Friedreich ataxia (FRDA) is the commonest autosomal recessive ataxic condition, with 95 % of cases caused by a homozygous GAA.TCC tri-nucleotide repeat expansion mutation within intron 1 of the FXN gene [6] leading to transcriptional repression of the mitochondrial protein frataxin [15, 46]. This evidence concerns the gene FXN and Friedreich ataxia.