101PathwayPropanoate MetabolismThis pathway depicts the metabolism of propionic acid. Propionic acid in mammals typically arises from the production of the acid by gut or skin microflora. Propionic acid producing bacteria (Propionibacterium sp.) are particularly common in sweat glands of mammals. After entering a cell, the propionic acid (propanoate) then enters the mitochondria where it is converted into propanol adenylate (or propionyl adenylate or propionyl-AMP) via propionyl-CoA synthetase and acetyl-CoA synthetase. The propionyl adenylate then is converted into propionyl coenzyme A (propionyl-CoA) via the same pair of enzymes. Propionyl-CoA is a relatively common compound that can also arise from the metabolic breakdown of fatty acids containing odd numbers of carbon atoms. Propionyl-CoA is also known to arise from the breakdown of some amino acids. Since propanoate has three carbons, propionyl-CoA cannot directly enter the beta-oxidation cycle (which requires two carbons from acetyl-CoA). Therefore, in most vertebrates, propionyl-CoA is carboxylated into D-methylmalonyl-CoA via propionyl-CoA carboxylase. The resulting compound is isomerized into L-methylmalonyl-CoA via methylmalonyl-CoA epimerase. A vitamin B12-dependent enzyme, called methylmalonyl CoA mutase catalyzes the rearrangement of L-methylmalonyl-CoA to succinyl-CoA, which is an intermediate of the citric acid cycle. Also depicted in this pathway is another propionic acid homolog called hydroxypropanoic acid (hydroxypropionate). This compound is also produced by bacteria and imported into cells. Hydroxypropionate can be converted into 3-hydroxypropionyl-CoA. This compound can be either enzymatically converted to acryloyl-CoA and then to propionyl-CoA or it can spontaneously convert to malonyl-CoA. Malonyl-CoA can convert into acetyl-CoA (via acetyl-CoA carboxylase in the cytoplasm or malonyl carboxylase in the mitochondria) whereupon it may enter a variety of pathways. In a rare genetic metabolic disorder called propionic acidemia, propionate acts as a metabolic toxin in liver cells by accumulating in the liver mitochondria as propionyl-CoA and its derivative methylcitrate. Both propionyl-CoA and methylcitrate are known TCA inhibitors. Glial cells are particularly susceptible to propionyl-CoA accumulation. In fact, when propionate is infused into rat brains and take up by the glial cells, it leads to behavioural changes that resemble autism (PMID: 16950524).MetabolicPW000149CenterPathwayVisualizationContext16437005500#000099PathwayVisualization93101Propanoate MetabolismThis pathway depicts the metabolism of propionic acid. Propionic acid in mammals typically arises from the production of the acid by gut or skin microflora. Propionic acid producing bacteria (Propionibacterium sp.) are particularly common in sweat glands of mammals. After entering a cell, the propionic acid (propanoate) then enters the mitochondria where it is converted into propanol adenylate (or propionyl adenylate or propionyl-AMP) via propionyl-CoA synthetase and acetyl-CoA synthetase. The propionyl adenylate then is converted into propionyl coenzyme A (propionyl-CoA) via the same pair of enzymes. Propionyl-CoA is a relatively common compound that can also arise from the metabolic breakdown of fatty acids containing odd numbers of carbon atoms. Propionyl-CoA is also known to arise from the breakdown of some amino acids. Since propanoate has three carbons, propionyl-CoA cannot directly enter the beta-oxidation cycle (which requires two carbons from acetyl-CoA). Therefore, in most vertebrates, propionyl-CoA is carboxylated into D-methylmalonyl-CoA via propionyl-CoA carboxylase. The resulting compound is isomerized into L-methylmalonyl-CoA via methylmalonyl-CoA epimerase. A vitamin B12-dependent enzyme, called methylmalonyl CoA mutase catalyzes the rearrangement of L-methylmalonyl-CoA to succinyl-CoA, which is an intermediate of the citric acid cycle. Also depicted in this pathway is another propionic acid homolog called hydroxypropanoic acid (hydroxypropionate). This compound is also produced by bacteria and imported into cells. Hydroxypropionate can be converted into 3-hydroxypropionyl-CoA. This compound can be either enzymatically converted to acryloyl-CoA and then to propionyl-CoA or it can spontaneously convert to malonyl-CoA. Malonyl-CoA can convert into acetyl-CoA (via acetyl-CoA carboxylase in the cytoplasm or malonyl carboxylase in the mitochondria) whereupon it may enter a variety of pathways. In a rare genetic metabolic disorder called propionic acidemia, propionate acts as a metabolic toxin in liver cells by accumulating in the liver mitochondria as propionyl-CoA and its derivative methylcitrate. Both propionyl-CoA and methylcitrate are known TCA inhibitors. Glial cells are particularly susceptible to propionyl-CoA accumulation. In fact, when propionate is infused into rat brains and take up by the glial cells, it leads to behavioural changes that resemble autism (PMID: 16950524).Metabolic111862584SubPathway120565940Compound41186262SubPathway120566808Compound4118627Intestinal Microflora SubPathway120567159Compound4120568552Compound411862886SubPathway120569988Compound411862962SubPathway12057040Compound4118630Cysteine and Methionine MetabolismSubPathway1205713Compound1118631Glycine, Serine, and Threonine MetabolismSubPathway1205723Compound1118632Tricarboxylate Transport SystemSubPathway120573940Compound12057463Compound47Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.101Pathway48Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.101Pathway1CellCL:00000002Platelet CL:00002335HepatocyteCL:00001823NeuronCL:00005404CardiomyocyteCL:00007468Beta cellCL:00006397Epithelial CellCL:00000666MyocyteCL:000018712AstrocyteCL:000012711Colorectal Cancer CellCL:00010641Homo sapiens9606EukaryoteHuman12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat10Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm3Escherichia coli562Prokaryote18Saccharomyces cerevisiae4932EukaryoteYeast4Arabidopsis thaliana3702EukaryoteThale cress2Bacteria2ProkaryoteBacteria23Pseudomonas aeruginosa287Prokaryote24Solanum lycopersicum4081EukaryoteTomato21Xenopus laevis8355EukaryoteAfrican clawed frog60Nitzschia sp.0001EukaryoteNitzschia449Bathymodiolus platifrons220390EukaryoteDeep sea mussel19Schizosaccharomyces pombe4896Eukaryote25Escherichia coli (strain K12)83333Prokaryote51Picea sitchensis3332EukaryoteSitka spruce202Spathaspora passalidarum340170EukaryoteSpathaspora passalidarum62Acinetobacter baylyi (strain ATCC 33305 / BD413 / ADP1)62977Prokaryote55Yarrowia lipolytica4952Eukaryoteyli135Felinus9685EukaryoteCat240Plasmodium falciparums121Eukaryote3Mitochondrial MatrixGO:00057592MitochondrionGO:00057391CytosolGO:00058295CytoplasmGO:000573731Periplasmic SpaceGO:000562035ChloroplastGO:000950713Endoplasmic ReticulumGO:000578311Extracellular SpaceGO:00056154PeroxisomeGO:000577712Mitochondrial Inner MembraneGO:00057438Smooth Endoplasmic Reticulum GO:000579010Cell MembraneGO:000588625Golgi ApparatusGO:00057947Endoplasmic Reticulum MembraneGO:000578914Mitochondrial Outer MembraneGO:000574115NucleusGO:000563427Peroxisome MembraneGO:000577832Inner MembraneGO:007025819Sarcoplasmic ReticulumGO:00165296LysosomeGO:000576416Lysosomal LumenGO:004320224Mitochondrial Intermembrane SpaceGO:000575818Melanosome MembraneGO:003316220Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520236MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032540PeriplasmGO:004259739Mitochondrial membraneGO:00319661LiverBTO:00007597294Adrenal MedullaBTO:000004971828StomachBTO:0001307155262Endothelium BTO:00003937Nervous SystemBTO:000148418PancreasBTO:000098825IntestineBTO:00006488Blood VesselBTO:000110274119MuscleBTO:00008871411824BrainBTO:000014289163Sympathetic Nervous SystemBTO:000183211HeartBTO:000056273106KidneyBTO:000067171815BoneBTO:000014022BladderBTO:00001234311PW_BS0000041333121PW_BS000133406351PW_BS0001151203171PW_BS0001204793101PW_BS000115501361PW_BS0001153211PW_BS0000031122121PW_BS000112407251PW_BS0001151192171PW_BS0001194812101PW_BS000115206261PW_BS0000242111PW_BS0000021321121PW_BS000132124151PW_BS0001241181171PW_BS0001182991101PW_BS000024388161PW_BS0001128511PW_BS000008107313PW_BS00010710813PW_BS0001081873118PW_BS000024224241PW_BS000024151141PW_BS0001512253541PW_BS000024310312PW_BS000024315123PW_BS0000243183123PW_BS0000241115121PW_BS000111122551PW_BS0001221355171PW_BS000135181311PW_BS00001815111PW_BS00001513013121PW_BS0001301141112PW_BS000114801111PW_BS0000801251351PW_BS0001254301151PW_BS00011513613171PW_BS00013646511171PW_BS000115103331PW_BS0001031553241PW_BS0001551613181PW_BS0001611783211PW_BS0001781601181PW_BS0001601632181PW_BS000163222341PW_BS0000243683601PW_BS0000282975101PW_BS000024205561PW_BS0000245411PW_BS000005171211PW_BS000017111811PW_BS00001114101PW_BS00001411PW_BS0000011985181PW_BS00002421013181PW_BS000024226441PW_BS0000241151012PW_BS00011513412121PW_BS0001343344121PW_BS0000283761017PW_BS00005317018PW_BS000170405105PW_BS000115408451PW_BS0001153841251PW_BS0001003744171PW_BS00005312112171PW_BS0001214781010PW_BS0001154824101PW_BS00011548012101PW_BS000115209106PW_BS000024502461PW_BS0001153911261PW_BS000112185321PW_BS00002413121PW_BS000013311511PW_BS000031204111PW_BS000020432511PW_BS00004349711PW_BS0000497028511PW_BS000070101531PW_BS000101100521PW_BS0001001901118PW_BS000024188118PW_BS0000242137181PW_BS0000242491341PW_BS0000242771218PW_BS0000242905491PW_BS00002429341PW_BS0000243331212PW_BS00002812915121PW_BS00012934141121PW_BS00002835625121PW_BS0000283317121PW_BS000028412125PW_BS0001154141551PW_BS0001154192551PW_BS000115383751PW_BS0001004461217PW_BS00011545015171PW_BS00011545525171PW_BS0001153987171PW_BS00011349025101PW_BS0001155072561PW_BS00011516212PW_BS000016221411PW_BS0000223211515PW_BS000032397113PW_BS000039101711PW_BS0000105811411PW_BS000058592711PW_BS00005927151PW_BS00002746114PW_BS00004629111PW_BS0000296618518PW_BS00006672513PW_BS000072612517PW_BS0000615181PW_BS000051231511PW_BS000023918511PW_BS000091541315PW_BS000054892PW_BS000089261115PW_BS000026711PW_BS000007971521PW_BS0000971041431PW_BS000104117131PW_BS00011714315191PW_BS0001431465191PW_BS0001461471241PW_BS00014716611PW_BS00016619914181PW_BS0000242111018PW_BS0000242164181PW_BS0000242171518PW_BS00002421815181PW_BS0000242811251PW_BS0000241644PW_BS0001642851041PW_BS0000242231241PW_BS0000243081011PW_BS0000243221231PW_BS000024253541PW_BS00002432914121PW_BS0000283361121PW_BS00002833217121PW_BS000028350114121PW_BS00002812815121PW_BS0001283511512PW_BS00002835325127PW_BS00002833527121PW_BS000028184121PW_BS000024943PW_BS000094109323PW_BS0001093821451PW_BS000100429151PW_BS0001151231751PW_BS00012343311451PW_BS0001154101551PW_BS0001154222751PW_BS000115435155PW_BS00011539914171PW_BS0001134641171PW_BS00011544717171PW_BS000115468114171PW_BS00011544415171PW_BS00011547225177PW_BS00011537527171PW_BS0000534701517PW_BS00011548414101PW_BS00011548515101PW_BS00011530013101PW_BS0000244957101PW_BS00011549127101PW_BS0001154991510PW_BS0001153891461PW_BS0001125161561PW_BS0001153951361PW_BS000113390761PW_BS0001125082761PW_BS000115517156PW_BS0001158911421PW_BS000552471914PW_BS00004731323PW_BS000024105113PW_BS000105409115PW_BS0001151371117PW_BS0001374831110PW_BS00011530635511PW_BS0000242941141PW_BS0000246131PW_BS0000061021231PW_BS00010216212181PW_BS0001621951318PW_BS0000242881441PW_BS0000241861221PW_BS0000242892491PW_BS0000249611PW_BS0000092811611PW_BS000028951721PW_BS00009514117191PW_BS0001411572241PW_BS00015729817101PW_BS0000241136121PW_BS00011334713125PW_BS0000284251355PW_BS000115126651PW_BS00012646013175PW_BS0001154436171PW_BS000115208116PW_BS000024422411PW_BS000042509516PW_BS00005085241011PW_BS00008522014PW_BS00002434524121PW_BS00002834695126PW_BS00002832711125PW_BS0000284182451PW_BS0001154239556PW_BS0001154241155PW_BS00011545424171PW_BS00011545895176PW_BS00011545911175PW_BS00011548924101PW_BS0001155062461PW_BS000115711113PW_BS000071207661PW_BS0000243201123PW_BS0000243016101PW_BS0000248424111PW_BS00008459724112PW_BS000336193513PW_BS00001921217181PW_BS0000245131761PW_BS000115331811PW_BS0000332441011PW_BS00002460251PW_BS0000603612011PW_BS0000363772113PW_BS00003793252011PW_BS000093110231PW_BS00011012711651PW_BS000127140103PW_BS00014015924PW_BS0001591802211PW_BS00018015284PW_BS00015221425181PW_BS0000242156181PW_BS0000242863641PW_BS0000242875341PW_BS0000242273441PW_BS00002465111PW_BS0000652916491PW_BS0000242924491PW_BS000024302116101PW_BS000024337116121PW_BS00002834318121PW_BS0000283522512PW_BS000028360410121PW_BS0000283702601PW_BS000028228361PW_BS000024232403PW_BS0000244151851PW_BS00011543441051PW_BS000115436255PW_BS000115448116171PW_BS00011545118171PW_BS000115469410171PW_BS0001154712517PW_BS00011548718101PW_BS0001155041861PW_BS00011551541061PW_BS0001157906111PW_BS0005248346111PW_BS00054915612241PW_BS00015617912211PW_BS0001793583912PW_BS00002836912601PW_BS000028372102PW_BS000028219314PW_BS0000243125231PW_BS00002430412PW_BS00002488231202PW_BS00055288312021PW_BS000552167311PW_BS000167168321PW_BS000168788241113PW_BS0005246531624PW_BS000508393151PW_BS00017384711PW_BS0005497851521PW_BS00052211812551PW_BS0005887132210111PW_BS00051270231351PW_BS000512102032401PW_BS0005771459Malonyl-CoA semialdehydeHMDB0002170Malonyl-CoA semialdehyde is involved in the propanoate metabolism pathway. Malonyl-CoA semialdehyde can be reversibly produced from malonyl-CoA and 3-hydroxy-propionyl-CoA. Malonic semialdehyde is formed in the alternative pathway of propionate metabolism and in the catabolism of beta-alanine. Studies of these pathways in cultured cells from a patient with mitochondrial malonyl-CoA decarboxylase deficiency indicate that malonic semialdehyde is directly converted into acetyl-CoA in man. (PMID: 6418146).6244-93-5C05989440865389702CC(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)CC=OC24H38N7O18P3SInChI=1S/C24H38N7O18P3S/c1-24(2,19(36)22(37)27-5-3-14(33)26-6-8-53-15(34)4-7-32)10-46-52(43,44)49-51(41,42)45-9-13-18(48-50(38,39)40)17(35)23(47-13)31-12-30-16-20(25)28-11-29-21(16)31/h7,11-13,17-19,23,35-36H,3-6,8-10H2,1-2H3,(H,26,33)(H,27,37)(H,41,42)(H,43,44)(H2,25,28,29)(H2,38,39,40)/t13-,17-,18-,19?,23-/m1/s1NMEYBPUHJHMRHU-UXYNFSPESA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy(3-hydroxy-2,2-dimethyl-3-{[2-({2-[(3-oxopropanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid837.581837.120687673-2.339[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3-hydroxy-2,2-dimethyl-3-{[2-({2-[(3-oxopropanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid0-4FDB0228821-thiomalonaldehydic acid s-ester with coenzyme a;3-oxopropionyl-coa;3-oxopropionyl-coenzyme a;Malonylsemialdehyde coenzyme a;Malonylsemialdehyde-coa;Malonylsemialdehyde-coenzyme a;S-(3-oxopropanoate;S-(3-oxopropanoate)coa;S-(3-oxopropanoate)coenzyme a;S-(3-oxopropanoic acidPW_C001459MaCoAsa227947862113312224940612480212012641247912797750129423-Hydroxypropionyl-CoAHMDB00068073-Hydroxypropionyl-CoA is an intermediate in b-Alanine (beta-alanine) metabolism. It can be produced from 3-hydroxypropanoic acid via the enzyme 3-hydroxyisobutyryl-Coenzyme A hydrolase (EC:3.1.2.4) or it can be generated from acrylyl CoA via the enzyme enoyl-CoA hydratase (EC:4.2.1.17). Acrylyl CoA is derived from propionyl CoA.157786-88-4C05668440753277623-HYDROXY-PROPIONYL-COA389623CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12)C(O)C(=O)NCCC(=O)NCCSC(=O)CCOC24H40N7O18P3SInChI=1S/C24H40N7O18P3S/c1-24(2,19(36)22(37)27-5-3-14(33)26-6-8-53-15(34)4-7-32)10-46-52(43,44)49-51(41,42)45-9-13-18(48-50(38,39)40)17(35)23(47-13)31-12-30-16-20(25)28-11-29-21(16)31/h11-13,17-19,23,32,35-36H,3-10H2,1-2H3,(H,26,33)(H,27,37)(H,41,42)(H,43,44)(H2,25,28,29)(H2,38,39,40)/t13-,17-,18-,19?,23-/m1/s1BERBFZCUSMQABM-UXYNFSPESA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy(3-hydroxy-3-{[2-({2-[(3-hydroxypropanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-2,2-dimethylpropoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid839.597839.136337737-2.2910[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3-hydroxy-3-{[2-({2-[(3-hydroxypropanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-2,2-dimethylpropoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid0-4FDB0240933-hydroxypropanoyl coenzymea;3-hydroxypropanoyl-coa;3-hydroxypropanoyl-coenzyme a;3-hydroxypropionyl coenzyme aPW_C0029423HP-CoA22804786221331222504061248031201264134791279785011560S-Methylmalonyl-CoAHMDB0002310S-Methylmalonyl-CoA belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain. S-Methylmalonyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). S-Methylmalonyl-CoA has been primarily detected in urine. Within the cell, S-methylmalonyl-CoA is primarily located in the cytoplasm, mitochondria and peroxisome. In humans, S-methylmalonyl-CoA is involved in the threonine and 2-oxobutanoate degradation pathway, the propanoate metabolism pathway, and the valine, leucine and isoleucine degradation pathway. S-Methylmalonyl-CoA is also involved in several metabolic disorders, some of which include the isovaleric aciduria pathway, the maple syrup urine disease pathway, isobutyryl-CoA dehydrogenase deficiency, and the 3-methylglutaconic aciduria type III pathway. Methylmalonyl-CoA is an intermediate in the metabolism of Propanoate. It is a substrate for Malonyl-CoA decarboxylase (mitochondrial), Methylmalonyl-CoA mutase (mitochondrial) and Methylmalonyl-CoA epimerase (mitochondrial).73173-91-8C0068321252287D-METHYL-MALONYL-COA13628334C[C@@H](C(O)=O)C(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12C25H40N7O19P3SInChI=1S/C25H40N7O19P3S/c1-12(23(37)38)24(39)55-7-6-27-14(33)4-5-28-21(36)18(35)25(2,3)9-48-54(45,46)51-53(43,44)47-8-13-17(50-52(40,41)42)16(34)22(49-13)32-11-31-15-19(26)29-10-30-20(15)32/h10-13,16-18,22,34-35H,4-9H2,1-3H3,(H,27,33)(H,28,36)(H,37,38)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t12-,13?,16+,17+,18-,22?/m0/s1MZFOKIKEPGUZEN-JDVCRUKVSA-N(2S)-3-[(2-{3-[(2R)-3-[({[({[(3S,4R)-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]-2-methyl-3-oxopropanoic acid867.607867.131252359-2.3910(2S)-3-[(2-{3-[(2R)-3-{[({[(3S,4R)-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]-2-methyl-3-oxopropanoic acid0-5FDB022959(s)-methylmalonyl-coa;(s)-methylmalonyl-coenzyme aPW_C001560S-MmCoA228642695378623133790321121215784071222514061241351191248041201264144791267304811279795011283222061523R-Methylmalonyl-CoAHMDB0002255R-Methylmalonyl-CoA belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain. R-Methylmalonyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). R-Methylmalonyl-CoA has been primarily detected in urine. Within the cell, R-methylmalonyl-CoA is primarily located in the cytoplasm. In humans, R-methylmalonyl-CoA is involved in the threonine and 2-oxobutanoate degradation pathway, the valine, leucine and isoleucine degradation pathway, and the propanoate metabolism pathway. R-Methylmalonyl-CoA is also involved in several metabolic disorders, some of which include the maple syrup urine disease pathway, the 3-methylglutaconic aciduria type IV pathway, 3-methylcrotonyl CoA carboxylase deficiency type I, and 3-hydroxyisobutyric acid dehydrogenase deficiency. Methylmalonyl-CoA is an intermediate in the metabolism of Propanoate. It is a substrate for Malonyl-CoA decarboxylase (mitochondrial), Methylmalonyl-CoA mutase (mitochondrial) and Methylmalonyl-CoA epimerase (mitochondrial).73173-92-9 C01213 22833590METHYL-MALONYL-COA17216177C[C@H](C(O)=O)C(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12C25H40N7O19P3SInChI=1S/C25H40N7O19P3S/c1-12(23(37)38)24(39)55-7-6-27-14(33)4-5-28-21(36)18(35)25(2,3)9-48-54(45,46)51-53(43,44)47-8-13-17(50-52(40,41)42)16(34)22(49-13)32-11-31-15-19(26)29-10-30-20(15)32/h10-13,16-18,22,34-35H,4-9H2,1-3H3,(H,27,33)(H,28,36)(H,37,38)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t12-,13?,16?,17?,18+,22?/m1/s1MZFOKIKEPGUZEN-YLYUOEEYSA-N(2R)-3-[(2-{3-[(2R)-3-[({[({[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]-2-methyl-3-oxopropanoic acid867.607867.131252359-2.3910(2R)-3-[(2-{3-[(2R)-3-{[({[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]-2-methyl-3-oxopropanoic acid0-5FDB022930PW_C001523R-MmCoA229042697243873786241337903513279204112121580407122252406122578124124138119124805120125150118126415479126733299127980501128326388704L-ValineHMDB0000883Valine (abbreviated as Val or V) is an -amino acid with the chemical formula HO2CCH(NH2)CH(CH3)2. It is named after the plant valerian. L-Valine is one of 20 proteinogenic amino acids. Its codons are GUU, GUC, GUA, and GUG. This essential amino acid is classified as nonpolar. Along with leucine and isoleucine, valine is a branched-chain amino acid. Branched chain amino acids (BCAA) are essential amino acids whose carbon structure is marked by a branch point. These three amino acids are critical to human life and are particularly involved in stress, energy and muscle metabolism. BCAA supplementation as therapy, both oral and intravenous, in human health and disease holds great promise. "BCAA" denotes valine, isoleucine and leucine which are branched chain essential amino acids. Despite their structural similarities, the branched amino acids have different metabolic routes, with valine going solely to carbohydrates, leucine solely to fats and isoleucine to both. The different metabolism accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg and 16 mg/kg of valine, leucine and isoleucine respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. Many types of inborn errors of BCAA metabolism exist, and are marked by various abnormalities. The most common form is the maple syrup urine disease, marked by a characteristic urinary odor. Other abnormalities are associated with a wide range of symptoms, such as mental retardation, ataxia, hypoglycemia, spinal muscle atrophy, rash, vomiting and excessive muscle movement. Most forms of BCAA metabolism errors are corrected by dietary restriction of BCAA and at least one form is correctable by supplementation with 10 mg of biotin daily. BCAA are decreased in patients with liver disease, such as hepatitis, hepatic coma, cirrhosis, extrahepatic biliary atresia or portacaval shunt; aromatic amino acids (AAA)tyrosine, tryptophan and phenylalanine, as well as methionineare increased in these conditions. Valine in particular, has been established as a useful supplemental therapy to the ailing liver. All the BCAA probably compete with AAA for absorption into the brain. Supplemental BCAA with vitamin B6 and zinc help normalize the BCAA:AAA ratio. (http://www.dcnutrition.com). In sickle-cell disease, valine substitutes for the hydrophilic amino acid glutamic acid in hemoglobin. Because valine is hydrophobic, the hemoglobin does not fold correctly. Valine is an essential amino acid, hence it must be ingested, usually as a component of proteins.72-18-4C00183628716414VAL6050DB00161CC(C)[C@H](N)C(O)=OC5H11NO2InChI=1S/C5H11NO2/c1-3(2)4(6)5(7)8/h3-4H,6H2,1-2H3,(H,7,8)/t4-/m0/s1KZSNJWFQEVHDMF-BYPYZUCNSA-N(2S)-2-amino-3-methylbutanoic acid117.1463117.0789786010.262L-valine00FDB000465(2s)-2-amino-3-methylbutanoate;(2s)-2-amino-3-methylbutanoic acid;(s)-2-amino-3-methylbutanoate;(s)-2-amino-3-methylbutanoic acid;(s)-2-amino-3-methylbutyrate;(s)-2-amino-3-methylbutyric acid;(s)-2-amino-3-methyl-butanoate;(s)-2-amino-3-methyl-butanoic acid;(s)-valine;(s)-a-amino-b-methylbutyrate;(s)-a-amino-b-methylbutyric acid;(s)-alpha-amino-beta-methylbutyrate;(s)-alpha-amino-beta-methylbutyric acid;2-amino-3-methylbutanoate;2-amino-3-methylbutanoic acid;2-amino-3-methylbutyrate;2-amino-3-methylbutyric acid;L-(+)-a-aminoisovalerate;L-(+)-a-aminoisovaleric acid;L-(+)-alpha-aminoisovalerate;L-(+)-alpha-aminoisovaleric acid;L-valine;L-a-amino-b-methylbutyrate;L-a-amino-b-methylbutyric acid;L-alpha-amino-beta-methylbutyrate;L-alpha-amino-beta-methylbutyric acid;Valine;L-valin;V;ValPW_C000704Val165182313456531075654108714418790692249070151907122542258310425413154256031878625133791781111215401221222544061240981351248071201264164791279825011500(S)-Methylmalonic acid semialdehydeHMDB0002217Methylmalonic semialdehyde is a metabolite in valine catabolism, inositol metabolism and propanoate metabolism. Methylmalonate-semialdehyde dehydrogenase (MMSDH) catalyses the NAD+ and coenzyme A-dependent conversion of methylmalonate semialdehyde to propionyl-CoA in the distal region of the L-valine catabolic pathway. MMSDH is located within the mitochondria; direct enzymatic assay of MMSDH is difficult since the substrate, methylmalonate semialdehyde, is both commercially unavailable and notoriously unstable as a b-keto acid. (PMID: 10947204).99043-16-0C060025462303278214575365C[C@@H](C=O)C(O)=OC4H6O3InChI=1S/C4H6O3/c1-3(2-5)4(6)7/h2-3H,1H3,(H,6,7)/t3-/m0/s1VOKUMXABRRXHAR-VKHMYHEASA-N(2S)-2-methyl-3-oxopropanoic acid102.0886102.0316940580.421(S)-methylmalonaldehydic acid0-1FDB022912(2s)-2-methyl-3-oxopropanoate;(2s)-2-methyl-3-oxopropanoic acid;(s)-methylmalonate semialdehyde;(s)-methylmalonic acid semialdehydePW_C00150022M3O167532314490872247862613379195112121570407122255406124127119124808120126417479127983501130Methylmalonic acidHMDB0000202Methylmalonic acid is a malonic acid derivative, which is a vital intermediate in the metabolism of fat and protein. In particular, the coenzyme A-linked form of methylmalonic acid, methylmalonyl-CoA, is converted into succinyl-CoA by methylmalonyl-CoA mutase in a reaction that requires vitamin B12 as a cofactor. In this way, methylmalonic acid enters the Krebs cycle and is thus part of one of the anaplerotic reactions. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This inborn error of metabolism is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA. Methylmalonic acid is also found to be associated with other inborn errors of metabolism, including cobalamin deficiency, cobalamin malabsorption, malonyl-CoA decarboxylase deficiency, and transcobalamin II deficiency. When present in sufficiently high levels, methylmalonic 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. Chronically high levels of methylmalonic acid are associated with at least 5 inborn errors of metabolism, including Malonyl CoA decarboxylase deficiency, Malonic Aciduria, Methylmalonate Semialdehyde Dehydrogenase Deficiency, Methylmalonic Aciduria and Methylmalonic Aciduria Due to Cobalamin-Related Disorders. Methylmalonic acid is an organic acid and abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, kidney abnormalities, liver damage, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.516-05-2C0217048730860473DB04183CC(C(O)=O)C(O)=OC4H6O4InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8)ZIYVHBGGAOATLY-UHFFFAOYSA-N2-methylpropanedioic acid118.088118.026608680.102methylmalonic acid0-2FDB0219051,1-ethanedicarboxylate;1,1-ethanedicarboxylic acid;2-methylmalonate;2-methylmalonic acid;Isosuccinate;Isosuccinic acid;Methyl-malonate;Methyl-malonic acid;Methyl-propanedioate;Methyl-propanedioic acid;Methylmalonate;Methylmalonic acid;Methylpropanedioate;Methylpropanedioic acid;Alpha-methylmalonic acid;2-methylpropanedioate;A-methylmalonate;A-methylmalonic acid;Alpha-methylmalonate;α-methylmalonate;α-methylmalonic acidPW_C000130Isosca16838231644565184567245691578627133792051117922913079231132792321148267780121287125121289430121584122122256406123858136123860465124142135124809120126418479127984501808Succinyl-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-CoA233410553366925378103603915560971616485178701516073611637474222771401337810111278576132800213681199784061207694071220141241227631201233651191245681181253584791261642991263064811269015011278682061401AdenosylcobalaminHMDB0002086Adenosylcobalamin is one of two metabolically active forms synthesized upon ingestion of vitamin B12 and is the predominant form in the liver; it acts as a coenzyme in the reaction catalyzed by methylmalonyl-CoA mutase. A cobalamin (cbl) derivative in which the substituent is deoxyadenosyl. It is one of two metabolically active forms synthesized upon ingestion of vitamin B12 and is the predominant form in the liver; it acts as a coenzyme in the reaction catalyzed by methylmalonyl-CoA mutase (MCM; E.C. 5.4.99.2). Inborn errors of vitamin B12 metabolism are autosomal recessive disorders and have been classified into nine distinct complementation classes. Disorders affecting adenosylcobalamin cause methylmalonic acidemia and metabolic acidosis. Methylmalonyl-CoA mutase catalyzes the conversion of L-methylmalonyl-CoA to succinyl-CoA and uses adenosylcobalamin (AdoCbl) as a cofactor. Cbl must be transported into mitochondria, reduced and adenosylated before it can be utilized by MCM. (PMID: 17011224).13870-90-1C0019418408ADENOSYLCOBALAMIN-5-P30791458[C@H]1(C[Co-3]2345[N+]6=C7C(C)=C8N2[C@]([H])([C@H](CC(=O)N)[C@]8(CCC(=O)NC[C@@H](C)OP([O-])(=O)O[C@@H]2[C@@H](CO)O[C@H](N8C=[N+]3C3=CC(C)=C(C)C=C83)[C@@H]2O)C)[C@@]2(C)[C@@](CC(=O)N)([C@H](CCC(=O)N)C(C(C)=C3[N+]4=C(C=C6C([C@@H]7CCC(=O)N)(C)C)[C@@H](CCC(=O)N)[C@@]3(CC(=O)N)C)=[N+]52)C)O[C@@H](N2C3=NC=NC(=C3N=C2)N)[C@H](O)[C@@H]1OC72H100CoN18O17PInChI=1S/C62H90N13O14P.C10H12N5O3.Co/c1-29-20-39-40(21-30(29)2)75(28-70-39)57-52(84)53(41(27-76)87-57)89-90(85,86)88-31(3)26-69-49(83)18-19-59(8)37(22-46(66)80)56-62(11)61(10,25-48(68)82)36(14-17-45(65)79)51(74-62)33(5)55-60(9,24-47(67)81)34(12-15-43(63)77)38(71-55)23-42-58(6,7)35(13-16-44(64)78)50(72-42)32(4)54(59)73-56;1-4-6(16)7(17)10(18-4)15-3-14-5-8(11)12-2-13-9(5)15;/h20-21,23,28,31,34-37,41,52-53,56-57,76,84H,12-19,22,24-27H2,1-11H3,(H15,63,64,65,66,67,68,69,71,72,73,74,77,78,79,80,81,82,83,85,86);2-4,6-7,10,16-17H,1H2,(H2,11,12,13);/q;;+2/p-2/t31-,34-,35-,36-,37+,41-,52-,53-,56-,57+,59-,60+,61+,62+;4-,6-,7-,10-;/m11./s1ZIHHMGTYZOSFRC-OUCXYWSSSA-L(10S,12R,13S,17R,23R,24R,25R,30S,35S,36S,40S,41S,42R,46R)-1-{[(2S,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl}-30,35,40-tris(2-carbamoylethyl)-24,36,41-tris(carbamoylmethyl)-46-hydroxy-12-(hydroxymethyl)-5,6,17,23,28,31,31,36,38,41,42-undecamethyl-15,20-dioxo-11,14,16-trioxa-2lambda5,9,19,26,43lambda5,44lambda5,45lambda5-heptaaza-15lambda5-phospha-1-cobaltadodecacyclo[27.14.1.1^{1,34}.1^{2,9}.1^{10,13}.0^{1,26}.0^{3,8}.0^{23,27}.0^{25,42}.0^{32,44}.0^{39,43}.0^{37,45}]heptatetraconta-2(47),3,5,7,27,29(44),32,34(45),37,39(43)-decaene-2,43,44,45-tetrakis(ylium)-1,1,1-triuid-15-olate1579.58181578.6583455712(10S,12R,13S,17R,23R,24R,25R,30S,35S,36S,40S,41S,42R,46R)-1-{[(2S,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl}-30,35,40-tris(2-carbamoylethyl)-24,36,41-tris(carbamoylmethyl)-46-hydroxy-12-(hydroxymethyl)-5,6,17,23,28,31,31,36,38,41,42-undecamethyl-15,20-dioxo-11,14,16-trioxa-2lambda5,9,19,26,43lambda5,44lambda5,45lambda5-heptaaza-15lambda5-phospha-1-cobaltadodecacyclo[27.14.1.1^{1,34}.1^{2,9}.1^{10,13}.0^{1,26}.0^{3,8}.0^{23,27}.0^{25,42}.0^{32,44}.0^{39,43}.0^{37,45}]heptatetraconta-2(47),3,5,7,27,29(44),32,34(45),37,39(43)-decaene-2,43,44,45-tetrakis(ylium)-1,1,1-triuid-15-olate00FDB022837(5'-deoxy-5'-adenosyl)cobamide coenzyme;5'-deoxy-5'-adenosyl vitamin b12;5'-deoxy-5'-adenosylcobalamin;Adenosylcobalamin 5'-phosphate;Calomide;Cobalamin coenzyme;Cobamamide;Cobamamide 5'-phosphate;Cobamide coenzyme;Coenzyme b12;Deoxyadenosylcobalamin;Dibencozide;Funacomide;Vitamin b12 coenzyme;Vitamin b12 coenzymes;Adenosylcob(iii)alamin;5,6-dimethylbenzimidazolyl-5-deoxyadenosyl-cobamide;(5,6-dimethylbenzimidazolyl)cobamide coenzyme;Alpha-(5,6-dimethylbenzimidazolyl)cobamide coenzyme;5'-deoxy-5'-adenosyl-5,6-dimethylbenzimidazolylcobamide;5,6-dimethylbenzimidazolyl-co-5'-deoxy-5'-adenosylcobamide;Dmbc coenzyme;A-(5,6-dimethylbenzimidazolyl)cobamide coenzyme;α-(5,6-dimethylbenzimidazolyl)cobamide coenzymePW_C001401Adnscbn2401378628112121582407124140119126419481127985206911Malonyl-CoAHMDB0001175Malonyl-CoA, also known as malonyl CoA or CoA, malonyl, belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain. Malonyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). Malonyl-CoA has been found throughout most human tissues, and has also been primarily detected in urine. Within the cell, malonyl-CoA is primarily located in the mitochondria, peroxisome and cytoplasm. In humans, malonyl-CoA is involved in fatty acid biosynthesis pathway, the pyruvate metabolism pathway, and the propanoate metabolism pathway. Malonyl-CoA is also involved in several metabolic disorders, some of which include the primary hyperoxaluria II, PH2 pathway, pyruvate decarboxylase E1 component deficiency (pdhe1 deficiency), malonyl-CoA decarboxylase deficiency, and pyruvate dehydrogenase complex deficiency. Malonyl-CoA is a coenzyme A derivative which plays a key role in the fatty acid synthesis in the cytoplasmic and microsomal systems.524-14-1C000831066315531MALONYL-COA10213DB04524CC(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)CC(O)=OC24H38N7O19P3SInChI=1S/C24H38N7O19P3S/c1-24(2,19(37)22(38)27-4-3-13(32)26-5-6-54-15(35)7-14(33)34)9-47-53(44,45)50-52(42,43)46-8-12-18(49-51(39,40)41)17(36)23(48-12)31-11-30-16-20(25)28-10-29-21(16)31/h10-12,17-19,23,36-37H,3-9H2,1-2H3,(H,26,32)(H,27,38)(H,33,34)(H,42,43)(H,44,45)(H2,25,28,29)(H2,39,40,41)/t12-,17-,18-,19?,23-/m1/s1LTYOQGRJFJAKNA-VFLPNFFSSA-N3-{[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}-3-oxopropanoic acid853.58853.115602295-2.18103-({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)-3-oxopropanoic acid0-5FDB001606Malonyl coa;Malonyl coenzyme a;Malonyl-coa;Malonyl-coenzyme a;Omega-carboxyacyl-coa;Omega-carboxyacyl-coenzyme a;S-(hydrogen malonyl)coenzyme a;S-(hydrogen propanedioate;S-(hydrogen propanedioate) coa;S-(hydrogen propanedioate) coenzyme a;S-(hydrogen propanedioic acidPW_C000911MalyCoA178882268442632692416012138151778301327862913378777111121454122122257406122454124124012135124810120125021118126082297126420479126606299127538205127987501128180388940Acetyl-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-CoA21343858842324162244652896173340114840145278103547612457331086025155607716163861647017869231607106163729119874602228245151827721012582226130122994261531577121133772911117756211277706132779941157835513478433334800073688063411980663376901241701199534061201454051203041221206324071224174081226263841227431201229591351231371181249863741252001211253434791255074781256332971265644821265724811267784801268865011270442091273942051276653881281375021281452061283743911407621851316Carbon dioxideHMDB0001967Carbon dioxide is a colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. Carbon dioxide is produced during respiration by all animals, fungi and microorganisms that depend on living and decaying plants for food, either directly or indirectly. It is, therefore, a major component of the carbon cycle. Additionally, carbon dioxide is used by plants during photosynthesis to make sugars which may either be consumed again in respiration or used as the raw material to produce polysaccharides such as starch and cellulose, proteins and the wide variety of other organic compounds required for plant growth and development. When inhaled at concentrations much higher than usual atmospheric levels, it can produce a sour taste in the mouth and a stinging sensation in the nose and throat. These effects result from the gas dissolving in the mucous membranes and saliva, forming a weak solution of carbonic acid. Carbon dioxide is used by the food industry, the oil industry, and the chemical industry. Carbon dioxide is used to produce carbonated soft drinks and soda water. Traditionally, the carbonation in beer and sparkling wine comes about through natural fermentation, but some manufacturers carbonate these drinks artificially.124-38-9C0001128016526274O=C=OCO2InChI=1S/CO2/c2-1-3CURLTUGMZLYLDI-UHFFFAOYSA-Nmethanedione44.009543.9898292440.630carbon dioxide00DBMET00423FDB014084Carbon oxide;Carbon-12 dioxide;Carbonic acid anhydride;Carbonic acid gas;Carbonic anhydride;[co2];Co2;E 290;E-290;E290;R-744PW_C001316CO25081211204448013503186403677316952080651133431638491745225511731447052831035320111575010857711015968100602615560781616471178663710769221907017160703516370611887163205730819873332137461222753021082152258223151915824911849277119081701246422612688290426263154352331876994293771221337717013277470333777391127775012977763341780771347840535678427334789413317922713080008368806751198071713594836384113291391115549121119954406120089122120155407120364412120556414120833419120922124120991408121284125121505383122744120123011446123190450123418455123489118123556374123855136124063398125344479125460297125516481125824490125870299125931482126280480126887501127052206127277507127331388127390502140798185414Adenosine 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_C000414ATP92214608266164142247813733327995934399763210518211210214649215614216058240559243427272646281229302966316372361661361751439923447431476891486454503289503526515575205975215100525010452911015313111534611253901035406117543011854431205542129555613255691335603135562110858461435854146587610758971475924151604815561091616230166649317868391886870160697619971572057184206720921072252137229211729819873022167390217740821874321637481222749919081862251184727711903170120102811203916412178285125782261269129013264223153273084232631542621322426943187702825377218134772333297746833377632336780373327804135078168128782143517824035378411335784941157885013078865331789193348002836880046184806741198562919482612411323494113282388116280109119914122119992406120154407120245382120362412121246429121392123121397433121471408121974410122065125122079383122083405122402422122444435122919399123009446123816464123951447123956468124029374124527444124616136124630398124634376124943472124972375125011470125304297125371479125392299125515481125595484126123485126220300126234495126240478126547491126596499126913501127123389127731516127781395127796390127801209128119508128167517140770891463Hydrogen carbonateHMDB0000595Bicarbonate, or hydrogen carbonate, is a simple single carbon molecule that plays surprisingly important roles in diverse biological processes. Among these are photosynthesis, the Krebs cycle, whole-body and cellular pH regulation, and volume regulation. Since bicarbonate is charged it is not permeable to lipid bilayers. Mammalian membranes thus contain bicarbonate transport proteins to facilitate the specific transmembrane movement of HCO3(-). Bicarbonate ion is an anion that consists of one central carbon atom surrounded by three oxygen atoms in a trigonal planar arrangement, with a hydrogen atom attached to one of the oxygens. The bicarbonate ion carries a negative one formal charge and is the conjugate base of carbonic acid, H2CO3. The carbonate radical is an elusive and strong one-electron oxidant. Bicarbonate in equilibrium with carbon dioxide constitutes the main physiological buffer. The bicarbonate-carbon dioxide pair stimulates the oxidation, peroxidation and nitration of several biological targets. The demonstration that the carbonate radical existed as an independent species in aqueous solutions at physiological pH and temperature renewed the interest in the pathophysiological roles of this radical and related species. The carbonate radical has been proposed to be a key mediator of the oxidative damage resulting from peroxynitrite production, xanthine oxidase turnover and superoxide dismutase1 peroxidase activity. The carbonate radical has also been proposed to be responsible for the stimulatory effects of the bicarbonate-carbon dioxide pair on oxidations mediated by hydrogen peroxide/transition metal ions. The ultimate precursor of the carbonate radical anion being bicarbonate, carbon dioxide, peroxymonocarbonate or complexes of transition metal ions with bicarbonate-derived species remains a matter of debate. The carbonate radical mediates some of the pathogenic effects of peroxynitrite. The carbonate radical as the oxidant produced from superoxide dismutase (EC 1.15.1.1, SOD1) peroxidase activity. Peroxymonocarbonate is a biological oxidant, whose existence is in equilibrium with hydrogen peroxide and bicarbonate. (PMID: 17505962, 17215880).71-52-3C0028876917544HCO3749OC(O)=OCH2O3InChI=1S/CH2O3/c2-1(3)4/h(H2,2,3,4)BVKZGUZCCUSVTD-UHFFFAOYSA-Ncarbonic acid62.024862.000393930.572carbonic acid0-1FDB022134Bicarbonate;Bicarbonate (hco3-);Bicarbonate anion;Bicarbonate ion;Bicarbonate ion (hco31-);Bicarbonate ions;Carbonate;Carbonate (hco31-);Carbonate ion (hco31-);Carbonic acid;Hydrocarbonate(1-);Hydrogen carbonate;Hydrogen carbonate (hco3-);Hydrogen carbonate anion;Hydrogen carbonate ion;Hydrogen carbonate ion (hco3-);Hydrogencarbonate;Hydrogentrioxocarbonate;Monohydrogen carbonate;[co2(oh)](-);Acid carbonate;Hco3(-);Hydrogen carbonic acid;Acid carbonic acid;Bicarbonic acid;Bicarbonic acid ionPW_C000463HCO322416878239332397226131531457053911035445120557113360491556110161649417874822229092224779591127863013278762111800293681199934061212094071214361221215571241237791191239941351241151181253724791260592971263602991265414811269145011275112051279223881281142061441Phosphoric acidHMDB0002142Phosphoric acid, also known as orthophosphoric acid or phosphoric(V) acid, is a mineral acid with the chemical formula H3PO4. Alternatively, orthophosphoric acid molecules can combine with themselves to form a variety of compounds referred to as phosphoric acids in a more general way. For a discussion of these, see Phosphoric acids and Phosphates. Appears to exist only as a food additive and produced synthetically. --Wikipedia.7664-38-2C00009100426078979OP(O)(O)=OH3O4PInChI=1S/H3O4P/c1-5(2,3)4/h(H3,1,2,3,4)NBIIXXVUZAFLBC-UHFFFAOYSA-Nphosphoric acid97.995297.9768950963phosphoric acid0-2FDB013380Acide phosphorique (french);Acido fosforico [italian];Acidum phosphoricum;Diphosphate tetrasodium;Fosforzuuroplossingen [dutch];Marphos;Nfb;Ortho- phosphoric acid;Orthophosphoric acid;Phosphoric acid (acd/name 4.0);Phosphorsaeure;Phosphorsaeureloesungen [german];Sodium pyrophosphate;Sodium pyrophosphate decahydrate;Sodium pyrophosphate decahydrate biochemica;Sonac;Tetra-sodium pyrophosphate;Tetrasodium pyrophosphate decahydrate;Tetrasodium pyrophosphate 10-hydrate;White phosphoric acid;[po(oh)3];Acide phosphorique;H3po4;Phosphate;Phosphorsaeureloesungen;OrthophosphatePW_C001441PhosfrA1718440448313159243167821902491910548563591314759431516891160117821981182821311829216118522771263831771051327733013377473333777791117800011278272356787063317870733480671135806771191199181221201261241201574071203444061203674121213834191219003831219024081228581181229941201230144461239424551244533981244553741253082971254932991255184811256644791260564901268552051270303881270532061271595011275085071034Adenosine diphosphateHMDB0001341Adenosine diphosphate, abbreviated ADP, is a nucleotide. It is an ester of pyrophosphoric acid with the nucleotide adenine. ADP consists of the pyrophosphate group, the pentose sugar ribose, and the nucleobase adenine. ADP is the product of ATP dephosphorylation by ATPases. ADP is converted back to ATP by ATP synthases.58-64-0C00008602216761ADP5800NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1OC10H15N5O10P2InChI=1S/C10H15N5O10P2/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(24-10)1-23-27(21,22)25-26(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1XTWYTFMLZFPYCI-KQYNXXCUSA-N[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]phosphonic acid427.2011427.029414749-2.126adenosine-diphosphate0-2FDB021817Adp;Adenosindiphosphorsaeure;Adenosine 5'-pyrophosphate;Adenosine diphosphate;Adenosine pyrophosphate;Adenosine-5'-diphosphate;Adenosine-5-diphosphate;Adenosine-diphosphate;5'-adenylphosphoric acid;Adenosine 5'-diphosphate;H3adp;5'-adenylphosphate;Adenosine 5'-diphosphoric acid;Adenosine-5'-diphosphoric acidPW_C001034ADP23413484152248213801596315978310611415182190149210418211310216158240859243527272847273646285529316572363561440023447631477091503626515775208975217100531511153491125392103544612055441295572133562410857411175764101584914358561465878107589914759261516050155611116162311666495178670094684118868721607159205718720672082107226213723121173001987303216739121774102187433163748322281872251185127711905170120132811218028513262223153293084232831542398313426223224269631877029253770871327721613477306329774723337766333678039332780433507817012878215351782443537841433578495115787053317884913078920334800303688062211880651135806761199482712411328338811620410911994412211999440612015640712031838212036641212124842912139412312139943312147240812189938312197641012206412512208540512240542212244543512297339912301344612381846412395344712395846812403037412445239812452944412461513612463637612494747212497537512501247012533429712537347912549229912551748112564548412612548512621930012623549512624247812655049112659749912691550112773351612778039512779739012780320912812250812816851712831338920BiotinHMDB0000030Biotin is an enzyme co-factor present in minute amounts in every living cell. Biotin is also known as vitamin H or B7 or coenzyme R. It occurs mainly bound to proteins or polypeptides and is abundant in liver, kidney, pancreas, yeast, and milk. Biotin has been recognized as an essential nutrient. Our biotin requirement is fulfilled in part through diet, through endogenous reutilization of biotin and perhaps through capture of biotin generated in the intestinal flora. The utilization of biotin for covalent attachment to carboxylases and its reutilization through the release of carboxylase biotin after proteolytic degradation constitutes the 'biotin cycle'. Biotin deficiency is associated with neurological manifestations, skin rash, hair loss and metabolic disturbances that are thought to relate to the various carboxylase deficiencies (metabolic ketoacidosis with lactic acidosis). It has also been suggested that biotin deficiency is associated with protein malnutrition, and that marginal biotin deficiency in pregnant women may be teratogenic. Biotin acts as a carboxyl carrier in carboxylation reactions. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lysine residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lys residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. Evidence is emerging that biotin participates in processes other than classical carboxylation reactions. Specifically, novel roles for biotin in cell signaling, gene expression, and chromatin structure have been identified in recent years. Human cells accumulate biotin by using both the sodium-dependent multivitamin transporter and monocarboxylate transporter 1. These transporters and other biotin-binding proteins partition biotin to compartments involved in biotin signaling: cytoplasm, mitochondria, and nuclei. The activity of cell signals such as biotinyl-AMP, Sp1 and Sp3, nuclear factor (NF)-kappaB, and receptor tyrosine kinases depends on biotin supply. Consistent with a role for biotin and its catabolites in modulating these cell signals, greater than 2000 biotin-dependent genes have been identified in various human tissues. Many biotin-dependent gene products play roles in signal transduction and localize to the cell nucleus, consistent with a role for biotin in cell signaling. Posttranscriptional events related to ribosomal activity and protein folding may further contribute to effects of biotin on gene expression. Finally, research has shown that biotinidase and holocarboxylase synthetase mediate covalent binding of biotin to histones (DNA-binding proteins), affecting chromatin structure; at least seven biotinylation sites have been identified in human histones. Biotinylation of histones appears to play a role in cell proliferation, gene silencing, and the cellular response to DNA repair. Roles for biotin in cell signaling and chromatin structure are consistent with the notion that biotin has a unique significance in cell biology. (PMID: 15992684, 16011464).58-85-5C0012017154815956BIOTIN149962DB00121[H][C@]12CS[C@@H](CCCCC(O)=O)[C@@]1([H])NC(=O)N2C10H16N2O3SInChI=1S/C10H16N2O3S/c13-8(14)4-2-1-3-7-9-6(5-16-7)11-10(15)12-9/h6-7,9H,1-5H2,(H,13,14)(H2,11,12,15)/t6-,7-,9-/m0/s1YBJHBAHKTGYVGT-ZKWXMUAHSA-N5-[(3aS,4S,6aR)-2-oxo-hexahydro-1H-thieno[3,4-d]imidazol-4-yl]pentanoic acid244.311244.088163078-2.3035-[(3aS,4S,6aR)-2-oxo-hexahydrothieno[3,4-d]imidazol-4-yl]pentanoic acid0-1FDB014510(+)-biotin;(+)-cis-hexahydro-2-oxo-1h-thieno[3,4]imidazole-4-valerate;(+)-cis-hexahydro-2-oxo-1h-thieno[3,4]imidazole-4-valeric acid;(3as,4s,6ar)-hexahydro-2-oxo-1h-thieno[3,4-d]imidazole-4-valerate;(3as,4s,6ar)-hexahydro-2-oxo-1h-thieno[3,4-d]imidazole-4-valeric acid;-(+)-biotin;1swk;1swn;1swr;5-(2-oxohexahydro-1h-thieno[3,4-d]imidazol-4-yl)pentanoate;5-(2-oxohexahydro-1h-thieno[3,4-d]imidazol-4-yl)pentanoic acid;Biodermatin;Bioepiderm;Bios ii;Bios h;Biotin;Coenzyme r;D(+)-biotin;D-(+)-biotin;D-biotin;D-biotin factor s;Factor s;Factor s (vitamin);Hexahydro-2-oxo-1h-thieno(3,4-d)imidazole-4-pentanoate;Hexahydro-2-oxo-1h-thieno(3,4-d)imidazole-4-pentanoic acid;Hexahydro-2-oxo-[3as-(3aa,4b,6aa)]-1h-thieno[3,4-d]imidazole-4-pentanoate;Hexahydro-2-oxo-[3as-(3aa,4b,6aa)]-1h-thieno[3,4-d]imidazole-4-pentanoic acid;Hexahydro-2-oxo-[3as-(3alpha,4beta,6alpha)]-1h-thieno[3,4-d]imidazole-4-pentanoate;Hexahydro-2-oxo-[3as-(3alpha,4beta,6alpha)]-1h-thieno[3,4-d]imidazole-4-pentanoic acid;Lutavit h2;Meribin;Rovimix h 2;Vitamin b7;Vitamin h;Vitamin-h;Cis-(+)-tetrahydro-2-oxothieno[3,4]imidazoline-4-valerate;Cis-(+)-tetrahydro-2-oxothieno[3,4]imidazoline-4-valeric acid;Cis-hexahydro-2-oxo-1h-thieno(3,4)imidazole-4-valeric acid;Cis-tetrahydro-2-oxothieno(3,4-d)imidazoline-4-valeric acid;Delta-(+)-biotin;Delta-biotin;Delta-biotin factor s;Biotina;Biotine;BiotinumPW_C000020Biotin2641358579151699322702529210152981055393103544912055461115551114557513360511556112161649617869251607484222778311327796011280031368806531351199954061201341221205034091212104071215591241231091371237801191241171181253744791255012971257184831264212991265424811269165011270382051279893881281152061027ManganeseHMDB0001333Manganese is an essential trace nutrient in all forms of life. Physiologically, it. exists as an ion in the body. It is concentrated in cell mitochondria, mostly in the pituitary gland, liver, pancreas, kidney, and bone, influences the synthesis of mucopolysaccharides, stimulates hepatic synthesis of cholesterol and fatty acids, and is a cofactor in many enzymes, including arginase and alkaline phosphatase in the liver.16397-91-4C196102785429035MN%2b325916[Mn++]MnInChI=1S/Mn/q+2WAEMQWOKJMHJLA-UHFFFAOYSA-Nmanganese(2+) ion54.93854.9380496360manganese(2+) ion22FDB003636Manganese;Manganese (ii) ion;Manganese(ii);Manganese, ion (mn2+);Manganous ion;Mn(2+);Mn2+PW_C001027Mn2+27447381486491553432271223943251314539410354501205576133605215561131616497178692616074852221188019811939225119581641247124913360151152213067705029477494111778321327796111278267356784901157852433179247293800323681199964061204011221210581241212114071212953831213784191224884051230441351236221181237811191238653981239374551250543761253754791259764951260514901260602971261582991265434811266424781269175011274293901275035071275122051277653881281162061282182091142Acetoacetyl-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_C001142ActaCoA5924792810493527910370021617292198735716375981608242226830621015239151772581337822411278914111901261701202834061207634071214651221229481201233591191240231351256224791260854811271495011275412061407961851099Coenzyme 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_C001099CoA21143868845387922892172407592414224595281329286231334211335118461810462958484214486554487965232102524710452801035477124573410857771016023155607516163841646817869301606961162697319970831887108163729319873472107458222822915190812269090224912417092151951301329915318249254884942616315769072937711913377222134772303297729211177550132775553347756311277633336776721297799611578047332780563507841333578567130792593337997433180005368806201188062737480635119806653769382838293834383986742881105553891105613901158423991158473981199514061201474051202313841203051221206344071207621171214061231214214331215211251216664291216824081217144141224044221227411201229041211229601351239654471239794681240791361242204641242654501249743751253414791255094781255794801255924841256342971260844811265494911265604821267463001268845011270462091271093911273012051275402061276673881281215081281335021283403951407511861407631851407678916753Malonic semialdehydeHMDB0011111Malonic semialdehyde is formed in the alternative pathway of propionate metabolism and in the catabolism of beta-alanine. Studies done on these pathways in cultured cells from a patient with mitochondrial malonyl-CoA decarboxylase deficiency show that malonic semialdehyde is directly converted into acetyl-CoA in man. (PMID: 6418146).926-61-4C0022286817960845OC(=O)CC=OC3H4O3InChI=1S/C3H4O3/c4-2-1-3(5)6/h2H,1H2,(H,5,6)OAKURXIZZOAYBC-UHFFFAOYSA-N3-oxopropanoic acid88.062188.0160439940.4613-oxopropanoic acid0-1C002223-oxopropanoate;3-oxopropanoic acid;3-oxopropionic acid;Formyl acetate;Formyl acetic acid;Formylacetic acid;Malonaldehydic acid;Malonate semialdehyde;FormylacetatePW_C006753MalSah4335414126822897752411277591133120085407120456406122832119123090120125456481125700479126994206127192501721NADHMDB0000902NAD (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_C000721NAD140415033538651101114211344312735146654222949277917283529310794807184813184819284902649603151679552381035334111536011254691235482125559013556101185696100573810858271415912147594215160241556072157607616163851646917867721176890160701218870971637174205719720674051987459222824122683592259085224118192161232224913006298130183001325622342404322426193157710413277120133772091347737033177650336776673347770233277709130779151137798334778406356800063688069011993825124110552388112750166112853941199291221199524061201714071208344191209844081211594251212421261212594291218173831226143841227421201231304471231411361234194551235493741237314601238124431238294641243703981251871211253192971253424791255304811258062991258254901259244821265154951267654801268855011272785071273835021280893901283603911284283951407571851144NADHHMDB0001487NADH 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_C001144NADH1434153349086481011152127551469542230492781172836293109948061848121848212849046495931516995524010353321115358112546612354791255593135569810057371085829141591514759451516027155607916163871647217867711176893160701118870991637172205719520674622228244226836022590862241180919811821216123202491300329813015300132552234240332242618315771071327712313377208134773713317765133677668334777003327770713077917113779863478000936880691119938221241105493881128549411583811811995540612017240712037812212098640812116242512124412612169342912181838312261638412274512012312744712313813612355137412373446012381444312424246412437139812518912112534547912553148112576229712580829912592648212651649512676748012688850112738550212809039012836239112842939514075918540β-AlanineHMDB0000056beta-Alanine is the only naturally occurring beta-amino acid - an amino acid in which the amino group is at the beta-position from the carboxylate group. It is formed in vivo by the degradation of dihydrouracil and carnosine. It is a component of the naturally occurring peptides carnosine and anserine and also of pantothenic acid (vitamin B-5), which itself is a component of coenzyme A. Under normal conditions, beta-alanine is metabolized into acetic acid. beta-Alanine can undergo a transanimation reaction with pyruvate to form malonate-semialdehyde and L-alanine. The malonate semialdehyde can then be converted into malonate via malonate-semialdehyde dehydrogenase. Malonate is then converted into malonyl-CoA and enter fatty acid biosynthesis. Since neuronal uptake and neuronal receptor sensitivity to beta-alanine have been demonstrated, beta-alanine may act as a false transmitter replacing gamma-aminobutyric acid. When present in sufficiently high levels, beta-alanine can act as a neurotoxin, a mitochondrial toxin, and a metabotoxin. A neurotoxin is a compound that damages the brain or nerve tissue. A mitochondrial toxin is a compound that damages mitochondria and reduces cellular respiration as well as oxidative phosphorylation. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of beta-alanine are associated with at least three inborn errors of metabolism, including GABA-transaminase deficiency, hyper-beta-alaninemia, and methylmalonate semialdehyde dehydrogenase deficiency. beta-Alanine is a central nervous system (CNS) depressant and is an inhibitor of GABA transaminase. The associated inhibition of GABA transaminase and displacement of GABA from CNS binding sites can also lead to GABAuria (high levels of GABA in the urine) and convulsions. In addition to its neurotoxicity, beta-alanine reduces cellular levels of taurine, which are required for normal respiratory chain function. Cellular taurine depletion is known to reduce respiratory function and elevate mitochondrial superoxide generation, which damages mitochondria and increases oxidative stress (PMID: 27023909). Individuals suffering from mitochondrial defects or mitochondrial toxicity typically develop neurotoxicity, hypotonia, respiratory distress, and cardiac failure. beta-Alanine is a biomarker for the consumption of meat, especially red meat.107-95-9C0009923916958B-ALANINE234DB03107NCCC(O)=OC3H7NO2InChI=1S/C3H7NO2/c4-2-1-3(5)6/h1-2,4H2,(H,5,6)UCMIRNVEIXFBKS-UHFFFAOYSA-N3-aminopropanoic acid89.093289.0476784730.742β alanine00FDB0022532-carboxyethylamine;3-amino-propanoate;3-amino-propanoic acid;3-aminopropanoate;3-aminopropanoic acid;3-aminopropionate;3-aminopropionic acid;Abufene;B-alanine;Omega-aminopropionate;Omega-aminopropionic acid;B-aminopropanoate;B-aminopropanoic acid;B-aminopropionate;B-aminopropionic acid;Beta alanine;Beta-alanine;Beta-aminopropanoate;Beta-aminopropanoic acid;Beta-aminopropionate;Beta-aminopropionic acid;β-alaninePW_C000040b-Ala413498527152320427472579410870711881268028912687290426453157752211277527111775781147815913278633133120083407120439409120445122121056124122260406122830119123073137123079135123620118124813120125454481125683483125689297126389299126424479126992206127178208127184205127950388127992501134Oxoglutaric acidHMDB0000208Oxoglutaric acid, also known as alpha-ketoglutarate, alpha-ketoglutaric acid, AKG, or 2-oxoglutaric acid, is classified as a gamma-keto acid or a gamma-keto acid derivative. gamma-Keto acids are organic compounds containing an aldehyde substituted with a keto group on the C4 carbon atom. alpha-Ketoglutarate is considered to be soluble (in water) and acidic. alpha-Ketoglutarate is a key molecule in the TCA cycle, playing a fundamental role in determining the overall rate of this important metabolic process (PMID: 26759695). In the TCA cycle, AKG is decarboxylated to succinyl-CoA and carbon dioxide by AKG dehydrogenase, which functions as a key control point of the TCA cycle. Additionally, AKG can be generated from isocitrate by oxidative decarboxylation catalyzed by the enzyme known as isocitrate dehydrogenase (IDH). In addition to these routes of production, AKG can be produced from glutamate by oxidative deamination via glutamate dehydrogenase, and as a product of pyridoxal phosphate-dependent transamination reactions (mediated by branched-chain amino acid transaminases) in which glutamate is a common amino donor. AKG is a nitrogen scavenger and a source of glutamate and glutamine that stimulates protein synthesis and inhibits protein degradation in muscles. In particular, AKG can decrease protein catabolism and increase protein synthesis to enhance bone tissue formation in skeletal muscles (PMID: 26759695). Interestingly, enteric feeding of AKG supplements can significantly increase circulating plasma levels of hormones such as insulin, growth hormone, and insulin-like growth factor-1 (PMID: 26759695). It has recently been shown that AKG can extend the lifespan of adult C. elegans by inhibiting ATP synthase and TOR (PMID: 24828042). In combination with molecular oxygen, alpha-ketoglutarate is required for the hydroxylation of proline to hydroxyproline in the production of type I collagen. A recent study has shown that alpha-ketoglutarate promotes TH1 differentiation along with the depletion of glutamine thereby favouring Treg (regulatory T-cell) differentiation (PMID: 26420908). alpha-Ketoglutarate has been found to be associated with fumarase deficiency, 2-ketoglutarate dehydrogenase complex deficiency, and D-2-hydroxyglutaric aciduria, which are all inborn errors of metabolism (PMID: 8338207).328-50-7C0002651309152-KETOGLUTARATE50DB02926OC(=O)CCC(=O)C(O)=OC5H6O5InChI=1S/C5H6O5/c6-3(5(9)10)1-2-4(7)8/h1-2H2,(H,7,8)(H,9,10)KPGXRSRHYNQIFN-UHFFFAOYSA-N2-oxopentanedioic acid146.0981146.021523302-0.442oxoglutarate0-2FDB0033612-ketoglutarate;2-ketoglutaric acid;2-oxo-1,5-pentanedioate;2-oxo-1,5-pentanedioic acid;2-oxoglutarate;2-oxoglutaric acid;2-oxopentanedioate;2-oxopentanedioic acid;Oxoglutarate;Alpha-ketoglutaric acid;Oxoglutaric acid;A-ketoglutarate;A-ketoglutaric acid;Alpha-ketoglutarate;α-ketoglutarate;α-ketoglutaric acidPW_C000134AKG152423141414684991867331110842126351447501455261467545375103541411754381185564132600814760361556069157609216164821786530857471222751522475191518209225837422011863198126812897705425377135133774811117752311277746129779673457797034677976327779843477842533480018368806941351131629411997240612002212412008440712017412212055241412081441812098940812114642312115242412116042512275712012283111912318645012339945412355437412371845812372445912373246012535747912540029912545548112553329712580048912592948212690050112694038812699320612706620512725550612738850295L-Glutamic acidHMDB0000148Glutamic acid (Glu), also referred to as glutamate (the anion), is one of the 20 proteinogenic amino acids. It is not among the essential amino acids. Glutamate is a key molecule in cellular metabolism. In humans, dietary proteins are broken down by digestion into amino acids, which serves as metabolic fuel or other functional roles in the body. Glutamate is the most abundant fast excitatory neurotransmitter in the mammalian nervous system. At chemical synapses, glutamate is stored in vesicles. Nerve impulses trigger release of glutamate from the pre-synaptic cell. In the opposing post-synaptic cell, glutamate receptors, such as the NMDA receptor, bind glutamate and are activated. Because of its role in synaptic plasticity, it is believed that glutamic acid is involved in cognitive functions like learning and memory in the brain. Glutamate transporters are found in neuronal and glial membranes. They rapidly remove glutamate from the extracellular space. In brain injury or disease, they can work in reverse and excess glutamate can accumulate outside cells. This process causes calcium ions to enter cells via NMDA receptor channels, leading to neuronal damage and eventual cell death, and is called excitotoxicity. The mechanisms of cell death include: * Damage to mitochondria from excessively high intracellular Ca2+. * Glu/Ca2+-mediated promotion of transcription factors for pro-apoptotic genes, or downregulation of transcription factors for anti-apoptotic genes. Excitotoxicity due to glutamate occurs as part of the ischemic cascade and is associated with stroke and diseases like amyotrophic lateral sclerosis, lathyrism, and Alzheimer's disease. glutamic acid has been implicated in epileptic seizures. Microinjection of glutamic acid into neurons produces spontaneous depolarization around one second apart, and this firing pattern is similar to what is known as paroxysmal depolarizing shift in epileptic attacks. This change in the resting membrane potential at seizure foci could cause spontaneous opening of voltage activated calcium channels, leading to glutamic acid release and further depolarization. (http://en.wikipedia.org/wiki/Glutamic_acid).56-86-0C000253303216015GLT30572DB00142N[C@@H](CCC(O)=O)C(O)=OC5H9NO4InChI=1S/C5H9NO4/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H,7,8)(H,9,10)/t3-/m0/s1WHUUTDBJXJRKMK-VKHMYHEASA-N(2S)-2-aminopentanedioic acid147.1293147.053157781-0.263L-glutamic acid0-1FDB012535(2s)-2-aminopentanedioate;(2s)-2-aminopentanedioic acid;(s)-(+)-glutamate;(s)-(+)-glutamic acid;(s)-2-aminopentanedioate;(s)-2-aminopentanedioic acid;(s)-glutamate;(s)-glutamic acid;1-amino-propane-1,3-dicarboxylate;1-amino-propane-1,3-dicarboxylic acid;1-aminopropane-1,3-dicarboxylate;1-aminopropane-1,3-dicarboxylic acid;2-aminoglutarate;2-aminoglutaric acid;2-aminopentanedioate;2-aminopentanedioic acid;Aciglut;Aminoglutarate;Aminoglutaric acid;E;Glt;Glu;Glusate;Glut;Glutacid;Glutamicol;Glutamidex;Glutaminate;Glutaminic acid;Glutaminol;Glutaton;L-(+)-glutamate;L-(+)-glutamic acid;L-glu;L-glutamate;L-glutaminate;L-glutaminic acid;L-a-aminoglutarate;L-a-aminoglutaric acid;L-alpha-aminoglutarate;L-alpha-aminoglutaric acid;A-aminoglutarate;A-aminoglutaric acid;A-glutamate;A-glutamic acid;Alpha-aminoglutarate;Alpha-aminoglutaric acid;Alpha-glutamate;Alpha-glutamic acid;Acide glutamique;Acido glutamico;Acidum glutamicum;Glutamate;Glutamic acid;L-glutaminsaeurePW_C000095Glu16244365811911384164149699110542144850145626146254532311153441135415117543911855651325631107563210858591056006147607115761919465318568381876844188709272709371716520571822077514224751815182082258373220117921981185516112004222126213112683289126972904234831542349318428453207702025377332133775251127797134677977327779813477829134580649135120023124120040122120086407120347406120692126120816418121147423121153424121157425122833119122997120123299443123401454123719458123725459123729460125401299125418297125457481125667479125769301125802489126941388126995206127162501127257506140738841407395971148Pyridoxal 5'-phosphateHMDB0001491This is the active form of vitamin B6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (pyridoxamine). -- Pubchem; Pyridoxal-phosphate (PLP, pyridoxal-5'-phosphate) is a cofactor of many enzymatic reactions. It is the active form of vitamin B6 which comprises three natural organic compounds, pyridoxal, pyridoxamine and pyridoxine. -- Wikipedia.54-47-7C00018105118405PYRIDOXAL_PHOSPHATE1022DB00114CC1=NC=C(COP(O)(O)=O)C(C=O)=C1OC8H10NO6PInChI=1S/C8H10NO6P/c1-5-8(11)7(3-10)6(2-9-5)4-15-16(12,13)14/h2-3,11H,4H2,1H3,(H2,12,13,14)NGVDGCNFYWLIFO-UHFFFAOYSA-N[(4-formyl-5-hydroxy-6-methylpyridin-3-yl)methoxy]phosphonic acid247.1419247.024573569-1.643pyridoxal phosphate0-2FDB021820Apolon b6;Biosechs;Codecarboxylase;Coenzyme b6;Hairoxal;Hexermin-p;Hi-pyridoxin;Hiadelon;Himitan;Pal-p;Plp;Phosphopyridoxal;Phosphopyridoxal coenzyme;Pidopidon;Piodel;Pydoxal;Pyridoxal 5'-phosphate;Pyridoxal 5-phosphate;Pyridoxal p;Pyridoxal phosphate;Pyridoxal-p;Pyridoxyl phosphate;Pyromijin;Sechvitan;Vitahexin-p;Vitazechs;3-hydroxy-2-methyl-5-[(phosphonooxy)methyl]-4-pyridinecarboxaldehyde;3-hydroxy-5-(hydroxymethyl)-2-methylisonicotinaldehyde 5-phosphate;Phosphoric acid mono-(4-formyl-5-hydroxy-6-methyl-pyridin-3-ylmethyl) ester;Pyridoxal 5-monophosphoric acid ester;Pyridoxal 5'-(dihydrogen phosphate);Pyridoxal-5'-phosphate;Pyridoxal 5'-phosphoric acid;3-hydroxy-5-(hydroxymethyl)-2-methylisonicotinaldehyde 5-phosphoric acid;Phosphate mono-(4-formyl-5-hydroxy-6-methyl-pyridin-3-ylmethyl) ester;Pyridoxal 5-monophosphate ester;Pyridoxal 5'-(dihydrogen phosphoric acid);Pyridoxal 5-phosphoric acid;Pyridoxal phosphoric acid;Pyridoxal-5'-phosphoric acidPW_C001148Pyr-5'P182324453518122140119696201110421450501458262120102150495325111541611754211035441118545512055671325581133653385701816071672057216212722221311858161121751511262331126281812684289126892907701725377037225770412937705222477526112777643417797334677979327782923457885533278862331806961359863071199121221200241241200294061200874071208174181211494231211554241220691231220763831228341191234024541237214581237274591246204471246273981253022971254022991254074791254584811258034891262242981262314951269423881269475011269962061272585061277865131277933901558Acrylyl-CoAHMDB0002307Acrylyl-CoA, also known as acryloyl-CoA or CoA S-acrylate, belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain. Acrylyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). Acrylyl-CoA has been primarily detected in urine. Within the cell, acrylyl-CoA is primarily located in the cytoplasm. Acrylyl-CoA exists in all living organisms, ranging from bacteria to humans. In humans, acrylyl-CoA is involved in the propanoate metabolism pathway. Acrylyl-CoA is also involved in a few metabolic disorders, which include the malonic aciduria pathway, malonyl-CoA decarboxylase deficiency, and the methylmalonic aciduria due to cobalamin-related disorders pathway. Acrylyl-CoA is involved in alternative pathways of propionate metabolism.5776-58-9C0089443934015513ACRYLYL-COA388468CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12)C(O)C(=O)NCCC(=O)NCCSC(=O)C=CC24H38N7O17P3SInChI=1S/C24H38N7O17P3S/c1-4-15(33)52-8-7-26-14(32)5-6-27-22(36)19(35)24(2,3)10-45-51(42,43)48-50(40,41)44-9-13-18(47-49(37,38)39)17(34)23(46-13)31-12-30-16-20(25)28-11-29-21(16)31/h4,11-13,17-19,23,34-35H,1,5-10H2,2-3H3,(H,26,32)(H,27,36)(H,40,41)(H,42,43)(H2,25,28,29)(H2,37,38,39)/t13-,17-,18-,19?,23-/m1/s1POODSGUMUCVRTR-UXYNFSPESA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy({3-hydroxy-2,2-dimethyl-3-[(2-{[2-(prop-2-enoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxy})phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid821.582821.125773051-2.299[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3-hydroxy-2,2-dimethyl-3-[(2-{[2-(prop-2-enoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid0-4FDB022958Acryloyl coenzyme a;Acryloyl-coa;Acryloyl-coenzyme a;Acrylyl coenzyme a;Acrylyl-coa;Acrylyl-coenzyme a;Coa s-2-propenoate;Coa s-2-propenoic acid;Coa s-acrylate;Coenzyme a s-2-propenoate;Coenzyme a s-2-propenoic acid;Coenzyme a s-acrylate;Propenoyl-coa;Thioacrylic acid s-ester with coenzyme aPW_C001558AcryCoA22814786341331222624061248151201264264791279945011420WaterHMDB0002111Water 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_C001420H2O5589491095139415131621448113526156242865210691207703382318838210943113774914655415904320182425322226786027274627781728052931437031647236346145983647273749419350302751567519597521410052279452361035297105531911153431135355112540211054701235483125549212655071275534130553711455411295591135560811856221085691657591405778101584114358531465877107589095591014759401516032155605915760871616123163613315962151621816664771786507180660015267131176840188688816071622057181207719320672112117228213723821472432157295198735021673882107401212746722274922247500190758817082012258237226841416292652611850277119221641201128112213285122502861226428712327249125202271263265126932901270529112715292130072981301930013025301130373021326122313327294153403084232731542695318436913227691429377019253771021327713113377215134773783317739733277471333775161157753633477628336777223377775934177816343779823477807132978235352782423537827035679113360800143688003937080591228806561199383038394794384110557390110639391115844398119879232119915122119963406120008407120046408120113124120365412120430405120438409120606415120794414121158425121240429121351121121381419121607434122118382122384436122753120122797374122804443123012446123064376123072137123131447123142136123162448123231451123384450123730460123810464123940455124165469124670399124938471124945472125305297125353479125386481125424482125480299125682483125707478125745487126054490126238495126273484126764480126896501126963502127017388127177208127199209127227504127506507127576515127836389128082395128176513140674790140675834140755185552Hydroxypropionic acidHMDB00007003-Hydroxypropionic acid is a carboxylic acid. It is an intermediate in the breakdown of branched-chain amino acids and propionic acid from the gut. Typically it originates from propionyl-CoA and a defect in the enzyme propionyl carboxylase. This leads to a buildup in propionyl-CoA in the mitochondria. Such a buildup can lead to a disruption of the esterified CoA:free CoA ratio and ultimately to mitochondrial toxicity. Detoxification of these metabolic end products occurs via the transfer of the propionyl moiety to carnitine-forming propionyl-carnitine, which is then transferred across the inner mitochondrial membrane. 3-Hydroxypropionic acid is then released as the free acid. As an industrial chemical, it is used in the production of various chemicals such as acrylates in industry. When present in sufficiently high levels, 3-hydroxypropionic 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. Chronically high levels of hydroxypropionic acid are associated with many inborn errors of metabolism including biotinidase deficiency, malonic aciduria, methylmalonate semialdehyde dehydrogenase deficiency, methylmalonic aciduria, methylmalonic aciduria due to cobalamin-related disorders, and propionic acidemia. Hydroxypropionic 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. Infants with acidosis have symptoms that include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of the 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.503-66-2C01013681523340461460DB03688OCCC(O)=OC3H6O3InChI=1S/C3H6O3/c4-2-1-3(5)6/h4H,1-2H2,(H,5,6)ALRHLSYJTWAHJZ-UHFFFAOYSA-N3-hydroxypropanoic acid90.077990.0316940580.862hydroxypropionic acid0-1FDB0221892-deoxyglycerate;2-deoxyglyceric acid;3-hydroxypropanoate;3-hydroxypropanoic acid;3-hydroxypropionate;3-hydroxypropionic acid;Ethylenelactate;Ethylenelactic acid;Hydracrylic acid;Hydroxypropionate;B-hydroxypropionate;B-hydroxypropionic acid;B-lactate;B-lactic acid;Beta-hydroxypropionate;Beta-hydroxypropionic acid;Beta-lactate;Beta-lactic acid;3-hydroxy-propanoic acid;3-hydroxy-propanoate;β-hydroxypropionate;β-hydroxypropionic acid;HydracrylatePW_C0005523OH2282478635133122264406124817120126428479127996501988Propionyl-CoAHMDB0001275Propionyl-CoA is an intermediate in the metabolism of propanoate. Propionic aciduria is caused by an autosomal recessive disorder of propionyl coenzyme A (CoA) carboxylase deficiency (EC 6.4.1.3). In propionic aciduria, propionyl CoA accumulates within the mitochondria in massive quantities; free carnitine is then esterified, creating propionyl carnitine, which is then excreted in the urine. Because the supply of carnitine in the diet and from synthesis is limited, such patients readily develop carnitine deficiency as a result of the increased loss of acylcarnitine derivatives. This condition demands supplementation of free carnitine above the normal dietary intake to continue to remove (detoxify) the accumulating organic acids. Propionyl-CoA is a substrate for Acyl-CoA dehydrogenase (medium-chain specific, mitochondrial), Acetyl-coenzyme A synthetase 2-like (mitochondrial), Propionyl-CoA carboxylase alpha chain (mitochondrial), Methylmalonate-semialdehyde dehydrogenase (mitochondrial), Trifunctional enzyme beta subunit (mitochondrial), 3-ketoacyl-CoA thiolase (peroxisomal), Acyl-CoA dehydrogenase (long-chain specific, mitochondrial), Malonyl-CoA decarboxylase (mitochondrial), Acetyl-coenzyme A synthetase (cytoplasmic), 3-ketoacyl-CoA thiolase (mitochondrial) and Propionyl-CoA carboxylase beta chain (mitochondrial). (PMID: 10650319).317-66-8C0010043916415539PROPIONYL-COA388310CCC(=O)SCCNC(=O)CCNC(=O)C(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=C2NC24H40N7O17P3SInChI=1S/C24H40N7O17P3S/c1-4-15(33)52-8-7-26-14(32)5-6-27-22(36)19(35)24(2,3)10-45-51(42,43)48-50(40,41)44-9-13-18(47-49(37,38)39)17(34)23(46-13)31-12-30-16-20(25)28-11-29-21(16)31/h11-13,17-19,23,34-35H,4-10H2,1-3H3,(H,26,32)(H,27,36)(H,40,41)(H,42,43)(H2,25,28,29)(H2,37,38,39)/t13-,17-,18-,19?,23-/m1/s1QAQREVBBADEHPA-UXYNFSPESA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy({3-hydroxy-2,2-dimethyl-3-[(2-{[2-(propanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxy})phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid823.597823.141423115-2.169[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-({[hydroxy([hydroxy(3-hydroxy-2,2-dimethyl-3-[(2-{[2-(propanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxy)phosphoryl]oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxyphosphonic acid0-4FDB0225292-methylacetyl-coa;2-methylacetyl-coenzyme a;Propanoyl-coa;Propanoyl-coenzyme a;Propionyl-coa;Propionyl-coenzyme a;Alpha-methylacetyl-coa;Alpha-methylacetyl-coenzyme aPW_C000988PropCoA12778169432285424455491413909122477641334784361127855611178636133120995122121576407121681408122266406123560135124133119124231374124819120125935297126430479126557482126568481127395205127997501128130502128141206964FADHMDB0001248FAD, 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_C000964FAD999114518681923216425317628288251884021188141489421612291622492133582536223723264602364688314741134758104881652681035285102533511154961265511127561311860301556054156608216161161626390164751786499179666610770391637175205732121374652227487223907622411818216118872151189921112296225123282491244315112519227125952261271029112720292130293011304130243623318770802937712613377152134775011137750711277518115775413347761513277726337780543297837534578930331792223367927235880012368800343698071411911995840611999938412005140812010740712043240512045312212049012412127842912129841812141738212148938312274812012277612112280237412282344312306637612308713512316644812384946412386845412397639912404739812534847912537848012542948212547448112569729712597948912610729912627748412689150112692039112696850212698720712701120612731020912743250612760238812784038914079018514079918632Adenosine 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_C000170Ppi1223546384292373532882221217316204924105928152941751448685450348952521045294101540911754241035433118545812055481115559132558413356061355655108587910762391666978199707318871341637272160731219873182138275151828321011869161120022221204116412315225123232491251228812579226126952901521930615375183476017425613154269731877235329773171287763533678416335789283317915311279950134799581308004737280417170856301947863849481412594819382986782231106343911132703951132753891155271361155323991199341221200171241200324061203304101209364071212614291213411211214863831224074221229854441235021191238314641240443981249773751253242971253952991254104791255974841256564851258764811265524911268692051269353881269505011273372061281245081407728912941Propinol adenylateHMDB0006806Propinol adenylate, also known as propionyl-amp, belongs to the class of organic compounds known as 5'-acylphosphoadenosines. These are ribonucleoside derivatives containing an adenoside moiety, where the phosphate group is acylated. Propinol adenylate is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). In humans, propinol adenylate is involved in the propanoate metabolism pathway. Propinol adenylate is also involved in a few metabolic disorders, which include the methylmalonic aciduria due to cobalamin-related disorders pathway, the malonic aciduria pathway, and malonyl-CoA decarboxylase deficiency. Propinol adenylate is involved in the propanoate metabolism pathway. Propinol adenylate can be reversibly produced from propanoate or propanoyl-CoA by acetyl-CoA synthetase [EC:6.2.1.1] and propionyl-CoA synthetase [EC:6.2.1.17].C0598344086362415389700CCC(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2NC13H18N5O8PInChI=1S/C13H18N5O8P/c1-2-7(19)26-27(22,23)24-3-6-9(20)10(21)13(25-6)18-5-17-8-11(14)15-4-16-12(8)18/h4-6,9-10,13,20-21H,2-3H2,1H3,(H,22,23)(H2,14,15,16)/t6-,9-,10-,13-/m1/s1ZGNGGJLVZZHLQM-ZRFIDHNTSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(propanoyloxy)phosphinic acid403.2845403.089299089-2.134propionyl-AMP0-1FDB024092Propionyladenylate;5'-adenylic acid propanoic acid anhydride;5'-adenylic acid propionic acid anhydride;5'-o-[hydroxy(propionyloxy)phosphoryl]adenosine;Propanoyl-adenosine monophosphate;Propionyl-adenosine monophosphate;Propionyl-amp;5'-adenylate propanoate anhydride;5'-adenylate propionate anhydride;Propanoyl-adenosine monophosphoric acid;Propionyl-adenosine monophosphoric acid;Propionyladenylic acidPW_C002941ProAdyl22924786371331222684061248211201264324791280005011887PantetheineHMDB0003426Pantetheine is the mercaptoethyl conjugated amide analogue of pantothenic acid (Vitamin B5). The dimer of this compound, pantethine is more commonly known, and is considered to be a more potent form of vitamin B5 than pantothenic acid. Pantetheine is an intermediate in the production of Coenzyme A by the body. An intermediate in the pathway of coenzyme A formation in mammalian liver and some microorganisms.496-65-1C0083147916753PANTETHEINE-P466CC(C)(CO)C(O)C(=O)NCCC(=O)NCCSC11H22N2O4SInChI=1S/C11H22N2O4S/c1-11(2,7-14)9(16)10(17)13-4-3-8(15)12-5-6-18/h9,14,16,18H,3-7H2,1-2H3,(H,12,15)(H,13,17)ZNXZGRMVNNHPCA-UHFFFAOYSA-N2,4-dihydroxy-3,3-dimethyl-N-{2-[(2-sulfanylethyl)carbamoyl]ethyl}butanamide278.368278.130027892-2.835pantetheine00FDB023172(r)-pantetheine;D-pantetheine;Lbf;Lactobacillus bulgaricus factor;PantotheinePW_C001887Panethn1144144610257581407844311578638132120847405122269124123428376124822118125834478126433299127287209128001388159Propionic acidHMDB0000237Propionic acid (PA) is widely used as an antifungal agent in food. It is present naturally at low levels in dairy products and occurs ubiquitously, together with other short-chain fatty acids (SCFA), in the gastro-intestinal tract of humans and other mammals as an end-product of the microbial digestion of carbohydrates. It has significant physiological activity in animals. PA is irritant but produces no acute systemic effects and has no demonstrable genotoxic potential. (PMID 1628870) Propionic aciduria is one of the most frequent organic acidurias, a disease that comprise many various disorders. The outcome of patients born with Propionic aciduria is poor intellectual development patterns, with 60% having an IQ less than 75 and requiring special education. Successful liver and/or renal transplantations, in a few patients, have resulted in better quality of life but have not necessarily prevented neurological and various visceral complications. These results emphasize the need for permanent metabolic follow-up whatever the therapeutic strategy. (PMID 15868474) Decreased early mortality, less severe symptoms at diagnosis, and more favorable short-term neurodevelopmental outcome were recorded in patients identified through expanded newborn screening. (PMID 16763906).79-09-4C00163103230768PROPIONATE1005DB03766CCC(O)=OC3H6O2InChI=1S/C3H6O2/c1-2-3(4)5/h2H2,1H3,(H,4,5)XBDQKXXYIPTUBI-UHFFFAOYSA-Npropanoic acid74.078574.0367794360.681propanoic acid0-1FDB008285Adofeed;Antischim b;Carboxyethane;Ethanecarboxylate;Ethanecarboxylic acid;Ethylformate;Ethylformic acid;Luprosil;Metacetonate;Metacetonic acid;Methylacetate;Methylacetic acid;Monoprop;Propanate;Propanoate;Propanoic acid;Propcorn;Propionate;Propkorn;Prozoin;Pseudoacetate;Pseudoacetic acid;Toxi-check;Acide propanoique;Acide propionique;Ch3-ch2-cooh;Pa;Propioic acid;Propionsaeure;Propoic acid;Propioate;PropoatePW_C0001593:02307445631863631076364108424683184246931578639133792281301212851251222724061238561361248251201264344791280035011051HydrogenHMDB0001362Hydrogen is a colorless, odorless, nonmetallic, tasteless, highly flammable diatomic gas with the molecular formula H2. With an atomic weight of 1.00794, hydrogen is the lightest element. Besides the common H1 isotope, hydrogen exists as the stable isotope Deuterium and the unstable, radioactive isotope Tritium. Hydrogen is the most abundant of the chemical elements, constituting roughly 75% of the universe's elemental mass. Hydrogen can form compounds with most elements and is present in water and most organic compounds. It plays a particularly important role in acid-base chemistry, in which many reactions involve the exchange of protons between soluble molecules. Oxidation of hydrogen, in the sense of removing its electron, formally gives H+, containing no electrons and a nucleus which is usually composed of one proton. That is why H+ is often called a proton. This species is central to discussion of acids. Under the Bronsted-Lowry theory, acids are proton donors, while bases are proton acceptors. A bare proton H+ cannot exist in solution because of its strong tendency to attach itself to atoms or molecules with electrons. However, the term 'proton' is used loosely to refer to positively charged or cationic hydrogen, denoted H+. H2 is a product of some types of anaerobic metabolism and is produced by several microorganisms, usually via reactions catalyzed by iron- or nickel-containing enzymes called hydrogenases. These enzymes catalyze the reversible redox reaction between H2 and its component two protons and two electrons. Creation of hydrogen gas occurs in the transfer of reducing equivalents produced during pyruvate fermentation to water.1333-74-0C002825883867318276ALPHA-GLUCOSE-16-BISPHOSPHATE762[H][H]H2InChI=1S/H2/h1HUFHFLCQGNIYNRP-UHFFFAOYSA-Ndihydrogen2.01592.0156500640dihydrogen00FDB016247Dihydrogen;Hydrogen;Hydrogen cation;Hydrogen gas;Hydrogen ion;Hydronium;Proton;E 949;E-949;E949;H2;Molecular hydrogenPW_C001051H21756823882269633146704952107033163704516012774151132702257859411278603132787721111131639412144812212203240712203712412400613512458611912459111812607329712618248112618829912752920512800538812832320632-Ketobutyric acidHMDB00000052-Ketobutyric acid is a substance that is involved in the metabolism of many amino acids (glycine, methionine, valine, leucine, serine, threonine, isoleucine) as well as propanoate metabolism and C-5 branched dibasic acid metabolism. More specifically, alpha-ketobutyric acid is a product of the lysis of cystathionine. It is also one of the degradation products of threonine. It can be converted into propionyl-CoA (and subsequently methylmalonyl CoA, which can be converted into succinyl CoA, a citric acid cycle intermediate), and thus enter the citric acid cycle.600-18-0C0010958308312-OXOBUTANOATE57DB04553CCC(=O)C(O)=OC4H6O3InChI=1S/C4H6O3/c1-2-3(5)4(6)7/h2H2,1H3,(H,6,7)TYEYBOSBBBHJIV-UHFFFAOYSA-N2-oxobutanoic acid102.0886102.031694058-0.1112-oxobutanoic acid0-1FDB0033592-ketobutanoate;2-ketobutanoic acid;2-ketobutyrate;2-oxo-butanoate;2-oxo-butanoic acid;2-oxo-butyrate;2-oxo-butyric acid;2-oxo-n-butyrate;2-oxo-n-butyric acid;2-oxobutanoate;2-oxobutanoic acid;2-oxobutyrate;2-oxobutyric acid;3-methylpyruvate;3-methylpyruvic acid;Methyl-pyruvate;Methyl-pyruvic acid;Propionyl-formate;Propionyl-formic acid;A-keto-n-butyrate;A-keto-n-butyric acid;A-ketobutyrate;A-ketobutyric acid;A-oxo-n-butyrate;A-oxo-n-butyric acid;A-oxobutyrate;A-oxobutyric acid;Alpha-keto-n-butyrate;Alpha-keto-n-butyric acid;Alpha-ketobutric acid;Alpha-ketobutyrate;Alpha-ketobutyric acid;Alpha-oxo-n-butyrate;Alpha-oxo-n-butyric acid;Alpha-oxobutyrate;Alpha-oxobutyric acid;2-ketobutyric acid;3-methyl pyruvic acid;3-methyl pyruvate;α-ketobutyrate;α-ketobutyric acid;α-oxo-n-butyrate;α-oxo-n-butyric acidPW_C0000032KBA3378186822692382741518383225422724781261327816311178643133790271121199221221221761241222744061225774071227161351247281181248291201251491191253122971263332991264384791267264811268582051278963881280075011283192061060Thiamine 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-O3-[(4-amino-2-methylpyrimidin-5-yl)methyl]-5-(2-{[hydroxy(phosphonooxy)phosphoryl]oxy}ethyl)-4-methyl-1,3-thiazol-3-ium425.314425.044967696-3.484thiamin pyrophosphate1-1FDB022584Tpp;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_C001060ThiamPP20541075311978127151736253661036028155608016163881647317874632221280622577124133782851127842333479018111791751328001036811995640612080240712090212212098240812153712412274612012338811912347313512354737412409511812534647912592248212609448112680229912688950112738150212754920612840038842-Hydroxybutyric acidHMDB00000082-Hydroxybutyric acid (alpha-hydroxybutyrate) is an organic acid derived from alpha-ketobutyrate. alpha-Ketobutyrate is produced by amino acid catabolism (threonine and methionine) and glutathione anabolism (cysteine formation pathway) and is metabolized to propionyl-CoA and carbon dioxide (PMID: 20526369). 2-Hydroxybutyric acid is formed as a by-product of the formation of alpha-ketobutyrate via a reaction catalyzed by lactate dehydrogenase (LDH) or alpha-hydroxybutyrate dehydrogenase (alphaHBDH). alpha-Hydroxybutyric acid is primarily produced in mammalian hepatic tissues that catabolize L-threonine or synthesize glutathione. Oxidative stress or detoxification of xenobiotics in the liver can dramatically increase the rate of hepatic glutathione synthesis. Under such metabolic stress conditions, supplies of L-cysteine for glutathione synthesis become limiting, so homocysteine is diverted from the transmethylation pathway (which forms methionine) into the transsulfuration pathway (which forms cystathionine). 2-Hydroxybutyrate is released as a byproduct when cystathionine is cleaved into cysteine that is incorporated into glutathione. Chronic shifts in the rate of glutathione synthesis may be reflected by urinary excretion of 2-hydroxybutyrate. 2-Hydroxybutyrate is an early marker for both insulin resistance and impaired glucose regulation that appears to arise due to increased lipid oxidation and oxidative stress (PMID: 20526369). 2-Hydroxybutyric acid is often found in the urine of patients suffering from lactic acidosis and ketoacidosis. 2-Hydroxybutyric acid generally appears at high concentrations in situations related to deficient energy metabolism (e.g. birth asphyxia) and also in inherited metabolic diseases affecting the central nervous system during neonatal development, such as "cerebral" lactic acidosis, glutaric aciduria type II, dihydrolipoyl dehydrogenase (E3) deficiency, and propionic acidemia. More recently it has been noted that elevated levels of alpha-hydroxybutyrate in the plasma is a good marker for early-stage type II diabetes (PMID: 19166731). It was concluded from studies done in the mid-1970's that an increased NADH2/NAD ratio was the most important factor for the production of 2-hydroxybutyric acid (PMID: 168632).600-15-7C05984112661148CPD-356410792CC[C@H](O)C(O)=OC4H8O3InChI=1S/C4H8O3/c1-2-3(5)4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m0/s1AFENDNXGAFYKQO-VKHMYHEASA-N(2S)-2-hydroxybutanoic acid104.105104.0473441180.672(S)-2-hydroxybutyric acid0-1FDB021867(rs)-2-hydroxybutyrate;(rs)-2-hydroxybutyric acid;2-hydroxy-butanoate;2-hydroxy-butanoic acid;2-hydroxy-dl-butyrate;2-hydroxy-dl-butyric acid;2-hydroxy-n-butyrate;2-hydroxy-n-butyric acid;2-hydroxybutanoate;2-hydroxybutanoic acid;2-hydroxybutyrate;Dl-2-hydroxybutanoate;Dl-2-hydroxybutanoic acid;Dl-a-hydroxybutyrate;Dl-a-hydroxybutyric acid;Dl-alpha-hydroxybutyrate;Dl-alpha-hydroxybutyric acid;A-hydroxy-n-butyrate;A-hydroxy-n-butyric acid;A-hydroxybutanoate;A-hydroxybutanoic acid;A-hydroxybutyrate;A-hydroxybutyric acid;Alpha-hydroxy-n-butyrate;Alpha-hydroxy-n-butyric acid;Alpha-hydroxybutanoate;Alpha-hydroxybutanoic acid;Alpha-hydroxybutyrate;Alpha-hydroxybutyric acid;α-hydroxybutanoate;α-hydroxybutanoic acid;α-hydroxybutyrate;α-hydroxybutyric acidPW_C0000042HBA4227347864813312227640612483012012644247912801050140034Hydrogen IonHMDB0059597Hydrogen ion is recommended by IUPAC as a general term for all ions of hydrogen and its isotopes. Depending on the charge of the ion, two different classes can be distinguished: positively charged ions and negatively charged ions. Under aqueous conditions found in biochemistry, hydrogen ions exist as the hydrated form hydronium, H3O+, but these are often still referred to as hydrogen ions or even protons by biochemists. [WikiPedia])C000801038153781010[H+]HInChI=1S/p+1GPRLSGONYQIRFK-UHFFFAOYSA-Nhydron1.00791.0078250320hydron10H+;H(+);Hydrogen cation;Hydron;ProtonPW_C040034H+2154670875315788318483111621463261464542231492780174250224254424547104576184694705241103532711153531125626108563910756991005720105574211759631476037155607015760931616130159623216664831786601152669210168431886910187710016371682057191206745321974542207472222752521375322107558212757216075901708195225821815182432268413162842022491391959155249119151641201528112181285122462861226628712521227132572231332529415330308423293154235431842401322424053124245432076912293771361337721013477372331778041147795513277990327779913477837934579929130800193688038731080388304807221199382312494823383110550388112855941132803901155373981155391181158563361162051091199734061201934071205491221205934091211704241211714251225694181226153841226871251227581201231831351232181371237424591237434601251414541251881211252731361253594791255504811257304831257362971258092991265174951267174891267664801268233001269025011272132081283085061283613911284303951406928821406938831406991671407071681407151414074278814074359714076018563Citric acidHMDB0000094Citric acid (citrate) is a weak acid that is formed in the tricarboxylic acid cycle or that may be introduced with diet. The evaluation of plasma citric acid is scarcely used in the diagnosis of human diseases. On the contrary urinary citrate excretion is a common tool in the differential diagnosis of kidney stones, renal tubular acidosis and it plays also a role in bone diseases. The importance of hypocitraturia should be considered with regard to bone mass, urine crystallization and urolithiasis. (PMID 12957820) The secretory epithelial cells of the prostate gland of humans and other animals posses a unique citrate-related metabolic pathway regulated by testosterone and prolactin. This specialized hormone-regulated metabolic activity is responsible for the major prostate function of the production and secretion of extraordinarily high levels of citrate. The key regulatory enzymes directly associated with citrate production in the prostate cells are mitochondrial aspartate aminotransferase, pyruvate dehydrogenase, and mitochondrial aconitase. testosterone and prolactin are involved in the regulation of the corresponding genes associated with these enzymes. The regulatory regions of these genes contain the necessary response elements that confer the ability of both hormones to control gene transcription. Protein kinase c (PKC) is the signaling pathway for the prolactin regulation of the metabolic genes in prostate cells. testosterone and prolactin regulation of these metabolic genes (which are constitutively expressed in all mammalian cells) is specific for these citrate-producing cells. (PMID 12198595) Citric acid is found in citrus fruits, most concentrated in lemons and limes, where it can comprise as much as 8% of the dry weight of the fruit. Citric acid is a natural preservative and is also used to add an acidic (sour) taste to foods and soft drinks. The salts of citric acid (citrates) can be used as anticoagulants due to their calcium chelating ability. Intolerance to citric acid in the diet is known to exist. Little information is available as the condition appears to be rare, but like other types of food intolerance it is often described as a "pseudo-allergic" reaction.77-92-9C001581978290430769CIT305DB04272OC(=O)CC(O)(CC(O)=O)C(O)=OC6H8O7InChI=1S/C6H8O7/c7-3(8)1-6(13,5(11)12)2-4(9)10/h13H,1-2H2,(H,7,8)(H,9,10)(H,11,12)KRKNYBCHXYNGOX-UHFFFAOYSA-N2-hydroxypropane-1,2,3-tricarboxylic acid192.1235192.02700261-0.264citric acid0-3FDB0125862-hydroxy-1,2,3-propanetricarboxylate;2-hydroxy-1,2,3-propanetricarboxylic acid;3-carboxy-3-hydroxypentane-1,5-dioate;3-carboxy-3-hydroxypentane-1,5-dioic acid;Aciletten;Anhydrous citrate;Anhydrous citric acid;Chemfill;Citraclean;Citrate;Citretten;Citric acid;Citro;E 330;Hydrocerol a;Kyselina citronova;Suby g;Uro-trainer;Beta-hydroxytricarballylate;Beta-hydroxytricarballylic acid;2-hydroxytricarballylic acid;Citronensaeure;E330;H3cit;2-hydroxytricarballylatePW_C000063CA21942415253721036034155608916164791787469222771321337905313280016368111712811996540612239712412275412012496711812535547912653829912689850112811138869electron-transfer flavoproteinCompoundPW_EC0000695086ChEBIETF70Reduced electron-transfer flavoproteinCompoundPW_EC0000705086ChEBIRETF250Methylmalonyl-CoA epimerase, mitochondrialQ96PE7HMDBP00256MCEE2p13.3AF36454715.1.99.1171432291426982251Methylmalonyl-CoA mutase, mitochondrialP22033Involved in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species.
HMDBP00257MUT6p12.3M3750015.4.99.2171542402316Malonyl-CoA decarboxylase, mitochondrialO95822Catalyzes the conversion of malonyl-CoA to acetyl-CoA. In the fatty acid biosynthesis MCD selectively removes malonyl-CoA and thus assures that methyl-malonyl-CoA is the only chain elongating substrate for fatty acid synthase and that fatty acids with multiple methyl side chains are produced. In peroxisomes it may be involved in degrading intraperoxisomal malonyl-CoA, which is generated by the peroxisomal beta-oxidation of odd chain-length dicarboxylic fatty acids.
HMDBP00016MLYCD16q24BC05259214.1.1.9226944574214106116645Acetyl-CoA carboxylase 1Q13085Catalyzes the rate-limiting reaction in the biogenesis of long-chain fatty acids. Carries out three functions: biotin carboxyl carrier protein, biotin carboxylase and carboxyltransferase.
HMDBP00047ACACA17q21AY31562016.4.1.2; 6.3.4.141789822722136677653140994393141059166141628261421613114332284736Acetyl-CoA acetyltransferase, mitochondrialP24752Plays a major role in ketone body metabolism.
HMDBP00038ACAT111q22.3CH47106512.3.1.9594410503528110313563313313965178514098213141289249Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrialQ02252Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA.
HMDBP00051ALDH6A114q24.3AJ24999411.2.1.18; 1.2.1.27544416953461322424-aminobutyrate aminotransferase, mitochondrialP80404Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine.
HMDBP00248ABAT16p13.2U8022612.6.1.19; 2.6.1.224631488542445752380Enoyl-CoA hydratase, mitochondrialP30084Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate.
HMDBP00388ECHS110q26.2-q26.3BT00712314.2.1.1759148533294417460525237103135623133143885118124253-hydroxyisobutyryl-CoA hydrolase, mitochondrialQ6NVY1Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.
HMDBP04709HIBCH2q32.2AK22297913.1.2.4167432283446082101Medium-chain specific acyl-CoA dehydrogenase, mitochondrialP11310This enzyme is specific for acyl chain lengths of 4 to 16.
HMDBP00104ACADM1p31M9143211.3.8.78974435334354346092527010313563013313785471340Acetyl-coenzyme A synthetase 2-like, mitochondrialQ9NUB1Important for maintaining normal body temperature during fasting and for energy homeostasis. Essential for energy expenditure under ketogenic conditions (By similarity). Converts acetate to acetyl-CoA so that it can be used for oxidation through the tricarboxylic cycle to produce ATP and CO(2).
HMDBP00042ACSS120p11.23-p11.21BC04458816.2.1.1791446112139656545247Acyl-CoA synthetase short-chain family member 3, mitochondrialQ9H6R3Activates acetate so that it can be used for lipid synthesis or for energy generation (By similarity).
HMDBP11602ACSS312q21.31CH47105416.2.1.12293446122253Propionyl-CoA carboxylase alpha chain, mitochondrialP05165HMDBP00259PCCA13q32AY03579516.4.1.3170032287446142252Propionyl-CoA carboxylase beta chain, mitochondrialP05166HMDBP00258PCCB3q21-q22M3116716.4.1.3170134615214284742232Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrialP11182The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
HMDBP03078DBT1p31J0320812.3.1.16815974174834359252Dihydrolipoyl 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-q32L1375711.8.1.421741080346708639411369657021421265414252510201425961202932-oxoisovalerate dehydrogenase subunit beta, mitochondrialP21953
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
HMDBP00299BCKDHB6q14.1X5244611.2.4.41589417382269332942-oxoisovalerate dehydrogenase subunit alpha, mitochondrialP12694The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
HMDBP00300BCKDHA19q13.1-q13.2Z1409311.2.4.41588417372269432357L-lactate dehydrogenase A-like 6BQ9BYZ2HMDBP03581LDHAL6B15q22.2AK05819211.1.1.27422704289Aldehyde dehydrogenase, mitochondrialP05091HMDBP00295ALDH212q24.2K0300111.2.1.35474132181871330092135492181415779561420219791434755413Acetyl-CoA acetyltransferase, cytosolicQ9BWD1HMDBP00013ACAT26q25.3BC00040812.3.1.97938926913465Methylmalonyl-CoA epimerase, mitochondrial1PW_P000465488250468Methylmalonyl-CoA mutase, mitochondrial1PW_P000468491251228614011590Malonyl-CoA decarboxylase, mitochondrial1PW_P00059063416493Acetyl-CoA carboxylase 11PW_P00049351745123920124010272179Acetyl-CoA acetyltransferase, mitochondrial1PW_P000179197364158Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial1PW_P000158176494543364-aminobutyrate aminotransferase, mitochondrial1PW_P00000672422711481406177Enoyl-CoA hydratase, mitochondrial1PW_P00017719538064583-hydroxyisobutyryl-CoA hydrolase, mitochondrial1PW_P0004584812425251Medium-chain specific acyl-CoA dehydrogenase, mitochondrial1PW_P00025127010141169641228Acetyl-coenzyme A synthetase 2-like, mitochondrial1PW_P000228246401591Acyl-CoA synthetase short-chain family member 3, mitochondrial1PW_P0005916355247684Propionyl-CoA carboxylase1PW_P00068477125317722521310201683branched-chain alpha-keto dehydrogenase complex1PW_P0006837672232176852176929317702941307106013089641309106015705 L-lactate dehydrogenase A-like 6B1PW_P005705131892357422714159Aldehyde dehydrogenase, mitochondrial1PW_P00015917728945454229Acetyl-CoA acetyltransferase, cytosolic1PW_P0002292471341254falsePW_R001254Both480314591Compoundfalse480429421Compoundfalse1263falsePW_R001263Right482915601Compoundfalse483015231Compoundfalse9764655.1.99.11281falsePW_R001281Right48997041Compoundfalse490015001Compoundfalse1282falsePW_R001282Right490115001Compoundfalse79207211Compoundtrue792114201Compoundtrue49021301Compoundfalse792211441Compoundtrue20871591.2.1.31264falsePW_R001264Both483115231Compoundfalse48328081Compoundfalse9774685.4.99.21247falsePW_R001247Right47829111Compoundfalse47839401Compoundfalse822013161Compoundtrue9615904.1.1.91048falsePW_R001048Right40454141Compoundtrue40464631Compoundtrue40479401Compoundfalse404814411Compoundtrue404910341Compoundtrue40509111Compoundfalse703493681falsePW_R000681Both27919402Compoundtrue279211421Compoundfalse279310991Compoundtrue1781792.3.1.95172292.3.1.9666falsePW_R000666Right273567531Compoundfalse273610991Compoundtrue27377211Compoundtrue27389401Compoundfalse273913161Compoundtrue274011441Compoundtrue1611585falsePW_R000005Right21401Compoundfalse221341Compoundfalse2367531Compoundfalse24951Compoundfalse662266PW_R002266Both82479111Compoundfalse824814591Compoundfalse1255falsePW_R001255Both480529421Compoundfalse480615581Compoundfalse824914201Compoundtrue9691774.2.1.171256falsePW_R001256Both480729421Compoundfalse825014201Compoundtrue48085521Compoundfalse825110991Compoundtrue9704583.1.2.41260falsePW_R001260Both481915581Compoundfalse8252691ElementCollectiontrue48209881Compoundfalse8253701ElementCollectiontrue9722511.3.8.71265falsePW_R001265Both48339881Compoundfalse8254321Compoundtrue82551701Compoundtrue483429411Compoundfalse82564141Compoundtrue825718871Compoundtrue9782286.2.1.11342falsePW_R001342Both511329411Compoundfalse82584141Compoundtrue825918871Compoundtrue51149881Compoundfalse8260321Compoundtrue82611701Compoundtrue10575916.2.1.11273falsePW_R001273Both486529411Compoundfalse48661591Compoundfalse8262321Compoundtrue9895916.2.1.11343falsePW_R001343Both511529411Compoundfalse51161591Compoundfalse8263321Compoundtrue10582286.2.1.11011falsePW_R001011Right393415001Compoundfalse790114201Compoundtrue79027211Compoundtrue790310991Compoundtrue39359881Compoundfalse790411441Compoundtrue79054631Compoundtrue6271581484falsePW_R001484Right56299881Compoundfalse56304631Compoundtrue56314141Compoundtrue563215601Compoundfalse563310341Compoundtrue563410511Compoundtrue12056846.4.1.31483falsePW_R001483Right562431Compoundfalse562510991Compoundtrue56267211Compoundtrue56279881Compoundfalse562811441Compoundtrue12046832.3.1.16872860PW_R072860Both25037241Compoundfalse2503737211Compoundtrue25037431Compoundfalse25037511441Compoundtrue250376400341Compoundtrue6298957051.1.1.2731706351459482false1768125710regular30028031706362942482false2428191710regular30028031706371560482false3733208710regular30028031706381523482false3733266710regular3002803170639704482false4418172210regular30028031706401500482false4418137210regular3002803170641130482false4788137210regular3002803170642808482false3168266710regular3002803170643140139false3563276210regular100253170644911482false838125210regular3002803170645940482false838175710regular30028031706461316252false1056167310regular78783170647414242false378162710regular50303170648463247false428168710regular787831706491441246false377141510regular444331706501034243false442136710regular503031706512029false418156719regular100253170652102729false418150719regular1002531706531142482false838225210regular30028031706541099385false873219710regular503031706556753482false1248225210regular30028031706561099385false1298214710regular50303170657721359false1483215210regular503031706581316352false1481189310regular787831706591144360false1373189710regular5030317066040482false2078225210regular3002803170661134482false1843255210regular300280317066295482false1478255210regular3002803170663114849false1763233710regular1003531706641558482false2428136710regular30028031706651420249false2423166710regular787831706661420249false2448225710regular78783170667552482false2428254710regular30028031706681099285false2478245710regular50303170669988482false3728137710regular300280317067096429false3303122210regular10025317067132244false3578167410regular50303170672170245false3636170210regular634331706732941482false2833137710regular3002803170674414242false3343166710regular503031706751887281false3163169710regular2001903170676414242false3293134210regular503031706771887281false3183143710regular200190317067832244false3583133910regular50303170679170245false3576144210regular63433170680159482false2843192210regular300280317068132244false3108187210regular5030317068232244false2813187210regular503031706831420249false4303155210regular78783170684721259false4303143210regular503031706851099285false4353141210regular503031706861144260false4068143210regular50303170687463247false4043155210regular78783170688463247false3723167210regular78783170689414242false3773176210regular503031706901034243false3780195710regular503031706911051255false3731199810regular787831706922029false3838183219regular1002531706933482false4263208710regular3002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1397 C2098 1397 2394 1560 2418 1577 5true18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339113M2428 2057 C2158 2055 2068 2057 1919 2058 C1919 1974 1918 1637 1918 1537 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345falsetrueM 1368.2145356150122 1092.0976086451406 L 1355 1085 L 1355.4605584146943 1099.9929278643851false4339114M3883 2367 C3883 2397 3883 2447 3883 2477 5false184339115M3883 2667 C3883 2637 3883 2577 3883 2547 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339116M4568 1722 C4569 1670 4569 1706 4568 1652 5false18trueM 2426.606529735972 1206.7538843850477 L 2440 1200 L 2427.4542294163116 1191.777856699044false4339117M4418 1512 C4388 1512 4544 1985 4568 2002 5true18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339118M4718 1512 C4757 1512 4751 1513 4788 1512 5false18trueM 2595.0644009829866 1323.611518094107 L 2610 1325 L 2603.734661094692 1311.371150877582false4339119M4788 1512 C4758 1512 3414 1810 3438 1827 5true18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339120M3733 2807 C3703 2807 3718 2807 3688 2807 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339121M3468 2807 C3498 2807 3498 2807 3528 2807 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339122M3518 2642 L3518 2692 L3568 2642 z10true184339123M988 1532 C988 1562 988 1562 988 1592 5false184339124M988 1757 C988 1727 988 1692 988 1662 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339125M1056 1712 C1014 1712 988 1692 988 1662 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339126M428 1642 C463 1639 468 1617 468 1587 5false184339127M467 1687 C467 1657 468 1617 468 1587 5false184339128M838 1897 C647 1892 468 1617 468 1587 5true184339129M421 1436.5 C476 1432.5 468 1487 468 1517 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339130M467 1397 C467 1427 468 1487 468 1517 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339131M838 1392 C629 1393 468 1457 468 1517 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339132M973 777 L973 827 L1023 777 z10true184339133M973 777 L973 827 L1023 777 z10true184339134M988 2037 C988 2067 988 2062 988 2092 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339135M988 2252 C988 2222 988 2207 988 2177 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339136M923 2212 C957 2211 988 2207 988 2177 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339137M1398 2252 C1400 2223 1397 2126 1398 2102 5false184339138M1348 2162 C1391 2160 1403 2123 1398 2102 5false184339139M1483 2172 C1441 2179 1396 2137 1398 2102 5false184339140M1138 1897 C1216 1892 1397 1901 1398 2017 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339141M1481 1932 C1434 1935 1395 1951 1398 2017 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339142M1398 1927 C1395 1956 1398 1981 1398 2017 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339143M2078 2392 C2048 2392 1918 2392 1888 2392 5false184339144M1993 2552 C1991 2508 1998 2394 1888 2392 5false184339145M1548 2392 C1578 2392 1708 2392 1738 2392 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339146M1628 2552 C1630 2510 1641 2392 1738 2392 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339147M973 777 L973 827 L1023 777 z10true184339148M1138 1392 C1168 1392 1949 1375 1973 1392 5true18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339149M1768 1397 C1738 1397 1236 1392 1138 1392 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345falsetrueM 987.9903810567666 757.5 L 975 765 L 987.9903810567666 772.5false4339150M2578 1917 C2578 1887 2578 1882 2578 1852 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339151M2578 1647 C2578 1677 2578 1737 2578 1767 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339152M2501 1706 C2549 1707 2578 1737 2578 1767 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339153M2578 2197 C2578 2227 2578 2327 2578 2357 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339154M2526 2296 C2580 2296 2578 2327 2578 2357 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339155M2578 2547 C2578 2517 2578 2457 2578 2427 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339156M2528 2472 C2563 2473 2578 2457 2578 2427 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339157M2578 1367 C2664 1273 3253 1282 3283 1282 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339158M3878 1377 C3802 1285 3453 1282 3423 1282 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339159M3153 1202 C3155 1248 3197 1276 3283 1282 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339160M3563 1202 C3563 1250 3508 1277 3423 1282 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339161M973 777 L973 827 L1023 777 z10true184339162M3731 1638 C3683 1638 3607 1639 3560 1639 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339163M3603 1674 C3604 1647 3610 1639 3560 1639 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339164M3667.5 1702 C3667.5 1673 3653 1640 3560 1639 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339165M3138 1639 C3180 1640 3318 1641 3410 1639 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false4339166M3368 1667 C3369 1643 3382 1638 3410 1639 5false18trueM 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