234Context2-Ketoglutarate Dehydrogenase Complex Deficiency2-Ketoglutarate dehydrogenase complex deficiency is a rare autosomal recessive disease. 2-ketoglutarate dehydrogenase is an enzyme of the Krebs cycle that catalyzes the oxidation of alpha-ketoglutarate to succinyl CoA. The deficiency of 2-Ketoglutarate dehydrogenase complex results in the disorder of Krebs cycle with accumulation of succinyl CoA. The primary manifestations include developmental delay, ataxia, opisthotonus, seizure and other neurological symptoms. DiseasePW000525CenterPathwayVisualizationContext55933503150#000099PathwayVisualization72Citric Acid CycleThe citric acid cycle, which is also known as the tricarboxylic acid cycle (TCA cycle) or the Krebs cycle, is a connected series of enzyme-catalyzed chemical reactions of central importance to all aerobic organisms (i.e. organisms that use oxygen for cellular respiration). The citric acid cycle is named after citrate or citric acid, a tricarboxylic acid that is both consumed and regenerated through this pathway. The citric acid cycle was discovered in 1937 by Hans Adolf Krebs while he worked at the University of Sheffield in England (PMID: 16746382). Krebs received the Nobel Prize for his discovery in 1953. Krebs’ extensive work on this pathway is also why the citric acid or TCA cycle is often referred to as the Krebs cycle. Metabolically, the citric acid cycle allows the release of energy (ultimately in the form of ATP) from carbohydrates, fats, and proteins through the oxidation of acetyl-CoA. The citric acid cycle also produces CO2, the precursors for several amino acids (aspartate, asparagine, glutamine, proline) and NADH – all of which are used in other important metabolic pathways, such as amino acid synthesis and oxidative phosphorylation (OxPhos). The net yield of one “turn” of the TCA cycle in terms of energy-containing compounds is one GTP, one FADH2, and three NADH molecules. The NADH molecules are used in oxidative phosphorylation to generate ATP. In eukaryotes, the citric acid cycle occurs in the mitochondrial matrix. In prokaryotes, the citric acid cycle occurs in the cytoplasm. In eukaryotes, the citric acid or TCA cycle has a total of 10 steps that are mediated by 8 different enzymes. Key to the whole cycle is the availability of acetyl-CoA. One of the primary sources of acetyl-CoA is from the breakdown of glucose (and other sugars) by glycolysis. This process generates pyruvate. Pyruvate is decarboxylated by pyruvate dehydrogenase to generate acetyl-CoA. The citric acid cycle begins with acetyl-CoA transferring its two-carbon acetyl group to the four-carbon acceptor compound (oxaloacetate) to form a six-carbon compound (citrate) through the enzyme citrate synthase. The resulting citrate is then converted to cis-aconitate and then isocitrate via the enzyme aconitase. The resulting isocitrate then combines with NAD+ to form oxalosuccinate and NADH, which is then converted into alpha-ketoglutarate (and CO2) through the action of the enzyme known as isocitrate dehydrogenase. The resulting alpha-ketoglutarate combines with NAD+ and CoA-SH to produce succinyl-CoA, NADH, and CO2. This step is mediated by the enzyme alpha-ketoglutarate dehydrogenase. The resulting succinyl-CoA combines with GDP and organic phosphate to produce succinate, CoA-SH, and GTP. This phosphorylation reaction is performed by succinyl-CoA synthase. The resulting succinate then combines with ubiquinone to produce two compounds, fumarate and ubiquinol through the action of the enzyme succinate dehydrogenase. The resulting fumarate is then hydrated by the enzyme known as fumarase to produce malate. The resulting malate is oxidized via NAD+ to produce oxaloacetate and NADH. This oxidation reaction is performed by malate dehydrogenase. The resulting oxaloacetate can then combine with acetyl-CoA and the TCA reaction cycle begins again. Overall, in the citric acid cycle, the starting six-carbon citrate molecule loses two carboxyl groups as CO2, leading to the production of a four-carbon oxaloacetate. The two-carbon acetyl-CoA that is the “fuel” for the TCA cycle can be generated by several metabolic pathways including glucose metabolism, fatty acid oxidation, and the metabolism of amino acids. The overall reaction for the citric acid cycle is as follows: acetyl-CoA + 3 NAD+ + FAD + GDP + P + 2H2O = CoA-SH + 3NADH + FADH2 + 3H+ + GTP + 2CO2. Many molecules in the citric acid cycle serve as key precursors for other molecules needed by cells. The citrate generated via the citric acid cycle can serve as an intermediate for fatty acid synthesis; alpha-ketoglutarate can serve as a precursor for glutamate, proline, and arginine; oxaloacetate can serve as a precursor for aspartate and asparagine; succinyl-CoA can serve as a precursor for porphyrins; and acetyl-CoA can serve as a precursor fatty acids, cholesterol, vitamin D, and various steroid hormones. There are several variations to the citric acid cycle that are known. Interestingly, most of the variation lies with the step involving succinyl-CoA production or conversion. Humans and other animals have two different types of succinyl-CoA synthetases. One produces GTP from GDP, while the other produces ATP from ADP (PMID: 9765291). On the other hand, plants have a succinyl-CoA synthetase that produces ATP (ADP-forming succinyl-CoA synthetase) (Jones RC, Buchanan BB, Gruissem W. (2000). Biochemistry & molecular biology of plants (1st ed.). Rockville, Md: American Society of Plant Physiologists. ISBN 0-943088-39-9.). In certain acetate-producing bacteria, such as Acetobacter aceti, an enzyme known as succinyl-CoA:acetate CoA-transferase performs this conversion (PMID: 18502856) while in Helicobacter pylori succinyl-CoA:acetoacetate CoA-transferase is responsible for this reaction (PMID: 9325289). The citric acid cycle is regulated in a number of ways but the primary mechanism is by product inhibition. For instance, NADH inhibits pyruvate dehydrogenase, isocitrate dehydrogenase, alpha-ketoglutarate dehydrogenase, and citrate synthase. Acetyl-CoA inhibits pyruvate dehydrogenase, while succinyl-CoA inhibits alpha-ketoglutarate dehydrogenase and citrate synthase. Additionally, ATP inhibits citrate synthase and alpha-ketoglutarate dehydrogenase. Calcium is another important regulator of the citric acid cycle. In particular, it activates pyruvate dehydrogenase phosphatase, which then activates pyruvate dehydrogenase. Calcium also activates isocitrate dehydrogenase and alpha-ketoglutarate dehydrogenase (PMID: 171557).Metabolic135277SubPathway545164Compound235376SubPathway546164Compound235484SubPathway547164Compound235560SubPathway548148Compound435677SubPathway549148Compound43573SubPathway550148Compound43584SubPathway551148Compound435984SubPathway552148Compound43604SubPathway55388Compound436187SubPathway55488Compound436215SubPathway55588Compound436378SubPathway55688Compound4364101SubPathway557174Compound4365348SubPathway558932Compound1736660SubPathway559134Compound43677SubPathway560134Compound43683SubPathway561134Compound436977SubPathway562134Compound437075SubPathway563134Compound437186SubPathway564940Compound437289SubPathway565940Compound437384SubPathway566940Compound437410SubPathway567940Compound437555SubPathway568940Compound437658SubPathway569940Compound4377342SubPathway570940Compound4378101SubPathway571940Compound4131Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.2Pathway132Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.2Pathway28028516746382Krebs HA, Johnson WA: Metabolism of ketonic acids in animal tissues. Biochem J. 1937 Apr;31(4):645-60. doi: 10.1042/bj0310645.2Pathway2802869765291Johnson JD, Mehus JG, Tews K, Milavetz BI, Lambeth DO: Genetic evidence for the expression of ATP- and GTP-specific succinyl-CoA synthetases in multicellular eucaryotes. J Biol Chem. 1998 Oct 16;273(42):27580-6. doi: 10.1074/jbc.273.42.27580.2Pathway28028718502856Mullins EA, Francois JA, Kappock TJ: A specialized citric acid cycle requiring succinyl-coenzyme A (CoA):acetate CoA-transferase (AarC) confers acetic acid resistance on the acidophile Acetobacter aceti. J Bacteriol. 2008 Jul;190(14):4933-40. doi: 10.1128/JB.00405-08. Epub 2008 May 23.2Pathway2802889325289Corthesy-Theulaz IE, Bergonzelli GE, Henry H, Bachmann D, Schorderet DF, Blum AL, Ornston LN: Cloning and characterization of Helicobacter pylori succinyl CoA:acetoacetate CoA-transferase, a novel prokaryotic member of the CoA-transferase family. J Biol Chem. 1997 Oct 10;272(41):25659-67. doi: 10.1074/jbc.272.41.25659.2Pathway280289171557Denton RM, Randle PJ, Bridges BJ, Cooper RH, Kerbey AL, Pask HT, Severson DL, Stansbie D, Whitehouse S: Regulation of mammalian pyruvate dehydrogenase. Mol Cell Biochem. 1975 Oct 31;9(1):27-53.2Pathway28036522628558Bricker DK, Taylor EB, Schell JC, Orsak T, Boutron A, Chen YC, Cox JE, Cardon CM, Van Vranken JG, Dephoure N, Redin C, Boudina S, Gygi SP, Brivet M, Thummel CS, Rutter J: A mitochondrial pyruvate carrier required for pyruvate uptake in yeast, Drosophila, and humans. Science. 2012 Jul 6;337(6090):96-100. doi: 10.1126/science.1218099. Epub 2012 May 24.2Pathway1CellCL:00000006MyocyteCL:00001875HepatocyteCL:00001824Cardiomyocyte CL:00007463NeuronCL:00005407Epithelial CellCL:00000662Platelet CL:00002338Beta cellCL:00006391Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote17Rattus norvegicus10116EukaryoteRat12Mus musculus10090EukaryoteMouse2Bacteria2ProkaryoteBacteria24Solanum lycopersicum4081EukaryoteTomato4Arabidopsis thaliana3702EukaryoteThale cress18Saccharomyces cerevisiae4932EukaryoteYeast21Xenopus laevis8355EukaryoteAfrican clawed frog60Nitzschia sp.0001EukaryoteNitzschia45Bos taurus9913EukaryoteCattle10Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm23Pseudomonas aeruginosa287Prokaryote19Schizosaccharomyces pombe4896Eukaryote49Bathymodiolus platifrons220390EukaryoteDeep sea mussel25Escherichia coli (strain K12)83333Prokaryote29Saccharomyces cerevisiae (strain ATCC 204508 / S288c)559292EukaryoteBaker's yeast51Picea sitchensis3332EukaryoteSitka spruce135Felinus9685EukaryoteCat1CytosolGO:00058293Mitochondrial MatrixGO:00057595CytoplasmGO:00057372MitochondrionGO:00057397Endoplasmic Reticulum MembraneGO:000578912Mitochondrial Inner MembraneGO:000574310Cell MembraneGO:000588635ChloroplastGO:000950713Endoplasmic ReticulumGO:000578314Mitochondrial Outer MembraneGO:000574127Peroxisome MembraneGO:00057784PeroxisomeGO:00057778Smooth Endoplasmic Reticulum GO:000579025Golgi apparatusGO:00057946LysosomeGO:000576416Lysosomal LumenGO:004320231Periplasmic SpaceGO:000562011Extracellular SpaceGO:000561524Mitochondrial Intermembrane SpaceGO:000575820Endoplasmic Reticulum LumenGO:000578834Plant-Type VacuoleGO:000032539Mitochondrial membraneGO:003196618Melanosome MembraneGO:003316221SynapseGO:004520215NucleusGO:000563436MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016840PeriplasmGO:004259732Inner MembraneGO:007025826Golgi apparatus membraneGO:000013919sarcoplasmic reticulumGO:00165299MuscleBTO:0000887141181LiverBTO:000075972924BrainBTO:000014289164Adrenal MedullaBTO:000004971828StomachBTO:00013071552625IntestineBTO:00006487Nervous SystemBTO:00014848Blood VesselBTO:0001102741111HeartBTO:000056273102Endothelium 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351PW_BS00051270421351PW_BS000512164Pyruvic acidHMDB0000243Pyruvic acid is an intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed.) Biological Source: Intermediate in primary metabolism including fermentation processes. Present in muscle in redox equilibrium with Lactic acid. A common constituent, as a chiral cyclic acetal linked to saccharide residues, of bacterial polysaccharides. Isolated from cane sugar fermentation broth and peppermint. Constituent of Bauhinia purpurea, Cicer arietinum (chickpea), Delonix regia, Pisum sativum (pea) and Trigonella caerulea (sweet trefoil) Use/Importance: Reagent for regeneration of carbonyl compdounds from semicarbazones, phenylhydrazones and oximes. Flavoring ingredient (Dictionary of Organic Compounds).127-17-3C00022106032816PYRUVATE1031DB00119CC(=O)C(O)=OC3H4O3InChI=1S/C3H4O3/c1-2(4)3(5)6/h1H3,(H,5,6)LCTONWCANYUPML-UHFFFAOYSA-N2-oxopropanoic acid88.062188.0160439940.181pyruvic acid0-1FDB0082932-oxopropanoate;2-oxopropanoic acid;2-oxopropionate;2-oxopropionic acid;Acetylformate;Acetylformic acid;Bts;Pyroracemate;Pyroracemic acid;Pyruvate;A-ketopropionate;A-ketopropionic acid;Alpha-ketopropionate;Alpha-ketopropionic acid;2-ketopropionic acid;2-oxopropansaeure;2-oxopropionsaeure;Acide pyruvique;Alpha-oxopropionsaeure;Brenztraubensaeure;Ch3cocooh;2-ketopropionate;α-ketopropionate;α-ketopropionic acid;A-oxopropionsaeure;α-oxopropionsaeurePW_C000164Pyr1722044228118131449501457265365103540511754401185444120556613255701335893955920147595115160221556067156607416161261606383164671786510177653285745722274952208200225126223115292249153491877310111779723467797832778090112800043688004236780695135112879941156831211199504061200111241201751221208784071211484231211544241234541191237204581237264591253404791253902991255342971258544811268835011269313881270672051278582061099Coenzyme 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_C001099CoA2114386884538792289217240759241422459528132928623133421133511846181046295848421448655448796523210252471045280103547712457341085777101602315560751616384164681786930160696116269731997083188710816372931987347210745822282291519081226909022491241709215195130132991531824925488494261631576907293771191337722213477230329772921117755013277555334775631127763333677672129779961157804733278056350784133357856713079259333799743318000536880620118806273748063511980665376938283829383438398674288110555389110561390115842399115847398119951406120147405120231384120305122120634407120762117121406123121421433121521125121666429121682408121714414122404422122741120122904121122960135123965447123979468124079136124220464124265450124974375125341479125509478125579480125592484125634297126084481126549491126560482126746300126884501127046209127109391127301205127540206127667388128121508128133502128340395721NADHMDB0000902NAD (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]1OC21H28N7O14P2InChI=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)/p+1/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BAWFJGJZGIEFAR-NNYOXOHSSA-O1-[(2R,3R,4S,5R)-5-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium664.433664.116946663-2.5981-[(2R,3R,4S,5R)-5-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium1-1FDB0223093-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_C000721NAD14041503353865110111421134431273514665422294927791728352931079480718481318481928490264960315167955238103533411153601125469123548212555901355610118569610057381085827141591214759421516024155607215760761616385164691786772117689016070121887097163717420571972067405198745922282412268359225908522411819216123222491300629813018300132562234240432242619315771041327712013377209134773703317765033677667334777023327770913077915113779833477840635680006368806901199382512411055238811275016611285394119929122119952406120171407120834419120984408121159425121242126121259429121817383122614384122742120123130447123141136123419455123549374123731460123812443123829464124370398125187121125319297125342479125530481125806299125825490125924482126515495126765480126885501127278507127383502128089390128360391128428395940Acetyl-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-CoA21343858842324162244652896173340114840145278103547612457331086025155607716163861647017869231607106163729119874602228245151827721012582226130122994261531577121133772911117756211277706132779941157835513478433334800073688063411980663376901241701199534061201454051203041221206324071224174081226263841227431201229591351231371181249863741252001211253434791255074781256332971265644821265724811267784801268865011270442091273942051276653881281375021281452061283743911316Carbon 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_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905021144NADHHMDB0001487NADH 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_C001144NADH143415334908648101115212755146954223049278117283629310994806184812184821284904649593151699552401035332111535811254661235479125559313556981005737108582914159151475945151602715560791616387164721786771117689316070111887099163717220571952067462222824422683602259086224118091981182121612320249130032981301530013255223424033224261831577107132771231337720813477371331776513367766833477700332777071307791711377986347800093688069111993822124110549388112854941158381181199554061201724071203781221209864081211624251212441261216934291218183831226163841227451201231274471231381361235513741237344601238144431242424641243713981251891211253454791255314811257622971258082991259264821265164951267674801268885011273855021280903901283623911284293951060Thiamine 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(CCO[P@](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_C001060ThiamPP205410753119781271517362536610360281556080161638816473178746322212806225771241337828511278423334790181117917513280010368119956406120802407120902122120982408121537124122746120123388119123473135123547374124095118125346479125922482126094481126802299126889501127381502127549206128400388769LipoamideHMDB0000962Lipoamide is the oxidized form of glutathione. (PMID:8957191). Lipoamide is a trivial name for 6,8-dithiooctanoic amide. It is 6,8-dithiooctanoic acid's functional form where the carboxyl group is attached to protein (or any other amine) by an amide linkage (containing -NH2) to an amino group. Lipoamide forms a thioester bond, oxidizing the disulfide bond, with acetaldehyde (pyruvate after it has been decarboxylated). It then transfers the acetaldehyde group to CoA which can then continue in the TCA cycle. (Wikipedia). Lipoamide is an intermediate in glycolysis/gluconeogenesis, citrate cycle (TCA cycle), alanine, aspartate and pyruvate metabolism, and valine, leucine and isoleucine degradation (KEGG:C00248). It is generated from dihydrolipoamide via the enzyme dihydrolipoamide dehydrogenase (EC:1.8.1.4) and then converted to S-glutaryl-dihydrolipoamide via the enzyme oxoglutarate dehydrogenase (EC:1.2.4.2).940-69-2C0024886317460LIPOAMIDE840NC(=O)CCCCC1CCSS1C8H15NOS2InChI=1S/C8H15NOS2/c9-8(10)4-2-1-3-7-5-6-11-12-7/h7H,1-6H2,(H2,9,10)FCCDDURTIIUXBY-UHFFFAOYSA-N5-(1,2-dithiolan-3-yl)pentanamide205.341205.059505487-3.311lipoamide00FDB0223401,2-dithiolane-3-pentanamide;5-(1,2-dithiolan-3-yl)-pentanamide;5-(1,2-dithiolan-3-yl)pentanamide;5-(1,2-dithiolan-3-yl)valeramide;5-(dithiolan-3-yl)valeramide;Dl-lipoamide;Dl-6-thioctic amide;Lipamide;Lipoacin;Lipoamid;Lipoicin;Lipozyme;Lypoaran;Pathoclon;Thioami;Thioctamid;Thioctamide;Thioctic acid amide;Thioctic acid amide (jan);Thiotomin;Ticolin;Vitamin n;Alpha-lipoate;Alpha-lipoic acid;Alpha-lipoic acid amide;A-lipoate amide;A-lipoic acid amide;Alpha-lipoate amide;α-lipoate amide;α-lipoic acid amide;Thioctate amidePW_C000769Lipoamd2024107331734246678536710360291556081161638916474178746422277125133782861127917313280011368119957406120803407121535124122747120123389119124093118125347479126078481126890501127534206964FADHMDB0001248FAD, also known as flavitan or adeflavin, belongs to the class of organic compounds known as flavin nucleotides. These are nucleotides containing a flavin moiety. Flavin is a compound that contains the tricyclic isoalloxazine ring system, which bears 2 oxo groups at the 2- and 4-positions. FAD is a drug which is used to treat eye diseases caused by vitamin b2 deficiency, such as keratitis and blepharitis. FAD is slightly soluble (in water) and a moderately acidic compound (based on its pKa). FAD has been found in human liver and muscle tissues, and has also been detected in multiple biofluids, such as feces and blood. Within the cell, FAD is primarily located in the cytoplasm, mitochondria, endoplasmic reticulum and peroxisome. FAD exists in all living organisms, ranging from bacteria to humans. In humans, FAD is involved in the risedronate action pathway, the ibandronate action pathway, the valine, leucine and isoleucine degradation pathway, and the pyrimidine metabolism pathway. FAD is also involved in several metabolic disorders, some of which include the oncogenic action OF L-2-hydroxyglutarate in hydroxygluaricaciduria pathway, gaba-transaminase deficiency, 4-hydroxybutyric aciduria/succinic semialdehyde dehydrogenase deficiency, and the saccharopinuria/hyperlysinemia II pathway. FAD is a condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972).146-14-5C0001664397516238FAD559059DB03147CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)CO[P@](O)(=O)O[P@@](O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=NC(=O)NC(=O)C1=N2C27H33N9O15P2InChI=1S/C27H33N9O15P2/c1-10-3-12-13(4-11(10)2)35(24-18(32-12)25(42)34-27(43)33-24)5-14(37)19(39)15(38)6-48-52(44,45)51-53(46,47)49-7-16-20(40)21(41)26(50-16)36-9-31-17-22(28)29-8-30-23(17)36/h3-4,8-9,14-16,19-21,26,37-41H,5-7H2,1-2H3,(H,44,45)(H,46,47)(H2,28,29,30)(H,34,42,43)/t14-,15+,16+,19-,20+,21+,26+/m0/s1VWWQXMAJTJZDQX-UYBVJOGSSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}[({[(2R,3S,4S)-5-{7,8-dimethyl-2,4-dioxo-2H,3H,4H,10H-benzo[g]pteridin-10-yl}-2,3,4-trihydroxypentyl]oxy}(hydroxy)phosphoryl)oxy]phosphinic acid785.5497785.157134455-2.279flavine-adenine dinucleotide0-3FDB0225111h-purin-6-amine flavin dinucleotide;1h-purin-6-amine flavine dinucleotide;Adenine-flavin dinucleotide;Adenine-flavine dinucleotide;Adenine-riboflavin dinuceotide;Adenine-riboflavin dinucleotide;Adenine-riboflavine dinucleotide;Fad;Flamitajin b;Flanin f;Flavin adenine dinucleotide;Flavin adenine dinucleotide oxidized;Flavin-adenine dinucleotide;Flavine adenosine diphosphate;Flavine-adenine dinucleotide;Flavitan;Flaziren;Isoalloxazine-adenine dinucleotide;Riboflavin 5'-adenosine diphosphate;Riboflavin-adenine dinucleotide;Riboflavine-adenine dinucleotide;AdeflavinPW_C000964FAD99911451868192321642531762828825188402118814148942161229162249213358253622372326460236468831474113475810488165268103528510253351115496126551112756131186030155605415660821616116162639016475178649917966661077039163717520573212137465222748722390762241181821611887215118992111229622512328249124431511251922712595226127102911272029213029301130413024362331877080293771261337715213477501113775071127751811577541334776151327772633778054329783753457893033179222336792723588001236880034369807141191199584061199993841200514081201074071204324051204531221204901241212784291212984181214173821214893831227481201227761211228023741228234431230663761230871351231664481238494641238684541239763991240473981253484791253784801254294821254744811256972971259794891261072991262774841268915011269203911269685021269872071270112061273102091274325061276023881278403891420WaterHMDB0002111Water 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_C001420H2O5589491095139415131621448113526156242865210691207703382318838210943113774914655415904320182425322226786027274627781728052931437031647236346145983647273749419350302751567519597521410052279452361035297105531911153431135355112540211054701235483125549212655071275534130553711455411295591135560811856221085691657591405778101584114358531465877107589095591014759401516032155605915760871616123163613315962151621816664771786507180660015267131176840188688816071622057181207719320672112117228213723821472432157295198735021673882107401212746722274922247500190758817082012258237226841416292652611850277119221641201128112213285122502861226428712327249125202271263265126932901270529112715292130072981301930013025301130373021326122313327294153403084232731542695318436913227691429377019253771021327713113377215134773783317739733277471333775161157753633477628336777223377775934177816343779823477807132978235352782423537827035679113360800143688003937080591228806561199383038394794384110557390110639391115844398119879232119915122119963406120008407120046408120113124120365412120430405120438409120606415120794414121158425121240429121351121121381419121607434122118382122384436122753120122797374122804443123012446123064376123072137123131447123142136123162448123231451123384450123730460123810464123940455124165469124670399124938471124945472125305297125353479125386481125424482125480299125682483125707478125745487126054490126238495126273484126764480126896501126963502127017388127177208127199209127227504127506507127576515127836389128082395128176513148Oxalacetic acidHMDB0000223Oxaloacetic acid, also known as oxosuccinic acid or oxalacetic acid, is a four-carbon dicarboxylic acid appearing as an intermediate of the citric acid cycle. In vivo, oxaloacetate (the ionized form of oxaloacetic acid) is formed by the oxidation of L-malate, catalyzed by malate dehydrogenase, and reacts with Acetyl-CoA to form citrate, catalyzed by citrate synthase.(wikipedia) A class of ketodicarboxylic acids derived from oxalic acid. Oxaloacetic acid is an intermediate in the citric acid cycle and is converted to aspartic acidD by a transamination reaction.328-42-7C0003697030744OXALACETIC_ACID945OC(=O)CC(=O)C(O)=OC4H4O5InChI=1S/C4H4O5/c5-2(4(8)9)1-3(6)7/h1H2,(H,6,7)(H,8,9)KHPXUQMNIQBQEV-UHFFFAOYSA-N2-oxobutanedioic acid132.0716132.005873238-0.362oxalacetate0-2FDB0014792-ketosuccinate;2-ketosuccinic acid;2-oxobutanedioate;2-oxobutanedioic acid;2-oxosuccinate;2-oxosuccinic acid;Ketosuccinate;Ketosuccinic acid;Oaa;Oxalacetate;Oxaloacetate;Oxaloacetic acid;Oxaloethanoate;Oxaloethanoic acid;Oxosuccinate;Oxosuccinic acid;A-ketosuccinate;A-ketosuccinic acid;Alpha-ketosuccinate;Alpha-ketosuccinic acid;3-carboxy-3-oxopropanoic acid;Keto-succinic acid;Oxalacetic acid;Oxobutanedioic acid;3-carboxy-3-oxopropanoate;Keto-succinate;OxobutanedioatePW_C000148Oaa2549691115109931109421113216888537110354481205574133603315560881616478178746822275132247517151837222083782251174411711891160127072911271729243792322775081327753311377538334779581127800911178290345800153688070013511996440612004840812006212612018012212041912412081541812120740712279937412281344312305511812340045412377711912535447912542648212543930112553729712580148912580729912689750112696550212697720712707020512725650612726138863Citric acidHMDB0000094Citric acid (citrate) is a weak acid that is formed in the tricarboxylic acid cycle or that may be introduced with diet. The evaluation of plasma citric acid is scarcely used in the diagnosis of human diseases. On the contrary urinary citrate excretion is a common tool in the differential diagnosis of kidney stones, renal tubular acidosis and it plays also a role in bone diseases. The importance of hypocitraturia should be considered with regard to bone mass, urine crystallization and urolithiasis. (PMID 12957820) The secretory epithelial cells of the prostate gland of humans and other animals posses a unique citrate-related metabolic pathway regulated by testosterone and prolactin. This specialized hormone-regulated metabolic activity is responsible for the major prostate function of the production and secretion of extraordinarily high levels of citrate. The key regulatory enzymes directly associated with citrate production in the prostate cells are mitochondrial aspartate aminotransferase, pyruvate dehydrogenase, and mitochondrial aconitase. testosterone and prolactin are involved in the regulation of the corresponding genes associated with these enzymes. The regulatory regions of these genes contain the necessary response elements that confer the ability of both hormones to control gene transcription. Protein kinase c (PKC) is the signaling pathway for the prolactin regulation of the metabolic genes in prostate cells. testosterone and prolactin regulation of these metabolic genes (which are constitutively expressed in all mammalian cells) is specific for these citrate-producing cells. (PMID 12198595) Citric acid is found in citrus fruits, most concentrated in lemons and limes, where it can comprise as much as 8% of the dry weight of the fruit. Citric acid is a natural preservative and is also used to add an acidic (sour) taste to foods and soft drinks. The salts of citric acid (citrates) can be used as anticoagulants due to their calcium chelating ability. Intolerance to citric acid in the diet is known to exist. Little information is available as the condition appears to be rare, but like other types of food intolerance it is often described as a "pseudo-allergic" reaction.77-92-9C001581978290430769CIT305DB04272OC(=O)CC(O)(CC(O)=O)C(O)=OC6H8O7InChI=1S/C6H8O7/c7-3(8)1-6(13,5(11)12)2-4(9)10/h13H,1-2H2,(H,7,8)(H,9,10)(H,11,12)KRKNYBCHXYNGOX-UHFFFAOYSA-N2-hydroxypropane-1,2,3-tricarboxylic acid192.1235192.02700261-0.264citric acid0-3FDB0125862-hydroxy-1,2,3-propanetricarboxylate;2-hydroxy-1,2,3-propanetricarboxylic acid;3-carboxy-3-hydroxypentane-1,5-dioate;3-carboxy-3-hydroxypentane-1,5-dioic acid;Aciletten;Anhydrous citrate;Anhydrous citric acid;Chemfill;Citraclean;Citrate;Citretten;Citric acid;Citro;E 330;Hydrocerol a;Kyselina citronova;Suby g;Uro-trainer;Beta-hydroxytricarballylate;Beta-hydroxytricarballylic acid;2-hydroxytricarballylic acid;Citronensaeure;E330;H3cit;2-hydroxytricarballylatePW_C000063CA21942415253721036034155608916164791787469222771321337905313280016368111712811996540612239712412275412012496711812535547912653829912689850112811138851cis-Aconitic acidHMDB0000072cis-Aconitic acid is an intermediate in the tricarboxylic acid cycle produced by the dehydration of citric acid. The enzyme aconitase (aconitate hydratase; EC 4.2.1.3) catalyses the stereo-specific isomerization of citrate to isocitrate via cis-aconitate in the tricarboxylic acid cycle.585-84-2C0041764375732805CIS-ACONITATE558863OC(=O)C\C(=C\C(O)=O)C(O)=OC6H6O6InChI=1S/C6H6O6/c7-4(8)1-3(6(11)12)2-5(9)10/h1H,2H2,(H,7,8)(H,9,10)(H,11,12)/b3-1-GTZCVFVGUGFEME-IWQZZHSRSA-N(1Z)-prop-1-ene-1,2,3-tricarboxylic acid174.1082174.016437924-1.413cis-aconitic acid0-3FDB008306(1z)-1-propene-1,2,3-tricarboxylate;(1z)-1-propene-1,2,3-tricarboxylic acid;(z)-1-propene-1,2,3-tricarboxylate;(z)-1-propene-1,2,3-tricarboxylic acid;(z)-aconitate;(z)-aconitic acid;1-propene-1,2,3-tricarboxylate;1-propene-1,2,3-tricarboxylic acid;1-cis-2,3-propenetricarboxylate;1-cis-2,3-propenetricarboxylic acid;Cis-1-propene-1,2,3-tricarboxylate;Cis-1-propene-1,2,3-tricarboxylic acid;Cis-aconate;Cis-aconic acid;Cis-aconitate;Cis-aconitic acid;Cis-oxaloacetate;Cis-oxaloacetic acidPW_C000051Aconiti222454011036058155612216165061787491222117451177715913380038368120007406122783120125385479126927501125Isocitric acidHMDB0000193The citrate oxidation to isocitrate is catalyzed by the enzyme aconitase. Human prostatic secretion is remarkably rich in citric acid and low aconitase activity will therefore play a significant role in enabling accumulation of high citrate levels (PubMed ID 8115279).320-77-4C00311119830887threo-d(s)-iso-citrate1161OC(C(CC(O)=O)C(O)=O)C(O)=OC6H8O7InChI=1S/C6H8O7/c7-3(8)1-2(5(10)11)4(9)6(12)13/h2,4,9H,1H2,(H,7,8)(H,10,11)(H,12,13)ODBLHEXUDAPZAU-UHFFFAOYSA-N1-hydroxypropane-1,2,3-tricarboxylic acid192.1235192.02700261-0.564isocitric acid0-3FDB0032811-hydroxy-1,2,3-propanetricarboxylate;1-hydroxy-1,2,3-propanetricarboxylic acid;1-hydroxypropane-1,2,3-tricarboxylate;1-hydroxypropane-1,2,3-tricarboxylic acid;1-hydroxytricarballylate;1-hydroxytricarballylic acid;3-carboxy-2,3-dideoxy-1-hydroxypropan-1,2,3-tricarboxylate;3-carboxy-2,3-dideoxy-1-hydroxypropan-1,2,3-tricarboxylic acid;3-carboxy-2,3-dideoxy-pentarate;3-carboxy-2,3-dideoxy-pentaric acid;D-isocitrate;I-cit;Isocitrate;Threo-d(s)-iso-citrate;Threo-ds-isocitratePW_C000125I-cita224450642537410360351556091161648117874702227713413380017368117827132119971406122672124122756120125247118125389479126805299126930501128403388134Oxoglutaric 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_C000134AKG152423141414684991867331110842126351447501455261467545375103541411754381185564132600814760361556069157609216164821786530857471222751522475191518209225837422011863198126812897705425377135133774811117752311277746129779673457797034677976327779843477842533480018368806941351131629411997240612002212412008440712017412212055241412081441812098940812114642312115242412116042512275712012283111912318645012339945412355437412371845812372445912373246012535747912540029912545548112553329712580048912592948212690050112694038812699320612706620512725550612738850240034Hydrogen IonHMDB0059597Hydrogen ion is recommended by IUPAC as a general term for all ions of hydrogen and its isotopes. Depending on the charge of the ion, two different classes can be distinguished: positively charged ions and negatively charged ions. Under aqueous conditions found in biochemistry, hydrogen ions exist as the hydrated form hydronium, H3O+, but these are often still referred to as hydrogen ions or even protons by biochemists. [WikiPedia])C000801038153781010[H+]HInChI=1S/p+1GPRLSGONYQIRFK-UHFFFAOYSA-Nhydron1.00791.0078250320hydron10H+;H(+);Hydrogen cation;Hydron;ProtonPW_C040034H+215467087531578831848311162146326146454223149278017425022425442454710457618469470524110353271115353112562610856391075699100572010557421175963147603715560701576093161613015962321666483178660115266921016843188691018771001637168205719120674532197454220747222275252137532210755821275721607590170819522582181518243226841316284202249139195915524911915164120152811218128512246286122662871252122713257223133252941533030842329315423543184240132242405312424543207691229377136133772101347737233177804114779551327799032777991347783793457992913080019368803873108038830480722119938231249482338311055038811285594113280390115537398115539118115856336116205109119973406120193407120549122120593409121170424121171425122569418122615384122687125122758120123183135123218137123742459123743460125141454125188121125273136125359479125550481125730483125736297125809299126517495126717489126766480126823300126902501127213208128308506128361391128430395423MagnesiumHMDB0000547Magnesium 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+86822742681647627272681158191888322936399833992211167461483491529431764142124102411592942233126293373745403147749148695449745652531045329111535611253761035906147593415160381556094161625016664841786594164688116069791997170205719420672272137233211725021473102167313198747322211763132118432101231222512324249125132881258122612729290152752851533730877137133772363297793733678393334784173357848911578522331785363567857413080020368800451848004837280623118806541358086515809652538184151938323839490027108596223110559390115687398119974406120070122120247382120702407120981408121181124121265429121319419121924125122086405122408422122759120122921399123307119123546374123835464123889455124477136124637376124978375125447297125598484125669479125777481125921482125947299125973495126000490126243478126553491126753300127125389127164501127380502127407388127451507127804209128125508128347395808Succinyl-CoAHMDB0001022Succinyl-CoA is an important intermediate in the citric acid cycle, where it is synthesized from α-Ketoglutarate by α-ketoglutarate dehydrogenase (EC 1.2.4.2) through decarboxylation, and is converted into succinate through the hydrolytic release of coenzyme A by succinyl-CoA synthetase (EC 6.2.1.5). Succinyl-CoA may be an end product of peroxisomal beta-oxidation of dicarboxylic fatty acids; the identification of an apparently specific succinyl-CoA thioesterase (ACOT4, EC 3.1.2.3, hydrolyzes succinyl-CoA) in peroxisomes strongly suggests that succinyl-CoA is formed in peroxisomes. Acyl-CoA thioesterases (ACOTs) are a family of enzymes that catalyze the hydrolysis of the CoA esters of various lipids to the free acids and coenzyme A, thereby regulating levels of these compounds. (PMID: 16141203).604-98-8C00091439161153803-METHYLBENZYLSUCCINYL-COA388307CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)C(O)C(=O)NCCC(=O)NCCSC(=O)CCC(O)=OC25H40N7O19P3SInChI=1S/C25H40N7O19P3S/c1-25(2,20(38)23(39)28-6-5-14(33)27-7-8-55-16(36)4-3-15(34)35)10-48-54(45,46)51-53(43,44)47-9-13-19(50-52(40,41)42)18(37)24(49-13)32-12-31-17-21(26)29-11-30-22(17)32/h11-13,18-20,24,37-38H,3-10H2,1-2H3,(H,27,33)(H,28,39)(H,34,35)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t13-,18-,19-,20?,24-/m1/s1VNOYUJKHFWYWIR-FZEDXVDRSA-N4-{[2-(3-{3-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido}propanamido)ethyl]sulfanyl}-4-oxobutanoic acid867.607867.131252359-2.35104-({2-[3-(3-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-2-hydroxy-3-methylbutanamido)propanamido]ethyl}sulfanyl)-4-oxobutanoic acid0-5FDB022375Coa s-(hydrogen succinate);Coa s-succinate;Coenzyme a s-(hydrogen succinate);Coenzyme a s-succinate;S-(hydrogen butanedioate;S-(hydrogen butanedioate) coa;S-(hydrogen butanedioate) coenzyme a;S-(hydrogen butanedioic acid;S-succinoylcoenzyme a;Suc-co-a;Suc-coa;Succ-coa;Succ-coenzyme a;Succ-s-coa;Succ-s-coenzyme a;Succ-s-coenzyme-a;Succ-coenzyme-a;Succino-1-yl-coenzyme a;Succinyl coa;Succinyl coenzyme a;Succinyl-s-coa;Succinyl-s-coenzyme a;Succinyl-s-coenzyme-a;Succinylcoenzyme-a;Succinylcoenzyme aPW_C000808Suc-CoA233410553366925378103603915560971616485178701516073611637474222771401337810111278576132800213681199784061207694071220141241227631201233651191245681181253584791261642991263064811269015011278682061104PhosphateHMDB0001429Phosphate is a salt of phosphoric acid. In organic chemistry, a phosphate, or organophosphate, is an ester of phosphoric acid. Organic phosphates are important in biochemistry, biogeochemistry and ecology. Phosphate (Pi) is an essential component of life. In biological systems, phosphorus is found as a free phosphate ion in solution and is called inorganic phosphate, to distinguish it from phosphates bound in various phosphate esters. Inorganic phosphate is generally denoted Pi and at physiological (neutral) pH primarily consists of a mixture of HPO<sup>2-</sup><sub>4</sub> and H<sub>2</sub>PO<sup>-</sup><sub>4</sub> ions. phosphates are most commonly found in the form of adenosine phosphates, (AMP, ADP and ATP) and in DNA and RNA and can be released by the hydrolysis of ATP or ADP. Similar reactions exist for the other nucleoside diphosphates and triphosphates. Phosphoanhydride bonds in ADP and ATP, or other nucleoside diphosphates and triphosphates, contain high amounts of energy which give them their vital role in all living organisms. Phosphate must be actively transported into cells against its electrochemical gradient. In vertebrates, two unrelated families of Na+-dependent Pi transporters carry out this task. Remarkably, the two families transport different Pi species: whereas type II Na+/Pi cotransporters (SCL34) prefer divalent HPO4(2), type III Na+/Pi cotransporters (SLC20) transport monovalent H2PO4. The SCL34 family comprises both electrogenic and electroneutral members that are expressed in various epithelia and other polarized cells. Through regulated activity in apical membranes of the gut and kidney, they maintain body Pi homeostasis, and in salivary and mammary glands, liver, and testes they play a role in modulating the Pi content of luminal fluids. Phosphate levels in the blood play an important role in hormone signaling and in bone homeostasis. In classical endocrine regulation, low serum phosphate induces the renal production of the seco-steroid hormone 1,25-dihydroxyvitamin D3 (1,25(OH)2D3).This active metabolite of vitamin D acts to restore circulating mineral (i.e. phosphate and calcium) levels by increasing absorption in the intestine, reabsorption in the kidney, and mobilization of calcium and phosphate from bone. Thus, chronic renal failure is associated with hyperparathyroidism, which in turn contributes to osteomalacia (softening of the bones). Another complication of chronic renal failure is hyperphosphatemia (low levels of phosphate in the blood). Hyperphosphatemia (excess levels of phosphate in the blood) is a prevalent condition in kidney dialysis patients and is associated with increased risk of mortality. Hypophosphatemia (hungry bone syndrome) has been associated to postoperative electrolyte aberrations and after parathyroidectomy. (PMID: 17581921, 11169009, 11039261, 9159312, 17625581)Fibroblast growth factor 23 (FGF-23) has recently been recognized as a key mediator of phosphate homeostasis, its most notable effect being promotion of phosphate excretion. FGF-23 was discovered to be involved in diseases such as autosomal dominant hypophosphatemic rickets, X-linked hypophosphatemia, and tumor-induced osteomalacia in which phosphate wasting was coupled to inappropriately low levels of 1,25(OH)2D3. FGF-23 is regulated by dietary phosphate in humans. In particular it was found that phosphate restriction decreased FGF-23, and phosphate loading increased FGF-23.14265-44-2C00009106118367CPD-85871032[O-]P([O-])([O-])=OO4PInChI=1S/H3O4P/c1-5(2,3)4/h(H3,1,2,3,4)/p-3NBIIXXVUZAFLBC-UHFFFAOYSA-Kphosphoric acid94.971494.953423phosphoric acid0-2DBMET00532FDB022617Nfb orthophosphate;O-phosphoric acid;Ortho-phosphate;Orthophosphate (po43-);Orthophosphate(3-);Phosphate;Phosphate (po43-);Phosphate anion(3-);Phosphate ion (po43-);Phosphate ion(3-);Phosphate trianion;Phosphate(3-);Phosphoric acid ion(3-);Pi;[po4](3-);Orthophosphate;Phosphate ion;Po4(3-);Phosphoric acid;Orthophosphoric acid;Phosphoric acid ionPW_C001104Pi2448488145818188312980317631417674925001027294727374631292931667236366138512342492244753150312751587520797521610053171115351112538110354471205543129557313356051355625108569365848143585514659111475941151604015561001616294107648717866911016714117684218868891607161205718920672122117306198738921074022127436163747522281962258258227101182411013425711748132117611151177321311904170119271641201428112728290132632233481917422553044235031542435318436923227701825377194293772171347794033677966130780483327805732978245353786693318002236889279308938313839479638411055839011064039111323594115845398116206109119982406120069122120699407121057124121216125121268429121352121121409123121423382121852405123304119123621118123786136123838464123968447123981399124405376124948472125362479125446297125774481125954299126221478126594300126604298126723484126904501127413388127783209128166395128177513128315389936Guanosine diphosphateHMDB0001201Guanosine 5'-(trihydrogen diphosphate). A guanine nucleotide containing two phosphate groups esterified to the sugar moiety. It is an ester of pyrophosphoric acid with the nucleoside guanosine. GDP consists of the pyrophosphate group, the pentose sugar ribose, and the nucleobase guanine. GDP is the product of GTP dephosphorylation by GTPases, e.g. the G-proteins that are involved in signal transduction.146-91-8C00035897717552GDP-4-DEHYDRO-6-DEOXY-D-MANNOSE8630NC1=NC2=C(N=CN2[C@@H]2O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]2O)C(=O)N1C10H15N5O11P2InChI=1S/C10H15N5O11P2/c11-10-13-7-4(8(18)14-10)12-2-15(7)9-6(17)5(16)3(25-9)1-24-28(22,23)26-27(19,20)21/h2-3,5-6,9,16-17H,1H2,(H,22,23)(H2,19,20,21)(H3,11,13,14,18)/t3-,5-,6-,9-/m1/s1QGWNDRXFNXRZMB-UUOKFMHZSA-N[({[(2R,3S,4R,5R)-5-(2-amino-6-oxo-6,9-dihydro-3H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]phosphonic acid443.2005443.024329371-2.007{[(2R,3S,4R,5R)-5-(2-amino-6-oxo-3H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy(hydroxy)phosphoryl}oxyphosphonic acid0-3FDB0224875'-gdp;Gdp;Guanosine 5'-(trihydrogen pyrophosphate);Guanosine 5'-diphosphate;Guanosine 5'-pyrophosphate;Guanosine mono(trihydrogen diphosphate);Guanosine pyrophosphate;Guanosine-5'-diphosphate;Guanosine-diphosphate;Ppg;Guanosine diphosphate;Guanosine 5'-diphosphoric acidPW_C000936GDP83823841762142391241547350078553821036041155610116164881787476222117541151177121111823198127272901339515169322177142133775461117795213280023368800803088012216489115253119983406120068122121205124121847405122766120122820135123775118124400376125363479125445297126905501126984205174Succinic 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_C000174Succini15232394502185078676311265542551753831036042155610216164541076455108648917867641176836166736216374552197456220747722211866198121421511325922342368318423693154240232277143133772131347748311177738112777491297842633480024368807211191128463081134281119984406120192407120385122120555414120990408122565384122767120123029135123189450123555374125138121125364479125549481125930482126713480126906501127082206127389502128304391986Guanosine triphosphateHMDB0001273Guanosine triphosphate (GTP) is a guanine nucleotide containing three phosphate groups esterified to the sugar moiety. GTP functions as a carrier of phosphates and pyrophosphates involved in channeling chemical energy into specific biosynthetic pathways. GTP activates the signal transducing G proteins which are involved in various cellular processes including proliferation, differentiation, and activation of several intracellular kinase cascades. Proliferation and apoptosis are regulated in part by the hydrolysis of GTP by small GTPases Ras and Rho. Another type of small GTPase, Rab, plays a role in the docking and fusion of vesicles and may also be involved in vesicle formation. In addition to its role in signal transduction, GTP also serves as an energy-rich precursor of mononucleotide units in the enzymatic biosynthesis of DNA and RNA.86-01-1C00044683015996GTP6569NC1=NC2=C(N=CN2[C@@H]2O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]2O)C(=O)N1C10H16N5O14P3InChI=1S/C10H16N5O14P3/c11-10-13-7-4(8(18)14-10)12-2-15(7)9-6(17)5(16)3(27-9)1-26-31(22,23)29-32(24,25)28-30(19,20)21/h2-3,5-6,9,16-17H,1H2,(H,22,23)(H,24,25)(H2,19,20,21)(H3,11,13,14,18)/t3-,5-,6-,9-/m1/s1XKMLYUALXHKNFT-UUOKFMHZSA-N({[({[(2R,3S,4R,5R)-5-(2-amino-6-oxo-6,9-dihydro-1H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)phosphonic acid523.1804522.990659781-1.708triphosphate, guanosine0-3FDB0225275'-gtp;Gtg;Gtp;Guanosine 5'-(tetrahydrogen triphosphate);Guanosine 5'-triphosphate;Guanosine 5'-triphosphorate;Guanosine 5'-triphosphoric acid;Guanosine triphosphate;Guanosine mono(tetrahydrogen triphosphate) (ester);H4gtp;Guanosine-5'-triphosphatePW_C000986GTP8182404193924091144153735006855384103604315561031616490178747822211753115117691981198115112725290693271769622257714413377544111779511328002536880088308801211648911325311998540612006612212120412412276812012281813512377411812536547912544329712648029912690750112698220512805138888Fumaric acidHMDB0000134Fumaric acid is a precursor to L-malate in the Krebs tricarboxylic acid cycle. It is formed by the oxidation of succinate by succinate dehydrogenase. Fumarate is converted by fumarase to malate. A fumarate is a salt or ester of the organic compound fumaric acid, a dicarboxylic acid. (wikipedia).110-17-8C001222188378818012FUM10197150DB04299OC(=O)\C=C\C(O)=OC4H4O4InChI=1S/C4H4O4/c5-3(6)1-2-4(7)8/h1-2H,(H,5,6)(H,7,8)/b2-1+VZCYOOQTPOCHFL-OWOJBTEDSA-N(2E)-but-2-enedioic acid116.0722116.010958616-0.682fumaric acid0-2FDB003291(2e)-but-2-enedioate;(2e)-but-2-enedioic acid;(e)-2-butenedioate;(e)-2-butenedioic acid;2-(e)-butenedioate;2-(e)-butenedioic acid;Allomaleate;Allomaleic acid;Boletate;Boletic acid;Fc 33;Fumarate;Fumaric acid;Lichenate;Lichenic acid;Sodium fumarate;Trans-1,2-ethylenedicarboxylate;Trans-1,2-ethylenedicarboxylic acid;Trans-2-butenedioate;Trans-2-butenedioic acid;Trans-butenedioate;Trans-butenedioic acid;(2e)-2-butenedioic acid;E297;Fumarsaeure;Trans-but-2-enedioic acid;(2e)-2-butenedioate;Trans-but-2-enedioatePW_C000088Fumarat10282541720042505345388102604715661071626458107645910864921796763117683716674802239065151118041981271329042400322424963184249731577148134774661117910713280027369117808133119989384120043122121599124122661406122772121122794135124157118125236120125369480125421297126793479126911391126960205127565388128391501101L-Malic acidHMDB0000156Malic acid is a tart-tasting organic dicarboxylic acid that plays a role in many sour or tart foods. Apples contain malic acid, which contributes to the sourness of a green apple. Malic acid can make a wine taste tart, although the amount decreases with increasing fruit ripeness. (wikipedia). In its ionized form malic acid is called malate. Malate is an intermediate of the TCA cycle along with fumarate. It can also be formed from pyruvate as one of the anaplerotic reactions. In humans, malic acid is both derived from food sources and synthesized in the body through the citric acid cycle or Krebs cycle which takes place in the mitochondria. Malate's importance to the production of energy in the body during both aerobic and anaerobic conditions is well established. Under aerobic conditions, the oxidation of malate to oxaloacetate provides reducing equivalents to the mitochondria through the malate-aspartate redox shuttle. During anaerobic conditions, where a buildup of excess of reducing equivalents inhibits glycolysis, malic acid's simultaneous reduction to succinate and oxidation to oxaloacetate is capable of removing the accumulating reducing equivalents. This allows malic acid to reverse hypoxia's inhibition of glycolysis and energy production. In studies on rats it has been found that only tissue malate is depleted following exhaustive physical activity. Other key metabolites from the citric acid cycle needed for energy production were found to be unchanged. Because of this, a deficiency of malic acid has been hypothesized to be a major cause of physical exhaustion. Notably, the administration of malic acid to rats has been shown to elevate mitochondrial malate and increase mitochondrial respiration and energy production.97-67-6C0014922265630797193317O[C@@H](CC(O)=O)C(O)=OC4H6O5InChI=1S/C4H6O5/c5-2(4(8)9)1-3(6)7/h2,5H,1H2,(H,6,7)(H,8,9)/t2-/m0/s1BJEPYKJPYRNKOW-REOHCLBHSA-N(2S)-2-hydroxybutanedioic acid134.0874134.0215233020.213(-)-malic acid0-2FDB001044(-)-(s)-malate;(-)-(s)-malic acid;(-)-hydroxysuccinate;(-)-hydroxysuccinic acid;(-)-l-malic acid;(-)-malic acid;(2s)-2-hydroxybutanedioate;(2s)-2-hydroxybutanedioic acid;(s)-(-)-hydroxysuccinate;(s)-(-)-hydroxysuccinic acid;(s)-hydroxybutanedioate;(s)-hydroxybutanedioic acid;(s)-malic acid;(s)-hydroxy-butanedioate;(s)-hydroxy-butanedioic acid;Apple acid;L-(-)-malic acid;L-apple acid;L-hydroxybutanedioate;L-hydroxybutanedioic acid;L-hydroxysuccinate;L-hydroxysuccinic acid;Malic acid;S-(-)-malate;S-(-)-malic acid;S-2-hydroxybutanedioate;S-2-hydroxybutanedioic acid;L-2-hydroxybutanedioic acid;L-malic acid;Malate;(-)-l-malate;(s)-malate;L-2-hydroxybutanedioate;L-malatePW_C000101Malate262410983172182387253871035735108604615561061616453107649117874512197452220747922242455318424563157714713378766111790511327905711280026368119988406121441122122394124122401407122771120123999135124964118124971119125368479126066297126535299126546481126910501127519205128108388128118206414Adenosine 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_C000414ATP92214608266164142247813733327995934399763210518211210214649215614216058240559243427272646281229302966316372361661361751439923447431476891486454503289503526515575205975215100525010452911015313111534611253901035406117543011854431205542129555613255691335603135562110858461435854146587610758971475924151604815561091616230166649317868391886870160697619971572057184206720921072252137229211729819873022167390217740821874321637481222749919081862251184727711903170120102811203916412178285125782261269129013264223153273084232631542621322426943187702825377218134772333297746833377632336780373327804135078168128782143517824035378411335784941157885013078865331789193348002836880046184806741198562919482612411323494113282388116280109119914122119992406120154407120245382120362412121246429121392123121397433121471408121974410122065125122079383122083405122402422122444435122919399123009446123816464123951447123956468124029374124527444124616136124630398124634376124943472124972375125011470125304297125371479125392299125515481125595484126123485126220300126234495126240478126547491126596499126913501127123389127731516127781395127796390127801209128119508128167517463Hydrogen carbonateHMDB0000595Bicarbonate, or hydrogen carbonate, is a simple single carbon molecule that plays surprisingly important roles in diverse biological processes. Among these are photosynthesis, the Krebs cycle, whole-body and cellular pH regulation, and volume regulation. Since bicarbonate is charged it is not permeable to lipid bilayers. Mammalian membranes thus contain bicarbonate transport proteins to facilitate the specific transmembrane movement of HCO3(-). Bicarbonate ion is an anion that consists of one central carbon atom surrounded by three oxygen atoms in a trigonal planar arrangement, with a hydrogen atom attached to one of the oxygens. The bicarbonate ion carries a negative one formal charge and is the conjugate base of carbonic acid, H2CO3. The carbonate radical is an elusive and strong one-electron oxidant. Bicarbonate in equilibrium with carbon dioxide constitutes the main physiological buffer. The bicarbonate-carbon dioxide pair stimulates the oxidation, peroxidation and nitration of several biological targets. The demonstration that the carbonate radical existed as an independent species in aqueous solutions at physiological pH and temperature renewed the interest in the pathophysiological roles of this radical and related species. The carbonate radical has been proposed to be a key mediator of the oxidative damage resulting from peroxynitrite production, xanthine oxidase turnover and superoxide dismutase1 peroxidase activity. The carbonate radical has also been proposed to be responsible for the stimulatory effects of the bicarbonate-carbon dioxide pair on oxidations mediated by hydrogen peroxide/transition metal ions. The ultimate precursor of the carbonate radical anion being bicarbonate, carbon dioxide, peroxymonocarbonate or complexes of transition metal ions with bicarbonate-derived species remains a matter of debate. The carbonate radical mediates some of the pathogenic effects of peroxynitrite. The carbonate radical as the oxidant produced from superoxide dismutase (EC 1.15.1.1, SOD1) peroxidase activity. Peroxymonocarbonate is a biological oxidant, whose existence is in equilibrium with hydrogen peroxide and bicarbonate. (PMID: 17505962, 17215880).71-52-3C0028876917544HCO3749OC([O-])=OCHO3InChI=1S/CH2O3/c2-1(3)4/h(H2,2,3,4)/p-1BVKZGUZCCUSVTD-UHFFFAOYSA-Mcarbonic acid61.016860.9925688980.572carbonic acid0-1FDB022134Bicarbonate;Bicarbonate (hco3-);Bicarbonate anion;Bicarbonate ion;Bicarbonate ion (hco31-);Bicarbonate ions;Carbonate;Carbonate (hco31-);Carbonate ion (hco31-);Carbonic acid;Hydrocarbonate(1-);Hydrogen carbonate;Hydrogen carbonate (hco3-);Hydrogen carbonate anion;Hydrogen carbonate ion;Hydrogen carbonate ion (hco3-);Hydrogencarbonate;Hydrogentrioxocarbonate;Monohydrogen carbonate;[co2(oh)](-);Acid carbonate;Hco3(-);Hydrogen carbonic acid;Acid carbonic acid;Bicarbonic acid;Bicarbonic acid ionPW_C000463HCO322416878239332397226131531457053911035445120557113360491556110161649417874822229092224779591127863013278762111800293681199934061212094071214361221215571241237791191239941351241151181253724791260592971263602991265414811269145011275112051279223881281142061034Adenosine diphosphateHMDB0001341Adenosine diphosphate, abbreviated ADP, is a nucleotide. It is an ester of pyrophosphoric acid with the nucleotide adenine. ADP consists of the pyrophosphate group, the pentose sugar ribose, and the nucleobase adenine. ADP is the product of ATP dephosphorylation by ATPases. ADP is converted back to ATP by ATP synthases.58-64-0C00008602216761ADP5800NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1OC10H15N5O10P2InChI=1S/C10H15N5O10P2/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(24-10)1-23-27(21,22)25-26(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1XTWYTFMLZFPYCI-KQYNXXCUSA-N[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]phosphonic acid427.2011427.029414749-2.126adenosine-diphosphate0-2FDB021817Adp;Adenosindiphosphorsaeure;Adenosine 5'-pyrophosphate;Adenosine diphosphate;Adenosine pyrophosphate;Adenosine-5'-diphosphate;Adenosine-5-diphosphate;Adenosine-diphosphate;5'-adenylphosphoric acid;Adenosine 5'-diphosphate;H3adp;5'-adenylphosphate;Adenosine 5'-diphosphoric acid;Adenosine-5'-diphosphoric acidPW_C001034ADP23413484152248213801596315978310611415182190149210418211310216158240859243527272847273646285529316572363561440023447631477091503626515775208975217100531511153491125392103544612055441295572133562410857411175764101584914358561465878107589914759261516050155611116162311666495178670094684118868721607159205718720672082107226213723121173001987303216739121774102187433163748322281872251185127711905170120132811218028513262223153293084232831542398313426223224269631877029253770871327721613477306329774723337766333678039332780433507817012878215351782443537841433578495115787053317884913078920334800303688062211880651135806761199482712411328338811620410911994412211999440612015640712031838212036641212124842912139412312139943312147240812189938312197641012206412512208540512240542212244543512297339912301344612381846412395344712395846812403037412445239812452944412461513612463637612494747212497537512501247012533429712537347912549229912551748112564548412612548512621930012623549512624247812655049112659749912691550112773351612778039512779739012780320912812250812816851712831338920BiotinHMDB0000030Biotin is an enzyme co-factor present in minute amounts in every living cell. Biotin is also known as vitamin H or B7 or coenzyme R. It occurs mainly bound to proteins or polypeptides and is abundant in liver, kidney, pancreas, yeast, and milk. Biotin has been recognized as an essential nutrient. Our biotin requirement is fulfilled in part through diet, through endogenous reutilization of biotin and perhaps through capture of biotin generated in the intestinal flora. The utilization of biotin for covalent attachment to carboxylases and its reutilization through the release of carboxylase biotin after proteolytic degradation constitutes the 'biotin cycle'. Biotin deficiency is associated with neurological manifestations, skin rash, hair loss and metabolic disturbances that are thought to relate to the various carboxylase deficiencies (metabolic ketoacidosis with lactic acidosis). It has also been suggested that biotin deficiency is associated with protein malnutrition, and that marginal biotin deficiency in pregnant women may be teratogenic. Biotin acts as a carboxyl carrier in carboxylation reactions. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lysine residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lys residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. Evidence is emerging that biotin participates in processes other than classical carboxylation reactions. Specifically, novel roles for biotin in cell signaling, gene expression, and chromatin structure have been identified in recent years. Human cells accumulate biotin by using both the sodium-dependent multivitamin transporter and monocarboxylate transporter 1. These transporters and other biotin-binding proteins partition biotin to compartments involved in biotin signaling: cytoplasm, mitochondria, and nuclei. The activity of cell signals such as biotinyl-AMP, Sp1 and Sp3, nuclear factor (NF)-kappaB, and receptor tyrosine kinases depends on biotin supply. Consistent with a role for biotin and its catabolites in modulating these cell signals, greater than 2000 biotin-dependent genes have been identified in various human tissues. Many biotin-dependent gene products play roles in signal transduction and localize to the cell nucleus, consistent with a role for biotin in cell signaling. Posttranscriptional events related to ribosomal activity and protein folding may further contribute to effects of biotin on gene expression. Finally, research has shown that biotinidase and holocarboxylase synthetase mediate covalent binding of biotin to histones (DNA-binding proteins), affecting chromatin structure; at least seven biotinylation sites have been identified in human histones. Biotinylation of histones appears to play a role in cell proliferation, gene silencing, and the cellular response to DNA repair. Roles for biotin in cell signaling and chromatin structure are consistent with the notion that biotin has a unique significance in cell biology. (PMID: 15992684, 16011464).58-85-5C0012017154815956BIOTIN149962DB00121[H][C@]12CS[C@@H](CCCCC(O)=O)[C@@]1([H])NC(=O)N2C10H16N2O3SInChI=1S/C10H16N2O3S/c13-8(14)4-2-1-3-7-9-6(5-16-7)11-10(15)12-9/h6-7,9H,1-5H2,(H,13,14)(H2,11,12,15)/t6-,7-,9-/m0/s1YBJHBAHKTGYVGT-ZKWXMUAHSA-N5-[(3aS,4S,6aR)-2-oxo-hexahydro-1H-thieno[3,4-d]imidazol-4-yl]pentanoic acid244.311244.088163078-2.3035-[(3aS,4S,6aR)-2-oxo-hexahydrothieno[3,4-d]imidazol-4-yl]pentanoic acid0-1FDB014510(+)-biotin;(+)-cis-hexahydro-2-oxo-1h-thieno[3,4]imidazole-4-valerate;(+)-cis-hexahydro-2-oxo-1h-thieno[3,4]imidazole-4-valeric acid;(3as,4s,6ar)-hexahydro-2-oxo-1h-thieno[3,4-d]imidazole-4-valerate;(3as,4s,6ar)-hexahydro-2-oxo-1h-thieno[3,4-d]imidazole-4-valeric acid;-(+)-biotin;1swk;1swn;1swr;5-(2-oxohexahydro-1h-thieno[3,4-d]imidazol-4-yl)pentanoate;5-(2-oxohexahydro-1h-thieno[3,4-d]imidazol-4-yl)pentanoic acid;Biodermatin;Bioepiderm;Bios ii;Bios h;Biotin;Coenzyme r;D(+)-biotin;D-(+)-biotin;D-biotin;D-biotin factor s;Factor s;Factor s (vitamin);Hexahydro-2-oxo-1h-thieno(3,4-d)imidazole-4-pentanoate;Hexahydro-2-oxo-1h-thieno(3,4-d)imidazole-4-pentanoic acid;Hexahydro-2-oxo-[3as-(3aa,4b,6aa)]-1h-thieno[3,4-d]imidazole-4-pentanoate;Hexahydro-2-oxo-[3as-(3aa,4b,6aa)]-1h-thieno[3,4-d]imidazole-4-pentanoic acid;Hexahydro-2-oxo-[3as-(3alpha,4beta,6alpha)]-1h-thieno[3,4-d]imidazole-4-pentanoate;Hexahydro-2-oxo-[3as-(3alpha,4beta,6alpha)]-1h-thieno[3,4-d]imidazole-4-pentanoic acid;Lutavit h2;Meribin;Rovimix h 2;Vitamin b7;Vitamin h;Vitamin-h;Cis-(+)-tetrahydro-2-oxothieno[3,4]imidazoline-4-valerate;Cis-(+)-tetrahydro-2-oxothieno[3,4]imidazoline-4-valeric acid;Cis-hexahydro-2-oxo-1h-thieno(3,4)imidazole-4-valeric acid;Cis-tetrahydro-2-oxothieno(3,4-d)imidazoline-4-valeric acid;Delta-(+)-biotin;Delta-biotin;Delta-biotin factor s;Biotina;Biotine;BiotinumPW_C000020Biotin2641358579151699322702529210152981055393103544912055461115551114557513360511556112161649617869251607484222778311327796011280031368806531351199954061201341221205034091212104071215591241231091371237801191241171181253744791255012971257184831264212991265424811269165011270382051279893881281152061027ManganeseHMDB0001333Manganese is an essential trace nutrient in all forms of life. Physiologically, it. exists as an ion in the body. It is concentrated in cell mitochondria, mostly in the pituitary gland, liver, pancreas, kidney, and bone, influences the synthesis of mucopolysaccharides, stimulates hepatic synthesis of cholesterol and fatty acids, and is a cofactor in many enzymes, including arginase and alkaline phosphatase in the liver.16397-91-4C196102785429035MN%2b325916[Mn++]MnInChI=1S/Mn/q+2WAEMQWOKJMHJLA-UHFFFAOYSA-Nmanganese(2+) ion54.93854.9380496360manganese(2+) ion22FDB003636Manganese;Manganese (ii) ion;Manganese(ii);Manganese, ion (mn2+);Manganous ion;Mn(2+);Mn2+PW_C001027Mn2+2744738148649155343227122394325131453941035450120557613360521556113161649717869261607485222118801981193922511958164124712491336015115221306770502947749411177832132779611127826735678490115785243317924729380032368119996406120401122121058124121211407121295383121378419122488405123044135123622118123781119123865398123937455125054376125375479125976495126051490126060297126158299126543481126642478126917501127429390127503507127512205127765388128116206128218209846Coenzyme Q10HMDB0001072Coenzyme Q10 (ubiquinone) is a naturally occurring compound widely distributed in animal organisms and in humans. The primary compounds involved in the biosynthesis of ubiquinone are 4-hydroxybenzoate and the polyprenyl chain. An essential role of coenzyme Q10 is as an electron carrier in the mitochondrial respiratory chain. Moreover, coenzyme Q10 is one of the most important lipophilic antioxidants, preventing the generation of free radicals as well as oxidative modifications of proteins, lipids, and DNA, it and can also regenerate the other powerful lipophilic antioxidant, alpha-tocopherol. Antioxidant action is a property of the reduced form of coenzyme Q10, ubiquinol (CoQ10H2), and the ubisemiquinone radical (CoQ10H*). Paradoxically, independently of the known antioxidant properties of coenzyme Q10, the ubisemiquinone radical anion (CoQ10-) possesses prooxidative properties. Decreased levels of coenzyme Q10 in humans are observed in many pathologies (e.g. cardiac disorders, neurodegenerative diseases, AIDS, cancer) associated with intensive generation of free radicals and their action on cells and tissues. In these cases, treatment involves pharmaceutical supplementation or increased consumption of coenzyme Q10 with meals as well as treatment with suitable chemical compounds (i.e. folic acid or B-group vitamins) which significantly increase ubiquinone biosynthesis in the organism. Estimation of coenzyme Q10 deficiency and efficiency of its supplementation requires a determination of ubiquinone levels in the organism. Therefore, highly selective and sensitive methods must be applied, such as HPLC with UV or coulometric detection. For a number of years, coenzyme Q (CoQ10 in humans) was known for its key role in mitochondrial bioenergetics; later studies demonstrated its presence in other subcellular fractions and in plasma, and extensively investigated its antioxidant role. These two functions constitute the basis on which research supporting the clinical use of CoQ10 is founded. Also at the inner mitochondrial membrane level, coenzyme Q is recognized as an obligatory co-factor for the function of uncoupling proteins and a modulator of the transition pore. Furthermore, recent data reveal that CoQ10 affects expression of genes involved in human cell signalling, metabolism, and transport and some of the effects of exogenously administered CoQ10 may be due to this property. Coenzyme Q is the only lipid soluble antioxidant synthesized endogenously. In its reduced form, CoQH2, ubiquinol, inhibits protein and DNA oxidation but it is the effect on lipid peroxidation that has been most deeply studied. Ubiquinol inhibits the peroxidation of cell membrane lipids and also that of lipoprotein lipids present in the circulation. Dietary supplementation with CoQ10 results in increased levels of ubiquinol-10 within circulating lipoproteins and increased resistance of human low-density lipoproteins to the initiation of lipid peroxidation. Moreover, CoQ10 has a direct anti-atherogenic effect, which has been demonstrated in apolipoprotein E-deficient mice fed with a high-fat diet. (PMID: 15928598, 17914161).303-98-0C11378528191546245UBIQUINONE-104445197COC1=C(OC)C(=O)C(C\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(\C)CC\C=C(/C)CC\C=C(/C)CCC=C(C)C)=C(C)C1=OC59H90O4InChI=1S/C59H90O4/c1-44(2)24-15-25-45(3)26-16-27-46(4)28-17-29-47(5)30-18-31-48(6)32-19-33-49(7)34-20-35-50(8)36-21-37-51(9)38-22-39-52(10)40-23-41-53(11)42-43-55-54(12)56(60)58(62-13)59(63-14)57(55)61/h24,26,28,30,32,34,36,38,40,42H,15-23,25,27,29,31,33,35,37,39,41,43H2,1-14H3/b45-26+,46-28+,47-30-,48-32+,49-34+,50-36+,51-38+,52-40+,53-42+ACTIUHUUMQJHFO-NBZSDRGLSA-N2-[(2E,6E,10E,14E,18E,22E,26E,30E,34E)-3,7,11,15,19,23,27,31,35,39-decamethyltetraconta-2,6,10,14,18,22,26,30,34,38-decaen-1-yl]-5,6-dimethoxy-3-methylcyclohexa-2,5-diene-1,4-dione863.3435862.683911368-6.650coenzyme-Q1000FDB014621(all-e)-2,3-dimethoxy-5-methyl-6-(3,7,11,15,19,23,27,31-octamethyl-2,6,10,14,18,22,26,30-dotriacontaoctaenyl)-2,5-cyclohexadiene-1,4-dione;(all-e)-2-(3,7,11,15,19,23,27,31,35,39-decamethyl-2,6,10,14,18,22,26,30,34,38-tetracontadecaenyl)-5,6-dimethoxy-3-methyl-2,5-cyclohexadiene-1,4-dione;2-(3,7,11,15,19,23,27,31,35,39-decamethyl-2,6,10,14,18,22,26,30,34,38-tetracontadecaenyl)-5,6-dimethoxy-3-methyl-p-benzoquinone;2-[(2e,6e,10e,14e,18e,22e,26e,30e,34e)-3,7,11,15,19,23,27,31,35,39-decamethyl-2,6,10,14,18,22,26,30,34,38-tetracontadecaenyl]-5,6-dimethoxy-3-methyl- 2,5-cyclohexadiene-1,4-dione;4-ethyl-5-fluoropyrimidine;Aqua q 10l10;Aqua q10;Bio-quinon;Bio-quinone q10;Coq10;Coenzyme q10;Ensorb;Kaneka q10;Kudesan;Li-q-sorb;Liquid-q;Neuquinon;Neuquinone;Puresorb q 40;Q 10aa;Q-gel;Q-gel 100;Ubidecarenone;Ubiquinone 10;Ubiquinone 50;Ubiquinone q10;Ubiquinone-10;Unbiquinone;Unispheres q 10;2-((all-e)-3,7,11,15,19,23,27,31,35,39-decamethyl-2,6,10,14,18,22,26,30,34,38-tetracontadecaenyl)-5,6-dimethoxy-3-methyl-p-benzoquinone;2-[(2e,6e,10e,14e,18e,22e,26e,30e,34e)-3,7,11,15,19,23,27,31,35,39-decamethyltetraconta-2,6,10,14,18,22,26,30,34,38-decaen-1-yl]-5,6-dimethoxy-3-methyl-1,4-benzoquinone;Adelir;All-trans-ubiquinone;Coq;Q;Q 199;Q10;UbiquinonePW_C000846Coq25217425142505245396102605315661151626498179748622377151134783713458003336911780713311999838412256241812266040612277512112513545412523512012537748012671048912679247912691939112830150612839050140098QH(2)HMDB0059661QH(2), also known as ubiquinol, belongs to the class of organic compounds known as ubiquinols. These are coenzyme Q derivatives containing a 5, 6-dimethoxy-3-methylbenzene-1,4-diol moiety to which an isoprenyl group is attached at ring position 2(or 6). QH(2) is considered to be a practically insoluble (in water) and relatively neutral molecule. Within the cell, QH(2) is primarily located in the membrane (predicted from logP) and cytoplasm. QH(2) exists in all living organisms, ranging from bacteria to humans. In humans, QH(2) is involved in the congenital lactic acidosis pathway, the citric Acid cycle pathway, and the oncogenic action OF 2-hydroxyglutarate pathway. QH(2) is also involved in several metabolic disorders, some of which include fumarase deficiency, cancer (via the Warburg effect), pyruvate dehydrogenase deficiency (e3), and mitochondrial complex II deficiency. Qh(2) is part of the Oxidative phosphorylation, Cardiac muscle contraction, Alzheimer\'s disease, Parkinson\'s disease, and Huntington\'s disease pathways. It is a substrate for: Cytochrome b-c1 complex subunit Rieske, mitochondrial.44792017976394877COC1=C(O)C(C)=C(CC=C(C)C)C(O)=C1OCC14H20O4InChI=1S/C14H20O4/c1-8(2)6-7-10-9(3)11(15)13(17-4)14(18-5)12(10)16/h6,15-16H,7H2,1-5H3TVLSKGDBUQMDPR-UHFFFAOYSA-N2,3-dimethoxy-5-methyl-6-(3-methylbut-2-en-1-yl)benzene-1,4-diol252.3062252.136159128-2.9222,3-dimethoxy-5-methyl-6-(3-methylbut-2-en-1-yl)benzene-1,4-diol00Ubiquinol(1)PW_C040098QH(2)2551750544539710260551566117162650017974882237715313480035369117809133120000384122662406122777121125237120125379480126794479126921391128392501932FADHHMDB0001197FADH is the reduced form of flavin adenine dinucleotide (FAD). FAD is synthesized from riboflavin and two molecules of ATP. Riboflavin is phosphorylated by ATP to give riboflavin 5-phosphate (FMN). FAD is then formed from FMN by the transfer of an AMP moiety from a second molecule of ATP. FADH is generated in each round of fatty acid oxidation, and the fatty acyl chain is shortened by two carbon atoms as a result of these reactions; because oxidation is on the beta carbon, this series of reactions is called the beta-oxidation pathway. In the citric acid cycle FADH is involved in harvesting of high-energy electrons from carbon fuels; citric acid cycle itself neither generates a large amount of ATP nor includes oxygen as a reactant. Instead, the citric acid cycle removes electrons from acetyl CoA and uses these electrons to form FADH. (Biochemistry. Berg, Jeremy M. Tymoczko, John L. and Stryer, Lubert. New York: W. H. Freeman and Co. 2002.).1910-41-4C0135244601317877FADH2393487CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=C(N2)C(=O)NC(=O)N1C27H35N9O15P2InChI=1S/C27H35N9O15P2/c1-10-3-12-13(4-11(10)2)35(24-18(32-12)25(42)34-27(43)33-24)5-14(37)19(39)15(38)6-48-52(44,45)51-53(46,47)49-7-16-20(40)21(41)26(50-16)36-9-31-17-22(28)29-8-30-23(17)36/h3-4,8-9,14-16,19-21,26,32,37-41H,5-7H2,1-2H3,(H,44,45)(H,46,47)(H2,28,29,30)(H2,33,34,42,43)/t14-,15+,16+,19-,20+,21+,26+/m0/s1YPZRHBJKEMOYQH-UYBVJOGSSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}[({[(2R,3S,4S)-5-{7,8-dimethyl-2,4-dioxo-1H,2H,3H,4H,5H,10H-benzo[g]pteridin-10-yl}-2,3,4-trihydroxypentyl]oxy}(hydroxy)phosphoryl)oxy]phosphinic acid787.5656787.172784519-2.4711fadh(.)0-2FDB0224831,5-dihydro-fad;1,5-dihydro-p-5-ester with adenosine;1,5-dihydro-riboflavin 5'-(trihydrogen diphosphate) p'->5'-ester with adenosine;Adenosine 5'-(trihydrogen pyrophosphate), 5'-5'-ester with 5,10-dihydro-7,8-dimethyl-10-(d-ribo-2,3,4,5-tetrahydroxypentyl)alloxazine;Adenosine 5'-(trihydrogen pyrophosphate), 5'->5'-ester with 5,10-dihydro-7,8-dimethyl-10-(d-ribo-2,3,4,5-tetrahydroxypentyl)alloxazine;Adenosine 5'-{3-[d-ribo-5-(7,8-dimethyl-2,4-dioxo-1,2,3,4,5,10-tetrahydrobenzo[g]pteridin-10-yl)-2,3,4-trihydroxypentyl] dihydrogen diphosphate};Adenosine 5-(trihydrogen pyrophosphate);Adenosine pyrophosphate 5'-5'-ester with 5,10-dihydro-7,8-dimethyl-10-(d-ribo-2,3,4,5-tetrahydroxypentyl)alloxazine;Adenosine pyrophosphate, 5'-5'-ester with 5,10-dihydro-7,8-dimethyl-10-(d-ribo-2,3,4,5-tetrahydroxypentyl)alloxazine;Adenosine pyrophosphate, 5'->5'-ester with 5,10-dihydro-7,8-dimethyl-10-(d-ribo-2,3,4,5-tetrahydroxypentyl)alloxazine;Benzo[g]pteridine riboflavin 5'-(trihydrogen diphosphate) deriv;Benzo[gr]pteridine riboflavin 5'-(trihydrogen diphosphate) deriv;Dihydro-fad;Dihydroflavine-adenine dinucleotide;Fadh2;Fda;Flavin adenine dinucleotide (reduced);Flavin adenine dinucleotide reduced;Reduced flavine adenine dinucleotidePW_C000932FADH2561710453149042505545398102605615661181626501179748922390772241252322712527249771541347852534580036369117810133120001384121299418122663406122778121123869454125238120125380480125980489126795479126922391127433506128393501407114Fe-4SHMDB0061380Tetrakis(λ¹-iron(1+) ion) tetrasulfane tetrasulfanide belongs to the class of inorganic compounds known as transition metal sulfides. These are inorganic compounds containing a sulfur atom of an oxidation state of -2, in which the heaviest atom bonded to the oxygen is a transition metal.33723S[Fe]12[S]3[Fe]4(S)[S]1[Fe]1(S)[S]2[Fe]3(S)[S]41Fe4H4S8InChI=1S/4Fe.4H2S.4S/h;;;;4*1H2;;;;/q4*+1;;;;;;;;/p-4CFWDOBXUEATMSD-UHFFFAOYSA-Jtetrakis(lambda1-iron(1+) ion) tetrasulfane tetrasulfanide483.932483.547634180tetrakis(lambda1-iron(1+) ion) tetrasulfane tetrasulfanide01PW_C0407114Fe-4S47383485985051450652540311060601576124163650818074932241261816477160112780031118004037080681135117797133117828132120009407120161122122658406122673124122784119125233120125248118125387481125522297126790479126806299126928206127057205128387501128404388405582Fe-2SHMDB0061344Bis(λ²-iron(2+) ion) disulfane tetrasulfanide belongs to the class of inorganic compounds known as transition metal sulfides. These are inorganic compounds containing a sulfur atom of an oxidation state of -2, in which the heaviest atom bonded to the oxygen is a transition metal.S[Fe]1(S)S[Fe](S)(S)S1Fe2H4S6InChI=1S/2Fe.4H2S.2S/h;;4*1H2;;/q2*+2;;;;;;/p-4TUVRTRPESCSNGS-UHFFFAOYSA-Jbis(lambda2-iron(2+) ion) disulfane tetrasulfanide308.112307.7336085340bis(lambda2-iron(2+) ion) disulfane tetrasulfanide02PW_C0405582Fe2S3742240128425717438934626547543148299483728505645497126551212770461601303030113042302777151137772733778377134783861327920711211781113312158640712179512412256738412266440612315244312316744812414411912434611812514012112523912012618929912671548012679647912768038812830639112839450144Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrialP08559The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.
HMDBP00046PDHA1Xp22.1M2915511.2.4.12064178036391113696270212Pyruvate dehydrogenase E1 component subunit beta, mitochondrialP11177The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.
HMDBP00012PDHB3p21.1-p14.2M3447911.2.4.12074172736392113696370253Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrialP10515The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.
HMDBP00055DLAT11q23.1AP00090712.3.1.122144178786393113696470252Dihydrolipoyl dehydrogenase, mitochondrialP09622Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
HMDBP00054DLD7q31-q32L1375711.8.1.4217410803467086394113696570241Citrate synthase, mitochondrialO75390HMDBP00043CS12q13.2AF04704212.3.3.122041316643136966702842Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrialP50213HMDBP00899IDH3A15q25.1-q25.2CH47113611.1.1.412284136967702840Isocitrate dehydrogenase [NAD] subunit beta, mitochondrialO43837HMDBP00897IDH3B20p13BC00196011.1.1.412294136968702843Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrialP51553HMDBP00900IDH3GXq28BC00190211.1.1.4123041369697024302-oxoglutarate dehydrogenase, mitochondrialQ02218
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).
HMDBP00439OGDH7p14-p13BC01461711.2.4.223441076346688136970702882Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrialP36957
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of 3 enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).
HMDBP00939DLST14q24.3AC00653012.3.1.6123541079346698136971702832Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrialP53597Catalyzes the ATP- or GTP-dependent ligation of succinate and CoA to form succinyl-CoA. The nature of the beta subunit determines the nucleotide specificity (By similarity).
HMDBP00889SUCLG12p11.2Z6820416.2.1.4; 6.2.1.52414136972702834Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrialQ96I99Catalyzes the GTP-dependent ligation of succinate and CoA to form succinyl-CoA (By similarity).
HMDBP00891SUCLG23p14.1AC11440116.2.1.42424136973702806Fumarate hydratase, mitochondrialP07954Also acts as a tumor suppressor.
HMDBP00861FH1q42.1U5930914.2.1.22634136974702803Malate dehydrogenase, mitochondrialP40926HMDBP00858MDH27cen-q22AK29077911.1.1.372654110031117219Pyruvate carboxylase, mitochondrialP11498Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate.
HMDBP00019PC11q13.4-q13.5K0228216.4.1.1284168982395323982136975702145Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrialP31040Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Can act as a tumor suppressor.
HMDBP00150SDHA5p15AK29131111.3.5.12571750574136976703188Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrialP21912Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
HMDBP00193SDHB1p36.1-p35U1724811.3.5.12581750584136977703116Succinate dehydrogenase cytochrome b560 subunit, mitochondrialQ99643Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
HMDBP00121SDHC1q23.3AK29430512591750594136978703174Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrialO14521Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (By similarity).
HMDBP00179SDHD11q23BC022350126017506041369797035479Mitochondrial pyruvate carrier 1Q9Y5U8Mediates the uptake of pyruvate into mitochondria.
HMDBP11834MPC16q27AF1518871844017688Aconitate hydratase, mitochondrialQ99798Catalyzes the isomerization of citrate to isocitrate via cis-aconitate (By similarity).
HMDBP00725ACO222q13.2U8793214.2.1.32234473931369807044308Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrialQ96HY7
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity).
HMDBP09099DHTKD110p14AC07316011.2.4.251513805Malate dehydrogenase, cytoplasmicP40925HMDBP00860MDH12p13.3AK31233111.1.1.37; 1.1.1.9617228239025061461Pyruvate dehydrogenase complex1PW_P00006168446069126070536071522434106013576913696424236457Citrate synthase, mitochondrial1PW_P00005760412218759Isocitrate dehydrogenase1PW_P000059628422638401648431304234225460Oxoglutarate dehydrogenase complex1PW_P0000606543081668821675223110601327691339642232462Succinyl-CoA ligase1PW_P000062728321738341237466Fumarate hydratase, mitochondrial1PW_P000066808064261468Malate dehydrogenase, mitochondrial1PW_P00006882803226445Pyruvate carboxylase, mitochondrial1PW_P000005619452016102748464Succinate dehydrogenase1PW_P00006475145176188177116178174138964142340558124317303Mitochondrial pyruvate carrier1PW_P0003033225479110971758Aconitate hydratase, mitochondrial1PW_P0000586168814604071112214473Malate dehydrogenase, cytoplasmic1PW_P0004734968051594falsePW_R000594Right25001641Compoundfalse250110991Compoundtrue25027211Compoundtrue25039401Compoundfalse250413161Compoundtrue250511441Compoundtrue69611.2.4.1590falsePW_R000590Right24769401Compoundfalse247714201Compoundtrue24781481Compoundfalse2479631Compoundfalse248010991Compoundtrue62572.3.3.1332falsePW_R000332Right13801251Compoundfalse13817211Compoundtrue13821341Compoundfalse138313161Compoundtrue138411441Compoundtrue1385400341Compoundtrue70591.1.1.41592falsePW_R000592Right24871341Compoundfalse24887211Compoundtrue248910991Compoundtrue24908081Compoundfalse249111441Compoundtrue2492400341Compoundtrue249313161Compoundtrue67601.2.4.2593falsePW_R000593Both24948081Compoundfalse249511041Compoundtrue24969361Compoundtrue24971741Compoundfalse249810991Compoundtrue24999861Compoundtrue6862269falsePW_R000269Both11451011Compoundfalse1146881Compoundfalse114714201Compoundtrue78664.2.1.2390falsePW_R000390Both16051011Compoundfalse16067211Compoundtrue16071481Compoundfalse160811441Compoundtrue1609400341Compoundtrue794734falsePW_R000004Right154141Compoundtrue161641Compoundfalse174631Compoundtrue1810341Compoundtrue1911041Compoundtrue201481Compoundfalse456.4.1.1233falsePW_R000233Both10101741Compoundfalse10118461Compoundtrue25129641Compoundtrue1012881Compoundfalse101310061Compoundtrue25139321Compoundtrue75641.3.5.189falsePW_R000089Both350631Compoundfalse351511Compoundfalse35214201Compoundtrue63584.2.1.390falsePW_R000090Both353511Compoundfalse248114201Compoundtrue3541251Compoundfalse64584.2.1.31PW_T00000111641Compound24Right63032013-07-08T16:38:23-06:002013-07-08T16:38:23-06:0017260164481false131584510regular2001902611099485false174098510regular5030262721459false1750103510regular5030263940482false1503137010regular3002802641316452false1494126610regular78782651144460false1746127510regular5030266106049false1705118510regular1003526776949false1705112510regular1002526896449false1510119510regular100252691420449false1643181010regular7878270148481false1078164510regular20019027163481false2113164510regular2001902721099485false1893182510regular503027351481false2558184010regular200190276125481false2558223510regular200190277721459false2728244010regular5030278134481false2309272010regular2001902791316452false2485263110regular78782801144460false2737265010regular503028140034455false2628277110regular787828242349false2713254210regular10025283721459false2144270010regular50302841099485false2174292010regular5030285808482false1434267610regular3002802861144460false1889269610regular503028740034455false1830291710regular78782881316452false1795263210regular7878289106049false1989272510regular1003529076949false1934287010regular1002529196449false2054287010regular100252921104446false1312270110regular4443293936453false1299291210regular5030294174481false734272010regular2001902951099485false1049270510regular5030296986454false1049291010regular5030309881781false509227010regular200190318101481false509185510regular2001903191420449false709220010regular7878320721459false689164010regular50303211144460false928164210regular503032240034455false929184810regular7878326414442false1002107510regular5030327463447false98398610regular78783281034443false999123510regular50303291104446false1012128810regular44433302049false1187120010regular10025331102749false1067120010regular1002533884617139false464276510regular503033996417137false384268010regular50303404009817140false464246510regular503034193217138false384250010regular5030342964179true619258710regular100251789164281false131523010regular200190102461420349false2613160010regular7878102474071139false2445169010regular10025102481420349false2773200510regular7878102494071139false2513212210regular1002569444136false156010858proteinregular175155701242true154011508subunitregular15070715342true149511708subunitregular15070725242true145011758subunitregular15070734146false172317008subunitregular160807684246false257824928subunitregular1608077840497false264325628subunitregular1507078843498false252825628subunitregular15070794304136false195427358proteinregular1751558088242true194428058subunitregular15070815242true199427658subunitregular1507082832497false112428038noneregular150708383442false112427838proteinregular150709380648false53921108subunitregular140859480346false75317008subunitregular16080961948false110711358subunitregular14085991451799false41425628noneregular150701001881798false44925678noneregular150701011161797false41425828noneregular15070102174172false43925878proteinregular1507068354791776false13406858subunitregular15070561868832false242517058subunitregular15070561968832false258321028subunitregular15070606174696970707171727235266326Cofactor36267327Cofactor37268328Cofactor615774737364597476767777787838282346Cofactor65607479798080818139289354Cofactor40290355Cofactor41291356Cofactor666274828283837366749393746874949476574969649330405Cofactor50331406Cofactor7964717999910010010110110210253342420Cofactor6363037676683458458735589561820521024716240Cofactor458558735590561920531024916244Cofactor320M1515 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17.380887721185843 25.575134323078345false333M1918 1825 C1917 1791 1913 1740 1883 1740 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false340M2658 2425 C2658 2455 2658 2462 2658 2492 5false18341M2728 2455 C2695 2455 2658 2462 2658 2492 5false18342M2509 2815 C2592 2813 2661 2694 2662 2621 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false343M2563 2670 C2587 2670 2662 2650 2662 2620 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false344M2737 2665 C2703 2666 2663 2652 2663 2622 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false345M2667 2771 C2667 2740 2662 2653 2662 2623 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false346M2398 2812.5 L2398 2862.5 L2448 2812.5 z10true18347M2309 2815 C2279 2815 2159 2815 2129 2815 5false18348M2169 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2238 609 2225 609 2195 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false391M609 1855 C609 1784 688 1740 753 1740 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false392M714 1670 C715 1708 723 1740 753 1740 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false393M1078 1740 C1048 1740 943 1740 913 1740 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false394M953 1672 C952 1705 943 1740 913 1740 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false395M968 1848 C968 1811 943 1740 913 1740 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false399M1052 1090 C1090 1090 1176 1101 1177 1135 5false18400M1315 940 C1222 962 1177 1053 1177 1135 5false18401M1061 1025 C1094 1025 1176 1096 1177 1135 5false18402M1049 1250 C1097 1250 1177 1250 1177 1220 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false403M1056 1309.5 C1088 1309.5 1178 1267 1177 1220 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false404M1178 1645 C1178 1615 1177 1250 1177 1220 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false405M200 250 L200 300 L250 250 z10true18406M200 250 L200 300 L250 250 z10true18414M734 2815 C634 2812 489 2744 489 2655 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false415M489 2765 C489 2723 486 2698 489 2655 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false416M434 2695 C465 2695 489 2685 489 2655 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false417M609 2460 C580 2497 489 2532 489 2562 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false418M489 2495 C489 2524 489 2498 489 2562 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false419M434 2515 C459 2515 489 2517 489 2562 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false420M200 250 L200 300 L250 250 z10true182582M1415 420 C1415 450 1415 655 1415 685 83false18trueM 1362.5 602.0096189432334 L 1370 615 L 1377.5 602.0096189432334false2583M1415 845 C1415 815 1415 785 1415 755 83false18trueM 1367.5 762.0096189432334 L 1375 775 L 1382.5 762.0096189432334false8471M1515 240 C1545 240 1700 240 1730 240 5false18trueM 1498.9903810567666 167.5 L 1486 175 L 1498.9903810567666 182.5false8472M1515 325 C1545 325 1700 325 1730 325 5false18trueM 1497.9903810567666 247.5 L 1485 255 L 1497.9903810567666 262.5false8473M1515 410 C1545 410 1700 410 1730 410 5false18trueM 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25.575134323078345false16243M2658 2235 C2658 2205 2658 2202 2658 2172 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false16244M200 250 L200 300 L250 250 z10true185975944250260320Left251261321Left252262322Left253263323Right254264324Right255265325Right5869606075904256263329Left257269330Left258270331Left259271332Right260272333Right5962616373324267276340Left268277341Left269278342Right270279343Right271280344Right272281345Right6270646475924273278347Left274283348Left275284349Left276285350Right277286351Right278287352Right279288353Right6367656575934280285357Left281292358Left282293359Left283294360Right284295361Right285296362Right6468667372694305318388Left306309389Right307319390Right7178737473904308318391Left309320392Left310270393Right311321394Right312322395Right72797476744315326399Left316260400Left317327401Left318328402Right319329403Right320270404Right7447679723317326294414Left327338415Left328339416Left329309417Right330340418Right331341419Right77757930687893977327116237Left977427316238Right97751024616239Right311963458430697903977627316241Left97771024816242Left977827616243Right3120644585521710317892582Left1042602583Right46366379352714false173020516regular35817898471Left380353714false173029016regular35917898472Right381354714false173037516regular36017898473Right382355714false853138516regular3612708478Left383356714false853147016regular3622708479Left384357714false1278209516regular3632708480Right385358714false1278201016regular3642708481Right386359714false1278192516regular3652708482Right387360714false229224516regular3663098483Right388361714false229233016regular3673098484Right389362714false849233016regular3683098485Right390363714false849241516regular3693098486Right391364714false759303516regular3702948487Left392365714false216260016regular3713418488Left393366714false2139244516regular3722788493Left394367714false2139252516regular3732788494Left395368714false2139260516regular3742788495Left396369714false2519295516regular3752788496Left397370714false2519303516regular3762788497Right398371714false1213134516regular3772638547Right399372714false1213142016regular3782638548Right400373714false1213149516regular3792638549Right401374714false1213157016regular3802638550Right402375714false1938134516regular3812638551Left403376714false1938142016regular3822638552Left404377714false1938149516regular3832638553Left405378714false1938157016regular3842638554Left3132101601.61.63602149024018713424952001.31.3021432726712M495 3133 C494 2937 494 2727 494 2558 C494 2396 250 2404 250 2557 C250 2729 247 2935 247 3135 84false6248.0578.099M126 226 C126 176 176 126 226 126 C1037 126 2091 126 2902 126 C2952 126 3002 176 3002 226 C3002 1104 3002 2245 3002 3123 C3002 3173 2952 3223 2902 3223 C2091 3223 1037 3223 226 3223 C176 3223 126 3173 126 3123 C126 2245 126 1104 126 226 1true62876.03097.0117M203 724 C1061 725 1978 720 2927 720 84false62724.04.0118M202 628 C1142 628 2240 627 2927 627 84false62725.01.0515Inner mitochondrial membrane1802875201.01.016015615Mitochondrial intermembrane space2802935201.01.016015715Cytosol1915195203.13.116015815Mitochondrial matrix2190710204.04.016015915Mitochondrion325205202.22.21601586215Inner mitochondrial membrane280655201.61.61601586315Outer mitochondrial membrane280560201.61.61601523667402202601292731344#FFEBEB4272525332-Ketoglutarate Dehydrogenase Complex Deficiency2-Ketoglutarate dehydrogenase complex deficiency is a rare autosomal recessive disease. 2-ketoglutarate dehydrogenase is an enzyme of the Krebs cycle that catalyzes the oxidation of alpha-ketoglutarate to succinyl CoA. The deficiency of 2-Ketoglutarate dehydrogenase complex results in the disorder of Krebs cycle with accumulation of succinyl CoA. The primary manifestations include developmental delay, ataxia, opisthotonus, seizure and other neurological symptoms. DiseasePW_X000234Context2341168430ProteinMutated1169150CompoundIncreased117088CompoundIncreased1171122CompoundIncreased1172134CompoundIncreased145324TissueDamaged14541TissueDamaged145511TissueDamaged1690[Metagen: 2-KETOGLUTARATE DEHYDROGENASE COMPLEX DEFICIENCY](http://metagene.de/program/d.prg?id_d=401)234Context1691[OMIM: 203740](http://omim.org/entry/203740})234Context2785321640293Bonnefont JP, Chretien D, Rustin P, Robinson B, Vassault A, Aupetit J, Charpentier C, Rabier D, Saudubray JM, Munnich A: Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis. J Pediatr. 1992 Aug;121(2):255-8.234Context27930923475850Kiss G, Konrad C, Doczi J, Starkov AA, Kawamata H, Manfredi G, Zhang SF, Gibson GE, Beal MF, Adam-Vizi V, Chinopoulos C: The negative impact of alpha-ketoglutarate dehydrogenase complex deficiency on matrix substrate-level phosphorylation. FASEB J. 2013 Jun;27(6):2392-406. doi: 10.1096/fj.12-220202. Epub 2013 Mar 8.234Context