In the genetic disease Friedreich’s Ataxia (FRDA), a homozygous GAA (Guanine-Adenine-Adenine) trinucleotide expansion in intron 1 of the FXN nuclear locus partly silences de novo transcription leading to a reduction in intracellular levels of FXN protein1, 2. Here, FXN is linked to Friedreich ataxia.