Formulation: A solid
Formal Name: S-(3-methylbutanoate) coenzyme A, sodium salt
Purity: ≥95%
Formula Markup: C26H44N7O17P3S • XNa
Formula Weight: 851,7
Shelf life (days): 1460
Notes: Isovaleryl-coenzyme A (isovaleryl-CoA) is a branched-chain acyl CoA and an intermediate in the metabolism of the essential amino acid L-leucine (Item No. 34342).{57686,67716} It is formed from L-leucine by the branched-chain α-keto acid dehydrogenase (BCKAD) complex and is converted to 3-methylcrotonyl-CoA by isovaleryl-CoA dehydrogenase (IVD), an enzyme involved in the inborn error of metabolism isovaleric acidemia.{58549} Isovaleryl-CoA increases the oxidation rate of the branched-chain 2-oxo acids 3-methyl-2-butanoate and 4-methyl-2-oxopentanoate in isolated rat muscle mitochondria in the absence of carnitine when used at a concentration of 1 mM.{55274} It also inhibits succinate-CoA ligase activity in rat liver mitochondria (IC50 = 273 µM).{67717}