Correction of Translational Defects in Patient-derived Mutant Mitochondria by Complex-mediated Import of a Cytoplasmic tRNA

Bidesh Mahata, Suvendra Nath Bhattacharyya, Saikat Mukherjee, Samit Adhya
2004 Journal of Biological Chemistry  
A variety of clinical disorders result from mutations in mitochondrial tRNA genes, leading to translational defects. We show here that a protein complex from the kinetoplastid protozoon Leishmania induces specific, ATP-dependent import of human cytoplasmic tRNA 1 Lys into human mitochondria in vitro. The imported tRNA undergoes efficient aminoacylation within the organelle and supports organellar protein synthesis. Moreover, translation in mitochondria from patients with myclonic epilepsy with
more » ... agged red fibers (MERRF) and Kearns-Sayre syndrome (KSS), containing mutant tRNA Lys genes, is stimulated to near-wild-type levels and the formation of aberrant polypeptides suppressed by complex-mediated import. These results suggest a novel way to introduce RNAs for the modulation of mitochondrial gene expression.
doi:10.1074/jbc.c400572200 pmid:15619607 fatcat:fgqcezw4onayjkejpzozp6zyzi