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Article

Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway.

Nature neuroscience - 1 Aug 2021

Lin Yen-Chen, Kumar Meenakshi Sundaram, Ramesh Nandini, Anderson Eric N, Nguyen Aivi T, Kim Boram, Cheung Simon, McDonough Justin A, Skarnes William C, Lopez-Gonzalez Rodrigo, Landers John E, Fawzi Nicolas L, Mackenzie Ian R A, Lee Edward B, Nickerson Jeffrey A, Grunwald David, Pandey Udai B, Bosco Daryl A

Abstract excerpt

Nucleocytoplasmic transport (NCT) decline occurs with aging and neurodegeneration. Here, we investigated the NCT pathway in models of amyotrophic lateral sclerosis-fused in sarcoma (ALS-FUS). Expression of ALS-FUS led to a reduction in NCT and nucleoporin (Nup) density within the nuclear membrane of human neurons. FUS and Nups were found to interact independently of RNA in cells and to alter the phase-separation...

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Identifiers

PMID: 34059832
DOI: 10.1038/s41593-021-00859-9
PMCID: PMC8832378

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