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': 'Enzyme deficient in maple syrup urine disease? \nSelect and explain. \nA. a-ketoacid decarboxylase, B. Transaminase, C. Isomerase, D. Mutase', 'role': 'user'}, {'content': 'Answer: A \nExplanation: Maple syrup urine disease is due to deficiency of a-ketoacid dehydrogenase (also called a-ketoacid decarboxylase). In maple syrup urine disease there is excretion of branched chain amino acids (isoleucine, leucine, valine) and their keto acids (a-keto b-methylvalerate, a-ketoisocaproate, a-ketoisovalerate) in urine.', 'role': 'assistant'}]