CEP164 and Bardet-Biedl syndrome: We further showed that CEP164, which is mutated in nephronophthisis and Bardet-Biedl syndrome (BBS), both of which are classified as ciliopathies [34, 35], directly interacts with and recruits Cby1 to the distal appendage/transition fiber of the mother centriole/basal body during primary ciliogenesis [25].