PALB2 (Partner and Localizer of BRCA2) was discovered as a BRCA2-interacting protein and later characterized as a tumor suppressor and a member (FANCN) of the Fanconi Anemia (FA) pathway, which also includes BRCA2 (FANCD1) and other HR proteins (BRCA1-FANCS, RAD51-FANCR, RAD51C-FANCO, BRIP1-FANCJ, XRCC2-FANCU) (Figure 2A) [11,192,193,194,195,196]. Here, BRIP1 is linked to Friedreich ataxia.