Satoru Hasegawa1, Mikako Ito1, Mayu Fukami2, Miki Hashimoto2, Masaaki Hirayama2, Kinji Ohno1. 1. a Division of Neurogenetics , Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine , Japan. 2. b Department of Pathophysiological Laboratory Sciences , Nagoya University Graduate School of Medicine , Japan.
Abstract
OBJECTIVE: Duchenne muscular dystrophy (DMD) is a devastating muscle disease caused by a mutation in DMD encoding dystrophin. Oxidative stress accounts for dystrophic muscle pathologies in DMD. We examined the effects of molecular hydrogen in mdx mice, a model animal for DMD. METHODS: The pregnant mother started to take supersaturated hydrogen water (>5 ppm) ad libitum from E15.5 up to weaning of the offspring. The mdx mice took supersaturated hydrogen water from weaning until age 10 or 24 weeks when they were sacrificed. RESULTS: Hydrogen water prevented abnormal body mass gain that is commonly observed in mdx mice. Hydrogen improved the spontaneous running distance that was estimated by a counter-equipped running-wheel, and extended the duration on the rota-rod. Plasma creatine kinase activities were decreased by hydrogen at ages 10 and 24 weeks. Hydrogen also decreased the number of central nuclei of muscle fibers at ages 10 and 24 weeks, and immunostaining for nitrotyrosine in gastrocnemius muscle at age 24 weeks. Additionally, hydrogen tended to increase protein expressions of antioxidant glutathione peroxidase 1, as well as anti-apoptotic Bcl-2, in skeletal muscle at age 10 weeks. DISCUSSION: Although molecular mechanisms of the diverse effects of hydrogen remain to be elucidated, hydrogen potentially improves muscular dystrophy in DMD patients.
OBJECTIVE:Duchenne muscular dystrophy (DMD) is a devastating muscle disease caused by a mutation in DMD encoding dystrophin. Oxidative stress accounts for dystrophic muscle pathologies in DMD. We examined the effects of molecular hydrogen in mdx mice, a model animal for DMD. METHODS: The pregnant mother started to take supersaturated hydrogen water (>5 ppm) ad libitum from E15.5 up to weaning of the offspring. The mdx mice took supersaturated hydrogen water from weaning until age 10 or 24 weeks when they were sacrificed. RESULTS:Hydrogen water prevented abnormal body mass gain that is commonly observed in mdx mice. Hydrogen improved the spontaneous running distance that was estimated by a counter-equipped running-wheel, and extended the duration on the rota-rod. Plasma creatine kinase activities were decreased by hydrogen at ages 10 and 24 weeks. Hydrogen also decreased the number of central nuclei of muscle fibers at ages 10 and 24 weeks, and immunostaining for nitrotyrosine in gastrocnemius muscle at age 24 weeks. Additionally, hydrogen tended to increase protein expressions of antioxidant glutathione peroxidase 1, as well as anti-apoptotic Bcl-2, in skeletal muscle at age 10 weeks. DISCUSSION: Although molecular mechanisms of the diverse effects of hydrogen remain to be elucidated, hydrogen potentially improves muscular dystrophy in DMDpatients.
Authors: Sonia Messina; Domenica Altavilla; M'hammed Aguennouz; Paolo Seminara; Letteria Minutoli; Maria C Monici; Alessandra Bitto; Anna Mazzeo; Herbert Marini; Francesco Squadrito; Giuseppe Vita Journal: Am J Pathol Date: 2006-03 Impact factor: 4.307
Authors: Olivier M Dorchies; Stéphanie Wagner; Ophélie Vuadens; Katri Waldhauser; Timo M Buetler; Pavel Kucera; Urs T Ruegg Journal: Am J Physiol Cell Physiol Date: 2006-02 Impact factor: 4.249