代谢亢进
骨骼肌
医学
去神经支配
能源消耗
神经科学
脑干
肌萎缩侧索硬化
肌萎缩
内科学
内分泌学
疾病
生物
作者
Silvia Scaricamazza,Illari Salvatori,Giacomo Giacovazzo,Jean Philippe Loeffler,René Frydman,Marco Rosina,Cyril Quessada,Daisy Proietti,Constantin Heil,Simona Rossi,Stefania Battistini,F. Giannini,Nila Volpi,Frederik J. Steyn,Shyuan T. Ngo,Elisabetta Ferraro,Luca Madaro,Roberto Coccurello,Cristiana Valle,Alberto Ferri
出处
期刊:iScience
[Elsevier]
日期:2020-05-01
卷期号:23 (5): 101087-101087
被引量:55
标识
DOI:10.1016/j.isci.2020.101087
摘要
Summary
Patients with ALS show, in addition to the loss of motor neurons in the spinal cord, brainstem, and cerebral cortex, an abnormal depletion of energy stores alongside hypermetabolism. In this study, we show that bioenergetic defects and muscle remodeling occur in skeletal muscle of the SOD1G93A mouse model of ALS mice prior to disease onset and before the activation of muscle denervation markers, respectively. These changes in muscle physiology were followed by an increase in energy expenditure unrelated to physical activity. Finally, chronic treatment of SOD1G93A mice with Ranolazine, an FDA-approved inhibitor of fatty acid β-oxidation, led to a decrease in energy expenditure in symptomatic SOD1G93A mice, and this occurred in parallel with a robust, albeit temporary, recovery of the pathological phenotype.
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