Novel taurine-containing uridine derivatives and mitochondrial human diseases.
Two novel modified uridines were identified from mammalian mitochondrial (mt) tRNAs. Mass spectrometric analysis revealed that they are modified uridines possessing a sulfonic acid group derived from taurine; 5-taurinomethyl-uridine from mt tRNAs for Trp and Leu(UUR), and 5-taurinomethyl-2-thiouridine from mt tRNAs for Lys, Gln and Glu. We have found lack of modification of these taurine-containing uridines in mutant mt tRNAs for Leu(UUR) and Lys from pathogenic cells of mitochondrial encephalomyopathies, MELAS and MERRF, respectively.
Suzuki
T
T
Dept of Integrated Biosciences, Graduate School of Frontier Sciences, Dept of Chemistry and Biotechnology, Graduate School of Engineering, University of Tokyo, 5-1-5 Kashiwanoha, Kashiwa, Chiba Prefecture, 277-8562, Japan.
Suzuki
T
T
Wada
T
T
Saigo
K
K
Watanabe
K
K
D006801:Humans; D013058:Mass Spectrometry; D017237:Mitochondrial Encephalomyopathies / Q000235:genetics*; D012313:RNA / Q000737:chemistry*; D000077278:RNA, Mitochondrial; D012343:RNA, Transfer / Q000737:chemistry*; D013654:Taurine / Q000737:chemistry*; D014529:Uridine / Q000031:analogs & derivatives*
D000077278:RNA, Mitochondrial; D013654:Taurine; D012313:RNA; D012343:RNA, Transfer; D014529:Uridine