This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: None, Subject name: Biochemistry
[{'content': 'Branched chain ketoacid decarboxylation is defective in \nSelect and explain. \nA. Maple Syrup urine disease, B. Hartnup disease, C. Alkaptonuria, D. GMI Gangliosidosis', 'role': 'user'}, {'content': 'Answer: A \nExplanation: Maple syrup urine disease is due to deficiency of the enzyme branched chain α-keto dehydrogenase which catalyzes decarboxylation of branched - chain amino acid.', 'role': 'assistant'}]