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References

  • Adam A. C., Bornhovd C., Prokisch H., Neupert W. and Hell K. (2006) The Nfs1 interacting protein Isd11 has an essential role in Fe/S cluster biogenesis in mitochondria. EMBO J. 25, 174183.
  • Adamec J., Rusnak F., Owen W. G., Naylor S., Benson L. M., Gacy A. M. and Isaya G. (2000) Iron-dependent self-assembly of recombinant yeast frataxin: implications for Friedreich ataxia. Am. J. Hum. Genet. 67, 549562.
  • Adinolfi S., Trifuoggi M., Politou A. S., Martin S. and Pastore A. (2002) A structural approach to understanding the iron-binding properties of phylogenetically different frataxins. Hum. Mol. Genet. 11, 18651877.
  • Adinolfi S., Nair M., Politou A., Bayer E., Martin S., Temussi P. and Pastore A. (2004) The factors governing the thermal stability of frataxin orthologues: how to increase a protein's stability. Biochemistry 43, 65116518.
  • Adinolfi S., Iannuzzi C., Prischi F., Pastore C., Iametti S., Martin S., Bonomi F. and Pastore A. (2009) Bacterial frataxin CyaY is the gate keeper of iron sulfur formation catalysed by IscS. Nat. Struct. Mol. Biol. 16, 390396.
  • Adrover M., Esposito V., Martorell G., Pastore A. and Temussi P. A. (2010) Understanding cold denaturation: the case study of Yfh1. J. Am. Chem. Soc. 132, 1624016246.
  • Adrover M., Martorell G., Martin S., Urosev D., Daura X., Konarev P., Svergun D., Temussi P. A. and Pastore A. (2012) The role of hydration in protein stability: comparison of the cold and heat unfolded states of Yfh1. J. Mol. Biol. 417, 413424.
  • Alberts B. (2012) The end of “small science”? Science 337, 1583.
  • Aloria K., Schilke B., Andrew A. and Craig E. A. (2004) Iron-induced oligomerization of yeast frataxin homologue Yfh1 is dispensable in vivo. EMBO Rep. 5, 10961101.
  • Bandyopadhyay S., Chandramouli K. and Johnson M. K. (2008) Iron-sulfur cluster biosynthesis. Biochem. Soc. Trans. 36, 11121119.
  • Bencze K. Z., Yoon T., Bradley P. B., Cowan J. A. and Stemmler T. L. (2007) Human frataxin: iron structure and ferrochelatase binding surface. Chem. Commun. 14, 17981800.
  • Bou-Abdallah F., Adinolfi S., Pastore A., Laue T. M. and Chasteen N. D. (2004) Iron binding and oxidation properties of the bacterial frataxin cyay of escherichia coli. J. Mol. Biol. 341, 605615.
  • Branda S. S., Cavadini P., Adamec J., Kalousek F., Taroni F. and Isaya G. (1999) Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase. J. Biol. Chem. 274, 2276322769.
  • Bridwell-Rabb J., Iannuzzi C., Pastore A. and Barondeau D. P. (2012) Effector role reversal during evolution: the case of Frataxin in FeS cluster biosynthesis. Biochemistry 51, 25062514.
  • Bulteau A. L., O'Neill H. A., Kennedy M. C., Ikeda-Saito M., Isaya G. and Szweda L. I. (2004) Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity. Science 305, 242245.
  • Campanella A., Isaya G., ONeill H. A., Santambrogio P., Cozzi A., Arosio P. and Levi S. (2004) The expression of human mitochondrial ferritin rescues respiratory function in frataxin-deficient yeast. Hum. Mol. Genet. 13, 22792288.
  • Campuzano V., Montermini L., Molto M. D. et al. (1996) Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion. Science 271, 14231427.
  • Campuzano V., Montermini L., Lutz Y. et al. (1997) Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes. Hum. Mol. Genet. 6, 17711780.
  • Cavadini P., Adamec J., Taroni F., Gakh O. and Isaya G. (2000) Two-step processing of human frataxin by mitochondrial processing peptidase. Precursor and intermediate forms are cleaved at different rates. J. Biol. Chem. 275, 4146941475.
  • Cavadini P., O'Neill H. A., Benada O. and Isaya G. (2002) Assembly and iron- binding properties of human frataxin, the protein deficient in Friedreich ataxia. Hum. Mol. Genet. 11, 217227.
  • Chen O. S., Hemenway S. and Kaplan J. (2002) Inhibition of Fe-S cluster biosynthesis decreasesmitochondrial iron export: evidence that Yfh1p affects Fe-S cluster synthesis. Proc. Natl Acad. Sci. USA 99, 1232112326.
  • Cho S. J., Lee M. G., Yang J. K., Lee J. Y., Song H. K. and Suh S. W. (2000) Crystal structure of Escherichia coli CyaY protein reveals a previously unidentified fold for the evolutionarily conserved frataxin family. Proc. Natl Acad. Sci. USA 97, 89328927.
  • Colin F., Martelli A., Clemancey M., Latour J.M., Gambarelli S., Birck C., Page A., Puccio H. and Olagnier de Choudens (2013) Mammalian frataxin controls sulfur production and iron entry during de novo Fe4S4 cluster assembly. J. Am. Chem. Soc. 135, 733740.
  • Condò I., Ventura N., Malisan F., Rufini A., Tomassini B. and Testi R. (2007) In vivo maturation of human frataxin. Hum. Mol. Genet. 16, 15341540.
  • Cook J. D., Bencze K. Z., Jankovic A. D., Crater A. K., Busch C. N., Bradley P. B., Stemmler A. J., Spaller M. R. and Stemmler T. L. (2006) Monomeric yeast frataxin is an iron binding protein. Biochemistry 45, 77677777.
  • Cook J. D., Kondapalli K. C., Rawat S., Childs W. C., Murugesan Y., Dancis A. and Stemmler T. L. (2010) Molecular details of the yeast frataxin–Isu1 interaction during mitochondrial Fe–S cluster assembly. Biochemistry 49, 87568765.
  • Correia A., Adinolfi S., Pastore A. and Gomes C. (2006) Conformational stability of human frataxin and effect of Friedreich's ataxia related mutations on protein folding. Biochem. J. 398, 605611.
  • Correia A. R., Pastore C., Adinolfi S., Pastore A. and Gomes C. M. (2008) Implications of Friedreich's ataxia mutations in frataxin structure and dynamics. FEBS J. 275, 36803690.
  • Cossée M., Dürr A., Schmitt M. et al. (1999) Friedreich's ataxia: point mutations and clinical presentation of compound heterozygotes. Ann. Neurol. 45, 200206.
  • Dhe-Paganon S., Shigeta R., Chi Y. I., Ristow M. and Shoelson S. E. (2000) Crystal structure of human frataxin. J. Biol. Chem. 275, 3075330756.
  • Dolezal P., Dancis A., Lesuisse E., Sutak R., Hrdy I., Embley T. M. and Tachezy J. (2007) Frataxin, a conserved mitochondrial protein in the hydrogenosome of trichomonas vaginalis. Eukaryot. Cell 6, 14311438.
  • Duby G., Foury F., Ramazzotti A., Herrmann J. and Lutz T. (2002) A non-essential function for yeast frataxin in iron-sulfur cluster assembly. Hum. Mol. Genet. 11, 26352643.
  • Foury F. and Cazzalini O. (1997) Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria. FEBS Lett. 411, 373377.
  • Foury F., Pastore A. and Trincal M. (2007) Acidic residues of yeast frataxin play an essential role in Fe-S cluster assembly. EMBO Rep. 8, 194199.
  • Gakh O., Adamec J., Gacy A. M., Twesten R. D., Owen W. G. and Isaya G. (2002) Physical evidence that yeast frataxin is an iron storage protein. Biochemistry 41, 67986804.
  • Gakh O., Park S., Liu G., Macomber L., Imlay J. A., Ferreira G. C. and Isaya G. (2006) Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity. Hum. Mol. Genet. 15, 467479.
  • Gakh O., Smith D. Y. IV and Isaya G. (2008) Assembly of the iron-binding protein Frataxin in Saccharomyces cerevisiae responds to dynamic changes in mitochondrial iron influx and stress level. J. Biol. Chem. 283, 3150031510.
  • Gakh O., Bedekovics T., Duncan S. F., Smith D. Y. IV, Berkholz D. S. and Isaya G. (2010) Normal and Friedreich ataxia cells express different isoforms of frataxin with complementary roles in iron-sulfur cluster assembly. J. Biol. Chem. 285, 3848638501.
  • Gerber J., Muhlenhoff U. and Lill R. (2003) An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1. EMBO Rep. 4, 906911.
  • Gibson T. J., Koonin E. V., Musco G., Pastore A. and Bork P. (1996) Friedreich's ataxia protein: phylogenetic evidence for mitochondrial dysfunction. Trends Neurosci. 19, 465468.
  • Gonzalez-Cabo P., Vazquez-Manrique R. P., Garcia-Gimeno M. A., Sanz P. and Palau F. (2005) Frataxin interacts functionally with mitochondrial electron transport chain proteins. Hum. Mol. Genet. 14, 20912098.
  • He Y., Alam S. L., Proteasa S. V., Zhang Y., Lesuisse E., Dancis A. and Stemmler T. L. (2004) Yeast frataxin solution structure, iron binding, and ferrochelatase interaction. Biochemistry 43, 1625416262.
  • Huang J., Dizin E. and Cowan J. A. (2008) Mapping iron binding sites on human frataxin: implications for cluster assembly on the ISU Fe–S cluster scaffold protein. J. Biol. Inorg. Chem. 13, 825836.
  • Huynen M. A., Snel B., Bork P. and Gibson T. J. (2001) The phylogenetic distribution of frataxin indicates a role in iron-sulfur cluster protein assembly. Hum. Mol. Genet. 10, 24632468.
  • Iannuzzi C., Adinolfi S., Howes B. D., Garcia-Serres R., Clémancey M., Latour J. M., Smulevich G. and Pastore A. (2011) The role of CyaY in iron sulfur cluster assembly on the E. coli IscU scaffold protein. PLoS ONE 6, e21992.
  • Karlberg T., Schagerlof U., Gakh O., Park S., Ryde U., Lindahl M., Leath K., Garman E., Isaya G. and Al-Karadaghi S. (2006) The structures of frataxin oligomers reveal the mechanism for the delivery and detoxification of iron. Structure 14, 15351546.
  • Koutnikova H., Campuzano V., Foury F., Dolle P., Cazzalini O. and Koenig M. (1997) Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin. Nat. Genet. 16, 345351.
  • Koutnikova H., Campuzano V. and Koenig M. (1998) Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase. Hum. Mol. Genet. 7, 14851489.
  • Labuda M., Poirier J. and Pandolfo M. (1999) A missense mutation (W155R) in an American patient with Friedreich ataxia. Hum. Mutat. 13, 506507.
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  • Layer G., Ollagnier de Choudens S., Sanakis Y. and Fontecave M. (2006) Iron-sulfur cluster biosynthesis: characterization of Escherichia coli cyay as an iron donor for the assembly of [2Fe-2S] clusters in the scaffold ISCU. J. Biol. Chem. 281, 1625616263.
  • Leidgens S., De Smet S. and Foury F. (2010) Frataxin interacts with Isu1 through a conserved tryptophan in its beta-sheet. Hum. Mol. Genet. 19, 276286.
  • Lesuisse E., Santos R., Matzanke B. F., Knight S. A., Camadro J. M. and Dancis A. (2003) Iron use for heme synthesis is under control of the yeast frataxin homologue (Yfh1). Hum. Mol. Genet. 12, 879889.
  • Levi S., Corsi B., Bosisio M., Invernizzi R., Volz A., Sanford D., Arosio P. and Drysdale J. (2001) A human mitochondrial ferritin encoded by an intronless gene. J. Biol. Chem. 276, 2443724440.
  • Martelli A., Napierala M. and Puccio H. (2012) Understanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular models. Dis. Model Mech. 5, 165176.
  • Mühlenhoff U., Richhardt N., Gerber J. and Lill R. (2002) Characterization of iron-sulfur protein assembly in isolated mitochondria. A requirement for ATP, NADH, and reduced iron. J. Biol. Chem. 277, 2981029816.
  • Musco G., Stier G., Kolmerer B., Adinolfi S., Martin S., Frenkiel T., Gibson T. and Pastore A. (2000) Towards a structural understanding of Friedreich's ataxia: the solution structure of frataxin.Structure. Fold Des. 8, 695707.
  • Nair M., Adinolfi S., Pastore C., Kelly G., Temussi P. and Pastore A. (2004) Solution structure of the bacterial frataxin ortholog, CyaY: mapping the iron binding sites. Structure 12, 20372048.
  • Nichol H., Gakh O., O'Neill H. A., Pickering I. J., Isaya G. and George G. N. (2003) Structure of frataxin iron cores: an X-ray absorption spectroscopic study. Biochemistry 42, 59715976.
  • O'Neill H. A., Gakh O., Park S., Cui J., Mooney S. M., Sampson M., Ferreira G. C. and Isaya G. (2005) Assembly of human frataxin is a mechanism for detoxifying redox-active iron. Biochemistry 44, 537545.
  • Pandey A., Yoon H., Lyver E. R., Dancis A. and Pain D. (2011) Isd11p protein activates the mitochondrial cysteine desulfurase Nfs1p protein. J. Biol. Chem. 286, 3824238252.
  • Pandolfo M. (2009) Friedreich ataxia: the clinical picture. J. Neurol. 256, 38.
  • Park S., Gakh O., O'Neill H. A., Mangravita A., Nichol H., Ferreira G. C. and Isaya G. (2003) Yeast frataxin sequentially chaperones and stores iron by coupling protein assembly with iron oxidation. J. Biol. Chem. 278, 3134031351.
  • Pastore A., Martin S. R., Politou A., Kondapalli K. C., Stemmler T. and Temussi P. A. (2007) Unbiased cold denaturation: low and high temperature unfolding of Yeast frataxin under physiological conditions. J. Am. Chem. Soc. 129, 53745375.
  • Prischi F., Giannini C., Adinolfi S. and Pastore A. (2009) The N-terminus of mature human frataxin is intrinsically unfolded. FEBS J. 276, 66696676.
  • Prischi F., Konarev P. V., Iannuzzi C., Pastore C., Adinolfi S., Martin S. R., Svergun D. I. and Pastore A. (2010) Structural bases for the interaction of frataxin with the central components of iron-sulfur cluster assembly. Nat. Commun. 1, 95.
  • Puccio H., Simon D., Cossée M., Criqui-Filipe P., Tiziano F., Melki J., Hindelang C., Matyas R., Rustin P. and Koenig M. (2001) Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits. Nat. Genet. 27, 181186.
  • Py B., Moreau P. L. and Barras F. (2011) Fe-S clusters, fragile sentinels of the cell. Curr. Opin. Microbiol. 14, 218223.
  • Ramazzotti A., Vanmansart V. and Foury F. (2004) Mitochondrial functional interactions between frataxin and Isu1p, the iron-sulfur cluster scaffold protein, in Saccharomyces cerevisiae. FEBS Lett. 557, 215220.
  • Sazanov L. A. and Hinchliffe P. (2006) Structure of the hydrophilic domain of respiratory complex I from Thermus thermophilus. Science 311, 14301436.
  • Schmucker S., Argentini M., Carelle-Calmels N., Martelli A. and Puccio H. (2008) The in vivo mitochondrial two-step maturation of human frataxin. Hum. Mol. Genet. 17, 35213531.
  • Schmucker S., Martelli A., Colin F., Page A., Wattenhofer-Donzé M., Reutenauer L. and Puccio H. (2011) Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex. PLoS ONE 6, e16199.
  • Schoenfeld R. A., Napoli E., Wong A. et al. (2005) Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian cells. Hum. Mol. Genet. 14, 37873799.
  • Seguin A., Bayot A., Dancis A., Rogowska-Wrzesinska A., Auchère F., Camadro J. M., Bulteau A. L. and Lesuisse E. (2009) Overexpression of the yeast frataxin homolog (Yfh1): contrasting effects on iron-sulfur cluster assembly, heme synthesis and resistance to oxidative stress. Mitochondrion 9, 130138.
  • Shan Y., Napoli E. and Cortopassi G. (2007) Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones. Hum. Mol. Genet. 16, 929941.
  • Shi R., Proteau A., Villarroya M., Moukadiri I., Zhang L., Trempe J. F., Matte A., Armengod M. E. and Cygler M. (2010) Structural basis for Fe-S cluster assembly and tRNA thiolation mediated by IscS protein-protein interactions. PLoS Biol. 8(), e1000354.
  • Söderberg C. A., Shkumatov A. V., Rajan S., Gakh O., Svergun D. I., Isaya G. and Al-Karadaghi S. (2011) Oligomerization propensity and flexibility of yeast frataxin studied by X-ray crystallography and small-angle X-ray scattering. J. Mol. Biol. 414, 783797.
  • Taketani S. (2005) Acquisition, mobilization and utilization of cellular iron and heme: endless findings and growing evidence of tight regulation. Tohoku J. Exp. Med. 205, 297318.
  • Tsai C. I. and Barondeau D. P. (2010) Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex. Biochemistry 49, 91324139.
  • Vaubel R. A. and Isaya G. (2012) Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia. Mol. Cell. Neurosci. [Epub ahead of publication].
  • Wiedemann N., Urzica E., Guiard B. et al. (2006) Essential role of Isd11 in mitochondrial iron-sulfur cluster synthesis on Isu scaffold proteins. EMBO J. 25, 184195.
  • Ye H. and Rouault T. A. (2010) Human iron-sulfur cluster assembly, cellular iron homeostasis, and disease. Biochemistry 49, 49454956.
  • Yoon T. and Cowan J. A. (2003) Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins. J. Am. Chem. Soc. 125, 60786084.
  • Yoon T. and Cowan J. A. (2004) Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis. J. Biol. Chem. 279, 2594325946.
  • Yoon T., Dizin E. and Cowan J. A. (2007) N-terminal iron-mediated self-cleavage of human frataxin: regulation of iron binding and complex formation with target proteins. J. Biol. Inorg. Chem. 12, 535542.
  • Zanella I., Derosas M., Corrado M., Cocco E., Cavadini P., Biasiotto G., Poli M., Verardi R. and Arosio P. (2008) The effects of frataxin silencing in HeLa cells are rescued by the expression of human mitochondrial ferritin. Biochim. Biophys. Acta 1782, 9098.