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': 'An inherited deficiency of branched chain a keto acid dehydrogenase result in which condition? \nSelect and explain. \nA. Maple syrup urine disease, B. Phenylketonuria, C. Homocystinuria, D. Alcaptonuria', 'role': 'user'}, {'content': 'Answer: A \nExplanation: Maple syrup urine disease is caused by a deficiency of branched-chain a-keto acid dehydrogenase. This enzyme is involved in the catabolism of the branched- chain amino acids leucine, isoleucine, and valine. The sweet-smelling intermediates that accumulate in the urine give the disease its name. Neurologic problems are common in patients with this disease. Phenylketonuria is caused by a deficiency of phenylalanine hydroxylase. Homocystinuria is caused by a deficiency of cystathionine synthetase. A deficiency of homogentisate oxidase results in alcaptonuria, and a lack of histidase causes histidinemia.', 'role': 'assistant'}]