Taurine ameliorates mitochondrial dysfunction and prevents stroke-like episodes in patients with MELAS

Abstract

In 1966, Francis Crick predicted that the first anticodon (“wobble”) nucleotide recognizes the third codon nucleotide through non-canonical Watson–Crick geometry. To date, post-transcriptional modifications in tRNAs are thought to play critical roles in deciphering the genetic code. In MELAS patients harbouring the A3243G-mutant mitochondrial DNA, post-transcriptional taurine modification at the wobble nucleotide is deficient in the mutant mitochondrial (mt) tRNALeu(UUR). Here, we show that addition of taurine to culture media ameliorated reduced oxygen consumption, decreased mitochondrial membrane potential, and increased oxidative stress in MELAS patient-derived pathogenic cells.