All are lysosomal disorders, except
High-Yield Explanation
Enzyme deficiencies of :Gaucher's disease - Beta-glucosidaseNiemann-Pick disease - SphingomyelinaseTay-Sachs disease - Hexosaminidase AGlycogen storage disease, Type l (von Gierke&;s disease)Enzyme-deficient-Glucose-6-phosphataseChromosome location-17Salient features-Hepatomegaly, cirrhosis, hypoglycemia, ketosis, hyperuricemiaRef: D M Vasudevan 7th edition Page no: 144, 430