title: Suppression of mutations in mitochondrial DNA by tRNAs imported from the cytoplasm.
abstract: Mitochondrial import of a cytoplasmic transfer RNA (tRNA) in yeast requires the preprotein import machinery and cytosolic factors. We investigated whether the tRNA import pathway can be used to correct respiratory deficiencies due to mutations in the mitochondrial DNA and whether this system can be transferred into human cells. We show that cytoplasmic tRNAs with altered aminoacylation identity can be specifically targeted to the mitochondria and participate in mitochondrial translation. We also show that human mitochondria, which do not normally import tRNAs, are able to internalize yeast tRNA derivatives in vitro and that this import requires an essential yeast import factor.
authors.affiliation: FRE 2168 du CNRS, Mécanismes Moléculaires de la Division Cellulaire et du Développement, 21 rue René Descartes, 67084 Strasbourg, France.
authors.forename: O A
authors.initials: OA
authors.lastname: Kolesnikova
authors.forename: N S
authors.initials: NS
authors.lastname: Entelis
authors.forename: H
authors.initials: H
authors.lastname: Mireau
authors.forename: T D
authors.initials: TD
authors.lastname: Fox
authors.forename: R P
authors.initials: RP
authors.lastname: Martin
authors.forename: I A
authors.initials: IA
authors.lastname: Tarassov
mesh_terms: D000215:Acylation; D001483:Base Sequence; D001692:Biological Transport; D003593:Cytoplasm / Q000378:metabolism; D004272:DNA, Mitochondrial / Q000235:genetics / Q000378:metabolism*; D005800:Genes, Fungal; D006801:Humans; D066298:In Vitro Techniques; D008928:Mitochondria / Q000378:metabolism*; D008969:Molecular Sequence Data; D012441:Saccharomyces cerevisiae / Q000235:genetics / Q000378:metabolism; D013489:Suppression, Genetic
chemical_list: D004272:DNA, Mitochondrial