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.
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December 2018
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Cover Image
Cover Image
The cover image shows microtubule catastrophe, the moment at which the polymer begins to fall apart by outward peeling of longitudinally connected stretches of tubulin, known as protofilaments. This mode of depolymerisation has long been observed thanks to nanometre-scale electron microscopy, but it lacked mechanistic explanation. Recent work addressed the structural basis of microtubule dynamic instability using cryo-electron microscopy (cryo-EM) at near-atomic resolution. This and other major insights into microtubule biology revealed by cryo-EM are reviewed by Manka and Moores in this issue (see pages 737–751). The atomic surface model was created in ChimeraX using experimentally determined tubulin conformations and was kindly provided by Szymon Manka (Birkbeck, University of London).
Review Article|
October 04 2018
Trafficking of ciliary membrane proteins by the intraflagellar transport/BBSome machinery
Jenna L. Wingfield;
Jenna L. Wingfield
1Department of Cellular Biology, University of Georgia, Athens, GA 30602, U.S.A.
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Karl-Ferdinand Lechtreck;
1Department of Cellular Biology, University of Georgia, Athens, GA 30602, U.S.A.
Correspondence: Esben Lorentzen (el@mbg.au.dk) or Karl-Ferdinand Lechtreck (lechtrek@uga.edu)
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Esben Lorentzen
2Department of Molecular Biology and Genetics, Aarhus University, Gustav Wieds Vej 10c, Aarhus C DK-8000, Denmark
Correspondence: Esben Lorentzen (el@mbg.au.dk) or Karl-Ferdinand Lechtreck (lechtrek@uga.edu)
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Publisher: Portland Press Ltd
Received:
June 29 2018
Revision Received:
September 04 2018
Accepted:
September 19 2018
Online ISSN: 1744-1358
Print ISSN: 0071-1365
© 2018 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society
2018
Essays Biochem (2018) 62 (6): 753–763.
Article history
Received:
June 29 2018
Revision Received:
September 04 2018
Accepted:
September 19 2018
Citation
James G. Wakefield, Carolyn A. Moores, Jenna L. Wingfield, Karl-Ferdinand Lechtreck, Esben Lorentzen; Trafficking of ciliary membrane proteins by the intraflagellar transport/BBSome machinery. Essays Biochem 7 December 2018; 62 (6): 753–763. doi: https://doi.org/10.1042/EBC20180030
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