Which of the following enzymes is involved in the degradation of branched-chain amino acids?
Explanation
The branched-chain ketoacid dehydrogenase complex is a mitochondrial enzyme complex that catalyzes the degradation of branched-chain amino acids (leucine, isoleucine, and valine). Deficiency of this enzyme complex leads to maple syrup urine disease.
Source
Scriver's The Metabolic and Molecular Bases of Inherited Disease