What is the primary enzyme responsible for the degradation 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 Dihydrolipoyl Transacetylase
C Isocitrate Dehydrogenase
D Pyruvate Dehydrogenase

Explanation

The primary enzyme responsible for the degradation of branched-chain amino acids is Branched-Chain Alpha-Keto Acid Dehydrogenase. Deficiency of this enzyme leads to Maple Syrup Urine Disease, characterized by the accumulation of branched-chain amino acids and their toxic byproducts.

Source

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