探寻发病机制：肌萎缩侧索硬化症病理中的TAR DNA结合蛋白43聚集、轴突运输及局部合成 - Suppr

Navigating the pathways: TAR-DNA-binding-protein-43 aggregation, axonal transport, and local synthesis in amyotrophic lateral sclerosis pathology.

作者信息

Bar Avi Ori, Perlson Eran

机构信息

Department of Physiology and Pharmacology, Faculty of Medical & Health Science, Tel-Aviv University, Tel-Aviv, Israel (Bar Avi O, Perlson E).

Sagol School of Neuroscience, Tel-Aviv University, Tel-Aviv, Israel (Perlson E).

出版信息

Neural Regen Res. 2025 Oct 1;20(10):2921-2922. doi: 10.4103/NRR.NRR-D-24-00726. Epub 2024 Oct 22.

DOI:10.4103/NRR.NRR-D-24-00726

PMID:39610104

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11826470/

Abstract

摘要

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5278/11826470/7084787d482e/NRR-20-2921-g001.jpg

相似文献

Navigating the pathways: TAR-DNA-binding-protein-43 aggregation, axonal transport, and local synthesis in amyotrophic lateral sclerosis pathology.探寻发病机制：肌萎缩侧索硬化症病理中的TAR DNA结合蛋白43聚集、轴突运输及局部合成

Neural Regen Res. 2025 Oct 1;20(10):2921-2922. doi: 10.4103/NRR.NRR-D-24-00726. Epub 2024 Oct 22.

The pathogenic mechanism of TAR DNA-binding protein 43 (TDP-43) in amyotrophic lateral sclerosis.肌萎缩侧索硬化症中TAR DNA结合蛋白43（TDP - 43）的致病机制

Neural Regen Res. 2024 Apr;19(4):800-806. doi: 10.4103/1673-5374.382233.

Optineurin defects cause TDP43-pathology with autophagic vacuolar formation.Optineurin 缺陷导致 TDP43 病理学伴自噬空泡形成。

Neurobiol Dis. 2021 Jan;148:105215. doi: 10.1016/j.nbd.2020.105215. Epub 2020 Dec 6.

Oligodendrocyte pathology exceeds axonal pathology in white matter in human amyotrophic lateral sclerosis.人肌萎缩性侧索硬化症的白质中少突胶质细胞病理超过轴突病理。

J Pathol. 2020 Jul;251(3):262-271. doi: 10.1002/path.5455. Epub 2020 Jun 8.

RNA dysfunction and aggrephagy at the centre of an amyotrophic lateral sclerosis/frontotemporal dementia disease continuum.RNA 功能障碍和聚集物自噬在肌萎缩侧索硬化症/额颞叶痴呆疾病连续体的中心。

Brain. 2013 May;136(Pt 5):1345-60. doi: 10.1093/brain/awt030. Epub 2013 Mar 9.

TAR DNA-binding protein of 43 kDa (TDP-43) and amyotrophic lateral sclerosis (ALS): a promising therapeutic target.TAR DNA 结合蛋白 43kDa（TDP-43）与肌萎缩侧索硬化症（ALS）：一个有前景的治疗靶点。

Expert Opin Ther Targets. 2022 Jun;26(6):575-592. doi: 10.1080/14728222.2022.2083958. Epub 2022 Jun 2.

Nuclear transport dysfunction: a common theme in amyotrophic lateral sclerosis and frontotemporal dementia.核转运功能障碍：肌萎缩侧索硬化症和额颞叶痴呆的共同特征

J Neurochem. 2016 Aug;138 Suppl 1:134-44. doi: 10.1111/jnc.13642. Epub 2016 Jun 15.

A candidate protective factor in amyotrophic lateral sclerosis: heterogenous nuclear ribonucleoprotein G.肌萎缩侧索硬化症中的一个潜在保护因素：不均一核核糖核蛋白G

Neural Regen Res. 2023 Jul;18(7):1527-1534. doi: 10.4103/1673-5374.357916.

MTHFSD and DDX58 are novel RNA-binding proteins abnormally regulated in amyotrophic lateral sclerosis.MTHFSD 和 DDX58 是肌萎缩侧索硬化症中异常调节的新型 RNA 结合蛋白。

Brain. 2016 Jan;139(Pt 1):86-100. doi: 10.1093/brain/awv308. Epub 2015 Nov 2.

The role of heat shock proteins in Amyotrophic Lateral Sclerosis: The therapeutic potential of Arimoclomol.热休克蛋白在肌萎缩侧索硬化症中的作用：阿立莫唑莫的治疗潜力。

Pharmacol Ther. 2014 Jan;141(1):40-54. doi: 10.1016/j.pharmthera.2013.08.003. Epub 2013 Aug 23.

本文引用的文献

Looking for answers far away from the soma-the (un)known axonal functions of TDP-43, and their contribution to early NMJ disruption in ALS.寻找远离神经元胞体的答案——TDP-43 的（未知）轴突功能，以及它们对 ALS 早期 NMJ 破坏的贡献。

Mol Neurodegener. 2023 May 31;18(1):35. doi: 10.1186/s13024-023-00623-6.

Lost in local translation: TDP-43 and FUS in axonal/neuromuscular junction maintenance and dysregulation in amyotrophic lateral sclerosis.在局部翻译中迷失：TDP-43 和 FUS 在肌萎缩侧索硬化症中的轴突/神经肌肉接头维持和失调中的作用。

Neuron. 2023 May 3;111(9):1355-1380. doi: 10.1016/j.neuron.2023.02.028. Epub 2023 Mar 23.

Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins.轴突 TDP-43 凝聚物通过抑制核编码线粒体蛋白的局部合成导致神经肌肉接头破坏。

Nat Commun. 2021 Nov 25;12(1):6914. doi: 10.1038/s41467-021-27221-8.

The role of TDP-43 mislocalization in amyotrophic lateral sclerosis.TDP-43 蛋白定位错误在肌萎缩侧索硬化症中的作用。

Mol Neurodegener. 2020 Aug 15;15(1):45. doi: 10.1186/s13024-020-00397-1.

Axonal transport: Driving synaptic function.轴突运输：驱动突触功能。

Science. 2019 Oct 11;366(6462). doi: 10.1126/science.aaw9997.

Axonal transport defects and neurodegeneration: Molecular mechanisms and therapeutic implications.轴突运输缺陷与神经退行性变：分子机制与治疗意义。

Semin Cell Dev Biol. 2020 Mar;99:133-150. doi: 10.1016/j.semcdb.2019.07.010. Epub 2019 Sep 18.

Axonal Transport and Mitochondrial Function in Neurons.神经元中的轴突运输与线粒体功能

Front Cell Neurosci. 2019 Aug 9;13:373. doi: 10.3389/fncel.2019.00373. eCollection 2019.

A Case for Microtubule Vulnerability in Amyotrophic Lateral Sclerosis: Altered Dynamics During Disease.肌萎缩侧索硬化症中微管易损性的一个案例：疾病过程中动力学的改变

Front Cell Neurosci. 2016 Sep 13;10:204. doi: 10.3389/fncel.2016.00204. eCollection 2016.

Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations.TDP-43 mRNA 颗粒的轴突运输被 ALS 致病突变所损害。

Neuron. 2014 Feb 5;81(3):536-543. doi: 10.1016/j.neuron.2013.12.018.

Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration.增益或损耗：TDP43 介导的神经退行性变的分子机制。

Nat Rev Neurosci. 2011 Nov 30;13(1):38-50. doi: 10.1038/nrn3121.

文献AI研究员

20分钟写一篇综述，助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型，支持多种主流文档格式。

立即体验

Navigating the pathways: TAR-DNA-binding-protein-43 aggregation, axonal transport, and local synthesis in amyotrophic lateral sclerosis pathology.

作者信息

机构信息

出版信息

相似文献

本文引用的文献

文献AI研究员

用中文搜PubMed

文档翻译

Suppr 超能文献

相似文献

本文引用的文献