Frataxin is a mitochondrial protein that, at concentrations below 40% of normal levels, leads to Friedreich ataxia (FA), a disease caused by the presence of large expansions of GAA triplet repeats in the first intron of the frataxin (FXN) gene, which results in a low transcription rate and, consequently, diminished frataxin expression [1]. The gene discussed is FXN; the disease is Friedreich ataxia.