A modified diet does not ameliorate muscle pathology in a mouse model for Duchenne muscular dystrophy

• Published in: PloS one 2019-04, Vol.14 (4), p.e0215335-e0215335
• Main Authors: Verhaart, Ingrid E C, van de Vijver, Davy, Boertje-van der Meulen, Joke W, Putker, Kayleigh, Adamzek, Kevin, Aartsma-Rus, Annemieke, van Putten, Maaike
• Format: Article
• Language: English
• Subjects: Age; Amino acids; Amino Acids, Branched-Chain - administration & dosage; Animals; Body composition; Body mass index; Branched chain amino acids; Calcification; Calcification (Physiology); Calcinosis - pathology; Chain branching; Cholecalciferol - administration & dosage; Diet; Dietary minerals; Dietary Proteins - metabolism; Disease Models, Animal; Duchenne muscular dystrophy; Duchenne's muscular dystrophy; Dystrophin; Dystrophin - deficiency; Dystrophin - genetics; Dystrophy; Fibrosis; Genetics; Humans; Laboratory rats; Male; Males; Metabolism; Mice; Mice, Inbred DBA; Mice, Inbred mdx; Muscle, Skeletal - metabolism; Muscle, Skeletal - pathology; Muscles; Muscular dystrophy; Muscular Dystrophy, Duchenne - diet therapy; Muscular Dystrophy, Duchenne - metabolism; Muscular Dystrophy, Duchenne - pathology; Musculoskeletal system; Nutrition; Nutrition research; Pathology; Proteins; Regeneration; Risk factors; Rodents; Studies; Triterpenes - administration & dosage; Ursolic Acid; Utrophin; Vitamin D3
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0215335

Duchenne muscular dystrophy (DMD) is caused by a lack of dystrophin protein. Next to direct effects on the muscles, this has also metabolic consequences. The influence of nutrition on disease progression becomes more and more recognized. Protein intake by DMD patients may be insufficient to meet the increased demand of the constantly regenerating muscle fibers. This led to the hypothesis that improving protein uptake by the muscles could have therapeutic effects. The present study examined the effects of a modified diet, which composition might stimulate muscle growth, on disease pathology in the D2-mdx mouse model. D2-mdx males were fed with either a control diet or modified diet, containing high amounts of branched-chain amino acids, vitamin D3 and ursolic acid, for six weeks. Our study indicates that the modified diet could not ameliorate the muscle pathology. No effects on bodyweight or weight of individual muscles were observed. Neither did the diet affect severity of fibrosis or calcification of the muscles.