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': 'Maple syrup urine disease is due to \nSelect and explain. \nA. a-ketoacid decarboxylase, B. Transaminase, C. Isomerase, D. Mutase', 'role': 'user'}, {'content': 'Answer: A \nExplanation: Maple syrup urine disease is also known as Branched chain ketonuria. The basic biochemical deformity is deficient decarboxylation of branched-chain keto acid. This is due to the deficiency of alpha-ketoacid decarboxylase.Ref: DM Vasudevan - Textbook of Biochemistry, 7th edition, page no: 230', 'role': 'assistant'}]