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Literature summary extracted from

  • Luis, P.B.; Ruiter, J.; Ijlst, L.; de Almeida, I.T.; Duran, M.; Wanders, R.J.; Silva, M.F.
    Valproyl-CoA inhibits the activity of ATP- and GTP-dependent succinate CoA ligases (2014), J. Inherit. Metab. Dis., 37, 353-357.
    View publication on PubMed

Inhibitors

EC Number Inhibitors Comment Organism Structure
6.2.1.4 additional information not inhibited by valproic acid Homo sapiens
6.2.1.4 valproyl-CoA 25-50% inhibition at 1 mM Homo sapiens
6.2.1.4 ZnCl2 complete inhibition at 1mM Homo sapiens
6.2.1.5 additional information not inhibited by valproic acid Homo sapiens
6.2.1.5 valproyl-CoA 45-55% inhibition at 1 mM Homo sapiens
6.2.1.5 ZnCl2 complete inhibition at 1mM Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
6.2.1.4 0.63
-
 GTP at pH 8.0 and 37°C Homo sapiens
6.2.1.5 0.25
-
 ATP at pH 8.0 and 37°C Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
6.2.1.4 mitochondrion
-
 Homo sapiens 5739
-
6.2.1.5 mitochondrion
-
 Homo sapiens 5739
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
6.2.1.4 GDP + phosphate + succinyl-CoA Homo sapiens
-
 GTP + succinate + CoA
-
 r
6.2.1.4 GTP + succinate + CoA Homo sapiens
-
 GDP + phosphate + succinyl-CoA
-
 r
6.2.1.5 ADP + phosphate + succinyl-CoA Homo sapiens
-
 ATP + succinate + CoA
-
 r
6.2.1.5 ATP + succinate + CoA Homo sapiens
-
 ADP + phosphate + succinyl-CoA
-
 r

Organism

EC Number Organism UniProt Comment Textmining
6.2.1.4 Homo sapiens
-
-
-
6.2.1.5 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
6.2.1.4 fibroblast
-
 Homo sapiens
-
6.2.1.5 fibroblast
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
6.2.1.4 GDP + phosphate + succinyl-CoA
-
 Homo sapiens GTP + succinate + CoA
-
 r
6.2.1.4 GTP + succinate + CoA
-
 Homo sapiens GDP + phosphate + succinyl-CoA
-
 r
6.2.1.5 ADP + phosphate + succinyl-CoA
-
 Homo sapiens ATP + succinate + CoA
-
 r
6.2.1.5 ATP + succinate + CoA
-
 Homo sapiens ADP + phosphate + succinyl-CoA
-
 r

Synonyms

EC Number Synonyms Comment Organism
6.2.1.4 G-SUCL
-
 Homo sapiens
6.2.1.4 GTP-dependent succinate:CoA ligase
-
 Homo sapiens
6.2.1.4 GTP-specific succinate:CoA ligase
-
 Homo sapiens
6.2.1.5 A-SUCL
-
 Homo sapiens
6.2.1.5 ATP-dependent succinate:CoA ligase
-
 Homo sapiens
6.2.1.5 ATP-specific succinate:CoA ligase
-
 Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
6.2.1.4 GTP
-
 Homo sapiens
6.2.1.5 ATP
-
 Homo sapiens

General Information

EC Number General Information Comment Organism
6.2.1.4 metabolism key role of the enzyme in the Krebs cycle Homo sapiens
6.2.1.5 malfunction inhibition of the enzyme might influence the activity of nucleoside diphosphate kinase inducing an imbalance of nucleotides in the mitochondria and eventually mitochondrial DNA depletion. This may account for the potential liver failure associated with valproate therapy, reported in patients with deficiencies within the mitochondrial DNA replicase system such as polymerase gamma 1 Homo sapiens
6.2.1.5 metabolism key role of the enzyme in the Krebs cycle Homo sapiens
6.2.1.5 physiological function the enzme is bound to the nucleoside diphosphate kinase, which is responsible for the mitochondrial (deoxy)nucleotide synthesis Homo sapiens