Mitochondrial translation optimization 1 (MTO1) and GTP-binding protein 3 (GTPBP3) are responsible for catalyzing the biosynthesis of 5-taurinomethyluridine.732 High-dose oral taurine has been shown to increase MTO1 expression,733 effectively preventing stroke-like episodes in MELAS by correcting the first anticodon nucleotide modification defect in mt-tRNALeu(UUR). The gene discussed is GTPBP3; the disease is stroke disorder.