The enzyme defect in von Gierke&;s disease (type-1 glycogen storage disease) is
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
Glycogen phosphorylase
Debranching enzyme
Phosphoglucomutase
Glucose-6-phosphate
Endocrinology