What is the primary enzyme responsible for the breakdown of branched-chain amino acids in the human body, and its deficiency leads to a condition known as Maple Syrup Urine Disease?

A Branched-Chain Alpha-Keto Acid Dehydrogenase ✓
B Phenylalanine Hydroxylase
C Tyrosinase
D Lactate Dehydrogenase

Explanation

Branched-Chain Alpha-Keto Acid Dehydrogenase is the enzyme responsible for breaking down 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

Harper's Biochemistry
Enzymes in Disease Biochemistry Amino Acid Metabolism Disorders Hard MEDICAL Confidence: high