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.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Animals
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Caveolin 1 / metabolism
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Cell Line, Tumor
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Cell Proliferation
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Cell Respiration
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Cells, Cultured
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Cytosol / metabolism*
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Electron Transport Complex IV / metabolism
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Endocytosis
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Humans
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Kearns-Sayre Syndrome / metabolism
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Leishmania tropica*
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MERRF Syndrome / metabolism
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Mitochondria / genetics
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Mitochondria / metabolism*
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Mitochondrial Proteins / metabolism*
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Mutation
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Oxygen Consumption
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Protein Biosynthesis
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Protozoan Proteins / metabolism*
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RNA, Transfer, Lys / genetics
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RNA, Transfer, Lys / metabolism*
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RNA-Binding Proteins / metabolism*
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Transfection
Substances
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Caveolin 1
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Mitochondrial Proteins
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Protozoan Proteins
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RNA, Transfer, Lys
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RNA-Binding Proteins
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Electron Transport Complex IV