The Yeast Frataxin Homologue Mediates Mitochondrial Iron Efflux
Open accessAbstract
Mutations in the nuclear gene encoding the mitochondrial protein frataxin are responsible for the neurological disorder Friedreich ataxia (FA). Yeast strains with a deletion in the frataxin homologue YFH1 accumulate excess iron in mitochondria and demonstrate mitochondrial damage. We show that in the absence of YFH1, mitochondrial damage is proportional to the concentration and duration of exposure to extracellular iron, establishing mitochondrial iron accumulation as causal to mitochondrial damage. Reintroduction of YFH1 results in the rapid export of accumulated mitochondrial iron into the cytosol as free, non-heme bound iron, demonstrating that mitochondrial iron in the yeast FA model can be made bioavailable. These results demonstrate a mitochondrial iron cycle in which Yfh1p regulates mitochondrial iron efflux.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
TIM-2-deficient mice showed increased levels of cytokines IL-4, IL-5, IL-6, and IL-10.
“TIM-2-deficient mice showed increased levels of cytokines IL-4, IL-5, IL-6, and IL-10, which may result in increased inflammation (256).”
From Intracellular iron transport and storage: from molecular mechanisms to health implications (2008) · cites this paper as [256]
Cites
Friedreich's Ataxia: Autosomal Recessive Disease Caused by an Intronic GAA Triplet Repeat Expansion
Campuzano, V., Montermini, L., Moltò, M. D., Pianese, L., Cossée, M., Cavalcanti, F., Monros, E., Rodius, F., Duclos, F., Monticelli, A., et al. (1996) · Science · doi:10.1126/science.271.5254.1423 · Cited by 2,786 · see citing works
Aconitase and mitochondrial iron–sulphur protein deficiency in Friedreich ataxia
Rötig, A., de Lonlay, P., Chretien, D., Foury, F., Koenig, M., Sidi, D., Munnich, A., & Rustin, P. (1997) · Nature Genetics · doi:10.1038/ng1097-215 · Cited by 991 · see citing works
Regulation of Mitochondrial Iron Accumulation by Yfh1p, a Putative Homolog of Frataxin
Babcock, M., de Silva, D., Oaks, R., Davis-Kaplan, S., Jiralerspong, S., Montermini, L., Pandolfo, M., & Kaplan, J. (1997) · Science · doi:10.1126/science.276.5319.1709 · Cited by 921 · see citing works
Self-citationFrataxin is Reduced in Friedreich Ataxia Patients and is Associated with Mitochondrial Membranes
Campuzano, V., Montermini, L., Lutz, Y., Cova, L., Hindelang, C., Jiralerspong, S., Trottier, Y., Kish, S. J., Faucheux, B. A., Trouillas, P., et al. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.11.1771 · Cited by 739 · see citing works
The FET3 gene of S. cerevisiae encodes a multicopper oxidase required for ferrous iron uptake.
Askwith, C., Eide, D., Van Ho, A., Bernard, P. S., Li, L., Davis-Kaplan, S., Sipe, D. M., & Kaplan, J. (1994) · Cell · doi:10.1016/0092-8674(94)90346-8 · Cited by 670 · see citing works
Self-citationPonka, P. (1997) · Blood · doi:10.1182/blood.v89.1.1 · Cited by 544 · see citing works
Bidichandani, S. I., Ashizawa, T., & Patel, P. I. (1998) · The American Journal of Human Genetics · doi:10.1086/301680 · Cited by 381 · see citing works
Foury, F., & Cazzalini, O. (1997) · FEBS Letters · doi:10.1016/s0014-5793(97)00734-5 · Cited by 378 · see citing works
Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue
Wilson, R. B., & Roof, D. M. (1997) · Nature Genetics · doi:10.1038/ng0897-352 · Cited by 345 · see citing works
The ABC transporter Atm1p is required for mitochondrial iron homeostasis
Kispál, G., Csere, P., Guiard, B., & Lill, R. (1997) · FEBS Letters · doi:10.1016/s0014-5793(97)01414-2 · Cited by 276 · see citing works
The Friedreich ataxia GAA triplet repeat: premutation and normal alleles
Montermini, L. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.8.1261 · Cited by 207 · see citing works
An ABC transporter in the mitochondrial inner membrane is required for normal growth of yeast.
Leighton, J. A., & Schatz, G. (1995) · The EMBO Journal · doi:10.1002/j.1460-2075.1995.tb06989.x · Cited by 197 · see citing works
Use of desferrioxamine in the treatment of aceruloplasminemia
Miyajima, H., Takahashi, Y., Kamata, T., Shimizu, H., Sakai, N., & Gitlin, J. D. (1997) · Annals of Neurology · doi:10.1002/ana.410410318 · Cited by 173 · see citing works
Gattermann, N., Retzlaff, S., Wang, Y., Hofhaus, G., Heinisch, J. J., Aul, C., & Schneider, W. (1997) · Blood · doi:10.1182/blood.v90.12.4961 · Cited by 159 · see citing works
Askwith, C. C., & Kaplan, J. (1997) · Journal of Biological Chemistry · doi:10.1074/jbc.272.1.401 · Cited by 148 · see citing works
Self-citationKushner, J. P., LEE, G. R., Wintrobe, M. M., & Cartwright, G. E. (1971) · Medicine · doi:10.1097/00005792-197105000-00001 · Cited by 120 · see citing works
Regulation of High Affinity Iron Uptake in the YeastSaccharomyces cerevisiae
Hassett, R. F., Romeo, A. M., & Kosman, D. J. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.13.7628 · Cited by 95 · see citing works
Chelation therapy in beta-thalassemia: the benefits and limitations of desferrioxamine.
Giardina, P. J., & Grady, R. W. (1995) · PubMed · Cited by 73 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
The roles of iron in health and disease
Lieu, P. T., Heiskala, M. K., Peterson, P. A., & Yang, Y. M. (2001) · Molecular Aspects of Medicine · doi:10.1016/s0098-2997(00)00006-6 · Cited by 813 · see citing works
Puccio, H., Simon, D., Cossée, M., Criqui-Filipe, P., Tiziano, F., Melki, J., Hindelang, C., Matyas, R., Rustin, P., & Koenig, M. (2001) · Nature Genetics · doi:10.1038/84818 · Cited by 738 · see citing works
Bush, A. (2000) · Current Opinion in Chemical Biology · doi:10.1016/s1367-5931(99)00073-3 · Cited by 731 · see citing works
The mitochondrial proteins Atm1p and Nfs1p are essential for biogenesis of cytosolic Fe/S proteins
Kispál, G., Csere, P., Prohl, C., & Lill, R. (1999) · The EMBO Journal · doi:10.1093/emboj/18.14.3981 · Cited by 699 · see citing works
I RON R EGULATORY P ROTEINS AND THE M OLECULAR C ONTROL OF M AMMALIAN I RON M ETABOLISM
Eisenstein, R. S. (2000) · Annual Review of Nutrition · doi:10.1146/annurev.nutr.20.1.627 · Cited by 652 · see citing works
Targeted gene disruption reveals an essential role for ceruloplasmin in cellular iron efflux
Harris, Z. L., Durley, A. P., Man, T. K., & Gitlin, J. D. (1999) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.96.19.10812 · Cited by 568 · see citing works
Involvement of NRAMP1 from Arabidopsis thaliana in iron transport
Curie, C., Alonso, J. M., Jean, M. L., Ecker, J. R., & Briat, J. (2000) · Biochemical Journal · doi:10.1042/bj3470749 · Cited by 552 · see citing works
Simcox, J. A., & McClain, D. A. (2013) · Cell Metabolism · doi:10.1016/j.cmet.2013.02.007 · Cited by 545 · see citing works
Intracellular iron transport and storage: from molecular mechanisms to health implications
Mackenzie, E. L., Iwasaki, K., & Tsuji, Y. (2008) · Antioxidants & Redox Signaling · doi:10.1089/ars.2007.1893 · Cited by 526 · see citing works
Energetics in the pathogenesis of neurodegenerative diseases
Beal, M. F. (2000) · Trends in Neurosciences · doi:10.1016/s0166-2236(00)01584-8 · Cited by 472 · see citing works
Grx5 Is a Mitochondrial Glutaredoxin Required for the Activity of Iron/Sulfur Enzymes
Rodrı́guez-Manzaneque, M. T., Tamarit, J., Bellı́, G., Ros, J., & Herrero, E. (2002) · Molecular Biology of the Cell · doi:10.1091/mbc.01-10-0517 · Cited by 464 · see citing works
Oxidative stress and mitochondrial dysfunction in neurodegenerative diseases
Trushina, E. V., & McMurray, C. T. (2007) · Neuroscience · doi:10.1016/j.neuroscience.2006.10.056 · Cited by 440 · see citing works
Iron–sulphur cluster biogenesis and mitochondrial iron homeostasis
Rouault, T. A., & Tong, W.-H. (2005) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm1620 · Cited by 432 · see citing works
Oxidative stress, mitochondrial dysfunction and cellular stress response in Friedreich's ataxia
Calabrese, V., Lodi, R., Tonon, C., D’Agata, V., Sapienza, M. A., Scapagnini, G., Mangiameli, A., Pennisi, G., Stella, A. M. G., & Butterfield, D. A. (2005) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2005.03.012 · Cited by 407 · see citing works
Eaton, J. W., & Qian, M. (2002) · Free Radical Biology and Medicine · doi:10.1016/s0891-5849(02)00772-4 · Cited by 389 · see citing works
Iron Toxicity in Diseases of Aging: Alzheimer's Disease, Parkinson's Disease and Atherosclerosis
Altamura, S., & Muckenthaler, M. U. (2009) · Journal of Alzheimer's Disease · doi:10.3233/jad-2009-1010 · Cited by 369 · see citing works
Brain iron pathways and their relevance to Parkinson's disease
Berg, D., Gerlach, M., Youdim, M. B. H., Double, K. L., Zecca, L., Riederer, P., & Becker, G. (2001) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2001.00608.x · Cited by 362 · see citing works
Friedreich ataxia: an overview
Delatycki, M. B., Williamson, R., & Forrest, S. M. (2000) · Journal of Medical Genetics · doi:10.1136/jmg.37.1.1 · Cited by 358 · see citing works
CCC1 Is a Transporter That Mediates Vacuolar Iron Storage in Yeast
Li, L., Chen, O. S., Ward, D. M., & Kaplan, J. (2001) · Journal of Biological Chemistry · doi:10.1074/jbc.m103944200 · Cited by 354 · see citing works
Self-citationNeurodegenerative disorders associated with diabetes mellitus
Ristow, M. (2004) · Journal of Molecular Medicine · doi:10.1007/s00109-004-0552-1 · Cited by 350 · see citing works
The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S proteins
Mühlenhoff, U. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.17.2025 · Cited by 326 · see citing works
Evidence for a conserved system for iron metabolism in the mitochondria of Saccharomyces cerevisiae
Schilke, B. A., Voisine, C., Beinert, H., & Craig, E. A. (1999) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.96.18.10206 · Cited by 284 · see citing works
Mitochondria and degenerative disorders
Orth, M., & Schapira, A. H. V. (2001) · American Journal of Medical Genetics · doi:10.1002/ajmg.1425 · Cited by 278 · see citing works
Nichol, H., Law, J. H., & Winzerling, J. J. (2002) · Annual Review of Entomology · doi:10.1146/annurev.ento.47.091201.145237 · Cited by 279 · see citing works
Costa, V. (2001) · Molecular Aspects of Medicine · doi:10.1016/s0098-2997(01)00012-7 · Cited by 272 · see citing works
A century of mitochondrial research: achievements and perspectives
Scheffler, I. E. (2001) · Mitochondrion · doi:10.1016/s1567-7249(00)00002-7 · Cited by 265 · see citing works
Iron-Dependent Self-Assembly of Recombinant Yeast Frataxin: Implications for Friedreich Ataxia
Adamec, J., Rusnak, F., Owen, W. G., Naylor, S., Benson, L. M., Gacy, A. M., & Isaya, G. (2000) · The American Journal of Human Genetics · doi:10.1086/303056 · Cited by 255 · see citing works
The pathogenesis of Friedreich ataxia and the structure and function of frataxin
Pandolfo, M., & Pastore, A. (2009) · Journal of Neurology · doi:10.1007/s00415-009-1003-2 · Cited by 242 · see citing works
Human Iron−Sulfur Cluster Assembly, Cellular Iron Homeostasis, and Disease
Ye, H., & Rouault, T. A. (2010) · Biochemistry · doi:10.1021/bi1004798 · Cited by 238 · see citing works
The long history of iron in the Universe and in health and disease
Sheftel, A. D., Mason, A. B., & Ponka, P. (2011) · Biochimica et Biophysica Acta (BBA) - General Subjects · doi:10.1016/j.bbagen.2011.08.002 · Cited by 234 · see citing works
Stehling, O., & Lill, R. (2013) · Cold Spring Harbor Perspectives in Biology · doi:10.1101/cshperspect.a011312 · Cited by 228 · see citing works
Role of Iron in Neurodegenerative Disorders
Berg, D., & Youdim, M. B. H. (2006) · Topics in Magnetic Resonance Imaging · doi:10.1097/01.rmr.0000245461.90406.ad · Cited by 227 · see citing works
Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1)
Lesuisse, E. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg096 · Cited by 226 · see citing works
Nikali, K., Suomalainen, A., Saharinen, J., Kuokkanen, M., Spelbrink, J. N., Lönnqvist, T. R. I., & Peltonen, L. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi328 · Cited by 223 · see citing works
Frataxin activates mitochondrial energy conversion and oxidative phosphorylation
Ristow, M., Pfister, M. F., Yee, A. J., Schubert, M., Michael, L., Zhang, C., Ueki, K., Michael, M. D., Lowell, B. B., & Kahn, C. R. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.220403797 · Cited by 221 · see citing works
Singh, N., Haldar, S., Tripathi, A. K., Horback, K., Wong, J., Sharma, D., Beserra, A., Suda, S., Anbalagan, C., Dev, S., et al. (2013) · Antioxidants and Redox Signaling · doi:10.1089/ars.2012.4931 · Cited by 205 · see citing works
Chen, O. S., Crisp, R. J., Valachovic, M., Bard, M., Winge, D. R., & Kaplan, J. (2004) · Journal of Biological Chemistry · doi:10.1074/jbc.m403209200 · Cited by 202 · see citing works
Self-citationLi, J., Kogan, M., Knight, S. A. B., Pain, D., & Dancis, A. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.46.33025 · Cited by 201 · see citing works
Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia
Cavadini, P. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.3.217 · Cited by 197 · see citing works
Distinct iron-sulfur cluster assembly complexes exist in the cytosol and mitochondria of human cells
Tong, W., & Rouault, T. (2000) · The EMBO Journal · doi:10.1093/emboj/19.21.5692 · Cited by 194 · see citing works
Friedreich Ataxia: Molecular Mechanisms, Redox Considerations, and Therapeutic Opportunities
Santos, R., Lefevre, S. D., Sliwa, D., Seguin, A., Camadro, J., & Lesuisse, E. (2010) · Antioxidants and Redox Signaling · doi:10.1089/ars.2009.3015 · Cited by 191 · see citing works
Desferrioxamine-mediated Iron Uptake in Saccharomyces cerevisiae
Yun, C., Ferea, T., Rashford, J., Ardon, O., Brown, P. O., Botstein, D., Kaplan, J., & Philpott, C. C. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.275.14.10709 · Cited by 183 · see citing works
Self-citationMitochondrial Control of Iron Homeostasis
Foury, F., & Talibi, D. (2001) · Journal of Biological Chemistry · doi:10.1074/jbc.m005804200 · Cited by 176 · see citing works
Glutathione in blood of patients with Friedreich's ataxia
Piemonte, F., Pastore, A., Tozzi, G., Tagliacozzi, D., Santorelli, F. M., Carrozzo, R., Casali, C., Damiano, M. G., Federici, G., & Bertini, E. (2001) · European Journal of Clinical Investigation · doi:10.1046/j.1365-2362.2001.00922.x · Cited by 174 · see citing works
Miranda, C. J., Santos, M. M., Ohshima, K., Smith, J., Li, L., Bunting, M., Cossée, M., Koenig, M., Sequeiros, J., Kaplan, J., et al. (2002) · FEBS Letters · doi:10.1016/s0014-5793(02)02251-2 · Cited by 172 · see citing works
Self-citationFoury, F., & Roganti, T. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m111789200 · Cited by 171 · see citing works
Towards a structural understanding of Friedreich’s ataxia: the solution structure of frataxin
Musco, G., Stier, G., Kolmerer, B., Adinolfi, S., Martin, S., Frenkiel, T., Gibson, T., & Pastore, A. (2000) · Structure · doi:10.1016/s0969-2126(00)00158-1 · Cited by 166 · see citing works
Siderophore-Iron Uptake in Saccharomyces cerevisiae
Yun, C., Tiedeman, J., Moore, R. E. B., & Philpott, C. C. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.m001456200 · Cited by 166 · see citing works
The Structure and Function of Frataxin
Bencze, K. Z., Kondapalli, K. C., Cook, J. D., McMahon, S., Millán‐Pacheco, C., Pastor, N., & Stemmler, T. L. (2006) · Critical Reviews in Biochemistry and Molecular Biology · doi:10.1080/10409230600846058 · Cited by 164 · see citing works
Yeast, a model organism for iron and copper metabolism studies
Freitas, J. M. D., Henri Wintz, A., Kim, J. H., Poynton, H. C., Fox, T. C., & Vulpe, C. D. (2003) · BioMetals · doi:10.1023/a:1020771000746 · Cited by 164 · see citing works
Metals in our minds: therapeutic implications for neurodegenerative disorders
Doraiswamy, P. M., & Finefrock, A. E. (2004) · The Lancet Neurology · doi:10.1016/s1474-4422(04)00809-9 · Cited by 161 · see citing works
Actin Glutathionylation Increases in Fibroblasts of Patients with Friedreich's Ataxia
Pastore, A., Tozzi, G., Gaeta, L. M., Bertini, E., Serafini, V., Cesare, S. D., Bonetto, V., Casoni, F., Carrozzo, R., Federici, G., et al. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m301872200 · Cited by 161 · see citing works
Friedreich ataxia- pathogenesis and implications for therapies
Delatycki, M. B., & Bidichandani, S. I. (2019) · Neurobiology of Disease · doi:10.1016/j.nbd.2019.104606 · Cited by 160 · see citing works
Shan, Y., Schoenfeld, R. A., Hayashi, G., Napoli, E., Akiyama, T., Carstens, M. I., Carstens, E. E., Pook, M. A., & Cortopassi, G. A. (2013) · Antioxidants and Redox Signaling · doi:10.1089/ars.2012.4537 · Cited by 157 · see citing works
Novel Nrf2-Inducer Prevents Mitochondrial Defects and Oxidative Stress in Friedreich’s Ataxia Models
Abeti, R., Baccaro, A., Esteras, N., & Giunti, P. (2018) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2018.00188 · Cited by 158 · see citing works
Chen, O. S., Hemenway, S., & Kaplan, J. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.192449599 · Cited by 152 · see citing works
Self-citationScheffler, I. E. (2001) · Advanced Drug Delivery Reviews · doi:10.1016/s0169-409x(01)00123-5 · Cited by 151 · see citing works
Friedreich Ataxia: current status and future prospects
Bürk, K. (2017) · Cerebellum & Ataxias · doi:10.1186/s40673-017-0062-x · Cited by 150 · see citing works
Luk, E., Carroll, M., Baker, M., & Culotta, V. (2003) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1632471100 · Cited by 146 · see citing works
Recent advances in the molecular pathogenesis of Friedreich ataxia
Puccio, H. M. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.6.887 · Cited by 146 · see citing works
Frataxin and Mitochondrial FeS Cluster Biogenesis
Stemmler, T. L., Lesuisse, E., Pain, D., & Dancis, A. (2010) · Journal of Biological Chemistry · doi:10.1074/jbc.r110.118679 · Cited by 145 · see citing works
Low iron concentration and aconitase deficiency in a yeast frataxin homologue deficient strain
Foury, F. (1999) · FEBS Letters · doi:10.1016/s0014-5793(99)00961-8 · Cited by 145 · see citing works
RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila
Anderson, P. R., Kirby, K., Hilliker, A. J., & Phillips, J. P. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi367 · Cited by 144 · see citing works
Human frataxin maintains mitochondrial iron homeostasis in Saccharomyces cerevisiae
Cavadini, P. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.17.2523 · Cited by 141 · see citing works
Srinivasan, C., Liba, A., Imlay, J. A., Valentine, J. S., & Gralla, E. B. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.m004239200 · Cited by 134 · see citing works
Adinolfi, S. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.16.1865 · Cited by 133 · see citing works
Iron Binding and Oxidation Kinetics in Frataxin CyaY of Escherichia coli
Bou‐Abdallah, F., Adinolfi, S., Pastore, A., Laue, T. M., & Chasteen, N. D. (2004) · Journal of Molecular Biology · doi:10.1016/j.jmb.2004.05.072 · Cited by 129 · see citing works
Characterization of iron binding in IscA, an ancient iron-sulphur cluster assembly protein
Ding, H., & CLARK, R. J. (2004) · Biochemical Journal · doi:10.1042/bj20031702 · Cited by 129 · see citing works
Monomeric Yeast Frataxin Is an Iron-Binding Protein
Cook, J. D., Bencze, K. Z., Janković, A., Crater, A. K., Busch, C. N., Bradley, P. B., Stemmler, A. J., Spaller, M. R., & Stemmler, T. L. (2006) · Biochemistry · doi:10.1021/bi060424r · Cited by 129 · see citing works
Friedreich Ataxia: From GAA Triplet–Repeat Expansion to Frataxin Deficiency
Patel, P. I., & Isaya, G. (2001) · The American Journal of Human Genetics · doi:10.1086/321283 · Cited by 126 · see citing works
General aspects of neurodegeneration
Jellinger, K. A. (2003) · Journal of Neural Transmission. Supplementa · doi:10.1007/978-3-7091-0643-3_7 · Cited by 126 · see citing works
Lutz, T., Westermann, B., Neupert, W., & Herrmann, J. M. (2001) · Journal of Molecular Biology · doi:10.1006/jmbi.2001.4527 · Cited by 125 · see citing works
Causative role of oxidative stress in a Drosophila model of Friedreich ataxia
Llorens, J. V., Navarro, J. A., Martínez-Sebastián, M. J., Baylies, M. K., Schneuwly, S., Botella, J., & Moltó, M. D. (2006) · The FASEB Journal · doi:10.1096/fj.05-5709com · Cited by 123 · see citing works
Calabrese, V., Butterfield, D. A., Scapagnini, G., Stella, A. M. G., & Maines, M. D. (2006) · Antioxidants and Redox Signaling · doi:10.1089/ars.2006.8.444 · Cited by 121 · see citing works
Adrenodoxin Reductase Homolog (Arh1p) of Yeast Mitochondria Required for Iron Homeostasis
Li, J., Saxena, S., Pain, D., & Dancis, A. (2001) · Journal of Biological Chemistry · doi:10.1074/jbc.m007198200 · Cited by 119 · see citing works
A non-essential function for yeast frataxin in iron-sulfur cluster assembly
Duby, G. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.21.2635 · Cited by 117 · see citing works
Iron: A New Target for Pharmacological Intervention in Neurodegenerative Diseases
Whitnall, M., & Richardson, D. R. (2006) · Seminars in Pediatric Neurology · doi:10.1016/j.spen.2006.08.008 · Cited by 113 · see citing works
Iron‐regulated transcription and capsule formation in the fungal pathogen Cryptococcus neoformans
Lian, T., Simmer, M. I., D’Souza, C. A., Steen, B. R., Zuyderduyn, S., Jones, S. J. M., Marra, M. A., & Kronstad, J. W. (2005) · Molecular Microbiology · doi:10.1111/j.1365-2958.2004.04474.x · Cited by 113 · see citing works
How Escherichia coli and Saccharomyces cerevisiae Build Fe/S Proteins
Barras, F., Loiseau, L., & Py, B. (2005) · Advances in microbial physiology/Advances in Microbial Physiology · doi:10.1016/s0065-2911(05)50002-x · Cited by 111 · see citing works
Friedreich ataxia: a paradigm for mitochondrial diseases
Puccio, H. M., & Koenig, M. (2002) · Current Opinion in Genetics & Development · doi:10.1016/s0959-437x(02)00298-8 · Cited by 111 · see citing works
Campanella, A. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddh232 · Cited by 109 · see citing works
A Mitochondrial-Vacuolar Signaling Pathway in Yeast That Affects Iron and Copper Metabolism
Li, L., & Kaplan, J. (2004) · Journal of Biological Chemistry · doi:10.1074/jbc.m403146200 · Cited by 109 · see citing works
Self-citationVyas, P. M., Tomamichel, W. J., Pride, P. M., Babbey, C. M., Wang, Q., Mercier, J., Martin, E. M., & Payne, R. M. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddr554 · Cited by 109 · see citing works
Molecular Pathogenesis of Friedreich Ataxia
Pandolfo, M. (1999) · Archives of Neurology · doi:10.1001/archneur.56.10.1201 · Cited by 108 · see citing works
Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question
Armstrong, J. S., Khdour, O., & Hecht, S. M. (2010) · The FASEB Journal · doi:10.1096/fj.09-143222 · Cited by 107 · see citing works
Modulation of thiol-dependent redox system by metal ionsviathioredoxin and glutaredoxin systems
Ouyang, Y., Peng, Y., Li, J., Holmgren, A., & Lu, J. (2018) · Metallomics · doi:10.1039/c7mt00327g · Cited by 106 · see citing works
Branda, S. S., Cavadini, P., Adamec, J., Kalousek, F., Taroni, F., & Isaya, G. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.32.22763 · Cited by 107 · see citing works
Anderson, P. R., Kirby, K., Orr, W. C., Hilliker, A. J., & Phillips, J. P. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0709691105 · Cited by 105 · see citing works
DNA repeat expansions and human disease
Usdin, K., & Grabczyk, E. (2000) · Cellular and Molecular Life Sciences · doi:10.1007/pl00000734 · Cited by 106 · see citing works
Richardson, D. R. (2004) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1306.026 · Cited by 104 · see citing works
Friedreich's ataxia: Past, present and future
Marmolino, D. (2011) · Brain Research Reviews · doi:10.1016/j.brainresrev.2011.04.001 · Cited by 103 · see citing works
Metal ion transporters in mammals: structure, function and pathological implications
Rolfs, A., & Hediger, M. A. (1999) · The Journal of Physiology · doi:10.1111/j.1469-7793.1999.0001r.x · Cited by 103 · see citing works
Reduction in frataxin causes progressive accumulation of mitochondrial damage
Karthikeyan, G. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg349 · Cited by 102 · see citing works
The role of oxidative stress in Friedreich's ataxia
Lupoli, F., Vannocci, T., Longo, G., Niccolai, N., & Pastore, A. (2017) · FEBS Letters · doi:10.1002/1873-3468.12928 · Cited by 102 · see citing works
Iron and its sensitive balance in the cell
Freitas, J. M. D., & Meneghini, R. (2001) · Mutation research. Fundamental and molecular mechanisms of mutagenesis · doi:10.1016/s0027-5107(01)00066-5 · Cited by 101 · see citing works
Hereditary Causes of Disturbed Iron Homeostasis in the Central Nervous System
Ponka, P. (2004) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1306.022 · Cited by 99 · see citing works
Iron in neurodegenerative disorders
Berg, D., Becker, G., Riederer, P. F., & Rieß, O. (2002) · Neurotoxicity Research · doi:10.1080/1029842021000045444 · Cited by 98 · see citing works
Li, L., & Kaplan, J. (2001) · Journal of Biological Chemistry · doi:10.1074/jbc.m008969200 · Cited by 99 · see citing works
Self-citationKoeppen, A. (1998) · Journal of Neuropathology and Experimental Neurology · doi:10.1097/00005072-199806000-00001 · Cited by 22 · see citing works
Mitochondrial DNA inheritance in Saccharomyces cerevisiae
Berger, K. H., & Yaffe, M. P. (2000) · Trends in Microbiology · doi:10.1016/s0966-842x(00)01862-x · Cited by 97 · see citing works
- Authors
- Radisky, D. C., Babcock, M. C., & Kaplan, J.
- Affiliations
- University of Utah (US)
- Corresponding author
- Kaplan, Jerry
- ORCID
- Radisky, Derek C.
- Venue
- Journal of Biological Chemistry
- Publisher
- Elsevier BV
- Published
- 1999-02
- Volume
- 274
- Issue
- 8
- Pages
- 4497-4499
- ISSN
- 0021-9258 (ISSN-L)
- OpenAlex
- W2009136033
- Cited by
- 277
- References
- 18
- Citations by year
- 2026: 1 · 2025: 5 · 2023: 5 · 2022: 5 · 2021: 1 · 2020: 1 · 2019: 2 · 2018: 7 · 2017: 2 · 2016: 3 · 2015: 3 · 2014: 7 · 2013: 12 · 2012: 3
- FWCI
- 13.46
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Biological Chemistry (publishedVersion, cc-by)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (5p50dk049219-05)
- National Cancer Institute (CA42014, P30CA042014)
- National Institute of Diabetes and Digestive and Kidney Diseases (DK 52380, R01DK052380, DK-49219)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
- Health Sciences › Medicine › Hematology › Iron Metabolism and Disorders
- Keywords
- frataxin, Yfh1, Friedreich's ataxia, mitochondrial iron accumulation, mitochondrial damage, yeast model, non-heme iron
- MeSH terms
- Frataxin, Biological Transport, Friedreich Ataxia, Iron, Mitochondria, Phosphotransferases (Alcohol Group Acceptor), Iron-Binding Proteins
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
