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Literature summary for 6.2.1.4 extracted from

  • Ostergaard, E.
    Disorders caused by deficiency of succinate-CoA ligase (2008), J. Inherit. Metab. Dis., 31, 226-229.
    View publication on PubMed

Protein Variants

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

Localization Comment Organism GeneOntology No. Textmining
mitochondrial matrix
-
Homo sapiens 5759
-
mitochondrion
-
Homo sapiens 5739
-

Metals/Ions

Metals/Ions Comment Organism Structure
Mg2+
-
Homo sapiens

Natural Substrates/ Products (Substrates)

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

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

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 and Products (Substrate)

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

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

Synonyms Comment Organism
G-SUCL
-
Homo sapiens
GDP-forming SUCL
-
Homo sapiens
succinate-CoA ligase
-
Homo sapiens
SUCL
-
Homo sapiens

Cofactor

Cofactor Comment Organism Structure
GDP
-
Homo sapiens
GTP
-
Homo sapiens

General Information

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