产品描述:
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}