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EC unknown
Reaction Schemes
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uridine1369 in 16S rRNA
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2'-O-methyluridine1369 in 16S rRNA
Synonyms
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S-adenosyl-L-methionine + uridine1369 in 16S rRNA = S-adenosyl-L-homocysteine + 2'-O-methyluridine1369 in 16S rRNA
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S-adenosyl-L-methionine:16S rRNA (uridine1369-2'-O-)-methyltransferase
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S-adenosyl-L-methionine + uridine1369 in 16S rRNA
S-adenosyl-L-homocysteine + 2'-O-methyluridine1369 in 16S rRNA
Substrates: -
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S-adenosyl-L-methionine + uridine1369 in 16S rRNA
S-adenosyl-L-homocysteine + 2'-O-methyluridine1369 in 16S rRNA
Substrates: -
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UniProt
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Highest Expressing Human Cell Lines
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Cell Line Links
Gene Links
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physiological function
MRM2 and MRM3 are responsible for modification of nucleotides of the 16S rRNA A-loop, an essential component of the peptidyl transxadferase center. Inactivation of MRM2 or MRM3 in human cells by RNA interference results in respiratory incompetence as a consequence of diminished mitochondrial translation. Ineffective translation in MRM2- and MRM3-depleted cells results from aberxadrant assembly of the large subunit of the mitochondrial ribosome
physiological function
siRNA directed against MRM2 results in loss of methylation at at residue U1369 of 16S RNA and in a general suppression of cell growth
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MRM2_HUMAN
246
0
27424
Swiss-Prot
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G189R
mutation identified in a 7-year-old boy with childhood-onset rapidly progressive encephalomyopathy and stroke-like episodes
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medicine
mutation G189R identified in a 7-year-old boy with childhood-onset rapidly progressive encephalomyopathy and stroke-like episodes. Multiple OXPHOS defects and decreased mtDNA copy number (40%) are detected in muscle homogenate. Symptoms are similar to mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome. A yeast MRM2 knockout mutant shows a defect in respiration and the reduction of the 2'-O-methylmodification at position U2791 in the yeast mitochondrial 21S rRNA. Complementation of the yeast knockout mutant with the human mutant Mrm2 fails to rescue the respiratory phenotype, which is instead completely rescued by expressing the wild-type allele
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Garone, C.; DSouza, A.R.; Dallabona, C.; Lodi, T.; Rebelo-Guiomar, P.; Rorbach, J.; Donati, M.A.; Procopio, E.; Montomoli, M.; Guerrini, R.; Zeviani, M.; Calvo, S.E.; Mootha, V.K.; DiMauro, S.; Ferrero, I.; Minczuk, M.
Defective mitochondrial rRNA methyltransferase MRM2 causes MELAS-like clinical syndrome
Hum. Mol. Genet.
26
4257-4266
2017
Homo sapiens (Q9UI43)
brenda
Lee, K.W.; Bogenhagen, D.F.
Assignment of 2'-O-methyltransferases to modification sites on the mammalian mitochondrial large subunit 16 S ribosomal RNA (rRNA)
J. Biol. Chem.
289
24936-24942
2014
Homo sapiens (Q9UI43)
brenda
Rorbach, J.; Boesch, P.; Gammage, P.A.; Nicholls, T.J.J.; Pearce, S.F.; Patel, D.; Hauser, A.; Perocchi, F.; Minczuk, M.
MRM2 and MRM3 are involved in biogenesis of the large subunit of the mitochondrial ribosome
Mol. Biol. Cell
25
2542-2555 /
2014
Homo sapiens (Q9UI43), Homo sapiens
brenda
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