Abnormalities in brain biochemistry associated with lack of dystrophin: studies of the mdx mouse

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• 作者：Rae ; Caroline ; Griffin ; Julian L. ; Blair ; Duncan H. ; Bothwell ; John H. ; Bubb ; William A. ; Maitland ; Annie ; et. al.
• 关键词：Dystrophin ; γ ; -aminobutyric acid_A receptor ; Muscimol ; Metabolism ; Osmotic shock ; Oxygen consumption ; mdx mouse
• 刊名：Neuromuscular Disorders
• 出版年：2002
• 出版时间：February, 2002
• 年：2002
• 卷：12
• 期：2
• 页码：121-129
• 全文大小：199 K

文摘

Biochemical abnormalities have been reported in dystrophin-deficient muscle of boys with Duchenne (severe Xp21) muscular dystrophy or in the murine (mdx) model of the disease. These abnormalities include altered energy metabolism and responses to osmotic shock. In contrast, the situation in brain is less well understood and it is probable that dystrophin is playing a different role (or roles) in this organ. In this study we conclude that the elevation in choline-containing compounds reported in mdx brain is confined to cerebellum and hippocampus in older (> 6 months) mice. We report alterations in glucose metabolism in mdx brain under normal, awake conditions, and a reduced response of brain metabolism to the γ-aminobutyric acid_A receptor agonist muscimol. Using brain cortical slices we found no difference in the response of dystrophic tissue to hypoosmotic shock, but increased, substrate-dependent oxygen consumption rates at low oxygen partial pressures.