Journal
CLINICAL NUTRITION
Volume 32, Issue 3, Pages 472-475Publisher
CHURCHILL LIVINGSTONE
DOI: 10.1016/j.clnu.2012.06.001
Keywords
N-Acetylcysteine; TNF-alpha; Mdx mice; Diaphragm muscle
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Funding
- Fundacao de Amparo a Pesquisa do Estado de Sao Paulo (FAPESP) [08/50731-3]
- Coordenacao de Aperfeicoamento de Pessoal de Nivel Superior (CAPES-PROEX)
- Conselho Nacional de Pesquisas (CNPq) [302006/09-5, 301386/07-2]
- CAPES
- CNPq
- FAPESP [10/01087-4]
- Fundacao de Amparo a Pesquisa do Estado de Sao Paulo (FAPESP) [10/01087-4] Funding Source: FAPESP
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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-alpha), 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-alpha 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-alpha 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. (C) 2012 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.
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