Disease on EC 2.8.3.5 - 3-oxoacid CoA-transferase and Organism(s) Homo sapiens
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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.
3-oxoacid coa-transferase deficiency
A new Japanese case of succinyl-CoA: 3-ketoacid CoA-transferase deficiency.
3-oxoacid coa-transferase deficiency
A Rare Cause of Life-Threatening Ketoacidosis: Novel Compound Heterozygous OXCT1 Mutations Causing Succinyl-CoA:3-Ketoacid CoA Transferase Deficiency.
3-oxoacid coa-transferase deficiency
Heterozygous carriers of succinyl-CoA:3-oxoacid CoA transferase deficiency can develop severe ketoacidosis.
3-oxoacid coa-transferase deficiency
Management and communication problems in a patient with succinyl-CoA transferase deficiency in pregnancy and labour.
3-oxoacid coa-transferase deficiency
Neonatal hypoglycaemia in severe succinyl-CoA: 3-oxoacid CoA-transferase deficiency.
3-oxoacid coa-transferase deficiency
Rare cause of high anion gap metabolic acidosis in an infant: Succinyl-CoA:3-ketoacid transferase deficiency.
3-oxoacid coa-transferase deficiency
Successful adaptation to ketosis by mice with tissue-specific deficiency of ketone body oxidation.
3-oxoacid coa-transferase deficiency
Succinyl-CoA: 3-ketoacid CoA-transferase deficiency. A cause for ketoacidosis in infancy.
3-oxoacid coa-transferase deficiency
Succinyl-CoA:3-oxoacid coenzyme A transferase (SCOT) deficiency: A rare and potentially fatal metabolic disease.
3-oxoacid coa-transferase deficiency
[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.
Acidosis
Rare cause of high anion gap metabolic acidosis in an infant: Succinyl-CoA:3-ketoacid transferase deficiency.
Acidosis
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.
Breast Neoplasms
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).
Carcinogenesis
The role of OXCT1 in the pathogenesis of cancer as a rate-limiting enzyme of ketone body metabolism.
Carcinoma, Hepatocellular
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.
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.
Diabetes Mellitus, Type 2
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
Novel LncRNA OXCT1-AS1 indicates poor prognosis and contributes to tumorigenesis by regulating miR-195/CDC25A axis in glioblastoma.
Glioma
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.
Hyperthyroidism
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.
Hypoglycemia
Successful adaptation to ketosis by mice with tissue-specific deficiency of ketone body oxidation.
Hypoglycemia
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.
Ketosis
A structural mapping of mutations causing succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency.
Ketosis
Clinical and molecular characterization of five patients with succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency.
Ketosis
Glutaconate CoA-transferase from Acidaminococcus fermentans: the crystal structure reveals homology with other CoA-transferases.
Ketosis
Heterozygous carriers of succinyl-CoA:3-oxoacid CoA transferase deficiency can develop severe ketoacidosis.
Ketosis
Identification and characterization of a temperature-sensitive R268H mutation in the human succinyl-CoA:3-ketoacid CoA transferase (SCOT) gene.
Ketosis
Management and communication problems in a patient with succinyl-CoA transferase deficiency in pregnancy and labour.
Ketosis
Neonatal hypoglycaemia in severe succinyl-CoA: 3-oxoacid CoA-transferase deficiency.
Ketosis
Patients homozygous for the T435N mutation of succinyl-CoA:3-ketoacid CoA Transferase (SCOT) do not show permanent ketosis.
Ketosis
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.
Metabolic Diseases
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
Low ketolytic enzyme levels in tumors predict ketogenic diet responses in cancer cell lines in vitro and in vivo.
Neoplasms
Metabolic substrate utilization by a tumour cell line which induces cachexia in vivo.
Neoplasms
The calorically restricted ketogenic diet, an effective alternative therapy for malignant brain cancer.
Neoplasms
The role of OXCT1 in the pathogenesis of cancer as a rate-limiting enzyme of ketone body metabolism.
Neoplasms
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
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.
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.