Which enzyme is responsible for the breakdown of branched-chain amino acids, and its deficiency leads to a condition known as maple syrup urine disease?
Explanation
Maple syrup urine disease is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex, leading to the accumulation of branched-chain amino acids and their toxic byproducts.
Mnemonic
Branched-chain = 'branch' out to multiple diseases
Source
Harper's Biochemistry