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Methylation of 12S rRNA is necessary for in vivo stability of the small subunit of the mammalian mitochondrial ribosome.

Authors
Metodiev, MD; Lesko, N; Park, CB; Cámara, Y; Shi, Y; Wibom, R; Hultenby, K; Gustafsson, CM; Larsson, NG
Citation
Cell metabolism, 9(4):386-397, 2009
Journal Title
Cell metabolism
ISSN
1550-41311932-7420
Abstract
The 3' end of the rRNA of the small ribosomal subunit contains two extremely highly conserved dimethylated adenines. This modification and the responsible methyltransferases are present in all three domains of life, but its function has remained elusive. We have disrupted the mouse Tfb1m gene encoding a mitochondrial protein homologous to bacterial dimethyltransferases and demonstrate here that loss of TFB1M is embryonic lethal. Disruption of Tfb1m in heart leads to complete loss of adenine dimethylation of the rRNA of the small mitochondrial ribosomal subunit, impaired assembly of the mitochondrial ribosome, and abolished mitochondrial translation. In addition, we present biochemical evidence that TFB1M does not activate or repress transcription in the presence of TFB2M. Our results thus show that TFB1M is a nonredundant dimethyltransferase in mammalian mitochondria. In addition, we provide a possible explanation for the universal conservation of adenine dimethylation of rRNA by showing a critical role in ribosome maintenance.
MeSH terms
AnimalsCardiomyopathies/metabolismCardiomyopathies/pathologyDNA, Mitochondrial/geneticsElectron TransportEmbryonic DevelopmentGene Expression Regulation, DevelopmentalMammals/metabolism*MethylationMiceMice, KnockoutMitochondria/geneticsMitochondria/metabolism*Mitochondria/ultrastructureMyocardium/pathologyMyocardium/ultrastructureOrgan SpecificityProtein BiosynthesisRNA, Messenger/geneticsRNA, Messenger/metabolismRNA, Ribosomal/metabolism*Ribosome Subunits, Small, Eukaryotic/metabolism*Transcription Factors/deficiencyTranscription Factors/geneticsTranscription Factors/metabolismTranscription, Genetic
DOI
10.1016/j.cmet.2009.03.001
PMID
19356719
Appears in Collections:
Journal Papers > School of Medicine / Graduate School of Medicine > Physiology
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