Related Experiment Videos
Novel taurine-containing uridine derivatives and mitochondrial human diseases
1Dept 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.
Abstract:
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.