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In conclusion, in this review, we briefly describe the mitochondrial structure, summarize the specific regulatory mechanisms of the mitochondrial quality control system and the interaction between various pathways, and finally elaborate the research progress of mitochondrial biogenesis, mitophagy and mitochondrial dynamics in several common neurodegenerative diseases, in order to understand more about the mitochondrial quality control system and provide new research targets and ideas for the treatment of neurodegenerative diseases