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
Times Cited: 159
Times Cited: 159
Times Cited
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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
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Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion.
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Identification of nonferritin mitochondrial iron deposits in a mouse model of Friedreich ataxia.
Megan Whitnall, Yohan Suryo Rahmanto, Michael L-H Huang, Federica Saletta, Hiu Chuen Lok, Lucía Gutiérrez, Francisco J Lázaro, Adam J Fleming, Tim G St Pierre, Marc R Mikhael,[...]. Proc Natl Acad Sci U S A 2012
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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia.
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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
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Mitochondrial iron trafficking and the integration of iron metabolism between the mitochondrion and cytosol.
Des R Richardson, Darius J R Lane, Erika M Becker, Michael L-H Huang, Megan Whitnall, Yohan Suryo Rahmanto, Alex D Sheftel, Prem Ponka. Proc Natl Acad Sci U S A 2010
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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.
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Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes.
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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.
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Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxia.
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Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex.
Chi-Lin Tsai, David P Barondeau. Biochemistry 2010
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Susan Michael, Simone V Petrocine, Jiang Qian, Jacques B Lamarche, Mitchell D Knutson, Michael D Garrick, Arnulf H Koeppen. Cerebellum 2006
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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.
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Iron-dependent regulation of frataxin expression: implications for treatment of Friedreich ataxia.
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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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Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model.
Yuxi Shan, Robert A Schoenfeld, Genki Hayashi, Eleonora Napoli, Tasuku Akiyama, Mirela Iodi Carstens, Earl E Carstens, Mark A Pook, Gino A Cortopassi. Antioxid Redox Signal 2013
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Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease.
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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
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Mitochondrial mayhem: the mitochondrion as a modulator of iron metabolism and its role in disease.
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Ferrochelatase forms an oligomeric complex with mitoferrin-1 and Abcb10 for erythroid heme biosynthesis.
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The dentate nucleus in Friedreich's ataxia: the role of iron-responsive proteins.
Arnulf H Koeppen, Susan C Michael, Mitchell D Knutson, David J Haile, Jiang Qian, Sonia Levi, Paolo Santambrogio, Michael D Garrick, Jacques B Lamarche. Acta Neuropathol 2007
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Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation.
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Friedreich ataxia: neuropathology revised.
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The dorsal root ganglion in Friedreich's ataxia.
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Molecular and functional alterations in a mouse cardiac model of Friedreich ataxia: activation of the integrated stress response, eIF2α phosphorylation, and the induction of downstream targets.
Michael Li-Hsuan Huang, Sutharshani Sivagurunathan, Samantha Ting, Patric J Jansson, Christopher J D Austin, Matthew Kelly, Christopher Semsarian, Daohai Zhang, Des R Richardson. Am J Pathol 2013
Michael Li-Hsuan Huang, Sutharshani Sivagurunathan, Samantha Ting, Patric J Jansson, Christopher J D Austin, Matthew Kelly, Christopher Semsarian, Daohai Zhang, Des R Richardson. Am J Pathol 2013
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Frataxin deficiency leads to reduced expression and impaired translocation of NF-E2-related factor (Nrf2) in cultured motor neurons.
Valentina D'Oria, Stefania Petrini, Lorena Travaglini, Chiara Priori, Emanuela Piermarini, Sara Petrillo, Barbara Carletti, Enrico Bertini, Fiorella Piemonte. Int J Mol Sci 2013
Valentina D'Oria, Stefania Petrini, Lorena Travaglini, Chiara Priori, Emanuela Piermarini, Sara Petrillo, Barbara Carletti, Enrico Bertini, Fiorella Piemonte. Int J Mol Sci 2013
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Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia.
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Cellular iron uptake, trafficking and metabolism: Key molecules and mechanisms and their roles in disease.
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Control of iron homeostasis by an iron-regulated ubiquitin ligase.
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An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1.
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Deletion of the mitochondrial carrier genes MRS3 and MRS4 suppresses mitochondrial iron accumulation in a yeast frataxin-deficient strain.
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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
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