Tauopathies are a class of neurodegenerative disorders characterized by neuronal and/or glial tau-positive inclusions. Clinically, tauopathies can present with a range of phenotypes that include cognitive/behavioral-disorders, movement disorders, language disorders and non-specific amnestic symptoms in advanced age. Pathologically, tauopathies can be classified based on the predominant tau isoforms that are present in the inclusion bodies (i.e., 3R, 4R or equal 3R:4R ratio). Imaging, cerebrospinal fluid (CSF) and blood-based tau biomarkers have the potential to be used as a routine diagnostic strategy and in the evaluation of patients with tauopathies. As tauopathies are strongly linked neuropathologically and genetically to tau protein abnormalities, there is a growing interest in pursuing of tau-directed therapeutics for the disorders. Here we synthesize emerging lessons on tauopathies from clinical, pathological, genetic, and experimental studies toward a unified concept of these disorders that may accelerate the therapeutics. Since tauopathies are still untreatable diseases, efforts have been made to depict clinical and pathological characteristics, identify biomarkers, elucidate underlying pathogenesis to achieve early diagnosis and develop disease-modifying therapies.

中文翻译：

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Tauopathy：新的观点和挑战

Tauopathies 是一类以神经元和/或胶质细胞 tau 阳性内含物为特征的神经退行性疾病。临床上，tau蛋白病可表现为一系列表型，包括认知/行为障碍、运动障碍、语言障碍和高龄非特异性遗忘症状。病理学上，可根据包涵体中存在的主要 tau 异构体（即 3R、4R 或相等的 3R:4R 比率）对 tau 病变进行分类。影像学、脑脊液 (CSF) 和基于血液的 tau 生物标志物有可能用作常规诊断策略和评估 tau 病患者。由于 tau 蛋白病在神经病理学和遗传上与 tau 蛋白异常密切相关，因此人们越来越关注针对 tau 蛋白的疾病进行治疗。在这里，我们综合了临床、病理学、遗传和实验研究中关于 tau 病变的新教训，以统一这些疾病的概念，从而加速治疗。由于 tau 蛋白病仍然是无法治疗的疾病，因此已努力描绘临床和病理特征、识别生物标志物、阐明潜在的发病机制以实现早期诊断和开发疾病缓解疗法。