochondrial Fe-S Proteins, The American Journal of Human Genetics, vol.89, issue.5, pp.656-667, 2011. ,
DOI : 10.1016/j.ajhg.2011.10.005
The human mitochondrial ISCA1, ISCA2, and IBA57 proteins are required for [4Fe-4S] protein maturation, Molecular Biology of the Cell, vol.23, issue.7, pp.1157-1166, 2012. ,
DOI : 10.1091/mbc.E11-09-0772
Mutation of the irone sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy, Hum. Mol. Genet, pp.22-2590, 2013. ,
Role of Saccharomyces cerevisiae ISA1 and ISA2 in Iron Homeostasis, Molecular and Cellular Biology, vol.20, issue.11, pp.3918-3927, 2000. ,
DOI : 10.1128/MCB.20.11.3918-3927.2000
Isa1p is a component of the mitochondrial machinery for maturation of cellular ironesulfur proteins and requires conserved cysteine residues for function, J. Biol. Chem, pp.275-15955, 2000. ,
Iron-binding activity of human ironesulfur cluster assembly protein hIscA1, Biochem. J, pp.428-125, 2010. ,
Distinct iron binding property of two putative iron donors for the ironesulfur cluster assembly: IscA and the bacterial frataxin ortholog CyaY under physiological and oxidative stress conditions, J. Biol. Chem, pp.282-7997, 2007. ,
Iron-Sulfur (Fe/S) Protein Biogenesis: Phylogenomic and Genetic Studies of A-Type Carriers, PLoS Genetics, vol.353, issue.5, p.1000497, 2009. ,
DOI : 10.1371/journal.pgen.1000497.s004
URL : https://hal.archives-ouvertes.fr/hal-00698313
Iron binding activity is essential for the function of IscA in ironesulphur cluster biogenesis, pp.42-3100, 2013. ,
IscA, Biochemistry, vol.51, issue.41, pp.8071-8084, 2012. ,
DOI : 10.1021/bi3006658
Characterization of iron-sulfur cluster assembly protein isca from Acidithiobacillus ferrooxidans, Biochemistry (Moscow), vol.78, issue.3, pp.244-251, 2013. ,
DOI : 10.1134/S000629791303005X
Specialized function of yeast Isa1 and Isa2 proteins in the maturation of mitochondrial [4Fee4S] proteins, J. Biol. Chem, pp.286-41205, 2011. ,
Mitochondrial Iba57p Is Required for Fe/S Cluster Formation on Aconitase and Activation of Radical SAM Enzymes, Molecular and Cellular Biology, vol.28, issue.5, pp.28-1851, 2008. ,
DOI : 10.1128/MCB.01963-07
High-throughput, pooled sequencing identifies mutations in NUBPL and FOXRED1 in human complex I deficiency, Nature Genetics, vol.1792, issue.10, pp.42-851, 2010. ,
DOI : 10.1093/hmg/7.6.981
The molecular basis of human complex I deficiency, IUBMB Life, vol.348, pp.63-669, 2011. ,
DOI : 10.1002/iub.495
NUBPL mutations in patients with complex I deficiency and a distinct MRI pattern, Neurology, vol.80, issue.17, pp.1577-1583, 2013. ,
DOI : 10.1212/WNL.0b013e31828f1914
Massive and exclusive pontocerebellar damage in mitochondrial disease and NUBPL mutations, Massive and exclusive pontocerebellar damage in mitochondrial disease and NUBPL mutations, p.391, 2012. ,
DOI : 10.1212/WNL.0b013e3182611232
The iron???sulphur protein Ind1 is required for effective complex I assembly, The EMBO Journal, vol.21, issue.12, pp.1736-1746, 2008. ,
DOI : 10.1038/emboj.2008.98
Ironesulfur cluster biosynthesis: characterization of a molten globule domain in human NFU Subcellular compartmentalization of human Nfu, an ironesulfur cluster scaffold protein, and its ability to assemble a [4Fe-4S] cluster, Biochemistry Proc. Natl. Acad. Sci. U. S. A, vol.48, issue.100, pp.9762-9767, 2003. ,
Iron sulfur cluster biosynthesis. Human NFU mediates sulfide delivery to ISU in the final step of [2Fe???2S] cluster assembly, Chemical Communications, vol.280, issue.30, pp.3192-3194, 2007. ,
DOI : 10.1039/b704928e
Molecular organization, biochemical function, cellular role and evolution of NfuA, an atypical Fe-S carrier, Molecular Microbiology, vol.183, issue.1, pp.155-171, 2012. ,
DOI : 10.1111/j.1365-2958.2012.08181.x
URL : https://hal.archives-ouvertes.fr/hal-01054360
Protein expression profiles in patients carrying NFU1 mutations. Contribution to the pathophysiology of the disease, Journal of Inherited Metabolic Disease, vol.44, issue.1, pp.36-841, 2013. ,
DOI : 10.3109/07853890.2011.598547
Homozygous missense mutation in BOLA3 causes multiple mitochondrial dysfunctions syndrome in two siblings, Journal of Inherited Metabolic Disease, vol.14, issue.3, pp.36-55, 2013. ,
DOI : 10.1007/s10545-012-9489-7
Human ABC7 transporter: gene structure and mutation causing X-linked sideroblastic anemia with ataxia with disruption of cytosolic ironesulfur protein maturation, Blood, vol.96, pp.3256-3264, 2000. ,
Cytosolic ironesulfur cluster assembly (CIA) system: factors, mechanism, and relevance to cellular iron regulation, J. Biol. Chem, p.285, 2010. ,
An essential function of the mitochondrial sulfhydryl oxidase Erv1p/ALR in the maturation of cytosolic Fe/S proteins, EMBO reports, vol.273, issue.8, pp.715-720, 2001. ,
DOI : 10.1093/embo-reports/kve161
Role of human mitochondrial Nfs1 in cytosolic ironesulfur protein biogenesis and iron regulation, Mol. Cell. Biol, pp.26-5675, 2006. ,
Mutation of a Putative Mitochondrial Iron Transporter Gene (ABC7) in X-Linked Sideroblastic Anemia and Ataxia (XLSA/A), Human Molecular Genetics, vol.8, issue.5, pp.743-749, 1999. ,
DOI : 10.1093/hmg/8.5.743
X-linked cerebellar ataxia and sideroblastic anaemia associated with a missense mutation in the ABC7 gene predicting V411L, British Journal of Haematology, vol.13, issue.4, pp.115-910, 2001. ,
DOI : 10.1093/hmg/7.13.2021
X-linked sideroblastic anemia and ataxia: a new family with identification of a fourth ABCB7 gene mutation, Eur. J. Paediatr. Neurol, vol.16, pp.730-735, 2012. ,
Abcb7, the gene responsible for X-linked sideroblastic anemia with ataxia, is essential for hematopoiesis, Blood, vol.109, issue.8, pp.3567-3569, 2007. ,
DOI : 10.1182/blood-2006-04-015768
The mitochondrial ATP-binding cassette transporter Abcb7 is essential in mice and participates in cytosolic ironesulfur cluster biogenesis, Hum. Mol. Genet, pp.15-953, 2006. ,
RNA silencing of the mitochondrial ABCB7 transporter in HeLa cells causes an iron-deficient phenotype with mitochondrial iron overload, Blood, vol.109, issue.8, pp.3552-3559, 2007. ,
DOI : 10.1182/blood-2006-08-041632
Maturation of cytosolic ironesulfur proteins requires glutathione, J. Biol. Chem, p.277, 2002. ,
Maturation of cytosolic and nuclear ironesulfur proteins, Trends Cell. Biol, 2013. ,
Human Nbp35 is essential for both cytosolic ironesulfur protein assembly and iron homeostasis, Mol. Cell. Biol, p.28, 2008. ,
The Cfd1???Nbp35 complex acts as a scaffold for iron-sulfur protein assembly in the yeast cytosol, Nature Chemical Biology, vol.126, issue.5, pp.278-286, 2007. ,
DOI : 10.1093/nar/22.25.5767
A Role for IOP1 in Mammalian Cytosolic Iron-Sulfur Protein Biogenesis, Journal of Biological Chemistry, vol.283, issue.14, pp.9231-9238, 2008. ,
DOI : 10.1074/jbc.M708077200
The hydrogenase-like Nar1p is essential for maturation of cytosolic and nuclear iron???sulphur proteins, The EMBO Journal, vol.179, issue.10, pp.2105-2115, 2004. ,
DOI : 10.1016/S0168-6445(01)00063-8
Structure of the Yeast WD40 Domain Protein Cia1, a Component Acting Late in Iron-Sulfur Protein Biogenesis, Structure, vol.15, issue.10, pp.1246-1257, 2007. ,
DOI : 10.1016/j.str.2007.08.009
The essential WD40 protein Cia1 is involved in a late step of cytosolic and nuclear ironesulfur protein assembly, Mol. Cell. Biol, p.25, 2005. ,
Dre2, a Conserved Eukaryotic Fe/S Cluster Protein, Functions in Cytosolic Fe/S Protein Biogenesis, Molecular and Cellular Biology, vol.28, issue.18, pp.5569-5582, 2008. ,
DOI : 10.1128/MCB.00642-08
Targeting of a Human Iron???Sulfur Cluster Assembly Enzyme, nifs, to Different Subcellular Compartments Is Regulated through Alternative AUG Utilization, Molecular Cell, vol.2, issue.6, pp.807-815, 1998. ,
DOI : 10.1016/S1097-2765(00)80295-6
Human ISD11 is essential for both iron-sulfur cluster assembly and maintenance of normal cellular iron homeostasis, Human Molecular Genetics, vol.18, issue.16, pp.3014-3025, 2009. ,
DOI : 10.1093/hmg/ddp239
Functions of mitochondrial ISCU and cytosolic ISCU in mammalian ironesulfur cluster biogenesis and iron homeostasis, Cell. Metab, vol.3, 2006. ,
The L-Cysteine Desulfurase NFS1 Is Localized in the Cytosol where it Provides the Sulfur for Molybdenum Cofactor Biosynthesis in Humans, PLoS ONE, vol.275, issue.4, p.60869, 2013. ,
DOI : 10.1371/journal.pone.0060869.s004
Evolution of the Friedreich's ataxia trinucleotide repeat expansion: Founder effect and premutations, Proceedings of the National Academy of Sciences, vol.94, issue.14, pp.94-7452, 1997. ,
DOI : 10.1073/pnas.94.14.7452
Friedreich's ataxia: Pathology, pathogenesis, and molecular genetics, Journal of the Neurological Sciences, vol.303, issue.1-2, pp.1-12, 2011. ,
DOI : 10.1016/j.jns.2011.01.010
The heart disease of Friedreich's ataxia: a clinical and electrocardiographic study of 115 patients, with an analysis of serial electrocardiographic changes in 30 cases, Q. J. Med, pp.52-489, 1983. ,
Aconitase and mitochondrial ironesulphur protein deficiency in Friedreich ataxia, Nat. Genet, pp.17-215, 1997. ,
SUMMARY:, Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques, vol.3, issue.04, pp.389-396, 1980. ,
DOI : 10.1002/path.1700580414
Increased levels of plasma malondialdehyde in Friedreich ataxia, Neurology, vol.55, issue.11, pp.1752-1753, 2000. ,
DOI : 10.1212/WNL.55.11.1752
Oxidative stress in patients with Friedreich ataxia, Neurology, vol.55, issue.11, pp.1719-1721, 2000. ,
DOI : 10.1212/WNL.55.11.1719
DNA triplet repeats mediate heterochromatin-protein-1-sensitive variegated gene silencing, Nature, vol.422, issue.6934, 2003. ,
DOI : 10.1038/nature01596
Understanding the molecular mechanisms of Friedreich's ataxia to develop therapeutic approaches, Human Molecular Genetics, vol.19, issue.R1, 2010. ,
DOI : 10.1093/hmg/ddq165
Friedreich's Ataxia: Autosomal Recessive Disease Caused by an Intronic GAA Triplet Repeat Expansion, Science, vol.271, issue.5254, pp.1423-1427, 1996. ,
DOI : 10.1126/science.271.5254.1423
Friedreich's ataxia: Point mutations and clinical presentation of compound heterozygotes, Annals of Neurology, vol.19, issue.2, p.45, 1999. ,
DOI : 10.1002/1531-8249(199902)45:2<200::AID-ANA10>3.0.CO;2-U
Frataxin gene point mutations in Italian Friedreich ataxia patients, Neurogenetics, vol.16, issue.4, pp.289-299, 2007. ,
DOI : 10.1007/s10048-007-0101-5
Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation, Human Molecular Genetics, vol.9, issue.8 ,
DOI : 10.1093/hmg/9.8.1219
Understanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular models, Disease Models & Mechanisms, vol.5, issue.2, pp.165-176, 2012. ,
DOI : 10.1242/dmm.008706
Primary and Secondary Drug Screening Assays for Friedreich Ataxia, Journal of Biomolecular Screening, vol.64, issue.3, 2012. ,
DOI : 10.1056/NEJM199610173351601
Hereditary metabolic myopathy with paroxysmal myoglobinuria due to abnormal glycolysis, Journal of Neurology, Neurosurgery & Psychiatry, vol.27, issue.5, pp.361-380, 1964. ,
DOI : 10.1136/jnnp.27.5.361
Mitochondrial myopathy with succinate dehydrogenase and aconitase deficiency. Abnormalities of several ironesulfur proteins, J. Clin. Invest, p.92, 1993. ,
Splice Mutation in the Iron-Sulfur Cluster Scaffold Protein ISCU Causes Myopathy with Exercise??Intolerance, The American Journal of Human Genetics, vol.82, issue.3, pp.652-660, 2008. ,
DOI : 10.1016/j.ajhg.2007.12.012
Myopathy with lactic acidosis is linked to chromosome 12q23.3-24.11 and caused by an intron mutation in the ISCU gene resulting in a splicing defect, Human Molecular Genetics, vol.17, issue.11, pp.539-544, 2008. ,
DOI : 10.1093/hmg/ddn057
Tissue-specific splicing of ISCU results in a skeletal muscle phenotype in myopathy with lactic acidosis, while complete loss of ISCU results in early embryonic death in mice, Human Genetics, vol.281, issue.38, pp.129-371, 2011. ,
DOI : 10.1007/s00439-010-0931-3
Clinical manifestation and a new ISCU mutation in ironesulphur cluster deficiency myopathy, pp.2170-2179, 2009. ,
Antisense oligonucleotide therapeutics for iron???sulphur cluster deficiency myopathy, Neuromuscular Disorders, vol.19, issue.12, pp.833-836, 2009. ,
DOI : 10.1016/j.nmd.2009.09.011
The human counterpart of zebrafish shiraz shows sideroblastic-like microcytic anemia and iron overload, Blood, vol.110, issue.4, 2007. ,
DOI : 10.1182/blood-2007-02-072520
Deficiency of glutaredoxin 5 reveals FeeS clusters are required for vertebrate haem synthesis, Nature, vol.436, 2005. ,
The Mitochondrial Disulfide Relay System Protein GFER Is Mutated in Autosomal-Recessive Myopathy with Cataract and Combined Respiratory-Chain Deficiency, The American Journal of Human Genetics, vol.84, issue.5, pp.594-604, 2009. ,
DOI : 10.1016/j.ajhg.2009.04.004
Hereditary sideroblastic anaemia and ataxia: an X linked recessive disorder., Journal of Medical Genetics, vol.22, issue.4, p.22, 1985. ,
DOI : 10.1136/jmg.22.4.267
Hammans, X-linked sideroblastic anaemia with ataxia: another mitochondrial disease?, J. Neurol. Neurosurg. Psychiatr, pp.70-65, 2001. ,
The Role of the Iron Transporter ABCB7 in Refractory Anemia with Ring Sideroblasts, PLoS ONE, vol.80, issue.4, p.1970, 2008. ,
DOI : 10.1371/journal.pone.0001970.t001
The transporter ABCB7 is a mediator of the phenotype of acquired refractory anemia with ring sideroblasts, Leukemia, vol.89, issue.4, pp.889-896, 2013. ,
DOI : 10.1182/blood-2011-12-399774
Recent advances in the understanding of inherited sideroblastic anaemia, British Journal of Haematology, vol.27, issue.1, pp.27-38, 2008. ,
DOI : 10.1111/j.1365-2141.2008.07290.x
Lipoic Acid Synthetase Deficiency Causes Neonatal-Onset Epilepsy, Defective Mitochondrial Energy Metabolism, and Glycine Elevation, The American Journal of Human Genetics, vol.89, issue.6, pp.792-797, 2011. ,
DOI : 10.1016/j.ajhg.2011.11.011
Alpha-lipoic acid as a dietary supplement: Molecular mechanisms and therapeutic potential, Biochimica et Biophysica Acta (BBA) - General Subjects, vol.1790, issue.10, p.1790, 2009. ,
DOI : 10.1016/j.bbagen.2009.07.026
Therapy for mitochondrial disorders: Little proof, high research activity, some promise, Seminars in Fetal and Neonatal Medicine, vol.16, issue.4, pp.236-240, 2011. ,
DOI : 10.1016/j.siny.2011.05.003
The ATM protein kinase: regulating the cellular response to genotoxic stress, and more, Nature Reviews Molecular Cell Biology, vol.13, issue.4, p.14, 2013. ,
DOI : 10.4161/cc.23592
Iron Regulatory Proteins Secure Mitochondrial Iron Sufficiency and Function, Cell Metabolism, vol.12, issue.2, 2010. ,
DOI : 10.1016/j.cmet.2010.06.007
Two to Tango: Regulation of Mammalian Iron Metabolism, Cell, vol.142, issue.1, pp.24-38, 2010. ,
DOI : 10.1016/j.cell.2010.06.028
Iron/sulfur proteins biogenesis in prokaryotes: Formation, regulation and diversity, Biochimica et Biophysica Acta (BBA) - Bioenergetics, vol.1827, issue.3, pp.455-469, 2013. ,
DOI : 10.1016/j.bbabio.2012.12.010
increase phosphorylation of peroxiredoxin 3 exacerbating oxidative stress-induced neuronal death, Human Mutation, vol.44, issue.12, pp.1390-1397, 2011. ,
DOI : 10.1002/humu.21582
[2Fe-2S] cluster transfer in iron-sulfur protein biogenesis, Proceedings of the National Academy of Sciences, vol.111, issue.17, pp.6203-6208, 2014. ,
DOI : 10.1073/pnas.1400102111
Mammalian Fe???S cluster biogenesis and its implication in disease, Biochimie, vol.100, pp.48-60, 2014. ,
DOI : 10.1016/j.biochi.2014.01.009
The iron???sulphur protein Ind1 is required for effective complex I assembly, The EMBO Journal, vol.21, issue.12, pp.1736-1746, 2008. ,
DOI : 10.1038/emboj.2008.98
The human counterpart of zebrafish shiraz shows sideroblastic-like microcytic anemia and iron overload, Blood, vol.110, issue.4, pp.1353-1358, 2007. ,
DOI : 10.1182/blood-2007-02-072520
Mutations in Iron-Sulfur Cluster Scaffold Genes NFU1 and BOLA3 Cause a Fatal Deficiency of Multiple Respiratory Chain and 2-Oxoacid Dehydrogenase Enzymes, The American Journal of Human Genetics, vol.89, issue.4, pp.486-495, 2011. ,
DOI : 10.1016/j.ajhg.2011.08.011
Cluster Assembly, Journal of the American Chemical Society, vol.135, issue.2, pp.733-740, 2013. ,
DOI : 10.1021/ja308736e
URL : https://hal.archives-ouvertes.fr/hal-01054359
Characterization of iron binding in IscA, an ancient iron-sulphur cluster assembly protein, Biochemical Journal, vol.379, issue.2, pp.433-440, 2004. ,
DOI : 10.1042/bj20031702
IscA Mediates Iron Delivery for Assembly of Iron-Sulfur Clusters in IscU under the Limited Accessible Free Iron Conditions, Journal of Biological Chemistry, vol.279, issue.36, pp.37499-37504, 2004. ,
DOI : 10.1074/jbc.M404533200
Optical and EPR characterization of different species of active and inactive aconitase, J Biol Chem, vol.258, issue.18, pp.11106-11111, 1983. ,
Analyzing chromatin remodeling complexes using shotgun proteomics and normalized spectral abundance factors, Methods, vol.40, issue.4, pp.303-311, 2006. ,
DOI : 10.1016/j.ymeth.2006.07.028
Inactivation-reactivation of aconitase in Escherichia coli. A sensitive measure of superoxide radical, J Biol Chem, vol.267, issue.13, pp.8757-8763, 1992. ,
Saccharomyces cerevisiae ISU1 and ISU2: members of a well-conserved gene family for iron-sulfur cluster assembly, Journal of Molecular Biology, vol.294, issue.4, pp.897-907, 1999. ,
DOI : 10.1006/jmbi.1999.3294
Mitochondrial Iba57p Is Required for Fe/S Cluster Formation on Aconitase and Activation of Radical SAM Enzymes, Molecular and Cellular Biology, vol.28, issue.5, pp.1851-1861, 2008. ,
DOI : 10.1128/MCB.01963-07
SufA, Coexpressed with SufBCDSE, Purifies as a [2Fe???2S] Protein and Acts as an Fe???S Transporter to Fe???S Target Enzymes, Journal of the American Chemical Society, vol.131, issue.17, pp.6149-6153, 2009. ,
DOI : 10.1021/ja807551e
URL : https://hal.archives-ouvertes.fr/hal-00374830
Role of Saccharomyces cerevisiae ISA1 and ISA2 in Iron Homeostasis, Molecular and Cellular Biology, vol.20, issue.11, pp.3918-3927, 2000. ,
DOI : 10.1128/MCB.20.11.3918-3927.2000
Isa1p Is a Component of the Mitochondrial Machinery for Maturation of Cellular Iron-Sulfur Proteins and Requires Conserved Cysteine Residues for Function, Journal of Biological Chemistry, vol.275, issue.21, pp.15955-15961, 2000. ,
DOI : 10.1074/jbc.M909502199
[2Fe-2S]-Ferredoxin Binds Directly to Cysteine Desulfurase and Supplies an Electron for Iron???Sulfur Cluster Assembly but Is Displaced by the Scaffold Protein or Bacterial Frataxin, Journal of the American Chemical Society, vol.135, issue.22, 2013. ,
DOI : 10.1021/ja401950a
Function and biogenesis of iron???sulphur proteins, Nature, vol.283, issue.7257, pp.831-838, 2009. ,
DOI : 10.1038/nature08301
ErpA, an iron sulfur (Fe S) protein of the A-type essential for respiratory metabolism in Escherichia coli, Proceedings of the National Academy of Sciences, vol.104, issue.34, pp.13626-13631, 2007. ,
DOI : 10.1073/pnas.0705829104
URL : https://hal.archives-ouvertes.fr/hal-00379510
Iron-binding activity of human iron???sulfur cluster assembly protein hIscA1, Biochemical Journal, vol.253, issue.1, pp.125-131, 2010. ,
DOI : 10.1016/j.febslet.2005.10.046
IscA, Biochemistry, vol.51, issue.41, pp.8056-8070, 2012. ,
DOI : 10.1021/bi300664j
Monothiol glutaredoxins and A-type proteins: partners in Fe???S cluster trafficking, Dalton Transactions, vol.279, issue.9, pp.3107-3115, 2013. ,
DOI : 10.1039/c2dt32263c
URL : https://hal.archives-ouvertes.fr/hal-01268219
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
Understanding mitochondrial complex I assembly in health and disease, Biochimica et Biophysica Acta (BBA) - Bioenergetics, vol.1817, issue.6, pp.851-862, 2012. ,
DOI : 10.1016/j.bbabio.2011.08.010
The Asymmetric IscA Homodimer with an Exposed [2Fe-2S] Cluster Suggests the Structural Basis of the Fe-S Cluster Biosynthetic Scaffold, Journal of Molecular Biology, vol.360, issue.1, pp.117-132, 2006. ,
DOI : 10.1016/j.jmb.2006.04.067
Specialized Function of Yeast Isa1 and Isa2 Proteins in the Maturation of Mitochondrial [4Fe-4S] Proteins, Journal of Biological Chemistry, vol.286, issue.48, pp.41205-41216, 2011. ,
DOI : 10.1074/jbc.M111.296152
A Fatal Mitochondrial Disease Is Associated with Defective NFU1 Function in the Maturation of a Subset of Mitochondrial Fe-S Proteins, The American Journal of Human Genetics, vol.89, issue.5, pp.656-667, 2011. ,
DOI : 10.1016/j.ajhg.2011.10.005
Iron-Sulfur Cluster Assembly: CHARACTERIZATION OF IscA AND EVIDENCE FOR A SPECIFIC AND FUNCTIONAL COMPLEX WITH FERREDOXIN, Journal of Biological Chemistry, vol.276, issue.25, pp.22604-22607, 2001. ,
DOI : 10.1074/jbc.M102902200
SufA/IscA: reactivity studies of a class of scaffold proteins involved in [Fe-S] cluster assembly, JBIC Journal of Biological Inorganic Chemistry, vol.275, issue.7, pp.828-838, 2004. ,
DOI : 10.1007/s00775-004-0581-9
Isd11p Protein Activates the Mitochondrial Cysteine Desulfurase Nfs1p Protein, Journal of Biological Chemistry, vol.286, issue.44, pp.38242-38252, 2011. ,
DOI : 10.1074/jbc.M111.288522
Mitochondrial Isa2p plays a crucial role in the maturation of cellular iron-sulfur proteins, FEBS Letters, vol.194, issue.3, pp.134-139, 2000. ,
DOI : 10.1016/S0014-5793(00)01711-7
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
In vivo iron-sulfur cluster formation, Proceedings of the National Academy of Sciences, vol.105, issue.25, pp.8591-8596, 2008. ,
DOI : 10.1073/pnas.0803173105
A Conserved Mitochondrial ATP-binding Cassette Transporter Exports Glutathione Polysulfide for Cytosolic Metal Cofactor Assembly, Journal of Biological Chemistry, vol.289, issue.34, pp.23264-23274, 2014. ,
DOI : 10.1074/jbc.M114.553438
The in vivo mitochondrial two-step maturation of human frataxin, Human Molecular Genetics, vol.17, issue.22, pp.3521-3531, 2008. ,
DOI : 10.1093/hmg/ddn244
URL : https://hal.archives-ouvertes.fr/inserm-00350838
Mammalian Frataxin: An Essential Function for Cellular Viability through an Interaction with a Preformed ISCU/NFS1/ISD11 Iron-Sulfur Assembly Complex, PLoS ONE, vol.1, issue.1, p.16199, 2011. ,
DOI : 10.1371/journal.pone.0016199.s009
Human Ind1, an Iron-Sulfur Cluster Assembly Factor for Respiratory Complex I, Molecular and Cellular Biology, vol.29, issue.22, pp.6059-6073, 2009. ,
DOI : 10.1128/MCB.00817-09
The human mitochondrial ISCA1, ISCA2, and IBA57 proteins are required for [4Fe-4S] protein maturation, Molecular Biology of the Cell, vol.23, issue.7, pp.1157-1166, 2012. ,
DOI : 10.1091/mbc.E11-09-0772
Cloning, sequencing, and overexpression of a [2Fe-2S] ferredoxin gene from Escherichia coli, J Biol Chem, vol.267, issue.16, pp.11120-11125, 1992. ,
under aerobic growth conditions, Biochemical Journal, vol.282, issue.3, pp.463-472, 2009. ,
DOI : 10.1074/jbc.M803395200
Crystal Structure of the YgfZ Protein from Escherichia coli Suggests a Folate-Dependent Regulatory Role in One-Carbon Metabolism, Journal of Bacteriology, vol.186, issue.21, pp.7134-7140, 2004. ,
DOI : 10.1128/JB.186.21.7134-7140.2004
Human Frataxin Is an Allosteric Switch That Activates the Fe???S Cluster Biosynthetic Complex, Biochemistry, vol.49, issue.43, pp.9132-9139, 2010. ,
DOI : 10.1021/bi1013062
The mitochondrial Hsp70 chaperone Ssq1 facilitates Fe/S cluster transfer from Isu1 to Grx5 by complex formation, Molecular Biology of the Cell, vol.24, issue.12, pp.1830-1841, 2013. ,
DOI : 10.1091/mbc.E12-09-0644
Iron-Sulfur (Fe/S) Protein Biogenesis: Phylogenomic and Genetic Studies of A-Type Carriers, PLoS Genetics, vol.353, issue.5, p.1000497, 2009. ,
DOI : 10.1371/journal.pgen.1000497.s004
URL : https://hal.archives-ouvertes.fr/hal-00698313
Architecture of mammalian respiratory complex I, Nature, vol.8, issue.7525, 2014. ,
DOI : 10.1038/nature13686
SufA involved in biosynthesis of iron-sulfur clusters: Implications for a functional dimer, FEBS Letters, vol.93, issue.29, pp.6543-6548, 2005. ,
DOI : 10.1016/j.febslet.2005.10.046
A role for tetrahydrofolates in the metabolism of iron-sulfur clusters in all domains of life, Proceedings of the National Academy of Sciences, vol.107, issue.23, pp.10412-10417, 2010. ,
DOI : 10.1073/pnas.0911586107
Peroxiredoxin-3 is overexpressed in prostate cancer and promotes cancer cell survival by protecting cells from oxidative stress, British Journal of Cancer, vol.13, issue.4, pp.983-993, 2013. ,
DOI : 10.1038/bjc.2013.396
Essential role of Isd11 in mitochondrial iron???sulfur cluster synthesis on Isu scaffold proteins, The EMBO Journal, vol.33, issue.1, pp.184-195, 2006. ,
DOI : 10.1038/sj.emboj.7600906
URL : https://hal.archives-ouvertes.fr/hal-00136402
Erratum: Deficiency of glutaredoxin 5 reveals Fe???S clusters are required for vertebrate haem synthesis, Nature, vol.437, issue.7060, pp.1035-1039, 2005. ,
DOI : 10.1038/nature03887
Glutaredoxin 5 deficiency causes sideroblastic anemia by specifically impairing heme biosynthesis and depleting cytosolic iron in human erythroblasts, Journal of Clinical Investigation, vol.120, issue.5, pp.1749-1761, 2010. ,
DOI : 10.1172/JCI40372DS1
The IscA from Acidithiobacillus ferrooxidans is an iron???sulfur protein which assemble the [Fe4S4] cluster with intracellular iron and sulfur, Archives of Biochemistry and Biophysics, vol.463, issue.2, pp.237-244, 2007. ,
DOI : 10.1016/j.abb.2007.03.024
Iron-Sulfur Cluster Biogenesis in Chloroplasts. Involvement of the Scaffold Protein CpIscA, PLANT PHYSIOLOGY, vol.138, issue.1, pp.161-172, 2005. ,
DOI : 10.1104/pp.104.058602
The Nfs1 interacting protein Isd11 has an essential role in Fe/S cluster biogenesis in mitochondriaAssembly of iron?sulfur clusters. Identification of an iscSUA? hscBA?fdx gene cluster from Azotobacter vinelandii, 174?183. References References References References References References References References References References References References References References References Zheng, pp.13264-13272, 1998. ,
Analysis of AAV Serotypes 1???9 Mediated Gene Expression and Tropism in Mice After Systemic Injection, Molecular Therapy, vol.16, issue.6, pp.1073-1080, 2008. ,
DOI : 10.1038/mt.2008.76
Statistical analysis of membrane proteome expression changes in Saccharomyces cerevisiae, 2339?2347. French summary des Fe?S pour les protéines cytosoliques et nucléaires (Beinert, 1997. ,
plusieurs fonctions ont été proposées : 1) donneur de fer lors de l'assemblage initiale du Fe?S (Ding and Clark, 2004. ,
2012); 2) protéine d'échafaudage des Fe?S alternative à ISCU, 2001. ,