Glucose-6 Phosphatase deficiency is seen in: (A) Von Gierke disease, (B) Tay sach disease, (C) Pompe disease, (D) Anderson disease
Answer is A. NameEnzyme DeficiencyClinical FeaturesVon Gierke diseaseGlucose-6-phosphataseGlycogen accumulation in liver and renal tubule cells;hypoglycemia; lactic acidemia; ketosis; hyperlipemiaRef: Harper&;s Biochemistry; 30th edition; Chapter 18 Metabolism of Glycogen; Page no: 179