2005
DOI: 10.1074/jbc.m504247200
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Mutations in MTO2 Related to tRNA Modification Impair Mitochondrial Gene Expression and Protein Synthesis in the Presence of a Paromomycin Resistance Mutation in Mitochondrial 15 S rRNA

Abstract: Nuclear gene(s) have been shown to modulate the phenotypic expression of mitochondrial DNA mutations. We report here the identification and characterization of the yeast nuclear gene MTO2 encoding an evolutionarily conserved protein involved in mitochondrial tRNA modification. Interestingly, mto2 null mutants expressed a respiratory-deficient phenotype when coexisting with the C1409G mutation of mitochondrial 15 S rRNA at the very conservative site for human deafness-associated 12 S rRNA A1491G and C1409T muta… Show more

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Cited by 44 publications
(75 citation statements)
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“…A typical mitochondrial staining pattern was observed with both antibodies, and superimposition of two panels showed complete overlap of the two patterns, demonstrating that human LARS2 localizes exclusively to mitochondria. This suggested that this protein, like other nucleus-encoding mitochondrial proteins such as MTO1, GTPBP3, and TRMU (29,30,49), was synthesized on cytoplasmic ribosomes and was imported into and then functioned in the mitochondrion.…”
Section: Resultsmentioning
confidence: 99%
See 1 more Smart Citation
“…A typical mitochondrial staining pattern was observed with both antibodies, and superimposition of two panels showed complete overlap of the two patterns, demonstrating that human LARS2 localizes exclusively to mitochondria. This suggested that this protein, like other nucleus-encoding mitochondrial proteins such as MTO1, GTPBP3, and TRMU (29,30,49), was synthesized on cytoplasmic ribosomes and was imported into and then functioned in the mitochondrion.…”
Section: Resultsmentioning
confidence: 99%
“…Resultant constructs were transfected into 143B cells using the SuperFect transfection reagent (Qiagen, Inc.) according to the manufacturer's protocol. Immunofluorescence analysis was performed as detailed elsewhere (29,30,49).…”
Section: Cell Lines and Culture Conditions 143btkmentioning
confidence: 99%
“…101,102 Proteins TRMU, MTO1 and MSS1 are required for splicing, likely because they are needed for the translation of maturases. 99,100,103,104 Strikingly, effects of MTO1 inactivation are more severe than those of MSS1 inactivation on splicing and accumulation of certain mttRNAs, 100 which might explain why mitochondrial translation is affected more in MTO1 than in MSS1 mutants. These observations suggest that MTO1 and MSS1, apart from their shared role in mttRNA modification, may play independent roles in other cellular functions.…”
Section: Glumentioning
confidence: 99%
“…Therefore, the function of the GidA, MnmE, and MnmA family proteins seems to be evolutionarily conserved. Human homologues have also been identified and been found to localize in mitochondria (23,25,46,49,50). It should be noted, however, that in humans taurine is incorporated into mitochondrial tRNA Lys and tRNA Leu(UUR) in place of cmnm (44).…”
mentioning
confidence: 99%
“…In yeast, the protein products of the nuclear genes MSS1, MTO1, and MTU1 (homologues of mnmE, gidA, and mnmA, respectively) localize in mitochondria, and their mutants are defective in modification of mitochondrial tRNA (8,9,46,49). Thus, mitochondrial tRNA Lys molecules isolated from yeast MTO1 and MSS1 deletion strains contain s 2 U instead of the cmnm 5 s 2 U found in the wild-type tRNA Lys molecules, whereas disruption of the MTU1 gene eliminates the 2-thio modification of mitochondrial tRNAs (Fig.…”
mentioning
confidence: 99%