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Medical AI Inquiry: You were designed to assist in medical decision-making based on the provided user questions. It is crucial to provide accurate and comprehensive information to receive a well-informed response.
user
Enzyme deficient in maple syrup urine disease?
assistant
The correct answer is  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.