產品屬性：

Cas No.: 35045-72-8

別名: N/A

化學名: N/A

分子式: C5H3D8NO2

分子量: 125.2

溶解度: PBS (pH 7.2): 1 mg/ml

儲存條件: -20°C
General tipsFor obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.

Shipping ConditionEvaluation sample solution : ship with blue ice

All other available size: ship with RT , or blue ice upon request

產品描述：

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.1,31.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)
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