| Modification Notes |
TRMU (tRNA 5-methylaminomethyl-2-thiouridylate methyltransferase), also known as MTO2, MTU1, TRMT, TRMT1 or TRNT1, is a 421 amino acid mitochondrial protein that is evolutionarily conserved and is involved in mitochondrial tRNA modification. Expressed ubiquitously with higher levels of expression in brain, heart, kidney and liver, TRMU functions to catalyze the 2-thiolation of uridine at the wobble position of select tRNA amino acids, including lysine, glutamine and glutamate. This event is required for the formation of 5-taurinomethyl-2-thiouridine, a hypermodified nucleotide that is essential for proper stabilization, aminoacylation and overall function of tRNAs. Polymorphisms in the gene encoding TRMU are thought to aggravate the mitochondrial dysfunction associated with aminoglycoside-induced and non-syndromic deafness, suggesting a role for TRMU in the development of this disorder. Multiple isoforms of TRMU exist due to alternative splicing events. |