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Literature summary extracted from

  • Huang, X.; Bedoyan, J.K.; Demirbas, D.; Harris, D.J.; Miron, A.; Edelheit, S.; Grahame, G.; DeBrosse, S.D.; Wong, L.J.; Hoppel, C.L.; Kerr, D.S.; Anselm, I.; Berry, G.T.
    Succinyl-CoA synthetase (SUCLA2) deficiency in two siblings with impaired activity of other mitochondrial oxidative enzymes in skeletal muscle without mitochondrial DNA depletion (2017), Mol. Genet. Metab., 120, 213-222 .
    View publication on PubMedView publication on EuropePMC

Protein Variants

EC Number Protein Variants Comment Organism
6.2.1.5 A307V the mutation is associated with succinyl-CoA ligase (ATP-forming) or succinyl-CoA synthetase (ADP-forming) deficiency Homo sapiens
6.2.1.5 M329V the mutation is associated with succinyl-CoA ligase (ATP-forming) or succinyl-CoA synthetase (ADP-forming) deficiency Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
6.2.1.5 1.33
-
succinate at pH 8.0 and 37°C Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
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
6.2.1.5 Homo sapiens Q9P2R7 subunit beta
-

Source Tissue

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

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
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
6.2.1.5 A-SCS
-
Homo sapiens
6.2.1.5 ATP-SCS
-
Homo sapiens
6.2.1.5 ATP-succinyl-CoA synthetase
-
Homo sapiens
6.2.1.5 succinyl-CoA ligase (ATP-forming)
-
Homo sapiens
6.2.1.5 Succinyl-CoA synthetase (ADP-forming)
-
Homo sapiens
6.2.1.5 SUCLA2
-
Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
6.2.1.5 ADP
-
Homo sapiens
6.2.1.5 ATP
-
Homo sapiens

General Information

EC Number General Information Comment Organism
6.2.1.5 malfunction enzyme mutations result in succinyl-CoA ligase (ATP-forming) or succinyl-CoA synthetase (ADP-forming) deficiency, a mitochondrial tricarboxylic acid cycle disorder Homo sapiens