Trafficking of ciliary membrane proteins by the intraflagellar transport/BBSome machinery
Open accessAbstract
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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BBSome proteins were initially thought to facilitate delivery of GPCRs to cilia, but it has been proposed that they primarily regulate ciliary export of signalling proteins.
“The BBSome proteins were initially thought to facilitate the delivery of GPCRs to cilia 73–75; however, it has been proposed that BBSome proteins primarily regulate ciliary export of signalling proteins 72, 76, 77, 78.”
From Cellular signalling by primary cilia in development, organ function and disease (2019) · cites this paper as [78]
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References 96 · Cited by 157
Research works printed but not linked: 1
- [14]Sloboda RD. Preface. Widespread interest in the primary cilium. Methods Cell Biol 2009; 94: xv– xvi. 19963094
- Authors
- Wingfield, J. L., Lechtreck, K.-F., & Lorentzen, E.
- Affiliations
- University of Georgia (US)
- Aarhus University (DK)
- Corresponding author
- Lechtreck, Karl-Ferdinand
- Lorentzen, Esben
- Venue
- Essays in Biochemistry
- Publisher
- Portland Press Ltd.
- Published
- 2018-10-04
- Volume
- 62
- Issue
- 6
- Pages
- 753-763
- ISSN
- 0071-1365 (ISSN-L)
- 1744-1358
- PMC
- PMC6737936
- OpenAlex
- W2895602084
- Cited by
- 157
- References
- 96
- Citations by year
- 2026: 14 · 2025: 20 · 2024: 17 · 2023: 23 · 2022: 24 · 2021: 21 · 2020: 24 · 2019: 14
- FWCI
- 2.03
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Essays in Biochemistry (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Novo Nordisk Fonden (NNF15OC0016050)
- National Institute of General Medical Sciences (R01GM110413)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Kidney Cyst Diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Keywords
- Bardet-Biedl syndrome, BBSome, intraflagellar transport, ciliary membrane proteins, cilia, cargo recognition, transmembrane proteins, peripheral membrane proteins, ciliary ultrastructure, molecular motors, microtubules
- MeSH terms
- Animals, Biological Transport, Cell Membrane, Cilia, Flagella, Humans, Membrane Proteins, Microtubules, Bardet-Biedl Syndrome
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