A 3-month-old infant presents with hepatosplenomegaly and failure to thrive. A liver biopsy reveals glycogen with an abnormal, amylopectin like structure with long outer chains and missing branches. Which of the following enzymes would most likely be deficient:
Disease is Anderson's disease/Amylopectinosis (GSD Type IV) Branching enzyme will be deficient Enzyme deficient Disease Features Branching enzyme Amylopectinosis Stored glycogen is abnormal long polysaccharide chains with few branch points resembles - Amylopectin Debranching enzyme Limit dextrinosis Limit dextrins (glycogen remains having shoer linear chains with very sho branch point)-accumulated Glucose-6-phosphatase Von-Gierke's disease Stored glycogen-Normal
Alpha amylase
Branching enzyme
Debranching enzyme
Glucose-6-phosphatase
Glycogen