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Metabogenic and Nutriceutical Approaches to Address Energy Dysregulation and Skeletal Muscle Wasting in Duchenne Muscular Dystrophy

机译:代谢和营养的方法来解决能量失调和骨骼肌萎缩在杜兴肌营养不良症

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Duchenne Muscular Dystrophy (DMD) is a fatal genetic muscle wasting disease with no current cure. A prominent, yet poorly treated feature of dystrophic muscle is the dysregulation of energy homeostasis which may be associated with intrinsic defects in key energy systems and promote muscle wasting. As such, supplementative nutriceuticals that target and augment the bioenergetical expansion of the metabolic pathways involved in cellular energy production have been widely investigated for their therapeutic efficacy in the treatment of DMD. We describe the metabolic nuances of dystrophin-deficient skeletal muscle and review the potential of various metabogenic and nutriceutical compounds to ameliorate the pathological and clinical progression of the disease.
机译:杜兴氏肌营养不良症(DMD)是一种致命的遗传性肌肉萎缩性疾病,目前尚无治愈方法。营养不良性肌肉的一个突出但尚未得到充分治疗的特征是能量稳态的失调,这可能与关键能量系统的固有缺陷有关,并促进了肌肉的浪费。这样,已经针对针对和增强参与细胞能量产生的代谢途径的生物能扩展的补充营养食品进行了DMD的治疗功效的研究。我们描述了肌营养不良蛋白缺乏者骨骼肌的代谢细微差别,并综述了各种代谢和营养化合物改善疾病的病理和临床进展的潜力。

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