Trafficking of ciliary membrane proteins by the intraflagellar transport/BBSome machinery

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  • Jenna L Wingfield, Department of Cellular Biology, University of Georgia, Athens, GA 30602, U.S.A.
  • ,
  • Karl-Ferdinand Lechtreck, Department of Cellular Biology, University of Georgia, Athens, GA 30602, U.S.A. el@mbg.au.dk lechtrek@uga.edu.
  • ,
  • Esben Lorentzen

Bardet-Biedl syndrome (BBS) is a rare inherited disease caused by defects in the BBSome, an octameric complex of BBS proteins. The BBSome is conserved in most organisms with cilia, which are microtubule (MT)-based cell organelles that protrude from the cell surface and function in motility and sensing. Cilia assembly, maintenance, and function require intraflagellar transport (IFT), a bidirectional motility of multi-megadalton IFT trains propelled by molecular motors along the ciliary MTs. IFT has been shown to transport structural proteins, including tubulin, into growing cilia. The BBSome is an adapter for the transport of ciliary membrane proteins and cycles through cilia via IFT. While both the loss and the abnormal accumulation of ciliary membrane proteins have been observed in bbs mutants, recent data converge on a model where the BBSome mainly functions as a cargo adapter for the removal of certain transmembrane and peripheral membrane proteins from cilia. Here, we review recent data on the ultrastructure of the BBSome and how the BBSome recognizes its cargoes and mediates their removal from cilia.

Original languageEnglish
JournalEssays in Biochemistry
Volume62
Issue6
Pages (from-to)753-763
ISSN0071-1365
DOIs
Publication statusPublished - 4 Oct 2018

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