What is the primary enzyme responsible for the breakdown of branched-chain amino acids, 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 Homogentisate 1,2-Dioxygenase
The enzyme Branched-Chain Alpha-Keto Acid Dehydrogenase is responsible for the breakdown of branched-chain amino acids. A deficiency in this enzyme leads to Maple Syrup Urine Disease, characterized by the accumulation of these amino acids and their toxic byproducts.
NCERT Class 12 Biochemistry
Enzymes in Disease Biochemistry Amino Acid Metabolism Disorders Medium MEDICAL Confidence: high