Internal medicine (Tokyo, Japan) · 2012
Taurine ameliorates impaired the mitochondrial function and prevents stroke-like episodes in patients with MELAS.
Клинический случайСтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Rikimaru Mitsue, Ohsawa Yutaka, Wolf Alexander M, Nishimaki Kiyomi, Ichimiya Harumi, Kamimura Naomi, Nishimatsu Shin-ichiro, Ohta Shigeo, Sunada Yoshihide
Аннотация
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.
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.