Ubiquitylation and the Fanconi anemia pathway

► The Fanconi anemia (FA) is a bone marrow and cancer predisposition syndrome. ► The FA pathway is responsible for repair of interstrand DNA crosslinks. ► FA core complex ubiquitylates FANCI and FANCD2 which localize to chromatin and direct repair. ► Ubiquitin interaction motifs are used to recruit nucleases to sites of damage. ► Deubiquitylation plays an important role in terminating DNA damage signaling.