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一种以显著星形胶质细胞TDP - 43病理为特征的肌萎缩侧索硬化症新神经病理亚型。

A Novel Neuropathological Subtype of Amyotrophic Lateral Sclerosis Characterised by Prominent Astroglial TDP-43 Pathology.

作者信息

Matsubara Tomoyasu, Izumi Yuishin, Hatanaka Yuki, Takahara Mika, Kondo Amuro, Matsukura Kiyoshi, Arakawa Akira, Haga Toshihiro, Miyamoto Ryosuke, Naruse Hiroya, Morino Hiroyuki, Toda Tatsushi, Murayama Shigeo, Saito Yuko

机构信息

Department of Neuropathology (The Brain Bank for Aging Research), Tokyo Metropolitan Institute for Geriatrics and Gerontology, Tokyo, Japan.

Department of Neurology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan.

出版信息

Neuropathol Appl Neurobiol. 2025 Oct;51(5):e70036. doi: 10.1111/nan.70036.

DOI:10.1111/nan.70036
PMID:40928424
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12422077/
Abstract

This study identified a novel amyotrophic lateral sclerosis subtype with prominent astroglial phosphorylated TDP‐43 inclusions and minimal neuronal inclusions. The patients shared a clinical phenotype of flail arm variant of ALS. These observations suggest a more critical role for astroglia than previously recognised.

摘要

本研究确定了一种新型肌萎缩侧索硬化症亚型，其特征为星形胶质细胞中存在显著的磷酸化TDP-43包涵体，而神经元包涵体极少。这些患者具有肌萎缩侧索硬化症连枷臂变异型的临床表型。这些观察结果表明，星形胶质细胞的作用比以往认识到的更为关键。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a9d/12422077/47b7bfe61f49/NAN-51-e70036-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a9d/12422077/1cf9cb38854f/NAN-51-e70036-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a9d/12422077/47b7bfe61f49/NAN-51-e70036-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a9d/12422077/1cf9cb38854f/NAN-51-e70036-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a9d/12422077/47b7bfe61f49/NAN-51-e70036-g003.jpg

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本文引用的文献

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Slow disease progression and characteristic TDP-43 inclusions in a patient with familial amyotrophic lateral sclerosis carrying a TARDBP G357S variant.携带TARDBP G357S变异的家族性肌萎缩侧索硬化症患者疾病进展缓慢且具有特征性TDP-43包涵体。
Neuropathol Appl Neurobiol. 2024 Feb;50(1):e12966. doi: 10.1111/nan.12966.
2
Regional spreading pattern is associated with clinical phenotype in amyotrophic lateral sclerosis.区域性扩散模式与肌萎缩侧索硬化症的临床表型相关。
Brain. 2023 Oct 3;146(10):4105-4116. doi: 10.1093/brain/awad129.
3
Interactions of glial cells with neuronal synapses, from astrocytes to microglia and oligodendrocyte lineage cells.
从星形胶质细胞到小胶质细胞和少突胶质细胞谱系细胞，胶质细胞与神经元突触的相互作用。
Glia. 2023 Jun;71(6):1383-1401. doi: 10.1002/glia.24343. Epub 2023 Feb 17.
4
Phagocytic astrocytes: Emerging from the shadows of microglia.吞噬性星形胶质细胞：从小胶质细胞的阴影中浮现。
Glia. 2022 Jun;70(6):1009-1026. doi: 10.1002/glia.24145. Epub 2022 Feb 10.
5
Spreading of TDP-43 pathology via pyramidal tract induces ALS-like phenotypes in TDP-43 transgenic mice.TDP-43 病理学通过锥体束传播可诱导 TDP-43 转基因小鼠出现类似 ALS 的表型。
Acta Neuropathol Commun. 2021 Jan 18;9(1):15. doi: 10.1186/s40478-020-01112-3.
6
Clinical features and outcomes of the flail arm and flail leg and pure lower motor neuron MND variants: a multicentre Italian study.连枷臂和连枷腿及纯下运动神经元型运动神经元病变体的临床特征与预后：一项意大利多中心研究
J Neurol Neurosurg Psychiatry. 2020 Sep;91(9):1001-1003. doi: 10.1136/jnnp-2020-323542. Epub 2020 Jul 10.
7
p.N345K mutation in TARDBP in a patient with familial amyotrophic lateral sclerosis: An autopsy case.TARDBP 中的 p.N345K 突变与家族性肌萎缩侧索硬化症：一例尸检病例。
Neuropathology. 2019 Aug;39(4):286-293. doi: 10.1111/neup.12559. Epub 2019 May 24.
8
An autopsy case of early-stage amyotrophic lateral sclerosis with TDP-43 immunoreactive neuronal, but not glial, inclusions.伴有 TDP-43 免疫反应性神经元、而非神经胶质内包涵体的早期肌萎缩性侧索硬化症的尸检病例。
Neuropathology. 2019 Jun;39(3):224-230. doi: 10.1111/neup.12554. Epub 2019 Apr 24.
9
Heterogeneity of cerebral TDP-43 pathology in sporadic amyotrophic lateral sclerosis: Evidence for clinico-pathologic subtypes.散发性肌萎缩侧索硬化症中 TDP-43 脑病理学的异质性：临床病理亚型的证据。
Acta Neuropathol Commun. 2016 Jun 23;4(1):61. doi: 10.1186/s40478-016-0335-2.
10
Lysosomal and phagocytic activity is increased in astrocytes during disease progression in the SOD1 (G93A) mouse model of amyotrophic lateral sclerosis.在肌萎缩侧索硬化症的SOD1（G93A）小鼠模型疾病进展过程中，星形胶质细胞中的溶酶体和吞噬活性增加。
Front Cell Neurosci. 2015 Oct 15;9:410. doi: 10.3389/fncel.2015.00410. eCollection 2015.