Department of Neurology, School of Medicine, Chengde Medical University Affiliated Hospital, Chengde Medical University, Anyuan Road, Chengde, People's Republic of China.
Department of Neurology, Chengde Medical University Affiliated Hospital, Chengde Medical University, No.36 Nanyingzi Road, Chengde, People's Republic of China.
Mol Neurobiol. 2022 Jul;59(7):4223-4241. doi: 10.1007/s12035-022-02847-x. Epub 2022 May 2.
In recent years, more and more neurodegenerative diseases, such as ALS, FTLD and AD, have been found to share a common pathological feature, which is the depletion of TDP-43 in the nucleus and the accumulation of TDP-43 in the cytoplasm through hyperphosphorylation, ubiquitination and cleavage. Therefore, this kind of neurodegenerative disease is also called TDP-43 proteinopathy. This suggests that TDP-43 plays a role in the pathogenesis of disease. Current studies show that the pathophysiological mechanism of TDP-43 in neurodegeneration is very complex. In this review, we describe the structure of TDP-43, its main physiological functions, the possible pathogenesis and how TDP-43 provides a new pathway to treat neurodegenerative diseases.
近年来，越来越多的神经退行性疾病，如 ALS、FTLD 和 AD，被发现具有共同的病理特征，即 TDP-43 在核内耗竭，通过过度磷酸化、泛素化和切割在细胞质中积累。因此，这种神经退行性疾病也称为 TDP-43 蛋白病。这表明 TDP-43 在疾病发病机制中起作用。目前的研究表明，TDP-43 在神经退行性变中的病理生理机制非常复杂。在这篇综述中，我们描述了 TDP-43 的结构、其主要生理功能、可能的发病机制以及 TDP-43 如何为治疗神经退行性疾病提供新途径。
