Tauopathies are a diverse group of progressive and fatal neurodegenerative diseases characterized by aberrant tau inclusions in the central nervous system. Tau protein forms pathologic fibrillar aggregates that are typically closely associated with neuronal cell death, leading to varied clinical phenotypes including dementia, movement disorders, and motor neuron disease. In this review, we describe the clinicopathologic features of tauopathies and highlight recent advances in understanding the mechanisms that lead to spread of pathologic aggregates through interconnected neuronal pathways. The cell-to-cell propagation of tauopathy is then linked to posttranslational modifications, tau fibril structural variants, and the breakdown of cellular protein quality control.

中文翻译：

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神经退行性疾病 tau 蛋白病


tau 蛋白病是一组多样化的进行性和致命性神经退行性疾病，其特征是中枢神经系统中异常的 tau 包涵体。Tau 蛋白形成病理原纤维聚集体，通常与神经元细胞死亡密切相关，导致不同的临床表型，包括痴呆、运动障碍和运动神经元疾病。在这篇综述中，我们描述了 tau 蛋白病的临床病理特征，并强调了在理解导致病理聚集体通过相互连接的神经元通路传播的机制方面的最新进展。然后，tau 蛋白病的细胞间增殖与翻译后修饰、tau 原纤维结构变异和细胞蛋白质质量控制的分解有关。