Enzyme deficiency in Von Gierke disease is
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
Glycogen synthase
Glucose-6-phosphatase
Branching enzyme
Muscle phosphorylase
Metabolism of carbohydrate