The catabolism of isoleucine initiates in muscle and yields NADH and FADH2 which can be utilized for ATP generation. The catabolism of amino acids uses the same enzymes in the first two steps. The first step in each case is a transamination using a single BCAA aminotransferase, with a-ketoglutarate as amine acceptor. As a result, three different a-keto acids are produced and are oxidized using a common branched-chain a-keto acid dehydrogenase, yielding the three different CoA derivatives. Subsequently the metabolic pathways diverge, producing many intermediates.
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L-Isoleucine is used in the treatment of nutritional deficiencies.