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首页> 外文期刊>Journal of neurology >Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases.
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Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases.

机译：肌萎缩性侧索硬化症，额颞叶痴呆及其他：TDP-43疾病。

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摘要

Ever since the significance of pathological 43-kDa transactivating responsive sequence DNA-binding protein (TDP-43) for human disease has been recognized in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin positive inclusions (FTLD-U), a number of publications have emerged reporting on this pathology in a variety of neurodegenerative diseases. Given the heterogeneous and, in part, conflicting nature of the recent findings, we here review pathological TDP-43 and its relationship to human disease with a special focus on ALS and FTLD-U. To this end, we propose a classification scheme in which pathological TDP-43 is the major disease defining pathology in one group, or is present in addition to other neurodegenerative hallmark pathologies in a second category. We conclude that the TDP-43 proteinopathies represent a novel class of neurodegenerative disorders akin to alpha-synucleinopathies and tauopathies, with the concept of ALS and FTLD-U to be widened to a broad clinico-pathological multisystem disease, i.e., TDP-43 proteinopathy.
机译：自从病理43-kDa反式激活序列DNA结合蛋白（TDP-43）对人类疾病的重要性在肌萎缩性侧索硬化症（ALS）和额颞叶变性伴泛素阳性包涵体（FTLD-U）的认识中得到了广泛认可关于神经退行性疾病的这种病理学的出版物已有报道。鉴于最近发现的异质性和部分矛盾性，我们在这里回顾病理性TDP-43及其与人类疾病的关系，并特别关注ALS和FTLD-U。为此，我们提出了一种分类方案，其中病理性TDP-43是一组中定义疾病的主要疾病，或者除第二类中的其他神经变性标志性病理外，还存在其他疾病。我们得出的结论是，TDP-43蛋白病代表一类新型的神经退行性疾病，类似于α-突触核蛋白病和tauopathies，ALS和FTLD-U的概念将扩大到广泛的临床病理多系统疾病，即TDP-43蛋白病。

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