NOX4-expressing PDGFRα+ cells were also found in the muscles of mice that model limb-girdle MD 2B (LGMD2B) (dysferlinopathy) and LGMD2F (δ-sarcoglycan deficiency) (Figure 2C); therefore, these observations are not restricted to dystrophin-deficient muscle. This evidence concerns the gene NOX4 and autosomal recessive limb-girdle muscular dystrophy type 2B.