• To facilitate gene and cell therapy experiments, we created severely immune-deficient mouse models of Duchenne muscular dystrophy (DMD), limb girdle muscular dystrophy 2B (LGMD2B), and limb girdle muscular dystrophy 2D (LGMD2D) by crossing mdx 4Cv , Bl/AJ, and Sgca-null mice with NRG immune-deficient mice. (plos.org)
  • Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophy, characterized by widespread degeneration of the skeletal, respiratory, and cardiac muscles, resulting in disability and premature death. (plos.org)
  • LGMD2B leads to a later-onset, milder form of muscular dystrophy characterized by progressive degeneration of skeletal muscles and resulting disability 1 , 4 . (plos.org)
  • To determine their therapeutic value, dystrophic mdx mice were subject to forced exercise to model the DMD cardiac phenotype. (ox.ac.uk)
  • By contrast, the phenotype of the BlAJ/NRG mice was milder in each case. (plos.org)
  • Treated mice also exhibited significantly reduced cardiac fibrosis and improved sarcolemmal integrity. (ox.ac.uk)
  • This work demonstrates that high levels of cardiac dystrophin restored by Pip peptide-AOs prevents further deterioration of cardiomyopathy and pathology following exercise in dystrophic DMD mice. (ox.ac.uk)
  • In skeletal muscles, the Dp71 isoform was ectopically expressed as a probable consequence of the mutation. (researchsquare.com)
  • In addition to skeletal muscle wasting, DMD patients develop cardiomyopathy, which significantly contributes to mortality. (ox.ac.uk)
  • We performed centronucleation, Evans blue dye, hydroxyproline, and treadmill assays on the disease model mice versus NRG controls to evaluate muscle histology and function. (plos.org)
  • These studies demonstrated that the mdx 4Cv /NRG and Sgca/NRG mice showed significant deficits in muscle structure and function in all the assays and were similar to each other. (plos.org)
  • These novel immune-deficient mouse models of DMD, LGMD2B, and LGMD2D will be useful for long-term gene and cell therapy studies involving transfer of foreign genes and cells. (plos.org)