TABLE 1 

Glycogen clearance comparison by muscle type between Reveglucosidase alfa and rhGAA in Pompe mice

Muscle TyperhGAA EC50aReveglucosidase Alfa EC50aEC50 Ratiob 
(rhGAA/Reveglucosidase Alfa)
mg/kgmg/kg
Heart13.2 (0.893)5.00 (0.298)2.85
Diaphragm32.2 (4.64)12.1 (1.96)2.88
Quadriceps25.2 (4.59)9.70 (1.12)2.81
Psoas54.6 (10.7)12.4 (2.82)4.77
Soleus27.8 (3.47)9.36 (1.48)3.22
  • a Mean (SE).

  • b To account for differences in molecular weight, a correction factor of 1.0825 was used to convert rhGAA doses in mg/kg to equivalent doses of Reveglucosidase alfa in mg/kg for EC50 ratio comparison (molecular weight: rhGAA = 109 kDA, Reveglucosidase alfa = 118 kDA).