人线粒体tRNA修饰与疾病  

Human Mitochondrial tRNA Modification and Diseases

作　　者：张勇 周小龙 ZHANG Yong;ZHOU Xiaolong(Key Laboratory of RNA Innovation,Science and Engineering,Center for Excellence in Molecular Cell Science,Shanghai Institute of Biochemistry and Cell Biology,Chinese Academy of Sciences,Shanghai 200031,China;University of Chinese Academy of Sciences,Beijing 100864,China;Key Laboratory of Systems Health Science of Zhejiang Province,School of Life Science,Hangzhou Institute for Advanced Study,Hangzhou 310024,China)

机构地区：[1]核糖核酸功能与应用全国重点实验室,中国科学院分子细胞科学卓越创新中心/生物化学与细胞生物学研究所,上海200031 [2]中国科学院大学,北京100864 [3]国科大杭州高等研究院生命与健康科学学院,浙江省系统健康科学重点实验室,杭州310024

出　　处：《中国细胞生物学学报》2024年第9期1623-1634,共12页Chinese Journal of Cell Biology

基　　金：国家重点研发计划(批准号:2021YFA1300800);中国科学院战略先导B项目(批准号:XDB0570000);中国科学院稳定支持基础研究领域青年团队计划(批准号:YSBR075)资助的课题;国家自然科学基金(批准号:32271300);上海市科学技术委员会项目(批准号:22ZR1481300和22JC1400503)。

摘　　要：人线粒体作为细胞中重要的能量工厂,拥有自己的基因组。线粒体基因组编码2种rRNA和22种tRNA,用于翻译产生13种氧化磷酸化复合物亚基。线粒体t RNA(mt-tRNA)作为接头分子携带特定氨基酸参与线粒体蛋白质合成。线粒体tRNA上含有18种转录后修饰。这些修饰对于稳定tRNA结构,介导密码子与反密码子的相互作用具有重要作用。因此,线粒体t RNA修饰水平和修饰酶的异常变化与线粒体功能紊乱及疾病的发生发展密切相关。该文综述部分线粒体tRNA修饰的研究进展并介绍它们与线粒体脑肌病、神经系统疾病和癌症的关系。As the pivotal energy hub of cells,human mitochondria have their own genomes and translation system to synthesize thirteen mitochondrial DNA-encoded subunits for OXPHOS(oxidative phosphorylation)complexes.Mt-tRNA(mitochondrial transfer ribonucleic acid)serves as an adaptor in mitochondrial protein synthesis by carrying the specific amino acid to the ribosome.Mt-tRNAs harbor 18 types of posttranscriptional modifications,which play important roles in stabilizing tRNA tertiary structure and/or mediating precise codon-anticodon interactions.Thus,abnormal mt-tRNA modification and modification enzymes are closely related to mitochondrial dysfunctions and human diseases.This review summarizes the recent advances in the study of mt-tRNA modifications and discusses their relevance to various mitochondrial encephalomyopathies,neurological disorders and cancers.

关 键 词：线粒体 tRNA修饰 线粒体脑肌病 神经系统疾病 癌症 

分 类 号：R363[医药卫生—病理学]