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TDP-43 dysfunction compromises UPF1-dependent mRNA metabolism in ALS

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sciencedirect.com:5037/science/article/pii/S0896627325008487
Dec 2025
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A tabletop blast device for the study of the long-term consequences of traumatic brain injury on brain organoids

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  DOI: 10.1016/j.crmeth.2025.101213
  
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pmc.ncbi.nlm.nih.gov/articles/PMC12664893/
Nov 2025
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A 16-amino acid peptide delays the progression of motor neuron degeneration and pathogenic symptoms in ALS models

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sciencedirect.com:5037/science/article/pii/S1878747925002843
54.241.53.176 54.241.53.176

Aberrant NSUN1 activity connects m5C-RNA modification to TDP-43 neurotoxicity in ALS/FTD

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  DOI: 10.26508/lsa.202503297
  
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54.241.53.176/29266c821148923660676f0aa34275a086b7a924/PMID_41188020-html.html
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Disparate leukemia mutations converge on nuclear phase-separated condensates

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  DOI: 10.1016/j.cell.2025.10.010
  
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sciencedirect.com/science/article/pii/S0092867425011493
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Phospho-proteome profiling in human neurons reveals targets of TBK1 in ALS/FTD-associated autophagy networks

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  DOI: 10.1016/j.celrep.2025.116494
  
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sciencedirect.com/science/article/pii/S2211124725012653
Oct 2025
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Single-nucleus RNA sequencing reveals GABAergic vulnerability and reactive gliosis driven by loss of TDP-43

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pmc.ncbi.nlm.nih.gov/articles/PMC12595131/
Sep 2025
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Maintenance of neuronal TDP-43 expression requires axonal lysosome transport

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  DOI: 10.7554/eLife.104057
  
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pmc.ncbi.nlm.nih.gov/articles/PMC12448747/
Jul 2025
52.53.155.43 52.53.155.43

watanabe-et-al-als-associated-rna-binding-proteins-promote-unc13a-transcription-through-rest-downregulation-html.html

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52.53.155.43/42423fb21c90eb6b2282f89153f3787d8f44c56d/watanabe-et-al-als-associated-rna-binding-proteins-promote-unc13a-transcription-through-rest-downregulation-html.html
www.nature.com www.nature.com

TRAF6 regulates ubiquitination-independent TDP-43 condensation and related neurodegeneration

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  DOI: 10.1038/s41380-025-03122-w
  
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nature.com/articles/s41380-025-03122-w
Jun 2025
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Critical impact of lysine 136 in TDP-43 phase separation, compartmentalization, and aggregation in living vertebrates

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  DOI: 10.1016/j.isci.2025.112761
  
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sciencedirect.com/science/article/pii/S2589004225010223
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The effect of G-quadruplexes on TDP43 condensation, distribution, and toxicity

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  DOI: 10.1016/j.str.2025.05.006
  
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cell.com/structure/fulltext/S0969-2126(25)00184-4
May 2025
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Neuroimmune signaling mediates astrocytic nucleocytoplasmic disruptions and stress granule formation associated with TDP-43 pathology

1
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  DOI: 10.1016/j.nbd.2025.106939
  
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sciencedirect.com/science/article/pii/S096999612500155X
52.53.155.43 52.53.155.43

1-s2.0-S2589004225009095-main-html.html

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  DOI: 10.1016/j.isci.2025.112648
  
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52.53.155.43/a6cce86e021e5bd6e2c85c9d52d91e003ece88d3/1-s2.0-S2589004225009095-main-html.html
Mar 2025
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TDP-43 seeding induces cytoplasmic aggregation heterogeneity and nuclear loss of function of TDP-43

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  DOI: 10.1016/j.neuron.2025.03.004
  
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sciencedirect.com/science/article/pii/S089662732500176X
Feb 2025
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TDP43 autoregulation gives rise to dominant negative isoforms that are tightly controlled by transcriptional and post-translational mechanisms

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  DOI: 10.1016/j.celrep.2024.115113
  
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pmc.ncbi.nlm.nih.gov/articles/PMC11848802/
Jan 2025
onlinelibrary.wiley.com onlinelibrary.wiley.com

Genetic Ablation of Sarm1 Mitigates Disease Acceleration after Traumatic Brain Injury in the SOD1G93A Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

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1. scibot 17 Jan 2025
  
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  DOI: 10.1002/ana.27174
  
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onlinelibrary.wiley.com/doi/10.1002/ana.27174
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Opposing roles of p38α-mediated phosphorylation and PRMT1-mediated arginine methylation in driving TDP-43 proteinopathy

1
1. scibot 16 Jan 2025
  
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  DOI: 10.1016/j.celrep.2024.115205
  
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sciencedirect.com/science/article/pii/S2211124724015560
Dec 2024
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Interrogation of RNA-bound proteome with XRNAX illuminates molecular alterations in the mouse brain affected with dysmyelination

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sciencedirect.com/science/article/pii/S2211124724014463
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DUX4-induced HSATII RNA accumulation drives protein aggregation impacting RNA processing pathways

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  DOI: 10.1101/2024.12.17.628988
  
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biorxiv.org/content/10.1101/2024.12.17.628988v1
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Design principles to tailor Hsp104 therapeutics

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sciencedirect.com/science/article/pii/S2211124724013561
Sep 2024
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ATAXIN-2 intermediate-length polyglutamine expansions elicit ALS-associated metabolic and immune phenotypes

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  DOI: 10.1038/s41467-024-51676-0
  
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ncbi.nlm.nih.gov/pmc/articles/PMC11362472/
Aug 2024
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Disruption of nuclear speckle integrity dysregulates RNA splicing in C9ORF72-FTD/ALS

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1. scibot 27 Aug 2024
  
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  DOI: 10.1016/j.neuron.2024.07.025
  
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sciencedirect.com/science/article/pii/S0896627324005701
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Untitled document

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  DOI: 10.1002/jnr.23999
  
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onlinelibrary.wiley.com/doi/10.1002/jnr.23999/full
Jul 2024
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Multivalent GU-rich oligonucleotides sequester TDP-43 in the nucleus by inducing high molecular weight RNP complexes

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  DOI: 10.1016/j.isci.2024.110109
  
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ncbi.nlm.nih.gov/pmc/articles/PMC11233918/
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Depletion of Mettl3 in cholinergic neurons causes adult-onset neuromuscular degeneration

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  DOI: 10.1016/j.celrep.2024.113999
  
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ncbi.nlm.nih.gov/pmc/articles/PMC11216409/
Jun 2024
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Identifying FUS amyotrophic lateral sclerosis disease signatures in patient dermal fibroblasts

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  DOI: 10.1016/j.devcel.2024.05.011
  
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sciencedirect.com/science/article/pii/S1534580724003289
May 2024
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LncRNA Hsrω nucleates TBPH and promotes intestinal stem cell differentiation upon heat shock

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  DOI: 10.1016/j.isci.2024.109732
  
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ncbi.nlm.nih.gov/pmc/articles/PMC11067334/
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TDP-43 pathology links innate and adaptive immunity in amyotrophic lateral sclerosis

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  DOI: 10.1101/2024.01.07.574541
  
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52.53.155.43/52328d22433e68f24f1d36a54def69a5a5ed912e/2024.01.07.574541v2.full-html.html
Apr 2024
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Time-resolved profiling of RNA binding proteins throughout the mRNA life cycle

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  DOI: 10.1016/j.molcel.2024.03.012
  
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sciencedirect.com/science/article/pii/S1097276524002223
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KCNJ2 inhibition mitigates mechanical injury in a human brain organoid model of traumatic brain injury

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  DOI: 10.1016/j.stem.2024.03.004
  
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sciencedirect.com/science/article/pii/S1934590924000857
Mar 2024
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The nuclear import receptor Kapβ2 modifies neurotoxicity mediated by poly(GR) in C9orf72-linked ALS/FTD

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  DOI: 10.1038/s42003-024-06071-2
  
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ncbi.nlm.nih.gov/pmc/articles/PMC10978903/
Feb 2024
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Homozygous ALS-linked mutations in TARDBP/TDP-43 lead to hypoactivity and synaptic abnormalities in human iPSC-derived motor neurons

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  DOI: 10.1016/j.isci.2024.109166
  
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  DOI: 10.1016/j.isci.2024.109166
  
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hnRNPA2B1 represses the disassembly of arsenite-induced stress granules and is essential for male fertility

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Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microglia

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Mouse oocytes sequester aggregated proteins in degradative super-organelles

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Targeting 14-3-3θ-mediated TDP-43 pathology in amyotrophic lateral sclerosis and frontotemporal dementia mice

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Astrocytic TDP-43 dysregulation impairs memory by modulating antiviral pathways and interferon-inducible chemokines

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TDP-43 chronic deficiency leads to dysregulation of transposable elements and gene expression by affecting R-loop and 5hmC crosstalk

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Aberrant axon initial segment plasticity and intrinsic excitability of ALS hiPSC motor neurons

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Targeted perturbation of signaling-driven condensates

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Dysregulation of the Progranulin-driven Autophagy-lysosomal Pathway Mediates Secretion of the Nuclear Protein TDP-43

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TTBK1 and CK1 inhibitors restore TDP-43 pathology and avoid disease propagation in lymphoblast from Alzheimer’s disease patients

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Tuning Hsp104 specificity to selectively detoxify α-synuclein

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Chaperone-mediated autophagy in neuronal dendrites utilizes activity-dependent lysosomal exocytosis for protein disposal

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Repeated mild traumatic brain injury triggers pathology in asymptomatic C9ORF72 transgenic mice

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Astrocytic response mediated by the CLU risk allele inhibits OPC proliferation and myelination in a human iPSC model

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The identification of high-performing antibodies for TDP-43 for use in Western Blot, immunoprecipitation and immunofluorescence

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Tandem detergent-extraction and immunoprecipitation of proteinopathy: Scalable enrichment of ALS-associated TDP-43 aggregates

1
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Hyperosmotic-stress-induced liquid-liquid phase separation of ALS-related proteins in the nucleus

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Astrocytic TDP-43 dysregulation impairs memory by modulating antiviral pathways and interferon-inducible chemokines

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ILF3 prion-like domain regulates gene expression and fear memory under chronic stress

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Loss of C9orf72 perturbs the Ran-GTPase gradient and nucleocytoplasmic transport, generating compositionally diverse Importin β-1 granules

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PIKFYVE inhibition mitigates disease in models of diverse forms of ALS

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SYF2 suppression mitigates neurodegeneration in models of diverse forms of ALS

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Genome-wide CRISPR screen reveals v-ATPase as a drug target to lower levels of ALS protein ataxin-2

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Impaired disassembly of the axon initial segment restricts mitochondrial entry into damaged axons

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Insulin-like growth factor 5 associates with human Aß plaques and promotes cognitive impairment

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Therapeutic modulation of GSTO activity rescues FUS-associated neurotoxicity via deglutathionylation in ALS disease models

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TDP-43 represses cryptic exon inclusion in the FTD–ALS gene UNC13A

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Homotypic fibrillization of TMEM106B across diverse neurodegenerative diseases

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Circ-Hdgfrp3 shuttles along neurites and is trapped in aggregates formed by ALS-associated mutant FUS

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NEAT1 is essential for metabolic changes that promote breast cancer growth and metastasis

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Decoding distinctive features of plasma extracellular vesicles in amyotrophic lateral sclerosis

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TDP-43 condensation properties specify its RNA-binding and regulatory repertoire

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Overlapping Role of SCYL1 and SCYL3 in Maintaining Motor Neuron Viability

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Untitled document

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Untitled document

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Rapid in vitro quantification of TDP-43 and FUS mislocalisation for screening of gene variants implicated in frontotemporal dementia and amyotrophic lateral sclerosis

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Saccadic Palsy following Cardiac Surgery: Possible Role of Perineuronal Nets

6
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Untitled document

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Phosphorylation of Trans-active response DNA binding protein-of 43 kDa promotes its cytoplasmic aggregation and modulates its function in tau mRNA stability and exon 10 alternative splicing

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DNA strand breaks and TDP-43 mislocation are absent in the murine hSOD1G93A model of amyotrophic lateral sclerosis in vivo and in vitro

2
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Neuronal VCP loss of function recapitulates FTLD-TDP pathology

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Mouse closed head traumatic brain injury replicates the histological tau pathology pattern of human disease: characterization of a novel model and systematic review of the literature

1
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Traumatic injury compromises nucleocytoplasmic transport and leads to TDP-43 pathology

1
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UBQLN2-HSP70 axis reduces poly-Gly-Ala aggregates and alleviates behavioral defects in the C9ORF72 animal model

1
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Pathogenic Huntingtin Repeat Expansions in Patients with Frontotemporal Dementia and Amyotrophic Lateral Sclerosis

1
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G4C2 Repeat RNA Initiates a POM121-Mediated Reduction in Specific Nucleoporins in C9orf72 ALS/FTD

1
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Stress Induces Dynamic, Cytotoxicity-Antagonizing TDP-43 Nuclear Bodies via Paraspeckle LncRNA NEAT1-Mediated Liquid-Liquid Phase Separation

1
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  DOI: 10.1016/j.molcel.2020.06.019
  
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TDP-43 maximizes nerve conduction velocity by repressing a cryptic exon for paranodal junction assembly in Schwann cells

1
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  DOI: 10.7554/eLife.64456
  
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ncbi.nlm.nih.gov/pmc/articles/PMC7946431/
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Therapeutic genetic variation revealed in diverse Hsp104 homologs

1
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  DOI: 10.7554/eLife.57457
  
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ncbi.nlm.nih.gov/pmc/articles/PMC7785292/
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Cytosolic calcium regulates cytoplasmic accumulation of TDP-43 through Calpain-A and Importin α3

1
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  DOI: 10.7554/eLife.60132
  
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Nuclear Import Receptors Directly Bind to Arginine-Rich Dipeptide Repeat Proteins and Suppress Their Pathological Interactions

1
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Amyotrophic lateral sclerosis associated mislocalisation of TDP‐43 to the cytoplasm causes cortical hyperexcitability and reduced excitatory neurotransmission in the motor cortex

1
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  rabbit polyclonal α‐TDP‐43
  
  DOI: 10.1111/jnc.15214
  
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Chimeric Peptide Species Contribute to Divergent Dipeptide Repeat Pathology in c9ALS/FTD and SCA36

1
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Extracellular Vesicles Isolated from Human Induced Pluripotent Stem Cell-Derived Neurons Contain a Transcriptional Network

1
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  DOI: 10.1007/s11064-020-03019-w
  
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Interactome Mapping Provides a Network of Neurodegenerative Disease Proteins and Uncovers Widespread Protein Aggregation in Affected Brains

1
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jcs.biologists.org jcs.biologists.org

TDP-43 a protein central to amyotrophic lateral sclerosis is destabilized by Tankyrase-1/2

3
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  RRID: AB_615042
  
  DOI: 10.1242/jcs.245811
  
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  RRID: AB_615042
  
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An ALS-linked mutation in TDP-43 disrupts normal protein interactions in the motor neuron response to oxidative stress

1
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  rabbit anti-TDP-43
  
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sciencedirect.com/science/article/pii/S0969996120303259
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Postmortem Cortex Samples Identify Distinct Molecular Subtypes of ALS: Retrotransposon Activation, Oxidative Stress, and Activated Glia

1
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Untitled document

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  DOI: 10.1016/j.celrep.2019.04.003
  
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Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis

1
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  DOI: 10.7554/eLife.40811
  
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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

1
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  RRID:AB_615042
  
  DOI: 10.1016/j.neuron.2019.02.038
  
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Cross-Regulation between TDP-43 and Paraspeckles Promotes Pluripotency-Differentiation Transition

1
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  RRID:AB_615042
  
  DOI: 10.1016/j.molcel.2019.03.041
  
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Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS

1
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  RRID:AB_615042
  
  DOI: 10.1016/j.celrep.2018.12.045
  
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Untitled document

1
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  RRID:AB_615042
  
  DOI: 10.1007/s00401-018-1942-8
  
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link.springer.com/article/10.1007/s00401-018-1942-8
Nov 2018
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TBK1 Suppresses RIPK1-Driven Apoptosis and Inflammation during Development and in Aging

1
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sciencedirect.com/science/article/pii/S0092867418309693
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Poly(ADP-Ribose) Prevents Pathological Phase Separation of TDP-43 by Promoting Liquid Demixing and Stress Granule Localization

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1. scibot 05 Nov 2018
  
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  RRID:AB_615042
  
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sciencedirect.com/science/article/pii/S1097276518305495
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Zc3h13 Regulates Nuclear RNA m6A Methylation and Mouse Embryonic Stem Cell Self-Renewal

1
1. scibot 05 Nov 2018
  
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  RRID:AB_615042
  
  Curator: @Jmenke
  
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sciencedirect.com/science/article/pii/S1097276518301345
www.jneurosci.org www.jneurosci.org

Untitled document

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1. scibot 05 Nov 2018
  
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jneurosci.org/content/38/10/2615
onlinelibrary.wiley.com onlinelibrary.wiley.com

Essential role of hippocampal noradrenaline in the regulation of spatial working memory and TDP‐43 tissue pathology

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1. scibot 05 Nov 2018
  
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  RRID:AB_615042
  
  Curator: @evieth
  
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onlinelibrary.wiley.com/doi/10.1002/cne.24397/full
Nov 2017
www.ncbi.nlm.nih.gov www.ncbi.nlm.nih.gov

TDP-43 and ubiquitinated cytoplasmic aggregates in sporadic ALS are low frequency and widely distributed in the lower motor neuron columns independent of disease spread

1
1. neurohugo 12 Nov 2017
  
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  Proteintech Group Inc. no. 10782-2-AP, rabbit, IgG, polyclonal;
  
  Proteintech Group Cat# 10782-2-AP Lot# RRID:AB_615042
  
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ncbi.nlm.nih.gov/pmc/articles/PMC4981144/
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TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics

1
1. scibot 08 Nov 2017
  
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  RRID:AB_615042
  
  Curator: @Jmenke
  
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sciencedirect.com/science/article/pii/S0896627317306475
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TDP-43 Depletion in Microglia Promotes Amyloid Clearance but Also Induces Synapse Loss

1
1. scibot 08 Nov 2017
  
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  AB_615042
  
  Curator: @chewbeccax
  
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sciencedirect.com/science/article/pii/S0896627317304750
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Regulatory Role of RNA Chaperone TDP-43 for RNA Misfolding and Repeat-Associated Translation in SCA31

1
1. scibot 08 Nov 2017
  
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  AB_615042
  
  Curator: @Zeljana_Babic
  
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sciencedirect.com/science/article/pii/S089662731730154X
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A Multi-network Approach Identifies Protein-Specific Co-expression in Asymptomatic and Symptomatic Alzheimer’s Disease

1
1. scibot 08 Nov 2017
  
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  TDP-43 antibody
  
  Curator: @kaitlinsung
  
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sciencedirect.com/science/article/pii/S2405471216303702
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C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles

1
1. scibot 08 Nov 2017
  
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  AB_615042
  
  Curator: @gabimpine
  
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sciencedirect.com/science/article/pii/S0092867416313836
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Saccadic Palsy following Cardiac Surgery: Possible Role of Perineuronal Nets

2
1. scibot 08 Nov 2017
  
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  AB_615042
  
  Curator: @jcabotaj
  
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ncbi.nlm.nih.gov/pmc/articles/PMC4489868/

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