Trafficking of ciliary membrane proteins by the intraflagellar transport/BBSome machinery
| Autor: | Jenna L Wingfield, Esben Lorentzen, Karl-Ferdinand Lechtreck |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
congenital hereditary and neonatal diseases and abnormalities BBSome Microtubules Biochemistry Article 03 medical and health sciences 0302 clinical medicine Microtubule Intraflagellar transport Animals Humans Cilia Bardet-Biedl Syndrome Molecular Biology Ciliary membrane Chemistry Cilium Cell Membrane Peripheral membrane protein Membrane Proteins Biological Transport Transmembrane protein Cell biology 030104 developmental biology Membrane protein Flagella sense organs 030217 neurology & neurosurgery |
| Zdroj: | Wingfield, J L, Lechtreck, K-F & Lorentzen, E 2018, ' Trafficking of ciliary membrane proteins by the intraflagellar transport/BBSome machinery ', Essays in Biochemistry, vol. 62, no. 6, pp. 753-763 . https://doi.org/10.1042/EBC20180030 |
| DOI: | 10.1042/EBC20180030 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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