关键词: Central nervous system Duchenne muscular dystrophy Muscle mdx

来  源:   DOI:10.1007/s12035-024-04258-6

Abstract:
Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder characterized by progressive skeletal muscle degeneration and systemic effects, including the central nervous system (CNS). This study aimed to assess the impact of a 14-day ketogenic diet (DCet) on biochemical and clinical parameters in a DMD mouse model. Young adult mice (50 days old) were fed DCet, while control groups received a standard diet. On the 14th day, memory and behavior tests were conducted, followed by biochemical evaluations of oxidative stress, inflammatory biomarkers, body weight, feed intake, and brain-derived neurotrophic factor (BDNF) levels. mdx + DCet mice showed reduced mass (0.2 g ± 2.49) and improved memory retention (p < 0.05) compared to controls. Oxidative damage in muscle tissue and CNS decreased, along with a significant cytokine level reduction (p <0.05). The protocol led to an increase in hippocampal BDNF and mitochondrial respiratory complex activity in muscle tissue and the central nervous system (CNS), while also decreasing creatine kinase activity only in the striatum. Overall, a 14-day DCet showed protective effects by improving spatial learning and memory through reductions in oxidative stress and immune response, as well as increases in BDNF levels, consistent with our study\'s findings.
摘要:
Duchenne型肌营养不良症(DMD)是一种X连锁隐性疾病,其特征是进行性骨骼肌变性和全身效应,包括中枢神经系统(CNS)。这项研究旨在评估14天生酮饮食(DCet)对DMD小鼠模型中生化和临床参数的影响。年轻的成年小鼠(50天大)饲喂DCet,而对照组接受标准饮食。第14天,进行了记忆和行为测试,接下来是氧化应激的生化评估,炎症生物标志物,体重,饲料摄入量,和脑源性神经营养因子(BDNF)水平。与对照相比,mdx+DCet小鼠显示出降低的质量(0.2g±2.49)和改善的记忆保留(p<0.05)。肌肉组织和中枢神经系统的氧化损伤减少,伴随着显著的细胞因子水平降低(p<0.05)。该方案导致肌肉组织和中枢神经系统(CNS)中海马BDNF和线粒体呼吸复合物活性增加,同时也降低仅在纹状体中的肌酸激酶活性。总的来说,14天的DCet通过减少氧化应激和免疫反应来改善空间学习和记忆,以及BDNF水平的增加,与我们的研究结果一致。
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