Physiological and pathological roles of branched-chain amino acids in the regulation of protein and energy metabolism and neurological functions

Abstract

Branched-chain amino acids (BCAAs: leucine, isoleucine, and valine) are essential amino acids for humans and play an important role as the building blocks of proteins. Recent studies have disclosed that free BCAAs in the tissue amino acid pool function not only as substrates for protein synthesis, but also as regulators of protein and energy metabolism. Furthermore, BCAAs are actively used as an amino group donor to synthesize glutamate in the brain. These functions of BCAAs are closely related to human health. This review summarizes the recent findings concerning physiological and pathological roles of free BCAAs in the metabolism and neurological functions.

Keywords: Branched-chain amino acids; Branched-chain α-keto acid dehydrogenase; Energy metabolism; Glucose metabolism; Neurological development; Protein metabolism.