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补充矢车菊素可阻碍肌肉萎缩症的进展。

Nutritional intervention with cyanidin hinders the progression of muscular dystrophy.

机构信息

Department of Biosciences, University of Milan, via Celoria 26, 20133, Milan, Italy.

Venetian Institute of Molecular Medicine (VIMM), Department of Biomedical Sciences, University of Padova, Padova, Italy.

出版信息

Cell Death Dis. 2020 Feb 18;11(2):127. doi: 10.1038/s41419-020-2332-4.

Abstract

Muscular Dystrophies are severe genetic diseases due to mutations in structural genes, characterized by progressive muscle wasting that compromises patients' mobility and respiratory functions. Literature underlined oxidative stress and inflammation as key drivers of these pathologies. Interestingly among different myofiber classes, type I fibers display a milder dystrophic phenotype showing increased oxidative metabolism. This work shows the benefits of a cyanidin-enriched diet, that promotes muscle fiber-type switch and reduced inflammation in dystrophic alpha-sarcoglyan (Sgca) null mice having, as a net outcome, morphological and functional rescue. Notably, this benefit is achieved also when the diet is administered in dystrophic animals when the signs of the disease are seriously evident. Our work provides compelling evidence that a cyanidin-rich diet strongly delays the progression of muscular dystrophies, paving the way for a combinatorial approach where nutritional-based reduction of muscle inflammation and oxidative stress facilitate the successful perspectives of definitive treatments.

摘要

肌肉萎缩症是一种严重的遗传性疾病,由于结构基因突变所致,其特征是进行性肌肉萎缩,损害患者的运动和呼吸功能。文献强调氧化应激和炎症是这些疾病的关键驱动因素。有趣的是,在不同的肌纤维类型中,I 型纤维表现出较轻的营养不良表型,显示出增强的氧化代谢。这项工作表明富含矢车菊素的饮食的益处,它促进肌肉纤维类型转换,并减少具有 net outcome 的营养不良 alpha- sarcoglycan (Sgca) 缺失小鼠的炎症,从而实现形态和功能的挽救。值得注意的是,当饮食在疾病症状明显时被给予营养不良的动物时,也能达到这种益处。我们的工作提供了令人信服的证据,表明富含矢车菊素的饮食能强烈延缓肌肉萎缩症的进展,为联合治疗铺平了道路,其中基于营养的减少肌肉炎症和氧化应激有助于确定治疗方法的成功前景。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd76/7028923/68bc71e7ccaa/41419_2020_2332_Fig1_HTML.jpg

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