Figure 14.

Inhibition of the proteasome pathway up-regulates the membrane expression of members of the dystrophin-glycoprotein complex in human muscle explants from DMD patients. Muscle explants were incubated in the absence or presence of Velcade at 1 and 10 μmol/L concentrations for 16 hours. Frozen skeletal muscle sections from untreated and Velcade-treated DMD explants were immunostained with specific antibodies directed against dystrophin (DYS), β-dystroglycan (β-DG), and α-sarcoglycan (α-SG). A-SG, β-DG were reduced in the skeletal muscle fibers from untreated muscle explants, whereas they displayed an enhanced and uniform membrane staining on the muscle plasma membrane following Velcade exposure. As expected in the untreated group, DYS is negative, while, in the Velcade samples, it displays positivity in few fibers. Final magnification, ×25. Representative images are shown.