NSUN4 methylates cytidine-841 of 12S rRNA yielding 5-methylcytidine-841

Stable Identifier
R-HSA-6793057
Type
Reaction [transition]
Species
Homo sapiens
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MTERF4 forms a complex with NSUN4 (Camara et al. 2011, Spahr et al. 2012, Yakubovskaya et al. 2012) and recruits NSUN4 to the ribosome where NSUN4 transfers a methyl group from S-adenosylmethionine to the 5 position of cytidine-841 in 12S rRNA (Camara et al. 2011). Knockout of MTERF4 in mice is lethal and cardiomyocyte-specific knockout is accompanied by mitochondrial cardiomyopathy (Camara et al. 2011).

Literature References
PubMed ID Title Journal Year
23022348 Structure of the essential MTERF4:NSUN4 protein complex reveals how an MTERF protein collaborates to facilitate rRNA modification

Yakubovskaya, E, Guja, KE, Mejia, E, Castano, S, Hambardjieva, E, Choi, WS, Garcia-Diaz, M

Structure 2012
22949673 Structure of the human MTERF4-NSUN4 protein complex that regulates mitochondrial ribosome biogenesis

Spåhr, H, Habermann, B, Gustafsson, CM, Larsson, NG, Hallberg, BM

Proc. Natl. Acad. Sci. U.S.A. 2012
21531335 MTERF4 regulates translation by targeting the methyltransferase NSUN4 to the mammalian mitochondrial ribosome

Cámara, Y, Asin-Cayuela, J, Park, CB, Metodiev, MD, Shi, Y, Ruzzenente, B, Kukat, C, Habermann, B, Wibom, R, Hultenby, K, Franz, T, Erdjument-Bromage, H, Tempst, P, Hallberg, BM, Gustafsson, CM, Larsson, NG

Cell Metab. 2011
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Title
rRNA (cytosine-C5-)-methyltransferase activity of MTERF4:NSUN4 [mitochondrial matrix]
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