与神经退行性疾病相关的RNA结合蛋白在线粒体损伤中的作用

Mitochondrial Damage Induced by RNA Binding Proteins Which Are Associated With Neurodegenerative Diseases

线粒体是细胞内制造能量的细胞器,它还负责各种细胞信号的整合,参与协调多种复杂的细胞功能.线粒体是动态变化的,连续不断地进行分裂与融合,这是其功能维持和增殖遗传的关键.在过去20年中,参与线粒体分裂与融合的核心因子陆续被发现,它们在进化上高度保守,但是在形成分裂与融合复合物中的详细分子机制还有待于深入研究.线粒体分裂与融合的动态变化,是线粒体质量控制的重要组成部分,其动态平衡在细胞发育和稳态维持中起重要作用.线粒体动态变化失衡和功能失调,则会导致多种神经退行性疾病的发生.这些研究的发现为探索线粒体生物学及与疾病的关系开拓了令人振奋的新方向.

The mitochondria are the energy-generating organelles in the cytoplasm of the cell. They also serve as signaling organelles that coordinate complex cellular functions. Mitochondria are highly dynamic and undergo fusion and fission processes continuously, which is crucial for the maintenance of mitochondrial homeostasis and the balance of mitochondrial turnover. Although most of the evolutionarily conserved core components of the mitochondrial fusion and fission machineries have been identified in the past decade, the mechanistic insights into their molecular functions remain to be investigated. Mitochondrial fusion and fission（collectively termed mitochondrial dynamics） takes part in cellular quality control system and play a key role in the development of the cell, tissue and organism. Dysfunctions of mitochondrial dynamics are implicated in various inherited and age-related neurodegenerative diseases. Thus, the research in the relationship between mitochondrial biology and diseases will remain an exciting field in the coming years.