<prompt> The enzyme defect in von Gierke;s disease type-1 glycogen storage disease isGlycogen phosphorylaseDebranching enzymePhosphoglucomutaseGlucose-6-phosphate  <response>: Von Gierke disease:DiseaseEnzyme deficiencyClinical featuresVon Gierke disease Glucose-6-phosphatase deficiencyGlycogen accumulation in liver and renal tubule cells; hypoglycemia; lactic acidemia; ketosis; hyperlipemiaReference: Harper;s Biochemistry; 30th edition; Chapter 18; Metabolism of Glycogen 