Glucose 6-phosphatase deficiency occurs in
(Von Gierke's disease) (269- U.S 3rd)GLYCOGEN-STORAGE DISEASETypeNAMEENZYME-DEFECTI* Von Gierke's disease (type I glycogenosis)Glucose-6 phosphataseII*Pompe's diseaseLysosomal a-1, 4 glucosidase (acid maltase)IIICori's disease (limit dextrinosis, Forbe's disease)Amylo a-1, 6- glucosidase (debranching enzyme)IVAnderson's disease (amylopectinosis)Glucosyl 4-6 transferase (branching enzyme)VMe Ardle's disease (type V glycogenosis)Muscle glycogen phosphorylaseVIHer's diseaseLiver glycogen phosphorylaseVIITarul's diseasePhospho fructokinase SPHINGOMYELINSCEREBROSIDES1.Niemann-Pick disease - defect in enzyme sphingomyelinase1.Gaucher's disease - due to b-glucosidase defect2.Faber's disease - defect in enzyme ceramidase2.Krabbe's disease - due to b-galactosidase enzyme defect
Gaucher's disease
Von Gierke's disease
Pompe's disease
Hurler's disease
Carbohydrates