Moreover, monoubiquitinated FANCD2 serves as a signal to recruit to the replication fork, DNA repair proteins that contain ubiquitin-binding motifs [101], such as FA complementation group P (FANCP, also called SLX4) and FA complementation group Q (FANCQ, also called ERCC), to remove the cross-linked DNA [54,55] (Figure 1C). The gene discussed is ERCC4; the disease is Friedreich ataxia.