In addition to RBS, there are other developmental syndromes caused by mutations in SCC components: (1) Cornelia de Lange syndrome (CdLS), caused by mutations in NIPBL, SMC1, SMC3, HDAC8 and RAD21 (Krantz et al., 2004; Tonkin et al., 2004; Musio et al., 2006; Deardorff et al., 2007; Liu and Krantz, 2008); (2) Warsaw breakage syndrome (WABS), caused by mutations in DDX11 (van der Lelij et al., 2010; Capo-Chichi et al., 2013); and (3) chronic atrial and intestinal dysrhythmia (CAID), caused by mutations in SGOL1 (Chetaille et al., 2014). The gene discussed is DDX11; the disease is chronic atrial and intestinal dysrhythmia.