Figure 4

Double immunofluorescence analysis of aquaporin-4 (AQP4) and dystrophin in a female carrier of Duchenne muscular dystrophy (DMD) and an amyotrophic lateral sclerosis patient (ALS). A female carrier of DMD showed a mosaic pattern of dystrophin-positive and dystrophin-negative fibers on sarcoplasmic membrane with a markedly increased variability of muscle fiber diameters, and the stainability of AQP4 was strikingly patchy; some large fibers showed positive staining for AQP4 whereas almost all the small fibers were negative (A1-A3). The muscle specimens of an ALS patient showed that AQP4 immunoreactivity was weak or negative on the myofiber surface with group atrophy, but dystrophin was clearly visualized on the denervated muscles (B1-B3).