The Fanconi anaemia (FA) pathway constitutes a central genome maintenance system that orchestrates the repair of DNA interstrand crosslinks (ICLs) through the coordinated monoubiquitination of FANCI and FANCD2, followed by recruitment of repair effectors such as BRCA1, REV1, and ERCC1 [32]. Here, BRCA1 is linked to Friedreich ataxia.