2311PathwayIsoleucine Biosynthesis Isoleucine biosynthesis begins with L-threonine from the threonine biosynthesis pathway. L-threonine interacts with a threonine dehydratase biosynthetic releasing water, a hydrogen ion and (2Z)-2-aminobut-2-enoate. This compound is isomerized into a 2-iminobutanoate which interacts with water and a hydrogen ion spontaneously, resulting in the release of ammonium and 2-ketobutyric acid. This compound reacts with pyruvic acid and hydrogen ion through an acetohydroxybutanoate synthase / acetolactate synthase 2 resulting in carbon dioxide and (S)-2-Aceto-2-hydroxybutanoic acid. The latter compound is reduced by an NADPH driven acetohydroxy acid isomeroreductase releasing NADP and acetohydroxy acid isomeroreductase. The latter compound is dehydrated by a dihydroxy acid dehydratase resulting in 3-methyl-2-oxovaleric acid.This compound reacts in a reversible reaction with L-glutamic acid through a Branched-chain-amino-acid aminotransferase resulting in oxoglutaric acid and L-isoleucine.MetabolicPW002537CenterPathwayVisualizationContext282314622802#000099PathwayVisualization22942311Isoleucine Biosynthesis Isoleucine biosynthesis begins with L-threonine from the threonine biosynthesis pathway. L-threonine interacts with a threonine dehydratase biosynthetic releasing water, a hydrogen ion and (2Z)-2-aminobut-2-enoate. This compound is isomerized into a 2-iminobutanoate which interacts with water and a hydrogen ion spontaneously, resulting in the release of ammonium and 2-ketobutyric acid. This compound reacts with pyruvic acid and hydrogen ion through an acetohydroxybutanoate synthase / acetolactate synthase 2 resulting in carbon dioxide and (S)-2-Aceto-2-hydroxybutanoic acid. The latter compound is reduced by an NADPH driven acetohydroxy acid isomeroreductase releasing NADP and acetohydroxy acid isomeroreductase. The latter compound is dehydrated by a dihydroxy acid dehydratase resulting in 3-methyl-2-oxovaleric acid.This compound reacts in a reversible reaction with L-glutamic acid through a Branched-chain-amino-acid aminotransferase resulting in oxoglutaric acid and L-isoleucine.Metabolic41100012329SubPathway109581109Compound1CellCL:00000005HepatocyteCL:00001824Cardiomyocyte CL:00007463NeuronCL:00005407Epithelial CellCL:00000666MyocyteCL:00001871Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote18Saccharomyces cerevisiae4932EukaryoteYeast4Arabidopsis thaliana3702EukaryoteThale cress23Pseudomonas aeruginosa287Prokaryote12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat10Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm2Bacteria2ProkaryoteBacteria24Solanum lycopersicum4081EukaryoteTomato21Xenopus laevis8355EukaryoteAfrican clawed frog25Escherichia coli (strain K12)83333Prokaryote60Nitzschia sp.0001EukaryoteNitzschia419Schizosaccharomyces pombe4896Eukaryote49Bathymodiolus platifrons220390EukaryoteDeep sea mussel11Extracellular SpaceGO:00056151CytosolGO:000582931Periplasmic SpaceGO:000562035ChloroplastGO:00095073Mitochondrial MatrixGO:00057595CytoplasmGO:00057372MitochondrionGO:00057397Endoplasmic Reticulum MembraneGO:000578912Mitochondrial Inner MembraneGO:000574314Mitochondrial Outer MembraneGO:000574124Mitochondrial Intermembrane SpaceGO:000575813Endoplasmic ReticulumGO:00057834PeroxisomeGO:000577710Cell MembraneGO:000588636MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032532Inner MembraneGO:00702586LysosomeGO:000576416Lysosomal LumenGO:004320218Melanosome MembraneGO:003316225Golgi apparatusGO:000579420Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520215NucleusGO:000563440PeriplasmGO:004259719sarcoplasmic reticulumGO:00165291LiverBTO:000075972928StomachBTO:0001307155268Blood VesselBTO:000110274114Adrenal MedullaBTO:000004971825IntestineBTO:00006487Nervous SystemBTO:000148411HeartBTO:000056273109MuscleBTO:00008871411824BrainBTO:000014289165cardiocyteBTO:000153915111PW_BS0000152111PW_BS000002107313PW_BS00010710813PW_BS000108105113PW_BS000105188118PW_BS0000241873118PW_BS0000242253541PW_BS0000243183123PW_BS000024315123PW_BS0000241321121PW_BS0001321141112PW_BS000114124151PW_BS000124409115PW_BS0001151181171PW_BS0001181371117PW_BS0001372991101PW_BS0000244831110PW_BS000115388161PW_BS000112208116PW_BS0000244311PW_BS0000048511PW_BS000008311511PW_BS0000313211PW_BS000003261115PW_BS000026541315PW_BS00005449711PW_BS000049171211PW_BS000017221411PW_BS000022422411PW_BS000042101711PW_BS000010181311PW_BS0000187028511PW_BS000070103331PW_BS0001031115121PW_BS0001111122121PW_BS000112100521PW_BS000100117131PW_BS0001171471241PW_BS0001471553241PW_BS0001551572241PW_BS0001571613181PW_BS00016115924PW_BS00015916611PW_BS0001661783211PW_BS00017815284PW_BS000152101531PW_BS0001011632181PW_BS000163205561PW_BS000024206261PW_BS000024219314PW_BS00002422014PW_BS000024222341PW_BS0000242137181PW_BS00002421013181PW_BS00002421217181PW_BS0000241601181PW_BS00016017018PW_BS000170151141PW_BS000151226441PW_BS00002416212181PW_BS000162224241PW_BS0000241951318PW_BS0000242491341PW_BS0000241644PW_BS0001642811251PW_BS0000242851041PW_BS0000242863641PW_BS0000242875341PW_BS0000242273441PW_BS0000242231241PW_BS0000242941141PW_BS0000243081011PW_BS0000243221231PW_BS0000243125231PW_BS0000243201123PW_BS00002429341PW_BS0000241333121PW_BS00013313412121PW_BS0001343317121PW_BS00002832711125PW_BS00002834713125PW_BS00002834524121PW_BS00002813013121PW_BS0001303683601PW_BS000028310312PW_BS00002430412PW_BS0000241192171PW_BS000119383751PW_BS000100943PW_BS000094390761PW_BS0001123987171PW_BS0001133361121PW_BS000028109323PW_BS000109406351PW_BS000115407251PW_BS000115122551PW_BS0001224241155PW_BS0001154251355PW_BS0001154182451PW_BS0001153841251PW_BS0001001251351PW_BS0001251203171PW_BS0001201355171PW_BS00013545911175PW_BS00011546013175PW_BS00011545424171PW_BS00011512112171PW_BS00012113613171PW_BS0001364793101PW_BS0001154812101PW_BS0001152975101PW_BS0000244957101PW_BS00011548924101PW_BS00011548012101PW_BS00011530013101PW_BS000024501361PW_BS0001155062461PW_BS0001153911261PW_BS0001123951361PW_BS0001139611PW_BS0000095411PW_BS00000514101PW_BS00001413121PW_BS0000132811611PW_BS000028204111PW_BS000020331811PW_BS000033432511PW_BS0000432441011PW_BS00002460251PW_BS00006046114PW_BS00004629111PW_BS00002972513PW_BS000072612517PW_BS0000613612011PW_BS0000363772113PW_BS00003793252011PW_BS00009327151PW_BS000027711PW_BS000007971521PW_BS0000971136121PW_BS000113110231PW_BS0001101231751PW_BS000123126651PW_BS00012612711651PW_BS00012712915121PW_BS0001296131PW_BS000006140103PW_BS00014014315191PW_BS0001431465191PW_BS000146951721PW_BS00009511PW_BS0000011802211PW_BS000180207661PW_BS0000242111018PW_BS00002421425181PW_BS0000242156181PW_BS0000241985181PW_BS0000242164181PW_BS0000241901118PW_BS0000242771218PW_BS00002465111PW_BS0000652905491PW_BS0000242916491PW_BS0000242924491PW_BS00002429817101PW_BS0000243016101PW_BS000024302116101PW_BS000024253541PW_BS00002433217121PW_BS0000283331212PW_BS0000281151012PW_BS0001153344121PW_BS000028337116121PW_BS00002834141121PW_BS00002834318121PW_BS00002832914121PW_BS0000283522512PW_BS00002835325127PW_BS00002835625121PW_BS000028360410121PW_BS0000283702601PW_BS000028228361PW_BS000024232403PW_BS000024408451PW_BS000115412125PW_BS000115405105PW_BS0001154151851PW_BS0001154141551PW_BS000115429151PW_BS0001154192551PW_BS00011543441051PW_BS0001153821451PW_BS000100436255PW_BS0001153744171PW_BS0000534436171PW_BS0001154461217PW_BS0001153761017PW_BS00005344717171PW_BS000115448116171PW_BS00011545118171PW_BS00011545015171PW_BS0001154641171PW_BS00011545525171PW_BS000115469410171PW_BS00011539914171PW_BS0001134712517PW_BS00011547225177PW_BS0001154824101PW_BS0001154781010PW_BS00011548718101PW_BS00011549025101PW_BS00011548414101PW_BS000115502461PW_BS000115209106PW_BS0000245041861PW_BS0001155072561PW_BS00011551541061PW_BS0001153891461PW_BS0001125131761PW_BS000115509516PW_BS00005015612241PW_BS0001561771211PW_BS00017785241011PW_BS00008534695126PW_BS0000283671601PW_BS0000284239556PW_BS00011545895176PW_BS0001153551914PW_BS000035711113PW_BS0000712892491PW_BS000024221324PW_BS0000243791541PW_BS000084380364PW_BS000084693741PW_BS000512109L-ThreonineHMDB0000167Threonine is an essential amino acid in humans. It is abundant in human plasma, particularly in newborns. Severe deficiency of threonine causes neurological dysfunction and lameness in experimental animals. Threonine is an immunostimulant which promotes the growth of thymus gland. It also can probably promote cell immune defense function. This amino acid has been useful in the treatment of genetic spasticity disorders and multiple sclerosis at a dose of 1 gram daily. It is highly concentrated in meat products, cottage cheese and wheat germ. (http://www.dcnutrition.com/AminoAcids/) The threonine content of most of the infant formulas currently on the market is approximately 20% higher than the threonine concentration in human milk. Due to this high threonine content the plasma threonine concentrations are up to twice as high in premature infants fed these formulas than in infants fed human milk. The whey proteins which are used for infant formulas are sweet whey proteins. Sweet whey results from cheese production. Threonine catabolism in mammals appears to be due primarily (70-80%) to the activity of threonine dehydrogenase (EC 1.1.1.103) that oxidizes threonine to 2-amino-3-oxobutyrate, which forms glycine and acetyl CoA, whereas threonine dehydratase (EC 4.2.1.16) that catabolizes threonine into 2-oxobutyrate and ammonia, is significantly less active. Increasing the threonine plasma concentrations leads to accumulation of threonine and glycine in the brain. Such accumulation affects the neurotransmitter balance which may have consequences for the brain development during early postnatal life. Thus, excessive threonine intake during infant feeding should be avoided. (PMID 9853925).72-19-5C00188628816857THR6051DB00156C[C@@H](O)[C@H](N)C(O)=OC4H9NO3InChI=1S/C4H9NO3/c1-2(6)3(5)4(7)8/h2-3,6H,5H2,1H3,(H,7,8)/t2-,3+/m1/s1AYFVYJQAPQTCCC-GBXIJSLDSA-N(2S,3R)-2-amino-3-hydroxybutanoic acid119.1192119.0582431590.603L-threonine00FDB011999Threonin;(2s,3r)-(-)-threonine;(2s,3r)-2-amino-3-hydroxybutyrate;(2s,3r)-2-amino-3-hydroxybutyric acid;(r-(r*,s*))-2-amino-3-hydroxybutanoate;(r-(r*,s*))-2-amino-3-hydroxybutanoic acid;(s)-threonine;2-amino-3-hydroxybutanoate;2-amino-3-hydroxybutanoic acid;2-amino-3-hydroxybutyrate;2-amino-3-hydroxybutyric acid;L-(-)-threonine;L-2-amino-3-hydroxybutyrate;L-2-amino-3-hydroxybutyric acid;L-alpha-amino-beta-hydroxybutyrate;L-alpha-amino-beta-hydroxybutyric acid;Threonine;[r-(r*,s*)]-2-amino-3-hydroxybutanoate;[r-(r*,s*)]-2-amino-3-hydroxybutanoic acid;[r-(r*,s*)]-2-amino-3-hydroxy-butanoate;[r-(r*,s*)]-2-amino-3-hydroxy-butanoic acid;(2s)-threonine;(2s,3r)-2-amino-3-hydroxybutanoic acid;L-threonin;T;Thr;(2s,3r)-2-amino-3-hydroxybutanoate;L-a-amino-b-hydroxybutyrate;L-a-amino-b-hydroxybutyric acid;L-α-amino-β-hydroxybutyrate;L-α-amino-β-hydroxybutyric acidPW_C000109Thr268915269025644107564510858851056908188690918783792254241431842415315790261327903811412257612412258040912514811812515213712672529912673448312831838812832820840034Hydrogen IonHMDB0059597Hydrogen ion is recommended by IUPAC as a general term for all ions of hydrogen and its isotopes. Depending on the charge of the ion, two different classes can be distinguished: positively charged ions and negatively charged ions. Under aqueous conditions found in biochemistry, hydrogen ions exist as the hydrated form hydronium, H3O+, but these are often still referred to as hydrogen ions or even protons by biochemists. [WikiPedia])C000801038153781010[H+]HInChI=1S/p+1GPRLSGONYQIRFK-UHFFFAOYSA-Nhydron1.00791.0078250320hydron10H+;H(+);Hydrogen cation;Hydron;ProtonPW_C040034H+2154670875315788318483111621463261464542231492780174250224254424547104576184694705241103532711153531125626108563910756991005720105574211759631476037155607015760931616130159623216664831786601152669210168431886910187710016371682057191206745321974542207472222752521375322107558212757216075901708195225821815182432268413162842022491391959155249119151641201528112181285122462861226628712521227132572231332529415330308423293154235431842401322424053124245432076912293771361337721013477372331778041147795513277990327779913477837934579929130800193688038731080388304807221199382312494823383110550388112855941132803901155373981155391181158563361162051091199734061201934071205491221205934091211704241211714251225694181226153841226871251227581201231831351232181371237424591237434601251414541251881211252731361253594791255504811257304831257362971258092991265174951267174891267664801268233001269025011272132081283085061283613911284303951420WaterHMDB0002111Water is a chemical substance that is essential to all known forms of life. It appears colorless to the naked eye in small quantities, though it is actually slightly blue in color. It covers 71% of Earth's surface. Current estimates suggest that there are 1.4 billion cubic kilometers (330 million m3) of it available on Earth, and it exists in many forms. It appears mostly in the oceans (saltwater) and polar ice caps, but it is also present as clouds, rain water, rivers, freshwater aquifers, lakes, and sea ice. Water in these bodies perpetually moves through a cycle of evaporation, precipitation, and runoff to the sea. Clean water is essential to human life. In many parts of the world, it is in short supply. From a biological standpoint, water has many distinct properties that are critical for the proliferation of life that set it apart from other substances. It carries out this role by allowing organic compounds to react in ways that ultimately allow replication. All known forms of life depend on water. Water is vital both as a solvent in which many of the body's solutes dissolve and as an essential part of many metabolic processes within the body. Metabolism is the sum total of anabolism and catabolism. In anabolism, water is removed from molecules (through energy requiring enzymatic chemical reactions) in order to grow larger molecules (e.g. starches, triglycerides and proteins for storage of fuels and information). In catabolism, water is used to break bonds in order to generate smaller molecules (e.g. glucose, fatty acids and amino acids to be used for fuels for energy use or other purposes). Water is thus essential and central to these metabolic processes. Water is also central to photosynthesis and respiration. Photosynthetic cells use the sun's energy to split off water's hydrogen from oxygen. Hydrogen is combined with CO2 (absorbed from air or water) to form glucose and release oxygen. All living cells use such fuels and oxidize the hydrogen and carbon to capture the sun's energy and reform water and CO2 in the process (cellular respiration). Water is also central to acid-base neutrality and enzyme function. An acid, a hydrogen ion (H+, that is, a proton) donor, can be neutralized by a base, a proton acceptor such as hydroxide ion (OH-) to form water. Water is considered to be neutral, with a pH (the negative log of the hydrogen ion concentration) of 7. Acids have pH values less than 7 while bases have values greater than 7. Stomach acid (HCl) is useful to digestion. However, its corrosive effect on the esophagus during reflux can temporarily be neutralized by ingestion of a base such as aluminum hydroxide to produce the neutral molecules water and the salt aluminum chloride. Human biochemistry that involves enzymes usually performs optimally around a biologically neutral pH of 7.4. (Wikipedia).7732-18-5C0000196215377937OH2OInChI=1S/H2O/h1H2XLYOFNOQVPJJNP-UHFFFAOYSA-Nwater18.015318.0105646861water00FDB013390Dihydrogen oxide;Steam;[oh2];Acqua;Agua;Aqua;Bound water;Dihydridooxygen;Eau;H2o;Hoh;Hydrogen hydroxide;WasserPW_C001420H2O558949109513941513162144811352615624286521069120770338231883821094311377491465541590432018242532222678602727462778172805293143703164723634614598364727374941935030275156751959752141005227945236103529710553191115343113535511254021105470123548312554921265507127553413055371145541129559113556081185622108569165759140577810158411435853146587710758909559101475940151603215560591576087161612316361331596215162181666477178650718066001526713117684018868881607162205718120771932067211211722821372382147243215729519873502167388210740121274672227492224750019075881708201225823722684141629265261185027711922164120112811221328512250286122642871232724912520227126326512693290127052911271529213007298130193001302530113037302132612231332729415340308423273154269531843691322769142937701925377102132771311337721513477378331773973327747133377516115775363347762833677722337777593417781634377982347780713297823535278242353782703567911336080014368800393708059122880656119938303839479438411055739011063939111584439811987923211991512211996340612000840712004640812011312412036541212043040512043840912060641512079441412115842512124042912135112112138141912160743412211838212238443612275312012279737412280444312301244612306437612307213712313144712314213612316244812323145112338445012373046012381046412394045512416546912467039912493847112494547212530529712535347912538648112542448212548029912568248312570747812574548712605449012623849512627348412676448012689650112696350212701738812717720812719920912722750412750650712757651512783638912808239512817651340939 (2Z)-2-aminobut-2-enoate(2Z)-2-Aminobut-2-enoate belongs to the class of organic compounds known as alpha amino acids. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon) (2Z)-2-Aminobut-2-enoate is soluble (in water) and a moderately acidic compound (based on its pKa) (2Z)-2-Aminobut-2-enoate may be a unique E.coli metabolite. Conjugate base of 2-aminobut-2-enoic acid.58739CC=C(N)C([O-])=OC4H6NO2InChI=1S/C4H7NO2/c1-2-3(5)4(6)7/h2H,5H2,1H3,(H,6,7)/p-1PAWSVPVNIXFKOS-UHFFFAOYSA-M2-aminobut-2-enoate100.098100.0404020170.4512-aminobut-2-enoate-10PW_C04093922A2E6591151838122511923164792371228005328580144293409402-iminobutanoate2-Iminobutanoate belongs to the class of organic compounds known as fatty acids and conjugates. These are aliphatic monocarboxylic acids with a saturated or unsaturated aliphatic tail (with at least 4 Carbon atoms). 2-Iminobutanoate is slightly soluble (in water) and a weakly acidic compound (based on its pKa).19796801CCC(=N)C([O-])=OC4H6NO2InChI=1S/C4H7NO2/c1-2-3(5)4(6)7/h5H,2H2,1H3,(H,6,7)/p-1WRBRCYPPGUCRHW-UHFFFAOYSA-M2-iminobutanoate100.098100.040402017-1.3812-iminobutanoate-10PW_C0409402ib65921518382225792381228005428522608AmmoniumHMDB0041827Ammonium is an important source of nitrogen for many plant species, especially those growing on hypoxic soils. However, it is also toxic to most crop species and is rarely applied as a sole nitrogen source. The ammonium (more obscurely: aminium) cation is a positively charged polyatomic cation with the chemical formula NH4+. It is formed by the protonation of ammonia (NH3). Ammonium is also a general name for positively charged or protonated substituted amines and quaternary ammonium cations (NR4+), where one or more hydrogen atoms are replaced by organic radical groups (indicated by R).14798-03-9C013421674114628938218[NH4+]H4NInChI=1S/H3N/h1H3/p+1QGZKDVFQNNGYKY-UHFFFAOYSA-Oazanium18.038518.0343741331azanium11Ammonium ion;Ammonia ion;Ammonium;Ammonium chloride;Ammonium(1+);Azanium;Nh4+;[nh4]+;[nh4](+);Nh4(+)PW_C022608Ammon5751108589295596910062261668273151836722511909170124702494262731511628110932-Ketobutyric acidHMDB00000052-Ketobutyric acid is a substance that is involved in the metabolism of many amino acids (glycine, methionine, valine, leucine, serine, threonine, isoleucine) as well as propanoate metabolism and C-5 branched dibasic acid metabolism. More specifically, alpha-ketobutyric acid is a product of the lysis of cystathionine. It is also one of the degradation products of threonine. It can be converted into propionyl-CoA (and subsequently methylmalonyl CoA, which can be converted into succinyl CoA, a citric acid cycle intermediate), and thus enter the citric acid cycle.600-18-0C0010958308312-OXOBUTANOATE57DB04553CCC(=O)C(O)=OC4H6O3InChI=1S/C4H6O3/c1-2-3(5)4(6)7/h2H2,1H3,(H,6,7)TYEYBOSBBBHJIV-UHFFFAOYSA-N2-oxobutanoic acid102.0886102.031694058-0.1112-oxobutanoic acid0-1FDB0033592-ketobutanoate;2-ketobutanoic acid;2-ketobutyrate;2-oxo-butanoate;2-oxo-butanoic acid;2-oxo-butyrate;2-oxo-butyric acid;2-oxo-n-butyrate;2-oxo-n-butyric acid;2-oxobutanoate;2-oxobutanoic acid;2-oxobutyrate;2-oxobutyric acid;3-methylpyruvate;3-methylpyruvic acid;Methyl-pyruvate;Methyl-pyruvic acid;Propionyl-formate;Propionyl-formic acid;A-keto-n-butyrate;A-keto-n-butyric acid;A-ketobutyrate;A-ketobutyric acid;A-oxo-n-butyrate;A-oxo-n-butyric acid;A-oxobutyrate;A-oxobutyric acid;Alpha-keto-n-butyrate;Alpha-keto-n-butyric acid;Alpha-ketobutric acid;Alpha-ketobutyrate;Alpha-ketobutyric acid;Alpha-oxo-n-butyrate;Alpha-oxo-n-butyric acid;Alpha-oxobutyrate;Alpha-oxobutyric acid;2-ketobutyric acid;3-methyl pyruvic acid;3-methyl pyruvate;α-ketobutyrate;α-ketobutyric acid;α-oxo-n-butyrate;α-oxo-n-butyric acidPW_C0000032KBA337818682269238274151838322542272478126132781631117864313379027112119922122122176124122274406122577407122716135124728118124829120125149119125312297126333299126438479126726481126858205127896388128007501128319206164Pyruvic acidHMDB0000243Pyruvic acid is an intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed.) Biological Source: Intermediate in primary metabolism including fermentation processes. Present in muscle in redox equilibrium with Lactic acid. A common constituent, as a chiral cyclic acetal linked to saccharide residues, of bacterial polysaccharides. Isolated from cane sugar fermentation broth and peppermint. Constituent of Bauhinia purpurea, Cicer arietinum (chickpea), Delonix regia, Pisum sativum (pea) and Trigonella caerulea (sweet trefoil) Use/Importance: Reagent for regeneration of carbonyl compdounds from semicarbazones, phenylhydrazones and oximes. Flavoring ingredient (Dictionary of Organic Compounds).127-17-3C00022106032816PYRUVATE1031DB00119CC(=O)C(O)=OC3H4O3InChI=1S/C3H4O3/c1-2(4)3(5)6/h1H3,(H,5,6)LCTONWCANYUPML-UHFFFAOYSA-N2-oxopropanoic acid88.062188.0160439940.181pyruvic acid0-1FDB0082932-oxopropanoate;2-oxopropanoic acid;2-oxopropionate;2-oxopropionic acid;Acetylformate;Acetylformic acid;Bts;Pyroracemate;Pyroracemic acid;Pyruvate;A-ketopropionate;A-ketopropionic acid;Alpha-ketopropionate;Alpha-ketopropionic acid;2-ketopropionic acid;2-oxopropansaeure;2-oxopropionsaeure;Acide pyruvique;Alpha-oxopropionsaeure;Brenztraubensaeure;Ch3cocooh;2-ketopropionate;α-ketopropionate;α-ketopropionic acid;A-oxopropionsaeure;α-oxopropionsaeurePW_C000164Pyr1722044228118131449501457265365103540511754401185444120556613255701335893955920147595115160221556067156607416161261606383164671786510177653285745722274952208200225126223115292249153491877310111779723467797832778090112800043688004236780695135112879941156831211199504061200111241201751221208784071211484231211544241234541191237204581237264591253404791253902991255342971258544811268835011269313881270672051278582061316Carbon dioxideHMDB0001967Carbon dioxide is a colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. Carbon dioxide is produced during respiration by all animals, fungi and microorganisms that depend on living and decaying plants for food, either directly or indirectly. It is, therefore, a major component of the carbon cycle. Additionally, carbon dioxide is used by plants during photosynthesis to make sugars which may either be consumed again in respiration or used as the raw material to produce polysaccharides such as starch and cellulose, proteins and the wide variety of other organic compounds required for plant growth and development. When inhaled at concentrations much higher than usual atmospheric levels, it can produce a sour taste in the mouth and a stinging sensation in the nose and throat. These effects result from the gas dissolving in the mucous membranes and saliva, forming a weak solution of carbonic acid. Carbon dioxide is used by the food industry, the oil industry, and the chemical industry. Carbon dioxide is used to produce carbonated soft drinks and soda water. Traditionally, the carbonation in beer and sparkling wine comes about through natural fermentation, but some manufacturers carbonate these drinks artificially.124-38-9C0001128016526274O=C=OCO2InChI=1S/CO2/c2-1-3CURLTUGMZLYLDI-UHFFFAOYSA-Nmethanedione44.009543.9898292440.630carbon dioxide00DBMET00423FDB014084Carbon oxide;Carbon-12 dioxide;Carbonic acid anhydride;Carbonic acid gas;Carbonic anhydride;[co2];Co2;E 290;E-290;E290;R-744PW_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905023002(S)-2-Aceto-2-hydroxybutanoic acidHMDB0006900(S)-2-Aceto-2-hydroxybutanoic acid, also known as (S)-2-hydroxy-2-ethyl-3-oxobutanoate or (S)-2-acetyl-2-hydroxybutyrate, belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms (S)-2-Aceto-2-hydroxybutanoic acid is soluble (in water) and a weakly acidic compound (based on its pKa) (S)-2-Aceto-2-hydroxybutanoic acid exists in all living organisms, ranging from bacteria to humans. (S)-2-Aceto-2-hydroxybutanoic acid is an intermediate in branched chain amino acid metabolism. It is converted from 2-oxobutanoate or 2-hydoxyethyl ThPP via acetolactate synthase.C0600644087527681389708CC[C@](O)(C(C)=O)C(O)=OC6H10O4InChI=1S/C6H10O4/c1-3-6(10,4(2)7)5(8)9/h10H,3H2,1-2H3,(H,8,9)/t6-/m0/s1VUQLHQFKACOHNZ-LURJTMIESA-N(2S)-2-ethyl-2-hydroxy-3-oxobutanoic acid146.1412146.0579088080.252(2S)-2-ethyl-2-hydroxy-3-oxobutanoic acid0-1FDB024147(2s)-2-ethyl-2-hydroxy-3-oxobutanoate;(2s)-2-ethyl-2-hydroxy-3-oxobutanoic acid;(s)-2-aceto-2-hydroxybutanoate;(s)-2-aceto-2-hydroxybutanoic acid;(s)-2-hydroxy-2-ethyl-3-oxobutanoate;(s)-2-hydroxy-2-ethyl-3-oxobutanoic acid;(s)-2-acetyl-2-hydroxybutyric acid;(s)-2-acetyl-2-hydroxybutyratePW_C003002S2A2ha146NADPHHMDB0000221Nicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed.).53-57-6C000052283351216474NADPH17215925NC(=O)C1=CN(C=CC1)[C@H]1O[C@@H](COP(O)(=O)OP(O)(=O)OC[C@@H]2O[C@@H]([C@@H](OP(O)(O)=O)[C@H]2O)N2C=NC3=C(N)N=CN=C23)[C@H](O)[C@@H]1OC21H30N7O17P3InChI=1S/C21H30N7O17P3/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(44-46(33,34)35)14(30)11(43-21)6-41-48(38,39)45-47(36,37)40-5-10-13(29)15(31)20(42-10)27-3-1-2-9(4-27)18(23)32/h1,3-4,7-8,10-11,13-16,20-21,29-31H,2,5-6H2,(H2,23,32)(H,36,37)(H,38,39)(H2,22,24,25)(H2,33,34,35)/t10-,11-,13-,14-,15-,16-,20-,21-/m0/s1ACFIXJIJDZMPPO-NCHANQSKSA-N{[(2S,3S,4S,5S)-2-(6-amino-9H-purin-9-yl)-5-[({[({[(2S,3R,4S,5S)-5-(3-carbamoyl-1,4-dihydropyridin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-4-hydroxyoxolan-3-yl]oxy}phosphonic acid745.4209745.091102105-2.149[(2S,3S,4S,5S)-2-(6-aminopurin-9-yl)-5-{[({[(2S,3R,4S,5S)-5-(3-carbamoyl-4H-pyridin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-4-hydroxyoxolan-3-yl]oxyphosphonic acid0-4FDB0219092'-(dihydrogen phosphate) 5'-(trihydrogen pyrophosphate) adenosine 5'-ester with 1,4-dihydro-1-b-d-ribofuranosylnicotinamide;2'-(dihydrogen phosphate) 5'-(trihydrogen pyrophosphate) adenosine 5'-ester with 1,4-dihydro-1-beta-delta-ribofuranosylnicotinamide;Adenosine 5'-(trihydrogen diphosphate) 2'-(dihydrogen phosphate) p'-5'-ester with 1,4-dihydro-1-beta-d-ribofuranosyl-3-pyridinecarboxamide;Adenosine 5'-(trihydrogen diphosphate) 2'-(dihydrogen phosphate) p'-5'-ester with 1,4-dihydro-1-beta-delta-ribofuranosyl-3-pyridinecarboxamide;Dihydrocodehydrogenase ii;Dihydronicotinamide adenine dinucleotide phosphate;Dihydronicotinamide adenine dinucleotide-p;Dihydrotriphosphopyridine nucleotide reduced;Nadp-reduced;Nadph;Nicotinamide-adenine-dinucleotide-phosphorate;Nicotinamide-adenine-dinucleotide-phosphoric acid;Reduced codehydrase ii;Reduced coenzyme ii;Reduced cozymase ii;Reduced triphosphopyridine nucleotide;Triphosphopyridine nucleotide reduced;B-nadph;B-nicotinamide-adenine-dinucleotide-phosphorate;B-nicotinamide-adenine-dinucleotide-phosphoric acid;Beta-nadph;Beta-nicotinamide-adenine-dinucleotide-phosphorate;Beta-nicotinamide-adenine-dinucleotide-phosphoric acid;Nicotinamide adenine dinucleotide phosphate - reducedPW_C000146NADPH18581903778107965821188372160929161549468731479314479714531011157891085972147612815962713567791177068188710316371542057205160731521373452107559212759117081942258219151842122411812198118932111200622212150164122452861259622612648249423433154374632276911293771661327738533177394332774601307750411277511115776233368071211911316494120105407120425405120452122120616123121141125121275429121402124121483383123059376123086135123241447123712136123846464123961118124041398125472481125696297126214299126529495127009206127572388128101390143NADPHMDB0000217Nicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5-phosphate (NMN) coupled by pyrophosphate linkage to the 5-phosphate adenosine 2,5-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed.) Hydrogen carrier in biochemical redox systems. In the hexose monophosphoric acid system it is reduced to Dihydrocoenzyme II and reoxidation in the presence of flavoproteins (Dictionary of Organic Compounds).53-59-8C00006588618009NAD(P)5675NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1OC21H29N7O17P3InChI=1S/C21H28N7O17P3/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(44-46(33,34)35)14(30)11(43-21)6-41-48(38,39)45-47(36,37)40-5-10-13(29)15(31)20(42-10)27-3-1-2-9(4-27)18(23)32/h1-4,7-8,10-11,13-16,20-21,29-31H,5-6H2,(H7-,22,23,24,25,32,33,34,35,36,37,38,39)/p+1/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1XJLXINKUBYWONI-NNYOXOHSSA-O1-[(2R,3R,4S,5R)-5-[({[({[(2R,3R,4R,5R)-5-(6-amino-9H-purin-9-yl)-3-hydroxy-4-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium744.4129744.083277073-2.2791-[(2R,3R,4S,5R)-5-{[({[(2R,3R,4R,5R)-5-(6-aminopurin-9-yl)-3-hydroxy-4-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium1-3FDB021908Adenine-nicotinamide dinucleotide phosphate;Codehydrase ii;Codehydrogenase ii;Coenzyme ii;Cozymase ii;Nad phosphate;Nadp;Nadp+;Nicotinamide adenine dinucleotide phosphate;Nicotinamide-adenine dinucleotide phosphate;Tpn;Triphosphopyridine nucleotide;B-nadp;B-nicotinamide adenine dinucleotide phosphate;B-tpn;Beta-nadp;Beta-nicotinamide adenine dinucleotide phosphate;Beta-tpn;Oxidized nicotinamide-adenine dinucleotide phosphate;B-nicotinamide adenine dinucleotide phosphoric acid;Beta-nicotinamide adenine dinucleotide phosphoric acid;β-nicotinamide adenine dinucleotide phosphate;β-nicotinamide adenine dinucleotide phosphoric acidPW_C000143NADP18381913768578010824188392161129161749468531479614480114530811157901086017147613215962733567781177069188710516371522057206160731721373462107562212758917081972258220151841922411811198118972111200822212152164122492861259722612650249423443154374532276913293771641327738433177396332774611307751511577624336778143347787011280713119113165941201064071204294051204501221206044081206181231211421251212774291214011241214853831230633761230841351232293741232434471237131361238484641239601181240433981254734811256942971257434821262152991265284951270102061272255021275703881281003907698(R) 2,3-Dihydroxy-3-methylvalerateHMDB0012140(R) 2,3-Dihydroxy-methylvalerate is an intermediate in valine, leucine and isoleucine biosynthesis. The pathway of valine biosynthesis is a four-step pathway that shares all of its steps with the parallel pathway of isoleucine biosynthesis. These entwined pathways are part of the superpathway of leucine, valine, and isoleucine biosynthesis, that generates not only isoleucine and valine, but also leucine. (R) 2,3-Dihydroxy-methylvalerate is generated from 3-Hydroxy-3-methyl-2-oxopentanoic acid via the enzyme ketol-acid reductoisomerase (EC 1.1.1.86) then it is converted to (S)-3-methyl-2-oxopentanoic via the dihydroxy-acid dehydratase (EC:4.2.1.9).562-43-6C06007448154275121-KETO-2-METHYLVALERATE395044CC[C@@](C)(O)[C@@H](O)C(O)=OC6H12O4InChI=1S/C6H12O4/c1-3-6(2,10)4(7)5(8)9/h4,7,10H,3H2,1-2H3,(H,8,9)/t4-,6+/m0/s1PDGXJDXVGMHUIR-UJURSFKZSA-N(2R,3R)-2,3-dihydroxy-3-methylpentanoic acid148.1571148.0735588720.523(2R,3R)-2,3-dihydroxy-3-methylpentanoic acid0-1C06007(2r,3r)-2,3-dihydroxy-3-methylpentanoate;(2r,3r)-2,3-dihydroxy-3-methylpentanoic acid;(r)-2,3-dihydroxy-3-methylpentanoate;(r)-2,3-dihydroxy-3-methylpentanoic acid;(r)-2,3-dihydroxy-3-methylvalerate;1-keto-2-methylvalerate;2,3-dihydroxy-3-methyl-valeric acid;2,3-dihydroxy-3-methylpentanoate;2,3-dihydroxy-3-methylpentanoic acid;2,3-dihydroxy-valerianic acid;4,5-dideoxy-3-c-methyl-pentonic acid;Cid8;Dmv;Alpha,beta-dihydroxy-beta-methylvaleric acid;(2r,3r)-2,3-dihydroxy-3-methylvaleric acid;(2r,3r)-2,3-dihydroxy-3-methylvaleratePW_C007698R23D3M62431513733-Methyl-2-oxovaleric acidHMDB00004913-Methyl-2-oxovaleric acid is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. 3-Methyl-2-oxovaleric acid is a neurotoxin, an acidogen, and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of 3-methyl-2-oxovaleric acid are associated with maple syrup urine disease. MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. The symptoms of MSUD often show in infancy and lead to severe brain damage if untreated. MSUD may also present later depending on the severity of the disease. If left untreated in older individuals, during times of metabolic crisis, symptoms of the condition include uncharacteristically inappropriate, extreme, or erratic behaviour and moods, hallucinations, anorexia, weight loss, anemia, diarrhea, vomiting, dehydration, lethargy, oscillating hypertonia and hypotonia, ataxia, seizures, hypoglycemia, ketoacidosis, opisthotonus, pancreatitis, rapid neurological decline, and coma. In maple syrup urine disease, the brain concentration of branched-chain ketoacids can increase 10- to 20-fold. This leads to a depletion of glutamate and a consequent reduction in the concentration of brain glutamine, aspartate, alanine, and other amino acids. The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis (PMID: 15930465). 3-Methyl-2-oxovaleric acid is a keto-acid, which is a subclass of organic acids. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated MSUD. Many affected children with organic acidemias experience intellectual disability or delayed development.1460-34-0C0346547359322-KETO-3-METHYL-VALERATE46CCC(C)C(=O)C(O)=OC6H10O3InChI=1S/C6H10O3/c1-3-4(2)5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)JVQYSWDUAOAHFM-UHFFFAOYSA-N3-methyl-2-oxopentanoic acid130.1418130.062994186-1.1213-methyl-2-oxopentanoic acid0-1FDB021447(3r)-3-methyl-2-oxopentanoate;(3r)-3-methyl-2-oxopentanoic acid;(r)-3-methyl-2-oxopentanoate;(r)-3-methyl-2-oxopentanoic acid;(s)-3-methyl-2-oxopentanoate;(s)-3-methyl-2-oxopentanoic acid;2-keto-3-methylvalerate;2-keto-3-methylvaleric acid;2-oxo-3-methyl-n-valerate;2-oxo-3-methyl-n-valeric acid;2-oxo-3-methylpentanoate;2-oxo-3-methylpentanoic acid;2-oxo-3-methylvalerate;2-oxo-3-methylvaleric acid;2-oxoisoleucine;2-oxokolavenate;2-oxokolavenic acid;3-methyl-2-oxo-valerate;3-methyl-2-oxo-valeric acid;3-methyl-2-oxo-pentanoate;3-methyl-2-oxo-pentanoic acid;3-methyl-2-oxopentanoate;3-methyl-2-oxopentanoic acid;3-methyl-2-oxovalerate;3-methyl-2-oxovaleric;A-keto-b-methyl-n-valerate;A-keto-b-methyl-n-valeric acid;A-keto-b-methylvalerate;A-keto-b-methylvaleric acid;A-oxo-b-methyl-n-valerate;A-oxo-b-methyl-n-valeric acid;A-oxo-b-methylvalerate;A-oxo-b-methylvaleric acid;Alpha-keto-beta-methyl-n-valerate;Alpha-keto-beta-methyl-n-valeric acid;Alpha-keto-beta-methylvalerate;Alpha-keto-beta-methylvaleric acid;Alpha-oxo-beta-methyl-n-valerate;Alpha-oxo-beta-methyl-n-valeric acid;Alpha-oxo-beta-methylvalerate;Alpha-oxo-beta-methylvaleric acid;3-ethyl-3-methylpyruvic acid;3-ethyl-3-methylpyruvate;α-keto-β-methyl-n-valerate;α-keto-β-methyl-n-valeric acid;α-keto-β-methylvalerate;α-keto-β-methylvaleric acid;α-oxo-β-methyl-n-valerate;α-oxo-β-methyl-n-valeric acid;α-oxo-β-methylvalerate;α-oxo-β-methylvaleric acidPW_C0003733M2Oxva1725862441517918411112154712212410513595L-Glutamic acidHMDB0000148Glutamic acid (Glu), also referred to as glutamate (the anion), is one of the 20 proteinogenic amino acids. It is not among the essential amino acids. Glutamate is a key molecule in cellular metabolism. In humans, dietary proteins are broken down by digestion into amino acids, which serves as metabolic fuel or other functional roles in the body. Glutamate is the most abundant fast excitatory neurotransmitter in the mammalian nervous system. At chemical synapses, glutamate is stored in vesicles. Nerve impulses trigger release of glutamate from the pre-synaptic cell. In the opposing post-synaptic cell, glutamate receptors, such as the NMDA receptor, bind glutamate and are activated. Because of its role in synaptic plasticity, it is believed that glutamic acid is involved in cognitive functions like learning and memory in the brain. Glutamate transporters are found in neuronal and glial membranes. They rapidly remove glutamate from the extracellular space. In brain injury or disease, they can work in reverse and excess glutamate can accumulate outside cells. This process causes calcium ions to enter cells via NMDA receptor channels, leading to neuronal damage and eventual cell death, and is called excitotoxicity. The mechanisms of cell death include: * Damage to mitochondria from excessively high intracellular Ca2+. * Glu/Ca2+-mediated promotion of transcription factors for pro-apoptotic genes, or downregulation of transcription factors for anti-apoptotic genes. Excitotoxicity due to glutamate occurs as part of the ischemic cascade and is associated with stroke and diseases like amyotrophic lateral sclerosis, lathyrism, and Alzheimer's disease. glutamic acid has been implicated in epileptic seizures. Microinjection of glutamic acid into neurons produces spontaneous depolarization around one second apart, and this firing pattern is similar to what is known as paroxysmal depolarizing shift in epileptic attacks. This change in the resting membrane potential at seizure foci could cause spontaneous opening of voltage activated calcium channels, leading to glutamic acid release and further depolarization. (http://en.wikipedia.org/wiki/Glutamic_acid).56-86-0C000253303216015GLT30572DB00142N[C@@H](CCC(O)=O)C(O)=OC5H9NO4InChI=1S/C5H9NO4/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H,7,8)(H,9,10)/t3-/m0/s1WHUUTDBJXJRKMK-VKHMYHEASA-N(2S)-2-aminopentanedioic acid147.1293147.053157781-0.263L-glutamic acid0-1FDB012535(2s)-2-aminopentanedioate;(2s)-2-aminopentanedioic acid;(s)-(+)-glutamate;(s)-(+)-glutamic acid;(s)-2-aminopentanedioate;(s)-2-aminopentanedioic acid;(s)-glutamate;(s)-glutamic acid;1-amino-propane-1,3-dicarboxylate;1-amino-propane-1,3-dicarboxylic acid;1-aminopropane-1,3-dicarboxylate;1-aminopropane-1,3-dicarboxylic acid;2-aminoglutarate;2-aminoglutaric acid;2-aminopentanedioate;2-aminopentanedioic acid;Aciglut;Aminoglutarate;Aminoglutaric acid;E;Glt;Glu;Glusate;Glut;Glutacid;Glutamicol;Glutamidex;Glutaminate;Glutaminic acid;Glutaminol;Glutaton;L-(+)-glutamate;L-(+)-glutamic acid;L-glu;L-glutamate;L-glutaminate;L-glutaminic acid;L-a-aminoglutarate;L-a-aminoglutaric acid;L-alpha-aminoglutarate;L-alpha-aminoglutaric acid;A-aminoglutarate;A-aminoglutaric acid;A-glutamate;A-glutamic acid;Alpha-aminoglutarate;Alpha-aminoglutaric acid;Alpha-glutamate;Alpha-glutamic acid;Acide glutamique;Acido glutamico;Acidum glutamicum;Glutamate;Glutamic acid;L-glutaminsaeurePW_C000095Glu16244365811911384164149699110542144850145626146254532311153441135415117543911855651325631107563210858591056006147607115761919465318568381876844188709272709371716520571822077514224751815182082258373220117921981185516112004222126213112683289126972904234831542349318428453207702025377332133775251127797134677977327779813477829134580649135120023124120040122120086407120347406120692126120816418121147423121153424121157425122833119122997120123299443123401454123719458123725459123729460125401299125418297125457481125667479125769301125802489126941388126995206127162501127257506134Oxoglutaric acidHMDB0000208Oxoglutaric acid, also known as alpha-ketoglutarate, alpha-ketoglutaric acid, AKG, or 2-oxoglutaric acid, is classified as a gamma-keto acid or a gamma-keto acid derivative. gamma-Keto acids are organic compounds containing an aldehyde substituted with a keto group on the C4 carbon atom. alpha-Ketoglutarate is considered to be soluble (in water) and acidic. alpha-Ketoglutarate is a key molecule in the TCA cycle, playing a fundamental role in determining the overall rate of this important metabolic process (PMID: 26759695). In the TCA cycle, AKG is decarboxylated to succinyl-CoA and carbon dioxide by AKG dehydrogenase, which functions as a key control point of the TCA cycle. Additionally, AKG can be generated from isocitrate by oxidative decarboxylation catalyzed by the enzyme known as isocitrate dehydrogenase (IDH). In addition to these routes of production, AKG can be produced from glutamate by oxidative deamination via glutamate dehydrogenase, and as a product of pyridoxal phosphate-dependent transamination reactions (mediated by branched-chain amino acid transaminases) in which glutamate is a common amino donor. AKG is a nitrogen scavenger and a source of glutamate and glutamine that stimulates protein synthesis and inhibits protein degradation in muscles. In particular, AKG can decrease protein catabolism and increase protein synthesis to enhance bone tissue formation in skeletal muscles (PMID: 26759695). Interestingly, enteric feeding of AKG supplements can significantly increase circulating plasma levels of hormones such as insulin, growth hormone, and insulin-like growth factor-1 (PMID: 26759695). It has recently been shown that AKG can extend the lifespan of adult C. elegans by inhibiting ATP synthase and TOR (PMID: 24828042). In combination with molecular oxygen, alpha-ketoglutarate is required for the hydroxylation of proline to hydroxyproline in the production of type I collagen. A recent study has shown that alpha-ketoglutarate promotes TH1 differentiation along with the depletion of glutamine thereby favouring Treg (regulatory T-cell) differentiation (PMID: 26420908). alpha-Ketoglutarate has been found to be associated with fumarase deficiency, 2-ketoglutarate dehydrogenase complex deficiency, and D-2-hydroxyglutaric aciduria, which are all inborn errors of metabolism (PMID: 8338207).328-50-7C0002651309152-KETOGLUTARATE50DB02926OC(=O)CCC(=O)C(O)=OC5H6O5InChI=1S/C5H6O5/c6-3(5(9)10)1-2-4(7)8/h1-2H2,(H,7,8)(H,9,10)KPGXRSRHYNQIFN-UHFFFAOYSA-N2-oxopentanedioic acid146.0981146.021523302-0.442oxoglutarate0-2FDB0033612-ketoglutarate;2-ketoglutaric acid;2-oxo-1,5-pentanedioate;2-oxo-1,5-pentanedioic acid;2-oxoglutarate;2-oxoglutaric acid;2-oxopentanedioate;2-oxopentanedioic acid;Oxoglutarate;Alpha-ketoglutaric acid;Oxoglutaric acid;A-ketoglutarate;A-ketoglutaric acid;Alpha-ketoglutarate;α-ketoglutarate;α-ketoglutaric acidPW_C000134AKG1524231414146849918673311108421263514475014552614675453751035414117543811855641326008147603615560691576092161648217865308574712227515224751915182092258374220118631981268128977054253771351337748111177523112777461297796734577970346779763277798434778425334800183688069413511316294119972406120022124120084407120174122120552414120814418120989408121146423121152424121160425122757120122831119123186450123399454123554374123718458123724459123732460125357479125400299125455481125533297125800489125929482126900501126940388126993206127066205127255506127388502112L-IsoleucineHMDB0000172Branched chain amino acids (BCAA) are essential amino acids whose carbon structure is marked by a branch point. These three amino acids are critical to human life and are particularly involved in stress, energy and muscle metabolism. BCAA supplementation as therapy, both oral and intravenous, in human health and disease holds great promise. "BCAA" denotes valine, isoleucine and leucine which are branched chain essential amino acids. Despite their structural similarities, the branched amino acids have different metabolic routes, with valine going solely to carbohydrates, leucine solely to fats and isoleucine to both. The different metabolism accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg and 16 mg/kg of valine, leucine and isoleucine respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. BCAA are decreased in patients with liver disease, such as hepatitis, hepatic coma, cirrhosis, extrahepatic biliary atresia or portacaval shunt; aromatic amino acids (AAA)-tyrosine, tryptophan and phenylalanine, as well as methionine-are increased in these conditions. Valine, in particular, has been established as a useful supplemental therapy to the ailing liver. All the BCAA probably compete with AAA for absorption into the brain. Supplemental BCAA with vitamin B6 and zinc help normalize the BCAA:AAA ratio. The BCAA are not without side effects. Leucine alone, for example, exacerbates pellagra and can cause psychosis in pellagra patients by increasing excretion of niacin in the urine. Leucine may lower brain serotonin and dopamine. A dose of 3 g of isoleucine added to the niacin regime has cleared leucine-aggravated psychosis in schizophrenic patients. Isoleucine may have potential as an antipsychotic treatment. Leucine is more highly concentrated in foods than other amino acids. A cup of milk contains 800 mg of leucine and only 500 mg of isoleucine and valine. A cup of wheat germ has about 1.6 g of leucine and 1 g of isoleucine and valine. The ratio evens out in eggs and cheese. One egg and an ounce of most cheeses each contain about 400 mg of leucine and 400 mg of valine and isoleucine. The ratio of leucine to other BCAA is greatest in pork, where leucine is 7 to 8 g and the other BCAA together are only 3 to 4 g. (http://www.dcnutrition.com).73-32-5C00407630617191ILE6067DB00167CC[C@H](C)[C@H](N)C(O)=OC6H13NO2InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1AGPKZVBTJJNPAG-WHFBIAKZSA-N(2S,3S)-2-amino-3-methylpentanoic acid131.1729131.094628665-0.062L-isoleucine00FDB012397(2s,3s)-2-amino-3-methylpentanoate;(2s,3s)-2-amino-3-methylpentanoic acid;(2s,3s)-2-amino-3-methyl-pentanoate;(2s,3s)-2-amino-3-methyl-pentanoic acid;(2s,3s)-a-amino-b-methyl-n-valerate;(2s,3s)-a-amino-b-methyl-n-valeric acid;(2s,3s)-a-amino-b-methylvalerate;(2s,3s)-a-amino-b-methylvaleric acid;(2s,3s)-alph-amino-beta-methylvalerate;(2s,3s)-alph-amino-beta-methylvaleric acid;(2s,3s)-alpha-amino-beta-merthyl-n-valerate;(2s,3s)-alpha-amino-beta-merthyl-n-valeric acid;(2s,3s)-alpha-amino-beta-merthylvalerate;(2s,3s)-alpha-amino-beta-merthylvaleric acid;(2s,3s)-alpha-amino-beta-methyl-n-valerate;(2s,3s)-alpha-amino-beta-methyl-n-valeric acid;(2s,3s)-alpha-amino-beta-methylvalerate;(2s,3s)-alpha-amino-beta-methylvaleric acid;(s)-isoleucine;(s,s)-isoleucine;2-amino-3-methylpentanoate;2-amino-3-methylpentanoic acid;2-amino-3-methylvalerate;2-amino-3-methylvaleric acid;2s,3s-isoleucine;Erythro-l-isoleucine;Ile;Iso-leucine;Isoleucine;L-(+)-isoleucine;L-ile;[s-(r*,r*)]-2-amino-3-methylpentanoate;[s-(r*,r*)]-2-amino-3-methylpentanoic acid;Alpha-amino-beta-methylvaleric acid;I;A-amino-b-methylvalerate;A-amino-b-methylvaleric acid;Alpha-amino-beta-methylvalerate;α-amino-β-methylvalerate;α-amino-β-methylvaleric acidPW_C000112Ile172485656107565710871471877148188425423154256231879183111121546122124104135430132-Keto-3-methyl-valerate(3S)-3-Methyl-2-oxopentanoate, also known as (S)-2-oxo-3-methylpentanoate or α-keto-methylvalerate, belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms (3S)-3-Methyl-2-oxopentanoate is soluble (in water) and a weakly acidic compound (based on its pKa) (3S)-3-Methyl-2-oxopentanoate can be biosynthesized from valerate..685740135146CC[C@H](C)C(=O)C([O-])=OC6H9O3InChI=1S/C6H10O3/c1-3-4(2)5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)/p-1/t4-/m0/s1JVQYSWDUAOAHFM-BYPYZUCNSA-M(3S)-3-methyl-2-oxopentanoate129.1339129.055169154-1.070(S)-3-methyl-2-oxopentanoate-1-1α-keto-β-methyl-valerate;-keto-methylvalerate;(3<i>s</i>)-3-methyl-2-oxopentanoate;(3r)-3-methyl-2-oxopentanoate;(3r)-3-methyl-2-oxopentanoic acid;(3s)-3-methyl-2-oxopentanoate;(3s)-3-methyl-2-oxopentanoic acid;(<i>s</i>)-2-oxo-3-methylpentanoate;(<i>s</i>)-3-methyl-2-oxopentanoate;(r)-3-methyl-2-oxopentanoate;(r)-3-methyl-2-oxopentanoic acid;(s)-2-oxo-3-methylpentanoate;(s)-2-oxo-3-methylpentanoic acid;(s)-3-methyl-2-oxopentanoate;(s)-3-methyl-2-oxopentanoic acid;(s)-3-methyl-2-oxovalerate;(s)-3-methyl-2-oxovaleric acid;2-keto-3-methyl-valerate;2-keto-3-methyl-valeric acid;2-keto-3-methylvalerate;2-keto-3-methylvaleric acid;2-oxo-3-methyl-n-valerate;2-oxo-3-methyl-n-valeric acid;2-oxo-3-methylpentanoate;2-oxo-3-methylpentanoic acid;2-oxo-3-methylvalerate;2-oxo-3-methylvaleric acid;2-oxoisoleucine;2-oxokolavenate;2-oxokolavenic acid;3-methyl-2-oxo-pentanoate;3-methyl-2-oxo-pentanoic acid;3-methyl-2-oxo-valerate;3-methyl-2-oxo-valeric acid;3-methyl-2-oxopentanoate;3-methyl-2-oxopentanoic acid;3-methyl-2-oxovalerate;3-methyl-2-oxovaleric;3-methyl-2-oxovaleric acid;A-keto-b-methyl-n-valerate;A-keto-b-methyl-n-valeric acid;A-keto-b-methylvalerate;A-keto-b-methylvaleric acid;A-oxo-b-methyl-n-valerate;A-oxo-b-methyl-n-valeric acid;A-oxo-b-methylvalerate;A-oxo-b-methylvaleric acid;Alpha-keto-beta-methyl-n-valerate;Alpha-keto-beta-methyl-n-valeric acid;Alpha-keto-beta-methyl-valerate;Alpha-keto-beta-methyl-valeric acid;Alpha-keto-beta-methylvalerate;Alpha-keto-beta-methylvaleric acid;Alpha-keto-methylvalerate;Alpha-keto-methylvaleric acid;Alpha-oxo-beta-methyl-n-valerate;Alpha-oxo-beta-methyl-n-valeric acid;Alpha-oxo-beta-methylvalerate;Alpha-oxo-beta-methylvaleric acidPW_C0430132K3MVal11158Threonine dehydratase biosynthetic, chloroplasticQ9ZSS6
Catalyzes the formation of alpha-ketobutyrate from threonine in a two-step reaction. The first step is a dehydration of threonine, followed by rehydration and liberation of ammonia.
OMR144.3.1.198163225111592-hydroxyacyl-CoA lyaseQ9LF46
Catalyzes a carbon-carbon cleavage reaction; cleaves a 2-hydroxy-3-methylacyl-CoA into formyl-CoA and a 2-methyl-branched fatty aldehyde.
HACL44.1.-.-81641519659UnknownUnknown45.4.99.-; 4.2.1.-5930151746622274902237494224817122592722218096225380963379809643801366072931370456931370512494922threonine deaminase4PW_P004922121751115844923acetohydroxybutanoate synthase / acetolactate synthase 4PW_P004923121769659212177965924924acetohydroxybutanoate synthase / acetolactate synthase 24PW_P004924121789659212179965924925acetolactate synthase / acetohydroxybutanoate synthase 34PW_P004925121809659212181965924926acetohydroxy acid isomeroreductase4PW_P00492612182965944927dihydroxy acid dehydratase4PW_P00492712183965924928branched-chain amino-acid 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2577 180 C2627 180 2677 230 2677 280 C2677 568 2677 944 2677 1232 C2677 1282 2627 1332 2577 1332 C1864 1332 938 1332 225 1332 C175 1332 125 1282 125 1232 C125 944 125 568 125 280 1true62552.01152.0783281544402256135056939#EBFFEB4948313