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ReportsFunctional Delivery of a Cytosolic tRNA into Mutant Mitochondria of Human Cells
Many maternally inherited and incurable neuromyopathies are caused by mutations in mitochondrial (mt) transfer RNA (tRNA) genes. Kinetoplastid protozoa, including Leishmania, have evolved specialized systems for importing nucleus-encoded tRNAs into mitochondria. We found that the Leishmania RNA import complex (RIC) could enter human cells by a caveolin-1dependent pathway, where it induced import of endogenous cytosolic tRNAs, including tRNALys, and restored mitochondrial function in a cybrid harboring a mutant mt tRNALys (MT-TK) gene. The use of protein complexes to modulate mitochondrial function may help in the management of such genetic disorders.
1 Genetic Engineering, Indian Institute of Chemical Biology, 4 Raja S. C. Mullick Road, Calcutta 700032, India.
2 Molecular Endocrinology, Indian Institute of Chemical Biology, 4 Raja S. C. Mullick Road, Calcutta 700032, India. * To whom correspondence should be addressed. E-mail: sadhya{at}iicb.res.in
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Science. ISSN 0036-8075 (print), 1095-9203 (online)