Protein Variants | Comment | Organism |
---|---|---|
A307V | the mutation is associated with succinyl-CoA ligase (ATP-forming) or succinyl-CoA synthetase (ADP-forming) deficiency | Homo sapiens |
M329V | the mutation is associated with succinyl-CoA ligase (ATP-forming) or succinyl-CoA synthetase (ADP-forming) deficiency | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
1.33 | - |
succinate | at pH 8.0 and 37°C | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
ADP + phosphate + succinyl-CoA | Homo sapiens | - |
ATP + succinate + CoA | - |
r | |
ATP + succinate + CoA | Homo sapiens | - |
ADP + phosphate + succinyl-CoA | - |
r |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | Q9P2R7 | subunit beta | - |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
fibroblast | - |
Homo sapiens | - |
skeletal muscle | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
ADP + phosphate + succinyl-CoA | - |
Homo sapiens | ATP + succinate + CoA | - |
r | |
ATP + succinate + CoA | - |
Homo sapiens | ADP + phosphate + succinyl-CoA | - |
r |
Synonyms | Comment | Organism |
---|---|---|
A-SCS | - |
Homo sapiens |
ATP-SCS | - |
Homo sapiens |
ATP-succinyl-CoA synthetase | - |
Homo sapiens |
succinyl-CoA ligase (ATP-forming) | - |
Homo sapiens |
Succinyl-CoA synthetase (ADP-forming) | - |
Homo sapiens |
SUCLA2 | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
ADP | - |
Homo sapiens | |
ATP | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
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 |