Whole eye proteomic analysis of bbs1 zebrafish mutants

Bardet-Biedl syndrome (BBS) is a ciliopathy characterized by retinal dystrophy, intellectual disability, polydactyly, obesity and renal anomalies. In photoreceptors (PR), light sensation occurs in outer segments (OSs), which are specialized primary cilia. BBS1, the major BBS gene, is part of a protein complex called “BBSome”, which is involved in intracellular protein transport. However, the precise function of BBS1/BBSome in controlling trafficking of ciliary proteins in PRs remains unclear. We generated a bbs1 knock out zebrafish mutant to investigate the role of Bbs1 and the BBSome in vivo. To detect global changes in the eye proteome and to verify the knock-out of Bbs1, we conducted a whole eyes proteomic investigation.