Stéphane Schmucker, Manuela Argentini, Nadège Carelle-Calmels, Alain Martelli, Hélène Puccio. Hum Mol Genet 2008
Times Cited: 95
Times Cited: 95
Times Cited
Times Co-cited
Similarity
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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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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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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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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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
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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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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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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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Two-step processing of human frataxin by mitochondrial processing peptidase. Precursor and intermediate forms are cleaved at different rates.
P Cavadini, J Adamec, F Taroni, O Gakh, G Isaya. J Biol Chem 2000
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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
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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
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Normal and Friedreich ataxia cells express different isoforms of frataxin with complementary roles in iron-sulfur cluster assembly.
Oleksandr Gakh, Tibor Bedekovics, Samantha F Duncan, Douglas Y Smith, Donald S Berkholz, Grazia Isaya. J Biol Chem 2010
Oleksandr Gakh, Tibor Bedekovics, Samantha F Duncan, Douglas Y Smith, Donald S Berkholz, Grazia Isaya. J Biol Chem 2010
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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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Friedreich's ataxia: point mutations and clinical presentation of compound heterozygotes.
M Cossée, A Dürr, M Schmitt, N Dahl, P Trouillas, P Allinson, M Kostrzewa, A Nivelon-Chevallier, K H Gustavson, A Kohlschütter,[...]. Ann Neurol 1999
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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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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
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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
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Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia.
Patrizia Cavadini, Heather A O'Neill, Oldrich Benada, Grazia Isaya. Hum Mol Genet 2002
Patrizia Cavadini, Heather A O'Neill, Oldrich Benada, Grazia Isaya. Hum Mol Genet 2002
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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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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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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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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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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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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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The structures of frataxin oligomers reveal the mechanism for the delivery and detoxification of iron.
Tobias Karlberg, Ulrika Schagerlöf, Oleksandr Gakh, Sungjo Park, Ulf Ryde, Martin Lindahl, Kirstin Leath, Elspeth Garman, Grazia Isaya, Salam Al-Karadaghi. Structure 2006
Tobias Karlberg, Ulrika Schagerlöf, Oleksandr Gakh, Sungjo Park, Ulf Ryde, Martin Lindahl, Kirstin Leath, Elspeth Garman, Grazia Isaya, Salam Al-Karadaghi. Structure 2006
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Oligomeric yeast frataxin drives assembly of core machinery for mitochondrial iron-sulfur cluster synthesis.
Hongqiao Li, Oleksandr Gakh, Douglas Y Smith, Grazia Isaya. J Biol Chem 2009
Hongqiao Li, Oleksandr Gakh, Douglas Y Smith, Grazia Isaya. J Biol Chem 2009
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The relationship between trinucleotide (GAA) repeat length and clinical features in Friedreich ataxia.
A Filla, G De Michele, F Cavalcanti, L Pianese, A Monticelli, G Campanella, S Cocozza. Am J Hum Genet 1996
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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
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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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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
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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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N-terminal iron-mediated self-cleavage of human frataxin: regulation of iron binding and complex formation with target proteins.
Taejin Yoon, Eric Dizin, J A Cowan. J Biol Inorg Chem 2007
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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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Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones.
Yuxi Shan, Eleonora Napoli, Gino Cortopassi. Hum Mol Genet 2007
Yuxi Shan, Eleonora Napoli, Gino Cortopassi. Hum Mol Genet 2007
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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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The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis.
A Wong, J Yang, P Cavadini, C Gellera, B Lonnerdal, F Taroni, G Cortopassi. Hum Mol Genet 1999
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Frataxin gene point mutations in Italian Friedreich ataxia patients.
Cinzia Gellera, Barbara Castellotti, Caterina Mariotti, Rossana Mineri, Viviana Seveso, Stefano Didonato, Franco Taroni. Neurogenetics 2007
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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
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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
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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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Oxidative stress in patients with Friedreich ataxia.
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Iron-sulfur cluster biosynthesis: characterization of Escherichia coli CYaY as an iron donor for the assembly of [2Fe-2S] clusters in the scaffold IscU.
Gunhild Layer, Sandrine Ollagnier-de Choudens, Yiannis Sanakis, Marc Fontecave. J Biol Chem 2006
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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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Friedreich's ataxia: pathology, pathogenesis, and molecular genetics.
Arnulf H Koeppen. J Neurol Sci 2011
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Dynamics, stability and iron-binding activity of frataxin clinical mutants.
Ana R Correia, Chiara Pastore, Salvatore Adinolfi, Annalisa Pastore, Cláudio M Gomes. FEBS J 2008
Ana R Correia, Chiara Pastore, Salvatore Adinolfi, Annalisa Pastore, Cláudio M Gomes. FEBS J 2008
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Conformational stability of human frataxin and effect of Friedreich's ataxia-related mutations on protein folding.
Ana R Correia, Salvatore Adinolfi, Annalisa Pastore, Cláudio M Gomes. Biochem J 2006
Ana R Correia, Salvatore Adinolfi, Annalisa Pastore, Cláudio M Gomes. Biochem J 2006
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Structural basis of the iron storage function of frataxin from single-particle reconstruction of the iron-loaded oligomer.
Ulrika Schagerlöf, Hans Elmlund, Oleksandr Gakh, Gustav Nordlund, Hans Hebert, Martin Lindahl, Grazia Isaya, Salam Al-Karadaghi. Biochemistry 2008
Ulrika Schagerlöf, Hans Elmlund, Oleksandr Gakh, Gustav Nordlund, Hans Hebert, Martin Lindahl, Grazia Isaya, Salam Al-Karadaghi. Biochemistry 2008
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Supramolecular assemblies of human frataxin are formed via subunit-subunit interactions mediated by a non-conserved amino-terminal region.
Heather A O'Neill, Oleksandr Gakh, Grazia Isaya. J Mol Biol 2005
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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.