title: Mutations in yeast mt tRNAs: specific and general suppression by nuclear encoded tRNA interactors.
abstract: Mutations in mitochondrial tRNA genes can produce alterations in tRNA structure resulting in defective mitochondrial protein synthesis and hence respiratory defects. Such defects are often at the origin of neurodegenerative diseases in humans and can be easily studied in yeast since respiratory deficient mutants are viable. Several nuclear encoded tRNA interactors have been shown to rescue the mitochondrial defects due to mutations in mitochondrial tRNAs. Among these, we have identified the gene for the mitochondrial protein synthesis elongation factor EF-Tu and the specific mt aminoacyl-tRNA synthetases. We also observed that the respiratory defects and the effect of the TUF1 over-expression were strongly strain dependent. The importance of the nuclear background in which the mitochondrial mutation is expressed was investigated by changing the nuclear context. Finally, we demonstrated, using the RT-PCR method, the existence of significantly variable levels of the TUF1 transcript among strains with functional and dysfunctional mitochondria.
authors.affiliation: Department of Cell and Developmental Biology, Pasteur Institute-Cenci Bolognetti Foundation, University of Rome I, Italy.
authors.forename: C
authors.initials: C
authors.lastname: De Luca
authors.forename: C
authors.initials: C
authors.lastname: Besagni
authors.forename: L
authors.initials: L
authors.lastname: Frontali
authors.forename: M
authors.initials: M
authors.lastname: Bolotin-Fukuhara
authors.forename: S
authors.initials: S
authors.lastname: Francisci
mesh_terms: D000604:Amino Acyl-tRNA Synthetases / Q000235:genetics; D001483:Base Sequence; D004271:DNA, Fungal / Q000235:genetics; D005800:Genes, Fungal; D009154:Mutation; D010444:Peptide Elongation Factor Tu / Q000235:genetics; D010641:Phenotype; D012313:RNA / Q000235:genetics*; D012331:RNA, Fungal / Q000235:genetics*; D000077278:RNA, Mitochondrial; D012343:RNA, Transfer / Q000235:genetics*; D012441:Saccharomyces cerevisiae / Q000235:genetics*; D013489:Suppression, Genetic; D014157:Transcription Factors / Q000235:genetics; D014158:Transcription, Genetic
chemical_list: D004271:DNA, Fungal; D012331:RNA, Fungal; D000077278:RNA, Mitochondrial; D014157:Transcription Factors; D012313:RNA; D012343:RNA, Transfer; D010444:Peptide Elongation Factor Tu; D000604:Amino Acyl-tRNA Synthetases