Background:GAA(Alpha-1,4-glucosidase) is a lysosomal enzyme involved in the degradation of glycogen within cellular vacuoles. After translation, GAA undergoes proteolytic processing to form two lengths of lysosomal a-glucosidase, and both N-terminal and C-terminal processing occur. The full sequence can be further processed into a mature form. The 105 kDa GAA can be cleaved into 70 kDa or 76 kDa form.