このエントリーをはてなブックマークに追加
ID 48781
本文ファイル
著者
Umeda, Noriko
Suzuki, Takeo
Ohya, Yoshikazu
Shindo, Heisaburo
Watanabe, Kimitsuna
Suzuki, Tsutomu
抄録(英)
Human mitochondrial (mt) tRNALys has a taurine-containing modified uridine, 5-taurinomethyl-2-thiouridine (τm5s2U), at its anticodon wobble position. We previously found that the mt tRNALys, carrying the A8344G mutation from cells of patients with myoclonus epilepsy associated with ragged-red fibers (MERRF), lacks the τm5s2U modification. Here we describe the identification and characterization of a tRNA-modifying enzyme MTU1 (mitochondrial tRNA-specific 2-thiouridylase 1) that is responsible for the 2-thiolation of the wobble position in human and yeast mt tRNAs. Disruption of the yeast MTU1 gene eliminated the 2-thio modification of mt tRNAs and impaired mitochondrial protein synthesis, which led to reduced respiratory activity. Furthermore, when MTO1 or MSS1, which are responsible for the C5 substituent of the modified uridine, was disrupted along with MTU1, a much more severe reduction in mitochondrial activity was observed. Thus, the C5 and 2-thio modifications act synergistically in promoting efficient cognate codon decoding. Partial inactivation of MTU1 in HeLa cells by small interference RNA also reduced their oxygen consumption and resulted in mitochondria with defective membrane potentials, which are similar phenotypic features observed in MERRF.
内容記述
This work was supported by grants-in-aid for scientific research on priority areas from the Ministry of Education, Science, Sports, and Culture of Japan, and by a grant from the New Energy and Industrial Technology Development Organization (to T. S.).
掲載誌名
Journal of Biological Chemistry
280巻
2号
開始ページ
1613
終了ページ
1624
出版年月日
2005-01-14
出版者
The American Society for Biochemistry and Molecular Biology, Inc.
ISSN
0021-9258
1083-351X
出版者DOI
言語
英語
NII資源タイプ
学術雑誌論文
広大資料タイプ
学術雑誌論文
DCMIタイプ
text
フォーマット
application/pdf
著者版フラグ
publisher
権利情報
This research was originally published in the Journal of Biological Chemistry. Noriko Umeda, Takeo Suzuki, Masashi Yukawa, Yoshikazu Ohya, Heisaburo Shindo, Kimitsuna Watanabe, and Tsutomu Suzuki. Mitochondria-specific RNA-modifying Enzymes Responsible for the Biosynthesis of the Wobble Base in Mitochondrial tRNAs. J Biol Chem. 2005; 280:1613-1624. © 2005 by The American Society for Biochemistry and Molecular Biology, Inc.
関連情報URL
部局名
統合生命科学研究科