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Nuclear and degradative functions of the ESCRT-III pathway: implications for neurodegenerative disease

Nucleus (Austin, Tex.), 2024-12, Vol.15 (1), p.2349085-2349085

2024

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Nuclear pore complexes - a doorway to neural injury in neurodegeneration

Nature reviews. Neurology, 2022-06, Vol.18 (6), p.348

2022

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SUN1 facilitates CHMP7 nuclear influx and injury cascades in sporadic amyotrophic lateral sclerosis

Brain (London, England : 1878), 2024-01, Vol.147 (1), p.109-121

2024

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Nuclear accumulation of CHMP7 initiates nuclear pore complex injury and subsequent TDP-43 dysfunction in sporadic and familial ALS

Science translational medicine, 2021-07, Vol.13 (604)

2021

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Nuclear pore complex and nucleocytoplasmic transport disruption in neurodegeneration

FEBS letters, 2023-10, Vol.597 (20), p.2546-2566

2023

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Stress Granule Assembly Disrupts Nucleocytoplasmic Transport

Cell, 2018-05, Vol.173 (4), p.958-971.e17

2018

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G4C2 Repeat RNA Initiates a POM121-Mediated Reduction in Specific Nucleoporins in C9orf72 ALS/FTD

Neuron (Cambridge, Mass.), 2020-09, Vol.107 (6), p.1124-1140.e11

2020

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CRISPR-Cas9 Screens Identify the RNA Helicase DDX3X as a Repressor of C9ORF72 (GGGGCC)n Repeat-Associated Non-AUG Translation

Neuron (Cambridge, Mass.), 2019-12, Vol.104 (5), p.885-898.e8

2019

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Endocytosis regulates TDP-43 toxicity and turnover

Nature communications, 2017-12, Vol.8 (1), p.2092-14, Article 2092

2017

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Futsch/MAP1B mRNA is a translational target of TDP-43 and is neuroprotective in a Drosophila model of amyotrophic lateral sclerosis

The Journal of neuroscience, 2014-11, Vol.34 (48), p.15962-15974

2014

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Drosophila models of amyotrophic lateral sclerosis with defects in RNA metabolism

Brain research, 2018-08, Vol.1693 (Pt A), p.109-120

2018

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The ESCRT-III protein VPS4, but not CHMP4B or CHMP2B, is pathologically increased in familial and sporadic ALS neuronal nuclei

Acta neuropathologica communications, 2021-07, Vol.9 (1), p.127-127, Article 127

2021

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Post-transcriptional Inhibition of Hsc70-4/HSPA8 Expression Leads to Synaptic Vesicle Cycling Defects in Multiple Models of ALS

Cell reports (Cambridge), 2017-10, Vol.21 (1), p.110-125

2017

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G2C4 targeting antisense oligonucleotides potently mitigate TDP-43 dysfunction in human C9orf72 ALS/FTD induced pluripotent stem cell derived neurons

Acta neuropathologica, 2024-06, Vol.147 (1), p.1-1, Article 1

2024

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Failure to Deliver and Translate-New Insights into RNA Dysregulation in ALS

Frontiers in cellular neuroscience, 2017-08, Vol.11, p.243-243

2017

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A Helping Hand: RNA-Binding Proteins Guide Gene-Binding Choices by Cohesin Complexes

PLoS genetics, 2016-11, Vol.12 (11), p.e1006419-e1006419

2016

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UPF1 reduces C9orf72 HRE-induced neurotoxicity in the absence of nonsense-mediated decay dysfunction

Cell reports (Cambridge), 2021-03, Vol.34 (13), p.108925-108925, Article 108925

2021

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Antibody Therapy Targeting RAN Proteins Rescues C9 ALS/FTD Phenotypes in C9orf72 Mouse Model

Neuron (Cambridge, Mass.), 2020-02, Vol.105 (4), p.645-662.e11

2020

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Nuclear lamina invaginations are not a pathological feature of C9orf72 ALS/FTD

Acta neuropathologica communications, 2021-03, Vol.9 (1), p.45-8, Article 45

2021

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Ribonuclease recruitment using a small molecule reduced c9ALS/FTD r(G 4 C 2 ) repeat expansion in vitro and in vivo ALS models

Science translational medicine, 2021-10, Vol.13 (617), p.eabd5991

2021

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Fragile X protein mitigates TDP-43 toxicity by remodeling RNA granules and restoring translation

Human molecular genetics, 2015-12, Vol.24 (24), p.6886-6898

2015

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Poly(ADP-ribose) promotes toxicity of C9ORF72 arginine-rich dipeptide repeat proteins

Science translational medicine, 2022-09, Vol.14 (662), p.eabq3215

2022

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PolyGR and polyPR knock-in mice reveal a conserved neuroprotective extracellular matrix signature in C9orf72 ALS/FTD neurons

Nature neuroscience, 2024-04, Vol.27 (4), p.643

2024

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Tau Protein Disrupts Nucleocytoplasmic Transport in Alzheimer’s Disease

Neuron (Cambridge, Mass.), 2018-09, Vol.99 (5), p.925-940.e7

2018

Volltextzugriff (PDF)

G 4 C 2 Repeat RNA Initiates a POM121-Mediated Reduction in Specific Nucleoporins in C9orf72 ALS/FTD

Neuron (Cambridge, Mass.), 2020-09, Vol.107 (6), p.1124

2020

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Nuclear and degradative functions of the ESCRT-III pathway: implications for neurodegenerative disease

Nuclear pore complexes - a doorway to neural injury in neurodegeneration

SUN1 facilitates CHMP7 nuclear influx and injury cascades in sporadic amyotrophic lateral sclerosis

Nuclear accumulation of CHMP7 initiates nuclear pore complex injury and subsequent TDP-43 dysfunction in sporadic and familial ALS

Nuclear pore complex and nucleocytoplasmic transport disruption in neurodegeneration

Stress Granule Assembly Disrupts Nucleocytoplasmic Transport

G4C2 Repeat RNA Initiates a POM121-Mediated Reduction in Specific Nucleoporins in C9orf72 ALS/FTD

CRISPR-Cas9 Screens Identify the RNA Helicase DDX3X as a Repressor of C9ORF72 (GGGGCC)n Repeat-Associated Non-AUG Translation

Endocytosis regulates TDP-43 toxicity and turnover

Futsch/MAP1B mRNA is a translational target of TDP-43 and is neuroprotective in a Drosophila model of amyotrophic lateral sclerosis

Drosophila models of amyotrophic lateral sclerosis with defects in RNA metabolism

The ESCRT-III protein VPS4, but not CHMP4B or CHMP2B, is pathologically increased in familial and sporadic ALS neuronal nuclei

Post-transcriptional Inhibition of Hsc70-4/HSPA8 Expression Leads to Synaptic Vesicle Cycling Defects in Multiple Models of ALS

G2C4 targeting antisense oligonucleotides potently mitigate TDP-43 dysfunction in human C9orf72 ALS/FTD induced pluripotent stem cell derived neurons

Failure to Deliver and Translate-New Insights into RNA Dysregulation in ALS

A Helping Hand: RNA-Binding Proteins Guide Gene-Binding Choices by Cohesin Complexes

UPF1 reduces C9orf72 HRE-induced neurotoxicity in the absence of nonsense-mediated decay dysfunction

Antibody Therapy Targeting RAN Proteins Rescues C9 ALS/FTD Phenotypes in C9orf72 Mouse Model

Nuclear lamina invaginations are not a pathological feature of C9orf72 ALS/FTD

Ribonuclease recruitment using a small molecule reduced c9ALS/FTD r(G 4 C 2 ) repeat expansion in vitro and in vivo ALS models

Fragile X protein mitigates TDP-43 toxicity by remodeling RNA granules and restoring translation

Poly(ADP-ribose) promotes toxicity of C9ORF72 arginine-rich dipeptide repeat proteins

PolyGR and polyPR knock-in mice reveal a conserved neuroprotective extracellular matrix signature in C9orf72 ALS/FTD neurons

Tau Protein Disrupts Nucleocytoplasmic Transport in Alzheimer’s Disease

G 4 C 2 Repeat RNA Initiates a POM121-Mediated Reduction in Specific Nucleoporins in C9orf72 ALS/FTD

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