rRNA modification in the mitochondrion

Stable Identifier
R-HSA-6793080
Type
Pathway
Species
Homo sapiens
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Five modified nucleotides have been detected in the 12S rRNA: 5-methylcytidine-841 catalyzed by NSUN4, 6-dimethyladenosine-936 catalyzed by TFB1M, 6-dimethyladenosine-937 catalyzed by TFB1M, 5-methyluridine-429, and 4-methylcytidine-839 (reviewed in Van Haute et al. 2015). Four modified nucleotides have been detected in 16S rRNA: 2'-O-methylguanosine-1145 catalyzed by MRM1, 2'-O-methylguanosine-1370 catalyzed by RNMTL1 (MRM3), 2'-O-methyluridine-1369 catalyzed by FTSJ2 (MRM2), and pseudouridine-1397. 2'-O-methyluridine-1369 and 2'-O-methylguanosone-1370 occur in the A-loop of rRNA which is located at the peptidyl transferase center of the large subunit. Here the modified residues play a role in interaction with the aminoacyl site of tRNA. Knockouts of TFB1M and NSUN4 are lethal in mice and mutations in TFB1M may be related to aminoglycoside-induced deafness (reviewed in Van Haute et al. 2015).

Literature References
PubMed ID Title Journal Year
26016801 Mitochondrial transcript maturation and its disorders

Van Haute, L, Pearce, SF, Powell, CA, D'Souza, AR, Nicholls, TJ, Minczuk, M

J. Inherit. Metab. Dis. 2015
22642575 The post-transcriptional life of mammalian mitochondrial RNA

Rorbach, J, Minczuk, M

Biochem. J. 2012
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