<prompt> 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:Alpha amylaseBranching enzymeDebranching enzymeGlucose-6-phosphatase  <response>: Disease is Andersons diseaseAmylopectinosis 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-Gierkes disease Stored glycogen-Normal 