Showing 1 - 20 results of 33 for search '"TDP-43"', query time: 0.07s Refine Results
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    Synaptic Paths to Neurodegeneration: The Emerging Role of TDP-43 and FUS in Synaptic Functions by Shuo-Chien Ling

    Published 2018-01-01
    “…TAR DNA-binding protein-43 KDa (TDP-43) and fused in sarcoma (FUS) as the defining pathological hallmarks for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), coupled with ALS-FTD-causing mutations in both genes, indicate that their dysfunctions damage the motor system and cognition. …”
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    TDP-43 nuclear retention is antagonized by hypo-phosphorylation of its C-terminus in the cytoplasm by Célia Rabhi, Nicolas Babault, Céline Martin, Bénédicte Desforges, Alexandre Maucuer, Vandana Joshi, Serhii Pankivskyi, Yitian Feng, Guillaume Bollot, Revital Rattenbach, David Pastré, Ahmed Bouhss

    Published 2025-01-01
    “…Abstract Protein aggregation is a hallmark of many neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS), in which TDP-43, a nuclear RNA-binding protein, forms cytoplasmic inclusions. …”
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    Glymphatic dysfunction and neurodegeneration in ALS: Longitudinal insights from rNLS8 TDP-43 mice by Akram Zamani, Adam K. Walker, David K. Wright

    Published 2025-03-01
    “…Dysfunctional Tar DNA binding protein-43 (TDP-43) is found in approximately 95 % of all people with amyotrophic lateral sclerosis (ALS). …”
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    Deciphering the interactome of Ataxin-2 and TDP-43 in iPSC-derived neurons for potential ALS targets. by Yuan Tian, Nicolette Heinsinger, Yinghui Hu, U-Ming Lim, Yi Wang, Aaron Zefrin Fernandis, Sophie Parmentier-Batteur, Becky Klein, Jason M Uslaner, Sean M Smith

    Published 2024-01-01
    “…Down-regulation of Ataxin-2 has been shown to mitigate TDP-43 proteinopathy in ALS models. To identify alternative therapeutic targets that can mitigate TDP-43 toxicity, we examined the interaction between Ataxin-2 and TDP-43. …”
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    Influence of Inflammatory Cytokines IL-1β and IFNγ on Sarcoplasmic Aggregation of p62 and TDP-43 in Myotubes by Bryony McCord, Richard M. Day

    Published 2023-01-01
    “…Non-inflammatory features are also observed, like the sarcoplasmic accumulation of proteins including TDP-43 and p62. This study aimed to investigate the effect of IFNγ and interleukin 1-β (IL-1β) on TDP-43 and p62 aggregation in vitro. …”
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    肌萎缩侧索硬化症基因TDP43致病突变对斑马鱼毒性的影响

    Published 2014-01-01
    “…摘 要: 【目的】 探讨肌萎缩侧索硬化症基因TDP43致病突变对斑马鱼毒性的影响。【方法】 以野生型AB系斑马鱼作为研究对象,构建携带红色荧光蛋白(RFP)的TDP43基因野生型(WT)和突变型(S292N)质粒,通过显微注射法转染斑马鱼单细胞期胚胎,建立转基因斑马鱼模型。…”
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    Decreased Hsp90 activity protects against TDP-43 neurotoxicity in a C. elegans model of amyotrophic lateral sclerosis. by Laura Garcia-Toscano, Heather N Currey, Joshua C Hincks, Jade G Stair, Nicolas J Lehrbach, Nicole F Liachko

    Published 2024-12-01
    “…Mutations in TARDBP, the gene coding for TDP-43, can cause some cases of familial inherited ALS (fALS), indicating dysfunction of TDP-43 drives disease. …”
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    Metabolic rate and insulin-independent glucose uptake increase in a TDP-43Q331K mouse model of amyotrophic lateral sclerosis by Tanya S. McDonald, Cedric S. Cui, Titaya Lerskiatiphanich, Jianina Marallag, John D. Lee

    Published 2025-02-01
    “…Here, we investigated energy balance and glucose control in TAR DNA-binding protein 43 (TDP-43)Q331K mice, a model of ALS, at both the early and late symptomatic stages of disease. …”
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    C9ORF72 poly-PR induces TDP-43 nuclear condensation via NEAT1 and is modulated by HSP70 activity by Diksha Agnihotri, Chi-Chang Lee, Po-Chao Lu, Ruei-Yu He, Yung-An Huang, Hung-Chih Kuo, Joseph Jen-Tse Huang

    Published 2025-01-01
    “…Under prolonged poly-PR stress, HSP70 delocalizes from TDP-43 NCs, leading to the oligomerization of TDP-43 within these condensates. …”
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