Animal models of nonsense-associated BBS and AS such as Bbs2-, Bbs4-, Alms1- and alms1-null mice and zebrafish have been previously reported and can be utilised for future studies [93,94] The restoration of full-length proteins BBS2 and ALMS1 and the correction of both anatomical and functional ciliary defects reported in our proof-of-concept study hold immense promise for future ciliopathy research and importantly, the patients affected by these syndromes. Here, BBS4 is linked to ciliopathy.