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L-Isoleucine-13C6 is intended for use as an internal standard for the quantification of L-isoleucine by GC- or LC-MS. L-Isoleucine is an essential and branched-chain amino acid.1 It is an isomer of L-leucine (Item No. 34342) and a diastereomer of L-alloisoleucine (Item No. 34904) that functions as a nitrogen donor and has roles in glucose consumption, fatty acid metabolism, and immune function.1,2 Urinary levels of L-isoleucine are increased in patients with deficiencies in β-ketothiolase, short/branched-chain specific acyl-CoA dehydrogenase (SBCAD), or 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD), inborn errors of metabolism characterized by episodic ketoacidosis and neurological impairments.3
WARNING This product is not for human or veterinary use.
1. Branched chain amino acids: Beyond nutrition metabolism. Int. J. Mol. Sci. 19(4), 954 (2018).
2. Novel metabolic and physiological functions of branched chain amino acids: A review. J. Anim. Sci. Biotechnol. 8, 10 (2017).
3. Inborn errors of isoleucine degradation: A review. Mol. Genet. Metab. 89(4), 289-299 (2006).