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Acidosis
Activation of oxoglutarate dehydrogenase in the kidney in response to acute acidosis.
Acidosis
Adaptation of renal tricarboxylic acid cycle metabolism to various acid-base states: study with [3-13C,5-15N]glutamine.
Acidosis
Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis.
Acidosis
Ammoniagenesis by cultured human renal cortical epithelial cells: study with 15N.
Acidosis
Biochemical pathways and modulators of renal ammoniagenesis.
Acidosis
Intramitochondrial pH and ammonium production in rat and dog kidney cortex.
Acidosis
Regulation of rat kidney mitochondrial metabolism in acute acidosis.
Acidosis, Lactic
Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis.
Acidosis, Lactic
Alterations of thiamine phosphorylation and of thiamine-dependent enzymes in Alzheimer's disease.
Acidosis, Lactic
Congenital lactic acidosis, alpha-ketoglutaric aciduria and variant form of maple syrup urine disease due to a single enzyme defect: dihydrolipoyl dehydrogenase deficiency.
Acidosis, Lactic
Deficiency of dihydrolipoyl dehydrogenase (a component of the pyruvate and alpha-ketoglutarate dehydrogenase complexes): a cause of congenital chronic lactic acidosis in infancy.
Acidosis, Lactic
Lactic acidosis in three sibs due to defects in both pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase complexes.
Acidosis, Lactic
Mutations in the lipoyltransferase LIPT1 gene cause a fatal disease associated with a specific lipoylation defect of the 2-ketoacid dehydrogenase complexes.
Adenocarcinoma
The CIMP-high phenotype is associated with energy metabolism alterations in colon adenocarcinoma.
Adenocarcinoma of Lung
Activation of Mitochondrial 2-Oxoglutarate Dehydrogenase by Cocarboxylase in Human Lung Adenocarcinoma Cells A549 Is p53/p21-Dependent and Impairs Cellular Redox State, Mimicking the Cisplatin Action.
Alzheimer Disease
Abnormality of the alpha-ketoglutarate dehydrogenase complex in fibroblasts from familial Alzheimer's disease.
Alzheimer Disease
Alpha-ketoglutarate dehydrogenase in Alzheimer brains bearing the APP670/671 mutation.
Alzheimer Disease
An update on the role of mitochondrial ?-ketoglutarate dehydrogenase in oxidative stress.
Alzheimer Disease
Association of the dihydrolipoamide dehydrogenase gene with Alzheimer's disease in an Ashkenazi Jewish population.
Alzheimer Disease
Benfotiamine treatment activates the Nrf2/ARE pathway and is neuroprotective in a transgenic mouse model of tauopathy.
Alzheimer Disease
Beta-amyloid inhibits integrated mitochondrial respiration and key enzyme activities.
Alzheimer Disease
Brain alpha-ketoglutarate dehydrogenase complex activity in Alzheimer's disease.
Alzheimer Disease
Brain energy metabolizing enzymes in Alzheimer's disease: alpha-ketoglutarate dehydrogenase complex and cytochrome oxidase.
Alzheimer Disease
Brain protein and alpha-ketoglutarate dehydrogenase complex activity in Alzheimer's disease.
Alzheimer Disease
Cause and consequence: Mitochondrial dysfunction initiates and propagates neuronal dysfunction, neuronal death and behavioral abnormalities in age-associated neurodegenerative diseases.
Alzheimer Disease
Cerebrospinal fluid levels of thiamine in patients with Alzheimer's disease.
Alzheimer Disease
Computational prediction of changes in brain metabolic fluxes during Parkinson's disease from mRNA expression.
Alzheimer Disease
Human 2-Oxoglutarate Dehydrogenase and 2-Oxoadipate Dehydrogenase Both Generate Superoxide/H2O2 in a Side Reaction and Each Could Contribute to Oxidative Stress in Mitochondria.
Alzheimer Disease
Implications for altered glutamate and GABA metabolism in the dorsolateral prefrontal cortex of aged schizophrenic patients.
Alzheimer Disease
Inhibition of ?-ketoglutarate-and pyruvate dehydrogenase complexes in E. coli by a glutathione S-transferase containing a pathological length poly-Q domain: A possible role of energy deficit in neurological diseases associated with poly-Q expansions?
Alzheimer Disease
Inhibition of the alpha-ketoglutarate dehydrogenase complex by the myeloperoxidase products, hypochlorous acid and mono-N-chloramine.
Alzheimer Disease
Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity.
Alzheimer Disease
Mitochondrial damage in Alzheimer's disease varies with apolipoprotein E genotype.
Alzheimer Disease
Mitochondrial enzymes and endoplasmic reticulum calcium stores as targets of oxidative stress in neurodegenerative diseases.
Alzheimer Disease
Modulation by DLST of the genetic risk of Alzheimer's disease in a very elderly population.
Alzheimer Disease
Normal glutamate metabolism in Alzheimer's disease fibroblasts deficient in alpha-ketoglutarate dehydrogenase complex activity.
Alzheimer Disease
Novel functions of the alpha-ketoglutarate dehydrogenase complex may mediate diverse oxidant-induced changes in mitochondrial enzymes associated with Alzheimer's disease.
Alzheimer Disease
Oxidative stress and a key metabolic enzyme in Alzheimer brains, cultured cells, and an animal model of chronic oxidative deficits.
Alzheimer Disease
Oxidative stress increases internal calcium stores and reduces a key mitochondrial enzyme.
Alzheimer Disease
Pseudogene of dihydrolipoyl succinyltransferase (E2k) found by PCR amplification and direct sequencing of rodent-human cell hybrid DNAs.
Alzheimer Disease
Quantitative alpha-ketoglutarate dehydrogenase activity staining in brain sections and in cultured cells.
Alzheimer Disease
Structural basis of the dysfunctioning of human 2-oxo acid dehydrogenase complexes.
Alzheimer Disease
The alpha-ketoglutarate dehydrogenase complex in neurodegeneration.
Alzheimer Disease
The Role of the Rare Variants in the Genes Encoding the Alpha-Ketoglutarate Dehydrogenase in Alzheimer's Disease.
Alzheimer Disease
Thiamine-dependent enzyme changes in temporal cortex of patients with Alzheimer's disease.
Anemia, Megaloblastic
Diabetes mellitus, thiamine-dependent megaloblastic anemia, and sensorineural deafness associated with deficient alpha-ketoglutarate dehydrogenase activity.
Anemia, Sideroblastic
Diabetes mellitus, thiamine-dependent megaloblastic anemia, and sensorineural deafness associated with deficient alpha-ketoglutarate dehydrogenase activity.
Brain Diseases
Activities of thiamine-dependent enzymes in two experimental models of thiamine-deficiency encephalopathy. 2. alpha-Ketoglutarate dehydrogenase.
Brain Diseases
Brain protein and alpha-ketoglutarate dehydrogenase complex activity in Alzheimer's disease.
Brain Diseases
Effects of thiamine deficiency on brain metabolism: implications for the pathogenesis of the Wernicke-Korsakoff syndrome.
Brain Diseases
Oxidative metabolites of 5-S-cysteinylnorepinephrine are irreversible inhibitors of mitochondrial complex I and the alpha-ketoglutarate dehydrogenase and pyruvate dehydrogenase complexes: possible implications for neurodegenerative brain disorders.
Brain Diseases
The alpha-ketoglutarate dehydrogenase complex.
Brain Injuries, Traumatic
Thiamine preserves mitochondrial function in a rat model of traumatic brain injury, preventing inactivation of the 2-oxoglutarate dehydrogenase complex.
Breast Neoplasms
Differential promoter methylation of kinesin family member 1a in plasma is associated with breast cancer and DNA repair capacity.
Breast Neoplasms
Discovery of rare coding variants in OGDHL and BRCA2 in relation to breast cancer risk in Chinese women.
Breast Neoplasms
Identification of a metabolic and canonical biomarker signature in Mexican HR+/HER2-, triple positive and triple-negative breast cancer patients.
Breast Neoplasms
Sources of superoxide/H2O2 during mitochondrial proline oxidation.
Carcinogenesis
A Novel Oxoglutarate Dehydrogenase-Like Mediated miR-214/TWIST1 Negative Feedback Loop Inhibits Pancreatic Cancer Growth and Metastasis.
Carcinogenesis
Mitochondrial Protein Lipoylation and the 2-Oxoglutarate Dehydrogenase Complex Controls HIF1? Stability in Aerobic Conditions.
Carcinogenesis
OGDHL is a modifier of AKT-dependent signaling and NF-?B function.
Carcinoma
A Novel Oxoglutarate Dehydrogenase-Like Mediated miR-214/TWIST1 Negative Feedback Loop Inhibits Pancreatic Cancer Growth and Metastasis.
Carcinoma, Hepatocellular
Calcium sensitive isocitrate and 2-oxoglutarate dehydrogenase activities in rat liver and AS-30D hepatoma mitochondria.
Carcinoma, Hepatocellular
Chemopreventive efficacy of selenium against N-nitrosodiethylamine-induced hepatoma in albino rats.
Carcinoma, Hepatocellular
Mitochondrial free fatty acid ?-oxidation supports oxidative phosphorylation and proliferation in cancer cells.
Carcinoma, Hepatocellular
OGDHL silencing promotes hepatocellular carcinoma by reprogramming glutamine metabolism.
Cardiomyopathies
Osteopontin Promotes Left Ventricular Diastolic Dysfunction Through a Mitochondrial Pathway.
Charcot-Marie-Tooth Disease
A nonsense mutation in DHTKD1 causes Charcot-Marie-Tooth disease type 2 in a large Chinese pedigree.
Charcot-Marie-Tooth Disease
DHTKD1 Deficiency Causes Charcot-Marie-Tooth Disease in Mice.
Charcot-Marie-Tooth Disease
Human 2-Oxoglutarate Dehydrogenase and 2-Oxoadipate Dehydrogenase Both Generate Superoxide/H2O2 in a Side Reaction and Each Could Contribute to Oxidative Stress in Mitochondria.
Charcot-Marie-Tooth Disease
Inhibition and Crystal Structure of the Human DHTKD1-Thiamin Diphosphate Complex.
Cholestasis
[Activity of carbohydrate metabolism enzyme activity in normal and pathologic parodontal tissues]
Colitis, Ulcerative
Emerging views of mitophagy in immunity and autoimmune diseases.
Colonic Neoplasms
Modulating effect of Withania somnifera on TCA cycle enzymes and electron transport chain in azoxymethane-induced colon cancer in mice.
Colorectal Neoplasms
Aberrant hypermethylation of OGDHL gene promoter in sporadic colorectal cancer.
Coma
[Intensity of glycolysis and activity of energy metabolism enzymes in rat brain after multiple exposures to hypoglycemic doses of insulin]
Coronary Disease
[The relation between oxidative processes and the glycogen content in the heart and liver of rabbits with chronic ischemic heart disease]
Crohn Disease
Emerging views of mitophagy in immunity and autoimmune diseases.
Deafness
Diabetes mellitus, thiamine-dependent megaloblastic anemia, and sensorineural deafness associated with deficient alpha-ketoglutarate dehydrogenase activity.
Dementia
Alterations of thiamine phosphorylation and of thiamine-dependent enzymes in Alzheimer's disease.
Dementia
Mitochondrial damage in Alzheimer's disease varies with apolipoprotein E genotype.
Dementia
Mitochondrial enzymes in schizophrenia.
Diabetes Mellitus
Diabetes mellitus, thiamine-dependent megaloblastic anemia, and sensorineural deafness associated with deficient alpha-ketoglutarate dehydrogenase activity.
Diabetes Mellitus, Type 2
Dysmorphic features, frontal cerebral cavernoma and hyperglycemia in a girl with a de novo deletion of 7.23 Mb in region 7p13-p12.1.
Diabetes Mellitus, Type 2
Knock-Out of DHTKD1 Alters Mitochondrial Respiration and Function, and May Represent a Novel Pathway in Cardiometabolic Disease Risk.
Diabetic Nephropathies
Fumarate accumulation involved in renal diabetic fibrosis in Goto-Kakizaki rats.
Diabetic Neuropathies
Mercury toxicity and antioxidants: Part 1: role of glutathione and alpha-lipoic acid in the treatment of mercury toxicity.
dihydrolipoyl dehydrogenase deficiency
The expanding clinical spectrum of mitochondrial diseases.
Eosinophilic Esophagitis
Human 2-Oxoglutarate Dehydrogenase and 2-Oxoadipate Dehydrogenase Both Generate Superoxide/H2O2 in a Side Reaction and Each Could Contribute to Oxidative Stress in Mitochondria.
Eosinophilic Esophagitis
Inhibition and Crystal Structure of the Human DHTKD1-Thiamin Diphosphate Complex.
Eosinophilic Esophagitis
Whole-exome sequencing uncovers oxidoreductases DHTKD1 and OGDHL as linkers between mitochondrial dysfunction and eosinophilic esophagitis.
Epilepsy
Triheptanoin alters [U-13C6]-glucose incorporation into glycolytic intermediates and increases TCA cycling by normalizing the activities of pyruvate dehydrogenase and oxoglutarate dehydrogenase in a chronic epilepsy mouse model.
Fragile X Syndrome
Identification of distinct genes with restricted expression in the somitic mesoderm in Xenopus embryo.
Friedreich Ataxia
Immunoreactive levels of alpha-ketoglutarate dehydrogenase subunits in Friedreich's ataxia and spinocerebellar ataxia type 1.
Friedreich Ataxia
Low activities of the pyruvate and oxoglutarate dehydrogenase complexes in five patients with Friedreich's ataxia.
fumarate hydratase deficiency
The expanding clinical spectrum of mitochondrial diseases.
Glycogen Storage Disease
Studies on alpha-ketoglutaric aciduria in type I glycogenosis.
Heart Failure
Osteopontin Promotes Left Ventricular Diastolic Dysfunction Through a Mitochondrial Pathway.
Hemangioma, Cavernous, Central Nervous System
Dysmorphic features, frontal cerebral cavernoma and hyperglycemia in a girl with a de novo deletion of 7.23 Mb in region 7p13-p12.1.
Hepatic Encephalopathy
Brain alpha-ketoglutarate dehydrogenase complex: kinetic properties, regional distribution, and effects of inhibitors.
Hepatic Encephalopathy
The two catalytic components of the 2-oxoglutarate dehydrogenase complex in rat cerebral synaptic and nonsynaptic mitochondria: comparison of the response to in vitro treatment with ammonia, hyperammonemia, and hepatic encephalopathy.
Hepatolenticular Degeneration
Cu2+ toxicity inhibition of mitochondrial dehydrogenases in vitro and in vivo.
Hepatolenticular Degeneration
Free radical-mediated neurotoxicity may be caused by inhibition of mitochondrial dehydrogenases in vitro and in vivo.
Huntington Disease
Human 2-Oxoglutarate Dehydrogenase and 2-Oxoadipate Dehydrogenase Both Generate Superoxide/H2O2 in a Side Reaction and Each Could Contribute to Oxidative Stress in Mitochondria.
Huntington Disease
Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity.
Hyperlactatemia
Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis.
Hyperparathyroidism, Secondary
Impaired activity of alpha-ketoglutarate dehydrogenase of heart mitochondria in chronic renal failure: role of secondary hyperparathyroidism.
Hyperthyroidism
Effects of hyperthyroidism on glucose, glutamine and ketone-body metabolism in the gut of the rat.
Hypokinesia
[Activity of oxidative enzymes of the tricarboxylic acid cycle in the liver of rats during hypokinesia]
Hypokinesia
[Activity of various oxidases and transaminases in the rat liver in the readaptation period after hypokinesia up to 30 days]
Hypokinesia
[Changes in the mitochondrial oxidative enzyme activity in the skeletal muscles od rats during the recovery period after hypokinesia of varying duration]
Hypokinesia
[Oxidative enzyme activity of the tricarboxylic acid cycle in rat skeletal muscles in hypokinesia]
Infections
Activities of membrane bound phosphatases, transaminases and mitochondrial enzymes in white spot syndrome virus infected tissues of Fenneropenaeus indicus.
Inflammatory Bowel Diseases
Emerging views of mitophagy in immunity and autoimmune diseases.
Insulin Resistance
2-Aminoadipic acid protects against obesity and diabetes.
Insulin Resistance
GM-CSF driven myeloid cells in adipose tissue link weight gain and insulin resistance via formation of 2-aminoadipate.
Intellectual Disability
Identification of distinct genes with restricted expression in the somitic mesoderm in Xenopus embryo.
Kidney Failure, Chronic
Impaired activity of alpha-ketoglutarate dehydrogenase of heart mitochondria in chronic renal failure: role of secondary hyperparathyroidism.
Korsakoff Syndrome
No association between DLST gene and Alzheimer's disease or Wernicke-Korsakoff syndrome.
Korsakoff Syndrome
Thiamine-dependent enzyme changes in the brains of alcoholics: relationship to the Wernicke-Korsakoff syndrome.
Leigh Disease
Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase.
Liver Cirrhosis, Biliary
Antibodies against mitochondrial dehydrogenase complexes in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Autoepitope mapping and reactivity of autoantibodies to the dihydrolipoamide dehydrogenase-binding protein (E3BP) and the glycine cleavage proteins in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Demonstration of peptide-specific and cross-reactive epitopes in proteins reacting with antimitochondrial antibodies of primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Emerging views of mitophagy in immunity and autoimmune diseases.
Liver Cirrhosis, Biliary
Epitope mapping and reactivity of autoantibodies to the E2 component of 2-oxoglutarate dehydrogenase complex in primary biliary cirrhosis using recombinant 2-oxoglutarate dehydrogenase complex.
Liver Cirrhosis, Biliary
Establishment and structural analysis of human mAb to the E2 component of the 2-oxoglutarate dehydrogenase complex generated from a patient with primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Frequency of IgG and IgM autoantibodies to four specific M2 mitochondrial autoantigens in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Immunoreactivity of porcine heart dihydrolipoamide acetyl- and succinyl-transferases (PDC-E2, OGDC-E2) with primary biliary cirrhosis sera: characterization of the autoantigenic region and effects of enzymatic delipoylation and relipoylation.
Liver Cirrhosis, Biliary
Inhibition of alpha-ketoglutarate dehydrogenase activity by a distinct population of autoantibodies recognizing dihydrolipoamide succinyltransferase in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Sera from patients with tuberculosis recognize the M2a-epitope (E2-subunit of pyruvate dehydrogenase) specific for primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Sjögren's syndrome and primary biliary cirrhosis: presence of autoantibodies to purified mitochondrial 2-OXO acid dehydrogenases.
Liver Cirrhosis, Biliary
Specific reactivity of recombinant human PDC-E1 alpha in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Tubulointerstitial nephritis and Fanconi syndrome in primary biliary cirrhosis.
Liver Diseases
Autoepitope mapping and reactivity of autoantibodies to the dihydrolipoamide dehydrogenase-binding protein (E3BP) and the glycine cleavage proteins in primary biliary cirrhosis.
Liver Failure
The two catalytic components of the 2-oxoglutarate dehydrogenase complex in rat cerebral synaptic and nonsynaptic mitochondria: comparison of the response to in vitro treatment with ammonia, hyperammonemia, and hepatic encephalopathy.
Liver Neoplasms
OGDHL Expression as a Prognostic Biomarker for Liver Cancer Patients.
Liver Neoplasms
OGDHL silencing promotes hepatocellular carcinoma by reprogramming glutamine metabolism.
Lung Neoplasms
Effect of mangiferin on benzo(a)pyrene induced lung carcinogenesis in experimental Swiss albino mice.
Lung Neoplasms
Modulation of TCA cycle enzymes and electron transport chain systems in experimental lung cancer.
Lupus Erythematosus, Systemic
Emerging views of mitophagy in immunity and autoimmune diseases.
Malaria
Absence of alpha-ketoglutarate dehydrogenase activity and presence of CO2-fixing activity in Plasmodium falciparum grown in vitro in human erythrocytes.
Maple Syrup Urine Disease
Congenital lactic acidosis, alpha-ketoglutaric aciduria and variant form of maple syrup urine disease due to a single enzyme defect: dihydrolipoyl dehydrogenase deficiency.
Melanoma
Emerging views of mitophagy in immunity and autoimmune diseases.
Metabolic Diseases
Structural alterations induced by ten disease-causing mutations of human dihydrolipoamide dehydrogenase analyzed by hydrogen/deuterium-exchange mass spectrometry: Implications for the structural basis of E3 deficiency.
Migraine Disorders
Mechanisms of action of valproate: a commentatory.
Movement Disorders
A biallelic pathogenic variant in the OGDH gene results in a neurological disorder with features of a mitochondrial disease.
Muscle Hypotonia
A biallelic pathogenic variant in the OGDH gene results in a neurological disorder with features of a mitochondrial disease.
Muscle Hypotonia
Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis.
nadh:ubiquinone reductase (h+-translocating) deficiency
Studies on the pathogenesis of Parkinson's disease in Japan.
Neoplasm Metastasis
A Novel Oxoglutarate Dehydrogenase-Like Mediated miR-214/TWIST1 Negative Feedback Loop Inhibits Pancreatic Cancer Growth and Metastasis.
Neoplasm Metastasis
miR-193a-5p promotes pancreatic cancer cell metastasis through SRSF6-mediated alternative splicing of OGDHL and ECM1.
Neoplasms
A Novel Oxoglutarate Dehydrogenase-Like Mediated miR-214/TWIST1 Negative Feedback Loop Inhibits Pancreatic Cancer Growth and Metastasis.
Neoplasms
A strategically designed small molecule attacks alpha-ketoglutarate dehydrogenase in tumor cells through a redox process.
Neoplasms
Aberrant hypermethylation of OGDHL gene promoter in sporadic colorectal cancer.
Neoplasms
Activation of Mitochondrial 2-Oxoglutarate Dehydrogenase by Cocarboxylase in Human Lung Adenocarcinoma Cells A549 Is p53/p21-Dependent and Impairs Cellular Redox State, Mimicking the Cisplatin Action.
Neoplasms
Alternol eliminates excessive ATP production by disturbing Krebs cycle in prostate cancer.
Neoplasms
Differential Aspartate Usage Identifies a Subset of Cancer Cells Particularly Dependent on OGDH.
Neoplasms
Emerging views of mitophagy in immunity and autoimmune diseases.
Neoplasms
Exclusive neuronal detection of KGDHC-specific subunits in the adult human brain cortex despite pancellular protein lysine succinylation.
Neoplasms
Identification of a metabolic and canonical biomarker signature in Mexican HR+/HER2-, triple positive and triple-negative breast cancer patients.
Neoplasms
Inhibition of mitochondrial 2-oxoglutarate dehydrogenase impairs viability of cancer cells in a cell-specific metabolism-dependent manner.
Neoplasms
Mitochondrial supercomplex assembly promotes breast and endometrial tumorigenesis by metabolic alterations and enhanced hypoxia tolerance.
Neoplasms
OGDHL closely associates with tumor microenvironment and can serve as a prognostic biomarker for papillary thyroid cancer.
Neoplasms
OGDHL is a modifier of AKT-dependent signaling and NF-?B function.
Neoplasms
OGDHL silencing promotes hepatocellular carcinoma by reprogramming glutamine metabolism.
Neoplasms
PIK3CA mutant tumors depend on oxoglutarate dehydrogenase.
Neoplasms
Structure-function relationships in the 2-oxo acid dehydrogenase family: substrate-specific signatures and functional predictions for the 2-oxoglutarate dehydrogenase-like proteins.
Neoplasms
Synthesis, in vitro and in vivo activity of thiamine antagonist transketolase inhibitors.
Neoplasms
The CIMP-high phenotype is associated with energy metabolism alterations in colon adenocarcinoma.
Neoplasms
Therapeutic effect of tamoxifen and energy-modulating vitamins on carbohydrate-metabolizing enzymes in breast cancer.
Neoplasms
Toward an Understanding of the Structural and Mechanistic Aspects of Protein-Protein Interactions in 2-Oxoacid Dehydrogenase Complexes.
Neoplasms
[Downregulation of OGDHL expression is associated with promoter hypermethylation in colorectal cancer].
Nervous System Diseases
Acute sources of mitochondrial NAD+ during respiratory chain dysfunction.
Nervous System Diseases
DHTKD1 Deficiency Causes Charcot-Marie-Tooth Disease in Mice.
Nervous System Diseases
Human 2-Oxoglutarate Dehydrogenase and 2-Oxoadipate Dehydrogenase Both Generate Superoxide/H2O2 in a Side Reaction and Each Could Contribute to Oxidative Stress in Mitochondria.
Nervous System Diseases
The alpha-ketoglutarate dehydrogenase complex.
Neuroblastoma
High doses of nicotinamide prevent oxidative mitochondrial dysfunction in a cellular model and improve motor deficit in a Drosophila model of Parkinson's disease.
Neuroblastoma
Metabolic impairment elicits brain cell type-selective changes in oxidative stress and cell death in culture.
Neuroblastoma
Mild mitochondrial metabolic deficits by ?-ketoglutarate dehydrogenase inhibition cause prominent changes in intracellular autophagic signaling: Potential role in the pathobiology of Alzheimer's disease.
Neuroblastoma
Sensitivity to thiamine deficiency in cultured human cells is dependent on cell type and is enhanced in cells from thiamine-responsive megaloblastic anemia patients.
Neurodegenerative Diseases
Alpha-ketoglutarate dehydrogenase: a target and generator of oxidative stress.
Neurodegenerative Diseases
An update on the role of mitochondrial ?-ketoglutarate dehydrogenase in oxidative stress.
Neurodegenerative Diseases
Behavioral impact of the regulation of the brain 2-oxoglutarate dehydrogenase complex by synthetic phosphonate analog of 2-oxoglutarate: implications into the role of the complex in neurodegenerative diseases.
Neurodegenerative Diseases
Bioenergetics in glutaryl-coenzyme A dehydrogenase deficiency: a role for glutaryl-coenzyme A.
Neurodegenerative Diseases
Cause and consequence: Mitochondrial dysfunction initiates and propagates neuronal dysfunction, neuronal death and behavioral abnormalities in age-associated neurodegenerative diseases.
Neurodegenerative Diseases
Distribution of the alpha-ketoglutarate dehydrogenase complex in rat brain.
Neurodegenerative Diseases
Human 2-Oxoglutarate Dehydrogenase and 2-Oxoadipate Dehydrogenase Both Generate Superoxide/H2O2 in a Side Reaction and Each Could Contribute to Oxidative Stress in Mitochondria.
Neurodegenerative Diseases
Inactivation and reactivation of the mitochondrial {alpha}-ketoglutarate dehydrogenase complex.
Neurodegenerative Diseases
Inhibition of Krebs cycle enzymes by hydrogen peroxide: A key role of [alpha]-ketoglutarate dehydrogenase in limiting NADH production under oxidative stress.
Neurodegenerative Diseases
Inhibition of the alpha-ketoglutarate dehydrogenase complex by the myeloperoxidase products, hypochlorous acid and mono-N-chloramine.
Neurodegenerative Diseases
Kinetic Modeling of the Mitochondrial Energy Metabolism of Neuronal Cells: The Impact of Reduced ?-Ketoglutarate Dehydrogenase Activities on ATP Production and Generation of Reactive Oxygen Species.
Neurodegenerative Diseases
Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity.
Neurodegenerative Diseases
Mice deficient in dihydrolipoyl succinyl transferase show increased vulnerability to mitochondrial toxins.
Neurodegenerative Diseases
Mild reduction in the activity of the alpha-ketoglutarate dehydrogenase complex elevates GABA shunt and glycolysis.
Neurodegenerative Diseases
Mitochondrial enzymes and endoplasmic reticulum calcium stores as targets of oxidative stress in neurodegenerative diseases.
Neurodegenerative Diseases
Mitochondrial enzymes in schizophrenia.
Neurodegenerative Diseases
Oxidative stress and a key metabolic enzyme in Alzheimer brains, cultured cells, and an animal model of chronic oxidative deficits.
Neurodegenerative Diseases
Phosphonate analogues of alpha-ketoglutarate inhibit the activity of the alpha-ketoglutarate dehydrogenase complex isolated from brain and in cultured cells.
Neurodegenerative Diseases
Quantitative alpha-ketoglutarate dehydrogenase activity staining in brain sections and in cultured cells.
Neurodegenerative Diseases
Reduction in the E2k subunit of the alpha-ketoglutarate dehydrogenase complex has effects independent of complex activity.
Neurodegenerative Diseases
Reductions in the mitochondrial enzyme ?-ketoglutarate dehydrogenase complex in neurodegenerative disease - beneficial or detrimental?
Neurodegenerative Diseases
Selective loss of KGDHC-enriched neurons in Alzheimer temporal cortex: does mitochondrial variation contribute to selective vulnerability?
Neurodegenerative Diseases
The alpha-ketoglutarate dehydrogenase complex in neurodegeneration.
Neurodegenerative Diseases
The alpha-ketoglutarate-dehydrogenase complex: a mediator between mitochondria and oxidative stress in neurodegeneration.
Neuroinflammatory Diseases
Thiamine preserves mitochondrial function in a rat model of traumatic brain injury, preventing inactivation of the 2-oxoglutarate dehydrogenase complex.
Olivopontocerebellar Atrophies
Cerebellar alpha-ketoglutarate dehydrogenase activity is reduced in spinocerebellar ataxia type 1.
Ovarian Neoplasms
Regulation of protein metabolism in cancer.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
2-ketoglutarate dehydrogenase deficiency with intermittent 2-ketoglutaric aciduria.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
2-Ketoglutarate dehydrogenase deficiency, a rare cause of primary hyperlactataemia: report of a new case.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
A novel mutation in the dihydrolipoamide dehydrogenase E3 subunit gene (DLD) resulting in an atypical form of alpha-ketoglutarate dehydrogenase deficiency.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
DHTKD1 Deficiency Causes Charcot-Marie-Tooth Disease in Mice.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
Dysmorphic features, frontal cerebral cavernoma and hyperglycemia in a girl with a de novo deletion of 7.23 Mb in region 7p13-p12.1.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
Expression of oxidative phosphorylation genes in muscle cell cultures from patients with mitochondrial myopathies.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
Immunochemical characterization of the deficiency of the alpha-ketoglutarate dehydrogenase complex in thiamine-deficient rat brain.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
Measurement of alpha-ketoglutarate dehydrogenase activity in tissue extracts and human platelets using reversed-phase high-performance liquid chromatography.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
Metabolic intermediates in lactic acidosis: compounds, samples and interpretation.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
The alpha-ketoglutarate dehydrogenase complex in neurodegeneration.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
The alpha-ketoglutarate dehydrogenase complex.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
The expanding clinical spectrum of mitochondrial diseases.
oxoglutarate dehydrogenase (succinyl-transferring) deficiency
[Alpha-ketoglutarate dehydrogenase deficiency]
Pancreatic Neoplasms
A Novel Oxoglutarate Dehydrogenase-Like Mediated miR-214/TWIST1 Negative Feedback Loop Inhibits Pancreatic Cancer Growth and Metastasis.
Pancreatic Neoplasms
Integrative Genomic Analysis of Gemcitabine Resistance in Pancreatic Cancer by Patient-derived Xenograft Models.
Pancreatic Neoplasms
miR-193a-5p promotes pancreatic cancer cell metastasis through SRSF6-mediated alternative splicing of OGDHL and ECM1.
Paramyxoviridae Infections
Emerging views of mitophagy in immunity and autoimmune diseases.
Parkinson Disease
An immunohistochemical study on alpha-ketoglutarate dehydrogenase complex in Parkinson's disease.
Parkinson Disease
Association between the gene encoding the E2 subunit of the alpha-ketoglutarate dehydrogenase complex and Parkinson's disease.
Parkinson Disease
Cerebrospinal fluid levels of thiamine in patients with Parkinson's disease.
Parkinson Disease
Human 2-Oxoglutarate Dehydrogenase and 2-Oxoadipate Dehydrogenase Both Generate Superoxide/H2O2 in a Side Reaction and Each Could Contribute to Oxidative Stress in Mitochondria.
Parkinson Disease
Inhibition of alpha-ketoglutarate dehydrogenase by isoquinoline derivatives structurally related to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP).
Parkinson Disease
Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity.
Parkinson Disease
Mitochondrial respiratory enzyme function and superoxide dismutase activity following brain glutathione depletion in the rat.
Parkinson Disease
Oxidative alpha-ketoglutarate dehydrogenase inhibition via subtle elevations in monoamine oxidase B levels results in loss of spare respiratory capacity: implications for Parkinson's disease.
Parkinson Disease
Oxidative metabolites of 5-S-cysteinyldopamine inhibit the alpha-ketoglutarate dehydrogenase complex: possible relevance to the pathogenesis of Parkinson's disease.
Parkinson Disease
The alpha-ketoglutarate dehydrogenase complex in neurodegeneration.
Parkinson Disease
The isomers of thioctic acid alter C-deoxyglucose incorporation in rat basal ganglia.
Parkinsonian Disorders
Emerging views of mitophagy in immunity and autoimmune diseases.
Prostatic Neoplasms
Alternol eliminates excessive ATP production by disturbing Krebs cycle in prostate cancer.
Pulmonary Disease, Chronic Obstructive
The CIMP-high phenotype is associated with energy metabolism alterations in colon adenocarcinoma.
Refsum Disease
Brain pyruvate and 2-oxoglutarate dehydrogenase complexes are mitochondrial targets of the CoA ester of the Refsum disease marker phytanic acid.
Respiratory Insufficiency
Characterization of oxygen-resistant Chinese hamster ovary cells. III. Relative resistance of succinate and alpha-ketoglutarate dehydrogenases to hyperoxic inactivation.
Seizures
Activities of thiamine-dependent enzymes in two experimental models of thiamine-deficiency encephalopathy. 2. alpha-Ketoglutarate dehydrogenase.
Spinocerebellar Ataxias
Cerebellar alpha-ketoglutarate dehydrogenase activity is reduced in spinocerebellar ataxia type 1.
Spinocerebellar Ataxias
Immunoreactive levels of alpha-ketoglutarate dehydrogenase subunits in Friedreich's ataxia and spinocerebellar ataxia type 1.
Spinocerebellar Ataxias
Problems in the congenital lactic acidoses.
Spinocerebellar Degenerations
Pyruvate dehydrogenase deficiency in spinocerebellar degenerations.
Starvation
Metabolism of glucose, glutamine, long-chain fatty acids and ketone bodies by lungs of the rat.
Starvation
Studies on the interactions of Ca2+ and pyruvate in the regulation of rat heart pyruvate dehydrogenase activity. Effects of starvation and diabetes.
Starvation
[Features of the interconversion of alpha-ketoglutarate--glutamate in brain mitochondria of exothermic animals during hibernation]
Stomach Diseases
Helicobacter pylori porCDAB and oorDABC genes encode distinct pyruvate:flavodoxin and 2-oxoglutarate:acceptor oxidoreductases which mediate electron transport to NADP.
Thiamine Deficiency
Chronic alcoholism in rats induces a compensatory response, preserving brain thiamine diphosphate, but the brain 2-oxo acid dehydrogenases are inactivated despite unchanged coenzyme levels.
Thiamine Deficiency
Effects of thiamine deficiency on brain metabolism: implications for the pathogenesis of the Wernicke-Korsakoff syndrome.
Thiamine Deficiency
Inhibition of the alpha-ketoglutarate dehydrogenase complex by the myeloperoxidase products, hypochlorous acid and mono-N-chloramine.
Thiamine Deficiency
No association between DLST gene and Alzheimer's disease or Wernicke-Korsakoff syndrome.
Thiamine Deficiency
Pathophysiologic mechanisms responsible for the reversible (thiamine-responsive) and irreversible (thiamine non-responsive) neurological symptoms of Wernicke's encephalopathy.
Thiamine Deficiency
Responses of the mitochondrial alpha-ketoglutarate dehydrogenase complex to thiamine deficiency may contribute to regional selective vulnerability.
Thiamine Deficiency
Role of mitochondrial dysfunction and oxidative stress in the pathogenesis of selective neuronal loss in Wernicke's encephalopathy.
Thiamine Deficiency
Thiamine deficiency decreases steady-state transketolase and pyruvate dehydrogenase but not alpha-ketoglutarate dehydrogenase mRNA levels in three human cell types.
Thiamine Deficiency
Thiamine deficiency-related brain dysfunction in chronic liver failure.
Thiamine Deficiency
Thiamine pyrophosphate-requiring enzymes are altered during pyrithiamine-induced thiamine deficiency in cultured human lymphoblasts.
Thiamine Deficiency
[Characteristics of carbohydrate metabolism in the rat liver in thiamine deficiency]
Thiamine Deficiency
[The activity of 2-oxoacid-dehydrogenases in thiamine deficiency of the rat, with a contribution to the activity measurement of oxoglutarate dehydrogenase]
Thyroid Cancer, Papillary
OGDHL closely associates with tumor microenvironment and can serve as a prognostic biomarker for papillary thyroid cancer.
Tuberculosis
An anaerobic-type alpha-ketoglutarate ferredoxin oxidoreductase completes the oxidative tricarboxylic acid cycle of Mycobacterium tuberculosis.
Tuberculosis
Corynebacterial protein kinase G controls 2-oxoglutarate dehydrogenase activity via the phosphorylation status of the OdhI protein.
Tuberculosis
Functional Plasticity and Allosteric Regulation of ?-Ketoglutarate Decarboxylase in Central Mycobacterial Metabolism.
Tuberculosis
Mycobacterium tuberculosis appears to lack alpha-ketoglutarate dehydrogenase and encodes pyruvate dehydrogenase in widely separated genes.
Tuberculosis
The glyoxylate shunt is essential for CO2-requiring oligotrophic growth of Rhodococcus erythropolis N9T-4.
Uterine Cervical Neoplasms
OGDHL is a modifier of AKT-dependent signaling and NF-?B function.
Wernicke Encephalopathy
Activities of thiamine-dependent enzymes in two experimental models of thiamine-deficiency encephalopathy. 2. alpha-Ketoglutarate dehydrogenase.
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1.2.4.2 oxoglutarate dehydrogenase (succinyl-transferring)
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The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable). 
