Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death

Fatima Gasset-Rosa; Shan Lu; Haiyang Yu; Cong Chen; Ze'ev Melamed; Lin Guo; James Shorter; Sandrine Da Cruz; Don W. Cleveland

doi:10.1016/j.neuron.2019.02.038

Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death

Fatima Gasset-Rosa, Shan Lu, Haiyang Yu, Cong Chen, Ze'ev Melamed, Lin Guo, James Shorter, Sandrine Da Cruz^*, Don W. Cleveland

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

297 Scopus citations

Abstract

TDP-43 aggregation is the major hallmark of multiple neurodegenerative diseases, including ALS and FTD. Gasset-Rosa et al. demonstrate that transient stress induces long-lasting cytoplasmic TDP-43 de-mixing independent of stress granules, driving nuclear import defects, nuclear TDP-43 clearance, and cell death.

Original language	English
Pages (from-to)	339-357.e7
Journal	Neuron
Volume	102
Issue number	2
DOIs	https://doi.org/10.1016/j.neuron.2019.02.038
State	Published - 17 Apr 2019
Externally published	Yes

Bibliographical note

Publisher Copyright:
© 2019 Elsevier Inc.

Keywords

ALS/FTD
RNA-binding proteins
TDP-43
TDP-43 de-mixing
iPSCs
liquid-liquid phase separation
low complexity domains
motor neurons
neurodegeneration
nucleocytoplasmic transport
stress granules

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10.1016/j.neuron.2019.02.038

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title = "Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death",

abstract = "TDP-43 aggregation is the major hallmark of multiple neurodegenerative diseases, including ALS and FTD. Gasset-Rosa et al. demonstrate that transient stress induces long-lasting cytoplasmic TDP-43 de-mixing independent of stress granules, driving nuclear import defects, nuclear TDP-43 clearance, and cell death.",

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AU - Lu, Shan

AU - Yu, Haiyang

AU - Chen, Cong

AU - Melamed, Ze'ev

AU - Guo, Lin

AU - Shorter, James

AU - Da Cruz, Sandrine

AU - Cleveland, Don W.

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KW - liquid-liquid phase separation

KW - low complexity domains

KW - motor neurons

KW - neurodegeneration

KW - nucleocytoplasmic transport

KW - stress granules

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Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death

Abstract

Bibliographical note

Keywords

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