116693PathwayAcylcarnitine (8Z,11Z,14Z,17Z)-icosa-8,11,14,17-tetraenoylcarnitineMetabolicPW125836CenterPathwayVisualizationContext12611220003000#000099PathwayVisualization116541116693Acylcarnitine (8Z,11Z,14Z,17Z)-icosa-8,11,14,17-tetraenoylcarnitineMetabolic11162602138SubPathway117190940Compound802116261354SubPathway117191111814Compound802117192940Compound8021CellCL:00000002Platelet CL:00002335HepatocyteCL:00001823NeuronCL:00005404Cardiomyocyte CL:00007468Beta cellCL:00006397Epithelial CellCL:000006611Colorectal Cancer CellCL:00010641Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote12Mus musculus10090EukaryoteMouse24Solanum lycopersicum4081EukaryoteTomato4Arabidopsis thaliana3702EukaryoteThale cress18Saccharomyces cerevisiae4932EukaryoteYeast21Xenopus laevis8355EukaryoteAfrican clawed frog6Caenorhabditis elegans6239EukaryoteRoundworm49Bathymodiolus platifrons220390EukaryoteDeep sea mussel60Nitzschia sp.0001EukaryoteNitzschia42Bacteria2ProkaryoteBacteria17Rattus norvegicus10116EukaryoteRat5Bos taurus9913EukaryoteCattle10Drosophila melanogaster7227EukaryoteFruit fly19Schizosaccharomyces pombe4896Eukaryote25Escherichia coli (strain K12)83333Prokaryote23Pseudomonas aeruginosa287Prokaryote51Picea sitchensis3332EukaryoteSitka spruce62Acinetobacter baylyi (strain ATCC 33305 / BD413 / ADP1)62977Prokaryote5CytoplasmGO:00057373Mitochondrial MatrixGO:00057591CytosolGO:00058294PeroxisomeGO:000577713Endoplasmic ReticulumGO:000578314Mitochondrial Outer MembraneGO:00057412MitochondrionGO:00057397Endoplasmic Reticulum MembraneGO:000578925Golgi ApparatusGO:000579410Cell MembraneGO:000588627Peroxisome MembraneGO:000577815NucleusGO:000563426Golgi Apparatus MembraneGO:000013935ChloroplastGO:000950711Extracellular SpaceGO:000561512Mitochondrial Inner MembraneGO:000574331Periplasmic SpaceGO:000562032Inner MembraneGO:00702588Smooth Endoplasmic Reticulum GO:000579024Mitochondrial Intermembrane SpaceGO:00057582Endothelium BTO:00003931LiverBTO:00007597297Nervous SystemBTO:00014845cardiocyteBTO:00015398Blood VesselBTO:0001102741118PancreasBTO:000098825IntestineBTO:00006484Adrenal MedullaBTO:000004971828StomachBTO:00013071552622BladderBTO:00001238511PW_BS0000084311PW_BS00000416212PW_BS0000162111PW_BS000002261115PW_BS0000265411PW_BS000005181311PW_BS000018221411PW_BS000022397113PW_BS0000393211PW_BS000003215114PW_BS00002146114PW_BS00004649711PW_BS000049432511PW_BS00004314101PW_BS000014101711PW_BS000010592711PW_BS000059231511PW_BS00002329111PW_BS000029711PW_BS000007311511PW_BS000031918511PW_BS000091541315PW_BS000054562611PW_BS0000561041431PW_BS0001041115121PW_BS0001111122121PW_BS000112103331PW_BS0001031471241PW_BS000147151141PW_BS0001511553241PW_BS0001551613181PW_BS00016116611PW_BS0001661783211PW_BS0001781644PW_BS0001641601181PW_BS00016019914181PW_BS000024205561PW_BS000024206261PW_BS0000242137181PW_BS0000242111018PW_BS00002421425181PW_BS0000242164181PW_BS0000241985181PW_BS000024222341PW_BS0000241321121PW_BS00013221013181PW_BS0000242253541PW_BS0000242491341PW_BS0000242881441PW_BS000024226441PW_BS0000242905491PW_BS0000242851041PW_BS0000243081011PW_BS0000241333121PW_BS00013332914121PW_BS0000283361121PW_BS0000283344121PW_BS00002833527121PW_BS0000281151012PW_BS0001153317121PW_BS00002835625121PW_BS00002813013121PW_BS0001303683601PW_BS000028184121PW_BS000024372102PW_BS0000281181171PW_BS0001181355171PW_BS00013515111PW_BS000015253541PW_BS0000245181PW_BS000051383751PW_BS00010027151PW_BS0000272231241PW_BS000024390761PW_BS0001123987171PW_BS000113406351PW_BS000115122551PW_BS0001223821451PW_BS000100407251PW_BS000115408451PW_BS000115124151PW_BS000124429151PW_BS0001154192551PW_BS0001151251351PW_BS000125405105PW_BS0001154222751PW_BS0001151203171PW_BS00012039914171PW_BS0001131192171PW_BS0001193744171PW_BS0000534641171PW_BS00011545525171PW_BS00011513613171PW_BS0001363761017PW_BS00005337527171PW_BS0000532975101PW_BS00002448414101PW_BS0001154793101PW_BS0001154812101PW_BS0001154824101PW_BS0001152991101PW_BS0000244957101PW_BS00011549025101PW_BS0001154781010PW_BS00011549127101PW_BS00011530013101PW_BS0000243891461PW_BS000112501361PW_BS000115502461PW_BS000115388161PW_BS0001125072561PW_BS000115209106PW_BS0000245082761PW_BS0001153951361PW_BS0001138911421PW_BS00055213121PW_BS0000133211515PW_BS0000325811411PW_BS0000586618518PW_BS00006672513PW_BS000072612517PW_BS000061892PW_BS000089971521PW_BS000097100521PW_BS000100101531PW_BS000101117131PW_BS00011712915121PW_BS00012910813PW_BS00010814315191PW_BS0001431465191PW_BS000146107313PW_BS000107188118PW_BS0000242171518PW_BS00002421815181PW_BS0000241632181PW_BS0001631901118PW_BS0000242771218PW_BS00002417018PW_BS0001702811251PW_BS000024315123PW_BS0000243221231PW_BS0000243183123PW_BS00002413412121PW_BS0001343331212PW_BS00002833217121PW_BS000028350114121PW_BS00002812815121PW_BS0001283511512PW_BS00002835325127PW_BS00002811PW_BS000001943PW_BS000094109323PW_BS000109412125PW_BS0001151231751PW_BS00012343311451PW_BS0001154101551PW_BS000115435155PW_BS0001154461217PW_BS00011544717171PW_BS000115468114171PW_BS00011544415171PW_BS00011547225177PW_BS0001154701517PW_BS00011548515101PW_BS0001154991510PW_BS0001155161561PW_BS000115517156PW_BS000115171211PW_BS000017111811PW_BS0000116131PW_BS0000061021231PW_BS00010216212181PW_BS000162224241PW_BS0000241951318PW_BS00002429341PW_BS0000243841251PW_BS0001004141551PW_BS00011512112171PW_BS00012145015171PW_BS00011548012101PW_BS0001153911261PW_BS0001121861221PW_BS000024185321PW_BS00002430635511PW_BS000024105113PW_BS0001051141112PW_BS000114409115PW_BS0001151371117PW_BS0001374831110PW_BS0001152941141PW_BS000024204111PW_BS0000207028511PW_BS00007034141121PW_BS000028422411PW_BS00004234524121PW_BS0000284182451PW_BS00011545424171PW_BS00011548924101PW_BS0001155062461PW_BS0001157132210111PW_BS0005126531624PW_BS000508393151PW_BS00017380231PW_BS000548423MagnesiumHMDB0000547Magnesium 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+86822742681647627272681158191888322936399833992211167461483491529431764142124102411592942233126293373745403147749148695449745652531045329111535611253761035906147593415160381556094161625016664841786594164688116069791997170205719420672272137233211725021473102167313198747322211763132118432101231222512324249125132881258122612729290152752851533730877137133772363297793733678393334784173357848911578522331785363567857413080020368800451848004837280623118806541358086515809652538184151938323839490027108596223110559390115687398119974406120070122120247382120702407120981408121181124121265429121319419121924125122086405122408422122759120122921399123307119123546374123835464123889455124477136124637376124978375125447297125598484125669479125777481125921482125947299125973495126000490126243478126553491126753300127125389127164501127380502127407388127451507127804209128125508128347395140773891111813(8Z,11Z,14Z,17Z)-icosa-8,11,14,17-tetraenoic acidCC\C=C/C\C=C/C\C=C/C\C=C/CCCCCCC(O)=OC20H32O2InChI=1S/C20H32O2/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-16-17-18-19-20(21)22/h3-4,6-7,9-10,12-13H,2,5,8,11,14-19H2,1H3,(H,21,22)/b4-3-,7-6-,10-9-,13-12-HQPCSDADVLFHHO-LTKCOYKYSA-N(8Z,11Z,14Z,17Z)-icosa-8,11,14,17-tetraenoic acid304.4669304.240230268-6.271(8Z,11Z,14Z,17Z)-icosa-8,11,14,17-tetraenoic acid0-1PW_C111813414Adenosine 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_C001099CoA2114386884538792289217240759241422459528132928623133421133511846181046295848421448655448796523210252471045280103547712457341085777101602315560751616384164681786930160696116269731997083188710816372931987347210745822282291519081226909022491241709215195130132991531824925488494261631576907293771191337722213477230329772921117755013277555334775631127763333677672129779961157804733278056350784133357856713079259333799743318000536880620118806273748063511980665376938283829383438398674288110555389110561390115842399115847398119951406120147405120231384120305122120634407120762117121406123121421433121521125121666429121682408121714414122404422122741120122904121122960135123965447123979468124079136124220464124265450124974375125341479125509478125579480125592484125634297126084481126549491126560482126746300126884501127046209127109391127301205127540206127667388128121508128133502128340395140751186140763185140767891111814(8Z,11Z,14Z,17Z)-icosa-8,11,14,17-tetraenoyl-CoACCC=CCC=CCC=CCC=CCCCCCCC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC41H66N7O17P3SInChI=1S/C41H66N7O17P3S/c1-4-5-6-7-8-9-10-11-12-13-14-15-16-17-18-19-20-21-32(50)69-25-24-43-31(49)22-23-44-39(53)36(52)41(2,3)27-62-68(59,60)65-67(57,58)61-26-30-35(64-66(54,55)56)34(51)40(63-30)48-29-47-33-37(42)45-28-46-38(33)48/h5-6,8-9,11-12,14-15,28-30,34-36,40,51-52H,4,7,10,13,16-27H2,1-3H3,(H,43,49)(H,44,53)(H,57,58)(H,59,60)(H2,42,45,46)(H2,54,55,56)PLHICYKOPITJJT-UHFFFAOYSA-N1053.991053.344875861PW_C11181432Adenosine monophosphateHMDB0000045Adenosine monophosphate, also known as 5'-adenylic acid and abbreviated AMP, is a nucleotide that is found in RNA. It is an ester of phosphoric acid with the nucleoside adenosine. AMP consists of the phosphate group, the pentose sugar ribose, and the nucleobase adenine. AMP can be produced during ATP synthesis by the enzyme adenylate kinase. AMP has recently been approved as a 'Bitter Blocker' additive to foodstuffs. When AMP is added to bitter foods or foods with a bitter aftertaste it makes them seem 'sweeter'. This potentially makes lower calorie food products more palatable.61-19-8C00020608316027AMP5858DB00131NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1C10H14N5O7PInChI=1S/C10H14N5O7P/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(22-10)1-21-23(18,19)20/h2-4,6-7,10,16-17H,1H2,(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1UDMBCSSLTHHNCD-KQYNXXCUSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}phosphonic acid347.2212347.063084339-2.025adenylate0-2DBMET00485FDB0218065'-amp;5'-adenosine monophosphate;5'-adenylate;5'-adenylic acid;Amp;Adenosine 5'-monophosphate;Adenosine 5'-phosphate;Adenosine 5'-phosphorate;Adenosine 5'-phosphoric acid;Adenosine phosphate;Adenosine-5'-monophosphorate;Adenosine-5'-monophosphoric acid;Adenosine-5-monophosphorate;Adenosine-5-monophosphoric acid;Adenosine-monophosphate;Adenosine-phosphate;Adenovite;Adenylate;Adenylic acid;Cardiomone;Lycedan;Muscle adenylate;Muscle adenylic acid;My-b-den;My-beta-den;Phosaden;Phosphaden;Phosphentaside;5'-o-phosphonoadenosine;Adenosine 5'-(dihydrogen phosphate);Adenosine monophosphate;Adenosine-5'p;Adenosini phosphas;Ado5'p;Fosfato de adenosina;Pa;Pado;Phosphate d'adenosine;5'-adenosine monophosphoric acid;Adenosine phosphoric acid;Adenosine 5'-(dihydrogen phosphoric acid);Adenosine 5'-monophosphoric acid;Adenosine monophosphoric acid;Adenosine-5'-monophosphate;Phosphoric acid d'adenosinePW_C000032AMP112344628270167343288122118914457254867545033895251104540811754231035432118545712055581325583133577910157951086977199707218811789198118681611198815112003222125802261263631126942901333122542266342646315772343297732511178392334788091157932011280399180684135809007119916122120016124120031406120246382120888405121954408122920399123464376124507374125306297125394299125409479125596484126853205126934388126949501127124389127311209127711502140771891170PyrophosphateHMDB0000250The anion, the salts, and the esters of pyrophosphoric acid are called pyrophosphates. The pyrophosphate anion is abbreviated PPi and is formed by the hydrolysis of ATP into AMP in cells. This hydrolysis is called pyrophosphorolysis. The pyrophosphate anion has the structure P2O74-, and is an acid anhydride of phosphate. It is unstable in aqueous solution and rapidly hydrolyzes into inorganic phosphate. Pyrophosphate is an osteotoxin (arrests bone development) and an arthritogen (promotes arthritis). It is also a metabotoxin (an endogenously produced metabolite that causes adverse health affects at chronically high levels). Chronically high levels of pyrophosphate are associated with hypophosphatasia. Hypophosphatasia (also called deficiency of alkaline phosphatase or phosphoethanolaminuria) is a rare, and sometimes fatal, metabolic bone disease. Hypophosphatasia is associated with a molecular defect in the gene encoding tissue non-specific alkaline phosphatase (TNSALP). TNSALP is an enzyme that is tethered to the outer surface of osteoblasts and chondrocytes. TNSALP hydrolyzes several substances, including inorganic pyrophosphate (PPi) and pyridoxal 5'-phosphate (PLP), a major form of vitamin B6. When TSNALP is low, inorganic pyrophosphate (PPi) accumulates outside of cells and inhibits the formation of hydroxyapatite, one of the main components of bone, causing rickets in infants and children and osteomalacia (soft bones) in adults. Vitamin B6 must be dephosphorylated by TNSALP before it can cross the cell membrane. Vitamin B6 deficiency in the brain impairs synthesis of neurotransmitters which can cause seizures. In some cases, a build-up of calcium pyrophosphate dihydrate crystals in the joints can cause pseudogout.14000-31-8C0001364410218361PPI559142DB04160OP(O)(=O)OP(O)(O)=OH4O7P2InChI=1S/H4O7P2/c1-8(2,3)7-9(4,5)6/h(H2,1,2,3)(H2,4,5,6)XPPKVPWEQAFLFU-UHFFFAOYSA-N(phosphonooxy)phosphonic acid177.9751177.9432255064pyrophosphoric acid0-3FDB021918(4-)diphosphoric acid ion;(p2o74-)diphosphate;Diphosphate;Diphosphoric acid;Ppi;Pyrometaphosphate;Pyrophosphate;Pyrophosphate tetraanion;Pyrophosphate(4-) ion;[o3popo3](4-);Diphosphat;P2o7(4-);Pyrophosphat;Pyrophosphate ion;Phosphonato phosphoric acid;Pyrophosphoric acid;Pyrophosphoric acid ionPW_C000170Ppi12235463842923735328822212173162049241059281529417514486854503489525210452941015409117542410354331185458120554811155591325584133560613556551085879107623916669781997073188713416372721607312198731821382751518283210118691611200222212041164123152251232324912512288125792261269529015219306153751834760174256131542697318772353297731712877635336784163357892833179153112799501347995813080047372804171708563019478638494814125948193829867822311063439111327039511327538911552713611553239911993412212001712412003240612033041012093640712126142912134112112148638312240742212298544412350211912383146412404439812497737512532429712539529912541047912559748412565648512587648112655249112686920512693538812695050112733720612812450814077289144L-CarnitineHMDB0000062Carnitine is not an essential amino acid; it can be synthesized in the body. However, it is so important in providing energy to muscles (including the heart) that some researchers are now recommending carnitine supplements in the diet, particularly for people who do not consume much red meat (the main food source for carnitine). Carnitine has been described as a vitamin, an amino acid, or a metabimin (i.e. an essential metabolite). Like the B vitamins, carnitine contains nitrogen and is very soluble in water, and to some researchers carnitine is a vitamin (Liebovitz 1984). It was found that an animal (yellow mealworm) could not grow without carnitine in its diet. However, as it turned out, almost all other animals, including humans, do make their own carnitine; thus, it is no longer considered a vitamin. Nevertheless, in certain circumstances, such as deficiencies of methionine, lysine, or vitamin C or kidney dialysis, carnitine shortages develop. Under these conditions, carnitine must be absorbed from food, and for this reason it is sometimes referred to as a "metabimin" or a conditionally essential metabolite. Like the other amino acids used or manufactured by the body, carnitine is an amine. But like choline, which is sometimes considered to be a B vitamin, carnitine is also an alcohol (specifically, a trimethylated carboxy-alcohol). Thus, carnitine is an unusual amino acid and has different functions than most other amino acids, which are usually employed by the body in the construction of protein. Carnitine is an essential factor in fatty acid metabolism in mammals. Its most important known metabolic function is to transport fat into the mitochondria of muscle cells, including those in the heart, for oxidation. This is how the heart gets most of its energy. In humans, about 25% of carnitine is synthesized in the liver, kidney, and brain from the amino acids lysine and methionine. Most of the carnitine in the body comes from dietary sources such as red meat and dairy products. Inborn errors of carnitine metabolism can lead to brain deterioration like that of Reye's syndrome, gradually worsening muscle weakness, Duchenne-like muscular dystrophy, and extreme muscle weakness with fat accumulation in muscles. Borum et al. (1979) describe carnitine as an essential nutrient for pre-term babies and individuals who are unable to eat a normal diet (e.g. non-ketotic hypoglycemics, kidney dialysis patients) (PMID: 115309). In conditions such as kwashiorkor, cirrhosis, and heart muscle disease (cardiomyopathy) as well as in inborn errors of metabolism such as type IV hyperlipidemia and propionic or organic aciduria (acid urine resulting from genetic or other anomalies), carnitine is essential to life and carnitine supplements are valuable. Carnitine therapy may also be useful in a wide variety of clinical conditions. Carnitine supplementation has improved some patients who have angina secondary to coronary artery disease. It may also be worth a trial for patients with any form of hyperlipidemia or muscle weakness. Carnitine supplements may also be useful in many forms of toxic or metabolic liver disease and in cases of heart muscle disease. Hearts undergoing severe arrhythmia quickly deplete their stores of carnitine. Athletes, particularly in Europe, have used carnitine supplements for improved endurance. Carnitine may improve muscle building by improving fat utilization and may even be useful in treating obesity. Carnitine joins a long list of nutrients which may be of value in treating pregnant women, hypothyroid individuals, and male infertility due to the low motility of sperm. Carnitine deficiency is noted in abnormal liver function, renal dialysis patients, and severe to moderate muscular weakness with associated anorexia (http://www.dcnutrition.com). Carnitine is a biomarker for the consumption of meat.541-15-1C003181091716347CARNITINE10455DB00583C[N+](C)(C)C[C@H](O)CC([O-])=OC7H15NO3InChI=1S/C7H15NO3/c1-8(2,3)5-6(9)4-7(10)11/h6,9H,4-5H2,1-3H3/t6-/m1/s1PHIQHXFUZVPYII-ZCFIWIBFSA-N(3R)-3-hydroxy-4-(trimethylazaniumyl)butanoate161.1989161.105193351-1.601L-carnitine00FDB000572(-)-(r)-3-hydroxy-4-(trimethylammonio)butyrate;(-)-carnitine;(r)-(3-carboxy-2-hydroxypropyl)trimethylammonium hydroxide;(r)-carnitine;(s)-carnitine;1-carnitine;3-carboxy-2-hydroxy-n,n,n-trimethyl-1-propanaminium;3-hydroxy-4-trimethylammoniobutanoate;3-hydroxy-4-trimethylammoniobutanoic acid;Bicarnesine;Carniking;Carniking 50;Carnilean;Carnipass;Carnipass 20;Carnitene;Carnitine;Carnitor;D-carnitine;Dl-carnitine;Karnitin;L-(-)-carnitine;L-carnitine;L-gamma-trimethyl-beta-hydroxybutyrobetaine;Levocarnitina;Levocarnitine;Levocarnitinum;R-(-)-3-hydroxy-4-trimethylaminobutyrate;Vitamin bt;Delta-carnitine;Gamma-trimethyl-ammonium-beta-hydroxybutirate;Gamma-trimethyl-beta-hydroxybutyrobetaine;Gamma-trimethyl-hydroxybutyrobetaine;(-)-l-carnitine;3-carboxy-2-hydroxy-n,n,n-trimethyl-1-propanaminium hydroxide, inner salt;CarnicorPW_C000044L-Carnt67531885228911724575251932675228858288745230102524510469591626971199772201347722832977558334775611127756713277748129783391117834513379258333806303748063311980639118120229384120241382120554414122412408122421407122522124122617122122621406122902121122915399123188450125191135125195120125577480125590484126558482126569481126675299126769297126773479127107391127119389128131502128142206128255388128364205128368501140749186140765891111812(8Z,11Z,14Z,17Z)-icosa-8,11,14,17-tetraenoylcarnitineHMDB0241588CCCCCC=CCC(O)C=CC=CCC=CCCCC(=O)OC(CC([O-])=O)C[N+](C)(C)CC27H45NO5InChI=1S/C27H45NO5/c1-5-6-7-8-13-16-19-24(29)20-17-14-11-9-10-12-15-18-21-27(32)33-25(22-26(30)31)23-28(2,3)4/h10-14,16-17,20,24-25,29H,5-9,15,18-19,21-23H2,1-4H3NOARSWRROUOLCM-UHFFFAOYSA-N463.659463.329773553PW_C11181220BiotinHMDB0000030Biotin is an enzyme co-factor present in minute amounts in every living cell. Biotin is also known as vitamin H or B7 or coenzyme R. It occurs mainly bound to proteins or polypeptides and is abundant in liver, kidney, pancreas, yeast, and milk. Biotin has been recognized as an essential nutrient. Our biotin requirement is fulfilled in part through diet, through endogenous reutilization of biotin and perhaps through capture of biotin generated in the intestinal flora. The utilization of biotin for covalent attachment to carboxylases and its reutilization through the release of carboxylase biotin after proteolytic degradation constitutes the 'biotin cycle'. Biotin deficiency is associated with neurological manifestations, skin rash, hair loss and metabolic disturbances that are thought to relate to the various carboxylase deficiencies (metabolic ketoacidosis with lactic acidosis). It has also been suggested that biotin deficiency is associated with protein malnutrition, and that marginal biotin deficiency in pregnant women may be teratogenic. Biotin acts as a carboxyl carrier in carboxylation reactions. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lysine residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lys residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. Evidence is emerging that biotin participates in processes other than classical carboxylation reactions. Specifically, novel roles for biotin in cell signaling, gene expression, and chromatin structure have been identified in recent years. Human cells accumulate biotin by using both the sodium-dependent multivitamin transporter and monocarboxylate transporter 1. These transporters and other biotin-binding proteins partition biotin to compartments involved in biotin signaling: cytoplasm, mitochondria, and nuclei. The activity of cell signals such as biotinyl-AMP, Sp1 and Sp3, nuclear factor (NF)-kappaB, and receptor tyrosine kinases depends on biotin supply. Consistent with a role for biotin and its catabolites in modulating these cell signals, greater than 2000 biotin-dependent genes have been identified in various human tissues. Many biotin-dependent gene products play roles in signal transduction and localize to the cell nucleus, consistent with a role for biotin in cell signaling. Posttranscriptional events related to ribosomal activity and protein folding may further contribute to effects of biotin on gene expression. Finally, research has shown that biotinidase and holocarboxylase synthetase mediate covalent binding of biotin to histones (DNA-binding proteins), affecting chromatin structure; at least seven biotinylation sites have been identified in human histones. Biotinylation of histones appears to play a role in cell proliferation, gene silencing, and the cellular response to DNA repair. Roles for biotin in cell signaling and chromatin structure are consistent with the notion that biotin has a unique significance in cell biology. (PMID: 15992684, 16011464).58-85-5C0012017154815956BIOTIN149962DB00121[H][C@]12CS[C@@H](CCCCC(O)=O)[C@@]1([H])NC(=O)N2C10H16N2O3SInChI=1S/C10H16N2O3S/c13-8(14)4-2-1-3-7-9-6(5-16-7)11-10(15)12-9/h6-7,9H,1-5H2,(H,13,14)(H2,11,12,15)/t6-,7-,9-/m0/s1YBJHBAHKTGYVGT-ZKWXMUAHSA-N5-[(3aS,4S,6aR)-2-oxo-hexahydro-1H-thieno[3,4-d]imidazol-4-yl]pentanoic acid244.311244.088163078-2.3035-[(3aS,4S,6aR)-2-oxo-hexahydrothieno[3,4-d]imidazol-4-yl]pentanoic acid0-1FDB014510(+)-biotin;(+)-cis-hexahydro-2-oxo-1h-thieno[3,4]imidazole-4-valerate;(+)-cis-hexahydro-2-oxo-1h-thieno[3,4]imidazole-4-valeric acid;(3as,4s,6ar)-hexahydro-2-oxo-1h-thieno[3,4-d]imidazole-4-valerate;(3as,4s,6ar)-hexahydro-2-oxo-1h-thieno[3,4-d]imidazole-4-valeric acid;-(+)-biotin;1swk;1swn;1swr;5-(2-oxohexahydro-1h-thieno[3,4-d]imidazol-4-yl)pentanoate;5-(2-oxohexahydro-1h-thieno[3,4-d]imidazol-4-yl)pentanoic acid;Biodermatin;Bioepiderm;Bios ii;Bios h;Biotin;Coenzyme r;D(+)-biotin;D-(+)-biotin;D-biotin;D-biotin factor s;Factor s;Factor s (vitamin);Hexahydro-2-oxo-1h-thieno(3,4-d)imidazole-4-pentanoate;Hexahydro-2-oxo-1h-thieno(3,4-d)imidazole-4-pentanoic acid;Hexahydro-2-oxo-[3as-(3aa,4b,6aa)]-1h-thieno[3,4-d]imidazole-4-pentanoate;Hexahydro-2-oxo-[3as-(3aa,4b,6aa)]-1h-thieno[3,4-d]imidazole-4-pentanoic acid;Hexahydro-2-oxo-[3as-(3alpha,4beta,6alpha)]-1h-thieno[3,4-d]imidazole-4-pentanoate;Hexahydro-2-oxo-[3as-(3alpha,4beta,6alpha)]-1h-thieno[3,4-d]imidazole-4-pentanoic acid;Lutavit h2;Meribin;Rovimix h 2;Vitamin b7;Vitamin h;Vitamin-h;Cis-(+)-tetrahydro-2-oxothieno[3,4]imidazoline-4-valerate;Cis-(+)-tetrahydro-2-oxothieno[3,4]imidazoline-4-valeric acid;Cis-hexahydro-2-oxo-1h-thieno(3,4)imidazole-4-valeric acid;Cis-tetrahydro-2-oxothieno(3,4-d)imidazoline-4-valeric acid;Delta-(+)-biotin;Delta-biotin;Delta-biotin factor s;Biotina;Biotine;BiotinumPW_C000020Biotin2641358579151699322702529210152981055393103544912055461115551114557513360511556112161649617869251607484222778311327796011280031368806531351199954061201341221205034091212104071215591241231091371237801191241171181253744791255012971257184831264212991265424811269165011270382051279893881281152061027ManganeseHMDB0001333Manganese is an essential trace nutrient in all forms of life. Physiologically, it. exists as an ion in the body. It is concentrated in cell mitochondria, mostly in the pituitary gland, liver, pancreas, kidney, and bone, influences the synthesis of mucopolysaccharides, stimulates hepatic synthesis of cholesterol and fatty acids, and is a cofactor in many enzymes, including arginase and alkaline phosphatase in the liver.16397-91-4C196102785429035MN%2b325916[Mn++]MnInChI=1S/Mn/q+2WAEMQWOKJMHJLA-UHFFFAOYSA-Nmanganese(2+) ion54.93854.9380496360manganese(2+) ion22FDB003636Manganese;Manganese (ii) ion;Manganese(ii);Manganese, ion (mn2+);Manganous ion;Mn(2+);Mn2+PW_C001027Mn2+2744738148649155343227122394325131453941035450120557613360521556113161649717869261607485222118801981193922511958164124712491336015115221306770502947749411177832132779611127826735678490115785243317924729380032368119996406120401122121058124121211407121295383121378419122488405123044135123622118123781119123865398123937455125054376125375479125976495126051490126060297126158299126543481126642478126917501127429390127503507127512205127765388128116206128218209940Acetyl-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-CoA2134385884232416224465289617334011484014527810354761245733108602515560771616386164701786923160710616372911987460222824515182772101258222613012299426153157712113377291111775621127770613277994115783551347843333480007368806341198066337690124170119953406120145405120304122120632407122417408122626384122743120122959135123137118124986374125200121125343479125507478125633297126564482126572481126778480126886501127044209127394205127665388128137502128145206128374391140762185911Malonyl-CoAHMDB0001175Malonyl-CoA, also known as malonyl CoA or CoA, malonyl, belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain. Malonyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). Malonyl-CoA has been found throughout most human tissues, and has also been primarily detected in urine. Within the cell, malonyl-CoA is primarily located in the mitochondria, peroxisome and cytoplasm. In humans, malonyl-CoA is involved in fatty acid biosynthesis pathway, the pyruvate metabolism pathway, and the propanoate metabolism pathway. Malonyl-CoA is also involved in several metabolic disorders, some of which include the primary hyperoxaluria II, PH2 pathway, pyruvate decarboxylase E1 component deficiency (pdhe1 deficiency), malonyl-CoA decarboxylase deficiency, and pyruvate dehydrogenase complex deficiency. Malonyl-CoA is a coenzyme A derivative which plays a key role in the fatty acid synthesis in the cytoplasmic and microsomal systems.524-14-1C000831066315531MALONYL-COA10213DB04524CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)C(O)C(=O)NCCC(=O)NCCSC(=O)CC(O)=OC24H38N7O19P3SInChI=1S/C24H38N7O19P3S/c1-24(2,19(37)22(38)27-4-3-13(32)26-5-6-54-15(35)7-14(33)34)9-47-53(44,45)50-52(42,43)46-8-12-18(49-51(39,40)41)17(36)23(48-12)31-11-30-16-20(25)28-10-29-21(16)31/h10-12,17-19,23,36-37H,3-9H2,1-2H3,(H,26,32)(H,27,38)(H,33,34)(H,42,43)(H,44,45)(H2,25,28,29)(H2,39,40,41)/t12-,17-,18-,19?,23-/m1/s1LTYOQGRJFJAKNA-VFLPNFFSSA-N3-{[2-(3-{3-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido}propanamido)ethyl]sulfanyl}-3-oxopropanoic acid853.58853.115602295-2.18103-({2-[3-(3-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-2-hydroxy-3-methylbutanamido)propanamido]ethyl}sulfanyl)-3-oxopropanoic acid0-5FDB001606Malonyl coa;Malonyl coenzyme a;Malonyl-coa;Malonyl-coenzyme a;Omega-carboxyacyl-coa;Omega-carboxyacyl-coenzyme a;S-(hydrogen malonyl)coenzyme a;S-(hydrogen propanedioate;S-(hydrogen propanedioate) coa;S-(hydrogen propanedioate) coenzyme a;S-(hydrogen propanedioic acidPW_C000911MalyCoA1788822684426326924160121381517783013278629133787771111214541221222574061224541241240121351248101201250211181260822971264204791266062991275382051279875011281803881316Carbon 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_C001316CO25081211204448013503186403677316952080651133431638491745225511731447052831035320111575010857711015968100602615560781616471178663710769221907017160703516370611887163205730819873332137461222753021082152258223151915824911849277119081701246422612688290426263154352331876994293771221337717013277470333777391127775012977763341780771347840535678427334789413317922713080008368806751198071713594836384113291391115549121119954406120089122120155407120364412120556414120833419120922124120991408121284125121505383122744120123011446123190450123418455123489118123556374123855136124063398125344479125460297125516481125824490125870299125931482126280480126887501127052206127277507127331388127390502140798185129L-AcetylcarnitineHMDB0000201L-Acetylcarnitine (ALCAR or ALC) is an acetic acid ester of carnitine that facilitates movement of acetyl-CoA into the matrices of mammalian mitochondria during the oxidation of fatty acids. In addition to his metabolic role, acetyl-L-carnitine posses unique neuroprotective, neuromodulatory, and neurotrophic properties this may play an important role in counteracting various disease processes (PMID ID: 15363640).3040-38-8C02571173024(-)o-acetylcarnitine21243783CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)CC9H17NO4InChI=1S/C9H17NO4/c1-7(11)14-8(5-9(12)13)6-10(2,3)4/h8H,5-6H2,1-4H3/t8-/m1/s1RDHQFKQIGNGIED-MRVPVSSYSA-N(3R)-3-(acetyloxy)-4-(trimethylazaniumyl)butanoate203.238203.115758031-2.860acetyl-L-carnitine00FDB021904(+-)-acetylcarnitine;(-)-acetylcarnitine;(r)-acetylcarnitine;Alcar;Acetyl-l-(-)-carnitine;Acetyl-l-carnitine;Acetyl-carnitine;Acetylcarnitine;L-acetylcarnitine;L-carnitine acetyl ester;L-o-acetylcarnitine;Levocarnitine acetyl;Nicetile;O-acetyl-l-carnitine;O-acetylcarnitine;3-(acetyloxy)-4-(trimethylammonio)butanoate;Acetyl-dl-carnitine;Dl-o-acetylcarnitine;3-(acetyloxy)-4-(trimethylammonio)butanoic acidPW_C000129L-Alcar24615247022506422515477559334775601337757313278441345806313748063212080645118122418408122424406122431418122527124124997454126565482126573479126579489126679299128138502128146501128152506128258388546Long-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.
HMDBP00577ACSL14q35CH47105616.2.1.388422241259261225254104132636166135628329137847713303Carnitine O-palmitoyltransferase 1, liver isoformP50416Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. Plays an important role in triglyceride metabolism.
HMDBP00309CPT1A11q13.2L3921112.3.1.2188722524810480321171356273291410514245Acetyl-CoA carboxylase 1Q13085Catalyzes the rate-limiting reaction in the biogenesis of long-chain fatty acids. Carries out three functions: biotin carboxyl carrier protein, biotin carboxylase and carboxyltransferase.
HMDBP00047ACACA17q21AY31562016.4.1.2; 6.3.4.14178982272213667765314099439314105916616Malonyl-CoA decarboxylase, mitochondrialO95822Catalyzes the conversion of malonyl-CoA to acetyl-CoA. In the fatty acid biosynthesis MCD selectively removes malonyl-CoA and thus assures that methyl-malonyl-CoA is the only chain elongating substrate for fatty acid synthase and that fatty acids with multiple methyl side chains are produced. In peroxisomes it may be involved in degrading intraperoxisomal malonyl-CoA, which is generated by the peroxisomal beta-oxidation of odd chain-length dicarboxylic fatty acids.
HMDBP00016MLYCD16q24BC05259214.1.1.92269445742141061166305Carnitine O-palmitoyltransferase 2, mitochondrialP23786HMDBP00311CPT21p32U0964312.3.1.218931726703267625233102135622134137852713141052614106380223795Long-chain fatty acid transport protein 1Q6PCB7
Mediates the ATP-dependent import of long-chain fatty acids (LCFA) into the cell by mediating their translocation at the plasma membrane (PubMed:28178239). Has also an acyl-CoA ligase activity for long-chain and very-long-chain fatty acids. May act directly as a bona fide transporter, or alternatively, in a cytoplasmic or membrane-associated multimeric protein complex to trap and draw fatty acids towards accumulation. Plays a pivotal role in regulating available LCFA substrates from exogenous sources in tissues undergoing high levels of beta-oxidation or triglyceride synthesis. May be involved in regulation of cholesterol metabolism (By similarity). Probably involved in fatty acid transport across the blood barrier (PubMed:21395585).
SLC27A116.2.1.152100Mitochondrial carnitine/acylcarnitine carrier proteinO43772Mediates the transport of acylcarnitines of different length across the mitochondrial inner membrane from the cytosol to the mitochondrial matrix for their oxidation by the mitochondrial fatty acid-oxidation pathway.
HMDBP02794SLC25A203p21.31Y1031918475171495Solute carrier family 22 member 5O76082Sodium-ion dependent, high affinity carnitine transporter. Involved in the active cellular uptake of carnitine. Transports one sodium ion with one molecule of carnitine. Also transports organic cations such as tetraethylammonium (TEA) without the involvement of sodium. Also relative uptake activity ratio of carnitine to TEA is 11.3HMDBP01612SLC22A55q31AB291606128Carnitine O-acetyltransferaseP43155Carnitine acetylase is specific for short chain fatty acids. Carnitine acetylase seems to affect the flux through the pyruvate dehydrogenase complex. It may be involved as well in the transport of acetyl-CoA into mitochondria.
HMDBP00028CRAT9q34.1X7982712.3.1.72460525203141053802246Long-chain-fatty-acid--CoA ligase 11PW_P00024626554611134231247Carnitine O-palmitoyltransferase 11PW_P0002472663036493Acetyl-CoA carboxylase 11PW_P00049351745123920124010272590Malonyl-CoA decarboxylase, mitochondrial1PW_P00059063416248Carnitine O-palmitoyltransferase 2, mitochondrial1PW_P000248267305115061Long-chain fatty acid transport protein 11PW_P015061250982379514105014644Mitochondrial carnitine/acylcarnitine carrier protein1PW_P0006447022100115062Organic cation/carnitine transporter 21PW_P01506225099149514106214628Carnitine O-acetyltransferase1PW_P000628674281217305falsePW_R217305Right8379961118131Compoundfalse8379974141Compoundtrue83799810991Compoundtrue8379991118141Compoundfalse838000321Compoundtrue8380011701Compoundtrue2069262466.2.1.3217306falsePW_R217306Both8380021118141Compoundfalse838003441Compoundtrue8380041118121Compoundfalse83800510991Compoundtrue2069272472.3.1.211248falsePW_R001248Right47849401Compoundfalse47859111Compoundfalse9624931247falsePW_R001247Right47829111Compoundfalse47839401Compoundfalse822013161Compoundtrue9615904.1.1.9217307falsePW_R217307Right8380061118121Compoundfalse83800710991Compoundfalse838008441Compoundfalse8380091118141Compoundfalse2069282481381falsePW_R001381Both52519401Compoundfalse5252441Compoundtrue52531291Compoundfalse525410991Compoundtrue11016282.3.1.713474PW_T013474Active138811118131Compound157Right4580150612021-04-17T03:39:06-06:002021-04-17T03:39:06-06:001413475PW_T013475Active138821118121Compound426Right45816442021-04-17T03:39:07-06:002021-04-17T03:39:07-06:001711315PW_T011315Active11722441Compound15166Right2422150622021-04-12T09:56:12-06:002021-04-12T09:56:12-06:00143511InhibitionPW_I0035117021911Compound17022247ProteinComplex1312984242329false117544010regular10025312984311181373false140044010regular1001403129844111813153false140010010regular1001403129845414242false134540510regular503031298461099285false134555010regular5030312984710994285false93771610regular50303129848109980285false927121110regular50303129849109985false1852138610regular5030312985011181423false92544110regular100140312985111181463false1267107610regular100140312985232244false104540110regular50303129853170245false103354110regular634331298544483false78038510regular1001003129855448023false1137118110regular100100312985644153false77910010regular100100312985744166156true54932010regular1001003129858443false1852118610regular1001003129859111812423false77775610regular100130312986011181263false777107110regular1001303129861201669false39537210regular10025312986210271669false39535210regular1002531298639403false1642107610regular10010031298649401663false22049510regular10010031298659111663false59047510regular1001003129866131616652false23177210regular787831298671293false1647143110regular100110120739854622false11504558subunitregular1507012073993034217false7405988subunitregular180851207400451662false3703878subunitregular150701207401161662false3756458subunitregular1507012074023058022false9879168subunitregular1507012074032379576false13752708subunitregular150701207404210076false7529218subunitregular150701207405149576false7542758proteinregular150701207406282false162212818subunitregular150709515182461165412120239812073984419931298424273573Cofactor9515192471165414212023991207399951520493116541166120240012074004420031298614273584Cofactor4420131298624273585Cofactor95152159011654116612024011207401951522248116541802120240212074029515231506111654112024031207403951524644116541120240412074049515251506211654112024051207405951526628116541120240612074064273568M1742 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42.646910088549894273603M1693 1176 C1696 1215 1692 1223 1696 1281 5false18trueM 825.6135420558836 552.9282433080148 L 810.6666870117188 551.6666870117188 L 817.0475747329045 565.2418213347971falsefalsefalse4273604M1699 1430 C1699 1397 1701 1386 1699 1351 5false18trueM 825.6135420558836 552.9282433080148 L 810.6666870117188 551.6666870117188 L 817.0475747329045 565.2418213347971false4273605M1852 1236 C1822 1236 1705 1237 1700 1279 5false18trueM 825.6135420558836 552.9282433080148 L 810.6666870117188 551.6666870117188 L 817.0475747329045 565.2418213347971false4273606M1852 1401 C1825 1403 1709 1398 1697 1351 5false18trueM 825.6135420558836 552.9282433080148 L 810.6666870117188 551.6666870117188 L 817.0475747329045 565.2418213347971false920536116541217305374652031298434273574Left374652131298454273575Left374652231298464273576Left374652331298504273577Right374652431298524273578Right374652531298534273579Right874912288951518920537116541217306374652631298504273580Left374652731298544273581Left374652831298594273582Right374652931298474273583Right8749132899515199205381165411248374653031298644273586Left374653131298654273587Right8749149629515209205391165411247374653231298654273588Left374653331298644273589Right374653431298664273590Right874915961951521920540116541217307374653531298604273591Left374653631298484273592Left374653731298554273593Right374653831298514273594Right8749162036659515229205411165411381374653931298634273603Left374654031298584273604Left374654131298674273605Right374654231298494273606Right87491711019515261020461347411654120564631298434273595Right20564731298444273596Left4815195152324181020471347511654120564831298594273597Left20564931298604273598Right4815295152424191020481131511654120565031298564273599Left20565131298574273600Right481539515252422107893511116541140699410084273601Right140709410084273602Right11604111626011654114false1788109116regular11357431298634273568Left11604211626111654114false1429109116regular11357531298514273569Left72697427357172698427357247290116714231.01.0-90290484497471279M123 410 C123 360 173 310 223 310 C777 310 1496 310 2050 310 C2100 310 2150 360 2150 410 C2150 771 2150 1241 2150 1602 C2150 1652 2100 1702 2050 1702 C1496 1702 777 1702 223 1702 C173 1702 123 1652 123 1602 C123 1241 123 771 123 410 1true62027.01392.0471280M698.9999847412109 644.3333282470703 C756.9999847412109 644.3333282470703 1973.999984741211 642.3333282470703 2026.999984741211 643.3333282470703 84false60.00.0471281M701.9999847412109 955.3333282470703 C758.9999847412109 956.3333282470703 1953.999984741211 948.3333282470703 2025.999984741211 949.3333282470703 84false60.00.087967115Mitochondrial Outer Membrane1804595201.31.31601587967215Mitochondrial Inner Membrane1803900201.31.31601587967315Extracellular Space1758180201.61.61601587967415Cytosol1753320201.61.61601587967515Intermembrane Space1334645201.61.61601587967615Mitochondrial Matrix7151475201.61.61601528086461811907016182027157639#FFEBEB41326958