| In the absence of Yfh1, Isu1 was found to exist in two forms, one mostly monomeric with limited tendency to dimerize, distinct structural features suggest that in physiological conditions the zinc-regulated abundance of monomeric vs. oligomeric Isu1 yields [Yfh1].[Isu1] complexes with different Isu1 configurations that afford unique functional properties for Fe-S cluster assembly and delivery. | Zinc and the iron donor frataxin regulate oligomerization of the scaffold protein to form new Fe-S cluster assembly centers.
Galeano BK, Ranatunga W, Gakh O, Smith DY, Thompson JR, Isaya G., Free PMC Article | 07/21/2018 |
| YFH1 is spontaneously unfolded at low and high temperatures. | Essential dynamics of the cold denaturation: pressure and temperature effects in yeast frataxin.
Espinosa YR, Grigera JR, Caffarena ER. | 07/29/2017 |
| Mutual exclusivity of cluster assembly factor (Nfs1/Yfh1) and cluster transfer factor (Jac1/Ssq1) binding to Isu has functional consequences for the transition from the assembly process to the transfer process during biogenesis of FeS cluster proteins. | 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.
Manicki M, Majewska J, Ciesielski S, Schilke B, Blenska A, Kominek J, Marszalek J, Craig EA, Dutkiewicz R., Free PMC Article | 01/3/2015 |
| The data presented here show that the Isu1 suppressor mimics the frataxin effects on Nfs1, explaining the bypassing activity. | 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.
Pandey A, Gordon DM, Pain J, Stemmler TL, Dancis A, Pain D., Free PMC Article | 02/22/2014 |
| At an iron-to-protein ratio of 2, the initially monomeric Yfh1 is converted to a trimeric form in solution. | The molecular basis of iron-induced oligomerization of frataxin and the role of the ferroxidation reaction in oligomerization.
Söderberg CAG, Rajan S, Shkumatov AV, Gakh O, Schaefer S, Ahlgren EC, Svergun DI, Isaya G, Al-Karadaghi S., Free PMC Article | 05/18/2013 |
| Frataxin (YFH1) deficiency in yeast cells leads to increased DNA base oxidation and requirement of Apn1 for DNA damage repair. | Apn1 AP-endonuclease is essential for the repair of oxidatively damaged DNA bases in yeast frataxin-deficient cells.
Lefevre S, Brossas C, Auchère F, Boggetto N, Camadro JM, Santos R., Free PMC Article | 01/26/2013 |
| The authors observe that the high- and low-temperature denatured states of Yfh1 have similar but not identical residual secondary structures, different kinetics and compactness and a remarkably different degree of hydration. | The role of hydration in protein stability: comparison of the cold and heat unfolded states of Yfh1.
Adrover M, Martorell G, Martin SR, Urosev D, Konarev PV, Svergun DI, Daura X, Temussi P, Pastore A. | 05/5/2012 |
| in yeast, frataxin-deficiency interferes with mitochondrial dynamics, which might therefore be relevant for the pathophysiology of Friedreich ataxia. | Oxidative stress induces mitochondrial fragmentation in frataxin-deficient cells.
Lefevre S, Sliwa D, Rustin P, Camadro JM, Santos R. | 04/14/2012 |
| Secondary inactivation of Aat1 contributes to the amplification of the respiratory defect observed in Deltayfh1 cells. | Inactivation of mitochondrial aspartate aminotransferase contributes to the respiratory deficit of yeast frataxin-deficient cells.
Sliwa D, Dairou J, Camadro JM, Santos R. | 03/17/2012 |
| Metacaspase is associated with mitochondrial respiration, intracellular redox control and genomic stability. | The yeast metacaspase is implicated in oxidative stress response in frataxin-deficient cells.
Lefevre S, Sliwa D, Auchère F, Brossas C, Ruckenstuhl C, Boggetto N, Lesuisse E, Madeo F, Camadro JM, Santos R. | 03/10/2012 |
| The results revealed the types of oligomers present in solution and supported our earlier suggestions that the trimer is the main building block of yeast frataxin oligomers. | Oligomerization propensity and flexibility of yeast frataxin studied by X-ray crystallography and small-angle X-ray scattering.
Söderberg CA, Shkumatov AV, Rajan S, Gakh O, Svergun DI, Isaya G, Al-Karadaghi S., Free PMC Article | 02/4/2012 |
| in Deltayfh1 cells, soluble phosphate is limiting, due to its co-precipitation with iron. | 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 JM, Lesuisse E., Free PMC Article | 04/16/2011 |
| Deltayfh1 mutant exposed to oxidative stress has a drop in energy production and reducing power of the cells due to the damage of glycolytic enzymes and decrease in activity of enzymes involved in protein protection and regeneration. | Dynamics of protein damage in yeast frataxin mutant exposed to oxidative stress.
Kim JH, Sedlak M, Gao Q, Riley CP, Regnier FE, Adamec J., Free PMC Article | 03/26/2011 |
| Frataxin depletion in yeast triggers up-regulation of iron transport systems before affecting iron-sulfur enzyme activities | Frataxin depletion in yeast triggers up-regulation of iron transport systems before affecting iron-sulfur enzyme activities.
Moreno-Cermeño A, Obis E, Bellí G, Cabiscol E, Ros J, Tamarit J., Free PMC Article | 02/26/2011 |
| The ratio of soluble-to-insoluble iron did not change when the amount of Yfh1p was increased up to 4500 molecules per cell. The results do not support the hypothesis of Yfh1p being an iron storage protein in vivo. | Evidence that yeast frataxin is not an iron storage protein in vivo.
Seguin A, Sutak R, Bulteau AL, Garcia-Serres R, Oddou JL, Lefevre S, Santos R, Dancis A, Camadro JM, Latour JM, Lesuisse E. | 06/28/2010 |
| YFH1 interacts with Isu1 through the conserved Trp-131. | Frataxin interacts with Isu1 through a conserved tryptophan in its beta-sheet.
Leidgens S, De Smet S, Foury F. | 03/22/2010 |
| Changing the conserved beta-sheet residues Asn122-Lys123-Gln124 had no effect on Yfh1 stability and plasticity. | Iron-binding activity in yeast frataxin entails a trade off with stability in the alpha1/beta1 acidic ridge region.
Correia AR, Wang T, Craig EA, Gomes CM., Free PMC Article | 03/8/2010 |
| The semi-conserved pocket of negative charge within the 'acidic ridge' region significantly increase the YFH1 structural flexibility. | Frataxin, a molecule of mystery: trading stability for function in its iron-binding site.
Lane DJ, Richardson DR. | 03/8/2010 |
| frataxin drives assembly of core machinery for mitochondrial iron-sulfur cluster synthesis | Oligomeric yeast frataxin drives assembly of core machinery for mitochondrial iron-sulfur cluster synthesis.
Li H, Gakh O, Smith DY 4th, Isaya G., Free PMC Article | 01/21/2010 |
| Major frataxin-related phenotypes ([Fe-S] cluster assembly and heme synthesis) can be split in vivo, suggesting that frataxin has independent roles in both processes, and that the optimal conditions for these independent roles are different. | Overexpression of the yeast frataxin homolog (Yfh1): contrasting effects on iron-sulfur cluster assembly, heme synthesis and resistance to oxidative stress.
Seguin A, Bayot A, Dancis A, Rogowska-Wrzesinska A, Auchère F, Camadro JM, Bulteau AL, Lesuisse E. | 01/21/2010 |
| Yfh1 assembly responds to dynamic changes in mitochondrial iron uptake or stress exposure in a highly controlled fashion | Assembly of the iron-binding protein frataxin in Saccharomyces cerevisiae responds to dynamic changes in mitochondrial iron influx and stress level.
Gakh O, Smith DY 4th, Isaya G., Free PMC Article | 01/21/2010 |
| Glutathione is a valuable indicator of the redox status of frataxin-deficient cells in a yeast model of Friedreich's ataxia. | 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 JM. | 01/21/2010 |
| These results 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. | Binding of yeast frataxin to the scaffold for Fe-S cluster biogenesis, Isu.
Wang T, Craig EA., Free PMC Article | 01/21/2010 |
| particle image reconstructions show the iron core of frataxin | Structural basis of the iron storage function of frataxin from single-particle reconstruction of the iron-loaded oligomer.
Schagerlöf U, Elmlund H, Gakh O, Nordlund G, Hebert H, Lindahl M, Isaya G, Al-Karadaghi S., Free PMC Article | 01/21/2010 |
| Consequently, we suggest a direct participation of the respiratory chain in the pathogenesis of the Friedreich ataxia, which we propose to be considered as an OXPHOS disease. | Frataxin interacts functionally with mitochondrial electron transport chain proteins.
González-Cabo P, Vázquez-Manrique RP, García-Gimeno MA, Sanz P, Palau F. | 01/21/2010 |