Which enzyme's deficiency is associated with the development of Pompe disease, a genetic disorder characterized by the accumulation of glycogen in muscles?

A Acid alpha-glucosidase ✓
B Alpha-galactosidase
C Beta-glucosidase
D Hexosaminidase

Explanation

Pompe disease is caused by a deficiency of the enzyme acid alpha-glucosidase, which breaks down glycogen in lysosomes.

Mnemonic

Pompe = Pump → acid alpha-glucosidase pumps glycogen out of lysosomes

Source

NCERT Class 12 Biochemistry
Enzymes in Disease Biochemistry Lysosomal Storage Diseases Medium MEDICAL Confidence: high