Mitochondrial rRNA methyltransferase 1 (MRM1)

Mitochondrial rRNA methyltransferase 1 (MRM1) is a nuclear-encoded, mitochondrial matrix-localized enzyme responsible for catalyzing the 2'-O-methylation of guanosine at position 1145 in 16S mitochondrial large subunit ribosomal RNA (mtLSU rRNA)[1][4][7]. This is a universally conserved modification vital for proper assembly and function of the mitochondrial ribosome, particularly affecting the peptidyl transferase domain required for efficient protein synthesis by mitochondria[2][4]. Deficiency or mutation of MRM1 has been associated with severe mitochondrial disorders such as combined oxidative phosphorylation deficiency and mitochondrial DNA depletion syndromes, reflecting its essential role in mitochondrial gene expression and cellular energy metabolism[1][2].

Other names

rRNA methyltransferase 1, mitochondrial16S rRNA (guanosine(1145)-2'-O)-methyltransferase16S rRNA [Gm1145] 2'-O-methyltransferaseFLJ22578CTB-75G16.3Mitochondrial large ribosomal RNA ribose methylaseA530065E19Rik (mouse ortholog)

For hypothetical targeting: Inhibition or modulation of MRM1 would affect 2'-O-methylation of 16S mt-rRNA, potentially disrupting mitochondrial protein synthesis. No extant drugs currently apply this mechanism.