L-Valine-d8 (Synonyms: (+)-Valine, (S)-Valine) |
| Catalog No.GC49371 |
L-발린-d8(L-VALINE-2,3,4,4,4,5,5,5-d8)은 L-발린의 중수소화된 형태입니다.
Products are for research use only. Not for human use. We do not sell to patients.
Cas No.: 35045-72-8
Sample solution is provided at 25 µL, 10mM.
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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)
Average Rating: 5 (Based on Reviews and 18 reference(s) in Google Scholar.)
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