L-Valine-d<sub>8</sub>

L-Valine-d8

CAT N°: 34830
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From 74.00 62.90

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.{54328} It is considered glucogenic due to its catabolism that ultimately forms succinyl-CoA, which enters the citric acid cycle.{45990} 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.{54328,60681}

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