Annalisa Pastore, Helene Puccio. J Neurochem 2013
Times Cited: 128
Times Cited: 128
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
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
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
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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Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex.
Chi-Lin Tsai, David P Barondeau. Biochemistry 2010
Chi-Lin Tsai, David P Barondeau. Biochemistry 2010
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Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS.
Salvatore Adinolfi, Clara Iannuzzi, Filippo Prischi, Chiara Pastore, Stefania Iametti, Stephen R Martin, Franco Bonomi, Annalisa Pastore. Nat Struct Mol Biol 2009
Salvatore Adinolfi, Clara Iannuzzi, Filippo Prischi, Chiara Pastore, Stefania Iametti, Stephen R Martin, Franco Bonomi, Annalisa Pastore. Nat Struct Mol Biol 2009
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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
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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Structural bases for the interaction of frataxin with the central components of iron-sulphur cluster assembly.
Filippo Prischi, Petr V Konarev, Clara Iannuzzi, Chiara Pastore, Salvatore Adinolfi, Stephen R Martin, Dmitri I Svergun, Annalisa Pastore. Nat Commun 2010
Filippo Prischi, Petr V Konarev, Clara Iannuzzi, Chiara Pastore, Salvatore Adinolfi, Stephen R Martin, Dmitri I Svergun, Annalisa Pastore. Nat Commun 2010
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Human frataxin activates Fe-S cluster biosynthesis by facilitating sulfur transfer chemistry.
Jennifer Bridwell-Rabb, Nicholas G Fox, Chi-Lin Tsai, Andrew M Winn, David P Barondeau. Biochemistry 2014
Jennifer Bridwell-Rabb, Nicholas G Fox, Chi-Lin Tsai, Andrew M Winn, David P Barondeau. Biochemistry 2014
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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
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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Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins.
Taejin Yoon, J A Cowan. J Am Chem Soc 2003
Taejin Yoon, J A Cowan. J Am Chem Soc 2003
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Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis.
Taejin Yoon, J A Cowan. J Biol Chem 2004
Taejin Yoon, J A Cowan. J Biol Chem 2004
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Clinical features of Friedreich's ataxia: classical and atypical phenotypes.
Michael H Parkinson, Sylvia Boesch, Wolfgang Nachbauer, Caterina Mariotti, Paola Giunti. J Neurochem 2013
Michael H Parkinson, Sylvia Boesch, Wolfgang Nachbauer, Caterina Mariotti, Paola Giunti. J Neurochem 2013
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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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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
Jana Gerber, Ulrich Mühlenhoff, Roland Lill. EMBO Rep 2003
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Functional reconstitution of mitochondrial Fe/S cluster synthesis on Isu1 reveals the involvement of ferredoxin.
Holger Webert, Sven-Andreas Freibert, Angelo Gallo, Torsten Heidenreich, Uwe Linne, Stefan Amlacher, Ed Hurt, Ulrich Mühlenhoff, Lucia Banci, Roland Lill. Nat Commun 2014
Holger Webert, Sven-Andreas Freibert, Angelo Gallo, Torsten Heidenreich, Uwe Linne, Stefan Amlacher, Ed Hurt, Ulrich Mühlenhoff, Lucia Banci, Roland Lill. Nat Commun 2014
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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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Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity.
Anne-Laure Bulteau, Heather A O'Neill, Mary Claire Kennedy, Masao Ikeda-Saito, Grazia Isaya, Luke I Szweda. Science 2004
Anne-Laure Bulteau, Heather A O'Neill, Mary Claire Kennedy, Masao Ikeda-Saito, Grazia Isaya, Luke I Szweda. Science 2004
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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
Ivano Condò, Natascia Ventura, Florence Malisan, Alessandra Rufini, Barbara Tomassini, Roberto Testi. Hum Mol Genet 2007
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The pathogenesis of Friedreich ataxia and the structure and function of frataxin.
Massimo Pandolfo, Annalisa Pastore. J Neurol 2009
Massimo Pandolfo, Annalisa Pastore. J Neurol 2009
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Structural basis for Fe-S cluster assembly and tRNA thiolation mediated by IscS protein-protein interactions.
Rong Shi, Ariane Proteau, Magda Villarroya, Ismaïl Moukadiri, Linhua Zhang, Jean-François Trempe, Allan Matte, M Eugenia Armengod, Miroslaw Cygler. PLoS Biol 2010
Rong Shi, Ariane Proteau, Magda Villarroya, Ismaïl Moukadiri, Linhua Zhang, Jean-François Trempe, Allan Matte, M Eugenia Armengod, Miroslaw Cygler. PLoS Biol 2010
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The role of mitochondria in cellular iron-sulfur protein biogenesis and iron metabolism.
Roland Lill, Bastian Hoffmann, Sabine Molik, Antonio J Pierik, Nicole Rietzschel, Oliver Stehling, Marta A Uzarska, Holger Webert, Claudia Wilbrecht, Ulrich Mühlenhoff. Biochim Biophys Acta 2012
Roland Lill, Bastian Hoffmann, Sabine Molik, Antonio J Pierik, Nicole Rietzschel, Oliver Stehling, Marta A Uzarska, Holger Webert, Claudia Wilbrecht, Ulrich Mühlenhoff. Biochim Biophys Acta 2012
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Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease.
Tracey A Rouault. Dis Model Mech 2012
Tracey A Rouault. Dis Model Mech 2012
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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
Rachael A Vaubel, Grazia Isaya. Mol Cell Neurosci 2013
14
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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Structure, function, and formation of biological iron-sulfur clusters.
Deborah C Johnson, Dennis R Dean, Archer D Smith, Michael K Johnson. Annu Rev Biochem 2005
Deborah C Johnson, Dennis R Dean, Archer D Smith, Michael K Johnson. Annu Rev Biochem 2005
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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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Iron-sulfur cluster biogenesis and trafficking in mitochondria.
Joseph J Braymer, Roland Lill. J Biol Chem 2017
Joseph J Braymer, Roland Lill. J Biol Chem 2017
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Components involved in assembly and dislocation of iron-sulfur clusters on the scaffold protein Isu1p.
Ulrich Mühlenhoff, Jana Gerber, Nadine Richhardt, Roland Lill. EMBO J 2003
Ulrich Mühlenhoff, Jana Gerber, Nadine Richhardt, Roland Lill. EMBO J 2003
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The mitochondrial Hsp70 chaperone Ssq1 facilitates Fe/S cluster transfer from Isu1 to Grx5 by complex formation.
Marta A Uzarska, Rafal Dutkiewicz, Sven-Andreas Freibert, Roland Lill, Ulrich Mühlenhoff. Mol Biol Cell 2013
Marta A Uzarska, Rafal Dutkiewicz, Sven-Andreas Freibert, Roland Lill, Ulrich Mühlenhoff. Mol Biol Cell 2013
13
Friedreich's ataxia: pathology, pathogenesis, and molecular genetics.
Arnulf H Koeppen. J Neurol Sci 2011
Arnulf H Koeppen. J Neurol Sci 2011
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Towards a structural understanding of Friedreich's ataxia: the solution structure of frataxin.
G Musco, G Stier, B Kolmerer, S Adinolfi, S Martin, T Frenkiel, T Gibson, A Pastore. Structure 2000
G Musco, G Stier, B Kolmerer, S Adinolfi, S Martin, T Frenkiel, T Gibson, A Pastore. Structure 2000
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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
David Herman, Kai Jenssen, Ryan Burnett, Elisabetta Soragni, Susan L Perlman, Joel M Gottesfeld. Nat Chem Biol 2006
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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
S Dhe-Paganon, R Shigeta, Y I Chi, M Ristow, S E Shoelson. J Biol Chem 2000
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Friedreich ataxia: neuropathology revised.
Arnulf H Koeppen, Joseph E Mazurkiewicz. J Neuropathol Exp Neurol 2013
Arnulf H Koeppen, Joseph E Mazurkiewicz. J Neuropathol Exp Neurol 2013
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Fixing frataxin: 'ironing out' the metabolic defect in Friedreich's ataxia.
A Anzovino, D J R Lane, M L-H Huang, D R Richardson. Br J Pharmacol 2014
A Anzovino, D J R Lane, M L-H Huang, D R Richardson. Br J Pharmacol 2014
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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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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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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
Emmanuel Lesuisse, Renata Santos, Berthold F Matzanke, Simon A B Knight, Jean-Michel Camadro, Andrew Dancis. Hum Mol Genet 2003
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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
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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Oxidative stress and altered lipid metabolism in Friedreich ataxia.
Jordi Tamarit, Èlia Obis, Joaquim Ros. Free Radic Biol Med 2016
Jordi Tamarit, Èlia Obis, Joaquim Ros. Free Radic Biol Med 2016
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The structure and function of frataxin.
Krisztina Z Bencze, Kalyan C Kondapalli, Jeremy D Cook, Stephen McMahon, César Millán-Pacheco, Nina Pastor, Timothy L Stemmler. Crit Rev Biochem Mol Biol 2006
Krisztina Z Bencze, Kalyan C Kondapalli, Jeremy D Cook, Stephen McMahon, César Millán-Pacheco, Nina Pastor, Timothy L Stemmler. Crit Rev Biochem Mol Biol 2006
11
Humans possess two mitochondrial ferredoxins, Fdx1 and Fdx2, with distinct roles in steroidogenesis, heme, and Fe/S cluster biosynthesis.
Alex D Sheftel, Oliver Stehling, Antonio J Pierik, Hans-Peter Elsässer, Ulrich Mühlenhoff, Holger Webert, Anna Hobler, Frank Hannemann, Rita Bernhardt, Roland Lill. Proc Natl Acad Sci U S A 2010
Alex D Sheftel, Oliver Stehling, Antonio J Pierik, Hans-Peter Elsässer, Ulrich Mühlenhoff, Holger Webert, Anna Hobler, Frank Hannemann, Rita Bernhardt, Roland Lill. Proc Natl Acad Sci U S A 2010
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Maturation of cytosolic and nuclear iron-sulfur proteins.
Daili J A Netz, Judita Mascarenhas, Oliver Stehling, Antonio J Pierik, Roland Lill. Trends Cell Biol 2014
Daili J A Netz, Judita Mascarenhas, Oliver Stehling, Antonio J Pierik, Roland Lill. Trends Cell Biol 2014
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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
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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Solution structure of the bacterial frataxin ortholog, CyaY: mapping the iron binding sites.
Margie Nair, Salvatore Adinolfi, Chiara Pastore, Geoff Kelly, Pierandrea Temussi, Annalisa Pastore. Structure 2004
Margie Nair, Salvatore Adinolfi, Chiara Pastore, Geoff Kelly, Pierandrea Temussi, Annalisa Pastore. Structure 2004
11
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
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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Molecular control of the cytosolic aconitase/IRP1 switch by extramitochondrial frataxin.
Ivano Condò, Florence Malisan, Ilaria Guccini, Dario Serio, Alessandra Rufini, Roberto Testi. Hum Mol Genet 2010
Ivano Condò, Florence Malisan, Ilaria Guccini, Dario Serio, Alessandra Rufini, Roberto Testi. Hum Mol Genet 2010
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Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase.
H Koutnikova, V Campuzano, M Koenig. Hum Mol Genet 1998
H Koutnikova, V Campuzano, M Koenig. Hum Mol Genet 1998
11
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
12
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.