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J. Biol. Chem., Vol. 280, Issue 7, 5141-5144, February 18, 2005

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

Bidesh Mahata, Suvendra Nath Bhattacharyya, Saikat Mukherjee, and Samit Adhya

From the Genetic Engineering Laboratory, Indian Institute of Chemical Biology, 4 Raja S. C. Mullick Road, Calcutta 7000032, India

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 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 ragged 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.

Received for publication, December 10, 2004 , and in revised form, December 23, 2004.

^* This work was supported by a grant from the Department of Science and Technology, Government of India, and by Research Fellowships from the Council of Scientific and Industrial Research awarded to B. M., S. N. B., and S. M.). The costs of publication of this article were defrayed in part by the payment of page charges. This article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

Present address: Friedrich Miescher Inst. for Biomedical Research, Novartis Research Foundation, Maulbeerstr. 66, 4058 Basel, Switzerland.

To whom correspondence should be addressed. Tel.: 91-33-2473-3491 (ext. 136); Fax: 91-33-2472-3967; E-mail: sadhya{at}iicb.res.in.

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