Functional Delivery of a Cytosolic tRNA into Mutant Mitochondria of Human Cells
- 20 October 2006
- journal article
- other
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 314 (5798) , 471-474
- https://doi.org/10.1126/science.1129754
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 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.Keywords
This publication has 23 references indexed in Scilit:
- A bifunctional tRNA import receptor from Leishmania mitochondriaProceedings of the National Academy of Sciences, 2006
- Correction of Translational Defects in Patient-derived Mutant Mitochondria by Complex-mediated Import of a Cytoplasmic tRNAJournal of Biological Chemistry, 2005
- Nuclear DNA-encoded tRNAs targeted into mitochondria can rescue a mitochondrial DNA mutation associated with the MERRF syndrome in cultured human cellsHuman Molecular Genetics, 2004
- Clathrin-mediated endocytosis in AP-2–depleted cellsThe Journal of cell biology, 2003
- “Ping-Pong” Interactions between Mitochondrial tRNA Import Receptors within a Multiprotein ComplexMolecular and Cellular Biology, 2003
- Analysis of mitochondrial morphology and function with novel fixable fluorescent stains.Journal of Histochemistry & Cytochemistry, 1996
- Mitochondrial diabetes mellitus: a reviewBiochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 1995
- MtDNA mutation in MERRF syndrome causes defective aminoacylation of tRNALys and premature translation terminationNature Genetics, 1995
- Clinical features of melas and mitochondrial DNA mutationsMuscle & Nerve, 1995
- Myoclonic epilepsy and ragged-red fiber disease (MERRF) is associated with a mitochondrial DNA tRNALys mutationCell, 1990