L-Isoleucine 13C6

Biological Activity

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 and a diastereomer of L-alloisoleucine 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

References

1.Nie, C., He, T., Zhang, W., et al.Branched chain amino acids: Beyond nutrition metabolismInt. J. Mol. Sci.19(4)954(2018) 2.Zhang, S., Zeng, X., Ren, M., et al.Novel metabolic and physiological functions of branched chain amino acids: A reviewJ. Anim. Sci. Biotechnol.810(2017) 3.Korman, S.H.Inborn errors of isoleucine degradation: A reviewMol. Genet. Metab.89(4)289-299(2006)