Effects of therapy on cation-independent mannose 6-phosphate receptor (CI-MPR) expression in GAA-KO mice. Expression of GAA with AAV2/8-MHCK7hGAApA induced CI-MPR expression in quadriceps and gastrocnemius. Western blotting was performed on three mice from each group, and signal intensity was quantified with Quantity One (Bio-Rad, Hercules, CA) for quadriceps, gastrocnemius, heart, and liver. CI-MPR was normalized to the signal for glyceraldehyde-3-phosphate dehydrogenase (GAPDH). Statistical analysis was performed by two-way analysis of variance with Bonferroni's comparison test, using both the untreated and combination therapy groups as the reference in separate analyses (*p < 0.05, **p < 0.01, ***p < 0.001). Shown are means and standard deviation. Clen, clenbuterol.