SLX4, together with associated nucleases, is important for the repair of DNA inter-strand crosslinks (ICLs) and, consequently, bi-allelic mutations in SLX4 (also known as FANCP) cause Fanconi anemia (FA) (Kim et al., 2011; Stoepker et al., 2011), a rare condition characterized by developmental defects, bone marrow failure and cancer predisposition (Moldovan and D'Andrea, 2009). Here, SLX4 is linked to Friedreich ataxia.