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神经变性疾病的病因和发病机制尚不明确,但它们却有着相似的病理特征—细胞内外异常蛋白的聚集。这提示不同的神经变性疾病可能有一个共同的发病机制,即与异常蛋白的形成有关。泛素蛋白酶体系统在处理这些异常蛋白质中起核心作用,故泛素蛋白酶体系统在神经变性疾病发病机制中的作用倍受关注。本文就典型的神经变性疾病与泛素蛋白酶体系统功能障碍之间关系的研究进展做一文献综述。
The etiology and pathogenesis of neurodegenerative diseases is not clear, but they have a similar pathological features - the accumulation of abnormal proteins inside and outside the cell. This suggests that different neurodegenerative diseases may have a common pathogenesis, that is, the formation of abnormal proteins. Ubiquitin proteasome systems play a central role in the processing of these aberrant proteins, and the role of the ubiquitin proteasome system in the pathogenesis of neurodegenerative diseases has drawn much attention. This review summarizes the progress of the research on the relationship between typical neurodegenerative diseases and dysfunction of the ubiquitin proteasome system.