A branched-chain acyl CoA and intermediate in L-leucine metabolism
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Isovaleryl-Coenzyme A (sodium salt)

Item No. 39999

Product Insert (PDF)Safety Data Sheet (SDS) (PDF)
Technical Information
Formal Name
S-(3-methylbutanoate) coenzyme A, sodium salt
Synonyms
  • Isovaleryl-CoA
Molecular Formula
C26H44N7O17P3S • XNa
Formula Weight
851.7
Purity
≥95%
Formulation
A solid
Solubility(Learn about Variance in Solubility)
Water: Soluble
SMILES
O[C@H]([C@@H]1O[P](O)(O)=O)[C@@H](O[C@@H]1CO[P](O[P](OCC(C)(C)[C@@H](O)C(NCCC(NCCSC(CC(C)C)=O)=O)=O)(O)=O)(O)=O)N2C3=NC=NC(N)=C3N=C2.[Na]
InChi Code
InChI=1S/C26H44N7O17P3S.Na/c1-14(2)9-17(35)54-8-7-28-16(34)5-6-29-24(38)21(37)26(3,4)11-47-53(44,45)50-52(42,43)46-10-15-20(49-51(39,40)41)19(36)25(48-15)33-13-32-18-22(27)30-12-31-23(18)33;/h12-15,19-21,25,36-37H,5-11H2,1-4H3,(H,28,34)(H,29,38)(H,42,43)(H,44,45)(H2,27,30,31)(H2,39,40,41);/t15-,19-,20-,21+,25-;/m1./s1
InChi Key
MIOHTOXKBKMCMT-WADKRTIVSA-N
Shipping & Storage Information
Storage
-20°C
Shipping
Wet ice in continental US; may vary elsewhere
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    1 mg

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    Product 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).5

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Duan, Y., Li, F., Li, Y., et al. The role of leucine and its metabolites in protein and energy metabolism. Amino Acids 48(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 acidemia. Biochemistry 37(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 metabolism. FEBS 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 mitochondria. Int. 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 mitochondria. Biochem. Med. 27(2), 154-160 (1982).