The loss of specific DGC proteins leads to dystrophic muscle characteristics

Abstract

In striated skeletal muscle, the dystrophin-glycoprotein complex (DGC) serves to link the extracellular matrix and the subsarcolemmal cytoskeleton while also protecting muscle fibers from damage caused by contraction. Muscular dystrophies are a group of genetic disorders that primarily affect skeletal muscle and are characterized by progressive muscle wasting. Major forms of muscular dystrophy have been linked to abnormalities of the DGC proteins. Due to this, mice with mutations affecting the DGC are significant models for studying muscular dystrophy and its pathology. By performing histological and morphometric analyses of skeletal muscle, we can establish a correlation between the skeletal muscle characteristics, and the abnormal DGC expression across the mouse genotypes. This study will expand our basic understanding of the DGC in both healthy and diseased skeletal muscle, and how the DGC mutations might contribute to the muscle fibers’ integrity.