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Nucleocytoplasmic Proteomic Analysis Uncovers eRF1 and Nonsense-Mediated Decay as Modifiers of ALS/FTD C9orf72 Toxicity

https://doi.org/10.1016/j.neuron.2020.01.020

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Nonsense-mediated mRNA decay in neuronal physiology and neurodegeneration

inferred← citeshttps://doi.org/10.1016/j.ygeno.2018.07.008openalex-snapshot

Characterization of proteins in different subcellular localizations for Escherichia coli K12

inferred← citeshttps://doi.org/10.1038/s41467-020-17129-0openalex-snapshot

C9orf72 arginine-rich dipeptide repeats inhibit UPF1-mediated RNA decay via translational repression

inferred← citeshttps://doi.org/10.1038/s41467-021-22627-wopenalex-snapshot

Loss of function mutations in GEMIN5 cause a neurodevelopmental disorder

inferred← citeshttps://doi.org/10.1038/s41467-021-26303-xopenalex-snapshot

A C. elegans model of C9orf72-associated ALS/FTD uncovers a conserved role for eIF2D in RAN translation

inferred← citeshttps://doi.org/10.1038/s41467-022-31098-6openalex-snapshot

NUP62 localizes to ALS/FTLD pathological assemblies and contributes to TDP-43 insolubility

inferred← citeshttps://doi.org/10.1038/s41467-026-69025-8openalex-snapshot

Supercharging-enhanced nDIA-MS enables global profiling of drug-induced proteome solubility shifts

inferred← citeshttps://doi.org/10.1038/s41531-025-00899-zopenalex-snapshot

Longitudinal multi-omics in alpha-synuclein Drosophila model discriminates disease- from age-associated pathologies in Parkinson’s disease

inferred← citeshttps://doi.org/10.1038/s41586-025-08950-yopenalex-snapshot

The expanding repertoire of ESCRT functions in cell biology and disease

inferred← citeshttps://doi.org/10.1038/s41593-021-00859-9openalex-snapshot

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

inferred← citeshttps://doi.org/10.1038/s41593-025-02096-wopenalex-snapshot

TDP-43-dependent mis-splicing of KCNQ2 triggers intrinsic neuronal hyperexcitability in ALS/FTD

inferred← citeshttps://doi.org/10.1038/s42003-025-07502-4openalex-snapshot

Reduced UPF1 levels in senescence impair nonsense-mediated mRNA decay

inferred← citeshttps://doi.org/10.1038/s44318-024-00352-6openalex-snapshot

RNA dysregulation in neurodegenerative diseases

inferred← citeshttps://doi.org/10.1042/etls20190167openalex-snapshot

Arginine-rich dipeptide-repeat proteins as phase disruptors in C9-ALS/FTD

inferred← citeshttps://doi.org/10.1080/14789450.2021.2004890openalex-snapshot

Advancing mechanistic understanding and biomarker development in amyotrophic lateral sclerosis

inferred← citeshttps://doi.org/10.1080/15384101.2021.1886661openalex-snapshot

Multiple ways to a dead end: diverse mechanisms by which ALS mutant genes induce cell death

inferred← citeshttps://doi.org/10.1093/hmg/ddab089openalex-snapshot

C9orf72 -associated arginine-rich dipeptide repeats induce RNA-dependent nuclear accumulation of Staufen in neurons

inferred← citeshttps://doi.org/10.1093/jmcb/mjab022openalex-snapshot

Regulation of nonsense-mediated mRNA decay in neural development and disease

inferred← citeshttps://doi.org/10.1093/nar/gkab834openalex-snapshot

ZNF598 co-translationally titrates poly(GR) protein implicated in the pathogenesis of C9ORF72 -associated ALS/FTD

inferred← citeshttps://doi.org/10.1101/2020.02.24.954453openalex-snapshot

Identification of a Nonsense-Mediated Decay pathway at the Endoplasmic Reticulum

inferred← citeshttps://doi.org/10.1101/2021.06.11.448082openalex-snapshot

Proximity-based labeling reveals DNA damage-induced N-terminal phosphorylation of fused in sarcoma (FUS) leads to distinct changes in the FUS protein interactome

inferred← citeshttps://doi.org/10.1101/2021.08.04.455154openalex-snapshot

Opposing roles of p38α-mediated phosphorylation and arginine methylation in driving TDP-43 proteinopathy

inferred← citeshttps://doi.org/10.1101/2024.01.31.578310openalex-snapshot

Counter-regulation of RNA stability by UPF1 and TDP43

inferred← citeshttps://doi.org/10.1101/2024.01.31.578311openalex-snapshot

ALS-Associated TDP-43 Dysfunction Compromises UPF1-Dependent mRNA Metabolism Pathways Including Alternative Polyadenylation and 3’UTR Length

inferred← citeshttps://doi.org/10.1101/2024.03.21.586073openalex-snapshot

Neurotrauma-induced vulnerability of C9orf72 ALS iPSC-motor neurons revealed by biofidelic stretch injury

inferred← citeshttps://doi.org/10.1101/2024.05.17.594716openalex-snapshot

The role of ADAR editing and nonsense-mediated decay in Parkinson’s Disease

inferred← citeshttps://doi.org/10.1101/2024.12.01.626216openalex-snapshot

The ALS-associated co-chaperone DNAJC7 mediates neuroprotection against proteotoxic stress by modulating HSF1 activity

inferred← citeshttps://doi.org/10.1101/2025.04.07.646890openalex-snapshot

Context-dependent Interactors Regulate TDP-43 Dysfunction in ALS/FTLD

inferred← citeshttps://doi.org/10.1101/gad.338061.120openalex-snapshot

Identification of a localized nonsense-mediated decay pathway at the endoplasmic reticulum

inferred← citeshttps://doi.org/10.1111/acel.14245openalex-snapshot

Pathogenic tau induces an adaptive elevation in mRNA translation rate at early stages of disease

inferred← citeshttps://doi.org/10.1111/bph.15224openalex-snapshot

Sigma 1 receptor as a therapeutic target for amyotrophic lateral sclerosis

inferred← citeshttps://doi.org/10.1111/jnc.15529openalex-snapshot

Proteostasis deregulation as a driver of C9ORF72 pathogenesis

inferred← citeshttps://doi.org/10.1111/jnc.15847openalex-snapshot

Aspects of degradation and translation of the expanded C9orf72 hexanucleotide repeat RNA

inferred← citeshttps://doi.org/10.1117/1.nph.9.s1.013001openalex-snapshot

Neurophotonic tools for microscopic measurements and manipulation: status report

inferred← citeshttps://doi.org/10.1126/sciadv.adf7997openalex-snapshot

CLIP-Seq analysis enables the design of protective ribosomal RNA bait oligonucleotides against C9ORF72 ALS/FTD poly-GR pathophysiology

inferred← citeshttps://doi.org/10.1126/sciadv.adi5548openalex-snapshot

Loss of function of the ALS-associated NEK1 kinase disrupts microtubule homeostasis and nuclear import

inferred← citeshttps://doi.org/10.1126/science.adv3301openalex-snapshot

Short RNA chaperones promote aggregation-resistant TDP-43 conformers to mitigate neurodegeneration

inferred← citeshttps://doi.org/10.1186/s40035-025-00477-6openalex-snapshot

Gene therapy breakthroughs in ALS: a beacon of hope for 20% of ALS patients

inferred← citeshttps://doi.org/10.1186/s40478-020-01060-yopenalex-snapshot

RNA dependent suppression of C9orf72 ALS/FTD associated neurodegeneration by Matrin-3

inferred← citeshttps://doi.org/10.1186/s40478-021-01150-5openalex-snapshot

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

inferred← citeshttps://doi.org/10.12688/f1000research.168181.1openalex-snapshot

Variations in ADAR editing of nonsense-mediated decay targets in PD males and females

inferred← citeshttps://doi.org/10.1371/journal.pbio.3001002openalex-snapshot

LSM12-EPAC1 defines a neuroprotective pathway that sustains the nucleocytoplasmic RAN gradient

inferred← citeshttps://doi.org/10.15252/embj.2023114378openalex-snapshot

Translation‐coupled mRNA quality control mechanisms

inferred← citeshttps://doi.org/10.26508/lsa.202402757openalex-snapshot

RuvBL1/2 reduce toxic dipeptide repeat protein burden in multiple models of C9orf72-ALS/FTD

inferred← citeshttps://doi.org/10.3389/fgene.2022.886563openalex-snapshot

RNA Helicases in Microsatellite Repeat Expansion Disorders and Neurodegeneration

inferred← citeshttps://doi.org/10.3389/fncel.2021.708181openalex-snapshot

RNA Is a Double-Edged Sword in ALS Pathogenesis

inferred← citeshttps://doi.org/10.3389/fnins.2020.00914openalex-snapshot

Tissue Clearing and Expansion Methods for Imaging Brain Pathology in Neurodegeneration: From Circuits to Synapses and Beyond

inferred← citeshttps://doi.org/10.3389/fnmol.2024.1328578openalex-snapshot

Invertebrate genetic models of amyotrophic lateral sclerosis

inferred← citeshttps://doi.org/10.3389/fnmol.2024.1456052openalex-snapshot

Histone post-translational modification and heterochromatin alterations in neurodegeneration: revealing novel disease pathways and potential therapeutics

inferred← citeshttps://doi.org/10.3389/fnmol.2024.1478110openalex-snapshot

Increased nuclear import characterizes aberrant nucleocytoplasmic transport in neurons from patients with spinocerebellar ataxia type 7

inferred← citeshttps://doi.org/10.3390/ijms27156986openalex-snapshot

RAN Translation-Coupled Decay of the C9orf72 GGGGCC Repeat Transcript by the RNA Exosome Suppresses Dipeptide Repeat Production

inferred← citeshttps://doi.org/10.6084/m9.figshare.14252098openalex-snapshot

Additional file 5 of Nuclear lamina invaginations are not a pathological feature of C9orf72 ALS/FTD

inferred← citeshttps://doi.org/10.70675/9a7778aaz88c7z40b1z92d3zaf5699b86a09openalex-snapshot

Comprendre et modéliser la Sclérose Latérale Amyotrophique à début précoce

inferred← citeshttps://doi.org/10.7554/elife.105342openalex-snapshot

The ALS-associated co-chaperone DNAJC7 mediates neuroprotection against proteotoxic stress by modulating HSF1 activity

inferred← citeshttps://doi.org/10.7554/elife.105342.1openalex-snapshot

The ALS-associated co-chaperone DNAJC7 mediates neuroprotection against proteotoxic stress by modulating HSF1 activity

inferred← citeshttps://doi.org/10.7554/elife.59419openalex-snapshot

TFEB/Mitf links impaired nuclear import to autophagolysosomal dysfunction in C9-ALS

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