Mitochondrial rRNA methyltransferase 2 (MRM2)

Mitochondrial rRNA methyltransferase 2 (MRM2) is an S-adenosyl-L-methionine-dependent 2'-O-methyltransferase that specifically methylates uridine at position 1369 (Um1369) in the 16S mitochondrial large subunit ribosomal RNA (mtLSU rRNA) in humans, a modification that is highly conserved in the peptidyl transferase center of ribosomes across species. This enzyme is essential for proper late-stage assembly of the mitochondrial large ribosomal subunit and efficient translation of mitochondrial-encoded proteins, which are critical for mitochondrial respiratory function. Loss or mutation of MRM2 impairs mitochondrial protein synthesis and is associated with diseases such as mitochondrial DNA depletion syndrome 17. MRM2 is the human ortholog of yeast Mrm2p and *E. coli* RrmJ/FtsJ, enzymes with a conserved function in ribosomal RNA methylation.

Other names

rRNA methyltransferase 2, mitochondrialS-adenosylmethionine-dependent methyltransferaseMRM2_HUMANFTSJ2 (paralog FTSJ3)Mrm2p (yeast ortholog)

Catalyzes 2'-O-methylation of uridine in mitochondrial 16S rRNA (modifies Um1369 in humans); required for mitochondrial ribosome assembly and function