Spatial control of nucleoporin condensation by fragile X-related proteins.

Détails

Ressource 1Télécharger: 32706158_BIB_F93EA3DA3D5C.pdf (5297.78 [Ko])
Etat: Public
Version: Final published version
Licence: CC BY-NC-ND 4.0
ID Serval
serval:BIB_F93EA3DA3D5C
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Spatial control of nucleoporin condensation by fragile X-related proteins.
Périodique
The EMBO journal
Auteur⸱e⸱s
Agote-Aran A., Schmucker S., Jerabkova K., Jmel Boyer I., Berto A., Pacini L., Ronchi P., Kleiss C., Guerard L., Schwab Y., Moine H., Mandel J.L., Jacquemont S., Bagni C., Sumara I.
ISSN
1460-2075 (Electronic)
ISSN-L
0261-4189
Statut éditorial
Publié
Date de publication
15/10/2020
Peer-reviewed
Oui
Volume
39
Numéro
20
Pages
e104467
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Résumé
Nucleoporins (Nups) build highly organized nuclear pore complexes (NPCs) at the nuclear envelope (NE). Several Nups assemble into a sieve-like hydrogel within the central channel of the NPCs. In the cytoplasm, the soluble Nups exist, but how their assembly is restricted to the NE is currently unknown. Here, we show that fragile X-related protein 1 (FXR1) can interact with several Nups and facilitate their localization to the NE during interphase through a microtubule-dependent mechanism. Downregulation of FXR1 or closely related orthologs FXR2 and fragile X mental retardation protein (FMRP) leads to the accumulation of cytoplasmic Nup condensates. Likewise, models of fragile X syndrome (FXS), characterized by a loss of FMRP, accumulate Nup granules. The Nup granule-containing cells show defects in protein export, nuclear morphology and cell cycle progression. Our results reveal an unexpected role for the FXR protein family in the spatial regulation of nucleoporin condensation.
Mots-clé
Acrylates/pharmacology, Animals, Cell Line, Cell Nucleus/metabolism, Cytoplasm/drug effects, Cytoplasm/metabolism, Down-Regulation, Fragile X Mental Retardation Protein/genetics, Fragile X Mental Retardation Protein/metabolism, Fragile X Syndrome/genetics, Fragile X Syndrome/metabolism, G1 Phase Cell Cycle Checkpoints/genetics, Humans, In Situ Hybridization, Fluorescence, Interphase/genetics, Mice, Microscopy, Electron, Transmission, Microtubules/drug effects, Microtubules/metabolism, Microtubules/ultrastructure, Myoblasts/drug effects, Myoblasts/metabolism, Nuclear Envelope/drug effects, Nuclear Envelope/metabolism, Nuclear Envelope/ultrastructure, Nuclear Pore Complex Proteins/genetics, Nuclear Pore Complex Proteins/metabolism, RNA, Small Interfering, RNA-Binding Proteins/genetics, RNA-Binding Proteins/metabolism, FXR1, dynein, fragile X syndrome, nucleoporins, phase separation
Pubmed
Web of science
Open Access
Oui
Création de la notice
11/08/2020 10:49
Dernière modification de la notice
25/01/2024 7:47
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