<prompt> Enzyme deficiency in Von Gierke disease isGlycogen synthaseGlucose-6-phosphataseBranching enzymeMuscle phosphorylase  <response>: 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 