线粒体转移核糖核酸(mt-tRNA)的牛磺酸修饰——纪念邹承鲁先生百年诞辰  

Mitochondrial Transfer Ribonucleic Acid(mt-tRNA)Taurine Modification

作　　者：彭桂鑫 王恩多 周小龙[1,2] PENG Gui-Xin;WANG En-Duo;ZHOU Xiao-Long(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;School of Life Science and Technology,ShanghaiTech University,Shanghai 201210,China)

机构地区：[1]中国科学院分子细胞科学卓越创新中心/上海生物化学与细胞生物学研究所,分子生物学国家重点实验室,上海200031 [2]中国科学院大学,北京100864 [3]上海科技大学生命科学与技术学院,上海201210

出　　处：《生物化学与生物物理进展》2023年第5期867-876,共10页Progress In Biochemistry and Biophysics

基　　金：国家重点研发计划(2021YFA1300800);国家自然科学基金(32271300)资助项目。

摘　　要：转移核糖核酸(tRNA)是转录后修饰种类最多和修饰最密集的RNA分子,特别是其反密码子环含有大量的修饰。线粒体具有相对独立的蛋白质合成系统,线粒体tRNA (mt-tRNA)全部由线粒体基因组编码。研究表明,5-牛磺酸甲基尿嘧啶核苷(5-taurinomethyluridine,τm5U)修饰只存在于高等真核生物mt-tRNA第34位,能够调节密码子和反密码子相互作用的精确性,控制翻译的速度和保真性。人类GTP结合蛋白质3(GTPBP3)和线粒体翻译优化蛋白1(MTO1)介导τm5U修饰,其缺陷可能引起线粒体脑肌病。本文综述了τm5U修饰及其修饰酶的生物学性质,为深入研究τm5U修饰的机制,及认识τm5U修饰缺陷导致线粒体疾病的致病机理提供一个新的视角。Transfer ribonucleic acid(tRNA)is the RNA molecule with the largest variety of post-transcriptional modifications.In particular its anticodon loop contains a large number of modifications.Mitochondria have a relatively independent protein synthesis system,mitochondrial tRNAs(mt-tRNAs)are all encoded by the mitochondrial genome.Studies have shown that 5-taurinomethyluridine modification(τm5 U)only exists at position 34 of mitochondrial tRNAs of higher eukaryotes,regulating the fidelity of codon and anticodon interaction and contributing to translation speed and fidelity.Human GTPBP3 and MTO1 mediate mitochondrialτm5 U modification,whose functional defects may cause mitochondrial encephalomyopathy.This review summarizes the biological properties ofτm5 U modification and its modifying enzymes,providing a new insight into the mechanism ofτm5 U modification and the pathogenesis of mitochondrial diseases caused byτm5 U modification defects.

关 键 词：转移核糖核酸 转录后修饰 线粒体疾病 生物学功能 

分 类 号：Q5[生物学—生物化学]