Variable reduction of dystrophin and sarcoglycans was observed in most of our patients with sarcoglycanopathies or dystrophinopathies, and a slight reduction of sarcoglycans and dystrophin was observed in a few patients with LGMD2I, suggesting that it is sometimes incorrect to predict the primary genetic defect based on muscle immunoanalysis due to secondary reduction of other DGC proteins [7–9]. Here, DMD is linked to autosomal recessive limb-girdle muscular dystrophy type 2I.