Post-transcriptional Processing and Modification of Mitochondrial RNA and Their Relationship with Human Mitochondrial Diseases / 中国生物化学与分子生物学报

ABSTRACT

Mitochondria are important organelles involved in energy generation and material metabolism in eukarvotic cells, and have their own genomic DNA.The regulation of mitochondrial gene expression is essential for the maintenance of mitochondrial function.According to the central law of molecular biology, genetic information is transferred from DNA to UNA, and then from RNA to proteins.Mitochondrial DNA (mtDNA) encodes 13 messenger RNAs (mRNAs) , 2 ribosomal RNAs (rRNAs) , and 22 transfer RNAs (tRNAs).The various precursor RNAs synthesized during transcription often have to undergo subsequent changes to transform into active mature RNA molecules, which is called "post-transcriptional processing and modification" .Herein we review the post-transcriptional processing and modification methods of the three RNAs encoded by the mammalian mitochondrial genome, and further explore the relationship between mammalian mitochondrial RNA post-transcriptional modification and mitochondrial diseases from the perspective of modification sites and modification enzymes.We aim to provide new ideas for the diagnosis and treatment of human mitochondrial diseases.