Pathophysiology of Primary Cilia: Signaling and Proteostasis Regulation.
E3 ligase
PKA
autophagy
cAMP
proteasome
signaling
ubiquitin
Journal
Frontiers in cell and developmental biology
ISSN: 2296-634X
Titre abrégé: Front Cell Dev Biol
Pays: Switzerland
ID NLM: 101630250
Informations de publication
Date de publication:
2022
2022
Historique:
received:
10
12
2021
accepted:
21
04
2022
entrez:
1
6
2022
pubmed:
2
6
2022
medline:
2
6
2022
Statut:
epublish
Résumé
Primary cilia are microtubule-based, non-motile sensory organelles present in most types of growth-arrested eukaryotic cells. They are transduction hubs that receive and transmit external signals to the cells in order to control growth, differentiation and development. Mutations of genes involved in the formation, maintenance or disassembly of ciliary structures cause a wide array of developmental genetic disorders, also known as ciliopathies. The primary cilium is formed during G1 in the cell cycle and disassembles at the G2/M transition. Following the completion of the cell division, the cilium reassembles in G1. This cycle is finely regulated at multiple levels. The ubiquitin-proteasome system (UPS) and the autophagy machinery, two main protein degradative systems in cells, play a fundamental role in cilium dynamics. Evidence indicate that UPS, autophagy and signaling pathways may act in synergy to control the ciliary homeostasis. However, the mechanisms involved and the links between these regulatory systems and cilium biogenesis, dynamics and signaling are not well defined yet. Here, we discuss the reciprocal regulation of signaling pathways and proteolytic machineries in the control of the assembly and disassembly of the primary cilium, and the impact of the derangement of these regulatory networks in human ciliopathies.
Identifiants
pubmed: 35646931
doi: 10.3389/fcell.2022.833086
pii: 833086
pmc: PMC9130585
doi:
Types de publication
Journal Article
Review
Langues
eng
Pagination
833086Informations de copyright
Copyright © 2022 Senatore, Iannucci, Chiuso, Delle Donne, Rinaldi and Feliciello.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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