Biochemistry The enzyme defect in von Gierke&;s disease (type-1 glycogen storage disease) is A Glycogen phosphorylase B Debranching enzyme C Phosphoglucomutase D Glucose-6-phosphate 💡 Explanation The enzyme defect in von Gierke&;s disease (type-1 glycogen storage disease) is ✓ Correct Answer: D. Glucose-6-phosphate