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...
Main Authors: | , , , , , , , , , , , , , , , , , |
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Format: | Online |
Language: | English |
Published: |
The Company of Biologists Limited
2013
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Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3634645/ |