In animal tissue, BCKDC catalyzes an irreversible step[REF] in the catabolism of the branched-chain amino acids L-isoleucine, L-valine, and L-leucine, acting on their deaminated derivatives (L-alpha-keto-beta-methylvalerate, alpha-ketoisovalerate, and alpha-ketoisocaproate, respectively) [REF].
The overall catabolic reaction catalyzed by the BCKDC is shown in Figure 1.