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由基因突变导致蛋白变性是形成众多神经系统变性疾病中细胞包涵体的主要原因,它进而引起特异脑区神经元功能丧失。遍在蛋白系统对清除机体内变性蛋白有重要的意义,此系统发生变异将导致有毒蛋白的聚集受包涵体的形成。有三个遍在蛋白酶体系统相关基因的突变参与了帕金森病的病理过程,深入了解这些基因突变在包涵体的形成及代谢过程中的作用对于神经系统变性疾病的病因了解及临床治疗有重要意义。
Protein denaturation, caused by mutations in genes, is the major cause of the formation of many of the cellular inclusions in degenerative diseases of the nervous system, which in turn leads to loss of function of neurons in specific brain regions. Ubiquitin protein system is very important for the removal of denatured protein in the body, and the variation of this system will lead to the accumulation of toxic protein by inclusion body formation. There are three ubiquitin proteasome system related gene mutations involved in the pathological process of Parkinson’s disease, in-depth understanding of these gene mutations in the formation of inclusion bodies and metabolic processes for the etiology of nervous system degenerative disease understanding and clinical treatment of great significance .