The X-linked dystrophin gene (Dmd) is highly expressed in muscle cells and encodes a cytoskeletal protein which localizes to the inner face of the sarcolemma. Dystrophin molecules bind to cytoskeletal F-actin and transmembrane beta-dystroglycan as part of a complex, multimolecular unit that mediates signaling between the intracellular cytoskeleton and the extracellular matrix. The structure and localization also suggest that dystrophin is important for stabilizing the plasma membrane, particularly during contraction. Like human patients who suffer from one of the most common neuromuscular diseases, Duchenne muscular dystrophy (DMD), the Dmdmdx mutants do not express dystrophin and therefore have been routinely used as an animal model of the disease even though the resultant myopathology is much less severe compared to the human disease course. When combined with the Prkdcscid allele, there is some amelioration of the mdx phenotype including a reduction in the rate of muscle fibrosis, higher endurance and decreased expression of active TGFB1. However, MDX/SCID mice continue to exhibit necrosis, centrally located nuclei and and the muscle degeneration characteristic of DMD. The MDX/SCID mouse may be used a dystrophic model for the transplantation of human donor cells to evaluate skeletal muscle regeneration.

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 MDX/SCID mice exhibit necrosis, centrally located nuclei and and the muscle degeneration characteristic of DMD and may be used a dystrophic model for the transplantation of human donor cells to evaluate skeletal muscle regeneration.

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