Protein Variants | Comment | Organism |
---|---|---|
additional information | patients with SUCLA2 mutations are expected to retain the activity of G-SUCL, and only the ATP production is expected to be compromised. In addition, in patients with SUCLA2 mutations, no liver symptoms are reported. The lactate level in patients with SUCLA2 mutations is normal or moderately elevated, phenotype, overview | Homo sapiens |
additional information | naturally occuring mutations g.32720del43ins5 and IVS4+1G>A cause 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. |
---|---|---|---|---|---|---|
ATP + succinate + CoA | Homo sapiens | - |
ADP + phosphate + succinyl-CoA | - |
r | |
ATP + succinate + CoA | Homo sapiens | the enzyme is part of the Krebs cycle | ADP + phosphate + succinyl-CoA | - |
? | |
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. GTP-specific succinate:CoA ligase, EC 6.2.1.4 | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
gene SUCLA1 encodes the alpha-subunit and gene SUCLA2 encodes the catalytic beta-subunit | - |
Homo sapiens | P53597 | alpha-subunit | - |
Homo sapiens | Q9P2R7 | beta-subunit | - |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
brain | - |
Homo sapiens | - |
brain | high level | Homo sapiens | - |
fibroblast | - |
Homo sapiens | - |
heart | high level | Homo sapiens | - |
additional information | the enzyme is preferably expressed in catabolic tissues | Homo sapiens | - |
skeletal muscle | - |
Homo sapiens | - |
skeletal muscle | high level | Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
ATP + succinate + CoA | - |
Homo sapiens | ADP + phosphate + succinyl-CoA | - |
? | |
ATP + succinate + CoA | - |
Homo sapiens | ADP + phosphate + succinyl-CoA | - |
r | |
ATP + succinate + CoA | the enzyme is part of the Krebs cycle | Homo sapiens | ADP + phosphate + succinyl-CoA | - |
? | |
additional information | succinate-CoA ligase catalyses the reversible conversion of succinyl-CoA and ADP or GDP to succinate and ATP or GTP, cf. GTP-specific succinate:CoA ligase, EC 6.2.1.4 | 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 |
---|---|---|
A-SUCL | - |
Homo sapiens |
ADP-forming SUCL | - |
Homo sapiens |
succinate-CoA ligase | - |
Homo sapiens |
SUCL | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
ADP | - |
Homo sapiens | |
ATP | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | in patients with enzyme mutations, muscle histology shows severe lipid accumulation, and a marked type I fibre predominance is found, with an increased variability in fibre diameter. mtDNA depletion in succinate-CoA ligase deficiency | Homo sapiens |