TRIM32-mediated deregulation of key muscle substrates, including actin, α-actinin, tropomyosin, and desmin, contribute to muscle atrophy (Cohen et al., 2014; Cohen et al., 2012; Kudryashova et al., 2005; Locke et al., 2009), but studies have not been performed to directly test this model in the context of LGMD2H. This evidence concerns the gene DES and autosomal recessive limb-girdle muscular dystrophy type 2H.