In accord with its role as a tumor suppressor, FANCJ was identified as the gene mutated in the J complementation group of Fanconi anemia (FA), a rare disorder characterized by progressive bone marrow failure, skeletal abnormalities, and cancer (Levitus et al., 2005; Levran et al., 2005; Litman et al., 2005). This evidence concerns the gene BRIP1 and Friedreich ataxia.