Document Detail

Taurine ameliorates impaired the mitochondrial function and prevents stroke-like episodes in patients with MELAS.
MedLine Citation:
PMID:  23257519     Owner:  NLM     Status:  MEDLINE    
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.
METHODS AND 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.
CONCLUSION: Taurine supplementation may be a novel potential treatment option for preventing the stroke-like episodes associated with MELAS.
Mitsue Rikimaru; Yutaka Ohsawa; Alexander M Wolf; Kiyomi Nishimaki; Harumi Ichimiya; Naomi Kamimura; Shin-ichiro Nishimatsu; Shigeo Ohta; Yoshihide Sunada
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't     Date:  2012-12-15
Journal Detail:
Title:  Internal medicine (Tokyo, Japan)     Volume:  51     ISSN:  1349-7235     ISO Abbreviation:  Intern. Med.     Publication Date:  2012  
Date Detail:
Created Date:  2012-12-21     Completed Date:  2013-05-28     Revised Date:  2013-06-20    
Medline Journal Info:
Nlm Unique ID:  9204241     Medline TA:  Intern Med     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  3351-7     Citation Subset:  IM    
Department of Neurology, Kawasaki Medical School, Japan.
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MeSH Terms
Cells, Cultured
MELAS Syndrome / complications*,  physiopathology*
Mitochondria / drug effects*,  physiology*
Stroke / etiology*,  prevention & control*
Taurine / pharmacology,  therapeutic use*
Young Adult
Reg. No./Substance:
Comment In:
Intern Med. 2013;52(9):1011   [PMID:  23648726 ]

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