Glutaconyl-CoA  (Synonyms: Glutaconyl-coenzyme A)

Glutaconyl-CoA (Glutaconyl-coenzyme A) is a key metabolic intermediate in the mitochondrial catabolic pathways of lysine, hydroxylysine and tryptophan. Glutaconyl-CoA competitively inhibits wild-type glutaryl-CoA dehydrogenase (GCDH) with a K_i value of 1.1 µM. Glutaconyl-CoA is generated via the oxidative decarboxylation of glutaryl-CoA catalyzed by GCDH, and is subsequently further metabolized. Glutaconyl-CoA can be used in the research of type I glutaric aciduria^[1].

Glutaconyl-CoA (28-30 µM; 10-30 min) is decarboxylated by Glu370Gln mutant human glutaryl-CoA dehydrogenase to crotonyl-CoA without flavin oxidation-reduction, forming a transient 725 nm-absorbing species and converting 95% of glutaconyl-CoA to crotonyl-CoA within 30 min at pH 7.6, 25 °C^[2].
Glutaconyl-CoA (28 µM; 10 min) is converted by wild-type human glutaryl-CoA dehydrogenase primarily to 3-hydroxybutyryl-CoA (99% product after 10 min) at pH 7.6, 25 °C^[2].
Glutaconyl-CoA (28 µM; 10 min) is converted by Arg94Gln mutant human glutaryl-CoA dehydrogenase primarily to crotonyl-CoA at pH 7.6, 25 °C^[2].

MedChemExpress (MCE) has not independently confirmed the accuracy of these methods. They are for reference only.

Glutaconyl-CoA exhibits significantly reduced glutathione levels in liver and brain of glutaryl-CoA dehydrogenase knock-out mice (a model of glutaric aciduria type I), consistent with the hypothesized toxic interaction of glutaconyl-CoA with sulfhydryl groups^[1].

MedChemExpress (MCE) has not independently confirmed the accuracy of these methods. They are for reference only.

C₂₆H₄₀N₇O₁₉P₃S

167357-83-7

O[C@@H]1[C@H](OP(O)(O)=O)[C@@H](COP(OP(OCC(C)(C)[C@@H](O)C(NCCC(NCCSC(/C=C/CC(O)=O)=O)=O)=O)(O)=O)(O)=O)O[C@H]1N2C=NC3=C(N)N=CN=C32

879.62

Room temperature in continental US; may vary elsewhere.

Please store the product under the recommended conditions in the Certificate of Analysis.

• [1]. Lehnert W, et al. Glutaconyl-CoA is the main toxic agent in glutaryl-CoA dehydrogenase deficiency (glutaric aciduria type I). Med Hypotheses. 2005;65(2):330-333.

  [2]. Westover JB, et al. Binding, hydration, and decarboxylation of the reaction intermediate glutaconyl-coenzyme A by human glutaryl-CoA dehydrogenase. Biochemistry. 2001;40(46):14106-14114.