1.昆明理工大学灵长类转化医学研究院,昆明 650500; 2.云南省中科灵长类生物医学重点实验室,昆明 650500
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1.Institute of Primate Translational Medicine of Kunming University of Science and Technology, Kunming 650500, China. 2. Yunnan Province Key Laboratory of Primate Biomedicine, Kunming 650500
帕金森病(Parinson’s disease,PD)是全球第二大中枢神经系统退行性疾病,其病理特征主要是黑质中 a-突触核蛋白(a-synuclein,a-syn)的聚集,其早期征兆是线粒体功能紊乱。 PD 相关蛋白 PINK1、Parkin、DJ-1 蛋 白和 a-syn 等参与线粒体自噬及质量控制过程,PD 相关蛋白突变时导致线粒体自噬及其功能异常,从而出现受损线粒体不能被选择性清除和线粒体功能障碍的现象,最终可能造成多巴胺能神经元的死亡。 因此 PD 相关蛋白的失控与 PD 的发生密切相关,本文将对线粒体自噬及功能障碍与帕金森病的关系做一综述。
Parkinson’ s disease ( PD) is the second most common central nervous system degenerative disease worldwide. One pathological feature is a-synuclein accumulation in the substantia nigra, and an early sign is mitochondrial dysfunction. The PD-related proteins PINK1, Parkin, DJ-1, and a-synuclein are involved in the process of mitochondrial autophagy and quality control. The mutation of PD-related proteins leads to abnormal autophagy and mitochondrial function. A failure of selective clearance of damaged mitochondria and mitochondrial dysfunction may eventually lead to dopaminergic neuron death. Therefore, the loss of control of PD-related proteins is closely related to PD occurrence. This article reviews the associations between mitochondrial autophagy, dysfunction, and Parkinson’s disease.
甘 雪,刘书一,王正波.线粒体自噬及功能障碍与帕金森病[J].中国比较医学杂志,2020,30(10):121~127.
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