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research article

Spatial control of nucleoporin condensation by fragile X-related proteins

Agote-Aran, Arantxa
•
Schmucker, Stephane
•
Jerabkova, Katerina
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July 24, 2020
Embo Journal

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.

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Type
research article
DOI
10.15252/embj.2020104467
Web of Science ID

WOS:000551520600001

Author(s)
Agote-Aran, Arantxa
Schmucker, Stephane
Jerabkova, Katerina
Boyer, Ines Jmel
Berto, Alessandro  
Pacini, Laura
Ronchi, Paolo
Kleiss, Charlotte
Guerard, Laurent
Schwab, Yannick
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Date Issued

2020-07-24

Publisher

WILEY

Published in
Embo Journal
Article Number

e104467

Subjects

Biochemistry & Molecular Biology

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Cell Biology

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dynein

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fragile x syndrome

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fxr1

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nucleoporins

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phase separation

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nuclear-pore complex

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annulate lamellae

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phase-separation

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fmrp

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progression

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expression

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maturation

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diffusion

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transport

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membrane

Editorial or Peer reviewed

REVIEWED

Written at

EPFL

EPFL units
UPGON  
Available on Infoscience
August 6, 2020
Use this identifier to reference this record
https://infoscience.epfl.ch/handle/20.500.14299/170656
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