Peter R Anderson, Kim Kirby, William C Orr, Arthur J Hilliker, John P Phillips. Proc Natl Acad Sci U S A 2008
Times Cited: 84
Times Cited: 84
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
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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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
45
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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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
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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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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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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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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
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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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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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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
25
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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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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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
23
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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Oxidative stress and protease dysfunction in the yeast model of Friedreich ataxia.
Anne-Laure Bulteau, Andrew Dancis, Monique Gareil, Jean-Jacques Montagne, Jean-Michel Camadro, Emmanuel Lesuisse. Free Radic Biol Med 2007
Anne-Laure Bulteau, Andrew Dancis, Monique Gareil, Jean-Jacques Montagne, Jean-Michel Camadro, Emmanuel Lesuisse. Free Radic Biol Med 2007
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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
23
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
21
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
21
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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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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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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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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Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities.
Renata Santos, Sophie Lefevre, Dominika Sliwa, Alexandra Seguin, Jean-Michel Camadro, Emmanuel Lesuisse. Antioxid Redox Signal 2010
Renata Santos, Sophie Lefevre, Dominika Sliwa, Alexandra Seguin, Jean-Michel Camadro, Emmanuel Lesuisse. Antioxid Redox Signal 2010
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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
25
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
17
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
17
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
17
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
17
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
16
Glutathione in blood of patients with Friedreich's ataxia.
F Piemonte, A Pastore, G Tozzi, D Tagliacozzi, F M Santorelli, R Carrozzo, C Casali, M Damiano, G Federici, E Bertini. Eur J Clin Invest 2001
F Piemonte, A Pastore, G Tozzi, D Tagliacozzi, F M Santorelli, R Carrozzo, C Casali, M Damiano, G Federici, E Bertini. Eur J Clin Invest 2001
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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
18
dfh is a Drosophila homolog of the Friedreich's ataxia disease gene.
J Cañizares, J M Blanca, J A Navarro, E Monrós, F Palau, M D Moltó. Gene 2000
J Cañizares, J M Blanca, J A Navarro, E Monrós, F Palau, M D Moltó. Gene 2000
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Frataxin deficiency in pancreatic islets causes diabetes due to loss of beta cell mass.
Michael Ristow, Hindrik Mulder, Doreen Pomplun, Tim J Schulz, Katrin Müller-Schmehl, Anja Krause, Malin Fex, Helene Puccio, Jörg Müller, Frank Isken,[...]. J Clin Invest 2003
Michael Ristow, Hindrik Mulder, Doreen Pomplun, Tim J Schulz, Katrin Müller-Schmehl, Anja Krause, Malin Fex, Helene Puccio, Jörg Müller, Frank Isken,[...]. J Clin Invest 2003
16
Oxidative stress, mitochondrial dysfunction and cellular stress response in Friedreich's ataxia.
Vittorio Calabrese, Raffaele Lodi, Caterina Tonon, Velia D'Agata, Maria Sapienza, Giovanni Scapagnini, Andrea Mangiameli, Giovanni Pennisi, A M Giuffrida Stella, D Allan Butterfield. J Neurol Sci 2005
Vittorio Calabrese, Raffaele Lodi, Caterina Tonon, Velia D'Agata, Maria Sapienza, Giovanni Scapagnini, Andrea Mangiameli, Giovanni Pennisi, A M Giuffrida Stella, D Allan Butterfield. J Neurol Sci 2005
16
Glutathione-dependent redox status of frataxin-deficient cells in a yeast model of Friedreich's ataxia.
Françoise Auchère, Renata Santos, Sara Planamente, Emmanuel Lesuisse, Jean-Michel Camadro. Hum Mol Genet 2008
Françoise Auchère, Renata Santos, Sara Planamente, Emmanuel Lesuisse, Jean-Michel Camadro. Hum Mol Genet 2008
21
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
16
Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation.
Or Kakhlon, Hila Manning, William Breuer, Naomi Melamed-Book, Chunye Lu, Gino Cortopassi, Arnold Munnich, Z Ioav Cabantchik. Blood 2008
Or Kakhlon, Hila Manning, William Breuer, Naomi Melamed-Book, Chunye Lu, Gino Cortopassi, Arnold Munnich, Z Ioav Cabantchik. Blood 2008
16
The dorsal root ganglion in Friedreich's ataxia.
Arnulf H Koeppen, Jennifer A Morral, Ashley N Davis, Jiang Qian, Simone V Petrocine, Mitchell D Knutson, Walter M Gibson, Matthew J Cusack, Danhong Li. Acta Neuropathol 2009
Arnulf H Koeppen, Jennifer A Morral, Ashley N Davis, Jiang Qian, Simone V Petrocine, Mitchell D Knutson, Walter M Gibson, Matthew J Cusack, Danhong Li. Acta Neuropathol 2009
16
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
16
Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question.
Jeffrey S Armstrong, Omar Khdour, Sidney M Hecht. FASEB J 2010
Jeffrey S Armstrong, Omar Khdour, Sidney M Hecht. FASEB J 2010
17
Deferiprone and idebenone rescue frataxin depletion phenotypes in a Drosophila model of Friedreich's ataxia.
Sirena Soriano, José V Llorens, Laura Blanco-Sobero, Lucía Gutiérrez, Pablo Calap-Quintana, M Puerto Morales, M Dolores Moltó, M José Martínez-Sebastián. Gene 2013
Sirena Soriano, José V Llorens, Laura Blanco-Sobero, Lucía Gutiérrez, Pablo Calap-Quintana, M Puerto Morales, M Dolores Moltó, M José Martínez-Sebastián. Gene 2013
46
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
Iron and iron-responsive proteins in the cardiomyopathy of Friedreich's ataxia.
Susan Michael, Simone V Petrocine, Jiang Qian, Jacques B Lamarche, Mitchell D Knutson, Michael D Garrick, Arnulf H Koeppen. Cerebellum 2006
Susan Michael, Simone V Petrocine, Jiang Qian, Jacques B Lamarche, Mitchell D Knutson, Michael D Garrick, Arnulf H Koeppen. Cerebellum 2006
15
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
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
15
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
15
Binding of yeast frataxin to the scaffold for Fe-S cluster biogenesis, Isu.
Tao Wang, Elizabeth A Craig. J Biol Chem 2008
Tao Wang, Elizabeth A Craig. J Biol Chem 2008
17
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.