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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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
Format: Artigo
Jezik:Inglês
Izdano: The Company of Biologists Limited 2013
Teme:
Research Article
Online dostop: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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