Which enzyme is involved in the degradation of branched-chain amino acids, and what disease is caused by its deficiency?

A Branched-chain alpha-keto acid dehydrogenase complex and Maple Syrup Urine Disease ✓
B Phenylalanine hydroxylase and Phenylketonuria
C Tyrosinase and Albinism
D Galactose-1-phosphate uridyltransferase and Galactosemia

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 the accumulation of these amino acids, causing Maple Syrup Urine Disease.

Source

NCERT Class 12 Biochemistry
Enzymes in Disease Biochemistry Amino Acid Metabolism Disorders Hard MEDICAL Confidence: high