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

EMBO Molecular Medicine

Volumn 6, Issue 2, 2014, Pages 155-157

  Tyynismaa, Henna ^a,b   Schon, Eric A ^c  

^a Research Programs Unit   (Finland)

^b UNIVERSITY OF HELSINKI   (Finland)

^c NewYork Presbyterian Hospital   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

AMINO ACID; MITOCHONDRIAL ENZYME; SYNTHETASE; TRANSFER RNA;

ARTICLE; ENZYME ANALYSIS; HUMAN; PRIORITY JOURNAL; RESPIRATORY TRACT DISEASE;

AMINO ACYL-TRNA SYNTHETASES; DNA, MITOCHONDRIAL; HUMANS; MITOCHONDRIA; MITOCHONDRIAL DISEASES; MUTATION; RNA, TRANSFER;

EID: 84893728705     PISSN: 17574676     EISSN: 17574684     Source Type: Journal    
DOI: 10.1002/emmm.201303586     Document Type: Article

References (14)

1. El Meziane A, Lehtinen SK, Hance N, Nijtmans LG, Dunbar D, Holt IJ, Jacobs HT (1998) A tRNA suppressor mutation in human mitochondria. Nat Genet 18: 350-353
2. Francisci S, Montanari A, De Luca C, Frontali L (2011) Peptides from aminoacyl-tRNA synthetases can cure the defects due to mutations in mt tRNA genes. Mitochondrion 11: 919-923
3. Guo M, Schimmel P (2013) Essential nontranslational functions of tRNA synthetases. Nat Chem Biol 9: 145-153
4. Hornig-Do HT, Montanari A, Rozanska A, Tuppen HA, Almalki AA, Abg-Kamaludin DP, Frontali L, Francisci S, Lightowlers RN, Chrzanowska-Lightowlers ZM (2014) Human mitochondrial leucyl tRNA synthetase can suppress non cognate pathogenic mt-tRNA mutations. EMBO Mol Med 6: 183-193
5. King MP, Attardi G (1993) Post-transcriptional regulation of the steady-state levels of mitochondrial tRNAs in HeLa cells. J Biol Chem 268: 10228-10237
6. Konovalova S, Tyynismaa H (2013) Mitochondrial aminoacyl-tRNA synthetases in human disease. Mol Genet Metab 108: 206-211
7. Labouesse M (1990) The yeast mitochondrial leucyl-tRNA synthetase is a splicing factor for the excision of several group I introns. Mol Gen Genet 224: 209-221
8. Meiklejohn CD, Holmbeck MA, Siddig MA, Abt DN, Rand DM, Montooth KL (2013) An incompatibility between a mitochondrial tRNA and its nuclear encoded tRNA synthetase compromises development and fitness in Drosophila. PLoS Genet 9: e1003238
9. Leu(UUR) gene. RNA 14: 2407-2416
10. Perli E, Giordano C, Pisano A, Montanari A, Campese AF, Reyes A, Ghezzi D, Nasca A, Tuppen HA, Orlandi M et al (2014) The isolated carboxy-terminal domain of human mitochondrial leucyl-tRNA synthetase rescues the pathological phenotype of mitochondrial tRNA mutations in human cells. EMBO Mol Med 6: 169-182
11. Ile mutation causing hypertrophic cardiomyopathy. Hum Mol Genet 21: 85-100
12. Ribas de Pouplana L, Schimmel P (2001) Two classes of tRNA synthetases suggested by sterically compatible dockings on tRNA acceptor stem. Cell 104: 191-193
13. Suzuki T, Suzuki T, Wada T, Saiqo K, Watanabe K (2002) Taurine as a constituent of mitochondrial tRNAs: new insight into the functions of taurine and human mitochondrial diseases. EMBO J 21: 6581-6589
14. Xu X, Zhang HM, Swindell EC, Marshall AG, Guo M, Kishi S, Yang XL (2012) Unique domain appended to vertebrate tRNA synthetase is essential for vascular development. Nat Commun 3: 681