Novel taurine-containing uridine derivatives and mitochondrial human diseases

Nucleic Acids Res Suppl. 2001:(1):257-8. doi: 10.1093/nass/1.1.257.

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.

MeSH terms

  • Humans
  • Mass Spectrometry
  • Mitochondrial Encephalomyopathies / genetics*
  • RNA / chemistry*
  • RNA, Mitochondrial
  • RNA, Transfer / chemistry*
  • Taurine / chemistry*
  • Uridine / analogs & derivatives*

Substances

  • RNA, Mitochondrial
  • Taurine
  • RNA
  • RNA, Transfer
  • Uridine