10/28/2023 0 Comments Maple syrup urine disease screening![]() Most BCAA transamination and oxidation occur in the skeletal muscle. The liver and kidney are responsible for the catabolism of 10% to 15% of BCAA. In the brain, BCKAD helps metabolize BCAA to facilitate cerebral GABA and glutamate synthesis. The catabolism of these amino acids is necessary to maintain various physiologic functions such as : The branched-chain amino acids are essential amino acids with hydrophobic side chains and are found in protein-rich food. ![]() ![]() These intermediates are then converted into succinyl-CoA, acetoacetate, and acetyl-CoA. Consequently, alpha-ketoacids are further metabolized into intermediates such as isovaleryl-coenzyme A, alpha-methylbutyryl-CoA, and isobutyrl-CoA. Alpha-ketoacids are then oxidatively decarboxylated by the branched-chain ketoacid dehydrogenase complex. Their respective yielded alpha-ketoacids include alpha-ketoisocaproic acid, alpha-keto-beta-methyl valeric acid, and alpha-ketoisovaleric acid. Therefore, within the mitochondria, branched-chain amino acids are first converted into their respective alpha-ketoacids by the enzyme branched-chain amino acid transaminase. The activity of branched-chain ketoacid dehydrogenase is further regulated by BCKAD phosphatase and BCKAD kinase. The E3 subunit helps reoxidize the lipoic acid residue in E2. The lipoic acid residue in E2 transfers the acyl group from E1 to CoA. In the presence of thiamin pyrophosphate, E1 decarboxylates the alpha ketoacids. Together with branched-chain amino acid transaminase, it helps mediate catabolism of branched-chain amino acids (BCAA). It is composed of three catalytic subunits (E1, E2, and E3). Branched-chain ketoacid dehydrogenase (BCKAD) is located within the inner mitochondrial membrane of various tissues such as skeletal muscle, liver, kidney, and the brain.
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