InterPro domain: IPR004416

tRNA uridine 5-carboxymethylaminomethyl modification enzyme MnmG (also known as GidA) is a tRNA modification enzyme found in bacteria and mitochondria. MnmG forms a complex with MnmE which is involved in the formation of methyluridine derivatives at the wobble uridine base in some tRNAs [ 1 , 2 , 3 ]. Sequence variations in the human mitochondrial protein may influence the severity of aminoglycoside-induced deafness [ 4 ].

1. Mitochondria-specific RNA-modifying enzymes responsible for the biosynthesis of the wobble base in mitochondrial tRNAs. Implications for the molecular pathogenesis of human mitochondrial diseases. J. Biol. Chem. 280, 1613-24
2. Translational misreading: a tRNA modification counteracts a +2 ribosomal frameshift. Genes Dev. 15, 2295-306
3. Evolutionarily conserved proteins MnmE and GidA catalyze the formation of two methyluridine derivatives at tRNA wobble positions. Nucleic Acids Res. 37, 7177-93
4. Phenotype of non-syndromic deafness associated with the mitochondrial A1555G mutation is modulated by mitochondrial RNA modifying enzymes MTO1 and GTPBP3. Mol. Genet. Metab. 83, 199-206