Whole-body metabolic fate of branched-chain amino acids

Oxidation of branched-chain amino acids (BCAAs) is tightly regulated in mammals. We review here the distribution and regulation of whole-body BCAA oxidation. Phosphorylation and dephosphorylation of the rate-limiting enzyme, branched-chain alpha ketoacid dehydrogenase complex directly regulates BCAA oxidation, and various other indirect mechanisms of regulation also exist. Most tissues throughout the body are capable of BCAA oxidation, and the flux of oxidative BCAA disposal in each tissue is influenced by three key factors: 1. tissue-specific preference for BCAA oxidation relative to other fuels, 2. the overall oxidative activity of mitochondria within a tissue, and 3. total tissue mass. Perturbations in BCAA oxidation have been implicated in many disease contexts, underscoring the importance of BCAA homeostasis in overall health.

Automated summary

The oxidation of branched-chain amino acids (BCAAs) is tightly regulated in mammals via direct and indirect mechanisms. Most tissues are capable of BCAA oxidation, influenced by tissue-specific preference, mitochondria activity, and total tissue mass. Perturbations in BCAA oxidation have been linked to various diseases, highlighting the importance of BCAA homeostasis in overall health.