Taurine Ameliorates Impaired the Mitochondrial Function and Prevents Stroke-like Episodes in Patients with MELAS
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- Rikimaru Mitsue
- Department of Neurology, Kawasaki Medical School, Japan
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- Ohsawa Yutaka
- Department of Neurology, Kawasaki Medical School, Japan
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- Wolf Alexander M.
- Department of Biochemistry and Cell Biology, Institute of Development and Aging Sciences, Nippon Medical School, Japan
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- Nishimaki Kiyomi
- Department of Biochemistry and Cell Biology, Institute of Development and Aging Sciences, Nippon Medical School, Japan
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- Ichimiya Harumi
- Department of Biochemistry and Cell Biology, Institute of Development and Aging Sciences, Nippon Medical School, Japan
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- Kamimura Naomi
- Department of Biochemistry and Cell Biology, Institute of Development and Aging Sciences, Nippon Medical School, Japan
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- Nishimatsu Shin-ichiro
- Department of Molecular and Developmental Biology, Kawasaki Medical School, Japan
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- Ohta Shigeo
- Department of Biochemistry and Cell Biology, Institute of Development and Aging Sciences, Nippon Medical School, Japan
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- Sunada Yoshihide
- Department of Neurology, Kawasaki Medical School, Japan
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Abstract
Objective Post-transcriptional taurine modification at the first anticodon ("wobble") nucleotide is deficient in A3243G-mutant mitochondrial (mt) tRNALeu(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.<br> 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.<br> Conclusion Taurine supplementation may be a novel potential treatment option for preventing the stroke-like episodes associated with MELAS.<br>
Journal
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- Internal Medicine
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Internal Medicine 51 (24), 3351-3357, 2012
The Japanese Society of Internal Medicine