神经变性疾病是以特定的神经细胞变性、脱失、死亡为主要病变的一组疾病,其临床表现多样,病因不明,发病机制复杂,以至于曾被称为神经难病。近年来,我们已经知道许多神经变性疾病是由于特定的蛋白质的空间构象发生变化,随之在细胞内外蓄积,形成特定的包涵体,从而诱导了细胞的死亡。根据中枢神经系统沉积的主要蛋白,将神经变性疾病分为Tau病、α-synuclein病、TDP-43蛋白病、FUS/FET蛋白病、朊病毒病、三核苷酸串联重复序 Neurodegenerative diseases are a group of diseases characterized by degeneration, loss and death of specific nerve cells. The clinical manifestations are diverse, the etiology is unknown, and the pathogenesis is so complicated that they have been called neuropathies. In recent years, we have learned that many neurodegenerative diseases are caused by changes in the spatial conformation of specific proteins, which then accumulate inside and outside the cells to form specific inclusion bodies, thereby inducing cell death. Neurodegenerative diseases are classified into Tau disease, α-synuclein disease, TDP-43 proteinopathy, FUS / FET proteinopathy, prion disease, trinucleotide tandem repeats, based on the major proteins deposited in the central nervous system