NLS-binding deficient Kapβ2 reduces neurotoxicity via selective interaction with C9orf72-ALS/FTD dipeptide repeats

Abstract Arginine-rich dipeptide repeat proteins (R-DPRs) are highly toxic proteins found in patients with C9orf72-linked amyotrophic lateral sclerosis and frontotemporal dementia (C9-ALS/FTD). R-DPRs can cause toxicity by disrupting the natural phase behavior of RNA-binding proteins (RBPs). Mitigat...

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Main Authors: Kevin M. Kim, Amandeep Girdhar, Maria E. Cicardi, Vaishnavi Kankate, Miyuki Hayashi, Ruoyu Yang, Jenny L. Carey, Charlotte M. Fare, James Shorter, Gino Cingolani, Davide Trotti, Lin Guo
Format: Article
Language:English
Published: Nature Portfolio 2025-01-01
Series:Communications Biology
Online Access:https://doi.org/10.1038/s42003-024-07412-x
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