Characterization of immune population of model murine of muscular dystrophy.

Muscular dystrophy comprises a group of genetic diseases leading to progressive weakness and muscle mass loss due to protein deficiency, such as delta-sarcoglycan protein involved in stabilization of the sarcolemma and in signal transduction. Dystrophic muscles exhibit a predominant inflammatory environment characterized by cell infiltrate capable of producing immunostimulatory signals. While it has been reported that myeloid cell infiltrate may contribute to the pathogenesis of chronic myopathies, the specific role of delta-sarcoglycan protein deficiency in the overall immune response profile has not been previously assessed. In this study, we used flow cytometry to evaluate B and T cell populations, macrophages, monocytes, and neutrophils in the spleens of delta-sarcoglycan null and wildtype mice. We observed significant differences only in the F4/80 macrophage population. These findings suggest that delta-sarcoglycan deficiency may play a role in the differentiation of immune cells, particularly macrophages.