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Mitochondrially encoded tRNA methionine (MT-TM)

Target
MT-TM
Molecular classification
tRNA, Non-coding RNA, Mitochondrial gene product, Other
01

Overview

Mitochondrially encoded tRNA methionine (MT-TM, also called trnM or mt-tRNA^Met^) is one of the 22 transfer RNAs encoded by the human mitochondrial genome. Its primary role is to transport methionine during mitochondrial protein synthesis, and it uniquely decodes both the canonical AUG codon and the non-canonical AUA codon as methionine in mitochondria. This tRNA undergoes essential post-transcriptional modifications, including methylation and formylation at the wobble base (C34), critical for accurate and flexible decoding. The sequential modification of C34 by NSUN3 and ABH1 is required for the unique ability of mt-tRNA^Met^ to decode the AUA codon as methionine. Mutations or failures in these modifications can cause mitochondrial translation defects and are associated with mitochondrial disease phenotypes. Unlike classic therapeutic targets such as enzymes or receptors, MT-TM functions as a core RNA component of the mitochondrial translation machinery and is not traditionally targeted by small-molecule drugs[1][2][3][4][5].

Other names
trnMmt-tRNA^Met^tRNA(Met) mitochondrialmitochondrial tRNA^Met^MT-TMmt-tRNAMet
02

Biological functions

Mitochondrial translationProtein biosynthesisCodon recognitionRNA metabolismInitiation of mitochondrial protein synthesis
03

Disease associations

Other (associated with mitochondrial diseases when mutated or modified)
04

Safety considerations

Mutations can cause mitochondrial dysfunction and diseasealteration in post-transcriptional modifications can impair mitochondrial translation and pathogenicity
05

Biomarkers

Mutations or modifications in MT-TM as biomarkers for specific mitochondrial disorders

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