The Fanconi anemia (FA) repair pathway promotes the excision of ICLs using at least 22 FANC proteins, including FANCS/BRCA1 and FANCD1/BRCA2 (Kottemann and Smogorzewska, 2013; Wang and Smogorzewska, 2015; Ceccaldi et al., 2016). This evidence concerns the gene BRCA2 and Friedreich ataxia.