A novel ion-pair RP-HPLC method for determination of five components in compound α-ketoacid tablets.

Journal of pharmaceutical and biomedical analysis 20120701

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

Selective editing of Val and Leu methyl groups in high molecular weight protein NMR.

Journal of biomolecular NMR 20120601

Experimental evolution of Legionella pneumophila in mouse macrophages leads to strains with altered determinants of environmental survival.

PLoS pathogens 20120501

Two novel mutations in the BCKDHB gene (R170H, Q346R) cause the classic form of maple syrup urine disease (MSUD).

Gene 20120425

Production of volatile compounds by Lactobacillus sakei from branched chain α-keto acids.

Food microbiology 20120401

Removal of the C-terminal regulatory domain of α-isopropylmalate synthase disrupts functional substrate binding.

Biochemistry 20120320

Mutation of zebrafish dihydrolipoamide branched-chain transacylase E2 results in motor dysfunction and models maple syrup urine disease.

Disease models & mechanisms 20120301

[Acute encephalopathy due to late-onset maple syrup urine disease in a school boy].

Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics 20120301

Fermentative production of isobutene.

Applied microbiology and biotechnology 20120201

A bio-catalytic approach to aliphatic ketones.

Scientific reports 20120101

UniDrug-target: a computational tool to identify unique drug targets in pathogenic bacteria.

PloS one 20120101

Complete Genome of Ignavibacterium album, a Metabolically Versatile, Flagellated, Facultative Anaerobe from the Phylum Chlorobi.

Frontiers in microbiology 20120101

Recent progress in synthetic biology for microbial production of C3-C10 alcohols.

Frontiers in microbiology 20120101

Cloning and characterization of a novel 2-ketoisovalerate reductase from the beauvericin producer Fusarium proliferatum LF061.

BMC biotechnology 20120101

Amino acid metabolism in the human fetus at term: leucine, valine, and methionine kinetics.

Pediatric research 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

Emerging perspectives on essential amino acid metabolism in obesity and the insulin-resistant state.

Advances in nutrition (Bethesda, Md.) 20111101

Use of the valine biosynthetic pathway to convert glucose into isobutanol.

Journal of industrial microbiology & biotechnology 20110901

Multimodal dynamic response of the Buchnera aphidicola pLeu plasmid to variations in leucine demand of its host, the pea aphid Acyrthosiphon pisum.

Molecular microbiology 20110901

The organellar genome and metabolic potential of the hydrogen-producing mitochondrion of Nyctotherus ovalis.

Molecular biology and evolution 20110801

Engineering Bacillus subtilis for isobutanol production by heterologous Ehrlich pathway construction and the biosynthetic 2-ketoisovalerate precursor pathway overexpression.

Applied microbiology and biotechnology 20110801

Characterization of organic anion-transporting polypeptide (Oatp) 1a1 and 1a4 null mice reveals altered transport function and urinary metabolomic profiles.

Toxicological sciences : an official journal of the Society of Toxicology 20110801

¹³C metabolic flux analysis identifies an unusual route for pyruvate dissimilation in mycobacteria which requires isocitrate lyase and carbon dioxide fixation.

PLoS pathogens 20110701

High-flux isobutanol production using engineered Escherichia coli: a bioreactor study with in situ product removal.

Applied microbiology and biotechnology 20110601

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

Metabonomic investigation of single and multiple strain Trypanosoma brucei brucei infections.

The American journal of tropical medicine and hygiene 20110105

From amino acid to glucosinolate biosynthesis: protein sequence changes in the evolution of methylthioalkylmalate synthase in Arabidopsis.

The Plant cell 20110101

Molecular breeding of advanced microorganisms for biofuel production.

Journal of biomedicine & biotechnology 20110101

Asp295 stabilizes the active-site loop structure of pyruvate dehydrogenase, facilitating phosphorylation of ser292 by pyruvate dehydrogenase-kinase.

Enzyme research 20110101

Housekeeping genes essential for pantothenate biosynthesis are plasmid-encoded in Rhizobium etli and Rhizobium leguminosarum.

BMC microbiology 20110101

The human postsynaptic density shares conserved elements with proteomes of unicellular eukaryotes and prokaryotes.

Frontiers in neuroscience 20110101

More than 200 genes required for methane formation from H₂ and CO₂ and energy conservation are present in Methanothermobacter marburgensis and Methanothermobacter thermautotrophicus.

Archaea (Vancouver, B.C.) 20110101

Systems biology of the qa gene cluster in Neurospora crassa.

PloS one 20110101

Genetics and molecular biology of the electron flow for sulfate respiration in desulfovibrio.

Frontiers in microbiology 20110101

Increased isobutanol production in Saccharomyces cerevisiae by overexpression of genes in valine metabolism.

Biotechnology for biofuels 20110101

Comparative analyses imply that the enigmatic Sigma factor 54 is a central controller of the bacterial exterior.

BMC genomics 20110101

Three Korean patients with maple syrup urine disease: four novel mutations in the BCKDHA gene.

Annals of clinical and laboratory science 20110101

Carbon metabolic pathways in phototrophic bacteria and their broader evolutionary implications.

Frontiers in microbiology 20110101

Chemometric analysis of biofluids from mice experimentally infected with Schistosoma mansoni.

Parasites & vectors 20110101

Detailed kinetics and regulation of mammalian 2-oxoglutarate dehydrogenase.

BMC biochemistry 20110101

Duodenal and faecal microbiota of celiac children: molecular, phenotype and metabolome characterization.

BMC microbiology 20110101

The transcriptome of the novel dinoflagellate Oxyrrhis marina (Alveolata: Dinophyceae): response to salinity examined by 454 sequencing.

BMC genomics 20110101

Proteomic and biochemical analyses reveal the activation of unfolded protein response, ERK-1/2 and ribosomal protein S6 signaling in experimental autoimmune myocarditis rat model.

BMC genomics 20110101

Identification of two novel BCKDHA mutations in a Chinese patient with maple syrup urine disease.

Journal of pediatric endocrinology & metabolism : JPEM 20110101

The fur transcription regulator and fur-regulated genes in Clostridium botulinum A ATCC 3502.

Journal of biomedicine & biotechnology 20110101

Impact of prenatal disorders on the metabolic profile of second trimester amniotic fluid: a nuclear magnetic resonance metabonomic study.

Journal of proteome research 20101105

Proteomic and metabolomic analysis of atrial profibrillatory remodelling in congestive heart failure.

Journal of molecular and cellular cardiology 20101101

Graph-based analysis of the metabolic exchanges between two co-resident intracellular symbionts, Baumannia cicadellinicola and Sulcia muelleri, with their insect host, Homalodisca coagulata.

PLoS computational biology 20100901

Metabolic changes reveal the development of schistosomiasis in mice.

PLoS neglected tropical diseases 20100801

Engineering Corynebacterium glutamicum for isobutanol production.

Applied microbiology and biotechnology 20100701

Characterization of the branched-chain amino acid aminotransferase enzyme family in tomato.

Plant physiology 20100701

LAP5 and LAP6 encode anther-specific proteins with similarity to chalcone synthase essential for pollen exine development in Arabidopsis.

Plant physiology 20100701

Microbial 1-butanol production: Identification of non-native production routes and in silico engineering interventions.

Biotechnology journal 20100701

Complete genome sequence of Thermosphaera aggregans type strain (M11TL).

Standards in genomic sciences 20100630

Incorporation of methyl-protonated valine and leucine residues into deuterated ocean pout type III antifreeze protein: expression, crystallization and preliminary neutron diffraction studies.

Acta crystallographica. Section F, Structural biology and crystallization communications 20100601

Integrated cytokine and metabolic analysis of pathological responses to parasite exposure in rodents.

Journal of proteome research 20100507

Mass spectrometry screening reveals widespread diversity in trichome specialized metabolites of tomato chromosomal substitution lines.

The Plant journal : for cell and molecular biology 20100501

Integration of metabolomics and transcriptomics data to aid biomarker discovery in type 2 diabetes.

Molecular bioSystems 20100501

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

Structural insights into the prereaction state of pyruvate decarboxylase from Zymomonas mobilis .

Biochemistry 20100302

Homeostatic interplay between bacterial cell-cell signaling and iron in virulence.

PLoS pathogens 20100301

The C-terminal regulatory domain is required for catalysis by Neisseria meningitidis alpha-isopropylmalate synthase.

Biochemical and biophysical research communications 20100226

Regulation of branched-chain amino acid catabolism: glucose limitation enhances the component of isovalerylspiramycin for the bitespiramycin production.

Bioprocess and biosystems engineering 20100201

Genome sequence of the pea aphid Acyrthosiphon pisum.

PLoS biology 20100201

Engineering the isobutanol biosynthetic pathway in Escherichia coli by comparison of three aldehyde reductase/alcohol dehydrogenase genes.

Applied microbiology and biotechnology 20100101

Pentanol isomer synthesis in engineered microorganisms.

Applied microbiology and biotechnology 20100101

The path to next generation biofuels: successes and challenges in the era of synthetic biology.

Microbial cell factories 20100101

The Thiamine diphosphate dependent Enzyme Engineering Database: a tool for the systematic analysis of sequence and structure relations.

BMC biochemistry 20100101

Prediction of novel synthetic pathways for the production of desired chemicals.

BMC systems biology 20100101

alpha-hydroxybutyrate is an early biomarker of insulin resistance and glucose intolerance in a nondiabetic population.

PloS one 20100101

Compartmentalization of the Edinburgh Human Metabolic Network.

BMC bioinformatics 20100101

Two origins for the gene encoding alpha-isopropylmalate synthase in fungi.

PloS one 20100101

Synthetic biology guides biofuel production.

Journal of biomedicine & biotechnology 20100101

Iron-sulfur world in aerobic and hyperthermoacidophilic archaea Sulfolobus.

Archaea (Vancouver, B.C.) 20100101

A genomic perspective on the potential of Actinobacillus succinogenes for industrial succinate production.

BMC genomics 20100101

Interaction with diurnal and circadian regulation results in dynamic metabolic and transcriptional changes during cold acclimation in Arabidopsis.

PloS one 20100101

A kinetic platform for in silico modeling of the metabolic dynamics in Escherichia coli.

Advances and applications in bioinformatics and chemistry : AABC 20100101

Adverse effect of fenofibrate on branched-chain alpha-ketoacid dehydrogenase complex in rat's liver.

Toxicology 20091221

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

Arabidopsis thaliana encodes a bacterial-type heterodimeric isopropylmalate isomerase involved in both Leu biosynthesis and the Met chain elongation pathway of glucosinolate formation.

Plant molecular biology 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

Identification of candidate genes affecting Delta9-tetrahydrocannabinol biosynthesis in Cannabis sativa.

Journal of experimental botany 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

Protein phosphatase 2Cm is a critical regulator of branched-chain amino acid catabolism in mice and cultured cells.

The Journal of clinical investigation 20090601

Inhibition of branched-chain alpha-ketoacid dehydrogenase kinase by thiamine pyrophosphate at different potassium ionic levels.

Bioscience, biotechnology, and biochemistry 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

Animal models of maple syrup urine disease.

Journal of inherited metabolic disease 20090401

Selective backbone labelling of ILV methyl labelled proteins.

Journal of biomolecular NMR 20090401

Modified method for purifying rat liver branched-chain alpha-ketoacid dehydrogenase complex.

Bioscience, biotechnology, and biochemistry 20090323

The standardized ileal digestible valine-to-lysine requirement ratio is at least seventy percent in postweaned piglets.

Journal of animal science 20090301

An efficient protocol for the complete incorporation of methyl-protonated alanine in perdeuterated protein.

Journal of biomolecular NMR 20090201

Combinatorial mutasynthesis of scrambled beauvericins, cyclooligomer depsipeptide cell migration inhibitors from Beauveria bassiana.

Chembiochem : a European journal of chemical biology 20090126

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

Regulation of alpha-ketoisovalerate decarboxylase expression in Lactococcus lactis IFPL730.

Journal of molecular microbiology and biotechnology 20090101

Modeling metabolic networks in C. glutamicum: a comparison of rate laws in combination with various parameter optimization strategies.

BMC systems biology 20090101

Isolation, sequence identification, and tissue expression profile of 3 novel porcine genes: NCF2, BCKDHB and BCKDHA.

Journal of applied genetics 20090101

Determination of branched chain amino acids, methionine, phenylalanine, tyrosine and alpha-keto acids in plasma and dried blood samples using HPLC with fluorescence detection.

Clinical chemistry and laboratory medicine 20090101

Molecular genetics of maple syrup urine disease in the Turkish population.

The Turkish journal of pediatrics 20090101

Characterization of alpha-isopropylmalate synthases containing different copy numbers of tandem repeats in Mycobacterium tuberculosis.

BMC microbiology 20090101

Molecular cloning, sequence identification and tissue expression profile of three novel sheep (Ovis aries) genes - BCKDHA, NAGA and HEXA.

Biological research 20090101

The amidase domain of lipoamidase specifically inactivates lipoylated proteins in vivo.

PloS one 20090101

Snapshots of catalysis in the E1 subunit of the pyruvate dehydrogenase multienzyme complex.

Structure (London, England : 1993) 20081212

Protein side-chain dynamics as observed by solution- and solid-state NMR spectroscopy: a similarity revealed.

Journal of the American Chemical Society 20081210

Impaired oxidation of branched-chain amino acids in the medial thalamus of thiamine-deficient rats.

Metabolic brain disease 20081201

Maple syrup urine disease due to a new large deletion at BCKDHA caused by non-homologous recombination.

Journal of inherited metabolic disease 20081201

Exploration of the molecular origin of the azinomycin epoxide: timing of the biosynthesis revealed.

Organic letters 20081106

alpha-Isopropylmalate, a leucine biosynthesis intermediate in yeast, is transported by the mitochondrial oxalacetate carrier.

The Journal of biological chemistry 20081017

Direct methods and residue type specific isotope labeling in NMR structure determination and model-driven sequential assignment.

Journal of biomolecular NMR 20081001

Maple syrup urine disease in Thai infants.

Journal of the Medical Association of Thailand = Chotmaihet thangphaet 20081001

Regulation of branched-chain amino acid catabolism in rat models for spontaneous type 2 diabetes mellitus.

Biochemical and biophysical research communications 20080815

Metabolic profiling of an Echinostoma caproni infection in the mouse for biomarker discovery.

PLoS neglected tropical diseases 20080701

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

A novel isoform of pantothenate synthetase in the Archaea.

The FEBS journal 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

A new family of D-2-hydroxyacid dehydrogenases that comprises D-mandelate dehydrogenases and 2-ketopantoate reductases.

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

Amino acids allosterically regulate the thiamine diphosphate-dependent alpha-keto acid decarboxylase from Mycobacterium tuberculosis.

The Journal of biological chemistry 20080227

A genomic analysis of the archaeal system Ignicoccus hospitalis-Nanoarchaeum equitans.

Genome biology 20080101

Is gene activity in plant cells affected by UMTS-irradiation? A whole genome approach.

Advances and applications in bioinformatics and chemistry : AABC 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

Description of the mutations in 15 subjects with variant forms of maple syrup urine disease.

Journal of inherited metabolic disease 20071101

Effect of the branched-chain alpha-keto acids accumulating in maple syrup urine disease on S100B release from glial cells.

Journal of the neurological sciences 20070915

Exercise training increases branched-chain oxoacid dehydrogenase kinase content in human skeletal muscle.

American journal of physiology. Regulatory, integrative and comparative physiology 20070901

Peroxisomal metabolism of propionic acid and isobutyric acid in plants.

The Journal of biological chemistry 20070824

L-glutamine in vitro regulates rat aortic glutamate content and modulates nitric oxide formation and contractility responses.

American journal of physiology. Cell physiology 20070701

Identification of the bkdAB gene cluster, a plausible source of the starter-unit for virginiamycin M production in Streptomyces virginiae.

Archives of microbiology 20070601

Biomass in the manufacture of industrial products--the use of proteins and amino acids.

Applied microbiology and biotechnology 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

MAM3 catalyzes the formation of all aliphatic glucosinolate chain lengths in Arabidopsis.

Plant physiology 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

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

Synaptic plasma membrane Na(+), K (+)-ATPase activity is significantly reduced by the alpha-keto acids accumulating in maple syrup urine disease in rat cerebral cortex.

Metabolic brain disease 20070301

Two Arabidopsis genes (IPMS1 and IPMS2) encode isopropylmalate synthase, the branchpoint step in the biosynthesis of leucine.

Plant physiology 20070201

Branched chain keto-acids exert biphasic effects on alpha-ketoglutarate-stimulated respiration in intact rat liver mitochondria.

Neurochemical research 20070101

The crystal structure of Escherichia coli TdcF, a member of the highly conserved YjgF/YER057c/UK114 family.

BMC structural biology 20070101

Metabolic network visualization eliminating node redundance and preserving metabolic pathways.

BMC systems biology 20070101

A comprehensive analysis of non-sequential alignments between all protein structures.

BMC structural biology 20070101

Single nucleotide polymorphism detection by polymerase chain reaction-restriction fragment length polymorphism.

Nature protocols 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

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

A relevant in vitro eukaryotic live-cell system for the evaluation of plasmodial protein localization.

Biochimie 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

Kinetic and chemical mechanism of alpha-isopropylmalate synthase from Mycobacterium tuberculosis.

Biochemistry 20060725

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

Metabolic complementarity and genomics of the dual bacterial symbiosis of sharpshooters.

PLoS biology 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

Hypobaric hypoxia affects endogenous levels of alpha-keto acids in murine heart ventricles.

Biochemical and biophysical research communications 20060414

IgA class antibodies to 2-oxo-acid dehydrogenase complex are not predictive markers of histopathological progression in primary biliary cirrhosis.

Autoimmunity 20060301

A versatile conformational switch regulates reactivity in human branched-chain alpha-ketoacid dehydrogenase.

Structure (London, England : 1993) 20060201

Creatine and antioxidant treatment prevent the inhibition of creatine kinase activity and the morphological alterations of C6 glioma cells induced by the branched-chain alpha-keto acids accumulating in maple syrup urine disease.

Cellular and molecular neurobiology 20060201

Production and characterization of murine models of classic and intermediate maple syrup urine disease.

BMC medical genetics 20060101

Metabolite profiling of Chlamydomonas reinhardtii under nutrient deprivation.

Plant physiology 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

Mechanism of the insulin-releasing action of alpha-ketoisocaproate and related alpha-keto acid anions.

Molecular pharmacology 20051001

Coordinated collagen and muscle protein synthesis in human patella tendon and quadriceps muscle after exercise.

The Journal of physiology 20050915

Branched-chain alpha-keto acids accumulating in maple syrup urine disease induce reorganization of phosphorylated GFAP in C6-glioma cells.

Metabolic brain disease 20050901

Studies on thiamine diphosphate-dependent enzymes.

Biochemical Society transactions 20050801

Slow-onset feedback inhibition: inhibition of Mycobacterium tuberculosis alpha-isopropylmalate synthase by L-leucine.

Journal of the American Chemical Society 20050720

alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats.

Metabolic brain disease 20050601

Biosynthesis of glyoxylate from glycine in Saccharomyces cerevisiae.

FEMS yeast research 20050501

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

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

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

Thermococcus profundus 2-ketoisovalerate ferredoxin oxidoreductase, a key enzyme in the archaeal energy-producing amino acid metabolic pathway.

Journal of biochemistry 20050101

A comprehensive update of the sequence and structure classification of kinases.

BMC structural biology 20050101

The mitochondrial branched-chain aminotransferase (AtBCAT-1) is capable to initiate degradation of leucine, isoleucine and valine in almost all tissues in Arabidopsis thaliana.

Plant molecular biology 20050101

Modeling Lactococcus lactis using a genome-scale flux model.

BMC microbiology 20050101

Molecular mechanism for regulation of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex by phosphorylation.

Structure (London, England : 1993) 20041201

Evidence that the branched-chain alpha-keto acids accumulating in maple syrup urine disease induce morphological alterations and death in cultured astrocytes from rat cerebral cortex.

Glia 20041115

Tissue-specific translation of murine branched-chain alpha-ketoacid dehydrogenase kinase mRNA is dependent upon an upstream open reading frame in the 5'-untranslated region.

The Journal of biological chemistry 20041022

Biochemical and molecular characterization of alpha-ketoisovalerate decarboxylase, an enzyme involved in the formation of aldehydes from amino acids by Lactococcus lactis.

FEMS microbiology letters 20040915

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

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

Crystallization and preliminary X-ray analysis of alpha-isopropylmalate synthase from Mycobacterium tuberculosis.

Acta crystallographica. Section D, Biological crystallography 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

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

A conditional marker gene allowing both positive and negative selection in plants.

Nature biotechnology 20040401

Leucine toxicity in a neuronal cell model with inhibited branched chain amino acid catabolism.

Brain research. Molecular brain research 20040330

Differential regulation of branched-chain alpha-ketoacid dehydrogenase kinase expression by glucocorticoids and acidification in LLC-PK1-GR101 cells.

American journal of physiology. Renal physiology 20040301

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

Lipoic acid-dependent oxidative catabolism of alpha-keto acids in mitochondria provides evidence for branched-chain amino acid catabolism in Arabidopsis.

Plant physiology 20040201

Alpha-keto-beta-methylvaleric acid increases the in vitro phosphorylation of intermediate filaments in cerebral cortex of young rats through the gabaergic system.

Journal of the neurological sciences 20040115

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

Branched-chain amino acid catabolism: unique segregation of pathway enzymes in organ systems and peripheral nerves.

American journal of physiology. Endocrinology and metabolism 20040101

Intrahippocampal administration of the alpha-keto acids accumulating in maple syrup urine disease provokes learning deficits in rats.

Pharmacology, biochemistry, and behavior 20040101

Catabolism of leucine to branched-chain fatty acids in Staphylococcus xylosus.

Journal of applied microbiology 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

Markers of inflammation, proteolysis, and apoptosis in ESRD.

American journal of kidney diseases : the official journal of the National Kidney Foundation 20031201

Inhibition of brain energy metabolism by the alpha-keto acids accumulating in maple syrup urine disease.

Biochimica et biophysica acta 20031120

Ile, Leu, and Val methyl assignments of the 723-residue malate synthase G using a new labeling strategy and novel NMR methods.

Journal of the American Chemical Society 20031112

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

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

Substrate specificity and kinetic isotope effect analysis of the Eschericia coli ketopantoate reductase.

Biochemistry 20030930

Structure of E. coli ketopantoate hydroxymethyl transferase complexed with ketopantoate and Mg2+, solved by locating 160 selenomethionine sites.

Structure (London, England : 1993) 20030801

Dietary carbohydrate deprivation increases 24-hour nitrogen excretion without affecting postabsorptive hepatic or whole body protein metabolism in healthy men.

The Journal of clinical endocrinology and metabolism 20030801

Reaction mechanism of the heterotetrameric (alpha2beta2) E1 component of 2-oxo acid dehydrogenase multienzyme complexes.

Biochemistry 20030617

MR diffusion imaging and MR spectroscopy of maple syrup urine disease during acute metabolic decompensation.

Neuroradiology 20030601

Development of a capillary electrophoretic method for the analysis of amino acids containing tablets.

Journal of chromatography. A 20030418

Regulatory effects of alanine-group amino acids on serine alkaline protease production by recombinant Bacillus licheniformis.

Biotechnology and applied biochemistry 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

A sensitive and robust method for obtaining intermolecular NOEs between side chains in large protein complexes.

Journal of biomolecular NMR 20030301

Reaction of 1-amino-2-methylenecyclopropane-1-carboxylate with 1-aminocyclopropane-1-carboxylate deaminase: analysis and mechanistic implications.

Biochemistry 20030225

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

Mycobacterium tuberculosis ketopantoate hydroxymethyltransferase: tetrahydrofolate-independent hydroxymethyltransferase and enolization reactions with alpha-keto acids.

Biochemistry 20030114

The genomics and proteomics of biofilm formation.

Genome biology 20030101

Evolution of mosaic operons by horizontal gene transfer and gene displacement in situ.

Genome biology 20030101

Clofibric acid stimulates branched-chain amino acid catabolism by three mechanisms.

Archives of biochemistry and biophysics 20021115

Folding kinetics of the lipoic acid-bearing domain of human mitochondrial branched chain alpha-ketoacid dehydrogenase complex.

FEBS letters 20021023

The C-terminal hinge region of lipoic acid-bearing domain of E2b is essential for domain interaction with branched-chain alpha-keto acid dehydrogenase kinase.

The Journal of biological chemistry 20021004

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

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

Effect of the branched-chain alpha-ketoacids accumulating in maple syrup urine disease on the high molecular weight neurofilament subunit (NF-H) in rat cerebral cortex.

Metabolic brain disease 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

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

[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

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

Structural basis of the dysfunctioning of human 2-oxo acid dehydrogenase complexes.

Current medicinal chemistry 20020201

Evidence of common ancestry for the maple syrup urine disease (MSUD) Y438N allele in non-Mennonite MSUD patients.

Molecular genetics and metabolism 20020101

The complex fate of alpha-ketoacids.

Annual review of plant biology 20020101

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

Primary biliary cirrhosis: lessons learned from an organ-specific disease.

Clinical and experimental medicine 20011201

Uteroplacental insufficiency alters liver and skeletal muscle branched-chain amino acid metabolism in intrauterine growth-restricted fetal rats.

Pediatric research 20011101

A gene controlling variation in Arabidopsis glucosinolate composition is part of the methionine chain elongation pathway.

Plant 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

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

Nitrogen source governs the patterns of growth and pristinamycin production in 'Streptomyces pristinaespiralis'.

Microbiology (Reading, England) 20010901

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

Psychrophilic valine dehydrogenase of the antarctic psychrophile, Cytophaga sp. KUC-1: purification, molecular characterization and expression.

European journal of biochemistry 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

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

Alteration of the co-substrate selectivity of deacetoxycephalosporin C synthase. The role of arginine 258.

The Journal of biological chemistry 20010525

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

Pharmacological evidence that alpha-ketoisovaleric acid induces convulsions through GABAergic and glutamatergic mechanisms in rats.

Brain research 20010309

Regulation of the activity of branched-chain 2-oxo acid dehydrogenase (BCODH) complex by binding BCODH kinase.

FEBS letters 20010223

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

The biosynthesis of coenzyme A in bacteria.

Vitamins and hormones 20010101

2-keto acid oxidoreductases from Pyrococcus furiosus and Thermococcus litoralis.

Methods in enzymology 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

Induction of expression of branched-chain aminotransferase and alpha-keto acid dehydrogenase in rat tissues during lactation.

Advances in experimental medicine and biology 20010101