在[具体模型名称未给出]中hTDP-43的神经元过表达模拟了TDP-43蛋白病中常见的细胞病理学特征。
Neuronal overexpression of hTDP-43 in mimics the cellular pathology commonly observed in TDP-43 proteinopathies.
作者信息
Koopman Mandy, Güngördü Lale, Seinstra Renée I, Hogewerf Wytse, Nollen Ellen A A
机构信息
European Research Institute for the Biology of Ageing, University of Groningen, University Medical Centre Groningen, The Netherlands.
出版信息
MicroPubl Biol. 2023 Apr 19;2023. doi: 10.17912/micropub.biology.000767. eCollection 2023.
Abstract
Inclusions consisting of transactive response DNA-binding protein 43 (TDP-43) are a characteristic feature of amyotrophic lateral sclerosis (ALS). has been instrumental in studying the underlying mechanisms of TDP-43 pathology. Here, we extend the possibilities of previous studies by examining a model expressing human wild-type ( ) pan-neuronally. We show that disease-related (hyper)phosphorylation and cytosolic localisation of hTDP-43 are present in hTDP-43 worms and that these features can be enhanced by adjusting the environmental temperature.
摘要
由反式激活反应DNA结合蛋白43（TDP-43）组成的包涵体是肌萎缩侧索硬化症（ALS）的一个特征性特征。在研究TDP-43病理学的潜在机制方面发挥了重要作用。在这里，我们通过检查一个在全神经元中表达人类野生型（ ）的模型，扩展了先前研究的可能性。我们表明，hTDP-43蠕虫中存在与疾病相关的（高）磷酸化和hTDP-43的胞质定位，并且这些特征可以通过调节环境温度来增强。
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