Our genetic analysis demonstrated that expression of the FANCJ-A349P mutant allele in FANCJ-null FA-J patient cells failed to rescue sensitivity to the DNA cross-linking agent MMC; similarly, expression of FANCJ-A349P in fancj null chicken DT40 cells failed to rescue cisplatin sensitivity (Wu et al., 2010). Here, BRIP1 is linked to Friedreich ataxia.