Proteasome inhibitors improve the function of mutant lysosomal α-glucosidase in fibroblasts from Pompe disease patient carrying c.546G>T mutation

► Proteasome inhibitors stabilized mutant GAA in patient cells with c.546G>T mutation. ► Proteasome inhibitors promoted the maturation of mutant GAA in patient cells. ► Proteasome inhibitors restored subcellular localization of patient GAA to lysosome. ► Proteasome inhibitors increased the GAA enzyme activity in patient cells.