Abstract:Myopathies are frequently caused by mutations in genes encoding for extracellular matrix (ECM) proteins, which gets progressively substituted by fibrotic tissue. Spinal muscular atrophy is a disorder caused by mutations in SMN gene. The same mutation in HSA-Cre, SmnF7/F7
mice generates a specific impairment of skeletal muscle with diaphragm displaying fibrosis and myofiber loss. Using a decellularized matrix obtained from healthy-mice diaphragm we aimed to ameliorate diaphragm condition of HSA-Cre, SmnF7/F7
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