Our experiments to model recombination at the UBE2T locus were prompted by an FA patient with inactivating duplication and deletion mutations in UBE2T and whose fibroblasts were hypersensitive to the genotoxins DEB and cisplatin, and defective in monoubiquitination of FANCD2 in response to MMC (40). Here, FANCD2 is linked to Friedreich ataxia.