MRM3 (RNMTL1) methylates guanosine-1370 of 16S rRNA yielding 2'-O-methylguanosine-1370

Stable Identifier
R-HSA-6793096
Type
Reaction [transition]
Species
Homo sapiens
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MRM3 (RNMTL1) associates with the large mitochondrial ribosomal subunit and transfers a methyl group from S-adenosylmethionine to the 2' hydroxyl group of guanosine-1370 of 16S rRNA (Lee et al. 2013, Lee and Bogenhagen 2014). Inactivation of RNMTL1 causes aberrant assembly of large ribosomal subunits, decreased mitochondrial translation, and respiratory incompetence (Rorbach et al. 2014).

Literature References
PubMed ID Title Journal Year
24036117 Mitochondrial ribosomal RNA (rRNA) methyltransferase family members are positioned to modify nascent rRNA in foci near the mitochondrial DNA nucleoid

Lee, KW, Okot-Kotber, C, LaComb, JF, Bogenhagen, DF

J. Biol. Chem. 2013
25074936 Assignment of 2'-O-methyltransferases to modification sites on the mammalian mitochondrial large subunit 16 S ribosomal RNA (rRNA)

Lee, KW, Bogenhagen, DF

J. Biol. Chem. 2014
25009282 MRM2 and MRM3 are involved in biogenesis of the large subunit of the mitochondrial ribosome

Rorbach, J, Boesch, P, Gammage, PA, Nicholls, TJ, Pearce, SF, Patel, D, Hauser, A, Perocchi, F, Minczuk, M

Mol. Biol. Cell 2014
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Catalyst Activity
Title
rRNA (guanosine-2'-O-)-methyltransferase activity of RNMTL1 [mitochondrial matrix]
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