Rab-like small GTPases in the regulation of ciliary Bardet-Biedl syndrome (BBS) complex transport

FEBS J. 2022 Dec;289(23):7359-7367. doi: 10.1111/febs.16232. Epub 2021 Oct 31.

Abstract

Primary cilia, microtubule-based hair-like structures protruding from most cells, contain membranes enriched in signaling molecules and function as sensory and regulatory organelles critical for development and tissue homeostasis. Intraflagellar transport (IFT), cilia-specific bidirectional transport, is required for the assembly, maintenance, and function of cilia. BBSome, the coat complex, acts as the adaptor between the IFT complex and membrane proteins and is therefore essential for establishing the specific compartmentalization of signaling molecules in the cilia. Recent findings have revealed that three ciliary Rab-like small GTPases, IFT27, IFT22, and Rabl2, play critical regulatory roles in ciliary BBSome transport. In this review, we provide an overview of these three Rab-like small GTPases and their relationship with BBSome.

Keywords: BBSome; Bardet-Biedl syndrome; Cilia; IFT22; IFT27; Rab-like; Rabl2; small GTPases.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Bardet-Biedl Syndrome* / genetics
  • Cilia
  • Humans
  • Monomeric GTP-Binding Proteins* / genetics

Substances

  • Monomeric GTP-Binding Proteins