YFH1

Summary

Gene Symbol: YFH1
Description: ferroxidase
Alias: ferroxidase
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Auchère F, Santos R, Planamente S, Lesuisse E, Camadro J. Glutathione-dependent redox status of frataxin-deficient cells in a yeast model of Friedreich's ataxia. Hum Mol Genet. 2008;17:2790-802 pubmed publisher
    ..In conclusion, our findings provide evidence for an impairment of glutathione homeostasis in a yeast model of Friedreich's ataxia and identify glutathione as a valuable indicator of the redox status of frataxin-deficient cells. ..
  2. Wilson R, Roof D. Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue. Nat Genet. 1997;16:352-7 pubmed
    ..We show that the yeast frataxin homologue, which we have named YFH1, localizes to mitochondria and is required to maintain mitochondrial DNA...
  3. Yoon H, Golla R, Lesuisse E, Pain J, Donald J, Lyver E, et al. Mutation in the Fe-S scaffold protein Isu bypasses frataxin deletion. Biochem J. 2012;441:473-80 pubmed publisher
    ..methionine to isoleucine) at position 107 in the mature form of Isu1 restored many deficient functions in ?yfh1 or frataxin-depleted yeast cells...
  4. Lefevre S, Sliwa D, Auchère F, Brossas C, Ruckenstuhl C, Boggetto N, et al. The yeast metacaspase is implicated in oxidative stress response in frataxin-deficient cells. FEBS Lett. 2012;586:143-8 pubmed publisher
    ..In addition, our results suggest that metacaspase is associated with mitochondrial respiration, intracellular redox control and genomic stability. ..
  5. Zhang Y, Lyver E, Knight S, Lesuisse E, Dancis A. Frataxin and mitochondrial carrier proteins, Mrs3p and Mrs4p, cooperate in providing iron for heme synthesis. J Biol Chem. 2005;280:19794-807 pubmed
    ..Deletion of the yeast frataxin homolog (YFH1) was combined with deletions of MRS3 and MRS4, mitochondrial carrier proteins implicated in iron homeostasis...
  6. Babcock M, de Silva D, Oaks R, Davis Kaplan S, Jiralerspong S, Montermini L, et al. Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin. Science. 1997;276:1709-12 pubmed
    ..The yeast gene (yeast frataxin homolog, YFH1) encodes a mitochondrial protein involved in iron homeostasis and respiratory function...
  7. Lesuisse E, Santos R, Matzanke B, Knight S, Camadro J, Dancis A. Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1). Hum Mol Genet. 2003;12:879-89 pubmed
    The YFH1 gene is the yeast homologue of the human FRDA gene, which encodes the frataxin protein. Saccharomyces cerevisiae cells lacking the YFH1 gene showed very low cytochrome content...
  8. Seguin A, Bayot A, Dancis A, Rogowska Wrzesinska A, Auchère F, Camadro J, et al. Overexpression of the yeast frataxin homolog (Yfh1): contrasting effects on iron-sulfur cluster assembly, heme synthesis and resistance to oxidative stress. Mitochondrion. 2009;9:130-8 pubmed publisher
    ..of Friedreich's ataxia to study the physiological consequences of modulating the expression of the frataxin gene (YFH1)...
  9. Adrover M, Martorell G, Martin S, Urosev D, Konarev P, Svergun D, et al. The role of hydration in protein stability: comparison of the cold and heat unfolded states of Yfh1. J Mol Biol. 2012;417:413-24 pubmed publisher
    ..have combined experimental and computational approaches to compare the high- and low-temperature unfolded states of Yfh1, a natural protein that, at physiologic pH, undergoes cold and heat denaturation around 0 °C and 40 °C without ..

More Information

Publications62

  1. Gakh O, Adamec J, Gacy A, Twesten R, Owen W, Isaya G. Physical evidence that yeast frataxin is an iron storage protein. Biochemistry. 2002;41:6798-804 pubmed
    ..These findings support the hypothesis that frataxin is an iron storage protein, which could explain the mitochondrial iron accumulation and oxidative damage associated with frataxin defects in yeast, mouse, and humans. ..
  2. Sliwa D, Dairou J, Camadro J, Santos R. Inactivation of mitochondrial aspartate aminotransferase contributes to the respiratory deficit of yeast frataxin-deficient cells. Biochem J. 2012;441:945-53 pubmed publisher
    ..we show that the malate-aspartate NADH shuttle is impaired in Saccharomyces cerevisiae frataxin-deficient cells (?yfh1) due to decreased activity of cytosolic and mitochondrial isoforms of malate dehydrogenase and to complete ..
  3. Moreno Cermeño A, Alsina D, Cabiscol E, Tamarit J, Ros J. Metabolic remodeling in frataxin-deficient yeast is mediated by Cth2 and Adr1. Biochim Biophys Acta. 2013;1833:3326-3337 pubmed publisher
    ..We performed transcriptomic and proteomic analyses of conditional Yeast Frataxin Homologue (Yfh1) mutants (tetO7-YFH1) to investigate metabolic remodeling upon Yfh1 depletion...
  4. Schagerlöf U, Elmlund H, Gakh O, Nordlund G, Hebert H, Lindahl M, et al. Structural basis of the iron storage function of frataxin from single-particle reconstruction of the iron-loaded oligomer. Biochemistry. 2008;47:4948-54 pubmed publisher
    ..These include the overall organization of the oligomers, the way they are stabilized, and the mechanisms of iron core nucleation. ..
  5. He Y, Alam S, Proteasa S, Zhang Y, Lesuisse E, Dancis A, et al. Yeast frataxin solution structure, iron binding, and ferrochelatase interaction. Biochemistry. 2004;43:16254-62 pubmed
    ..NMR spectroscopy was further used to identify the intermolecular binding interface between ferrochelatase and frataxin. Ferrochelatase appears to bind to frataxin's helical plane in a manner that includes its iron-binding interface. ..
  6. Lefevre S, Sliwa D, Rustin P, Camadro J, Santos R. Oxidative stress induces mitochondrial fragmentation in frataxin-deficient cells. Biochem Biophys Res Commun. 2012;418:336-41 pubmed publisher
    ..Yeast cells lacking frataxin (?yfh1 mutant) showed an increased proportion of fragmented mitochondria compared to wild-type...
  7. Adamec J, Rusnak F, Owen W, Naylor S, Benson L, Gacy A, et al. Iron-dependent self-assembly of recombinant yeast frataxin: implications for Friedreich ataxia. Am J Hum Genet. 2000;67:549-62 pubmed
    ..We propose that iron-dependent self-assembly of recombinant mYfh1p reflects a physiological role for frataxin in mitochondrial iron sequestration and bioavailability. ..
  8. Chen O, Kaplan J. CCC1 suppresses mitochondrial damage in the yeast model of Friedreich's ataxia by limiting mitochondrial iron accumulation. J Biol Chem. 2000;275:7626-32 pubmed
    Deletion of YFH1 in Saccharomyces cerevisiae leads to a loss of respiratory competence due to excessive mitochondrial iron accumulation...
  9. Zhang Y, Lyver E, Knight S, Pain D, Lesuisse E, Dancis A. Mrs3p, Mrs4p, and frataxin provide iron for Fe-S cluster synthesis in mitochondria. J Biol Chem. 2006;281:22493-502 pubmed
    ..These findings show that Mrs3/4p transports iron into mitochondria, whereas frataxin makes iron already within mitochondria available for Fe-S cluster synthesis. ..
  10. Ranatunga W, Gakh O, Galeano B, Smith D, Söderberg C, Al Karadaghi S, et al. Architecture of the Yeast Mitochondrial Iron-Sulfur Cluster Assembly Machinery: THE SUB-COMPLEX FORMED BY THE IRON DONOR, Yfh1 PROTEIN, AND THE SCAFFOLD, Isu1 PROTEIN. J Biol Chem. 2016;291:10378-98 pubmed publisher
    ..We reconstituted a stable, functional complex consisting of the iron donor, Yfh1 (yeast frataxin homologue 1), and the Fe-S cluster scaffold, Isu1, with 1:1 stoichiometry, [Yfh1]24·[Isu1]24 ..
  11. Wang T, Craig E. Binding of yeast frataxin to the scaffold for Fe-S cluster biogenesis, Isu. J Biol Chem. 2008;283:12674-9 pubmed publisher
    ..support the idea that the surface of the beta-sheet, adjacent to the acidic, iron binding ridge, is important for interaction of Yfh1 with the Fe-S cluster scaffold and point to a critical role for frataxin in Fe-S cluster biogenesis.
  12. Gakh O, Smith D, Isaya G. Assembly of the iron-binding protein frataxin in Saccharomyces cerevisiae responds to dynamic changes in mitochondrial iron influx and stress level. J Biol Chem. 2008;283:31500-10 pubmed publisher
    ..In Saccharomyces cerevisiae, only monomeric yeast frataxin (Yfh1) was detected in unstressed cells when mitochondrial iron uptake was maintained at a steady, low nanomolar level...
  13. Branda S, Cavadini P, Adamec J, Kalousek F, Taroni F, Isaya G. Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase. J Biol Chem. 1999;274:22763-9 pubmed
    ..MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to mature size protein. In this way, MPP could influence frataxin function and indirectly affect mitochondrial iron homeostasis. ..
  14. Chen O, Hemenway S, Kaplan J. Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: evidence that Yfh1p affects Fe-S cluster synthesis. Proc Natl Acad Sci U S A. 2002;99:12321-6 pubmed
    ..These results demonstrate a direct role of Yfh1p in the formation of Fe-S clusters and indicate that mitochondrial iron export requires Fe-S cluster biosynthesis. ..
  15. Chen O, Hemenway S, Kaplan J. Genetic analysis of iron citrate toxicity in yeast: implications for mammalian iron homeostasis. Proc Natl Acad Sci U S A. 2002;99:16922-7 pubmed
    Deletion of the yeast homologue of frataxin, YFH1, results in mitochondrial iron accumulation and respiratory deficiency (petite formation)...
  16. Yoon H, Knight S, Pandey A, Pain J, Turkarslan S, Pain D, et al. Turning Saccharomyces cerevisiae into a Frataxin-Independent Organism. PLoS Genet. 2015;11:e1005135 pubmed publisher
    Frataxin (Yfh1 in yeast) is a conserved protein and deficiency leads to the neurodegenerative disease Friedreich's ataxia...
  17. Park S, Gakh O, O Neill H, Mangravita A, Nichol H, Ferreira G, et al. Yeast frataxin sequentially chaperones and stores iron by coupling protein assembly with iron oxidation. J Biol Chem. 2003;278:31340-51 pubmed
    ..Here we show that mYfh1p assembly is driven by two sequential iron oxidation reactions: A ferroxidase reaction catalyzed by mYfh1p induces the first assembly step (alpha --> alpha3), followed by a slower ..
  18. Muhlenhoff U, Gerber J, Richhardt N, Lill R. Components involved in assembly and dislocation of iron-sulfur clusters on the scaffold protein Isu1p. EMBO J. 2003;22:4815-25 pubmed
    ..We propose a model that dissects Fe/S cluster biogenesis into two major steps and assigns its central components to one of these two steps. ..
  19. Vaubel R, Rustin P, Isaya G. Mutations in the dimer interface of dihydrolipoamide dehydrogenase promote site-specific oxidative damages in yeast and human cells. J Biol Chem. 2011;286:40232-45 pubmed publisher
    ..We conclude that the cryptic activities of DLD promote oxidative damage to neighboring molecules and thus contribute to the clinical severity of DLD mutations. ..
  20. Branda S, Yang Z, Chew A, Isaya G. Mitochondrial intermediate peptidase and the yeast frataxin homolog together maintain mitochondrial iron homeostasis in Saccharomyces cerevisiae. Hum Mol Genet. 1999;8:1099-110 pubmed
    ..In Saccharomyces cerevisiae, lack of the yeast frataxin homolog ( YFH1 gene, Yfh1p polypeptide) results in mitochondrial iron accumulation, suggesting that frataxin is required for ..
  21. Foury F, Pastore A, Trincal M. Acidic residues of yeast frataxin have an essential role in Fe-S cluster assembly. EMBO Rep. 2007;8:194-9 pubmed
    ..It has been reported that alanine substitution of residues in an acidic ridge of yeast frataxin (Yfh1) elicits loss of iron binding in vitro but has no effect on Fe-S cluster synthesis in vivo...
  22. Bulteau A, O Neill H, Kennedy M, Ikeda Saito M, Isaya G, Szweda L. Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity. Science. 2004;305:242-5 pubmed
    ..Thus, frataxin is an iron chaperone protein that protects the aconitase [4Fe-4S]2+ cluster from disassembly and promotes enzyme reactivation. ..
  23. Bedekovics T, Li H, Gajdos G, Isaya G. Leucine biosynthesis regulates cytoplasmic iron-sulfur enzyme biogenesis in an Atm1p-independent manner. J Biol Chem. 2011;286:40878-88 pubmed publisher
    ..Thus, still largely unknown transcriptional and post-transcriptional mechanisms control ISC distribution between mitochondria and other cellular compartments...
  24. Yoon H, Zhang Y, Pain J, Lyver E, Lesuisse E, Pain D, et al. Rim2, a pyrimidine nucleotide exchanger, is needed for iron utilization in mitochondria. Biochem J. 2011;440:137-46 pubmed publisher
    ..Several genes were identified, including an isolate mutated for Yfh1, the yeast frataxin homologue...
  25. Moreno Cermeño A, Obis E, Belli G, Cabiscol E, Ros J, Tamarit J. Frataxin depletion in yeast triggers up-regulation of iron transport systems before affecting iron-sulfur enzyme activities. J Biol Chem. 2010;285:41653-64 pubmed publisher
    ..Using a yeast model of conditional expression of the frataxin homologue YFH1, we analyzed the primary effects of YFH1 depletion...
  26. Lane D, Richardson D. Frataxin, a molecule of mystery: trading stability for function in its iron-binding site. Biochem J. 2010;426:e1-3 pubmed publisher
    ..investigate the relationship between structure and function at the putative iron-binding site of Yfh1 (yeast frataxin)...
  27. Söderberg C, Rajan S, Shkumatov A, Gakh O, Schaefer S, Ahlgren E, et al. The molecular basis of iron-induced oligomerization of frataxin and the role of the ferroxidation reaction in oligomerization. J Biol Chem. 2013;288:8156-67 pubmed publisher
    ..we describe the solution structure of the oligomers formed during the iron-dependent assembly of yeast (Yfh1) and Escherichia coli (CyaY) frataxin...
  28. Aloria K, Schilke B, Andrew A, Craig E. Iron-induced oligomerization of yeast frataxin homologue Yfh1 is dispensable in vivo. EMBO Rep. 2004;5:1096-101 pubmed
    ..The in vivo role of frataxin is under debate. Frataxin, as well as its yeast homologue Yfh1, binds multiple iron atoms as an oligomer and has been proposed to function as a crucial iron-storage protein...
  29. Espinosa Y, Grigera J, Caffarena E. Essential dynamics of the cold denaturation: pressure and temperature effects in yeast frataxin. Proteins. 2017;85:125-136 pubmed publisher
    ..We have studied the process of cold denaturation using molecular dynamics simulations of the frataxin system Yfh1, which has a dynamic experimental characterization of unfolding at low and high temperatures...
  30. Li L, Miao R, Bertram S, Jia X, Ward D, Kaplan J. A role for iron-sulfur clusters in the regulation of transcription factor Yap5-dependent high iron transcriptional responses in yeast. J Biol Chem. 2012;287:35709-21 pubmed publisher
    ..that mutation or reduced expression of other genes involved in mitochondrial iron-sulfur cluster synthesis (YFH1, ISU1) prevented induction of the Yap5 response...
  31. Correia A, Wang T, Craig E, Gomes C. Iron-binding activity in yeast frataxin entails a trade off with stability in the alpha1/beta1 acidic ridge region. Biochem J. 2010;426:197-203 pubmed publisher
    ..Alterations on the conserved beta-sheet residues have only a modest impact on the protein stability, highlighting the functional importance of residues 122-124...
  32. Irazusta V, Moreno Cermeño A, Cabiscol E, Ros J, Tamarit J. Major targets of iron-induced protein oxidative damage in frataxin-deficient yeasts are magnesium-binding proteins. Free Radic Biol Med. 2008;44:1712-23 pubmed publisher
    ..In yeast cells lacking the frataxin ortholog YFH1, we have identified a set of 14 carbonylated proteins, which include mitochondrial ATP synthase, phosphoglycerate ..
  33. Lefevre S, Brossas C, Auchère F, Boggetto N, Camadro J, Santos R. Apn1 AP-endonuclease is essential for the repair of oxidatively damaged DNA bases in yeast frataxin-deficient cells. Hum Mol Genet. 2012;21:4060-72 pubmed publisher
    ..we present evidence that one of the pleiotropic effects of oxidative stress in frataxin-deficient yeast cells (?yfh1 mutant) is damage to nuclear DNA and that repair requires the Apn1 AP-endonuclease of the base excision repair ..
  34. Li H, Gakh O, Smith D, Isaya G. Oligomeric yeast frataxin drives assembly of core machinery for mitochondrial iron-sulfur cluster synthesis. J Biol Chem. 2009;284:21971-80 pubmed publisher
    ..Analysis of highly conserved components of the yeast ISC assembly machinery shows that the iron-chaperone, Yfh1, and the sulfur-donor complex, Nfs1-Isd11, directly bind to each other...
  35. Gerber J, Muhlenhoff U, Lill R. An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1. EMBO Rep. 2003;4:906-11 pubmed
    ..The function of frataxin is unclear, although recent studies have suggested a function of frataxin (yeast Yfh1) in iron/sulphur (Fe/S) protein biogenesis...
  36. Söderberg C, Gillam M, Ahlgren E, Hunter G, Gakh O, Isaya G, et al. The Structure of the Complex between Yeast Frataxin and Ferrochelatase: CHARACTERIZATION AND PRE-STEADY STATE REACTION OF FERROUS IRON DELIVERY AND HEME SYNTHESIS. J Biol Chem. 2016;291:11887-98 pubmed publisher
    ..and single-particle reconstruction from negatively stained electron microscopic images were used to verify the Yfh1-ferrochelatase interactions...
  37. Chen O, Kaplan J. YFH1-mediated iron homeostasis is independent of mitochondrial respiration. FEBS Lett. 2001;509:131-4 pubmed
    The human gene frataxin and its yeast homolog YFH1 affect mitochondrial function. Deficits in frataxin result in Friedreich ataxia, while deletion of YFH1 results in respiratory incompetence...
  38. Seguin A, Santos R, Pain D, Dancis A, Camadro J, Lesuisse E. Co-precipitation of phosphate and iron limits mitochondrial phosphate availability in Saccharomyces cerevisiae lacking the yeast frataxin homologue (YFH1). J Biol Chem. 2011;286:6071-9 pubmed publisher
    Saccharomyces cerevisiae cells lacking the yeast frataxin homologue (?yfh1) accumulate iron in the mitochondria in the form of nanoparticles of ferric phosphate...
  39. Pandey A, Gordon D, Pain J, Stemmler T, Dancis A, Pain D. Frataxin directly stimulates mitochondrial cysteine desulfurase by exposing substrate-binding sites, and a mutant Fe-S cluster scaffold protein with frataxin-bypassing ability acts similarly. J Biol Chem. 2013;288:36773-86 pubmed publisher
    ..Using purified proteins and isolated mitochondria, we show here that the yeast frataxin homolog (Yfh1) directly and specifically stimulates cysteine binding to Nfs1 by exposing substrate-binding sites...
  40. Kim J, Sedlak M, Gao Q, Riley C, Regnier F, Adamec J. Dynamics of protein damage in yeast frataxin mutant exposed to oxidative stress. OMICS. 2010;14:689-99 pubmed publisher
    ..Predicted 3D structures of peroxiredoxin (TSA1) and thioredoxin II (TRX2) revealed close proximity of all oxidized amino acid residues to the protein active sites. ..
  41. Leidgens S, De Smet S, Foury F. Frataxin interacts with Isu1 through a conserved tryptophan in its beta-sheet. Hum Mol Genet. 2010;19:276-86 pubmed publisher
    ..It has been shown that Yfh1, the yeast frataxin homologue, interacts functionally and physically with Isu1, the scaffold protein on which the ..
  42. Park S, Gakh O, Mooney S, Isaya G. The ferroxidase activity of yeast frataxin. J Biol Chem. 2002;277:38589-95 pubmed
    ..A ferroxidase reaction with a stoichiometry of 2 Fe(II)/O(2) is detected at Fe(II)/mYfh1p ratios of < or = 0.5...
  43. Gonzalez Cabo P, Vázquez Manrique R, García Gimeno M, Sanz P, Palau F. Frataxin interacts functionally with mitochondrial electron transport chain proteins. Hum Mol Genet. 2005;14:2091-8 pubmed
    ..Genetic synthetic interaction experiments confirmed a functional relationship between YFH1 and succinate dehydrogenase genes SDH1 and SDH2...
  44. Cook J, Kondapalli K, Rawat S, Childs W, Murugesan Y, Dancis A, et al. Molecular details of the yeast frataxin-Isu1 interaction during mitochondrial Fe-S cluster assembly. Biochemistry. 2010;49:8756-65 pubmed publisher
    ..Here we provide molecular details of how yeast frataxin (Yfh1) interacts with Isu1 as a structural module to improve our understanding of the multiprotein complex assembly that ..
  45. Seguin A, Sutak R, Bulteau A, Garcia Serres R, Oddou J, Lefevre S, et al. Evidence that yeast frataxin is not an iron storage protein in vivo. Biochim Biophys Acta. 2010;1802:531-8 pubmed publisher
    ..Our results do not privilege the hypothesis of Yfh1p being an iron storage protein in vivo. ..
  46. Yoon H, Knight S, Pandey A, Pain J, Zhang Y, Pain D, et al. Frataxin-bypassing Isu1: characterization of the bypass activity in cells and mitochondria. Biochem J. 2014;459:71-81 pubmed publisher
    ..The persulfide-forming activity of the cysteine desulfurase was diminished in the frataxin deletion (∆yfh1 ISU1) and was improved by expression of the bypass Isu1 (∆yfh1 M107I ISU1)...
  47. Söderberg C, Shkumatov A, Rajan S, Gakh O, Svergun D, Isaya G, et al. Oligomerization propensity and flexibility of yeast frataxin studied by X-ray crystallography and small-angle X-ray scattering. J Mol Biol. 2011;414:783-97 pubmed publisher
    ..They also indicate that different mechanisms may control oligomer stability and oligomerization in vivo. ..
  48. Kucej M, Foury F. Iron toxicity protection by truncated Ras2 GTPase in yeast strain lacking frataxin. Biochem Biophys Res Commun. 2003;310:986-91 pubmed
    Yeast strain deleted for the YFH1 gene, which encodes the orthologue of human frataxin, accumulates iron in mitochondria, constitutively activates the high-affinity iron import system in the plasma membrane, and is sensitive to high iron ..
  49. Irazusta V, Cabiscol E, Reverter Branchat G, Ros J, Tamarit J. Manganese is the link between frataxin and iron-sulfur deficiency in the yeast model of Friedreich ataxia. J Biol Chem. 2006;281:12227-32 pubmed
  50. Manicki M, Majewska J, Ciesielski S, Schilke B, Blenska A, Kominek J, et al. Overlapping binding sites of the frataxin homologue assembly factor and the heat shock protein 70 transfer factor on the Isu iron-sulfur cluster scaffold protein. J Biol Chem. 2014;289:30268-78 pubmed publisher
    ..of Isu with both Nfs1, the cysteine desulfurase serving as a sulfur donor, and the yeast frataxin homolog (Yfh1) serving as a regulator of desulfurase activity and/or iron donor...
  51. Ramazzotti A, Vanmansart V, Foury F. Mitochondrial functional interactions between frataxin and Isu1p, the iron-sulfur cluster scaffold protein, in Saccharomyces cerevisiae. FEBS Lett. 2004;557:215-20 pubmed
    ..A synthetic lethal screen on high iron media with the mild G107D yfh1 mutant has specifically identified Isu1p...
  52. Cook J, Bencze K, Jankovic A, Crater A, Busch C, Bradley P, et al. Monomeric yeast frataxin is an iron-binding protein. Biochemistry. 2006;45:7767-77 pubmed
    ..On the basis of our results, we have developed a model for how we believe yeast frataxin interacts with iron. ..
  53. Gakh O, Park S, Liu G, Macomber L, Imlay J, Ferreira G, et al. Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity. Hum Mol Genet. 2006;15:467-79 pubmed