N-乙酰半胱氨酸治疗可降低 mdx 小鼠膈肌肌肉中的 TNF-α 水平和肌坏死。
N-acetylcysteine treatment reduces TNF-α levels and myonecrosis in diaphragm muscle of mdx mice.
机构信息
Departamento de Biologia Estrutural e Funcional, Instituto de Biologia, Universidade Estadual de Campinas (UNICAMP), Campinas, São Paulo 13083-970, Brazil.
出版信息
Clin Nutr. 2013 Jun;32(3):472-5. doi: 10.1016/j.clnu.2012.06.001. Epub 2012 Jun 23.
BACKGROUND & AIMS: Duchenne muscular dystrophy (DMD) is a genetic muscle disease caused by the absence of dystrophin. An established animal model of DMD is the mdx mouse, which is unable to express dystrophin. Inflammation, particularly the proinflammatory cytokine tumor necrosis factor alpha (TNF-α), strongly contributes to necrosis in the dystrophin-deficient fibers of the mdx mice and in DMD. In this study we investigated whether the antioxidant N-acetylcysteine (NAC) decreases TNF-α levels and protects the diaphragm muscle of mdx mice against necrosis.
METHODS
Mdx mice (14 days old) received daily intraperitoneal injections of NAC for 14 days, followed by removal of the diaphragm muscle. Control mdx mice were injected with saline.
RESULTS
NAC reduced TNF-α and 4-HNE-protein adducts levels, inflammation, creatine kinase levels, and myonecrosis in diaphragm muscle.
CONCLUSIONS
NAC may be used as a complementary treatment for dystrophinopathies. However, clinical trials are needed to determine the appropriate dose for patients with Duchenne muscular dystrophy.
背景与目的
杜氏肌营养不良症（DMD）是一种由肌营养不良蛋白缺失引起的遗传性肌肉疾病。已建立的 DMD 动物模型是 mdx 小鼠，其无法表达肌营养不良蛋白。炎症，特别是促炎细胞因子肿瘤坏死因子-α（TNF-α），强烈导致 mdx 小鼠和 DMD 中肌营养不良蛋白缺陷纤维的坏死。在这项研究中，我们研究了抗氧化剂 N-乙酰半胱氨酸（NAC）是否降低 TNF-α 水平并保护 mdx 小鼠的膈肌免受坏死。
方法
14 天大的 mdx 小鼠每天接受腹腔内注射 NAC，持续 14 天，然后取出膈肌。对照 mdx 小鼠注射生理盐水。
结果
NAC 降低了 TNF-α 和 4-HNE-蛋白质加合物水平、炎症、肌酸激酶水平和膈肌的肌坏死。
结论
NAC 可作为肌营养不良蛋白病的辅助治疗方法。然而，需要进行临床试验以确定 Duchenne 肌营养不良症患者的合适剂量。
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