Accumulation of glycogen in lysosomes is characteristic in the deficiency of
Glycogen synthase
Liver debranching enzyme
Acid maltase
Muscle phosphorylase
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
Metabolism of carbohydrate