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Isovaleryl-Coenzyme A (sodium salt)

(Synonyms: Isovaleryl-CoA) 目录号 : GC91817

Isovaleryl-coenzyme A (isovaleryl-CoA) is a branched-chain acyl CoA and an intermediate in the metabolism of the essential amino acid L-leucine .

Isovaleryl-Coenzyme A (sodium salt) Chemical Structure

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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 .[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).[5]

References:
[1].Duan, Y., Li, F., Li, Y., et al.The role of leucine and its metabolites in protein and energy metabolismAmino Acids48(1)41-51(2016).
[2].Mohsen, A.-W.A., Anderson, B.D., Volchenboum, S.L., et al.Characterization of molecular defects in isovaleryl-CoA dehydrogenase in patients with isovaleric acidemiaBiochemistry37(28)10325-10335(1998).
[3].Mack, M., Schniegler-Mattox, U., Peters, V., et al.Biochemical characterization of human 3-methylglutaconyl-CoA hydratase and its role in leucine metabolismFEBS J.273(9)2012-2022(2006).
[4].Veerkamp, J.H., van Moerkerk, H.T.B., and Wagenmakers, A.J.M.Interaction of short-chain and branched-chain fatty acids and their carnitine and CoA esters and of various metabolites and agents with branced-chain 2-oxo acid oxidation in rat muscle and liver mitochondriaInt. J. Biochem.17(9)967-974(1985).
[5].Bergen, B.J., Stumpf, D.A., Haas, R., et al.A mechanism of toxicity of isovaleric acid in rat liver mitochondriaBiochem. Med.27(2)154-160(1982).

化学性质

Cas No. N/A SDF Download SDF
别名 Isovaleryl-CoA
分子式 C26H44N7O17P3S•XNa 分子量 851.7
溶解度 Water: Soluble 储存条件 -20°C
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溶解性数据

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1 mg 5 mg 10 mg
1 mM 1.1741 mL 5.8706 mL 11.7412 mL
5 mM 0.2348 mL 1.1741 mL 2.3482 mL
10 mM 0.1174 mL 0.5871 mL 1.1741 mL
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