Succinate-CoA Ligases (Succinyl Coenzyme A Synthetases)

Enzymes that catalyze the first step leading to the oxidation of succinic acid by the reversible formation of succinyl-CoA from succinate and CoA with the concomitant cleavage of ATP to ADP (EC 6.2.1.5) or GTP to GDP (EC 6.2.1.4) and orthophosphate. Itaconate can act instead of succinate and ITP instead of GTP.EC 6.2.1.-.

Also Known As:

Succinyl Coenzyme A Synthetases; CoA Synthetase, Succinyl; CoA Synthetases, Succinyl; Ligase, Succinate-CoA; Ligases, Succinate-CoA; Succinate CoA Ligase; Succinate CoA Ligases; Synthetase, Succinyl CoA; Synthetases, Succinyl CoA; Thiokinase, Succinic; Thiokinases, Succinic; Succinate-CoA Ligase; Succinate-CoA Ligase (GDP-Forming); Succinate-CoA Ligases (ADP-Forming); Succinic Thiokinase; Succinic Thiokinases; Succinyl CoA Synthetase; Succinyl CoA Synthetases; Succinyl Coenzyme A Synthetase; Succinate:CoA ligase (GDP- or ADP-forming)

Networked: 18 relevant articles (0 outcomes, 1 trials/studies)

Bio-Agent Context: Research Results

Enzymes and Coenzymes: 1
- Enzymes: 152586
  - Ligases: 5553
    - Carbon-Sulfur Ligases
      - Coenzyme A Ligases: 197
        Succinate-CoA Ligases: 18
        Drosophila ScsbetaA protein
        human SUCLA2 protein
        human SUCLG1 protein
        human SUCLG2 protein
        mouse Sucla2 protein
        Neocallimastix frontalis succinyl CoA hydrogenosomal beta subunit
        O-succinylbenzoate - CoA ligase

Experts

1.	Batbayli, Mustafa: 2 articles (03/2016 - 02/2010)
2.	Holme, Elisabeth: 2 articles (03/2016 - 02/2010)
3.	Ostergaard, Elsebet: 2 articles (03/2016 - 02/2010)
4.	Craigen, William J: 2 articles (01/2016 - 02/2014)
5.	Donti, Taraka R: 2 articles (01/2016 - 02/2014)
6.	Graham, Brett H: 2 articles (01/2016 - 02/2014)
7.	Chen, Danlei: 1 article (01/2022)
8.	Chen, Wei: 1 article (01/2022)
9.	Chen, Yi-Ming: 1 article (01/2022)
10.	Chen, Yuan: 1 article (01/2022)

Related Diseases

1.	Prostatic Neoplasms (Prostate Cancer) 10/01/2020 - "In this study, we identified that ADT induced upregulation of the succinate-CoA ligase GDP-forming beta subunit (SUCLG2), which regulates succinate metabolism and NE differentiation of prostate cancer. "
2.	Methylmalonic acidemia 06/01/2010 - "Deficiencies in two subunits of the succinyl-coenzyme A synthetase (SCS) have been involved in patients with encephalomyopathy and mild methylmalonic aciduria (MMA). " 03/01/2016 - "The encephalomyopathic mtDNA depletion syndrome with methylmalonic aciduria is associated with deficiency of succinate-CoA ligase, caused by mutations in SUCLA2 or SUCLG1. " 04/01/2018 - "On stratification by type of disorders leading to methylmalonic acidurias, 9(22%) had methylmalonic acidemia, 12(29%) had Cobalamin-related remethylation disorders, nonspecific methylmalonic acidurias in 16(39%), while 2(5%) each had succinyl coenzyme A synthetase and Vitamin B12 deficiency. " 01/01/2022 - "Succinate-CoA ligase/synthetase (SCS) deficiency is responsible for encephalomyopathy with mitochondrial DNA depletion and mild methylmalonic aciduria. " 01/01/2016 - "Deficiency of the TCA cycle enzyme Succinyl-CoA Synthetase/Ligase (SCS), due to pathogenic variants in subunits encoded by SUCLG1 and SUCLA2, causes mitochondrial encephalomyopathy, methylmalonic acidemia, and mitochondrial DNA (mtDNA) depletion. "
3.	Mitochondrial Encephalomyopathies (Mitochondrial Encephalomyopathy) 06/27/2023 - "Succinyl-CoA Synthetase Dysfunction as a Mechanism of Mitochondrial Encephalomyopathy: More than Just an Oxidative Energy Deficit." 06/27/2023 - "Biallelic pathogenic variants in subunits of succinyl-CoA synthetase (SCS), a tricarboxylic acid (TCA) cycle enzyme, are associated with mitochondrial encephalomyopathy in humans. " 10/31/2023 - "Deficiency of succinyl-CoA synthetase (SCS), the tricarboxylic acid cycle enzyme catalyzing the interconversion of succinyl-CoA to succinate, results in mitochondrial encephalomyopathy in humans. " 02/01/2014 - "Mutations in subunits of succinyl-CoA synthetase/ligase (SCS), a component of the citric acid cycle, are associated with mitochondrial encephalomyopathy, elevation of methylmalonic acid (MMA), and mitochondrial DNA (mtDNA) depletion. " 01/01/2016 - "Deficiency of the TCA cycle enzyme Succinyl-CoA Synthetase/Ligase (SCS), due to pathogenic variants in subunits encoded by SUCLG1 and SUCLA2, causes mitochondrial encephalomyopathy, methylmalonic acidemia, and mitochondrial DNA (mtDNA) depletion. "
4.	Succinate-Coa Ligase Deficiency 03/01/2019 - "Succinyl-CoA synthetase or succinate-CoA ligase deficiency can result from biallelic mutations in SUCLG1 gene that encodes for the alpha subunit of the succinyl-CoA synthetase. " 02/01/2010 - "Mitochondrial DNA depletion, encephalomyopathic form, with methylmalonic aciduria is associated with mutations in SUCLA2, the gene encoding a beta subunit of succinate-CoA ligase, where 17 patients have been reported. "
5.	Vitamin B 12 Deficiency (Vitamin B12 Deficiency) 04/01/2018 - "On stratification by type of disorders leading to methylmalonic acidurias, 9(22%) had methylmalonic acidemia, 12(29%) had Cobalamin-related remethylation disorders, nonspecific methylmalonic acidurias in 16(39%), while 2(5%) each had succinyl coenzyme A synthetase and Vitamin B12 deficiency. "

Related Drugs and Biologics

1.	Succinic Acid (Succinate)
2.	Enzymes
3.	Mitochondrial DNA (mtDNA)
4.	Ligases (Synthetase)
5.	Chromogranin B
6.	Vitamin B 12 (Cyanocobalamin)
7.	Tricarboxylic Acids
8.	Succinate Dehydrogenase (Fumarate Reductase)
9.	Methylmalonic Acid
10.	Heme (Haem)