Or Kakhlon, Hila Manning, William Breuer, Naomi Melamed-Book, Chunye Lu, Gino Cortopassi, Arnold Munnich, Z Ioav Cabantchik. Blood 2008
Times Cited: 96
Times Cited: 96
Times Cited
Times Co-cited
Similarity
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
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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
A Rötig, P de Lonlay, D Chretien, F Foury, M Koenig, D Sidi, A Munnich, P Rustin. Nat 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
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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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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Redistribution of accumulated cell iron: a modality of chelation with therapeutic implications.
Yang-Sung Sohn, William Breuer, Arnold Munnich, Z Ioav Cabantchik. Blood 2008
Yang-Sung Sohn, William Breuer, Arnold Munnich, Z Ioav Cabantchik. Blood 2008
28
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
A Wong, J Yang, P Cavadini, C Gellera, B Lonnerdal, F Taroni, G Cortopassi. Hum Mol Genet 1999
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Iron-dependent regulation of frataxin expression: implications for treatment of Friedreich ataxia.
Kuanyu Li, Edward K Besse, Dung Ha, Gennadiy Kovtunovych, Tracey A Rouault. Hum Mol Genet 2008
Kuanyu Li, Edward K Besse, Dung Ha, Gennadiy Kovtunovych, Tracey A Rouault. Hum Mol Genet 2008
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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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Deferiprone targets aconitase: implication for Friedreich's ataxia treatment.
Sergio Goncalves, Vincent Paupe, Emmanuel P Dassa, Pierre Rustin. BMC Neurol 2008
Sergio Goncalves, Vincent Paupe, Emmanuel P Dassa, Pierre Rustin. BMC Neurol 2008
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Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia.
J L Bradley, J C Blake, S Chamberlain, P K Thomas, J M Cooper, A H Schapira. Hum Mol Genet 2000
J L Bradley, J C Blake, S Chamberlain, P K Thomas, J M Cooper, A H Schapira. Hum Mol Genet 2000
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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
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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Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a randomised, placebo-controlled trial.
Nicholas A Di Prospero, Angela Baker, Neal Jeffries, Kenneth H Fischbeck. Lancet Neurol 2007
Nicholas A Di Prospero, Angela Baker, Neal Jeffries, Kenneth H Fischbeck. Lancet Neurol 2007
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Oxidative stress in patients with Friedreich ataxia.
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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
20
Combined therapy with idebenone and deferiprone in patients with Friedreich's ataxia.
Daniel Velasco-Sánchez, Asuncion Aracil, Raquel Montero, Ana Mas, Lorenzo Jiménez, Mar O'Callaghan, Maria Tondo, Antoni Capdevila, Josep Blanch, Rafael Artuch,[...]. Cerebellum 2011
Daniel Velasco-Sánchez, Asuncion Aracil, Raquel Montero, Ana Mas, Lorenzo Jiménez, Mar O'Callaghan, Maria Tondo, Antoni Capdevila, Josep Blanch, Rafael Artuch,[...]. Cerebellum 2011
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Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study.
P Rustin, J C von Kleist-Retzow, K Chantrel-Groussard, D Sidi, A Munnich, A Rötig. Lancet 1999
P Rustin, J C von Kleist-Retzow, K Chantrel-Groussard, D Sidi, A Munnich, A Rötig. Lancet 1999
19
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
19
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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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
18
Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.
R Lodi, J M Cooper, J L Bradley, D Manners, P Styles, D J Taylor, A H Schapira. Proc Natl Acad Sci U S A 1999
R Lodi, J M Cooper, J L Bradley, D Manners, P Styles, D J Taylor, A H Schapira. Proc Natl Acad Sci U S A 1999
18
The MCK mouse heart model of Friedreich's ataxia: Alterations in iron-regulated proteins and cardiac hypertrophy are limited by iron chelation.
Megan Whitnall, Yohan Suryo Rahmanto, Robert Sutak, Xiangcong Xu, Erika M Becker, Marc R Mikhael, Prem Ponka, Des R Richardson. Proc Natl Acad Sci U S A 2008
Megan Whitnall, Yohan Suryo Rahmanto, Robert Sutak, Xiangcong Xu, Erika M Becker, Marc R Mikhael, Prem Ponka, Des R Richardson. Proc Natl Acad Sci U S A 2008
18
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
17
Increased levels of plasma malondialdehyde in Friedreich ataxia.
M Emond, G Lepage, M Vanasse, M Pandolfo. Neurology 2000
M Emond, G Lepage, M Vanasse, M Pandolfo. Neurology 2000
17
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
17
Increased iron in the dentate nucleus of patients with Friedrich's ataxia.
D Waldvogel, P van Gelderen, M Hallett. Ann Neurol 1999
D Waldvogel, P van Gelderen, M Hallett. Ann Neurol 1999
16
Friedreich ataxia: the oxidative stress paradox.
Hervé Seznec, Delphine Simon, Cécile Bouton, Laurence Reutenauer, Ariane Hertzog, Pawel Golik, Vincent Procaccio, Manisha Patel, Jean-Claude Drapier, Michel Koenig,[...]. Hum Mol Genet 2005
Hervé Seznec, Delphine Simon, Cécile Bouton, Laurence Reutenauer, Ariane Hertzog, Pawel Golik, Vincent Procaccio, Manisha Patel, Jean-Claude Drapier, Michel Koenig,[...]. Hum Mol Genet 2005
16
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
16
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
17
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
16
HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model.
Myriam Rai, Elisabetta Soragni, Kai Jenssen, Ryan Burnett, David Herman, Giovanni Coppola, Daniel H Geschwind, Joel M Gottesfeld, Massimo Pandolfo. PLoS One 2008
Myriam Rai, Elisabetta Soragni, Kai Jenssen, Ryan Burnett, David Herman, Giovanni Coppola, Daniel H Geschwind, Joel M Gottesfeld, Massimo Pandolfo. PLoS One 2008
15
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
15
Friedreich's ataxia, no changes in mitochondrial labile iron in human lymphoblasts and fibroblasts: a decrease in antioxidative capacity?
Brigitte Sturm, Ute Bistrich, Matthias Schranzhofer, Joseph P Sarsero, Ursula Rauen, Barbara Scheiber-Mojdehkar, Herbert de Groot, Panos Ioannou, Frank Petrat. J Biol Chem 2005
Brigitte Sturm, Ute Bistrich, Matthias Schranzhofer, Joseph P Sarsero, Ursula Rauen, Barbara Scheiber-Mojdehkar, Herbert de Groot, Panos Ioannou, Frank Petrat. J Biol Chem 2005
23
RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila.
Peter R Anderson, Kim Kirby, Arthur J Hilliker, John P Phillips. Hum Mol Genet 2005
Peter R Anderson, Kim Kirby, Arthur J Hilliker, John P Phillips. Hum Mol Genet 2005
14
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
14
Disabled early recruitment of antioxidant defenses in Friedreich's ataxia.
K Chantrel-Groussard, V Geromel, H Puccio, M Koenig, A Munnich, A Rötig, P Rustin. Hum Mol Genet 2001
K Chantrel-Groussard, V Geromel, H Puccio, M Koenig, A Munnich, A Rötig, P Rustin. Hum Mol Genet 2001
14
The yeast frataxin homologue mediates mitochondrial iron efflux. Evidence for a mitochondrial iron cycle.
D C Radisky, M C Babcock, J Kaplan. J Biol Chem 1999
D C Radisky, M C Babcock, J Kaplan. J Biol Chem 1999
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Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia.
Peter R Anderson, Kim Kirby, William C Orr, Arthur J Hilliker, John P Phillips. Proc Natl Acad Sci U S A 2008
Peter R Anderson, Kim Kirby, William C Orr, Arthur J Hilliker, John P Phillips. Proc Natl Acad Sci U S A 2008
16
Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPARgamma pathway as a therapeutic target in Friedreich's ataxia.
Giovanni Coppola, Daniele Marmolino, Daning Lu, Qing Wang, Miriam Cnop, Myriam Rai, Fabio Acquaviva, Sergio Cocozza, Massimo Pandolfo, Daniel H Geschwind. Hum Mol Genet 2009
Giovanni Coppola, Daniele Marmolino, Daning Lu, Qing Wang, Miriam Cnop, Myriam Rai, Fabio Acquaviva, Sergio Cocozza, Massimo Pandolfo, Daniel H Geschwind. Hum Mol Genet 2009
15
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
14
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
13
Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant.
Rafael P Vázquez-Manrique, Pilar González-Cabo, Sheila Ros, Homera Aziz, Howard A Baylis, Francesc Palau. FASEB J 2006
Rafael P Vázquez-Manrique, Pilar González-Cabo, Sheila Ros, Homera Aziz, Howard A Baylis, Francesc Palau. FASEB J 2006
18
Idebenone treatment in Friedreich patients: one-year-long randomized placebo-controlled trial.
C Mariotti, A Solari, D Torta, L Marano, C Fiorentini, S Di Donato. Neurology 2003
C Mariotti, A Solari, D Torta, L Marano, C Fiorentini, S Di Donato. Neurology 2003
13
Idebenone in Friedreich ataxia cardiomyopathy-results from a 6-month phase III study (IONIA).
Sarah J Lagedrost, Martin St John Sutton, Meryl S Cohen, Gary M Satou, Beth D Kaufman, Susan L Perlman, Christian Rummey, Thomas Meier, David R Lynch. Am Heart J 2011
Sarah J Lagedrost, Martin St John Sutton, Meryl S Cohen, Gary M Satou, Beth D Kaufman, Susan L Perlman, Christian Rummey, Thomas Meier, David R Lynch. Am Heart J 2011
16
A phase 3, double-blind, placebo-controlled trial of idebenone in friedreich ataxia.
David R Lynch, Susan L Perlman, Thomas Meier. Arch Neurol 2010
David R Lynch, Susan L Perlman, Thomas Meier. Arch Neurol 2010
13
Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues.
Alain Martelli, Marie Wattenhofer-Donzé, Stéphane Schmucker, Samuel Bouvet, Laurence Reutenauer, Hélène Puccio. Hum Mol Genet 2007
Alain Martelli, Marie Wattenhofer-Donzé, Stéphane Schmucker, Samuel Bouvet, Laurence Reutenauer, Hélène Puccio. Hum Mol Genet 2007
12
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
12
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
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.