Disease on EC 1.2.1.51 - pyruvate dehydrogenase (NADP+)
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3-hydroxyisobutyryl-coa hydrolase deficiency
Paroxysmal Dyskinesias Revealing 3-Hydroxy-Isobutyryl-CoA Hydrolase (HIBCH) Deficiency.
Acanthosis Nigricans
Sensitivity of pyruvate dehydrogenase to insulin in activated T lymphocytes. Lack of responsiveness to insulin in patients with polycystic ovarian disease and diabetes.
Acidosis
"Cerebral" lactic acidosis: defects in pyruvate metabolism with profound brain damage and minimal systemic acidosis.
Acidosis
Continuous pH monitoring using the Paratrend 7 inserted into a peripheral vein in a patient with shock and congenital lactic acidosis.
Acidosis
Dichloroacetate attenuates myocardial acidosis and metabolic changes induced by partial occlusion of the coronary artery in dogs.
Acidosis
Effect of induced metabolic acidosis on human skeletal muscle metabolism during exercise.
Acidosis
Effect of phenformin on the metabolism of glucose, pyruvate and acetate in guinea-pig heart.
Acidosis
Effects of work and acidosis on pyruvate dehydrogenase activity in perfused rat hearts.
Acidosis
[Lactic acid acidosis with mitochondrial myopathy due to a pyruvate dehydrogenase deficiency]
Acidosis
[Sodium dichloracetate: its application to the therapeutics of experimental hyperiactatemia (author's transl)]
Acidosis, Lactic
A new case of pyruvate dehydrogenase deficiency due to a novel mutation in the PDX1 gene.
Acidosis, Lactic
A pathogenic glutamate-to-aspartate substitution (D296E) in the pyruvate dehydrogenase E1 subunit gene PDHA1.
Acidosis, Lactic
A zebrafish model for pyruvate dehydrogenase deficiency: rescue of neurological dysfunction and embryonic lethality using a ketogenic diet.
Acidosis, Lactic
Cerebral palsy and pyruvate dehydrogenase deficiency: identification of two new mutations in the E1alpha gene.
Acidosis, Lactic
Clinical and genetic spectrum of pyruvate dehydrogenase deficiency: dihydrolipoamide acetyltransferase (E2) deficiency.
Acidosis, Lactic
Clinical manifestations in two patients with pyruvate dehydrogenase deficiency and long-term survival.
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
Congenital lactic acidosis: evaluation of the properties of the a199t natural variant of human pyruvate dehydrogenase e1alpha by in vitro mutation.
Acidosis, Lactic
Continuous pH monitoring using the Paratrend 7 inserted into a peripheral vein in a patient with shock and congenital lactic acidosis.
Acidosis, Lactic
Deficiency of pyruvate dehydrogenase caused by novel and known mutations in the E1alpha subunit.
Acidosis, Lactic
Deficiency of the alpha and beta subunits of pyruvate dehydrogenase in a patient with lactic acidosis and unexpected sudden death.
Acidosis, Lactic
Deficiency of the pyruvate dehydrogenase complex and of mitochondrial fatty acid oxidation.
Acidosis, Lactic
Detection of pyruvate dehydrogenase E1 alpha-subunit deficiencies in females by immunohistochemical demonstration of mosaicism in cultured fibroblasts.
Acidosis, Lactic
Diagnostic accuracy of blood lactate-to-pyruvate molar ratio in the differential diagnosis of congenital lactic acidosis.
Acidosis, Lactic
Dichloroacetate for lactic acidosis in severe malaria: a pharmacokinetic and pharmacodynamic assessment.
Acidosis, Lactic
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
Acidosis, Lactic
Effect of dichloracetate on infarct size in a primate model of focal cerebral ischaemia.
Acidosis, Lactic
Fatal lactic acidosis in a newborn attributable to a congenital defect of pyruvate dehydrogenase.
Acidosis, Lactic
Follow-up of a child with pyruvate dehydrogenase deficiency on a less restrictive ketogenic diet.
Acidosis, Lactic
Heterogeneous expression of protein and mRNA in pyruvate dehydrogenase deficiency.
Acidosis, Lactic
Hyperammonaemia and lactic acidosis in a patient with pyruvate dehydrogenase deficiency.
Acidosis, Lactic
Immunochemical analysis of normal and mutant forms of human pyruvate dehydrogenase.
Acidosis, Lactic
In utero central nervous system damage in pyruvate dehydrogenase deficiency.
Acidosis, Lactic
Lactic acidosis and developmental delay due to deficiency of E3 binding protein (protein X) of the pyruvate dehydrogenase complex.
Acidosis, Lactic
Lactic acidosis caused by repressed lactate dehydrogenase subunit B expression down-regulates mitochondrial oxidative phosphorylation via the pyruvate dehydrogenase (PDH)-PDH kinase axis.
Acidosis, Lactic
Lactic acidosis in three sibs due to defects in both pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase complexes.
Acidosis, Lactic
Leigh syndrome: pyruvate dehydrogenase defect. A case with peripheral neuropathy.
Acidosis, Lactic
Mutations in the lipoyltransferase LIPT1 gene cause a fatal disease associated with a specific lipoylation defect of the 2-ketoacid dehydrogenase complexes.
Acidosis, Lactic
Mutations in the X-linked pyruvate dehydrogenase (E1) alpha subunit gene (PDHA1) in patients with a pyruvate dehydrogenase complex deficiency.
Acidosis, Lactic
Neonatal pyruvate dehydrogenase deficiency due to a R302H mutation in the PDHA1 gene: MRI findings.
Acidosis, Lactic
Prenatal diagnosis of pyruvate dehydrogenase deficiency using magnetic resonance imaging.
Acidosis, Lactic
Pyruvate dehydrogenase complex deficiency is linked to regulatory loop disorder in the ?V138M variant of human pyruvate dehydrogenase.
Acidosis, Lactic
Pyruvate dehydrogenase deficiency presenting as isolated paroxysmal exercise induced dystonia successfully reversed with thiamine supplementation. Case report and mini-review.
Acidosis, Lactic
Pyruvate dehydrogenase deficiency. Clinical presentation and molecular genetic characterization of five new patients.
Acidosis, Lactic
Pyruvate dehydrogenase E1alpha subunit deficiency in a female patient: evidence of antenatal origin of brain damage and possible etiology of infantile spasms.
Acidosis, Lactic
Pyruvate dehydrogenase subcomplex with lipoamide dehydrogenase deficiency in a patient with lactic acidosis and branched chain ketoaciduria.
Acidosis, Lactic
Reconciling diabetes management and the ketogenic diet in a child with pyruvate dehydrogenase deficiency.
Acidosis, Lactic
Sensitivity to carbohydrate in a patient with familial intermittent lactic acidosis and pyruvate dehydrogenase deficiency.
Acidosis, Lactic
Sodium 2-chloropropionate: its effects on experimental hyperlactatemia in the dog.
Acidosis, Lactic
Splicing error in E1alpha pyruvate dehydrogenase mRNA caused by novel intronic mutation responsible for lactic acidosis and mental retardation.
Acidosis, Lactic
The SR protein SC35 is responsible for aberrant splicing of the E1alpha pyruvate dehydrogenase mRNA in a case of mental retardation with lactic acidosis.
Acidosis, Lactic
Unilateral periventricular leukomalacia in association with pyruvate dehydrogenase deficiency.
Acidosis, Lactic
Variant non ketotic hyperglycinemia is caused by mutations in LIAS, BOLA3 and the novel gene GLRX5.
Acidosis, Lactic
Vitamin-responsive pyruvate dehydrogenase deficiency in a young girl with external ophthalmoplegia, myopathy and lactic acidosis.
Acidosis, Lactic
[A case of congenital lactic acidosis caused by deficiency of pyruvate dehydrogenase]
Acidosis, Lactic
[Pyruvate dehydrogenase deficiency in a child with persistent lactic acidosis]
Acidosis, Lactic
[Pyruvate dehydrogenase deficit associated to the C515T mutation in exon 6 of the E1alpha gene.]
acyl-coa dehydrogenase deficiency
Clinical Therapeutic Management of Human Mitochondrial Disorders.
Adenocarcinoma
Pyruvate dehydrogenase, the citrate condensing enzyme and the utilization of 14 C-labeled lactate, pyruvate and alanine by slices of lactating mammary gland and adenocarcinoma of mouse mammary gland.
Adenocarcinoma of Lung
PDK4 promotes tumorigenesis and cisplatin resistance in lung adenocarcinoma via transcriptional regulation of EPAS1.
Adenocarcinoma of Lung
The microRNA-182-PDK4 axis regulates lung tumorigenesis by modulating pyruvate dehydrogenase and lipogenesis.
Agenesis of Corpus Callosum
Morphological correlates of mitochondrial dysfunction in children.
Aicardi Syndrome
The clinical spectrum of nodular heterotopias in children: Report of 31 patients.
Alkalosis
Effect of induced metabolic alkalosis on human skeletal muscle metabolism during exercise.
Alzheimer Disease
A?PP-Transgenic 2576 Mice Mimic Cell Type-Specific Aspects of Acetyl-CoA-Linked Metabolic Deficits in Alzheimer's Disease.
Alzheimer Disease
An immunochemical study of the pyruvate dehydrogenase deficit in Alzheimer's disease brain.
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
Coenzyme A-acetylating enzymes in Alzheimer's disease: possible cholinergic 'compartment' of pyruvate dehydrogenase.
Alzheimer Disease
Enzyme activities in relation to pH and lactate in postmortem brain in Alzheimer-type and other dementias.
Alzheimer Disease
Immunocapture and microplate-based activity measurement of mammalian pyruvate dehydrogenase complex.
amp deaminase deficiency
[Diagnostic significance of muscle biopsies in metabolic myopathies. II. Clinical biochemistry]
Anemia, Hypochromic
A Nuclear Mutation in Nicotiana sylvestris Causing a Thiamine-Reversible Defect in Synthesis of Chloroplast Pigments.
Anemia, Hypochromic
Spermine ameliorates prolonged fluoride toxicity in soil-grown rice seedlings by activating the antioxidant machinery and glyoxalase system.
Argininosuccinic Aciduria
Aminoacidopathies: Prevalence, Etiology, Screening, and Treatment Options.
aromatic-l-amino-acid decarboxylase deficiency
Vitamin-Responsive Movement Disorders in Children.
Arthritis, Rheumatoid
Autoantibodies to mitochondrial and centromere antigens in primary biliary cirrhosis and systemic sclerosis.
arylsulfatase (type i) deficiency
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
aspartate-ammonia ligase deficiency
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
Ataxia
Clinical and genetic spectrum of pyruvate dehydrogenase deficiency: dihydrolipoamide acetyltransferase (E2) deficiency.
Ataxia
Deficiency of the pyruvate dehydrogenase complex and of mitochondrial fatty acid oxidation.
Ataxia
Genetic and clinical features of Chinese patients with mitochondrial ataxia identified by targeted next-generation sequencing.
Ataxia
Immunochemical analysis of pyruvate dehydrogenase complex in 2 boys with primary lactic acidemia.
Ataxia
Pyruvate dehydrogenase, lipoamide dehydrogenase and citrate synthase activity in fibroblasts from patients with Friedreich's and Charlevoix-Saguenay ataxia.
Autoimmune Diseases
A flow cytometric method to detect anti-pyruvate dehydrogenase antibody in primary biliary cirrhosis.
Autoimmune Diseases
Anti-mitochondrial antibodies and other immunological tests in primary biliary cirrhosis.
Autoimmune Diseases
Antimitochondrial autoantibodies of primary biliary cirrhosis as a novel probe in the study of 2-oxo acid dehydrogenases in patients with mitochondrial myopathies.
Autoimmune Diseases
Primary biliary cirrhosis and Sjögren's syndrome: Autoimmune epithelitis.
Autoimmune Diseases
T cell responses to natural human proteins in primary biliary cirrhosis.
Autoimmune Diseases
TCR? repertoire of memory T cell reveals potential role for Escherichia coli in the pathogenesis of primary biliary cholangitis.
Autoimmune Diseases
The Critical Role of Chemokine (C-C Motif) Receptor 2-Positive Monocytes in Autoimmune Cholangitis.
Avitaminosis
[Comparative evaluation of 2 methods for the determination of pyruvate dehydrogenase activity in tissues in avitaminosis B 1 induced by various methods]
Brain Diseases
A?PP-Transgenic 2576 Mice Mimic Cell Type-Specific Aspects of Acetyl-CoA-Linked Metabolic Deficits in Alzheimer's Disease.
Brain Diseases
Activities of thiamine-dependent enzymes in two experimental models of thiamine-deficiency encephalopathy: 1. The pyruvate dehydrogenase complex.
Brain Diseases
Aggravated effects of coexisting marginal thiamine deficits and zinc excess on SN56 neuronal cells.
Brain Diseases
Characteristic changes on brain CT in a case of Leigh encephalopathy with deficiency of pyruvate dehydrogenase.
Brain Diseases
Leigh syndrome: pyruvate dehydrogenase defect. A case with peripheral neuropathy.
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
Pyruvate dehydrogenase deficiency. Clinical presentation and molecular genetic characterization of five new patients.
Brain Diseases
Reconciling diabetes management and the ketogenic diet in a child with pyruvate dehydrogenase deficiency.
Brain Diseases
Severe encephalopathy associated to pyruvate dehydrogenase mutations and unbalanced coenzyme Q10 content.
Brain Edema
Brain magnetic resonance imaging findings and radiologic review of maple syrup urine disease: Report of three cases.
Brain Injuries
Dichloroacetate treatment improves mitochondrial metabolism and reduces brain injury in neonatal mice.
Brain Injuries
Role of pyruvate dehydrogenase complex in traumatic brain injury and Measurement of pyruvate dehydrogenase enzyme by dipstick test.
Brain Injuries, Traumatic
Glucose metabolism after traumatic brain injury: estimation of pyruvate carboxylase and pyruvate dehydrogenase flux by mass isotopomer analysis.
Brain Injuries, Traumatic
Pyruvate Dehydrogenase and Tricarboxylic Acid Cycle Enzymes Are Sensitive Targets of Traumatic Brain Injury Induced Metabolic Derangement.
Brain Injuries, Traumatic
Pyruvate dehydrogenase enzyme dipstick test in traumatic brain injury: A concern.
Brain Injuries, Traumatic
Role of pyruvate dehydrogenase complex in traumatic brain injury and Measurement of pyruvate dehydrogenase enzyme by dipstick test.
Brain Ischemia
Effect of nicardipine, a Ca2+ channel blocker, on pyruvate dehydrogenase activity and energy metabolites during cerebral ischemia and reperfusion in gerbil brain.
Brain Ischemia
Hyperoxic reperfusion after global ischemia decreases hippocampal energy metabolism.
Brain Ischemia
Neuronal subclass-selective loss of pyruvate dehydrogenase immunoreactivity following canine cardiac arrest and resuscitation.
Brain Ischemia
Pyruvate dehydrogenase activity in the rat cerebral cortex following cerebral ischemia.
Brain Ischemia
The effect of duration of cerebral ischemia on brain pyruvate dehydrogenase activity, energy metabolites, and blood flow during reperfusion in gerbil brain.
Brain Ischemia
[Effect of duration of cerebral ischemia on pyruvate dehydrogenase activity (PDH) and metabolites in the gerbil brain]
Brain Neoplasms
mTORC2 links growth factor signaling with epigenetic regulation of iron metabolism in glioblastoma.
branched-chain alpha-keto acid dehydrogenase system deficiency
Pyruvate dehydrogenase E1alpha subunit deficiency in a female patient: evidence of antenatal origin of brain damage and possible etiology of infantile spasms.
Breast Neoplasms
Differential expression of pyruvate dehydrogenase E1A and its inactive phosphorylated form among breast cancer subtypes.
Breast Neoplasms
Effect of Doxorubicin on Myocardial Bicarbonate Production From Pyruvate Dehydrogenase in Women With Breast Cancer.
Breast Neoplasms
Impairment of HIF-1?-mediated metabolic adaption by NRF2-silencing in breast cancer cells.
Breast Neoplasms
Suppression of PDHX by microRNA-27b deregulates cell metabolism and promotes growth in breast cancer.
Breast Neoplasms
The novel function of tumor protein D54 in regulating pyruvate dehydrogenase and metformin cytotoxicity in breast cancer.
Burkitt Lymphoma
Hypoxia-Inducible Factor 1 and Dysregulated c-Myc Cooperatively Induce Vascular Endothelial Growth Factor and Metabolic Switches Hexokinase 2 and Pyruvate Dehydrogenase Kinase 1.
Carcinogenesis
A combination of alpha lipoic acid and calcium hydroxycitrate is efficient against mouse cancer models: preliminary results.
Carcinogenesis
Overexpression of Pyruvate dehydrogenase E1a subunit Inhibits Warburg effect and Induces Cell Apoptosis through Mitochondria-mediated Pathway in Hepatocellular Carcinoma.
Carcinogenesis
Ras-mediated modulation of pyruvate dehydrogenase activity regulates mitochondrial reserve capacity and contributes to glioblastoma tumorigenesis.
Carcinogenesis
The microRNA-182-PDK4 axis regulates lung tumorigenesis by modulating pyruvate dehydrogenase and lipogenesis.
Carcinoma
Decreased expression of pyruvate dehydrogenase A1 predicts an unfavorable prognosis in ovarian carcinoma.
Carcinoma
Expression parameters of the metabolic pathway genes pyruvate dehydrogenase kinase-1 (PDK-1) and DJ-1/PARK7 in renal cell carcinoma (RCC).
Carcinoma
Hexokinase II promotes the Warburg effect by phosphorylating alpha subunit of pyruvate dehydrogenase.
Carcinoma
Lung Cancer: A Comparative Study of Metabolism Related Protein Expression in Cancer Cells and Tumor Associated Stroma.
Carcinoma
Metformin increases PDH and suppresses HIF-1? under hypoxic conditions and induces cell death in oral squamous cell carcinoma.
Carcinoma
[S35 lipoic acid distribution and its effect on pyruvate dehydrogenase activity in rats with Walker carcinoma]
Carcinoma, Ehrlich Tumor
Ammonium ions enhance the flow through the pyruvate dehydrogenase in Ehrlich ascites tumor cells.
Carcinoma, Ehrlich Tumor
Kinetic and regulatory properties of pyruvate dehydrogenase from Ehrlich ascites tumor cells.
Carcinoma, Ehrlich Tumor
Multiple effects of amobarbital on Ehrlich ascites tumor cells. Inhibition of pyruvate dehydrogenase.
Carcinoma, Hepatocellular
Active pyruvate dehydrogenase and impaired gluconeogenesis in orthotopic hepatomas of rats.
Carcinoma, Hepatocellular
Dexamethasone permits the release of an inhibitor of pyruvate dehydrogenase from Reuber H-35 hepatoma cells in response to insulin.
Carcinoma, Hepatocellular
Effects of fasting on the control of fatty-acid synthesis in hepatoma 7777 and host liver. Role of long-chain fatty acyl-CoA,, the mitochondrial citrate transporter and pyruvate dehydrogenase activity.
Carcinoma, Hepatocellular
Inhibition of pyruvate dehydrogenase kinase?1 by dicoumarol enhances the sensitivity of hepatocellular carcinoma cells to oxaliplatin via metabolic reprogramming.
Carcinoma, Hepatocellular
Overexpression of Pyruvate dehydrogenase E1a subunit Inhibits Warburg effect and Induces Cell Apoptosis through Mitochondria-mediated Pathway in Hepatocellular Carcinoma.
Carcinoma, Hepatocellular
Partial purification from hepatoma cells of an intracellular substance which mediates the effects of insulin on pyruvate dehydrogenase and low Km cyclic AMP phosphodiesterase.
Carcinoma, Hepatocellular
Pyruvate dehydrogenase levels in Morris hepatomas with different growth rate.
Carcinoma, Hepatocellular
Regulation of mammalian pyruvate dehydrogenase alpha subunit gene expression by glucose in HepG2 cells.
Carcinoma, Hepatocellular
The chemical mediators of insulin action: possible targets for postreceptor defects.
Carcinoma, Hepatocellular
The HGF-MET axis coordinates liver cancer metabolism and autophagy for chemotherapeutic resistance.
Carcinoma, Renal Cell
Expression parameters of the metabolic pathway genes pyruvate dehydrogenase kinase-1 (PDK-1) and DJ-1/PARK7 in renal cell carcinoma (RCC).
Carcinoma, Renal Cell
Hexokinase II promotes the Warburg effect by phosphorylating alpha subunit of pyruvate dehydrogenase.
Carcinosarcoma
[Lactate and pyruvate metabolism in the tissues of rats with Walker carcinosarcoma injected with oxythiamine]
Cardiomegaly
Role of pyruvate dehydrogenase inhibition in the development of hypertrophy in the hyperthyroid rat heart: a combined magnetic resonance imaging and hyperpolarized magnetic resonance spectroscopy study.
Cardiomyopathies
Pyruvate dehydrogenase activity in cardiac mitochondria from genetically diabetic mice.
Cardiomyopathies
Pyruvate dehydrogenase as a therapeutic target for obesity cardiomyopathy.
Cerebellar Ataxia
A possible mechanism for selective cerebellar damage in partial pyruvate dehydrogenase deficiency.
Cerebral Palsy
Cerebral palsy and pyruvate dehydrogenase deficiency: identification of two new mutations in the E1alpha gene.
Charcot-Marie-Tooth Disease
Ultrastructural examination of skin biopsies may assist in diagnosing mitochondrial cytopathy when muscle biopsies yield negative results.
Cholangitis
Detection of anti-mitochondrial antibodies by immunoprecipitation in patients with systemic sclerosis.
Cholangitis
Hepatitic bile duct injuries in chronic hepatitis C: histopathologic and immunohistochemical studies.
Cholangitis
Induction of autoimmune cholangitis in non-obese diabetic (NOD).1101 mice following a chemical xenobiotic immunization.
Cholangitis
TCR? repertoire of memory T cell reveals potential role for Escherichia coli in the pathogenesis of primary biliary cholangitis.
Cholangitis
The Critical Role of Chemokine (C-C Motif) Receptor 2-Positive Monocytes in Autoimmune Cholangitis.
Cholangitis, Sclerosing
Antibodies to carbonic anhydrase in patients with immune cholangiopathies.
Cholestasis
[Activity of carbohydrate metabolism enzyme activity in normal and pathologic parodontal tissues]
Chondrodysplasia Punctata, Rhizomelic
Lactate oxidation for the detection of mitochondrial dysfunction in human skin fibroblasts.
Colitis
Quantitative analysis of mucosal oxygenation using ex vivo imaging of healthy and inflamed mammalian colon tissue.
Colonic Neoplasms
Vitamin C activates pyruvate dehydrogenase (PDH) targeting the mitochondrial tricarboxylic acid (TCA) cycle in hypoxic KRAS mutant colon cancer.
Colorectal Neoplasms
Cellular Metabolism and Dose Reveal Carnitine-Dependent and -Independent Mechanisms of Butyrate Oxidation in Colorectal Cancer Cells.
Colorectal Neoplasms
Hemistepsin A suppresses colorectal cancer growth through inhibiting pyruvate dehydrogenase kinase activity.
Colorectal Neoplasms
miR-26a enhances colorectal cancer cell growth by targeting RREB1 deacetylation to activate AKT-mediated glycolysis.
Coma
Changes in pyruvate dehydrogenase complex activity during and following severe insulin-induced hypoglycemia.
Coma
Selective increase of brain lactate synthesis in experimental acute liver failure: results of a [H-C] nuclear magnetic resonance study.
Coma
[Intensity of glycolysis and activity of energy metabolism enzymes in rat brain after multiple exposures to hypoglycemic doses of insulin]
Congenital Abnormalities
Fatal lactic acidosis in a newborn attributable to a congenital defect of pyruvate dehydrogenase.
Congenital Disorders of Glycosylation
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
Coronary Artery Disease
Myocardial metabolic and hemodynamic effects of dichloroacetate in coronary artery disease.
Coronary Occlusion
Dichloroacetate attenuates myocardial acidosis and metabolic changes induced by partial occlusion of the coronary artery in dogs.
Cushing Syndrome
The relationship of mitochondrial dysfunction and the development of insulin resistance in Cushing's syndrome.
Cysts
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
Cytochrome-c Oxidase Deficiency
Determination of [U-13C]glucose turnover into various metabolite pools for the differential diagnosis of lactic acidemias.
Cytochrome-c Oxidase Deficiency
Nonviability of cells with oxidative defects in galactose medium: a screening test for affected patient fibroblasts.
Deficiency Diseases
Pyruvate dehydrogenase deficiency disease detected by the enzyme activity of peripheral leukocytes.
Dementia
Enzyme activities in relation to pH and lactate in postmortem brain in Alzheimer-type and other dementias.
Diabetes Mellitus
Effect of streptozotocin-induced diabetes mellitus on the turnover of rat liver pyruvate carboxylase and pyruvate dehydrogenase.
Diabetes Mellitus
Hyperpolarized NMR study of the impact of pyruvate dehydrogenase kinase inhibition on the pyruvate dehydrogenase and TCA flux in type 2 diabetic rat muscle.
Diabetes Mellitus
Insulin resistance in the liver in fasting and diabetes mellitus: the failure of insulin to stimulate the release of a chemical modulator of pyruvate dehydrogenase.
Diabetes Mellitus, Experimental
Changes of pyruvate dehydrogenase in brain during alloxan diabetes.
Diabetes Mellitus, Experimental
Differential response of liver and kidney adenine nucleotide translocase and pyruvate dehydrogenase activity to alloxan diabetes. The possible regulatory role of long chain acyl CoA.
Diabetes Mellitus, Type 1
13C-nuclear magnetic resonance spectroscopy studies of hepatic glucose metabolism in normal subjects and subjects with insulin-dependent diabetes mellitus.
Diabetes Mellitus, Type 2
Adipocyte glycogen synthase and pyruvate dehydrogenase in obese and type II diabetic subjects.
Diabetes Mellitus, Type 2
Derangements of pyruvate dehydrogenase in circulating lymphocytes of NIDDM patients and their healthy offspring.
Diabetes Mellitus, Type 2
Dose-response characteristics of impaired glucose oxidation in non-insulin-dependent diabetes mellitus.
Diabetes Mellitus, Type 2
Effect of sulfonylurea agents on pyruvate dehydrogenase activity in circulating lymphocytes from patients with non-insulin-dependent diabetes mellitus (NIDDM).
Diabetes Mellitus, Type 2
Fasting hyperglycemia normalizes oxidative and nonoxidative pathways of insulin-stimulated glucose metabolism in noninsulin-dependent diabetes mellitus.
Diabetes Mellitus, Type 2
Hyperpolarized NMR study of the impact of pyruvate dehydrogenase kinase inhibition on the pyruvate dehydrogenase and TCA flux in type 2 diabetic rat muscle.
Diabetes Mellitus, Type 2
Impaired Glucose Partitioning in Primary Myotubes from Severely Obese Women with Type 2 Diabetes.
Diabetes Mellitus, Type 2
In obese individuals dexfenfluramine corrects molecular derangements reflecting insulin resistance.
Diabetes Mellitus, Type 2
Increasing Pyruvate Dehydrogenase Flux as a Treatment for Diabetic Cardiomyopathy: A Combined 13C Hyperpolarized Magnetic Resonance and Echocardiography Study.
Diabetes Mellitus, Type 2
Insulin resistance in obese subjects and newly diagnosed NIDDM patients and derangements of pyruvate dehydrogenase in their circulating lymphocytes.
Diabetes Mellitus, Type 2
Molecular effects of sulphonylurea agents in circulating lymphocytes of patients with non-insulin-dependent diabetes mellitus.
Diabetes Mellitus, Type 2
Pregnancy induces molecular alterations reflecting impaired insulin control over glucose oxidative pathways that only in women with a family history of Type 2 diabetes last beyond pregnancy.
Diabetic Cardiomyopathies
Increasing Pyruvate Dehydrogenase Flux as a Treatment for Diabetic Cardiomyopathy: A Combined 13C Hyperpolarized Magnetic Resonance and Echocardiography Study.
Diabetic Ketoacidosis
Pyruvate dehydrogenase activity is decreased in emergency department patients with diabetic ketoacidosis.
Diabetic Ketoacidosis
Reconciling diabetes management and the ketogenic diet in a child with pyruvate dehydrogenase deficiency.
Diabetic Neuropathies
Pyruvate Dehydrogenase Kinase-mediated Glycolytic Metabolic Shift in the Dorsal Root Ganglion Drives Painful Diabetic Neuropathy.
Diabetic Retinopathy
A pyruvate dehydrogenase kinase inhibitor prevents retinal cell death and improves energy metabolism in rat retinas after ischemia/reperfusion injury.
dihydrolipoyl dehydrogenase deficiency
Newborn screening for dihydrolipoamide dehydrogenase deficiency: Citrulline as a useful analyte.
dihydrolipoyl dehydrogenase deficiency
Pyruvate dehydrogenase subcomplex with lipoamide dehydrogenase deficiency in a patient with lactic acidosis and branched chain ketoaciduria.
Down Syndrome
Enzyme activities in relation to pH and lactate in postmortem brain in Alzheimer-type and other dementias.
Down Syndrome
Severe Brain Malformations in an Infant With Pyruvate Dehydrogenase Deficiency and Down Syndrome.
Drug Resistant Epilepsy
Current Perspectives On The Role Of The Ketogenic Diet In Epilepsy Management.
Drug Resistant Epilepsy
Food and Food Products on the Italian Market for Ketogenic Dietary Treatment of Neurological Diseases.
Dyskinesias
Pyruvate dehydrogenase complex-E2 deficiency causes paroxysmal exercise-induced dyskinesia.
Dystonia
Clinical and genetic spectrum of pyruvate dehydrogenase deficiency: dihydrolipoamide acetyltransferase (E2) deficiency.
Dystonia
Pyruvate dehydrogenase deficiency presenting as isolated paroxysmal exercise induced dystonia successfully reversed with thiamine supplementation. Case report and mini-review.
Dystonia
Pyruvate dehydrogenase E2 deficiency: A potentially treatable cause of episodic dystonia.
Epilepsy
Animal Models of Metabolic Epilepsy and Epilepsy Associated Metabolic Dysfunction: A Systematic Review.
Epilepsy
Apparent identity of alpha-subunit of pyruvate dehydrogenase and the protein phosphorylated in the presence of glutamate in P2-fractions of rat cerebral cortex.
Epilepsy
Impairments in Oxidative Glucose Metabolism in Epilepsy and Metabolic Treatments Thereof.
Epilepsy
Reconciling diabetes management and the ketogenic diet in a child with pyruvate dehydrogenase deficiency.
Epilepsy
Summary of recommendations for the management of infantile seizures: Task Force Report for the ILAE Commission of Pediatrics.
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.
Epileptic Syndromes
Current Perspectives On The Role Of The Ketogenic Diet In Epilepsy Management.
Fatigue Syndrome, Chronic
Tenuous link between chronic fatigue syndrome and pyruvate dehydrogenase deficiency.
Fatty Liver
Pyruvate dehydrogenase kinase 4 mRNA is increased in the hypertrophied ventricles of carnitine-deficient juvenile visceral steatosis (JVS) mice.
Fetal Alcohol Spectrum Disorders
Derangement of pyruvate dehydrogenase activity in circulating lymphocytes of a newborn with fetal alcohol syndrome.
Fetal Alcohol Spectrum Disorders
Impairment of pyruvate dehydrogenase activity by acetaldehyde.
Friedreich Ataxia
Active pyruvate dehydrogenase in platelets from Friedreich's ataxia patients.
Friedreich Ataxia
Friedreich's ataxia: I. Normal pyruvate dehydrogenase complex activity in platelets.
Friedreich Ataxia
Long-term treatment with thiamine as possible medical therapy for Friedreich ataxia.
Friedreich Ataxia
Pyruvate dehydrogenase, lipoamide dehydrogenase and citrate synthase activity in fibroblasts from patients with Friedreich's and Charlevoix-Saguenay ataxia.
Galactosemias
Impact of selected inborn errors of metabolism on prenatal and neonatal development.
Genetic Diseases, Inborn
Optic neuropathy in a patient with pyruvate dehydrogenase deficiency.
Glaucoma
A pyruvate dehydrogenase kinase inhibitor prevents retinal cell death and improves energy metabolism in rat retinas after ischemia/reperfusion injury.
Glioblastoma
mTORC2 links growth factor signaling with epigenetic regulation of iron metabolism in glioblastoma.
Glioblastoma
Ras-mediated modulation of pyruvate dehydrogenase activity regulates mitochondrial reserve capacity and contributes to glioblastoma tumorigenesis.
Glioblastoma
Specific inhibition by synthetic analogs of pyruvate reveals that the pyruvate dehydrogenase reaction is essential for metabolism and viability of glioblastoma cells.
Glioma
Effects of methyl mercury and triethyllead on Na+K+ATPase and pyruvate dehydrogenase activities in glioma C6 cells.
Glioma
Metabolic reprogramming of pyruvate dehydrogenase is essential for the proliferation of glioma cells expressing mutant IDH1.
Glioma
MiR-363-3p modulates cell growth and invasion in glioma by directly targeting pyruvate dehydrogenase B.
Glioma
Nodal regulates energy metabolism in glioma cells by inducing expression of hypoxia-inducible factor 1?.
Glioma
Phosphorylated form of pyruvate dehydrogenase ?1 mediates tumor necrosis factor ?-induced glioma cell migration.
Glioma
Pyruvate dehydrogenase kinase as a potential therapeutic target for malignant gliomas.
Glioma
Reversing tozasertib resistance in glioma through inhibition of pyruvate dehydrogenase kinases.
Glomerulonephritis
Lycium barbarum polysaccharides attenuate rat anti-Thy-1 glomerulonephritis through mediating pyruvate dehydrogenase.
Glucose Intolerance
Regulation of hepatic pyruvate dehydrogenase phosphorylation in offspring glucose intolerance induced by intrauterine hyperglycemia.
Glucose Intolerance
The antianginal ranolazine mitigates obesity-induced nonalcoholic fatty liver disease and increases hepatic pyruvate dehydrogenase activity.
Glycogen Storage Disease
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
Glycogen Storage Disease Type I
Urinary lactate excretion in normal children and in children with enzyme defects of carbohydrate metabolism.
Heart Arrest
Activation of Pyruvate Dehydrogenase Activity Bydichloroacetate Improves Survival and Neurologic Outcomes After Cardiac Arrest in Rats.
Heart Arrest
Energy-linked regulation of glucose and pyruvate oxidation in isolated perfused rat heart. Role of pyruvate dehydrogenase.
Heart Arrest
Enhanced pyruvate dehydrogenase activity improves cardiac outcomes in a murine model of cardiac arrest.
Heart Arrest
Neuronal subclass-selective loss of pyruvate dehydrogenase immunoreactivity following canine cardiac arrest and resuscitation.
Heart Failure
Cardiac mitochondrial proteome dynamics with heavy water reveals stable rate of mitochondrial protein synthesis in heart failure despite decline in mitochondrial oxidative capacity.
Heart Failure
Development of Dihydroxyphenyl Sulfonylisoindoline Derivatives as Liver-Targeting Pyruvate Dehydrogenase Kinase Inhibitors.
Heart Failure
Increasing carbohydrate oxidation improves contractile reserves and prevents hypertrophy in porcine right heart failure.
Heart Failure
Metabolic markers of myocardium insulin resistance in dogs with heart failure.
Heart Failure
The effects of hypertrophy and diabetes on cardiac pyruvate dehydrogenase activity.
Hepatitis
Autoantibodies of primary biliary cirrhosis recognize dihydrolipoamide acetyltransferase and inhibit enzyme function.
Hepatitis
Identification of the dihydrolipoamide acetyltransferase subunit of the human pyruvate dehydrogenase complex as an autoantigen in halothane hepatitis. Molecular mimicry of trifluoroacetyl-lysine by lipoic acid.
Hepatitis, Autoimmune
Antibodies to carbonic anhydrase in patients with immune cholangiopathies.
Hepatitis, Autoimmune
Immunoreactivity to pyruvate dehydrogenase complex-E2 in well-defined patients with autoimmune hepatitis: Western blot analysis.
Hepatitis, Chronic
Autoantibodies of primary biliary cirrhosis recognize dihydrolipoamide acetyltransferase and inhibit enzyme function.
Hepatitis, Chronic
Hepatitic bile duct injuries in chronic hepatitis C: histopathologic and immunohistochemical studies.
Hepatolenticular Degeneration
Free radical-mediated neurotoxicity may be caused by inhibition of mitochondrial dehydrogenases in vitro and in vivo.
Huntington Disease
Beneficial effect of (-)schisandrin B against 3-nitropropionic acid-induced cell death in PC12 cells.
Huntington Disease
Distribution of phosphate-activated glutaminase, succinic dehydrogenase, pyruvate dehydrogenase and gamma-glutamyl transpeptidase in post-mortem brain from Huntington's disease and agonal cases.
Huntington Disease
Enzyme activities in relation to pH and lactate in postmortem brain in Alzheimer-type and other dementias.
Huntington Disease
Histone Deacetylase Inhibitors Protect Against Pyruvate Dehydrogenase Dysfunction in Huntington's Disease.
Hyperargininemia
Aminoacidopathies: Prevalence, Etiology, Screening, and Treatment Options.
Hyperemia
Pyruvate-enhanced phosphorylation potential and inotropism in normoxic and postischemic isolated working heart. Near-complete prevention of reperfusion contractile failure.
Hyperglycemia
Effect of Chronic Hyperglycemia on Glucose Metabolism in Subjects with Normal Glucose Tolerance.
Hyperglycemia
Effect of hyperglycemia on pyruvate dehydrogenase activity and energy metabolites during ischemia and reperfusion in gerbil brain.
Hyperglycemia
Fasting hyperglycemia normalizes oxidative and nonoxidative pathways of insulin-stimulated glucose metabolism in noninsulin-dependent diabetes mellitus.
Hyperglycemia
Pimozide Alleviates Hyperglycemia in Diet-Induced Obesity by Inhibiting Skeletal Muscle Ketone Oxidation.
Hyperglycemia
Preischemic hyperglycemia and postischemic alteration of rat brain pyruvate dehydrogenase activity.
Hyperglycemia
Regulation of hepatic pyruvate dehydrogenase phosphorylation in offspring glucose intolerance induced by intrauterine hyperglycemia.
Hyperglycinemia, Nonketotic
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
Hyperglycinemia, Nonketotic
Inborn errors of metabolism: a cause of abnormal brain development.
Hyperglycinemia, Nonketotic
LIPT1 deficiency presenting as early infantile epileptic encephalopathy, Leigh disease, and secondary pyruvate dehydrogenase complex deficiency.
Hyperglycinemia, Nonketotic
[Cerebral malformation in the newborn: holoprosencephaly and agenesis of the corpus callosum]
Hyperglycinemia, Nonketotic
[Contribution of CSF analysis to the diagnosis of inborn errors of metabolism in adult patients]
Hyperinsulinism
Effects of blockade of fatty acid oxidation on whole body and tissue-specific glucose metabolism in rats.
Hyperinsulinism
Interaction between glucose and free fatty acid metabolism in human skeletal muscle.
Hyperlactatemia
Evidence for a role of exogenous or endogenous hyperlactatemia in insulin secretion in the dog.
Hyperlactatemia
Sodium 2-chloropropionate: its effects on experimental hyperlactatemia in the dog.
Hyperlipidemias
Hepatic HAX-1 inactivation prevents metabolic diseases by enhancing mitochondrial activity and bile salt export.
Hypertension
Epigenetic Metabolic Reprogramming of Right Ventricular Fibroblasts in Pulmonary Arterial Hypertension: A Pyruvate Dehydrogenase Kinase-Dependent Shift in Mitochondrial Metabolism Promotes Right Ventricular Fibrosis.
Hypertension
Pyruvate dehydrogenase inhibition by the inflammatory cytokine TNF? contributes to the pathogenesis of pulmonary arterial hypertension.
Hypertension
The Endocannabinoid System Affects Myocardial Glucose Metabolism in the DOCA-Salt Model of Hypertension.
Hypertension, Pulmonary
Time-dependent PPAR? Modulation of HIF-1? Signaling in Hypoxic Pulmonary Artery Smooth Muscle Cells.
Hyperthyroidism
Interaction between insulin and thyroid hormones on the control of carbohydrate and lipid metabolism in rat adipose tissue.
Hyperthyroidism
Role of pyruvate dehydrogenase inhibition in the development of hypertrophy in the hyperthyroid rat heart: a combined magnetic resonance imaging and hyperpolarized magnetic resonance spectroscopy study.
Hypoglycemia
Changes in pyruvate dehydrogenase complex activity during and following severe insulin-induced hypoglycemia.
Hypoglycemia
Starvation and seizures. Observation on the electroconvulsive threshold and cerebral metabolism of the starved adult rat.
Hypoglycemia
The Effects of Sodium Dichloroacetate on Mitochondrial Dysfunction and Neuronal Death Following Hypoglycemia-Induced Injury.
Hypoglycemia
Ultrastructural examination of the axillary skin biopsy in the diagnosis of metabolic diseases.
Hypokinesia
[Activity of various oxidases and transaminases in the rat liver in the readaptation period after hypokinesia up to 30 days]
Hypophosphatasia
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
Hypophosphatemia
Mapping of the glycine receptor alpha 2-subunit gene and the GABAA alpha 3-subunit gene on the mouse X chromosome.
Hypothyroidism
Interaction between insulin and thyroid hormones on the control of carbohydrate and lipid metabolism in rat adipose tissue.
Infections
Disruption of the pdhB pyruvate dehydrogenase [corrected] gene affects colony morphology, in vitro growth and cell invasiveness of Mycoplasma agalactiae.
Infections
Down-regulation of expression of rat pyruvate dehydrogenase E1alpha gene by self-complementary adeno-associated virus-mediated small interfering RNA delivery.
Infections
Energy Metabolism in the Placenta and the Role of Disturbances in the Development of Placental Insufficiency at an Exacerbation of Cytomegalovirus Infection.
Infections
Metabolic responses of primary and transformed cells to intracellular Listeria monocytogenes.
Infections
Microsporidian mitochondrial proteins: expression in Antonospora locustae spores and identification of genes coding for two further proteins.
Infections
Mycoplasma genitalium rapidly disseminates to the upper reproductive tracts and knees of female mice following vaginal inoculation.
Infections
Plasmodium pyruvate dehydrogenase activity is only essential for the parasite's progression from liver infection to blood infection.
Insulin Resistance
Circulating factors and insulin resistance. II. The action of the novel myo-inositol cyclic 1,2-inositol phosphate phosphoglycan insulin antagonist from human plasma in regulating pyruvate dehydrogenase phosphatase.
Insulin Resistance
Decreased insulin-generation of pyruvate dehydrogenase inhibitor in insulin resistant states.
Insulin Resistance
Development of insulin sensitivity in white adipose tissue during the suckling-weaning transition in the rat. Involvement of glucose transport and lipogenesis.
Insulin Resistance
Different types of postinsulin receptor defects contribute to insulin resistance in hearts of obese Zucker rats.
Insulin Resistance
Enhanced blood insulin overcomes pyruvate dehydrogenase derangements that reflect systemic insulin resistance in obese adolescents.
Insulin Resistance
Genetic activation of pyruvate dehydrogenase alters oxidative substrate selection to induce skeletal muscle insulin resistance.
Insulin Resistance
Genetic inactivation of pyruvate dehydrogenase kinases improves hepatic insulin resistance induced diabetes.
Insulin Resistance
Growth hormone-induced insulin resistance in human subjects involves reduced pyruvate dehydrogenase activity.
Insulin Resistance
Hepatic HAX-1 inactivation prevents metabolic diseases by enhancing mitochondrial activity and bile salt export.
Insulin Resistance
Hexose-independent activation of glycogen synthase and pyruvate dehydrogenase by insulin is dissociated in the mouse BC3H-1 cell line.
Insulin Resistance
High dietary fructose induces a hepatic stress response resulting in cholesterol and lipid dysregulation.
Insulin Resistance
Hyperpolarized NMR study of the impact of pyruvate dehydrogenase kinase inhibition on the pyruvate dehydrogenase and TCA flux in type 2 diabetic rat muscle.
Insulin Resistance
In obese individuals dexfenfluramine corrects molecular derangements reflecting insulin resistance.
Insulin Resistance
Insulin activates phospholipase C in fat cells: similarity with the activation of pyruvate dehydrogenase.
Insulin Resistance
Insulin resistance in obese subjects and newly diagnosed NIDDM patients and derangements of pyruvate dehydrogenase in their circulating lymphocytes.
Insulin Resistance
Insulin resistance in the liver in fasting and diabetes mellitus: the failure of insulin to stimulate the release of a chemical modulator of pyruvate dehydrogenase.
Insulin Resistance
Insulin resistance induced by growth hormone is linked to lipolysis and associated with suppressed pyruvate dehydrogenase activity in skeletal muscle: a 2?×?2 factorial, randomised, crossover study in human individuals.
Insulin Resistance
Insulin resistance of glucose metabolism in isolated brown adipocytes of lactating rats. Evidence for a post-receptor defect in insulin action.
Insulin Resistance
Metabolic diseases drug discovery world summit. July 28-29, 2003, San Diego, CA, USA.
Insulin Resistance
News and views in myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS): The role of co-morbidity and novel treatments.
Insulin Resistance
Opposing actions of dehydroepiandrosterone and testosterone on insulin sensitivity. In vivo and in vitro studies of hyperandrogenic females.
Insulin Resistance
Pyruvate dehydrogenase and ATP citrate (pro-3S)-lyase activities in adipose tissue and liver of the young lean and the older obese rat.
Insulin Resistance
Pyruvate dehydrogenase inactivity is not responsible for sepsis-induced insulin resistance.
Insulin Resistance
Pyruvate dehydrogenase, Randle cycle, and skeletal muscle insulin resistance.
Insulin Resistance
Regulation of glucose metabolism by altered pyruvate dehydrogenase activity. I. Potential site of insulin resistance in sepsis.
Insulin Resistance
Regulation of hepatic fat and glucose oxidation in rats with lipid-induced hepatic insulin resistance.
Insulin Resistance
Sensitivity of pyruvate dehydrogenase to insulin in activated T lymphocytes. Lack of responsiveness to insulin in patients with polycystic ovarian disease and diabetes.
Insulin Resistance
The effect of diet composition on weight gain and pyruvate dehydrogenase activity in heart muscle in the gold thioglucose obese mouse.
Insulin Resistance
The Importance of the Fatty Acid Transporter L-Carnitine in Non-Alcoholic Fatty Liver Disease (NAFLD).
Insulinoma
Regulation of islet beta-cell pyruvate metabolism: interactions of prolactin, glucose, and dexamethasone.
Intellectual Disability
Immunochemical analysis of pyruvate dehydrogenase complex in 2 boys with primary lactic acidemia.
Intellectual Disability
Splicing error in E1alpha pyruvate dehydrogenase mRNA caused by novel intronic mutation responsible for lactic acidosis and mental retardation.
Intellectual Disability
The SR protein SC35 is responsible for aberrant splicing of the E1alpha pyruvate dehydrogenase mRNA in a case of mental retardation with lactic acidosis.
Ischemic Attack, Transient
Effect of dichloroacetate on regional energy metabolites and pyruvate dehydrogenase activity during ischemia and reperfusion in gerbil brain.
Ischemic Attack, Transient
Ischemic tolerance phenomenon from an approach of energy metabolism and the mitochondrial enzyme activity of pyruvate dehydrogenase in gerbils.
Ketosis
Pyruvate dehydrogenase subcomplex with lipoamide dehydrogenase deficiency in a patient with lactic acidosis and branched chain ketoaciduria.
Ketosis
Starvation and seizures. Observation on the electroconvulsive threshold and cerebral metabolism of the starved adult rat.
Lafora Disease
Animal Models of Metabolic Epilepsy and Epilepsy Associated Metabolic Dysfunction: A Systematic Review.
Leigh Disease
Biochemical and molecular analysis of an X-linked case of Leigh syndrome associated with thiamin-responsive pyruvate dehydrogenase deficiency.
Leigh Disease
Leigh syndrome: pyruvate dehydrogenase defect. A case with peripheral neuropathy.
Leigh Disease
Outcome of thiamine treatment in a child with Leigh disease due to thiamine-responsive pyruvate dehydrogenase deficiency.
Leigh Disease
Paroxysmal Dyskinesias Revealing 3-Hydroxy-Isobutyryl-CoA Hydrolase (HIBCH) Deficiency.
Leigh Disease
Pyruvate dehydrogenase activity is not deficient in the brain of three autopsied cases with Leigh disease (subacute necrotizing encephalomyelopathy, SNE).
Leigh Disease
Pyruvate dehydrogenase phosphate (PDHb) phosphatase activity in fibroblasts from Leigh's disease.
Leigh Disease
Ultrastructural examination of the axillary skin biopsy in the diagnosis of metabolic diseases.
Lennox Gastaut Syndrome
Ultrastructural examination of the axillary skin biopsy in the diagnosis of metabolic diseases.
Leukemia
Oncophosphosignaling Favors a Glycolytic Phenotype in Human Drug Resistant Leukemia.
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Tyrosine kinase inhibition in leukemia induces an altered metabolic state sensitive to mitochondrial perturbations.
Leukodystrophy, Metachromatic
Ultrastructural examination of the axillary skin biopsy in the diagnosis of metabolic diseases.
Leukomalacia, Periventricular
Unilateral periventricular leukomalacia in association with pyruvate dehydrogenase deficiency.
Liver Cirrhosis, Biliary
An experimental animal model of primary biliary cirrhosis induced by lipopolysaccharide and pyruvate dehydrogenase.
Liver Cirrhosis, Biliary
Anti-M4 antibodies in primary biliary cirrhosis react with sulphite oxidase, an enzyme of the mitochondrial inter-membrane space.
Liver Cirrhosis, Biliary
Anti-mitochondrial autoantibodies of primary biliary cirrhosis as a novel probe in the study of the biosynthetic regulation of the yeast 2-oxo acid dehydrogenase complexes.
Liver Cirrhosis, Biliary
Antibodies to carbonic anhydrase in patients with immune cholangiopathies.
Liver Cirrhosis, Biliary
Antibodies to pyruvate dehydrogenase in primary biliary cirrhosis: correlation with histology.
Liver Cirrhosis, Biliary
Antibody binding and inhibition of pyruvate dehydrogenase (PDH) in sera from patients with primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Antimitochondrial antibody heterogeneity and the xenobiotic etiology of primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Antimitochondrial antibody recognition and structural integrity of the inner lipoyl domain of the e2 subunit of pyruvate dehydrogenase complex.
Liver Cirrhosis, Biliary
Antimitochondrial autoantibodies in anti-glomerular basement membrane disease.
Liver Cirrhosis, Biliary
Antimitochondrial autoantibodies of primary biliary cirrhosis as a novel probe in the study of 2-oxo acid dehydrogenases in patients with mitochondrial myopathies.
Liver Cirrhosis, Biliary
Autoantibodies against subunits of pyruvate dehydrogenase and citrate synthase in a case of paediatric biliary cirrhosis.
Liver Cirrhosis, Biliary
Autoantibodies in breast cancer sera are not epiphenomena and may participate in carcinogenesis.
Liver Cirrhosis, Biliary
Autoantibodies to mitochondrial and centromere antigens in primary biliary cirrhosis and systemic sclerosis.
Liver Cirrhosis, Biliary
Autoreactivity to lipoate and a conjugated form of lipoate in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Characterization of the autoantibody responses to recombinant E3 binding protein (protein X) of pyruvate dehydrogenase in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Comparative epitope mapping of murine monoclonal and human autoantibodies to human PDH-E2, the major mitochondrial autoantigen of primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Cryptic antigenic determinants on the extracellular pyruvate dehydrogenase complex/mimeotope found in primary biliary cirrhosis. A probe by affinity mass spectrometry.
Liver Cirrhosis, Biliary
Demonstration of peptide-specific and cross-reactive epitopes in proteins reacting with antimitochondrial antibodies of primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Detection of anti-mitochondrial antibodies by immunoprecipitation in patients with systemic sclerosis.
Liver Cirrhosis, Biliary
Disease-specific cross-reactivity between mimicking peptides of heat shock protein of Mycobacterium gordonae and dominant epitope of E2 subunit of pyruvate dehydrogenase is common in Spanish but not British patients with primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Distinct costimulation dependent and independent autoreactive T-cell clones in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Distribution of dihydrolipoamide acetyltransferase (E2) in the liver and portal lymph nodes of patients with primary biliary cirrhosis: an immunohistochemical study.
Liver Cirrhosis, Biliary
Distribution of pyruvate dehydrogenase dihydrolipoamide acetyltransferase (PDC-E2) and another mitochondrial marker in salivary gland and biliary epithelium from patients with primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Enzyme inhibitory antibody to pyruvate dehydrogenase: diagnostic utility in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Enzyme-linked immunosorbent assays for the determination of IgG, IgA, and IgM autoantibodies to pyruvate dehydrogenase in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Fine phenotypic and functional characterization of effector CD8(+) T cells in patients with primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Gut-liver axis: an immune link between celiac disease and primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Hepatitic bile duct injuries in chronic hepatitis C: histopathologic and immunohistochemical studies.
Liver Cirrhosis, Biliary
Heterogeneity of combinatorial human autoantibodies against PDC-E2 and biliary epithelial cells in patients with primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Immunization of experimental animals with dihydrolipoamide acetyltransferase, as a purified recombinant polypeptide, generates mitochondrial antibodies but not primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Immunoblotting as a confirmatory test for antimitochondrial antibodies in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Immunoglobulin gene usage and immunohistochemical characteristics of human monoclonal antibodies to the mitochondrial autoantigens of primary biliary cirrhosis induced in the XenoMouse.
Liver Cirrhosis, Biliary
Immunoreactivity of organic mimeotopes of the E2 component of pyruvate dehydrogenase: connecting xenobiotics with primary biliary cirrhosis.
Liver Cirrhosis, Biliary
In situ nucleic acid hybridization of pyruvate dehydrogenase complex-E2 in primary biliary cirrhosis: pyruvate dehydrogenase complex-E2 messenger RNA is expressed in hepatocytes but not in biliary epithelium.
Liver Cirrhosis, Biliary
Induction of autoimmune cholangitis in non-obese diabetic (NOD).1101 mice following a chemical xenobiotic immunization.
Liver Cirrhosis, Biliary
Infectious agents in the pathogenesis of primary biliary cirrhosis.
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
Inhibition of enzyme function by human autoantibodies to an autoantigen pyruvate dehydrogenase E2: different epitope for spontaneous human and induced rabbit autoantibodies.
Liver Cirrhosis, Biliary
Isolation of human anti-branched chain alpha-oxo acid dehydrogenase-E2 recombinant antibodies by Ig repertoire cloning in idiopathic dilated cardiomyopathy.
Liver Cirrhosis, Biliary
Microbial mimics are major targets of crossreactivity with human pyruvate dehydrogenase in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Molecular mimicry in primary biliary cirrhosis. Evidence for biliary epithelial expression of a molecule cross-reactive with pyruvate dehydrogenase complex-E2.
Liver Cirrhosis, Biliary
Oral tolerance and pyruvate dehydrogenase in patients with primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Phenotypical and functional alterations of CD8 regulatory T cells in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Primary biliary cirrhosis and Sjögren's syndrome: Autoimmune epithelitis.
Liver Cirrhosis, Biliary
Primary biliary cirrhosis. Inhibition of pyruvate dehydrogenase complex activity by autoantibodies specific for E1 alpha, a non-lipoic acid containing mitochondrial enzyme.
Liver Cirrhosis, Biliary
Pyruvate dehydrogenase specific T cells in primary biliary cirrhosis show restricted antigen recognition sites.
Liver Cirrhosis, Biliary
Serum reactivity against bacterial pyruvate dehydrogenase: increasing the specificity of anti-mitochondrial antibodies for the diagnosis of 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
Structural requirement for autoreactivity on human pyruvate dehydrogenase-E2, the major autoantigen of primary biliary cirrhosis. Implication for a conformational autoepitope.
Liver Cirrhosis, Biliary
T cell responses to natural human proteins in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
TCR? repertoire of memory T cell reveals potential role for Escherichia coli in the pathogenesis of primary biliary cholangitis.
Liver Cirrhosis, Biliary
The antinuclear autoantibodies Sp100 and gp210 persist after orthotopic liver transplantation in patients with primary biliary cirrhosis.
Liver Cirrhosis, Biliary
The Critical Role of Chemokine (C-C Motif) Receptor 2-Positive Monocytes in Autoimmune Cholangitis.
Liver Cirrhosis, Biliary
The human biliary epithelial cell plasma membrane antigen in primary biliary cirrhosis: pyruvate dehydrogenase X?
Liver Cirrhosis, Biliary
Tubulointerstitial nephritis and Fanconi syndrome in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Ursodeoxycholic acid treatment lowers the serum level of antibodies against pyruvate dehydrogenase and influences their inhibitory capacity for the enzyme complex in patients with primary biliary cirrhosis.
Liver Cirrhosis, Biliary
[Abnormal expression of pyruvate dehydrogenase on bile ducts opens new fields in the pathogenesis of primary biliary cirrhosis]
Liver Cirrhosis, Biliary
[Primary biliary cirrhosis--specific anti-mitochondrial antibodies]
Liver Diseases
Autoantibodies to mitochondrial and centromere antigens in primary biliary cirrhosis and systemic sclerosis.
Liver Diseases
Hepatic pyruvate dehydrogenase activity in humans: effect of cirrhosis, transplantation, and dichloroacetate.
Liver Diseases
Inhibition of enzyme function by human autoantibodies to an autoantigen pyruvate dehydrogenase E2: different epitope for spontaneous human and induced rabbit autoantibodies.
Liver Diseases
Pyruvate Dehydrogenase as a Therapeutic Target for Nonalcoholic Fatty Liver Disease.
Liver Diseases
The antianginal ranolazine mitigates obesity-induced nonalcoholic fatty liver disease and increases hepatic pyruvate dehydrogenase activity.
Liver Diseases
The antinuclear autoantibodies Sp100 and gp210 persist after orthotopic liver transplantation in patients with primary biliary cirrhosis.
Liver Failure, Acute
Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis.
Liver Neoplasms, Experimental
Regulation of energy metabolism in Morris hepatoma 7777 and 7800.
Lung Neoplasms
Inhibiting Glycine Decarboxylase Suppresses Pyruvate-to-Lactate Metabolism in Lung Cancer Cells.
Lung Neoplasms
Pyruvate dehydrogenase and pyruvate dehydrogenase kinase expression in non small cell lung cancer and tumor-associated stroma.
Lysosomal Storage Diseases
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
Macular Degeneration
Pyruvate dehydrogenase kinase/lactate axis: a therapeutic target for neovascular age-related macular degeneration identified by metabolomics.
Malaria
Dichloroacetate for lactic acidosis in severe malaria: a pharmacokinetic and pharmacodynamic assessment.
Malaria, Cerebral
Dichloroacetate (DCA) reduces brain lactate but increases brain glutamine in experimental cerebral malaria: a 1H-NMR study.
Maple Syrup Urine Disease
Aminoacidopathies: Prevalence, Etiology, Screening, and Treatment Options.
Maple Syrup Urine Disease
Branched chain keto-acids exert biphasic effects on alpha-ketoglutarate-stimulated respiration in intact rat liver mitochondria.
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.
Maple Syrup Urine Disease
Newborn screening for dihydrolipoamide dehydrogenase deficiency: Citrulline as a useful analyte.
Maple Syrup Urine Disease
Pyruvate dehydrogenase subcomplex with lipoamide dehydrogenase deficiency in a patient with lactic acidosis and branched chain ketoaciduria.
Melanoma
A key role for mitochondrial gatekeeper pyruvate dehydrogenase in oncogene-induced senescence.
Melanoma
MiR-370 functions as oncogene in melanoma by direct targeting pyruvate dehydrogenase B.
MELAS Syndrome
Alternating hemiplegia of childhood: metabolic studies in the largest European series of patients.
Metabolic Diseases
Hepatic HAX-1 inactivation prevents metabolic diseases by enhancing mitochondrial activity and bile salt export.
Metabolic Diseases
Loss of metabolic flexibility as a result of overexpression of pyruvate dehydrogenase kinases in muscle, liver and the immune system: Therapeutic targets in metabolic diseases.
Metabolic Diseases
Mutations in the X-linked pyruvate dehydrogenase (E1) alpha subunit gene (PDHA1) in patients with a pyruvate dehydrogenase complex deficiency.
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.
Microphthalmos
Pyruvate dehydrogenase has a major role in mast cell function, and its activity is regulated by mitochondrial microphthalmia transcription factor.
Mitochondrial Diseases
Acute flaccid paralysis as initial symptom in 4 patients with novel e1alpha mutations of the pyruvate dehydrogenase complex.
Mitochondrial Diseases
An immunocytochemical approach to detection of mitochondrial disorders.
Mitochondrial Diseases
Animal Models of Metabolic Epilepsy and Epilepsy Associated Metabolic Dysfunction: A Systematic Review.
Mitochondrial Diseases
Cell culture studies on patients with mitochondrial diseases: molecular defects in pyruvate dehydrogenase.
Mitochondrial Diseases
Diagnostic accuracy of blood lactate-to-pyruvate molar ratio in the differential diagnosis of congenital lactic acidosis.
Mitochondrial Diseases
Leukoencephalopathy with cysts and hyperglycinemia may result from NFU1 deficiency.
Mitochondrial Diseases
Mendeliome sequencing enables differential diagnosis and treatment of neonatal lactic acidosis.
Mitochondrial Diseases
The first case with FBXL4 mutation successfully treated with a parenteral ketogenic diet for lactic acidosis.
Mitochondrial Myopathies
[Lactic acid acidosis with mitochondrial myopathy due to a pyruvate dehydrogenase deficiency]
Multiple Acyl Coenzyme A Dehydrogenase Deficiency
Ultrastructural examination of the axillary skin biopsy in the diagnosis of metabolic diseases.
Multiple Myeloma
Proteomic analysis of oridonin-induced apoptosis in multiple myeloma cells.
Muscle Hypotonia
Case Report of Pyruvate Dehydrogenase Deficiency With Unusual Increase of Fats During Ketogenic Diet Treatment.
Muscle Hypotonia
Clinical and genetic spectrum of pyruvate dehydrogenase deficiency: dihydrolipoamide acetyltransferase (E2) deficiency.
Muscle Hypotonia
Leigh syndrome: pyruvate dehydrogenase defect. A case with peripheral neuropathy.
Muscular Diseases
A metabolic switching hypothesis for the first step in the hypolipidemic effects of fibrates.
Muscular Diseases
Vitamin-responsive pyruvate dehydrogenase deficiency in a young girl with external ophthalmoplegia, myopathy and lactic acidosis.
Mycoplasma Infections
Effect of mycoplasma infection on pyruvate dehydrogenase complex activity of normal and pyruvate dehydrogenase complex-deficient fibroblasts.
Myocardial Ischemia
Effects of ischemia and reperfusion on pyruvate dehydrogenase activity in isolated rat hearts.
Myocardial Ischemia
Synthesis of ranolazine metabolites and their anti-myocardial ischemia activities.
Myocardial Ischemia
[Efficiency of trimetazidine treatment of experimental ischemic heart disease in age aspect].
Myocardial Ischemia
[Ischemic heart disease and left ventricular dysfunction: the role of trimetazidine]
nadh:ubiquinone reductase (h+-translocating) deficiency
Nonviability of cells with oxidative defects in galactose medium: a screening test for affected patient fibroblasts.
nadh:ubiquinone reductase (h+-translocating) deficiency
Ultrastructural examination of skin biopsies may assist in diagnosing mitochondrial cytopathy when muscle biopsies yield negative results.
Nasopharyngeal Carcinoma
Pyruvate dehydrogenase B promoted the growth and migration of the nasopharyngeal carcinoma cells.
Neoplasm Metastasis
Novel molecular mechanisms of antitumor action of dichloroacetate against T cell lymphoma: Implication of altered glucose metabolism, pH homeostasis and cell survival regulation.
Neoplasms
A combination of alpha lipoic acid and calcium hydroxycitrate is efficient against mouse cancer models: preliminary results.
Neoplasms
A key role for mitochondrial gatekeeper pyruvate dehydrogenase in oncogene-induced senescence.
Neoplasms
Ammonium ions enhance the flow through the pyruvate dehydrogenase in Ehrlich ascites tumor cells.
Neoplasms
Animal Models of Metabolic Epilepsy and Epilepsy Associated Metabolic Dysfunction: A Systematic Review.
Neoplasms
Antiglioma pseurotin A from marine Bacillus sp. FS8D regulating tumour metabolic enzymes.
Neoplasms
Assessment of early diabetic renal changes with hyperpolarized [1-(13) C]pyruvate.
Neoplasms
Cell-surface G-protein-coupled receptors for tumor-associated metabolites: A direct link to mitochondrial dysfunction in cancer.
Neoplasms
Compartmentalized activities of the pyruvate dehydrogenase complex sustain lipogenesis in prostate cancer.
Neoplasms
Development of Dihydroxyphenyl Sulfonylisoindoline Derivatives as Liver-Targeting Pyruvate Dehydrogenase Kinase Inhibitors.
Neoplasms
Development of pyruvate dehydrogenase kinase inhibitors in medicinal chemistry with particular emphasis as anticancer agents.
Neoplasms
Development of the First Generation of Disulfide-Based Subtype-Selective and Potent Covalent Pyruvate Dehydrogenase Kinase 1 (PDK1) Inhibitors.
Neoplasms
Dichloroacetate and Pyruvate Metabolism: Pyruvate Dehydrogenase Kinases as Targets Worth Investigating for Effective Therapy of Toxoplasmosis.
Neoplasms
Discovery of novel pyruvate dehydrogenase kinases inhibitors by screening of an in-house small molecule library for anti-lung cancer therapeutics.
Neoplasms
Effects of HIF-1alpha and HIF2alpha on Growth and Metabolism of Clear-Cell Renal Cell Carcinoma 786-0 Xenografts.
Neoplasms
Epigallocatechin-3-gallate downregulates PDHA1 interfering the metabolic pathways in human herpesvirus 8 harboring primary effusion lymphoma cells.
Neoplasms
Expression of type 2 hexokinase and mitochondria-related genes in gastric carcinoma tissues and cell lines.
Neoplasms
GSTZ1 genotypes correlate with dichloroacetate pharmacokinetics and chronic side effects in multiple myeloma patients in a pilot phase 2 clinical trial.
Neoplasms
Hemistepsin A suppresses colorectal cancer growth through inhibiting pyruvate dehydrogenase kinase activity.
Neoplasms
High-dose vitamin B1 reduces proliferation in cancer cell lines analogous to dichloroacetate.
Neoplasms
High-throughput screening of novel pyruvate dehydrogenase kinases inhibitors and biological evaluation of their in vitro and in vivo antiproliferative activity.
Neoplasms
Hypoxic repression of pyruvate dehydrogenase activity is necessary for metabolic reprogramming and growth of model tumours.
Neoplasms
Identification of an AMP-activatable pyruvate dehydrogenase isozyme in embryos and tumors.
Neoplasms
Identification of Novel Pyruvate Dehydrogenase Kinase 1 (PDK1) Inhibitors by Kinase Activity-Based High-Throughput Screening for Anticancer Therapeutics.
Neoplasms
Identification of Novel Resorcinol Amide Derivatives as Potent and Specific Pyruvate Dehydrogenase Kinase (PDHK) Inhibitors.
Neoplasms
Identification of pyruvate dehydrogenase kinase 1 inhibitors with anti-osteosarcoma activity.
Neoplasms
Ilimaquinone Induces the Apoptotic Cell Death of Cancer Cells by Reducing Pyruvate Dehydrogenase Kinase 1 Activity.
Neoplasms
In vitro cytotoxicity screening to identify novel anti-osteosarcoma therapeutics targeting pyruvate dehydrogenase kinase 2.
Neoplasms
Inhibiting Glycine Decarboxylase Suppresses Pyruvate-to-Lactate Metabolism in Lung Cancer Cells.
Neoplasms
Kinetic and regulatory properties of pyruvate dehydrogenase from Ehrlich ascites tumor cells.
Neoplasms
Lactate-mediated mitoribosomal defects impair mitochondrial oxidative phosphorylation and promote hepatoma cell invasiveness.
Neoplasms
Lung Cancer: A Comparative Study of Metabolism Related Protein Expression in Cancer Cells and Tumor Associated Stroma.
Neoplasms
Metabolic Flexibility in Cancer: Targeting the Pyruvate Dehydrogenase Kinase:Pyruvate Dehydrogenase Axis.
Neoplasms
Microenvironmental control of glucose metabolism in tumors by regulation of pyruvate dehydrogenase.
Neoplasms
miR-26a enhances colorectal cancer cell growth by targeting RREB1 deacetylation to activate AKT-mediated glycolysis.
Neoplasms
miR?21?5p targets PDHA1 to regulate glycolysis and cancer progression in gastric cancer.
Neoplasms
Modelling pyruvate dehydrogenase under hypoxia and its role in cancer metabolism.
Neoplasms
Multiple effects of amobarbital on Ehrlich ascites tumor cells. Inhibition of pyruvate dehydrogenase.
Neoplasms
Novel molecular mechanisms of antitumor action of dichloroacetate against T cell lymphoma: Implication of altered glucose metabolism, pH homeostasis and cell survival regulation.
Neoplasms
Obesity-associated, but not obesity-independent, tumors respond to insulin by increasing mitochondrial glucose oxidation.
Neoplasms
Overexpression of pyruvate dehydrogenase kinase 3 increases drug resistance and early recurrence in colon cancer.
Neoplasms
PDHA1 gene knockout in prostate cancer cells results in metabolic reprogramming towards greater glutamine dependence.
Neoplasms
PDK-1 mediated Hippo-YAP-IRS2 signaling pathway and involved in the apoptosis of non-small cell lung cancer cells.
Neoplasms
PDK-1 regulates lactate production in hypoxia and is associated with poor prognosis in head and neck squamous cancer.
Neoplasms
Phosphorylated form of pyruvate dehydrogenase ?1 mediates tumor necrosis factor ?-induced glioma cell migration.
Neoplasms
Poldip2 is an oxygen-sensitive protein that controls PDH and ?KGDH lipoylation and activation to support metabolic adaptation in hypoxia and cancer.
Neoplasms
Pyruvate Dehydrogenase PDH-E1? Controls Tumor Progression by Altering the Metabolic Status of Cancer Cells.
Neoplasms
Rational combination with PDK1 inhibition overcomes cetuximab resistance in head and neck squamous cell carcinoma.
Neoplasms
Re: Jinjing Chen, Ilaria Guccini, Diletta Di Mitri, et al. Compartmentalized Activities of the Pyruvate Dehydrogenase Complex Sustain Lipogenesis in Prostate Cancer. Nat Genet 2018;50:219-28: Lipid Metabolism in Prostate Cancer: Expanding Patient Therapeutic Opportunities.
Neoplasms
SIRT3 deacetylates and increases pyruvate dehydrogenase activity in cancer cells.
Neoplasms
Src drives the Warburg effect and therapy resistance by inactivating pyruvate dehydrogenase through tyrosine-289 phosphorylation.
Neoplasms
Subpopulation targeting of pyruvate dehydrogenase and GLUT1 decouples metabolic heterogeneity during collective cancer cell invasion.
Neoplasms
Succinate aggravates NAFLD progression to liver cancer on the onset of obesity: An in silico model.
Neoplasms
Synthesis and biological evaluation of (R)-3,3,3-trifluoro-2-hydroxy-2-methylpropionamides as pyruvate dehydrogenase kinase 1 (PDK1) inhibitors to reduce the growth of cancer cells.
Neoplasms
Synthesis, biological evaluation and structure-activity relationship of novel dichloroacetophenones targeting pyruvate dehydrogenase kinases with potent anticancer activity.
Neoplasms
Synthetic Essentiality of Metabolic Regulator PDHK1 in PTEN-Deficient Cells and Cancers.
Neoplasms
Targeting PDK1 with dichloroacetophenone to inhibit acute myeloid leukemia (AML) cell growth.
Neoplasms
Targeting thiamine-dependent enzymes for metabolic therapies in oral squamous cell carcinoma?
Neoplasms
Targeting Tumor Metabolism for Cancer Treatment: Is Pyruvate Dehydrogenase Kinases (PDKs) a Viable Anticancer Target?
Neoplasms
The HGF-MET axis coordinates liver cancer metabolism and autophagy for chemotherapeutic resistance.
Neoplasms
The Importance of Gender-Related Anticancer Research on Mitochondrial Regulator Sodium Dichloroacetate in Preclinical Studies In Vivo.
Neoplasms
The novel function of tumor protein D54 in regulating pyruvate dehydrogenase and metformin cytotoxicity in breast cancer.
Neoplasms
The right ventricle in pulmonary arterial hypertension: disorders of metabolism, angiogenesis and adrenergic signaling in right ventricular failure.
Neoplasms
Theoretical aspects of weight loss in patients with cancer. Possible importance of pyruvate dehydrogenase.
Neoplasms
Therapeutic Targeting of the Pyruvate Dehydrogenase Complex/Pyruvate Dehydrogenase Kinase (PDC/PDK) Axis in Cancer.
Neoplasms
Tyr-94 phosphorylation inhibits pyruvate dehydrogenase phosphatase 1 and promotes tumor growth.
Neoplasms
Unexpected Discovery of Dichloroacetate Derived Adenosine Triphosphate Competitors Targeting Pyruvate Dehydrogenase Kinase To Inhibit Cancer Proliferation.
Neoplasms
[Effects of stromal cells derived from the normal prostate on the glycolysis of prostate cancer cells].
Neoplasms
[S35 lipoic acid distribution and its effect on pyruvate dehydrogenase activity in rats with Walker carcinoma]
Nephritis
HUANGKUISIWUFANG inhibits pyruvate dehydrogenase to improve glomerular injury in anti-Thy1 nephritis model.
Nervous System Diseases
Metabolic reprogramming by the pyruvate dehydrogenase kinase-lactic acid axis: Linking metabolism and diverse neuropathophysiologies.
Nervous System Diseases
Pyruvate dehydrogenase deficiency presenting as isolated paroxysmal exercise induced dystonia successfully reversed with thiamine supplementation. Case report and mini-review.
Neuroblastoma
Cellular effects of endotoxin in vitro. II. Reversibility of endotoxic damage.
Neuroblastoma
RS-alpha-lipoic acid protects cholinergic cells against sodium nitroprusside and amyloid-beta neurotoxicity through restoration of acetyl-CoA level.
Neurodegenerative Diseases
CoA-dependent activation of mitochondrial acyl carrier protein links four neurodegenerative diseases.
Neuroinflammatory Diseases
Mitochondrial metabolism: a common link between neuroinflammation and neurodegeneration.
Neurologic Manifestations
Cerebral Developmental Abnormalities in a Mouse with Systemic Pyruvate Dehydrogenase Deficiency.
Neurologic Manifestations
Clinical and genetic spectrum of pyruvate dehydrogenase deficiency: dihydrolipoamide acetyltransferase (E2) deficiency.
Non-alcoholic Fatty Liver Disease
Pyruvate Dehydrogenase as a Therapeutic Target for Nonalcoholic Fatty Liver Disease.
Non-alcoholic Fatty Liver Disease
The antianginal ranolazine mitigates obesity-induced nonalcoholic fatty liver disease and increases hepatic pyruvate dehydrogenase activity.
Obesity
Adipocyte glycogen synthase and pyruvate dehydrogenase in obese and type II diabetic subjects.
Obesity
Age-related changes in liver and adipose tissue pyruvate dehydrogenase of genetically obese mice.
Obesity
Changes in glycogen metabolism in liver of gold thioglucose injected mice during the development of obesity.
Obesity
Development of Dihydroxyphenyl Sulfonylisoindoline Derivatives as Liver-Targeting Pyruvate Dehydrogenase Kinase Inhibitors.
Obesity
Different types of postinsulin receptor defects contribute to insulin resistance in hearts of obese Zucker rats.
Obesity
Dose-response characteristics of impaired glucose oxidation in non-insulin-dependent diabetes mellitus.
Obesity
Inactivation of pyruvate dehydrogenase complex in heart muscle mitochondria of gold-thioglucose-induced obese mice is not due to a stable increase in activity of pyruvate dehydrogenase kinase.
Obesity
Postnatal induction of muscle fatty acid oxidation in mice differing in propensity to obesity: a role of pyruvate dehydrogenase.
Obesity
Proportion of active dephosphorylated pyruvate dehydrogenase complex in heart and isolated heart mitochondria is decreased in obese hyperinsulinaemic mice.
Obesity
Pyruvate and hepatic pyruvate dehydrogenase levels in rat strains sensitive and resistant to dietary obesity.
Obesity
The activity of the pyruvate dehydrogenase complex in heart and liver from mice during the development of obesity and insulin resistance.
Obesity
The effect of diet composition on weight gain and pyruvate dehydrogenase activity in heart muscle in the gold thioglucose obese mouse.
Obesity
Tissue differences in the response of the pyruvate dehydrogenase complex to a glucose load during the development of obesity in gold-thioglucose-obese mice.
Ophthalmoplegia
Vitamin-responsive pyruvate dehydrogenase deficiency in a young girl with external ophthalmoplegia, myopathy and lactic acidosis.
Ophthalmoplegia, Chronic Progressive External
Renal manifestations of primary mitochondrial disorders.
Optic Atrophy, Autosomal Dominant
Deterioration of mitochondrial bioenergetics and ultrastructure impairment in skeletal muscle of a transgenic minipig model in the early stages of Huntington's disease.
Optic Atrophy, Hereditary, Leber
Clinical Therapeutic Management of Human Mitochondrial Disorders.
Optic Nerve Diseases
Thiamine responsive pyruvate dehydrogenase deficiency in an adult with peripheral neuropathy and optic neuropathy.
Optic Neuropathy, Ischemic
A pyruvate dehydrogenase kinase inhibitor prevents retinal cell death and improves energy metabolism in rat retinas after ischemia/reperfusion injury.
ornithine carbamoyltransferase deficiency
Impact of selected inborn errors of metabolism on prenatal and neonatal development.
Ornithine Carbamoyltransferase Deficiency Disease
Impact of selected inborn errors of metabolism on prenatal and neonatal development.
Ovarian Diseases
Sensitivity of pyruvate dehydrogenase to insulin in activated T lymphocytes. Lack of responsiveness to insulin in patients with polycystic ovarian disease and diabetes.
Ovarian Neoplasms
Multiple blocks in the engagement of oxidative phosphorylation in putative ovarian cancer stem cells: implication for maintenance therapy with glycolysis inhibitors.
peptidyl-glutamate 4-carboxylase deficiency
Defects of pyruvate metabolism and the Krebs cycle.
Perinatal Death
Prenatal diagnosis of pyruvate dehydrogenase deficiency using magnetic resonance imaging.
Peripheral Nervous System Diseases
Leigh syndrome: pyruvate dehydrogenase defect. A case with peripheral neuropathy.
Peripheral Nervous System Diseases
Thiamine responsive pyruvate dehydrogenase deficiency in an adult with peripheral neuropathy and optic neuropathy.
Peroxisomal Disorders
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
Peroxisomal Disorders
Lactate oxidation for the detection of mitochondrial dysfunction in human skin fibroblasts.
Persistent Infection
Mycoplasma genitalium rapidly disseminates to the upper reproductive tracts and knees of female mice following vaginal inoculation.
Phenylketonuria, Maternal
Inborn errors of metabolism: a cause of abnormal brain development.
Phenylketonurias
Aminoacidopathies: Prevalence, Etiology, Screening, and Treatment Options.
Phenylketonurias
Effect of phenylpyruvate on pyruvate dehydrogenase activity in rat brain mitochondria.
Pick Disease of the Brain
Enzyme activities in relation to pH and lactate in postmortem brain in Alzheimer-type and other dementias.
Polycystic Ovary Syndrome
Opposing actions of dehydroepiandrosterone and testosterone on insulin sensitivity. In vivo and in vitro studies of hyperandrogenic females.
Polymicrogyria
Cerebral white matter involvement in children with mitochondrial encephalopathies.
Prostatic Neoplasms
Compartmentalized activities of the pyruvate dehydrogenase complex sustain lipogenesis in prostate cancer.
Prostatic Neoplasms
Differential effects of specific amino acid restriction on glucose metabolism, reduction/oxidation status and mitochondrial damage in DU145 and PC3 prostate cancer cells.
Prostatic Neoplasms
Pyruvate dehydrogenase alpha 1 as a target of omega-3 polyunsaturated fatty acids in human prostate cancer through a global phosphoproteomic analysis.
Prostatic Neoplasms
Pyruvate dehydrogenase expression is negatively associated with cell stemness and worse clinical outcome in prostate cancers.
Prostatic Neoplasms
Re: Jinjing Chen, Ilaria Guccini, Diletta Di Mitri, et al. Compartmentalized Activities of the Pyruvate Dehydrogenase Complex Sustain Lipogenesis in Prostate Cancer. Nat Genet 2018;50:219-28: Lipid Metabolism in Prostate Cancer: Expanding Patient Therapeutic Opportunities.
Protein Deficiency
A new case of pyruvate dehydrogenase deficiency due to a novel mutation in the PDX1 gene.
Protein Deficiency
Lactic acidosis and developmental delay due to deficiency of E3 binding protein (protein X) of the pyruvate dehydrogenase complex.
Protein Deficiency
Mendeliome sequencing enables differential diagnosis and treatment of neonatal lactic acidosis.
Protein Deficiency
Primary pyruvate dehydrogenase E3 binding protein deficiency with mild hyperlactataemia and hyperalaninaemia.
Pulmonary Arterial Hypertension
Epigenetic Metabolic Reprogramming of Right Ventricular Fibroblasts in Pulmonary Arterial Hypertension: A Pyruvate Dehydrogenase Kinase-Dependent Shift in Mitochondrial Metabolism Promotes Right Ventricular Fibrosis.
Pulmonary Arterial Hypertension
Pyruvate dehydrogenase inhibition by the inflammatory cytokine TNF? contributes to the pathogenesis of pulmonary arterial hypertension.
pyruvate carboxylase deficiency
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
pyruvate carboxylase deficiency
Prenatal sonographic description of fetuses affected by pyruvate dehydrogenase or pyruvate carboxylase deficiency.
Pyruvate Carboxylase Deficiency Disease
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
Pyruvate Carboxylase Deficiency Disease
Prenatal sonographic description of fetuses affected by pyruvate dehydrogenase or pyruvate carboxylase deficiency.
pyruvate decarboxylase deficiency
Outcome of pyruvate dehydrogenase deficiency treated with ketogenic diets. Studies in patients with identical mutations.
pyruvate dehydrogenase (acetyl-transferring) deficiency
An animal model of PDH deficiency using AAV8-siRNA vector-mediated knockdown of pyruvate dehydrogenase E1?.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Autopsy findings in pyruvate dehydrogenase E1alpha deficiency: case report.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Beta cell-specific pyruvate dehydrogenase deficiency impairs glucose-stimulated insulin secretion.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Gene therapy for pyruvate dehydrogenase E1alpha deficiency using recombinant adeno-associated virus 2 (rAAV2) vectors.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Immunocapture and microplate-based activity measurement of mammalian pyruvate dehydrogenase complex.
pyruvate dehydrogenase (acetyl-transferring) deficiency
In vivo pyruvate detected by MR spectroscopy in neonatal pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Nonspecific mitochondrial disease with epilepsy in children: diagnostic approaches and epileptic phenotypes.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Phenyl butyrate inhibits pyruvate dehydrogenase kinase 1 and contributes to its anti-cancer effect.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Pyruvate dehydrogenase deficiency caused by deletion of a 7-bp repeat sequence in the E1 alpha gene.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Pyruvate dehydrogenase deficiency due to a 20-bp deletion in exon II of the pyruvate dehydrogenase (PDH) E1 alpha gene.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Sequential deletion of C-terminal amino acids of the E(1)alpha component of the pyruvate dehydrogenase (PDH) complex leads to reduced steady-state levels of functional E(1)alpha(2)beta(2) tetramers: implications for patients with PDH deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
[Analysis of PDHA1 gene variant in a patient with pyruvate dehydrogenase E1alpha deficiency and pyramidal tract involvement].
pyruvate dehydrogenase (nadp+) deficiency
A combined therapeutic approach for pyruvate dehydrogenase deficiency using self-complementary adeno-associated virus serotype-specific vectors and dichloroacetate.
pyruvate dehydrogenase (nadp+) deficiency
A four-nucleotide insertion at the E1 alpha gene in a patient with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
A new case of pyruvate dehydrogenase deficiency due to a novel mutation in the PDX1 gene.
pyruvate dehydrogenase (nadp+) deficiency
A novel gross deletion caused by non-homologous recombination of the PDHX gene in a patient with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
A pathogenic glutamate-to-aspartate substitution (D296E) in the pyruvate dehydrogenase E1 subunit gene PDHA1.
pyruvate dehydrogenase (nadp+) deficiency
A possible mechanism for selective cerebellar damage in partial pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
A putative exonic splicing enhancer in exon 7 of the PDHA1 gene affects splicing of adjacent exons.
pyruvate dehydrogenase (nadp+) deficiency
A zebrafish model for pyruvate dehydrogenase deficiency: rescue of neurological dysfunction and embryonic lethality using a ketogenic diet.
pyruvate dehydrogenase (nadp+) deficiency
Acute flaccid paralysis as initial symptom in 4 patients with novel e1alpha mutations of the pyruvate dehydrogenase complex.
pyruvate dehydrogenase (nadp+) deficiency
Agenesis of the Corpus Callosum and Skeletal Deformities in Two Unrelated Patients: Analysis via MRI and Radiography.
pyruvate dehydrogenase (nadp+) deficiency
Alternating hemiplegia of childhood: metabolic studies in the largest European series of patients.
pyruvate dehydrogenase (nadp+) deficiency
An immunochemical assay model system for the sensitive detection of pyruvate dehydrogenase complex (PDHc) and its decarboxylating subunit pyruvate dehydrogenase (E1).
pyruvate dehydrogenase (nadp+) deficiency
An improved spectrophotometric assay of pyruvate dehydrogenase in lactate dehydrogenase contaminated mitochondrial preparations from human skeletal muscle.
pyruvate dehydrogenase (nadp+) deficiency
Anaesthesia and pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Anaesthesia in pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Anesthesia in a child with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Anesthesia in a child with pyruvate dehydrogenase deficiency: a case report.
pyruvate dehydrogenase (nadp+) deficiency
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
pyruvate dehydrogenase (nadp+) deficiency
Arginine 302 mutations in the pyruvate dehydrogenase E1alpha subunit gene: identification of further patients and in vitro demonstration of pathogenicity.
pyruvate dehydrogenase (nadp+) deficiency
Beta cell-specific pyruvate dehydrogenase deficiency impairs glucose-stimulated insulin secretion.
pyruvate dehydrogenase (nadp+) deficiency
Bi-allelic mutations of LONP1 encoding the mitochondrial LonP1 protease cause pyruvate dehydrogenase deficiency and profound neurodegeneration with progressive cerebellar atrophy.
pyruvate dehydrogenase (nadp+) deficiency
Biochemical and molecular analysis of an X-linked case of Leigh syndrome associated with thiamin-responsive pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Biochemical and structural brain alterations in female mice with cerebral pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Blood-brain barrier, ion homeostatis and epilepsy: possible implications towards the understanding of ketogenic diet mechanisms.
pyruvate dehydrogenase (nadp+) deficiency
Brain metabolism modulates neuronal excitability in a mouse model of pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Case Report of Pyruvate Dehydrogenase Deficiency With Unusual Increase of Fats During Ketogenic Diet Treatment.
pyruvate dehydrogenase (nadp+) deficiency
Cerebral Developmental Abnormalities in a Mouse with Systemic Pyruvate Dehydrogenase Deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Cerebral dysgenesis and lactic acidemia: an MRI/MRS phenotype associated with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Cerebral palsy and pyruvate dehydrogenase deficiency: identification of two new mutations in the E1alpha gene.
pyruvate dehydrogenase (nadp+) deficiency
Cerebral white matter involvement in children with mitochondrial encephalopathies.
pyruvate dehydrogenase (nadp+) deficiency
Characterization of point mutations in patients with pyruvate dehydrogenase deficiency: role of methionine-181, proline-188, and arginine-349 in the alpha subunit.
pyruvate dehydrogenase (nadp+) deficiency
Characterization of the mutations in three patients with pyruvate dehydrogenase E1 alpha deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Clinical and biochemical characterization of four patients with mutations in ECHS1.
pyruvate dehydrogenase (nadp+) deficiency
Clinical and genetic spectrum of pyruvate dehydrogenase deficiency: dihydrolipoamide acetyltransferase (E2) deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Clinical diversity of pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Clinical manifestations in two patients with pyruvate dehydrogenase deficiency and long-term survival.
pyruvate dehydrogenase (nadp+) deficiency
Clinical Therapeutic Management of Human Mitochondrial Disorders.
pyruvate dehydrogenase (nadp+) deficiency
Considerations on the management of pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Continuous pH monitoring using the Paratrend 7 inserted into a peripheral vein in a patient with shock and congenital lactic acidosis.
pyruvate dehydrogenase (nadp+) deficiency
Controlled clinical trial of dichloroacetate for treatment of congenital lactic acidosis in children.
pyruvate dehydrogenase (nadp+) deficiency
Cortical metabolism in pyruvate dehydrogenase deficiency revealed by ex vivo multiplet (13)C NMR of the adult mouse brain.
pyruvate dehydrogenase (nadp+) deficiency
Defects of pyruvate metabolism and the Krebs cycle.
pyruvate dehydrogenase (nadp+) deficiency
Determination of pyruvate dehydrogenase in cultured human fibroblasts and amniotic fluid cells.
pyruvate dehydrogenase (nadp+) deficiency
Development of subdural effusions in association with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Diagnostic accuracy of blood lactate-to-pyruvate molar ratio in the differential diagnosis of congenital lactic acidosis.
pyruvate dehydrogenase (nadp+) deficiency
Dichloroacetate stabilizes the mutant E1alpha subunit in pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
pyruvate dehydrogenase (nadp+) deficiency
DNA diagnosis of pyruvate dehydrogenase deficiency in female patients with congenital lactic acidaemia.
pyruvate dehydrogenase (nadp+) deficiency
E1 pyruvate dehydrogenase deficiency in a child with motor neuropathy.
pyruvate dehydrogenase (nadp+) deficiency
Enzymatic pyruvate measurement by Cobas 6000 open channel assay.
pyruvate dehydrogenase (nadp+) deficiency
Evoked potentials in children with oxidative metabolic defects leading to Leigh syndrome.
pyruvate dehydrogenase (nadp+) deficiency
Females with PDHA1 gene mutations: a diagnostic challenge.
pyruvate dehydrogenase (nadp+) deficiency
Follow-up of a child with pyruvate dehydrogenase deficiency on a less restrictive ketogenic diet.
pyruvate dehydrogenase (nadp+) deficiency
Food and Food Products on the Italian Market for Ketogenic Dietary Treatment of Neurological Diseases.
pyruvate dehydrogenase (nadp+) deficiency
Four novel PDHA1 mutations in pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Gene regulation and genetic defects in the pyruvate dehydrogenase complex.
pyruvate dehydrogenase (nadp+) deficiency
Genetic and clinical features of Chinese patients with mitochondrial ataxia identified by targeted next-generation sequencing.
pyruvate dehydrogenase (nadp+) deficiency
Glucose transporter 1 deficiency syndrome and other glycolytic defects.
pyruvate dehydrogenase (nadp+) deficiency
Glutamate and pyruvate dehydrogenase deficiency in spinocerebellar degeneration.
pyruvate dehydrogenase (nadp+) deficiency
Heterogeneous expression of protein and mRNA in pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Histological changes of muscle in a patient with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Hyperammonaemia and lactic acidosis in a patient with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Immunological approaches to the characterization and diagnosis of mitochondrial disease.
pyruvate dehydrogenase (nadp+) deficiency
Impact of selected inborn errors of metabolism on prenatal and neonatal development.
pyruvate dehydrogenase (nadp+) deficiency
In utero central nervous system damage in pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
In vivo pyruvate detected by MR spectroscopy in neonatal pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Inborn errors of metabolism: a cause of abnormal brain development.
pyruvate dehydrogenase (nadp+) deficiency
Intermittent peripheral weakness as the presenting feature of pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Isolated capillary proliferation in Leigh's syndrome.
pyruvate dehydrogenase (nadp+) deficiency
Ketogenic diet and childhood neurological disorders other than epilepsy: an overview.
pyruvate dehydrogenase (nadp+) deficiency
Ketogenic Diet: A New Light Shining on Old but Gold Biochemistry.
pyruvate dehydrogenase (nadp+) deficiency
Ketogenic diet: an early option for epilepsy treatment, instead of a last choice only.
pyruvate dehydrogenase (nadp+) deficiency
Ketone Bodies as a Possible Adjuvant to Ketogenic Diet in PDHc Deficiency but Not in GLUT1 Deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Ketonic diet in the management of pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Lactic acidosis and developmental delay due to deficiency of E3 binding protein (protein X) of the pyruvate dehydrogenase complex.
pyruvate dehydrogenase (nadp+) deficiency
Lacticacidaemia due to pyruvate dehydrogenase deficiency, with evidence of protein polymorphism in the alpha-subunit of the enzyme.
pyruvate dehydrogenase (nadp+) deficiency
Late-onset presentation of pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Leigh syndrome: pyruvate dehydrogenase defect. A case with peripheral neuropathy.
pyruvate dehydrogenase (nadp+) deficiency
Leukoencephalopathy with cysts and hyperglycinemia may result from NFU1 deficiency.
pyruvate dehydrogenase (nadp+) deficiency
LIPT1 deficiency presenting as early infantile epileptic encephalopathy, Leigh disease, and secondary pyruvate dehydrogenase complex deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Massive parallel sequencing identifies RAPSN and PDHA1 mutations causing fetal akinesia deformation sequence.
pyruvate dehydrogenase (nadp+) deficiency
Mechanisms of expression of pyruvate dehydrogenase deficiency caused by an E1alpha subunit mutation.
pyruvate dehydrogenase (nadp+) deficiency
Metabolite studies in HIBCH and ECHS1 defects: Implications for screening.
pyruvate dehydrogenase (nadp+) deficiency
Mitochondrial dysfunction in a patient with Joubert syndrome.
pyruvate dehydrogenase (nadp+) deficiency
Mitochondrial respiratory chain disease discrimination by retrospective cohort analysis of blood metabolites.
pyruvate dehydrogenase (nadp+) deficiency
Molecular genetic analysis of a female patient with pyruvate dehydrogenase deficiency: detection of a new mutation and differential expression of mutant gene product in cultured cells.
pyruvate dehydrogenase (nadp+) deficiency
Morphological correlates of mitochondrial dysfunction in children.
pyruvate dehydrogenase (nadp+) deficiency
Moving Metabolism to Make Inroads in a Model of Mitochondrial Epilepsy.
pyruvate dehydrogenase (nadp+) deficiency
MRI features of 4 female patients with pyruvate dehydrogenase E1 alpha deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Mutation of E1 alpha gene in a female patient with pyruvate dehydrogenase deficiency due to rapid degradation of E1 protein.
pyruvate dehydrogenase (nadp+) deficiency
Mutations in the gene for the E1beta subunit: a novel cause of pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Mutations of the E1beta subunit gene (PDHB) in four families with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Neonatal pyruvate dehydrogenase deficiency due to a R302H mutation in the PDHA1 gene: MRI findings.
pyruvate dehydrogenase (nadp+) deficiency
Neonatal pyruvate dehydrogenase deficiency with lipoate responsive lactic acidaemia and hyperammonaemia.
pyruvate dehydrogenase (nadp+) deficiency
Optic neuropathy in a patient with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Orthopaedic Problems in 35 Patients With Organic Acid Disorders.
pyruvate dehydrogenase (nadp+) deficiency
Outcome of pyruvate dehydrogenase deficiency treated with ketogenic diets. Studies in patients with identical mutations.
pyruvate dehydrogenase (nadp+) deficiency
Outcome of thiamine treatment in a child with Leigh disease due to thiamine-responsive pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Partial complementation of pyruvate dehydrogenase deficiency by independently expressed lipoyl and catalytic domains of the dihydrolipoamide acetyltransferase component.
pyruvate dehydrogenase (nadp+) deficiency
Phenotypic and Neuropathological Characterization of Fetal Pyruvate Dehydrogenase Deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Pitfalls of relying on genetic testing only to diagnose inherited metabolic disorders in non-western populations - 5 cases of pyruvate dehydrogenase deficiency from South Africa.
pyruvate dehydrogenase (nadp+) deficiency
Potential role of stress-induced gluconeogenesis in disease aggravation and mortality in pyruvate dehydrogenase deficiency: A case-based hypothesis.
pyruvate dehydrogenase (nadp+) deficiency
Prenatal diagnosis of pyruvate dehydrogenase deficiency using magnetic resonance imaging.
pyruvate dehydrogenase (nadp+) deficiency
Prenatal sonographic description of fetuses affected by pyruvate dehydrogenase or pyruvate carboxylase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency - morphological and metabolic effects, creation of animal model to study and research for treatment therapy.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency as a result of splice-site mutations in the PDX1 gene.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency caused by a 33 base pair duplication in the PDH E1 alpha subunit.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency caused by a 5 base pair duplication in the E1 alpha subunit.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency caused by a four-nucleotide insertion in the E1 alpha subunit gene.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency caused by a new mutation of PDHX gene in two Moroccan patients.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency caused by deletion of a 7-bp repeat sequence in the E1 alpha gene.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency disease detected by the enzyme activity of peripheral leukocytes.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency due to a 20-bp deletion in exon II of the pyruvate dehydrogenase (PDH) E1 alpha gene.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency due to a mutation of the E1 alpha subunit.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency in a child responsive to thiamine treatment.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency in a female due to a 4 base pair deletion in exon 10 of the E1 alpha gene.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency in a male caused by a point mutation (F205L) in the E1 alpha subunit.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency in a Sussex spaniel.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency in spinocerebellar degenerations.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency presenting as dystonia in childhood.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency presenting as intermittent isolated acute ataxia.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency presenting as isolated paroxysmal exercise induced dystonia successfully reversed with thiamine supplementation. Case report and mini-review.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency restricted to brain.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency. Clinical presentation and molecular genetic characterization of five new patients.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency: clinical and biochemical diagnosis.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency: identification of a novel mutation in the PDHA1 gene which responds to amino acid supplementation.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency: molecular basis for intrafamilial heterogeneity.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase deficiency: the relation of the E1 alpha mutation to the E1 beta subunit deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase E2 deficiency: A potentially treatable cause of episodic dystonia.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate dehydrogenase E3 binding protein deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Pyruvate Dehydrogenase-E1? Deficiency Presenting as Recurrent Demyelination: An Unusual Presentation and a Novel Mutation.
pyruvate dehydrogenase (nadp+) deficiency
Rapid diagnosis of pyruvate and ketoglutarate dehydrogenase deficiencies in platelet-enriched preparations from blood.
pyruvate dehydrogenase (nadp+) deficiency
Reconciling diabetes management and the ketogenic diet in a child with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Renal manifestations of primary mitochondrial disorders.
pyruvate dehydrogenase (nadp+) deficiency
Sensitivity to carbohydrate in a patient with familial intermittent lactic acidosis and pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Sensory-motor polyneuropathy occurring in variant maple syrup urine disease.
pyruvate dehydrogenase (nadp+) deficiency
Severe Brain Malformations in an Infant With Pyruvate Dehydrogenase Deficiency and Down Syndrome.
pyruvate dehydrogenase (nadp+) deficiency
Severe Neonatal Presentation of Mitochondrial Citrate Carrier (SLC25A1) Deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Somatic mosaicism for a PDHA1 mutation in a female with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Studies of the pathophysiology of pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Summary of recommendations for the management of infantile seizures: Task Force Report for the ILAE Commission of Pediatrics.
pyruvate dehydrogenase (nadp+) deficiency
Tenuous link between chronic fatigue syndrome and pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
The clinical spectrum of nodular heterotopias in children: Report of 31 patients.
pyruvate dehydrogenase (nadp+) deficiency
The first case with FBXL4 mutation successfully treated with a parenteral ketogenic diet for lactic acidosis.
pyruvate dehydrogenase (nadp+) deficiency
The genetic heterogeneity of lactic acidosis: occurrence of recognizable inborn errors of metabolism in pediatric population with lactic acidosis.
pyruvate dehydrogenase (nadp+) deficiency
The human pyruvate dehydrogenase complex. Isolation of cDNA clones for the E1 alpha subunit, sequence analysis, and characterization of the mRNA.
pyruvate dehydrogenase (nadp+) deficiency
The importance of cerebrospinal fluid lactate in the evaluation of congenital lactic acidosis.
pyruvate dehydrogenase (nadp+) deficiency
The Importance of Gender-Related Anticancer Research on Mitochondrial Regulator Sodium Dichloroacetate in Preclinical Studies In Vivo.
pyruvate dehydrogenase (nadp+) deficiency
The ketogenic diet: uses in epilepsy and other neurologic illnesses.
pyruvate dehydrogenase (nadp+) deficiency
Therapeutic efficacy of a case of pyruvate dehydrogenase complex deficiency monitored by localized proton magnetic resonance spectroscopy.
pyruvate dehydrogenase (nadp+) deficiency
Thiamine dependency in a patient with congenital lacticacidaemia due to pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Thiamine responsive pyruvate dehydrogenase deficiency in an adult with peripheral neuropathy and optic neuropathy.
pyruvate dehydrogenase (nadp+) deficiency
Thiamine responsive pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Thiamine-responsive pyruvate dehydrogenase deficiency in two patients caused by a point mutation (F205L and L216F) within the thiamine pyrophosphate binding region.
pyruvate dehydrogenase (nadp+) deficiency
Two Patients with Clinically Distinct Manifestation of Pyruvate Dehydrogenase Deficiency Due to Mutations in PDHA1 Gene.
pyruvate dehydrogenase (nadp+) deficiency
Ultrastructural examination of skin biopsies may assist in diagnosing mitochondrial cytopathy when muscle biopsies yield negative results.
pyruvate dehydrogenase (nadp+) deficiency
Ultrastructural examination of the axillary skin biopsy in the diagnosis of metabolic diseases.
pyruvate dehydrogenase (nadp+) deficiency
Unilateral periventricular leukomalacia in association with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Urinary lactate excretion in normal children and in children with enzyme defects of carbohydrate metabolism.
pyruvate dehydrogenase (nadp+) deficiency
Vitamin-Responsive Movement Disorders in Children.
pyruvate dehydrogenase (nadp+) deficiency
Vitamin-responsive pyruvate dehydrogenase deficiency in a young girl with external ophthalmoplegia, myopathy and lactic acidosis.
pyruvate dehydrogenase (nadp+) deficiency
X-chromosome localization of the functional gene for the E1 alpha subunit of the human pyruvate dehydrogenase complex.
pyruvate dehydrogenase (nadp+) deficiency
Zonisamide ameliorates symptoms of secondary paroxysmal dystonia.
pyruvate dehydrogenase (nadp+) deficiency
[A case of pyruvate dehydrogenase deficiency with low density areas in white matter noticed by CT scan]
pyruvate dehydrogenase (nadp+) deficiency
[Inborn errors of metabolism in adult neurology].
pyruvate dehydrogenase (nadp+) deficiency
[Lactic acid acidosis with mitochondrial myopathy due to a pyruvate dehydrogenase deficiency]
pyruvate dehydrogenase (nadp+) deficiency
[Pyruvate dehydrogenase deficiency and cerebral malformations]
pyruvate dehydrogenase (nadp+) deficiency
[Pyruvate dehydrogenase deficiency in a child with persistent lactic acidosis]
pyruvate dehydrogenase (nadp+) deficiency
[Pyruvate-dehydrogenase deficiency. Lethal course of the disease during infancy (author's transl)]
Pyruvate Dehydrogenase Complex Deficiency Disease
A combined therapeutic approach for pyruvate dehydrogenase deficiency using self-complementary adeno-associated virus serotype-specific vectors and dichloroacetate.
Pyruvate Dehydrogenase Complex Deficiency Disease
A family with pyruvate dehydrogenase complex deficiency due to a novel C>T substitution at nucleotide position 407 in exon 4 of the X-linked Epsilon1alpha gene.
Pyruvate Dehydrogenase Complex Deficiency Disease
A four-nucleotide insertion at the E1 alpha gene in a patient with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
A mutation in the E1 alpha subunit of pyruvate dehydrogenase associated with variable expression of pyruvate dehydrogenase complex deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
A new case of pyruvate dehydrogenase deficiency due to a novel mutation in the PDX1 gene.
Pyruvate Dehydrogenase Complex Deficiency Disease
A novel gross deletion caused by non-homologous recombination of the PDHX gene in a patient with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
A pathogenic glutamate-to-aspartate substitution (D296E) in the pyruvate dehydrogenase E1 subunit gene PDHA1.
Pyruvate Dehydrogenase Complex Deficiency Disease
A possible mechanism for selective cerebellar damage in partial pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
A putative exonic splicing enhancer in exon 7 of the PDHA1 gene affects splicing of adjacent exons.
Pyruvate Dehydrogenase Complex Deficiency Disease
A zebrafish model for pyruvate dehydrogenase deficiency: rescue of neurological dysfunction and embryonic lethality using a ketogenic diet.
Pyruvate Dehydrogenase Complex Deficiency Disease
Acute flaccid paralysis as initial symptom in 4 patients with novel e1alpha mutations of the pyruvate dehydrogenase complex.
Pyruvate Dehydrogenase Complex Deficiency Disease
Agenesis of the Corpus Callosum and Skeletal Deformities in Two Unrelated Patients: Analysis via MRI and Radiography.
Pyruvate Dehydrogenase Complex Deficiency Disease
Alternating hemiplegia of childhood: metabolic studies in the largest European series of patients.
Pyruvate Dehydrogenase Complex Deficiency Disease
An animal model of PDH deficiency using AAV8-siRNA vector-mediated knockdown of pyruvate dehydrogenase E1?.
Pyruvate Dehydrogenase Complex Deficiency Disease
An improved spectrophotometric assay of pyruvate dehydrogenase in lactate dehydrogenase contaminated mitochondrial preparations from human skeletal muscle.
Pyruvate Dehydrogenase Complex Deficiency Disease
Anaesthesia and pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Anaesthesia in pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Anesthesia in a child with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Anesthesia in a child with pyruvate dehydrogenase deficiency: a case report.
Pyruvate Dehydrogenase Complex Deficiency Disease
Anesthesia in pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
Pyruvate Dehydrogenase Complex Deficiency Disease
Arginine 302 mutations in the pyruvate dehydrogenase E1alpha subunit gene: identification of further patients and in vitro demonstration of pathogenicity.
Pyruvate Dehydrogenase Complex Deficiency Disease
Beta cell-specific pyruvate dehydrogenase deficiency impairs glucose-stimulated insulin secretion.
Pyruvate Dehydrogenase Complex Deficiency Disease
Bi-allelic mutations of LONP1 encoding the mitochondrial LonP1 protease cause pyruvate dehydrogenase deficiency and profound neurodegeneration with progressive cerebellar atrophy.
Pyruvate Dehydrogenase Complex Deficiency Disease
Biochemical and molecular analysis of an X-linked case of Leigh syndrome associated with thiamin-responsive pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Biochemical and structural brain alterations in female mice with cerebral pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Blood-brain barrier, ion homeostatis and epilepsy: possible implications towards the understanding of ketogenic diet mechanisms.
Pyruvate Dehydrogenase Complex Deficiency Disease
Brain metabolism modulates neuronal excitability in a mouse model of pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Case Report of Pyruvate Dehydrogenase Deficiency With Unusual Increase of Fats During Ketogenic Diet Treatment.
Pyruvate Dehydrogenase Complex Deficiency Disease
Cerebral Developmental Abnormalities in a Mouse with Systemic Pyruvate Dehydrogenase Deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Cerebral dysgenesis and lactic acidemia: an MRI/MRS phenotype associated with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Cerebral palsy and pyruvate dehydrogenase deficiency: identification of two new mutations in the E1alpha gene.
Pyruvate Dehydrogenase Complex Deficiency Disease
Cerebral white matter involvement in children with mitochondrial encephalopathies.
Pyruvate Dehydrogenase Complex Deficiency Disease
Characterization of point mutations in patients with pyruvate dehydrogenase deficiency: role of methionine-181, proline-188, and arginine-349 in the alpha subunit.
Pyruvate Dehydrogenase Complex Deficiency Disease
Characterization of the mutations in three patients with pyruvate dehydrogenase E1 alpha deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Clinical and biochemical characterization of four patients with mutations in ECHS1.
Pyruvate Dehydrogenase Complex Deficiency Disease
Clinical and genetic spectrum of pyruvate dehydrogenase deficiency: dihydrolipoamide acetyltransferase (E2) deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Clinical diversity of pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Clinical manifestations in two patients with pyruvate dehydrogenase deficiency and long-term survival.
Pyruvate Dehydrogenase Complex Deficiency Disease
Clinical Therapeutic Management of Human Mitochondrial Disorders.
Pyruvate Dehydrogenase Complex Deficiency Disease
Considerations on the management of pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Continuous pH monitoring using the Paratrend 7 inserted into a peripheral vein in a patient with shock and congenital lactic acidosis.
Pyruvate Dehydrogenase Complex Deficiency Disease
Controlled clinical trial of dichloroacetate for treatment of congenital lactic acidosis in children.
Pyruvate Dehydrogenase Complex Deficiency Disease
Cortical metabolism in pyruvate dehydrogenase deficiency revealed by ex vivo multiplet (13)C NMR of the adult mouse brain.
Pyruvate Dehydrogenase Complex Deficiency Disease
Defects of pyruvate metabolism and the Krebs cycle.
Pyruvate Dehydrogenase Complex Deficiency Disease
Defects of thiamine transport and metabolism.
Pyruvate Dehydrogenase Complex Deficiency Disease
Determination of pyruvate dehydrogenase in cultured human fibroblasts and amniotic fluid cells.
Pyruvate Dehydrogenase Complex Deficiency Disease
Development of subdural effusions in association with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Diagnostic accuracy of blood lactate-to-pyruvate molar ratio in the differential diagnosis of congenital lactic acidosis.
Pyruvate Dehydrogenase Complex Deficiency Disease
Dichloroacetate stabilizes the mutant E1alpha subunit in pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Disorders of the pyruvate dehydrogenase complex.
Pyruvate Dehydrogenase Complex Deficiency Disease
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
Pyruvate Dehydrogenase Complex Deficiency Disease
DNA diagnosis of pyruvate dehydrogenase deficiency in female patients with congenital lactic acidaemia.
Pyruvate Dehydrogenase Complex Deficiency Disease
E1 pyruvate dehydrogenase deficiency in a child with motor neuropathy.
Pyruvate Dehydrogenase Complex Deficiency Disease
Evoked potentials in children with oxidative metabolic defects leading to Leigh syndrome.
Pyruvate Dehydrogenase Complex Deficiency Disease
Fatal case of pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Females with PDHA1 gene mutations: a diagnostic challenge.
Pyruvate Dehydrogenase Complex Deficiency Disease
Follow-up of a child with pyruvate dehydrogenase deficiency on a less restrictive ketogenic diet.
Pyruvate Dehydrogenase Complex Deficiency Disease
Food and Food Products on the Italian Market for Ketogenic Dietary Treatment of Neurological Diseases.
Pyruvate Dehydrogenase Complex Deficiency Disease
Four novel PDHA1 mutations in pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Gene regulation and genetic defects in the pyruvate dehydrogenase complex.
Pyruvate Dehydrogenase Complex Deficiency Disease
Genetic and clinical features of Chinese patients with mitochondrial ataxia identified by targeted next-generation sequencing.
Pyruvate Dehydrogenase Complex Deficiency Disease
Glucose transporter 1 deficiency syndrome and other glycolytic defects.
Pyruvate Dehydrogenase Complex Deficiency Disease
Glutamate and pyruvate dehydrogenase deficiency in spinocerebellar degeneration.
Pyruvate Dehydrogenase Complex Deficiency Disease
Heterogeneous expression of protein and mRNA in pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Histological changes of muscle in a patient with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Hyperammonaemia and lactic acidosis in a patient with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Impact of selected inborn errors of metabolism on prenatal and neonatal development.
Pyruvate Dehydrogenase Complex Deficiency Disease
In utero central nervous system damage in pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
In vivo pyruvate detected by MR spectroscopy in neonatal pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Inborn errors of metabolism: a cause of abnormal brain development.
Pyruvate Dehydrogenase Complex Deficiency Disease
Intermittent peripheral weakness as the presenting feature of pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Isolated capillary proliferation in Leigh's syndrome.
Pyruvate Dehydrogenase Complex Deficiency Disease
Ketogenic diet and childhood neurological disorders other than epilepsy: an overview.
Pyruvate Dehydrogenase Complex Deficiency Disease
Ketogenic Diet: A New Light Shining on Old but Gold Biochemistry.
Pyruvate Dehydrogenase Complex Deficiency Disease
Ketogenic diet: an early option for epilepsy treatment, instead of a last choice only.
Pyruvate Dehydrogenase Complex Deficiency Disease
Ketone Bodies as a Possible Adjuvant to Ketogenic Diet in PDHc Deficiency but Not in GLUT1 Deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Ketonic diet in the management of pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Lactic acidosis and developmental delay due to deficiency of E3 binding protein (protein X) of the pyruvate dehydrogenase complex.
Pyruvate Dehydrogenase Complex Deficiency Disease
Lacticacidaemia due to pyruvate dehydrogenase deficiency, with evidence of protein polymorphism in the alpha-subunit of the enzyme.
Pyruvate Dehydrogenase Complex Deficiency Disease
Late-onset presentation of pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Leigh syndrome: pyruvate dehydrogenase defect. A case with peripheral neuropathy.
Pyruvate Dehydrogenase Complex Deficiency Disease
Leukoencephalopathy with cysts and hyperglycinemia may result from NFU1 deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
LIPT1 deficiency presenting as early infantile epileptic encephalopathy, Leigh disease, and secondary pyruvate dehydrogenase complex deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Massive parallel sequencing identifies RAPSN and PDHA1 mutations causing fetal akinesia deformation sequence.
Pyruvate Dehydrogenase Complex Deficiency Disease
Mechanisms of expression of pyruvate dehydrogenase deficiency caused by an E1alpha subunit mutation.
Pyruvate Dehydrogenase Complex Deficiency Disease
Metabolite studies in HIBCH and ECHS1 defects: Implications for screening.
Pyruvate Dehydrogenase Complex Deficiency Disease
Mitochondrial dysfunction in a patient with Joubert syndrome.
Pyruvate Dehydrogenase Complex Deficiency Disease
Mitochondrial respiratory chain disease discrimination by retrospective cohort analysis of blood metabolites.
Pyruvate Dehydrogenase Complex Deficiency Disease
Molecular genetic analysis of a female patient with pyruvate dehydrogenase deficiency: detection of a new mutation and differential expression of mutant gene product in cultured cells.
Pyruvate Dehydrogenase Complex Deficiency Disease
Morphological correlates of mitochondrial dysfunction in children.
Pyruvate Dehydrogenase Complex Deficiency Disease
Moving Metabolism to Make Inroads in a Model of Mitochondrial Epilepsy.
Pyruvate Dehydrogenase Complex Deficiency Disease
MRI features of 4 female patients with pyruvate dehydrogenase E1 alpha deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Mutation of E1 alpha gene in a female patient with pyruvate dehydrogenase deficiency due to rapid degradation of E1 protein.
Pyruvate Dehydrogenase Complex Deficiency Disease
Mutations in the gene for the E1beta subunit: a novel cause of pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Mutations in the X-linked pyruvate dehydrogenase (E1) alpha subunit gene (PDHA1) in patients with a pyruvate dehydrogenase complex deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Mutations of the E1beta subunit gene (PDHB) in four families with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Neonatal pyruvate dehydrogenase deficiency due to a R302H mutation in the PDHA1 gene: MRI findings.
Pyruvate Dehydrogenase Complex Deficiency Disease
Neonatal pyruvate dehydrogenase deficiency with lipoate responsive lactic acidaemia and hyperammonaemia.
Pyruvate Dehydrogenase Complex Deficiency Disease
Nonspecific mitochondrial disease with epilepsy in children: diagnostic approaches and epileptic phenotypes.
Pyruvate Dehydrogenase Complex Deficiency Disease
Optic neuropathy in a patient with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Orthopaedic Problems in 35 Patients With Organic Acid Disorders.
Pyruvate Dehydrogenase Complex Deficiency Disease
Outcome of pyruvate dehydrogenase deficiency treated with ketogenic diets. Studies in patients with identical mutations.
Pyruvate Dehydrogenase Complex Deficiency Disease
Outcome of thiamine treatment in a child with Leigh disease due to thiamine-responsive pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Partial complementation of pyruvate dehydrogenase deficiency by independently expressed lipoyl and catalytic domains of the dihydrolipoamide acetyltransferase component.
Pyruvate Dehydrogenase Complex Deficiency Disease
Phenotypic and Neuropathological Characterization of Fetal Pyruvate Dehydrogenase Deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Phenyl butyrate inhibits pyruvate dehydrogenase kinase 1 and contributes to its anti-cancer effect.
Pyruvate Dehydrogenase Complex Deficiency Disease
Phenylbutyrate increases pyruvate dehydrogenase complex activity in cells harboring a variety of defects.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pitfalls of relying on genetic testing only to diagnose inherited metabolic disorders in non-western populations - 5 cases of pyruvate dehydrogenase deficiency from South Africa.
Pyruvate Dehydrogenase Complex Deficiency Disease
Potential role of stress-induced gluconeogenesis in disease aggravation and mortality in pyruvate dehydrogenase deficiency: A case-based hypothesis.
Pyruvate Dehydrogenase Complex Deficiency Disease
Prenatal diagnosis of pyruvate dehydrogenase deficiency using magnetic resonance imaging.
Pyruvate Dehydrogenase Complex Deficiency Disease
Prenatal sonographic description of fetuses affected by pyruvate dehydrogenase or pyruvate carboxylase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase complex deficiency is linked to regulatory loop disorder in the ?V138M variant of human pyruvate dehydrogenase.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase complex deficiency with multiple minor anomalies.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency - morphological and metabolic effects, creation of animal model to study and research for treatment therapy.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency and epilepsy.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency and the brain.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency as a result of splice-site mutations in the PDX1 gene.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency caused by a 33 base pair duplication in the PDH E1 alpha subunit.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency caused by a 5 base pair duplication in the E1 alpha subunit.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency caused by a four-nucleotide insertion in the E1 alpha subunit gene.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency caused by a new mutation of PDHX gene in two Moroccan patients.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency caused by deletion of a 7-bp repeat sequence in the E1 alpha gene.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency disease detected by the enzyme activity of peripheral leukocytes.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency due to a 20-bp deletion in exon II of the pyruvate dehydrogenase (PDH) E1 alpha gene.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency due to a mutation of the E1 alpha subunit.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency in 35 patients.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency in a child responsive to thiamine treatment.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency in a female due to a 4 base pair deletion in exon 10 of the E1 alpha gene.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency in a male caused by a point mutation (F205L) in the E1 alpha subunit.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency in a Sussex spaniel.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency in spinocerebellar degenerations.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency presenting as dystonia in childhood.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency presenting as intermittent isolated acute ataxia.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency presenting as isolated paroxysmal exercise induced dystonia successfully reversed with thiamine supplementation. Case report and mini-review.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency restricted to brain.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency. Clinical presentation and molecular genetic characterization of five new patients.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency: clinical and biochemical diagnosis.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency: identification of a novel mutation in the PDHA1 gene which responds to amino acid supplementation.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency: molecular basis for intrafamilial heterogeneity.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase deficiency: the relation of the E1 alpha mutation to the E1 beta subunit deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase E2 deficiency: A potentially treatable cause of episodic dystonia.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase E3 binding protein deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate Dehydrogenase-E1? Deficiency Presenting as Recurrent Demyelination: An Unusual Presentation and a Novel Mutation.
Pyruvate Dehydrogenase Complex Deficiency Disease
Reconciling diabetes management and the ketogenic diet in a child with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Renal manifestations of primary mitochondrial disorders.
Pyruvate Dehydrogenase Complex Deficiency Disease
Sensitivity to carbohydrate in a patient with familial intermittent lactic acidosis and pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Sensory-motor polyneuropathy occurring in variant maple syrup urine disease.
Pyruvate Dehydrogenase Complex Deficiency Disease
Sequential deletion of C-terminal amino acids of the E(1)alpha component of the pyruvate dehydrogenase (PDH) complex leads to reduced steady-state levels of functional E(1)alpha(2)beta(2) tetramers: implications for patients with PDH deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Severe Brain Malformations in an Infant With Pyruvate Dehydrogenase Deficiency and Down Syndrome.
Pyruvate Dehydrogenase Complex Deficiency Disease
Severe Neonatal Presentation of Mitochondrial Citrate Carrier (SLC25A1) Deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Somatic mosaicism for a PDHA1 mutation in a female with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Studies of the pathophysiology of pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Summary of recommendations for the management of infantile seizures: Task Force Report for the ILAE Commission of Pediatrics.
Pyruvate Dehydrogenase Complex Deficiency Disease
Tenuous link between chronic fatigue syndrome and pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
The clinical spectrum of nodular heterotopias in children: Report of 31 patients.
Pyruvate Dehydrogenase Complex Deficiency Disease
The first case with FBXL4 mutation successfully treated with a parenteral ketogenic diet for lactic acidosis.
Pyruvate Dehydrogenase Complex Deficiency Disease
The genetic heterogeneity of lactic acidosis: occurrence of recognizable inborn errors of metabolism in pediatric population with lactic acidosis.
Pyruvate Dehydrogenase Complex Deficiency Disease
The human pyruvate dehydrogenase complex. Isolation of cDNA clones for the E1 alpha subunit, sequence analysis, and characterization of the mRNA.
Pyruvate Dehydrogenase Complex Deficiency Disease
The importance of cerebrospinal fluid lactate in the evaluation of congenital lactic acidosis.
Pyruvate Dehydrogenase Complex Deficiency Disease
The Importance of Gender-Related Anticancer Research on Mitochondrial Regulator Sodium Dichloroacetate in Preclinical Studies In Vivo.
Pyruvate Dehydrogenase Complex Deficiency Disease
The ketogenic diet: uses in epilepsy and other neurologic illnesses.
Pyruvate Dehydrogenase Complex Deficiency Disease
Therapeutic efficacy of a case of pyruvate dehydrogenase complex deficiency monitored by localized proton magnetic resonance spectroscopy.
Pyruvate Dehydrogenase Complex Deficiency Disease
Thiamine dependency in a patient with congenital lacticacidaemia due to pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Thiamine responsive pyruvate dehydrogenase deficiency in an adult with peripheral neuropathy and optic neuropathy.
Pyruvate Dehydrogenase Complex Deficiency Disease
Thiamine responsive pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Thiamine-responsive pyruvate dehydrogenase deficiency in two patients caused by a point mutation (F205L and L216F) within the thiamine pyrophosphate binding region.
Pyruvate Dehydrogenase Complex Deficiency Disease
Two Patients with Clinically Distinct Manifestation of Pyruvate Dehydrogenase Deficiency Due to Mutations in PDHA1 Gene.
Pyruvate Dehydrogenase Complex Deficiency Disease
Ultrastructural examination of skin biopsies may assist in diagnosing mitochondrial cytopathy when muscle biopsies yield negative results.
Pyruvate Dehydrogenase Complex Deficiency Disease
Ultrastructural examination of the axillary skin biopsy in the diagnosis of metabolic diseases.
Pyruvate Dehydrogenase Complex Deficiency Disease
Unilateral periventricular leukomalacia in association with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Urinary lactate excretion in normal children and in children with enzyme defects of carbohydrate metabolism.
Pyruvate Dehydrogenase Complex Deficiency Disease
Vitamin-Responsive Movement Disorders in Children.
Pyruvate Dehydrogenase Complex Deficiency Disease
Vitamin-responsive pyruvate dehydrogenase deficiency in a young girl with external ophthalmoplegia, myopathy and lactic acidosis.
Pyruvate Dehydrogenase Complex Deficiency Disease
X-chromosome localization of the functional gene for the E1 alpha subunit of the human pyruvate dehydrogenase complex.
Pyruvate Dehydrogenase Complex Deficiency Disease
Zonisamide ameliorates symptoms of secondary paroxysmal dystonia.
Pyruvate Dehydrogenase Complex Deficiency Disease
[A case of pyruvate dehydrogenase deficiency with low density areas in white matter noticed by CT scan]
Pyruvate Dehydrogenase Complex Deficiency Disease
[Inborn errors of metabolism in adult neurology].
Pyruvate Dehydrogenase Complex Deficiency Disease
[Lactic acid acidosis with mitochondrial myopathy due to a pyruvate dehydrogenase deficiency]
Pyruvate Dehydrogenase Complex Deficiency Disease
[Leigh's syndrome and mitochondrial myopathy]
Pyruvate Dehydrogenase Complex Deficiency Disease
[Pyruvate dehydrogenase deficiency and cerebral malformations]
Pyruvate Dehydrogenase Complex Deficiency Disease
[Pyruvate dehydrogenase deficiency in a child with persistent lactic acidosis]
Pyruvate Dehydrogenase Complex Deficiency Disease
[Pyruvate-dehydrogenase deficiency. Lethal course of the disease during infancy (author's transl)]
Pyruvate Dehydrogenase Complex Deficiency Disease
[Treatable hereditary neuro-metabolic diseases]
pyruvate dehydrogenase system deficiency
Diagnosis of partial deficiency of the pyruvate dehydrogenase complex in biopsied muscle.
pyruvate dehydrogenase system deficiency
Mutations in the X-linked pyruvate dehydrogenase (E1) alpha subunit gene (PDHA1) in patients with a pyruvate dehydrogenase complex deficiency.
Renal Insufficiency, Chronic
Chronic kidney disease reduces muscle mitochondria and exercise endurance and its exacerbation by dietary protein through inactivation of pyruvate dehydrogenase.
Reperfusion Injury
Neuroprotective effect of ethanol and Modafinil on focal cerebral ischemia in rats.
Respiratory Insufficiency
Continuous pH monitoring using the Paratrend 7 inserted into a peripheral vein in a patient with shock and congenital lactic acidosis.
Retinal Vein Occlusion
A pyruvate dehydrogenase kinase inhibitor prevents retinal cell death and improves energy metabolism in rat retinas after ischemia/reperfusion injury.
Retinopathy of Prematurity
A pyruvate dehydrogenase kinase inhibitor prevents retinal cell death and improves energy metabolism in rat retinas after ischemia/reperfusion injury.
Reye Syndrome
Deficient activity of hepatic pyruvate dehydrogenase and pyruvate carboxylase in Reye's syndrome.
Rhabdomyolysis
A metabolic switching hypothesis for the first step in the hypolipidemic effects of fibrates.
Scleroderma, Systemic
Autoantibodies to mitochondrial and centromere antigens in primary biliary cirrhosis and systemic sclerosis.
Scleroderma, Systemic
Detection of anti-mitochondrial antibodies by immunoprecipitation in patients with systemic sclerosis.
Seizures
Animal Models of Metabolic Epilepsy and Epilepsy Associated Metabolic Dysfunction: A Systematic Review.
Seizures
Blood-brain barrier, ion homeostatis and epilepsy: possible implications towards the understanding of ketogenic diet mechanisms.
Seizures
Case Report of Pyruvate Dehydrogenase Deficiency With Unusual Increase of Fats During Ketogenic Diet Treatment.
Seizures
Impaired hippocampal glucose metabolism during and after flurothyl-induced seizures in mice: Reduced phosphorylation coincides with reduced activity of pyruvate dehydrogenase.
Seizures
Ketogenic diet and childhood neurological disorders other than epilepsy: an overview.
Seizures
Ketone Bodies as a Possible Adjuvant to Ketogenic Diet in PDHc Deficiency but Not in GLUT1 Deficiency.
Sepsis
Dichloroacetate-induced metabolic reprogramming improves lifespan in a Drosophila model of surviving sepsis.
Sepsis
Glucose kinetics and pyruvate dehydrogenase activity in septic rats treated with dichloroacetate.
Sepsis
Immunocapture and microplate-based activity measurement of mammalian pyruvate dehydrogenase complex.
Sepsis
Metabolic effects of partial reversal of pyruvate dehydrogenase activity by dichloroacetate in sepsis.
Sepsis
Pharmacological reversal of abnormal glucose regulation, BCAA utilization, and muscle catabolism in sepsis by dichloroacetate.
Sepsis
Potentiation of decreased pyruvate dehydrogenase activity by inflammatory stimuli in sepsis.
Sepsis
Pyruvate Dehydrogenase Activity Is Decreased in the Peripheral Blood Mononuclear Cells of Patients with Sepsis. A Prospective Observational Trial.
Sepsis
Pyruvate dehydrogenase inactivity is not responsible for sepsis-induced insulin resistance.
Sepsis
Regulation of glucose metabolism by altered pyruvate dehydrogenase activity. I. Potential site of insulin resistance in sepsis.
Sepsis
Role of insulin and glucose oxidation in mediating the protein catabolism of burns and sepsis.
Small Cell Lung Carcinoma
Pyruvate dehydrogenase and pyruvate dehydrogenase kinase expression in non small cell lung cancer and tumor-associated stroma.
Spasms, Infantile
Pyruvate dehydrogenase E1alpha subunit deficiency in a female patient: evidence of antenatal origin of brain damage and possible etiology of infantile spasms.
Spinocerebellar Degenerations
Glutamate and pyruvate dehydrogenase deficiency in spinocerebellar degeneration.
Spinocerebellar Degenerations
Pyruvate dehydrogenase deficiency in spinocerebellar degenerations.
Squamous Cell Carcinoma of Head and Neck
HPV-associated differential regulation of tumor metabolism in oropharyngeal head and neck cancer.
Squamous Cell Carcinoma of Head and Neck
Metformin increases PDH and suppresses HIF-1? under hypoxic conditions and induces cell death in oral squamous cell carcinoma.
Starvation
Active and inactive forms of pyruvate dehydrogenase in rat liver. Effect of starvation and refeeding and of insulin treatment on pyruvate-dehydrogenase interconversion.
Starvation
Characterization of the isozymes of pyruvate dehydrogenase phosphatase: implications for the regulation of pyruvate dehydrogenase activity.
Starvation
Cyclic AMP and free fatty acids in the longer-term regulation of pyruvate dehydrogenase kinase in rat soleus muscle.
Starvation
Diversion of the metabolic flux from pyruvate dehydrogenase to pyruvate oxidase decreases oxidative stress during glucose metabolism in nongrowing Escherichia coli cells incubated under aerobic, phosphate starvation conditions.
Starvation
Effect of starvation and insulin in vivo on the activity of the pyruvate dehydrogenase complex in rat skeletal muscles.
Starvation
Effect of starvation on the local and systemic metabolic effects of the lambda-carrageenan wound.
Starvation
Effects of re-feeding after prolonged starvation on pyruvate dehydrogenase activities in heart, diaphragm and selected skeletal muscles of the rat.
Starvation
Effects of starvation, diabetes and 2-bromo-alpha-ergocryptine treatment on pyruvate dehydrogenase activity in lactating rat mammary gland.
Starvation
Glucose metabolism in perfused skeletal muscle. Pyruvate dehydrogenase activity in starvation, diabetes and exercise.
Starvation
Glucose utilization in heart, diaphragm and skeletal muscle during the fed-to-starved transition.
Starvation
Halothane anaesthesia can block insulin stimulation of pyruvate dehydrogenase activity in mammary glands of 24-hour starved lactating rats.
Starvation
Hepatic glycogen synthesis on carbohydrate re-feeding after starvation. A regulatory role for pyruvate dehydrogenase in liver and extrahepatic tissues.
Starvation
Hepatic pyruvate dehydrogenase kinase activities during the starved-to-fed transition.
Starvation
Immunocapture and microplate-based activity measurement of mammalian pyruvate dehydrogenase complex.
Starvation
Inactivation of pyruvate dehydrogenase complex in heart muscle mitochondria of gold-thioglucose-induced obese mice is not due to a stable increase in activity of pyruvate dehydrogenase kinase.
Starvation
Insulin reverses effects of starvation on the activity of pyruvate dehydrogenase kinase in cultured hepatocytes.
Starvation
Longer-term regulation of pyruvate dehydrogenase kinase in cultured rat cardiac myocytes.
Starvation
Modifications of citric acid cycle activity and gluconeogenesis in streptozotocin-induced diabetes and effects of metformin.
Starvation
Nutrient deprivation induces the Warburg effect through ROS/AMPK-dependent activation of pyruvate dehydrogenase kinase.
Starvation
Occupancy of sites of phosphorylation in inactive rat heart pyruvate dehydrogenase phosphate in vivo.
Starvation
Pyruvate dehydrogenase activities during the fed-to-starved transition and on re-feeding after acute or prolonged starvation.
Starvation
Pyruvate dehydrogenase kinase/activator in rat heart mitochondria, Assay, effect of starvation, and effect of protein-synthesis inhibitors of starvation.
Starvation
Regulation of fatty acid synthesis in lactating rat mammary gland in the fed to starved transition: asynchronous control of pyruvate dehydrogenase, phosphofructokinase and acetyl-CoA carboxylase.
Starvation
Regulation of pyruvate dehydrogenase and pyruvate dehydrogenase phosphate phosphatase activity in rat epididymal fat-pads. Effects of starvation, alloxan-diabetes and high-fat diet.
Starvation
Reversible phosphorylation of pyruvate dehydrogenase in rat skeletal-muscle mitochondria. Effects of starvation and diabetes.
Starvation
Studies on the interactions of Ca2+ and pyruvate in the regulation of rat heart pyruvate dehydrogenase activity. Effects of starvation and diabetes.
Starvation
The mode of regulation of pyruvate dehydrogenase of lactating rat mammary gland. Effects of starvation and insulin.
Starvation
Time courses of the responses of pyruvate dehydrogenase activities to short-term starvation in diaphragm and selected skeletal muscles of the rat.
Stomach Neoplasms
Dichloroacetate attenuates hypoxia-induced resistance to 5-fluorouracil in gastric cancer through the regulation of glucose metabolism.
Stomach Neoplasms
Expression of pyruvate dehydrogenase is an independent prognostic marker in gastric cancer.
Stomach Neoplasms
Expression of pyruvate dehydrogenase kinase-1 in gastric cancer as a potential therapeutic target.
Stomach Neoplasms
Phosphorylated AMP-activated protein kinase expression associated with prognosis for patients with gastric cancer treated with cisplatin-based adjuvant chemotherapy.
Stroke
Inhibition of prolyl hydroxylases by dimethyloxaloylglycine after stroke reduces ischemic brain injury and requires hypoxia inducible factor-1?.
Stroke
[Influence of heart work and substrate uptake on the regulation of pyruvate dehydrogenase activity in isolated guinea pig hearts (author's transl)]
Subarachnoid Hemorrhage
First Description of Reduced Pyruvate Dehydrogenase Enzyme Activity Following Subarachnoid Hemorrhage (SAH).
succinate-semialdehyde dehydrogenase [nad(p)+] deficiency
Urinary organic acids in succinic semialdehyde dehydrogenase deficiency: evidence of alpha-oxidation of 4-hydroxybutyric acid, interaction of succinic semialdehyde with pyruvate dehydrogenase and possible secondary inhibition of mitochondrial beta-oxidation.
Synostosis
Agenesis of the Corpus Callosum and Skeletal Deformities in Two Unrelated Patients: Analysis via MRI and Radiography.
Thiamine Deficiency
Acetyl-CoA deficit in brain mitochondria in experimental thiamine deficiency encephalopathy.
Thiamine Deficiency
Activities of thiamine-dependent enzymes in two experimental models of thiamine-deficiency encephalopathy: 1. The pyruvate dehydrogenase complex.
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
Clinical evaluation and biochemical analyses of thiamine deficiency in Pacific harbor seals (Phoca vitulina) maintained at a zoological facility.
Thiamine Deficiency
Disturbances of acetyl-CoA, energy and acetylcholine metabolism in some encephalopathies.
Thiamine Deficiency
Matched Retrospective Cohort Study of Thiamine to Treat Persistent Hyperlactatemia in Pediatric Septic Shock.
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
[Changes in the activity of pyruvate dehydrogenase in thiamine deficiency]
Thiamine Deficiency
[Coenzyme-dependent induction of pyruvate dehydrogenase in thiamine deficiency]
Thiamine Deficiency
[Importance of water-soluble vitamins as regulatory factors of genetic expression]
Thiamine Deficiency
[Regulation of pyruvate dehydrogenase activity and dynamics of the isolated perfused guinea pig heart in thiamine deficiency]
Thrombosis
Dichloroacetate, an inhibitor of pyruvate dehydrogenase kinases, inhibits platelet aggregation and arterial thrombosis.
Toxoplasmosis
Dichloroacetate and Pyruvate Metabolism: Pyruvate Dehydrogenase Kinases as Targets Worth Investigating for Effective Therapy of Toxoplasmosis.
transaldolase deficiency
Antenatal manifestations of inborn errors of metabolism: autopsy findings suggestive of a metabolic disorder.
Tuberculosis
Dihydrolipoamide dehydrogenase-Lpd (Rv0462)-specific T cell recall responses are higher in healthy household contacts of TB: a novel immunodominant antigen from M. tuberculosis.
Tuberculosis
Mycobacterium tuberculosis appears to lack alpha-ketoglutarate dehydrogenase and encodes pyruvate dehydrogenase in widely separated genes.
Unverricht-Lundborg Syndrome
Ultrastructural examination of the axillary skin biopsy in the diagnosis of metabolic diseases.
Ventricular Dysfunction
Effects of L-propionylcarnitine on ischemia-induced myocardial dysfunction in men with angina pectoris.
[pyruvate dehydrogenase (acetyl-transferring)]-phosphatase deficiency
A family with pyruvate dehydrogenase complex deficiency due to a novel C>T substitution at nucleotide position 407 in exon 4 of the X-linked Epsilon1alpha gene.
[pyruvate dehydrogenase (acetyl-transferring)]-phosphatase deficiency
A mutation in the E1 alpha subunit of pyruvate dehydrogenase associated with variable expression of pyruvate dehydrogenase complex deficiency.
[pyruvate dehydrogenase (acetyl-transferring)]-phosphatase deficiency
Mutations in the X-linked pyruvate dehydrogenase (E1) alpha subunit gene (PDHA1) in patients with a pyruvate dehydrogenase complex deficiency.
[pyruvate dehydrogenase (acetyl-transferring)]-phosphatase deficiency
Pyruvate dehydrogenase complex deficiency is linked to regulatory loop disorder in the ?V138M variant of human pyruvate dehydrogenase.
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