Functional 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 impo...

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Bibliographic Details
Published in:Science (American Association for the Advancement of Science) 2006-10, Vol.314 (5798), p.471-474
Main Authors: Mahata, Bidesh, Mukherjee, Saikat, Mishra, Sumita, Bandyopadhyay, Arun, Adhya, Samit
Format: Article
Language:English
Subjects:
Animals
Biological and medical sciences
Caveolin 1 - metabolism
Cell growth
Cell Line, Tumor
Cell lines
Cell Proliferation
Cell Respiration
Cells, Cultured
Cultured cells
Cytosol - metabolism
Electron Transport Complex IV - metabolism
Endocytosis
General aspects
Genetic disorders
Genetic engineering
Genetic mutation
Hep G2 cells
Humans
Imports
Kearns-Sayre Syndrome - metabolism
Leishmania
Leishmania tropica
Medical sciences
MERRF Syndrome - metabolism
Mitochondria
Mitochondria - genetics
Mitochondria - metabolism
Mitochondrial Proteins - metabolism
Mutation
Neurological disorders
Oxygen Consumption
Protein Biosynthesis
Proteins
Protozoan Proteins - metabolism
Reverse transcriptase polymerase chain reaction
Ribonucleic acid
RNA
RNA, Transfer, Lys - genetics
RNA, Transfer, Lys - metabolism
RNA-Binding Proteins - metabolism
Transfection
Transfer RNA
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Summary: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 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.
ISSN:0036-8075
1095-9203
DOI:10.1126/science.1129754