What Does BCKD Stand For?

BCKD stands for Branched Chain Alpha Ketoacid Dehydrogenase

BCKD, or Branched Chain Alpha Ketoacid Dehydrogenase, is a crucial enzyme complex involved in the catabolism of branched-chain amino acids, specifically leucine, isoleucine, and valine. This multi-enzyme complex facilitates the decarboxylation of branched-chain alpha-keto acids, converting them into acyl-CoA derivatives for further energy production and metabolic pathways. Proper function of BCKD is essential for maintaining amino acid homeostasis and energy balance, and its deficiencies are associated with metabolic disorders, notably maple syrup urine disease.