ライフサイエンスコーパス: frataxin

1 uced expression of the mitochondrial protein frataxin.

2 and/or function of the mitochondrial protein frataxin.

3 ticed, potential ubiquitin-binding domain in frataxin.

4 of yeast (Yfh1) and Escherichia coli (CyaY) frataxin.

5 ess and pathology observed in the absence of frataxin.

6 a genetic disease caused by deficiencies in frataxin.

7 by a deficiency in the mitochondrial protein frataxin.

8 eads to epigenetic modifications and reduced frataxin.

9 processing of cytosolic precursors, such as frataxin.

10 in the first intron of the gene that encodes frataxin.

11 s transcription leading to the deficiency of frataxin.

12 generative disorder caused by a reduction in frataxin.

13 c insufficiency of the mitochondrial protein frataxin.

14 loss of the essential mitochondrial protein frataxin.

15 ent oligomers contribute to the functions of frataxin.

16 nal activities of superoxide dismutase 2 and frataxin, 2 common target genes involved in radical dism

17 ced in these models, is assumed to be mature frataxin (78-207) by analogy with human mature frataxin

18 rily in the cytosol of mouse liver; whereas, frataxin (78-207) is primarily present in the mitochondr

19 Mature mouse frataxin (78-207) only contributes 7-15% to the total fr

20 dominantly a 129-amino acid truncated mature frataxin (79-207) in which the N-terminal lysine residue

21 We have also found that truncated mature frataxin (79-207) is present primarily in the cytosol of

22 Reduced levels of mature frataxin (81-20) in human subjects caused by the genetic

23 ataxin (78-207) by analogy with human mature frataxin (81-210).

24 eich ataxia is caused by reduced activity of frataxin, a conserved iron-binding protein of the mitoch

25 Frataxin, a conserved nuclear-encoded mitochondrial prot

26 nerative disease caused by the deficiency of frataxin, a mitochondrial protein crucial for iron-sulfu

27 FXN gene, which codes for the 210 amino acid frataxin, a mitochondrial protein involved in iron-sulfu

28 Frataxin, a mitochondrial protein that is directly invol

29 ch's ataxia (FRDA) is caused by mutations in frataxin, a mitochondrial protein whose function remains

30 fect is made possible by the choice of yeast frataxin, a protein that undergoes cold denaturation abo

31 e functional absence of the FXN gene product frataxin, a protein whose exact function still remains u

32 We investigate this phenomenon by studying frataxin, a protein whose normal function is to facilita

33 caused by a decreased level of expression of frataxin, a putative iron chaperone.

34 Frataxin, a small mitochondrial protein linked to the ne

35 e disease caused by reduced transcription of frataxin, a ubiquitously expressed protein.

36 Mutations in the hydrophobic core of frataxin affected stability whereas surface residue muta

37 Monomeric frataxin also binds to Isu, the scaffold protein require

38 Frataxin amino acids affected by the presence of iron ar

39 We observed decreased levels of Yfh1/frataxin, an essential component of the iron-sulfur biog

40 Deficiency of frataxin, an essential mitochondrial protein, leads to p

41 Reduced levels of frataxin, an essential protein of as yet unknown functio

42 cted with empty vector compared to wild-type frataxin and (II) lymphoblasts from FRDA patients show t

43 GRP75 increases the levels of both wild-type frataxin and clinically relevant missense frataxin varia

44 Here we report on the complex between yeast frataxin and ferrochelatase, the terminal enzyme of heme

45 Interactions between frataxin and ISD11, and frataxin and GRP75 were confirmed by co-immunoprecipitat

46 p and group* HAX-1 interaction revealed that frataxin and HAX-1 are associated both at mRNA and prote

47 Moreover, correlation between frataxin and HAX-1 was further evaluated in peripheral b

48 Interactions between frataxin and ISD11, and frataxin and GRP75 were confirme

49 ss-linking confirmed the interaction between frataxin and ISU in the presence of iron and validated t

50 and whether in vivo the interaction between frataxin and Isu is mediated by adaptor proteins is a ma

51 nd presumably the iron chaperone function of frataxin and its interactions with target proteins.

52 ssion, providing a link between increases in frataxin and neurophysiological function.

53 ding site of CyaY, the bacterial ortholog of frataxin and sits in a cavity close to the enzyme active

54 persulfide, but this step is independent of frataxin and strictly dependent on Isd11.

55 T and P509S impair the binding of GRP75 with frataxin and the effect of GRP75 on frataxin levels.

56 there are two interaction interfaces between frataxin and the ferrochelatase dimer.

57 monstrated that ISD11 co-localized with both frataxin and with mitochondria.

58 In E. cuniculi, the iron (frataxin) and sulphur (cysteine desulphurase, Nfs1) dono

59 riers with approximately 50% decrease of the frataxin are asymptomatic.

60 but surprisingly the main pools of Isu1 and frataxin are cytosolic, creating a conundrum of how thes

61 ve investigated how these different forms of frataxin are regulated in vivo.

62 a new paradigm for understanding the role of frataxin as a regulator of IscS functions.

63 possibility of enhancing levels of residual frataxin as a treatment for FRDA.

64 tes that the ferroxidation reaction controls frataxin assembly and presumably the iron chaperone func

65 ish the levels of both complex formation and frataxin-based activation, whereas ferrous iron further

66 Frataxin binding dramatically changes the K(M) for cyste

67 Frataxin binds Isu in an iron-dependent manner in vitro.

68 These data suggest that frataxin binds the iron-sulfur biogenesis Nfs1/ISCU comp

69 Here we provide in vitro evidence that human frataxin binds to a Nfs1, Isd11, and Isu2 complex to gen

70 talin/mthsp70/PBP74, directly interacts with frataxin both in vivo in mouse cortex and in vitro in co

71 Nevertheless, derepression of frataxin by a histone deacetylase inhibitor leads to a d

72 nthesized and tested for specific binding to frataxin by an UF-LC/MS based ligand-binding assay.

73 The point mutations I154F and W155R in frataxin cause FRDA and are clustered to one surface of

74 ited deficiency of the mitochondrial protein frataxin causes Friedreich's ataxia (FRDA); the mechanis

75 s, decreased amounts or impaired function of frataxin causes the autosomal recessive neurodegenerativ

76 ound that Fdx, IscU, and CyaY (the bacterial frataxin) compete for overlapping binding sites on IscS.

77 s associated with a sustained improvement in frataxin concentrations towards those seen in asymptomat

78 This study also suggests that frataxin could be a potential target for FRDA drug devel

79 ng an in vitro disease model, we studied how frataxin deficiency affects beta-cell function and survi

80 thesis that the respiratory chain defects in frataxin deficiency alter mitochondrial protein acetylat

81 In addition, GRP75 overexpression rescues frataxin deficiency and abnormal cellular phenotypes suc

82 ed to arrest transcription, which results in frataxin deficiency and eventual neurodegeneration.

83 dependent, and that multiple consequences of frataxin deficiency are duplicated by ISD11 deficiency.

84 Humans with frataxin deficiency have Friedreich's ataxia, a neurodeg

85 f nicotinamide and its ability to ameliorate frataxin deficiency in Friedreich's ataxia is warranted.

86 Collectively, cardiac frataxin deficiency reduces Nrf2 levels via two potentia

87 planation for the elevated oxylipins is that frataxin deficiency results in increased COX activity.

88 Frataxin deficiency sensitized beta cells to oleate-indu

89 ged in cells from patients with pathological frataxin deficiency, and a core set of these genes were

90 eart and skeletal muscle in a mouse model of frataxin deficiency, and found molecular evidence of inc

91 ctor Srebp1 in cellular and animal models of frataxin deficiency, and in cells from FRDA patients, wh

92 a neurodegenerative disorder resulting from frataxin deficiency, is thought to involve progressive c

93 s known that DRG are inherently sensitive to frataxin deficiency, recent observations also indicate t

94 Frataxin deficiency, responsible for Friedreich's ataxia

95 iodegenerative disease resulting from marked frataxin deficiency.

96 on have been conducted using mouse models of frataxin deficiency.

97 ial dysfunction conducted in mouse models of frataxin deficiency.

98 nherited neurodegenerative disease caused by frataxin deficiency.

99 ion of this pathway could play a key role in frataxin deficiency.

100 levels and/or alleviate the consequences of frataxin deficiency.

101 eration is due to the sensitivity of DRGs to frataxin deficiency; however, the progressive nature of

102 n potentially further compromise function in frataxin-deficient cells by decreasing frataxin expressi

103 inferred cytosolic iron depletion occurs as frataxin-deficient cells overload their mitochondria wit

104 Frataxin-deficient mice, which had higher mitochondrial

105 We propose that pathology in the frataxin-deficient nervous system involves decreased MMP

106 These findings are in contrast to the frataxin-deficient skeletal muscle, where Nrf2 was not d

107 which the expanded repeats contribute to the frataxin deficit in FRDA.

108 s, expression of partially functional mutant frataxin delays age of onset and reduces diabetes mellit

109 e that mirrors the disease have demonstrated frataxin deletion alters cardiac Fe metabolism.

110 A conditional mouse model with complete frataxin deletion in cardiac and skeletal muscle (Mck-Cr

111 Namely, frataxin deletion induces a signaling mechanism to incre

112 effect of heart and skeletal muscle-specific frataxin deletion on systemic Fe metabolism.

113 ve shown that either monomeric or oligomeric frataxin delivers iron to other proteins, whereas ferrit

114 This novel function of frataxin does not require iron, Isu1, or Isd11.

115 Frataxin downregulation is associated with robust change

116 ynthesis of the mitochondrial iron chaperone frataxin due to impaired gene transcription, which leads

117 ere show that the Isu1 suppressor mimics the frataxin effects on Nfs1, explaining the bypassing activ

118 Moreover, later administration of the frataxin-expressing vector, after the onset of heart fai

119 In Drosophila larvae with reduced frataxin expression (DfhIR), we evaluated possible mecha

120 view on articles pertaining to activation of frataxin expression (Friedreich's ataxia) and production

121 our study, we investigated the regulation of frataxin expression by iron and demonstrated that fratax

122 Alterations up or down of frataxin expression caused compensatory changes in HSC20

123 diabetes mellitus, compared to those with no frataxin expression from the non-expanded allele.

124 s cytosolic iron levels to maximize residual frataxin expression in FA patients.

125 n across repressive GAA repeats that silence frataxin expression in Friedreich's ataxia, a terminal n

126 ic Fe levels and Fe loading in tissues where frataxin expression is intact (i.e., liver, kidney, and

127 indicate that approaches aimed to reactivate frataxin expression should simultaneously address defici

128 orrelated with cytokine-induced increases in frataxin expression, providing a link between increases

129 d and significant (p<0.0001) upregulation of frataxin expression, which was accompanied by a reductio

130 The primary outcome was the upregulation of frataxin expression.

131 on in frataxin-deficient cells by decreasing frataxin expression.

132 XN gene causes Friedreich ataxia by reducing frataxin expression.

133 ic modifications of the FXN gene to increase frataxin expression.

134 ogenesis of the disease) and a regulators of frataxin expression.

135 ur in 96% of affected individuals and reduce frataxin expression.

136 ene leading to transcriptional repression of frataxin expression.

137 N FIXATION S-LIKE1 (NFS1) and its interactor FRATAXIN (FH), when silenced in Nicotiana benthamiana, c

138 lts show a continuous compaction of unfolded frataxin from 274 to 320 K, with a slight re-expansion a

139 The ability of frataxin from different organisms to populate multiple o

140 us exists on the details of the mechanism of frataxin function and oligomerization.

141 understanding of the mechanistic features of frataxin function requires detailed knowledge of the int

142 Most researchers agree that frataxin functions in the biogenesis of Fe-S clusters, b

143 Together, these results indicate human frataxin functions with Fe(2+) as an allosteric activato

144 nt mutation in Isu1 was found to bypass many frataxin functions.

145 Mutations in Frataxin (FXN) cause Friedreich's ataxia (FRDA), a reces

146 ited deficiency in the mitochondrial protein frataxin (FXN) causes the rare disease Friedreich's atax

147 The molecular mechanisms of reduced frataxin (FXN) expression in Friedreich's ataxia (FRDA)

148 ed mitochondrial function due to the loss of frataxin (FXN) expression.

149 GAA . TTC repeat in the first intron of the frataxin (FXN) gene causes an mRNA deficit that results

150 Expanded GAA repeats within intron 1 of the frataxin (FXN) gene lead to its heterochromatinisation a

151 n of an intronic trinucleotide repeat in the frataxin (FXN) gene yielding diminished FXN expression a

152 r caused by transcriptional silencing of the frataxin (FXN) gene, resulting in loss of the essential

153 enerative disease caused by mutations in the frataxin (FXN) gene, resulting in reduced expression of

154 onic GAA.TTC triplet repeat expansion in the frataxin (FXN) gene.

155 ding to the transcriptional silencing of the frataxin (FXN) gene.

156 ditary ataxia, is caused by mutations in the frataxin (FXN) gene.

157 elated with the number of GAA repeats in the frataxin (FXN) gene: every 100 GAA repeats on the smalle

158 The function of frataxin (FXN) has garnered great scientific interest si

159 edreich's ataxia (FRDA) patients, diminished frataxin (FXN) in sensory neurons is thought to yield th

160 The protein frataxin (FXN) is an allosteric activator that binds the

161 his expansion leads to reduced expression of frataxin (FXN) protein and evidence suggests that transc

162 ted with the loss of function of the protein frataxin (FXN) that results from low FXN levels due to a

163 ecessive mutations that reduce the levels of frataxin (FXN), a mitochondrial iron binding protein.

164 disease caused by insufficient expression of frataxin (FXN), a mitochondrial iron-binding protein req

165 FRDA is caused by reduced levels of frataxin (FXN), an essential mitochondrial protein invol

166 and associates with assembly proteins ISCU2, frataxin (FXN), and ferredoxin to synthesize Fe-S cluste

167 steine desulfurase NFS1 that is activated by frataxin (FXN), scaffold protein ISCU, accessory protein

168 ysteine desulfurase complex (NFS1/ISD11) and frataxin (FXN), the protein deficient in Friedreich's at

169 ited deficiency of the mitochondrial protein Frataxin (FXN), which has no approved therapy and is an

170 ne normally encodes the iron-binding protein frataxin (FXN), which is critical for mitochondrial iron

171 m a deficiency of the mitochondrial protein, frataxin (FXN), which is encoded in the nucleus.

172 ssive mutations in the mitochondrial protein frataxin (FXN).

173 odegenerative disease caused by mutations in Frataxin (FXN).

174 TTC) cause transcriptional repression of the Frataxin gene (FXN) leading to Friedreich's ataxia (FRDA

175 DA), expanded GAA repeats in intron 1 of the frataxin gene (FXN) reduce FXN mRNA levels in averaged c

176 d by large GAA expansions in intron 1 of the frataxin gene (FXN), which lead to reduced FXN expressio

177 A repeat length on the smaller allele of the frataxin gene (hazard ratio [HR], 1.85; 95% CI, 1.28-2.6

178 GAA)n repeats within the first intron of the frataxin gene reduce its expression, resulting in a here

179 nerative disorder caused by mutations in the frataxin gene that produces a predominantly mitochondria

180 the (GAA)n repeat in the first intron of the frataxin gene.

181 a GAA trinucleotide repeat expansion in the frataxin gene.

182 d products of human disease genes, including frataxin, GLRX5, ISCU, and ABCB7, have important roles i

183 We show that loss of frataxin homolog (fh) in Drosophila leads to iron toxici

184 We recently showed that loss of frataxin homolog (fh), a Drosophila homolog of FXN, caus

185 ed mitochondria, we show here that the yeast frataxin homolog (Yfh1) directly and specifically stimul

186 ase serving as a sulfur donor, and the yeast frataxin homolog (Yfh1) serving as a regulator of desulf

187 We have identified a frataxin homolog in fission yeast, and we have analyzed

188 nd iron binding properties of the Drosophila frataxin homologue (Dfh).

189 ex consisting of the iron donor, Yfh1 (yeast frataxin homologue 1), and the Fe-S cluster scaffold, Is

190 We also show that like frataxin, HSC20 interacts with multiple proteins involve

191 rescued the defective interaction of mutant frataxin I154F and W155R with ISD11.

192 er scaffold and point to a critical role for frataxin in Fe-S cluster biogenesis.

193 The role of the mitochondrial protein frataxin in iron storage and detoxification, iron delive

194 acts with the Friedreich ataxia gene product frataxin in iron-sulfur cluster biosynthesis.

195 tified multiple interactors of mitochondrial frataxin in mammalian cells.

196 spectrometry, we have discovered that mature frataxin in mouse heart (77%), brain (86%), and liver (4

197 support for the role of extra-mitochondrial frataxin in the etiology of Friedreich's ataxia, also ha

198 mphoblastoid cells stably reconstituted with frataxin, indicated HS-1-associated protein X-1 (HAX-1)

199 imum activity as follows: one is mediated by frataxin interaction that exposes the "buried" substrate

200 Frataxin interacts with Isu, iron, and the cysteine desu

201 Previously we demonstrated that frataxin interacts with multiple components of the mamma

202 Biochemical and genetic studies have shown frataxin interacts with the iron-sulfur cluster assembly

203 Here we demonstrate that frataxin interacts with the mammalian mitochondrial chap

204 Frataxin is a conserved mitochondrial protein that contr

205 Frataxin is a conserved mitochondrial protein that plays

206 Frataxin is a mitochondrial iron-binding protein involve

207 Frataxin is a mitochondrial protein involved in iron hom

208 Cellular depletion of the human protein frataxin is correlated with the neurodegenerative diseas

209 Here, we report findings that frataxin is degraded via the ubiquitin-proteasomal pathw

210 Mature frataxin is essential for the assembly of iron-sulfur cl

211 Frataxin is implicated in the process, although it is un

212 Frataxin is thought to transiently interact with ISU, th

213 The frataxin/ISD11 interaction was also decreased by the che

214 A theoretical model of the frataxin-K(147)/Ub complex, constructed by combining bio

215 RNA interference-mediated frataxin knockdown impaired glucose-stimulated insulin s

216 Using a mouse conditional frataxin knockout (KO) model in the heart and skeletal m

217 using the muscle creatine kinase conditional frataxin knockout (KO) mouse; this mouse develops a seve

218 the muscle creatine kinase (MCK) conditional frataxin knockout mouse that mirrors the disease have de

219 Frataxin KO results in fatal cardiomyopathy, whereas ske

220 rt the hypothesis that reduced expression of frataxin leads to elevation of COX2-mediated oxylipin sy

221 How the reduced expression of frataxin leads to neurological and other systemic sympto

222 Both G-CSF and SCF had pronounced effects on frataxin levels (the primary molecular defect in the pat

223 urrent therapeutic strategies aim to elevate frataxin levels and/or alleviate the consequences of fra

224 hat posttranslational regulation of residual frataxin levels can rescue some of the functional defici

225 modulation of the PPARgamma pathway affects frataxin levels in vitro, supporting PPARgamma as a nove

226 We propose a model in which cellular frataxin levels regulate human Fe-S cluster biosynthesis

227 for developing therapies aimed at increasing frataxin levels to treat this debilitating disease.

228 nd expanded FXN loci with rapid detection of frataxin levels.

229 vitro treatment with compounds that increase frataxin levels.

230 P75 with frataxin and the effect of GRP75 on frataxin levels.

231 the importance of a tight regulation of the frataxin levels.

232 SUD-C adopts a frataxin like fold and has structural similarity to DNA-

233 ed at preventing the debilitating effects of frataxin loss and preventing the signs and symptoms asso

234 organization, and apoptosis are affected by frataxin loss in neurons of the CNS and peripheral nervo

235 ndings observed in FXTAS cells (lower mature frataxin, lower Complex IV and aconitase activities) alo

236 Because frataxin may participate in transient interactions with

237 urea] 50% approximately 2.4 M) of Drosophila frataxin, measured using circular dichroism (CD) and flu

238 Frataxin measurements from peripheral tissues can be use

239 Furthermore, they support the proposal that frataxin-mediated delivery of this potentially toxic sub

240 Finally, frataxin mediates the delivery of Fe(II) to Isu, promoti

241 The effect of GRP75 on frataxin might be in part mediated by the physical inter

242 urements demonstrated that in the absence of frataxin, mitochondria contained biomineral Fe aggregate

243 protein levels are indeed regulated through frataxin modulation.

244 rial processing peptidase (MPP), which makes frataxin more accessible to MPP.

245 ymphoblasts from FRDA patients show that low frataxin mRNA and protein expression correspond to reduc

246 rataxin show comparatively reduced levels of frataxin mRNA and protein expression, decreased aconitas

247 In addition, frataxin mRNA and protein levels decrease in fibroblast

248 xin expression by iron and demonstrated that frataxin mRNA levels decrease significantly in multiple

249 lity and in the mechanism underlying reduced frataxin mRNA levels in Friedreich Ataxia.

250 This integrated analysis of categorized frataxin mutations and their correlation with clinical o

251 Frataxin mutations were examined using structural modeli

252 estigates the participation of the bacterial frataxin ortholog CyaY and the YggX protein, which are p

253 ed the iron binding property of IscA and the frataxin ortholog CyaY from Escherichia coli under physi

254 nsistent with the structurally characterized frataxin orthologs.

255 d strand 1 in the structurally characterized frataxin orthologs.

256 Frataxin overexpression and silencing were also performe

257 imised the technique to study the effects of frataxin overexpression in a cellular model of Friedreic

258 We probed the effects of frataxin overexpression in the presence of oxidative str

259 ron to other proteins, whereas ferritin-like frataxin particles convert redox-active iron to an inert

260 t, there are two, Isu1 and Isu2), indicating frataxin plays a direct role in cluster assembly, possib

261 Our data indicate that frataxin point mutations have complex biochemical effect

262 Frataxin prevents reactive oxygen species-induced oxidat

263 e compound heterozygote groups; (2) null (no frataxin produced); (3) moderate/strong impact; and (4)

264 by blocking binding of factors that increase frataxin promoter activity.

265 response relation for proportional change in frataxin protein concentration from baseline to 8 h post

266 .5-times increase and 7.5 g in a doubling of frataxin protein concentration.

267 Higher P:M, and lower ZnT6 and mature frataxin protein expression suggested defective zinc and

268 Frataxin protein levels from multiple cell types in cont

269 (78-207) only contributes 7-15% to the total frataxin protein present in mouse tissues.

270 ffects as well as for increases in FXN mRNA, frataxin protein, and chromatin modification in blood ce

271 ACi 109/RG2833 increases FXN mRNA levels and frataxin protein, with concomitant changes in the epigen

272 erative disorder caused by deficiency of the frataxin protein.

273 e FXN gene, leading to reduced expression of frataxin protein.

274 The degree of frataxin reduction correlates with GAA.TTC tract length,

275 Defects in frataxin result in Friedreich ataxia, a genetic disease

276 Loss of frataxin results in mitochondrial dysfunction and oxidat

277 nt FRDA mice that express only human-derived frataxin show comparatively reduced levels of frataxin m

278 e results indicate that HSC20 interacts with frataxin structurally and functionally and is important

279 disease-causing mutations and the impact on frataxin structure/function and clinical outcome in FRDA

280 However, mouse frataxin that is reduced in these models, is assumed to

281 ken together, these results indicate that in frataxin the competition between folding and function cr

282 eserved, whereas mRNA and protein levels for frataxin, the oxidative stress-regulated mitochondrial a

283 s mutation impacts the maturation process of frataxin, the protein which is depleted in Friedreich at

284 Independent reports have linked frataxin to iron-sulphur cluster assembly through intera

285 controlled fashion and that this may enable frataxin to simultaneously promote respiratory function

286 involves one ferrochelatase monomer and one frataxin trimer, with conserved polar and charged amino

287 s with micromolar activity in disrupting the frataxin/Ub interaction.

288 l molecule (compound (+)-11) able to prevent frataxin ubiquitination and degradation.

289 pe frataxin and clinically relevant missense frataxin variants in human embryonic kidney 293 cells, w

290 Higher P:M of ATPase beta-subunit (ATPB) and frataxin were also observed in cortex from patients that

291 A regression model for frataxin which included HAX-1, group membership and grou

292 conserved, surface-exposed residues of yeast frataxin, which have deleterious effects on cell growth,

293 Frataxin, which is significantly reduced in patients wit

294 sed expression of the mitochondrial protein, frataxin, which leads to alterations in mitochondrial ir

295 we investigated the unfolded state of yeast frataxin, whose cold denaturation occurs at temperatures

296 these mutations decrease the interaction of frataxin with ISD11.

297 raction of CyaY (the bacterial orthologue of frataxin) with the IscS/IscU complex.

298 re we provide molecular details of how yeast frataxin (Yfh1) interacts with Isu1 as a structural modu

299 ccharomyces cerevisiae, only monomeric yeast frataxin (Yfh1) was detected in unstressed cells when mi

300 s similar to that which accumulates in yeast frataxin Yfh1p-deleted or yeast ferredoxin Yah1p-deplete