22PathwayButyrate MetabolismButyrate metabolism (Butanoate metabolism) describes the metabolic fate of a number of short chain fatty acids or short chain alcohols that are typically produced by intestinal fermentation. Many of these molecules are eventually used in the production of ketone bodies, the creation of short-chain lipids or as precursors to the citrate cycle, glycolysis or glutamate synthesis. The molecule for which this pathway is named, butyric acid, is a four-carbon fatty acid that is formed in the human colon by bacterial fermentation of carbohydrates (including dietary fiber). It is found in rancid butter, parmesan cheese, and vomit, and has an unpleasant odor and acrid taste, with a sweet aftertaste (similar to ether).MetabolicPW000014CenterPathwayVisualizationContext1431003400#000099PathwayVisualization2022Butyrate MetabolismButyrate metabolism (Butanoate metabolism) describes the metabolic fate of a number of short chain fatty acids or short chain alcohols that are typically produced by intestinal fermentation. Many of these molecules are eventually used in the production of ketone bodies, the creation of short-chain lipids or as precursors to the citrate cycle, glycolysis or glutamate synthesis. The molecule for which this pathway is named, butyric acid, is a four-carbon fatty acid that is formed in the human colon by bacterial fermentation of carbohydrates (including dietary fiber). It is found in rancid butter, parmesan cheese, and vomit, and has an unpleasant odor and acrid taste, with a sweet aftertaste (similar to ether).Metabolic117Intestinal MicrofloraSubPathway3128Compound887055SubPathway139442Compound4158Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.22Pathway159Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.22Pathway28039427346602Stilling RM, van de Wouw M, Clarke G, Stanton C, Dinan TG, Cryan JF: The neuropharmacology of butyrate: The bread and butter of the microbiota-gut-brain axis? Neurochem Int. 2016 Oct;99:110-132. doi: 10.1016/j.neuint.2016.06.011. Epub 2016 Jun 23.22Pathway28039519222573Louis P, Flint HJ: Diversity, metabolism and microbial ecology of butyrate-producing bacteria from the human large intestine. FEMS Microbiol Lett. 2009 May;294(1):1-8. doi: 10.1111/j.1574-6968.2009.01514.x. Epub 2009 Feb 13.22Pathway28039612740060Macfarlane S, Macfarlane GT: Regulation of short-chain fatty acid production. Proc Nutr Soc. 2003 Feb;62(1):67-72. doi: 10.1079/PNS2002207.22Pathway2803979406136Cummings JH, Macfarlane GT: Role of intestinal bacteria in nutrient metabolism. JPEN J Parenter Enteral Nutr. 1997 Nov-Dec;21(6):357-65. doi: 10.1177/0148607197021006357.22Pathway28039824757212Vital M, Howe AC, Tiedje JM: Revealing the bacterial butyrate synthesis pathways by analyzing (meta)genomic data. MBio. 2014 Apr 22;5(2):e00889. doi: 10.1128/mBio.00889-14.22Pathway1CellCL:00000005HepatocyteCL:00001824CardiomyocyteCL:00007463NeuronCL:00005407Epithelial CellCL:00000662Platelet CL:00002338Beta cellCL:000063911Colorectal Cancer CellCL:00010641Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote18Saccharomyces cerevisiae4932EukaryoteYeast12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat10Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm2Bacteria2ProkaryoteBacteria4Arabidopsis thaliana3702EukaryoteThale cress19Schizosaccharomyces pombe4896Eukaryote24Solanum lycopersicum4081EukaryoteTomato21Xenopus laevis8355EukaryoteAfrican clawed frog25Escherichia coli (strain K12)83333Prokaryote49Bathymodiolus platifrons220390EukaryoteDeep sea mussel23Pseudomonas aeruginosa287Prokaryote60Nitzschia sp.0001EukaryoteNitzschia451Picea sitchensis3332EukaryoteSitka spruce55Yarrowia lipolytica4952Eukaryoteyli3Mitochondrial MatrixGO:00057592MitochondrionGO:00057391CytosolGO:00058294PeroxisomeGO:00057775CytoplasmGO:00057376LysosomeGO:000576410Cell MembraneGO:000588611Extracellular SpaceGO:000561516Lysosomal LumenGO:00432027Endoplasmic Reticulum MembraneGO:000578918Melanosome MembraneGO:003316213Endoplasmic ReticulumGO:000578325Golgi ApparatusGO:000579414Mitochondrial Outer MembraneGO:000574112Mitochondrial Inner MembraneGO:000574320Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520215NucleusGO:000563431Periplasmic SpaceGO:000562035ChloroplastGO:000950736MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032540PeriplasmGO:00425978Smooth Endoplasmic Reticulum GO:000579027Peroxisome MembraneGO:000577832Inner MembraneGO:007025826Golgi Apparatus MembraneGO:000013924Mitochondrial Intermembrane SpaceGO:000575839Mitochondrial membraneGO:00319661LiverBTO:00007597294Adrenal MedullaBTO:000004971825IntestineBTO:000064828StomachBTO:0001307155267Nervous SystemBTO:00014848Blood VesselBTO:0001102741111HeartBTO:000056273106KidneyBTO:00006717182Endothelium BTO:000039318PancreasBTO:00009885cardiocyteBTO:000153915BoneBTO:000014022BladderBTO:00001234311PW_BS0000043211PW_BS000003103331PW_BS0001031613181PW_BS0001611632181PW_BS0001631333121PW_BS0001331122121PW_BS000112406351PW_BS000115407251PW_BS0001151203171PW_BS0001201192171PW_BS0001194793101PW_BS000115501361PW_BS000115185321PW_BS000024943PW_BS000094151141PW_BS000151226441PW_BS0000248511PW_BS0000089611PW_BS0000095411PW_BS00000514101PW_BS00001413121PW_BS00001315111PW_BS0000152811611PW_BS000028101711PW_BS000010204111PW_BS000020331811PW_BS000033181311PW_BS0000182111PW_BS000002311511PW_BS00003149711PW_BS000049541315PW_BS000054432511PW_BS0000432441011PW_BS000024221411PW_BS00002260251PW_BS00006046114PW_BS000046171211PW_BS00001729111PW_BS0000297028511PW_BS00007072513PW_BS000072612517PW_BS0000613612011PW_BS0000363772113PW_BS00003793252011PW_BS00009327151PW_BS000027711PW_BS000007971521PW_BS000097100521PW_BS000100105113PW_BS0001051115121PW_BS0001111136121PW_BS000113110231PW_BS0001101231751PW_BS0001231251351PW_BS000125126651PW_BS00012612711651PW_BS00012713013121PW_BS0001301141112PW_BS00011412915121PW_BS0001291355171PW_BS0001351181171PW_BS00011810813PW_BS0001086131PW_BS000006140103PW_BS000140101531PW_BS00010114315191PW_BS0001431465191PW_BS000146107313PW_BS000107951721PW_BS0000951471241PW_BS0001471553241PW_BS0001551572241PW_BS00015715924PW_BS00015911PW_BS00000116611PW_BS0001661783211PW_BS0001781802211PW_BS00018015284PW_BS000152117131PW_BS000117188118PW_BS0000241601181PW_BS000160205561PW_BS000024207661PW_BS000024206261PW_BS0000242111018PW_BS0000242137181PW_BS00002421425181PW_BS0000242156181PW_BS0000241985181PW_BS0000242164181PW_BS00002421013181PW_BS00002421217181PW_BS000024222341PW_BS000024224241PW_BS0000241901118PW_BS00002417018PW_BS0001702253541PW_BS00002416212181PW_BS000162261115PW_BS0000262771218PW_BS0000241644PW_BS0001642811251PW_BS0000242851041PW_BS0000242863641PW_BS0000242875341PW_BS0000242491341PW_BS0000242273441PW_BS00002465111PW_BS0000652905491PW_BS0000242916491PW_BS0000242924491PW_BS00002429817101PW_BS00002430013101PW_BS0000243016101PW_BS000024302116101PW_BS0000242231241PW_BS0000242941141PW_BS0000243081011PW_BS000024315123PW_BS0000243183123PW_BS0000243221231PW_BS00002429341PW_BS000024253541PW_BS0000241321121PW_BS00013213412121PW_BS0001343317121PW_BS00002833217121PW_BS0000283331212PW_BS0000281151012PW_BS0001153344121PW_BS0000283361121PW_BS000028337116121PW_BS00002834141121PW_BS00002834318121PW_BS00002834713125PW_BS00002832914121PW_BS0000283522512PW_BS00002835325127PW_BS00002835625121PW_BS000028360410121PW_BS0000283683601PW_BS0000283702601PW_BS000028228361PW_BS000024383751PW_BS0001003841251PW_BS000100390761PW_BS0001123911261PW_BS0001123987171PW_BS000113232403PW_BS000024122551PW_BS000122408451PW_BS000115124151PW_BS000124412125PW_BS000115405105PW_BS000115409115PW_BS0001154151851PW_BS0001154141551PW_BS0001154251355PW_BS000115429151PW_BS00011512112171PW_BS0001214192551PW_BS00011543441051PW_BS0001153821451PW_BS000100436255PW_BS0001153744171PW_BS0000534436171PW_BS0001154461217PW_BS0001153761017PW_BS0000531371117PW_BS00013744717171PW_BS00011513613171PW_BS000136448116171PW_BS00011545118171PW_BS00011545015171PW_BS00011546013175PW_BS0001154641171PW_BS00011545525171PW_BS000115469410171PW_BS00011539914171PW_BS0001134712517PW_BS00011547225177PW_BS0001152975101PW_BS0000244812101PW_BS0001154824101PW_BS0001152991101PW_BS0000244831110PW_BS0001154781010PW_BS00011548718101PW_BS00011549025101PW_BS0001154957101PW_BS00011548414101PW_BS00011548012101PW_BS000115502461PW_BS000115388161PW_BS000112208116PW_BS000024209106PW_BS0000245041861PW_BS0001155072561PW_BS00011551541061PW_BS0001153891461PW_BS0001123951361PW_BS0001135131761PW_BS0001157906111PW_BS0005248346111PW_BS00054914117191PW_BS000141111811PW_BS000011592711PW_BS0000595811411PW_BS0000581021231PW_BS0001021041431PW_BS00010419914181PW_BS0000241951318PW_BS000024350114121PW_BS00002833527121PW_BS0000282881441PW_BS00002443311451PW_BS0001154222751PW_BS000115468114171PW_BS00011537527171PW_BS00005349127101PW_BS0001155082761PW_BS0001151861221PW_BS0000248911421PW_BS000552219314PW_BS00002422014PW_BS00002416212PW_BS0000163211515PW_BS000032397113PW_BS0000396618518PW_BS0000665181PW_BS000051231511PW_BS000023918511PW_BS000091892PW_BS0000892171518PW_BS00002421815181PW_BS00002412815121PW_BS0001283511512PW_BS000028184121PW_BS000024109323PW_BS0001094101551PW_BS000115435155PW_BS00011544415171PW_BS0001154701517PW_BS00011548515101PW_BS0001154991510PW_BS0001155161561PW_BS000115517156PW_BS00011530635511PW_BS000024372102PW_BS000028215114PW_BS000021562611PW_BS000056422411PW_BS00004215612241PW_BS00015617912211PW_BS00017934524121PW_BS0000283583912PW_BS00002836912601PW_BS0000284182451PW_BS00011545424171PW_BS00011548924101PW_BS0001155062461PW_BS00011511812551PW_BS0005887851521PW_BS0005227132210111PW_BS000512856Butyryl-CoAHMDB0001088Butyryl-CoA is an intermediate in the metabolism of Butanoate. It is a substrate for Acyl-coenzyme A oxidase 3 (peroxisomal), 3-ketoacyl-CoA thiolase (mitochondrial), 3-ketoacyl-CoA thiolase (peroxisomal), Acyl-coenzyme A oxidase 1 (peroxisomal), Acyl-CoA dehydrogenase (medium-chain specific, mitochondrial), Acyl-CoA dehydrogenase (long-chain specific, mitochondrial), Acyl-coenzyme A oxidase 2 (peroxisomal), Acetyl-CoA acetyltransferase (mitochondrial), Acetyl-CoA acetyltransferase (cytosolic), Acyl-CoA dehydrogenase (short-chain specific, mitochondrial) and Trifunctional enzyme beta subunit (mitochondrial).2140-48-9C0013626557371CPD-12174260CCCC(=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=C2NC25H42N7O17P3SInChI=1S/C25H42N7O17P3S/c1-4-5-16(34)53-9-8-27-15(33)6-7-28-23(37)20(36)25(2,3)11-46-52(43,44)49-51(41,42)45-10-14-19(48-50(38,39)40)18(35)24(47-14)32-13-31-17-21(26)29-12-30-22(17)32/h12-14,18-20,24,35-36H,4-11H2,1-3H3,(H,27,33)(H,28,37)(H,41,42)(H,43,44)(H2,26,29,30)(H2,38,39,40)/t14-,18-,19-,20+,24-/m1/s1CRFNGMNYKDXRTN-CITAKDKDSA-N(2R)-4-({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)-N-(2-{[2-(butanoylsulfanyl)ethyl]-C-hydroxycarbonimidoyl}ethyl)-2-hydroxy-3,3-dimethylbutanimidic acid837.624837.157073179-2.329(2R)-4-[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]-N-(2-{[2-(butanoylsulfanyl)ethyl]-C-hydroxycarbonimidoyl}ethyl)-2-hydroxy-3,3-dimethylbutanimidic acid0-4FDB022419Butanoyl-coa;Butanoyl-coenzyme a;Butyryl-coa;Butyryl-coenzyme a;CoA(4:0)PW_C000856Btyl-CA5874843352751036999161710716377255133778721121202804061206334071229451201232571191256194791271465011407931851345Crotonoyl-CoAHMDB0002009Crotonoyl-CoA is an important component in several metabolic pathways, notably fatty acid and amino acid metabolism. It is the substrate of a group of enzymes acyl-Coenzyme A oxidases 1, 2, 3 (E.C.: 1.3.3.6) corresponding to palmitoyl, branched chain, and pristanoyl, respectively, in the peroxisomal fatty acid beta-oxidation, producing hydrogen peroxide. Abnormality of this group of enzymes is linked to coma, dehydration, diabetes, fatty liver, hyperinsulinemia, hyperlipidemia, and leukodystrophy. It is also a substrate of a group of enzymes called acyl-Coenzyme A dehydrogenase (E.C.:1.3.99-, including 1.3.99.2, 1.3.99.3) in the metabolism of fatty acids or branched chain amino acids in the mitochondria (Rozen et al., 1994). Acyl-Coenzyme A dehydrogenase (1.3.99.3) has shown to contribute to kidney-associated diseases, such as adrenogential syndrome, kidney failure, kidney tubular necrosis, homocystinuria, as well as other diseases including cretinism, encephalopathy, hypoglycemia, medium chain acyl-CoA dehydrogenase deficiency. The gene (ACADS) also plays a role in theta oscillation during sleep. In addition, crotonoyl-CoA is the substrate of enoyl coenzyme A hydratase (E.C.4.2.1.17) in the mitochondria during lysine degradation and tryptophan metabolism, benzoate degradation via CoA ligation; in contrast it is the product of this enzyme in the butanoate metabolism. Moreover, it is produced from multiple enzymes in the butanoate metabolism pathway, including 3-Hydroxybutyryl-CoA dehydratase (E.C.:4.2.1.55), glutaconyl-CoA decarboxylase (E.C.: 4.1.1.70), vinylacetyl-CoA Δ-isomerase (E.C.: 5.3.3.3), and trans-2-enoyl-CoA reductase (NAD+) (E.C.: 1.3.1.44). In lysine degradation and tryptophan metabolism, crotonoyl CoA is produced by glutaryl-Coenzyme A dehydrogenase (E.C.:1.3.99.7) lysine and tryptophan metabolic pathway. This enzyme is linked to type-1glutaric aciduria, metabolic diseases, movement disorders, myelinopathy, and nervous system diseases.102680-35-3C00877528038115473CPD-10834444072C\C=C\C(=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=C2NC25H40N7O17P3SInChI=1S/C25H40N7O17P3S/c1-4-5-16(34)53-9-8-27-15(33)6-7-28-23(37)20(36)25(2,3)11-46-52(43,44)49-51(41,42)45-10-14-19(48-50(38,39)40)18(35)24(47-14)32-13-31-17-21(26)29-12-30-22(17)32/h4-5,12-14,18-20,24,35-36H,6-11H2,1-3H3,(H,27,33)(H,28,37)(H,41,42)(H,43,44)(H2,26,29,30)(H2,38,39,40)/b5-4+/t14-,18-,19-,20+,24-/m1/s1KFWWCMJSYSSPSK-PAXLJYGASA-N(2R)-4-({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)-N-[2-({2-[(2E)-but-2-enoylsulfanyl]ethyl}-C-hydroxycarbonimidoyl)ethyl]-2-hydroxy-3,3-dimethylbutanimidic acid835.608835.141423115-2.179(2R)-4-[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]-N-[2-({2-[(2E)-but-2-enoylsulfanyl]ethyl}-C-hydroxycarbonimidoyl)ethyl]-2-hydroxy-3,3-dimethylbutanimidic acid0-4FDB0227922-butenoyl-coa;2-butenoyl-coenzyme a;But-2-enoyl-coa;But-2-enoyl-coenzyme a;Crotonyl-coenzyme a;S-but-2-enoylcoenzyme a;Trans-but-2-enoyl-coa;Trans-but-2-enoyl-coenzyme aPW_C001345CrtylCA58841083352269452761037000161823615177256133782811121202814061207914071229461201233811191256204791271475011407941859043-Hydroxybutyryl-CoAHMDB00011663-Hydroxybutyryl-CoA, also known as 3-hydroxybutanoyl-CoA or 3-OH-butyryl-CoA, belongs to the class of organic compounds known as (r)-3-hydroxyacyl coas. These are organic compounds containing a (R)-3-hydroxyl acylated coenzyme A derivative. 3-Hydroxybutyryl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). 3-Hydroxybutyryl-CoA has been primarily detected in urine. Within the cell, 3-hydroxybutyryl-CoA is primarily located in the mitochondria, peroxisome and cytoplasm. In humans, 3-hydroxybutyryl-CoA is involved in the lysine degradation pathway, the pyridoxine dependency with seizures pathway, the fatty acid metabolism pathway, and the butyrate metabolism pathway. 3-Hydroxybutyryl-CoA is also involved in several metabolic disorders, some of which include medium chain acyl-CoA dehydrogenase deficiency (mcad), the ethylmalonic encephalopathy pathway, the hyperlysinemia II or saccharopinuria pathway, and very-long-chain acyl CoA dehydrogenase deficiency (vlcad). 3-Hydroxybutyryl-CoA is a substrate for Enoyl-CoA hydratase (mitochondrial), Trifunctional enzyme alpha subunit (mitochondrial) and Peroxisomal bifunctional enzyme.2871-66-1C03561440045154522-METHYL-3-HYDROXY-BUTYRYL-COA389056C[C@H](O)CC(=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=C2NC25H42N7O18P3SInChI=1S/C25H42N7O18P3S/c1-13(33)8-16(35)54-7-6-27-15(34)4-5-28-23(38)20(37)25(2,3)10-47-53(44,45)50-52(42,43)46-9-14-19(49-51(39,40)41)18(36)24(48-14)32-12-31-17-21(26)29-11-30-22(17)32/h11-14,18-20,24,33,36-37H,4-10H2,1-3H3,(H,27,34)(H,28,38)(H,42,43)(H,44,45)(H2,26,29,30)(H2,39,40,41)/t13-,14+,18+,19+,20-,24+/m0/s1QHHKKMYHDBRONY-VKBDFPRVSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[(3R)-3-hydroxy-3-({2-[(2-{[(3S)-3-hydroxybutanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)-2,2-dimethylpropoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid853.623853.151987801-2.1410(S)-3-hydroxybutanoyl-coa0-4FDB022460(3r)-3-hydroxybutanoyl-coa;(3r)-3-hydroxybutanoyl-coenzyme a;(r)-3-hydroxybutanoyl-coa;(r)-3-hydroxybutanoyl-coenzyme a;(s)-3-hydroxybutanoyl-coa;(s)-3-hydroxybutanoyl-coenzyme a;3-hydroxybutanoyl-coa;3-hydroxybutanoyl-coenzyme a;3-hydroxybutyryl-coa;3-hydroxybutyryl-coenzyme a;3-oh-butyryl-coa;3-oh-butyryl-coenzyme a;Hydroxy-butyryl-coa;Hydroxy-butyryl-coenzyme a;Beta-hydroxybutyryl-coa;Beta-hydroxybutyryl-coenzyme a;Beta-hydroxybutyryl-s-coa;Beta-hydroxybutyryl-s-coenzyme aPW_C0009043HB-CoA5904108535277103700116182382261523815177257133782801121202824061207904071229471201233801191256214791271485011407951851420WaterHMDB0002111Water 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_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-N1-[(2R,3R,4S,5R)-5-({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl phosphono}oxy)(hydroxy)phosphoryl]oxy}methyl)-3,4-dihydroxyoxolan-2-yl]-3-(C-hydroxycarbonimidoyl)-1lambda5-pyridin-1-ylium663.4251663.109121631-2.5281-[(2R,3R,4S,5R)-5-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl phosphono}oxy(hydroxy)phosphoryl)oxy]methyl}-3,4-dihydroxyoxolan-2-yl]-3-(C-hydroxycarbonimidoyl)-1lambda5-pyridin-1-ylium0-1FDB0223093-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_C000721NAD140415033538651101114211344312735146654222949277917283529310794807184813184819284902649603151679552381035334111536011254691235482125559013556101185696100573810858271415912147594215160241556072157607616163851646917867721176890160701218870971637174205719720674051987459222824122683592259085224118192161232224913006298130183001325622342404322426193157710413277120133772091347737033177650336776673347770233277709130779151137798334778406356800063688069011993825124110552388112750166112853941199291221199524061201714071208344191209844081211594251212421261212594291218173831226143841227421201231304471231411361234194551235493741237314601238124431238294641243703981251871211253192971253424791255304811258062991258254901259244821265154951267654801268855011272785071273835021280893901283603911284283951407571851142Acetoacetyl-CoAHMDB0001484Acetoacetyl-CoA is an intermediate in the metabolism of Butanoate. It is a substrate for Succinyl-CoA:3-ketoacid-coenzyme A transferase 1 (mitochondrial), Hydroxymethylglutaryl-CoA synthase (mitochondrial), Short chain 3-hydroxyacyl-CoA dehydrogenase (mitochondrial), Trifunctional enzyme beta subunit (mitochondrial), Hydroxymethylglutaryl-CoA synthase (cytoplasmic), Peroxisomal bifunctional enzyme, Acetyl-CoA acetyltransferase (cytosolic), Acetyl-CoA acetyltransferase (mitochondrial), 3-hydroxyacyl-CoA dehydrogenase type II, Succinyl-CoA:3-ketoacid-coenzyme A transferase 2 (mitochondrial), 3-ketoacyl-CoA thiolase (mitochondrial), 3-ketoacyl-CoA thiolase (peroxisomal) and Trifunctional enzyme alpha subunit (mitochondrial).1420-36-6C0033243921415345ACETOACETYL-COA388353CC(=O)CC(=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=C2NC25H40N7O18P3SInChI=1S/C25H40N7O18P3S/c1-13(33)8-16(35)54-7-6-27-15(34)4-5-28-23(38)20(37)25(2,3)10-47-53(44,45)50-52(42,43)46-9-14-19(49-51(39,40)41)18(36)24(48-14)32-12-31-17-21(26)29-11-30-22(17)32/h11-12,14,18-20,24,36-37H,4-10H2,1-3H3,(H,27,34)(H,28,38)(H,42,43)(H,44,45)(H2,26,29,30)(H2,39,40,41)/t14-,18-,19-,20+,24-/m1/s1OJFDKHTZOUZBOS-CITAKDKDSA-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-[(3-oxobutanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid851.607851.136337737-2.169acetoacetyl-coa0-4FDB0226483-acetoacetyl-coa;3-acetoacetyl-coenzyme a;3-oxobutyryl-coa;3-oxobutyryl-coenzyme a;Acetoacetyl coa;Acetoacetyl coenzyme a;Acetoacetyl-coa;Acetoacetyl-coenzyme a;S-acetoacetylcoenzyme aPW_C001142ActaCoA5924792810493527910370021617292198735716375981608242226830621015239151772581337822411278914111901261701202834061207634071214651221229481201233591191240231351256224791260854811271495011275412061407961851144NADHHMDB0001487NADH 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_C001144NADH14341533490864810111521275514695422304927811728362931099480618481218482128490464959315169955240103533211153581125466123547912555931355698100573710858291415915147594515160271556079161638716472178677111768931607011188709916371722057195206746222282442268360225908622411809198118212161232024913003298130153001325522342403322426183157710713277123133772081347737133177651336776683347770033277707130779171137798634780009368806911199382212411054938811285494115838118119955406120172407120378122120986408121162425121244126121693429121818383122616384122745120123127447123138136123551374123734460123814443124242464124371398125189121125345479125531481125762297125808299125926482126516495126767480126888501127385502128090390128362391128429395140759185940Acetyl-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-CoA21343858842324162244652896173340114840145278103547612457331086025155607716163861647017869231607106163729119874602228245151827721012582226130122994261531577121133772911117756211277706132779941157835513478433334800073688063411980663376901241701199534061201454051203041221206324071224174081226263841227431201229591351231371181249863741252001211253434791255074781256332971265644821265724811267784801268865011270442091273942051276653881281375021281452061283743911407621851099Coenzyme 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_C001099CoA211438688453879228921724075924142245952813292862313342113351184618104629584842144865544879652321025247104528010354771245734108577710160231556075161638416468178693016069611626973199708318871081637293198734721074582228229151908122690902249124170921519513013299153182492548849426163157690729377119133772221347723032977292111775501327755533477563112776333367767212977996115780473327805635078413335785671307925933379974331800053688062011880627374806351198066537693828382938343839867428811055538911056139011584239911584739811995140612014740512023138412030512212063440712076211712140612312142143312152112512166642912168240812171441412240442212274112012290412112296013512396544712397946812407913612422046412426545012497437512534147912550947812557948012559248412563429712608448112654949112656048212674630012688450112704620912710939112730120512754020612766738812812150812813350212834039514075118614076318514076789110633-Hydroxy-3-methylglutaryl-CoAHMDB00013753-hydroxy-3-methylglutaryl CoA (HMG-CoA) is formed when Acetyl-CoA condenses with acetoacetyl-CoA in a reaction that is catalyzed by the enzyme HMG-CoA synthase in the mevalonate pathway or mevalonate-dependent (MAD) route, an important cellular metabolic pathway present in virtually all organisms. HMG-CoA reductase (EC 1.1.1.34) inhibitors, more commonly known as statins, are cholesterol-lowering drugs that have been widely used for many years to reduce the incidence of adverse cardiovascular events. HMG-CoA reductase catalyzes the rate-limiting step in the mevalonate pathway and these agents lower cholesterol by inhibiting its synthesis in the liver and in peripheral tissues. Androgen also stimulates lipogenesis in human prostate cancer cells directly by increasing transcription of the fatty acid synthase and HMG-CoA-reductase genes. (PMID: 14689582).1553-55-5C00356439218154673-HYDROXY-3-METHYL-GLUTARYL-COA388357C[C@](O)(CC(O)=O)CC(=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=C2NC27H44N7O20P3SInChI=1S/C27H44N7O20P3S/c1-26(2,21(40)24(41)30-5-4-15(35)29-6-7-58-17(38)9-27(3,42)8-16(36)37)11-51-57(48,49)54-56(46,47)50-10-14-20(53-55(43,44)45)19(39)25(52-14)34-13-33-18-22(28)31-12-32-23(18)34/h12-14,19-21,25,39-40,42H,4-11H2,1-3H3,(H,29,35)(H,30,41)(H,36,37)(H,46,47)(H,48,49)(H2,28,31,32)(H2,43,44,45)/t14-,19-,20-,21+,25-,27+/m1/s1CABVTRNMFUVUDM-VRHQGPGLSA-N(3S)-5-[(2-{3-[(2R)-3-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido]propanamido}ethyl)sulfanyl]-3-hydroxy-3-methyl-5-oxopentanoic acid911.659911.157467109-2.3511HMG-CoA0-5FDB022587(s)-3-hydroxy-3-methylglutaryl-coa;(s)-3-hydroxy-3-methylglutaryl-coenzyme a;3-hydroxy-3-methyl-glutaryl-coa;3-hydroxy-3-methyl-glutaryl-coenzyme a;3-hydroxy-3-methylglutaryl-coa;3-hydroxy-3-methylglutaryl-coenzyme a;Hmg-coa;Hmg-coenzyme a;Hydroxymethylglutaroyl coenzyme a;Hydroxymethylglutaryl-coa;Hydroxymethylglutaryl-coenzyme a;S-(hydrogen 3-hydroxy-3-methylglutaryl)coenzyme a;S-(hydrogen 3-hydroxy-3-methylpentanedioate;S-(hydrogen 3-hydroxy-3-methylpentanedioate) coenzyme a;S-(hydrogen 3-hydroxy-3-methylpentanedioic acidPW_C001063HMG-CoA595479421051315208729619873581638278210152401511524122277693133782251127891611112050940612076540712146712212311512012336111912402513542Acetoacetic acidHMDB0000060Acetoacetic acid (AcAc) is a weak organic acid that can be produced in the human liver under certain conditions of poor metabolism leading to excessive fatty acid breakdown (diabetes mellitus leading to diabetic ketoacidosis). It is then partially converted into acetone by decarboxylation and excreted either in urine or through respiration. Persistent mild hyperketonemia is a common finding in newborns. Ketone bodies serve as an indispensable source of energy for extrahepatic tissues, especially the brain and lung of developing rats. Another important function of ketone bodies is to provide acetoacetyl-CoA and acetyl-CoA for synthesis of cholesterol, fatty acids, and complex lipids. During the early postnatal period, acetoacetate and beta-hydroxybutyrate are preferred over glucose as substrates for synthesis of phospholipids and sphingolipids in accord with requirements for brain growth and myelination. Thus, during the first two weeks of postnatal development, when the accumulation of cholesterol and phospholipids accelerates, the proportion of ketone bodies incorporated into these lipids increases. On the other hand, an increased proportion of ketone bodies are utilized for cerebroside synthesis during the period of active myelination. In the lung, AcAc serves better than glucose as a precursor for the synthesis of lung phospholipids. The synthesized lipids, particularly dipalmityl phosphatidylcholine, are incorporated into surfactant, and thus have a potential role in supplying adequate surfactant lipids to maintain lung function during the early days of life (PMID: 3884391). The acid is also present in the metabolism of those undergoing starvation or prolonged physical exertion as part of gluconeogenesis. When ketone bodies are measured by way of urine concentration, acetoacetic acid, along with beta-hydroxybutyric acid or acetone, is what is detected.541-50-4C0016496153443-KETOBUTYRATE94DB01762CC(=O)CC(O)=OC4H6O3InChI=1S/C4H6O3/c1-3(5)2-4(6)7/h2H2,1H3,(H,6,7)WDJHALXBUFZDSR-UHFFFAOYSA-N3-oxobutanoic acid102.0886102.0316940580.371acetoacetic acid0-1FDB0218013-ketobutyrate;3-ketobutyric acid;3-oxo-butanoate;3-oxo-butanoic acid;3-oxobutyrate;3-oxobutyric acid;Acetoacetate;Diacetate;Diacetic acid;3-oxobutanoic acid;Beta-ketobutyric acid;3-oxobutanoate;B-ketobutyrate;B-ketobutyric acid;Beta-ketobutyrate;β-ketobutyrate;β-ketobutyric acidPW_C000042LIN59741053313028200326929160736016390661511524222277694133778351327822711278483111120511406120767407121019122121598124123117120123363119123584135124156118126609299127564388808Succinyl-CoAHMDB0001022Succinyl-CoA is an important intermediate in the citric acid cycle, where it is synthesized from α-Ketoglutarate by α-ketoglutarate dehydrogenase (EC 1.2.4.2) through decarboxylation, and is converted into succinate through the hydrolytic release of coenzyme A by succinyl-CoA synthetase (EC 6.2.1.5). Succinyl-CoA may be an end product of peroxisomal beta-oxidation of dicarboxylic fatty acids; the identification of an apparently specific succinyl-CoA thioesterase (ACOT4, EC 3.1.2.3, hydrolyzes succinyl-CoA) in peroxisomes strongly suggests that succinyl-CoA is formed in peroxisomes. Acyl-CoA thioesterases (ACOTs) are a family of enzymes that catalyze the hydrolysis of the CoA esters of various lipids to the free acids and coenzyme A, thereby regulating levels of these compounds. (PMID: 16141203).604-98-8C00091439161153803-METHYLBENZYLSUCCINYL-COA388307CC(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)CCC(O)=OC25H40N7O19P3SInChI=1S/C25H40N7O19P3S/c1-25(2,20(38)23(39)28-6-5-14(33)27-7-8-55-16(36)4-3-15(34)35)10-48-54(45,46)51-53(43,44)47-9-13-19(50-52(40,41)42)18(37)24(49-13)32-12-31-17-21(26)29-11-30-22(17)32/h11-13,18-20,24,37-38H,3-10H2,1-2H3,(H,27,33)(H,28,39)(H,34,35)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t13-,18-,19-,20?,24-/m1/s1VNOYUJKHFWYWIR-FZEDXVDRSA-N4-{[2-(3-{3-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido}propanamido)ethyl]sulfanyl}-4-oxobutanoic acid867.607867.131252359-2.19104-({2-[3-(3-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-2-hydroxy-3-methylbutanamido)propanamido]ethyl}sulfanyl)-4-oxobutanoic acid0-5FDB022375Coa s-(hydrogen succinate);Coa s-succinate;Coenzyme a s-(hydrogen succinate);Coenzyme a s-succinate;S-(hydrogen butanedioate;S-(hydrogen butanedioate) coa;S-(hydrogen butanedioate) coenzyme a;S-(hydrogen butanedioic acid;S-succinoylcoenzyme a;Suc-co-a;Suc-coa;Succ-coa;Succ-coenzyme a;Succ-s-coa;Succ-s-coenzyme a;Succ-s-coenzyme-a;Succ-coenzyme-a;Succino-1-yl-coenzyme a;Succinyl coa;Succinyl coenzyme a;Succinyl-s-coa;Succinyl-s-coenzyme a;Succinyl-s-coenzyme-a;Succinylcoenzyme-a;Succinylcoenzyme aPW_C000808Suc-CoA23341055336692537810360391556097161648517870151607361163747422277140133781011127857613280021368119978406120769407122014124122763120123365119124568118125358479126164299126306481126901501127868206174Succinic acidHMDB0000254Succinic acid is a dicarboxylic acid. The anion, succinate, is a component of the citric acid cycle capable of donating electrons to the electron transfer chain. Succinate dehydrogenase (SDH) plays an important role in the mitochondria, being both part of the respiratory chain and the Krebs cycle. SDH with a covalently attached FAD prosthetic group, binds enzyme substrates (succinate and fumarate) and physiological regulators (oxaloacetate and ATP). Oxidizing succinate links SDH to the fast-cycling Krebs cycle portion where it participates in the breakdown of acetyl-CoA throughout the whole Krebs cycle. The succinate can readily be imported into the mitochondrial matrix by the n-butylmalonate- (or phenylsuccinate-) sensitive dicarboxylate carrier in exchange with inorganic phosphate or another organic acid, e. g. malate. (PMID 16143825) Mutations in the four genes encoding the subunits of the mitochondrial respiratory chain succinate dehydrogenase are associated with a wide spectrum of clinical presentations (i.e.: Huntington's disease. (PMID 11803021).110-15-6C00042111015741SUC1078DB00139OC(=O)CCC(O)=OC4H6O4InChI=1S/C4H6O4/c5-3(6)1-2-4(7)8/h1-2H2,(H,5,6)(H,7,8)KDYFGRWQOYBRFD-UHFFFAOYSA-Nbutanedioic acid118.088118.026608680.252succinic acid0-2FDB0019311,2-ethanedicarboxylate;1,2-ethanedicarboxylic acid;1,4-butanedioate;1,4-butanedioic acid;Amber acid;Asuccin;Dihydrofumarate;Dihydrofumaric acid;Katasuccin;Succinate;Wormwood acid;Acide butanedioique;Acide succinique;Acidum succinicum;Bernsteinsaeure;Butandisaeure;Butanedionic acid;E363;Ethylenesuccinic acid;Hooc-ch2-ch2-cooh;Spirit of amber;Butanedionate;EthylenesuccinatePW_C000174Succini15232394502185078676311265542551753831036042155610216164541076455108648917867641176836166736216374552197456220747722211866198121421511325922342368318423693154240232277143133772131347748311177738112777491297842633480024368807211191128463081134281119984406120192407120385122120555414120990408122565384122767120123029135123189450123555374125138121125364479125549481125930482126713480126906501127082206127389502128304391414Adenosine 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_C000414ATP9221460826616414224781373332799593439976321051821121021464921561421605824055924342727264628122930296631637236166136175143992344743147689148645450328950352651557520597521510052501045291101531311153461125390103540611754301185443120554212955561325569133560313556211085846143585414658761075897147592415160481556109161623016664931786839188687016069761997157205718420672092107225213722921172981987302216739021774082187432163748122274991908186225118472771190317012010281120391641217828512578226126912901326422315327308423263154262132242694318770282537721813477233329774683337763233678037332780413507816812878214351782403537841133578494115788501307886533178919334800283688004618480674119856291948261241132349411328238811628010911991412211999240612015440712024538212036241212124642912139212312139743312147140812197441012206512512207938312208340512240242212244443512291939912300944612381646412395144712395646812402937412452744412461613612463039812463437612494347212497237512501147012530429712537147912539229912551548112559548412612348512622030012623449512624047812654749112659649912691350112712338912773151612778139512779639012780120912811950812816751714077089128Butyric acidHMDB0000039Butyric acid, a four-carbon fatty acid, is formed in the human colon by bacterial fermentation of carbohydrates (including dietary fiber), and putatively suppresses colorectal cancer (CRC). Butyrate has diverse and apparently paradoxical effects on cellular proliferation, apoptosis and differentiation that may be either pro-neoplastic or anti-neoplastic, depending upon factors such as the level of exposure, availability of other metabolic substrate and the intracellular milieu. In humans, the relationship between luminal butyrate exposure and CRC has been examined only indirectly in case-control studies, by measuring fecal butyrate concentrations, although this may not accurately reflect effective butyrate exposure during carcinogenesis. Perhaps not surprisingly, results of these investigations have been mutually contradictory. The direct effect of butyrate on tumorigenesis has been assessed in a no. of in vivo animal models, which have also yielded conflicting results. In part, this may be explained by methodology: differences in the amount and route of butyrate administration, which are likely to significantly influence delivery of butyrate to the distal colon. (PMID: 16460475) Butyric acid is a carboxylic acid found in rancid butter, parmesan cheese, and vomit, and has an unpleasant odor and acrid taste, with a sweetish aftertaste (similar to ether). Butyric acid is a fatty acid occurring in the form of esters in animal fats and plant oils. Interestingly, low-molecular-weight esters of butyric acid, such as methyl butyrate, have mostly pleasant aromas or tastes. As a consequence, they find use as food and perfume additives. Butyrate is produced as end-product of a fermentation process solely performed by obligate anaerobic bacteria.107-92-6C0024626430772259DB03568CCCC(O)=OC4H8O2InChI=1S/C4H8O2/c1-2-3-4(5)6/h2-3H2,1H3,(H,5,6)FERIUCNNQQJTOY-UHFFFAOYSA-Nbutanoic acid88.105188.05242950.431butyric acid0-1FDB0120621-butanoate;1-butanoic acid;1-butyrate;1-butyric acid;1-propanecarboxylate;1-propanecarboxylic acid;Butanate;Butanic acid;Butanoate;Butanoic acid;Buttersaeure;Butyrate;Butyric acid;Ethylacetate;Ethylacetic acid;Honey robber;Kyselina maselna;N-butanoate;N-butanoic acid;N-butyrate;N-butyric acid;Propanecarboxylate;Propanecarboxylic acid;Propylformate;Propylformic acid;4:0;Acide butanoique;Acide butyrique;Butoic acid;C4:0;Ch3-[ch2]2-cooh;ButoatePW_C0000284:06044606842882693316077695133776961117783813212051840612052112212246012412312012012312313512502711812661329912818638832Adenosine 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_C000032AMP112344628270167343288122118914457254867545033895251104540811754231035432118545712055581325583133577910157951086977199707218811789198118681611198815112003222125802261263631126942901333122542266342646315772343297732511178392334788091157932011280399180684135809007119916122120016124120031406120246382120888405121954408122920399123464376124507374125306297125394299125409479125596484126853205126934388126949501127124389127311209127711502140771891170PyrophosphateHMDB0000250The 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.14000-31-8C0001364410218361PPI559142DB04160OP(O)(=O)OP(O)(O)=OH4O7P2InChI=1S/H4O7P2/c1-8(2,3)7-9(4,5)6/h(H2,1,2,3)(H2,4,5,6)XPPKVPWEQAFLFU-UHFFFAOYSA-N(phosphonooxy)phosphonic acid177.9751177.9432255064pyrophosphoric 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_C000170Ppi122354638429237353288222121731620492410592815294175144868545034895252104529410154091175424103543311854581205548111555913255841335606135565510858791076239166697819970731887134163727216073121987318213827515182832101186916112002222120411641231522512323249125122881257922612695290152193061537518347601742561315426973187723532977317128776353367841633578928331791531127995013479958130800473728041717085630194786384948141259481938298678223110634391113270395113275389115527136115532399119934122120017124120032406120330410120936407121261429121341121121486383122407422122985444123502119123831464124044398124977375125324297125395299125410479125597484125656485125876481126552491126869205126935388126950501127337206128124508140772891423MagnesiumHMDB0000547Magnesium salts are essential in nutrition, being required for the activity of many enzymes, especially those concerned with oxidative phosphorylation. Physiologically, it exists as an ion in the body. It is a component of both intra- and extracellular fluids and is excreted in the urine and feces. Deficiency causes irritability of the nervous system with tetany, vasodilatation, convulsions, tremors, depression, and psychotic behavior. Magnesium ion in large amounts is an ionic laxative, and magnesium sulfate (Epsom salts) is sometimes used for this purpose. So-called "milk of magnesia" is a water suspension of one of the few insoluble magnesium compounds, magnesium hydroxide; the undissolved particles give rise to its appearance and name. Milk of magnesia is a mild base, and is commonly used as an antacid.22537-22-0C003058881842013-HYDROXY-MAGNESIUM-PROTOPORP865DB01378[Mg++]MgInChI=1S/Mg/q+2JLVVSXFLKOJNIY-UHFFFAOYSA-Nmagnesium(2+) ion24.30523.9850418980magnesium(2+) ion22FDB003518Magnesium;Magnesium ions;Magnesium ion;Magnesium, doubly charged positive ion;Magnesium, ion (mg(2+));Mg(2+);Mg2+PW_C000423Mg2+86822742681647627272681158191888322936399833992211167461483491529431764142124102411592942233126293373745403147749148695449745652531045329111535611253761035906147593415160381556094161625016664841786594164688116069791997170205719420672272137233211725021473102167313198747322211763132118432101231222512324249125132881258122612729290152752851533730877137133772363297793733678393334784173357848911578522331785363567857413080020368800451848004837280623118806541358086515809652538184151938323839490027108596223110559390115687398119974406120070122120247382120702407120981408121181124121265429121319419121924125122086405122408422122759120122921399123307119123546374123835464123889455124477136124637376124978375125447297125598484125669479125777481125921482125947299125973495126000490126243478126553491126753300127125389127164501127380502127407388127451507127804209128125508128347395140773891964FADHMDB0001248FAD, 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-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}[({[(2R,3S,4S)-5-{7,8-dimethyl-2,4-dioxo-2H,3H,4H,10H-benzo[g]pteridin-10-yl}-2,3,4-trihydroxypentyl]oxy}(hydroxy)phosphoryl)oxy]phosphinic acid785.5497785.157134455-2.279flavine-adenine dinucleotide0-3FDB0225111h-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_C000964FAD999114518681923216425317628288251884021188141489421612291622492133582536223723264602364688314741134758104881652681035285102533511154961265511127561311860301556054156608216161161626390164751786499179666610770391637175205732121374652227487223907622411818216118872151189921112296225123282491244315112519227125952261271029112720292130293011304130243623318770802937712613377152134775011137750711277518115775413347761513277726337780543297837534578930331792223367927235880012368800343698071411911995840611999938412005140812010740712043240512045312212049012412127842912129841812141738212148938312274812012277612112280237412282344312306637612308713512316644812384946412386845412397639912404739812534847912537848012542948212547448112569729712597948912610729912627748412689150112692039112696850212698720712701120612731020912743250612760238812784038914079018514079918669electron-transfer flavoproteinCompoundPW_EC0000695086ChEBIETF70Reduced electron-transfer flavoproteinCompoundPW_EC0000705086ChEBIRETF380Enoyl-CoA hydratase, mitochondrialP30084Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate.
HMDBP00388ECHS110q26.2-q26.3BT00712314.2.1.17591485332944174605252371031356231331438851181373Hydroxyacyl-coenzyme A dehydrogenase, mitochondrialQ16836Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
HMDBP00381HADH4q22-q26AF00190311.1.1.3559341086336Acetyl-CoA acetyltransferase, mitochondrialP24752Plays a major role in ketone body metabolism.
HMDBP00038ACAT111q22.3CH47106512.3.1.9594410503528110313563313313965178514098213141289238Hydroxymethylglutaryl-CoA synthase, mitochondrialP54868This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.
HMDBP00040HMGCS21p13-p12U8185612.3.3.10596410523803338137841713676Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrialP55809Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate.
HMDBP00712OXCT15p13.1AB02957612.8.3.55984105639Hydroxymethylglutaryl-CoA lyase, mitochondrialP35914Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism.
HMDBP00009HMGCL1p36.1-p35AK31386914.1.3.4603410543140984132405Acyl-coenzyme A synthetase ACSM1, mitochondrialQ08AH1Has medium-chain fatty acid:CoA ligase activity with broad substrate specificity (in vitro). Acts on acids from C(4) to C(11) and on the corresponding 3-hydroxy- and 2,3- or 3,4-unsaturated acids (in vitro). Functions as GTP-dependent lipoate-activating enzyme that generates the substrate for lipoyltransferase (By similarity).
HMDBP04188ACSM116p12.3AB06250316.2.1.2605448705414412326100Short-chain specific acyl-CoA dehydrogenase, mitochondrialP16219HMDBP00103ACADS12q24.31U8399211.3.8.158944365352721031356321333713-hydroxyacyl-CoA dehydrogenase type-2Q99714Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/TRMT10C, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5'-ends. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD).
HMDBP00379HSD17B10Xp11.2BC00870811.1.1.35; 1.1.1.51; 1.1.1.178849326694491061431161008143882118113Acetyl-CoA acetyltransferase, cytosolicQ9BWD1HMDBP00013ACAT26q25.3BC00040812.3.1.979389269131496Short/branched chain specific acyl-CoA dehydrogenase, mitochondrialP45954Has greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
HMDBP01613ACADSB10q26.13AF26067811.3.8.5926443643488265271103135631133101Medium-chain specific acyl-CoA dehydrogenase, mitochondrialP11310This enzyme is specific for acyl chain lengths of 4 to 16.
HMDBP00104ACADM1p31M9143211.3.8.78974435334354346092527010313563013313785471399Long-chain specific acyl-CoA dehydrogenase, mitochondrialP28330HMDBP00102ACADL2q34CH47106311.3.8.88964469035269103135629133137853713177Enoyl-CoA hydratase, mitochondrial1PW_P0001771953806178Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial1PW_P0001781963732179Acetyl-CoA acetyltransferase, mitochondrial1PW_P000179197364180Hydroxymethylglutaryl-CoA synthase, mitochondrial1PW_P0001801983821813-oxoacid CoA-transferase 11PW_P0001811996762182Hydroxymethylglutaryl-CoA lyase, mitochondrial1PW_P00018220092183Acyl-coenzyme A synthetase ACSM1, mitochondrial1PW_P00018320124051814231176Short-chain specific acyl-CoA dehydrogenase, mitochondrial1PW_P00017619410048096412423-hydroxyacyl-CoA dehydrogenase type-21PW_P0002422603714229Acetyl-CoA acetyltransferase, cytosolic1PW_P000229247134252Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial1PW_P000252271149641179641251Medium-chain specific acyl-CoA dehydrogenase, mitochondrial1PW_P00025127010141169641250Long-chain specific acyl-CoA dehydrogenase, mitochondrial1PW_P0002502699941159641679falsePW_R000679Both27849041Compoundfalse278513451Compoundfalse278614201Compoundtrue1761774.2.1.17680falsePW_R000680Right27879041Compoundfalse27887211Compoundtrue278911421Compoundfalse279011441Compoundtrue5616400341Compoundtrue1771781.1.1.351199242681falsePW_R000681Both27919402Compoundtrue279211421Compoundfalse279310991Compoundtrue1781792.3.1.95172292.3.1.9682falsePW_R000682Right27949401Compoundfalse279511421Compoundfalse279614201Compoundtrue279710631Compoundfalse279810991Compoundtrue20891802.3.3.10685falsePW_R000685Both2803421Compoundfalse28048081Compoundfalse28051741Compoundfalse280611421Compoundfalse1821812.8.3.5686falsePW_R000686Right280710631Compoundfalse2808421Compoundfalse28099401Compoundfalse1831824.1.3.4688falsePW_R000688Right28124141Compoundtrue2813281Compoundfalse281410991Compoundtrue2815321Compoundtrue28161701Compoundtrue28178561Compoundfalse1851836.2.1.2678falsePW_R000678Both27828561Compoundfalse8490691ElementCollectiontrue278313451Compoundfalse8491701ElementCollectiontrue1751761.3.8.13072521.3.8.53082511.3.8.73092501.3.8.813PW_T00001313281Compound84Right828856482false115091510regular3002808291345482false37591510regular300280831904482false375147510regular3002808321420449false590120510regular7878833721459false740152010regular50308341142482false1155147510regular3002808351144460false1010151510regular5030836940482false1905147510regular3002808371099485false1520150510regular50308381420449false1830174510regular78788391063482false1720220010regular3002808401099485false1950202010regular503084142481false1210224510regular200190842808482false895198510regular300280843174481false980176010regular200190847940482false1280245010regular300280848414442false184595010regular503084928481false201096010regular2001908501099485false1855114010regular503085132444false159595510regular5030852170445false1585114010regular634385342349false1690100010regular1002585428881false278596010regular2001901127696439false857100510regular1002526869373false1020112512regular1009026970373false720111512regular10090260380417false43513008subunitregular1808526137346false81515758subunitregular160802623648false160515758subunitregular140852633846false179019058subunitregular1608026467646false123019228subunitregular16080266946false148723008subunitregular16080267240542false167010208subunitregular15070593010038false83710158subunitregular140852351772042602602361782042612612371792042622622381802042632632391812042642642411822042662662421832042672671128531152Cofactor48811762035901593023191127617610Cofactor1121M525 1475 C525 1445 525 1413 525 1383 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1122M525 1195 C525 1225 525 1270 525 1300 5false18falsefalse1123M590 1244 C556 1244 525 1270 525 1300 5false18falsefalse1124M675 1615 C705 1615 785 1615 815 1615 5false181125M765 1550 C766 1574 785 1615 815 1615 5false181126M1155 1615 C1125 1615 1005 1615 975 1615 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1127M1030 1540 C1030 1570 1005 1615 975 1615 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1128M1905 1615 C1875 1615 1775 1615 1745 1615 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1129M1455 1615 C1485 1615 1575 1615 1605 1615 5false18falsefalse1130M1545 1535 C1545 1568 1575 1615 1605 1615 5false18falsefalse1131M2055 1755 C2057 1832 1870 1863 1870 1905 5false181132M1455 1615 C1693 1611 1869 1853 1870 1905 5false181133M1869 1823 C1869 1853 1870 1875 1870 1905 5false181134M1870 2200 C1870 2170 1870 2015 1870 1985 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1135M1950 2035 C1921 2035 1870 2015 1870 1985 5false18trueM 1502.0096189432334 1742.5 L 1515 1735 L 1502.0096189432334 1727.5false1136M1310 2245 C1310 2215 1310 2032 1310 2002 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1137M1195 2125 C1243 2125 1310 2032 1310 2002 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1138M1180 1855 C1233 1855 1310 1892 1310 1922 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1139M1305 1755 C1305 1824 1310 1826 1310 1922 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1143M1720 2340 C1690 2340 1677 2340 1647 2340 5false181144M1410 2340 C1440 2340 1457 2340 1487 2340 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1145M1430 2450 C1430 2423 1436 2340 1487 2340 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1146M1870 980 C1870 1019 1850 1055 1820 1055 5false181147M2010 1055 C1980 1055 1850 1055 1820 1055 5false181148M1880 1140 C1880 1107 1850 1055 1820 1055 5false181149M1620 985 C1620 1023 1640 1055 1670 1055 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1150M1616.5 1140 C1616.5 1112 1640 1055 1670 1055 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1151M1450 1055 C1480 1055 1640 1055 1670 1055 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1152M1545 1060 L1545 1110 L1595 1060 z10true181153M2785 1055 C2755 1055 2254 1054 2210 1055 83false18trueM 1817.8166168619703 827.2067765197284 L 1805 835 L 1818.1574379422698 842.2029040529026false1154M2210 1055 C2240 1055 1986 1038 2010 1055 83true181158M2885 960 C2885 930 2885 355 2885 325 83false18trueM 2977.5 772.0096189432334 L 2985 785 L 2992.5 772.0096189432334false17608M1150 1055 C1120 1055 1007 1055 977 1055 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false17609M675 1055 C705 1055 807 1055 837 1055 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false17610M987.5 1080 L987.5 1130 L1037.5 1080 z10true1817611M1070 1125 C1072 1066 1007 1055 977 1055 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false17612M770 1115 C770 1069 807 1055 837 1055 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false17637M1210 2340 C1174 2339 1200 2339 1165 2340 5false18trueM 955.2359593727941 2135.776641855654 L 960 2150 L 969.935769166525 2138.7625407200057false2492067948978311121Left8988291122Right8998321123Right2331762352502068049008311124Left9018331125Left9028341126Right9038351127Right2341772362512068149048361128Left9058341129Right9068371130Right2351782372522068249078361131Left9088341132Left9098381133Left9108391134Right9118401135Right2361792382532068549128411136Left9138421137Left9148431138Right9158341139Right2371822392552068649188391143Left9198411144Right9208471145Right2391832412562068849218481146Left9228491147Left9238501148Left9248511149Right9258521150Right9268281151Right24018524232422067831066682817608Left1066782917609Right28026817611Left28126917612Right34111754881251320498541153Left508491154Right15172086false27359516regular208541158Left9648702014false1015230516regular89884117637Left19136604400.90.9021490240187195257821001.91.90314327267479806270123061.61.6-1052328036038M229 822 C229 772 279 722 329 722 C969 722 1802 722 2442 722 C2492 722 2542 772 2542 822 C2542 1414 2542 2184 2542 2776 C2542 2826 2492 2876 2442 2876 C1802 2876 969 2876 329 2876 C279 2876 229 2826 229 2776 C229 2184 229 1414 229 822 84true62313.02154.039M315 905 C315 855 365 805 415 805 C1002 805 1766 805 2353 805 C2403 805 2453 855 2453 905 C2453 1437 2453 2130 2453 2662 C2453 2712 2403 2762 2353 2762 C1766 2762 1002 2762 415 2762 C365 2762 315 2712 315 2662 C315 2130 315 1437 315 905 84true62138.01957.0167M124 474 C124 424 174 374 224 374 C1118 374 2281 374 3175 374 C3225 374 3275 424 3275 474 C3275 1202 3275 2147 3275 2875 C3275 2925 3225 2975 3175 2975 C2281 2975 1118 2975 224 2975 C174 2975 124 2925 124 2875 C124 2147 124 1202 124 474 1true63151.02601.041235Mitochondrion725420201.31.32001542235Mitochondrial matrix780875201.31.3200154315Inner Mitochondria Membrane2300950201.31.3160154415Outer Mitochondria Membrane2485865201.31.31601545235Intracellular Space2410410201.31.32001546235Extracellular Space2420230201.31.320015901235Intracellular Space1245435201.31.320015902235Extracellular Space1225245201.31.3200158848526202695256729044#FFEBEB423652209