Which of the following enzymes is involved in the degradation of branched-chain amino acids, and its deficiency leads to a condition called maple syrup urine disease?
Explanation
The branched-chain ketoacid dehydrogenase complex is involved in the degradation of branched-chain amino acids. Its deficiency leads to maple syrup urine disease, a condition characterized by a distinctive sweet odor of the urine.
Source
Lehninger Principles of Biochemistry