Figure 4.

Restoration of the dystrophin-associated glycoprotein complex in treated golden retriever muscular dystrophy dog (GRMD) muscles and utrophin relocation. (a) Upper, middle, and lower rows show sections from biceps femoris muscles of normal dog, untreated and treated GRMD, respectively. Sections were immunoassayed for (a,e,l) dystrophin, (b,f,j) α-sarcoglycan, (c,g,k) β-sarcoglycan, and (d,h,l) β-dystroglycan. The same cluster of revertant fibers displaying dystrophin, as well as the associated glycoprotein complex, is shown on the serial sections from untreated GRMD. (b) Serial sections of (a,d) normal, (b,e) GRMD, and treated (c,f) GRMD muscles immunoassayed for (a–c) dystrophin (NCL-DYS2) and (d–f) utrophin (NCL-DRP2), respectively. Note that only the very few dystrophin-negative fibers are still stained for utrophin (arrows and highlighted square indicating a cluster of dystrophin-negative fibers).