Heterozygous mutations of BRCA2, BRIP1 (FANCJ) and PALB2 (FANCN) are not sufficient to develop FA phenotypes, but they are associated with a dramatic increased risk for breast, ovarian and other cancers (Berwick et al. 2007; Catucci et al. 2014; Seal et al. 2006). Here, BRCA2 is linked to Friedreich ataxia.