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Amino Acids

, Volume 46, Issue 1, pp 47–56 | Cite as

Role of taurine in the pathologies of MELAS and MERRF

  • Stephen W. SchafferEmail author
  • Chian Ju Jong
  • Takashi Ito
  • Junichi Azuma
Review Article

Abstract

Taurine is an abundant β-amino acid that concentrates in the mitochondria, where it participates in the conjugation of tRNAs for leucine, lysine, glutamate and glutamine. The formation of 5-taurinomethyluridine-tRNA strengthens the interaction of the anticodon with the codon, thereby promoting the decoding of several codons, including those for AAG, UUG, CAG and GAG. By preventing these series of events, taurine deficiency appears to diminish the formation of 5-taurinomethyluridine and causes inefficient decoding for the mitochondrial codons of leucine, lysine, glutamate and glutamine. The resulting reduction in the biosynthesis of mitochondria-encoded proteins deprives the respiratory chain of subunits required for the assembly of respiratory chain complexes. Hence, taurine deficiency is associated with a reduction in oxygen consumption, an elevation in glycolysis and lactate production and a decline in ATP production. A similar sequence of events takes place in mitochondrial diseases MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) and MERRF (myoclonic epilepsy and ragged-red fiber syndrome). In both diseases, mutations in their respective tRNAs interfere with the formation of 5-taurinomethyluridine in the wobble position. Hence, the taurine-deficient phenotype resembles the phenotypes of MELAS and MERRF.

Keywords

Taurine deficiency Mitochondrial diseases Wobble hypothesis Mitochondrial protein synthesis Electron transport chain function 

Notes

Conflict of interest

The authors declare that there are no conflicts of interest.

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Copyright information

© Springer-Verlag Wien 2012

Authors and Affiliations

  • Stephen W. Schaffer
    • 1
    Email author
  • Chian Ju Jong
    • 1
  • Takashi Ito
    • 2
  • Junichi Azuma
    • 2
  1. 1.Department of Pharmacology, College of MedicineUniversity of South AlabamaMobileUSA
  2. 2.School of PharmacyHyogo University of Health SciencesKobeJapan

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