Editing activity for eliminating mischarged tRNAs is essential in mammalian mitochondria | |
Hilander, Taru1; Konovalova, Svetlana1; Euro, Liliya1; Chilov, Dmitri1; Tyynismaa, Henna1,6; Zhou, Xiao-Long2,3; Wang, En-Duo2,3; Zhang, Fu-Ping4; Poutanen, Matti4; Chihade, Joseph5 | |
刊名 | NUCLEIC ACIDS RESEARCH |
2018 | |
卷号 | 46期号:2页码:849-860 |
关键词 | Quality-control Human-disease Synthetase Mutations Protein-synthesis Aminoacylation Defects Mistranslation Recognition Polymerase Proteases |
ISSN号 | 0305-1048 |
DOI | 10.1093/nar/gkx1231 |
文献子类 | Article |
英文摘要 | Accuracy of protein synthesis is enabled by the selection of amino acids for tRNA charging by aminoacyl-tRNA synthetases (ARSs), and further enhanced by the proofreading functions of some of these enzymes for eliminating tRNAs mischarged with noncognate amino acids. Mouse models of editing-defective cytoplasmic alanyl-tRNA synthetase (AlaRS) have previously demonstrated the importance of proofreading for cytoplasmic protein synthesis, with embryonic lethal and progressive neurodegeneration phenotypes. Mammalian mitochondria import their own set of nuclear-encoded ARSs for translating critical polypeptides of the oxidative phosphorylation system, but the importance of editing by the mitochondrial ARSs for mitochondrial proteostasis has not been known. We demonstrate here that the human mitochondrial AlaRS is capable of editing mischarged tRNAs in vitro, and that loss of the proofreading activity causes embryonic lethality in mice. These results indicate that tRNA proofreading is essential in mammalian mitochondria, and cannot be overcome by other quality control mechanisms. |
电子版国际标准刊号 | 1362-4962 |
WOS研究方向 | Biochemistry & Molecular Biology |
语种 | 英语 |
WOS记录号 | WOS:000423812300033 |
内容类型 | 期刊论文 |
版本 | 出版稿 |
源URL | [http://202.127.25.143/handle/331003/3396] |
专题 | 生化所2018年发文 |
通讯作者 | Tyynismaa, Henna; Wang, En-Duo |
作者单位 | 1.Univ Helsinki, Res Programs Unit, Mol Neurol, FIN-00290 Helsinki, Finland; 2.Chinese Acad Sci, State Key Lab Mol Biol, CAS Ctr Excellence Mol Cell Sci, Shanghai Inst Biochem & Cell Biol, Shanghai 200031, Peoples R China; 3.Univ Chinese Acad Sci, Shanghai 200031, Peoples R China; 4.Univ Turku, Inst Biomed, Turku Ctr Dis Modeling, FIN-20520 Turku, Finland; 5.Carleton Coll, Dept Chem, Northfield, MN 55057 USA; 6.Univ Helsinki, Dept Med & Clin Genet, FIN-00290 Helsinki, Finland |
推荐引用方式 GB/T 7714 | Hilander, Taru,Konovalova, Svetlana,Euro, Liliya,et al. Editing activity for eliminating mischarged tRNAs is essential in mammalian mitochondria[J]. NUCLEIC ACIDS RESEARCH,2018,46(2):849-860. |
APA | Hilander, Taru.,Konovalova, Svetlana.,Euro, Liliya.,Chilov, Dmitri.,Tyynismaa, Henna.,...&Chihade, Joseph.(2018).Editing activity for eliminating mischarged tRNAs is essential in mammalian mitochondria.NUCLEIC ACIDS RESEARCH,46(2),849-860. |
MLA | Hilander, Taru,et al."Editing activity for eliminating mischarged tRNAs is essential in mammalian mitochondria".NUCLEIC ACIDS RESEARCH 46.2(2018):849-860. |
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