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Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway
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Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway
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Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway
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Journal Article
Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway
2021
Overview
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 properties of each other in vitro. FUS–Nup interactions were not localized to nuclear pores, but were enriched in the nucleus of control neurons versus the cytoplasm of mutant neurons. Our data indicate that the effect of ALS-linked mutations on the cytoplasmic mislocalization of FUS, rather than on the physiochemical properties of the protein itself, underlie our reported NCT defects. An aberrant interaction between mutant FUS and Nups is underscored by studies in
Drosophila
, whereby reduced Nup expression rescued multiple toxic FUS-induced phenotypes, including abnormal nuclear membrane morphology in neurons.
ALS-linked mutations in
FUS
trigger defects within the nucleocytoplasmic transport pathway in human neurons and
Drosophila
. Aberrant interactions between FUS and nucleoporins are thought to underlie these defects.
Publisher
Nature Publishing Group US,Nature Publishing Group
Subject
/ Active Transport, Cell Nucleus - physiology
/ Aging
/ Amyotrophic lateral sclerosis
/ Amyotrophic Lateral Sclerosis - genetics
/ Amyotrophic Lateral Sclerosis - metabolism
/ Animal Genetics and Genomics
/ Animals
/ Animals, Genetically Modified
/ Biomedical and Life Sciences
/ Cellular signal transduction
/ Defects
/ FUS gene
/ Humans
/ Insects
/ Mutants
/ Mutation
/ Neurons
/ Nuclear Pore Complex Proteins - metabolism
/ Protein-protein interactions
/ RNA-Binding Protein FUS - genetics
/ RNA-Binding Protein FUS - metabolism
/ Sarcoma
