A patient with a diagnosis of maple syrup urine disease is most likely to have a deficiency of which of the following enzymes?
Explanation
Maple syrup urine disease is a rare genetic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex. This enzyme is necessary for the breakdown of certain amino acids, and its deficiency leads to the accumulation of toxic compounds that can cause serious health problems.
Source
Nelson Textbook of Pediatrics











