Anna Stepanova, Jordi Magrané. Mol Cell Neurosci 2020
Times Cited: 12
Times Cited: 12
Times Cited
Times Co-cited
Similarity
Therapeutic Prospects for Friedreich's Ataxia.
Siyuan Zhang, Marek Napierala, Jill S Napierala. Trends Pharmacol Sci 2019
Siyuan Zhang, Marek Napierala, Jill S Napierala. Trends Pharmacol Sci 2019
33
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
33
Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia.
Sunil Sahdeo, Brian D Scott, Marissa Z McMackin, Mittal Jasoliya, Brandon Brown, Heike Wulff, Susan L Perlman, Mark A Pook, Gino A Cortopassi. Hum Mol Genet 2014
Sunil Sahdeo, Brian D Scott, Marissa Z McMackin, Mittal Jasoliya, Brandon Brown, Heike Wulff, Susan L Perlman, Mark A Pook, Gino A Cortopassi. Hum Mol Genet 2014
25
Friedreich ataxia- pathogenesis and implications for therapies.
Martin B Delatycki, Sanjay I Bidichandani. Neurobiol Dis 2019
Martin B Delatycki, Sanjay I Bidichandani. Neurobiol Dis 2019
25
Frataxin deficiency impairs mitochondrial biogenesis in cells, mice and humans.
Mittal J Jasoliya, Marissa Z McMackin, Chelsea K Henderson, Susan L Perlman, Gino A Cortopassi. Hum Mol Genet 2017
Mittal J Jasoliya, Marissa Z McMackin, Chelsea K Henderson, Susan L Perlman, Gino A Cortopassi. Hum Mol Genet 2017
25
Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia.
Hong Lin, Jordi Magrane, Amy Rattelle, Anna Stepanova, Alexander Galkin, Elisia M Clark, Yi Na Dong, Sarah M Halawani, David R Lynch. Dis Model Mech 2017
Hong Lin, Jordi Magrane, Amy Rattelle, Anna Stepanova, Alexander Galkin, Elisia M Clark, Yi Na Dong, Sarah M Halawani, David R Lynch. Dis Model Mech 2017
25
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
25
Mitochondrial dysfunction induced by frataxin deficiency is associated with cellular senescence and abnormal calcium metabolism.
Arantxa Bolinches-Amorós, Belén Mollá, David Pla-Martín, Francesc Palau, Pilar González-Cabo. Front Cell Neurosci 2014
Arantxa Bolinches-Amorós, Belén Mollá, David Pla-Martín, Francesc Palau, Pilar González-Cabo. Front Cell Neurosci 2014
25
New developments in pharmacotherapy for Friedreich ataxia.
Alexandra Clay, Patrick Hearle, Kim Schadt, David R Lynch. Expert Opin Pharmacother 2019
Alexandra Clay, Patrick Hearle, Kim Schadt, David R Lynch. Expert Opin Pharmacother 2019
25
Nrf2-Inducers Counteract Neurodegeneration in Frataxin-Silenced Motor Neurons: Disclosing New Therapeutic Targets for Friedreich's Ataxia.
Sara Petrillo, Emanuela Piermarini, Anna Pastore, Gessica Vasco, Tommaso Schirinzi, Rosalba Carrozzo, Enrico Bertini, Fiorella Piemonte. Int J Mol Sci 2017
Sara Petrillo, Emanuela Piermarini, Anna Pastore, Gessica Vasco, Tommaso Schirinzi, Rosalba Carrozzo, Enrico Bertini, Fiorella Piemonte. Int J Mol Sci 2017
16
Molecular Mechanisms and Therapeutics for the GAA·TTC Expansion Disease Friedreich Ataxia.
Joel M Gottesfeld. Neurotherapeutics 2019
Joel M Gottesfeld. Neurotherapeutics 2019
16
Compound heterozygous FXN mutations and clinical outcome in friedreich ataxia.
Charles A Galea, Aamira Huq, Paul J Lockhart, Geneieve Tai, Louise A Corben, Eppie M Yiu, Lyle C Gurrin, David R Lynch, Sarah Gelbard, Alexandra Durr,[...]. Ann Neurol 2016
Charles A Galea, Aamira Huq, Paul J Lockhart, Geneieve Tai, Louise A Corben, Eppie M Yiu, Lyle C Gurrin, David R Lynch, Sarah Gelbard, Alexandra Durr,[...]. Ann Neurol 2016
16
Adding a temporal dimension to the study of Friedreich's ataxia: the effect of frataxin overexpression in a human cell model.
Tommaso Vannocci, Roberto Notario Manzano, Ombretta Beccalli, Barbara Bettegazzi, Fabio Grohovaz, Gianfelice Cinque, Antonio de Riso, Luca Quaroni, Franca Codazzi, Annalisa Pastore. Dis Model Mech 2018
Tommaso Vannocci, Roberto Notario Manzano, Ombretta Beccalli, Barbara Bettegazzi, Fabio Grohovaz, Gianfelice Cinque, Antonio de Riso, Luca Quaroni, Franca Codazzi, Annalisa Pastore. Dis Model Mech 2018
16
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
16
Dimethyl fumarate dosing in humans increases frataxin expression: A potential therapy for Friedreich's Ataxia.
Mittal Jasoliya, Francesco Sacca, Sunil Sahdeo, Frederic Chedin, Chiara Pane, Vincenzo Brescia Morra, Alessandro Filla, Mark Pook, Gino Cortopassi. PLoS One 2019
Mittal Jasoliya, Francesco Sacca, Sunil Sahdeo, Frederic Chedin, Chiara Pane, Vincenzo Brescia Morra, Alessandro Filla, Mark Pook, Gino Cortopassi. PLoS One 2019
16
Selected missense mutations impair frataxin processing in Friedreich ataxia.
Elisia Clark, Jill S Butler, Charles J Isaacs, Marek Napierala, David R Lynch. Ann Clin Transl Neurol 2017
Elisia Clark, Jill S Butler, Charles J Isaacs, Marek Napierala, David R Lynch. Ann Clin Transl Neurol 2017
16
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
16
Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase.
H Koutnikova, V Campuzano, M Koenig. Hum Mol Genet 1998
H Koutnikova, V Campuzano, M Koenig. Hum Mol Genet 1998
16
Safety, pharmacodynamics, and potential benefit of omaveloxolone in Friedreich ataxia.
David R Lynch, Jennifer Farmer, Lauren Hauser, Ian A Blair, Qing Qing Wang, Clementina Mesaros, Nathaniel Snyder, Sylvia Boesch, Melanie Chin, Martin B Delatycki,[...]. Ann Clin Transl Neurol 2018
David R Lynch, Jennifer Farmer, Lauren Hauser, Ian A Blair, Qing Qing Wang, Clementina Mesaros, Nathaniel Snyder, Sylvia Boesch, Melanie Chin, Martin B Delatycki,[...]. Ann Clin Transl Neurol 2018
16
Emerging critical roles of Fe-S clusters in DNA replication and repair.
Jill O Fuss, Chi-Lin Tsai, Justin P Ishida, John A Tainer. Biochim Biophys Acta 2015
Jill O Fuss, Chi-Lin Tsai, Justin P Ishida, John A Tainer. Biochim Biophys Acta 2015
16
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
16
Reversible Axonal Dystrophy by Calcium Modulation in Frataxin-Deficient Sensory Neurons of YG8R Mice.
Belén Mollá, Diana C Muñoz-Lasso, Fátima Riveiro, Arantxa Bolinches-Amorós, Federico V Pallardó, Angel Fernandez-Vilata, María de la Iglesia-Vaya, Francesc Palau, Pilar Gonzalez-Cabo. Front Mol Neurosci 2017
Belén Mollá, Diana C Muñoz-Lasso, Fátima Riveiro, Arantxa Bolinches-Amorós, Federico V Pallardó, Angel Fernandez-Vilata, María de la Iglesia-Vaya, Francesc Palau, Pilar Gonzalez-Cabo. Front Mol Neurosci 2017
16
The mitochondrial cocktail: rationale for combined nutraceutical therapy in mitochondrial cytopathies.
M A Tarnopolsky. Adv Drug Deliv Rev 2008
M A Tarnopolsky. Adv Drug Deliv Rev 2008
16
Deferiprone in Friedreich ataxia: a 6-month randomized controlled trial.
Massimo Pandolfo, Javier Arpa, Martin B Delatycki, Kim Hanh Le Quan Sang, Caterina Mariotti, Arnold Munnich, Irene Sanz-Gallego, Geneieve Tai, Mark A Tarnopolsky, Franco Taroni,[...]. Ann Neurol 2014
Massimo Pandolfo, Javier Arpa, Martin B Delatycki, Kim Hanh Le Quan Sang, Caterina Mariotti, Arnold Munnich, Irene Sanz-Gallego, Geneieve Tai, Mark A Tarnopolsky, Franco Taroni,[...]. Ann Neurol 2014
16
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
16
Elamipretide (SS-31) improves mitochondrial dysfunction, synaptic and memory impairment induced by lipopolysaccharide in mice.
Weixing Zhao, Zhipeng Xu, Jiangbei Cao, Qiang Fu, Yishuang Wu, Xiaoying Zhang, Yue Long, Xuan Zhang, Yitian Yang, Yunfeng Li,[...]. J Neuroinflammation 2019
Weixing Zhao, Zhipeng Xu, Jiangbei Cao, Qiang Fu, Yishuang Wu, Xiaoying Zhang, Yue Long, Xuan Zhang, Yitian Yang, Yunfeng Li,[...]. J Neuroinflammation 2019
16
Co-enzyme Q10 and idebenone use in Friedreich's ataxia.
Michael H Parkinson, Jörg B Schulz, Paola Giunti. J Neurochem 2013
Michael H Parkinson, Jörg B Schulz, Paola Giunti. J Neurochem 2013
16
16
Frataxin activates mitochondrial energy conversion and oxidative phosphorylation.
M Ristow, M F Pfister, A J Yee, M Schubert, L Michael, C Y Zhang, K Ueki, M D Michael, B B Lowell, C R Kahn. Proc Natl Acad Sci U S A 2000
M Ristow, M F Pfister, A J Yee, M Schubert, L Michael, C Y Zhang, K Ueki, M D Michael, B B Lowell, C R Kahn. Proc Natl Acad Sci U S A 2000
16
Antioxidant Therapies and Oxidative Stress in Friedreich´s Ataxia: The Right Path or Just a Diversion?
Laura R Rodríguez, Tamara Lapeña, Pablo Calap-Quintana, María Dolores Moltó, Pilar Gonzalez-Cabo, Juan Antonio Navarro Langa. Antioxidants (Basel) 2020
Laura R Rodríguez, Tamara Lapeña, Pablo Calap-Quintana, María Dolores Moltó, Pilar Gonzalez-Cabo, Juan Antonio Navarro Langa. Antioxidants (Basel) 2020
28
Current experience in testing mitochondrial nutrients in disorders featuring oxidative stress and mitochondrial dysfunction: rational design of chemoprevention trials.
Giovanni Pagano, Annarita Aiello Talamanca, Giuseppe Castello, Mario D Cordero, Marco d'Ischia, Maria Nicola Gadaleta, Federico V Pallardó, Sandra Petrović, Luca Tiano, Adriana Zatterale. Int J Mol Sci 2014
Giovanni Pagano, Annarita Aiello Talamanca, Giuseppe Castello, Mario D Cordero, Marco d'Ischia, Maria Nicola Gadaleta, Federico V Pallardó, Sandra Petrović, Luca Tiano, Adriana Zatterale. Int J Mol Sci 2014
16
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
16
NFS1 undergoes positive selection in lung tumours and protects cells from ferroptosis.
Samantha W Alvarez, Vladislav O Sviderskiy, Erdem M Terzi, Thales Papagiannakopoulos, Andre L Moreira, Sylvia Adams, David M Sabatini, Kıvanç Birsoy, Richard Possemato. Nature 2017
Samantha W Alvarez, Vladislav O Sviderskiy, Erdem M Terzi, Thales Papagiannakopoulos, Andre L Moreira, Sylvia Adams, David M Sabatini, Kıvanç Birsoy, Richard Possemato. Nature 2017
16
Frataxin directly stimulates mitochondrial cysteine desulfurase by exposing substrate-binding sites, and a mutant Fe-S cluster scaffold protein with frataxin-bypassing ability acts similarly.
Alok Pandey, Donna M Gordon, Jayashree Pain, Timothy L Stemmler, Andrew Dancis, Debkumar Pain. J Biol Chem 2013
Alok Pandey, Donna M Gordon, Jayashree Pain, Timothy L Stemmler, Andrew Dancis, Debkumar Pain. J Biol Chem 2013
16
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
16
Restoring mitofusin balance prevents axonal degeneration in a Charcot-Marie-Tooth type 2A model.
Yueqin Zhou, Sharon Carmona, A K M G Muhammad, Shaughn Bell, Jesse Landeros, Michael Vazquez, Ritchie Ho, Antonietta Franco, Bin Lu, Gerald W Dorn,[...]. J Clin Invest 2021
Yueqin Zhou, Sharon Carmona, A K M G Muhammad, Shaughn Bell, Jesse Landeros, Michael Vazquez, Ritchie Ho, Antonietta Franco, Bin Lu, Gerald W Dorn,[...]. J Clin Invest 2021
100
The cerebellar component of Friedreich's ataxia.
Arnulf H Koeppen, Ashley N Davis, Jennifer A Morral. Acta Neuropathol 2011
Arnulf H Koeppen, Ashley N Davis, Jennifer A Morral. Acta Neuropathol 2011
16
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
16
Mitochondrial and metabolic dysfunction in Friedreich ataxia: update on pathophysiological relevance and clinical interventions.
David R Lynch, Garrett Farmer. Neuronal Signal 2021
David R Lynch, Garrett Farmer. Neuronal Signal 2021
25
The Role of Iron in Friedreich's Ataxia: Insights From Studies in Human Tissues and Cellular and Animal Models.
José Vicente Llorens, Sirena Soriano, Pablo Calap-Quintana, Pilar Gonzalez-Cabo, María Dolores Moltó. Front Neurosci 2019
José Vicente Llorens, Sirena Soriano, Pablo Calap-Quintana, Pilar Gonzalez-Cabo, María Dolores Moltó. Front Neurosci 2019
16
Longitudinal evaluation of iron concentration and atrophy in the dentate nuclei in friedreich ataxia.
Phillip G D Ward, Ian H Harding, Thomas G Close, Louise A Corben, Martin B Delatycki, Elsdon Storey, Nellie Georgiou-Karistianis, Gary F Egan. Mov Disord 2019
Phillip G D Ward, Ian H Harding, Thomas G Close, Louise A Corben, Martin B Delatycki, Elsdon Storey, Nellie Georgiou-Karistianis, Gary F Egan. Mov Disord 2019
16
Engineering the AAVS1 locus for consistent and scalable transgene expression in human iPSCs and their differentiated derivatives.
Fabian Oceguera-Yanez, Shin-Il Kim, Tomoko Matsumoto, Ghee Wan Tan, Long Xiang, Takeshi Hatani, Takayuki Kondo, Makoto Ikeya, Yoshinori Yoshida, Haruhisa Inoue,[...]. Methods 2016
Fabian Oceguera-Yanez, Shin-Il Kim, Tomoko Matsumoto, Ghee Wan Tan, Long Xiang, Takeshi Hatani, Takayuki Kondo, Makoto Ikeya, Yoshinori Yoshida, Haruhisa Inoue,[...]. Methods 2016
8
The role of oxidative stress in Friedreich's ataxia.
Federica Lupoli, Tommaso Vannocci, Giovanni Longo, Neri Niccolai, Annalisa Pastore. FEBS Lett 2018
Federica Lupoli, Tommaso Vannocci, Giovanni Longo, Neri Niccolai, Annalisa Pastore. FEBS Lett 2018
8
Overexpression of human and fly frataxins in Drosophila provokes deleterious effects at biochemical, physiological and developmental levels.
Juan A Navarro, José V Llorens, Sirena Soriano, José A Botella, Stephan Schneuwly, María J Martínez-Sebastián, María D Moltó. PLoS One 2011
Juan A Navarro, José V Llorens, Sirena Soriano, José A Botella, Stephan Schneuwly, María J Martínez-Sebastián, María D Moltó. PLoS One 2011
8
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
8
p53 directly regulates the transcription of the human frataxin gene and its lack of regulation in tumor cells decreases the utilization of mitochondrial iron.
Rina Shimizu, Nguyen Ngoc Lan, Tran Tien Tai, Yuka Adachi, Asako Kawazoe, Anfeng Mu, Shigeru Taketani. Gene 2014
Rina Shimizu, Nguyen Ngoc Lan, Tran Tien Tai, Yuka Adachi, Asako Kawazoe, Anfeng Mu, Shigeru Taketani. Gene 2014
8
Friedreich's ataxia--a case of aberrant transcription termination?
Jill Sergesketter Butler, Marek Napierala. Transcription 2015
Jill Sergesketter Butler, Marek Napierala. Transcription 2015
8
Intracerebroventricular viral injection of the neonatal mouse brain for persistent and widespread neuronal transduction.
Ji-Yoen Kim, Stacy D Grunke, Yona Levites, Todd E Golde, Joanna L Jankowsky. J Vis Exp 2014
Ji-Yoen Kim, Stacy D Grunke, Yona Levites, Todd E Golde, Joanna L Jankowsky. J Vis Exp 2014
8
Engineering adeno-associated virus vectors for gene therapy.
Chengwen Li, R Jude Samulski. Nat Rev Genet 2020
Chengwen Li, R Jude Samulski. Nat Rev Genet 2020
8
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.