Description
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).1,2 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.3 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.4 It also inhibits succinate-CoA ligase activity in rat liver mitochondria (IC50 = 273 µM).5WARNING This product is not for human or veterinary use.
References
[1] YEHUI DUAN. The role of leucine and its metabolites in protein and energy metabolism[J]. Amino Acids, 2015, 48 1: 41-51. DOI:
10.1007/s00726-015-2067-1[2] AL-WALID A. MOHSEN. Characterization of Molecular Defects in Isovaleryl-CoA Dehydrogenase in Patients with Isovaleric Acidemia†[J]. Biochemistry Biochemistry, 1998, 37 28: 10325-10335. DOI:
10.1021/bi973096r[3] MATTHIAS MACK. Biochemical characterization of human 3-methylglutaconyl-CoA hydratase and its role in leucine metabolism[J]. The FEBS journal, 2006, 273 9: 2012-2022. DOI:
10.1111/j.1742-4658.2006.05218.x[4] J.H. VEERKAMP A. J M W H T B van Moerkerk. Interaction of short-chain and branched-chain fatty acids and their carnitine and CoA esters and of various metabolites and agents with branched-chain 2-oxo acid oxidation in rat muscle and liver mitochondria[J]. International Journal of Biochemistry, 1985, 17 9: Pages 967-974. DOI:
10.1016/0020-711x(85)90241-1[5] BARRY J. BERGEN . A mechanism of toxicity of isovaleric acid in rat liver mitochondria[J]. Biochemical medicine, 1982, 27 2: Pages 154-160. DOI:
10.1016/0006-2944(82)90017-5
