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Spatial control of nucleoporin condensation by fragile X‐related proteins
Author(s) -
AgoteAran Arantxa,
Schmucker Stephane,
Jerabkova Katerina,
Jmel Boyer Inès,
Berto Alessandro,
Pacini Laura,
Ronchi Paolo,
Kleiss Charlotte,
Guerard Laurent,
Schwab Yannick,
Moine Hervé,
Mandel JeanLouis,
Jacquemont Sebastien,
Bagni Claudia,
Sumara Izabela
Publication year - 2020
Publication title -
the embo journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.484
H-Index - 392
eISSN - 1460-2075
pISSN - 0261-4189
DOI - 10.15252/embj.2020104467
Subject(s) - humanities , national library , political science , library science , art , computer science
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.