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| Content Provider | Springer Nature Link |
|---|---|
| Author | Schaffer, Stephen W. Jong, Chian Ju Ito, Takashi Azuma, Junichi |
| Copyright Year | 2012 |
| 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. |
| Starting Page | 47 |
| Ending Page | 56 |
| Page Count | 10 |
| File Format | |
| ISSN | 09394451 |
| Journal | Amino Acids |
| Volume Number | 46 |
| Issue Number | 1 |
| e-ISSN | 14382199 |
| Language | English |
| Publisher | Springer Vienna |
| Publisher Date | 2012-11-20 |
| Publisher Place | Vienna |
| Access Restriction | Subscribed |
| Subject Keyword | Taurine deficiency Mitochondrial diseases Wobble hypothesis Mitochondrial protein synthesis Electron transport chain function Biochemistry Analytical Chemistry Biochemical Engineering Life Sciences Proteomics Neurobiology |
| Content Type | Text |
| Resource Type | Article |
| Subject | Organic Chemistry Biochemistry Clinical Biochemistry |
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