63PathwayPhenylacetate MetabolismPhenylacetate (or phenylacetic acid) metabolism involves two steps. The first step is the conversion of phenylacetate into phenylacetyl-CoA which is catalyzed by acyl-coenzyme A synthetase ACSM1 or acyl-coenzyme A synthetase ACSM2B. Coenzyme A and ATP are also involved in this first step and AMP and pyrophosphate will be generated during the first step of metabolism. In the second step, phenylacetyl-CoA and L-glutamine interacts with glycine N-acyltransferase to generate coenzyme A as well as phenylacetylglutamine, of which the latter will be excreted in the urine. Phenylacetate metabolism provides a route that facilitates the excretion of nitrogen for patients with urea cycle defects; hence, it is important for clinical purposes.MetabolicPW000033CenterPathwayVisualizationContext3323002400#000099PathwayVisualization4363Phenylacetate MetabolismPhenylacetate (or phenylacetic acid) metabolism involves two steps. The first step is the conversion of phenylacetate into phenylacetyl-CoA which is catalyzed by acyl-coenzyme A synthetase ACSM1 or acyl-coenzyme A synthetase ACSM2B. Coenzyme A and ATP are also involved in this first step and AMP and pyrophosphate will be generated during the first step of metabolism. In the second step, phenylacetyl-CoA and L-glutamine interacts with glycine N-acyltransferase to generate coenzyme A as well as phenylacetylglutamine, of which the latter will be excreted in the urine. Phenylacetate metabolism provides a route that facilitates the excretion of nitrogen for patients with urea cycle defects; hence, it is important for clinical purposes.Metabolic18513SubPathway154500Compound4867SubPathway155500Compound4316Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.63Pathway317Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.63Pathway1CellCL:00000002Platelet CL:00002335HepatocyteCL:00001823NeuronCL:00005404CardiomyocyteCL:00007468Beta cellCL:00006397Epithelial CellCL:00000661Homo sapiens9606EukaryoteHuman12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat2Bacteria2ProkaryoteBacteria3Escherichia coli562Prokaryote19Schizosaccharomyces pombe4896Eukaryote24Solanum lycopersicum4081EukaryoteTomato4Arabidopsis thaliana3702EukaryoteThale cress18Saccharomyces cerevisiae4932EukaryoteYeast21Xenopus laevis8355EukaryoteAfrican clawed frog6Caenorhabditis elegans6239EukaryoteRoundworm25Escherichia coli (strain K12)83333Prokaryote49Bathymodiolus platifrons220390EukaryoteDeep sea mussel23Pseudomonas aeruginosa287Prokaryote60Nitzschia sp.0001EukaryoteNitzschia410Drosophila melanogaster7227EukaryoteFruit fly51Picea sitchensis3332EukaryoteSitka spruce3Mitochondrial MatrixGO:00057591CytosolGO:00058295CytoplasmGO:000573714Mitochondrial Outer MembraneGO:00057412MitochondrionGO:000573915NucleusGO:00056344PeroxisomeGO:000577713Endoplasmic ReticulumGO:00057837Endoplasmic Reticulum MembraneGO:000578910Cell MembraneGO:000588627Peroxisome MembraneGO:000577831Periplasmic SpaceGO:000562011Extracellular SpaceGO:000561535ChloroplastGO:000950712Mitochondrial Inner MembraneGO:000574332Inner MembraneGO:00702588Smooth Endoplasmic Reticulum GO:000579025Golgi ApparatusGO:000579426Golgi Apparatus MembraneGO:00001392Endothelium BTO:00003931LiverBTO:00007597297Nervous SystemBTO:000148418PancreasBTO:000098825IntestineBTO:00006488Blood VesselBTO:000110274115cardiocyteBTO:00015396KidneyBTO:00006717184311PW_BS0000041333121PW_BS000133406351PW_BS0001151203171PW_BS0001202111PW_BS0000028511PW_BS00000816212PW_BS000016221411PW_BS00002213121PW_BS0000133211515PW_BS0000325411PW_BS000005397113PW_BS0000393211PW_BS000003181311PW_BS000018101711PW_BS00001049711PW_BS00004914101PW_BS0000145811411PW_BS000058592711PW_BS00005927151PW_BS00002746114PW_BS00004629111PW_BS0000296618518PW_BS00006672513PW_BS000072612517PW_BS0000615181PW_BS000051231511PW_BS000023311511PW_BS000031918511PW_BS000091541315PW_BS000054892PW_BS000089261115PW_BS000026711PW_BS000007971521PW_BS000097100521PW_BS0001001041431PW_BS000104101531PW_BS0001011115121PW_BS0001111122121PW_BS000112103331PW_BS000103117131PW_BS0001171181171PW_BS00011812915121PW_BS0001291321121PW_BS0001321355171PW_BS00013510813PW_BS00010814315191PW_BS0001431465191PW_BS000146107313PW_BS0001071471241PW_BS000147151141PW_BS0001511553241PW_BS0001551613181PW_BS00016116611PW_BS0001661783211PW_BS000178188118PW_BS0000241601181PW_BS00016019914181PW_BS000024205561PW_BS000024206261PW_BS00002421013181PW_BS0000242137181PW_BS0000242111018PW_BS0000241985181PW_BS0000242164181PW_BS0000242171518PW_BS00002421815181PW_BS0000241632181PW_BS000163222341PW_BS0000241901118PW_BS0000242253541PW_BS0000242771218PW_BS00002417018PW_BS0001702811251PW_BS0000241644PW_BS0001642851041PW_BS000024226441PW_BS0000242905491PW_BS0000242231241PW_BS0000243081011PW_BS000024315123PW_BS0000243221231PW_BS0000243183123PW_BS000024253541PW_BS00002413412121PW_BS00013432914121PW_BS0000283331212PW_BS0000283361121PW_BS00002833217121PW_BS000028350114121PW_BS00002812815121PW_BS0001283511512PW_BS00002835325127PW_BS00002833527121PW_BS0000281151012PW_BS00011513013121PW_BS0001303317121PW_BS0000283344121PW_BS0000283683601PW_BS000028184121PW_BS0000241192171PW_BS00011911PW_BS000001124151PW_BS000124943PW_BS000094388161PW_BS000112109323PW_BS000109122551PW_BS000122407251PW_BS0001153821451PW_BS000100412125PW_BS000115429151PW_BS0001151231751PW_BS00012343311451PW_BS000115408451PW_BS0001154101551PW_BS0001151251351PW_BS000125383751PW_BS000100405105PW_BS0001154222751PW_BS000115435155PW_BS00011539914171PW_BS0001134461217PW_BS0001154641171PW_BS00011544717171PW_BS000115468114171PW_BS0001153744171PW_BS00005344415171PW_BS00011513613171PW_BS0001363987171PW_BS0001133761017PW_BS00005347225177PW_BS00011537527171PW_BS0000534701517PW_BS0001152975101PW_BS0000244793101PW_BS0001152991101PW_BS0000244812101PW_BS00011548414101PW_BS00011548515101PW_BS00011530013101PW_BS0000244957101PW_BS0001154781010PW_BS00011549127101PW_BS0001154991510PW_BS000115501361PW_BS0001153891461PW_BS0001125161561PW_BS0001153951361PW_BS000113390761PW_BS000112209106PW_BS0000245082761PW_BS000115517156PW_BS0001158911421PW_BS000552171211PW_BS000017111811PW_BS0000116131PW_BS0000061021231PW_BS00010216212181PW_BS000162224241PW_BS0000241951318PW_BS0000242491341PW_BS00002429341PW_BS0000242881441PW_BS0000243841251PW_BS0001004141551PW_BS00011512112171PW_BS00012145015171PW_BS00011548012101PW_BS0001154824101PW_BS0001153911261PW_BS000112502461PW_BS0001151861221PW_BS000024185321PW_BS00002430635511PW_BS000024372102PW_BS000028215114PW_BS000021432511PW_BS000043562611PW_BS00005621425181PW_BS00002435625121PW_BS00002815111PW_BS0000154192551PW_BS00011545525171PW_BS00011549025101PW_BS0001155072561PW_BS0001151572241PW_BS0001571141112PW_BS00011411612PW_BS000116409115PW_BS0001151371117PW_BS0001374831110PW_BS000115208116PW_BS0000246361PW_BS000063342612PW_BS00002842065PW_BS000115456617PW_BS000115135Phenylacetic acidHMDB0000209Phenyl acetate (or phenylacetate) is a carboxylic acid ester that has been found in the biofluids of patients with nephritis and/or hepatitis as well as patients with phenylketonuria (PKU). Excess phenylalanine in the body can be disposed of through a transamination process leading to the production of phenylpyruvate. The phenylpyruvate can be further metabolized into a number of products. Decarboxylation of phenylpyruvate gives phenylacetate, while a reduction reaction gives phenyllactate. The phenylacetate can be further conjugated with glutamine to give phenylacetyl glutamine. All of these metabolites can be detected in serum and urine of PKU patients. Phenyl acetate is also produced endogenously as the metabolite of 2-Phenylethylamine, which is mainly metabolized by monoamine oxidase to form phenyl acetate. 2-phenylethylamine is an "endogenous amphetamine" which may modulate central adrenergic functions, and the urinary phenyl acetate levels have been postulated as a marker for depression. (PMID: 17978765, 476920, 6857245). Phenylacetate is also found in essential oils, e.g. neroli, rose oil, free and as esters' and in many fruits. As a result it is used as a perfumery and flavoring ingredient.103-82-2C0708699930745PHENYLACETATE10181341OC(=O)CC1=CC=CC=C1C8H8O2InChI=1S/C8H8O2/c9-8(10)6-7-4-2-1-3-5-7/h1-5H,6H2,(H,9,10)WLJVXDMOQOGPHL-UHFFFAOYSA-N2-phenylacetic acid136.1479136.0524295-1.581ω-phenylacetic acid0-1FDB0105582-phenylacetate;2-phenylacetic acid;2-phenylethanoate;2-phenylethanoic acid;Benzeneacetate;Benzeneacetic acid;Benzylformate;Benzylformic acid;Omega-phenylacetate;Omega-phenylacetic acid;Phenylacetate;Phenylacetic acid;Phenylethanoate;Phenylethanoic acid;A-toluate;A-toluic acid;Alpha-toluate;Alpha-toluic acid;W-phenylacetate;W-phenylacetic acid;Pa;α-toluate;α-toluic acidPW_C000135PAA1171478458133120869406123445120414Adenosine 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_C000414ATP922146082661641422478137333279959343997632105182112102146492156142160582405592434272726462812293029663163723616613617514399234474314768914864545032895035265155752059752151005250104529110153131115346112539010354061175430118544312055421295556132556913356031355621108584614358541465876107589714759241516048155610916162301666493178683918868701606976199715720571842067209210722521372292117298198730221673902177408218743216374812227499190818622511847277119031701201028112039164121782851257822612691290132642231532730842326315426213224269431877028253772181347723332977468333776323367803733278041350781681287821435178240353784113357849411578850130788653317891933480028368800461848067411985629194826124113234941132823881162801091199141221199924061201544071202453821203624121212464291213921231213974331214714081219744101220651251220793831220834051224024221224444351229193991230094461238164641239514471239564681240293741245274441246161361246303981246343761249434721249723751250114701253042971253714791253922991255154811255954841261234851262203001262344951262404781265474911265964991269135011271233891277315161277813951277963901278012091281195081281675171407708911099Coenzyme 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_C001099CoA21143868845387922892172407592414224595281329286231334211335118461810462958484214486554487965232102524710452801035477124573410857771016023155607516163841646817869301606961162697319970831887108163729319873472107458222822915190812269090224912417092151951301329915318249254884942616315769072937711913377222134772303297729211177550132775553347756311277633336776721297799611578047332780563507841333578567130792593337997433180005368806201188062737480635119806653769382838293834383986742881105553891105613901158423991158473981199514061201474051202313841203051221206344071207621171214061231214214331215211251216664291216824081217144141224044221227411201229041211229601351239654471239794681240791361242204641242654501249743751253414791255094781255794801255924841256342971260844811265494911265604821267463001268845011270462091271093911273012051275402061276673881281215081281335021283403951407511861407631851407678912778Phenylacetyl-CoAHMDB0006503Phenylacetyl-CoA was found to be a very potent inhibitor of choline acetyltransferase, competitive for acetyl-CoA with Ki of 3.1 X 10(-7)M. Phenylacetate exerts its neurotoxic action through its metabolic product, phenylacetyl-CoA, which could severely decrease the availability of acetyl-CoA (PMID: 6142928).7532-39-0C005821656201553734-DIHYDROXYPHENYLACETYL-COA145148CC(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@@H](O)C(=O)NCCC(=O)NCCSC(=O)CC1=CC=CC=C1C29H42N7O17P3SInChI=1S/C29H42N7O17P3S/c1-29(2,24(40)27(41)32-9-8-19(37)31-10-11-57-20(38)12-17-6-4-3-5-7-17)14-50-56(47,48)53-55(45,46)49-13-18-23(52-54(42,43)44)22(39)28(51-18)36-16-35-21-25(30)33-15-34-26(21)36/h3-7,15-16,18,22-24,28,39-40H,8-14H2,1-2H3,(H,31,37)(H,32,41)(H,45,46)(H,47,48)(H2,30,33,34)(H2,42,43,44)/t18-,22-,23-,24+,28-/m1/s1ZIGIFDRJFZYEEQ-CECATXLMSA-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-[(2-phenylacetyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid885.667885.157073179-2.449phenylacetyl-coa0-4FDB023945Coenzyme a, s-(benzeneacetate);Phenylacetyl coenzyme a;Phenylacetyl-coenzyme a;Coenzyme a, s-(benzeneacetic acid)PW_C002778PhnaCoA1172478459133120870406123446120170PyrophosphateHMDB0000250The 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_C000170Ppi12235463842923735328822212173162049241059281529417514486854503489525210452941015409117542410354331185458120554811155591325584133560613556551085879107623916669781997073188713416372721607312198731821382751518283210118691611200222212041164123152251232324912512288125792261269529015219306153751834760174256131542697318772353297731712877635336784163357892833179153112799501347995813080047372804171708563019478638494814125948193829867822311063439111327039511327538911552713611553239911993412212001712412003240612033041012093640712126142912134112112148638312240742212298544412350211912383146412404439812497737512532429712539529912541047912559748412565648512587648112655249112686920512693538812695050112733720612812450814077289132Adenosine 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_C000032AMP112344628270167343288122118914457254867545033895251104540811754231035432118545712055581325583133577910157951086977199707218811789198118681611198815112003222125802261263631126942901333122542266342646315772343297732511178392334788091157932011280399180684135809007119916122120016124120031406120246382120888405121954408122920399123464376124507374125306297125394299125409479125596484126853205126934388126949501127124389127311209127711502140771891423MagnesiumHMDB0000547Magnesium salts are essential in nutrition, being required for the activity of many enzymes, especially those concerned with oxidative phosphorylation. Physiologically, it exists as an ion in the body. It is a component of both intra- and extracellular fluids and is excreted in the urine and feces. Deficiency causes irritability of the nervous system with tetany, vasodilatation, convulsions, tremors, depression, and psychotic behavior. Magnesium ion in large amounts is an ionic laxative, and magnesium sulfate (Epsom salts) is sometimes used for this purpose. So-called "milk of magnesia" is a water suspension of one of the few insoluble magnesium compounds, magnesium hydroxide; the undissolved particles give rise to its appearance and name. Milk of magnesia is a mild base, and is commonly used as an antacid.22537-22-0C003058881842013-HYDROXY-MAGNESIUM-PROTOPORP865DB01378[Mg++]MgInChI=1S/Mg/q+2JLVVSXFLKOJNIY-UHFFFAOYSA-Nmagnesium(2+) ion24.30523.9850418980magnesium(2+) ion22FDB003518Magnesium;Magnesium ions;Magnesium ion;Magnesium, doubly charged positive ion;Magnesium, ion (mg(2+));Mg(2+);Mg2+PW_C000423Mg2+86822742681647627272681158191888322936399833992211167461483491529431764142124102411592942233126293373745403147749148695449745652531045329111535611253761035906147593415160381556094161625016664841786594164688116069791997170205719420672272137233211725021473102167313198747322211763132118432101231222512324249125132881258122612729290152752851533730877137133772363297793733678393334784173357848911578522331785363567857413080020368800451848004837280623118806541358086515809652538184151938323839490027108596223110559390115687398119974406120070122120247382120702407120981408121181124121265429121319419121924125122086405122408422122759120122921399123307119123546374123835464123889455124477136124637376124978375125447297125598484125669479125777481125921482125947299125973495126000490126243478126553491126753300127125389127164501127380502127407388127451507127804209128125508128347395140773891500L-GlutamineHMDB0000641Glutamine (Gln) is one of the 20 amino acids encoded by the standard genetic code. Its side chain is an amide; it is formed by replacing a side-chain hydroxyl of glutamic acid with an amine functional group. glutamine is found in foods high in proteins, such as fish, red meat, beans, and dairy products. glutamine is a supplement that is used in weightlifting, bodybuilding, endurance and other sports, as well as by those who suffer from muscular cramps or pain particularly elderly people. The main use of glutamine within the diet of either group is as a means of replenishing the body's stores of amino acids that have been used during exercise or everyday activities. Studies which are looking into problems with excessive consumption of glutamine thus far have proved inconclusive. However, normal supplementation is healthy mainly because glutamine is supposed to be supplemented after prolonged periods of exercise (for example, a workout or exercise in which amino acids are required for use) and replenishes amino acid stores; this being the main reason glutamine is recommended during fasting or for people who suffer from physical trauma, immune deficiencies, or cancer. There is a significant body of evidence that links glutamine-enriched diets with intestinal effects; aiding maintenance of gut barrier function, intestinal cell proliferation and differentiation, as well as generally reducing septic morbidity and the symptoms of Irritable Bowel Syndrome. The reason for such "cleansing" properties is thought to stem from the fact that the intestinal extraction rate of glutamine is higher than that for other amino acids, and is therefore thought to be the most viable option when attempting to alleviate conditions relating to the gastrointestinal tract. These conditions were discovered after comparing plasma concentration within the gut between glutamine-enriched and non glutamine-enriched diets. However, even though glutamine is thought to have "cleansing" properties and effects, it is unknown to what extent glutamine has clinical benefits, due to the varied concentrations of glutamine in varieties of food. It is also known that glutamine has various effects in reducing healing time after operations. Hospital waiting times after abdominal surgery are reduced by providing parenteral nutrition regimens containing amounts of glutamine to patients. Clinical trials have revealed that patients on supplementation regimes containing glutamine have improved nitrogen balances, generation of cysteinyl-leukotrienes from polymorphonuclear neutrophil granulocytes and improved lymphocyte recovery and intestinal permeability (in postoperative patients) - in comparison to those who had no glutamine within their dietary regime; all without any side-effects. (http://en.wikipedia.org/wiki/glutamine).56-85-9C00064596118050GLN5746DB00130N[C@@H](CCC(N)=O)C(O)=OC5H10N2O3InChI=1S/C5H10N2O3/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H2,7,8)(H,9,10)/t3-/m0/s1ZDXPYRJPNDTMRX-VKHMYHEASA-N(2S)-2-amino-4-carbamoylbutanoic acid146.1445146.069142196-0.173L-glutamine00FDB012164(2s)-2,5-diamino-5-oxopentanoate;(2s)-2,5-diamino-5-oxopentanoic acid;(2s)-2-amino-4-carbamoylbutanoate;(2s)-2-amino-4-carbamoylbutanoic acid;(s)-2,5-diamino-5-oxopentanoate;(s)-2,5-diamino-5-oxopentanoic acid;2-aminoglutaramic acid;Cebrogen;Glavamin;Glumin;Glutamic acid 5-amide;Glutamic acid amide;Glutamine;L-(+)-glutamine;L-2-aminoglutaramic acid;L-2-aminoglutaramidic acid;L-glutamic acid 5-amide;L-glutamic acid gamma-amide;L-glutamid;L-glutamide;L-glutamin;L-glutamine;L-glutaminsaeure-5-amid;Levoglutamid;Levoglutamida;Levoglutamide;Levoglutamidum;Levoglutamina;Polyglutamine;Stimulina;Gamma-glutamine;Q;Glutamate 5-amide;Glutamate amidePW_C000500Gln61844342701230211556581075659108606815768461666847383902251179319812692290423523184235331577016253772961117733313378395132791641147926411680652135113207941162071091200381221203484061212194091219611241229981201237891371245141181254162971256684791263592991268124831269562051271635011277183881284112082728PhenylacetylglutamineHMDB0006344Phenylacetylglutamine is a product formed from the conjugation of phenylacetate and glutamine. Technically, it is the amino acid acetylation product of phenylacetate (or phenylbutyrate after beta-oxidation). Phenylacetylglutamine is a normal constituent of human urine, but other mammals such as the dog, cat, rat, monkey, sheep, and horse do not excrete this compound. Phenylacetyl-CoA and L-glutamine react to form phenylacetylglutamine and coenzyme A. The enzyme (glutamine N-acetyl transferase) that catalyzes this reaction has been purified from human liver mitochondria and shown to be a polypeptide species distinct from glycine-N-acyltransferase. Phenylacetylglutamine is a major nitrogenous metabolite that accumulates in uremia (PMID: 2791363, 8972626). It has been shown that over 50% of urine phenylacetylglutamine may be derived from kidney conjugation of free plasma phenylacetic acid and/or from the kidney's preferential filtration of conjugated phenylacetic acid (PMID: 6420430).28047-15-6C041489225817884CPD-109783292NC(=O)CC[C@H](NC(=O)CC1=CC=CC=C1)C(O)=OC13H16N2O4InChI=1S/C13H16N2O4/c14-11(16)7-6-10(13(18)19)15-12(17)8-9-4-2-1-3-5-9/h1-5,10H,6-8H2,(H2,14,16)(H,15,17)(H,18,19)/t10-/m0/s1JFLIEFSWGNOPJJ-JTQLQIEISA-N(2S)-4-carbamoyl-2-(2-phenylacetamido)butanoic acid264.2771264.11100701-2.833phenylacetylglutamine0-1FDB023896N2-(2-Phenylacetyl)-L-glutamine;N(2)-(2-Phenylacetyl)-L-glutamine;N-Phenylacetylglutamine;N2-(Phenylacetyl)-L-glutamine;N(2)-(Phenylacetyl)-L-glutamine;Phenylacetyl-L-glutamine;α-N-Phenylacetylglutamine;α-N-Phenylacetyl-L-glutamine;alpha-N-Phenylacetylglutamine;alpha-N-Phenylacetyl-L-glutamine;PW_C002728Phace11744434924350297846013378461132784623367934963796853421208711241208734201234471181234494562405Acyl-coenzyme A synthetase ACSM1, mitochondrialQ08AH1Has medium-chain fatty acid:CoA ligase activity with broad substrate specificity (in vitro). Acts on acids from C(4) to C(11) and on the corresponding 3-hydroxy- and 2,3- or 3,4-unsaturated acids (in vitro). Functions as GTP-dependent lipoate-activating enzyme that generates the substrate for lipoyltransferase (By similarity).
HMDBP04188ACSM116p12.3AB06250316.2.1.26054487054144123262591Glycine N-acyltransferaseQ6IB77Mitochondrial acyltransferase which transfers an acyl group to the N-terminus of glycine and glutamine, although much less efficiently. Can conjugate numerous substrates to form a variety of N-acylglycines, with a preference for benzoyl-CoA over phenylacetyl-CoA as acyl donors. Thereby detoxify xenobiotics, such as benzoic acid or salicylic acid, and endogenous organic acids, such as isovaleric acid.
HMDBP07349GLYAT11q12.1AF02346612.3.1.13; 2.3.1.7111754140964311440646144124263916Acyl-coenzyme A synthetase ACSM2B, mitochondrialQ68CK6Has medium-chain fatty acid:CoA ligase activity with broad substrate specificity (in vitro). Acts on acids from C(4) to C(11) and on the corresponding 3-hydroxy- and 2,3- or 3,4-unsaturated acids (in vitro).
HMDBP08700ACSM2B16p12.3AC14105316.2.1.211734183Acyl-coenzyme A synthetase ACSM1, mitochondrial1PW_P00018320124051814231323Glycine N-acyltransferase1PW_P00032334425911322Acyl-coenzyme A synthetase ACSM2B, mitochondrial1PW_P000322343391611434231842falsePW_R000842Right34111351Compoundfalse34124141Compoundtrue341310991Compoundtrue341427781Compoundfalse34151701Compoundtrue3416321Compoundtrue3921836.2.1.23933226.2.1.2843falsePW_R000843Right341727781Compoundfalse34185001Compoundfalse341927281Compoundfalse342010991Compoundtrue394323228PW_T00022826527281Compound263Right1901135481false43086510regular2001901902414442false69072510regular503019031099485false61573010regular503019042778481false104086810regular2001901905170445false96871610regular6343190632444false90072110regular5030190742349false77075510regular100251909500481false1320101310regular20019019111099485false1640103310regular50302438414442false675118010regular503024391099485false610119510regular50302440170445false963118310regular6343244132444false905118810regular5030244242349false770116010regular1002515811942728281false174887010regular200190158119527286381false1748186010regular200190733240542false7457708subunitregular15070735259142false14709288subunitregular15070902391642false74511008subunitregular1507068418343472673328819072757Cofactor68632343472873585032243489590239124423572Cofactor2751M530 865 C530 836 529 823 530 806 C574 806 697 807 745 805 5false18trueM 341.15380027238456 737.7904550494351 L 328 745 L 340.8205492131073 752.7867527169092false2752M715 755 C715 780 715 805 745 805 5false182753M640 760 C642 791 710 804 745 805 5false182754M1140 868 C1140 834 1140 829 1140 801 C1055 803 1024 803 895 805 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2755M999.5 759 C999.5 803 925 805 895 805 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2756M925 751 C925 778 925 805 895 805 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2757M400 300 L400 350 L450 300 z10true183566M530 1055 C531 1099 531 1079 530 1138 C564 1139 661 1137 745 1135 5false18trueM 256.7069699286199 986.5507462441477 L 271 982 L 259.9124231107014 971.8972459832038false3567M700 1180 C700 1160 715 1135 745 1135 5false183568M635 1195 C638 1160 715 1135 745 1135 5false183569M1140 1058 C1141 1085 1141 1092 1143 1134 C1091 1135 969 1136 895 1135 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3570M994.5 1183 C989.5 1146 925 1135 895 1135 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3571M930 1188 C930 1166 925 1135 895 1135 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3572M400 300 L400 350 L450 300 z10true183573M1420 1203 C1420 1233 1420 1253 1420 1283 5false18trueM 1117.5 990.9903810567666 L 1110 978 L 1102.5 990.9903810567666false3574M1520 1108 C1616 1108 1640 1157 1640 1218 5false18trueM 1172.9903810567666 920.5 L 1160 928 L 1172.9903810567666 935.5false2154197M1828 1065 C1828 1095 1848 1830 1848 1860 83true182154198M1848 1860 C1850 1712 1846 1153 1848 1060 83false18trueM 838.9468550441649 643.261556296296 L 824 642 L 830.3808877211858 655.5751343230784false2154199M1240 963 C1270 963 1440 963 1470 963 5false182154200M1420 1013 C1421 979 1440 963 1470 963 5false182154201M1748 965 C1718 965 1650 963 1620 963 5false18trueM 927.9468550441649 605.261556296296 L 913 604 L 919.3808877211858 617.5751343230784false2154202M1665 1033 C1660 988 1650 963 1620 963 5false18trueM 927.9468550441649 605.261556296296 L 913 604 L 919.3808877211858 617.5751343230784false555438424204619012751Left204719022752Left204819032753Left204919042754Right205019052755Right205119062756Right531392684743438424268019013566Left268124383567Left268224393568Left268319043569Right268424403570Right268524413571Right685393850486631438434195089119042154199Left195089219092154200Left195089315811942154201Right195089419112154202Right44519839468622831228434636915811942154197Left4637015811952154198Right123854314false1345128316regular9519093573Right124864314false1565121816regular9619093574Right901316251751.31.3031432726761371164016701.31.30282924004798186003250.60.602149024047981917804501.01.0-20523280360125M123 225 C123 175 173 125 223 125 C814 125 1582 125 2173 125 C2223 125 2273 175 2273 225 C2273 614 2273 1120 2273 1509 C2273 1559 2223 1609 2173 1609 C1582 1609 814 1609 223 1609 C173 1609 123 1559 123 1509 C123 1120 123 614 123 225 1true62150.01484.0475375M275 696 C275 646 325 596 375 596 C875 596 1526 596 2026 596 C2076 596 2126 646 2126 696 C2126 908 2126 1183 2126 1395 C2126 1445 2076 1495 2026 1495 C1526 1495 875 1495 375 1495 C325 1495 275 1445 275 1395 C275 1183 275 908 275 696 84true61851.0899.0475376M376 762 C376 712 426 662 476 662 C921 662 1499 662 1944 662 C1994 662 2044 712 2044 762 C2044 925 2044 1137 2044 1300 C2044 1350 1994 1400 1944 1400 C1499 1400 921 1400 476 1400 C426 1400 376 1350 376 1300 C376 1137 376 925 376 762 84true61668.0738.0132235Intracellular Space860195201.31.320015133235Extracellular Space91020201.31.320015621235Kidney18551710201.01.020015704235Mitochondria585540201.31.320015890901235Mitochondrial Matrix1395710201.31.32001546730386254567215315224#FFEBEB41899955