71PathwayPhytanic Acid Peroxisomal Oxidation Phytanic acid, a branched chain fatty acid, is an important component of fatty acid intake, occuring in meat, fish and dairy products. Due to its methylation, it cannot be a substrate for acyl-CoA dehydrogenase and cannot enter the mitochondrial beta oxidation pathway. Phytanic acid is instead activated to its CoA ester form by a CoA synthetase to phytanoyl-CoA, where it can begin the first cycle of alpha oxidation. Phytanoyl-CoA is a substrate for a specific alpha-hydroxylase (Phytanoyl-CoA hydroxylase), which adds a hydroxyl group to the α-carbon of phytanic acid, creating the 19-carbon homologue, pristanic acid. Pristanic acid then undergoes further metabolism through beta oxidation. MetabolicPW000041CenterPathwayVisualizationContext4146002200#000099PathwayVisualization5171Phytanic Acid Peroxisomal Oxidation Phytanic acid, a branched chain fatty acid, is an important component of fatty acid intake, occuring in meat, fish and dairy products. Due to its methylation, it cannot be a substrate for acyl-CoA dehydrogenase and cannot enter the mitochondrial beta oxidation pathway. Phytanic acid is instead activated to its CoA ester form by a CoA synthetase to phytanoyl-CoA, where it can begin the first cycle of alpha oxidation. Phytanoyl-CoA is a substrate for a specific alpha-hydroxylase (Phytanoyl-CoA hydroxylase), which adds a hydroxyl group to the α-carbon of phytanic acid, creating the 19-carbon homologue, pristanic acid. Pristanic acid then undergoes further metabolism through beta oxidation. Metabolic167Beta OxidationSubPathway128635Compound5129940Compound8130988Compound8131960Compound875471SubPathway1244635Compound51CellCL:00000002Platelet CL:00002335HepatocyteCL:00001823NeuronCL:00005404CardiomyocyteCL:00007468Beta cellCL:00006397Epithelial CellCL:00000666MyocyteCL:00001871Homo sapiens9606EukaryoteHuman12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat10Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm2Bacteria2ProkaryoteBacteria3Escherichia coli562Prokaryote19Schizosaccharomyces pombe4896Eukaryote24Solanum lycopersicum4081EukaryoteTomato4Arabidopsis thaliana3702EukaryoteThale cress18Saccharomyces cerevisiae4932EukaryoteYeast21Xenopus laevis8355EukaryoteAfrican clawed frog25Escherichia coli (strain K12)83333Prokaryote49Bathymodiolus platifrons220390EukaryoteDeep sea mussel23Pseudomonas aeruginosa287Prokaryote60Nitzschia sp.0001EukaryoteNitzschia451Picea sitchensis3332EukaryoteSitka spruce5CytoplasmGO:000573727Peroxisome MembraneGO:00057781CytosolGO:00058293Mitochondrial MatrixGO:000575914Mitochondrial Outer MembraneGO:00057412MitochondrionGO:000573915NucleusGO:00056344PeroxisomeGO:000577713Endoplasmic ReticulumGO:00057837Endoplasmic Reticulum MembraneGO:000578910Cell MembraneGO:000588631Periplasmic SpaceGO:000562011Extracellular SpaceGO:000561535ChloroplastGO:000950712Mitochondrial Inner MembraneGO:000574332Inner MembraneGO:00702588Smooth Endoplasmic Reticulum GO:000579019Sarcoplasmic ReticulumGO:001652924Mitochondrial Intermembrane SpaceGO:00057586LysosomeGO:000576416Lysosomal LumenGO:004320218Melanosome MembraneGO:003316236MembraneGO:001602025Golgi ApparatusGO:000579426Golgi Apparatus MembraneGO:000013920Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520253Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032540PeriplasmGO:004259739Mitochondrial membraneGO:00319662Endothelium BTO:00003931LiverBTO:00007597297Nervous SystemBTO:000148418PancreasBTO:000098825IntestineBTO:00006488Blood VesselBTO:000110274119MuscleBTO:00008871411824BrainBTO:000014289164Adrenal MedullaBTO:000004971828StomachBTO:0001307155265cardiocyteBTO:000153911HeartBTO:000056273106KidneyBTO:00006717188511PW_BS000008592711PW_BS00005933527121PW_BS0000281115121PW_BS000111122551PW_BS0001224222751PW_BS0001151355171PW_BS00013537527171PW_BS0000532975101PW_BS00002449127101PW_BS000115205561PW_BS0000245082761PW_BS0001152111PW_BS0000024311PW_BS00000416212PW_BS000016221411PW_BS00002213121PW_BS0000133211515PW_BS0000325411PW_BS000005397113PW_BS0000393211PW_BS000003181311PW_BS000018101711PW_BS00001049711PW_BS00004914101PW_BS0000145811411PW_BS00005827151PW_BS00002746114PW_BS00004629111PW_BS0000296618518PW_BS00006672513PW_BS000072612517PW_BS0000615181PW_BS000051231511PW_BS000023311511PW_BS000031918511PW_BS000091541315PW_BS000054892PW_BS000089261115PW_BS000026711PW_BS000007971521PW_BS000097100521PW_BS0001001041431PW_BS000104101531PW_BS0001011122121PW_BS000112103331PW_BS000103117131PW_BS0001171181171PW_BS0001181203171PW_BS00012012915121PW_BS0001291321121PW_BS0001321333121PW_BS00013310813PW_BS00010814315191PW_BS0001431465191PW_BS000146107313PW_BS0001071471241PW_BS000147151141PW_BS0001511553241PW_BS0001551613181PW_BS00016116611PW_BS0001661783211PW_BS000178188118PW_BS0000241601181PW_BS00016019914181PW_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_BS0000281151012PW_BS00011513013121PW_BS0001303317121PW_BS0000283344121PW_BS0000283683601PW_BS000028184121PW_BS0000241192171PW_BS00011911PW_BS000001124151PW_BS000124943PW_BS000094388161PW_BS000112109323PW_BS000109406351PW_BS000115407251PW_BS0001153821451PW_BS000100412125PW_BS000115429151PW_BS0001151231751PW_BS00012343311451PW_BS000115408451PW_BS0001154101551PW_BS0001151251351PW_BS000125383751PW_BS000100405105PW_BS000115435155PW_BS00011539914171PW_BS0001134461217PW_BS0001154641171PW_BS00011544717171PW_BS000115468114171PW_BS0001153744171PW_BS00005344415171PW_BS00011513613171PW_BS0001363987171PW_BS0001133761017PW_BS00005347225177PW_BS0001154701517PW_BS0001154793101PW_BS0001152991101PW_BS0000244812101PW_BS00011548414101PW_BS00011548515101PW_BS00011530013101PW_BS0000244957101PW_BS0001154781010PW_BS0001154991510PW_BS000115501361PW_BS0001153891461PW_BS0001125161561PW_BS0001153951361PW_BS000113390761PW_BS000112209106PW_BS000024517156PW_BS0001158911421PW_BS000552171211PW_BS000017111811PW_BS0000116131PW_BS0000061021231PW_BS00010216212181PW_BS000162224241PW_BS0000241951318PW_BS0000242491341PW_BS00002429341PW_BS0000242881441PW_BS0000243841251PW_BS0001004141551PW_BS00011512112171PW_BS00012145015171PW_BS00011548012101PW_BS0001154824101PW_BS0001153911261PW_BS000112502461PW_BS0001151861221PW_BS000024185321PW_BS00002415111PW_BS000015471914PW_BS00004731323PW_BS00002430635511PW_BS000024372102PW_BS000028422411PW_BS000042509516PW_BS0000501572241PW_BS00015785241011PW_BS00008522014PW_BS0000242892491PW_BS00002434524121PW_BS00002834695126PW_BS00002832711125PW_BS00002834713125PW_BS0000284182451PW_BS0001154239556PW_BS0001154241155PW_BS0001154251355PW_BS00011545424171PW_BS00011545895176PW_BS00011545911175PW_BS00011546013175PW_BS00011548924101PW_BS0001155062461PW_BS0001159611PW_BS0000092811611PW_BS000028204111PW_BS000020331811PW_BS0000332441011PW_BS000024126651PW_BS00012612711651PW_BS00012715924PW_BS00015921217181PW_BS0000242156181PW_BS0000242863641PW_BS0000242916491PW_BS0000242924491PW_BS00002429817101PW_BS0000243016101PW_BS000024302116101PW_BS0000242941141PW_BS0000241136121PW_BS000113337116121PW_BS00002834141121PW_BS0000281141112PW_BS00011434318121PW_BS000028360410121PW_BS000028409115PW_BS0001154151851PW_BS00011543441051PW_BS0001154436171PW_BS000115448116171PW_BS0001151371117PW_BS00013745118171PW_BS000115469410171PW_BS0001154831110PW_BS000115207661PW_BS000024208116PW_BS0000245041861PW_BS00011551541061PW_BS000115219314PW_BS000024432511PW_BS0000437028511PW_BS00007035625121PW_BS0000284192551PW_BS00011545525171PW_BS00011549025101PW_BS0001155072561PW_BS000115215114PW_BS000021562611PW_BS00005621425181PW_BS000024951721PW_BS00009514117191PW_BS00014160251PW_BS0000603612011PW_BS0000363772113PW_BS00003793252011PW_BS000093105113PW_BS000105110231PW_BS000110140103PW_BS0001401802211PW_BS00018015284PW_BS0001522875341PW_BS0000242273441PW_BS00002465111PW_BS0000653522512PW_BS0000283702601PW_BS000028228361PW_BS000024232403PW_BS000024436255PW_BS0001154712517PW_BS00011548718101PW_BS0001155131761PW_BS0001157906111PW_BS0005248346111PW_BS0005493093911PW_BS000024639Phytanic acidHMDB0000801Phytanic acid (or 3,7,11,15-tetramethylhexadecanoic acid) is a 20-carbon branched-chain fatty acid that humans can obtain through the consumption of dairy products, ruminant animal fats, and certain fish. It is primarily formed by bacterial degradation of chlorophyll in the intestinal tract of ruminants. Unlike most fatty acids, phytanic acid cannot be metabolized by beta-oxidation (because of a methyl group in the beta position). Instead, it undergoes alpha-oxidation in the peroxisome, where it is converted into pristanic acid by the removal of one carbon. Pristanic acid can undergo several rounds of beta-oxidation in the peroxisome to form medium-chain fatty acids that can be converted into carbon dioxide and water in mitochondria. Refsum disease, an autosomal recessive neurological disorder caused by mutations in the PHYH gene, is characterized by having impaired alpha-oxidation activity. Individuals with Refsum disease accumulate large stores of phytanic acid in their blood and tissues. This frequently leads to peripheral polyneuropathy, cerebellar ataxia, retinitis pigmentosa, anosmia, and hearing loss. Therefore, chronically high levels of phytanic acid can be neurotoxic. Phytanic acid's neurotoxicity appears to lie in its ability to initiate astrocyte/neural cell death by activating the mitochondrial route of apoptosis. In particular, phytanic acid can induce the substantial generation of reactive oxygen species in isolated mitochondria as well as in intact cells. It also induces the release of cytochrome c from mitochondria.14721-66-5C01607268401628525001CC(C)CCCC(C)CCCC(C)CCCC(C)CC(O)=OC20H40O2InChI=1S/C20H40O2/c1-16(2)9-6-10-17(3)11-7-12-18(4)13-8-14-19(5)15-20(21)22/h16-19H,6-15H2,1-5H3,(H,21,22)RLCKHJSFHOZMDR-UHFFFAOYSA-N3,7,11,15-tetramethylhexadecanoic acid312.5304312.302830524-6.581phytanic acid0-1FDB0222523,7,11,15-tetramethyl-hexadecanoate;3,7,11,15-tetramethyl-hexadecanoic acid;3,7,11,15-tetramethyl-hexadecansaeure;3,7,11,15-tetramethylhexadecanoate;3,7,11,15-tetramethylhexadecanoic acid;3,7,11,15-tetramethylhexadecoanoate;3,7,11,15-tetramethylhexadecoanoic acid;Phytanate;Phytanoate;Phytanoic acid;3,7,11,15-tetramethyl hexadecanoic acid;3,7,11,15-tetramethyl hexadecanoatePW_C000639Phytana126082485597843433578552111120975122122419422123540135124987375125915297126566491127374205128139508414Adenosine 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_C001099CoA21143868845387922892172407592414224595281329286231334211335118461810462958484214486554487965232102524710452801035477124573410857771016023155607516163841646817869301606961162697319970831887108163729319873472107458222822915190812269090224912417092151951301329915318249254884942616315769072937711913377222134772303297729211177550132775553347756311277633336776721297799611578047332780563507841333578567130792593337997433180005368806201188062737480635119806653769382838293834383986742881105553891105613901158423991158473981199514061201474051202313841203051221206344071207621171214061231214214331215211251216664291216824081217144141224044221227411201229041211229601351239654471239794681240791361242204641242654501249743751253414791255094781255794801255924841256342971260844811265494911265604821267463001268845011270462091271093911273012051275402061276673881281215081281335021283403951407511861407631851407678911049Phytanoyl-CoAHMDB0001359Phytanoyl CoA is a coenzyme A derivative of phytanic acid. Phytanic acid is present in human diet or in animal tissues where it may be derived from chlorophyll in plant extracts. Specifically it is an epimeric metabolite of the isoprenoid side chain of chlorophyll. Owing to the presence of its epimeric beta-methyl group, phytanic acid cannot be metabolized by beta-oxidation. Instead, it is metabolized in peroxisomes via alpha-oxidation to give pristanic acid, which is then oxidized by beta-oxidation. PhyH (phytanoyl-CoA 2-hydroxylase) catalyses hydroxylation of phytanoyl-CoA. Mutations of PhyH can lead to phytanic acid accumulation. High levels of phytanic acid are found in patients suffering from Refsum's syndrome. This inherited neurological disorder is characterized by an accumulation of phytanic acid in blood and tissues. Clinically it is characterized by adult onset retinitis pigmentosa, anosmia, sensory neuropathy, and phytanic acidaemia. This disorder has been found to be related to deficiency in the α-oxidation pathway in the liver. (PMID: 17956235). Phytanoyl CoA and other branched-chain fatty acid CoA products are potent inducers of the peroxisome proliferator-activated receptor PPARalpha, a nuclear receptor that enhances transcription of peroxisomal enzymes mediating beta-oxidation of these potentially toxic fatty acids (PMID: 16768463). Pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase are strongly inhibited by phytanoyl-CoA. Decreased activity of these important mitochondrial metabolism complexes might therefore contribute to neurological symptoms upon accumulation of phytanic acid in Refsum disease (PMID: 16737698).146622-45-9C0206043964015538CPD-206388712CC(C)CCC[C@@H](C)CCC[C@@H](C)CCC[C@H](C)CC(=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=C2NC41H74N7O17P3SInChI=1S/C41H74N7O17P3S/c1-26(2)11-8-12-27(3)13-9-14-28(4)15-10-16-29(5)21-32(50)69-20-19-43-31(49)17-18-44-39(53)36(52)41(6,7)23-62-68(59,60)65-67(57,58)61-22-30-35(64-66(54,55)56)34(51)40(63-30)48-25-47-33-37(42)45-24-46-38(33)48/h24-30,34-36,40,51-52H,8-23H2,1-7H3,(H,43,49)(H,44,53)(H,57,58)(H,59,60)(H2,42,45,46)(H2,54,55,56)/t27-,28-,29+,30-,34-,35-,36?,40-/m1/s1NRJQGHHZMSOUEN-IYJVDCLDSA-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-{[(3S,7R,11R)-3,7,11,15-tetramethylhexadecanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid1062.0491061.407474203-3.149[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3-hydroxy-2,2-dimethyl-3-({2-[(2-{[(3S,7R,11R)-3,7,11,15-tetramethylhexadecanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)propoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid0-4FDB0225773,7,11,15-tetramethyl hexadecanoyl coa;3,7,11,15-tetramethyl hexadecanoyl coenzyme a;Phytanoyl coa;Phytanoyl coenzyme a;Phytanoyl-coa;Phytanoyl-coenzyme a;Phytanyl coa;Phytanyl coenzyme aPW_C001049PhytCoA1261824285248427842433478435132785531111209761221209884081224201241235411351235533741249881181259162971259284821265672991273752051273875021281403881034Adenosine 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_C001034ADP234134841522482138015963159783106114151821901492104182113102161582408592435272728472736462855293165723635614400234476314770915036265157752089752171005315111534911253921035446120554412955721335624108574111757641015849143585614658781075899147592615160501556111161623116664951786700946841188687216071592057187206720821072262137231211730019873032167391217741021874331637483222818722511851277119051701201328112180285132622231532930842328315423983134262232242696318770292537708713277216134773063297747233377663336780393327804335078170128782153517824435378414335784951157870533178849130789203348003036880622118806511358067611994827124113283388116204109119944122119994406120156407120318382120366412121248429121394123121399433121472408121899383121976410122064125122085405122405422122445435122973399123013446123818464123953447123958468124030374124452398124529444124615136124636376124947472124975375125012470125334297125373479125492299125517481125645484126125485126219300126235495126242478126550491126597499126915501127733516127780395127797390127803209128122508128168517128313389170PyrophosphateHMDB0000250The 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_C000170Ppi122354638429237353288222121731620492410592815294175144868545034895252104529410154091175424103543311854581205548111555913255841335606135565510858791076239166697819970731887134163727216073121987318213827515182832101186916112002222120411641231522512323249125122881257922612695290152193061537518347601742561315426973187723532977317128776353367841633578928331791531127995013479958130800473728041717085630194786384948141259481938298678223110634391113270395113275389115527136115532399119934122120017124120032406120330410120936407121261429121341121121486383122407422122985444123502119123831464124044398124977375125324297125395299125410479125597484125656485125876481126552491126869205126935388126950501127337206128124508140772891134Oxoglutaric 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_C000134AKG15242314141468499186733111084212635144750145526146754537510354141175438118556413260081476036155606915760921616482178653085747122275152247519151820922583742201186319812681289770542537713513377481111775231127774612977967345779703467797632777984347784253348001836880694135113162941199724061200221241200844071201741221205524141208144181209894081211464231211524241211604251227571201228311191231864501233994541235543741237184581237244591237324601253574791254002991254554811255332971258004891259294821269005011269403881269932061270662051272555061273885021065OxygenHMDB0001377Oxygen is the third most abundant element in the universe after hydrogen and helium and the most abundant element by mass in the Earth's crust. Diatomic oxygen gas constitutes 20.9% of the volume of air. All major classes of structural molecules in living organisms, such as proteins, carbohydrates, and fats, contain oxygen, as do the major inorganic compounds that comprise animal shells, teeth, and bone. Oxygen in the form of O2 is produced from water by cyanobacteria, algae and plants during photosynthesis and is used in cellular respiration for all living organisms. Green algae and cyanobacteria in marine environments provide about 70% of the free oxygen produced on earth and the rest is produced by terrestrial plants. Oxygen is used in mitochondria to help generate adenosine triphosphate (ATP) during oxidative phosphorylation. For animals, a constant supply of oxygen is indispensable for cardiac viability and function. To meet this demand, an adult human, at rest, inhales 1.8 to 2.4 grams of oxygen per minute. This amounts to more than 6 billion tonnes of oxygen inhaled by humanity per year. At a resting pulse rate, the heart consumes approximately 8-15 ml O2/min/100 g tissue. This is significantly more than that consumed by the brain (approximately 3 ml O2/min/100 g tissue) and can increase to more than 70 ml O2/min/100 g myocardial tissue during vigorous exercise. As a general rule, mammalian heart muscle cannot produce enough energy under anaerobic conditions to maintain essential cellular processes; thus, a constant supply of oxygen is indispensable to sustain cardiac function and viability. However, the role of oxygen and oxygen-associated processes in living systems is complex, and they and can be either beneficial or contribute to cardiac dysfunction and death (through reactive oxygen species). Reactive oxygen species (ROS) are a family of oxygen-derived free radicals that are produced in mammalian cells under normal and pathologic conditions. Many ROS, such as the superoxide anion (O2-)and hydrogen peroxide (H2O2), act within blood vessels, altering mechanisms mediating mechanical signal transduction and autoregulation of cerebral blood flow. Reactive oxygen species are believed to be involved in cellular signaling in blood vessels in both normal and pathologic states. The major pathway for the production of ROS is by way of the one-electron reduction of molecular oxygen to form an oxygen radical, the superoxide anion (O2-). Within the vasculature there are several enzymatic sources of O2-, including xanthine oxidase, the mitochondrial electron transport chain, and nitric oxide (NO) synthases. Studies in recent years, however, suggest that the major contributor to O2- levels in vascular cells is the membrane-bound enzyme NADPH-oxidase. Produced O2- can react with other radicals, such as NO, or spontaneously dismutate to produce hydrogen peroxide (H2O2). In cells, the latter reaction is an important pathway for normal O2- breakdown and is usually catalyzed by the enzyme superoxide dismutase (SOD). Once formed, H2O2 can undergo various reactions, both enzymatic and nonenzymatic. The antioxidant enzymes catalase and glutathione peroxidase act to limit ROS accumulation within cells by breaking down H2O2 to H2O. Metabolism of H2O2 can also produce other, more damaging ROS. For example, the endogenous enzyme myeloperoxidase uses H2O2 as a substrate to form the highly reactive compound hypochlorous acid. Alternatively, H2O2 can undergo Fenton or Haber-Weiss chemistry, reacting with Fe2+/Fe3+ ions to form toxic hydroxyl radicals (-.OH). (PMID: 17027622, 15765131).7782-44-7C0000797715379CPD-6641952O=OO2InChI=1S/O2/c1-2MYMOFIZGZYHOMD-UHFFFAOYSA-Ndioxygen31.998831.9898292440singlet oxygen00FDB022589Dioxygen;Molecular oxygen;O2;Oxygen;Oxygen molecule;[oo];Dioxygene;Disauerstoff;E 948;E-948;E948PW_C001065O2959110524516500185058549146252863836491067431688207541576347693383621375492016242531222803294260424747135467123548012554931265508127580910859731476129159700618870321637050160731921375332107560212839515111816216118641981188321511894211120572251206316412247286122792261232524912706291127162921300429813016300130263011303830213260223422761742657315769102937704429477214134773501117736313077377331773953327749711377512115775373347762633677723337777361127774712977756341778051147781213378070329781511327838134578805343791113601200474081203831221204264051205424071205534141205944091206014061208834151210451241211043831216054341216564291221173821225734181226893841227983741228224431230271351230603761231284471231391361231634481231761191231874501232191371232261201234594511236091181236693981241634691242144641246693991251454541252751211254254821257064781257314831257372971257404791258844811261002991262724841265224951267214891268254801269645021269862071271982091272142081272192051272225011273055041273452061275573881275745151278353891280813951280953901283125061284323919992-Hydroxyphytanoyl-CoAHMDB00012952-Hydroxyphytanoyl-CoA belongs to the class of organic compounds known as long-chain fatty acyl coas. These are acyl CoAs where the group acylated to the coenzyme A moiety is a long aliphatic chain of 13 to 21 carbon atoms. Thus, 2-hydroxyphytanoyl-CoA is considered to be a fatty ester lipid molecule. 2-Hydroxyphytanoyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). 2-Hydroxyphytanoyl-CoA has been primarily detected in urine. Within the cell, 2-hydroxyphytanoyl-CoA is primarily located in the peroxisome, cytoplasm and mitochondria. In humans, 2-hydroxyphytanoyl-CoA is involved in the oxidation OF branched chain fatty acids pathway and the phytanic Acid peroxisomal oxidation pathway. 2-Hydroxyphytanoyl-CoA is also involved in the metabolic disorder called the refsum disease pathway. 2-Hydroxyphytanoyl-CoA is a substrate for Phytanoyl-CoA dioxygenase (peroxisomal).172787-73-4C07343441263154752-HYDROXYPHYTANOYL-COA390032CC(C)CCC[C@@H](C)CCC[C@@H](C)CCC[C@H](C)C(O)C(=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=C2NC41H74N7O18P3SInChI=1S/C41H74N7O18P3S/c1-25(2)11-8-12-26(3)13-9-14-27(4)15-10-16-28(5)32(50)40(54)70-20-19-43-30(49)17-18-44-38(53)35(52)41(6,7)22-63-69(60,61)66-68(58,59)62-21-29-34(65-67(55,56)57)33(51)39(64-29)48-24-47-31-36(42)45-23-46-37(31)48/h23-29,32-35,39,50-52H,8-22H2,1-7H3,(H,43,49)(H,44,53)(H,58,59)(H,60,61)(H2,42,45,46)(H2,55,56,57)/t26-,27-,28+,29-,32?,33-,34-,35?,39-/m1/s1WNVFJMYPVBOLKV-PJDIVXIPSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[3-hydroxy-3-({2-[(2-{[(3S,7R,11R)-2-hydroxy-3,7,11,15-tetramethylhexadecanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)-2,2-dimethylpropoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid1078.0491077.402388825-2.8810[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3-hydroxy-3-({2-[(2-{[(3S,7R,11R)-2-hydroxy-3,7,11,15-tetramethylhexadecanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)-2,2-dimethylpropoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid0-4FDB022539(3s)-2-hydroxyphytanoyl-coa;(3s)-2-hydroxyphytanoyl-coenzyme a;(3s,7r,11r)-2-hydroxy-3,7,11,15-tetramethylhexadecanoyl-coa;(3s,7r,11r)-2-hydroxy-3,7,11,15-tetramethylhexadecanoyl-coenzyme a;2-hydroxyphytanoyl-coa;2-hydroxyphytanoyl-coenzyme a;3(s)-2-hydroxyphytanoyl-coa;3(s)-2-hydroxyphytanoyl-coenzyme a;3s2hphy-coa;3s2hphy-coenzyme aPW_C0009992HPCoA1264578421334120978408123543374125918482127377502174Succinic acidHMDB0000254Succinic acid is a dicarboxylic acid. The anion, succinate, is a component of the citric acid cycle capable of donating electrons to the electron transfer chain. Succinate dehydrogenase (SDH) plays an important role in the mitochondria, being both part of the respiratory chain and the Krebs cycle. SDH with a covalently attached FAD prosthetic group, binds enzyme substrates (succinate and fumarate) and physiological regulators (oxaloacetate and ATP). Oxidizing succinate links SDH to the fast-cycling Krebs cycle portion where it participates in the breakdown of acetyl-CoA throughout the whole Krebs cycle. The succinate can readily be imported into the mitochondrial matrix by the n-butylmalonate- (or phenylsuccinate-) sensitive dicarboxylate carrier in exchange with inorganic phosphate or another organic acid, e. g. malate. (PMID 16143825) Mutations in the four genes encoding the subunits of the mitochondrial respiratory chain succinate dehydrogenase are associated with a wide spectrum of clinical presentations (i.e.: Huntington's disease. (PMID 11803021).110-15-6C00042111015741SUC1078DB00139OC(=O)CCC(O)=OC4H6O4InChI=1S/C4H6O4/c5-3(6)1-2-4(7)8/h1-2H2,(H,5,6)(H,7,8)KDYFGRWQOYBRFD-UHFFFAOYSA-Nbutanedioic acid118.088118.026608680.252succinic acid0-2FDB0019311,2-ethanedicarboxylate;1,2-ethanedicarboxylic acid;1,4-butanedioate;1,4-butanedioic acid;Amber acid;Asuccin;Dihydrofumarate;Dihydrofumaric acid;Katasuccin;Succinate;Wormwood acid;Acide butanedioique;Acide succinique;Acidum succinicum;Bernsteinsaeure;Butandisaeure;Butanedionic acid;E363;Ethylenesuccinic acid;Hooc-ch2-ch2-cooh;Spirit of amber;Butanedionate;EthylenesuccinatePW_C000174Succini152323945021850786763112655425517538310360421556102161645410764551086489178676411768361667362163745521974562207477222118661981214215113259223423683184236931542402322771431337721313477483111777381127774912978426334800243688072111911284630811342811199844061201924071203851221205554141209904081225653841227671201230291351231894501235553741251381211253644791255494811259304821267134801269065011270822061273895021283043911316Carbon 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_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905021407981859794IronHMDB0015531Iron is a metallic element found in certain minerals, in nearly all soils, and in mineral waters. Iron is required for life. It exists in all living species, ranging from bacteria to humans. It can be found primarily in blood and it is an essential constituent of hemoglobin, cytochrome, and other components of respiratory enzyme systems. Its chief functions are in the transport of oxygen to tissue (hemoglobin) and in cellular oxidation mechanisms. Depletion of iron stores may result in iron-deficiency anemia. Iron is used to build up the blood in anemia. In humans, iron is involved in several metabolic pathways, some of which include the rofecoxib pathway, magnesium salicylate action pathway, etodolac pathway, and diclofenac pathway. Iron is also involved in several metabolic disorders, some of which include adenine phosphoribosyltransferase deficiency (APRT), porphyria variegata (PV), adenylosuccinate lyase deficiency, and AICA-ribosiduria. The major activity of supplemental iron is in the prevention and treatment of iron-deficiency anemia. Iron has putative immune-enhancing, anticarcinogenic, and cognition-enhancing activities. Iron can be found in a number of food items such as chinese water chestnut, hyssop, daikon radish, and peppermint, which makes it a potential biomarker for the consumption of these food products.7439-89-6C00023239251824822368DB01592[Fe++]FeInChI=1S/Fe/q+2CWYNVVGOOAEACU-UHFFFAOYSA-Nlambda2-iron(2+) ion55.84555.9349421330lambda2-iron(2+) ion22C0002326fe;Eisen;Fe;Fer;Ferrum;HierroPW_C009794Iron11388126651533216354931192943411873302131205622577683130779283367826113278409111784283347893833112083812212099240812125642912137112412150238312172712512342313512355737412382646412393011812406039812427813612582929712593248212604429912728220512739150212749638831Ascorbic acidHMDB0000044Ascorbic acid is found naturally in citrus fruits and many vegetables and is an essential nutrient in human diets. It is necessary to maintain connective tissue and bone. The biologically active form of ascorbic acid is vitamin C. Vitamin C is a water soluble vitamin. Primates (including humans) and a few other species in all divisions of the animal kingdom, notably the guinea pig, have lost the ability to synthesize ascorbic acid and must obtain it in their food. Vitamin C functions as a reducing agent and coenzyme in several metabolic pathways. Vitamin C is considered an antioxidant. [PubChem] Ascorbic acid is an electron donor for enzymes involved in collagen hydroxylation, biosynthesis of carnitine and norepinephrine, tyrosine metabolism, and amidation of peptide hormones. Ascrobic acid (vitamin C) deficiency causes scurvy. The amount of vitamin C necessary to prevent scurvy may not be adequate to maintain optimal health. The ability of vitamin C to donate electrons also makes it a potent water-soluble antioxidant that readily scavenges free radicals such as molecular oxygen, superoxide, hydroxyl radical, and hypochlorous acid. In this setting, several mechanisms could account for a link between vitamin C and heart disease. One is the relation between LDL oxidation and vitamins C and E. Vitamin C in vitro can recycle vitamin E, which can donate electrons to prevent LDL oxidation in vitro. As the lipid-phase vitamin E is oxidized, it can be regenerated by aqueous vitamin C. Other possibilities are that vitamin C could decrease cholesterol by mechanisms not well characterized, or could improve vasodilatation and vascular reactivity, perhaps by decreasing the interactions of nitric oxide with oxidants. (PMID: 10799361).50-81-7C000725467006729073ASCORBATE10189562DB00126[H][C@@]1(OC(=O)C(O)=C1O)[C@@H](O)COC6H8O6InChI=1S/C6H8O6/c7-1-2(8)5-3(9)4(10)6(11)12-5/h2,5,7-10H,1H2/t2-,5+/m0/s1CIWBSHSKHKDKBQ-JLAZNSOCSA-N(5R)-5-[(1S)-1,2-dihydroxyethyl]-3,4-dihydroxy-2,5-dihydrofuran-2-one176.1241176.0320879880.144vitamin C0-1FDB001224(+)-sodium l-ascorbate;(+)-ascorbate;(+)-ascorbic acid;3-keto-l-gulofuranolactone;3-oxo-l-gulofuranolactone;Adenex;Allercorb;Antiscorbic vitamin;Antiscorbutic vitamin;Arco-cee;Ascoltin;Ascor-b.i.d.;Ascorb;Ascorbajen;Ascorbate;Ascorbic acid;Ascorbicab;Ascorbicap;Ascorbicin;Ascorbin;Ascorbutina;Ascorin;Ascorteal;Ascorvit;C-level;C-long;C-quin;C-span;C-vimin;Cantan;Cantaxin;Catavin c;Ce lent;Ce-mi-lin;Ce-vi-sol;Cebicure;Cebid;Cebion;Cebione;Cecon;Cee-caps td;Cee-vite;Cegiolan;Ceglion;Ceklin;Celaskon;Celin;Cell c;Cemagyl;Cemill;Cenetone;Cenolate;Cereon;Cergona;Cescorbat;Cetamid;Cetane;Cetane-caps tc;Cetane-caps td;Cetebe;Cetemican;Cevalin;Cevatine;Cevex;Cevi-bid;Cevimin;Cevital;Cevitamate;Cevitamic acid;Cevitamin;Cevitan;Cevitex;Cewin;Chewcee;Ciamin;Cipca;Citriscorb;Citrovit;Colascor;Concemin;Davitamon c;Dora-c-500;Duoscorb;Ferrous ascorbate;Hicee;Hybrin;Ido-c;Juvamine;Kangbingfeng;Kyselina askorbova;L(+)-ascorbate;L(+)-ascorbic acid;L-(+)-ascorbate;L-(+)-ascorbic acid;L-3-ketothreohexuronic acid lactone;L-ascorbate;L-ascorbic acid;L-lyxoascorbate;L-lyxoascorbic acid;L-threo-ascorbic acid;L-xyloascorbate;L-xyloascorbic acid;Laroscorbine;Lemascorb;Liqui-cee;Meri-c;Natrascorb;Natrascorb injectable;Planavit c;Proscorbin;Redoxon;Ribena;Ronotec 100;Rontex 100;Roscorbic;Rovimix c;Scorbacid;Scorbu c;Scorbu-c;Secorbate;Sodascorbate;Suncoat vc 40;Testascorbic;Vasc;Vicelat;Vicin;Vicomin c;Viforcit;Viscorin;Viscorin 100m;Vitace;Vitacee;Vitacimin;Vitacin;Vitamin c;Vitamisin;Vitascorbol;Xitix;Gamma-lactone l-threo-hex-2-enonate;Gamma-lactone l-threo-hex-2-enonic acid;Acide ascorbique;Acido ascorbico;Acidum ascorbicum;Acidum ascorbinicum;Ascorbinsaeure;E 300;E-300;E300PW_C000031VitC679316982012675202114625210712146151122972251340222242506318777521297776634178429334791161151158553361205584141209934081216104051231924501235583741241683761259334821273925021275792091309PristanalHMDB0001958Pristanal belongs to the class of organic compounds known as acyclic diterpenoids. These are diterpenoids (compounds made of four consecutive isoprene units) that do not contain a cycle. Thus, pristanal is considered to be an isoprenoid lipid molecule. Pristanal is considered to be a practically insoluble (in water) and relatively neutral molecule. Within the cell, pristanal is primarily located in the cytoplasm, membrane (predicted from logP) and peroxisome. In humans, pristanal is involved in the oxidation OF branched chain fatty acids pathway and the phytanic Acid peroxisomal oxidation pathway. Pristanal is also involved in the metabolic disorder called the refsum disease pathway. Intermediate in the metabolism of phytanic acid and pristanic acid.105373-75-9146710604918913628282CC(C)CCCC(C)CCCC(C)CCCC(C)C=OC19H38OInChI=1S/C19H38O/c1-16(2)9-6-10-17(3)11-7-12-18(4)13-8-14-19(5)15-20/h15-19H,6-14H2,1-5H3IZJRIIWUSIGEAJ-UHFFFAOYSA-N2,6,10,14-tetramethylpentadecanal282.5044282.292265838-7.210pristanal00FDB022765(2r)-pristanal;(2r,6r,10r)-2,6,10,14-tetramethylpentadecanal;(2s)-pristanal;(2s,6r,10r)-2,6,10,14-tetramethylpentadecanal;2(r)-pristanal;2(s)-pristanal;2rpr-al (2r,6r,10r,14)-tetramethylpentadecanal;2spr-alPW_C001309Pristal126952424378419112785543341209794081224104071235443741249801191259194821265554811273785021281272061883Formyl-CoAHMDB0003419Formyl-CoA is formed during the alpha-oxidation process in liver peroxisomes, as a result of the alpha-oxidation of 3-methyl-substituted fatty acids. The amount of formyl-CoA formed constitutes 2 - 5% of the total formate. The formyl-CoA formed is not due to activation of formate - until now presumed to be the primary end-product of alpha-oxidation - but is rather than formate the end-product of alpha-oxidation. The cleavage of 2-hydroxy-3-methylhexadecanoyl-CoA to 2-methylpentadecanal and formate (formyl-CoA) is probably due to the presence of a specific lyase. (PMID: 9276483, 9166898).13131-49-2C0079843931315522FORMYL-COA388444CC(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=OC22H36N7O17P3SInChI=1S/C22H36N7O17P3S/c1-22(2,17(33)20(34)25-4-3-13(31)24-5-6-50-11-30)8-43-49(40,41)46-48(38,39)42-7-12-16(45-47(35,36)37)15(32)21(44-12)29-10-28-14-18(23)26-9-27-19(14)29/h9-12,15-17,21,32-33H,3-8H2,1-2H3,(H,24,31)(H,25,34)(H,38,39)(H,40,41)(H2,23,26,27)(H2,35,36,37)/t12-,15-,16-,17?,21-/m1/s1SXMOKYXNAPLNCW-BWGWEBPHSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-2-[({[({3-[(2-{[2-(formylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]-3-hydroxy-2,2-dimethylpropoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-4-hydroxyoxolan-3-yl]oxy}phosphonic acid795.544795.110122987-2.259[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-2-{[({3-[(2-{[2-(formylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]-3-hydroxy-2,2-dimethylpropoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-4-hydroxyoxolan-3-yl]oxyphosphonic acid0-4FDB023169Formyl coenzyme aPW_C001883FormCoA1270578422334120980408123545374125920482127379502423MagnesiumHMDB0000547Magnesium 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+868227426816476272726811581918883229363998339922111674614834915294317641421241024115929422331262933737454031477491486954497456525310453291115356112537610359061475934151603815560941616250166648417865941646881160697919971702057194206722721372332117250214731021673131987473222117631321184321012312225123242491251328812581226127292901527528515337308771371337723632977937336783933347841733578489115785223317853635678574130800203688004518480048372806231188065413580865158096525381841519383238394900271085962231105593901156873981199744061200701221202473821207024071209814081211811241212654291213194191219241251220864051224084221227591201229213991233071191235463741238354641238894551244771361246373761249783751254472971255984841256694791257774811259214821259472991259734951260004901262434781265534911267533001271253891271645011273805021274073881274515071278042091281255081283473951407738911060Thiamine 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_C001060ThiamPP205410753119781271517362536610360281556080161638816473178746322212806225771241337828511278423334790181117917513280010368119956406120802407120902122120982408121537124122746120123388119123473135123547374124095118125346479125922482126094481126802299126889501127381502127549206128400388721NADHMDB0000902NAD (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_C000721NAD140415033538651101114211344312735146654222949277917283529310794807184813184819284902649603151679552381035334111536011254691235482125559013556101185696100573810858271415912147594215160241556072157607616163851646917867721176890160701218870971637174205719720674051987459222824122683592259085224118192161232224913006298130183001325622342404322426193157710413277120133772091347737033177650336776673347770233277709130779151137798334778406356800063688069011993825124110552388112750166112853941199291221199524061201714071208344191209844081211594251212421261212594291218173831226143841227421201231304471231411361234194551235493741237314601238124431238294641243703981251871211253192971253424791255304811258062991258254901259244821265154951267654801268855011272785071273835021280893901283603911284283951407571851420WaterHMDB0002111Water 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_C001420H2O5589491095139415131621448113526156242865210691207703382318838210943113774914655415904320182425322226786027274627781728052931437031647236346145983647273749419350302751567519597521410052279452361035297105531911153431135355112540211054701235483125549212655071275534130553711455411295591135560811856221085691657591405778101584114358531465877107589095591014759401516032155605915760871616123163613315962151621816664771786507180660015267131176840188688816071622057181207719320672112117228213723821472432157295198735021673882107401212746722274922247500190758817082012258237226841416292652611850277119221641201128112213285122502861226428712327249125202271263265126932901270529112715292130072981301930013025301130373021326122313327294153403084232731542695318436913227691429377019253771021327713113377215134773783317739733277471333775161157753633477628336777223377775934177816343779823477807132978235352782423537827035679113360800143688003937080591228806561199383038394794384110557390110639391115844398119879232119915122119963406120008407120046408120113124120365412120430405120438409120606415120794414121158425121240429121351121121381419121607434122118382122384436122753120122797374122804443123012446123064376123072137123131447123142136123162448123231451123384450123730460123810464123940455124165469124670399124938471124945472125305297125353479125386481125424482125480299125682483125707478125745487126054490126238495126273484126764480126896501126963502127017388127177208127199209127227504127506507127576515127836389128082395128176513140674790140675834140755185635Pristanic acidHMDB0000795Pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) is a terpenoid acid present at micromolar concentrations in the plasma of healthy individuals. It is also found in the lipids from many sources such as freshwater sponges, krill, earthworms, whales, human milk fat, bovine depot fat, butterfat or Californian petroleum. It is usually present in combination with phytanic acid. In humans, pristanic acid is obtained from two sources: either directly from the diet or as the alpha oxidation product of phytanic acid. At physiological concentrations pristanic acid is a natural ligand for PPARalpha. In liver, pristanic acid is degraded by peroxisomal beta oxidation to propionyl-CoA. Together with phytanic acid, pristanic acid accumulates in several inherited disorders such as Zellweger syndrome. Pristanic acid is a branched chain fatty acid that arises from the breakdown of phytanic acid. It is present at micromolar concentrations in the plasma of healthy individuals. Pristanic acid is normally degraded by peroxisomal beta-oxidation. In patients affected with generalized peroxisomal disorders, degradation of both phytanic acid and pristanic acid is impaired owing to absence of functional peroxisomes. Pristanic acid has been found to activate the peroxisome proliferator-activated receptor {alpha} (PPAR{alpha}) in a concentration dependent manner.1189-37-312392951340110458CC(C)CCCC(C)CCCC(C)CCCC(C)C(O)=OC19H38O2InChI=1S/C19H38O2/c1-15(2)9-6-10-16(3)11-7-12-17(4)13-8-14-18(5)19(20)21/h15-18H,6-14H2,1-5H3,(H,20,21)PAHGJZDQXIOYTH-UHFFFAOYSA-N2,6,10,14-tetramethylpentadecanoic acid298.511298.287180464-6.341pristanic acid0-1FDB012993(2s)-pristanic acid;(2s,6r,10r)-2,6,10,14-tetramethylpentadecanoate;(2s,6r,10r)-2,6,10,14-tetramethylpentadecanoic acid;(2s,6r,10r)-pristanic acid;2,6,10,14-tetramethylpentadecanoate;2,6,10,14-tetramethylpentadecanoic acid;Pristanate:(2s,6r,10r)-pristanate;2,6,10,14-tetramethyl-pentadecansaeure;2,6,10,14-tetramethylpentadecylic acid;Acide pristanique;Acido pristanico;Pristaninsaeure;Pristanate;2,6,10,14-tetramethylpentadecylatePW_C000635Pristan1274524065978412335785553341209854081224034221235503741249733751259254821265484911273845021281205081144NADHHMDB0001487NADH is the reduced form of NAD+, and NAD+ is the oxidized form of NADH, A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). It forms NADP with the addition of a phosphate group to the 2' position of the adenosyl nucleotide through an ester linkage.(Dorland, 27th ed).58-68-4C0000443915316908NADH388299DB00157NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1OC21H29N7O14P2InChI=1S/C21H29N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1,3-4,7-8,10-11,13-16,20-21,29-32H,2,5-6H2,(H2,23,33)(H,34,35)(H,36,37)(H2,22,24,25)/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BOPGDPNILDQYTO-NNYOXOHSSA-N[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]({[(2R,3S,4R,5R)-5-(3-carbamoyl-1,4-dihydropyridin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy})phosphinic acid665.441665.124771695-2.358NADH0-2FDB0226491,4-dihydronicotinamide adenine dinucleotide;Dpnh;Dihydrocodehydrogenase i;Dihydrocozymase;Dihydronicotinamide adenine dinucleotide;Dihydronicotinamide mononucleotide;Enada;Nadh;Nadh2;Reduced codehydrogenase i;Reduced diphosphopyridine nucleotide;Reduced nicotinamide adenine diphosphate;Reduced nicotinamide-adenine dinucleotide;B-dpnh;B-nadh;Beta-dpnh;Beta-nadh;Nicotinamide adenine dinucleotide (reduced);Reduced nicotinamide adenine dinucleotidePW_C001144NADH14341533490864810111521275514695422304927811728362931099480618481218482128490464959315169955240103533211153581125466123547912555931355698100573710858291415915147594515160271556079161638716472178677111768931607011188709916371722057195206746222282442268360225908622411809198118212161232024913003298130153001325522342403322426183157710713277123133772081347737133177651336776683347770033277707130779171137798634780009368806911199382212411054938811285494115838118119955406120172407120378122120986408121162425121244126121693429121818383122616384122745120123127447123138136123551374123734460123814443124242464124371398125189121125345479125531481125762297125808299125926482126516495126767480126888501127385502128090390128362391128429395140759185940Acetyl-CoAHMDB0001206The main function of coenzyme A is to carry acyl groups (such as the acetyl group) or thioesters. Acetyl-CoA is an important molecule itself. It is the precursor to HMG CoA, which is a vital component in cholesterol and ketone synthesis. (wikipedia). acetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.72-89-9C0002444449315351ACETYL-COA392413CC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC23H38N7O17P3SInChI=1S/C23H38N7O17P3S/c1-12(31)51-7-6-25-14(32)4-5-26-21(35)18(34)23(2,3)9-44-50(41,42)47-49(39,40)43-8-13-17(46-48(36,37)38)16(33)22(45-13)30-11-29-15-19(24)27-10-28-20(15)30/h10-11,13,16-18,22,33-34H,4-9H2,1-3H3,(H,25,32)(H,26,35)(H,39,40)(H,41,42)(H2,24,27,28)(H2,36,37,38)/t13-,16-,17-,18+,22-/m1/s1ZSLZBFCDCINBPY-ZSJPKINUSA-N{[(2R,3S,4R,5R)-2-({[({[(3R)-3-[(2-{[2-(acetylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]-3-hydroxy-2,2-dimethylpropoxy](hydroxy)phosphoryl}oxy)(hydroxy)phosphoryl]oxy}methyl)-5-(6-amino-9H-purin-9-yl)-4-hydroxyoxolan-3-yl]oxy}phosphonic acid809.571809.125773051-2.279acetyl-CoA0-4FDB022491Ac-coa;Ac-coenzyme a;Ac-s-coa;Ac-s-coenzyme a;Acetyl coenzyme-a;Acetyl-coa;Acetyl-coenzyme a;Acetyl-s-coa;Acetyl-s-coenzyme a;Acetylcoenzyme-a;S-acetate coa;S-acetate coenzyme a;S-acetyl coenzyme a;Accoa;Acetyl coenzyme a;S-acetyl-coa;S-acetyl-coenzyme a;Acetylcoenzyme aPW_C000940Ac-CoA2134385884232416224465289617334011484014527810354761245733108602515560771616386164701786923160710616372911987460222824515182772101258222613012299426153157712113377291111775621127770613277994115783551347843333480007368806341198066337690124170119953406120145405120304122120632407122417408122626384122743120122959135123137118124986374125200121125343479125507478125633297126564482126572481126778480126886501127044209127394205127665388128137502128145206128374391140762185988Propionyl-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_C000988PropCoA12778169432285424455491413909122477641334784361127855611178636133120995122121576407121681408122266406123560135124133119124231374124819120125935297126430479126557482126568481127395205127997501128130502128141206960Isobutyryl-CoAHMDB0001243Isobutyryl-CoA, also known as isobutanoyl-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. Thus, isobutyryl-CoA is considered to be a fatty ester lipid molecule. Isobutyryl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). Isobutyryl-CoA has been primarily detected in urine. Within the cell, isobutyryl-CoA is primarily located in the mitochondria and cytoplasm. Isobutyryl-CoA exists in all living organisms, ranging from bacteria to humans. In humans, isobutyryl-CoA is involved in the phytanic Acid peroxisomal oxidation pathway and the valine, leucine and isoleucine degradation pathway. Isobutyryl-CoA is also involved in several metabolic disorders, some of which include methylmalonate semialdehyde dehydrogenase deficiency, the maple syrup urine disease pathway, the 3-methylglutaconic aciduria type I pathway, and the isovaleric acidemia pathway. Isobutyryl-CoA is a substrate for Acyl-CoA dehydrogenase (short-chain specific, mitochondrial), Acyl-CoA dehydrogenase (medium-chain specific, mitochondrial) and Acyl-CoA dehydrogenase (long-chain specific, mitochondrial).15621-60-0C00630303693115479ISOBUTYRYL-COA2300823CC(C)C(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC25H42N7O17P3SInChI=1S/C25H42N7O17P3S/c1-13(2)24(37)53-8-7-27-15(33)5-6-28-22(36)19(35)25(3,4)10-46-52(43,44)49-51(41,42)45-9-14-18(48-50(38,39)40)17(34)23(47-14)32-12-31-16-20(26)29-11-30-21(16)32/h11-14,17-19,23,34-35H,5-10H2,1-4H3,(H,27,33)(H,28,36)(H,41,42)(H,43,44)(H2,26,29,30)(H2,38,39,40)/t14-,17-,18-,19+,23-/m1/s1AEWHYWSPVRZHCT-NDZSKPAWSA-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-methylpropanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid837.624837.157073179-2.349isobutyryl- coa0-4FDB0225082-methylpropanoyl-coa;2-methylpropanoyl-coenzyme a;2-methylpropionyl-coa;2-methylpropionyl-coenzyme a;Isobutyryl- coa;Isobutyryl- coenzyme a;Isobutyryl-coa;S-(2-methylpropanoate;S-(2-methylpropanoate)coa;S-(2-methylpropanoate)coenzyme a;S-(2-methylpropanoic acid;S-(2-methylpropanoyl)-coa;S-(2-methylpropanoyl)-coenzyme a;Alpha-methylpropionyl-coa;Alpha-methylpropionyl-coenzyme a;Isobutanoyl-coaPW_C000960IsoBCoA127881668478557111791901331209961221215624061235611351241201201259362971273962051694Very long-chain acyl-CoA synthetaseO14975Acyl-CoA synthetase probably involved in bile acid metabolism. Proposed to activate C27 precurors of bile acids to their CoA thioesters derivatives before side chain cleavage via peroxisomal beta-oxidation occurs. In vitro, activates 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanate (THCA), the C27 precursor of cholic acid deriving from the de novo synthesis from cholesterol. Does not utilize C24 bile acids as substrates. In vitro, also activates long- and branched-chain fatty acids and may have additional roles in fatty acid metabolism. May be involved in translocation of long-chain fatty acids (LFCA) across membranes (By similarity).
HMDBP01946SLC27A215q21.2AF09629016.2.1.-; 6.2.1.312628144237309144238308264Phytanoyl-CoA dioxygenase, peroxisomalO14832Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
HMDBP00270PHYH10p13AF24238411.14.11.181268539452-hydroxyacyl-CoA lyase 1Q9UJ83Catalyzes a carbon-carbon cleavage reaction; cleaves a 2-hydroxy-3-methylacyl-CoA into formyl-CoA and a 2-methyl-branched fatty aldehyde.
HMDBP08729HACL13p25.1AJ13175314.1.-.-12725290Fatty aldehyde dehydrogenaseP51648Catalyzes the oxidation of long-chain aliphatic aldehydes to fatty acids. Active on a variety of saturated and unsaturated aliphatic aldehydes between 6 and 24 carbons in length. Responsible for conversion of the sphingosine 1-phosphate (S1P) degradation product hexadecenal to hexadecenoic acid.
HMDBP00296ALDH3A217p11.2U7528611.2.1.3127655714ATP-binding cassette sub-family D member 1P33897Z3100618448595715ATP-binding cassette sub-family D member 2Q9UBJ2BC1049011844959546Long-chain-fatty-acid--CoA ligase 1P33121Activation of long-chain fatty acids for both synthesis of cellular lipids, and degradation via beta-oxidation. Preferentially uses palmitoleate, oleate and linoleate.
HMDBP00577ACSL14q35CH47105616.2.1.38842224125926122525410413263616613562832913784771314113631353Very long-chain acyl-CoA synthetase1PW_P00035337416941354Phytanoyl-CoA dioxygenase, peroxisomal1PW_P0003543752641159979411603113552-hydroxyacyl-CoA lyase 11PW_P00035537639454161423416210604356Fatty aldehyde dehydrogenase1PW_P0003563772901636ATP-binding cassette sub-family D1PW_P0006366875714168857151246Long-chain-fatty-acid--CoA ligase 11PW_P00024626554611134231862falsePW_R000862Right34896391Compoundfalse34904141Compoundtrue349110991Compoundtrue349210491Compoundfalse349310341Compoundtrue34941701Compoundtrue43135311142466.2.1.3864falsePW_R000864Right35019991Compoundfalse350213091Compoundfalse350318831Compoundtrue4333554.1.-.-865falsePW_R000865Right350413091Compoundfalse35057211Compoundtrue350614201Compoundtrue35076351Compoundfalse350811441Compoundtrue4343561.2.1.3863falsePW_R000863Right349510491Compoundfalse34961341Compoundtrue349710651Compoundtrue34989991Compoundfalse34991741Compoundtrue350013161Compoundtrue4323541.14.11.181569PW_T001569Active181210491Compound85Right9956362018-07-31T12:35:06-06:002018-07-31T12:35:06-06:00592054639882false126523010regular3002802055414842false151553510regular503020561099885false127053510regular503020571049882false126882510regular30028020581034843false151873510regular50302059170845false126672810regular63432060134581false1120170510regular20019020611065565false1516176110regular78782062999582false1270212010regular3002802063174581false1120192510regular20019020641316552false1516197110regular78782065979459false1370185719regular1002520663159false1370183720regular1002520671309582false1270279010regular30028020681883581false1575261510regular200190206942359false1375255520regular100252070106059false1375252020regular100352071721559false1280310510regular503020721420549false1510308010regular78782073635582false1270337510regular30028020741144560false1280330510regular50302075940882false865391010regular3002802076988882false1650391010regular3002802077960882false1275411510regular30028089351049582false1270142010regular300280780169482false13406158subunitregular1507078126452false134518728subunitregular15070782394558false135025508subunitregular1408578329052false134531858subunitregular15070615836571476false126812078subunitregular15070615837571576false141812078subunitregular1507073035351877378073135451577478131220652987Cofactor31320662988Cofactor73235551577578231420692992Cofactor31520702993Cofactor733356515776783503046636516143316158366143326158372975M1415 510 C1415 540 1415 585 1415 615 5false182976M1515 550 C1480 550 1415 585 1415 615 5false182977M1320 550 C1355 549 1414 587 1415 615 5false182978M1418 825 C1418 795 1415 715 1415 685 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2979M1518 750 C1491 750 1415 715 1415 685 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2980M1329 749.5 C1353 749.5 1415 715 1415 685 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2987M1365 1852 L1365 1902 L1415 1852 z10true182988M1365 1832 L1365 1882 L1415 1832 z10true182989M1420 2400 C1420 2430 1420 2520 1420 2550 5false182990M1420 2790 C1420 2760 1420 2665 1420 2635 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2991M1575 2710 C1543 2710 1420 2723 1420 2635 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2992M1505 2340 L1505 2390 L1555 2340 z10true182993M1505 2340 L1505 2390 L1555 2340 z10true182994M1420 3070 C1420 3100 1420 3155 1420 3185 5false182995M1330 3120 C1364 3120 1420 3155 1420 3185 5false182996M1510 3119 C1478 3119 1420 3155 1420 3185 5false182997M1420 3375 C1420 3345 1420 3285 1420 3255 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2998M1330 3320 C1361 3320 1420 3285 1420 3255 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2999M1420 3655 C1420 3685 1420 3680 1420 3710 5false183000M1165 4050 C1253 4049 1422 3948 1420 3780 5false18trueM 977.9903810567666 4057.5 L 965 4065 L 977.9903810567666 4072.5false3001M1650 4050 C1513 4049 1421 3899 1420 3780 5false18trueM 1457.0096189432334 4047.5 L 1470 4040 L 1457.0096189432334 4032.5false3002M1425 4115 C1425 4085 1420 3810 1420 3780 5false18trueM 1212.5 4147.009618943233 L 1220 4160 L 1227.5 4147.009618943233false13900M1570 3515 C1600 3515 1645 3515 1675 3515 5false18trueM 1462.0096189432334 3422.5 L 1475 3415 L 1462.0096189432334 3407.5false2259224M1420 1700 C1420 1730 1420 1842 1420 1872 5false182259225M1320 1800 C1389 1801 1420 1842 1420 1872 5false182259226M1516 1800 C1470 1799 1420 1842 1420 1872 5false182259227M1420 2120 C1420 2090 1420 1972 1420 1942 5false18trueM 540.9468550441649 13.26155629629604 L 526 12 L 532.3808877211858 25.575134323078345false2259228M1320 2020 C1389 2021 1420 1972 1420 1942 5false18trueM 540.9468550441649 13.26155629629604 L 526 12 L 532.3808877211858 25.575134323078345false2259229M1516 2010 C1472 2010 1420 1989 1420 1942 5false18trueM 540.9468550441649 13.26155629629604 L 526 12 L 532.3808877211858 25.575134323078345false2260230M1418 1105 C1418 1135 1418 1212 1418 1242 83false62260231M1420 1420 C1420 1390 1418 1272 1418 1242 83false6trueM 714.9468550441649 827.261556296296 L 700 826 L 706.3808877211858 839.5751343230784false605518628223520542975Left223620552976Left223720562977Left223820572978Right223920582979Right224020592980Right576431730607518645224720622989Left224820672990Right224920682991Right578433732608518655225020672994Left225120712995Left225220722996Left225320732997Right225420742998Right579434733507963518635203873989352259224Left203874020602259225Left203874120612259226Left203874220622259227Right203874320632259228Right203874420642259229Right466146432731268081569515421720572260230Left5421889352260231Right224745030469951046751217false1345371016regular7020732999Left7120753000Right7220763001Right7320773002Right8387545114false1675348016regular793207313900Left966732011501.51.502439934018744723527052.22.2031432726747983638529691.91.9-110232803604798374408301.11.1-4521490240135M1027 1335 C1027 1285 1077 1235 1127 1235 C1322 1235 1576 1235 1771 1235 C1821 1235 1871 1285 1871 1335 C1871 2059 1871 2999 1871 3723 C1871 3773 1821 3823 1771 3823 C1576 3823 1322 3823 1127 3823 C1077 3823 1027 3773 1027 3723 C1027 2999 1027 2059 1027 1335 94true6844.02588.0136M126 226 C126 176 176 126 226 126 C757 126 1446 126 1977 126 C2027 126 2077 176 2077 226 C2077 1484 2077 3118 2077 4376 C2077 4426 2027 4476 1977 4476 C1446 4476 757 4476 226 4476 C176 4476 126 4426 126 4376 C126 3118 126 1484 126 226 1true61951.04350.0145235Peroxisome4901155201.61.620015146235Intracellular Space685280201.31.320015147235Extracellular Space69520201.31.320015507471336100112101897385039#FBEBFC48962640