What is the primary enzyme responsible for the breakdown of glycogen in lysosomes, and its deficiency leads to a rare genetic disorder?

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

Explanation

The primary enzyme responsible for the breakdown of glycogen in lysosomes is acid alpha-glucosidase, and its deficiency leads to Pompe disease, a rare genetic disorder.

Mnemonic

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

Source

NCBI GeneReviews
Enzymes in Disease Biochemistry Lysosomal Storage Disorders Hard MEDICAL Confidence: high