Machida et al. (36) and Alpi et al. (37) have shown that UBE2T is the E2 conjugating ligase in the FA pathway and that genetic deficiency in UBE2T−/− DT40 cells leads to the classical cellular phenotypes of FA, including hypersensitivity to low doses of DNA ICL agents and high frequencies of chromosomal abnormalities. This evidence concerns the gene UBE2T and Friedreich ataxia.