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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Detalhes bibliográficos
Main Authors: 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: Artigo
Idioma:Inglês
Publicado em: The Company of Biologists Limited 2013
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Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3634645/
https://ncbi.nlm.nih.gov/pubmed/23136396
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1242/dmm.010900
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