%0 Review
%T Organization, functions, and mechanisms of the BBSome in development, ciliopathies, and beyond.
%A Tian X
%A Zhao H
%A Zhou J
%J Elife
%V 12
%N 0
%D 2023 07 19
%M 37466224
%F 8.713
%R 10.7554/eLife.87623
%X The BBSome is an octameric protein complex that regulates ciliary transport and signaling. Mutations in BBSome subunits are closely associated with ciliary defects and lead to ciliopathies, notably Bardet-Biedl syndrome. Over the past few years, there has been significant progress in elucidating the molecular organization and functions of the BBSome complex. An improved understanding of BBSome-mediated biological events and molecular mechanisms is expected to help advance the development of diagnostic and therapeutic approaches for BBSome-related diseases. Here, we review the current literature on the structural assembly, transport regulation, and molecular functions of the BBSome, emphasizing its roles in cilium-related processes. We also provide perspectives on the pathological role of the BBSome in ciliopathies as well as how these can be exploited for therapeutic benefit.