Pathophysiology of human mitochondrial tRNA metabolism

Jian-Hui Zhang; Gilbert Eriani; Xiao-Long Zhou

doi:10.1016/j.tem.2024.01.002

Pathophysiology of human mitochondrial tRNA metabolism

Trends Endocrinol Metab. 2024 Apr;35(4):285-289. doi: 10.1016/j.tem.2024.01.002. Epub 2024 Feb 1.

Authors

Jian-Hui Zhang¹, Gilbert Eriani², Xiao-Long Zhou³

Affiliations

¹ Key Laboratory of Systems Health Science of Zhejiang Province, School of Life Science, Hangzhou Institute for Advanced Study, University of Chinese Academy of Sciences, Hangzhou 310024, China; Key Laboratory of RNA Science and Engineering, CAS Center for Excellence in Molecular Cell Science, Shanghai Institute of Biochemistry and Cell Biology, Chinese Academy of Sciences, University of Chinese Academy of Sciences, 320 Yue Yang Road, Shanghai 200031, China.
² Architecture et Réactivité de l'ARN, Institut de Biologie Moléculaire et Cellulaire du CNRS, Université de Strasbourg, 2 allée Konrad Roentgen, 67084 Strasbourg, France. Electronic address: g.eriani@ibmc-cnrs.unistra.fr.
³ Key Laboratory of Systems Health Science of Zhejiang Province, School of Life Science, Hangzhou Institute for Advanced Study, University of Chinese Academy of Sciences, Hangzhou 310024, China; Key Laboratory of RNA Science and Engineering, CAS Center for Excellence in Molecular Cell Science, Shanghai Institute of Biochemistry and Cell Biology, Chinese Academy of Sciences, University of Chinese Academy of Sciences, 320 Yue Yang Road, Shanghai 200031, China. Electronic address: xlzhou@sibcb.ac.cn.

PMID: 38307811
DOI: 10.1016/j.tem.2024.01.002

Abstract

Mitochondria play multiple critical roles in cellular activity. In particular, mitochondrial translation is pivotal in the regulation of mitochondrial and cellular homeostasis. In this forum article, we discuss human mitochondrial tRNA metabolism and highlight its tight connection with various mitochondrial diseases caused by mutations in aminoacyl-tRNA synthetases, tRNAs, and tRNA-modifying enzymes.

MeSH terms

Amino Acyl-tRNA Synthetases* / genetics
Amino Acyl-tRNA Synthetases* / metabolism
Humans
Mitochondria* / genetics
Mitochondria* / metabolism
RNA, Transfer / genetics
RNA, Transfer / metabolism

Substances

Amino Acyl-tRNA Synthetases
RNA, Transfer