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Information on EC 6.2.1.4 - succinate-CoA ligase (GDP-forming)
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6.2.1.4 succinate-CoA ligase (GDP-forming)IUBMB Comments
Itaconate can act instead of succinate, and ITP instead of GTP.
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Word Map
- 6.2.1.4
-
tricarboxylic
- tca
- malate
- sucla2
-
citric
- mtdna
-
methylmalonic
-
krebs
- alpha-ketoglutarate
-
adp-forming
-
substrate-level
- fumarase
-
aciduria
- hydrogenosomes
- dguok
-
phosphohistidine
-
coa-transferase
- phosphoenzyme
-
encephalomyopathic
-
nishimura
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria, Archaea
Synonyms
succinyl-coa synthetase, suclg1, suclg2, succinate-coa ligase, succinate thiokinase, succinyl coa synthetase, succinyl-coa synthase, succinic thiokinase, succinyl coenzyme a synthetase, g-scs, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
ATP-specific SCS
G-SCS
G-STK
-
-
-
-
G-SUCL
GTP-specific succinyl-CoA synthetase
SCS
SCS-alpha
-
-
-
-
SCS-beta
SCS-betaG
-
-
-
-
SCSalpha
SucCDAm
Succinate thiokinase
-
-
-
-
succinate-CoA ligase
Succinic thiokinase
-
-
-
-
Succinyl CoA synthetase
Succinyl coenzyme A synthetase
Succinyl coenzyme A synthetase (GDP-forming)
-
-
-
-
Succinyl coenzyme A synthetase (guanosine diphosphate-forming)
-
-
-
-
Succinyl-CoA synthetase
Succinyl-CoA synthetase (GDP-forming)
-
-
-
-
SUCLG2
Synthetase, succinyl coenzyme A (guanosine diphosphate-forming)
-
-
-
-
SCS
-
-
-
-
SCS-beta
-
-
-
-
Succinyl CoA synthetase
-
-
-
-
Succinyl coenzyme A synthetase
-
-
-
-
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
GTP + succinate + CoA = GDP + phosphate + succinyl-CoA
-
-
-
-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
Acid-thiol ligation
-
-
-
-
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PATHWAY SOURCE
PATHWAYS
BRENDA
KEGG
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SYSTEMATIC NAME
IUBMB Comments
succinate:CoA ligase (GDP-forming)
Itaconate can act instead of succinate, and ITP instead of GTP.
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CAS REGISTRY NUMBER
COMMENTARY
9014-36-2
-
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
-
?
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
-
?
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
-
?
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
Brevibacterium leucinophagum
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
-
?
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
-
enzyme has a low Km-value for ADP and for GDP
-
-
?
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
-
?
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
Mima polymorpha
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
-
?
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
-
enzyme has a low Km-value for ADP and for GDP
-
-
?
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
-
enzyme has a low Km-value for ADP and for GDP
-
-
?
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
-
enzyme has a low Km-value for ADP and for GDP
-
-
?
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
-
enzyme has a low Km-value for ADP and for GDP
-
-
?
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
-
enzyme has a low Km-value for ADP and for GDP
-
-
?
ATP + succinate + CoA
ADP + phosphate + succinyl-CoA
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
-
?
GDP + phosphate + succinyl-CoA
GTP + succinate + CoA
-
GTP generated by the activation of succinyl-CoA synthetase could promote key functional roles in the mitochondrial metabolism leading to insulin secretion
-
?
GTP + 3-sulfinopropionate + CoA
GDP + phosphate + 3-sulfinopropionyl-CoA
SucCDAm is unspecific regarding ATP or GTP
determination of 3-sulfinopropionyl-CoA structure by using liquid chromatography-electrospray ionization-mass spectrometry
-
?
GTP + 3-sulfinopropionate + CoA
GDP + phosphate + 3-sulfinopropionyl-CoA
SucCDAm is unspecific regarding ATP or GTP
determination of 3-sulfinopropionyl-CoA structure by using liquid chromatography-electrospray ionization-mass spectrometry
-
?
GTP + itaconate + CoA
GDP + phosphate + itaconyl-CoA
SucCDAm is unspecific regarding ATP or GTP
-
-
?
GTP + itaconate + CoA
GDP + phosphate + itaconyl-CoA
SucCDAm is unspecific regarding ATP or GTP
-
-
?
GTP + succinate + CoA
?
-
recycling of succinate to succinyl-CoA at the expense of GTP, involvement in heme biosynthesis
-
-
?
GTP + succinate + CoA
?
-
recycling of succinate to succinyl-CoA at the expense of GTP, involvement in heme biosynthesis
-
-
?
GTP + succinate + CoA
?
-
production of succinyl-CoA for ketone body metabolism and as a precursor for porphyrins
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
SucCDAm is unspecific regarding ATP or GTP
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
succinate is the best substrate, SucCDAm is unspecific regarding ATP or GTP
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
SucCDAm is unspecific regarding ATP or GTP
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
succinate is the best substrate, SucCDAm is unspecific regarding ATP or GTP
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
substrate for mutant enzymes K46E/K114D and K46E/K114D/V113L/L227F
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
Blastocystis hominis NandII
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substrate for mutant enzymes K46E/K114D and K46E/K114D/V113L/L227F
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
Brevibacterium leucinophagum
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
enzyme has a low Km-value for ADP and for GDP
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
-
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
succinyl CoA synthetase converts succinyl CoA to succinate in the Krebs cycle. The enzyme activity is reduced, when Helicobacter pylori transforms to the coccoid form, a viable but non-culturable form with reduced metabolic activity, overview
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
Mima polymorpha
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
enzyme has a low Km-value for ADP and for GDP
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
enzyme has a low Km-value for ADP and for GDP
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
enzyme has a low Km-value for ADP and for GDP
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
enzyme has a low Km-value for ADP and for GDP
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
enzyme has a low Km-value for ADP and for GDP
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
GTP is the preferred cosubstrate
-
-
r
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
enzyme has a very high Km-value for ADP and a low Km-value for GDP
-
?
additional information
?
-
-
the wild type enzyme has no activity with GTP
-
-
?
additional information
?
-
Blastocystis hominis NandII
-
the wild type enzyme has no activity with GTP
-
-
?
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
-
-
?
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
-
-
?
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
-
-
?
additional information
?
-
-
the enzyme supports GTP-dependent anabolic processes
-
-
?
additional information
?
-
no activity with citrate, fumarate and oxaloacetate
-
-
?
additional information
?
-
-
no activity with citrate, fumarate and oxaloacetate
-
-
?
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
GDP + phosphate + succinyl-CoA
GTP + succinate + CoA
-
GTP generated by the activation of succinyl-CoA synthetase could promote key functional roles in the mitochondrial metabolism leading to insulin secretion
-
?
GTP + succinate + CoA
?
-
recycling of succinate to succinyl-CoA at the expense of GTP, involvement in heme biosynthesis
-
-
?
GTP + succinate + CoA
?
-
recycling of succinate to succinyl-CoA at the expense of GTP, involvement in heme biosynthesis
-
-
?
GTP + succinate + CoA
?
-
production of succinyl-CoA for ketone body metabolism and as a precursor for porphyrins
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
SucCDAm is unspecific regarding ATP or GTP
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
SucCDAm is unspecific regarding ATP or GTP
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
succinyl CoA synthetase converts succinyl CoA to succinate in the Krebs cycle. The enzyme activity is reduced, when Helicobacter pylori transforms to the coccoid form, a viable but non-culturable form with reduced metabolic activity, overview
-
-
?
GTP + succinate + CoA
GDP + phosphate + succinyl-CoA
-
GTP is the preferred cosubstrate
-
-
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
-
-
?
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
-
-
?
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
-
-
?
additional information
?
-
-
the enzyme supports GTP-dependent anabolic processes
-
-
?
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
-
nucleotide-binding site, binding structure crystal structure analysis, modling, detailed overview
-
GDP
-
allosteric regulator, at high concentration it serves as a substrate. However 100fold lower concentrations of GDP, which do not bind to the catalytic site to induce succinyl-CoA synthetase dephosphorylation, stimulate phosphorylation of p36, the alpha-subunit of succinyl-CoA synthetase
GDP
-
low concentrations stimulate phosphoenzyme formation by either GTP, or succinyl-CoA and phosphate
GDP
-
low concentrations stimulate phosphoenzyme formation by either GTP, or succinyl-CoA and phosphate
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
Mg2+
Mn2+
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
NaI
-
41% inhibition at 10 mM, the CoAT activity is inhibited by applying NaI to exponentially growing cell cultures, but the colony-forming ability is not affected
Urea
-
the Thermus aquaticus enzyme loses activity at 4-5.5 M urea, but it regains activity with increasing concentrations of urea, reaching 70% of its original activity in 8.5 M urea
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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Acidosis
Marked mitochondrial DNA depletion associated with a novel SUCLG1 gene mutation resulting in lethal neonatal acidosis, multi-organ failure, and interrupted aortic arch.
Acidosis, Lactic
A novel missense mutation in SUCLG1 associated with mitochondrial DNA depletion, encephalomyopathic form, with methylmalonic aciduria.
Acidosis, Lactic
Fatal infantile lactic acidosis and a novel homozygous mutation in the SUCLG1 gene: a mitochondrial DNA depletion disorder.
Acidosis, Lactic
Neonatal lactic acidosis with methylmalonic aciduria by novel mutations in the SUCLG1 gene.
Adenocarcinoma, Mucinous
Multi-omics analyses of human colorectal cancer revealed three mitochondrial genes potentially associated with poor outcomes of patients.
Adenoma
Identification of novel biomarker and therapeutic target candidates for diagnosis and treatment of follicular carcinoma.
Adenoma
Multi-omics analyses of human colorectal cancer revealed three mitochondrial genes potentially associated with poor outcomes of patients.
Alzheimer Disease
SUCLG2 identified as both a determinator of CSF A?1-42 levels and an attenuator of cognitive decline in Alzheimer's disease.
Anemia, Sideroblastic
Interaction between succinyl CoA synthetase and the heme-biosynthetic enzyme ALAS-E is disrupted in sideroblastic anemia.
Anemia, Sideroblastic
X-linked Sideroblastic Anemia Due to Carboxyl-terminal ALAS2 Mutations That Cause Loss of Binding to the ?-Subunit of Succinyl-CoA Synthetase (SUCLA2).
Brain Diseases
Contribution of nuclear and mitochondrial gene mutations in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome.
Carcinoma
Identification of novel biomarker and therapeutic target candidates for diagnosis and treatment of follicular carcinoma.
Cardiomyopathies
Contribution of nuclear and mitochondrial gene mutations in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome.
Cardiomyopathy, Hypertrophic
Succinate-CoA ligase deficiency due to mutations in SUCLA2 and SUCLG1: phenotype and genotype correlations in 71 patients.
Colonic Neoplasms
Effect of Citric Acid Cycle Genetic Variants and Their Interactions with Obesity, Physical Activity and Energy Intake on the Risk of Colorectal Cancer: Results from a Nested Case-Control Study in the UK Biobank.
Diabetes Mellitus, Experimental
Inhibition by streptozotocin of the activity of succinyl-CoA synthetase in vitro and in vivo.
Down Syndrome
Co-occurring Down syndrome and SUCLA2-related mitochondrial depletion syndrome.
Endometriosis
Changes in eutopic endometrial gene expression during the progression of experimental endometriosis in the baboon, papio anubis.
Epilepsy
Contribution of nuclear and mitochondrial gene mutations in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome.
Epilepsy
Succinate-CoA ligase deficiency due to mutations in SUCLA2 and SUCLG1: phenotype and genotype correlations in 71 patients.
Glioma
Elucidation of the genetic and epigenetic landscape alterations in RNA binding proteins in glioblastoma.
Hearing Loss, Sensorineural
Contribution of nuclear and mitochondrial gene mutations in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome.
Heart Failure
Defining decreased protein succinylation of failing human cardiac myofibrils in ischemic cardiomyopathy.
Mitochondrial Diseases
A SUCLG1 mutation in a patient with mitochondrial DNA depletion and congenital anomalies.
Mitochondrial Diseases
Contribution of nuclear and mitochondrial gene mutations in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome.
Mitochondrial Diseases
New SUCLG1 patients expanding the phenotypic spectrum of this rare cause of mild methylmalonic aciduria.
Mitochondrial Diseases
[Diagnosis of mitochondrial disorders in children with next generation sequencing].
Mitochondrial Encephalomyopathies
Clinical, Molecular, and Computational Analysis in two cases with mitochondrial encephalomyopathy associated with SUCLG1 mutation in a consanguineous family.
Mitochondrial Encephalomyopathies
Expanding the phenotypic spectrum of Succinyl-CoA ligase deficiency through functional validation of a new SUCLG1 variant.
Mitochondrial Encephalomyopathies
SUCLG1 mutations and mitochondrial encephalomyopathy: a case study and review of the literature.
Muscle Hypotonia
Mitochondrial DNA Depletion Syndromes: Review and Updates of Genetic Basis, Manifestations, and Therapeutic Options.
Neoplasms
EGFR-upregulated LIFR promotes SUCLG2-dependent castration resistance and neuroendocrine differentiation of prostate cancer.
Neoplasms
Proteomic Analysis of Hippocampus and Cortex in Streptozotocin-Induced Diabetic Model Mice Showing Dementia.
Obesity
Extracting time-dependent obese-diabetic specific networks in hepatic proteome analysis.
Paraganglioma
Another Weak Link in the Electron Transport Chain: Variants in SUCLG2 and Risk for Pheochromocytoma and Paraganglioma.
Paraganglioma
Germline SUCLG2 Variants in Patients with Pheochromocytoma and Paraganglioma.