759-05-7 | 3-Methyl-2-oxo-butanoic acid

Packsize	Purity	Availability	Price	Discounted Price
250mg	>95%	in stock	$29.00	$20.00
1g	>95%	in stock	$59.00	$41.00
5g	98%	in stock	$206.00	$144.00
25g	95%	in stock	$884.00	$619.00
100g	95%	in stock	$2,625.00	$1,837.00

Description

• Catalog Number: AB44630
• Chemical Name: 3-Methyl-2-oxo-butanoic acid
• CAS Number: 759-05-7
• Molecular Formula: C5H8O3
• Molecular Weight: 116.1152
• MDL Number: MFCD00040427
• SMILES: CC(C(=O)C(=O)O)C

Computed Properties

• Complexity: 115
• Covalently-Bonded Unit Count: 1
• Heavy Atom Count: 8
• Hydrogen Bond Acceptor Count: 3
• Hydrogen Bond Donor Count: 1
• Rotatable Bond Count: 2
• XLogP3: 0.7

Literature

• Improved 2-methyl-1-propanol production in an engineered Bacillus subtilis by constructing inducible pathways.

  Biotechnology letters 20121201

• 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

• Engineering a metabolic pathway for isobutanol biosynthesis in Bacillus subtilis.

  Applied biochemistry and biotechnology 20120901

• Tissue-specific and nutrient regulation of the branched-chain α-keto acid dehydrogenase phosphatase, protein phosphatase 2Cm (PP2Cm).

  The Journal of biological chemistry 20120706

• 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