734PathwayCitric Acid Cycle 1434561204The citric acid cycle, which is also known as the tricarboxylic acid cycle (TCA cycle) or the Krebs cycle, is a series of enzyme-catalyzed chemical reactions of key importance in all living cells that use oxygen as part of cellular respiration. In eukaryotes, the citric acid cycle occurs in the mitochondrial matrix. The TCA 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). The citrate then goes through a series of chemical transformations, losing first one, then a second carboxyl group as CO2. The carbons lost as CO2 originate from what was oxaloacetate, not directly from acetyl-CoA. The carbons donated by acetyl-CoA become part of the oxaloacetate carbon backbone after the first turn of the citric acid cycle. Loss of the acetyl-CoA-donated carbons as CO2 requires several turns of the citric acid cycle. However, because of the role of the citric acid cycle in anabolism, they may not be lost since many TCA cycle intermediates are also used as precursors for the biosynthesis of other molecules. Most of the energy made available by the oxidative steps of the cycle is transferred as energy-rich electrons to NAD+, forming NADH. For each acetyl group that enters the citric acid cycle, three molecules of NADH are produced. At the end of each cycle, the four-carbon oxaloacetate has been regenerated, and the cycle continues - PathWhiz. MetabolicPW000970CenterPathwayVisualizationContext125732003100#000099PathwayVisualization718734Citric Acid Cycle 1434561204The citric acid cycle, which is also known as the tricarboxylic acid cycle (TCA cycle) or the Krebs cycle, is a series of enzyme-catalyzed chemical reactions of key importance in all living cells that use oxygen as part of cellular respiration. In eukaryotes, the citric acid cycle occurs in the mitochondrial matrix. The TCA 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). The citrate then goes through a series of chemical transformations, losing first one, then a second carboxyl group as CO2. The carbons lost as CO2 originate from what was oxaloacetate, not directly from acetyl-CoA. The carbons donated by acetyl-CoA become part of the oxaloacetate carbon backbone after the first turn of the citric acid cycle. Loss of the acetyl-CoA-donated carbons as CO2 requires several turns of the citric acid cycle. However, because of the role of the citric acid cycle in anabolism, they may not be lost since many TCA cycle intermediates are also used as precursors for the biosynthesis of other molecules. Most of the energy made available by the oxidative steps of the cycle is transferred as energy-rich electrons to NAD+, forming NADH. For each acetyl group that enters the citric acid cycle, three molecules of NADH are produced. At the end of each cycle, the four-carbon oxaloacetate has been regenerated, and the cycle continues - PathWhiz. Metabolic18168777SubPathway2171164Compound160168876SubPathway2172164Compound160168984SubPathway2173164Compound160169060SubPathway2174148Compound161169177SubPathway2175148Compound16116923SubPathway2176148Compound16116934SubPathway2177148Compound161169484SubPathway2178148Compound16116954SubPathway217988Compound161169687SubPathway218088Compound161169715SubPathway218188Compound161169878SubPathway218288Compound1611699101SubPathway2183174Compound1611700348SubPathway2184932Compound162170160SubPathway2185134Compound16117027SubPathway2186134Compound16117033SubPathway2187134Compound161170477SubPathway2188134Compound161170575SubPathway2189134Compound161170686SubPathway2190940Compound161170789SubPathway2191940Compound161170884SubPathway2192940Compound161170910SubPathway2193940Compound161171055SubPathway2194940Compound161171158SubPathway2195940Compound1611712342SubPathway2196940Compound1611713101SubPathway2197940Compound1611CellCL:00000006MyocyteCL:00001875HepatocyteCL:00001824Cardiomyocyte CL:00007463NeuronCL:00005407Epithelial CellCL:00000662Platelet CL:00002338Beta cellCL:00006391Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote17Rattus norvegicus10116EukaryoteRat12Mus musculus10090EukaryoteMouse2Bacteria2ProkaryoteBacteria24Solanum lycopersicum4081EukaryoteTomato4Arabidopsis thaliana3702EukaryoteThale cress18Saccharomyces cerevisiae4932EukaryoteYeast21Xenopus laevis8355EukaryoteAfrican clawed frog5Bos taurus9913EukaryoteCattle38homo sapiens9608Eukaryote19Schizosaccharomyces pombe4896Eukaryote6Caenorhabditis elegans6239EukaryoteRoundworm29Saccharomyces cerevisiae (strain ATCC 204508 / S288c)559292EukaryoteBaker's yeast1CytosolGO:00058293Mitochondrial MatrixGO:00057595CytoplasmGO:00057372MitochondrionGO:00057397Endoplasmic Reticulum MembraneGO:000578912Mitochondrial Inner MembraneGO:000574310Cell MembraneGO:000588635chloroplastGO:000950714Mitochondrial Outer MembraneGO:000574127Peroxisome MembraneGO:00057784PeroxisomeGO:00057778Smooth Endoplasmic Reticulum GO:000579013Endoplasmic ReticulumGO:000578325Golgi apparatusGO:00057946LysosomeGO:000576416Lysosomal LumenGO:004320231Periplasmic SpaceGO:000562011Extracellular SpaceGO:000561524Mitochondrial Intermembrane SpaceGO:000575820Endoplasmic Reticulum LumenGO:000578818Melanosome MembraneGO:003316221SynapseGO:004520215NucleusGO:000563426Golgi apparatus membraneGO:000013919sarcoplasmic reticulumGO:001652934Plant-Type VacuoleGO:000032532Inner MembraneGO:00702589MuscleBTO:0000887141181LiverBTO:000075972924BrainBTO:000014289164Adrenal MedullaBTO:000004971828StomachBTO:00013071552625IntestineBTO:00006487Nervous SystemBTO:00014848Blood VesselBTO:000110274112Endothelium BTO:00003935cardiocyteBTO:000153918PancreasBTO:00009882111PW_BS0000024311PW_BS0000048511PW_BS0000083211PW_BS000003509516PW_BS000050261115PW_BS000026103331PW_BS000103117131PW_BS0001171181171PW_BS0001181203171PW_BS0001201321121PW_BS0001321333121PW_BS000133951721PW_BS0000951471241PW_BS000147151141PW_BS0001511553241PW_BS00015515612241PW_BS0001561613181PW_BS0001611601181PW_BS00016011PW_BS0000011783211PW_BS0001781771211PW_BS00017785241011PW_BS000085222341PW_BS00002422014PW_BS0000242253541PW_BS000024221411PW_BS000022171211PW_BS000017592711PW_BS0000595411PW_BS00000529111PW_BS000029311511PW_BS000031111811PW_BS000011181311PW_BS000018101711PW_BS0000105811411PW_BS00005814101PW_BS000014541315PW_BS0000546131PW_BS0000061021231PW_BS0001021041431PW_BS000104124151PW_BS00012410813PW_BS000108101531PW_BS00010116212181PW_BS00016219914181PW_BS000024188118PW_BS0000241632181PW_BS0001631985181PW_BS00002421013181PW_BS000024226441PW_BS000024224241PW_BS00002417018PW_BS00017025012381PW_BS0000242513381PW_BS00002425214381PW_BS0000241951318PW_BS000024432511PW_BS00004349711PW_BS0000499611PW_BS0000092811611PW_BS0000281115121PW_BS0001111122121PW_BS0001121231751PW_BS0001231251351PW_BS0001251355171PW_BS000135100521PW_BS00010014117191PW_BS0001411572241PW_BS00015717117381PW_BS000171205561PW_BS000024206261PW_BS00002413121PW_BS000013204111PW_BS0000207028511PW_BS000070107313PW_BS0001071901118PW_BS0000242137181PW_BS0000242491341PW_BS000024422411PW_BS0000423612011PW_BS000036126651PW_BS00012612711651PW_BS00012717912211PW_BS0001792231241PW_BS00002415111PW_BS000015331811PW_BS0000332441011PW_BS00002460251PW_BS00006046114PW_BS00004672513PW_BS000072612517PW_BS0000613772113PW_BS00003793252011PW_BS00009327151PW_BS000027711PW_BS000007971521PW_BS000097943PW_BS000094105113PW_BS0001051136121PW_BS000113110231PW_BS00011013013121PW_BS0001301141112PW_BS00011412915121PW_BS000129140103PW_BS00014014315191PW_BS0001431465191PW_BS00014615924PW_BS00015916611PW_BS00016617315381PW_BS0001731765381PW_BS0001761802211PW_BS00018015284PW_BS000152207661PW_BS0000242111018PW_BS00002421425181PW_BS0000242156181PW_BS0000242164181PW_BS00002421217181PW_BS00002417211385PW_BS0001721873118PW_BS000024219314PW_BS00002416212PW_BS000016397113PW_BS000039215114PW_BS000021231511PW_BS000023918511PW_BS000091562611PW_BS0000561644PW_BS000164471914PW_BS0000472273441PW_BS000024241529PW_BS00002425715291PW_BS000024731013PW_BS0000733211515PW_BS0000326618518PW_BS0000665181PW_BS000051892PW_BS0000892171518PW_BS00002421815181PW_BS0000241893218PW_BS000024254518PW_BS000024164Pyruvic acidHMDB00243Pyruvic 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 acidPW_C00016417220442281181314495014572653651035405117544011854441205566132557013358939559201475951151602215560671566074161612616063831646717865101776532857457222749522082002251099Coenzyme AHMDB01423Coenzyme 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. 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 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 protein 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(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-DRCCLKDXSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[3-hydroxy-2,2-dimethyl-3-({2-[(2-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_C0010992114386884538792289217240759241422459528132928623133421133511846181046295848421448655448796523210252471045280103547712457341085777101602315560751616384164681786930160696116269731997083188710816372931987347210745822282291519081226909022491241709165250917725191812529215195721NADHMDB00902NAD (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-1λ⁵-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-1λ⁵-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 trihydratePW_C00072114041503353865110111421134431273514665422294927791728352931079480718481318481928490264960315167955238103533411153601125469123548212555901355610118569610057381085827141591214759421516024155607215760761616385163961716469178677211768901607012188709716371742057197206740519874592228241226835922590852249171251940Acetyl-CoAHMDB01206The 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 APW_C00094021343858842324162244652896173340114840145278103547612457331086025155607716163861647017869231607106163729119874602228245151827721091762511316Carbon dioxideHMDB01967Carbon 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 anhydridePW_C0013165081211204448013503186403677316952080651133431638491745225511731447052831035320111575010857711015968100602615560781616471178663710769221907017160703516370611887163205730819873332137461222753021082152258223151915824992122511144NADHHMDB01487NADH 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-NADHPW_C001144143415334908648101115212755146954223049278117283629310994806184812184821284904649593151699552401035332111535811254661235479125559313556981005737108582914159151475945151602715560791616387163981716472178677111768931607011188709916371722057195206746222282442268360225908622491732511060Thiamine pyrophosphateHMDB01372Thiamine 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-pyrophosphatePW_C0010602054107531197812715173625366103602815560801616388164731787463222769LipoamideHMDB00962Lipoamide 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 amidePW_C00076920241073317342466785367103602915560811616389164741787464222964FADHMDB01248FAD 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}(hydroxy)phosphoryl)oxy]({[(2R,3S,4S)-5-{7,8-dimethyl-2,4-dioxo-2H,3H,4H,10H-benzo[g]pteridin-10-yl}-2,3,4-trihydroxypentyl]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 dinucleotidePW_C0009649991145186819232164253176282882518840211881414894216122916224921335825362237232646023646883147411347581048816526810352851025335111549612655111275613118603015560541566082161611616263901647517864991796666107703916371752057321213746522274872239076224920425192132501420WaterHMDB02111Water 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;SteamPW_C001420558949109513941513162144811352615624286521069120770338231883821094311377491465541590432018242532222678602727462778172805293143703164723634614598364727374941935030275156751959752141005227945236103529710553191115343113535511254021105470123548312554921265507127553413055371145541129559113556081185622108569165759140577810158411435853146587710758909559101475940151603215560591576087161612316361331596215162181666420173643717664771786507180660015267131176840188688816071622057181207719320672112117228213723821472432157295198735021673882107401212746722274922247500190758817082012258237226841416291692519265172148Oxalacetic acidHMDB00223Oxaloacetic 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 acidPW_C00014825496911151099311094211132168885371103544812055741336033155608816164781787468222751322475171518372220837822563Citric acidHMDB00094Citric 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 acidPW_C00006321942415253721036034155608916164791787469222125Isocitric acidHMDB00193The 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_C00012522445064253741036035155609116164811787470222134Oxoglutaric acidHMDB00208Alpha-ketoglutaric acid is an important biological compound and is a key intermediate in the Krebs cycle. Alpha-ketoglutaric acid occurs naturally within cells. One of its functions is to combine with ammonia to form glutamic acid and then glutamine. Another function is to combine with nitrogen released in the cell, therefore preventing nitrogen overload. (wikipedia).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;OxoglutaratePW_C000134152423141414684991867331110842126351447501455261467545375103541411754381185564132600814760361556069157609216164821786530857471222751522475191518209225837422040034Hydrogen IonHMDB59597Hydrogen 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-Nhydron1.00791.0078250320hydron10PW_C04003421546708753157883184831116214632614645422314927801742502242544245471045761846947052411035327111535311256261085639107569910057201055742117596314760371556070157609316161301596232166648317866011526692101684318869101877100163716820571912067453219745422074722227525213753221075582127572160759017081952258218151824322684131628420224913919591552499174251423MagnesiumHMDB00547Magnesium 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 PW_C0004238682274268164762727268115819188832293639983399221116746148349152943176414212410241159294223312629337374540314774914869544974565253104532911153561125376103590614759341516038155609416162501666484178659416468811606979199717020571942067227213723321172502147310216731319874732229187252808Succinyl-CoAHMDB01022Succinyl-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-APW_C0008082334105533669253781036039155609716164851787015160736116374742221104PhosphateHMDB01429Phosphate 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-Kphosphate94.971494.953420phosphate-3-2DBMET00532FDB022617NFB 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-);PiPW_C0011042448488145818188312980317631417674925001027294727374631292931667236366138512342492244753150312751587520797521610053171115351112538110354471205543129557313356051355625108569365848143585514659111475941151604015561001616294107643217364391766487178669110167141176842188688916071612057189206721221173061987389210740221274361637475222819622582582271011824110134257936Guanosine diphosphateHMDB01201Guanosine 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-1H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]phosphonic acid443.2005443.024329371-2.007GDP0-2FDB0224875'-GDP;GDP;Guanosine 5'-(trihydrogen pyrophosphate);Guanosine 5'-diphosphate;Guanosine 5'-pyrophosphate;Guanosine mono(trihydrogen diphosphate);Guanosine pyrophosphate;Guanosine-5'-diphosphate;Guanosine-diphosphate;PpGPW_C00093683823841762142391241547350078553821036041155610116164881787476222174Succinic acidHMDB00254Succinic 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 acidPW_C000174152323945021850786763112655425517538310360421556102161645410764551086489178676411768361667362163745521974562207477222986Guanosine triphosphateHMDB01273Guanosine 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);H4gtpPW_C00098681824041939240911441537350068553841036043155610316164901787478222101L-Malic acidHMDB00156Malic 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 acidPW_C000101262410983172182387253871035735108604615561061616453107649117874512197452220747922288Fumaric acidHMDB00134Fumaric 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 acidPW_C00008810282541720042505345388102604715661071626458107645910864921796763117683716674802239065151414Adenosine triphosphateHMDB00538Adenosine 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 esterPW_C0004149221460826616414224781373332799593439976321051821121021464921561421605824055924342727264628122930296631637236166136175143992344743147689148645450328950352651557520597521510052501045291101531311153461125390103540611754301185443120554212955561325569133560313556211085846143585414658761075897147592415160481556109161623016664301736438176649317868391886870160697619971572057184206720921072252137229211729819873022167390217740821874321637481222749919081862259184252463Hydrogen carbonateHMDB00595Bicarbonate, 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-Mhydrogen carbonate61.016860.9925688980.971bicarbonate-1-1FDB022134Bicarbonate;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 carbonatePW_C000463224168782393323972261315314570539110354451205571133604915561101616494178748222290922241034Adenosine diphosphateHMDB01341Adenosine 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-diphosphatePW_C00103423413484152248213801596315978310611415182190149210418211310216158240859243527272847273646285529316572363561440023447631477091503626515775208975217100531511153491125392103544612055441295572133562410857411175764101584914358561465878107589914759261516050155611116162311666433173644017664951786700946841188687216071592057187206720821072262137231211730019873032167391217741021874331637483222818722520BiotinHMDB00030Biotin 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]imidazolidin-4-yl]pentanoic acid244.311244.088163078-2.303biotin0-1FDB014510(+)-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 SPW_C00002026413585791516993227025292101529810553931035449120554611155511145575133605115561121616496178692516074842221027ManganeseHMDB01333Manganese 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+) ion22FDB003636ManganesePW_C0010272744738148649155343227122394325131453941035450120557613360521556113161649717869261607485222846Coenzyme Q10HMDB01072Coenzyme 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-UPTCCGCDSA-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 10PW_C00084625217425142505245396102605315661151626498179748622340098QH(2)HMDB59661Qh(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-diol00PW_C040098255175054453971026055156611716265001797488223932FADHHMDB01197FADH 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}(hydroxy)phosphoryl)oxy]({[(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})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_C00093225617104531490425055453981026056156611816265011797489223907722451cis-Aconitic acidHMDB00072cis-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_C000051222454011036058155612216165061787491222407114Fe-4SHMDB6138033723S[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-J483.932483.54763418000PW_C04071147383485985051450652540311060601576124163650818074932249969UnknownUnknown1860831616121162684518968851607036163706318872102107213211721721272232137237214724121572831907301198730521673922177412218927525410292Pyruvate dehydrogenase E1 component subunit beta, mitochondrialP32473
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2).
PDB1291.2.4.1653516110293Pyruvate dehydrogenase complex protein X component, mitochondrialP16451
Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.
PDX129653616110294Glutathione reductaseP41921
Maintains high levels of reduced glutathione in the cytosol.
GLR1291.8.1.7653716110295Citrate synthase 3P43635CIT3292.3.3.16653816110296Homoisocitrate dehydrogenase, mitochondrialP40495
Catalyzes the NAD(+)-dependent conversion of homoisocitrate to alpha-ketoadipate.
LYS12291.1.1.87653916110297Homoisocitrate dehydrogenase, mitochondrialP40495
Catalyzes the NAD(+)-dependent conversion of homoisocitrate to alpha-ketoadipate.
LYS12291.1.1.87654016110300Succinyl-CoA ligase [ADP-forming] subunit alpha, mitochondrialP53598LSC1296.2.1.5654316110301Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrialP53312LSC2296.2.1.5654416110302Fumarate hydratase, mitochondrialP08417FUM1294.2.1.2654516110303Acetyl-CoA carboxylaseQ00955
Carries out three functions: biotin carboxyl carrier protein, biotin carboxylase and carboxyltransferase. Involved in the synthesis of very-long-chain fatty acid synthesis which is required to maintain a functional nuclear envelope. Required for acylation and vacuolar membrane association of VAC8 which is necessary to maintain a normal morphology of the vacuole.
ACC1296.4.1.2, 6.3.4.14654616110304Fumarate reductase 1P32614
Irreversibly catalyzes the reduction of fumarate to succinate. Together with the second isozyme of soluble fumarate reductase (OSM1), essential for anaerobic growth. Involved in maintaining redox balance. Reduction of fumarate is the main source of succinate during fermentation, and under anaerobic conditions, the formation of succinate is strictly required for the reoxidation of FADH(2).
FRD1291.3.1.6654716210305Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrialP21801
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). SDH1 and SDH2 form the catalytic dimer. Electrons flow from succinate to the FAD bound to SDH1, and sequentially through the iron-sulfur clusters bound to SDH2 and enter the membrane dimer formed by SDH3 and SDH4.
SDH2291.3.5.16548162103063-isopropylmalate dehydrataseP07264
Catalyzes the isomerization between 2-isopropylmalate and 3-isopropylmalate, via the formation of 2-isopropylmaleate.
LEU1294.2.1.33654916310307Mitochondrial pyruvate carrier 1P53157
Mediates the uptake of pyruvate into mitochondria.
MPC1299970Pyruvate dehydrogenase E1 component subunit beta, mitochondrialP32473
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2).
PDB1291.2.4.1608416179481639971Pyruvate dehydrogenase complex protein X component, mitochondrialP16451
Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.
PDX1296085161794916079501639972Glutathione reductaseP41921
Maintains high levels of reduced glutathione in the cytosol.
GLR1291.8.1.76086161794616079471639973Citrate synthase 3P43635CIT3292.3.3.1660901619974Homoisocitrate dehydrogenase, mitochondrialP40495
Catalyzes the NAD(+)-dependent conversion of homoisocitrate to alpha-ketoadipate.
LYS12291.1.1.8760951619975Homoisocitrate dehydrogenase, mitochondrialP40495
Catalyzes the NAD(+)-dependent conversion of homoisocitrate to alpha-ketoadipate.
LYS12291.1.1.8760961619978Succinyl-CoA ligase [ADP-forming] subunit alpha, mitochondrialP53598LSC1296.2.1.561041619979Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrialP53312LSC2296.2.1.561051619980Fumarate hydratase, mitochondrialP08417FUM1294.2.1.261081619981Acetyl-CoA carboxylaseQ00955
Carries out three functions: biotin carboxyl carrier protein, biotin carboxylase and carboxyltransferase. Involved in the synthesis of very-long-chain fatty acid synthesis which is required to maintain a functional nuclear envelope. Required for acylation and vacuolar membrane association of VAC8 which is necessary to maintain a normal morphology of the vacuole.
ACC1296.4.1.2, 6.3.4.14611416179531639982Fumarate reductase 1P32614
Irreversibly catalyzes the reduction of fumarate to succinate. Together with the second isozyme of soluble fumarate reductase (OSM1), essential for anaerobic growth. Involved in maintaining redox balance. Reduction of fumarate is the main source of succinate during fermentation, and under anaerobic conditions, the formation of succinate is strictly required for the reoxidation of FADH(2).
FRD1291.3.1.661191629983Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrialP21801
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). SDH1 and SDH2 form the catalytic dimer. Electrons flow from succinate to the FAD bound to SDH1, and sequentially through the iron-sulfur clusters bound to SDH2 and enter the membrane dimer formed by SDH3 and SDH4.
SDH2291.3.5.161201629984Homoaconitase, mitochondrialP49367
Catalyzes the reversible hydration of cis-homoaconitate to (2R,3S)-homoisocitrate, a step in the alpha-aminoadipate pathway for lysine biosynthesis.
LYS4294.2.1.3661251639985Mitochondrial pyruvate carrier 1P53157
Mediates the uptake of pyruvate into mitochondria.
MPC1293524Pyruvate dehydrogenase complex18PW_P003524103509969601035199706010352997160103539972243525Citrate synthase, mitochondrial18PW_P00352510354997323526Isocitrate dehydrogenase18PW_P0035261035599742103569969110357997513528Succinyl-CoA ligase18PW_P003528103619978110362997913529Fumarate hydratase, mitochondrial18PW_P00352910363998043531Pyruvate carboxylase, mitochondrial18PW_P00353110365998143532Succinate dehydrogenase18PW_P00353210366998211036799831103689969110369996913533Aconitate hydratase, mitochondrial18PW_P00353310370998413534Mitochondrial pyruvate carrier18PW_P00353410371998513530Malate dehydrogenase, 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