Spatial control of nucleoporin condensation by fragile X-related proteins

Agote-Aran, Arantxa and Schmucker, Stephane and Jerabkova, Katerina and Jmel Boyer, Inès and Berto, Alessandro and Pacini, Laura and Ronchi, Paolo and Kleiss, Charlotte and Guerard, Laurent and Schwab, Yannick and Moine, Hervé and Mandel, Jean-Louis and Jacquemont, Sebastien and Bagni, Claudia and Sumara, Izabela. (2020) Spatial control of nucleoporin condensation by fragile X-related proteins. The EMBO Journal, 39 (20). e104467.

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Official URL: https://edoc.unibas.ch/79585/

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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.
Faculties and Departments:05 Faculty of Science > Departement Biozentrum > Services Biozentrum > Imaging Core Facility (Biehlmaier)
UniBasel Contributors:Guerard, Laurent
Item Type:Article, refereed
Article Subtype:Research Article
Publisher:Nature Publishing Group
Note:Publication type according to Uni Basel Research Database: Journal article
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Last Modified:27 Jan 2022 14:44
Deposited On:27 Jan 2022 14:44

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