This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Metabolism of carbohydrate, Subject name: Biochemistry
Enzyme deficiency in Von Gierke disease is 
Select and explain. 
A. Glycogen synthase, B. Glucose-6-phosphatase, C. Branching enzyme, D. Muscle phosphorylase
B: Glucose-6-phosphatase
Answer: B: Glucose-6-phosphatase 
Explanation: Type Name Enzyme Deficiency Clinical Features Ia Von Gierke disease Glucose-6-phosphatase Glycogen accumulation in liver and renal tubule cells Hypoglycemia Lactic acidemia Ketosis Hyperlipidemia Reference: Harper; 30th edition; Table: 18-2; Page no: 179