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Information on EC 1.2.4.1 - pyruvate dehydrogenase (acetyl-transferring)
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1.2.4.1 pyruvate dehydrogenase (acetyl-transferring)IUBMB Comments
Contains thiamine diphosphate. It is a component (in multiple copies) of the multienzyme pyruvate dehydrogenase complex, EC 1.2.1.104, in which it is bound to a core of molecules of EC 2.3.1.12, dihydrolipoyllysine-residue acetyltransferase, which also binds multiple copies of EC 1.8.1.4, dihydrolipoyl dehydrogenase. It does not act on free lipoamide or lipoyllysine, but only on the lipoyllysine residue in EC 2.3.1.12.
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Word Map
- 1.2.4.1
- acetyl-coa
- dichloroacetate
- cardiac
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tricarboxylic
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lipoylation
- glycogen
- tca
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pituitary-dependent
- acidosis
- hyperadrenocorticism
- dihydrolipoamide
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x-linked
- lipoamide
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warburg
- stearothermophilus
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lipoic
- acth
- cortisol
- lipoate
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hyperpolarized
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crustacean
- histoplasmosis
- leigh
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pigment-dispersing
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vastus
- hypercortisolism
-
ketogenic
- phosphocreatine
- transacetylase
-
maple
- histoplasma
- trilostane
- pyranose
-
anaplerosis
- capsulatum
-
hernia
-
alpha2beta2
- prephenate
- acetylcarnitine
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antimitochondrial
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interbody
- agriculture
- medicine
- biotechnology
- synthesis
The enzyme appears in viruses and cellular organisms
Reaction Schemes
Synonyms
pdh, pdha1, mitochondrial pyruvate dehydrogenase, e1alpha, pyruvate dehydrogenase multienzyme complex, pyruvate dehydrogenase e1, pdhc-e1, mtpdc, pdhc e1, pyruvate dehydrogenase e1 component, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
thiamin-dependent pyruvate dehydrogenase
part of the pyruvate dehydrogenase multienzyme complex PDHc as component E1
dehydrogenase, pyruvate
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MtPDC
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PDH
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pyruvate decarboxylase
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pyruvate dehydrogenase
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pyruvate dehydrogenase complex
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pyruvic acid dehydrogenase
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pyruvic dehydrogenase
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VEG220
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Vegetative protein 220
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
redox reaction
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oxidation
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reduction
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PATHWAY SOURCE
PATHWAYS
KEGG
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SYSTEMATIC NAME
IUBMB Comments
pyruvate:[dihydrolipoyllysine-residue acetyltransferase]-lipoyllysine 2-oxidoreductase (decarboxylating, acceptor-acetylating)
Contains thiamine diphosphate. It is a component (in multiple copies) of the multienzyme pyruvate dehydrogenase complex, EC 1.2.1.104, in which it is bound to a core of molecules of EC 2.3.1.12, dihydrolipoyllysine-residue acetyltransferase, which also binds multiple copies of EC 1.8.1.4, dihydrolipoyl dehydrogenase. It does not act on free lipoamide or lipoyllysine, but only on the lipoyllysine residue in EC 2.3.1.12.
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CAS REGISTRY NUMBER
COMMENTARY
9014-20-4
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
3-hydroxyisobutyryl-coa hydrolase deficiency
Thiamine-Responsive and Non-responsive Patients with PDHC-E1 Deficiency: A Retrospective Assessment.
Acanthamoeba Keratitis
A DNA dot hybridization model for molecular diagnosis of parasitic keratitis.
Acidosis
Effect of induced metabolic acidosis on human skeletal muscle metabolism during exercise.
Acidosis, Lactic
A pathogenic glutamate-to-aspartate substitution (D296E) in the pyruvate dehydrogenase E1 subunit gene PDHA1.
Acidosis, Lactic
Biochemical characterization of two mutants of human pyruvate dehydrogenase, F205L and T231A of the E1alpha subunit.
Acidosis, Lactic
Caveats when considering ketogenic diets for the treatment of pyruvate dehydrogenase complex deficiency.
Acidosis, Lactic
Characterization of two cDNA clones for pyruvate dehydrogenase E1 beta subunit and its regulation in tricarboxylic acid cycle-deficient fibroblast.
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
Defective gene in lactic acidosis: abnormal pyruvate dehydrogenase E1 alpha-subunit caused by a frame shift.
Acidosis, Lactic
Deletion at chromosomal band Xp22.12-Xp22.13 involving PDHA1 in a patient with congenital lactic acidosis.
Acidosis, Lactic
Development of a less toxic dichloroacetate analogue by docking and descriptor analysis.
Acidosis, Lactic
Dichloroacetate stabilizes the mutant E1alpha subunit in pyruvate dehydrogenase deficiency.
Acidosis, Lactic
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
Acidosis, Lactic
Enzymatic testing sensitivity, variability and practical diagnostic algorithm for pyruvate dehydrogenase complex (PDC) deficiency.
Acidosis, Lactic
Hepatic pyruvate dehydrogenase activity in humans: effect of cirrhosis, transplantation, and dichloroacetate.
Acidosis, Lactic
Heterogeneous expression of protein and mRNA in pyruvate dehydrogenase deficiency.
Acidosis, Lactic
Mild Phenotype in a Male with Pyruvate Dehydrogenase Complex Deficiency Associated with Novel Hemizygous In-Frame Duplication of the E1? Subunit Gene (PDHA1).
Acidosis, Lactic
Mouse mutants carrying deletions that remove the genes mutated in Coffin-Lowry syndrome and lactic acidosis.
Acidosis, Lactic
Neurodevelopmental abnormalities and lactic acidosis in a girl with a 20-bp deletion in the X-linked pyruvate dehydrogenase E1 alpha subunit gene.
Acidosis, Lactic
Neuropathological findings of a patient with pyruvate dehydrogenase E1 alpha deficiency presenting as a cerebral lactic acidosis.
Acidosis, Lactic
Novel mutations in IBA57 are associated with leukodystrophy and variable clinical phenotypes.
Acidosis, Lactic
Novel presentations associated with a PDHA1 variant - Alternating hemiplegia in Hemizygote proband and Guillain Barre Syndrome in Heterozygote mother.
Acidosis, Lactic
Partial pyruvate decarboxylase deficiency with profound lactic acidosis and hyperammonemia: responses to dichloroacetate and benzoate.
Acidosis, Lactic
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.
Acidosis, Lactic
Pyruvate dehydrogenase complex deficiency is linked to regulatory loop disorder in the ?V138M variant of human pyruvate dehydrogenase.
Acidosis, Lactic
Recombinant adeno-associated virus vector-based gene transfer for defects in oxidative metabolism.
Acidosis, Lactic
Splicing error in E1alpha pyruvate dehydrogenase mRNA caused by novel intronic mutation responsible for lactic acidosis and mental retardation.
Acidosis, Lactic
Stabilization of the pyruvate dehydrogenase E1alpha subunit by dichloroacetate.
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
X-linked pyruvate dehydrogenase E1 alpha subunit deficiency in heterozygous females: variable manifestation of the same mutation.
Acquired Immunodeficiency Syndrome
Clinical review: progressive disseminated histoplasmosis in the AIDS patient.
Acquired Immunodeficiency Syndrome
Direct kinetic evidence for half-of-the-sites reactivity in the E1 component of the human pyruvate dehydrogenase multienzyme complex through alternating sites cofactor activation.
Acquired Immunodeficiency Syndrome
Disseminated histoplasmosis in AIDS. Clinicopathologic features in seven patients from a non-endemic area.
Acquired Immunodeficiency Syndrome
Histoplasma Antigen Clearance during Treatment of Histoplasmosis in Patients with AIDS Determined by a Quantitative Antigen Enzyme Immunoassay.
Acquired Immunodeficiency Syndrome
Histoplasma capsulatum fungemia in patients with acquired immunodeficiency syndrome: detection by lysis-centrifugation blood-culturing technique.
Acquired Immunodeficiency Syndrome
Histoplasmosis among human immunodeficiency virus-infected people in europe: report of 4 cases and review of the literature.
Acquired Immunodeficiency Syndrome
Histoplasmosis in patients with acquired immunodeficiency syndrome. Hematologic and bone marrow manifestations.
Acquired Immunodeficiency Syndrome
Progressive disseminated histoplasmosis among human immunodeficiency virus-infected patients from West-Africa: report of four imported cases in Italy.
Acquired Immunodeficiency Syndrome
Progressive disseminated histoplasmosis in the acquired immunodeficiency syndrome: a model for disseminated disease.
ACTH-Secreting Pituitary Adenoma
Ki-67 and minichromosome maintenance-7 (MCM7) expression in canine pituitary corticotroph adenomas.
Adenoma
Dynamic computed tomography of the pituitary gland in dogs with pituitary-dependent hyperadrenocorticism.
Adrenocortical Hyperfunction
Alterations in anterior pituitary function of dogs with pituitary-dependent hyperadrenocorticism.
Adrenocortical Hyperfunction
Comparison of Survival Times for Dogs with Pituitary-Dependent Hyperadrenocorticism in a Primary-Care Hospital: Treated with Trilostane versus Untreated.
Adrenocortical Hyperfunction
Effect of trilostane on serum concentrations of aldosterone, cortisol, and potassium in dogs with pituitary-dependent hyperadrenocorticism.
Adrenocortical Hyperfunction
Feline pituitary-dependent hyperadrenocorticism and insulin resistance due to a plurihormonal adenoma.
Adrenocortical Hyperfunction
Plasma cortisol response to ketoconazole administration in dogs with hyperadrenocorticism.
Adrenocortical Hyperfunction
Regulation of prolactin secretion in canine pituitary-dependent hyperadrenocorticism.
Adrenocortical Hyperfunction
Results of cyproheptadine treatment in dogs with pituitary-dependent hyperadrenocorticism.
Adrenocortical Hyperfunction
Serum cholecystokinin concentrations in dogs with naturally acquired pituitary-dependent hyperadrenocorticism.
Adrenocortical Hyperfunction
Systemic arterial blood pressure and urine protein/creatinine ratio in dogs with hyperadrenocorticism.
Adrenocortical Hyperfunction
Urinary corticoid/creatinine ratios in the differentiation between pituitary-dependent hyperadrenocorticism and hyperadrenocorticism due to adrenocortical tumour in the dog.
Albuminuria
Association of Renal Resistive Index, Renal Pulsatility Index, Systemic Hypertension, and Albuminuria with Survival in Dogs with Pituitary-Dependent Hyperadrenocorticism.
Alexander Disease
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
Alkalosis
Effect of induced metabolic alkalosis on human skeletal muscle metabolism during exercise.
Alkalosis, Respiratory
Na(+), K(+)-ATPase content in skeletal muscle of dogs with pituitary-dependent hyperadrenocorticism.
Alzheimer Disease
An immunochemical study of the pyruvate dehydrogenase deficit in Alzheimer's disease brain.
Alzheimer Disease
Beta-amyloid inhibits integrated mitochondrial respiration and key enzyme activities.
Alzheimer Disease
Immunocapture and microplate-based activity measurement of mammalian pyruvate dehydrogenase complex.
Alzheimer Disease
Pyruvate Carboxylase and Pentose Phosphate Fluxes are Reduced in A?PP-PS1 Mouse Model of Alzheimer's Disease: A 13C NMR Study.
Anthrax
Structural and biochemical analysis of Bacillus anthracis prephenate dehydrogenase reveals an unusual mode of inhibition by tyrosine via the ACT domain.
Asthma
Changes over time in the prevalence of asthma, rhinitis and atopic eczema in adolescents from Taubaté, São Paulo, Brazil (2005-2012): Relationship with living near a heavily travelled highway.
Ataxia
Genetic and clinical features of Chinese patients with mitochondrial ataxia identified by targeted next-generation sequencing.
Ataxia
Mutations of the E1beta subunit gene (PDHB) in four families with pyruvate dehydrogenase deficiency.
Ataxia
Novel presentations associated with a PDHA1 variant - Alternating hemiplegia in Hemizygote proband and Guillain Barre Syndrome in Heterozygote mother.
Autoimmune Diseases
Mitochondrial p32/C1qbp Is a Critical Regulator of Dendritic Cell Metabolism and Maturation.
Bacterial Infections
Evaluation of a Histoplasma antigen lateral flow assay for the rapid diagnosis of progressive disseminated histoplasmosis in Colombian patients with AIDS.
Blindness
Blindness in dogs with pituitary dependent hyperadrenocorticism: Relationship with glucose, cortisol and triglyceride concentration and with ophthalmic blood flow.
Blindness
Interleukin-6 and insulin incrase and nitric oxide and adiponectin decrease in blind dogs with pituitary-dependent hyperadrenocorticism.
Blindness
Structural Insights into the Drosophila melanogaster Retinol Dehydrogenase, a Member of the Short-Chain Dehydrogenase/Reductase Family.
Brain Diseases
Analysis of pyruvate dehydrogenase expression in embryonic mouse brain: localization and developmental regulation.
Brain Diseases
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
Brain Diseases
Novel presentations associated with a PDHA1 variant - Alternating hemiplegia in Hemizygote proband and Guillain Barre Syndrome in Heterozygote mother.
Brain Diseases
The SR protein SC35 is responsible for aberrant splicing of the E1alpha pyruvate dehydrogenase mRNA in a case of mental retardation with lactic acidosis.
Brain Edema
Relation of postischemic delayed hypoperfusion and cerebral edema after transient forebrain ischemia.
Brain Injuries
Activation of Pyruvate Dehydrogenase Activity Bydichloroacetate Improves Survival and Neurologic Outcomes After Cardiac Arrest in Rats.
Brain Injuries
Role of pyruvate dehydrogenase complex in traumatic brain injury and Measurement of pyruvate dehydrogenase enzyme by dipstick test.
Brain Ischemia
Effect of dichloroacetate on regional energy metabolites and pyruvate dehydrogenase activity during ischemia and reperfusion in gerbil brain.
Brain Ischemia
[Cerebral hemodynamics of post-ischemic delayed hypoperfusion (PDH) and the effects of nicardipine on the PDH]
Brain Ischemia
[Effect of duration of cerebral ischemia on pyruvate dehydrogenase activity (PDH) and metabolites in the gerbil brain]
Brain Ischemia
[Studies on brain pyruvate dehydrogenase (PDH) activity and energy metabolites during ischemia and reperfusion]
Brain Ischemia
[The effects of post-ischemic delayed hypoperfusion on the process of recovery of brain function]
Breast Neoplasms
Altered regulation of PDK4 expression promotes antiestrogen resistance in human breast cancer cells.
Breast Neoplasms
Estrogen modulates metabolic pathway adaptation to available glucose in breast cancer cells.
Breast Neoplasms
Prolyl-hydroxylase PHD3 interacts with pyruvate dehydrogenase (PDH)-E1? and regulates the cellular PDH activity.
Breast Neoplasms
Pyruvate Dehydrogenase PDH-E1? Controls Tumor Progression by Altering the Metabolic Status of Cancer Cells.
Breast Neoplasms
The novel function of tumor protein D54 in regulating pyruvate dehydrogenase and metformin cytotoxicity in breast cancer.
Breast Neoplasms
The oncoprotein HBXIP promotes glucose metabolism reprogramming via downregulating SCO2 and PDHA1 in breast cancer.
Canavan Disease
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
Carcinoma
Decreased expression of pyruvate dehydrogenase A1 predicts an unfavorable prognosis in ovarian carcinoma.
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
Pyruvate dehydrogenase expression is negatively associated with cell stemness and worse clinical outcome in prostate cancers.
Carcinoma, Hepatocellular
Active pyruvate dehydrogenase and impaired gluconeogenesis in orthotopic hepatomas of rats.
Carcinoma, Hepatocellular
Insulin induces phosphorylation of pyruvate dehydrogenase through RhoA activation pathway in HepG2 cells.
Carcinoma, Hepatocellular
Insulin mediator stimulates pyruvate dehydrogenase of intact liver mitochondria.
Carcinoma, Hepatocellular
Lactic acidosis caused by repressed lactate dehydrogenase subunit B expression down-regulates mitochondrial oxidative phosphorylation via the pyruvate dehydrogenase (PDH)-PDH kinase axis.
Carcinoma, Hepatocellular
Mitochondrial free fatty acid ?-oxidation supports oxidative phosphorylation and proliferation in cancer cells.
Carcinoma, Hepatocellular
Overexpression of Pyruvate dehydrogenase E1a subunit Inhibits Warburg effect and Induces Cell Apoptosis through Mitochondria-mediated Pathway in Hepatocellular Carcinoma.
Carcinoma, Hepatocellular
Phosphorylation of the pyruvate dehydrogenase complex precedes HIF-1-mediated effects and pyruvate dehydrogenase kinase 1 upregulation during the first hours of hypoxic treatment in hepatocellular carcinoma cells.
Carcinoma, Hepatocellular
The HGF-MET axis coordinates liver cancer metabolism and autophagy for chemotherapeutic resistance.
Carcinoma, Non-Small-Cell Lung
Drug evaluation based on phosphomimetic PDHA1 reveals the complexity of activity-related cell death in A549 non-small cell lung cancer cells.
Carcinoma, Squamous Cell
Determination of Pyruvate Metabolic Fates Modulates Head and Neck Tumorigenesis.
Cardiomegaly
Comparative Proteome Profiling during Cardiac Hypertrophy and Myocardial Infarction Reveals Altered Glucose Oxidation by Differential Activation of Pyruvate Dehydrogenase E1 Component Subunit ?
Cardiotoxicity
Doxorubicin triggers bioenergetic failure and p53 activation in mouse stem cell-derived cardiomyocytes.
Cataract
Exclusion of three candidate genes, Grpr, Cxn33, and Pdha1, for the X-linked cataract gene on the distal region of the mouse chromosome X.
Cerebral Palsy
Cerebral palsy and pyruvate dehydrogenase deficiency: identification of two new mutations in the E1alpha gene.
Cholangiocarcinoma
PGC1? promotes cholangiocarcinoma metastasis by upregulating PDHA1 and MPC1 expression to reverse the Warburg effect.
Cholangitis
Identification of a xenobiotic as a potential environmental trigger in primary biliary cholangitis.
Cobblestone Lissencephaly
The Fetus with Ganglionic Eminence Abnormality: Head Size and Extracranial Sonographic Findings Predict Genetic Diagnoses and Postnatal Outcomes.
Coffin-Lowry Syndrome
Mouse mutants carrying deletions that remove the genes mutated in Coffin-Lowry syndrome and lactic acidosis.
Coinfection
Clinical and Laboratory Profile of Persons Living with Human Immunodeficiency Virus/Acquired Immune Deficiency Syndrome and Histoplasmosis from a Colombian Hospital.
Coinfection
Diagnostic accuracy cohort study and clinical value of the Histoplasma urine antigen (ALPHA Histoplasma EIA) for disseminated histoplasmosis among HIV infected patients: A multicenter study.
Colorectal Neoplasms
Mitochondrial dysfunction induces radioresistance in colorectal cancer by activating [Ca2+]m-PDP1-PDH-histone acetylation retrograde signaling.
Congenital Abnormalities
[Joint effects of nano-selenium and nano-cerium on the male reproductive function of mice exposed to microwave radiation].
Cushing Syndrome
Computed tomography and low-field magnetic resonance imaging of the pituitary gland in dogs with pituitary-dependent hyperadrenocorticism: 11 cases (2001-2003).
Cushing Syndrome
Evaluation of cortisol precursors for the diagnosis of pituitary-dependent hypercortisolism in dogs.
Cushing Syndrome
Feline plasma adrenocorticotropic hormone: validation of a chemiluminescent assay and concentrations in cats with hypercortisolism, primary hypoadrenocorticism and other diseases.
Cushing Syndrome
Regulation of prolactin secretion in canine pituitary-dependent hyperadrenocorticism.
Cysts
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
Dehydration
Biochemical response of hybrid black poplar tissue culture (Populus × canadensis) on water stress.
Dehydration
Reciprocal regulation of delta 1-pyrroline-5-carboxylate synthetase and proline dehydrogenase genes controls proline levels during and after osmotic stress in plants.
Diabetes Mellitus
Conditional knockout of pyruvate dehydrogenase in mouse pancreatic ??cells causes morphological and functional changes.
Diabetes Mellitus
Involvement of glucagon-like peptide 1 in the glucose homeostasis regulation in obese and pituitary-dependent hyperadrenocorticism affected dogs.
Diabetes Mellitus, Type 1
In vivo assessment of pyruvate dehydrogenase flux in the heart using hyperpolarized carbon-13 magnetic resonance.
Diabetes Mellitus, Type 1
The role of taurine in the pathogenesis of the cardiomyopathy of 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
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
Hyperglycemia normalizes insulin-stimulated skeletal muscle glucose oxidation and storage in noninsulin-dependent diabetes mellitus.
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
Lactate production and pyruvate dehydrogenase activity in fat and skeletal muscle from diabetic rats.
Diabetes Mellitus, Type 2
Molecular effects of sulphonylurea agents in circulating lymphocytes of patients with non-insulin-dependent diabetes mellitus.
Diabetes Mellitus, Type 2
Regulation of Muscle Pyruvate Dehydrogenase Complex in Insulin Resistance: Effects of Exercise and Dichloroacetate.
Diabetes Mellitus, Type 2
Sensitivity of pyruvate dehydrogenase to insulin in activated T lymphocytes. Lack of responsiveness to insulin in patients with polycystic ovarian disease and diabetes.
Diabetes Mellitus, Type 2
The role of taurine in the pathogenesis of the cardiomyopathy of insulin-dependent diabetes mellitus.
Diabetic Cardiomyopathies
FoxO1 inhibition alleviates type 2 diabetes-related diastolic dysfunction by increasing myocardial pyruvate dehydrogenase activity.
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.
Dyskinesias
The Fetus with Ganglionic Eminence Abnormality: Head Size and Extracranial Sonographic Findings Predict Genetic Diagnoses and Postnatal Outcomes.
Dyslexia
An Extension of the Procedural Deficit Hypothesis from Developmental Language Disorders to Mathematical Disability.
Dystonia
Pyruvate dehydrogenase deficiency presenting as intermittent isolated acute ataxia.
Ear Diseases
The World Health Organisation and the prevention of deafness and hearing impairment caused by noise.
Eczema
Changes over time in the prevalence of asthma, rhinitis and atopic eczema in adolescents from Taubaté, São Paulo, Brazil (2005-2012): Relationship with living near a heavily travelled highway.
Endarteritis
Conditions associated with relapse of amphotericin B-treated disseminated histoplasmosis.
Endocarditis
Conditions associated with relapse of amphotericin B-treated disseminated histoplasmosis.
Endometrial Hyperplasia
Differential Expression Patterns of Glycolytic Enzymes and Mitochondria-Dependent Apoptosis in PCOS Patients with Endometrial Hyperplasia, an Early Hallmark of Endometrial Cancer, In Vivo and the Impact of Metformin In Vitro.
Epilepsy
A Warburg-like metabolic program coordinates Wnt, AMPK, and mTOR signaling pathways in epileptogenesis.
Epilepsy
Brain metabolism modulates neuronal excitability in a mouse model of pyruvate dehydrogenase deficiency.
Esophageal Neoplasms
PDHA1 Gene Knockout In Human Esophageal Squamous Cancer Cells Resulted In Greater Warburg Effect And Aggressive Features In Vitro And In Vivo.
Gallbladder Diseases
Clinical Relationship between Cholestatic Disease and Pituitary-Dependent Hyperadrenocorticism in Dogs: A Retrospective Case Series.
Glioblastoma
Mitochondria-Translocated PGK1 Functions as a Protein Kinase to Coordinate Glycolysis and the TCA Cycle in Tumorigenesis.
Glioma
Metabolic response of glioma to dichloroacetate measured in vivo by hyperpolarized 13C magnetic resonance spectroscopic imaging.
Glucose Intolerance
"In vitro" effects of insulin on the PDH complex of the isolated perfused heart of rats fed a sucrose-rich diet.
Glucose Intolerance
Exercise-induced Pyruvate Dehydrogenase Activation Is Not Affected by Seven Days of Bed Rest.
Glucose Intolerance
Regulation of hepatic pyruvate dehydrogenase phosphorylation in offspring glucose intolerance induced by intrauterine hyperglycemia.
Glucose Metabolism Disorders
Metabolic disorder induces fatty liver in Japanese seabass, Lateolabrax japonicas fed a full plant protein diet and regulated by cAMP-JNK/NF-kB-caspase signal pathway.
Guillain-Barre Syndrome
Intermittent peripheral weakness as the presenting feature of pyruvate dehydrogenase deficiency.
Guillain-Barre Syndrome
Novel presentations associated with a PDHA1 variant - Alternating hemiplegia in Hemizygote proband and Guillain Barre Syndrome in Heterozygote mother.
Head Injuries, Closed
Role of pyruvate dehydrogenase complex in traumatic brain injury and Measurement of pyruvate dehydrogenase enzyme by dipstick test.
Hearing Loss, Noise-Induced
The World Health Organisation and the prevention of deafness and hearing impairment caused by noise.
Heart Arrest
Activation of Pyruvate Dehydrogenase Activity Bydichloroacetate Improves Survival and Neurologic Outcomes After Cardiac Arrest in Rats.
Heart Diseases
Validation of the in vivo assessment of pyruvate dehydrogenase activity using hyperpolarised (13)C MRS.
Heart Failure
The effects of hypertrophy and diabetes on cardiac pyruvate dehydrogenase activity.
Heart Failure
The metabolic modulators, Etomoxir and NVP-LAB121, fail to reverse pressure overload induced heart failure in vivo.
Heart Failure, Systolic
Adaptations in Protein Expression and Regulated Activity of Pyruvate Dehydrogenase Multienzyme Complex in Human Systolic Heart Failure.
Hemiplegia
Novel presentations associated with a PDHA1 variant - Alternating hemiplegia in Hemizygote proband and Guillain Barre Syndrome in Heterozygote mother.
Hepatitis
Antibody binding and inhibition of pyruvate dehydrogenase (PDH) in sera from patients with primary biliary cirrhosis.
Hepatitis, Chronic
Antibody binding and inhibition of pyruvate dehydrogenase (PDH) in sera from patients with primary biliary cirrhosis.
Hepatolenticular Degeneration
Cu2+ toxicity inhibition of mitochondrial dehydrogenases in vitro and in vivo.
Hepatomegaly
Clinical and Laboratory Profile of Persons Living with Human Immunodeficiency Virus/Acquired Immune Deficiency Syndrome and Histoplasmosis from a Colombian Hospital.
Histoplasmosis
Clinical and Laboratory Profile of Persons Living with Human Immunodeficiency Virus/Acquired Immune Deficiency Syndrome and Histoplasmosis from a Colombian Hospital.
Histoplasmosis
Disseminated Histoplasmosis as AIDS-presentation. Case Report and Comprehensive Review of Current Literature.
Histoplasmosis
Disseminated histoplasmosis in patients infected with human immunodeficiency virus.
Histoplasmosis
Histoplasma capsulatum 100-kilodalton antigen: recombinant production, characterization, and evaluation of its possible application in the diagnosis of histoplasmosis.
HIV Infections
Progressive disseminated histoplasmosis in children living with HIV: a case series study.
HIV Infections
Progressive disseminated histoplasmosis in the acquired immunodeficiency syndrome: a model for disseminated disease.
Huntington Disease
Histone Deacetylase Inhibitors Protect Against Pyruvate Dehydrogenase Dysfunction in Huntington's Disease.
Hyperalgesia
Regulation of mitochondrial function by Epac2 contributes to acute inflammatory hyperalgesia.
Hyperglycemia
Effect of hyperglycemia on pyruvate dehydrogenase activity and energy metabolites during ischemia and reperfusion in gerbil brain.
Hyperglycemia
Hyperglycemia normalizes insulin-stimulated skeletal muscle glucose oxidation and storage in noninsulin-dependent diabetes mellitus.
Hyperglycemia
Mitochondrial Uncoupling Coordinated With PDH Activation Safely Ameliorates Hyperglycemia Via Promoting Glucose Oxidation.
Hyperglycemia
Pyruvate dehydrogenase activity is decreased in emergency department patients with diabetic ketoacidosis.
Hyperglycemia
Regulation of hepatic pyruvate dehydrogenase phosphorylation in offspring glucose intolerance induced by intrauterine hyperglycemia.
Hyperinsulinism
Effects of insulin infusion on human skeletal muscle pyruvate dehydrogenase, phosphofructokinase, and glycogen synthase. Evidence for their role in oxidative and nonoxidative glucose metabolism.
Hyperinsulinism
Interaction between glucose and free fatty acid metabolism in human skeletal muscle.
Hyperinsulinism
Longitudinal study of tissue- and subunit-specific obesity-induced regulation of the pyruvate dehydrogenase complex.
Hyperkalemia
Na(+), K(+)-ATPase content in skeletal muscle of dogs with pituitary-dependent hyperadrenocorticism.
Hyperlactatemia
TNF binding protein prevents hyperlactatemia and inactivation of PDH complex in skeletal muscle during sepsis.
Hyperlipidemias
Hepatic HAX-1 inactivation prevents metabolic diseases by enhancing mitochondrial activity and bile salt export.
Hyperlipidemias
Regulation of PDK mRNA by high fatty acid and glucose in pancreatic islets.
Hypersensitivity
Association of pyrazolone drug hypersensitivity with HLA-DQ and DR antigens.
Hypersensitivity
Changes over time in the prevalence of asthma, rhinitis and atopic eczema in adolescents from Taubaté, São Paulo, Brazil (2005-2012): Relationship with living near a heavily travelled highway.
Hypersensitivity
Diagnosis of pyrazolone drug sensitivity: clinical history versus skin testing and in vitro testing.
Hypersensitivity
Picumast dihydrochloride (Auteral), a new anti-allergic inhibitor of mediator release and action.
Hypersensitivity
Toxicity of free proline revealed in an arabidopsis T-DNA-tagged mutant deficient in proline dehydrogenase.
Hypertension
Association of Renal Resistive Index, Renal Pulsatility Index, Systemic Hypertension, and Albuminuria with Survival in Dogs with Pituitary-Dependent Hyperadrenocorticism.
Hypertension
Investigation of the role of aldosterone in hypertension associated with spontaneous pituitary-dependent hyperadrenocorticism in dogs.
Hypertension
Postoperative causes of death in pediatric surgery: analysis and conclusions for the therapy.
Hypertension
Systemic arterial blood pressure and urine protein/creatinine ratio in dogs with hyperadrenocorticism.
Hyperthyroidism
Regulation of renal and hepatic pyruvate dehydrogenase complex on carbohydrate re-feeding after starvation. Possible mechanisms and a regulatory role for thyroid hormone.
Hypoglycemia
Mendeliome sequencing enables differential diagnosis and treatment of neonatal lactic acidosis.
Hypokinesia
[Changes in the mitochondrial oxidative enzyme activity in the skeletal muscles od rats during the recovery period after hypokinesia of varying duration]
Hypophosphatemia, Familial
Urinary excretion of calcium and phosphate in dogs with pituitary-dependent hypercortisolism: case control study in 499 dogs.
Infections
AAV3-mediated transfer and expression of the pyruvate dehydrogenase E1 alpha subunit gene causes metabolic remodeling and apoptosis of human liver cancer cells.
Infections
Defective gene in lactic acidosis: abnormal pyruvate dehydrogenase E1 alpha-subunit caused by a frame shift.
Infections
Development and evaluation of an enzyme-linked immunosorbent assay (ELISA) to detect Histoplasma capsulatum antigenuria in immunocompromised patients.
Infections
Diisopropylamine dichloroacetate, a novel pyruvate dehydrogenase kinase 4 inhibitor, as a potential therapeutic agent for metabolic disorders and multiorgan failure in severe influenza.
Infections
Down-regulation of expression of rat pyruvate dehydrogenase E1alpha gene by self-complementary adeno-associated virus-mediated small interfering RNA delivery.
Infections
Glycine decarboxylase mediates a postbinding step in duck hepatitis B virus infection.
Infections
Hypoxia inducible factor -1 regulates WSSV-induced glycolytic genes in the white shrimp Litopenaeus vannamei.
Infections
Identification of immunogenic proteins and evaluation of recombinant PDHA1 and GAPDH as potential vaccine candidates against Streptococcus iniae infection in flounder (Paralichthys olivaceus).
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
pdh modulate virulence through reducing stress tolerance and biofilm formation of Streptococcus suis serotype 2.
Infections
Progressive disseminated histoplasmosis mimicking a flare of systemic lupus erythematosus: a European case report.
Infections
Subunits of the Pyruvate Dehydrogenase Cluster of Mycoplasma pneumoniae Are Surface-Displayed Proteins that Bind and Activate Human Plasminogen.
Infections
[Study on the replication of hepatitis B virus compared with that of duck hepatitis B virus in primary duck hepatocytes]
Infertility, Male
Antisense inhibition of mitochondrial pyruvate dehydrogenase E1alpha subunit in anther tapetum causes male sterility.
Influenza, Human
Diisopropylamine dichloroacetate, a novel pyruvate dehydrogenase kinase 4 inhibitor, as a potential therapeutic agent for metabolic disorders and multiorgan failure in severe influenza.
Insulin Resistance
A preliminary evaluation of the circulating leptin/adiponectin ratio in dogs with pituitary-dependent hyperadrenocorticism and concurrent diabetes mellitus.
Insulin Resistance
Acute activation of pyruvate dehydrogenase increases glucose oxidation in muscle without changing glucose uptake.
Insulin Resistance
Ang II causes insulin resistance and induces cardiac metabolic switch and inefficiency: a critical role of PDK4.
Insulin Resistance
Effect of dexamethasone on adipose tissue and liver pyruvate dehydrogenase and its stimulation by insulin-generated chemical mediator.
Insulin Resistance
Effect of exercise training on skeletal muscle protein expression in relation to insulin sensitivity: Per-protocol analysis of a randomized controlled trial (GO-ACTIWE).
Insulin Resistance
Enhanced blood insulin overcomes pyruvate dehydrogenase derangements that reflect systemic insulin resistance in obese adolescents.
Insulin Resistance
Enhanced post-receptor insulin effects in women following dehydroepiandrosterone infusion.
Insulin Resistance
Exercise Alleviates Lipid-Induced Insulin Resistance in Human Skeletal Muscle-Signaling Interaction at the Level of TBC1 Domain Family Member 4.
Insulin Resistance
Feline pituitary-dependent hyperadrenocorticism and insulin resistance due to a plurihormonal adenoma.
Insulin Resistance
Genetic activation of pyruvate dehydrogenase alters oxidative substrate selection to induce skeletal muscle insulin resistance.
Insulin Resistance
Hepatic HAX-1 inactivation prevents metabolic diseases by enhancing mitochondrial activity and bile salt export.
Insulin Resistance
In obese individuals dexfenfluramine corrects molecular derangements reflecting insulin resistance.
Insulin Resistance
Insulin resistance in obese subjects and newly diagnosed NIDDM patients and derangements of pyruvate dehydrogenase in their circulating lymphocytes.
Insulin Resistance
Involvement of glucagon-like peptide 1 in the glucose homeostasis regulation in obese and pituitary-dependent hyperadrenocorticism affected dogs.
Insulin Resistance
Mitochondrial Uncoupling Coordinated With PDH Activation Safely Ameliorates Hyperglycemia Via Promoting Glucose Oxidation.
Insulin Resistance
Molecular mechanisms in skeletal muscle underlying insulin resistance in lean women with polycystic ovary syndrome.
Insulin Resistance
Opposing actions of dehydroepiandrosterone and testosterone on insulin sensitivity. In vivo and in vitro studies of hyperandrogenic females.
Insulin Resistance
Perpetual muscle PDH activation in PDH kinase knockout mice protects against high-fat feeding-induced muscle insulin resistance.
Insulin Resistance
Regulation of Muscle Pyruvate Dehydrogenase Complex in Insulin Resistance: Effects of Exercise and Dichloroacetate.
Insulin Resistance
Reply to Constantin-Teodosiu et al.: Mice with genetic PDH activation are not protected from high-fat diet-induced muscle 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.
Insulinoma
Regulation of islet beta-cell pyruvate metabolism: interactions of prolactin, glucose, and dexamethasone.
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.
Intervertebral Disc Degeneration
Quantitative radiographic analysis of foraminal re-stenosis after posterior cervical foraminotomy with laminoplasty.
Intestinal Obstruction
Left Paraduodenal Hernia Presenting as Closed Loop Jejunal Obstruction in a Young Female: An Enigmatic and Perilous Differential of Acute Abdomen.
Intestinal Obstruction
Paraduodenal hernias in children: Etiology, treatment, and outcomes of a rare but real cause of bowel obstruction.
Intestinal Obstruction
Timing of Abdominal CT Evaluation Impacts the Diagnosis of Paraduodenal Hernia.
Ischemic Attack, Transient
Ischemic tolerance phenomenon from an approach of energy metabolism and the mitochondrial enzyme activity of pyruvate dehydrogenase in gerbils.
Ischemic Stroke
Sodium Dichloroacetate Stimulates Angiogenesis by Improving Endothelial Precursor Cell Function in an AKT/GSK-3?/Nrf2 Dependent Pathway in Vascular Dementia Rats.
Ketosis
Pyruvate dehydrogenase activity is decreased in emergency department patients with diabetic ketoacidosis.
Kidney Failure, Chronic
Reduction of mitochondria and up regulation of pyruvate dehydrogenase kinase 4 of skeletal muscle in patients with chronic kidney disease.
Language Disorders
An Extension of the Procedural Deficit Hypothesis from Developmental Language Disorders to Mathematical Disability.
Leber Congenital Amaurosis
Structural Insights into the Drosophila melanogaster Retinol Dehydrogenase, a Member of the Short-Chain Dehydrogenase/Reductase Family.
Leigh Disease
Beneficial effect of pyruvate therapy on Leigh syndrome due to a novel mutation in PDH E1? gene.
Leigh Disease
Clinical exome sequencing reveals a mutation in PDHA1 in Leigh syndrome: A case of a Chinese boy with lethal neuropathy.
Leigh Disease
Leigh syndrome due to pyruvate dehydrogenase E1 alpha deficiency (point mutation R263G) in a Spanish boy.
Leigh Disease
Novel presentations associated with a PDHA1 variant - Alternating hemiplegia in Hemizygote proband and Guillain Barre Syndrome in Heterozygote mother.
Leigh Disease
PDH E(1)beta deficiency with novel mutations in two patients with Leigh syndrome.
Leigh Disease
Pyruvate dehydrogenase complex deficiency: four neurological phenotypes with differing pathogenesis.
Leigh Disease
[Leigh syndrome due to pyruvate dehydrogenase E1 alpha subunit gene mutation: a complicated and difficult case study]
Leptospirosis
[Experimental study of the effect of dexamethasone on pulmonary diffuse hemorrhage and energy metabolism of mitochondria in the liver of guinea pig infected with leptospirosis]
Leukemia
De novo methylation of tumor suppressor gene p16/INK4a is a frequent finding in multiple myeloma patients at diagnosis.
Leukemia
Reactive oxygen species are involved in arsenic trioxide inhibition of pyruvate dehydrogenase activity.
Leukemia
The requirement for pyruvate dehydrogenase in leukemogenesis depends on cell lineage.
Leukemia
Tyr-301 phosphorylation inhibits pyruvate dehydrogenase by blocking substrate binding and promotes the Warburg effect.
Leukemia, Plasma Cell
De novo methylation of tumor suppressor gene p16/INK4a is a frequent finding in multiple myeloma patients at diagnosis.
Leukomalacia, Periventricular
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
Leukomalacia, Periventricular
MRI features of 4 female patients with pyruvate dehydrogenase E1 alpha deficiency.
Lewy Body Disease
Alteration of mitochondrial protein PDHA1 in Lewy body disease and PARK14.
Liver Cirrhosis, Biliary
Analysis of rearranged T cell receptor (TCR) V beta transcripts in livers of primary biliary cirrhosis: preferential V beta usage suggests antigen-driven selection.
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
Enzyme inhibitory autoantibodies to pyruvate dehydrogenase complex in primary biliary cirrhosis: applications of a semiautomated assay.
Liver Cirrhosis, Biliary
Identification and characterization of antimitochondrial autoantibodies in sera of patients with monoclonal gammopathies.
Liver Cirrhosis, Biliary
Identification of a xenobiotic as a potential environmental trigger in primary biliary cholangitis.
Liver Cirrhosis, Biliary
Liver autoimmunity triggered by microbial activation of natural killer T cells.
Liver Cirrhosis, Biliary
Membrane dihydrolipoamide acetyltransferase (E2) on human biliary epithelial cells in primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Mitochondrial pyruvate dehydrogenase complex subunits as autoantigens in human primary biliary cirrhosis.
Liver Cirrhosis, Biliary
Natural and disease associated autoantibodies to the autoantigen, dihydrolipoamide acetyltransferase, recognise different epitopes.
Liver Cirrhosis, Biliary
Sidechain biology and the immunogenicity of PDC-E2, the major autoantigen of primary biliary cirrhosis.
Liver Cirrhosis, Biliary
[Primary biliary cirrhosis--specific anti-mitochondrial antibodies]
Liver Diseases
Hepatic pyruvate dehydrogenase activity in humans: effect of cirrhosis, transplantation, and dichloroacetate.
Liver Diseases
Identification of a xenobiotic as a potential environmental trigger in primary biliary cholangitis.
Liver Neoplasms
AAV3-mediated transfer and expression of the pyruvate dehydrogenase E1 alpha subunit gene causes metabolic remodeling and apoptosis of human liver cancer cells.
Lordosis
Contralateral radiculopathy after unilateral transforaminal lumbar interbody fusion: causes and prevention.
Lung Neoplasms
Drug evaluation based on phosphomimetic PDHA1 reveals the complexity of activity-related cell death in A549 non-small cell lung cancer cells.
Lung Neoplasms
Subpopulation targeting of pyruvate dehydrogenase and GLUT1 decouples metabolic heterogeneity during collective cancer cell invasion.
Lung Neoplasms
The microRNA-182-PDK4 axis regulates lung tumorigenesis by modulating pyruvate dehydrogenase and lipogenesis.
Lupus Erythematosus, Systemic
Antibody binding and inhibition of pyruvate dehydrogenase (PDH) in sera from patients with primary biliary cirrhosis.
Lymphatic Metastasis
Expression of pyruvate dehydrogenase is an independent prognostic marker in gastric cancer.
Lymphoma
Epigallocatechin-3-gallate downregulates PDHA1 interfering the metabolic pathways in human herpesvirus 8 harboring primary effusion lymphoma cells.
Lymphoma, Primary Effusion
Epigallocatechin-3-gallate downregulates PDHA1 interfering the metabolic pathways in human herpesvirus 8 harboring primary effusion lymphoma cells.
Malaria
Plasmodium pyruvate dehydrogenase activity is only essential for the parasite's progression from liver infection to blood infection.
Malaria
The malaria parasite Plasmodium falciparum has only one pyruvate dehydrogenase complex, which is located in the apicoplast.
Maple Syrup Urine Disease
Analysis of gene mutations among South Indian patients with maple syrup urine disease: identification of four novel mutations.
Maple Syrup Urine Disease
Functional characterization of the novel intronic nucleotide change c.288+9C>T within the BCKDHA gene: understanding a variant presentation of maple syrup urine disease.
Maple Syrup Urine Disease
Impaired assembly of E1 decarboxylase of the branched-chain alpha-ketoacid dehydrogenase complex in type IA maple syrup urine disease.
Maple Syrup Urine Disease
Molecular genetics of maple syrup urine disease in the Turkish population.
Melanoma
A key role for mitochondrial gatekeeper pyruvate dehydrogenase in oncogene-induced senescence.
Melanoma
ROS production induced by BRAF inhibitor treatment rewires metabolic processes affecting cell growth of melanoma cells.
MELAS Syndrome
Diagnostic accuracy of blood and CSF lactate in identifying children with mitochondrial diseases affecting the central nervous system.
Metabolic Diseases
Hepatic HAX-1 inactivation prevents metabolic diseases by enhancing mitochondrial activity and bile salt export.
Metabolic Diseases
Real-time hyperpolarized 13C magnetic resonance detects increased pyruvate oxidation in pyruvate dehydrogenase kinase 2/4-double knockout mouse livers.
Metabolic Diseases
[Leigh syndrome due to pyruvate dehydrogenase E1 alpha subunit gene mutation: a complicated and difficult case study]
Metabolic Syndrome
Regulation of Muscle Pyruvate Dehydrogenase Complex in Insulin Resistance: Effects of Exercise and Dichloroacetate.
Microcephaly
Somatic mosaicism for a novel PDHA1 mutation in a male with severe pyruvate dehydrogenase complex deficiency.
Mitochondrial Diseases
A 1.1 million base pair X-chromosomal deletion covering the PDHA1 and CDKL5 genes in a female patient with west syndrome and pyruvate oxidation deficiency.
Mitochondrial Diseases
Dichloroacetate stabilizes the mutant E1alpha subunit in pyruvate dehydrogenase deficiency.
Mitochondrial Diseases
Mitochondrial Glutathione Transferase Zeta 1 is Inactivated More Rapidly by Dichloroacetate than the Cytosolic Enzyme in Adult and Juvenile Rat Liver.
Mitochondrial Diseases
Severe encephalopathy associated to pyruvate dehydrogenase mutations and unbalanced coenzyme Q10 content.
Mitochondrial Diseases
Stabilization of the pyruvate dehydrogenase E1alpha subunit by dichloroacetate.
Mitochondrial Diseases
[Diagnosis of mitochondrial disorders in children with next generation sequencing].
Movement Disorders
Pyruvate dehydrogenase deficiency presenting as intermittent isolated acute ataxia.
Mucocele
Serum cholecystokinin concentrations in dogs with naturally acquired pituitary-dependent hyperadrenocorticism.
Multiple System Atrophy
Alteration of mitochondrial protein PDHA1 in Lewy body disease and PARK14.
Muscle Hypotonia
Association of cerebral dysgenesis and lactic acidemia with X-linked PDH E1 alpha subunit mutations in females.
Muscle Spasticity
The genetic etiology in cerebral palsy mimics: The results from a Greek tertiary care center.
Muscle Weakness
Reduction of mitochondria and up regulation of pyruvate dehydrogenase kinase 4 of skeletal muscle in patients with chronic kidney disease.
Muscular Dystrophy, Duchenne
PDH-mediated metabolic flow is critical for skeletal muscle stem cell differentiation and myotube formation during regeneration in mice.
Myocardial Infarction
Cardiac-Specific Deletion of the Pdha1 Gene Sensitizes Heart to Toxicological Actions of Ischemic Stress.
Myocardial Infarction
Comparative Proteome Profiling during Cardiac Hypertrophy and Myocardial Infarction Reveals Altered Glucose Oxidation by Differential Activation of Pyruvate Dehydrogenase E1 Component Subunit ?
Myocardial Infarction
[Chronic heart failure: effect and evaluation of therapy with positive inotropic substances]
nadh:ubiquinone reductase (h+-translocating) deficiency
Improvement of lesions shown on MRI and CT scan by administration of dichloroacetate in patients with Leigh syndrome.
Neoplasm Metastasis
AMPK maintains TCA cycle through sequential phosphorylation of PDHA to promote tumor metastasis.
Neoplasm Metastasis
Expression of pyruvate dehydrogenase is an independent prognostic marker in gastric cancer.
Neoplasm Metastasis
PGC1? promotes cholangiocarcinoma metastasis by upregulating PDHA1 and MPC1 expression to reverse the Warburg effect.
Neoplasm Metastasis
Src drives the Warburg effect and therapy resistance by inactivating pyruvate dehydrogenase through tyrosine-289 phosphorylation.
Neoplasms
A mitochondria-K+ channel axis is suppressed in cancer and its normalization promotes apoptosis and inhibits cancer growth.
Neoplasms
Active pyruvate dehydrogenase and impaired gluconeogenesis in orthotopic hepatomas of rats.
Neoplasms
Amrinone prevents the inhibition of muscle pyruvate dehydrogenase complex activity during sepsis.
Neoplasms
Cancer incidence in municipalities near two former nuclear materials processing facilities in Pennsylvania--an update.
Neoplasms
Comparison of two low-dose dexamethasone suppression protocols as screening and discrimination tests in dogs with hyperadrenocorticism.
Neoplasms
Compartmentalized activities of the pyruvate dehydrogenase complex sustain lipogenesis in prostate cancer.
Neoplasms
Conditions associated with relapse of amphotericin B-treated disseminated histoplasmosis.
Neoplasms
De novo methylation of tumor suppressor gene p16/INK4a is a frequent finding in multiple myeloma patients at diagnosis.
Neoplasms
Decoding Warburg's hypothesis: tumor-related mutations in the mitochondrial respiratory chain.
Neoplasms
Decreased expression of pyruvate dehydrogenase A1 predicts an unfavorable prognosis in ovarian carcinoma.
Neoplasms
Dichloroacetate, the Pyruvate Dehydrogenase Complex and the Modulation of mESC Pluripotency.
Neoplasms
Does Scavenging of Mitochondrial Superoxide Attenuate Cancer Prosurvival Signaling Pathways?
Neoplasms
Drug evaluation based on phosphomimetic PDHA1 reveals the complexity of activity-related cell death in A549 non-small cell lung cancer cells.
Neoplasms
Dynamic adrenal function testing in eight dogs with hyperadrenocorticism associated with adrenocortical neoplasia.
Neoplasms
Effect of colon cancer and surgical resection on skeletal muscle mitochondrial enzyme activity in colon cancer patients: a pilot study.
Neoplasms
Exosomal miR-21-5p derived from cisplatin-resistant SKOV3 ovarian cancer cells promotes glycolysis and inhibits chemosensitivity of its progenitor SKOV3 cells by targeting PDHA1.
Neoplasms
Expression of a p16INK4a-specific ribozyme downmodulates p16INK4a abundance and accelerates cell proliferation.
Neoplasms
Expression of pyruvate dehydrogenase is an independent prognostic marker in gastric cancer.
Neoplasms
High-dose vitamin B1 reduces proliferation in cancer cell lines analogous to dichloroacetate.
Neoplasms
Hypoxic repression of pyruvate dehydrogenase activity is necessary for metabolic reprogramming and growth of model tumours.
Neoplasms
Identification of PDHX as a metabolic target for esophageal squamous cell carcinoma.
Neoplasms
In vitro cytotoxicity screening to identify novel anti-osteosarcoma therapeutics targeting pyruvate dehydrogenase kinase 2.
Neoplasms
Inhibition of Pyruvate Dehydrogenase Kinase Enhances the Antitumor Efficacy of Oncolytic Reovirus.
Neoplasms
Ki-67 and minichromosome maintenance-7 (MCM7) expression in canine pituitary corticotroph adenomas.
Neoplasms
Lung Cancer: A Comparative Study of Metabolism Related Protein Expression in Cancer Cells and Tumor Associated Stroma.
Neoplasms
Metabolic cooperation between co-cultured lung cancer cells and lung fibroblasts.
Neoplasms
Metabolic plasticity maintains proliferation in pyruvate dehydrogenase deficient cells.
Neoplasms
miR?21?5p targets PDHA1 to regulate glycolysis and cancer progression in gastric cancer.
Neoplasms
Mitochondrial dysfunction induces radioresistance in colorectal cancer by activating [Ca2+]m-PDP1-PDH-histone acetylation retrograde signaling.
Neoplasms
Mitochondrial Glutathione Transferase Zeta 1 is Inactivated More Rapidly by Dichloroacetate than the Cytosolic Enzyme in Adult and Juvenile Rat Liver.
Neoplasms
Overexpression of Pyruvate dehydrogenase E1a subunit Inhibits Warburg effect and Induces Cell Apoptosis through Mitochondria-mediated Pathway in Hepatocellular Carcinoma.
Neoplasms
PDHA1 Gene Knockout In Human Esophageal Squamous Cancer Cells Resulted In Greater Warburg Effect And Aggressive Features In Vitro And In Vivo.
Neoplasms
PDHA1 gene knockout in prostate cancer cells results in metabolic reprogramming towards greater glutamine dependence.
Neoplasms
Phenyl butyrate inhibits pyruvate dehydrogenase kinase 1 and contributes to its anti-cancer effect.
Neoplasms
Plasma aldosterone concentrations and plasma renin activity in healthy dogs and dogs with hyperadrenocorticism.
Neoplasms
Poldip2 is an oxygen-sensitive protein that controls PDH and ?KGDH lipoylation and activation to support metabolic adaptation in hypoxia and cancer.
Neoplasms
Post-translational regulation of metabolism in fumarate hydratase deficient cancer cells.
Neoplasms
Pyruvate dehydrogenase and pyruvate dehydrogenase kinase expression in non small cell lung cancer and tumor-associated stroma.
Neoplasms
Pyruvate dehydrogenase expression is negatively associated with cell stemness and worse clinical outcome in prostate cancers.
Neoplasms
Pyruvate Dehydrogenase PDH-E1? Controls Tumor Progression by Altering the Metabolic Status of Cancer Cells.
Neoplasms
Regulation of prolactin secretion in canine pituitary-dependent hyperadrenocorticism.
Neoplasms
Serum pyruvate dehydrogenase kinase as a prognostic marker for cholangiocarcinoma.
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
Systemic arterial blood pressure and urine protein/creatinine ratio in dogs with hyperadrenocorticism.
Neoplasms
The HGF-MET axis coordinates liver cancer metabolism and autophagy for chemotherapeutic resistance.
Neoplasms
The microRNA-182-PDK4 axis regulates lung tumorigenesis by modulating pyruvate dehydrogenase and lipogenesis.
Neoplasms
The novel function of tumor protein D54 in regulating pyruvate dehydrogenase and metformin cytotoxicity in breast cancer.
Neoplasms
The PI3K/Akt Pathway Regulates Oxygen Metabolism via Pyruvate Dehydrogenase (PDH)-E1? Phosphorylation.
Neoplasms
The recent insights into the function of ACAT1: A possible anti-cancer therapeutic target.
Neoplasms
The requirement for pyruvate dehydrogenase in leukemogenesis depends on cell lineage.
Neoplasms
Theoretical aspects of weight loss in patients with cancer. Possible importance of pyruvate dehydrogenase.
Neoplasms
TNF binding protein prevents hyperlactatemia and inactivation of PDH complex in skeletal muscle during sepsis.
Neoplasms
Transformation linked decrease of pyruvate dehydrogenase complex in human epidermis.
Neoplasms
Treatment with pravastatin attenuates progression of chronic pancreatitis in rat.
Neoplasms
Tyr phosphorylation of PDP1 toggles recruitment between ACAT1 and SIRT3 to regulate the pyruvate dehydrogenase complex.
Neoplasms
Tyr-301 phosphorylation inhibits pyruvate dehydrogenase by blocking substrate binding and promotes the Warburg effect.
Neoplasms
Tyr-94 phosphorylation inhibits pyruvate dehydrogenase phosphatase 1 and promotes tumor growth.
Neoplasms
Tyrosine phosphorylation of mitochondrial pyruvate dehydrogenase kinase 1 is important for cancer metabolism.
Neoplasms
Ultrasonographic characteristics of the adrenal glands in dogs with pituitary-dependent hyperadrenocorticism: comparison with normal dogs.
Neoplasms, Squamous Cell
PDHA1 Gene Knockout In Human Esophageal Squamous Cancer Cells Resulted In Greater Warburg Effect And Aggressive Features In Vitro And In Vivo.
Neuralgia
Regulation of mitochondrial function by Epac2 contributes to acute inflammatory hyperalgesia.
Neurodegenerative Diseases
Management of canine pituitary-dependent hyperadrenocorticism with l-deprenyl (Anipryl).
Neurologic Manifestations
Magnetic resonance imaging for diagnosis of pituitary macrotumors in dogs.
Neurologic Manifestations
One-year follow-up evaluation of magnetic resonance imaging of the brain in dogs with pituitary-dependent hyperadrenocorticism.
Non-alcoholic Fatty Liver Disease
Pyruvate Dehydrogenase as a Therapeutic Target for Nonalcoholic Fatty Liver Disease.
Obesity
Adipocyte glycogen synthase and pyruvate dehydrogenase in obese and type II diabetic subjects.
Obesity
Elevated (Pro)renin Receptor Expression Contributes to Maintaining Aerobic Metabolism in Growth Hormone Deficiency.
Obesity
Longitudinal study of tissue- and subunit-specific obesity-induced regulation of the pyruvate dehydrogenase complex.
Obesity
Obesity-related elevations in plasma leucine are associated with alterations in enzymes involved in branched-chain amino acid metabolism.
Obesity
Pyruvate and hepatic pyruvate dehydrogenase levels in rat strains sensitive and resistant to dietary obesity.
Obesity
Pyruvate dehydrogenase activity in adipose tissue mitochondria from normal and obese humans.
Obesity
Skeletal muscle type comparison of pyruvate dehydrogenase phosphatase activity and isoform expression: effects of obesity and endurance training.
Obesity
Thiamine Prevents Obesity and Obesity-Associated Metabolic Disorders in OLETF Rats.
Opportunistic Infections
Disseminated histoplasmosis in AIDS. Clinicopathologic features in seven patients from a non-endemic area.
Otitis Media
The World Health Organisation and the prevention of deafness and hearing impairment caused by noise.
Ovarian Neoplasms
Decreased expression of pyruvate dehydrogenase A1 predicts an unfavorable prognosis in ovarian carcinoma.
Ovarian Neoplasms
Exosomal miR-21-5p derived from cisplatin-resistant SKOV3 ovarian cancer cells promotes glycolysis and inhibits chemosensitivity of its progenitor SKOV3 cells by targeting PDHA1.
Ovarian Neoplasms
Proteomic alterations of fibroblasts induced by ovarian cancer cells reveal potential cancer targets.
Pancreatic Neoplasms
Hypoxic repression of pyruvate dehydrogenase activity is necessary for metabolic reprogramming and growth of model tumours.
Panniculitis
Histoplasmosis and subcutaneous nodules in a kidney transplant recipient: erythema nodosum versus fungal panniculitis.
Paralysis
Acute flaccid paralysis as initial symptom in 4 patients with novel e1alpha mutations of the pyruvate dehydrogenase complex.
Parkinsonian Disorders
Alteration of mitochondrial protein PDHA1 in Lewy body disease and PARK14.
Peroxisomal Disorders
Lactic acidosis and mitochondrial dysfunction in two children with peroxisomal disorders.
Persistent Infection
Mycoplasma genitalium rapidly disseminates to the upper reproductive tracts and knees of female mice following vaginal inoculation.
Pharyngitis
Ethnopharmacology, phytochemistry, and pharmacology of Sterculia lychnophora Hance (Pangdahai).
Pituitary Neoplasms
Computed tomography and low-field magnetic resonance imaging of the pituitary gland in dogs with pituitary-dependent hyperadrenocorticism: 11 cases (2001-2003).
Pituitary Neoplasms
Diagnostic imaging findings and endocrine test results in dogs with pituitary-dependent hyperadrenocorticism that did or did not have neurologic abnormalities: 157 cases (1989-2005).
Pituitary Neoplasms
Efficacy of cobalt 60 radiotherapy in dogs with pituitary-dependent hyperadrenocorticism.
Pituitary Neoplasms
Expression of Ki-67, PCNA, and p27kip1 in canine pituitary corticotroph adenomas.
Pituitary Neoplasms
Plasma pro-opiomelanocortin, pro-adrenocorticotropin hormone, and pituitary adenoma size in dogs with Cushing's disease.
Pituitary Neoplasms
Progress in transsphenoidal hypophysectomy for treatment of pituitary-dependent hyperadrenocorticism in dogs and cats.
Pituitary Neoplasms
Transsphenoidal Surgery in Canines: Safety, Efficacy and Patient Selection.
Pituitary Neoplasms
Treating canine Cushing's syndrome: Current options and future prospects.
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
PDHA1 gene knockout in prostate cancer cells results in metabolic reprogramming towards greater glutamine dependence.
Prostatic Neoplasms
Pyruvate dehydrogenase expression is negatively associated with cell stemness and worse clinical outcome in prostate cancers.
Proteinuria
Systemic arterial blood pressure and urine protein/creatinine ratio in dogs with hyperadrenocorticism.
Pulmonary Embolism
Frequency of pulmonary mineralization and hypoxemia in 21 dogs with pituitary-dependent hyperadrenocorticism.
pyruvate decarboxylase deficiency
Beneficial effect of feeding a ketogenic diet to mothers on brain development in their progeny with a murine model of pyruvate dehydrogenase complex deficiency.
pyruvate decarboxylase deficiency
Differential phenotypic expression of a novel PDHA1 mutation in a female monozygotic twin pair.
pyruvate decarboxylase deficiency
Down-regulation of expression of rat pyruvate dehydrogenase E1alpha gene by self-complementary adeno-associated virus-mediated small interfering RNA delivery.
pyruvate decarboxylase deficiency
Enzymatic testing sensitivity, variability and practical diagnostic algorithm for pyruvate dehydrogenase complex (PDC) deficiency.
pyruvate decarboxylase deficiency
Novel presentations associated with a PDHA1 variant - Alternating hemiplegia in Hemizygote proband and Guillain Barre Syndrome in Heterozygote mother.
pyruvate decarboxylase deficiency
Pyruvate dehydrogenase complex deficiency: updating the clinical, metabolic and mutational landscapes in a cohort of Portuguese patients.
pyruvate decarboxylase deficiency
Structural and functional impact of clinically relevant E1? variants causing pyruvate dehydrogenase complex deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
A cognitively normal PDH-deficient 18-year-old man carrying the R263G mutation in the PDHA1 gene.
pyruvate dehydrogenase (acetyl-transferring) deficiency
A combined therapeutic approach for pyruvate dehydrogenase deficiency using self-complementary adeno-associated virus serotype-specific vectors and dichloroacetate.
pyruvate dehydrogenase (acetyl-transferring) deficiency
A zebrafish model for pyruvate dehydrogenase deficiency: rescue of neurological dysfunction and embryonic lethality using a ketogenic diet.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Aberrant splicing of exon 6 in the pyruvate dehydrogenase-E1 alpha mRNA linked to a silent mutation in a large family with Leigh's encephalomyelopathy.
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
Association of cerebral dysgenesis and lactic acidemia with X-linked PDH E1 alpha subunit mutations in females.
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
Bi-allelic mutations of LONP1 encoding the mitochondrial LonP1 protease cause pyruvate dehydrogenase deficiency and profound neurodegeneration with progressive cerebellar atrophy.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Biochemical and genetic studies of four patients with pyruvate dehydrogenase E1 alpha deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Cerebral palsy and pyruvate dehydrogenase deficiency: identification of two new mutations in the E1alpha gene.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Diagnostic accuracy of blood and CSF lactate in identifying children with mitochondrial diseases affecting the central nervous system.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Dichloroacetate stabilizes the mutant E1alpha subunit in pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Down-regulation of expression of rat pyruvate dehydrogenase E1alpha gene by self-complementary adeno-associated virus-mediated small interfering RNA delivery.
pyruvate dehydrogenase (acetyl-transferring) deficiency
E4F1 controls a transcriptional program essential for pyruvate dehydrogenase activity.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Energy substrate metabolism in pyruvate dehydrogenase complex deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Four novel PDHA1 mutations in pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
FoxO1 inhibition alleviates type 2 diabetes-related diastolic dysfunction by increasing myocardial pyruvate dehydrogenase activity.
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
Immunochemical analysis of normal and mutant forms of human pyruvate dehydrogenase.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Impairment of pyruvate dehydrogenase activity by acetaldehyde.
pyruvate dehydrogenase (acetyl-transferring) deficiency
In vivo pyruvate detected by MR spectroscopy in neonatal pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Intermittent peripheral weakness as the presenting feature of pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Ketonic diet in the management of pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Lactate oxidation for the detection of mitochondrial dysfunction in human skin fibroblasts.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Mechanisms of expression of pyruvate dehydrogenase deficiency caused by an E1alpha subunit mutation.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Mendeliome sequencing enables differential diagnosis and treatment of neonatal lactic acidosis.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Metabolic flux analysis of wild-type Escherichia coli and mutants deficient in pyruvate-dissimilating enzymes during fermentative metabolism of glucuronate.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Metabolic plasticity maintains proliferation in pyruvate dehydrogenase deficient cells.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Mutations in the gene for the E1beta subunit: a novel cause of pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (acetyl-transferring) 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) deficiency
Mutations of the E1beta subunit gene (PDHB) in four families with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Neonatal pyruvate dehydrogenase deficiency due to a R302H mutation in the PDHA1 gene: MRI findings.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Neuropathological findings of a patient with pyruvate dehydrogenase E1 alpha deficiency presenting as a cerebral lactic acidosis.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Nonspecific mitochondrial disease with epilepsy in children: diagnostic approaches and epileptic phenotypes.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Optic neuropathy in a patient with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Partial pyruvate decarboxylase deficiency with profound lactic acidosis and hyperammonemia: responses to dichloroacetate and benzoate.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Phenotypic and Neuropathological Characterization of Fetal Pyruvate Dehydrogenase Deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Phenyl butyrate inhibits pyruvate dehydrogenase kinase 1 and contributes to its anti-cancer effect.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Potential role of stress-induced gluconeogenesis in disease aggravation and mortality in pyruvate dehydrogenase deficiency: A case-based hypothesis.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Prenatal diagnosis of pyruvate dehydrogenase E1 alpha subunit deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Pyruvate dehydrogenase complex deficiency and absence of subunit X.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Pyruvate dehydrogenase complex deficiency and its relationship with epilepsy frequency--An overview.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Pyruvate dehydrogenase complex deficiency caused by ubiquitination and proteasome-mediated degradation of the E1 subunit.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Pyruvate dehydrogenase deficiency and epilepsy.
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
Pyruvate dehydrogenase deficiency presenting as dystonia in childhood.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Pyruvate dehydrogenase deficiency presenting as intermittent isolated acute ataxia.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Pyruvate dehydrogenase deficiency. Clinical presentation and molecular genetic characterization of five new patients.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Pyruvate dehydrogenase deficiency: clinical and biochemical diagnosis.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Pyruvate dehydrogenase E1 alpha deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Pyruvate dehydrogenase E3 binding protein (protein X) deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Pyruvate dehydrogenase E3 binding protein deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Recombinant adeno-associated virus vector-based gene transfer for defects in oxidative metabolism.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Reduction of mitochondria and up regulation of pyruvate dehydrogenase kinase 4 of skeletal muscle in patients with chronic kidney disease.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Role of dichloroacetate in the treatment of genetic mitochondrial diseases.
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
Stabilization of the pyruvate dehydrogenase E1alpha subunit by dichloroacetate.
pyruvate dehydrogenase (acetyl-transferring) deficiency
The pyruvate dehydrogenase complex as a target for gene therapy.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Thiamin-Responsive PDH Deficiency due to a PDHA1 Variant.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Thiamine-Responsive and Non-responsive Patients with PDHC-E1 Deficiency: A Retrospective Assessment.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Unilateral periventricular leukomalacia in association with pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
X chromosome inactivation and the diagnosis of X linked disease in females.
pyruvate dehydrogenase (acetyl-transferring) deficiency
X-linked pyruvate dehydrogenase E1 alpha subunit deficiency in heterozygous females: variable manifestation of the same mutation.
pyruvate dehydrogenase (acetyl-transferring) deficiency
[Analysis of PDHA1 gene variant in a patient with pyruvate dehydrogenase E1alpha deficiency and pyramidal tract involvement].
pyruvate dehydrogenase (acetyl-transferring) deficiency
[Familial partial pyruvic dehydrogenase deficiency (author's transl)]
pyruvate dehydrogenase (acetyl-transferring) deficiency
[Leigh syndrome due to pyruvate dehydrogenase E1 alpha subunit gene mutation: a complicated and difficult case study]
pyruvate dehydrogenase (acetyl-transferring) deficiency
[Pyruvate dehydrogenase deficiency and cerebral malformations]
pyruvate dehydrogenase (acetyl-transferring) deficiency
[Pyruvate dehydrogenase deficit associated to the C515T mutation in exon 6 of the E1alpha gene.]
pyruvate dehydrogenase (nadp+) deficiency
A pathogenic glutamate-to-aspartate substitution (D296E) in the pyruvate dehydrogenase E1 subunit gene PDHA1.
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
Arginine 302 mutations in the pyruvate dehydrogenase E1alpha subunit gene: identification of further patients and in vitro demonstration of pathogenicity.
pyruvate dehydrogenase (nadp+) deficiency
Cerebral palsy and pyruvate dehydrogenase deficiency: identification of two new mutations in the E1alpha gene.
pyruvate dehydrogenase (nadp+) deficiency
Defects of pyruvate metabolism and the Krebs cycle.
pyruvate dehydrogenase (nadp+) deficiency
Females with PDHA1 gene mutations: a diagnostic challenge.
pyruvate dehydrogenase (nadp+) deficiency
Four novel PDHA1 mutations in pyruvate dehydrogenase deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Massive parallel sequencing identifies RAPSN and PDHA1 mutations causing fetal akinesia deformation sequence.
pyruvate dehydrogenase (nadp+) deficiency
MRI features of 4 female patients with pyruvate dehydrogenase E1 alpha deficiency.
pyruvate dehydrogenase (nadp+) deficiency
Neonatal pyruvate dehydrogenase deficiency due to a R302H mutation in the PDHA1 gene: MRI findings.
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: identification of a novel mutation in the PDHA1 gene which responds to amino acid supplementation.
pyruvate dehydrogenase (nadp+) deficiency
Somatic mosaicism for a PDHA1 mutation in a female with pyruvate dehydrogenase deficiency.
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
Two Patients with Clinically Distinct Manifestation of Pyruvate Dehydrogenase Deficiency Due to Mutations in PDHA1 Gene.
pyruvate dehydrogenase (nadp+) deficiency
X-chromosome localization of the functional gene for the E1 alpha subunit of the human pyruvate dehydrogenase complex.
Pyruvate Dehydrogenase Complex Deficiency Disease
A 1.1 million base pair X-chromosomal deletion covering the PDHA1 and CDKL5 genes in a female patient with west syndrome and pyruvate oxidation deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
A cognitively normal PDH-deficient 18-year-old man carrying the R263G mutation in the PDHA1 gene.
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 Korean female patient with thiamine-responsive pyruvate dehydrogenase complex deficiency due to a novel point mutation (Y161C)in the PDHA1 gene.
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 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
Aberrant splicing of exon 6 in the pyruvate dehydrogenase-E1 alpha mRNA linked to a silent mutation in a large family with Leigh's encephalomyelopathy.
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
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
Association of cerebral dysgenesis and lactic acidemia with X-linked PDH E1 alpha subunit mutations in females.
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
Cerebral palsy and pyruvate dehydrogenase deficiency: identification of two new mutations in the E1alpha gene.
Pyruvate Dehydrogenase Complex Deficiency Disease
Clinical exome sequencing reveals a mutation in PDHA1 in Leigh syndrome: A case of a Chinese boy with lethal neuropathy.
Pyruvate Dehydrogenase Complex Deficiency Disease
Defects of pyruvate metabolism and the Krebs cycle.
Pyruvate Dehydrogenase Complex Deficiency Disease
Diagnostic accuracy of blood and CSF lactate in identifying children with mitochondrial diseases affecting the central nervous system.
Pyruvate Dehydrogenase Complex Deficiency Disease
Dichloroacetate stabilizes the mutant E1alpha subunit in pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Dihydrolipoamide dehydrogenase (DLD) deficiency in a Spanish patient with myopathic presentation due to a new mutation in the interface domain.
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
Energy substrate metabolism in pyruvate dehydrogenase complex deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Females with PDHA1 gene mutations: a diagnostic challenge.
Pyruvate Dehydrogenase Complex Deficiency Disease
Four novel PDHA1 mutations in pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
FoxO1 inhibition alleviates type 2 diabetes-related diastolic dysfunction by increasing myocardial pyruvate dehydrogenase activity.
Pyruvate Dehydrogenase Complex Deficiency Disease
Immunocapture and microplate-based activity measurement of mammalian pyruvate dehydrogenase complex.
Pyruvate Dehydrogenase Complex Deficiency Disease
Immunochemical analysis of normal and mutant forms of human pyruvate dehydrogenase.
Pyruvate Dehydrogenase Complex Deficiency Disease
Impairment of pyruvate dehydrogenase activity by acetaldehyde.
Pyruvate Dehydrogenase Complex Deficiency Disease
In vivo pyruvate detected by MR spectroscopy in neonatal pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Intermittent peripheral weakness as the presenting feature of pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Ketonic diet in the management of pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Lactate oxidation for the detection of mitochondrial dysfunction in human skin fibroblasts.
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
Mendeliome sequencing enables differential diagnosis and treatment of neonatal lactic acidosis.
Pyruvate Dehydrogenase Complex Deficiency Disease
Metabolic flux analysis of wild-type Escherichia coli and mutants deficient in pyruvate-dissimilating enzymes during fermentative metabolism of glucuronate.
Pyruvate Dehydrogenase Complex Deficiency Disease
Metabolic plasticity maintains proliferation in pyruvate dehydrogenase deficient cells.
Pyruvate Dehydrogenase Complex Deficiency Disease
Mild Phenotype in a Male with Pyruvate Dehydrogenase Complex Deficiency Associated with Novel Hemizygous In-Frame Duplication of the E1? Subunit Gene (PDHA1).
Pyruvate Dehydrogenase Complex Deficiency Disease
MRI features of 4 female patients with pyruvate dehydrogenase E1 alpha deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Mutation analysis of the pyruvate dehydrogenase E1 alpha gene in eight patients with a pyruvate dehydrogenase complex deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Mutational study in the PDHA1 gene of 40 patients suspected of pyruvate dehydrogenase complex deficiency.
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
Neonatal pyruvate dehydrogenase deficiency due to a R302H mutation in the PDHA1 gene: MRI findings.
Pyruvate Dehydrogenase Complex Deficiency Disease
Neuropathological findings of a patient with pyruvate dehydrogenase E1 alpha deficiency presenting as a cerebral lactic acidosis.
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
Partial pyruvate decarboxylase deficiency with profound lactic acidosis and hyperammonemia: responses to dichloroacetate and benzoate.
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
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
Pyruvate dehydrogenase complex deficiency and absence of subunit X.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase complex deficiency caused by ubiquitination and proteasome-mediated degradation of the E1 subunit.
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 deficiency caused by a 33 base pair duplication in the PDH E1 alpha subunit.
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 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 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. 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 E1 alpha deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Pyruvate dehydrogenase E3 binding protein (protein X) deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Recombinant adeno-associated virus vector-based gene transfer for defects in oxidative metabolism.
Pyruvate Dehydrogenase Complex Deficiency Disease
Reduction of mitochondria and up regulation of pyruvate dehydrogenase kinase 4 of skeletal muscle in patients with chronic kidney disease.
Pyruvate Dehydrogenase Complex Deficiency Disease
Role of dichloroacetate in the treatment of genetic mitochondrial diseases.
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
Somatic mosaicism for a novel PDHA1 mutation in a male with severe pyruvate dehydrogenase complex deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Somatic mosaicism for a PDHA1 mutation in a female with pyruvate dehydrogenase deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Somatic mosaicism for PDHA1 mutation in a male with pyruvate dehydrogenase complex deficiency.
Pyruvate Dehydrogenase Complex Deficiency Disease
Stabilization of the pyruvate dehydrogenase E1alpha subunit by dichloroacetate.
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
Thiamin-Responsive PDH Deficiency due to a PDHA1 Variant.
Pyruvate Dehydrogenase Complex Deficiency Disease
Thiamine Responsive Pyruvate Dehydrogenase Complex Deficiency: A Potentially Treatable Cause of Leigh's Disease.
Pyruvate Dehydrogenase Complex Deficiency Disease
Thiamine-Responsive and Non-responsive Patients with PDHC-E1 Deficiency: A Retrospective Assessment.
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
Unilateral periventricular leukomalacia in association with pyruvate dehydrogenase deficiency.
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
[Pyruvate dehydrogenase deficiency and cerebral malformations]
Pyruvate Dehydrogenase Complex Deficiency Disease
[Pyruvate dehydrogenase deficit associated to the C515T mutation in exon 6 of the E1alpha gene.]
pyruvate dehydrogenase system deficiency
Analysis of exonic mutations leading to exon skipping in patients with pyruvate dehydrogenase E1 alpha deficiency.
pyruvate dehydrogenase system deficiency
Deficiency of pyruvate dehydrogenase caused by novel and known mutations in the E1alpha subunit.
Radiculopathy
Contralateral radiculopathy after unilateral transforaminal lumbar interbody fusion: causes and prevention.
Reperfusion Injury
Hyperpolarized (13) C magnetic resonance evaluation of renal ischemia reperfusion injury in a murine model.
Rhinitis
Changes over time in the prevalence of asthma, rhinitis and atopic eczema in adolescents from Taubaté, São Paulo, Brazil (2005-2012): Relationship with living near a heavily travelled highway.
Rhinitis, Allergic
Picumast dihydrochloride (Auteral), a new anti-allergic inhibitor of mediator release and action.
Scleroderma, Systemic
Effects of prostaglandin E1alpha cyclodextrin [corrected] treatment on endothelial dysfunction in patients with systemic sclerosis.
Seizures
Mild Phenotype in a Male with Pyruvate Dehydrogenase Complex Deficiency Associated with Novel Hemizygous In-Frame Duplication of the E1? Subunit Gene (PDHA1).
Seizures
Somatic mosaicism for a novel PDHA1 mutation in a male with severe pyruvate dehydrogenase complex deficiency.
Sepsis
Activities of respiratory chain complexes and pyruvate dehydrogenase in isolated muscle mitochondria in premature neonates.
Sepsis
Amrinone prevents the inhibition of muscle pyruvate dehydrogenase complex activity during 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
Pyruvate Dehydrogenase Activity Is Decreased in the Peripheral Blood Mononuclear Cells of Patients with Sepsis. A Prospective Observational Trial.
Sepsis
Regulation of glucose metabolism by altered pyruvate dehydrogenase activity. I. Potential site of insulin resistance in sepsis.
Sepsis
TNF binding protein prevents hyperlactatemia and inactivation of PDH complex in skeletal muscle during sepsis.
Spasms, Infantile
A 1.1 million base pair X-chromosomal deletion covering the PDHA1 and CDKL5 genes in a female patient with west syndrome and pyruvate oxidation deficiency.
Spastic Paraplegia, Hereditary
The genetic etiology in cerebral palsy mimics: The results from a Greek tertiary care center.
Spinocerebellar Degenerations
Pyruvate dehydrogenase deficiency in spinocerebellar degenerations.
Squamous Cell Carcinoma of Head and Neck
Determination of Pyruvate Metabolic Fates Modulates Head and Neck Tumorigenesis.
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
Immunocapture and microplate-based activity measurement of mammalian pyruvate dehydrogenase complex.
Starvation
Kinase activator protein mediates longer-term effects of starvation on activity of pyruvate dehydrogenase kinase in rat liver mitochondria.
Starvation
Pyruvate dehydrogenase activities and rates of lipogenesis during the fed-to-starved transition in liver and brown adipose tissue of the rat.
Starvation
Pyruvate dehydrogenase activities during the fed-to-starved transition and on re-feeding after acute or prolonged starvation.
Starvation
Reduction of mitochondrial pyruvate dehydrogenase phosphatase activity in lactating rat mammary gland following starvation or insulin deprivation.
Starvation
Regulation of pyruvate dehydrogenase (PDH) in the hibernating ground squirrel, (Ictidomys tridecemlineatus).
Stomach Neoplasms
A Retrospective Analysis for Patients with HER2-Positive Gastric Cancer Who Were Treated with Trastuzumab-Based Chemotherapy: In the Perspectives of Ethnicity and Histology.
Stomach Neoplasms
miR?21?5p targets PDHA1 to regulate glycolysis and cancer progression in gastric cancer.
Stroke
Combination therapy of normobaric oxygen with hypothermia or ethanol modulates pyruvate dehydrogenase complex in thromboembolic cerebral ischemia.
Thyroid Cancer, Papillary
[Effects of UHRF1 on Estrogen Receptor and Proliferation, Invasion and Migration of BCPAP Cells in Thyroid Papillary Carcinoma].
Thyroid Carcinoma, Anaplastic
Inactivation of PDH can Reduce Anaplastic Thyroid Cancer Cells' Sensitivity to Artemisinin.
Thyroiditis
Antibody binding and inhibition of pyruvate dehydrogenase (PDH) in sera from patients with primary biliary cirrhosis.
Tuberculosis
Amino-terminal residues 1-45 of the Escherichia coli pyruvate dehydrogenase complex E1 subunit interact with the E2 subunit and are required for activity of the complex but not for reductive acetylation of the E2 subunit.
Tuberculosis
Clinical and Laboratory Profile of Persons Living with Human Immunodeficiency Virus/Acquired Immune Deficiency Syndrome and Histoplasmosis from a Colombian Hospital.
Tuberculosis
Purification and characterization of a functionally active Mycobacterium tuberculosis prephenate dehydrogenase.
Urinary Bladder Neoplasms
The Role of Pyruvate Dehydrogenase Kinase-4 (PDK4) in Bladder Cancer and Chemoresistance.
Wasting Syndrome
Disseminated histoplasmosis in patients infected with human immunodeficiency virus.
[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 Korean female patient with thiamine-responsive pyruvate dehydrogenase complex deficiency due to a novel point mutation (Y161C)in the PDHA1 gene.
[pyruvate dehydrogenase (acetyl-transferring)]-phosphatase deficiency
Clinical exome sequencing reveals a mutation in PDHA1 in Leigh syndrome: A case of a Chinese boy with lethal neuropathy.
[pyruvate dehydrogenase (acetyl-transferring)]-phosphatase deficiency
Mutation analysis of the pyruvate dehydrogenase E1 alpha gene in eight patients with a pyruvate dehydrogenase complex deficiency.
[pyruvate dehydrogenase (acetyl-transferring)]-phosphatase deficiency
Mutational study in the PDHA1 gene of 40 patients suspected of pyruvate dehydrogenase complex deficiency.
[pyruvate dehydrogenase (acetyl-transferring)]-phosphatase deficiency
Somatic mosaicism for a novel PDHA1 mutation in a male with severe pyruvate dehydrogenase complex deficiency.
[pyruvate dehydrogenase (acetyl-transferring)]-phosphatase deficiency
Somatic mosaicism for PDHA1 mutation in a male with pyruvate dehydrogenase complex deficiency.
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
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UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable).
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable). ODPA_HUMAN
390
0
43296
Swiss-Prot
Mitochondrion (Reliability: 1)
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PDB
SCOP
CATH
UNIPROT
ORGANISM
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POSTTRANSLATIONAL MODIFICATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
phosphoprotein
the PDC is tightly regulated by reversible phosphorylation at three known phosphorylation sites on PDHE1a: Ser293, Ser300, and Ser232
phosphorylation
phosphorylation of Ser264 slows the preceding binding of substrate to the enzyme's active site via the substrate channel and the subsequent reductive acetylation of the E2 component
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CRYSTALLIZATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
vapour diffusion method with 14-18% PEG 3350, 0.1 mM sodium azide, and 200 mM NaSCN in 50 mM potassium phosphate (pH 8.0)
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PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
S264E
pseudophosphorylation mutant, the preceding binding of substrate to the enzyme's active site via the substrate channel and the subsequent reductive acetylation of the E2 component are severely slowed in the mutant enzyme
S264Q
selectively deficient in pyruvate binding and reductive acetylation of component E2
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CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Seifert, F.; Ciszak, E.; Korotchkina, L.; Golbik, R.; Spinka, M.; Dominiak, P.; Sidhu, S.; Brauer, J.; Patel, M.S.; Tittmann, K.
Phosphorylation of serine 264 impedes active site accessibility in the E1 component of the human pyruvate dehydrogenase multienzyme complex
Biochemistry
46
6277-6287
2007
Homo sapiens (P08559), Homo sapiens
brenda
Rardin, M.J.; Wiley, S.E.; Naviaux, R.K.; Murphy, A.N.; Dixon, J.E.
Monitoring phosphorylation of the pyruvate dehydrogenase complex
Anal. Biochem.
389
157-164
2009
Mus musculus, Homo sapiens (P08559)
brenda
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Release 2023.1 (January 2023)
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