Essential Biochemistry - Protein Metabolism (Section 2 - No. 41)
Which of the following is NOT a characteristic of maple syrup urine disease?
A deficiency in branched-chain alpha-keto acid dehydrogenase
Sweet-smelling urine
Accumulation of branched-chain amino acids
Excessive levels of cystine in the blood
Explanation
Maple syrup urine disease is not characterized by increased levels of cystine.
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