Morphometric analysis of mdx diaphragm muscle fibres. Comparison with hindlimb muscles

Valproic Acid Activates the PI3K/Akt/mTOR Pathway in Muscle and Ameliorates Pathology in a Mouse Model of Duchenne Muscular Dystrophy

“…In DMD patients type VI collagen is increased approximately twofold to threefold above normal and extensive fibrosis contributes to the dystrophic pathology and severely limits the mobility. 19,23,24 Western blotting of protein extracted from the quadriceps of mdx/utrn Ϫ/Ϫ mice treated with VPA showed an approximate threefold decrease in levels of this collagen. Mice treated with VPA also had greater myofiber integrity, as shown by decreased Evans blue dye uptake.…”

Evidence ofmdx mouse skeletal muscle fragility in vivo by eccentric running exercise

“…The skeletal muscles do not show extensive fibrosis, except the diaphragm, which is one of the most constantly solicited skeletal muscles. 24,43 The muscle fibers are frequently split and show variations in diameter. Depending on the age of the mouse and the muscle considered, 30-90% of the fibers are centronucleated, which is a characteristic of adult regenerated fibers.…”

Regenerative capacity of the dystrophic (mdx) diaphragm after induced injury

“…A murine model of DMD, the mdx mouse, also lacks dystrophin due to a nonsense point mutation in exon 23 of the dystrophin gene (45). However, most skeletal muscles of mdx mice show little fibrosis or functional alteration until late in life (28,37,48). The relative sparing of muscle function in mdx mice, which is in striking contrast to the destructive muscle disease found in humans with the same genetic defect, has often been attributed to a superior regenerative capacity of skeletal muscle in the mouse model (35).…”