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Review
. 2014 Feb;6(2):155-7.
doi: 10.1002/emmm.201303586. Epub 2014 Jan 28.

Mixing and matching mitochondrial aminoacyl synthetases and their tRNAs: a new way to treat respiratory chain disorders?

Henna Tyynismaa  1 , Eric A Schon
Affiliations
Review

Mixing and matching mitochondrial aminoacyl synthetases and their tRNAs: a new way to treat respiratory chain disorders?

Henna Tyynismaa et al. EMBO Mol Med. 2014 Feb.

Abstract

Mutations in mitochondrial DNA are an important cause of human disease and from a therapeutic standpoint, these disorders are currently untreatable. New studies now show that a non‐cognate mitochondrial aminoacyl tRNA synthetase can overcome the respiratory defect caused by an mt‐tRNA mutation and that the isolated carboxy‐terminal domain of human mt‐leucyl tRNA synthetase can ameliorate the pathologic phenotype.

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Figures

Figure 1
Figure 1
The human mitochondrial DNA 16.6-kb genome encodes 7 subunits of complex I (ND1, 2, 3, 4L, 4, 5, and 6), 1 subunit of complex III (Cyt b), 3 subunits of complex IV (COX1, 2, and 3), and 2 subunits of complex V (A6 and 8), as well as 2 rRNAs (12S and 16S) and 22 tRNAs (3-letter notation).
See this image and copyright information in PMC

References

    1. El Meziane A, Lehtinen SK, Hance N, Nijtmans LG, Dunbar D, Holt IJ, Jacobs HT. A tRNA suppressor mutation in human mitochondria. Nat Genet. 1998;18:350–353. - PubMed
    1. Francisci S, Montanari A, De Luca C, Frontali L. Peptides from aminoacyl-tRNA synthetases can cure the defects due to mutations in mt tRNA genes. Mitochondrion. 2011;11:919–923. - PMC - PubMed
    1. Guo M, Schimmel P. Essential nontranslational functions of tRNA synthetases. Nat Chem Biol. 2013;9:145–153. - PMC - PubMed
    1. Hornig-Do HT, Montanari A, Rozanska A, Tuppen HA, Almalki AA, Abg-Kamaludin DP, Frontali L, Francisci S, Lightowlers RN, Chrzanowska-Lightowlers ZM. Human mitochondrial leucyl tRNA synthetase can suppress non cognate pathogenic mt-tRNA mutations. EMBO Mol Med. 2014;6:183–193. - PMC - PubMed
    1. King MP, Attardi G. Post-transcriptional regulation of the steady-state levels of mitochondrial tRNAs in HeLa cells. J Biol Chem. 1993;268:10228–10237. - PubMed

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