人线粒体RNA加工及调控

Human Mitochondrial RNA Processing and Regulation

线粒体是细胞内氧化磷酸化(oxidative phosphorylation,OXPHOS)和合成三磷酸腺苷(adenosine triphosphate,ATP)的细胞器,是细胞能量代谢的“动力工厂”。线粒体几乎存在于所有真核生物中,参与细胞凋亡、钙稳态以及先天免疫反应的调节等过程,对细胞行使正常的生理功能至关重要。线粒体是半自主细胞器,拥有自身的基因组DNA,编码37个基因,包括2个rRNA基因、13个m RNA基因和22个tRNA基因。线粒体的基因表达需要经过复杂的转录和转录后加工过程,包括多顺反子RNA的切割、RNA的修饰以及RNA的末端加工等过程。异常的线粒体RNA加工会导致线粒体RNA表达谱发生变化、线粒体翻译紊乱、线粒体功能失常等,从而造成多种线粒体相关疾病。本文综述了线粒体DNA的转录、RNA转录后加工以及影响RNA加工的因素方面的最新研究进展。

Mitochondria are semi-autonomous cellular organelles responsible for oxidative phosphorylation(OXPHOS)and adenosine triphosphate(ATP)synthesis and are the powerhouses of cellular metabolism.Mitochondria are present in almost all eukaryotic organisms and are involved in apoptosis,calcium homeostasis,and regulation of the innate immune responses,which play a vital role in normal physiological processes.Mitochondria contain their own DNA that encodes 37 genes,including 2 rRNAs,13 mRNA,and 22 tRNAs genes.Gene expression in mitochondria involves complex transcriptional and post-transcriptional processes,including cleavage of polycistronic RNA,RNA modification,and terminal processing of RNA.These processes require the coordinated spatiotemporal action of several enzymes,and many different factors are involved in the regulation and control of protein synthesis to maintain the stability and turnover of mitochondrial RNA.Disorders in mitochondrial RNA processing lead to changes in RNA expression profiles,interfere with protein translation,cause mitochondrial dysfunction,and result in a variety of mitochondria-related diseases.Although substantial progress has been made in the field of mitochondrial RNA processing and regulation,there are still many controversies and unknowns.This article reviews the latest research progress on mitochondrial DNA transcription,RNA post-transcriptional processing,and factors affecting RNA processing.