rRNA modification in the mitochondrion

Summary
Organism
Homo sapiens (human)
Reactome
R-HSA-6793080
PubChem
R-HSA-6793080
Description
  • 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).
Click on a node on the pathway to see its details. Glycoproteins are marked with a glycoprotein icon in their name.
Displaying all 3 entries
UniProt ID Protein Name Gene Symbol Pathway Viewer
Q96E29 Transcription termination factor 3, mitochondrial
  • CGI-12
  • MTERF3
  • MTERFD1
view
Q9HC36 rRNA methyltransferase 3, mitochondrial
  • HC90
  • MRM3
  • RNMTL1
view
Q9NPE2 Neugrin
  • FI58G
  • HT020
  • NGRN
view

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Acknowledgements

Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01


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