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Literature summary for 6.2.1.5 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 (2013), J. Inherit. Metab. Dis., 37, 353-357.
    View publication on PubMed
Search for more literature for 6.2.1.5

Inhibitors

Inhibitors Comment Organism Structure
additional information no inhibition by valproic acid Homo sapiens
valproyl-CoA 45-55% inhibition at 1 mM Homo sapiens
zinc chloride
-
 Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
 Homo sapiens 5739
-

Natural Substrates/ Products (Substrates)

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + succinate + CoA
-
 Homo sapiens ADP + phosphate + succinyl-CoA
-
 r

Synonyms

Synonyms Comment Organism
ATP-specific succinate:CoA ligase
-
 Homo sapiens
ATP-specific SUCL
-
 Homo sapiens

Cofactor

Cofactor Comment Organism Structure
ADP
-
 Homo sapiens
ATP
-
 Homo sapiens

General Information

General Information Comment Organism
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
metabolism key role of the enzyme in the Krebs cycle Homo sapiens
physiological function the enzme is bound to the nucleoside diphosphate kinase, which is responsible for the mitochondrial (deoxy)nucleotide synthesis Homo sapiens