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Disease on EC 2.8.3.5 - 3-oxoacid CoA-transferase and Organism(s) Homo sapiens

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
3-oxoacid coa-transferase deficiency
A Case of Succinyl-CoA:3-Oxoacid CoA Transferase Deficiency Presenting with Severe Acidosis in a 14-Month-Old Female: Evidence for Pathogenicity of a Point Mutation in the OXCT1 Gene.
A new Japanese case of succinyl-CoA: 3-ketoacid CoA-transferase deficiency.
A Rare Cause of Life-Threatening Ketoacidosis: Novel Compound Heterozygous OXCT1 Mutations Causing Succinyl-CoA:3-Ketoacid CoA Transferase Deficiency.
Heterozygous carriers of succinyl-CoA:3-oxoacid CoA transferase deficiency can develop severe ketoacidosis.
Management and communication problems in a patient with succinyl-CoA transferase deficiency in pregnancy and labour.
Neonatal hypoglycaemia in severe succinyl-CoA: 3-oxoacid CoA-transferase deficiency.
Rare cause of high anion gap metabolic acidosis in an infant: Succinyl-CoA:3-ketoacid transferase deficiency.
Successful adaptation to ketosis by mice with tissue-specific deficiency of ketone body oxidation.
Succinyl-CoA: 3-ketoacid CoA-transferase deficiency. A cause for ketoacidosis in infancy.
Succinyl-CoA:3-oxoacid coenzyme A transferase (SCOT) deficiency: A rare and potentially fatal metabolic disease.
[Succinyl-CoA: 3-ketoacid CoA transferase deficiency]
acetyl-coa c-acetyltransferase deficiency
Influence of ketone bodies on oxidative stress parameters in brain of developing rats in vitro.
Acidosis
A Case of Succinyl-CoA:3-Oxoacid CoA Transferase Deficiency Presenting with Severe Acidosis in a 14-Month-Old Female: Evidence for Pathogenicity of a Point Mutation in the OXCT1 Gene.
Rare cause of high anion gap metabolic acidosis in an infant: Succinyl-CoA:3-ketoacid transferase deficiency.
Successful Management of Pregnancies in Patients with Inherited Disorders of Ketone Body Metabolism.
Brain Neoplasms
The calorically restricted ketogenic diet, an effective alternative therapy for malignant brain cancer.
Breast Neoplasms
?-hydroxybutyrate does not alter the effects of glucose deprivation on breast cancer cells.
Mitoketoscins: Novel mitochondrial inhibitors for targeting ketone metabolism in cancer stem cells (CSCs).
Carcinogenesis
Mitoketoscins: Novel mitochondrial inhibitors for targeting ketone metabolism in cancer stem cells (CSCs).
The role of OXCT1 in the pathogenesis of cancer as a rate-limiting enzyme of ketone body metabolism.
Carcinoma, Hepatocellular
Acetoacetate coenzyme A transferase activity in rat hepatomas.
Regulation of succinyl coenzyme A:acetoacetyl coenzyme A transferase in rat hepatoma cell lines.
Carcinosarcoma
Failure of systemic ketosis to control cachexia and the growth rate of the Walker 256 carcinosarcoma in rats.
Colitis
Physiological activity of E. coli engineered to produce butyric acid.
Dehydration
Characterization of (R)-2-hydroxyisocaproate dehydrogenase and a family III coenzyme A transferase involved in reduction of L-leucine to isocaproate by Clostridium difficile.
Diabetes Mellitus
Increased Glucose Availability Attenuates Myocardial Ketone Body Utilization.
Diabetes Mellitus, Experimental
Physical training reverses defect in 3-ketoacid CoA-transferase activity in skeletal muscle of diabetic rats.
Diabetes Mellitus, Type 2
Increased Glucose Availability Attenuates Myocardial Ketone Body Utilization.
Lower succinyl-CoA:3-ketoacid-CoA transferase (SCOT) and ATP citrate lyase in pancreatic islets of a rat model of type 2 diabetes: knockdown of SCOT inhibits insulin release in rat insulinoma cells.
Diabetic Ketoacidosis
Ketone body utilization and its metabolic effect in resting muscles of normal and streptozotocin-diabetic rats.
Genetic Diseases, Inborn
A 6-bp deletion at the splice donor site of the first intron resulted in aberrant splicing using a cryptic splice site within exon 1 in a patient with succinyl-CoA: 3-Ketoacid CoA transferase (SCOT) deficiency.
Glioma
Ketone-body metabolism in glioma and neuroblastoma cells.
Novel LncRNA OXCT1-AS1 indicates poor prognosis and contributes to tumorigenesis by regulating miR-195/CDC25A axis in glioblastoma.
Turnover of succinyl-CoA:3-oxoacid CoA-transferase in glioma and neuroblastoma cells. Specific influence of acetoacetate in neuroblastoma cells.
glycoprotein-fucosylgalactoside alpha-n-acetylgalactosaminyltransferase deficiency
Succinyl-CoA:3-oxoacid coenzyme A transferase (SCOT) deficiency: A rare and potentially fatal metabolic disease.
Heart Failure
Increased Glucose Availability Attenuates Myocardial Ketone Body Utilization.
Hyperthyroidism
Differential action of thyroid hormones on the activity of certain enzymes in rat kidney and brain.
Ketone-body metabolism in hyperthyroid rats: reduced activity of D-3-hydroxybutyrate dehydrogenase in both liver and heart and of succinyl-coenzyme A: 3-oxoacid coenzyme A-transferase in heart.
Hypoglycemia
Impact of peripheral ketolytic deficiency on hepatic ketogenesis and gluconeogenesis during the transition to birth.
Successful adaptation to ketosis by mice with tissue-specific deficiency of ketone body oxidation.
Successful Management of Pregnancies in Patients with Inherited Disorders of Ketone Body Metabolism.
Infertility, Male
Ketone bodies could support the motility but not the acrosome reaction of mouse sperm.
Insulinoma
Lower succinyl-CoA:3-ketoacid-CoA transferase (SCOT) and ATP citrate lyase in pancreatic islets of a rat model of type 2 diabetes: knockdown of SCOT inhibits insulin release in rat insulinoma cells.
Ketosis
A Rare Cause of Life-Threatening Ketoacidosis: Novel Compound Heterozygous OXCT1 Mutations Causing Succinyl-CoA:3-Ketoacid CoA Transferase Deficiency.
A structural mapping of mutations causing succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency.
Clinical and molecular characterization of five patients with succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency.
Glutaconate CoA-transferase from Acidaminococcus fermentans: the crystal structure reveals homology with other CoA-transferases.
Heterozygous carriers of succinyl-CoA:3-oxoacid CoA transferase deficiency can develop severe ketoacidosis.
Identification and characterization of a temperature-sensitive R268H mutation in the human succinyl-CoA:3-ketoacid CoA transferase (SCOT) gene.
Inborn errors of ketone body utilization.
Management and communication problems in a patient with succinyl-CoA transferase deficiency in pregnancy and labour.
Neonatal hypoglycaemia in severe succinyl-CoA: 3-oxoacid CoA-transferase deficiency.
Patients homozygous for the T435N mutation of succinyl-CoA:3-ketoacid CoA Transferase (SCOT) do not show permanent ketosis.
Structure of the mammalian CoA transferase from pig heart.
Succinyl-CoA: 3-ketoacid CoA-transferase deficiency. A cause for ketoacidosis in infancy.
Liver Neoplasms, Experimental
Subcellular localization of acetoacetate coenzyme A transferase in rat hepatomas.
Lymphoma
Signalling pathways identified in salivary glands from primary Sjögren's syndrome patients reveal enhanced adipose tissue development.
Metabolic Diseases
Glutaconate CoA-transferase from Acidaminococcus fermentans: the crystal structure reveals homology with other CoA-transferases.
Succinyl-CoA:3-oxoacid coenzyme A transferase (SCOT) deficiency: A rare and potentially fatal metabolic disease.
Neoplasm Metastasis
Mitoketoscins: Novel mitochondrial inhibitors for targeting ketone metabolism in cancer stem cells (CSCs).
Neoplasms
Acetoacetate coenzyme A transferase activity in rat hepatomas.
Low ketolytic enzyme levels in tumors predict ketogenic diet responses in cancer cell lines in vitro and in vivo.
Metabolic substrate utilization by a tumour cell line which induces cachexia in vivo.
Role of acetoacetyl-CoA synthetase in acetoacetate utilization by tumor cells.
The calorically restricted ketogenic diet, an effective alternative therapy for malignant brain cancer.
The role of OXCT1 in the pathogenesis of cancer as a rate-limiting enzyme of ketone body metabolism.
Treatment of glioma patients with ketogenic diets: report of two cases treated with an IRB-approved energy-restricted ketogenic diet protocol and review of the literature.
Neuroblastoma
Ketone-body metabolism in glioma and neuroblastoma cells.
Turnover of succinyl-CoA:3-oxoacid CoA-transferase in glioma and neuroblastoma cells. Specific influence of acetoacetate in neuroblastoma cells.
Ovarian Neoplasms
3-Oxoacid CoA transferase 1 as a therapeutic target gene for cisplatin-resistant ovarian cancer.
Phenylketonurias
When one disease is not enough: succinyl-CoA: 3-oxoacid coenzyme A transferase (SCOT) deficiency due to a novel mutation in OXCT1 in an infant with known phenylketonuria.
Sleep Deprivation
Ketone body metabolism and sleep homeostasis in mice.
Starvation
Activities of some key enzymes of carbohydrate, ketone body, adenosine and glutamine metabolism in liver, and brown and white adipose tissues of the rat.
Stomach Neoplasms
Growth-inhibitory effects of the ketone body, monoacetoacetin, on human gastric cancer cells with succinyl-CoA: 3-oxoacid CoA-transferase (SCOT) deficiency.
Thymoma
[Analysis of Gene Variation in Thymoma by Microarray].