Peter R Anderson, Kim Kirby, Arthur J Hilliker, John P Phillips. Hum Mol Genet 2005
Times Cited: 107
Times Cited: 107
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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53
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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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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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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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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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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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
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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
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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
A Wong, J Yang, P Cavadini, C Gellera, B Lonnerdal, F Taroni, G Cortopassi. Hum Mol Genet 1999
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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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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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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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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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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
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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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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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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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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
24
Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia.
Delphine Simon, Hervé Seznec, Anne Gansmuller, Nadège Carelle, Philipp Weber, Daniel Metzger, Pierre Rustin, Michel Koenig, Hélène Puccio. J Neurosci 2004
Delphine Simon, Hervé Seznec, Anne Gansmuller, Nadège Carelle, Philipp Weber, Daniel Metzger, Pierre Rustin, Michel Koenig, Hélène Puccio. J Neurosci 2004
24
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
J B Schulz, T Dehmer, L Schöls, H Mende, C Hardt, M Vorgerd, K Bürk, W Matson, J Dichgans, M F Beal,[...]. Neurology 2000
22
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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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
H Koutnikova, V Campuzano, F Foury, P Dollé, O Cazzalini, M Koenig. Nat Genet 1997
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Altered lipid metabolism in a Drosophila model of Friedreich's ataxia.
Juan A Navarro, Elisabeth Ohmann, Diego Sanchez, José A Botella, Gerhard Liebisch, María D Moltó, María D Ganfornina, Gerd Schmitz, Stephan Schneuwly. Hum Mol Genet 2010
Juan A Navarro, Elisabeth Ohmann, Diego Sanchez, José A Botella, Gerhard Liebisch, María D Moltó, María D Ganfornina, Gerd Schmitz, Stephan Schneuwly. Hum Mol Genet 2010
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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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Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria.
F Foury, O Cazzalini. FEBS Lett 1997
F Foury, O Cazzalini. FEBS Lett 1997
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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
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Overexpression of frataxin in the mitochondria increases resistance to oxidative stress and extends lifespan in Drosophila.
Alexander P Runko, Anthony J Griswold, Kyung-Tai Min. FEBS Lett 2008
Alexander P Runko, Anthony J Griswold, Kyung-Tai Min. FEBS Lett 2008
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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
19
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
19
Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia.
Hervé Seznec, Delphine Simon, Laurent Monassier, Paola Criqui-Filipe, Anne Gansmuller, Pierre Rustin, Michel Koenig, Hélène Puccio. Hum Mol Genet 2004
Hervé Seznec, Delphine Simon, Laurent Monassier, Paola Criqui-Filipe, Anne Gansmuller, Pierre Rustin, Michel Koenig, Hélène Puccio. Hum Mol Genet 2004
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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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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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dfh is a Drosophila homolog of the Friedreich's ataxia disease gene.
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J Cañizares, J M Blanca, J A Navarro, E Monrós, F Palau, M D Moltó. Gene 2000
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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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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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Defects in mitochondrial axonal transport and membrane potential without increased reactive oxygen species production in a Drosophila model of Friedreich ataxia.
Yujiro Shidara, Peter J Hollenbeck. J Neurosci 2010
Yujiro Shidara, Peter J Hollenbeck. J Neurosci 2010
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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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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
Gunhild Layer, Sandrine Ollagnier-de Choudens, Yiannis Sanakis, Marc Fontecave. J Biol Chem 2006
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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
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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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Assembly of human frataxin is a mechanism for detoxifying redox-active iron.
Heather A O'Neill, Oleksandr Gakh, Sungjo Park, Jin Cui, Steven M Mooney, Matthew Sampson, Gloria C Ferreira, Grazia Isaya. Biochemistry 2005
Heather A O'Neill, Oleksandr Gakh, Sungjo Park, Jin Cui, Steven M Mooney, Matthew Sampson, Gloria C Ferreira, Grazia Isaya. Biochemistry 2005
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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
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Friedreich's ataxia protein: phylogenetic evidence for mitochondrial dysfunction.
T J Gibson, E V Koonin, G Musco, A Pastore, P Bork. Trends Neurosci 1996
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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
15
Frataxin, iron-sulfur clusters, heme, ROS, and aging.
Eleonora Napoli, Franco Taroni, Gino A Cortopassi. Antioxid Redox Signal 2006
Eleonora Napoli, Franco Taroni, Gino A Cortopassi. Antioxid Redox Signal 2006
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Frataxin knockdown causes loss of cytoplasmic iron-sulfur cluster functions, redox alterations and induction of heme transcripts.
Chunye Lu, Gino Cortopassi. Arch Biochem Biophys 2007
Chunye Lu, Gino Cortopassi. Arch Biochem Biophys 2007
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The cardiomyopathy of Friedreich's ataxia morphological observations in 3 cases.
J B Lamarche, M Côté, B Lemieux. Can J Neurol Sci 1980
J B Lamarche, M Côté, B Lemieux. Can J Neurol Sci 1980
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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
15
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
15
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.