Literature summary for 6.2.1.5 extracted from

Huang, X.; Bedoyan, J.K.; Demirbas, D.; Harris, D.J.; Miron, A.; Edelheit, S.; Grahame, G.; DeBrosse, S.D.; Wong, L.J.; Hoppel, C.L.; Kerr, D.S.; Anselm, I.; Berry, G.T.
Succinyl-CoA synthetase (SUCLA2) deficiency in two siblings with impaired activity of other mitochondrial oxidative enzymes in skeletal muscle without mitochondrial DNA depletion (2017), Mol. Genet. Metab., 120, 213-222 .

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Protein Variants

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 [mM]	KM Value Maximum [mM]	Substrate	Comment	Organism	Structure
1.33	-	succinate	at pH 8.0 and 37°C	Homo sapiens

Natural Substrates/ Products (Substrates)

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

Organism	UniProt	Comment	Textmining
Homo sapiens	Q9P2R7	subunit beta	-

Source Tissue

Source Tissue	Comment	Organism	Textmining
fibroblast	-	Homo sapiens	-
skeletal muscle	-	Homo sapiens	-

Substrates and Products (Substrate)

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

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

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

General Information

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

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Release 2023.1 (January 2023)