Myriam Rai, Elisabetta Soragni, Kai Jenssen, Ryan Burnett, David Herman, Giovanni Coppola, Daniel H Geschwind, Joel M Gottesfeld, Massimo Pandolfo. PLoS One 2008
Times Cited: 161
Times Cited: 161
Times Cited
Times Co-cited
Similarity
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
71
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
58
Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model.
Chiranjeevi Sandi, Ricardo Mouro Pinto, Sahar Al-Mahdawi, Vahid Ezzatizadeh, Glenn Barnes, Steve Jones, James R Rusche, Joel M Gottesfeld, Mark A Pook. Neurobiol Dis 2011
Chiranjeevi Sandi, Ricardo Mouro Pinto, Sahar Al-Mahdawi, Vahid Ezzatizadeh, Glenn Barnes, Steve Jones, James R Rusche, Joel M Gottesfeld, Mark A Pook. Neurobiol Dis 2011
47
Two new pimelic diphenylamide HDAC inhibitors induce sustained frataxin upregulation in cells from Friedreich's ataxia patients and in a mouse model.
Myriam Rai, Elisabetta Soragni, C James Chou, Glenn Barnes, Steve Jones, James R Rusche, Joel M Gottesfeld, Massimo Pandolfo. PLoS One 2010
Myriam Rai, Elisabetta Soragni, C James Chou, Glenn Barnes, Steve Jones, James R Rusche, Joel M Gottesfeld, Massimo Pandolfo. PLoS One 2010
39
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
Sahar Al-Mahdawi, Ricardo Mouro Pinto, Ozama Ismail, Dhaval Varshney, Stefania Lymperi, Chiranjeevi Sandi, Daniah Trabzuni, Mark Pook. Hum Mol Genet 2008
38
Repeat-induced epigenetic changes in intron 1 of the frataxin gene and its consequences in Friedreich ataxia.
Eriko Greene, Lata Mahishi, Ali Entezam, Daman Kumari, Karen Usdin. Nucleic Acids Res 2007
Eriko Greene, Lata Mahishi, Ali Entezam, Daman Kumari, Karen Usdin. Nucleic Acids Res 2007
31
DNA triplet repeats mediate heterochromatin-protein-1-sensitive variegated gene silencing.
Alexander Saveliev, Christopher Everett, Tammy Sharpe, Zoë Webster, Richard Festenstein. Nature 2003
Alexander Saveliev, Christopher Everett, Tammy Sharpe, Zoë Webster, Richard Festenstein. Nature 2003
31
Chemical probes identify a role for histone deacetylase 3 in Friedreich's ataxia gene silencing.
Chunping Xu, Elisabetta Soragni, C James Chou, David Herman, Heather L Plasterer, James R Rusche, Joel M Gottesfeld. Chem Biol 2009
Chunping Xu, Elisabetta Soragni, C James Chou, David Herman, Heather L Plasterer, James R Rusche, Joel M Gottesfeld. Chem Biol 2009
32
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
30
The GAA triplet-repeat expansion in Friedreich ataxia interferes with transcription and may be associated with an unusual DNA structure.
S I Bidichandani, T Ashizawa, P I Patel. Am J Hum Genet 1998
S I Bidichandani, T Ashizawa, P I Patel. Am J Hum Genet 1998
27
Epigenetic silencing in Friedreich ataxia is associated with depletion of CTCF (CCCTC-binding factor) and antisense transcription.
Irene De Biase, Yogesh K Chutake, Paul M Rindler, Sanjay I Bidichandani. PLoS One 2009
Irene De Biase, Yogesh K Chutake, Paul M Rindler, Sanjay I Bidichandani. PLoS One 2009
31
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
24
Mortality in Friedreich ataxia.
Amy Y Tsou, Erin K Paulsen, Sarah J Lagedrost, Susan L Perlman, Katherine D Mathews, George R Wilmot, Bernard Ravina, Arnulf H Koeppen, David R Lynch. J Neurol Sci 2011
Amy Y Tsou, Erin K Paulsen, Sarah J Lagedrost, Susan L Perlman, Katherine D Mathews, George R Wilmot, Bernard Ravina, Arnulf H Koeppen, David R Lynch. J Neurol Sci 2011
23
Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features.
A E Harding. Brain 1981
A E Harding. Brain 1981
22
Long intronic GAA repeats causing Friedreich ataxia impede transcription elongation.
Tanel Punga, Marc Bühler. EMBO Mol Med 2010
Tanel Punga, Marc Bühler. EMBO Mol Med 2010
25
Repeat expansion affects both transcription initiation and elongation in friedreich ataxia cells.
Daman Kumari, Rea Erika Biacsi, Karen Usdin. J Biol Chem 2011
Daman Kumari, Rea Erika Biacsi, Karen Usdin. J Biol Chem 2011
34
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
21
DNA methylation in intron 1 of the frataxin gene is related to GAA repeat length and age of onset in Friedreich ataxia patients.
I Castaldo, M Pinelli, A Monticelli, F Acquaviva, M Giacchetti, A Filla, S Sacchetti, S Keller, V E Avvedimento, L Chiariotti,[...]. J Med Genet 2008
I Castaldo, M Pinelli, A Monticelli, F Acquaviva, M Giacchetti, A Filla, S Sacchetti, S Keller, V E Avvedimento, L Chiariotti,[...]. J Med Genet 2008
30
Heterochromatinization induced by GAA-repeat hyperexpansion in Friedreich's ataxia can be reduced upon HDAC inhibition by vitamin B3.
Ping K Chan, Raul Torres, Cihangir Yandim, Pui P Law, Sanjay Khadayate, Marta Mauri, Crina Grosan, Nadine Chapman-Rothe, Paola Giunti, Mark Pook,[...]. Hum Mol Genet 2013
Ping K Chan, Raul Torres, Cihangir Yandim, Pui P Law, Sanjay Khadayate, Marta Mauri, Crina Grosan, Nadine Chapman-Rothe, Paola Giunti, Mark Pook,[...]. Hum Mol Genet 2013
42
Hyperexpansion of GAA repeats affects post-initiation steps of FXN transcription in Friedreich's ataxia.
Eunah Kim, Marek Napierala, Sharon Y R Dent. Nucleic Acids Res 2011
Eunah Kim, Marek Napierala, Sharon Y R Dent. Nucleic Acids Res 2011
35
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
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
20
A TAT-frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse model.
Piyush M Vyas, Wendy J Tomamichel, P Melanie Pride, Clifford M Babbey, Qiujuan Wang, Jennifer Mercier, Elizabeth M Martin, R Mark Payne. Hum Mol Genet 2012
Piyush M Vyas, Wendy J Tomamichel, P Melanie Pride, Clifford M Babbey, Qiujuan Wang, Jennifer Mercier, Elizabeth M Martin, R Mark Payne. Hum Mol Genet 2012
31
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
20
Recombinant human erythropoietin: effects on frataxin expression in vitro.
B Sturm, D Stupphann, C Kaun, S Boesch, M Schranzhofer, J Wojta, H Goldenberg, B Scheiber-Mojdehkar. Eur J Clin Invest 2005
B Sturm, D Stupphann, C Kaun, S Boesch, M Schranzhofer, J Wojta, H Goldenberg, B Scheiber-Mojdehkar. Eur J Clin Invest 2005
25
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
19
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
18
The GAA*TTC triplet repeat expanded in Friedreich's ataxia impedes transcription elongation by T7 RNA polymerase in a length and supercoil dependent manner.
E Grabczyk, K Usdin. Nucleic Acids Res 2000
E Grabczyk, K Usdin. Nucleic Acids Res 2000
18
Inhibitory effects of expanded GAA.TTC triplet repeats from intron I of the Friedreich ataxia gene on transcription and replication in vivo.
K Ohshima, L Montermini, R D Wells, M Pandolfo. J Biol Chem 1998
K Ohshima, L Montermini, R D Wells, M Pandolfo. J Biol Chem 1998
18
FXN methylation predicts expression and clinical outcome in Friedreich ataxia.
Marguerite V Evans-Galea, Nissa Carrodus, Simone M Rowley, Louise A Corben, Geneieve Tai, Richard Saffery, John C Galati, Nicholas C Wong, Jeffrey M Craig, David R Lynch,[...]. Ann Neurol 2012
Marguerite V Evans-Galea, Nissa Carrodus, Simone M Rowley, Louise A Corben, Geneieve Tai, Richard Saffery, John C Galati, Nicholas C Wong, Jeffrey M Craig, David R Lynch,[...]. Ann Neurol 2012
21
Epigenetic and neurological effects and safety of high-dose nicotinamide in patients with Friedreich's ataxia: an exploratory, open-label, dose-escalation study.
Vincenzo Libri, Cihangir Yandim, Stavros Athanasopoulos, Naomi Loyse, Theona Natisvili, Pui Pik Law, Ping Kei Chan, Tariq Mohammad, Marta Mauri, Kin Tung Tam,[...]. Lancet 2014
Vincenzo Libri, Cihangir Yandim, Stavros Athanasopoulos, Naomi Loyse, Theona Natisvili, Pui Pik Law, Ping Kei Chan, Tariq Mohammad, Marta Mauri, Kin Tung Tam,[...]. Lancet 2014
21
Epigenetic therapy for Friedreich ataxia.
Elisabetta Soragni, Wenyan Miao, Marco Iudicello, David Jacoby, Stefania De Mercanti, Marinella Clerico, Filomena Longo, Antonio Piga, Sherman Ku, Erica Campau,[...]. Ann Neurol 2014
Elisabetta Soragni, Wenyan Miao, Marco Iudicello, David Jacoby, Stefania De Mercanti, Marinella Clerico, Filomena Longo, Antonio Piga, Sherman Ku, Erica Campau,[...]. Ann Neurol 2014
19
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
17
Long intronic GAA*TTC repeats induce epigenetic changes and reporter gene silencing in a molecular model of Friedreich ataxia.
E Soragni, D Herman, S Y R Dent, J M Gottesfeld, R D Wells, M Napierala. Nucleic Acids Res 2008
E Soragni, D Herman, S Y R Dent, J M Gottesfeld, R D Wells, M Napierala. Nucleic Acids Res 2008
30
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
17
Friedreich's ataxia: pathology, pathogenesis, and molecular genetics.
Arnulf H Koeppen. J Neurol Sci 2011
Arnulf H Koeppen. J Neurol Sci 2011
17
Erythropoietin in Friedreich ataxia: no effect on frataxin in a randomized controlled trial.
Caterina Mariotti, Roberto Fancellu, Serena Caldarazzo, Lorenzo Nanetti, Daniela Di Bella, Massimo Plumari, Giuseppe Lauria, Maria D Cappellini, Lorena Duca, Alessandra Solari,[...]. Mov Disord 2012
Caterina Mariotti, Roberto Fancellu, Serena Caldarazzo, Lorenzo Nanetti, Daniela Di Bella, Massimo Plumari, Giuseppe Lauria, Maria D Cappellini, Lorena Duca, Alessandra Solari,[...]. Mov Disord 2012
44
The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice.
Elizabeth A Thomas, Giovanni Coppola, Paula A Desplats, Bin Tang, Elisabetta Soragni, Ryan Burnett, Fuying Gao, Kelsey M Fitzgerald, Jenna F Borok, David Herman,[...]. Proc Natl Acad Sci U S A 2008
Elizabeth A Thomas, Giovanni Coppola, Paula A Desplats, Bin Tang, Elisabetta Soragni, Ryan Burnett, Fuying Gao, Kelsey M Fitzgerald, Jenna F Borok, David Herman,[...]. Proc Natl Acad Sci U S A 2008
16
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
16
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
A Filla, G De Michele, F Cavalcanti, L Pianese, A Monticelli, G Campanella, S Cocozza. Am J Hum Genet 1996
16
Pimelic diphenylamide 106 is a slow, tight-binding inhibitor of class I histone deacetylases.
C James Chou, David Herman, Joel M Gottesfeld. J Biol Chem 2008
C James Chou, David Herman, Joel M Gottesfeld. J Biol Chem 2008
16
Interferon gamma upregulates frataxin and corrects the functional deficits in a Friedreich ataxia model.
Barbara Tomassini, Gaetano Arcuri, Silvia Fortuni, Chiranjeevi Sandi, Vahid Ezzatizadeh, Carlo Casali, Ivano Condò, Florence Malisan, Sahar Al-Mahdawi, Mark Pook,[...]. Hum Mol Genet 2012
Barbara Tomassini, Gaetano Arcuri, Silvia Fortuni, Chiranjeevi Sandi, Vahid Ezzatizadeh, Carlo Casali, Ivano Condò, Florence Malisan, Sahar Al-Mahdawi, Mark Pook,[...]. Hum Mol Genet 2012
40
Frataxin knockin mouse.
Carlos J Miranda, Manuela M Santos, Keiichi Ohshima, Julie Smith, Liangtao Li, Michaeline Bunting, Mireille Cossée, Michael Koenig, Jorge Sequeiros, Jerry Kaplan,[...]. FEBS Lett 2002
Carlos J Miranda, Manuela M Santos, Keiichi Ohshima, Julie Smith, Liangtao Li, Michaeline Bunting, Mireille Cossée, Michael Koenig, Jorge Sequeiros, Jerry Kaplan,[...]. FEBS Lett 2002
15
A persistent RNA.DNA hybrid formed by transcription of the Friedreich ataxia triplet repeat in live bacteria, and by T7 RNAP in vitro.
Ed Grabczyk, Miriam Mancuso, Mimi C Sammarco. Nucleic Acids Res 2007
Ed Grabczyk, Miriam Mancuso, Mimi C Sammarco. Nucleic Acids Res 2007
15
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
15
Evolution of the Friedreich's ataxia trinucleotide repeat expansion: founder effect and premutations.
M Cossée, M Schmitt, V Campuzano, L Reutenauer, C Moutou, J L Mandel, M Koenig. Proc Natl Acad Sci U S A 1997
M Cossée, M Schmitt, V Campuzano, L Reutenauer, C Moutou, J L Mandel, M Koenig. Proc Natl Acad Sci U S A 1997
14
Sticky DNA: self-association properties of long GAA.TTC repeats in R.R.Y triplex structures from Friedreich's ataxia.
N Sakamoto, P D Chastain, P Parniewski, K Ohshima, M Pandolfo, J D Griffith, R D Wells. Mol Cell 1999
N Sakamoto, P D Chastain, P Parniewski, K Ohshima, M Pandolfo, J D Griffith, R D Wells. Mol Cell 1999
14
Friedreich's ataxia induced pluripotent stem cells model intergenerational GAA⋅TTC triplet repeat instability.
Sherman Ku, Elisabetta Soragni, Erica Campau, Elizabeth A Thomas, Gulsah Altun, Louise C Laurent, Jeanne F Loring, Marek Napierala, Joel M Gottesfeld. Cell Stem Cell 2010
Sherman Ku, Elisabetta Soragni, Erica Campau, Elizabeth A Thomas, Gulsah Altun, Louise C Laurent, Jeanne F Loring, Marek Napierala, Joel M Gottesfeld. Cell Stem Cell 2010
14
Neurological effects of recombinant human erythropoietin in Friedreich's ataxia: a clinical pilot trial.
Sylvia Boesch, Brigitte Sturm, Sascha Hering, Barbara Scheiber-Mojdehkar, Hannes Steinkellner, Hans Goldenberg, Werner Poewe. Mov Disord 2008
Sylvia Boesch, Brigitte Sturm, Sascha Hering, Barbara Scheiber-Mojdehkar, Hannes Steinkellner, Hans Goldenberg, Werner Poewe. Mov Disord 2008
25
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
14
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.