Organization, functions, and mechanisms of the BBSome in development, ciliopathies, and beyond.
BBSome
Cilia
cell biology
ciliopathy
cytoskeleton
Journal
eLife
ISSN: 2050-084X
Titre abrégé: Elife
Pays: England
ID NLM: 101579614
Informations de publication
Date de publication:
19 07 2023
19 07 2023
Historique:
received:
10
03
2023
accepted:
06
07
2023
medline:
21
7
2023
pubmed:
19
7
2023
entrez:
19
7
2023
Statut:
epublish
Résumé
The BBSome is an octameric protein complex that regulates ciliary transport and signaling. Mutations in BBSome subunits are closely associated with ciliary defects and lead to ciliopathies, notably Bardet-Biedl syndrome. Over the past few years, there has been significant progress in elucidating the molecular organization and functions of the BBSome complex. An improved understanding of BBSome-mediated biological events and molecular mechanisms is expected to help advance the development of diagnostic and therapeutic approaches for BBSome-related diseases. Here, we review the current literature on the structural assembly, transport regulation, and molecular functions of the BBSome, emphasizing its roles in cilium-related processes. We also provide perspectives on the pathological role of the BBSome in ciliopathies as well as how these can be exploited for therapeutic benefit.
Identifiants
pubmed: 37466224
doi: 10.7554/eLife.87623
pii: 87623
pmc: PMC10356136
doi:
pii:
Types de publication
Review
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
© 2023, Tian, Zhao et al.
Déclaration de conflit d'intérêts
XT The authors declare no competing interests, HZ, JZ No competing interests declared
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