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Literature summary for 6.2.1.5 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 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

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.
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

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

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 and Products (Substrate)

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

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
A-SUCL
-
Homo sapiens
ADP-forming SUCL
-
Homo sapiens
succinate-CoA ligase
-
Homo sapiens
SUCL
-
Homo sapiens

Cofactor

Cofactor Comment Organism Structure
ADP
-
Homo sapiens
ATP
-
Homo sapiens

General Information

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