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Isoleucine is a mixture of the diastereomers L-isoleucine, L-alloisoleucine, D-isoleucine, and D-alloisoleucine. L-Isoleucine is an essential branched-chain amino acid that acts as a nitrogen donor and has roles in glucose consumption, fatty acid metabolism, and immune function.1,2,3 L-Alloisoleucine is formed from L-isoleucine by transamination, and plasma levels of L-alloisoleucine are increased in patients with maple syrup urine disease (MSUD), an inborn error of metabolism characterized by branched-chain a-keto acid dehydrogenase (BCKAD) deficiency.4,5 D-Isoleucine and D-alloisoleucine are enantiomers of L-isoleucine and L-alloisoleucine, respectively.
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1. Branched-
2. Branched chain amino acids: Beyond nutrition metabolism. Int. J. Mol. Sci. 19(4), 954 (2018).
3. Novel metabolic and physiological functions of branched chain amino acids: A review. J. Anim. Sci. Biotechnol. 8, 10 (2017).
4. Formation of L-
5. Significance of ʟ-