FANCL is an E3 ubiquitin ligase and is a component of the multiprotein core complex in the FA pathway.[12,13] FANCL interacts with FANCT, which serves as the E2-ubiquitin conjugating enzyme, and together they participate in the transfer of ubiquitin moieties to FANCD2 and FANCI. The gene discussed is UBE2E2; the disease is Friedreich ataxia.