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

• Main Authors: Aurore Hick (Author), Marie Wattenhofer-Donzé (Author), Satyan Chintawar (Author), Philippe Tropel (Author), Jodie P. Simard (Author), Nadège Vaucamps (Author), David Gall (Author), Laurie Lambot (Author), Cécile André (Author), Laurence Reutenauer (Author), Myriam Rai (Author), Marius Teletin (Author), Nadia Messaddeq (Author), Serge N. Schiffmann (Author), Stéphane Viville (Author), Christopher E. Pearson (Author), Massimo Pandolfo (Author), Hélène Puccio (Author)
• Format: Book
• Published: The Company of Biologists, 2013-05-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

SUMMARY 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 biosyn...