In vivo maturation of human frataxin

Three-dimensional tracking of axonal projections in the brain by magnetic resonance imaging

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Friedreich's Ataxia: Autosomal Recessive Disease Caused by an Intronic GAA Triplet Repeat Expansion

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Aconitase and mitochondrial iron–sulphur protein deficiency in Friedreich ataxia

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Regulation of Mitochondrial Iron Accumulation by Yfh1p, a Putative Homolog of Frataxin

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Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits

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Mitochondrial processing peptidases

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Iron–sulphur cluster biogenesis and mitochondrial iron homeostasis

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Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation

Cossee, M. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.8.1219 · Cited by 377 · see citing works

The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis

Wong, A. (1999) · Human Molecular Genetics · doi:10.1093/hmg/8.3.425 · Cited by 374 · see citing works

Frataxin Acts as an Iron Chaperone Protein to Modulate Mitochondrial Aconitase Activity

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Iron−Sulfur Cluster Biosynthesis. Characterization of Frataxin as an Iron Donor for Assembly of [2Fe-2S] Clusters in ISU-Type Proteins

Yoon, T., & Cowan, J. A. (2003) · Journal of the American Chemical Society · doi:10.1021/ja027967i · Cited by 353 · see citing works

Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia

Bradley, J. L. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.2.275 · Cited by 339 · see citing works

Frataxin-mediated Iron Delivery to Ferrochelatase in the Final Step of Heme Biosynthesis

Yoon, T., & Cowan, J. A. (2004) · Journal of Biological Chemistry · doi:10.1074/jbc.c400107200 · Cited by 281 · see citing works

Iron-Sulfur Cluster Biosynthesis

Layer, G., de Choudens, S. O., Sanakis, Y., & Fontecave, M. (2006) · Journal of Biological Chemistry · doi:10.1074/jbc.m513569200 · Cited by 257 · see citing works

Crystal Structures of Mitochondrial Processing Peptidase Reveal the Mode for Specific Cleavage of Import Signal Sequences

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Crystal Structure of Human Frataxin

Dhe-Paganon, S., Shigeta, R., Chi, Y.-I., Ristow, M., & Shoelson, S. E. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.c000407200 · Cited by 226 · see citing works

Frataxin activates mitochondrial energy conversion and oxidative phosphorylation

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Friedreich Ataxia Mouse Models with Progressive Cerebellar and Sensory Ataxia Reveal Autophagic Neurodegeneration in Dorsal Root Ganglia

Simon, D., Seznec, H., Gansmuller, A., Carelle, N., Weber, P., Metzger, D., Rustin, P., Koenig, M., & Puccio, H. (2004) · The Journal of Neuroscience · doi:10.1523/jneurosci.4549-03.2004 · Cited by 205 · 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

Disabled early recruitment of antioxidant defenses in Friedreich's ataxia

Chantrel-Groussard, K. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.19.2061 · Cited by 189 · see citing works

Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity

Gakh, O., Park, S., Liu, G., Macomber, L., Imlay, J. A., Ferreira, G. C., & Isaya, G. (2006) · Human Molecular Genetics · doi:10.1093/hmg/ddi461 · Cited by 184 · see citing works

Sticky DNA, a Self-associated Complex Formed at Long GAA·TTC Repeats in Intron 1 of the Frataxin Gene, Inhibits Transcription

Sakamoto, N., Ohshima, K., Montermini, L., Pandolfo, M., & Wells, R. D. (2001) · Journal of Biological Chemistry · doi:10.1074/jbc.m101879200 · Cited by 181 · see citing works

Iron–sulfur protein maturation in human cells: evidence for a function of frataxin

Stehling, O., Elsässer, H.-P., Brückel, B., Mühlenhoff, U., & Lill, R. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddh324 · Cited by 176 · see citing works

Towards a structural understanding of Friedreich’s ataxia: the solution structure of frataxin

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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

Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: Evidence that Yfh1p affects Fe-S cluster synthesis

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

Reversible redox-dependent modulation of mitochondrial aconitase and proteolytic activity during in vivo cardiac ischemia/reperfusion

Bulteau, A., Lundberg, K. C., Ikeda‐Saito, M., Isaya, G., & Szweda, L. I. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0501519102 · Cited by 131 · see citing works

Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase

Koutníková, H. (1998) · Human Molecular Genetics · doi:10.1093/hmg/7.9.1485 · Cited by 122 · see citing works

Two-step Processing of Human Frataxin by Mitochondrial Processing Peptidase

Cavadini, P., Adamec, J., Taroni, F., Gakh, O., & Isaya, G. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.m006539200 · Cited by 114 · see citing works

Yeast and Human Frataxin Are Processed to Mature Form in Two Sequential Steps by the Mitochondrial Processing Peptidase

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

Iron‐induced oligomerization of yeast frataxin homologue Yfh1 is dispensable in vivo

Aloria, K., Schilke, B. A., Andrew, A. J., & Craig, E. A. (2004) · EMBO Reports · doi:10.1038/sj.embor.7400272 · Cited by 104 · see citing works

A Pool of Extramitochondrial Frataxin That Promotes Cell Survival

Condò, I., Ventura, N., Malisan, F., Tomassini, B., & Testi, R. (2006) · Journal of Biological Chemistry · doi:10.1074/jbc.m511960200 · Cited by 90 · see citing works

Frataxin expression rescues mitochondrial dysfunctions in FRDA cells

Tan, G. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.19.2099 · Cited by 82 · see citing works

Friedreich ataxia

Pandolfo, M. (2003) · Seminars in Pediatric Neurology · doi:10.1016/s1071-9091(03)00025-1 · Cited by 81 · see citing works

Import of rat ornithine transcarbamylase precursor into mitochondria: two-step processing of the leader peptide.

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Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells

Jiralerspong, S., Ge, B., Hudson, T. J., & Pandolfo, M. (2001) · FEBS Letters · doi:10.1016/s0014-5793(01)03140-4 · Cited by 78 · see citing works

Supramolecular Assemblies of Human Frataxin are Formed via Subunit–Subunit Interactions Mediated by a Non-conserved Amino-terminal Region

O’Neill, H. A., Gakh, O., & Isaya, G. (2004) · Journal of Molecular Biology · doi:10.1016/j.jmb.2004.10.074 · Cited by 57 · see citing works

Maturation of Frataxin Within Mammalian and Yeast Mitochondria: One-Step Processing by Matrix Processing Peptidase

Gordon, D. M., Shi, Q., Dancis, A., & Pain, D. (1999) · Human Molecular Genetics · doi:10.1093/hmg/8.12.2255 · Cited by 46 · see citing works

N-terminal iron-mediated self-cleavage of human frataxin: regulation of iron binding and complex formation with target proteins

Yoon, T., Dizin, E., & Cowan, J. A. (2007) · JBIC Journal of Biological Inorganic Chemistry · doi:10.1007/s00775-007-0205-2 · Cited by 41 · 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

Friedreich's ataxia: Pathology, pathogenesis, and molecular genetics

Koeppen, A. H. (2011) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2011.01.010 · Cited by 447 · see citing works

Aconitases: non-redox iron-sulfur proteins sensitive to reactive species

Castro, L., Tórtora, V., Mansilla, S., & Radi, R. (2019) · Accounts of Chemical Research · doi:10.1021/acs.accounts.9b00150 · Cited by 146 · see citing works

Advancements in the pathophysiology of Friedreich’s Ataxia and new prospects for treatments

Babady, N. E., Carelle, N., Wells, R. D., Rouault, T. A., Hirano, M., Lynch, D. R., Delatycki, M. B., Wilson, R. B., Isaya, G., & Puccio, H. (2007) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2007.05.009 · Cited by 73 · 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

Mrs3p, Mrs4p, and Frataxin Provide Iron for Fe-S Cluster Synthesis in Mitochondria

Zhang, Y., Lyver, E. R., Knight, S. A. B., Pain, D., Lesuisse, E., & Dancis, A. (2006) · Journal of Biological Chemistry · doi:10.1074/jbc.m604246200 · Cited by 98 · see citing works

Iron–sulfur protein maturation in human cells: evidence for a function of frataxin

Stehling, O., Elsässer, H.-P., Brückel, B., Mühlenhoff, U., & Lill, R. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddh324 · Cited by 176 · see citing works

Frataxin Acts as an Iron Chaperone Protein to Modulate Mitochondrial Aconitase Activity

Bulteau, A.-L., O’Neill, H. A., Kennedy, M. C., Ikeda‐Saito, M., Isaya, G., & Szweda, L. I. (2004) · Science · doi:10.1126/science.1098991 · Cited by 372 · 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