Our results demonstrate, for the first time, (i) that tocotrienol induces in FRDA patients the upregulation of the FXN-3 mRNA isoform and (ii) that FXN-3 and FXN-2 proteins share the same 3D structure as FXN-1 and are apparently able to complex with NFS1/ISCU, thus suggesting that they may play the same functions. Here, NFS1 is linked to Friedreich ataxia.