- 著者
- Mitsue Rikimaru Yutaka Ohsawa Alexander M. Wolf Kiyomi Nishimaki Harumi Ichimiya Naomi Kamimura Shin-ichiro Nishimatsu Shigeo Ohta Yoshihide Sunada
- 出版者
- The Japanese Society of Internal Medicine
- 雑誌
- Internal Medicine (ISSN:09182918)
- 巻号頁・発行日
- vol.51, no.24, pp.3351-3357, 2012 (Released:2012-12-15)
- 参考文献数
- 14
- 被引用文献数
- 10 51
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. 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.