Aconitase is an Fe–S-containing enzyme that exists in both mitochondrial and cytosolic forms, and a deficit in aconitase activity has previously been identified as a major effect of decreased frataxin in FRDA patients, mouse models and yeast models (Al-Mahdawi et al., 2006; Bradley et al., 2000; Puccio et al., 2001; Rotig et al., 1997). This evidence concerns the gene FXN and Friedreich ataxia.