The BBSome is a stable complex that includes eight proteins (BBS1, 2, 4, 5, 7, 8, 9, and 18), encoded by causative genes of Bardet–Bield Syndrome (BBS), and the small GTPase ARL6/BBS3, which connects the complex with the ciliary membrane by its interaction with BBS1 (Jin et al., 2010; Prevo et al., 2017). Here, BBS1 is linked to Bardet-Biedl syndrome.