Peptides from aminoacyl-tRNA synthetases can cure the defects due to mutations in mt tRNA genes

► We studied the rescuing of human equivalent yeast mutants in mttRNALeu, Val and Ile. ► Thecarboxy-terminal of human and yeast mtleuRShas full suppressing activity. ► The overexpressed human C-terminal domain isfound to be localizedin mitochondria. ► Smaller peptides from C-terminal maintain rescuing capability. ► Suppression of defective phenotypes could be due to an RNA stabilizing capability.