In maple syrup urine disease the amino acids excreted in the urine are______________?

Correct answer: D. All of the above

  • A. Leucine
  • B. Isoleucine
  • C. Valine
  • D. All of the above

Explanation

Maple syrup urine disease results from deficiency of the branched-chain alpha-ketoacid dehydrogenase complex, causing leucine, isoleucine and valine to accumulate and be excreted. Therefore, all three listed amino acids are involved.

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