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CAS No: 3206-73-3 Catalog No: AG003PT7 MDL No:
Title | Journal |
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Lipoamide channel-binding sulfonamides selectively inhibit mycobacterial lipoamide dehydrogenase. | Biochemistry 20131223 |
Radical reactions of thiamin pyrophosphate in 2-oxoacid oxidoreductases. | Biochimica et biophysica acta 20121101 |
Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy. | Science (New York, N.Y.) 20121019 |
Neuroscience. Preventable forms of autism? | Science (New York, N.Y.) 20121019 |
Pyruvate dehydrogenase complex: mRNA and protein expression patterns of E1α subunit genes in human spermatogenesis. | Gene 20120910 |
In situ regeneration of NADH via lipoamide dehydrogenase-catalyzed electron transfer reaction evidenced by spectroelectrochemistry. | Bioelectrochemistry (Amsterdam, Netherlands) 20120801 |
Quantitative imaging of electron transfer flavoprotein autofluorescence reveals the dynamics of lipid partitioning in living pancreatic islets. | Integrative biology : quantitative biosciences from nano to macro 20120801 |
Mapping an X-linked locus that influences heat-induced febrile seizures in mice. | Epilepsia 20120801 |
Tissue-specific and nutrient regulation of the branched-chain α-keto acid dehydrogenase phosphatase, protein phosphatase 2Cm (PP2Cm). | The Journal of biological chemistry 20120706 |
Prospective study of changes in the metabolomic profiles of men during their first three months of androgen deprivation therapy for prostate cancer. | Clinical cancer research : an official journal of the American Association for Cancer Research 20120701 |
Thiamine and Parkinson's disease. | Journal of the neurological sciences 20120515 |
A case of pyruvate dehydrogenase E1α subunit deficiency with antenatal brain dysgenesis demonstrated by prenatal sonography and magnetic resonance imaging. | Journal of clinical ultrasound : JCU 20120501 |
Unilateral periventricular leukomalacia in association with pyruvate dehydrogenase deficiency. | Developmental medicine and child neurology 20120501 |
Intermittent-relapsing pyruvate dehydrogenase complex deficiency: a case with clinical, biochemical, and neuroradiological reversibility. | Developmental medicine and child neurology 20120501 |
Pyruvate dehydrogenase deficiency and the brain. | Developmental medicine and child neurology 20120501 |
Two novel mutations in the BCKDHB gene (R170H, Q346R) cause the classic form of maple syrup urine disease (MSUD). | Gene 20120425 |
Predicting the fission yeast protein interaction network. | G3 (Bethesda, Md.) 20120401 |
Probing lactate dehydrogenase activity in tumors by measuring hydrogen/deuterium exchange in hyperpolarized l-[1-(13)C,U-(2)H]lactate. | Journal of the American Chemical Society 20120314 |
Beneficial effect of pyruvate therapy on Leigh syndrome due to a novel mutation in PDH E1α gene. | Brain & development 20120201 |
A-MYB (MYBL1) stimulates murine testis-specific Ldhc expression via the cAMP-responsive element (CRE) site. | Biology of reproduction 20120201 |
Five new amicoumacins isolated from a marine-derived bacterium Bacillus subtilis. | Marine drugs 20120201 |
Contrasting effects of selenite and tellurite on lipoamide dehydrogenase activity suggest a different biological behaviour of the two chalcogens. | Archives of biochemistry and biophysics 20120101 |
Mitochondria-ros crosstalk in the control of cell death and aging. | Journal of signal transduction 20120101 |
Differential expression of iron acquisition genes by Brucella melitensis and Brucella canis during macrophage infection. | PloS one 20120101 |
Differences in nitrogen metabolism between Cryptococcus neoformans and C. gattii, the two etiologic agents of cryptococcosis. | PloS one 20120101 |
Understanding delayed T-cell priming, lung recruitment, and airway luminal T-cell responses in host defense against pulmonary tuberculosis. | Clinical & developmental immunology 20120101 |
Demethylation of the coding region triggers the activation of the human testis-specific PDHA2 gene in somatic tissues. | PloS one 20120101 |
Arsenic toxicity: the effects on plant metabolism. | Frontiers in physiology 20120101 |
Analysis of the organic hydroperoxide response of Chromobacterium violaceum reveals that OhrR is a cys-based redox sensor regulated by thioredoxin. | PloS one 20120101 |
Lipoic acid synthetase deficiency causes neonatal-onset epilepsy, defective mitochondrial energy metabolism, and glycine elevation. | American journal of human genetics 20111209 |
Molecular characterization of 82 patients with pyruvate dehydrogenase complex deficiency. Structural implications of novel amino acid substitutions in E1 protein. | Molecular genetics and metabolism 20111201 |
Activation of α-keto acid-dependent dioxygenases: application of an {FeNO}7/{FeO2}8 methodology for characterizing the initial steps of O2 activation. | Journal of the American Chemical Society 20111116 |
AMP-activated protein kinase and metabolic regulation in cold-hardy insects. | Journal of insect physiology 20111101 |
Mapping the lipoylation site of Arabidopsis thaliana plastidial dihydrolipoamide S-acetyltransferase using mass spectrometry and site-directed mutagenesis. | Plant physiology and biochemistry : PPB 20111101 |
Increased superoxide accumulation in pyruvate dehydrogenase complex deficient fibroblasts. | Molecular genetics and metabolism 20111101 |
Emerging perspectives on essential amino acid metabolism in obesity and the insulin-resistant state. | Advances in nutrition (Bethesda, Md.) 20111101 |
Dihydroquinazolines as a novel class of Trypanosoma brucei trypanothione reductase inhibitors: discovery, synthesis, and characterization of their binding mode by protein crystallography. | Journal of medicinal chemistry 20111013 |
Growth factor withdrawal in combination with sodium butyrate addition extends culture longevity and enhances antibody production in CHO cells. | Journal of biotechnology 20110910 |
Use of the valine biosynthetic pathway to convert glucose into isobutanol. | Journal of industrial microbiology & biotechnology 20110901 |
Exercise-induced pyruvate dehydrogenase activation is not affected by 7 days of bed rest. | Journal of applied physiology (Bethesda, Md. : 1985) 20110901 |
[Role of lipoic acid in health and disease]. | Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego 20110901 |
Differential expression of Trypanosoma cruzi I associated with clinical forms of Chagas disease: overexpression of oxidative stress proteins in acute patient isolate. | Journal of proteomics 20110824 |
Mycobacterium tuberculosislpdC, Rv0462, induces dendritic cell maturation and Th1 polarization. | Biochemical and biophysical research communications 20110805 |
Stimulation of reactive oxygen species generation by disease-causing mutations of lipoamide dehydrogenase. | Human molecular genetics 20110801 |
Lipoamide dehydrogenase is essential for both bloodstream and procyclic Trypanosoma brucei. | Molecular microbiology 20110801 |
Lipoic acid stimulates cAMP production via G protein-coupled receptor-dependent and -independent mechanisms. | The Journal of nutritional biochemistry 20110701 |
MRI features of 4 female patients with pyruvate dehydrogenase E1 alpha deficiency. | Pediatric neurology 20110701 |
Glycine cleavage enzyme complex: molecular cloning and expression of the H-protein cDNA from cultured human skin fibroblasts. | Biochemistry and cell biology = Biochimie et biologie cellulaire 20110601 |
Detection of DNA hybridisation in a diluted serum matrix by surface plasmon resonance and film bulk acoustic resonators. | Analytical and bioanalytical chemistry 20110501 |
Substrate and inhibitor specificities differ between human cytosolic and mitochondrial thioredoxin reductases: Implications for development of specific inhibitors. | Free radical biology & medicine 20110315 |
Overexpression of alpha-ketoglutarate dehydrogenase in Yarrowia lipolytica and its effect on production of organic acids. | Applied microbiology and biotechnology 20110301 |
Lipoamide or lipoic acid stimulates mitochondrial biogenesis in 3T3-L1 adipocytes via the endothelial NO synthase-cGMP-protein kinase G signalling pathway. | British journal of pharmacology 20110301 |
A high-fat diet elicits differential responses in genes coordinating oxidative metabolism in skeletal muscle of lean and obese individuals. | The Journal of clinical endocrinology and metabolism 20110301 |
In vivo neuroprotective effect of L-carnitine against oxidative stress in maple syrup urine disease. | Metabolic brain disease 20110301 |
Phenylbutyrate therapy for maple syrup urine disease. | Human molecular genetics 20110215 |
Successful TAT-mediated enzyme replacement therapy in a mouse model of mitochondrial E3 deficiency. | Journal of molecular medicine (Berlin, Germany) 20110201 |
Virulence of Mycobacterium tuberculosis depends on lipoamide dehydrogenase, a member of three multienzyme complexes. | Cell host & microbe 20110120 |
Toxin-induced and genetic animal models of Parkinson's disease. | Parkinson's disease 20110101 |
Mitochondrial dysfunction in Parkinson's disease: pathogenesis and neuroprotection. | Parkinson's disease 20110101 |
Animal products, diseases and drugs: a plea for better integration between agricultural sciences, human nutrition and human pharmacology. | Lipids in health and disease 20110101 |
Obesity resistant mechanisms in the Lean polygenic mouse model as indicated by liver transcriptome and expression of selected genes in skeletal muscle. | BMC genomics 20110101 |
Lipoic acid: energy metabolism and redox regulation of transcription and cell signaling. | Journal of clinical biochemistry and nutrition 20110101 |
Genomic stability over 9 years of an isoniazid resistant Mycobacterium tuberculosis outbreak strain in Sweden. | PloS one 20110101 |
The self-organizing fractal theory as a universal discovery method: the phenomenon of life. | Theoretical biology & medical modelling 20110101 |
Modulation of gene expression in heart and liver of hibernating black bears (Ursus americanus). | BMC genomics 20110101 |
Two patients with clinically distinct manifestation of pyruvate dehydrogenase deficiency due to mutations in PDHA1 gene. | Prague medical report 20110101 |
Zinc oxide nanoparticles as selective killers of proliferating cells. | International journal of nanomedicine 20110101 |
VapC toxins from Mycobacterium tuberculosis are ribonucleases that differentially inhibit growth and are neutralized by cognate VapB antitoxins. | PloS one 20110101 |
Three Korean patients with maple syrup urine disease: four novel mutations in the BCKDHA gene. | Annals of clinical and laboratory science 20110101 |
An Examination by Site-Directed Mutagenesis of Putative Key Residues in the Determination of Coenzyme Specificity in Clostridial NAD-Dependent Glutamate Dehydrogenase. | Enzyme research 20110101 |
Brain region specific mitophagy capacity could contribute to selective neuronal vulnerability in Parkinson's disease. | Proteome science 20110101 |
Novel Conjugates of 1,3-Diacylglycerol and Lipoic Acid: Synthesis, DPPH Assay, and RP-LC-MS-APCI Analysis. | Journal of lipids 20110101 |
Critical assessment of human metabolic pathway databases: a stepping stone for future integration. | BMC systems biology 20110101 |
Serine biosynthesis with one carbon catabolism and the glycine cleavage system represents a novel pathway for ATP generation. | PloS one 20110101 |
Structural-functional characterization and physiological significance of ferredoxin-NADP reductase from Xanthomonas axonopodis pv. citri. | PloS one 20110101 |
Diabetes and alpha lipoic Acid. | Frontiers in pharmacology 20110101 |
Identification of two novel BCKDHA mutations in a Chinese patient with maple syrup urine disease. | Journal of pediatric endocrinology & metabolism : JPEM 20110101 |
Radiation-induced signaling results in mitochondrial impairment in mouse heart at 4 weeks after exposure to X-rays. | PloS one 20110101 |
Dietary lipoic acid influences antioxidant capability and oxidative status of broilers. | International journal of molecular sciences 20110101 |
Nutrigenetics, nutrigenomics, and selenium. | Frontiers in genetics 20110101 |
Functional proteomic analysis for regulatory T cell surveillance of the HIV-1-infected macrophage. | Journal of proteome research 20101203 |
Is it time to reassess alpha lipoic acid and niacinamide therapy in schizophrenia? | Medical hypotheses 20101201 |
PGC-1alpha increases PDH content but does not change acute PDH regulation in mouse skeletal muscle. | American journal of physiology. Regulatory, integrative and comparative physiology 20101101 |
Liver-specific pyruvate dehydrogenase complex deficiency upregulates lipogenesis in adipose tissue and improves peripheral insulin sensitivity. | Lipids 20101101 |
Synthesis and SAR studies of 1,4-benzoxazine MenB inhibitors: novel antibacterial agents against Mycobacterium tuberculosis. | Bioorganic & medicinal chemistry letters 20101101 |
Deletion at chromosomal band Xp22.12-Xp22.13 involving PDHA1 in a patient with congenital lactic acidosis. | Molecular genetics and metabolism 20100901 |
Improved functionality of antibody-colloidal gold conjugates with the aid of lipoamide-grafted N-[tris(hydroxymethyl)methyl]acrylamide polymers. | Journal of colloid and interface science 20100801 |
A stable and controllable Prussian blue layer electrodeposited on self-assembled monolayers for constructing highly sensitive glucose biosensor. | The Analyst 20100801 |
Somatic mosaicism for PDHA1 mutation in a male with pyruvate dehydrogenase complex deficiency. | Molecular genetics and metabolism 20100701 |
Disruption of ptLPD1 or ptLPD2, genes that encode isoforms of the plastidial lipoamide dehydrogenase, confers arsenate hypersensitivity in Arabidopsis. | Plant physiology 20100701 |
A technical surgery for refractory gastroparesis in a patient with a mitochondrial disorder. | Pediatric surgery international 20100601 |
Solution structure and characterisation of the human pyruvate dehydrogenase complex core assembly. | Journal of molecular biology 20100528 |
Mutational study in the PDHA1 gene of 40 patients suspected of pyruvate dehydrogenase complex deficiency. | Clinical genetics 20100501 |
A combination of alpha lipoic acid and calcium hydroxycitrate is efficient against mouse cancer models: preliminary results. | Oncology reports 20100501 |
Protection by Exogenously Added Coenzyme Q(9) against Free Radical-Induced Injuries in Human Liver Cells. | Journal of clinical biochemistry and nutrition 20100501 |
Communication between thiamin cofactors in the Escherichia coli pyruvate dehydrogenase complex E1 component active centers: evidence for a 'direct pathway' between the 4'-aminopyrimidine N1' atoms. | The Journal of biological chemistry 20100409 |
Human testis-specific PDHA2 gene: methylation status of a CpG island in the open reading frame correlates with transcriptional activity. | Molecular genetics and metabolism 20100401 |
Analysis of branched-chain alpha-keto acid dehydrogenase complex activity in rat tissues using alpha-keto[1-13C]isocaproate as substrate. | Analytical biochemistry 20100401 |
DNA carrier testing and newborn screening for maple syrup urine disease in Old Order Mennonite communities. | Genetic testing and molecular biomarkers 20100401 |
The effects of branched-chain amino acid interactions on growth performance, blood metabolites, enzyme kinetics and transcriptomics in weaned pigs. | The British journal of nutrition 20100401 |
Triazaspirodimethoxybenzoyls as selective inhibitors of mycobacterial lipoamide dehydrogenase . | Biochemistry 20100302 |
Transhydrogenase and the anaerobic mitochondrial metabolism of adult Hymenolepis diminuta. | Parasitology 20100301 |
Gene cloning and characterization of dihydrolipoamide dehydrogenase from Microbacterium luteolum: A useful enzymatic regeneration system of NAD+ from NADH. | Journal of bioscience and bioengineering 20100301 |
Regulation of branched-chain amino acid catabolism: glucose limitation enhances the component of isovalerylspiramycin for the bitespiramycin production. | Bioprocess and biosystems engineering 20100201 |
Cytokines interleukin-1beta and tumor necrosis factor-alpha regulate different transcriptional and alternative splicing networks in primary beta-cells. | Diabetes 20100201 |
Multiple roles of PPARalpha in brown adipose tissue under constitutive and cold conditions. | Genes to cells : devoted to molecular & cellular mechanisms 20100201 |
Plasmodium pyruvate dehydrogenase activity is only essential for the parasite's progression from liver infection to blood infection. | Molecular microbiology 20100201 |
JS-K, a nitric oxide prodrug, has enhanced cytotoxicity in colon cancer cells with knockdown of thioredoxin reductase 1. | PloS one 20100101 |
Mycobacterial nucleoside diphosphate kinase blocks phagosome maturation in murine RAW 264.7 macrophages. | PloS one 20100101 |
Neonatal maternal deprivation response and developmental changes in gene expression revealed by hypothalamic gene expression profiling in mice. | PloS one 20100101 |
Differential proteomic analysis of Arabidopsis thaliana genotypes exhibiting resistance or susceptibility to the insect herbivore, Plutella xylostella. | PloS one 20100101 |
Two-photon microscopy for non-invasive, quantitative monitoring of stem cell differentiation. | PloS one 20100101 |
Regulation of hemolysin expression and virulence of Staphylococcus aureus by a serine/threonine kinase and phosphatase. | PloS one 20100101 |
Water buffalo genome science comes of age. | International journal of biological sciences 20100101 |
An animal model of PDH deficiency using AAV8-siRNA vector-mediated knockdown of pyruvate dehydrogenase E1α. | Molecular genetics and metabolism 20100101 |
In vivo expression of innate immunity markers in patients with Mycobacterium tuberculosis infection. | BMC infectious diseases 20100101 |
Mitochondrial redox metabolism in trypanosomatids is independent of tryparedoxin activity. | PloS one 20100101 |
Coordinate Transcriptomic and Metabolomic Effects of the Insulin Sensitizer Rosiglitazone on Fundamental Metabolic Pathways in Liver, Soleus Muscle, and Adipose Tissue in Diabetic db/db Mice. | PPAR research 20100101 |
The LabelHash algorithm for substructure matching. | BMC bioinformatics 20100101 |
Omega-6 fat supplementation alters lipogenic gene expression in bovine subcutaneous adipose tissue. | Gene regulation and systems biology 20100101 |
Exogenous coenzyme Q10 modulates MMP-2 activity in MCF-7 cell line as a breast cancer cellular model. | Nutrition journal 20100101 |
A genomic perspective on the potential of Actinobacillus succinogenes for industrial succinate production. | BMC genomics 20100101 |
Complex and multidimensional lipid raft alterations in a murine model of Alzheimer's disease. | International journal of Alzheimer's disease 20100101 |
Adverse effect of fenofibrate on branched-chain alpha-ketoacid dehydrogenase complex in rat's liver. | Toxicology 20091221 |
Effect of extracellular osmolality on metabolism in contracting mammalian skeletal muscle in vitro. | Applied physiology, nutrition, and metabolism = Physiologie appliquee, nutrition et metabolisme 20091201 |
Lipoamide dehydrogenase and diaphorase catalyzed conversion of some NO donors to NO and reduction of NO scavenger 2-phenyl-4,4,5,5-tetramethylimidazoline-1-oxyl-3-oxide (PTIO). | General physiology and biophysics 20091201 |
Nitric oxide and dihydrolipoic acid modulate the activity of caspase 3 in HepG2 cells. | FEBS letters 20091103 |
AAV3-mediated transfer and expression of the pyruvate dehydrogenase E1 alpha subunit gene causes metabolic remodeling and apoptosis of human liver cancer cells. | Molecular genetics and metabolism 20091101 |
An anaerobic-type alpha-ketoglutarate ferredoxin oxidoreductase completes the oxidative tricarboxylic acid cycle of Mycobacterium tuberculosis. | PLoS pathogens 20091101 |
Antioxidant properties of an endogenous thiol: Alpha-lipoic acid, useful in the prevention of cardiovascular diseases. | Journal of cardiovascular pharmacology 20091101 |
Decreased enzyme activity and contents of hepatic branched-chain alpha-keto acid dehydrogenase complex subunits in a rat model for type 2 diabetes mellitus. | Metabolism: clinical and experimental 20091001 |
Maple syrup urine disease in Cypriot families: identification of three novel mutations and biochemical characterization of the p.Thr211Met mutation in the E1alpha subunit. | Genetic testing and molecular biomarkers 20091001 |
Chemical and functional aspects of posttranslational modification of proteins. | Acta naturae 20091001 |
Carbohydrate refeeding after a high-fat diet rapidly reverses the adaptive increase in human skeletal muscle PDH kinase activity. | American journal of physiology. Regulatory, integrative and comparative physiology 20090901 |
HIF-1 prevents the overproduction of mitochondrial ROS after cytokine stimulation through induction of PDK-1. | Cell cycle (Georgetown, Tex.) 20090901 |
Global analysis of extracytoplasmic stress signaling in Escherichia coli. | PLoS genetics 20090901 |
Hepatocyte transplantation improves phenotype and extends survival in a murine model of intermediate maple syrup urine disease. | Molecular therapy : the journal of the American Society of Gene Therapy 20090701 |
Chaperones of F1-ATPase. | The Journal of biological chemistry 20090619 |
Protein phosphatase 2Cm is a critical regulator of branched-chain amino acid catabolism in mice and cultured cells. | The Journal of clinical investigation 20090601 |
Subunit and catalytic component stoichiometries of an in vitro reconstituted human pyruvate dehydrogenase complex. | The Journal of biological chemistry 20090508 |
Protective effects of compound FLZ, a novel synthetic analogue of squamosamide, on beta-amyloid-induced rat brain mitochondrial dysfunction in vitro. | Acta pharmacologica Sinica 20090501 |
Revisiting MSUD in Portuguese Gypsies: evidence for a founder mutation and for a mutational hotspot within the BCKDHA gene. | Annals of human genetics 20090501 |
Reaction mechanisms of thiamin diphosphate enzymes: redox reactions. | The FEBS journal 20090501 |
Traumatic brain injury-induced expression and phosphorylation of pyruvate dehydrogenase: a mechanism of dysregulated glucose metabolism. | Neuroscience letters 20090417 |
Heterologous expression of pyruvate dehydrogenase E1 subunits of the microsporidium Paranosema (Antonospora) locustae and immunolocalization of the mitochondrial protein in amitochondrial cells. | FEMS microbiology letters 20090401 |
Animal models of maple syrup urine disease. | Journal of inherited metabolic disease 20090401 |
Modified method for purifying rat liver branched-chain alpha-ketoacid dehydrogenase complex. | Bioscience, biotechnology, and biochemistry 20090323 |
Changes in the effective gravitational field strength affect the state of phosphorylation of stress-related proteins in callus cultures of Arabidopsis thaliana. | Journal of experimental botany 20090301 |
The basal flux of Akt in the mitochondria is mediated by heat shock protein 90. | Journal of neurochemistry 20090301 |
Identification of additional players in the alternative biosynthesis pathway to isovaleryl-CoA in the myxobacterium Myxococcus xanthus. | Chembiochem : a European journal of chemical biology 20090105 |
RamB is an activator of the pyruvate dehydrogenase complex subunit E1p gene in Corynebacterium glutamicum. | Journal of molecular microbiology and biotechnology 20090101 |
Pyruvate dehydrogenase deficiency: identification of a novel mutation in the PDHA1 gene which responds to amino acid supplementation. | European journal of pediatrics 20090101 |
Production of L-malic acid with fixation of HCO3(-) by malic enzyme-catalyzed reaction based on regeneration of coenzyme on electrode modified by layer-by-layer self-assembly method. | Journal of bioscience and bioengineering 20090101 |
Isolation, sequence identification, and tissue expression profile of 3 novel porcine genes: NCF2, BCKDHB and BCKDHA. | Journal of applied genetics 20090101 |
Identification of IbeR as a stationary-phase regulator in meningitic Escherichia coli K1 that carries a loss-of-function mutation in rpoS. | Journal of biomedicine & biotechnology 20090101 |
Practical and reliable FRET/FLIM pair of fluorescent proteins. | BMC biotechnology 20090101 |
Molecular profiles of Quadriceps muscle in myostatin-null mice reveal PI3K and apoptotic pathways as myostatin targets. | BMC genomics 20090101 |
Knockout studies reveal an important role of Plasmodium lipoic acid protein ligase A1 for asexual blood stage parasite survival. | PloS one 20090101 |
Myocardial overexpression of Mecr, a gene of mitochondrial FAS II leads to cardiac dysfunction in mouse. | PloS one 20090101 |
Targeted tandem affinity purification of PSD-95 recovers core postsynaptic complexes and schizophrenia susceptibility proteins. | Molecular systems biology 20090101 |
Molecular genetics of maple syrup urine disease in the Turkish population. | The Turkish journal of pediatrics 20090101 |
Molecular cloning, sequence identification and tissue expression profile of three novel sheep (Ovis aries) genes - BCKDHA, NAGA and HEXA. | Biological research 20090101 |
Effect of CAR activation on selected metabolic pathways in normal and hyperlipidemic mouse livers. | BMC genomics 20090101 |
The amidase domain of lipoamidase specifically inactivates lipoylated proteins in vivo. | PloS one 20090101 |
Identification of novel androgen-responsive genes by sequencing of LongSAGE libraries. | BMC genomics 20090101 |
Transcription analysis of central metabolism genes in Escherichia coli. Possible roles of sigma38 in their expression, as a response to carbon limitation. | PloS one 20090101 |
An insight into the role of phosphotransacetylase (pta) and the acetate/acetyl-CoA node in Escherichia coli. | Microbial cell factories 20090101 |
Snapshots of catalysis in the E1 subunit of the pyruvate dehydrogenase multienzyme complex. | Structure (London, England : 1993) 20081212 |
Structural basis for inactivation of the human pyruvate dehydrogenase complex by phosphorylation: role of disordered phosphorylation loops. | Structure (London, England : 1993) 20081210 |
Impaired oxidation of branched-chain amino acids in the medial thalamus of thiamine-deficient rats. | Metabolic brain disease 20081201 |
The involvement of the pyruvate dehydrogenase E1alpha subunit, in Streptococcus mutans acid tolerance. | FEMS microbiology letters 20081201 |
Maple syrup urine disease due to a new large deletion at BCKDHA caused by non-homologous recombination. | Journal of inherited metabolic disease 20081201 |
ATP-dependent ligases in trypanothione biosynthesis--kinetics of catalysis and inhibition by phosphinic acid pseudopeptides. | The FEBS journal 20081101 |
Skeletal muscle type comparison of pyruvate dehydrogenase phosphatase activity and isoform expression: effects of obesity and endurance training. | American journal of physiology. Regulatory, integrative and comparative physiology 20081001 |
Maple syrup urine disease in Thai infants. | Journal of the Medical Association of Thailand = Chotmaihet thangphaet 20081001 |
Potato skin proteome is enriched with plant defence components. | Journal of experimental botany 20080901 |
Somatic mosaicism for a PDHA1 mutation in a female with pyruvate dehydrogenase deficiency. | Human genetics 20080901 |
Regulation of branched-chain amino acid catabolism in rat models for spontaneous type 2 diabetes mellitus. | Biochemical and biophysical research communications 20080815 |
Hamster sperm capacitation: role of pyruvate dehydrogenase A and dihydrolipoamide dehydrogenase. | Biology of reproduction 20080801 |
Immunoadsorption therapy for patients with dilated cardiomyopathy and heart failure. | Current cardiology reviews 20080801 |
Cytotoxic interactions of methylene blue with trypanosomatid-specific disulfide reductases and their dithiol products. | Molecular and biochemical parasitology 20080701 |
Glycine cleavage system: reaction mechanism, physiological significance, and hyperglycinemia. | Proceedings of the Japan Academy. Series B, Physical and biological sciences 20080701 |
Role of lipoamide dehydrogenase and metallothionein on 1-methyl-4-phenyl-1,2,3,6- tetrahydropyridine-induced neurotoxicity. | Neurochemical research 20080601 |
Structural bases for the specific interactions between the E2 and E3 components of the Thermus thermophilus 2-oxo acid dehydrogenase complexes. | Journal of biochemistry 20080601 |
Molecular and structural analyses of maple syrup urine disease and identification of a founder mutation in a Portuguese Gypsy community. | Molecular genetics and metabolism 20080601 |
Structure-function relationships in the 2-oxo acid dehydrogenase family: substrate-specific signatures and functional predictions for the 2-oxoglutarate dehydrogenase-like proteins. | Proteins 20080501 |
Neonatal pyruvate dehydrogenase deficiency due to a R302H mutation in the PDHA1 gene: MRI findings. | Pediatric radiology 20080501 |
Mutations of the E1beta subunit gene (PDHB) in four families with pyruvate dehydrogenase deficiency. | Molecular genetics and metabolism 20080401 |
A combined therapeutic approach for pyruvate dehydrogenase deficiency using self-complementary adeno-associated virus serotype-specific vectors and dichloroacetate. | Molecular genetics and metabolism 20080401 |
Lipoamide protects retinal pigment epithelial cells from oxidative stress and mitochondrial dysfunction. | Free radical biology & medicine 20080401 |
TAT-mediated delivery of LAD restores pyruvate dehydrogenase complex activity in the mitochondria of patients with LAD deficiency. | Molecular therapy : the journal of the American Society of Gene Therapy 20080401 |
Characterization of a dihydrolipoyl dehydrogenase having diaphorase activity of Clostridium kluyveri. | Bioscience, biotechnology, and biochemistry 20080401 |
Branched chain alpha-keto acid dehydrogenase, E1-beta subunit gene is associated with premature ovarian failure. | Fertility and sterility 20080301 |
Two silent substitutions in the PDHA1 gene cause exon 5 skipping by disruption of a putative exonic splicing enhancer. | Molecular genetics and metabolism 20080301 |
A putative exonic splicing enhancer in exon 7 of the PDHA1 gene affects splicing of adjacent exons. | Human mutation 20080301 |
Succinate dehydrogenase flavoprotein subunit expression in Saccharomyces cerevisiae--involvement of the mitochondrial FAD transporter, Flx1p. | The FEBS journal 20080301 |
Amino acids allosterically regulate the thiamine diphosphate-dependent alpha-keto acid decarboxylase from Mycobacterium tuberculosis. | The Journal of biological chemistry 20080227 |
Pyruvate dehydrogenase deficiency presenting as intermittent isolated acute ataxia. | Neuropediatrics 20080201 |
Pyruvate dehydrogenase complex deficiency caused by ubiquitination and proteasome-mediated degradation of the E1 subunit. | The Journal of biological chemistry 20080104 |
The acute effects of differential dietary fatty acids on human skeletal muscle pyruvate dehydrogenase activity. | Journal of applied physiology (Bethesda, Md. : 1985) 20080101 |
Regulation of PDH in human arm and leg muscles at rest and during intense exercise. | American journal of physiology. Endocrinology and metabolism 20080101 |
Protein interactions in human genetic diseases. | Genome biology 20080101 |
Cell death by SecTRAPs: thioredoxin reductase as a prooxidant killer of cells. | PloS one 20080101 |
Metabolic pathways of the wheat (Triticum aestivum) endosperm amyloplast revealed by proteomics. | BMC plant biology 20080101 |
Arsenic-based antineoplastic drugs and their mechanisms of action. | Metal-based drugs 20080101 |
Collagen density promotes mammary tumor initiation and progression. | BMC medicine 20080101 |
Depletion of Plasmodium berghei plasmoredoxin reveals a non-essential role for life cycle progression of the malaria parasite. | PloS one 20080101 |
Early vertebrate chromosome duplications and the evolution of the neuropeptide Y receptor gene regions. | BMC evolutionary biology 20080101 |
Genetic basis of virulence attenuation revealed by comparative genomic analysis of Mycobacterium tuberculosis strain H37Ra versus H37Rv. | PloS one 20080101 |
Articulation of three core metabolic processes in Arabidopsis: fatty acid biosynthesis, leucine catabolism and starch metabolism. | BMC plant biology 20080101 |
[The molecular background of Leigh syndrome]. | Neurologia i neurochirurgia polska 20080101 |
The genome-scale metabolic model iIN800 of Saccharomyces cerevisiae and its validation: a scaffold to query lipid metabolism. | BMC systems biology 20080101 |
Analysis of the goldfish Carassius auratus olfactory epithelium transcriptome reveals the presence of numerous non-olfactory GPCR and putative receptors for progestin pheromones. | BMC genomics 20080101 |
A critical role for glycine transporters in hyperexcitability disorders. | Frontiers in molecular neuroscience 20080101 |
The integrated analysis of metabolic and protein interaction networks reveals novel molecular organizing principles. | BMC systems biology 20080101 |
NADPH-dependent coenzyme Q reductase is the main enzyme responsible for the reduction of non-mitochondrial CoQ in cells. | BioFactors (Oxford, England) 20080101 |
Large-scale differential proteome analysis in Plasmodium falciparum under drug treatment. | PloS one 20080101 |
Obesity-related elevations in plasma leucine are associated with alterations in enzymes involved in branched-chain amino acid metabolism. | American journal of physiology. Endocrinology and metabolism 20071201 |
Structure of the branched-chain keto acid decarboxylase (KdcA) from Lactococcus lactis provides insights into the structural basis for the chemoselective and enantioselective carboligation reaction. | Acta crystallographica. Section D, Biological crystallography 20071201 |
Effects of zinc on SN56 cholinergic neuroblastoma cells. | Journal of neurochemistry 20071101 |
Description of the mutations in 15 subjects with variant forms of maple syrup urine disease. | Journal of inherited metabolic disease 20071101 |
Enzymatic characterization of dihydrolipoamide dehydrogenase from Streptococcus pneumoniae harboring its own substrate. | The Journal of biological chemistry 20071005 |
A sensor for superoxide in aqueous and organic/aqueous media based on immobilized cytochrome c on binary self-assembled monolayers. | Biosensors & bioelectronics 20070930 |
A dynamic loop at the active center of the Escherichia coli pyruvate dehydrogenase complex E1 component modulates substrate utilization and chemical communication with the E2 component. | The Journal of biological chemistry 20070921 |
Theoretical calculation of the binding free energies for pyruvate dehydrogenase E1 binding with ligands. | Bioorganic & medicinal chemistry letters 20070915 |
Four weeks one-leg training and high fat diet does not alter PPARalpha protein or mRNA expression in human skeletal muscle. | European journal of applied physiology 20070901 |
The quality control assessment of commercially available coenzyme q(10)-containing dietary and health supplements in Japan. | Journal of clinical biochemistry and nutrition 20070901 |
Zinc irreversibly damages major enzymes of energy production and antioxidant defense prior to mitochondrial permeability transition. | The Journal of biological chemistry 20070817 |
Lipoamide dehydrogenase mediates retention of coronin-1 on BCG vacuoles, leading to arrest in phagosome maturation. | Journal of cell science 20070815 |
E1 component of pyruvate dehydrogenase complex does not regulate the expression of NADPH-ferredoxin reductase in Azotobacter vinelandii. | FEMS microbiology letters 20070801 |
Recognition of the inner lipoyl-bearing domain of dihydrolipoyl transacetylase and of the blood glucose-lowering compound AZD7545 by pyruvate dehydrogenase kinase 2. | Biochemistry 20070724 |
Oxidative metabolism of pyruvate is required for meiotic maturation of murine oocytes in vivo. | Biology of reproduction 20070701 |
L-glutamine in vitro regulates rat aortic glutamate content and modulates nitric oxide formation and contractility responses. | American journal of physiology. Cell physiology 20070701 |
Down-regulation of expression of rat pyruvate dehydrogenase E1alpha gene by self-complementary adeno-associated virus-mediated small interfering RNA delivery. | Mitochondrion 20070701 |
Evolution of function in the 'two dinucleotide binding domains' flavoproteins. | PLoS computational biology 20070701 |
Targeting lipoic acid to mitochondria: synthesis and characterization of a triphenylphosphonium-conjugated alpha-lipoyl derivative. | Free radical biology & medicine 20070615 |
Identification of the bkdAB gene cluster, a plausible source of the starter-unit for virginiamycin M production in Streptomyces virginiae. | Archives of microbiology 20070601 |
Thioredoxin glutathione reductase from Schistosoma mansoni: an essential parasite enzyme and a key drug target. | PLoS medicine 20070601 |
[Leigh syndrome due to pyruvate dehydrogenase E1 alpha subunit gene mutation: a complicated and difficult case study]. | Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics 20070601 |
Inhibition of PI3-kinase signaling by glucocorticoids results in increased branched-chain amino acid degradation in renal epithelial cells. | American journal of physiology. Cell physiology 20070501 |
Protective effects of coenzyme q(10) on decreased oxidative stress resistance induced by simvastatin. | Journal of clinical biochemistry and nutrition 20070501 |
The two active sites in human branched-chain alpha-keto acid dehydrogenase operate independently without an obligatory alternating-site mechanism. | The Journal of biological chemistry 20070420 |
Transcriptional analysis of L-methionine catabolism in Brevibacterium linens ATCC9175. | Applied microbiology and biotechnology 20070401 |
Carnitine revisited: potential use as adjunctive treatment in diabetes. | Diabetologia 20070401 |
Clinical and molecular survey in 124 Chinese patients with Leigh or Leigh-like syndrome. | Journal of inherited metabolic disease 20070401 |
Lowered concentrations of branched-chain amino acids result in impaired growth and neurological problems: insights from a branched-chain alpha-keto acid dehydrogenase complex kinase-deficient mouse model. | Nutrition reviews 20070401 |
Molecular docking and three-dimensional quantitative structure-activity relationship studies on the binding modes of herbicidal 1-(substituted phenoxyacetoxy)alkylphosphonates to the E1 component of pyruvate dehydrogenase. | Journal of agricultural and food chemistry 20070307 |
Absence of an adipogenic effect of rosiglitazone on mature 3T3-L1 adipocytes: increase of lipid catabolism and reduction of adipokine expression. | Diabetologia 20070301 |
Lipoic acid as a novel treatment for Alzheimer's disease and related dementias. | Pharmacology & therapeutics 20070101 |
Linear array of conserved sequence motifs to discriminate protein subfamilies: study on pyridine nucleotide-disulfide reductases. | BMC bioinformatics 20070101 |
Alterations in cytoskeletal and immune function-related proteome profiles in whole rat lung following intratracheal instillation of heparin. | Respiratory research 20070101 |
Mapping phosphoproteins in Mycoplasma genitalium and Mycoplasma pneumoniae. | BMC microbiology 20070101 |
An analysis of expression patterns of genes encoding proteins with catalytic activities. | BMC genomics 20070101 |
Spectrum of disease-causing mutations in protein secondary structures. | BMC structural biology 20070101 |
Sensitive detection of p65 homodimers using red-shifted and fluorescent protein-based FRET couples. | PloS one 20070101 |
Single nucleotide polymorphism detection by polymerase chain reaction-restriction fragment length polymorphism. | Nature protocols 20070101 |
Polymorphisms in promoter regions of PDHB, SORBS1, and EDG1 genes showing marbling-associated expression changes. | Research communications in molecular pathology and pharmacology 20070101 |
Trypanothione reductase: a viable chemotherapeutic target for antitrypanosomal and antileishmanial drug design. | Drug target insights 20070101 |
A synchronized substrate-gating mechanism revealed by cubic-core structure of the bovine branched-chain alpha-ketoacid dehydrogenase complex. | The EMBO journal 20061213 |
Determinants of substrate specificity in KdcA, a thiamin diphosphate-dependent decarboxylase. | Bioorganic chemistry 20061201 |
Spectrally resolved time-correlated single photon counting: a novel approach for characterization of endogenous fluorescence in isolated cardiac myocytes. | European biophysics journal : EBJ 20061201 |
Spectroscopic and electronic structure studies of the role of active site interactions in the decarboxylation reaction of alpha-keto acid-dependent dioxygenases. | Journal of inorganic biochemistry 20061201 |
Impaired growth and neurological abnormalities in branched-chain alpha-keto acid dehydrogenase kinase-deficient mice. | The Biochemical journal 20061115 |
The case for regulating indispensable amino acid metabolism: the branched-chain alpha-keto acid dehydrogenase kinase-knockout mouse. | The Biochemical journal 20061115 |
Activity of pyruvate dehydrogenase A (PDHA) in hamster spermatozoa correlates positively with hyperactivation and is associated with sperm capacitation. | Biology of reproduction 20061101 |
PDH-E1alpha dephosphorylation and activation in human skeletal muscle during exercise: effect of intralipid infusion. | Diabetes 20061101 |
Changes in gene expression involved in energy utilization during chicken follicle development. | Animal reproduction science 20061001 |
A relevant in vitro eukaryotic live-cell system for the evaluation of plasmodial protein localization. | Biochimie 20061001 |
A Korean female patient with thiamine-responsive pyruvate dehydrogenase complex deficiency due to a novel point mutation (Y161C)in the PDHA1 gene. | Journal of Korean medical science 20061001 |
Structure of the subunit binding domain and dynamics of the di-domain region from the core of human branched chain alpha-ketoacid dehydrogenase complex. | The Journal of biological chemistry 20060922 |
Demonstration of PDC-E1 subunits as major antigens in the complement-fixing fraction M4 and re-evaluation of PDC-E1-specific antibodies in PBC patients. | Liver international : official journal of the International Association for the Study of the Liver 20060901 |
[Pyruvate dehydrogenase deficit associated to the C515T mutation in exon 6 of the E1alpha gene]. | Revista de neurologia 20060901 |
An NADH-tetrazolium-coupled sensitive assay for malate dehydrogenase in mitochondria and crude tissue homogenates. | Journal of biochemical and biophysical methods 20060831 |
Modifications of the lipoamide-containing mitochondrial subproteome in a yeast mutant defective in cysteine desulfurase. | Molecular & cellular proteomics : MCP 20060801 |
Clofibrate treatment promotes branched-chain amino acid catabolism and decreases the phosphorylation state of mTOR, eIF4E-BP1, and S6K1 in rat liver. | Life sciences 20060717 |
Mutational spectrum of maple syrup urine disease in Spain. | Human mutation 20060701 |
Cellular disulfide-reducing capacity: an integrated measure of cell redox capacity. | Biochemical and biophysical research communications 20060616 |
Hyperoxia decreases muscle glycogenolysis, lactate production, and lactate efflux during steady-state exercise. | American journal of physiology. Endocrinology and metabolism 20060601 |
Females with PDHA1 gene mutations: a diagnostic challenge. | Mitochondrion 20060601 |
Leucine-induced activation of translational initiation is partly regulated by the branched-chain alpha-keto acid dehydrogenase complex in C2C12 cells. | Biochemical and biophysical research communications 20060519 |
Effect of lpdA gene knockout on the metabolism in Escherichia coli based on enzyme activities, intracellular metabolite concentrations and metabolic flux analysis by 13C-labeling experiments. | Journal of biotechnology 20060323 |
[Preliminary proteomics analysis of the total proteins of HL Type cytoplasmic male sterility rice anther]. | Yi chuan = Hereditas 20060301 |
IgA class antibodies to 2-oxo-acid dehydrogenase complex are not predictive markers of histopathological progression in primary biliary cirrhosis. | Autoimmunity 20060301 |
Somatic mosaicism in a male with an exon skipping mutation in PDHA1 of the pyruvate dehydrogenase complex results in a milder phenotype. | Molecular genetics and metabolism 20060201 |
A versatile conformational switch regulates reactivity in human branched-chain alpha-ketoacid dehydrogenase. | Structure (London, England : 1993) 20060201 |
Biochemical and molecular diagnosis of lipoamide dehydrogenase deficiency in a North American Ashkenazi Jewish family. | Journal of inherited metabolic disease 20060201 |
Regulatory roles of the N-terminal domain based on crystal structures of human pyruvate dehydrogenase kinase 2 containing physiological and synthetic ligands. | Biochemistry 20060117 |
Hydrogen peroxide scavenging is not a virulence determinant in the pathogenesis of Haemophilus influenzae type b strain Eagan. | BMC microbiology 20060101 |
Production and characterization of murine models of classic and intermediate maple syrup urine disease. | BMC medical genetics 20060101 |
NAD(H) recycling activity of an engineered bifunctional enzyme galactose dehydrogenase/lactate dehydrogenase. | International journal of biological sciences 20060101 |
Melatonin in Alzheimer's disease and other neurodegenerative disorders. | Behavioral and brain functions : BBF 20060101 |
Mitochondrial oxidative stress and nitrate tolerance--comparison of nitroglycerin and pentaerithrityl tetranitrate in Mn-SOD+/- mice. | BMC cardiovascular disorders 20060101 |
Metabolic network driven analysis of genome-wide transcription data from Aspergillus nidulans. | Genome biology 20060101 |
Prooxidant cytotoxicity of chromate in mammalian cells: the opposite roles of DT-diaphorase and glutathione reductase. | Zeitschrift fur Naturforschung. C, Journal of biosciences 20060101 |
First characterization of a large deletion of the PDHA 1 gene. | Molecular genetics and metabolism 20051201 |
Human microRNA (miR29b) expression controls the amount of branched chain alpha-ketoacid dehydrogenase complex in a cell. | Human molecular genetics 20051115 |
Folding of the protein domain hbSBD. | Biophysical journal 20051101 |
Plasmodium falciparum possesses organelle-specific alpha-keto acid dehydrogenase complexes and lipoylation pathways. | Biochemical Society transactions 20051101 |
Crystal structure and functional analysis of lipoamide dehydrogenase from Mycobacterium tuberculosis. | The Journal of biological chemistry 20051007 |
Neuroprotective therapy in Parkinson's disease: current status and new directions from experimental and genetic clues. | Journal of clinical neurology (Seoul, Korea) 20051001 |
Pyruvate decarboxylase provides growing pollen tubes with a competitive advantage in petunia. | The Plant cell 20050801 |
Mycobacterium tuberculosis appears to lack alpha-ketoglutarate dehydrogenase and encodes pyruvate dehydrogenase in widely separated genes. | Molecular microbiology 20050801 |
'Pyruvate dehydrogenase deficiency presenting as dystonia and responding to levodopa'. | Developmental medicine and child neurology 20050701 |
Glutamate 636 of the Escherichia coli pyruvate dehydrogenase-E1 participates in active center communication and behaves as an engineered acetolactate synthase with unusual stereoselectivity. | The Journal of biological chemistry 20050603 |
pH-dependent substrate preference of pig heart lipoamide dehydrogenase varies with oligomeric state: response to mitochondrial matrix acidification. | The Journal of biological chemistry 20050422 |
Structure of P-protein of the glycine cleavage system: implications for nonketotic hyperglycinemia. | The EMBO journal 20050420 |
Activation of hepatic branched-chain alpha-keto acid dehydrogenase complex by tumor necrosis factor-alpha in rats. | Biochemical and biophysical research communications 20050325 |
A novel mutation in the dihydrolipoamide dehydrogenase E3 subunit gene (DLD) resulting in an atypical form of alpha-ketoglutarate dehydrogenase deficiency. | Human mutation 20050301 |
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. | European journal of pediatrics 20050201 |
Precursor and temperature modulation of fatty acid composition and growth of Listeria monocytogenes cold-sensitive mutants with transposon-interrupted branched-chain alpha-keto acid dehydrogenase. | Microbiology (Reading, England) 20050201 |
Short-term feeding of fish oil down-regulates the expression of pyruvate dehydrogenase E1 alpha subunit mRNA in mouse brain. | Bioscience, biotechnology, and biochemistry 20050201 |
Dissociation of branched-chain alpha-keto acid dehydrogenase kinase (BDK) from branched-chain alpha-keto acid dehydrogenase complex (BCKDC) by BDK inhibitors. | Journal of nutritional science and vitaminology 20050201 |
The human malaria parasite Plasmodium falciparum possesses two distinct dihydrolipoamide dehydrogenases. | Molecular microbiology 20050101 |
Microsporidian mitochondrial proteins: expression in Antonospora locustae spores and identification of genes coding for two further proteins. | The Journal of eukaryotic microbiology 20050101 |
Predictors of primary breast cancers responsiveness to preoperative epirubicin/cyclophosphamide-based chemotherapy: translation of microarray data into clinically useful predictive signatures. | Journal of translational medicine 20050101 |
[Alpha-lipoic--dihydrolipoic acids--active bioantioxidant and bioregulatory system]. | Ukrains'kyi biokhimichnyi zhurnal (1999 ) 20050101 |
Characterization of a new member of the flavoprotein disulfide reductase family of enzymes from Mycobacterium tuberculosis. | The Journal of biological chemistry 20041210 |
Molecular mechanism for regulation of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex by phosphorylation. | Structure (London, England : 1993) 20041201 |
Crystal structure of T-protein of the glycine cleavage system. Cofactor binding, insights into H-protein recognition, and molecular basis for understanding nonketotic hyperglycinemia. | The Journal of biological chemistry 20041126 |
Deficiency of pyruvate dehydrogenase caused by novel and known mutations in the E1alpha subunit. | American journal of medical genetics. Part A 20041115 |
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. | Biochemistry 20041109 |
Alpha lipoic acid inhibits human T-cell migration: implications for multiple sclerosis. | Journal of neuroscience research 20041101 |
Protein thiol modifications visualized in vivo. | PLoS biology 20041101 |
Biochemistry. How active sites communicate in thiamine enzymes. | Science (New York, N.Y.) 20041029 |
A molecular switch and proton wire synchronize the active sites in thiamine enzymes. | Science (New York, N.Y.) 20041029 |
Pyruvate dehydrogenase deficiency presenting as dystonia in childhood. | Developmental medicine and child neurology 20041001 |
Conformational stability and thermodynamic characterization of the lipoic acid bearing domain of human mitochondrial branched chain alpha-ketoacid dehydrogenase. | Protein science : a publication of the Protein Society 20040901 |
The molecular origin of the thiamine diphosphate-induced spectral bands of ThDP-dependent enzymes. | Proteins 20040801 |
Cross-talk between thiamin diphosphate binding and phosphorylation loop conformation in human branched-chain alpha-keto acid decarboxylase/dehydrogenase. | The Journal of biological chemistry 20040730 |
Quantitative NAD(P)H/flavoprotein autofluorescence imaging reveals metabolic mechanisms of pancreatic islet pyruvate response. | The Journal of biological chemistry 20040723 |
Intermolecular interactions in the AhpC/AhpD antioxidant defense system of Mycobacterium tuberculosis. | Archives of biochemistry and biophysics 20040701 |
Dihydrolipoamide dehydrogenase from porcine heart catalyzes NADH-dependent scavenging of nitric oxide. | FEBS letters 20040618 |
IAR4, a gene required for auxin conjugate sensitivity in Arabidopsis, encodes a pyruvate dehydrogenase E1alpha homolog. | Plant physiology 20040601 |
Glutamine synthesis in the developing porcine placenta. | Biology of reproduction 20040501 |
A proteomic investigation of drug-induced steatosis in rat liver. | Chemical research in toxicology 20040501 |
Photosensitized NADH formation system with multilayer TiO2 film. | Chemical communications (Cambridge, England) 20040407 |
Ligand-induced conformational changes and a reaction intermediate in branched-chain 2-oxo acid dehydrogenase (E1) from Thermus thermophilus HB8, as revealed by X-ray crystallography. | Journal of molecular biology 20040402 |
Diversity of L-leucine catabolism in various microorganisms involved in dairy fermentations, and identification of the rate-controlling step in the formation of the potent flavour component 3-methylbutanal. | Applied microbiology and biotechnology 20040401 |
Identification of 36 kDa phosphoprotein in fibrous sheath of hamster spermatozoa. | Comparative biochemistry and physiology. Part B, Biochemistry & molecular biology 20040401 |
Leucine toxicity in a neuronal cell model with inhibited branched chain amino acid catabolism. | Brain research. Molecular brain research 20040330 |
Structural determinants of enzyme binding affinity: the E1 component of pyruvate dehydrogenase from Escherichia coli in complex with the inhibitor thiamin thiazolone diphosphate. | Biochemistry 20040309 |
Effects of liver failure on branched-chain alpha-keto acid dehydrogenase complex in rat liver and muscle: comparison between acute and chronic liver failure. | Journal of hepatology 20040301 |
[Effects of bkdAB interruption on avermectin biosynthesis]. | Sheng wu gong cheng xue bao = Chinese journal of biotechnology 20040301 |
Organization of the cores of the mammalian pyruvate dehydrogenase complex formed by E2 and E2 plus the E3-binding protein and their capacities to bind the E1 and E3 components. | The Journal of biological chemistry 20040220 |
Lipoic acid-dependent oxidative catabolism of alpha-keto acids in mitochondria provides evidence for branched-chain amino acid catabolism in Arabidopsis. | Plant physiology 20040201 |
Effects of liver failure on the enzymes in the branched-chain amino acid catabolic pathway. | Biochemical and biophysical research communications 20040109 |
Mechanisms responsible for regulation of branched-chain amino acid catabolism. | Biochemical and biophysical research communications 20040109 |
Riboflavin uptake and FAD synthesis in Saccharomyces cerevisiae mitochondria: involvement of the Flx1p carrier in FAD export. | The Journal of biological chemistry 20040102 |
Branched-chain amino acid catabolism: unique segregation of pathway enzymes in organ systems and peripheral nerves. | American journal of physiology. Endocrinology and metabolism 20040101 |
Pyruvate dehydrogenase E1alpha subunit deficiency in a female patient: evidence of antenatal origin of brain damage and possible etiology of infantile spasms. | Brain & development 20040101 |
Flavoprotein disulfide reductases: advances in chemistry and function. | Progress in nucleic acid research and molecular biology 20040101 |
Protein requirements for endurance athletes. | Nutrition (Burbank, Los Angeles County, Calif.) 20040101 |
A simple and rapid enzymatic assay for the branched-chain alpha-ketoacid dehydrogenase complex using high-performance liquid chromatography. | Journal of inherited metabolic disease 20040101 |
Branched chain amino acids (BCAAs) in heart diseases (ischaemic heart disease and myocardial infarction). | Annales Universitatis Mariae Curie-Sklodowska. Sectio D: Medicina 20040101 |
A pathogenic glutamate-to-aspartate substitution (D296E) in the pyruvate dehydrogenase E1 subunit gene PDHA1. | Human mutation 20031201 |
Markers of inflammation, proteolysis, and apoptosis in ESRD. | American journal of kidney diseases : the official journal of the National Kidney Foundation 20031201 |
Assembly of the covalent linkage between lipoic acid and its cognate enzymes. | Chemistry & biology 20031201 |
Protein components of mitochondrial DNA nucleoids in higher eukaryotes. | Molecular & cellular proteomics : MCP 20031101 |
Identification of twelve novel mutations in patients with classic and variant forms of maple syrup urine disease. | Human mutation 20031101 |
NarE: a novel ADP-ribosyltransferase from Neisseria meningitidis. | Molecular microbiology 20031101 |
Roles of His291-alpha and His146-beta' in the reductive acylation reaction catalyzed by human branched-chain alpha-ketoacid dehydrogenase: refined phosphorylation loop structure in the active site. | The Journal of biological chemistry 20031031 |
Premorbid weight, body mass, and varsity athletics in ALS. | Neurology 20031014 |
Epitope mapping on E1alpha subunit of pyruvate dehydrogenase complex with autoantibodies of patients with primary biliary cirrhosis. | Liver international : official journal of the International Association for the Study of the Liver 20031001 |
Towards a phosphoproteome map of Corynebacterium glutamicum. | Proteomics 20030801 |
Phosphorylation of formate dehydrogenase in potato tuber mitochondria. | The Journal of biological chemistry 20030711 |
Impairment of glucose metabolism and energy transfer in the rat heart. | Molecular and cellular biochemistry 20030701 |
Reaction mechanism of the heterotetrameric (alpha2beta2) E1 component of 2-oxo acid dehydrogenase multienzyme complexes. | Biochemistry 20030617 |
Structural basis for flip-flop action of thiamin pyrophosphate-dependent enzymes revealed by human pyruvate dehydrogenase. | The Journal of biological chemistry 20030606 |
MR diffusion imaging and MR spectroscopy of maple syrup urine disease during acute metabolic decompensation. | Neuroradiology 20030601 |
Aerobic growth deficient Haemophilus influenzae mutants are non-virulent: implications on metabolism. | International journal of medical microbiology : IJMM 20030601 |
Prevention of oxidant-induced cell death by lysosomotropic iron chelators. | Free radical biology & medicine 20030515 |
Differential effect of DCA treatment on the pyruvate dehydrogenase complex in patients with severe PDHC deficiency. | Pediatric research 20030501 |
Splicing error in E1alpha pyruvate dehydrogenase mRNA caused by novel intronic mutation responsible for lactic acidosis and mental retardation. | The Journal of biological chemistry 20030404 |
Antisense inhibition of mitochondrial pyruvate dehydrogenase E1alpha subunit in anther tapetum causes male sterility. | The Plant journal : for cell and molecular biology 20030401 |
2-Oxo acid dehydrogenase complexes of multicellular organisms. | European journal of biochemistry 20030301 |
2-Oxo acid dehydrogenase complexes in redox regulation. | European journal of biochemistry 20030301 |
Identification of 36-kDa flagellar phosphoproteins associated with hamster sperm motility. | Journal of biochemistry 20030301 |
Catalysis of diaphorase reactions by Mycobacterium tuberculosis lipoamide dehydrogenase occurs at the EH4 level. | Biochemistry 20030225 |
Selenite sensitizes mitochondrial permeability transition pore opening in vitro and in vivo: a possible mechanism for chemo-protection. | The Biochemical journal 20030215 |
A new case of pyruvate dehydrogenase deficiency due to a novel mutation in the PDX1 gene. | Annals of neurology 20030201 |
Encapsulation of an 86-kDa assembly intermediate inside the cavities of GroEL and its single-ring variant SR1 by GroES. | The Journal of biological chemistry 20030124 |
[Arsenite-induced lipid peroxidation in Saccharomyces cerevisiae]. | Mikrobiologiia 20030101 |
Regeneration of the antioxidant ubiquinol by lipoamide dehydrogenase, thioredoxin reductase and glutathione reductase. | BioFactors (Oxford, England) 20030101 |
Biochemical characterization of two mutants of human pyruvate dehydrogenase, F205L and T231A of the E1alpha subunit. | Journal of inherited metabolic disease 20030101 |
Antioxidant activities of novel alpha-lipoic acid derivatives: N-(6, 8-dimercaptooctanoyl)-2-aminoethanesulfonate- and N-(6, 8-dimercaptooctanoyl)-L-aspartate-zinc complex. | Research communications in molecular pathology and pharmacology 20030101 |
Histidine 407, a phantom residue in the E1 subunit of the Escherichia coli pyruvate dehydrogenase complex, activates reductive acetylation of lipoamide on the E2 subunit. An explanation for conservation of active sites between the E1 subunit and transketolase. | Biochemistry 20021231 |
The lipoamide dehydrogenase from Mycobacterium tuberculosis permits the direct observation of flavin intermediates in catalysis. | Biochemistry 20021210 |
Clofibric acid stimulates branched-chain amino acid catabolism by three mechanisms. | Archives of biochemistry and biophysics 20021115 |
Elongation factor Tu and E1 beta subunit of pyruvate dehydrogenase complex act as fibronectin binding proteins in Mycoplasma pneumoniae. | Molecular microbiology 20021101 |
Folding kinetics of the lipoic acid-bearing domain of human mitochondrial branched chain alpha-ketoacid dehydrogenase complex. | FEBS letters 20021023 |
Tissue-specific effects of chronic dietary leucine and norleucine supplementation on protein synthesis in rats. | American journal of physiology. Endocrinology and metabolism 20021001 |
Insulin increases branched-chain alpha-ketoacid dehydrogenase kinase expression in Clone 9 rat cells. | American journal of physiology. Endocrinology and metabolism 20021001 |
Inactivation of the 2-oxo acid dehydrogenase complexes upon generation of intrinsic radical species. | European journal of biochemistry 20021001 |
Diagnosis and molecular analysis of three male patients with thiamine-responsive pyruvate dehydrogenase complex deficiency. | Journal of the neurological sciences 20020915 |
Gene therapy for pyruvate dehydrogenase E1alpha deficiency using recombinant adeno-associated virus 2 (rAAV2) vectors. | Molecular therapy : the journal of the American Society of Gene Therapy 20020901 |
Glutaredoxins catalyze the reduction of glutathione by dihydrolipoamide with high efficiency. | Biochemical and biophysical research communications 20020802 |
A novel Y243S mutation in the pyruvate dehydrogenase El alpha gene subunit: correlation with thiamine pyrophosphate interaction. | Journal of inherited metabolic disease 20020801 |
Detection of pyruvate dehydrogenase E1 alpha-subunit deficiencies in females by immunohistochemical demonstration of mosaicism in cultured fibroblasts. | The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society 20020701 |
Interchangeable enzyme modules. Functional replacement of the essential linker of the biotinylated subunit of acetyl-CoA carboxylase with a linker from the lipoylated subunit of pyruvate dehydrogenase. | The Journal of biological chemistry 20020621 |
Primary biliary cirrhosis: seeking the silent partner of autoimmunity. | Gut 20020601 |
Diagnosis and treatment of maple syrup disease: a study of 36 patients. | Pediatrics 20020601 |
Solution structure and dynamics of the lipoic acid-bearing domain of human mitochondrial branched-chain alpha-keto acid dehydrogenase complex. | The Journal of biological chemistry 20020503 |
Two-photon fluorescence spectroscopy and microscopy of NAD(P)H and flavoprotein. | Biophysical journal 20020501 |
The E1beta and E2 subunits of the Bacillus subtilis pyruvate dehydrogenase complex are involved in regulation of sporulation. | Journal of bacteriology 20020501 |
Maple syrup urine disease: mutation analysis in Turkish patients. | Journal of inherited metabolic disease 20020501 |
Recurrent intragenomic recombination leading to sequence homogenization during the evolution of the lipoyl-binding domain. | FEMS microbiology letters 20020409 |
[Pyruvate dehydrogenase complex deficiency]. | Nihon rinsho. Japanese journal of clinical medicine 20020401 |
[Maple syrup urine disease]. | Nihon rinsho. Japanese journal of clinical medicine 20020401 |
Substrate channelling in 2-oxo acid dehydrogenase multienzyme complexes. | Biochemical Society transactions 20020401 |
Cerebral white matter involvement in children with mitochondrial encephalopathies. | Neuropediatrics 20020401 |
Zinc is a potent inhibitor of thiol oxidoreductase activity and stimulates reactive oxygen species production by lipoamide dehydrogenase. | The Journal of biological chemistry 20020322 |
Glucose-stimulated insulin secretion does not require activation of pyruvate dehydrogenase: impact of adenovirus-mediated overexpression of PDH kinase and PDH phosphate phosphatase in pancreatic islets. | Biochemical and biophysical research communications 20020308 |
Out of Africa again and again. | Nature 20020307 |
Glycogen phosphorylase and pyruvate dehydrogenase transformation in white muscle of trout during high-intensity exercise. | American journal of physiology. Regulatory, integrative and comparative physiology 20020301 |
Activation of the promoters of Arabidopsis genes for the branched-chain alpha-keto acid dehydrogenase complex in transgenic tobacco BY-2 cells under sugar starvation. | Plant & cell physiology 20020301 |
Clinical significance of positive immunoblotting but negative immunofluorescence for antimitochondrial antibodies in patients with liver diseases other than primary biliary cirrhosis. | Autoimmunity 20020301 |
Metabolic enzymes of mycobacteria linked to antioxidant defense by a thioredoxin-like protein. | Science (New York, N.Y.) 20020208 |
Pyruvate dehydrogenase E3 binding protein deficiency. | Human genetics 20020201 |
Structural basis of the dysfunctioning of human 2-oxo acid dehydrogenase complexes. | Current medicinal chemistry 20020201 |
Characterization of a missense mutation at histidine-44 in a pyruvate dehydrogenase-deficient patient. | Biochimica et biophysica acta 20020102 |
Evidence of common ancestry for the maple syrup urine disease (MSUD) Y438N allele in non-Mennonite MSUD patients. | Molecular genetics and metabolism 20020101 |
Atrophic corpus gastritis and Helicobacter pylori infection in primary biliary cirrhosis. | Digestive diseases and sciences 20020101 |
The complex fate of alpha-ketoacids. | Annual review of plant biology 20020101 |
[Inherited metabolic diseases associated with West syndrome]. | Ryoikibetsu shokogun shirizu 20020101 |
Regulation of mammalian pyruvate dehydrogenase complex by phosphorylation: complexity of multiple phosphorylation sites and kinases. | Experimental & molecular medicine 20011231 |
3D structure and significance of the GPhiXXG helix packing motif in tetramers of the E1beta subunit of pyruvate dehydrogenase from the archeon Pyrobaculum aerophilum. | Biochemistry 20011204 |
Dietary L-carnitine suppresses mitochondrial branched-chain keto acid dehydrogenase activity and enhances protein accretion and carcass characteristics of swine. | Journal of animal science 20011201 |
Gender-specific occurrence of West syndrome in patients with pyruvate dehydrogenase complex deficiency. | Neuropediatrics 20011201 |
Primary biliary cirrhosis: lessons learned from an organ-specific disease. | Clinical and experimental medicine 20011201 |
Theoretical study of the conformation of the lipoamide arm in a mutant H protein. | Proteins 20011115 |
Uteroplacental insufficiency alters liver and skeletal muscle branched-chain amino acid metabolism in intrauterine growth-restricted fetal rats. | Pediatric research 20011101 |
Differential effects of two mutations at arginine-234 in the alpha subunit of human pyruvate dehydrogenase. | Archives of biochemistry and biophysics 20011101 |
Purification and characterization of an enzyme from Mycobacterium sp. Pyr-1, with nitroreductase activity and an N-terminal sequence similar to lipoamide dehydrogenase. | Archives of microbiology 20011101 |
Inactivation of the murine pyruvate dehydrogenase (Pdha1) gene and its effect on early embryonic development. | Molecular genetics and metabolism 20011101 |
The effect of high-intensity exhaustive exercise studied in isolated mitochondria from human skeletal muscle. | Pflugers Archiv : European journal of physiology 20011101 |
Natural osmolyte trimethylamine N-oxide corrects assembly defects of mutant branched-chain alpha-ketoacid decarboxylase in maple syrup urine disease. | The Journal of biological chemistry 20011026 |
A trail of research from lipoic acid to alpha-keto acid dehydrogenase complexes. | The Journal of biological chemistry 20011019 |
Maple syrup urine disease: identification and carrier-frequency determination of a novel founder mutation in the Ashkenazi Jewish population. | American journal of human genetics 20011001 |
Characterization of two cDNAs encoding mitochondrial lipoamide dehydrogenase from Arabidopsis. | Plant physiology 20011001 |
Alpha and beta subunits of pyruvate dehydrogenase E1 from the microsporidian Nosema locustae: mitochondrion-derived carbon metabolism in microsporidia. | Molecular and biochemical parasitology 20011001 |
A case of PDH-E1 alpha mosaicism in a male patient with severe metabolic lactic acidosis. | Journal of inherited metabolic disease 20011001 |
Modification by exercise training of activity and enzyme expression of hepatic branched-chain alpha-ketoacid dehydrogenase complex in streptozotocin-induced diabetic rats. | Journal of nutritional science and vitaminology 20011001 |
Biochemical basis of type IB (E1beta ) mutations in maple syrup urine disease. A prevalent allele in patients from the Druze kindred in Israel. | The Journal of biological chemistry 20010928 |
Mycobacterium tuberculosis lipoamide dehydrogenase is encoded by Rv0462 and not by the lpdA or lpdB genes. | Biochemistry 20010925 |
Fluorescence correlation spectroscopy of flavins and flavoenzymes: photochemical and photophysical aspects. | Spectrochimica acta. Part A, Molecular and biomolecular spectroscopy 20010914 |
Regulation of branched-chain amino acid catabolism: nutritional and hormonal regulation of activity and expression of the branched-chain alpha-keto acid dehydrogenase kinase. | Current opinion in clinical nutrition and metabolic care 20010901 |
Effects of bezafibrate on beta-cell function of rat pancreatic islets. | European journal of pharmacology 20010831 |
Lipoamide dehydrogenase from Corynebacterium glutamicum: molecular and physiological analysis of the lpd gene and characterization of the enzyme. | Microbiology (Reading, England) 20010801 |
Tissue-specific and developmental-specific expression of an Arabidopsis thaliana gene encoding the lipoamide dehydrogenase component of the plastid pyruvate dehydrogenase complex. | Plant molecular biology 20010801 |
Leucine and its keto acid enhance the coordinated expression of genes for branched-chain amino acid catabolism in Arabidopsis under sugar starvation. | FEBS letters 20010615 |
Electrocatalytic reduction of lipoic acid and electroenzymatic reduction of NAD(P)(+) for integrated dehydrogenase biosensors. | Biosensors & bioelectronics 20010601 |
Autoantibodies of sera from patients with primary biliary cirrhosis recognize the alpha subunit of the decarboxylase component of human branched-chain 2-oxo acid dehydrogenase complex. | Journal of hepatology 20010601 |
Carrier detection and rapid newborn diagnostic test for the common Y393N maple syrup urine disease allele by PCR-RFLP: culturally permissible testing in the Mennonite community. | Journal of inherited metabolic disease 20010601 |
Human populations show reduced DNA sequence variation at the factor IX locus. | Current biology : CB 20010515 |
Acidification and glucocorticoids independently regulate branched-chain alpha-ketoacid dehydrogenase subunit genes. | American journal of physiology. Cell physiology 20010501 |
Protection by thiols of the mitochondrial complexes from 4-hydroxy-2-nonenal. | Free radical biology & medicine 20010501 |
[Mitochondrial DNA T to G mutation 8993 in Leigh encephalopathy and organic aciduria]. | No to hattatsu = Brain and development 20010501 |
Reduction of ubiquinone by lipoamide dehydrogenase. An antioxidant regenerating pathway. | European journal of biochemistry 20010301 |
Inhibitory effect of alpha-lipoic acid and its positively charged amide analogue on nitric oxide production in RAW 264.7 macrophages. | Biochemical pharmacology 20010301 |
Congenital lactic acidosis: evaluation of the properties of the a199t natural variant of human pyruvate dehydrogenase e1alpha by in vitro mutation. | Molecular genetics and metabolism 20010301 |
Regulation of the activity of branched-chain 2-oxo acid dehydrogenase (BCODH) complex by binding BCODH kinase. | FEBS letters 20010223 |
2- and 3-substituted 1,4-naphthoquinone derivatives as subversive substrates of trypanothione reductase and lipoamide dehydrogenase from Trypanosoma cruzi: synthesis and correlation between redox cycling activities and in vitro cytotoxicity. | Journal of medicinal chemistry 20010215 |
Reaction mechanism for mammalian pyruvate dehydrogenase using natural lipoyl domain substrates. | Archives of biochemistry and biophysics 20010215 |
Roles of active site and novel K+ ion-binding site residues in human mitochondrial branched-chain alpha-ketoacid decarboxylase/dehydrogenase. | The Journal of biological chemistry 20010209 |
cDNA cloning of the chicken branched-chain alpha-keto acid dehydrogenase complex. Chicken-specific residues of the acyltransferase affect the overall activity and the interaction with the dehydrogenase. | European journal of biochemistry 20010201 |
Multiple display of peptides and proteins on a macromolecular scaffold derived from a multienzyme complex. | Journal of molecular biology 20010112 |
Gene preference in maple syrup urine disease. | American journal of human genetics 20010101 |
Effect of starvation on branched-chain alpha-keto acid dehydrogenase activity in rat heart and skeletal muscle. | Physiological research 20010101 |
Channeling of substrates and intermediates in enzyme-catalyzed reactions. | Annual review of biochemistry 20010101 |
[Disorders of pyruvate metabolism and TCA cycle]. | Ryoikibetsu shokogun shirizu 20010101 |
Alpha-lipoic acid and alpha-lipoamide prevent oxidant-induced lysosomal rupture and apoptosis. | Redox report : communications in free radical research 20010101 |
Induction of expression of branched-chain aminotransferase and alpha-keto acid dehydrogenase in rat tissues during lactation. | Advances in experimental medicine and biology 20010101 |
Lipid peroxidation of erythrocyte membrane induced by lipoamide dehydrogenase in the presence of ADP-Fe3+. | Pharmacology & toxicology 19921001 |
Thioredoxin fragment 31-36 is reduced by dihydrolipoamide and reduces oxidized protein. | Biochemical and biophysical research communications 19880115 |
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