2194PathwayLysine MetabolismThe biosynthesis of lysine starts with oxoglutaric acid interacting with acetyl-coa through a homocitrate synthase resulting in the release of homocitric acid. This reaction may happen in the cytosol or in the mitochondria. The homocitric acid spontaneously releases water an is transformed into cis-homoaconitate, The cis-homoaconitate reacts with homoaconitase resulting in the release of water and a homoisocitrate. Homoisocitrate reacts with a NAD dependent homoisocitrate dehydrogenase resulting in the release of a carbon dioxide, a NADH and a oxoadipic acid. These set of reactions happen in the mitochondria. Oxoadipic acid reacts with a glutamic acid resulting in the release of oxoglutaric acid and aminoadipic acid. The aminoadipic acid reacts with a holo-[LYS2 peptidyl-carrier-protein] through an ATP driven L-2-aminoadipate reductase resulting in the release of AMP, pyrophosphate and L-2-aminoadipyl-[lys2 peptidyl-carrier-protein]. This resulting element reacts with a NADPH dependent L-2-aminoadipate reductase resulting in the release of allysine. Allysine reacts with a glutamic acid through a NADPH dependent saccharopine dehydrogenase resulting in the release of water, NADP and saccharopine. Saccharopine reacts with a water molecule and a NAD dependent saccharopine dehydrogenase resulting in the release of oxoglutaric acid and L-lysine. This last reaction is reversible and leads to the degradation of lysineMetabolicPW002420CenterPathwayVisualizationContext270627502347#000099PathwayVisualization21772194Lysine MetabolismThe biosynthesis of lysine starts with oxoglutaric acid interacting with acetyl-coa through a homocitrate synthase resulting in the release of homocitric acid. This reaction may happen in the cytosol or in the mitochondria. The homocitric acid spontaneously releases water an is transformed into cis-homoaconitate, The cis-homoaconitate reacts with homoaconitase resulting in the release of water and a homoisocitrate. Homoisocitrate reacts with a NAD dependent homoisocitrate dehydrogenase resulting in the release of a carbon dioxide, a NADH and a oxoadipic acid. These set of reactions happen in the mitochondria. Oxoadipic acid reacts with a glutamic acid resulting in the release of oxoglutaric acid and aminoadipic acid. The aminoadipic acid reacts with a holo-[LYS2 peptidyl-carrier-protein] through an ATP driven L-2-aminoadipate reductase resulting in the release of AMP, pyrophosphate and L-2-aminoadipyl-[lys2 peptidyl-carrier-protein]. This resulting element reacts with a NADPH dependent L-2-aminoadipate reductase resulting in the release of allysine. Allysine reacts with a glutamic acid through a NADPH dependent saccharopine dehydrogenase resulting in the release of water, NADP and saccharopine. Saccharopine reacts with a water molecule and a NAD dependent saccharopine dehydrogenase resulting in the release of oxoglutaric acid and L-lysine. This last reaction is reversible and leads to the degradation of lysineMetabolic18509610975256El Alami M, Feller A, Pierard A, Dubois E: Characterisation of a tripartite nuclear localisation sequence in the regulatory protein Lys14 of Saccharomyces cerevisiae. Curr Genet. 2000 Aug;38(2):78-86.2194Pathway509710103047Feller A, Ramos F, Pierard A, Dubois E: In Saccharomyces cerevisae, feedback inhibition of homocitrate synthase isoenzymes by lysine modulates the activation of LYS gene expression by Lys14p. Eur J Biochem. 1999 Apr;261(1):163-70.2194Pathway5098Hinnebusch, A. General and pathway-specific regulatory mechanisms controlling the synthesis of amino acid biosynthetic enzymes in Saccharomyces cerevisiae. The Molecular and Cellular Biology of the yeast Saccharomyces: Gene Expression. 1992;2:319-414.2194Pathway509910320345Ehmann DE, Gehring AM, Walsh CT: Lysine biosynthesis in Saccharomyces cerevisiae: mechanism of alpha-aminoadipate reductase (Lys2) involves posttranslational phosphopantetheinylation by Lys5. Biochemistry. 1999 May 11;38(19):6171-7. doi: 10.1021/bi9829940.2194Pathway1CellCL:00000006MyocyteCL:00001875HepatocyteCL:00001824Cardiomyocyte CL:00007463NeuronCL:00005407Epithelial CellCL:00000662Platelet CL:00002338Beta cellCL:00006391Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote17Rattus norvegicus10116EukaryoteRat12Mus musculus10090EukaryoteMouse24Solanum lycopersicum4081EukaryoteTomato18Saccharomyces cerevisiae4932EukaryoteYeast21Xenopus laevis8355EukaryoteAfrican clawed frog4Arabidopsis thaliana3702EukaryoteThale cress49Bathymodiolus platifrons220390EukaryoteDeep sea mussel60Nitzschia sp.0001EukaryoteNitzschia45Bos taurus9913EukaryoteCattle10Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm23Pseudomonas aeruginosa287Prokaryote2Bacteria2ProkaryoteBacteria19Schizosaccharomyces pombe4896Eukaryote25Escherichia coli (strain K12)83333Prokaryote51Picea sitchensis3332EukaryoteSitka spruce7Chlamydomonas reinhardtii3055Eukaryote1CytosolGO:00058292MitochondrionGO:00057393Mitochondrial MatrixGO:00057595CytoplasmGO:000573713Endoplasmic ReticulumGO:000578324Mitochondrial Intermembrane SpaceGO:00057584PeroxisomeGO:000577710Cell MembraneGO:000588635ChloroplastGO:000950712Mitochondrial Inner MembraneGO:00057438Smooth Endoplasmic Reticulum GO:00057906LysosomeGO:000576411Extracellular SpaceGO:000561516Lysosomal LumenGO:00432027Endoplasmic Reticulum MembraneGO:000578918Melanosome MembraneGO:003316225Golgi apparatusGO:000579414Mitochondrial Outer MembraneGO:000574120Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520215NucleusGO:000563431Periplasmic SpaceGO:000562036MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032540PeriplasmGO:004259727Peroxisome MembraneGO:000577832Inner MembraneGO:007025819sarcoplasmic reticulumGO:001652917NucleoplasmGO:00056541LiverBTO:00007597299MuscleBTO:00008871411824BrainBTO:000014289164Adrenal MedullaBTO:000004971825IntestineBTO:000064828StomachBTO:0001307155267Nervous SystemBTO:00014848Blood VesselBTO:0001102741111HeartBTO:000056273102Endothelium 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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_C000134AKG1524231414146849918673311108421263514475014552614675453751035414117543811855641326008147603615560691576092161648217865308574712227515224751915182092258374220118631981268128977054253771351337748111177523112777461297796734577970346779763277798434778425334800183688069413511316294119972406120022124120084407120174122120552414120814418120989408121146423121152424121160425122757120122831119123186450123399454123554374123718458123724459123732460125357479125400299125455481125533297125800489125929482126900501126940388126993206127066205127255506127388502940Acetyl-CoAHMDB0001206The main function of coenzyme A is to carry acyl groups (such as the acetyl group) or thioesters. Acetyl-CoA is an important molecule itself. It is the precursor to HMG CoA, which is a vital component in cholesterol and ketone synthesis. (wikipedia). acetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.72-89-9C0002444449315351ACETYL-COA392413CC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC23H38N7O17P3SInChI=1S/C23H38N7O17P3S/c1-12(31)51-7-6-25-14(32)4-5-26-21(35)18(34)23(2,3)9-44-50(41,42)47-49(39,40)43-8-13-17(46-48(36,37)38)16(33)22(45-13)30-11-29-15-19(24)27-10-28-20(15)30/h10-11,13,16-18,22,33-34H,4-9H2,1-3H3,(H,25,32)(H,26,35)(H,39,40)(H,41,42)(H2,24,27,28)(H2,36,37,38)/t13-,16-,17-,18+,22-/m1/s1ZSLZBFCDCINBPY-ZSJPKINUSA-N{[(2R,3S,4R,5R)-2-({[({[(3R)-3-[(2-{[2-(acetylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]-3-hydroxy-2,2-dimethylpropoxy](hydroxy)phosphoryl}oxy)(hydroxy)phosphoryl]oxy}methyl)-5-(6-amino-9H-purin-9-yl)-4-hydroxyoxolan-3-yl]oxy}phosphonic acid809.571809.125773051-2.279acetyl-CoA0-4FDB022491Ac-coa;Ac-coenzyme a;Ac-s-coa;Ac-s-coenzyme a;Acetyl coenzyme-a;Acetyl-coa;Acetyl-coenzyme a;Acetyl-s-coa;Acetyl-s-coenzyme a;Acetylcoenzyme-a;S-acetate coa;S-acetate coenzyme a;S-acetyl coenzyme a;Accoa;Acetyl coenzyme a;S-acetyl-coa;S-acetyl-coenzyme a;Acetylcoenzyme aPW_C000940Ac-CoA21343858842324162244652896173340114840145278103547612457331086025155607716163861647017869231607106163729119874602228245151827721012582226130122994261531577121133772911117756211277706132779941157835513478433334800073688063411980663376901241701199534061201454051203041221206324071224174081226263841227431201229591351231371181249863741252001211253434791255074781256332971265644821265724811267784801268865011270442091273942051276653881281375021281452061283743911420WaterHMDB0002111Water 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_C001420H2O55894910951394151316214481135261562428652106912077033823188382109431137749146554159043201824253222267860272746277817280529314370316472363461459836472737494193503027515675195975214100522794523610352971055319111534311353551125402110547012354831255492126550712755341305537114554112955911355608118562210856916575914057781015841143585314658771075890955910147594015160321556059157608716161231636133159621516218166647717865071806600152671311768401886888160716220571812077193206721121172282137238214724321572951987350216738821074012127467222749222475001907588170820122582372268414162926526118502771192216412011281122132851225028612264287123272491252022712632651269329012705291127152921300729813019300130253011303730213261223133272941534030842327315426953184369132276914293770192537710213277131133772151347737833177397332774713337751611577536334776283367772233777759341778163437798234778071329782353527824235378270356791133608001436880039370805912288065611993830383947943841105573901106393911158443981198792321199151221199634061200084071200464081201131241203654121204304051204384091206064151207944141211584251212404291213511211213814191216074341221183821223844361227531201227973741228044431230124461230643761230721371231314471231421361231624481232314511233844501237304601238104641239404551241654691246703991249384711249454721253052971253534791253864811254244821254802991256824831257074781257454871260544901262384951262734841267644801268965011269635021270173881271772081271992091272275041275065071275765151278363891280823951281765131099Coenzyme AHMDB0001423Coenzyme A (CoA, CoASH, or HSCoA) is a coenzyme notable for its role in the synthesis and oxidization of fatty acids and the oxidation of pyruvate in the citric acid cycle. It is adapted from beta-mercaptoethylamine, panthothenate, and adenosine triphosphate. It is also a parent compound for other transformation products, including but not limited to, phenylglyoxylyl-CoA, tetracosanoyl-CoA, and 6-hydroxyhex-3-enoyl-CoA. Coenzyme A is synthesized in a five-step process from pantothenate and cysteine. In the first step pantothenate (vitamin B5) is phosphorylated to 4'-phosphopantothenate by the enzyme pantothenate kinase (PanK, CoaA, CoaX). In the second step, a cysteine is added to 4'-phosphopantothenate by the enzyme phosphopantothenoylcysteine synthetase (PPC-DC, CoaB) to form 4'-phospho-N-pantothenoylcysteine (PPC). In the third step, PPC is decarboxylated to 4'-phosphopantetheine by phosphopantothenoylcysteine decarboxylase (CoaC). In the fourth step, 4'-phosphopantetheine is adenylylated to form dephospho-CoA by the enzyme phosphopantetheine adenylyl transferase (CoaD). Finally, dephospho-CoA is phosphorylated using ATP to coenzyme A by the enzyme dephosphocoenzyme A kinase (CoaE). Since coenzyme A is, in chemical terms, a thiol, it can react with carboxylic acids to form thioesters, thus functioning as an acyl group carrier. CoA assists in transferring fatty acids from the cytoplasm to the mitochondria. A molecule of coenzyme A carrying an acetyl group is also referred to as acetyl-CoA. When it is not attached to an acyl group, it is usually referred to as 'CoASH' or 'HSCoA'. Coenzyme A is also the source of the phosphopantetheine group that is added as a prosthetic group to proteins such as acyl carrier proteins and formyltetrahydrofolate dehydrogenase. Acetyl-CoA is an important molecule itself. It is the precursor to HMG CoA which is a vital component in cholesterol and ketone synthesis. Furthermore, it contributes an acetyl group to choline to produce acetylcholine in a reaction catalysed by choline acetyltransferase. Its main task is conveying the carbon atoms within the acetyl group to the citric acid cycle to be oxidized for energy production (Wikipedia).85-61-0C0001068161146900CO-A6557CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)[C@@H](O)C(=O)NCCC(=O)NCCSC21H36N7O16P3SInChI=1S/C21H36N7O16P3S/c1-21(2,16(31)19(32)24-4-3-12(29)23-5-6-48)8-41-47(38,39)44-46(36,37)40-7-11-15(43-45(33,34)35)14(30)20(42-11)28-10-27-13-17(22)25-9-26-18(13)28/h9-11,14-16,20,30-31,48H,3-8H2,1-2H3,(H,23,29)(H,24,32)(H,36,37)(H,38,39)(H2,22,25,26)(H2,33,34,35)/t11-,14-,15-,16+,20-/m1/s1RGJOEKWQDUBAIZ-IBOSZNHHSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[(3R)-3-hydroxy-2,2-dimethyl-3-({2-[(2-sulfanylethyl)carbamoyl]ethyl}carbamoyl)propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid767.534767.115208365-2.2210coenzyme A0-4FDB022614Acetoacetyl coenzyme a sodium salt;Coa;Coa hydrate;Coa-sh;Coash;Coenzyme a;Coenzyme a hydrate;Coenzyme a-sh;Coenzyme ash;Coenzymes a;Depot-zeel;Propionyl coa;Propionyl coenzyme a;S-propanoate;S-propanoate coa;S-propanoate coenzyme a;S-propanoic acid;S-propionate coa;S-propionate coenzyme a;Zeel;[(2r,3s,4r,5r)-5-(6-amino-9h-purin-9-yl)-4-hydroxy-3-(phosphonooxy)tetrahydrofuran-2-yl]methyl 3-hydroxy-4-({3-oxo-3-[(2-sulfanylethyl)amino]propyl}amino)-2,2-dimethyl-4-oxobutyl dihydrogen diphosphatePW_C001099CoA21143868845387922892172407592414224595281329286231334211335118461810462958484214486554487965232102524710452801035477124573410857771016023155607516163841646817869301606961162697319970831887108163729319873472107458222822915190812269090224912417092151951301329915318249254884942616315769072937711913377222134772303297729211177550132775553347756311277633336776721297799611578047332780563507841333578567130792593337997433180005368806201188062737480635119806653769382838293834383986742881105553891105613901158423991158473981199514061201474051202313841203051221206344071207621171214061231214214331215211251216664291216824081217144141224044221227411201229041211229601351239654471239794681240791361242204641242654501249743751253414791255094781255794801255924841256342971260844811265494911265604821267463001268845011270462091271093911273012051275402061276673881281215081281335021283403951911Homocitric acidHMDB0003518Homocitric acid is a normal urinary organic acid. (PMID: 14708889). Homocitric acid is a citric acid analogue found as a minor metabolite in urine samples from patients with propionic acidaemia. Homocitric acid is formed by citrate synthase due to propionyl CoA carboxylase deficiency (by the citrate synthase condensation reaction of a-ketoglutarate with acetyl coenzyme A and propionyl coenzyme A). (PMID: 7850997).3562-74-1C0125143945952222HOMO-CIT388564OC(=O)CC[C@@](O)(CC(O)=O)C(O)=OC7H10O7InChI=1S/C7H10O7/c8-4(9)1-2-7(14,6(12)13)3-5(10)11/h14H,1-3H2,(H,8,9)(H,10,11)(H,12,13)/t7-/m1/s1XKJVEVRQMLKSMO-SSDOTTSWSA-N(2R)-2-hydroxybutane-1,2,4-tricarboxylic acid206.1501206.042652674-0.034(R)-homocitric acid0-3FDB023186(r)-2-hydroxy-1,2,4-butanetricarboxylate;(r)-2-hydroxy-1,2,4-butanetricarboxylic acid;2-hydroxybutane-1,2,4-tricarboxylate;3-hydroxy-3-carboxyadipate;3-hydroxy-3-carboxyadipic acid;Homocitrate;(r)-2-hydroxybutane-1,2,4-tricarboxylic acid;(r)-homocitric acid;Homocitric acid;(2r)-2-hydroxybutane-1,2,4-tricarboxylate;(r)-2-hydroxybutane-1,2,4-tricarboxylate;(r)-homocitratePW_C001911Homocit40034Hydrogen 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+21546708753157883184831116214632614645422314927801742502242544245471045761846947052411035327111535311256261085639107569910057201055742117596314760371556070157609316161301596232166648317866011526692101684318869101877100163716820571912067453219745422074722227525213753221075582127572160759017081952258218151824322684131628420224913919591552491191516412015281121812851224628612266287125212271325722313325294153303084232931542354318424013224240531242454320769122937713613377210134773723317780411477955132779903277799134778379345799291308001936880387310803883048072211993823124948233831105503881128559411328039011553739811553911811585633611620510911997340612019340712054912212059340912117042412117142512256941812261538412268712512275812012318313512321813712374245912374346012514145412518812112527313612535947912555048112573048312573629712580929912651749512671748912676648012682330012690250112721320812830850612836139112843039544558cis-homoaconitateBut-1-ene-1,2,4-tricarboxylate, also known as cis-homoaconitate or 3-carboxyhex-2-enedioate, belongs to the class of organic compounds known as tricarboxylic acids and derivatives. These are carboxylic acids containing exactly three carboxyl groups. But-1-ene-1,2,4-tricarboxylate is soluble (in water) and a moderately acidic compound (based on its pKa).21158450[H]\C(C([O-])=O)=C(/CCC([O-])=O)C([O-])=OC7H5O6InChI=1S/C7H8O6/c8-5(9)2-1-4(7(12)13)3-6(10)11/h3H,1-2H2,(H,8,9)(H,10,11)(H,12,13)/p-3/b4-3-BJYPZFUWWJSAKC-ARJAWSKDSA-K(1Z)-but-1-ene-1,2,4-tricarboxylate185.113185.010258621-1.060cis-homoaconitate-3-33-carboxyhex-2-enedioate, (z)-but-1-ene-1,2,4-tricarboxylate, homo-cis-aconitate, (z)-1,2,4-but-1-enetricarboxylic acidPW_C044558cishomo44559(1R,2S)-homoisocitrate(1R,2S)-Homoisocitrate, also known as homoisocitric acid or homo-i-cit, belongs to the class of organic compounds known as tricarboxylic acids and derivatives. These are carboxylic acids containing exactly three carboxyl groups (1R,2S)-Homoisocitrate is soluble (in water) and a weakly acidic compound (based on its pKa).5459812[H][C@](O)(C([O-])=O)[C@]([H])(CCC([O-])=O)C([O-])=OC7H7O7InChI=1S/C7H10O7/c8-4(9)2-1-3(6(11)12)5(10)7(13)14/h3,5,10H,1-2H2,(H,8,9)(H,11,12)(H,13,14)/p-3/t3-,5+/m0/s1OEJZZCGRGVFWHK-WVZVXSGGSA-K(1R,2S)-1-hydroxybutane-1,2,4-tricarboxylate203.128203.020823305-0.451(-)-homoisocitrate(3-)-3-3(-)-threo-isohomocitrate, (-)-1-hydroxy-1,2,4-butanetricarboxylate, homo-i-cit, 1-hydroxy-1,2,4-butanetricarboxylate, 2-hydroxy-3-carboxyadipate, homo-iso-citrate, 1-(r)-hydroxy-2-(s)-1,2,4-butanetricarboxylic acid, 1-hydroxybutane-1,2,4-tricarboxylate, (1r,2s)-1-hydroxybutane-1,2,4-tricarboxylate, homoisocitratePW_C04455912H721NADHMDB0000902NAD (or Nicotinamide adenine dinucleotide) is used extensively in glycolysis and the citric acid cycle of cellular respiration. The reducing potential stored in NADH can be converted to ATP through the electron transport chain or used for anabolic metabolism. ATP "energy" is necessary for an organism to live. Green plants obtain ATP through photosynthesis, while other organisms obtain it by cellular respiration. (wikipedia). Nicotinamide adenine dinucleotide is a A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed).53-84-9C00003589315846NAD5682NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1OC21H28N7O14P2InChI=1S/C21H27N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1-4,7-8,10-11,13-16,20-21,29-32H,5-6H2,(H5-,22,23,24,25,33,34,35,36,37)/p+1/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BAWFJGJZGIEFAR-NNYOXOHSSA-O1-[(2R,3R,4S,5R)-5-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium664.433664.116946663-2.5981-[(2R,3R,4S,5R)-5-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium1-1FDB0223093-carbamoyl-1-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;3-carbamoyl-1-beta-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-beta-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;Adenine-nicotinamide dinucleotide;Co-i;Codehydrase i;Codehydrogenase i;Coenzyme i;Cozymase;Cozymase i;Diphosphopyridine nucleotide;Diphosphopyridine nucleotide oxidized;Endopride;Nad trihydrate;Nad-oxidized;Nicotinamide adenine dinucleotide;Nicotinamide adenine dinucleotide oxidized;Nicotinamide dinucleotide;Nicotineamide adenine dinucleotide;Oxidized diphosphopyridine nucleotide;Pyridine nucleotide diphosphate;[(3s,2r,4r,5r)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl {[(3s,2r,4r,5r)-5-(3-carbamoylpyridyl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxyphosphoryl) hydrogen phosphate;[adenylate-32-p]-nad;Beta-diphosphopyridine nucleotide;Beta-nad;Beta-nicotinamide adenine dinucleotide;Beta-nicotinamide adenine dinucleotide trihydrate;Dpn;Nad;Nad+;Nadide;B-nad;β-nadPW_C000721NAD140415033538651101114211344312735146654222949277917283529310794807184813184819284902649603151679552381035334111536011254691235482125559013556101185696100573810858271415912147594215160241556072157607616163851646917867721176890160701218870971637174205719720674051987459222824122683592259085224118192161232224913006298130183001325622342404322426193157710413277120133772091347737033177650336776673347770233277709130779151137798334778406356800063688069011993825124110552388112750166112853941199291221199524061201714071208344191209844081211594251212421261212594291218173831226143841227421201231304471231411361234194551235493741237314601238124431238294641243703981251871211253192971253424791255304811258062991258254901259244821265154951267654801268855011272785071273835021280893901283603911284283951316Carbon 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_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905021144NADHHMDB0001487NADH is the reduced form of NAD+, and NAD+ is the oxidized form of NADH, A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). It forms NADP with the addition of a phosphate group to the 2' position of the adenosyl nucleotide through an ester linkage.(Dorland, 27th ed).58-68-4C0000443915316908NADH388299DB00157NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1OC21H29N7O14P2InChI=1S/C21H29N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1,3-4,7-8,10-11,13-16,20-21,29-32H,2,5-6H2,(H2,23,33)(H,34,35)(H,36,37)(H2,22,24,25)/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BOPGDPNILDQYTO-NNYOXOHSSA-N[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]({[(2R,3S,4R,5R)-5-(3-carbamoyl-1,4-dihydropyridin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy})phosphinic acid665.441665.124771695-2.358NADH0-2FDB0226491,4-dihydronicotinamide adenine dinucleotide;Dpnh;Dihydrocodehydrogenase i;Dihydrocozymase;Dihydronicotinamide adenine dinucleotide;Dihydronicotinamide mononucleotide;Enada;Nadh;Nadh2;Reduced codehydrogenase i;Reduced diphosphopyridine nucleotide;Reduced nicotinamide adenine diphosphate;Reduced nicotinamide-adenine dinucleotide;B-dpnh;B-nadh;Beta-dpnh;Beta-nadh;Nicotinamide adenine dinucleotide (reduced);Reduced nicotinamide adenine dinucleotidePW_C001144NADH14341533490864810111521275514695422304927811728362931099480618481218482128490464959315169955240103533211153581125466123547912555931355698100573710858291415915147594515160271556079161638716472178677111768931607011188709916371722057195206746222282442268360225908622411809198118212161232024913003298130153001325522342403322426183157710713277123133772081347737133177651336776683347770033277707130779171137798634780009368806911199382212411054938811285494115838118119955406120172407120378122120986408121162425121244126121693429121818383122616384122745120123127447123138136123551374123734460123814443124242464124371398125189121125345479125531481125762297125808299125926482126516495126767480126888501127385502128090390128362391128429395150Oxoadipic acidHMDB00002252-Oxoadipic acid is produced from lysine in the cytosol of cells via the saccharopine and the pipecolic acid pathways. Catabolites of hydroxylysine and tryptophan enter these pathways as 2-aminoadipic-semialdehyde and 2-oxoadipate, respectively. In the mitochondrial matrix, 2-oxoadipate is decarboxylated to glutaryl-CoA by the 2-oxoadipate dehydrogenase complex and then converted into acetyl-CoA. Chronically high levels of oxoadipic acid are associated with at least two inborn errors of metabolism, including 2-aminoadipic aciduria and 2-oxoadipic aciduria. 2-Oxoadipic aciduria is an inborn error of metabolism involving lysine, tryptophan, and hydroxylysine, in which abnormal quantities of 2-aminoadipic acid are found in body fluids along with 2-oxoadipic acid. Patients with 2-oxoadipic acidemias are mentally retarded with hypotonia or seizures. 2-Oxoadipic aciduria can occur in patients with Kearns-Sayre syndrome, a progressive disorder with onset prior to 20 years of age in which multiple organ systems are affected. Affected individuals have progressive external ophthalmoplegia (PEO) and retinopathy, both of which are classically associated with abnormalities in cardiac conduction, cerebellar signs, and elevated cerebrospinal fluid protein (PMID: 10655159, 16183823, 11083877). When present in sufficiently high levels, oxoadipic acid can act as an acidogen and a metabotoxin. 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. Oxoadipic acid is an organic acid. 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 abnormalities, kidney abnormalities, liver damage, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.3184-35-8C0032271157532K-ADIPATE70OC(=O)CCCC(=O)C(O)=OC6H8O5InChI=1S/C6H8O5/c7-4(6(10)11)2-1-3-5(8)9/h1-3H2,(H,8,9)(H,10,11)FGSBNBBHOZHUBO-UHFFFAOYSA-N2-oxohexanedioic acid160.1247160.037173366-0.832oxoadipate0-2FDB0033622-keto-adipate;2-ketoadipate;2-ketoadipic acid;2-oxo-hexanedioate;2-oxo-hexanedioic acid;2-oxoadipate;2-oxoadipic acid;2-oxohexanedioate;2-oxohexanedioic acid;2-oxohexanedionic acid;Oxoadipate;A-ketoadipate;A-ketoadipic acid;A-oxoadipate;A-oxoadipic acid;Alpha-ketoadipate;Alpha-ketoadipic acid;Alpha-oxoadipate;Alpha-oxoadipic acidPW_C000150Oxoadip1071382281517827911212078740712337811995L-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_C000095Glu16244365811911384164149699110542144850145626146254532311153441135415117543911855651325631107563210858591056006147607115761919465318568381876844188709272709371716520571822077514224751815182082258373220117921981185516112004222126213112683289126972904234831542349318428453207702025377332133775251127797134677977327779813477829134580649135120023124120040122120086407120347406120692126120816418121147423121153424121157425122833119122997120123299443123401454123719458123725459123729460125401299125418297125457481125667479125769301125802489126941388126995206127162501127257506390Aminoadipic acidHMDB0000510Aminoadipic acid (2-aminoadipate) is a metabolite in the principal biochemical pathway of lysine. It is an intermediate in the metabolism (i.e. breakdown or degradation) of lysine and saccharopine. It antagonizes neuroexcitatory activity modulated by the glutamate receptor N-methyl-D-aspartate (NMDA). Aminoadipic acid has also been shown to inhibit the production of kynurenic acid, a broad spectrum excitatory amino acid receptor antagonist, in brain tissue slices (PMID: 8566117). Recent studies have shown that aminoadipic acid is elevated in prostate biopsy tissues from prostate cancer patients (PMID: 23737455). Mutations in DHTKD1 (dehydrogenase E1 and transketolase domain-containing protein 1) have been shown to cause human 2-aminoadipic aciduria and 2-oxoadipic aciduria via impaired decarboxylation of 2-oxoadipate to glutaryl-CoA, which is the last step in the lysine degradation pathway (PMID: 23141293). Aging, diabetes, sepsis, and renal failure are known to catalyze the oxidation of lysyl residues to form 2-aminoadipic acid in human skin collagen and potentially other tissues (PMID: 18448817). Proteolytic breakdown of these tissues can lead to the release of free 2-aminoadipic acid. Studies in rats indicate that aminoadipic acid (along with the three branched-chain amino acids - leucine, valine, and isoleucine) levels are elevated in the pre-diabetic phase and so aminoadipic acid may serve as a predictive biomarker for the development of diabetes (PMID: 15389298). Long-term hyperglycemia of endothelial cells can also lead to elevated levels of aminoadipate which is thought to be a sign of lysine breakdown through oxidative stress and reactive oxygen species (ROS) (PMID: 21961526). 2-Aminoadipate is a potential small-molecule marker of oxidative stress (PMID: 21647514). Therefore, depending on the circumstances aminoadipic acid can act as an acidogen, a diabetogen, an atherogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A diabetogen is a compound that can lead to type 2 diabetes. An atherogen is a compound that leads to atherosclerosis and cardiovascular disease. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of aminoadipic acid are associated with at least two inborn errors of metabolism including, 2-aminoadipic aciduria and 2-oxoadipic aciduria. Aminoadipic acid is an organic acid and 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 abnormalities, kidney abnormalities, liver damage, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures. As a diabetogen, serum aminoadipic levels appear to regulate glucose homeostasis and have been highly predictive of individuals who later develop diabetes (PMID: 24091325). In particular, aminoadipic acid lowers fasting plasma glucose levels and enhances insulin secretion from human islets. As an atherogen, aminoadipic acid has been found to be produced at high levels via protein lysine oxidation in atherosclerotic plaques (PMID: 28069522).542-32-5C0095646937024CPD-468456NC(CCCC(O)=O)C(O)=OC6H11NO4InChI=1S/C6H11NO4/c7-4(6(10)11)2-1-3-5(8)9/h4H,1-3,7H2,(H,8,9)(H,10,11)OYIFNHCXNCRBQI-UHFFFAOYSA-N2-aminohexanedioic acid161.1558161.068807845-0.693aminoadipic acid0-1FDB021812(+/-)-2-aminoadipate;(+/-)-2-aminoadipic acid;2-aminoadipate;2-aminoadipic acid;Aminoadipate;Dl-2-aminoadipate;Dl-2-aminoadipic acid;Dl-2-aminohexanedioate;Dl-2-aminohexanedioic acid;Dl-a-aminoadipate;Dl-a-aminoadipic acid;Dl-alpha-aminoadipate;Dl-alpha-aminoadipic acid;L-2-aminoadipate;L-2-aminoadipic acid;L-2-aminohexanedioate;L-2-aminohexanedioic acid;L-alpha-aminoadipate;L-alpha-aminoadipic acid;A-aminoadipate;A-aminoadipic acid;Alpha-amino-adipic acid;Alpha-aminoadipate;Alpha-aminoadipic acid;Aad;Aminoadipic acidPW_C000390AAD10693822715178278112120785407123376119414Adenosine triphosphateHMDB0000538Adenosine triphosphate (ATP) is a nucleotide consisting of a purine base (adenine) attached to the first carbon atom of ribose (a pentose sugar). Three phosphate groups are esterified at the fifth carbon atom of the ribose. ATP is incorporated into nucleic acids by polymerases in the processes of DNA replication and transcription. ATP contributes to cellular energy charge and participates in overall energy balance, maintaining cellular homeostasis. ATP can act as an extracellular signaling molecule via interactions with specific purinergic receptors to mediate a wide variety of processes as diverse as neurotransmission, inflammation, apoptosis, and bone remodelling. Extracellular ATP and its metabolite adenosine have also been shown to exert a variety of effects on nearly every cell type in human skin, and ATP seems to play a direct role in triggering skin inflammatory, regenerative, and fibrotic responses to mechanical injury, an indirect role in melanocyte proliferation and apoptosis, and a complex role in Langerhans cell-directed adaptive immunity. During exercise, intracellular homeostasis depends on the matching of adenosine triphosphate (ATP) supply and ATP demand. Metabolites play a useful role in communicating the extent of ATP demand to the metabolic supply pathways. Effects as different as proliferation or differentiation, chemotaxis, release of cytokines or lysosomal constituents, and generation of reactive oxygen or nitrogen species are elicited upon stimulation of blood cells with extracellular ATP. The increased concentration of adenosine triphosphate (ATP) in erythrocytes from patients with chronic renal failure (CRF) has been observed in many studies but the mechanism leading to these abnormalities still is controversial. (PMID: 15490415, 15129319, 14707763, 14696970, 11157473).56-65-5C00002595715422ATP5742DB00171NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1OC10H16N5O13P3InChI=1S/C10H16N5O13P3/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(26-10)1-25-30(21,22)28-31(23,24)27-29(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H,23,24)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1ZKHQWZAMYRWXGA-KQYNXXCUSA-N({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)phosphonic acid507.181506.995745159-2.057adenosine triphosphate0-3FDB0218135'-(tetrahydrogen triphosphate) adenosine;5'-atp;Atp;Adenosine 5'-triphosphate;Adenosine 5'-triphosphorate;Adenosine 5'-triphosphoric acid;Adenosine triphosphate;Adenylpyrophosphorate;Adenylpyrophosphoric acid;Adephos;Adetol;Adynol;Atipi;Atriphos;Cardenosine;Fosfobion;Glucobasin;Myotriphos;Phosphobion;Striadyne;Triadenyl;Triphosphaden;Triphosphoric acid adenosine ester;Adenosine-5'-triphosphate;H4atp;Adenosine triphosphoric acid;Adenosine-5'-triphosphoric acidPW_C000414ATP92214608266164142247813733327995934399763210518211210214649215614216058240559243427272646281229302966316372361661361751439923447431476891486454503289503526515575205975215100525010452911015313111534611253901035406117543011854431205542129555613255691335603135562110858461435854146587610758971475924151604815561091616230166649317868391886870160697619971572057184206720921072252137229211729819873022167390217740821874321637481222749919081862251184727711903170120102811203916412178285125782261269129013264223153273084232631542621322426943187702825377218134772333297746833377632336780373327804135078168128782143517824035378411335784941157885013078865331789193348002836880046184806741198562919482612411323494113282388116280109119914122119992406120154407120245382120362412121246429121392123121397433121471408121974410122065125122079383122083405122402422122444435122919399123009446123816464123951447123956468124029374124527444124616136124630398124634376124943472124972375125011470125304297125371479125392299125515481125595484126123485126220300126234495126240478126547491126596499126913501127123389127731516127781395127796390127801209128119508128167517170PyrophosphateHMDB0000250The anion, the salts, and the esters of pyrophosphoric acid are called pyrophosphates. The pyrophosphate anion is abbreviated PPi and is formed by the hydrolysis of ATP into AMP in cells. This hydrolysis is called pyrophosphorolysis. The pyrophosphate anion has the structure P2O74-, and is an acid anhydride of phosphate. It is unstable in aqueous solution and rapidly hydrolyzes into inorganic phosphate. Pyrophosphate is an osteotoxin (arrests bone development) and an arthritogen (promotes arthritis). It is also a metabotoxin (an endogenously produced metabolite that causes adverse health affects at chronically high levels). Chronically high levels of pyrophosphate are associated with hypophosphatasia. Hypophosphatasia (also called deficiency of alkaline phosphatase or phosphoethanolaminuria) is a rare, and sometimes fatal, metabolic bone disease. Hypophosphatasia is associated with a molecular defect in the gene encoding tissue non-specific alkaline phosphatase (TNSALP). TNSALP is an enzyme that is tethered to the outer surface of osteoblasts and chondrocytes. TNSALP hydrolyzes several substances, including inorganic pyrophosphate (PPi) and pyridoxal 5'-phosphate (PLP), a major form of vitamin B6. When TSNALP is low, inorganic pyrophosphate (PPi) accumulates outside of cells and inhibits the formation of hydroxyapatite, one of the main components of bone, causing rickets in infants and children and osteomalacia (soft bones) in adults. Vitamin B6 must be dephosphorylated by TNSALP before it can cross the cell membrane. Vitamin B6 deficiency in the brain impairs synthesis of neurotransmitters which can cause seizures. In some cases, a build-up of calcium pyrophosphate dihydrate crystals in the joints can cause pseudogout.14000-31-8C0001364410218361PPI559142DB04160[O-]P([O-])(=O)OP([O-])([O-])=OO7P2InChI=1S/H4O7P2/c1-8(2,3)7-9(4,5)6/h(H2,1,2,3)(H2,4,5,6)/p-4XPPKVPWEQAFLFU-UHFFFAOYSA-J(phosphonooxy)phosphonic acid173.9433173.9119253784pyrophosphoric acid0-3FDB021918(4-)diphosphoric acid ion;(p2o74-)diphosphate;Diphosphate;Diphosphoric acid;Ppi;Pyrometaphosphate;Pyrophosphate;Pyrophosphate tetraanion;Pyrophosphate(4-) ion;[o3popo3](4-);Diphosphat;P2o7(4-);Pyrophosphat;Pyrophosphate ion;Phosphonato phosphoric acid;Pyrophosphoric acid;Pyrophosphoric acid ionPW_C000170Ppi12235463842923735328822212173162049241059281529417514486854503489525210452941015409117542410354331185458120554811155591325584133560613556551085879107623916669781997073188713416372721607312198731821382751518283210118691611200222212041164123152251232324912512288125792261269529015219306153751834760174256131542697318772353297731712877635336784163357892833179153112799501347995813080047372804171708563019478638494814125948193829867822311063439111327039511327538911552713611553239911993412212001712412003240612033041012093640712126142912134112112148638312240742212298544412350211912383146412404439812497737512532429712539529912541047912559748412565648512587648112655249112686920512693538812695050112733720612812450832Adenosine monophosphateHMDB0000045Adenosine monophosphate, also known as 5'-adenylic acid and abbreviated AMP, is a nucleotide that is found in RNA. It is an ester of phosphoric acid with the nucleoside adenosine. AMP consists of the phosphate group, the pentose sugar ribose, and the nucleobase adenine. AMP can be produced during ATP synthesis by the enzyme adenylate kinase. AMP has recently been approved as a 'Bitter Blocker' additive to foodstuffs. When AMP is added to bitter foods or foods with a bitter aftertaste it makes them seem 'sweeter'. This potentially makes lower calorie food products more palatable.61-19-8C00020608316027AMP5858DB00131NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1C10H14N5O7PInChI=1S/C10H14N5O7P/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(22-10)1-21-23(18,19)20/h2-4,6-7,10,16-17H,1H2,(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1UDMBCSSLTHHNCD-KQYNXXCUSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}phosphonic acid347.2212347.063084339-2.025adenylate0-2DBMET00485FDB0218065'-amp;5'-adenosine monophosphate;5'-adenylate;5'-adenylic acid;Amp;Adenosine 5'-monophosphate;Adenosine 5'-phosphate;Adenosine 5'-phosphorate;Adenosine 5'-phosphoric acid;Adenosine phosphate;Adenosine-5'-monophosphorate;Adenosine-5'-monophosphoric acid;Adenosine-5-monophosphorate;Adenosine-5-monophosphoric acid;Adenosine-monophosphate;Adenosine-phosphate;Adenovite;Adenylate;Adenylic acid;Cardiomone;Lycedan;Muscle adenylate;Muscle adenylic acid;My-b-den;My-beta-den;Phosaden;Phosphaden;Phosphentaside;5'-o-phosphonoadenosine;Adenosine 5'-(dihydrogen phosphate);Adenosine monophosphate;Adenosine-5'p;Adenosini phosphas;Ado5'p;Fosfato de adenosina;Pa;Pado;Phosphate d'adenosine;5'-adenosine monophosphoric acid;Adenosine phosphoric acid;Adenosine 5'-(dihydrogen phosphoric acid);Adenosine 5'-monophosphoric acid;Adenosine monophosphoric acid;Adenosine-5'-monophosphate;Phosphoric acid d'adenosinePW_C000032AMP112344628270167343288122118914457254867545033895251104540811754231035432118545712055581325583133577910157951086977199707218811789198118681611198815112003222125802261263631126942901333122542266342646315772343297732511178392334788091157932011280399180684135809007119916122120016124120031406120246382120888405121954408122920399123464376124507374125306297125394299125409479125596484126853205126934388126949501127124389127311209127711502146NADPHHMDB0000221Nicotinamide 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_C000143NADP1838191376857801082418839216112916174946853147961448011453081115790108601714761321596273356778117706918871051637152205720616073172137346210756221275891708197225822015184192241181119811897211120082221215216412249286125972261265024942344315437453227691329377164132773843317739633277461130775151157762433677814334778701128071311911316594120106407120429405120450122120604408120618123121142125121277429121401124121485383123063376123084135123229374123243447123713136123848464123960118124043398125473481125694297125743482126215299126528495127010206127225502127570388128100390976AllysineHMDB0001263Allysine is a derivative of Lysine, used in the production of elastin and collagen. It is produced by the actions of the enzyme lysyl oxidase in the extracellular matrix and is essential in the crosslink formation that stabilizes collagen and elastin.1962-83-0C0147520717027ALLYSINE202NC(CCCC=O)C(O)=OC6H11NO3InChI=1S/C6H11NO3/c7-5(6(9)10)3-1-2-4-8/h4-5H,1-3,7H2,(H,9,10)GFXYTQPNNXGICT-UHFFFAOYSA-N2-amino-6-oxohexanoic acid145.1564145.073893223-0.472allysine00FDB022519(2s)-2-amino-6-oxohexanoate;(2s)-2-amino-6-oxohexanoic acid;2-amino-5-formylvalerate;2-amino-5-formylvaleric acid;2-amino-hexanedioate;2-amino-hexanedioic acid;2-amino-hexanedioic acid semialdhyde;2-aminoadipate 6-semialdehyde;2-aminoadipate semialdehyde;2-aminoadipate-6-semialdehyde;5-formyl-norvaline;6-oxo-l-norleucine;6-oxo-norleucine;Allysine;L-2-aminoadipate 6-semialdehyde;L-6-oxonorleucine;L-allysine;Alpha-aminoadipic acid delta-semialdehyde;Alpha-aminoadipic delta-semialdehyde;Alpha-aminoadipic semialdehyde;6-oxonorleucine;Hco-[ch2]3-ch(nh2)-cooh;2-amino-6-oxohexanoate;2-aminoadipic acid 6-semialdehyde;A-aminoadipate delta-semialdehyde;A-aminoadipic acid delta-semialdehyde;Alpha-aminoadipate delta-semialdehyde;α-aminoadipate δ-semialdehyde;α-aminoadipic acid δ-semialdehyde;A-aminoadipic delta-semialdehyde;α-aminoadipic δ-semialdehyde;A-aminoadipate δ-semialdehyde;A-aminoadipic acid δ-semialdehyde;A-aminoadipic δ-semialdehydePW_C000976Alysine1068378277112120783407123375119189SaccharopineHMDB0000279Saccharopine is an intermediate in the degradation of lysine, formed by the condensation of lysine and alpha-ketoglutarate. The saccharopine pathway is the main route for lysine degradation in mammals, and its first two reactions are catalyzed by enzymatic activities known as lysine-oxoglutarate reductase (LOR) and saccharopine dehydrogenase (SDH), which reside on a single bifunctional polypeptide (LOR/SDH) (EC 1.5.1.8). The reactions involved with saccharopine dehydrogenases have very strict substrate specificity for L-lysine, 2-oxoglutarate, and NADPH. LOR/SDH has been detected in a number of mammalian tissues, mainly in the liver and kidney, contributing not only to the general nitrogen balance in the organism but also to the controlled conversion of lysine into ketone bodies. A tetrameric form has also been observed in human liver and placenta. LOR activity has also been detected in brain mitochondria during embryonic development, and this opens up the question of whether or not lysine degradation has any functional significance during brain development. As a result, there is now a new focus on the nutritional requirements for lysine in gestation and infancy. Finally, LOR and/or SDH deficiencies seem to be involved in a human autosomal genetic disorder known as familial hyperlysinemia, which is characterized by serious defects in the functioning of the nervous system and characterized by a deficiency in lysine-ketoglutarate reductase, saccharopine dehydrogenase, and saccharopine oxidoreductase activities. Saccharopinuria (high amounts of saccharopine in the urine) and saccharopinemia (an excess of saccharopine in the blood) are conditions present in some inherited disorders of lysine degradation (PMID: 463877, 10567240, 10772957, 4809305). If present in sufficiently high levels, saccharopine can act as an acidogen and a metabotoxin. 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. Saccharopine is an organic acid. 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). Many affected children with organic acidemias experience intellectual disability or delayed development.997-68-2C0044916055616927SACCHAROPINE141086DB04207N[C@@H](CCCCN[C@@H](CCC(O)=O)C(O)=O)C(O)=OC11H20N2O6InChI=1S/C11H20N2O6/c12-7(10(16)17)3-1-2-6-13-8(11(18)19)4-5-9(14)15/h7-8,13H,1-6,12H2,(H,14,15)(H,16,17)(H,18,19)/t7-,8-/m0/s1ZDGJAHTZVHVLOT-YUMQZZPRSA-N(2S)-2-{[(5S)-5-amino-5-carboxypentyl]amino}pentanedioic acid276.2863276.132136382-1.725saccharopine0-1FDB000461(s)-n-(5-amino-5-carboxypentyl)-l-glutamic acid;L-n-(5-amino-5-carboxypentyl)-glutamic acid;L-saccharopin;L-saccharopine;N(6)-(l-1,3-dicarboxypropyl)-l-lysine;N-(5-amino-5-carboxypentyl)-l-glutamic acid;N-(5-amino-5-carboxypentyl)-glutamic acid;N-[(5s)-5-amino-5-carboxypentyl]-l-glutamic acid;N6-(l-1,3-dicarboxypropyl)-l-lysine;Saccharopin;Epsilon-n-(l-glutar-2-yl)-l-lysine;N-[(s)-5-amino-5-carboxypentyl]-l-glutamic acid;SaccharopinePW_C000189Sacchar10663822115178276112120781407123373119118L-LysineHMDB0000182L-lysine is an essential amino acid. Normal requirements for lysine have been found to be about 8 g per day or 12 mg/kg in adults. Children and infants need more- 44 mg/kg per day for an eleven to-twelve-year old, and 97 mg/kg per day for three-to six-month old. Lysine is highly concentrated in muscle compared to most other amino acids. Lysine is high in foods such as wheat germ, cottage cheese and chicken. Of meat products, wild game and pork have the highest concentration of lysine. Fruits and vegetables contain little lysine, except avocados. Normal lysine metabolism is dependent upon many nutrients including niacin, vitamin B6, riboflavin, vitamin C, glutamic acid and iron. Excess arginine antagonizes lysine. Several inborn errors of lysine metabolism are known. Most are marked by mental retardation with occasional diverse symptoms such as absence of secondary sex characteristics, undescended testes, abnormal facial structure, anemia, obesity, enlarged liver and spleen, and eye muscle imbalance. Lysine also may be a useful adjunct in the treatment of osteoporosis. Although high protein diets result in loss of large amounts of calcium in urine, so does lysine deficiency. Lysine may be an adjunct therapy because it reduces calcium losses in urine. Lysine deficiency also may result in immunodeficiency. Requirements for this amino acid are probably increased by stress. Lysine toxicity has not occurred with oral doses in humans. Lysine dosages are presently too small and may fail to reach the concentrations necessary to prove potential therapeutic applications. Lysine metabolites, amino caproic acid and carnitine have already shown their therapeutic potential. Thirty grams daily of amino caproic acid has been used as an initial daily dose in treating blood clotting disorders, indicating that the proper doses of lysine, its precursor, have yet to be used in medicine. Low lysine levels have been found in patients with Parkinson's, hypothyroidism, kidney disease, asthma and depression. The exact significance of these levels is unclear, yet lysine therapy can normalize the level and has been associated with improvement of some patients with these conditions. Abnormally elevated hydroxylysines have been found in virtually all chronic degenerative diseases and coumadin therapy. The levels of this stress marker may be improved by high doses of vitamin C. Lysine is particularly useful in therapy for marasmus (wasting) and herpes simplex. It stops the growth of herpes simplex in culture, and has helped to reduce the number and occurrence of cold sores in clinical studies. Dosing has not been adequately studied, but beneficial clinical effects occur in doses ranging from 100 mg to 4 g a day. Higher doses may also be useful, and toxicity has not been reported in doses as high as 8 g per day. Diets high in lysine and low in arginine can be useful in the prevention and treatment of herpes. Some researchers think herpes simplex virus is involved in many other diseases related to cranial nerves such as migraines, Bell's palsy and Meniere's disease. Herpes blister fluid will produce fatal encephalitis in the rabbit. (http://www.dcnutrition.com).56-87-1C00047596218019LYS5747DB00123NCCCC[C@H](N)C(O)=OC6H14N2O2InChI=1S/C6H14N2O2/c7-4-2-1-3-5(8)6(9)10/h5H,1-4,7-8H2,(H,9,10)/t5-/m0/s1KDXKERNSBIXSRK-YFKPBYRVSA-N(2S)-2,6-diaminohexanoic acid146.1876146.105527702-0.143L-lysine01FDB000474(+)-s-lysine;(s)-2,6-diaminohexanoate;(s)-2,6-diaminohexanoic acid;(s)-2,6-diamino-hexanoate;(s)-2,6-diamino-hexanoic acid;(s)-lysine;(s)-a,e-diaminocaproate;(s)-a,e-diaminocaproic acid;2,6-diaminohexanoate;2,6-diaminohexanoic acid;6-amino-aminutrin;6-amino-l-norleucine;Aminutrin;L-(+)-lysine;L-2,6-diainohexanoate;L-2,6-diainohexanoic acid;L-2,6-diaminocaproate;L-2,6-diaminocaproic acid;L-lys;Lys;Lysine;Lysine acid;A-lysine;Alpha-lysine;H-lys-oh;(s)-alpha,epsilon-diaminocaproic acid;6-ammonio-l-norleucine;K;L-lysin;(s)-a,epsilon-diaminocaproate;(s)-a,epsilon-diaminocaproic acid;(s)-alpha,epsilon-diaminocaproate;(s)-α,epsilon-diaminocaproate;(s)-α,epsilon-diaminocaproic acidPW_C000118Lys58115632301065310918529910553031075304108555211482142254237031842371315777303387827511278287111120504409120536413120780407120807122123110137123170449123372119123392135125719483261holo-[LYS2 peptidyl-carrier-protein]ProteinComplexProteinComplexPW_EC000261HLPCP262L-2-aminoadipyl-[LYS2 peptidyl-carrier-protein]ProteinComplexProteinComplexPW_EC000262L2ALPCP10700Homocitrate synthase, mitochondrialQ12122LYS21292.3.3.14785316313610864610701Homocitrate synthase, cytosolic isozymeP48570LYS20292.3.3.14785716010702Homoaconitase, mitochondrialP49367
Catalyzes the reversible hydration of cis-homoaconitate to (2R,3S)-homoisocitrate, a step in the alpha-aminoadipate pathway for lysine biosynthesis.
LYS4294.2.1.367854160785516310105Homoisocitrate dehydrogenase, mitochondrialP40495
Catalyzes the NAD(+)-dependent conversion of homoisocitrate to alpha-ketoadipate.
LYS12291.1.1.876304161785616310703L-2-aminoadipate reductaseP07702
Catalyzes the activation of alpha-aminoadipate by ATP-dependent adenylation and the reduction of activated alpha-aminoadipate by NADPH. The activated alpha-aminoadipate is bound to the phosphopantheinyl group of the enzyme itself before it is reduced to (S)-2-amino-6-oxohexanoate.
LYS2291.2.1.95785816010704Saccharopine dehydrogenase [NADP(+), L-glutamate-forming]P38999LYS9291.5.1.10785916010705Saccharopine dehydrogenase [NAD(+), L-lysine-forming]P38998
Catalyzes the NAD(+)-dependent cleavage of saccharopine to L-lysine and 2-oxoglutarate.
LYS1291.5.1.778601604372Homocitrate synthase, mitochondrial18PW_P00437211451107004373Homocitrate synthase, cytosolic isozyme18PW_P00437311452107014374Homoaconitase, mitochondrial18PW_P00437411453107024375Homoisocitrate dehydrogenase, mitochondrial18PW_P00437511454101054376L-2-aminoadipate reductase18PW_P00437611455107034377Saccharopine dehydrogenase [NADP(+), L-glutamate-forming]18PW_P00437711456107044378Saccharopine dehydrogenase [NAD(+), L-lysine-forming]18PW_P00437811457107056469falsePW_R006469Right265371341Compoundfalse265389401Compoundfalse2653914201Compoundfalse2654010991Compoundfalse2654119111Compoundfalse26542400341Compoundfalse639543722.3.3.146470falsePW_R006470Right265431341Compoundfalse265449401Compoundfalse2654514201Compoundfalse2654619111Compoundfalse2654710991Compoundfalse26548400341Compoundfalse639643732.3.3.146471truePW_R006471Right2654919111Compoundfalse2655014201Compoundfalse26551445581Compoundfalse6472falsePW_R006472Right26552445581Compoundfalse2655314201Compoundfalse26554445591Compoundfalse639743744.2.1.366473falsePW_R006473Right26555445591Compoundfalse265567211Compoundfalse2655713161Compoundfalse2655811441Compoundfalse265591501Compoundfalse639843751.1.1.876474PW_R006474Right265601501Compoundfalse26561951Compoundfalse265621341Compoundfalse265633901Compoundfalse6475falsePW_R006475Right265643901Compoundfalse265654141Compoundfalse265662611ElementCollectionfalse265671701Compoundfalse26568321Compoundfalse265692621ElementCollectionfalse639943761.2.1.956476falsePW_R006476Right26570400341Compoundfalse265711461Compoundfalse265722621ElementCollectionfalse265731431Compoundfalse265742611ElementCollectionfalse265759761Compoundfalse640043766477falsePW_R006477Right265769761Compoundfalse265771461Compoundfalse26578951Compoundfalse26579400341Compoundfalse2658014201Compoundfalse265811431Compoundfalse265821891Compoundfalse640143771.5.1.106478falsePW_R006478Right265831891Compoundfalse265847211Compoundfalse2658514201Compoundfalse2658611441Compoundfalse26587400341Compoundfalse265881341Compoundfalse265891181Compoundfalse640243781.5.1.7785431343false40079110regular100110785449403false52070110regular10010078545142049false53689610regular787878546109985false86578110regular50307854719113false97079110regular100110785484003455false86689110regular7878785491343false500149010regular100110785509403false635140010regular10010078551142049false641159510regular78787855219113false1050149010regular10011078553109985false970148010regular5030785544003455false946160010regular787878555142049false119673510regular787878557142049false1241141910regular787878558445583false129578710regular10011078559142049false165172110regular787878560445593false174578410regular1001107856172159false184091410regular503078562131652false1679108010regular787878563114460false1842111410regular5030785641503false1747119410regular10010078565953false1642138910regular100110785661343false1647163910regular100110785673903false1747179410regular1001107856841442false1597178410regular50307856917045false1257189210regular6343785703244false1265177910regular5030785714003455false966173610regular78787857214662false995188410regular50307857314361false695176710regular5030785749763false390179710regular1001007857514662false495201410regular503078576953false295192910regular100110785774003455false486189910regular787878578142049false301218710regular78787857914361false490222510regular5030785801893false390228510regular1001007858172159false570226710regular503078582142049false526237710regular787878583114460false825238210regular5030785844003455false921237710regular7878785851343false1010228010regular100110785861183false820219010regular1001001561261144false1572189412regular150701562262144false1070181412regular150701563261144false620189212regular1507037641107001632false6608138subunitregular1507037642107011602false76015128subunitregular1507037643107021632false14708128subunitregular1507037644101051632false17229868subunitregular1507037645107031602false136918148subunitregular1507037646107031602false77718128subunitregular1507037647107041602false36520858subunitregular1507037648107051602false64522988subunitregular150703069043722177369783764130691437321773697937642306924374217736980376433069343752177369813764430694437621773698237645306954376217736983376463069643772177369843764730697437821773698537648113012M500 846 C530 846 630 848 660 848 5false18113013M570 801 C572 845 630 848 660 848 5false18113014M575 896 C575 847 630 848 660 848 5false18113015M890 811 C889 850 840 848 810 848 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false113016M970 846 C940 846 840 848 810 848 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false113017M905 891 C905 842 840 848 810 848 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false113018M600 1545 C630 1545 730 1547 760 1547 5false18113019M685 1500 C685 1549 730 1547 760 1547 5false18113020M680 1595 C680 1553 730 1547 760 1547 5false18113021M1050 1545 C1020 1545 940 1547 910 1547 5false18trueM 25.946855044164835 462.261556296296 L 11 461 L 17.380887721185843 474.5751343230783false113022M995 1510 C995 1552 940 1547 910 1547 5false18trueM 25.946855044164835 462.261556296296 L 11 461 L 17.380887721185843 474.5751343230783false113023M985 1600 C984 1544 940 1547 910 1547 5false18trueM 25.946855044164835 462.261556296296 L 11 461 L 17.380887721185843 474.5751343230783false113027M1150 1545 C1180 1545 1145 1545 1175 1545 5false18113028M1280 1497 C1279 1542 1180 1545 1150 1545 5false18trueM 1318.9468550441647 1035.261556296296 L 1304 1034 L 1310.380887721186 1047.5751343230784false113029M1345 897 C1345 942 1348 1465 1348 1544 C1216 1544 1282 1545 1150 1545 5false18trueM 1318.9468550441647 1035.261556296296 L 1304 1034 L 1310.380887721186 1047.5751343230784false113030M1399 847 C1429 847 1440 847 1470 847 5false18113031M1690 799 C1689 846 1650 847 1620 847 5false18trueM 1513.9468550441647 973.261556296296 L 1499 972 L 1505.380887721186 985.5751343230784false113032M1745 845 C1715 845 1650 847 1620 847 5false18trueM 1513.9468550441647 973.261556296296 L 1499 972 L 1505.380887721186 985.5751343230784false113033M1797 895 C1797 925 1797 956 1797 986 5false18113034M1840 929 C1800 930 1797 956 1797 986 5false18113035M1757 1119 C1792 1119 1797 1086 1797 1056 5false18trueM 1884.9468550441647 334.261556296296 L 1870 333 L 1876.380887721186 346.5751343230783false113036M1842 1129 C1796 1129 1797 1086 1797 1056 5false18trueM 1884.9468550441647 334.261556296296 L 1870 333 L 1876.380887721186 346.5751343230783false113037M1797 1194 C1797 1164 1797 1086 1797 1056 5false18trueM 1884.9468550441647 334.261556296296 L 1870 333 L 1876.380887721186 346.5751343230783false113038M1797 1294 C1797 1324 1797 1764 1797 1794 5false18113039M1742 1444 C1799 1444 1797 1508 1797 1538 5false18113040M1747 1694 C1793 1695 1797 1632 1797 1602 5false18trueM 2107.9468550441647 787.261556296296 L 2093 786 L 2099.380887721186 799.5751343230784false113041M1797 1794 C1797 1764 1797 1324 1797 1294 5false18trueM 2107.9468550441647 787.261556296296 L 2093 786 L 2099.380887721186 799.5751343230784false113042M1747 1849 C1714 1850 1560 1849 1519 1849 5false18113043M1622 1814 C1622 1837 1609 1848 1519 1849 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