Von Geirke's occurs due to deficiency of -
Ans. is 'a' i.e., Glucose-6-phosphate (19-2)o Glucose - 6- phosphatase is deficient in Von Gierke's disease.Type Enzyme deficiencyOrgan (s) affected1von Gierke's diseaseGlucose 6-phosphataseLiver, kidney11Pompe's diseasea (1 - 4) Glucosidase (acid maltase)All organsIIICori's disease/Forbe's diseaseDebranching enzymeMuscle, liverIVAndersen's diseaseBranching enzymeLiver, myocardiumVMcArdle's diseasePhosphorylaseMuscleVIHers' diseasePhosphorylaseLiverVIITarui's diseasePhosphofructokinaseMuscle, RBCsVIII Phosphorylase kinaseLivero There is also on X-ltnked form of phosphorylase kinase deficiency. This is sole exception as all other glycogen storage diseases are inherited as autosomal recessive trait.
Glucose-6-phosphatase
Liver Phosphoryiase
Muscle phosphoryiase
Debranching enzyme
Glycogen Metabolism and Glycogen Storage