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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >TDP-43 pathology in polyglutamine diseases: With reference to amyotrphic lateral sclerosis
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TDP-43 pathology in polyglutamine diseases: With reference to amyotrphic lateral sclerosis

机译：TDP-43在多谷氨酰胺疾病中的病理学：关于肌萎缩性侧索硬化症

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A nuclear protein, transactivation response (TAR) DNA binding protein 43kDa (TDP-43), is the major component of neuronal cytoplasmic inclusions (NCIs) in frontotemporal lobar degeneration with ubiquitin inclusions (FTLD-U) and sporadic amyotrophic lateral sclerosis (SALS). While initially thought to be relatively specific to FTLD-U and ALS, TDP-43 pathology has now been detected in a number of other neurodegenerative diseases, including Alzheimer's disease and Parkinson's disease. In such tauopathies and α-synucleinopathies, occurrence of TDP-43-positive neuronal cytoplasmic inclusions may be associated with other distinct molecular pathologic processes primarily involving their own pathological proteins, tau and α-synuclein, respectively (secondary TDP-43 proteinopathies). On the other hand, in several polyglutamine (polyQ) diseases, TDP-43 appears to play an important pathomechanistic role. Interestingly, intermediate-length polyQ expansions (27-33 Qs) in ataxin 2, the causative gene of spinocerebellar ataxia type 2, have recently been reported to be a genetic risk factor for SALS. Here, with a review of the literature, we discuss the relationship between ALS and polyQ diseases from the viewpoint of TDP-43 neuropathology.
机译：核蛋白，反转录激活应答（TAR）DNA结合蛋白43kDa（TDP-43），是额颞叶变性伴泛素包涵体（FTLD-U）和偶发性肌萎缩性侧索硬化症（SALS）的神经元胞浆内含物（NCI）的主要成分。尽管最初认为TDP-43病理学对FTLD-U和ALS相对特定，但现在已在许多其他神经退行性疾病中检测到TDP-43病理，包括阿尔茨海默氏病和帕金森氏病。在此类陶氏病和α-突触核蛋白病中，TDP-43阳性神经元胞浆内含物的出现可能与其他不同的分子病理学过程相关，主要涉及其自身的病理蛋白，tau和α-突触核蛋白（继发性TDP-43蛋白病）。另一方面，在几种聚谷氨酰胺（polyQ）疾病中，TDP-43似乎起着重要的发病机制作用。有趣的是，最近已经报道了紫杉醇2（脊髓小脑性共济失调2型的致病基因）中长度的polyQ扩增（27-33 Qs）是SALS的遗传危险因素。在这里，通过文献综述，我们从TDP-43神经病理学的角度讨论了ALS与polyQ疾病之间的关系。

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