L-缬氨酸-d8 (L-VALINE-2,3,4,4,4,5,5,5-d8) 是 L-缬氨酸的氘代形式。
货号:ajcx25698
CAS:35045-72-8
分子式:C5H3D8NO2
分子量：125.2
溶解度:PBS (pH 7.2): 1 mg/ml
纯度：98%
存储：Store at -20°C
库存：现货

Background:

L-Valine-d₈is 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 obtainedviathe diet.¹It is considered glucogenic due to its catabolism that ultimately forms succinyl-CoA, which enters the citric acid cycle.²A 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)