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Cell Death Dis ; 11(2): 127, 2020 02 18.
Article in English | MEDLINE | ID: mdl-32071288

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.


Subject(s)
Anthocyanins/administration & dosage , Dietary Supplements , Inflammation Mediators/metabolism , Mitochondria, Muscle/metabolism , Muscle, Skeletal/metabolism , Oxidative Stress , Sarcoglycanopathies/diet therapy , Animals , Disease Models, Animal , Disease Progression , Female , Male , Mice, Knockout , Mitochondria, Muscle/pathology , Muscle, Skeletal/pathology , Organelle Biogenesis , Phenotype , Protein Carbonylation , Sarcoglycanopathies/genetics , Sarcoglycanopathies/metabolism , Sarcoglycanopathies/pathology , Sarcoglycans/deficiency , Sarcoglycans/genetics
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