Abstract：
Duchenne muscular dystrophy (DMD) is the most common muscular disorder affecting children. It affects nearly 1 male birth over 5000. Oxidative stress is a pervasive feature in the pathogenesis of DMD. Recent work shows that the main generators of ROS are NADPH oxidases (NOX), suggesting that they are an early and promising target in DMD. In addition, skeletal muscles of mdx mice, a murine model of DMD, overexpress NOXes. We investigated the impact of diapocynin, a dimer of the NOX inhibitor apocynin, on the chronic disease phase of mdx5Cv mice. Treatment of these mice with diapocynin from 7 to 10 months of age resulted in decreased hypertrophy of several muscles, prevented force loss induced by tetanic and eccentric contractions, improved muscle and respiratory functions, decreased fibrosis of the diaphragm and positively regulated the expression of disease modifiers. These encouraging results ensure the potential role of diapocynin in future treatment strategies.
摘要：
杜氏肌营养不良症（DMD）是影响儿童的最常见的肌肉疾病。它影响了近1名5000岁以上的男性出生。氧化应激是DMD发病机制中的普遍特征。最近的工作表明，ROS的主要发生器是NADPH氧化酶（NOX），这表明它们是DMD的早期和有希望的目标。此外,mdx小鼠的骨骼肌，DMD的鼠模型,过度表达NOXes。我们调查了Diaposynin的影响，NOX抑制剂apocynin的二聚体，在mdx5Cv小鼠的慢性疾病阶段。用7至10月龄的diaposynin治疗这些小鼠导致几块肌肉的肥大减少，防止强直收缩和偏心收缩引起的力损失，改善肌肉和呼吸功能，减少diaphragm膜的纤维化，并积极调节疾病修饰剂的表达。这些令人鼓舞的结果确保了diaposynin在未来治疗策略中的潜在作用。
