Oxidative stress is known to be associated with genome instability,11 and in FRDA cells that have decreased frataxin expression, reduced capacity for DNA damage repair is evident.8, 12 Differential expression of genes associated with genotoxicity stress, including oxidative phosphorylation, has also been found in peripheral blood mononuclear cells of FRDA patients, where mitochondrial and nuclear DNA damage is increased.13 In the yeast model of FRDA, reduced levels of frataxin correlate with DNA damage and recombination, mutation events and genome instability. This evidence concerns the gene FXN and Friedreich ataxia.