title: Taurine ameliorates impaired the mitochondrial function and prevents stroke-like episodes in patients with MELAS.
abstract: OBJECTIVE
  Post-transcriptional taurine modification at the first anticodon ("wobble") nucleotide is deficient in A3243G-mutant mitochondrial (mt) tRNA(Leu(UUR)) of patients with myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). Wobble nucleotide modifications in tRNAs have recently been identified to be important in the accurate and efficient deciphering of codons. We herein examined whether taurine can alleviate mitochondrial dysfunction in patient-derived pathogenic cells and prevent clinical symptoms in MELAS patients.
  
  
  RESULTS
  The addition of taurine to the culture media ameliorated the reduced oxygen consumption, decreased the mitochondrial membrane potential, and increased the oxidative stress in MELAS patient-derived cells. Moreover, high dose oral administration of taurine (0.25 g/kg/day) completely prevented stroke-like episodes in two MELAS patients for more than nine years.
  
  
  CONCLUSIONS
  Taurine supplementation may be a novel potential treatment option for preventing the stroke-like episodes associated with MELAS.
authors.affiliation: Department of Neurology, Kawasaki Medical School, Japan.
authors.forename: Mitsue
authors.initials: M
authors.lastname: Rikimaru
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Ohsawa
authors.forename: Alexander M
authors.initials: AM
authors.lastname: Wolf
authors.forename: Kiyomi
authors.initials: K
authors.lastname: Nishimaki
authors.forename: Harumi
authors.initials: H
authors.lastname: Ichimiya
authors.forename: Naomi
authors.initials: N
authors.lastname: Kamimura
authors.forename: Shin-ichiro
authors.initials: S
authors.lastname: Nishimatsu
authors.forename: Shigeo
authors.initials: S
authors.lastname: Ohta
authors.forename: Yoshihide
authors.initials: Y
authors.lastname: Sunada
mesh_terms: D000328:Adult; D002478:Cells, Cultured; D005260:Female; D006801:Humans; D017241:MELAS Syndrome / Q000150:complications* / Q000503:physiopathology*; D008928:Mitochondria / Q000187:drug effects* / Q000502:physiology*; D020521:Stroke / Q000209:etiology* / Q000517:prevention & control*; D013654:Taurine / Q000494:pharmacology / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D013654:Taurine