This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Metabolism of protein and amino acid, Subject name: Biochemistry
[{'content': 'A 7 day old infant presented with lethargy, decreased feeding, emesis, poor weight gain,hypotonia, a high-pitched cry, seizures, and the characteristic maple syrup smell of the urine.The following features are due to defect in \nSelect and explain. \nA. Oxidaton, B. Deamination, C. Carboxylation, D. Decarboxylation', 'role': 'user'}, {'content': 'Answer: D \nExplanation: Maple syrup urine disease (MSUD)-1 is due to impaired Alpha-Ketoacid decarboxylase component of BCKAD (branched chain alpha keto acid dehydrogenase) complex,leading to defective decarboxylation reaction and the formation of branched-chain amino acids (leucine, isoleucine, and valine) and their toxic byproducts (keto-acids) in blood and urine. A peculiar odor (burnt sugar) is found in urine, sweat, and cerumen. Treatmet:Diet lacking in branched chain amino acids Reference: Harpers illustrated biochemistry 30th edition', 'role': 'assistant'}]