L-Valine-d8 is intended for use as an internal standard for the quantification of L-valine by GC- or LC-MS. L-Valine is a branched-chain essential amino acid that is obtained via the diet.1 It is considered glucogenic due to its catabolism that ultimately forms succinyl-CoA, which enters the citric acid cycle.2 A deficiency in branched-chain dehydrogenase, the enzyme that catabolizes valine, isoleucine, and leucine, leads to maple syrup urine disease, a disorder characterized by progressive ketoacidosis, failure to thrive, neurological dysfunction, and, potentially, death.1,3
1.Berg, J.M., Tymoczko, J.L., and Stryer, L.Biochemistry(2002) 2.Massey, K.A., Blakeslee, C.H., and Pitkow, H.S.A review of physiological and metabolic effects of essential amino acidsAmino Acids14(4)271-300(1998) 3.Farias, H.R., Gabriel, J.R., Cecconi, M.L., et al.The metabolic effect of α-ketoisocaproic acid: In vivo and in vitro studiesMetab. Brain Dis.36(1)185-192(2021)