Thus, the identification of UBE2T as the 18th FA gene is consistent with current models of the FA pathway and confirms again the intimate connection between the ‘early’ proteins in the FA pathway, including FANCA/B/C/E/F/G/L/M and UBE2T (T), which together mediate the central step activation step, monoubiquitination of FANCD2 and FANCI. Here, UBE2T is linked to Friedreich ataxia.