Protein Variants | Comment | Organism |
---|---|---|
additional information | the severe disorder in patients with SUCLG1 mutations is likely caused by the absence of both ASUCL and G-SUCL, and thereby a compromised formation of both ATP and GTP. Severe lactic acidosis is found in patients with SUCLG1 mutations, phenotype, overview | Homo sapiens |
additional information | naturally occuring mutation c.113_114delAT causes succinate-CoA ligase deficiency | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrial matrix | - |
Homo sapiens | 5759 | - |
mitochondrion | - |
Homo sapiens | 5739 | - |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Mg2+ | - |
Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
GTP + succinate + CoA | Homo sapiens | - |
GDP + phosphate + succinyl-CoA | - |
? | |
GTP + succinate + CoA | Homo sapiens | - |
GDP + phosphate + succinyl-CoA | - |
r | |
additional information | Homo sapiens | succinate-CoA ligase catalyses the reversible conversion of succinyl-CoA and ADP or GDP to succinate and ATP or GTP, cf. ATP-specific succinate:CoA ligase, EC 6.2.1.5 | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
gene SUCLG1 encodes the alpha-subunit and SUCLG2 encodes the catalytic beta-subunit | - |
Homo sapiens | P53597 | alpha-subunit | - |
Homo sapiens | Q96I99 | beta-subunit | - |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
fibroblast | - |
Homo sapiens | - |
kidney | high level | Homo sapiens | - |
liver | - |
Homo sapiens | - |
liver | high level | Homo sapiens | - |
additional information | the enzyme is preferably expressed in anabolic tissues | Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
GTP + succinate + CoA | - |
Homo sapiens | GDP + phosphate + succinyl-CoA | - |
? | |
GTP + succinate + CoA | - |
Homo sapiens | GDP + phosphate + succinyl-CoA | - |
r | |
additional information | succinate-CoA ligase catalyses the reversible conversion of succinyl-CoA and ADP or GDP to succinate and ATP or GTP, cf. ATP-specific succinate:CoA ligase, EC 6.2.1.5 | Homo sapiens | ? | - |
? |
Subunits | Comment | Organism |
---|---|---|
heterodimer | the enzyme is composed of an alpha subunit, encoded by SUCLG1, and a beta subunit, encoded by either SUCLA2 or SUCLG2. The alpha-subunit forms a heterodimer with either of its beta-subunits, resulting in an ADP-forming succinate-CoA ligase, EC 6.2.1.5, and a GDP-forming succinate-CoA ligase, EC 6.2.1.4, respectively | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
G-SUCL | - |
Homo sapiens |
GDP-forming SUCL | - |
Homo sapiens |
succinate-CoA ligase | - |
Homo sapiens |
SUCL | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
GDP | - |
Homo sapiens | |
GTP | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | a patient with a SUCLG1 mutation shows steatosis in liver histology and severe lactic acidosis, one patient shows combined deficiency of respiratory chain complexes I, III and IV in liver. The accumulated succinyl-CoA inhibits the reaction catalysed by methylmalonyl-CoA mutase or causes an equilibrium shift. mtDNA depletion in succinate-CoA ligase deficiency | Homo sapiens |
malfunction | a patient with a SUCLG1 mutation shows steatosis in liver hstology and severe lactic acidosis, one patient shows combined deficiency of respiratory chain complexes I, III and IV in liver. The accumulated succinyl-CoA inhibits the reaction catalysed by methylmalonyl-CoA mutase or causes an equilibrium shift. mtDNA depletion in succinate-CoA ligase deficiency | Homo sapiens |