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许多神经退行性疾病的发生是由蛋白质错误折叠和聚集引起的,这类疾病统称为神经变性构象病。基因突变、环境因素以及蛋白质质控系统(泛素-蛋白酶体系统和自吞噬-溶酶体系统)功能的失常,都能导致蛋白质错误折叠并聚集而引发疾病。本文综述了引起蛋白质错误折叠和聚集的相关因素和机制、蛋白质错误折叠在神经退行性疾病发病中的作用以及诱导热休克蛋白作为一种新途径治疗神经变性构象病的研究进展。
Many neurodegenerative diseases are caused by misfolding and aggregation of proteins, which are collectively referred to as neurodegenerative conformation diseases. Genetic mutations, environmental factors and disorders in the functions of the protein control system (ubiquitin-proteasome system and autophagy-lysosomal system) can cause proteins to misfolded and aggregate to cause disease. This review summarizes the relevant factors and mechanisms that cause protein misfolding and aggregation, the role of protein misfolding in the development of neurodegenerative diseases, and the progress in the induction of heat shock protein as a new approach to the treatment of neurodegenerative conformation diseases.