Human Mitochondrial tRNA Modification and Diseases
ZHANG Yong1,2, ZHOU Xiaolong1,2,3*
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) com plexes. 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 interac tions. Thus, abnormal mt-tRNA modification and modification enzymes are closely related to mitochondrial dys functions 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.
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