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Mitochondrion. 2011 Nov;11(6):919-23. doi: 10.1016/j.mito.2011.08.006. Epub 2011 Aug 31.

Peptides from aminoacyl-tRNA synthetases can cure the defects due to mutations in mt tRNA genes.

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  • 1Department of Biology and Biotechnologies Charles Darwin, Pasteur Institute-Cenci Bolognetti Foundation and Sapienza University of Rome, Piazzale A. Moro 5, 00185 Rome, Italy.


Recent results from several laboratories have confirmed that human and yeast leucyl- and valyl-tRNA synthetases can rescue the respiratory defects due to mutations in mitochondrial tRNA genes. In this report we show that this effect cannot be ascribed to the catalytic activity per se and that isolated domains of aminoacyl-tRNA synthetases and even short peptides thereof have suppressing effects.

Copyright © 2011 Elsevier B.V. and Mitochondria Research Society. All rights reserved. All rights reserved.

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