Branched-Chain Amino Acid Metabolism Disorders By Matt Demczko, MD, Mitochondrial Medicine, Children's Hospital of Philadelphia Reviewed / Revised Oct 2021 | Modified Sep 2022 View Patient Education More Information Topic Resources Branched-Chain Amino Acid *... Valine, leucine, and isoleucine are branched-chain amino acids; deficiency of enzymes involved in their metabolism leads to accumulation of organic acids with severe metabolic acidosis.