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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Dettagli Bibliografici
Autori principali: 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
Natura: Artigo
Lingua:Inglês
Pubblicazione: The Company of Biologists Limited 2013
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Research Article
Accesso online: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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