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

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

266 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 (US)
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 - Apr 17 2019

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

ASJC Scopus subject areas

General Neuroscience

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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.",

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",

author = "Fatima Gasset-Rosa and Shan Lu and Haiyang Yu and Cong Chen and Ze'ev Melamed and Lin Guo and James Shorter and {Da Cruz}, Sandrine and Cleveland, {Don W.}",

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AU - Gasset-Rosa, Fatima

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.

PY - 2019/4/17

Y1 - 2019/4/17

N2 - 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.

AB - 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.

KW - ALS/FTD

KW - RNA-binding proteins

KW - TDP-43

KW - TDP-43 de-mixing

KW - iPSCs

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

Keywords

ASJC Scopus subject areas

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