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6.2.1.4: succinate-CoA ligase (GDP-forming)

This is an abbreviated version!
For detailed information about succinate-CoA ligase (GDP-forming), go to the full flat file.

Word Map on EC 6.2.1.4

Reaction

GTP
+
succinate
 +
CoA
=
GDP
 +
phosphate
 +
succinyl-CoA

Synonyms

ATP-specific SCS, G-SCS, G-STK, G-SUCL, GDP-forming SUCL, GTP-dependent succinate:CoA ligase, GTP-forming SUCL, GTP-specific succinate:CoA ligase, GTP-specific succinyl-CoA synthetase, GTP-specific SUCL, GTPSCS, SCS, SCS-alpha, SCS-beta, SCS-betaG, ScsA, 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 synthase, Succinyl-CoA synthetase, Succinyl-CoA synthetase (GDP-forming), succinyl-CoA synthetase subunit beta, succinyl-coenzyme A synthetase, SUCL, SUCLG1, SUCLG2, Synthetase, succinyl coenzyme A (guanosine diphosphate-forming)

ECTree

     6 Ligases
         6.2 Forming carbon-sulfur bonds
             6.2.1 Acid-thiol ligases
                6.2.1.4 succinate-CoA ligase (GDP-forming)

Disease

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Disease on EC 6.2.1.4 - succinate-CoA ligase (GDP-forming)

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Acidosis
Disorders caused by deficiency of succinate-CoA ligase.
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.
Fatal infantile lactic acidosis and a novel homozygous mutation in the SUCLG1 gene: a mitochondrial DNA depletion disorder.
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.
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.
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.
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.
Contribution of nuclear and mitochondrial gene mutations in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome.
New SUCLG1 patients expanding the phenotypic spectrum of this rare cause of mild methylmalonic aciduria.
[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.
Expanding the phenotypic spectrum of Succinyl-CoA ligase deficiency through functional validation of a new SUCLG1 variant.
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.
Germline SUCLG2 Variants in Patients with Pheochromocytoma and Paraganglioma.
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.
Germline SUCLG2 Variants in Patients with Pheochromocytoma and Paraganglioma.
Pheochromocytoma
Another Weak Link in the Electron Transport Chain: Variants in SUCLG2 and Risk for Pheochromocytoma and Paraganglioma.
Germline SUCLG2 Variants in Patients with Pheochromocytoma and Paraganglioma.
Porphyrias, Hepatic
Physiological roles of animal succinate thiokinases. Specific association of the guanine nucleotide-linked enzyme with haem biosynthesis.
Prostatic Neoplasms
EGFR-upregulated LIFR promotes SUCLG2-dependent castration resistance and neuroendocrine differentiation of prostate cancer.
pyruvate dehydrogenase system deficiency
Neonatal onset of mitochondrial disorders in 129 patients: clinical and laboratory characteristics and a new approach to diagnosis.
Seizures
Contribution of nuclear and mitochondrial gene mutations in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome.
Starvation
Deficiency of succinyl-CoA synthetase ? subunit delays development, impairs locomotor activity and reduces survival under starvation in Drosophila.
succinate-coa ligase (gdp-forming) deficiency
Expanding the phenotypic spectrum of Succinyl-CoA ligase deficiency through functional validation of a new SUCLG1 variant.
Five novel SUCLG1 mutations in three Chinese patients with succinate-CoA ligase deficiency noticed by mild methylmalonic aciduria.
Novel mutation in SUCLA2 identified on sequencing analysis.
Phenotypic variability in deficiency of the ? subunit of succinate-CoA ligase.
Succinate-CoA ligase deficiency due to mutations in SUCLA2 and SUCLG1: phenotype and genotype correlations in 71 patients.
SUCLA2 mutations are associated with mild methylmalonic aciduria, Leigh-like encephalomyopathy, dystonia and deafness.
The severity of phenotype linked to SUCLG1 mutations could be correlated with residual amount of SUCLG1 protein.
Two transgenic mouse models for beta subunit components of succinate-CoA ligase yielding pleiotropic metabolic alterations.
[Clinical and laboratory studies on four Chinese patients with succinate-CoA ligase deficiency noticed by mild methylmalonic aciduria].
Vitamin B 12 Deficiency
Diagnostic dilemma of patients with methylmalonic aciduria: Experience from a tertiary care centre in Pakistan.