Which enzyme is involved in the degradation of branched-chain amino acids, and its deficiency leads to Maple Syrup Urine Disease?

A Branched-chain alpha-keto acid dehydrogenase complex ✓
B Phenylalanine hydroxylase
C Tyrosinase
D Homogentisate 1,2-dioxygenase

Explanation

Branched-chain alpha-keto acid dehydrogenase complex is responsible for degrading branched-chain amino acids, and its deficiency leads to Maple Syrup Urine Disease.

Mnemonic

Maple = Branch → enzyme branches out to degrade amino acids

Source

Online Mendelian Inheritance in Man (OMIM)
Enzymes in Disease Metabolic Disorders Amino Acid Metabolism Hard MEDICAL Confidence: high