Suppression of Mutations in Mitochondrial DNA by tRNAs Imported from the Cytoplasm

• Published in: Science (American Association for the Advancement of Science) Vol. 289; no. 5486; pp. 1931 - 1933
• Main Authors: Kolesnikova, O. A., Entelis, N. S., Mireau, H., Fox, T. D., Martin, R. P., Tarassov, I. A.
• Format: Journal Article
• Language: English
• Published: United States American Society for the Advancement of Science 15-09-2000; American Association for the Advancement of Science; The American Association for the Advancement of Science; American Association for the Advancement of Science (AAAS)
• Subjects: Acylation; Base Sequence; Biological Transport; Cell culture techniques; Cellular Biology; Coding; Codon; Codons; Cytoplasm - metabolism; Deoxyribonucleic acid; DNA; DNA, Mitochondrial - genetics; DNA, Mitochondrial - metabolism; Genes, Fungal; Genetic mutation; Hep G2 cells; Humans; Imports; In Vitro Techniques; Life Sciences; Mitochondria; Mitochondria - metabolism; Mitochondrial DNA; Mitochondrial membrane; Mitochondrial membranes; Molecular Sequence Data; Mutation; Prostaglandins; Ribonucleic acid; RNA; Saccharomyces cerevisiae; Saccharomyces cerevisiae - genetics; Saccharomyces cerevisiae - metabolism; Suppression, Genetic; Transfer RNA; Translation; tRNA import; Yeast; Yeasts; United States; ARNT
• ISSN: 0036-8075; 1095-9203
• DOI: 10.1126/science.289.5486.1931

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.