{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,5]],"date-time":"2026-03-05T20:28:23Z","timestamp":1772742503050,"version":"3.50.1"},"reference-count":33,"publisher":"Oxford University Press (OUP)","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2006,3,15]]},"DOI":"10.1093\/hmg\/ddl007","type":"journal-article","created":{"date-parts":[[2006,1,31]],"date-time":"2006-01-31T01:33:04Z","timestamp":1138671184000},"page":"897-904","source":"Crossref","is-referenced-by-count":33,"title":["Acquisition of the wobble modification in mitochondrial tRNALeu(CUN) bearing the G12300A mutation suppresses the MELAS molecular defect"],"prefix":"10.1093","volume":"15","author":[{"given":"Yohei","family":"Kirino","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takehiro","family":"Yasukawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sanna K.","family":"Marjavaara","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Howard T.","family":"Jacobs","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ian J.","family":"Holt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kimitsuna","family":"Watanabe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tsutomu","family":"Suzuki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2006,1,30]]},"reference":[{"key":"key\n\t\t\t\t20171012074728_DDL007C1","unstructured":"Schon, E.A., Bonilla, E. and DiMauro, S. (1997) Mitochondrial DNA mutations and pathogenesis. J. Bioenerg. Biomembr., 29, 131\u2013149."},{"key":"key\n\t\t\t\t20171012074728_DDL007C2","unstructured":"Brandon, M.C., Lott, M.T., Nguyen, K.C., Spolim, S., Navathe, S.B., Baldi, P. and Wallace, D.C. (2005) MITOMAP: a human mitochondrial genome database\u20142004 update. Nucleic Acids Res., 33, D611\u2013D613."},{"key":"key\n\t\t\t\t20171012074728_DDL007C3","doi-asserted-by":"crossref","unstructured":"Jacobs, H.T. (2003) Disorders of mitochondrial protein synthesis. Hum. Mol. Genet., 12, R293\u2013R301. Epub 2003 August 19.","DOI":"10.1093\/hmg\/ddg285"},{"key":"key\n\t\t\t\t20171012074728_DDL007C4","unstructured":"Levinger, L., Morl, M. and Florentz, C. (2004) Mitochondrial tRNA 3\u2032 end metabolism and human disease. Nucleic Acids Res., 32, 5430\u20135441."},{"key":"key\n\t\t\t\t20171012074728_DDL007C5","unstructured":"Kirino, Y. and Suzuki, T. (2004) Human mitochondrial diseases associated with tRNA wobble modification deficiency. RNA Biol., 1, 145\u2013148."},{"key":"key\n\t\t\t\t20171012074728_DDL007C6","doi-asserted-by":"crossref","unstructured":"Yasukawa, T., Suzuki, T., Ohta, S. and Watanabe, K. (2002) Wobble modification defect suppresses translational activity of tRNAs with MERRF and MELAS mutations. Mitochondrion, 2, 129\u2013141.","DOI":"10.1016\/S1567-7249(02)00033-8"},{"key":"key\n\t\t\t\t20171012074728_DDL007C7","doi-asserted-by":"crossref","unstructured":"Goto, Y., Nonaka, I. and Horai, S. (1990) A mutation in the tRNA(Leu)(UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies. Nature, 348, 651\u2013653.","DOI":"10.1038\/348651a0"},{"key":"key\n\t\t\t\t20171012074728_DDL007C8","unstructured":"Kobayashi, Y., Momoi, M.Y., Tominaga, K., Momoi, T., Nihei, K., Yanagisawa, M., Kagawa, Y. and Ohta, S. (1990) A point mutation in the mitochondrial tRNA(Leu)(UUR) gene in MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes). Biochem. Biophys. Res. Commun., 173, 816\u2013822."},{"key":"key\n\t\t\t\t20171012074728_DDL007C9","unstructured":"Goto, Y., Nonaka, I. and Horai, S. (1991) A new mtDNA mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS). Biochim. Biophys. Acta, 1097, 238\u2013240."},{"key":"key\n\t\t\t\t20171012074728_DDL007C10","doi-asserted-by":"crossref","unstructured":"Suzuki, T., Suzuki, T., Wada, T., Saigo, K. and Watanabe, K. (2002) Taurine as a constituent of mitochondrial tRNAs: new insights into the functions of taurine and human mitochondrial diseases. EMBO J., 21, 6581\u20136589.","DOI":"10.1093\/emboj\/cdf656"},{"key":"key\n\t\t\t\t20171012074728_DDL007C11","unstructured":"Yasukawa, T., Suzuki, T., Suzuki, T., Ueda, T., Ohta, S. and Watanabe, K. (2000) Modification defect at anticodon wobble nucleotide of mitochondrial tRNAs(Leu)(UUR) with pathogenic mutations of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. J. Biol. Chem., 275, 4251\u20134257."},{"key":"key\n\t\t\t\t20171012074728_DDL007C12","doi-asserted-by":"crossref","unstructured":"Kirino, Y., Goto, Y., Campos, Y., Arenas, J. and Suzuki, T. (2005) Specific correlation between the wobble modification deficiency in mutant tRNAs and the clinical features of a human mitochondrial disease. Proc. Natl Acad. Sci. USA, 102, 7127\u20137132.","DOI":"10.1073\/pnas.0500563102"},{"key":"key\n\t\t\t\t20171012074728_DDL007C13","doi-asserted-by":"crossref","unstructured":"Suzuki, T. (2005) Biosynthesis and function of tRNA wobble modifications. Topics in Current Genetics. Springer-Verlag, NY, Vol. 12, pp. 24\u201369.","DOI":"10.1007\/b106361"},{"key":"key\n\t\t\t\t20171012074728_DDL007C14","doi-asserted-by":"crossref","unstructured":"Yokoyama, S. and Nishimura, S. (1995) Modified nucleosides and codon recognition. In S\u00f6ll, D. and RajBhandary, U.L. (eds), tRNA: Structure, Biosynthesis, and Function. American Society for Microbiology, Washington, D.C., pp. 207\u2013224.","DOI":"10.1128\/9781555818333.ch12"},{"key":"key\n\t\t\t\t20171012074728_DDL007C15","doi-asserted-by":"crossref","unstructured":"Bjork, G. (1995) Biosynthesis and function of modified nucleosides. In S\u00f6ll, D. and RajBhandary, U.L. (eds), tRNA: Structure, Biosynthesis, and Function. American Society for Microbiology, Washington, D.C., pp. 165\u2013205.","DOI":"10.1128\/9781555818333.ch11"},{"key":"key\n\t\t\t\t20171012074728_DDL007C16","doi-asserted-by":"crossref","unstructured":"Kirino, Y., Yasukawa, T., Ohta, S., Akira, S., Ishihara, K., Watanabe, K. and Suzuki, T. (2004) Codon-specific translational defect caused by a wobble modification deficiency in mutant tRNA from a human mitochondrial disease. Proc. Natl Acad. Sci. USA, 101, 15070\u201315075.","DOI":"10.1073\/pnas.0405173101"},{"key":"key\n\t\t\t\t20171012074728_DDL007C17","unstructured":"El Meziane, A., Lehtinen, S.K., Hance, N., Nijtmans, L.G., Dunbar, D., Holt, I.J. and Jacobs, H.T. (1998) A tRNA suppressor mutation in human mitochondria. Nat. Genet., 18, 350\u2013353."},{"key":"key\n\t\t\t\t20171012074728_DDL007C18","doi-asserted-by":"crossref","unstructured":"Lehtinen, S.K., Hance, N., El Meziane, A., Juhola, M.K., Juhola, K.M., Karhu, R., Spelbrink, J.N., Holt, I.J. and Jacobs, H.T. (2000) Genotypic stability, segregation and selection in heteroplasmic human cell lines containing np 3243 mutant mtDNA. Genetics, 154, 363\u2013380.","DOI":"10.1093\/genetics\/154.1.363"},{"key":"key\n\t\t\t\t20171012074728_DDL007C19","doi-asserted-by":"crossref","unstructured":"Watanabe, K. and Osawa, S. (1995) tRNA sequences and variations in the genetic code. In S\u00f6ll, D. and RajBhandary, U.L. (eds), tRNA: Structure, Biosynthesis, and Function. American Society for Microbiology, Washington, D.C., pp. 225\u2013250.","DOI":"10.1128\/9781555818333.ch13"},{"key":"key\n\t\t\t\t20171012074728_DDL007C20","unstructured":"Donis-Keller, H. (1980) Phy M: an RNase activity specific for U and A residues useful in RNA sequence analysis. Nucleic Acids Res., 8, 3133\u20133142."},{"key":"key\n\t\t\t\t20171012074728_DDL007C21","unstructured":"Kuchino, Y., Hanyu, N. and Nishimura, S. (1987) Analysis of modified nucleosides and nucleotide sequence of tRNA. Methods Enzymol., 155, 379\u2013396."},{"key":"key\n\t\t\t\t20171012074728_DDL007C22","doi-asserted-by":"crossref","unstructured":"Kaneko, T., Suzuki, T., Kapushoc, S.T., Rubio, M.A., Ghazvini, J., Watanabe, K., Simpson, L. and Suzuki, T. (2003) Wobble modification differences and subcellular localization of tRNAs in Leishmania tarentolae: implication for tRNA sorting mechanism. EMBO J., 22, 657\u2013667.","DOI":"10.1093\/emboj\/cdg066"},{"key":"key\n\t\t\t\t20171012074728_DDL007C23","unstructured":"Soma, A., Ikeuchi, Y., Kanemasa, S., Kobayashi, K., Ogasawara, N., Ote, T., Kato, J., Watanabe, K., Sekine, Y. and Suzuki, T. (2003) An RNA-modifying enzyme that governs both the codon and amino acid specificities of isoleucine tRNA. Mol. Cell, 12, 689\u2013698."},{"key":"key\n\t\t\t\t20171012074728_DDL007C24","doi-asserted-by":"crossref","unstructured":"Hanada, T., Suzuki, T., Yokogawa, T., Takemoto-Hori, C., Sprinzl, M. and Watanabe, K. (2001) Translation ability of mitochondrial tRNAsSer with unusual secondary structures in an in vitro translation system of bovine mitochondria. Genes Cells, 6, 1019\u20131030.","DOI":"10.1046\/j.1365-2443.2001.00491.x"},{"key":"key\n\t\t\t\t20171012074728_DDL007C25","unstructured":"Yasukawa, T., Suzuki, T., Ishii, N., Ohta, S. and Watanabe, K. (2001) Wobble modification defect in tRNA disturbs codon\u2013anticodon interaction in a mitochondrial disease. EMBO J., 20, 4794\u20134802."},{"key":"key\n\t\t\t\t20171012074728_DDL007C26","unstructured":"King, M.P. and Attardi, G. (1993) Post-transcriptional regulation of the steady-state levels of mitochondrial tRNAs in HeLa cells. J. Biol. Chem., 268, 10228\u201310237."},{"key":"key\n\t\t\t\t20171012074728_DDL007C27","unstructured":"Elseviers, D., Petrullo, L.A. and Gallagher, P.J. (1984) Novel E. coli mutants deficient in biosynthesis of 5-methylaminomethyl-2-thiouridine. Nucleic Acids Res., 12, 3521\u20133534."},{"key":"key\n\t\t\t\t20171012074728_DDL007C28","doi-asserted-by":"crossref","unstructured":"Nakayashiki, T. and Inokuchi, H. (1998) Novel temperature-sensitive mutants of Escherichia coli that are unable to grow in the absence of wild-type tRNA6Leu. J. Bacteriol., 180, 2931\u20132935.","DOI":"10.1128\/JB.180.11.2931-2935.1998"},{"key":"key\n\t\t\t\t20171012074728_DDL007C29","doi-asserted-by":"crossref","unstructured":"Bregeon, D., Colot, V., Radman, M. and Taddei, F. (2001) Translational misreading: a tRNA modification counteracts a +2 ribosomal frameshift. Genes Dev., 15, 2295\u20132306.","DOI":"10.1101\/gad.207701"},{"key":"key\n\t\t\t\t20171012074728_DDL007C30","unstructured":"Umeda, N., Suzuki, T., Yukawa, M., Ohya, Y., Shindo, H., Watanabe, K. and Suzuki, T. (2005) Mitochondria-specific RNA-modifying enzymes responsible for the biosynthesis of the wobble base in mitochondrial tRNAs. Implications for the molecular pathogenesis of human mitochondrial diseases. J. Biol. Chem., 280, 1613\u20131624."},{"key":"key\n\t\t\t\t20171012074728_DDL007C31","unstructured":"Li, R., Li, X., Yan, Q., Qin Mo, J. and Guan, M.X. (2003) Identification and characterization of mouse MTO1 gene related to mitochondrial tRNA modification. Biochim. Biophys. Acta, 1629, 53\u201359."},{"key":"key\n\t\t\t\t20171012074728_DDL007C32","unstructured":"Colby, G., Wu, M. and Tzagoloff, A. (1998) MTO1 codes for a mitochondrial protein required for respiration in paromomycin-resistant mutants of Saccharomyces cerevisiae. J. Biol. Chem., 273, 27945\u201327952."},{"key":"key\n\t\t\t\t20171012074728_DDL007C33","unstructured":"Sprinzl, M., Horn, C., Brown, M., Ioudovitch, A. and Steinberg, S. (1998) Compilation of tRNA sequences and sequences of tRNA genes. Nucleic Acids Res., 26, 148\u2013153."}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/15\/6\/897\/17243184\/ddl007.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,7,23]],"date-time":"2021-07-23T09:55:47Z","timestamp":1627034147000},"score":1,"resource":{"primary":{"URL":"http:\/\/academic.oup.com\/hmg\/article\/15\/6\/897\/582342\/Acquisition-of-the-wobble-modification-in"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,1,30]]},"references-count":33,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2006,1,30]]},"published-print":{"date-parts":[[2006,3,15]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddl007","relation":{},"ISSN":["1460-2083","0964-6906"],"issn-type":[{"value":"1460-2083","type":"electronic"},{"value":"0964-6906","type":"print"}],"subject":[],"published-other":{"date-parts":[[2006,3,15]]},"published":{"date-parts":[[2006,1,30]]}}}