Which enzyme is involved in the breakdown of branched-chain amino acids, and its deficiency leads to a condition known as maple syrup urine disease?
Explanation
The branched-chain alpha-keto acid dehydrogenase complex is an enzyme involved in the breakdown of branched-chain amino acids. A deficiency in this enzyme leads to maple syrup urine disease, a condition characterized by the accumulation of toxic compounds in the body.
Source
Clinical Biochemistry textbook