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

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Science  15 Sep 2000:
Vol. 289, Issue 5486, pp. 1931-1933
DOI: 10.1126/science.289.5486.1931

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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.

  • * These authors contributed equally to this work.

  • Present address: INRA, route de Saint-Cyr, Versailles 78026, France.

  • To whom correspondence should be addressed. E-mail: I.Tarassov{at}

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