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  • Title: Functional delivery of a cytosolic tRNA into mutant mitochondria of human cells.
    Author: Mahata B, Mukherjee S, Mishra S, Bandyopadhyay A, Adhya S.
    Journal: Science; 2006 Oct 20; 314(5798):471-4. PubMed ID: 17053148.
    Abstract:
    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-1-dependent pathway, where it induced import of endogenous cytosolic tRNAs, including tRNA(Lys), and restored mitochondrial function in a cybrid harboring a mutant mt tRNA(Lys) (MT-TK) gene. The use of protein complexes to modulate mitochondrial function may help in the management of such genetic disorders.
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