期刊
INTERNAL MEDICINE
卷 51, 期 24, 页码 3351-3357出版社
JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.51.7529
关键词
MELAS; post-transcriptional modification; taurine; stroke-like episodes
资金
- Intramural Neurological and Psychiatric Disorders from the National Center of Neurology and Psychiatry [20B-13, 23-5]
- Comprehensive Research on Disability Health and Welfare from the Ministry of Health, Labour and Welfare of Japan [H20-018]
- Japan Society for the Promotion of Science [C-20591013, C-21591101, C-23591261]
- Kawasaki Medical School [22-A24, 21-T1, 23-P601, 23-T1]
- Grants-in-Aid for Scientific Research [24591281, 23591261, 24700762] Funding Source: KAKEN
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.
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