113251Pathway2-Amino-3-Carboxymuconate Semialdehyde DegradationThis pathway is part of a major route of the degradation of L-tryptophan. It begins with 2-amino-3-carboxymuconate-6-semialdehyde which is generated from L-tryptophan degradation. The 2-amino-3-carboxymuconate-6-semialdehyde first is acted upon by a decarboxylase, forming 2-aminomuconic acid semialdehyde, which is then dehydrogenated by 2-aminomuconic semialdehyde dehydrogenase to form 2-aminomuconic acid. An unknown protein forms a 2-aminomuconate deaminase which forms (3E)-2-oxohex-3-enedioate, and a second unknown protein forms a 2-aminomuconate reductase, which forms oxoadipic acid from (3E)-2-oxohex-3-enedioate. Finally, within the mitochondria, oxoadipic acid is dehydrogenated and a coenzyme A is attached by the organelle’s oxoglutarate dehydrogenase complex, forming glutaryl-CoA. Glutaryl-CoA can then be further degraded.MetabolicPW122411CenterPathwayVisualizationContext12268711971800#000099PathwayVisualization1131141132512-Amino-3-Carboxymuconate Semialdehyde DegradationThis pathway is part of a major route of the degradation of L-tryptophan. It begins with 2-amino-3-carboxymuconate-6-semialdehyde which is generated from L-tryptophan degradation. The 2-amino-3-carboxymuconate-6-semialdehyde first is acted upon by a decarboxylase, forming 2-aminomuconic acid semialdehyde, which is then dehydrogenated by 2-aminomuconic semialdehyde dehydrogenase to form 2-aminomuconic acid. An unknown protein forms a 2-aminomuconate deaminase which forms (3E)-2-oxohex-3-enedioate, and a second unknown protein forms a 2-aminomuconate reductase, which forms oxoadipic acid from (3E)-2-oxohex-3-enedioate. Finally, within the mitochondria, oxoadipic acid is dehydrogenated and a coenzyme A is attached by the organelle’s oxoglutarate dehydrogenase complex, forming glutaryl-CoA. Glutaryl-CoA can then be further degraded.Metabolic1110142L-tryptophan degradationSubPathway1097451026Compound110143Glutaryl-CoA degradationSubPathway1097461032Compound27994616267312Colabroy KL, Begley TP: Tryptophan catabolism: identification and characterization of a new degradative pathway. J Bacteriol. 2005 Nov;187(22):7866-9. doi: 10.1128/JB.187.22.7866-7869.2005.113251Pathway27994723141293Danhauser K, Sauer SW, Haack TB, Wieland T, Staufner C, Graf E, Zschocke J, Strom TM, Traub T, Okun JG, Meitinger T, Hoffmann GF, Prokisch H, Kolker S: DHTKD1 mutations cause 2-aminoadipic and 2-oxoadipic aciduria. Am J Hum Genet. 2012 Dec 7;91(6):1082-7. doi: 10.1016/j.ajhg.2012.10.006. Epub 2012 Nov 8.113251Pathway27994812140278Fukuoka S, Ishiguro K, Yanagihara K, Tanabe A, Egashira Y, Sanada H, Shibata K: Identification and expression of a cDNA encoding human alpha-amino-beta-carboxymuconate-epsilon-semialdehyde decarboxylase (ACMSD). A key enzyme for the tryptophan-niacine pathway and "quinolinate hypothesis". J Biol Chem. 2002 Sep 20;277(38):35162-7. doi: 10.1074/jbc.M200819200. Epub 2002 Jul 24.113251Pathway27995114275130ICHIYAMA A, NAKAMURA S, KAWAI H, HONJO T, NISHIZUKA Y, HAYAISHI O, SENOH S: STUDIES ON THE METABOLISM OF THE BENZENE RING OF TRYPTOPHAN IN MAMMALIAN TISSUES. II. ENZYMIC FORMATION OF ALPHA-AMINOMUCONIC ACID FROM 3-HYDROXYANTHRANILIC ACID. J Biol Chem. 1965 Feb;240:740-9.113251Pathway27995219942660Liu X, Dong Y, Li X, Ren Y, Li Y, Wang W, Wang L, Feng L: Characterization of the anthranilate degradation pathway in Geobacillus thermodenitrificans NG80-2. Microbiology. 2010 Feb;156(Pt 2):589-95. doi: 10.1099/mic.0.031880-0. Epub 2009 Nov 26.113251Pathway27995312620844Muraki T, Taki M, Hasegawa Y, Iwaki H, Lau PC: Prokaryotic homologs of the eukaryotic 3-hydroxyanthranilate 3,4-dioxygenase and 2-amino-3-carboxymuconate-6-semialdehyde decarboxylase in the 2-nitrobenzoate degradation pathway of Pseudomonas fluorescens strain KU-7. Appl Environ Microbiol. 2003 Mar;69(3):1564-72.113251Pathway28009117288562Pucci L, Perozzi S, Cimadamore F, Orsomando G, Raffaelli N: Tissue expression and biochemical characterization of human 2-amino 3-carboxymuconate 6-semialdehyde decarboxylase, a key enzyme in tryptophan catabolism. FEBS J. 2007 Feb;274(3):827-40. doi: 10.1111/j.1742-4658.2007.05635.x.113251Pathway2800929721300He Z, Davis JK, Spain JC: Purification, characterization, and sequence analysis of 2-aminomuconic 6-semialdehyde dehydrogenase from Pseudomonas pseudoalcaligenes JS45. J Bacteriol. 1998 Sep;180(17):4591-5.113251Pathway2800939573204He Z, Spain JC: A novel 2-aminomuconate deaminase in the nitrobenzene degradation pathway of Pseudomonas pseudoalcaligenes JS45. J Bacteriol. 1998 May;180(9):2502-6.113251Pathway1CellCL:00000005HepatocyteCL:00001823NeuronCL:000054012AstrocyteCL:00001274CardiomyocyteCL:00007467Epithelial CellCL:00000666MyocyteCL:000018728MacrophageCL:00002352Platelet CL:00002331Homo sapiens9606EukaryoteHuman12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat6Caenorhabditis elegans6239EukaryoteRoundworm3Escherichia coli562Prokaryote2Bacteria2ProkaryoteBacteria24Solanum lycopersicum4081EukaryoteTomato18Saccharomyces cerevisiae4932EukaryoteYeast21Xenopus laevis8355EukaryoteAfrican clawed frog4Arabidopsis thaliana3702EukaryoteThale cress25Escherichia coli (strain K12)83333Prokaryote23Pseudomonas aeruginosa287Prokaryote60Nitzschia sp.0001EukaryoteNitzschia410Drosophila melanogaster7227EukaryoteFruit fly202Spathaspora passalidarum340170EukaryoteSpathaspora passalidarum49Bathymodiolus platifrons220390EukaryoteDeep sea mussel19Schizosaccharomyces pombe4896Eukaryote196Homo1924EukaryoteHuman157Acinetobacter baumannii 107673Prokaryote280Bacteroides fragilis55247009Prokaryote209Clostridium difficile1496Eukaryote135Felinus9685EukaryoteCat240Plasmodium falciparums121Eukaryote330Canis lupus familiaris9615EukaryoteDog62Acinetobacter baylyi (strain ATCC 33305 / BD413 / ADP1)62977Prokaryote1CytosolGO:00058293Mitochondrial MatrixGO:00057595CytoplasmGO:000573711Extracellular SpaceGO:00056152MitochondrionGO:00057397Endoplasmic Reticulum MembraneGO:000578912Mitochondrial Inner MembraneGO:000574314Mitochondrial Outer MembraneGO:000574124Mitochondrial Intermembrane SpaceGO:000575813Endoplasmic ReticulumGO:000578331Periplasmic SpaceGO:000562035ChloroplastGO:00095074PeroxisomeGO:000577710Cell MembraneGO:000588636MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032532Inner MembraneGO:007025825Golgi ApparatusGO:00057946LysosomeGO:000576416Lysosomal LumenGO:004320218Melanosome MembraneGO:003316220Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520215NucleusGO:000563440PeriplasmGO:004259727Peroxisome MembraneGO:00057788Smooth Endoplasmic Reticulum GO:000579039Mitochondrial membraneGO:003196637Basolateral cell membraneGO:001632349Nuclear EnvelopeGO:000563556Basal Cell MembraneGO:000992538Apical cell membraneGO:001632458Cell WallGO:000561830Lysosomal MembraneGO:00057651LiverBTO:000075972928StomachBTO:0001307155268Blood VesselBTO:0001102741124BrainBTO:000014289164Adrenal MedullaBTO:000004971825IntestineBTO:00006487Nervous SystemBTO:000148411HeartBTO:000056273106KidneyBTO:00006717189MuscleBTO:00008871411814EyeBTO:0000439153242111PW_BS0000021321121PW_BS000132124151PW_BS0001241181171PW_BS000118388161PW_BS0001124311PW_BS0000048511PW_BS00000815111PW_BS000015311511PW_BS0000313211PW_BS000003261115PW_BS000026541315PW_BS00005449711PW_BS000049171211PW_BS000017221411PW_BS000022422411PW_BS000042101711PW_BS000010181311PW_BS0000187028511PW_BS000070103331PW_BS0001031115121PW_BS0001111122121PW_BS00011210813PW_BS000108107313PW_BS000107100521PW_BS000100105113PW_BS000105117131PW_BS0001171471241PW_BS0001471553241PW_BS0001551572241PW_BS0001571613181PW_BS00016115924PW_BS00015916611PW_BS0001661783211PW_BS00017815284PW_BS000152101531PW_BS000101188118PW_BS0000241873118PW_BS0000241632181PW_BS000163205561PW_BS000024206261PW_BS000024219314PW_BS00002422014PW_BS000024222341PW_BS0000242137181PW_BS00002421013181PW_BS00002421217181PW_BS0000241601181PW_BS00016017018PW_BS0001702253541PW_BS000024151141PW_BS000151226441PW_BS00002416212181PW_BS000162224241PW_BS0000241951318PW_BS0000242491341PW_BS0000241644PW_BS0001642811251PW_BS0000242851041PW_BS0000242863641PW_BS0000242875341PW_BS0000242273441PW_BS0000242231241PW_BS0000242941141PW_BS0000243081011PW_BS000024315123PW_BS0000243183123PW_BS0000243221231PW_BS0000243125231PW_BS0000243201123PW_BS00002429341PW_BS0000241333121PW_BS00013313412121PW_BS0001343317121PW_BS0000281141112PW_BS00011432711125PW_BS00002834713125PW_BS00002834524121PW_BS00002813013121PW_BS0001303683601PW_BS000028310312PW_BS00002430412PW_BS0000241192171PW_BS000119383751PW_BS000100943PW_BS000094390761PW_BS0001123987171PW_BS0001133361121PW_BS000028109323PW_BS000109406351PW_BS000115407251PW_BS000115122551PW_BS000122409115PW_BS0001154241155PW_BS0001154251355PW_BS0001154182451PW_BS0001153841251PW_BS0001001251351PW_BS0001251203171PW_BS0001201355171PW_BS0001351371117PW_BS00013745911175PW_BS00011546013175PW_BS00011545424171PW_BS00011512112171PW_BS00012113613171PW_BS0001364793101PW_BS0001154812101PW_BS0001154831110PW_BS0001152975101PW_BS0000242991101PW_BS0000244957101PW_BS00011548924101PW_BS00011548012101PW_BS00011530013101PW_BS000024501361PW_BS000115208116PW_BS0000245062461PW_BS0001153911261PW_BS0001123951361PW_BS00011388231202PW_BS00055288312021PW_BS000552167311PW_BS000167168321PW_BS00016814101PW_BS000014788241113PW_BS00052459724112PW_BS000336185321PW_BS00002411PW_BS00000113121PW_BS000013204111PW_BS0000205411PW_BS000005432511PW_BS0000431901118PW_BS0000241985181PW_BS0000242771218PW_BS0000242905491PW_BS0000243331212PW_BS00002812915121PW_BS00012934141121PW_BS00002835625121PW_BS0000283344121PW_BS000028412125PW_BS0001154141551PW_BS0001154192551PW_BS000115408451PW_BS0001154461217PW_BS00011545015171PW_BS00011545525171PW_BS0001153744171PW_BS00005349025101PW_BS0001154824101PW_BS0001155072561PW_BS000115502461PW_BS0001159611PW_BS0000092811611PW_BS000028331811PW_BS0000332441011PW_BS00002460251PW_BS00006046114PW_BS00004629111PW_BS00002972513PW_BS000072612517PW_BS0000613612011PW_BS0000363772113PW_BS00003793252011PW_BS00009327151PW_BS000027711PW_BS000007971521PW_BS0000971136121PW_BS000113110231PW_BS0001101231751PW_BS000123126651PW_BS00012612711651PW_BS0001276131PW_BS000006140103PW_BS00014014315191PW_BS0001431465191PW_BS000146951721PW_BS0000951802211PW_BS000180207661PW_BS0000242111018PW_BS00002421425181PW_BS0000242156181PW_BS0000242164181PW_BS00002465111PW_BS0000652916491PW_BS0000242924491PW_BS00002429817101PW_BS0000243016101PW_BS000024302116101PW_BS000024253541PW_BS00002433217121PW_BS0000281151012PW_BS000115337116121PW_BS00002834318121PW_BS00002832914121PW_BS0000283522512PW_BS00002835325127PW_BS000028360410121PW_BS0000283702601PW_BS000028228361PW_BS000024232403PW_BS000024405105PW_BS0001154151851PW_BS000115429151PW_BS00011543441051PW_BS0001153821451PW_BS000100436255PW_BS0001154436171PW_BS0001153761017PW_BS00005344717171PW_BS000115448116171PW_BS00011545118171PW_BS0001154641171PW_BS000115469410171PW_BS00011539914171PW_BS0001134712517PW_BS00011547225177PW_BS0001154781010PW_BS00011548718101PW_BS00011548414101PW_BS000115209106PW_BS0000245041861PW_BS00011551541061PW_BS0001153891461PW_BS0001125131761PW_BS0001157906111PW_BS0005248346111PW_BS00054914117191PW_BS000141592711PW_BS000059111811PW_BS0000115811411PW_BS0000581021231PW_BS0001021041431PW_BS00010419914181PW_BS000024350114121PW_BS00002833527121PW_BS0000282881441PW_BS00002443311451PW_BS0001154222751PW_BS000115468114171PW_BS00011537527171PW_BS00005349127101PW_BS0001155082761PW_BS0001151861221PW_BS0000248911421PW_BS00055215612241PW_BS00015617912211PW_BS0001793583912PW_BS00002836912601PW_BS0000285311015PW_BS000053229371PW_BS0000245181PW_BS0000517684911PW_BS0005173093911PW_BS00002483265611PW_BS00054848113PW_BS000048184121PW_BS00002483163811PW_BS00054887724101961PW_BS0005529312011PW_BS000559958111211PW_BS000563959111411PW_BS000563828111011PW_BS000548372102PW_BS000028231511PW_BS00002399611315PW_BS0005699731715PW_BS000569787241013PW_BS00052481124111573PW_BS0005485291016PW_BS00005275251017PW_BS000075106532196PW_BS000578114311415PW_BS0005839611215PW_BS000564114814915PW_BS0005831168810128PW_BS000583118412801PW_BS000588810101574PW_BS000548231391PW_BS000024311322PW_BS00002412195821PW_BS000588903011PW_BS0000901452141011PW_BS00063514541411PW_BS0006358178512PW_BS0005481212102091PW_BS00058884711PW_BS0005497625117PW_BS00007678924113PW_BS000524509516PW_BS00005070231351PW_BS000512102032401PW_BS000577139533301PW_BS00063017283621PW_BS00070610262-Amino-3-carboxymuconic acid semialdehydeHMDB00013302-amino-3-carboxymuconic acid semialdehyde is an intermediate metabolite of the tryptophan-niacin catabolic pathway. Current interest in the degradation of tryptophan is mostly due to the role of quinolinate and other metabolites in several neuropathological conditions. Quinolinate is a neurotoxin formed nonenzymatically from 2-amino-3-carboxymuconic semialdehyde in mammalian tissues. 2-Amino-3-carboxymuconic semialdehyde is enzymatically converted to 2-aminomuconate via 2-aminomuconic semialdehyde. (PMID: 10510494, 16267312, 14275129).16597-58-3C0440952806739952-AMINO-3-CARBOXYMUCONATE_SEMIAL4444266N\C(C(O)=O)=C(\C=C/C=O)/C(O)=OC7H7NO5InChI=1S/C7H7NO5/c8-5(7(12)13)4(6(10)11)2-1-3-9/h1-3H,8H2,(H,10,11)(H,12,13)/b2-1-,5-4-KACPVQQHDVBVFC-OIFXTYEKSA-N185.1342185.032422339FDB0225592-amino-3-(3-oxoprop-1-en-1-yl)but-2-enedioate;2-amino-3-(3-oxoprop-1-en-1-yl)but-2-enedioic acid;2-amino-3-(3-oxoprop-1-enyl)-but-2-enedioate;2-amino-3-(3-oxoprop-1-enyl)-but-2-enedioic acid;2-amino-3-carboxymuconate semialdehyde;2-amino-3-carboxymuconate-6-semialdehyde;Acs;Amino-3-(3-oxoprop-1-en-1-yl)but-2-enedioate;Amino-3-(3-oxoprop-1-en-1-yl)but-2-enedioic acidPW_C0010262Am3CSa300127907913212176412412431511812764938840034Hydrogen 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-N1.00791.007825032H+;H(+);Hydrogen cation;Hydron;ProtonPW_C040034H+215467087531578831848311162146326146454223149278017425022425442454710457618469470524110353271115353112562610856391075699100572010557421175963147603715560701576093161613015962321666483178660115266921016843188691018771001637168205719120674532197454220747222275252137532210755821275721607590170819522582181518243226841316284202249139195915524911915164120152811218128512246286122662871252122713257223133252941533030842329315423543184240132242405312424543207691229377136133772101347737233177804114779551327799032777991347783793457992913080019368803873108038830480722119938231249482338311055038811285594113280390115537398115539118115856336116205109119973406120193407120549122120593409121170424121171425122569418122615384122687125122758120123183135123218137123742459123743460125141454125188121125273136125359479125550481125730483125736297125809299126517495126717489126766480126823300126902501127213208128308506128361391128430395140692882140693883140699167140707168140715141407427881407435971407601859912-Aminomuconic acid semialdehydeHMDB00012802-aminomuconic semialdehyde is an intermediate in the oxidative metabolism of tryptophan in mammals, and takes place via the kynurenine pathway, which is also used for NAD biosynthesis in all eukaryotic organisms. 2-Aminomuconic semialdehyde is reported to be unstable and spontaneously converted to picolinic acid (regarded as metabolically inert and is excreted in the urine as a glycine conjugate), and enzymatically converted to 2-aminomuconic acid through the action of 2-aminomuconic semialdehyde dehydrogenase. (PMID: 10510494, 16267312, 14275129).245128-91-0C038245280625157452-AMINOMUCONATE_SEMIALDEHYDE4444230N\C(=C\C=C/C=O)C(O)=OC6H7NO3InChI=1S/C6H7NO3/c7-5(6(9)10)3-1-2-4-8/h1-4H,7H2,(H,9,10)/b2-1-,5-3+QCGTZPZKJPTAEP-REDYYMJGSA-N141.1247141.042593095FDB0225322-aminomuconate 6-semialdehyde;2-aminomuconate semialdehyde;2-aminomuconic semialdehyde;2-aminomuconic acid 6-semialdehyde;(2e,4z)-2-amino-6-oxohexa-2,4-dienoate;2-aminomuconic acid semialdehydePW_C0009912-AaSa30022790801321217671241243181181276523881316Carbon 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-N44.009543.989829244DBMET00423FDB014084Carbon oxide;Carbon-12 dioxide;Carbonic acid anhydride;Carbonic acid gas;Carbonic anhydride;[co2];Co2;E 290;E-290;E290;R-744PW_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905021407981851420WaterHMDB0002111Water 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-N18.015318.010564686FDB013390Dihydrogen oxide;Steam;[oh2];Acqua;Agua;Aqua;Bound water;Dihydridooxygen;Eau;H2o;Hoh;Hydrogen hydroxide;WasserPW_C001420H2O5589491095139415131621448113526156242865210691207703382318838210943113774914655415904320182425322226786027274627781728052931437031647236346145983647273749419350302751567519597521410052279452361035297105531911153431135355112540211054701235483125549212655071275534130553711455411295591135560811856221085691657591405778101584114358531465877107589095591014759401516032155605915760871616123163613315962151621816664771786507180660015267131176840188688816071622057181207719320672112117228213723821472432157295198735021673882107401212746722274922247500190758817082012258237226841416292652611850277119221641201128112213285122502861226428712327249125202271263265126932901270529112715292130072981301930013025301130373021326122313327294153403084232731542695318436913227691429377019253771021327713113377215134773783317739733277471333775161157753633477628336777223377775934177816343779823477807132978235352782423537827035679113360800143688003937080591228806561199383038394794384110557390110639391115844398119879232119915122119963406120008407120046408120113124120365412120430405120438409120606415120794414121158425121240429121351121121381419121607434122118382122384436122753120122797374122804443123012446123064376123072137123131447123142136123162448123231451123384450123730460123810464123940455124165469124670399124938471124945472125305297125353479125386481125424482125480299125682483125707478125745487126054490126238495126273484126764480126896501126963502127017388127177208127199209127227504127506507127576515127836389128082395128176513140674790140675834140755185721NADHMDB0000902NAD (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]1OC21H27N7O14P2InChI=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)/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BAWFJGJZGIEFAR-NNYOXOHSSA-N663.4251663.109121631FDB0223093-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_C000721NAD140415033538651101114211344312735146654222949277917283529310794807184813184819284902649603151679552381035334111536011254691235482125559013556101185696100573810858271415912147594215160241556072157607616163851646917867721176890160701218870971637174205719720674051987459222824122683592259085224118192161232224913006298130183001325622342404322426193157710413277120133772091347737033177650336776673347770233277709130779151137798334778406356800063688069011993825124110552388112750166112853941199291221199524061201714071208344191209844081211594251212421261212594291218173831226143841227421201231304471231411361234194551235493741237314601238124431238294641243703981251871211253192971253424791255304811258062991258254901259244821265154951267654801268855011272785071273835021280893901283603911284283951407571859582-Aminomuconic acidHMDB00012412-Aminomuconic acid is a product of the Tryptophan metabolism degradation pathway (kinurenine pathway), in a reaction catabolized by the enzyme aminocarboxymuconate semialdehyde decarboxylase [EC:4.1.1.45]. The kynurenine pathway is the major route of L-tryptophan degradation in mammals. (BioCyc).4548-99-6C022205280499157452-AMINO-MUCONATE4444139N\C(=C\C=C/C(O)=O)C(O)=OC6H7NO4InChI=1S/C6H7NO4/c7-4(6(10)11)2-1-3-5(8)9/h1-3H,7H2,(H,8,9)(H,10,11)/b3-1-,4-2+ZRHONLCTYUYMIQ-HSFFGMMNSA-N157.1241157.037507717FDB0225072-aminohexa-2,4-dienedioate;2-aminohexa-2,4-dienedioic acid;2-aminomuconate;2-aminomuconic acid;O-aminomuconatePW_C0009582-Ama1144NADHHMDB0001487NADH 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-N665.441665.124771695FDB0226491,4-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_C001144NADH14341533490864810111521275514695422304927811728362931099480618481218482128490464959315169955240103533211153581125466123547912555931355698100573710858291415915147594515160271556079161638716472178677111768931607011188709916371722057195206746222282442268360225908622411809198118212161232024913003298130153001325522342403322426183157710713277123133772081347737133177651336776683347770033277707130779171137798634780009368806911199382212411054938811285494115838118119955406120172407120378122120986408121162425121244126121693429121818383122616384122745120123127447123138136123551374123734460123814443124242464124371398125189121125345479125531481125762297125808299125926482126516495126767480126888501127385502128090390128362391128429395140759185104699(3E)-2-oxohex-3-enedioate(3E)-2-Oxohex-3-enedioate has the chemical formula C6H4O5, and an average molecular weight of 156.094. (3E)-2-Oxohex-3-enedioate is involved in the 2-Amino-3-Carboxymuconate Semialdehyde Degradation Pathway.2524515664908[H]\C(CC([O-])=O)=C(\[H])C(=O)C([O-])=OC6H4O5InChI=1S/C6H6O5/c7-4(6(10)11)2-1-3-5(8)9/h1-2H,3H2,(H,8,9)(H,10,11)/p-2/b2-1+QTHJXLFFFTVYJC-OWOJBTEDSA-L156.094156.006970389PW_C1046993E2o3e22608AmmoniumHMDB0041827Ammonium 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-O18.038518.034374133Ammonium ion;Ammonia ion;Ammonium;Ammonium chloride;Ammonium(1+);Azanium;Nh4+;[nh4]+;[nh4](+);Nh4(+)PW_C022608Ammon57511085892955969100622616682731518367225119091701247024942627315116281109150Oxoadipic 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-N160.1247160.037173366FDB0033622-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_C000150Oxoadip107138228151782791121207874071233781191099Coenzyme 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-N767.534767.115208365FDB022614Acetoacetyl 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_C001099CoA21143868845387922892172407592414224595281329286231334211335118461810462958484214486554487965232102524710452801035477124573410857771016023155607516163841646817869301606961162697319970831887108163729319873472107458222822915190812269090224912417092151951301329915318249254884942616315769072937711913377222134772303297729211177550132775553347756311277633336776721297799611578047332780563507841333578567130792593337997433180005368806201188062737480635119806653769382838293834383986742881105553891105613901158423991158473981199514061201474051202313841203051221206344071207621171214061231214214331215211251216664291216824081217144141224044221227411201229041211229601351239654471239794681240791361242204641242654501249743751253414791255094781255794801255924841256342971260844811265494911265604821267463001268845011270462091271093911273012051275402061276673881281215081281335021283403951407511861407631851407678911032Glutaryl-CoAHMDB0001339Glutaryl-CoA is a substrate for 2-oxoglutarate dehydrogenase E1 component (mitochondrial), Dihydrolipoyllysine-residue succinyltransferase component of 2- oxoglutarate dehydrogenase complex (mitochondrial) and Glutaryl-CoA dehydrogenase (mitochondrial).103192-48-9C0052743925215524GLUTARYL-COA388388CC(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(O)C(=O)NCCC(=O)NCCSC(=O)CCCC(O)=OC26H42N7O19P3SInChI=1S/C26H42N7O19P3S/c1-26(2,21(39)24(40)29-7-6-15(34)28-8-9-56-17(37)5-3-4-16(35)36)11-49-55(46,47)52-54(44,45)48-10-14-20(51-53(41,42)43)19(38)25(50-14)33-13-32-18-22(27)30-12-31-23(18)33/h12-14,19-21,25,38-39H,3-11H2,1-2H3,(H,28,34)(H,29,40)(H,35,36)(H,44,45)(H,46,47)(H2,27,30,31)(H2,41,42,43)/t14-,19-,20-,21?,25-/m1/s1SYKWLIJQEHRDNH-KRPIADGTSA-N881.633881.146902423FDB022563Glutaryl-coa;Glutaryl-coenzyme aPW_C001032GlutCoA92941077352821037004161823015177259133782841121202854061207984071229501201233871191256244791271505011407971851060Thiamine pyrophosphateHMDB0001372Thiamine pyrophosphate is the active form of thiamine, and it serves as a cofactor for several enzymes involved primarily in carbohydrate catabolism. The enzymes are important in the biosynthesis of a number of cell constituents, including neurotransmitters, and for the production of reducing equivalents used in oxidant stress defenses and in biosyntheses and for synthesis of pentoses used as nucleic acid precursors. The chemical structure of TPP is that of an aromatic methylaminopyrimidine ring, linked via a methylene bridge to a methylthiazolium ring with a pyrophosphate group attached to a hydroxyethyl side chain. In non-enzymatic model studies it has been demonstrated that the thiazolium ring can catalyse reactions which are similar to those of TPP-dependent enzymes but several orders of magnitude slower. Using infrared and NMR spectrophotometry it has been shown that the dissociation of the proton from C2 of the thiazolium ring is necessary for catalysis; the abstraction of the proton leads to the formation of a carbanion (ylid) with the potential for a nucleophilic attack on the carbonyl group of the substrate. In all TPP-dependent enzymes the abstraction of the proton from the C2 atom is the first step in catalysis, which is followed by a nucleophilic attack of this carbanion on the substrate. Subsequent cleavage of a C-C bond releases the first product with formation of a second carbanion (2-greek small letter alpha-carbanion or enamine). The formation of this 2-greek small letter alpha-carbanion is the second feature of TPP catalysis common to all TPP-dependent enzymes. Depending on the enzyme and the substrate(s), the reaction intermediates and products differ. Methyl-branched fatty acids, as phytanic acid, undergo peroxisomal beta-oxidation in which they are shortened by 1 carbon atom. This process includes four steps: activation, 2-hydroxylation, thiamine pyrophosphate dependent cleavage and aldehyde dehydrogenation. In the third step, 2-hydroxy-3-methylacyl-CoA is cleaved in the peroxisomal matrix by 2-hydroxyphytanoyl-CoA lyase (2-HPCL), which uses thiamine pyrophosphate (TPP) as cofactor. The thiamine pyrophosphate dependence of the third step is unique in peroxisomal mammalian enzymology. Human pathology due to a deficient alpha-oxidation is mostly linked to mutations in the gene coding for the second enzyme of the sequence, phytanoyl-CoA hydroxylase (EC 1.14.11.18). (PMID: 12694175, 11899071, 9924800).154-87-0C00068113295322-(alpha-lactyl)-thpp1100CC1=C(CCOP(O)(=O)OP(O)(O)=O)SC=[N+]1CC1=CN=C(C)N=C1NC12H19N4O7P2SInChI=1S/C12H18N4O7P2S/c1-8-11(3-4-22-25(20,21)23-24(17,18)19)26-7-16(8)6-10-5-14-9(2)15-12(10)13/h5,7H,3-4,6H2,1-2H3,(H4-,13,14,15,17,18,19,20,21)/p+1AYEKOFBPNLCAJY-UHFFFAOYSA-O425.314425.044967696FDB022584Tpp;Thpp;Thaimine pyrophosphate;Thiamin diphosphate;Thiamin pyrophosphate;Thiamin-ppi;Thiamine diphosphate;Thiamine pyrophosphate;Thiamine-ppi;Thiamine-pyrophosphate;Thiamin diphosphoric acid;Thiamine(1+) diphosphoric acid;Thiamin pyrophosphoric acid;Thiamine diphosphoric acidPW_C001060ThiamPP205410753119781271517362536610360281556080161638816473178746322212806225771241337828511278423334790181117917513280010368119956406120802407120902122120982408121537124122746120123388119123473135123547374124095118125346479125922482126094481126802299126889501127381502127549206128400388769LipoamideHMDB0000962Lipoamide is the oxidized form of glutathione. (PMID:8957191). Lipoamide is a trivial name for 6,8-dithiooctanoic amide. It is 6,8-dithiooctanoic acid's functional form where the carboxyl group is attached to protein (or any other amine) by an amide linkage (containing -NH2) to an amino group. Lipoamide forms a thioester bond, oxidizing the disulfide bond, with acetaldehyde (pyruvate after it has been decarboxylated). It then transfers the acetaldehyde group to CoA which can then continue in the TCA cycle. (Wikipedia). Lipoamide is an intermediate in glycolysis/gluconeogenesis, citrate cycle (TCA cycle), alanine, aspartate and pyruvate metabolism, and valine, leucine and isoleucine degradation (KEGG:C00248). It is generated from dihydrolipoamide via the enzyme dihydrolipoamide dehydrogenase (EC:1.8.1.4) and then converted to S-glutaryl-dihydrolipoamide via the enzyme oxoglutarate dehydrogenase (EC:1.2.4.2).940-69-2C0024886317460LIPOAMIDE840NC(=O)CCCCC1CCSS1C8H15NOS2InChI=1S/C8H15NOS2/c9-8(10)4-2-1-3-7-5-6-11-12-7/h7H,1-6H2,(H2,9,10)FCCDDURTIIUXBY-UHFFFAOYSA-N205.341205.059505487FDB0223401,2-dithiolane-3-pentanamide;5-(1,2-dithiolan-3-yl)-pentanamide;5-(1,2-dithiolan-3-yl)pentanamide;5-(1,2-dithiolan-3-yl)valeramide;5-(dithiolan-3-yl)valeramide;Dl-lipoamide;Dl-6-thioctic amide;Lipamide;Lipoacin;Lipoamid;Lipoicin;Lipozyme;Lypoaran;Pathoclon;Thioami;Thioctamid;Thioctamide;Thioctic acid amide;Thioctic acid amide (jan);Thiotomin;Ticolin;Vitamin n;Alpha-lipoate;Alpha-lipoic acid;Alpha-lipoic acid amide;A-lipoate amide;A-lipoic acid amide;Alpha-lipoate amide;α-lipoate amide;α-lipoic acid amide;Thioctate amidePW_C000769Lipoamd2024107331734246678536710360291556081161638916474178746422277125133782861127917313280011368119957406120803407121535124122747120123389119124093118125347479126078481126890501127534206964FADHMDB0001248FAD, also known as flavitan or adeflavin, belongs to the class of organic compounds known as flavin nucleotides. These are nucleotides containing a flavin moiety. Flavin is a compound that contains the tricyclic isoalloxazine ring system, which bears 2 oxo groups at the 2- and 4-positions. FAD is a drug which is used to treat eye diseases caused by vitamin b2 deficiency, such as keratitis and blepharitis. FAD is slightly soluble (in water) and a moderately acidic compound (based on its pKa). FAD has been found in human liver and muscle tissues, and has also been detected in multiple biofluids, such as feces and blood. Within the cell, FAD is primarily located in the cytoplasm, mitochondria, endoplasmic reticulum and peroxisome. FAD exists in all living organisms, ranging from bacteria to humans. In humans, FAD is involved in the risedronate action pathway, the ibandronate action pathway, the valine, leucine and isoleucine degradation pathway, and the pyrimidine metabolism pathway. FAD is also involved in several metabolic disorders, some of which include the oncogenic action OF L-2-hydroxyglutarate in hydroxygluaricaciduria pathway, gaba-transaminase deficiency, 4-hydroxybutyric aciduria/succinic semialdehyde dehydrogenase deficiency, and the saccharopinuria/hyperlysinemia II pathway. FAD is a condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972).146-14-5C0001664397516238FAD559059DB03147CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=NC(=O)NC(=O)C1=N2C27H33N9O15P2InChI=1S/C27H33N9O15P2/c1-10-3-12-13(4-11(10)2)35(24-18(32-12)25(42)34-27(43)33-24)5-14(37)19(39)15(38)6-48-52(44,45)51-53(46,47)49-7-16-20(40)21(41)26(50-16)36-9-31-17-22(28)29-8-30-23(17)36/h3-4,8-9,14-16,19-21,26,37-41H,5-7H2,1-2H3,(H,44,45)(H,46,47)(H2,28,29,30)(H,34,42,43)/t14-,15+,16+,19-,20+,21+,26+/m0/s1VWWQXMAJTJZDQX-UYBVJOGSSA-N785.5497785.157134455FDB0225111h-purin-6-amine flavin dinucleotide;1h-purin-6-amine flavine dinucleotide;Adenine-flavin dinucleotide;Adenine-flavine dinucleotide;Adenine-riboflavin dinuceotide;Adenine-riboflavin dinucleotide;Adenine-riboflavine dinucleotide;Fad;Flamitajin b;Flanin f;Flavin adenine dinucleotide;Flavin adenine dinucleotide oxidized;Flavin-adenine dinucleotide;Flavine adenosine diphosphate;Flavine-adenine dinucleotide;Flavitan;Flaziren;Isoalloxazine-adenine dinucleotide;Riboflavin 5'-adenosine diphosphate;Riboflavin-adenine dinucleotide;Riboflavine-adenine dinucleotide;AdeflavinPW_C000964FAD99911451868192321642531762828825188402118814148942161229162249213358253622372326460236468831474113475810488165268103528510253351115496126551112756131186030155605415660821616116162639016475178649917966661077039163717520573212137465222748722390762241181821611887215118992111229622512328249124431511251922712595226127102911272029213029301130413024362331877080293771261337715213477501113775071127751811577541334776151327772633778054329783753457893033179222336792723588001236880034369807141191199584061199993841200514081201074071204324051204531221204901241212784291212984181214173821214893831227481201227761211228023741228234431230663761230871351231664481238494641238684541239763991240473981253484791253784801254294821254744811256972971259794891261072991262774841268915011269203911269685021269872071270112061273102091274325061276023881278403891407901851407991862552-amino-3-carboxymuconate-6-semialdehyde decarboxylaseQ8TDX5Converts alpha-amino-beta-carboxymuconate-epsilon-semialdehyde (ACMS) to alpha-aminomuconate semialdehyde (AMS). ACMS can be converted non-enzymatically to quinolate (QA), a key precursor of NAD, and a potent endogenous excitotoxin of neuronal cells which is implicated in the pathogenesis of various neurodegenerative disorders. In the presence of ACMSD, ACMS is converted to AMS, a benign catabolite. ACMSD ultimately controls the metabolic fate of tryptophan catabolism along the kynurenine pathway.
HMDBP00261ACMSD2q21.3BC01601814.1.1.4530032226822-aminomuconic semialdehyde dehydrogenaseQ9H2A2
Catalyzes the NAD-dependent oxidation of 2-aminomuconic semialdehyde of the kynurenine metabolic pathway in L-tryptophan degradation.
ALDH8A111.2.1.32136176210207UnknownUnknown12.3.1.85; 2.3.1.38; 2.3.1.39; 2.3.1.41; 1.1.1.100; 4.2.1.59; 1.3.1.39; 3.1.2.14; 3.5.99.5; 1.1.1.-64041064293164345391661791704918222116783116852121248121291412133229135973511361787140020308140156768140576309140589832140864601409334814095418414126183114127587714143293114158495814158595914158682814215526142194151422653721423322314267129142673996142683973142813787143272811143273521433557514339810651434741143143476131434789611435151148143934116814395111841439598101440942311441223111442314214424612191442849014562414521456271454145678817145749121214577684714583476145952789146059504308Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrialQ96HY7
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity).
HMDBP09099DHTKD110p14AC07316011.2.4.2515131422304882Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrialP36957
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of 3 enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).
HMDBP00939DLST14q24.3AC00653012.3.1.612354107934669813697170214253110201426021201443141031454151395149940172852Dihydrolipoyl dehydrogenase, mitochondrialP09622Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
HMDBP00054DLD7q31-q32L1375711.8.1.42174108034670863941136965702142126541425251020142596120144315103145409139514584411914993417287452-amino-3-carboxymuconate-6-semialdehyde decarboxylase1PW_P0007458402551130352-Aminomuconate-semialdehyde dehydrogenase1PW_P01303522842226821361772130362-aminomuconate deaminase1PW_P0130362284310207130372-aminomuconate reductase1PW_P013037228441020760Oxoglutarate dehydrogenase complex1PW_P00006065430816688216752231106013276913396422324179637falsePW_R179637Right67796010261Compoundfalse677961400341Compoundfalse6779629911Compoundfalse67796313161Compoundfalse169519745179638falsePW_R179638Right6779649911Compoundfalse67796514201Compoundfalse6779667211Compoundfalse6779679581Compoundfalse677968400341Compoundfalse67796911441Compoundfalse169520130351.2.1.32179639falsePW_R179639Right6779709581Compoundfalse67797114201Compoundfalse6779721046991Compoundfalse677973226081Compoundfalse16952113036 3.5.99.5179640falsePW_R179640Right6779741046991Compoundfalse677975400341Compoundfalse67797611441Compoundfalse6779771501Compoundfalse6779787211Compoundfalse16952213037 1.1.1.-179641falsePW_R179641Right6779791501Compoundfalse67798010991Compoundfalse6779817211Compoundfalse67798210321Compoundfalse67798311441Compoundfalse67798413161Compoundfalse169523601.2.4.22682067102623false39929410regular100130268206840034255false50020410regular7878268206999123false87930110regular10012026820701316252false81020610regular787826820711420249false99020810regular78782682072721259false101945610regular5030268207395823false139930710regular100110268207440034255false130021210regular787826820751144260false130445010regular503026820761420749false153637310regular7878268207710469973false139964210regular10012026820782260873false152460710regular100100268207940034755false130056910regular787826820801144760false129978210regular5030268208115073false87965010regular1001002682082721759false101978010regular503026820831099385false83459010regular50302682084721359false83480010regular50302682085103233false39964810regular10010026820861144360false52979310regular503026820871316352false49556310regular78782682088106039false65951510regular10035268208976939false65949510regular10025268209096439false65947510regular1002594957625522false6193268subunitregular150709495772268222false11093268subunitregular150709495781020772false13744898subunitregular150709495791020772false11096678subunitregular15070949580430832false6345408subunitregular1507094958188232false6346008subunitregular150709495825236false6296608subunitregular1608080407374511311429454079495768040741303511311429454089495778040751303611311479454099495788040761303711311479454109495798040776011311439454119495809454129495819454139495823647326820883665598Cofactor3647426820893665599Cofactor3647526820903665600Cofactor3665573M499.3333282470703 359 C529.3333282470703 359 589.3333282470703 361 619.3333282470703 361 5false183665574M578.3333282470703 243 C577.3333282470703 266 589.3333282470703 361 619.3333282470703 361 5false183665575M879.3333282470703 361 C849.3333282470703 361 799.3333282470703 361 769.3333282470703 361 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3665576M810.3333282470703 245 C809.3333282470703 281 799.3333282470703 361 769.3333282470703 361 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3665577M979.3333282470703 361 C1009.3333282470703 361 1079.3333282470703 361 1109.3333282470703 361 5false183665578M1068.3333282470703 247 C1070.3333282470703 277 1079.3333282470703 361 1109.3333282470703 361 5false183665579M1069.3333282470703 471 C1071.3333282470703 440 1079.3333282470703 361 1109.3333282470703 361 5false183665580M1399.3333282470703 362 C1369.3333282470703 362 1289.3333282470703 361 1259.3333282470703 361 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3665581M1300.3333282470703 251 C1299.3333282470703 267 1289.3333282470703 361 1259.3333282470703 361 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3665582M1304.3333282470703 465 C1303.3333282470703 443 1289.3333282470703 361 1259.3333282470703 361 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3665583M1449.3333282470703 417 C1449.3333282470703 447 1449.3333282470703 459 1449.3333282470703 489 5false183665584M1575.3333282470703 451 C1553.3333282470703 448 1449.3333282470703 459 1449.3333282470703 489 5false183665585M1449.3333282470703 642 C1449.3333282470703 612 1449.3333282470703 589 1449.3333282470703 559 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3665586M1574.3333282470703 607 C1554.3333282470703 605 1449.3333282470703 589 1449.3333282470703 559 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3665587M1399.3333282470703 702 C1369.3333282470703 702 1289.3333282470703 702 1259.3333282470703 702 5false183665588M1300.3333282470703 608 C1304.3333282470703 634 1289.3333282470703 702 1259.3333282470703 702 5false183665589M1299.3333282470703 797 C1298.3333282470703 778 1289.3333282470703 702 1259.3333282470703 702 5false183665590M979.3333282470703 700 C1009.3333282470703 700 1079.3333282470703 702 1109.3333282470703 702 5false18trueM 394.65059039572736 13.26155629629604 L 379.7037353515625 12 L 386.08462307274834 25.575134323078345false3665591M1069.3333282470703 795 C1069.3333282470703 781 1079.3333282470703 702 1109.3333282470703 702 5false18trueM 394.65059039572736 13.26155629629604 L 379.7037353515625 12 L 386.08462307274834 25.575134323078345false3665592M879.3333282470703 700 C849.3333282470703 700 819.3333282470703 700 789.3333282470703 700 5false183665593M834.3333282470703 605 C835.3333282470703 622 819.3333282470703 700 789.3333282470703 700 5false183665594M834.3333282470703 815 C834.3333282470703 782 819.3333282470703 700 789.3333282470703 700 5false183665595M499.3333282470703 698 C529.3333282470703 698 599.3333282470703 700 629.3333282470703 700 5false18trueM 394.65059039572736 13.26155629629604 L 379.7037353515625 12 L 386.08462307274834 25.575134323078345false3665596M579.3333282470703 808 C582.3333282470703 788 599.3333282470703 700 629.3333282470703 700 5false18trueM 394.65059039572736 13.26155629629604 L 379.7037353515625 12 L 386.08462307274834 25.575134323078345false3665597M573.3333282470703 602 C572.3333282470703 629 599.3333282470703 700 629.3333282470703 700 5false18trueM 394.65059039572736 13.26155629629604 L 379.7037353515625 12 L 386.08462307274834 25.575134323078345false3665598M464.3333282470703 520 L464.3333282470703 570 L514.3333282470703 520 z10true183665599M464.3333282470703 500 L464.3333282470703 550 L514.3333282470703 500 z10true183665600M464.3333282470703 480 L464.3333282470703 530 L514.3333282470703 480 z10true183668911M399 359 C369 359 380 357 350 357 5false18trueM 301.0096189432334 351.5 L 314 344 L 301.0096189432334 336.5false3668912M399 698 C369 698 374 696 344 696 5false18trueM 236.99038105676658 673.5 L 224 681 L 236.99038105676658 688.5false7984531131141796372322703126820673665573Left322703226820683665574Left322703326820693665575Right322703426820703665576Right7540321695198040737984541131141796382322703526820693665577Left322703626820713665578Left322703726820723665579Left322703826820733665580Right322703926820743665581Right322704026820753665582Right7540331695208040747984551131141796397322704126820733665583Left322704226820763665584Left322704326820773665585Right322704426820783665586Right7540341695218040757984561131141796407322704526820773665587Left322704626820793665588Left322704726820803665589Left322704826820813665590Right322704926820823665591Right7540351695228040767984571131141796413322705026820813665592Left322705126820833665593Left322705226820843665594Left322705326820853665595Right322705426820863665596Right322705526820873665597Right754036169523804077109839110142113114217false20032216regular10652126820673668911Right109840110143113114217false19466116regular10652226820853668912Left1867305688690.60.602149024018673110588040.80.80214327267369726M124 222 C124 172 174 122 224 122 C642 122 1185 122 1603 122 C1653 122 1703 172 1703 222 C1703 454 1703 755 1703 987 C1703 1037 1653 1087 1603 1087 C1185 1087 642 1087 224 1087 C174 1087 124 1037 124 987 C124 755 124 454 124 222 1true61579.0965.045068315Mitochondria388434201.31.31601545068415Cytosol1353954201.61.61601518050373590638846399984941#FFEBEB4611386