Nucleic Acids Research · 2013 · 57 citations · 39 references
MitophagyRespiratory Complex FormationGtpase ActivityMolecular BiologyMitochondrial BiologyProtein SynthesisMitochondrial BiogenesisProtein FoldingHuman G-proteinsLarge Ribosomal SubunitsProteomicsBiochemistryRespiratory ComplexesCell BiologySignal TransductionMitochondrial FunctionNatural SciencesCellular BiochemistryMedicine
The bacterial homologues of ObgH1 and Mtg1, ObgE and RbgA, respectively, have been suggested to be involved in the assembly of large ribosomal subunits. We sought to elucidate the functions of ObgH1 and Mtg1 in ribosome biogenesis in human mitochondria. ObgH1 and Mtg1 are localized in mitochondria in association with the inner membrane, and are exposed on the matrix side. Mtg1 and ObgH1 specifically associate with the large subunit of the mitochondrial ribosome in GTP-dependent manner. The large ribosomal subunit stimulated the GTPase activity of Mtg1, whereas only the intrinsic GTPase activity was detectable with ObgH1. The knockdown of Mtg1 decreased the overall mitochondrial translation activity, and caused defects in the formation of respiratory complexes. On the other hand, the depletion of ObgH1 led to the specific activation of the translation of subunits of Complex V, and disrupted its proper formation. Our results suggested that Mtg1 and ObgH1 function with the large subunit of the mitochondrial ribosome, and are also involved in both the translation and assembly of respiratory complexes. The fine coordination of ribosome assembly, translation and respiratory complex formation in mammalian mitochondria is affirmed.
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Sequence and organization of the human mitochondrial genome
Stephen K. Anderson, Alan T. Bankier, B. G. Barrell et al. · Nature · 1981 · 9.6K citations
Defective mitochondrial translation caused by a ribosomal protein (MRPS16) mutation
Chaya Miller, Ann Saada, Nava Shaul et al. · Annals of Neurology · 2004 · 228 citations