Taurine Supplemental Therapy In Prevention Of Stroke-Like Episodes In Melas

In 1966, Francis Crick predicted that the first anticodon (“wobble”) nucleotide recognizes the third codon nucleotide through non-canonical Watson–Crick geometry. To date, several post-transcriptional modifications have been discovered in the wobble nucleotide. We discovered taurine modification at the wobble nucleotide is deficient in the mutant mitochondrial (mt) tRNALeu(UUR) in MELAS patients harboring the A3243G-mutant mt DNA. Because the taurine modification defect in the mutant mt tRNALeu(UUR) causes a deficiency in deciphering codons, we regard MELAS as a first-ever tRNA-modification disorder. Indeed, high-dose taurine supplementation ameliorates impaired mt dysfunction in patient-derived cells and prevents stroke-like episodes in two MELAS patients for more than nine years. Here we carried out a multi-center, open, phase III trial to prove the clinical efficiencies of oral taurine supplementation on preventing stroke-like episodes in patients with MELAS. We enrolled 10 patients suffering from repeated stroke-like episodes in the trial. One-year oral administration of taurine significantly decreased annual relapsing rates. We were also able to detect a significant increase in the taurine modification ratio of the mt tRNALeu(UUR) from white blood cells. These findings provide a new evidence that the taurine supplementation therapy prevents stroke-like episodes by reversing impaired taurine modification in mt tRNALeu(UUR).