Type Name Enzyme Deficiency Clinical Features II Pompe disease Lysosomal a1 - 4 and a1 - 6 glucosidase (acid maltase) Accumulation of glycogen in lysosomes: juvenile onset variant, Muscle hypotonia, Death from hea failure by age 2 Adult-onset variant, muscle dystrophy Reference: Harper; 30th edition; Table: 18-2; Page no: 179