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Introduction Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder affecting 1 in 5000 newborn males worldwide, leading to progressive skeletal muscle weakness and cardiomyopathy. Truncating mutations in the DMD gene resulting in the absence of the dystrophin protein are the primary cause of DMD. Lack of dystrophin leads to chronic muscle damage, inflammation, fibrosis and impaired regeneration. We characterized the differences in three key pathological pathways (fibrosis, inflammation and muscle de/regeneration) in mdx/BL10 and mdx/utrn-/-mouse models compared to wild type mice. Moreover, we studied differences in differentiation between myoblasts derived from wild type and dystrophic muscle.

Methods Quadriceps and diaphragm muscles were isolated from 2-and 10-month old wild type, mdx and 2-month old mdx/utrn-/-mice (6 mice per genotype). Gene expression changes were analyzed with quantitative RT-PCR in relation to age and disease severity. For differentiation assay, myoblasts were isolated from the extensor digitorum longus muscle of 5-8 weeks old wild type, mdx and mdx/utrn-/-mice (6 mice per genotype).