BBS2 and Bardet-Biedl syndrome: To examine the potential effect of BBS mutations on kidney organoid formation we focused on BBS10. BBS10 mutations are associated with the most severe renal BBS phenotypes (Imhoff et al., 2011; Esposito et al., 2017; Forsythe et al., 2017) and over 20 pathogenic variants have been reported in the United Kingdom (Forsythe et al., 2017).