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Disease on EC 1.2.4.2 - oxoglutarate dehydrogenase (succinyl-transferring)

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Acidosis
Activation of oxoglutarate dehydrogenase in the kidney in response to acute acidosis.
Adaptation of renal tricarboxylic acid cycle metabolism to various acid-base states: study with [3-13C,5-15N]glutamine.
Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis.
Ammoniagenesis by cultured human renal cortical epithelial cells: study with 15N.
Biochemical pathways and modulators of renal ammoniagenesis.
Intramitochondrial pH and ammonium production in rat and dog kidney cortex.
Regulation of rat kidney mitochondrial metabolism in acute acidosis.
Acidosis, Lactic
Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis.
Alterations of thiamine phosphorylation and of thiamine-dependent enzymes in Alzheimer's disease.
Congenital lactic acidosis, alpha-ketoglutaric aciduria and variant form of maple syrup urine disease due to a single enzyme defect: dihydrolipoyl dehydrogenase deficiency.
Deficiency of dihydrolipoyl dehydrogenase (a component of the pyruvate and alpha-ketoglutarate dehydrogenase complexes): a cause of congenital chronic lactic acidosis in infancy.
Lactic acidosis in three sibs due to defects in both pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase complexes.
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.
Alpha-ketoglutarate dehydrogenase in Alzheimer brains bearing the APP670/671 mutation.
An update on the role of mitochondrial ?-ketoglutarate dehydrogenase in oxidative stress.
Association of the dihydrolipoamide dehydrogenase gene with Alzheimer's disease in an Ashkenazi Jewish population.
Benfotiamine treatment activates the Nrf2/ARE pathway and is neuroprotective in a transgenic mouse model of tauopathy.
Beta-amyloid inhibits integrated mitochondrial respiration and key enzyme activities.
Brain alpha-ketoglutarate dehydrogenase complex activity in Alzheimer's disease.
Brain energy metabolizing enzymes in Alzheimer's disease: alpha-ketoglutarate dehydrogenase complex and cytochrome oxidase.
Brain protein and alpha-ketoglutarate dehydrogenase complex activity in Alzheimer's disease.
Cause and consequence: Mitochondrial dysfunction initiates and propagates neuronal dysfunction, neuronal death and behavioral abnormalities in age-associated neurodegenerative diseases.
Cerebrospinal fluid levels of thiamine in patients with Alzheimer's disease.
Computational prediction of changes in brain metabolic fluxes during Parkinson's disease from mRNA expression.
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.
Implications for altered glutamate and GABA metabolism in the dorsolateral prefrontal cortex of aged schizophrenic patients.
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?
Inhibition of the alpha-ketoglutarate dehydrogenase complex by the myeloperoxidase products, hypochlorous acid and mono-N-chloramine.
Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity.
Mitochondrial damage in Alzheimer's disease varies with apolipoprotein E genotype.
Mitochondrial enzymes and endoplasmic reticulum calcium stores as targets of oxidative stress in neurodegenerative diseases.
Modulation by DLST of the genetic risk of Alzheimer's disease in a very elderly population.
Normal glutamate metabolism in Alzheimer's disease fibroblasts deficient in alpha-ketoglutarate dehydrogenase complex activity.
Novel functions of the alpha-ketoglutarate dehydrogenase complex may mediate diverse oxidant-induced changes in mitochondrial enzymes associated with Alzheimer's disease.
Oxidative stress and a key metabolic enzyme in Alzheimer brains, cultured cells, and an animal model of chronic oxidative deficits.
Oxidative stress increases internal calcium stores and reduces a key mitochondrial enzyme.
Pseudogene of dihydrolipoyl succinyltransferase (E2k) found by PCR amplification and direct sequencing of rodent-human cell hybrid DNAs.
Quantitative alpha-ketoglutarate dehydrogenase activity staining in brain sections and in cultured cells.
Structural basis of the dysfunctioning of human 2-oxo acid dehydrogenase complexes.
The alpha-ketoglutarate dehydrogenase complex in neurodegeneration.
The Role of the Rare Variants in the Genes Encoding the Alpha-Ketoglutarate Dehydrogenase in Alzheimer's 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 protein and alpha-ketoglutarate dehydrogenase complex activity in Alzheimer's disease.
Effects of thiamine deficiency on brain metabolism: implications for the pathogenesis of the Wernicke-Korsakoff syndrome.
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.
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.
Discovery of rare coding variants in OGDHL and BRCA2 in relation to breast cancer risk in Chinese women.
Identification of a metabolic and canonical biomarker signature in Mexican HR+/HER2-, triple positive and triple-negative breast cancer patients.
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.
Mitochondrial Protein Lipoylation and the 2-Oxoglutarate Dehydrogenase Complex Controls HIF1? Stability in Aerobic Conditions.
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.
Chemopreventive efficacy of selenium against N-nitrosodiethylamine-induced hepatoma in albino rats.
Mitochondrial free fatty acid ?-oxidation supports oxidative phosphorylation and proliferation in cancer cells.
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.
DHTKD1 Deficiency Causes Charcot-Marie-Tooth Disease in Mice.
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.
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.
Mitochondrial damage in Alzheimer's disease varies with apolipoprotein E genotype.
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.
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.
Inhibition and Crystal Structure of the Human DHTKD1-Thiamin Diphosphate Complex.
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.
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.
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.
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.
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]
[Activity of various oxidases and transaminases in the rat liver in the readaptation period after hypokinesia up to 30 days]
[Changes in the mitochondrial oxidative enzyme activity in the skeletal muscles od rats during the recovery period after hypokinesia of varying duration]
[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.
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.
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.
Autoepitope mapping and reactivity of autoantibodies to the dihydrolipoamide dehydrogenase-binding protein (E3BP) and the glycine cleavage proteins in primary biliary cirrhosis.
Demonstration of peptide-specific and cross-reactive epitopes in proteins reacting with antimitochondrial antibodies of primary biliary cirrhosis.
Emerging views of mitophagy in immunity and autoimmune diseases.
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.
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.
Frequency of IgG and IgM autoantibodies to four specific M2 mitochondrial autoantigens in primary biliary cirrhosis.
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.
Inhibition of alpha-ketoglutarate dehydrogenase activity by a distinct population of autoantibodies recognizing dihydrolipoamide succinyltransferase in primary biliary cirrhosis.
Sera from patients with tuberculosis recognize the M2a-epitope (E2-subunit of pyruvate dehydrogenase) specific for primary biliary cirrhosis.
Sjögren's syndrome and primary biliary cirrhosis: presence of autoantibodies to purified mitochondrial 2-OXO acid dehydrogenases.
Specific reactivity of recombinant human PDC-E1 alpha in primary biliary cirrhosis.
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.
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.
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.
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.
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.
A strategically designed small molecule attacks alpha-ketoglutarate dehydrogenase in tumor cells through a redox process.
Aberrant hypermethylation of OGDHL gene promoter in sporadic colorectal cancer.
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.
Alternol eliminates excessive ATP production by disturbing Krebs cycle in prostate cancer.
Differential Aspartate Usage Identifies a Subset of Cancer Cells Particularly Dependent on OGDH.
Emerging views of mitophagy in immunity and autoimmune diseases.
Exclusive neuronal detection of KGDHC-specific subunits in the adult human brain cortex despite pancellular protein lysine succinylation.
Identification of a metabolic and canonical biomarker signature in Mexican HR+/HER2-, triple positive and triple-negative breast cancer patients.
Inhibition of mitochondrial 2-oxoglutarate dehydrogenase impairs viability of cancer cells in a cell-specific metabolism-dependent manner.
Mitochondrial supercomplex assembly promotes breast and endometrial tumorigenesis by metabolic alterations and enhanced hypoxia tolerance.
OGDHL closely associates with tumor microenvironment and can serve as a prognostic biomarker for papillary thyroid cancer.
OGDHL is a modifier of AKT-dependent signaling and NF-?B function.
OGDHL silencing promotes hepatocellular carcinoma by reprogramming glutamine metabolism.
PIK3CA mutant tumors depend on oxoglutarate dehydrogenase.
Structure-function relationships in the 2-oxo acid dehydrogenase family: substrate-specific signatures and functional predictions for the 2-oxoglutarate dehydrogenase-like proteins.
Synthesis, in vitro and in vivo activity of thiamine antagonist transketolase inhibitors.
The CIMP-high phenotype is associated with energy metabolism alterations in colon adenocarcinoma.
Therapeutic effect of tamoxifen and energy-modulating vitamins on carbohydrate-metabolizing enzymes in breast cancer.
Toward an Understanding of the Structural and Mechanistic Aspects of Protein-Protein Interactions in 2-Oxoacid Dehydrogenase Complexes.
[Downregulation of OGDHL expression is associated with promoter hypermethylation in colorectal cancer].
Nervous System Diseases
Acute sources of mitochondrial NAD+ during respiratory chain dysfunction.
DHTKD1 Deficiency Causes Charcot-Marie-Tooth Disease in Mice.
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.
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.
Metabolic impairment elicits brain cell type-selective changes in oxidative stress and cell death in culture.
Mild mitochondrial metabolic deficits by ?-ketoglutarate dehydrogenase inhibition cause prominent changes in intracellular autophagic signaling: Potential role in the pathobiology of Alzheimer's disease.
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.
An update on the role of mitochondrial ?-ketoglutarate dehydrogenase in oxidative stress.
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.
Bioenergetics in glutaryl-coenzyme A dehydrogenase deficiency: a role for glutaryl-coenzyme A.
Cause and consequence: Mitochondrial dysfunction initiates and propagates neuronal dysfunction, neuronal death and behavioral abnormalities in age-associated neurodegenerative diseases.
Distribution of the alpha-ketoglutarate dehydrogenase complex in rat brain.
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.
Inactivation and reactivation of the mitochondrial {alpha}-ketoglutarate dehydrogenase complex.
Inhibition of Krebs cycle enzymes by hydrogen peroxide: A key role of [alpha]-ketoglutarate dehydrogenase in limiting NADH production under oxidative stress.
Inhibition of the alpha-ketoglutarate dehydrogenase complex by the myeloperoxidase products, hypochlorous acid and mono-N-chloramine.
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.
Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity.
Mice deficient in dihydrolipoyl succinyl transferase show increased vulnerability to mitochondrial toxins.
Mild reduction in the activity of the alpha-ketoglutarate dehydrogenase complex elevates GABA shunt and glycolysis.
Mitochondrial enzymes and endoplasmic reticulum calcium stores as targets of oxidative stress in neurodegenerative diseases.
Mitochondrial enzymes in schizophrenia.
Oxidative stress and a key metabolic enzyme in Alzheimer brains, cultured cells, and an animal model of chronic oxidative deficits.
Phosphonate analogues of alpha-ketoglutarate inhibit the activity of the alpha-ketoglutarate dehydrogenase complex isolated from brain and in cultured cells.
Quantitative alpha-ketoglutarate dehydrogenase activity staining in brain sections and in cultured cells.
Reduction in the E2k subunit of the alpha-ketoglutarate dehydrogenase complex has effects independent of complex activity.
Reductions in the mitochondrial enzyme ?-ketoglutarate dehydrogenase complex in neurodegenerative disease - beneficial or detrimental?
Selective loss of KGDHC-enriched neurons in Alzheimer temporal cortex: does mitochondrial variation contribute to selective vulnerability?
The alpha-ketoglutarate dehydrogenase complex in neurodegeneration.
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.
2-Ketoglutarate dehydrogenase deficiency, a rare cause of primary hyperlactataemia: report of a new case.
A novel mutation in the dihydrolipoamide dehydrogenase E3 subunit gene (DLD) resulting in an atypical form of alpha-ketoglutarate dehydrogenase deficiency.
Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis.
DHTKD1 Deficiency Causes Charcot-Marie-Tooth Disease in Mice.
Dysmorphic features, frontal cerebral cavernoma and hyperglycemia in a girl with a de novo deletion of 7.23 Mb in region 7p13-p12.1.
Expression of oxidative phosphorylation genes in muscle cell cultures from patients with mitochondrial myopathies.
Immunochemical characterization of the deficiency of the alpha-ketoglutarate dehydrogenase complex in thiamine-deficient rat brain.
Measurement of alpha-ketoglutarate dehydrogenase activity in tissue extracts and human platelets using reversed-phase high-performance liquid chromatography.
Metabolic intermediates in lactic acidosis: compounds, samples and interpretation.
The alpha-ketoglutarate dehydrogenase complex in neurodegeneration.
The alpha-ketoglutarate dehydrogenase complex.
The expanding clinical spectrum of mitochondrial diseases.
[Alpha-ketoglutarate dehydrogenase deficiency]
Pancreatic Neoplasms
A Novel Oxoglutarate Dehydrogenase-Like Mediated miR-214/TWIST1 Negative Feedback Loop Inhibits Pancreatic Cancer Growth and Metastasis.
Integrative Genomic Analysis of Gemcitabine Resistance in Pancreatic Cancer by Patient-derived Xenograft Models.
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.
Association between the gene encoding the E2 subunit of the alpha-ketoglutarate dehydrogenase complex and Parkinson's disease.
Cerebrospinal fluid levels of thiamine in patients with Parkinson's 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.
Inhibition of alpha-ketoglutarate dehydrogenase by isoquinoline derivatives structurally related to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP).
Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity.
Mitochondrial respiratory enzyme function and superoxide dismutase activity following brain glutathione depletion in the rat.
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.
Oxidative metabolites of 5-S-cysteinyldopamine inhibit the alpha-ketoglutarate dehydrogenase complex: possible relevance to the pathogenesis of Parkinson's disease.
The alpha-ketoglutarate dehydrogenase complex in neurodegeneration.
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.
Immunoreactive levels of alpha-ketoglutarate dehydrogenase subunits in Friedreich's ataxia and spinocerebellar ataxia type 1.
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.
Studies on the interactions of Ca2+ and pyruvate in the regulation of rat heart pyruvate dehydrogenase activity. Effects of starvation and diabetes.
[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.
Effects of thiamine deficiency on brain metabolism: implications for the pathogenesis of the Wernicke-Korsakoff syndrome.
Inhibition of the alpha-ketoglutarate dehydrogenase complex by the myeloperoxidase products, hypochlorous acid and mono-N-chloramine.
No association between DLST gene and Alzheimer's disease or Wernicke-Korsakoff syndrome.
Pathophysiologic mechanisms responsible for the reversible (thiamine-responsive) and irreversible (thiamine non-responsive) neurological symptoms of Wernicke's encephalopathy.
Responses of the mitochondrial alpha-ketoglutarate dehydrogenase complex to thiamine deficiency may contribute to regional selective vulnerability.
Role of mitochondrial dysfunction and oxidative stress in the pathogenesis of selective neuronal loss in Wernicke's encephalopathy.
Thiamine deficiency decreases steady-state transketolase and pyruvate dehydrogenase but not alpha-ketoglutarate dehydrogenase mRNA levels in three human cell types.
Thiamine deficiency-related brain dysfunction in chronic liver failure.
Thiamine pyrophosphate-requiring enzymes are altered during pyrithiamine-induced thiamine deficiency in cultured human lymphoblasts.
[Characteristics of carbohydrate metabolism in the rat liver in 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.
Corynebacterial protein kinase G controls 2-oxoglutarate dehydrogenase activity via the phosphorylation status of the OdhI protein.
Functional Plasticity and Allosteric Regulation of ?-Ketoglutarate Decarboxylase in Central Mycobacterial Metabolism.
Mycobacterium tuberculosis appears to lack alpha-ketoglutarate dehydrogenase and encodes pyruvate dehydrogenase in widely separated genes.
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.