This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Proteins and Amino Acids, Subject name: Biochemistry
[{'content': 'Enzyme deficient in maple syrup urine disease: \nSelect and explain. \nA. Branched chain alpha keto acid decarboxylase, B. Methionine adenosyl transferase, C. Fumarylacetoacetate hydrolase, D. Tyrosine aminotransferase', 'role': 'user'}, {'content': 'Answer: A \nExplanation: Ans. A Branched chain alpha keto acid decarboxylase* Maple syrup urine disease is an inherited disorder characterized by the deficiency of branched-chain alpha- keto acid decarboxylase complex.* This enzyme is required for breakdown of specific amino acid in body like valine, isoleucine and leucine.* Due to the enzyme deficiency, these amino acid is not metabolized and hence there is accumulation in cells and body fluids - producing burnt sugar/maple syrup smelling urine.', 'role': 'assistant'}]