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

The branched-chain alpha-keto acid dehydrogenase complex is an enzyme involved in the degradation of branched-chain amino acids. Its deficiency leads to Maple Syrup Urine Disease, a condition characterized by the accumulation of these amino acids and their toxic byproducts.

Source

OMIM database
Enzymes in Disease Biochemistry Amino Acid Metabolism Disorders Hard MEDICAL Confidence: high