This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Metabolism of carbohydrate, Subject name: Biochemistry
[{'content': 'Branched-chain ketoaciduria is due to deficiency of \nSelect and explain. \nA. Carboxylase, B. a-ketoacid dehydrogenase, C. Methyl-malonyl CoA mutase, D. Fumaryacetoacetate hydrolase', 'role': 'user'}, {'content': 'Answer: B \nExplanation: Maple syrup urine disease (MSUD), also called branched-chain ketoaciduria, is an autosomal recessive metabolic disorder affecting branched-chain amino acids. It is one type of organic acidemia. The condition gets its name from the distinctive sweet odor of affected infants&; urine, paicularly prior to diagnosis, and during times of acute illness MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine.', 'role': 'assistant'}]