Dysfunctional muscle and liver glycogen metabolism in mdx dystrophic mice

• Published in: PloS one 2014-03, Vol.9 (3), p.e91514-e91514
• Main Authors: Stapleton, David I, Lau, Xianzhong, Flores, Marcelo, Trieu, Jennifer, Gehrig, Stefan M, Chee, Annabel, Naim, Timur, Lynch, Gordon S, Koopman, René
• Format: Article
• Language: English
• Subjects: Analysis; Animal models; Animals; Biology; Cyclic AMP-Dependent Protein Kinases - metabolism; Diabetes; Disease Models, Animal; Dystrophin; Dystrophin - physiology; Dystrophy; Endocrinology; Enzymatic activity; Enzymes; Glucose; Glucose Intolerance; Glucose metabolism; Glycogen; Glycogen - metabolism; Glycogen Phosphorylase - metabolism; Glycogen synthesis; Kinases; Liver; Liver - metabolism; Liver - physiopathology; Liver diseases; Male; Medicine; Metabolism; Mice; Mice, Inbred C57BL; Mice, Inbred mdx; Mice, Transgenic; Muscle, Skeletal - metabolism; Muscle, Skeletal - physiopathology; Muscular dystrophy; Mutation; Phenotype; Physiological aspects; Physiology; Protein binding; Proteins; Rodents; Skeletal muscle; Utrophin
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0091514

Duchenne muscular dystrophy (DMD) is a severe, genetic muscle wasting disorder characterised by progressive muscle weakness. DMD is caused by mutations in the dystrophin (dmd) gene resulting in very low levels or a complete absence of the dystrophin protein, a key structural element of muscle fibres which is responsible for the proper transmission of force. In the absence of dystrophin, muscle fibres become damaged easily during contraction resulting in their degeneration. DMD patients and mdx mice (an animal model of DMD) exhibit altered metabolic disturbances that cannot be attributed to the loss of dystrophin directly. We tested the hypothesis that glycogen metabolism is defective in mdx dystrophic mice. Dystrophic mdx mice had increased skeletal muscle glycogen (79%, (P