Glycogen storage disease type II (MIM: 232300) known as Pompe disease (PD) is an autosomal recessive disease caused by a defect in α-glucosidase (AαGlu, EC: 3.2.1.20) encoded by GAA. PD manifests as the accumulation of lysosomal glycogen in the heart and skeletal muscles (Martiniuk et al., 1986); massive accumulation eventually leads to death from cardiorespiratory failure (Mellies and Lofaso, 2009). The gene discussed is GAA; the disease is glycogen storage disease II.