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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Bibliographic Details
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: Online
Language:English
Published: The Company of Biologists Limited 2013
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3634645/