What is the primary enzyme responsible for the breakdown of glycogen in lysosomes, and its deficiency leads to Pompe disease?

A Acid alpha-glucosidase ✓
B Alpha-amylase
C Glucose-6-phosphatase
D Debranching enzyme

Explanation

Acid alpha-glucosidase is the enzyme responsible for breaking down glycogen in lysosomes, and its deficiency leads to the accumulation of glycogen in cells, causing Pompe disease.

Mnemonic

Alpha = Acid, Glucosidase = Glycogen breakdown

Source

NCBI Gene Review
Enzymes in Disease Biochemistry Lysosomal Storage Diseases Medium MEDICAL Confidence: high