Neurons and cardiomyocytes derived from induced pluripotent stem cells as a model for mitochondrial defects in Friedreich’s ataxia

Friedreich’s ataxia (FRDA) is a recessive neurodegenerative disorder commonly associated with hypertrophic cardiomyopathy. FRDA is due to expanded GAA repeats within the first intron of the gene encoding frataxin, a conserved mitochondrial protein involved in iron-sulphur cluster biosynthesis. This...

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Detalles Bibliográficos
Autores principales: Hick, Aurore, Wattenhofer-Donzé, Marie, Chintawar, Satyan, Tropel, Philippe, Simard, Jodie P., Vaucamps, Nadège, Gall, David, Lambot, Laurie, André, Cécile, Reutenauer, Laurence, Rai, Myriam, Teletin, Marius, Messaddeq, Nadia, Schiffmann, Serge N., Viville, Stéphane, Pearson, Christopher E., Pandolfo, Massimo, Puccio, Hélène
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Limited 2013
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3634645/
https://www.ncbi.nlm.nih.gov/pubmed/23136396
http://dx.doi.org/10.1242/dmm.010900

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