(A) GAA activity in the indicated striated muscles, 18 weeks following vector administration. AAV-CK1hGAApA (CK1), either 1×1011 (1E+11; n=3) or 1×1012 (1E+12; n=3) vp/mouse, and AAV-MHCK7hGAApA (MHCK7), either 1×1011 (1E+11; n=4) or 1×1012 (1E+12; n=5) vp/mouse were injected intravenously at 3 months of age. Mock-treated GAA-KO mice were negative controls (PBS; n=4). (B) Glycogen content for GAA-KO mice in A. (C) Vector genome quantitation with realtime PCR for GAA-KO mice in A. (D) Ratio of GAA activity to vector genome quantity for GAA-KO mice in A. Mean +/- s.d. shown. P<0.05 (*) and P<0.001(**) indicated for the values for groups of mice following AAV-MHCK7hGAApA injection, in comparison to the group receiving the equivalent number of AAV-CK1hGAApA vector particles.