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ACBN Nutritionist Exam Prep

Category - ACBN

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Maple-syrup urine disease (MSUD) is an autosomal recessive disorder in infants caused by:
  1. The inability to metabolize branched-chain amino acids
  2. Plasma leucine levels exceeding 10 mg/dl
  3. A defect in the gene that controls the activity of alpha-keto acid hydrogenase
  4. A & C
Explanation
Answer: D - Maple-syrup urine disease (MSUD) in an autosomal recessive disorder in infants caused by the inability of the body to metabolize the branched-chain amino acids leucine, isoleucine, and valine. The inability to break down branched-chain amino acids is due to a mutation in the gene that controls the activity of the enzyme alpha-keto acid dehyrogenase. Plasma levels of leucine exceeding 10 mg/dl are associated with the symptoms of MSUD, including lethargy, vomiting, and neurologic changes.
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