The intronic GAA repeat expansion causing Friedreich ataxia is located in intron one of the frataxin (FXN) gene, and results in reduced expression of the FXN transcript, potentially due to the formation of a triplex DNA structure and “sticky DNA” (Sakamoto et al. 1999).The mechanism by which reduced frataxin, a mitochondrial protein involved in iron-sulphur cluster regulation, causes neurodegeneration is debated, but recent research support a neuroinflammatory mechanism (Adinolfi et al. 2009; Hayashi et al. 2014). This evidence concerns the gene FXN and Friedreich ataxia.