Essential Biochemistry - Protein Metabolism (Section 11 - No. 37)
Which metabolic defect causes maple syrup urine disease?
Deficiency in phenylalanine hydroxylase
Defect in branched-chain amino acid metabolism
Deficiency in the urea cycle
Defect in the transport of amino acids
Explanation
Maple syrup urine disease is a defect in branched-chain amino acid metabolism.
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