Anne-Laure Bulteau, Heather A O'Neill, Mary Claire Kennedy, Masao Ikeda-Saito, Grazia Isaya, Luke I Szweda. Science 2004
Times Cited: 289
Times Cited: 289
Times Cited
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Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion.
V Campuzano, L Montermini, M D Moltò, L Pianese, M Cossée, F Cavalcanti, E Monros, F Rodius, F Duclos, A Monticelli,[...]. Science 1996
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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia.
A Rötig, P de Lonlay, D Chretien, F Foury, M Koenig, D Sidi, A Munnich, P Rustin. Nat Genet 1997
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Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes.
V Campuzano, L Montermini, Y Lutz, L Cova, C Hindelang, S Jiralerspong, Y Trottier, S J Kish, B Faucheux, P Trouillas,[...]. Hum Mol Genet 1997
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Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin.
M Babcock, D de Silva, R Oaks, S Davis-Kaplan, S Jiralerspong, L Montermini, M Pandolfo, J Kaplan. Science 1997
M Babcock, D de Silva, R Oaks, S Davis-Kaplan, S Jiralerspong, L Montermini, M Pandolfo, J Kaplan. Science 1997
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Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex.
Stéphane Schmucker, Alain Martelli, Florent Colin, Adeline Page, Marie Wattenhofer-Donzé, Laurence Reutenauer, Hélène Puccio. PLoS One 2011
Stéphane Schmucker, Alain Martelli, Florent Colin, Adeline Page, Marie Wattenhofer-Donzé, Laurence Reutenauer, Hélène Puccio. PLoS One 2011
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Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits.
H Puccio, D Simon, M Cossée, P Criqui-Filipe, F Tiziano, J Melki, C Hindelang, R Matyas, P Rustin, M Koenig. Nat Genet 2001
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Friedreich ataxia: neuropathology revised.
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Crystal structure of human frataxin.
S Dhe-Paganon, R Shigeta, Y I Chi, M Ristow, S E Shoelson. J Biol Chem 2000
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Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1).
Emmanuel Lesuisse, Renata Santos, Berthold F Matzanke, Simon A B Knight, Jean-Michel Camadro, Andrew Dancis. Hum Mol Genet 2003
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An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1.
Jana Gerber, Ulrich Mühlenhoff, Roland Lill. EMBO Rep 2003
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Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex.
Chi-Lin Tsai, David P Barondeau. Biochemistry 2010
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Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia.
Morgane Perdomini, Brahim Belbellaa, Laurent Monassier, Laurence Reutenauer, Nadia Messaddeq, Nathalie Cartier, Ronald G Crystal, Patrick Aubourg, Hélène Puccio. Nat Med 2014
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Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin.
H Koutnikova, V Campuzano, F Foury, P Dollé, O Cazzalini, M Koenig. Nat Genet 1997
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Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS.
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Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia.
Rachael A Vaubel, Grazia Isaya. Mol Cell Neurosci 2013
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In vivo maturation of human frataxin.
Ivano Condò, Natascia Ventura, Florence Malisan, Alessandra Rufini, Barbara Tomassini, Roberto Testi. Hum Mol Genet 2007
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The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis.
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Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation.
M Cossée, H Puccio, A Gansmuller, H Koutnikova, A Dierich, M LeMeur, K Fischbeck, P Dollé, M Koenig. Hum Mol Genet 2000
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Frataxin interacts functionally with mitochondrial electron transport chain proteins.
Pilar González-Cabo, Rafael P Vázquez-Manrique, M Adelaida García-Gimeno, Pascual Sanz, Francesc Palau. Hum Mol Genet 2005
Pilar González-Cabo, Rafael P Vázquez-Manrique, M Adelaida García-Gimeno, Pascual Sanz, Francesc Palau. Hum Mol Genet 2005
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Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model.
Yuxi Shan, Robert A Schoenfeld, Genki Hayashi, Eleonora Napoli, Tasuku Akiyama, Mirela Iodi Carstens, Earl E Carstens, Mark A Pook, Gino A Cortopassi. Antioxid Redox Signal 2013
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Iron-dependent self-assembly of recombinant yeast frataxin: implications for Friedreich ataxia.
J Adamec, F Rusnak, W G Owen, S Naylor, L M Benson, A M Gacy, G Isaya. Am J Hum Genet 2000
J Adamec, F Rusnak, W G Owen, S Naylor, L M Benson, A M Gacy, G Isaya. Am J Hum Genet 2000
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Frataxin activates mitochondrial energy conversion and oxidative phosphorylation.
M Ristow, M F Pfister, A J Yee, M Schubert, L Michael, C Y Zhang, K Ueki, M D Michael, B B Lowell, C R Kahn. Proc Natl Acad Sci U S A 2000
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Clinical and genetic abnormalities in patients with Friedreich's ataxia.
A Dürr, M Cossee, Y Agid, V Campuzano, C Mignard, C Penet, J L Mandel, A Brice, M Koenig. N Engl J Med 1996
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GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology.
Sahar Al-Mahdawi, Ricardo Mouro Pinto, Dhaval Varshney, Lorraine Lawrence, Margaret B Lowrie, Sian Hughes, Zoe Webster, Julian Blake, J Mark Cooper, Rosalind King,[...]. Genomics 2006
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Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia.
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The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues.
Sahar Al-Mahdawi, Ricardo Mouro Pinto, Ozama Ismail, Dhaval Varshney, Stefania Lymperi, Chiranjeevi Sandi, Daniah Trabzuni, Mark Pook. Hum Mol Genet 2008
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Frataxin and mitochondrial FeS cluster biogenesis.
Timothy L Stemmler, Emmanuel Lesuisse, Debkumar Pain, Andrew Dancis. J Biol Chem 2010
Timothy L Stemmler, Emmanuel Lesuisse, Debkumar Pain, Andrew Dancis. J Biol Chem 2010
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Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia.
David Herman, Kai Jenssen, Ryan Burnett, Elisabetta Soragni, Susan L Perlman, Joel M Gottesfeld. Nat Chem Biol 2006
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Mammalian frataxin controls sulfur production and iron entry during de novo Fe4S4 cluster assembly.
Florent Colin, Alain Martelli, Martin Clémancey, Jean-Marc Latour, Serge Gambarelli, Laura Zeppieri, Catherine Birck, Adeline Page, Hélène Puccio, Sandrine Ollagnier de Choudens. J Am Chem Soc 2013
Florent Colin, Alain Martelli, Martin Clémancey, Jean-Marc Latour, Serge Gambarelli, Laura Zeppieri, Catherine Birck, Adeline Page, Hélène Puccio, Sandrine Ollagnier de Choudens. J Am Chem Soc 2013
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Monomeric yeast frataxin is an iron-binding protein.
Jeremy D Cook, Krisztina Z Bencze, Ana D Jankovic, Anna K Crater, Courtney N Busch, Patrick B Bradley, Ann J Stemmler, Mark R Spaller, Timothy L Stemmler. Biochemistry 2006
Jeremy D Cook, Krisztina Z Bencze, Ana D Jankovic, Anna K Crater, Courtney N Busch, Patrick B Bradley, Ann J Stemmler, Mark R Spaller, Timothy L Stemmler. Biochemistry 2006
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Oxidative stress in patients with Friedreich ataxia.
J B Schulz, T Dehmer, L Schöls, H Mende, C Hardt, M Vorgerd, K Bürk, W Matson, J Dichgans, M F Beal,[...]. Neurology 2000
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Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant.
Michael Li-Hsuan Huang, Erika M Becker, Megan Whitnall, Yohan Suryo Rahmanto, Prem Ponka, Des R Richardson. Proc Natl Acad Sci U S A 2009
Michael Li-Hsuan Huang, Erika M Becker, Megan Whitnall, Yohan Suryo Rahmanto, Prem Ponka, Des R Richardson. Proc Natl Acad Sci U S A 2009
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Yeast frataxin sequentially chaperones and stores iron by coupling protein assembly with iron oxidation.
Sungjo Park, Oleksandr Gakh, Heather A O'Neill, Arianna Mangravita, Helen Nichol, Gloria C Ferreira, Grazia Isaya. J Biol Chem 2003
Sungjo Park, Oleksandr Gakh, Heather A O'Neill, Arianna Mangravita, Helen Nichol, Gloria C Ferreira, Grazia Isaya. J Biol Chem 2003
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Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features.
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Causative role of oxidative stress in a Drosophila model of Friedreich ataxia.
José V Llorens, Juan A Navarro, Maria J Martínez-Sebastián, Mary K Baylies, S Schneuwly, José A Botella, Maria D Moltó. FASEB J 2007
José V Llorens, Juan A Navarro, Maria J Martínez-Sebastián, Mary K Baylies, S Schneuwly, José A Botella, Maria D Moltó. FASEB J 2007
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Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity.
Oleksandr Gakh, Sungjo Park, Gang Liu, Lee Macomber, James A Imlay, Gloria C Ferreira, Grazia Isaya. Hum Mol Genet 2006
Oleksandr Gakh, Sungjo Park, Gang Liu, Lee Macomber, James A Imlay, Gloria C Ferreira, Grazia Isaya. Hum Mol Genet 2006
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Iron-sulfur protein maturation in human cells: evidence for a function of frataxin.
Oliver Stehling, Hans-Peter Elsässer, Bernd Brückel, Ulrich Mühlenhoff, Roland Lill. Hum Mol Genet 2004
Oliver Stehling, Hans-Peter Elsässer, Bernd Brückel, Ulrich Mühlenhoff, Roland Lill. Hum Mol Genet 2004
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Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation.
Alain Martelli, Hélène Puccio. Front Pharmacol 2014
Alain Martelli, Hélène Puccio. Front Pharmacol 2014
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Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxia.
Vincent Paupe, Emmanuel P Dassa, Sergio Goncalves, Françoise Auchère, Maria Lönn, Arne Holmgren, Pierre Rustin. PLoS One 2009
Vincent Paupe, Emmanuel P Dassa, Sergio Goncalves, Françoise Auchère, Maria Lönn, Arne Holmgren, Pierre Rustin. PLoS One 2009
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Redox-dependent modulation of aconitase activity in intact mitochondria.
Anne-Laure Bulteau, Masao Ikeda-Saito, Luke I Szweda. Biochemistry 2003
Anne-Laure Bulteau, Masao Ikeda-Saito, Luke I Szweda. Biochemistry 2003
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Mitochondrial iron trafficking and the integration of iron metabolism between the mitochondrion and cytosol.
Des R Richardson, Darius J R Lane, Erika M Becker, Michael L-H Huang, Megan Whitnall, Yohan Suryo Rahmanto, Alex D Sheftel, Prem Ponka. Proc Natl Acad Sci U S A 2010
Des R Richardson, Darius J R Lane, Erika M Becker, Michael L-H Huang, Megan Whitnall, Yohan Suryo Rahmanto, Alex D Sheftel, Prem Ponka. Proc Natl Acad Sci U S A 2010
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Yeast frataxin solution structure, iron binding, and ferrochelatase interaction.
Yanan He, Steven L Alam, Simona V Proteasa, Yan Zhang, Emmanuel Lesuisse, Andrew Dancis, Timothy L Stemmler. Biochemistry 2004
Yanan He, Steven L Alam, Simona V Proteasa, Yan Zhang, Emmanuel Lesuisse, Andrew Dancis, Timothy L Stemmler. Biochemistry 2004
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A structural approach to understanding the iron-binding properties of phylogenetically different frataxins.
S Adinolfi, M Trifuoggi, A S Politou, S Martin, A Pastore. Hum Mol Genet 2002
S Adinolfi, M Trifuoggi, A S Politou, S Martin, A Pastore. Hum Mol Genet 2002
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The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S proteins.
Ulrich Mühlenhoff, Nadine Richhardt, Michael Ristow, Gyula Kispal, Roland Lill. Hum Mol Genet 2002
Ulrich Mühlenhoff, Nadine Richhardt, Michael Ristow, Gyula Kispal, Roland Lill. Hum Mol Genet 2002
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Selective iron chelation in Friedreich ataxia: biologic and clinical implications.
Nathalie Boddaert, Kim Hanh Le Quan Sang, Agnès Rötig, Anne Leroy-Willig, Serge Gallet, Francis Brunelle, Daniel Sidi, Jean-Christophe Thalabard, Arnold Munnich, Z Ioav Cabantchik. Blood 2007
Nathalie Boddaert, Kim Hanh Le Quan Sang, Agnès Rötig, Anne Leroy-Willig, Serge Gallet, Francis Brunelle, Daniel Sidi, Jean-Christophe Thalabard, Arnold Munnich, Z Ioav Cabantchik. Blood 2007
10
Co-cited is the co-citation frequency, indicating how many articles cite the article together with the query article. Similarity is the co-citation as percentage of the times cited of the query article or the article in the search results, whichever is the lowest. These numbers are calculated for the last 100 citations when articles are cited more than 100 times.