342PathwayOxidation of Branched-Chain Fatty AcidsIn the majority of organisms, fatty acid degradation occurs mostly through the beta-oxidation cycle. In plants, this cycle only happens in the peroxisome, while in mammals this cycle happens in both the peroxisomes and mitochondria. Unfortunately, traditional fatty acid oxidation does not work for branched-chain fatty acids, or fatty acids that do not have an even number of carbons, like the fatty acid phytanic acid, found in animal milk. This acid can not be oxidized through beta-oxidation, as problems arise when water is added at the branched beta-carbon. To be able to oxidize this fatty acid, the carbon is oxidized by oxygen, which removes the initial carboxyl group, which shortens the chain. Now lacking a methyl group, this chain can be beta-oxidized. Now moving to the mitochondria, there are four reactions that occur, and are repeated for each molecule of the fatty acid. Each time the cycle of these reactions is completed, the chain is relieved of two carbons, which are oxidized and are taken away by NADH and FADH2, energy carriers that collect the carbons energy. After beta-oxidation in the cycle of reactions, an acetyl-CoA unit is released and is recycled into the cycle of reactions in the mitochondria, until the chain is fully broken down into acetyl-CoA, and can enter the TCA cycle. Once in the TCA cycle, it is converted to NADH and FADH2, which in turn help move along mitochondrial ATP production. Acetyl-CoA also helps produce ketone bodies that are further converted to energy in the heart and the brain.
MetabolicPW000155CenterPathwayVisualizationContext17033004800#000099PathwayVisualization100342Oxidation of Branched-Chain Fatty AcidsIn the majority of organisms, fatty acid degradation occurs mostly through the beta-oxidation cycle. In plants, this cycle only happens in the peroxisome, while in mammals this cycle happens in both the peroxisomes and mitochondria. Unfortunately, traditional fatty acid oxidation does not work for branched-chain fatty acids, or fatty acids that do not have an even number of carbons, like the fatty acid phytanic acid, found in animal milk. This acid can not be oxidized through beta-oxidation, as problems arise when water is added at the branched beta-carbon. To be able to oxidize this fatty acid, the carbon is oxidized by oxygen, which removes the initial carboxyl group, which shortens the chain. Now lacking a methyl group, this chain can be beta-oxidized. Now moving to the mitochondria, there are four reactions that occur, and are repeated for each molecule of the fatty acid. Each time the cycle of these reactions is completed, the chain is relieved of two carbons, which are oxidized and are taken away by NADH and FADH2, energy carriers that collect the carbons energy. After beta-oxidation in the cycle of reactions, an acetyl-CoA unit is released and is recycled into the cycle of reactions in the mitochondria, until the chain is fully broken down into acetyl-CoA, and can enter the TCA cycle. Once in the TCA cycle, it is converted to NADH and FADH2, which in turn help move along mitochondrial ATP production. Acetyl-CoA also helps produce ketone bodies that are further converted to energy in the heart and the brain.
Metabolic1213Beta OxidationSubPathway3241381Compound5325988Compound5214Beta OxidationSubPathway3281381Compound5329940Compound5215Beta OxidationSubPathway3301381Compound53317198Compound5236Beta OxidationSubPathway3577198Compound3358940Compound375Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.342Pathway76Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.342Pathway280503444549Lawson LD, Kummerow FA: beta-Oxidation of the coenzyme A esters of elaidic, oleic, and stearic acids and their full-cycle intermediates by rat heart mitochondria. Biochim Biophys Acta. 1979 May 25;573(2):245-54. doi: 10.1016/0005-2760(79)90058-4.342Pathway28050415466478Yu W, Liang X, Ensenauer RE, Vockley J, Sweetman L, Schulz H: Leaky beta-oxidation of a trans-fatty acid: incomplete beta-oxidation of elaidic acid is due to the accumulation of 5-trans-tetradecenoyl-CoA and its hydrolysis and conversion to 5-trans-tetradecenoylcarnitine in the matrix of rat mitochondria. J Biol Chem. 2004 Dec 10;279(50):52160-7. doi: 10.1074/jbc.M409640200. Epub 2004 Oct 4.342Pathway28050510407780Wanders RJ, Vreken P, den Boer ME, Wijburg FA, van Gennip AH, IJlst L: Disorders of mitochondrial fatty acyl-CoA beta-oxidation. J Inherit Metab Dis. 1999 Jun;22(4):442-87.342Pathway28050610527927Zammit VA: The malonyl-CoA-long-chain acyl-CoA axis in the maintenance of mammalian cell function. Biochem J. 1999 Nov 1;343 Pt 3:505-15.342Pathway28050710051453Muoio DM, Seefeld K, Witters LA, Coleman RA: AMP-activated kinase reciprocally regulates triacylglycerol synthesis and fatty acid oxidation in liver and muscle: evidence that sn-glycerol-3-phosphate acyltransferase is a novel target. Biochem J. 1999 Mar 15;338 ( Pt 3):783-91.342Pathway2805098978480Watkins PA, Howard AE, Gould SJ, Avigan J, Mihalik SJ: Phytanic acid activation in rat liver peroxisomes is catalyzed by long-chain acyl-CoA synthetase. J Lipid Res. 1996 Nov;37(11):2288-95.342Pathway28051017613526,Westin MA, Hunt MC, Alexson SE: Peroxisomes contain a specific phytanoyl-CoA/pristanoyl-CoA thioesterase acting as a novel auxiliary enzyme in alpha- and beta-oxidation of methyl-branched fatty acids in mouse. J Biol Chem. 2007 Sep 14;282(37):26707-16. doi: 10.1074/jbc.M703718200. Epub 2007 Jul 5.342Pathway1CellCL:00000002Platelet CL:00002335HepatocyteCL:00001823NeuronCL:00005404CardiomyocyteCL:00007468Beta cellCL:00006397Epithelial CellCL:00000666MyocyteCL:000018711Colorectal Cancer CellCL:00010641Homo sapiens9606EukaryoteHuman2Bacteria2ProkaryoteBacteria3Escherichia coli562Prokaryote12Mus musculus10090EukaryoteMouse17Rattus norvegicus10116EukaryoteRat19Schizosaccharomyces pombe4896Eukaryote24Solanum lycopersicum4081EukaryoteTomato4Arabidopsis thaliana3702EukaryoteThale cress18Saccharomyces cerevisiae4932EukaryoteYeast21Xenopus laevis8355EukaryoteAfrican clawed frog6Caenorhabditis elegans6239EukaryoteRoundworm25Escherichia coli (strain K12)83333Prokaryote49Bathymodiolus platifrons220390EukaryoteDeep sea mussel23Pseudomonas aeruginosa287Prokaryote60Nitzschia sp.0001EukaryoteNitzschia45Bos taurus9913EukaryoteCattle10Drosophila melanogaster7227EukaryoteFruit fly51Picea sitchensis3332EukaryoteSitka spruce230Ambystoma mexicanum8296Eukaryoteaxolotl301Gallus Gallus1758Prokaryote1CytosolGO:00058293Mitochondrial MatrixGO:00057595CytoplasmGO:000573714Mitochondrial Outer MembraneGO:00057412MitochondrionGO:000573915NucleusGO:00056344PeroxisomeGO:000577713Endoplasmic ReticulumGO:00057837Endoplasmic Reticulum MembraneGO:000578910Cell MembraneGO:000588627Peroxisome MembraneGO:000577831Periplasmic SpaceGO:000562011Extracellular SpaceGO:000561535ChloroplastGO:000950712Mitochondrial Inner MembraneGO:000574332Inner MembraneGO:00702588Smooth Endoplasmic Reticulum GO:000579019Sarcoplasmic ReticulumGO:001652925Golgi ApparatusGO:000579426Golgi Apparatus MembraneGO:000013924Mitochondrial Intermembrane SpaceGO:00057586LysosomeGO:000576416Lysosomal LumenGO:004320218Melanosome MembraneGO:003316236MembraneGO:00160202Endothelium BTO:00003931LiverBTO:00007597297Nervous SystemBTO:000148418PancreasBTO:000098825IntestineBTO:00006488Blood VesselBTO:000110274115cardiocyteBTO:00015399MuscleBTO:00008871411824BrainBTO:000014289164Adrenal MedullaBTO:000004971828StomachBTO:00013071552622BladderBTO:000012311HeartBTO:000056273102111PW_BS0000024311PW_BS0000048511PW_BS00000816212PW_BS000016221411PW_BS00002213121PW_BS0000133211515PW_BS0000325411PW_BS000005397113PW_BS0000393211PW_BS000003181311PW_BS000018101711PW_BS00001049711PW_BS00004914101PW_BS0000145811411PW_BS000058592711PW_BS00005927151PW_BS00002746114PW_BS00004629111PW_BS0000296618518PW_BS00006672513PW_BS000072612517PW_BS0000615181PW_BS000051231511PW_BS000023311511PW_BS000031918511PW_BS000091541315PW_BS000054892PW_BS000089261115PW_BS000026711PW_BS000007971521PW_BS000097100521PW_BS0001001041431PW_BS000104101531PW_BS0001011115121PW_BS0001111122121PW_BS000112103331PW_BS000103117131PW_BS0001171181171PW_BS0001181203171PW_BS00012012915121PW_BS0001291321121PW_BS0001321333121PW_BS0001331355171PW_BS00013510813PW_BS00010814315191PW_BS0001431465191PW_BS000146107313PW_BS0001071471241PW_BS000147151141PW_BS0001511553241PW_BS0001551613181PW_BS00016116611PW_BS0001661783211PW_BS000178188118PW_BS0000241601181PW_BS00016019914181PW_BS000024205561PW_BS000024206261PW_BS00002421013181PW_BS0000242137181PW_BS0000242111018PW_BS0000241985181PW_BS0000242164181PW_BS0000242171518PW_BS00002421815181PW_BS0000241632181PW_BS000163222341PW_BS0000241901118PW_BS0000242253541PW_BS0000242771218PW_BS00002417018PW_BS0001702811251PW_BS0000241644PW_BS0001642851041PW_BS000024226441PW_BS0000242905491PW_BS0000242231241PW_BS0000243081011PW_BS000024315123PW_BS0000243221231PW_BS0000243183123PW_BS000024253541PW_BS00002413412121PW_BS00013432914121PW_BS0000283331212PW_BS0000283361121PW_BS00002833217121PW_BS000028350114121PW_BS00002812815121PW_BS0001283511512PW_BS00002835325127PW_BS00002833527121PW_BS0000281151012PW_BS00011513013121PW_BS0001303317121PW_BS0000283344121PW_BS0000283683601PW_BS000028184121PW_BS0000241192171PW_BS00011911PW_BS000001124151PW_BS000124943PW_BS000094388161PW_BS000112109323PW_BS000109122551PW_BS000122406351PW_BS000115407251PW_BS0001153821451PW_BS000100412125PW_BS000115429151PW_BS0001151231751PW_BS00012343311451PW_BS000115408451PW_BS0001154101551PW_BS0001151251351PW_BS000125383751PW_BS000100405105PW_BS0001154222751PW_BS000115435155PW_BS00011539914171PW_BS0001134461217PW_BS0001154641171PW_BS00011544717171PW_BS000115468114171PW_BS0001153744171PW_BS00005344415171PW_BS00011513613171PW_BS0001363987171PW_BS0001133761017PW_BS00005347225177PW_BS00011537527171PW_BS0000534701517PW_BS0001152975101PW_BS0000244793101PW_BS0001152991101PW_BS0000244812101PW_BS00011548414101PW_BS00011548515101PW_BS00011530013101PW_BS0000244957101PW_BS0001154781010PW_BS00011549127101PW_BS0001154991510PW_BS000115501361PW_BS0001153891461PW_BS0001125161561PW_BS0001153951361PW_BS000113390761PW_BS000112209106PW_BS0000245082761PW_BS000115517156PW_BS0001158911421PW_BS0005524824101PW_BS000115502461PW_BS000115171211PW_BS000017111811PW_BS0000116131PW_BS0000061021231PW_BS00010216212181PW_BS000162224241PW_BS0000241951318PW_BS0000242491341PW_BS00002429341PW_BS0000242881441PW_BS0000243841251PW_BS0001004141551PW_BS00011512112171PW_BS00012145015171PW_BS00011548012101PW_BS0001153911261PW_BS0001121861221PW_BS000024185321PW_BS00002415111PW_BS000015471914PW_BS00004731323PW_BS00002430635511PW_BS000024372102PW_BS000028215114PW_BS000021432511PW_BS000043562611PW_BS00005621425181PW_BS00002435625121PW_BS0000284192551PW_BS00011545525171PW_BS00011549025101PW_BS0001155072561PW_BS000115422411PW_BS000042509516PW_BS0000501572241PW_BS00015785241011PW_BS00008522014PW_BS0000242892491PW_BS00002434524121PW_BS00002834695126PW_BS00002832711125PW_BS00002834713125PW_BS0000284182451PW_BS0001154239556PW_BS0001154241155PW_BS0001154251355PW_BS00011545424171PW_BS00011545895176PW_BS00011545911175PW_BS00011546013175PW_BS00011548924101PW_BS0001155062461PW_BS0001159611PW_BS0000092811611PW_BS000028204111PW_BS000020331811PW_BS0000332441011PW_BS000024126651PW_BS00012612711651PW_BS00012715924PW_BS00015921217181PW_BS0000242156181PW_BS0000242863641PW_BS0000242916491PW_BS0000242924491PW_BS00002429817101PW_BS0000243016101PW_BS000024302116101PW_BS0000242941141PW_BS0000241136121PW_BS000113337116121PW_BS00002834141121PW_BS0000281141112PW_BS00011434318121PW_BS000028360410121PW_BS000028409115PW_BS0001154151851PW_BS00011543441051PW_BS0001154436171PW_BS000115448116171PW_BS0001151371117PW_BS00013745118171PW_BS000115469410171PW_BS0001154831110PW_BS000115207661PW_BS000024208116PW_BS0000245041861PW_BS00011551541061PW_BS000115219314PW_BS0000247028511PW_BS0000707132210111PW_BS00051295611211PW_BS000563979132301PW_BS000569129253011PW_BS00058880231PW_BS0005485311015PW_BS0000535511215PW_BS000055414Adenosine 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-N507.181506.995745159FDB0218135'-(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_C000414ATP92214608266164142247813733327995934399763210518211210214649215614216058240559243427272646281229302966316372361661361751439923447431476891486454503289503526515575205975215100525010452911015313111534611253901035406117543011854431205542129555613255691335603135562110858461435854146587610758971475924151604815561091616230166649317868391886870160697619971572057184206720921072252137229211729819873022167390217740821874321637481222749919081862251184727711903170120102811203916412178285125782261269129013264223153273084232631542621322426943187702825377218134772333297746833377632336780373327804135078168128782143517824035378411335784941157885013078865331789193348002836880046184806741198562919482612411323494113282388116280109119914122119992406120154407120245382120362412121246429121392123121397433121471408121974410122065125122079383122083405122402422122444435122919399123009446123816464123951447123956468124029374124527444124616136124630398124634376124943472124972375125011470125304297125371479125392299125515481125595484126123485126220300126234495126240478126547491126596499126913501127123389127731516127781395127796390127801209128119508128167517140770891635Pristanic acidHMDB0000795Pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) is a terpenoid acid present at micromolar concentrations in the plasma of healthy individuals. It is also found in the lipids from many sources such as freshwater sponges, krill, earthworms, whales, human milk fat, bovine depot fat, butterfat or Californian petroleum. It is usually present in combination with phytanic acid. In humans, pristanic acid is obtained from two sources: either directly from the diet or as the alpha oxidation product of phytanic acid. At physiological concentrations pristanic acid is a natural ligand for PPARalpha. In liver, pristanic acid is degraded by peroxisomal beta oxidation to propionyl-CoA. Together with phytanic acid, pristanic acid accumulates in several inherited disorders such as Zellweger syndrome. Pristanic acid is a branched chain fatty acid that arises from the breakdown of phytanic acid. It is present at micromolar concentrations in the plasma of healthy individuals. Pristanic acid is normally degraded by peroxisomal beta-oxidation. In patients affected with generalized peroxisomal disorders, degradation of both phytanic acid and pristanic acid is impaired owing to absence of functional peroxisomes. Pristanic acid has been found to activate the peroxisome proliferator-activated receptor {alpha} (PPAR{alpha}) in a concentration dependent manner.1189-37-312392951340110458CC(C)CCCC(C)CCCC(C)CCCC(C)C(O)=OC19H38O2InChI=1S/C19H38O2/c1-15(2)9-6-10-16(3)11-7-12-17(4)13-8-14-18(5)19(20)21/h15-18H,6-14H2,1-5H3,(H,20,21)PAHGJZDQXIOYTH-UHFFFAOYSA-N298.511298.287180464FDB012993(2s)-pristanic acid;(2s,6r,10r)-2,6,10,14-tetramethylpentadecanoate;(2s,6r,10r)-2,6,10,14-tetramethylpentadecanoic acid;(2s,6r,10r)-pristanic acid;2,6,10,14-tetramethylpentadecanoate;2,6,10,14-tetramethylpentadecanoic acid;Pristanate:(2s,6r,10r)-pristanate;2,6,10,14-tetramethyl-pentadecansaeure;2,6,10,14-tetramethylpentadecylic acid;Acide pristanique;Acido pristanico;Pristaninsaeure;Pristanate;2,6,10,14-tetramethylpentadecylatePW_C000635Pristan1274524065978412335785553341209854081224034221235503741249733751259254821265484911273845021281205081099Coenzyme 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-N767.534767.115208365FDB022614Acetoacetyl 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_C001099CoA21143868845387922892172407592414224595281329286231334211335118461810462958484214486554487965232102524710452801035477124573410857771016023155607516163841646817869301606961162697319970831887108163729319873472107458222822915190812269090224912417092151951301329915318249254884942616315769072937711913377222134772303297729211177550132775553347756311277633336776721297799611578047332780563507841333578567130792593337997433180005368806201188062737480635119806653769382838293834383986742881105553891105613901158423991158473981199514061201474051202313841203051221206344071207621171214061231214214331215211251216664291216824081217144141224044221227411201229041211229601351239654471239794681240791361242204641242654501249743751253414791255094781255794801255924841256342971260844811265494911265604821267463001268845011270462091271093911273012051275402061276673881281215081281335021283403951407511861407631851407678911034Adenosine 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-N427.2011427.029414749FDB021817Adp;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_C001034ADP2341348415224821380159631597831061141518219014921041821131021615824085924352727284727364628552931657236356144002344763147709150362651577520897521710053151115349112539210354461205544129557213356241085741117576410158491435856146587810758991475926151605015561111616231166649517867009468411886872160715920571872067208210722621372312117300198730321673912177410218743316374832228187225118512771190517012013281121802851326222315329308423283154239831342622322426963187702925377087132772161347730632977472333776633367803933278043350781701287821535178244353784143357849511578705331788491307892033480030368806221188065113580676119948271241132833881162041091199441221199944061201564071203183821203664121212484291213941231213994331214724081218993831219764101220641251220854051224054221224454351229733991230134461238184641239534471239584681240303741244523981245294441246151361246363761249474721249753751250124701253342971253734791254922991255174811256454841261254851262193001262354951262424781265504911265974991269155011277335161277803951277973901278032091281225081281685171283133891381Pristanoyl-CoAHMDB0002057(R) Pristanoyl-CoA is converted by alpha-methylacyl-CoA racemase (E.C. 5.1.99.4) (S) pristanoyl-CoA, which is then degraded via peroxisomal beta-oxidation. Deficiency in this enzyme results in neuropathy, hypogonadism of adult onset; and in infant, defective bile acid synthesis has been observed. Pristanoyl-CoA is the substrate of propionyl-CoA C(2)-trimethyltridecanoyltransferase (E.C.2.3.1.154). It is the substrate of peroxisomal pristanoyl-CoA oxidase (E.C.1.3.3.6). A genetic disorder called Zellweger syndrome (OMIM: 214100), also known as neonatal adrenoleukodystrophy, NALD) is the result of a lack of pristanoyl-CoA oxidase, and the subsequent accumulation of phytanic acid and pristanic acid.C07297441253285423-OXOPRISTANOYL-COA390025CC(C)CCCC(C)CCCC(C)CCCC(C)C(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC40H72N7O17P3SInChI=1S/C40H72N7O17P3S/c1-25(2)11-8-12-26(3)13-9-14-27(4)15-10-16-28(5)39(52)68-20-19-42-30(48)17-18-43-37(51)34(50)40(6,7)22-61-67(58,59)64-66(56,57)60-21-29-33(63-65(53,54)55)32(49)38(62-29)47-24-46-31-35(41)44-23-45-36(31)47/h23-29,32-34,38,49-50H,8-22H2,1-7H3,(H,42,48)(H,43,51)(H,56,57)(H,58,59)(H2,41,44,45)(H2,53,54,55)/t26?,27?,28?,29-,32-,33-,34+,38-/m1/s1XYJPSQPVCBNZHT-TUKYSRJDSA-N1048.031047.391826054FDB0228213-oxopristanoyl-coa;3-oxopristanoyl-coenzyme a;Pristanoyl coa;Pristanoyl coenzyme aPW_C001381PrisCoA24095924228242357841511178420334122406122122411408124976135124981374126551297126556482128123205128128502170PyrophosphateHMDB0000250The anion, the salts, and the esters of pyrophosphoric acid are called pyrophosphates. The pyrophosphate anion is abbreviated PPi and is formed by the hydrolysis of ATP into AMP in cells. This hydrolysis is called pyrophosphorolysis. The pyrophosphate anion has the structure P2O74-, and is an acid anhydride of phosphate. It is unstable in aqueous solution and rapidly hydrolyzes into inorganic phosphate. Pyrophosphate is an osteotoxin (arrests bone development) and an arthritogen (promotes arthritis). It is also a metabotoxin (an endogenously produced metabolite that causes adverse health affects at chronically high levels). Chronically high levels of pyrophosphate are associated with hypophosphatasia. Hypophosphatasia (also called deficiency of alkaline phosphatase or phosphoethanolaminuria) is a rare, and sometimes fatal, metabolic bone disease. Hypophosphatasia is associated with a molecular defect in the gene encoding tissue non-specific alkaline phosphatase (TNSALP). TNSALP is an enzyme that is tethered to the outer surface of osteoblasts and chondrocytes. TNSALP hydrolyzes several substances, including inorganic pyrophosphate (PPi) and pyridoxal 5'-phosphate (PLP), a major form of vitamin B6. When TSNALP is low, inorganic pyrophosphate (PPi) accumulates outside of cells and inhibits the formation of hydroxyapatite, one of the main components of bone, causing rickets in infants and children and osteomalacia (soft bones) in adults. Vitamin B6 must be dephosphorylated by TNSALP before it can cross the cell membrane. Vitamin B6 deficiency in the brain impairs synthesis of neurotransmitters which can cause seizures. In some cases, a build-up of calcium pyrophosphate dihydrate crystals in the joints can cause pseudogout.14000-31-8C0001364410218361PPI559142DB04160OP(O)(=O)OP(O)(O)=OH4O7P2InChI=1S/H4O7P2/c1-8(2,3)7-9(4,5)6/h(H2,1,2,3)(H2,4,5,6)XPPKVPWEQAFLFU-UHFFFAOYSA-N177.9751177.943225506FDB021918(4-)diphosphoric acid ion;(p2o74-)diphosphate;Diphosphate;Diphosphoric acid;Ppi;Pyrometaphosphate;Pyrophosphate;Pyrophosphate tetraanion;Pyrophosphate(4-) ion;[o3popo3](4-);Diphosphat;P2o7(4-);Pyrophosphat;Pyrophosphate ion;Phosphonato phosphoric acid;Pyrophosphoric acid;Pyrophosphoric acid ionPW_C000170Ppi122354638429237353288222121731620492410592815294175144868545034895252104529410154091175424103543311854581205548111555913255841335606135565510858791076239166697819970731887134163727216073121987318213827515182832101186916112002222120411641231522512323249125122881257922612695290152193061537518347601742561315426973187723532977317128776353367841633578928331791531127995013479958130800473728041717085630194786384948141259481938298678223110634391113270395113275389115527136115532399119934122120017124120032406120330410120936407121261429121341121121486383122407422122985444123502119123831464124044398124977375125324297125395299125410479125597484125656485125876481126552491126869205126935388126950501127337206128124508140772891423MagnesiumHMDB0000547Magnesium 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-N24.30523.985041898FDB003518Magnesium;Magnesium ions;Magnesium ion;Magnesium, doubly charged positive ion;Magnesium, ion (mg(2+));Mg(2+);Mg2+PW_C000423Mg2+868227426816476272726811581918883229363998339922111674614834915294317641421241024115929422331262933737454031477491486954497456525310453291115356112537610359061475934151603815560941616250166648417865941646881160697919971702057194206722721372332117250214731021673131987473222117631321184321012312225123242491251328812581226127292901527528515337308771371337723632977937336783933347841733578489115785223317853635678574130800203688004518480048372806231188065413580865158096525381841519383238394900271085962231105593901156873981199744061200701221202473821207024071209814081211811241212654291213194191219241251220864051224084221227591201229213991233071191235463741238354641238894551244771361246373761249783751254472971255984841256694791257774811259214821259472991259734951260004901262434781265534911267533001271253891271645011273805021274073881274515071278042091281255081283473951407738911309PristanalHMDB0001958Pristanal belongs to the class of organic compounds known as acyclic diterpenoids. These are diterpenoids (compounds made of four consecutive isoprene units) that do not contain a cycle. Thus, pristanal is considered to be an isoprenoid lipid molecule. Pristanal is considered to be a practically insoluble (in water) and relatively neutral molecule. Within the cell, pristanal is primarily located in the cytoplasm, membrane (predicted from logP) and peroxisome. In humans, pristanal is involved in the oxidation OF branched chain fatty acids pathway and the phytanic Acid peroxisomal oxidation pathway. Pristanal is also involved in the metabolic disorder called the refsum disease pathway. Intermediate in the metabolism of phytanic acid and pristanic acid.105373-75-9146710604918913628282CC(C)CCCC(C)CCCC(C)CCCC(C)C=OC19H38OInChI=1S/C19H38O/c1-16(2)9-6-10-17(3)11-7-12-18(4)13-8-14-19(5)15-20/h15-19H,6-14H2,1-5H3IZJRIIWUSIGEAJ-UHFFFAOYSA-N282.5044282.292265838FDB022765(2r)-pristanal;(2r,6r,10r)-2,6,10,14-tetramethylpentadecanal;(2s)-pristanal;(2s,6r,10r)-2,6,10,14-tetramethylpentadecanal;2(r)-pristanal;2(s)-pristanal;2rpr-al (2r,6r,10r,14)-tetramethylpentadecanal;2spr-alPW_C001309Pristal126952424378419112785543341209794081224104071235443741249801191259194821265554811273785021281272069992-Hydroxyphytanoyl-CoAHMDB00012952-Hydroxyphytanoyl-CoA belongs to the class of organic compounds known as long-chain fatty acyl coas. These are acyl CoAs where the group acylated to the coenzyme A moiety is a long aliphatic chain of 13 to 21 carbon atoms. Thus, 2-hydroxyphytanoyl-CoA is considered to be a fatty ester lipid molecule. 2-Hydroxyphytanoyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). 2-Hydroxyphytanoyl-CoA has been primarily detected in urine. Within the cell, 2-hydroxyphytanoyl-CoA is primarily located in the peroxisome, cytoplasm and mitochondria. In humans, 2-hydroxyphytanoyl-CoA is involved in the oxidation OF branched chain fatty acids pathway and the phytanic Acid peroxisomal oxidation pathway. 2-Hydroxyphytanoyl-CoA is also involved in the metabolic disorder called the refsum disease pathway. 2-Hydroxyphytanoyl-CoA is a substrate for Phytanoyl-CoA dioxygenase (peroxisomal).172787-73-4C07343441263154752-HYDROXYPHYTANOYL-COA390032CC(C)CCC[C@@H](C)CCC[C@@H](C)CCC[C@H](C)C(O)C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC41H74N7O18P3SInChI=1S/C41H74N7O18P3S/c1-25(2)11-8-12-26(3)13-9-14-27(4)15-10-16-28(5)32(50)40(54)70-20-19-43-30(49)17-18-44-38(53)35(52)41(6,7)22-63-69(60,61)66-68(58,59)62-21-29-34(65-67(55,56)57)33(51)39(64-29)48-24-47-31-36(42)45-23-46-37(31)48/h23-29,32-35,39,50-52H,8-22H2,1-7H3,(H,43,49)(H,44,53)(H,58,59)(H,60,61)(H2,42,45,46)(H2,55,56,57)/t26-,27-,28+,29-,32?,33-,34-,35?,39-/m1/s1WNVFJMYPVBOLKV-PJDIVXIPSA-N1078.0491077.402388825FDB022539(3s)-2-hydroxyphytanoyl-coa;(3s)-2-hydroxyphytanoyl-coenzyme a;(3s,7r,11r)-2-hydroxy-3,7,11,15-tetramethylhexadecanoyl-coa;(3s,7r,11r)-2-hydroxy-3,7,11,15-tetramethylhexadecanoyl-coenzyme a;2-hydroxyphytanoyl-coa;2-hydroxyphytanoyl-coenzyme a;3(s)-2-hydroxyphytanoyl-coa;3(s)-2-hydroxyphytanoyl-coenzyme a;3s2hphy-coa;3s2hphy-coenzyme aPW_C0009992HPCoA12645784213341209784081235433741259184821273775021883Formyl-CoAHMDB0003419Formyl-CoA is formed during the alpha-oxidation process in liver peroxisomes, as a result of the alpha-oxidation of 3-methyl-substituted fatty acids. The amount of formyl-CoA formed constitutes 2 - 5% of the total formate. The formyl-CoA formed is not due to activation of formate - until now presumed to be the primary end-product of alpha-oxidation - but is rather than formate the end-product of alpha-oxidation. The cleavage of 2-hydroxy-3-methylhexadecanoyl-CoA to 2-methylpentadecanal and formate (formyl-CoA) is probably due to the presence of a specific lyase. (PMID: 9276483, 9166898).13131-49-2C0079843931315522FORMYL-COA388444CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)C(O)C(=O)NCCC(=O)NCCSC=OC22H36N7O17P3SInChI=1S/C22H36N7O17P3S/c1-22(2,17(33)20(34)25-4-3-13(31)24-5-6-50-11-30)8-43-49(40,41)46-48(38,39)42-7-12-16(45-47(35,36)37)15(32)21(44-12)29-10-28-14-18(23)26-9-27-19(14)29/h9-12,15-17,21,32-33H,3-8H2,1-2H3,(H,24,31)(H,25,34)(H,38,39)(H,40,41)(H2,23,26,27)(H2,35,36,37)/t12-,15-,16-,17?,21-/m1/s1SXMOKYXNAPLNCW-BWGWEBPHSA-N795.544795.110122987FDB023169Formyl coenzyme aPW_C001883FormCoA12705784223341209804081235453741259204821273795021060Thiamine pyrophosphateHMDB0001372Thiamine pyrophosphate is the active form of thiamine, and it serves as a cofactor for several enzymes involved primarily in carbohydrate catabolism. The enzymes are important in the biosynthesis of a number of cell constituents, including neurotransmitters, and for the production of reducing equivalents used in oxidant stress defenses and in biosyntheses and for synthesis of pentoses used as nucleic acid precursors. The chemical structure of TPP is that of an aromatic methylaminopyrimidine ring, linked via a methylene bridge to a methylthiazolium ring with a pyrophosphate group attached to a hydroxyethyl side chain. In non-enzymatic model studies it has been demonstrated that the thiazolium ring can catalyse reactions which are similar to those of TPP-dependent enzymes but several orders of magnitude slower. Using infrared and NMR spectrophotometry it has been shown that the dissociation of the proton from C2 of the thiazolium ring is necessary for catalysis; the abstraction of the proton leads to the formation of a carbanion (ylid) with the potential for a nucleophilic attack on the carbonyl group of the substrate. In all TPP-dependent enzymes the abstraction of the proton from the C2 atom is the first step in catalysis, which is followed by a nucleophilic attack of this carbanion on the substrate. Subsequent cleavage of a C-C bond releases the first product with formation of a second carbanion (2-greek small letter alpha-carbanion or enamine). The formation of this 2-greek small letter alpha-carbanion is the second feature of TPP catalysis common to all TPP-dependent enzymes. Depending on the enzyme and the substrate(s), the reaction intermediates and products differ. Methyl-branched fatty acids, as phytanic acid, undergo peroxisomal beta-oxidation in which they are shortened by 1 carbon atom. This process includes four steps: activation, 2-hydroxylation, thiamine pyrophosphate dependent cleavage and aldehyde dehydrogenation. In the third step, 2-hydroxy-3-methylacyl-CoA is cleaved in the peroxisomal matrix by 2-hydroxyphytanoyl-CoA lyase (2-HPCL), which uses thiamine pyrophosphate (TPP) as cofactor. The thiamine pyrophosphate dependence of the third step is unique in peroxisomal mammalian enzymology. Human pathology due to a deficient alpha-oxidation is mostly linked to mutations in the gene coding for the second enzyme of the sequence, phytanoyl-CoA hydroxylase (EC 1.14.11.18). (PMID: 12694175, 11899071, 9924800).154-87-0C00068113295322-(alpha-lactyl)-thpp1100CC1=C(CCOP(O)(=O)OP(O)(O)=O)SC=[N+]1CC1=CN=C(C)N=C1NC12H19N4O7P2SInChI=1S/C12H18N4O7P2S/c1-8-11(3-4-22-25(20,21)23-24(17,18)19)26-7-16(8)6-10-5-14-9(2)15-12(10)13/h5,7H,3-4,6H2,1-2H3,(H4-,13,14,15,17,18,19,20,21)/p+1AYEKOFBPNLCAJY-UHFFFAOYSA-O425.314425.044967696FDB022584Tpp;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_C001060ThiamPP2054107531197812715173625366103602815560801616388164731787463222128062257712413378285112784233347901811179175132800103681199564061208024071209021221209824081215371241227461201233881191234731351235473741240951181253464791259224821260944811268022991268895011273815021275492061284003881049Phytanoyl-CoAHMDB0001359Phytanoyl CoA is a coenzyme A derivative of phytanic acid. Phytanic acid is present in human diet or in animal tissues where it may be derived from chlorophyll in plant extracts. Specifically it is an epimeric metabolite of the isoprenoid side chain of chlorophyll. Owing to the presence of its epimeric beta-methyl group, phytanic acid cannot be metabolized by beta-oxidation. Instead, it is metabolized in peroxisomes via alpha-oxidation to give pristanic acid, which is then oxidized by beta-oxidation. PhyH (phytanoyl-CoA 2-hydroxylase) catalyses hydroxylation of phytanoyl-CoA. Mutations of PhyH can lead to phytanic acid accumulation. High levels of phytanic acid are found in patients suffering from Refsum's syndrome. This inherited neurological disorder is characterized by an accumulation of phytanic acid in blood and tissues. Clinically it is characterized by adult onset retinitis pigmentosa, anosmia, sensory neuropathy, and phytanic acidaemia. This disorder has been found to be related to deficiency in the α-oxidation pathway in the liver. (PMID: 17956235). Phytanoyl CoA and other branched-chain fatty acid CoA products are potent inducers of the peroxisome proliferator-activated receptor PPARalpha, a nuclear receptor that enhances transcription of peroxisomal enzymes mediating beta-oxidation of these potentially toxic fatty acids (PMID: 16768463). Pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase are strongly inhibited by phytanoyl-CoA. Decreased activity of these important mitochondrial metabolism complexes might therefore contribute to neurological symptoms upon accumulation of phytanic acid in Refsum disease (PMID: 16737698).146622-45-9C0206043964015538CPD-206388712CC(C)CCC[C@@H](C)CCC[C@@H](C)CCC[C@H](C)CC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC41H74N7O17P3SInChI=1S/C41H74N7O17P3S/c1-26(2)11-8-12-27(3)13-9-14-28(4)15-10-16-29(5)21-32(50)69-20-19-43-31(49)17-18-44-39(53)36(52)41(6,7)23-62-68(59,60)65-67(57,58)61-22-30-35(64-66(54,55)56)34(51)40(63-30)48-25-47-33-37(42)45-24-46-38(33)48/h24-30,34-36,40,51-52H,8-23H2,1-7H3,(H,43,49)(H,44,53)(H,57,58)(H,59,60)(H2,42,45,46)(H2,54,55,56)/t27-,28-,29+,30-,34-,35-,36?,40-/m1/s1NRJQGHHZMSOUEN-IYJVDCLDSA-N1062.0491061.407474203FDB0225773,7,11,15-tetramethyl hexadecanoyl coa;3,7,11,15-tetramethyl hexadecanoyl coenzyme a;Phytanoyl coa;Phytanoyl coenzyme a;Phytanoyl-coa;Phytanoyl-coenzyme a;Phytanyl coa;Phytanyl coenzyme aPW_C001049PhytCoA126182428524842784243347843513278553111120976122120988408122420124123541135123553374124988118125916297125928482126567299127375205127387502128140388134Oxoglutaric 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-N146.0981146.021523302FDB0033612-ketoglutarate;2-ketoglutaric acid;2-oxo-1,5-pentanedioate;2-oxo-1,5-pentanedioic acid;2-oxoglutarate;2-oxoglutaric acid;2-oxopentanedioate;2-oxopentanedioic acid;Oxoglutarate;Alpha-ketoglutaric acid;Oxoglutaric acid;A-ketoglutarate;A-ketoglutaric acid;Alpha-ketoglutarate;α-ketoglutarate;α-ketoglutaric acidPW_C000134AKG15242314141468499186733111084212635144750145526146754537510354141175438118556413260081476036155606915760921616482178653085747122275152247519151820922583742201186319812681289770542537713513377481111775231127774612977967345779703467797632777984347784253348001836880694135113162941199724061200221241200844071201741221205524141208144181209894081211464231211524241211604251227571201228311191231864501233994541235543741237184581237244591237324601253574791254002991254554811255332971258004891259294821269005011269403881269932061270662051272555061273885021065OxygenHMDB0001377Oxygen is the third most abundant element in the universe after hydrogen and helium and the most abundant element by mass in the Earth's crust. Diatomic oxygen gas constitutes 20.9% of the volume of air. All major classes of structural molecules in living organisms, such as proteins, carbohydrates, and fats, contain oxygen, as do the major inorganic compounds that comprise animal shells, teeth, and bone. Oxygen in the form of O2 is produced from water by cyanobacteria, algae and plants during photosynthesis and is used in cellular respiration for all living organisms. Green algae and cyanobacteria in marine environments provide about 70% of the free oxygen produced on earth and the rest is produced by terrestrial plants. Oxygen is used in mitochondria to help generate adenosine triphosphate (ATP) during oxidative phosphorylation. For animals, a constant supply of oxygen is indispensable for cardiac viability and function. To meet this demand, an adult human, at rest, inhales 1.8 to 2.4 grams of oxygen per minute. This amounts to more than 6 billion tonnes of oxygen inhaled by humanity per year. At a resting pulse rate, the heart consumes approximately 8-15 ml O2/min/100 g tissue. This is significantly more than that consumed by the brain (approximately 3 ml O2/min/100 g tissue) and can increase to more than 70 ml O2/min/100 g myocardial tissue during vigorous exercise. As a general rule, mammalian heart muscle cannot produce enough energy under anaerobic conditions to maintain essential cellular processes; thus, a constant supply of oxygen is indispensable to sustain cardiac function and viability. However, the role of oxygen and oxygen-associated processes in living systems is complex, and they and can be either beneficial or contribute to cardiac dysfunction and death (through reactive oxygen species). Reactive oxygen species (ROS) are a family of oxygen-derived free radicals that are produced in mammalian cells under normal and pathologic conditions. Many ROS, such as the superoxide anion (O2-)and hydrogen peroxide (H2O2), act within blood vessels, altering mechanisms mediating mechanical signal transduction and autoregulation of cerebral blood flow. Reactive oxygen species are believed to be involved in cellular signaling in blood vessels in both normal and pathologic states. The major pathway for the production of ROS is by way of the one-electron reduction of molecular oxygen to form an oxygen radical, the superoxide anion (O2-). Within the vasculature there are several enzymatic sources of O2-, including xanthine oxidase, the mitochondrial electron transport chain, and nitric oxide (NO) synthases. Studies in recent years, however, suggest that the major contributor to O2- levels in vascular cells is the membrane-bound enzyme NADPH-oxidase. Produced O2- can react with other radicals, such as NO, or spontaneously dismutate to produce hydrogen peroxide (H2O2). In cells, the latter reaction is an important pathway for normal O2- breakdown and is usually catalyzed by the enzyme superoxide dismutase (SOD). Once formed, H2O2 can undergo various reactions, both enzymatic and nonenzymatic. The antioxidant enzymes catalase and glutathione peroxidase act to limit ROS accumulation within cells by breaking down H2O2 to H2O. Metabolism of H2O2 can also produce other, more damaging ROS. For example, the endogenous enzyme myeloperoxidase uses H2O2 as a substrate to form the highly reactive compound hypochlorous acid. Alternatively, H2O2 can undergo Fenton or Haber-Weiss chemistry, reacting with Fe2+/Fe3+ ions to form toxic hydroxyl radicals (-.OH). (PMID: 17027622, 15765131).7782-44-7C0000797715379CPD-6641952O=OO2InChI=1S/O2/c1-2MYMOFIZGZYHOMD-UHFFFAOYSA-N31.998831.989829244FDB022589Dioxygen;Molecular oxygen;O2;Oxygen;Oxygen molecule;[oo];Dioxygene;Disauerstoff;E 948;E-948;E948PW_C001065O295911052451650018505854914625286383649106743168820754157634769338362137549201624253122280329426042474713546712354801255493126550812758091085973147612915970061887032163705016073192137533210756021283951511181621611864198118832151189421112057225120631641224728612279226123252491270629112716292130042981301630013026301130383021326022342276174265731576910293770442947721413477350111773631307737733177395332774971137751211577537334776263367772333777736112777471297775634177805114778121337807032978151132783813457880534379111360120047408120383122120426405120542407120553414120594409120601406120883415121045124121104383121605434121656429122117382122573418122689384122798374122822443123027135123060376123128447123139136123163448123176119123187450123219137123226120123459451123609118123669398124163469124214464124669399125145454125275121125425482125706478125731483125737297125740479125884481126100299126272484126522495126721489126825480126964502126986207127198209127214208127219205127222501127305504127345206127557388127574515127835389128081395128095390128312506128432391174Succinic 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-N118.088118.02660868FDB0019311,2-ethanedicarboxylate;1,2-ethanedicarboxylic acid;1,4-butanedioate;1,4-butanedioic acid;Amber acid;Asuccin;Dihydrofumarate;Dihydrofumaric acid;Katasuccin;Succinate;Wormwood acid;Acide butanedioique;Acide succinique;Acidum succinicum;Bernsteinsaeure;Butandisaeure;Butanedionic acid;E363;Ethylenesuccinic acid;Hooc-ch2-ch2-cooh;Spirit of amber;Butanedionate;EthylenesuccinatePW_C000174Succini152323945021850786763112655425517538310360421556102161645410764551086489178676411768361667362163745521974562207477222118661981214215113259223423683184236931542402322771431337721313477483111777381127774912978426334800243688072111911284630811342811199844061201924071203851221205554141209904081225653841227671201230291351231894501235553741251381211253644791255494811259304821267134801269065011270822061273895021283043911316Carbon dioxideHMDB0001967Carbon dioxide is a colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. Carbon dioxide is produced during respiration by all animals, fungi and microorganisms that depend on living and decaying plants for food, either directly or indirectly. It is, therefore, a major component of the carbon cycle. Additionally, carbon dioxide is used by plants during photosynthesis to make sugars which may either be consumed again in respiration or used as the raw material to produce polysaccharides such as starch and cellulose, proteins and the wide variety of other organic compounds required for plant growth and development. When inhaled at concentrations much higher than usual atmospheric levels, it can produce a sour taste in the mouth and a stinging sensation in the nose and throat. These effects result from the gas dissolving in the mucous membranes and saliva, forming a weak solution of carbonic acid. Carbon dioxide is used by the food industry, the oil industry, and the chemical industry. Carbon dioxide is used to produce carbonated soft drinks and soda water. Traditionally, the carbonation in beer and sparkling wine comes about through natural fermentation, but some manufacturers carbonate these drinks artificially.124-38-9C0001128016526274O=C=OCO2InChI=1S/CO2/c2-1-3CURLTUGMZLYLDI-UHFFFAOYSA-N44.009543.989829244DBMET00423FDB014084Carbon oxide;Carbon-12 dioxide;Carbonic acid anhydride;Carbonic acid gas;Carbonic anhydride;[co2];Co2;E 290;E-290;E290;R-744PW_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905021407981859794IronHMDB0015531Iron is a metallic element found in certain minerals, in nearly all soils, and in mineral waters. Iron is required for life. It exists in all living species, ranging from bacteria to humans. It can be found primarily in blood and it is an essential constituent of hemoglobin, cytochrome, and other components of respiratory enzyme systems. Its chief functions are in the transport of oxygen to tissue (hemoglobin) and in cellular oxidation mechanisms. Depletion of iron stores may result in iron-deficiency anemia. Iron is used to build up the blood in anemia. In humans, iron is involved in several metabolic pathways, some of which include the rofecoxib pathway, magnesium salicylate action pathway, etodolac pathway, and diclofenac pathway. Iron is also involved in several metabolic disorders, some of which include adenine phosphoribosyltransferase deficiency (APRT), porphyria variegata (PV), adenylosuccinate lyase deficiency, and AICA-ribosiduria. The major activity of supplemental iron is in the prevention and treatment of iron-deficiency anemia. Iron has putative immune-enhancing, anticarcinogenic, and cognition-enhancing activities. Iron can be found in a number of food items such as chinese water chestnut, hyssop, daikon radish, and peppermint, which makes it a potential biomarker for the consumption of these food products.7439-89-6C00023239251824822368DB01592[Fe++]FeInChI=1S/Fe/q+2CWYNVVGOOAEACU-UHFFFAOYSA-N55.84555.934942133C0002326fe;Eisen;Fe;Fer;Ferrum;HierroPW_C009794Iron11388126651533216354931192943411873302131205622577683130779283367826113278409111784283347893833112083812212099240812125642912137112412150238312172712512342313512355737412382646412393011812406039812427813612582929712593248212604429912728220512739150212749638831Ascorbic acidHMDB0000044Ascorbic acid is found naturally in citrus fruits and many vegetables and is an essential nutrient in human diets. It is necessary to maintain connective tissue and bone. The biologically active form of ascorbic acid is vitamin C. Vitamin C is a water soluble vitamin. Primates (including humans) and a few other species in all divisions of the animal kingdom, notably the guinea pig, have lost the ability to synthesize ascorbic acid and must obtain it in their food. Vitamin C functions as a reducing agent and coenzyme in several metabolic pathways. Vitamin C is considered an antioxidant. [PubChem] Ascorbic acid is an electron donor for enzymes involved in collagen hydroxylation, biosynthesis of carnitine and norepinephrine, tyrosine metabolism, and amidation of peptide hormones. Ascrobic acid (vitamin C) deficiency causes scurvy. The amount of vitamin C necessary to prevent scurvy may not be adequate to maintain optimal health. The ability of vitamin C to donate electrons also makes it a potent water-soluble antioxidant that readily scavenges free radicals such as molecular oxygen, superoxide, hydroxyl radical, and hypochlorous acid. In this setting, several mechanisms could account for a link between vitamin C and heart disease. One is the relation between LDL oxidation and vitamins C and E. Vitamin C in vitro can recycle vitamin E, which can donate electrons to prevent LDL oxidation in vitro. As the lipid-phase vitamin E is oxidized, it can be regenerated by aqueous vitamin C. Other possibilities are that vitamin C could decrease cholesterol by mechanisms not well characterized, or could improve vasodilatation and vascular reactivity, perhaps by decreasing the interactions of nitric oxide with oxidants. (PMID: 10799361).50-81-7C000725467006729073ASCORBATE10189562DB00126[H][C@@]1(OC(=O)C(O)=C1O)[C@@H](O)COC6H8O6InChI=1S/C6H8O6/c7-1-2(8)5-3(9)4(10)6(11)12-5/h2,5,7-10H,1H2/t2-,5+/m0/s1CIWBSHSKHKDKBQ-JLAZNSOCSA-N176.1241176.032087988FDB001224(+)-sodium l-ascorbate;(+)-ascorbate;(+)-ascorbic acid;3-keto-l-gulofuranolactone;3-oxo-l-gulofuranolactone;Adenex;Allercorb;Antiscorbic vitamin;Antiscorbutic vitamin;Arco-cee;Ascoltin;Ascor-b.i.d.;Ascorb;Ascorbajen;Ascorbate;Ascorbic acid;Ascorbicab;Ascorbicap;Ascorbicin;Ascorbin;Ascorbutina;Ascorin;Ascorteal;Ascorvit;C-level;C-long;C-quin;C-span;C-vimin;Cantan;Cantaxin;Catavin c;Ce lent;Ce-mi-lin;Ce-vi-sol;Cebicure;Cebid;Cebion;Cebione;Cecon;Cee-caps td;Cee-vite;Cegiolan;Ceglion;Ceklin;Celaskon;Celin;Cell c;Cemagyl;Cemill;Cenetone;Cenolate;Cereon;Cergona;Cescorbat;Cetamid;Cetane;Cetane-caps tc;Cetane-caps td;Cetebe;Cetemican;Cevalin;Cevatine;Cevex;Cevi-bid;Cevimin;Cevital;Cevitamate;Cevitamic acid;Cevitamin;Cevitan;Cevitex;Cewin;Chewcee;Ciamin;Cipca;Citriscorb;Citrovit;Colascor;Concemin;Davitamon c;Dora-c-500;Duoscorb;Ferrous ascorbate;Hicee;Hybrin;Ido-c;Juvamine;Kangbingfeng;Kyselina askorbova;L(+)-ascorbate;L(+)-ascorbic acid;L-(+)-ascorbate;L-(+)-ascorbic acid;L-3-ketothreohexuronic acid lactone;L-ascorbate;L-ascorbic acid;L-lyxoascorbate;L-lyxoascorbic acid;L-threo-ascorbic acid;L-xyloascorbate;L-xyloascorbic acid;Laroscorbine;Lemascorb;Liqui-cee;Meri-c;Natrascorb;Natrascorb injectable;Planavit c;Proscorbin;Redoxon;Ribena;Ronotec 100;Rontex 100;Roscorbic;Rovimix c;Scorbacid;Scorbu c;Scorbu-c;Secorbate;Sodascorbate;Suncoat vc 40;Testascorbic;Vasc;Vicelat;Vicin;Vicomin c;Viforcit;Viscorin;Viscorin 100m;Vitace;Vitacee;Vitacimin;Vitacin;Vitamin c;Vitamisin;Vitascorbol;Xitix;Gamma-lactone l-threo-hex-2-enonate;Gamma-lactone l-threo-hex-2-enonic acid;Acide ascorbique;Acido ascorbico;Acidum ascorbicum;Acidum ascorbinicum;Ascorbinsaeure;E 300;E-300;E300PW_C000031VitC67931698201267520211462521071214615112297225134022224250631877752129777663417842933479116115115855336120558414120993408121610405123192450123558374124168376125933482127392502127579209988Propionyl-CoAHMDB0001275Propionyl-CoA is an intermediate in the metabolism of propanoate. Propionic aciduria is caused by an autosomal recessive disorder of propionyl coenzyme A (CoA) carboxylase deficiency (EC 6.4.1.3). In propionic aciduria, propionyl CoA accumulates within the mitochondria in massive quantities; free carnitine is then esterified, creating propionyl carnitine, which is then excreted in the urine. Because the supply of carnitine in the diet and from synthesis is limited, such patients readily develop carnitine deficiency as a result of the increased loss of acylcarnitine derivatives. This condition demands supplementation of free carnitine above the normal dietary intake to continue to remove (detoxify) the accumulating organic acids. Propionyl-CoA is a substrate for Acyl-CoA dehydrogenase (medium-chain specific, mitochondrial), Acetyl-coenzyme A synthetase 2-like (mitochondrial), Propionyl-CoA carboxylase alpha chain (mitochondrial), Methylmalonate-semialdehyde dehydrogenase (mitochondrial), Trifunctional enzyme beta subunit (mitochondrial), 3-ketoacyl-CoA thiolase (peroxisomal), Acyl-CoA dehydrogenase (long-chain specific, mitochondrial), Malonyl-CoA decarboxylase (mitochondrial), Acetyl-coenzyme A synthetase (cytoplasmic), 3-ketoacyl-CoA thiolase (mitochondrial) and Propionyl-CoA carboxylase beta chain (mitochondrial). (PMID: 10650319).317-66-8C0010043916415539PROPIONYL-COA388310CCC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC24H40N7O17P3SInChI=1S/C24H40N7O17P3S/c1-4-15(33)52-8-7-26-14(32)5-6-27-22(36)19(35)24(2,3)10-45-51(42,43)48-50(40,41)44-9-13-18(47-49(37,38)39)17(34)23(46-13)31-12-30-16-20(25)28-11-29-21(16)31/h11-13,17-19,23,34-35H,4-10H2,1-3H3,(H,26,32)(H,27,36)(H,40,41)(H,42,43)(H2,25,28,29)(H2,37,38,39)/t13-,17-,18-,19?,23-/m1/s1QAQREVBBADEHPA-UXYNFSPESA-N823.597823.141423115FDB0225292-methylacetyl-coa;2-methylacetyl-coenzyme a;Propanoyl-coa;Propanoyl-coenzyme a;Propionyl-coa;Propionyl-coenzyme a;Alpha-methylacetyl-coa;Alpha-methylacetyl-coenzyme aPW_C000988PropCoA12778169432285424455491413909122477641334784361127855611178636133120995122121576407121681408122266406123560135124133119124231374124819120125935297126430479126557482126568481127395205127997501128130502128141206940Acetyl-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-N809.571809.125773051FDB022491Ac-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-CoA213438588423241622446528961733401148401452781035476124573310860251556077161638616470178692316071061637291198746022282451518277210125822261301229942615315771211337729111177562112777061327799411578355134784333348000736880634119806633769012417011995340612014540512030412212063240712241740812262638412274312012295913512313711812498637412520012112534347912550747812563329712656448212657248112677848012688650112704420912739420512766538812813750212814520612837439114076218571984,8-Dimethylnonanoyl-CoAHMDB00116044,8-dimethylnonanoyl-CoA is produced when both phytanic acid and pristanic acid are oxidized in peroxisomes. It is converted to the corresponding acylcarnitine (presumably by peroxisomal carnitine octanoyltransferase), and exported to the mitochondrion. (PMID: 9469587).204120-61-653481003CC(C)CCCC(C)CCC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC32H56N7O17P3SInChI=1S/C32H56N7O17P3S/c1-19(2)7-6-8-20(3)9-10-23(41)60-14-13-34-22(40)11-12-35-30(44)27(43)32(4,5)16-53-59(50,51)56-58(48,49)52-15-21-26(55-57(45,46)47)25(42)31(54-21)39-18-38-24-28(33)36-17-37-29(24)39/h17-21,25-27,31,42-43H,6-16H2,1-5H3,(H,34,40)(H,35,44)(H,48,49)(H,50,51)(H2,33,36,37)(H2,45,46,47)/t20?,21-,25-,26-,27?,31-/m1/s1YGNKJFPEXQCWDB-DPSOZLMZSA-N935.81935.2666236274(r)-8-dimethyl-nonanoyl-coa;4(r)-8-dimethyl-nonanoyl-coenzyme a;S-(4,8-dimethylnonanoate;S-(4,8-dimethylnonanoate)-coa;S-(4,8-dimethylnonanoate)-coenzyme a;S-(4,8-dimethylnonanoic acidPW_C00719848DmnCA2447525213784313347843811212241540812242540712498437412499111912656248212657448112813550212814720644L-CarnitineHMDB0000062Carnitine is not an essential amino acid; it can be synthesized in the body. However, it is so important in providing energy to muscles (including the heart) that some researchers are now recommending carnitine supplements in the diet, particularly for people who do not consume much red meat (the main food source for carnitine). Carnitine has been described as a vitamin, an amino acid, or a metabimin (i.e. an essential metabolite). Like the B vitamins, carnitine contains nitrogen and is very soluble in water, and to some researchers carnitine is a vitamin (Liebovitz 1984). It was found that an animal (yellow mealworm) could not grow without carnitine in its diet. However, as it turned out, almost all other animals, including humans, do make their own carnitine; thus, it is no longer considered a vitamin. Nevertheless, in certain circumstances, such as deficiencies of methionine, lysine, or vitamin C or kidney dialysis, carnitine shortages develop. Under these conditions, carnitine must be absorbed from food, and for this reason it is sometimes referred to as a "metabimin" or a conditionally essential metabolite. Like the other amino acids used or manufactured by the body, carnitine is an amine. But like choline, which is sometimes considered to be a B vitamin, carnitine is also an alcohol (specifically, a trimethylated carboxy-alcohol). Thus, carnitine is an unusual amino acid and has different functions than most other amino acids, which are usually employed by the body in the construction of protein. Carnitine is an essential factor in fatty acid metabolism in mammals. Its most important known metabolic function is to transport fat into the mitochondria of muscle cells, including those in the heart, for oxidation. This is how the heart gets most of its energy. In humans, about 25% of carnitine is synthesized in the liver, kidney, and brain from the amino acids lysine and methionine. Most of the carnitine in the body comes from dietary sources such as red meat and dairy products. Inborn errors of carnitine metabolism can lead to brain deterioration like that of Reye's syndrome, gradually worsening muscle weakness, Duchenne-like muscular dystrophy, and extreme muscle weakness with fat accumulation in muscles. Borum et al. (1979) describe carnitine as an essential nutrient for pre-term babies and individuals who are unable to eat a normal diet (e.g. non-ketotic hypoglycemics, kidney dialysis patients) (PMID: 115309). In conditions such as kwashiorkor, cirrhosis, and heart muscle disease (cardiomyopathy) as well as in inborn errors of metabolism such as type IV hyperlipidemia and propionic or organic aciduria (acid urine resulting from genetic or other anomalies), carnitine is essential to life and carnitine supplements are valuable. Carnitine therapy may also be useful in a wide variety of clinical conditions. Carnitine supplementation has improved some patients who have angina secondary to coronary artery disease. It may also be worth a trial for patients with any form of hyperlipidemia or muscle weakness. Carnitine supplements may also be useful in many forms of toxic or metabolic liver disease and in cases of heart muscle disease. Hearts undergoing severe arrhythmia quickly deplete their stores of carnitine. Athletes, particularly in Europe, have used carnitine supplements for improved endurance. Carnitine may improve muscle building by improving fat utilization and may even be useful in treating obesity. Carnitine joins a long list of nutrients which may be of value in treating pregnant women, hypothyroid individuals, and male infertility due to the low motility of sperm. Carnitine deficiency is noted in abnormal liver function, renal dialysis patients, and severe to moderate muscular weakness with associated anorexia (http://www.dcnutrition.com). Carnitine is a biomarker for the consumption of meat.541-15-1C003181091716347CARNITINE10455DB00583C[N+](C)(C)C[C@H](O)CC([O-])=OC7H15NO3InChI=1S/C7H15NO3/c1-8(2,3)5-6(9)4-7(10)11/h6,9H,4-5H2,1-3H3/t6-/m1/s1PHIQHXFUZVPYII-ZCFIWIBFSA-N161.1989161.105193351FDB000572(-)-(r)-3-hydroxy-4-(trimethylammonio)butyrate;(-)-carnitine;(r)-(3-carboxy-2-hydroxypropyl)trimethylammonium hydroxide;(r)-carnitine;(s)-carnitine;1-carnitine;3-carboxy-2-hydroxy-n,n,n-trimethyl-1-propanaminium;3-hydroxy-4-trimethylammoniobutanoate;3-hydroxy-4-trimethylammoniobutanoic acid;Bicarnesine;Carniking;Carniking 50;Carnilean;Carnipass;Carnipass 20;Carnitene;Carnitine;Carnitor;D-carnitine;Dl-carnitine;Karnitin;L-(-)-carnitine;L-carnitine;L-gamma-trimethyl-beta-hydroxybutyrobetaine;Levocarnitina;Levocarnitine;Levocarnitinum;R-(-)-3-hydroxy-4-trimethylaminobutyrate;Vitamin bt;Delta-carnitine;Gamma-trimethyl-ammonium-beta-hydroxybutirate;Gamma-trimethyl-beta-hydroxybutyrobetaine;Gamma-trimethyl-hydroxybutyrobetaine;(-)-l-carnitine;3-carboxy-2-hydroxy-n,n,n-trimethyl-1-propanaminium hydroxide, inner salt;CarnicorPW_C000044L-Carnt67531885228911724575251932675228858288745230102524510469591626971199772201347722832977558334775611127756713277748129783391117834513379258333806303748063311980639118120229384120241382120554414122412408122421407122522124122617122122621406122902121122915399123188450125191135125195120125577480125590484126558482126569481126675299126769297126773479127107391127119389128131502128142206128255388128364205128368501140749186140765891658PropionylcarnitineHMDB0000824Propionylcarnitine is present in high abundance in the urine of patients with Methylmalonyl-CoA mutase (MUT) deficiency, (together with Methylmalonic acid). MUT is a mitochondrial enzyme that catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA (OMIM 609058 ).17298-37-2C030171077382886796904CCC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)CC10H19NO4InChI=1S/C10H19NO4/c1-5-10(14)15-8(6-9(12)13)7-11(2,3)4/h8H,5-7H2,1-4H3/t8-/m1/s1UFAHZIUFPNSHSL-MRVPVSSYSA-N217.265217.131408096FDB022268(+/-)-propionylcarnitine chloride;(3-carboxy-2-hydroxypropyl)trimethyl-hydroxide ammonium inner salt;3-carboxy-n,n,n-trimethyl-2-(1-oxopropoxy)-1-propanaminium inner salt;L-propionylcarnitine;O-propanoylcarnitine;O-propionylcarnitine;Propionyl carnitine;Propionyl-l-carnitine;Propionyl-carnitine;Propionylcarnitine;3-propanoyloxy-4-(trimethylazaniumyl)butanoic acidPW_C000658Propion245852469225054225084784303347843713378440345122413408122422406122430418124982374124989120124996454126559482126570479126578489128132502128143501128151506129L-AcetylcarnitineHMDB0000201L-Acetylcarnitine (ALCAR or ALC) is an acetic acid ester of carnitine that facilitates movement of acetyl-CoA into the matrices of mammalian mitochondria during the oxidation of fatty acids. In addition to his metabolic role, acetyl-L-carnitine posses unique neuroprotective, neuromodulatory, and neurotrophic properties this may play an important role in counteracting various disease processes (PMID ID: 15363640).3040-38-8C02571173024(-)o-acetylcarnitine21243783CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)CC9H17NO4InChI=1S/C9H17NO4/c1-7(11)14-8(5-9(12)13)6-10(2,3)4/h8H,5-6H2,1-4H3/t8-/m1/s1RDHQFKQIGNGIED-MRVPVSSYSA-N203.238203.115758031FDB021904(+-)-acetylcarnitine;(-)-acetylcarnitine;(r)-acetylcarnitine;Alcar;Acetyl-l-(-)-carnitine;Acetyl-l-carnitine;Acetyl-carnitine;Acetylcarnitine;L-acetylcarnitine;L-carnitine acetyl ester;L-o-acetylcarnitine;Levocarnitine acetyl;Nicetile;O-acetyl-l-carnitine;O-acetylcarnitine;3-(acetyloxy)-4-(trimethylammonio)butanoate;Acetyl-dl-carnitine;Dl-o-acetylcarnitine;3-(acetyloxy)-4-(trimethylammonio)butanoic acidPW_C000129L-Alcar2461524702250642251547755933477560133775731327844134580631374806321208064511812241840812242440612243141812252712412499745412656548212657347912657948912667929912813850212814650112815250612825838826594,8 Dimethylnonanoyl carnitineHMDB00062024,8 dimethylnonanoyl carnitine is an intermediate in phytanic and pristanic acid metabolism. Both phytanic acid and pristanic acid are initially oxidized in peroxisomes to 4,8-dimethylnonanoyl-CoA, which is then converted to to 4,8-dimethylnonanoyl carnitine (presumably by peroxisomal carnitine octanoyltransferase), and exported to the mitochondrion. After transport across the mitochondrial membrane and transfer of the acylgroup to coenzyme A, further oxidation to 2,6-dimethylheptanoyl-CoA occurs (PMID: 9469587). 4,8 dimethylnonanoyl carnitine is not a substrate for carnitine acetyltransferase, another acyltransferase localized in peroxisomes, which catalyzes the formation of carnitine esters of the other products of pristanic acid beta-oxidation, namely acetyl-CoA and propionyl-CoA. (PMID: 10486279). Earlier studies have shown that pristanic acid undergoes three cycles of beta-oxidation in peroxisomes to produce 4,8-dimethylnonanoyl-CoA (DMN-CoA) which is then transported to the mitochondria for full oxidation to CO(2) and H(2)O. In principle, this can be done via two different mechanisms in which DMN-CoA is either converted into the corresponding carnitine ester or hydrolyzed to 4,8-dimethylnonanoic acid plus CoASH.(PMID: 11785945). Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) and pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) are branched-chain fatty acids that are constituents of the human diet. As phytanic acid possesses a beta-methyl group, it cannot be degraded by beta-oxidation. Instead, phytanic acid is first degraded by alpha-oxidation, yielding pristanic acid, which is subsequently degraded by beta-oxidation. Phytanic acid alpha-oxidation is thought to occur partly, and pristanic acid beta-oxidation exclusively, in peroxisomes. Accumulation of phytanic acid and pristanic acid is found in blood and tissues of patients affected with generalized peroxisomal disorders.534778016387421233922CC(C)CCCC(C)CCC(=O)OC(CC([O-])=O)C[N+](C)(C)CC18H35NO4InChI=1S/C18H35NO4/c1-14(2)8-7-9-15(3)10-11-18(22)23-16(12-17(20)21)13-19(4,5)6/h14-16H,7-13H2,1-6H3DDTDJDZHDFMZED-UHFFFAOYSA-N329.4748329.256608613FDB0238344,8-dimethylnonanoylcarnitinePW_C00265948DnCn246552473225074225164784323347843913378442345122416408122426406122432418124985374124992120124998454126563482126575479126580489128136502128148501128153506639Phytanic acidHMDB0000801Phytanic acid (or 3,7,11,15-tetramethylhexadecanoic acid) is a 20-carbon branched-chain fatty acid that humans can obtain through the consumption of dairy products, ruminant animal fats, and certain fish. It is primarily formed by bacterial degradation of chlorophyll in the intestinal tract of ruminants. Unlike most fatty acids, phytanic acid cannot be metabolized by beta-oxidation (because of a methyl group in the beta position). Instead, it undergoes alpha-oxidation in the peroxisome, where it is converted into pristanic acid by the removal of one carbon. Pristanic acid can undergo several rounds of beta-oxidation in the peroxisome to form medium-chain fatty acids that can be converted into carbon dioxide and water in mitochondria. Refsum disease, an autosomal recessive neurological disorder caused by mutations in the PHYH gene, is characterized by having impaired alpha-oxidation activity. Individuals with Refsum disease accumulate large stores of phytanic acid in their blood and tissues. This frequently leads to peripheral polyneuropathy, cerebellar ataxia, retinitis pigmentosa, anosmia, and hearing loss. Therefore, chronically high levels of phytanic acid can be neurotoxic. Phytanic acid's neurotoxicity appears to lie in its ability to initiate astrocyte/neural cell death by activating the mitochondrial route of apoptosis. In particular, phytanic acid can induce the substantial generation of reactive oxygen species in isolated mitochondria as well as in intact cells. It also induces the release of cytochrome c from mitochondria.14721-66-5C01607268401628525001CC(C)CCCC(C)CCCC(C)CCCC(C)CC(O)=OC20H40O2InChI=1S/C20H40O2/c1-16(2)9-6-10-17(3)11-7-12-18(4)13-8-14-19(5)15-20(21)22/h16-19H,6-15H2,1-5H3,(H,21,22)RLCKHJSFHOZMDR-UHFFFAOYSA-N312.5304312.302830524FDB0222523,7,11,15-tetramethyl-hexadecanoate;3,7,11,15-tetramethyl-hexadecanoic acid;3,7,11,15-tetramethyl-hexadecansaeure;3,7,11,15-tetramethylhexadecanoate;3,7,11,15-tetramethylhexadecanoic acid;3,7,11,15-tetramethylhexadecoanoate;3,7,11,15-tetramethylhexadecoanoic acid;Phytanate;Phytanoate;Phytanoic acid;3,7,11,15-tetramethyl hexadecanoic acid;3,7,11,15-tetramethyl hexadecanoatePW_C000639Phytana126082485597843433578552111120975122122419422123540135124987375125915297126566491127374205128139508546Long-chain-fatty-acid--CoA ligase 1P33121Activation of long-chain fatty acids for both synthesis of cellular lipids, and degradation via beta-oxidation. Preferentially uses palmitoleate, oleate and linoleate.
HMDBP00577ACSL14q35CH47105616.2.1.38842224125926122525410413263616613562832913784771314113631289Aldehyde dehydrogenase, mitochondrialP05091HMDBP00295ALDH212q24.2K0300111.2.1.354741321818713300921354921814157795614202197914347554144669129214583712039452-hydroxyacyl-CoA lyase 1Q9UJ83Catalyzes a carbon-carbon cleavage reaction; cleaves a 2-hydroxy-3-methylacyl-CoA into formyl-CoA and a 2-methyl-branched fatty aldehyde.
HMDBP08729HACL13p25.1AJ13175314.1.-.-12725264Phytanoyl-CoA dioxygenase, peroxisomalO14832Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
HMDBP00270PHYH10p13AF24238411.14.11.18126855712Peroxisomal membrane protein PEX13Q92968AF04875518473595713Peroxisomal membrane protein PEX14O75381AF045186184745928Carnitine O-acetyltransferaseP43155Carnitine acetylase is specific for short chain fatty acids. Carnitine acetylase seems to affect the flux through the pyruvate dehydrogenase complex. It may be involved as well in the transport of acetyl-CoA into mitochondria.
HMDBP00028CRAT9q34.1X7982712.3.1.724605252031410538021411284301Peroxisomal carnitine O-octanoyltransferaseQ9UKG9Beta-oxidation of fatty acids. The highest activity concerns the C6 to C10 chain length substrate. Converts the end product of pristanic acid beta oxidation, 4,8-dimethylnonanoyl-CoA, to its corresponding carnitine ester.
HMDBP00307CROT7q21.1CH23694912.3.1.13724665252235714ATP-binding cassette sub-family D member 1P33897Z3100618448595715ATP-binding cassette sub-family D member 2Q9UBJ2BC10490118449592100Mitochondrial carnitine/acylcarnitine carrier proteinO43772Mediates the transport of acylcarnitines of different length across the mitochondrial inner membrane from the cytosol to the mitochondrial matrix for their oxidation by the mitochondrial fatty acid-oxidation pathway.
HMDBP02794SLC25A203p21.31Y103191847517305Carnitine O-palmitoyltransferase 2, mitochondrialP23786HMDBP00311CPT21p32U0964312.3.1.218931726703267625233102135622134137852713141052614106380214113053141131551694Very long-chain acyl-CoA synthetaseO14975Acyl-CoA synthetase probably involved in bile acid metabolism. Proposed to activate C27 precurors of bile acids to their CoA thioesters derivatives before side chain cleavage via peroxisomal beta-oxidation occurs. In vitro, activates 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanate (THCA), the C27 precursor of cholic acid deriving from the de novo synthesis from cholesterol. Does not utilize C24 bile acids as substrates. In vitro, also activates long- and branched-chain fatty acids and may have additional roles in fatty acid metabolism. May be involved in translocation of long-chain fatty acids (LFCA) across membranes (By similarity).
HMDBP01946SLC27A215q21.2AF09629016.2.1.-; 6.2.1.312628144237309144238308144329808246Long-chain-fatty-acid--CoA ligase 11PW_P00024626554611134231159Aldehyde dehydrogenase, mitochondrial1PW_P000159177289454543552-hydroxyacyl-CoA lyase 11PW_P00035537639454161423416210604354Phytanoyl-CoA dioxygenase, peroxisomal1PW_P000354375264115997941160311620Peroxisomal membrane protein1PW_P0006206645712166557131628Carnitine O-acetyltransferase1PW_P000628674281629Peroxisomal carnitine O-octanoyltransferase1PW_P0006296753011636ATP-binding cassette sub-family D1PW_P0006366875714168857151644Mitochondrial carnitine/acylcarnitine carrier protein1PW_P00064470221001248Carnitine O-palmitoyltransferase 2, mitochondrial1PW_P0002482673051353Very long-chain acyl-CoA synthetase1PW_P000353374169411350falsePW_R001350Right51294141Compoundtrue51306351Compoundfalse513110991Compoundtrue513210341Compoundtrue513313811Compoundfalse51341701Compoundtrue10662466.2.1.31354falsePW_R001354Right514913091Compoundfalse515010991Compoundtrue515113811Compoundfalse10701591.2.1.3864falsePW_R000864Right35019991Compoundfalse350213091Compoundfalse350318831Compoundtrue4333554.1.-.-863falsePW_R000863Right349510491Compoundfalse34961341Compoundtrue349710651Compoundtrue34989991Compoundfalse34991741Compoundtrue350013161Compoundtrue4323541.14.11.181379falsePW_R001379Both52459881Compoundfalse5246441Compoundtrue52476581Compoundfalse524810991Compoundtrue10996282.3.1.71384falsePW_R001384Both525971981Compoundfalse5260441Compoundtrue526126591Compoundfalse526210991Compoundtrue11042482.3.1.211381falsePW_R001381Both52519401Compoundfalse5252441Compoundtrue52531291Compoundfalse525410991Compoundtrue11016282.3.1.7862falsePW_R000862Right34896391Compoundfalse34904141Compoundtrue349110991Compoundtrue349210491Compoundfalse349310341Compoundtrue34941701Compoundtrue43135311142466.2.1.371PW_T0000718113811Compound85Right376202013-08-16T15:27:58-06:002013-08-16T15:27:58-06:005973PW_T000073836581Compound52Right386202013-08-19T11:05:58-06:002013-08-19T11:05:58-06:005974PW_T000074841291Compound52Right396202013-08-19T11:06:47-06:002013-08-19T11:06:47-06:005975PW_T0000758526591Compound52Right406202013-08-19T11:07:48-06:002013-08-19T11:07:48-06:005976PW_T0000768610491Compound52Right416362013-08-19T11:57:41-06:002013-08-19T11:57:41-06:005977PW_T000077876581Compound424Right426442013-08-19T15:22:15-06:002013-08-19T15:22:15-06:001778PW_T000078881291Compound24Right436442013-08-19T15:28:26-06:002013-08-19T15:28:26-06:001779PW_T0000798926591Compound424Right446442013-08-19T15:29:50-06:002013-08-19T15:29:50-06:001746034145942false148559410regular503046046355982false160525910regular300280460510995985false157561910regular5030460610345943false123559510regular503046071381882false89026010regular30028046081705945false116361510regular63434609423599false134074910regular1002546221381582false895123510regular30028046241309382false375166010regular30028046251099385false565128510regular50304633999582false1340214510regular30028046341883581false790196010regular200190463542359false1065232010regular100254636106059false1065222510regular1003546371049582false2140214310regular3002804638134581false1920204510regular20019046391065565false1961235110regular78784640174581false1650204510regular20019046411316552false1721235110regular78784642979459false1825223510regular1002546433159false1830230510regular100254730988582false150084510regular3002804731940582false1495123510regular30028047327198582false1490158010regular300280475144581false1820109010regular2001904752658582false227084510regular30028047531099585false217090010regular50304756129582false2270123510regular300280476044581false1820179010regular20019047612659582false2270158010regular30028047621099585false2170164010regular503047666584282false285584510regular30028047671294282false2860123010regular300280477026594282false2860157510regular30028047721099585false2180129510regular503047781049282false2140275310regular30028047796395982false1415275710regular30028047804145942false1765269710regular5030478110995985false1680271710regular5030478210345943false1990269510regular503047831705945false2063271310regular63434784423599false1855258210regular100254838658482false346584510regular3002804839129482false3465123010regular30028048402659482false3465157510regular3002804848988382false421584510regular300280484944381false3990108510regular20019048501099385false378090010regular50304854940382false4215123010regular30028048551099385false3795129010regular503048567198382false4215157510regular3002804865940382false4215204010regular300280519244381false3995178510regular20019051931099385false3805164010regular50301751546592false13157598subunitregular15070175528938false69513358subunitregular140851758394558false104522458subunitregular14085175926452false180522508subunitregular15070177257125976false10457758subunitregular15070177357135976false8957758subunitregular1507018102852false20009508subunitregular15070181930152false201016858subunitregular15070182357125976false26609658subunitregular15070182457135976false26609208subunitregular15070182557125976false267013558subunitregular15070182657135976false267013108subunitregular15070183157125976false266517008subunitregular15070183257135976false266516558subunitregular1507018382852false201213408subunitregular15070184957145976false228024888subunitregular15070185057155976false217024888subunitregular150701851546592false183025928subunitregular15070190221001776false32609508subunitregular15070190321001776false326513358subunitregular15070190421001776false326516808subunitregular1507019082832false38509508subunitregular1507019102832false386213358subunitregular15070212030532false388216808subunitregular150701628246100591744175180346096888Cofactor1632159100317481755163535510051751175880646356923Cofactor80746366924Cofactor163635410051752175980846426931Cofactor80946436932Cofactor16496201001765177217661773168562810051803181016936291005181218191697620100181618231817182416986201001818182518191826170262010018241831182518321708628100518311838171563610018421849184318501716246100591844185184247847164Cofactor1755644100189519021756644100189619031757644100189719041761628100319011908176362810031903191019152481003211321206882M1510 624 C1508 678 1495 794 1465 794 5false186883M1755 539 C1757 661 1621 793 1465 794 5false186884M1600 649 C1599 701 1550 793 1465 794 5false186885M1260 625 C1260 661 1285 794 1315 794 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false6886M1040 540 C1037 646 1227 791 1315 794 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false6887M1194.5 658 C1195.5 715 1252 791 1315 794 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false6888M1385 824 L1385 874 L1435 824 z10true186904M1040 540 C1040 570 1045 1212 1045 1235 83true18falsefalse6905M1045 1235 C1045 1205 1040 608 1040 540 83false7trueM 1252.5 847.0096189432334 L 1260 860 L 1267.5 847.0096189432334false6908M525 1660 C525 1531 586 1373 695 1375 5false186909M590 1315 C590 1352 649 1375 695 1375 5false186910M895 1375 C865 1375 865 1375 835 1375 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false6920M1340 2285 C1310 2285 1215 2285 1185 2285 5false186921M525 1940 C524 2162 689 2279 1045 2285 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false6922M890 2150 C892 2230 1010 2285 1045 2285 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false6923M765 1945 L765 1995 L815 1945 z10true186924M765 1945 L765 1995 L815 1945 z10true186925M2140 2283 C2110 2283 1985 2285 1955 2285 5false186926M2020 2235 C2020 2259 1985 2285 1955 2285 5false186927M2000 2351 C2000 2327 1985 2285 1955 2285 5false186928M1640 2285 C1670 2285 1775 2285 1805 2285 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false6929M1750 2235 C1750 2261 1775 2285 1805 2285 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false6930M1760 2351 C1760 2325 1775 2285 1805 2285 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false6931M1018 -25 L1018 25 L1068 -25 z10true186932M1018 -25 L1018 25 L1068 -25 z10true187057M1045 1235 C1044 1119 1152 991 1280 985 5false187058M1500 985 C1470 985 1460 985 1430 985 5false18trueM 2152.0096189432334 932.5 L 2165 925 L 2152.0096189432334 917.5false7059M1195 1375 C1225 1375 1255 1375 1285 1375 5false187060M1495 1375 C1465 1375 1465 1375 1435 1375 5false18trueM 2152.0096189432334 1132.5 L 2165 1125 L 2152.0096189432334 1117.5false7061M1045 1515 C1048 1619 1123 1718 1285 1720 5false187062M1490 1720 C1460 1720 1465 1720 1435 1720 5false18trueM 2152.0096189432334 1332.5 L 2165 1325 L 2152.0096189432334 1317.5false7095M1800 985 C1830 985 1970 985 2000 985 5false187096M1920 1090 C1918 1044 1934 985 2000 985 5false187097M2270 985 C2240 985 2180 985 2150 985 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7098M2195 930 C2195 957 2180 985 2150 985 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7111M1790 1720 C1820 1720 1980 1720 2010 1720 5false187112M1920 1790 C1920 1770 1922 1720 2010 1720 5false187113M2270 1720 C2240 1720 2190 1720 2160 1720 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7114M2195 1670 C2195 1690 2190 1720 2160 1720 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7120M2570 985 C2600 985 2825 985 2855 985 83true187121M2855 985 C2825 985 2600 985 2570 985 83false77122M2570 1375 C2600 1375 2830 1370 2860 1370 83true187123M2860 1370 C2830 1370 2600 1375 2570 1375 83false77128M2570 1720 C2600 1720 2830 1715 2860 1715 83true187129M2860 1715 C2830 1715 2600 1720 2570 1720 83false77141M1795 1375 C1825 1375 1982 1375 2012 1375 5false187142M1920 1280 C1922 1324 1946 1373 2012 1375 5false187143M2270 1375 C2240 1375 2192 1375 2162 1375 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7144M2205 1325 C2205 1347 2192 1375 2162 1375 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7156M2290 2423 C2290 2453 2425 2318 2425 2348 83true18trueM 2332.5 1905.9903810567666 L 2325 1893 L 2317.5 1905.9903810567666false7157M2290 2753 C2290 2723 2290 2453 2290 2423 83false7trueM 2317.5 2190.0096189432334 L 2325 2203 L 2332.5 2190.0096189432334false7158M1565 2757 C1567 2705 1720 2627 1830 2627 5false187159M1790 2697 C1790 2678 1800 2627 1830 2627 5false187160M1705 2717 C1726 2682 1781 2627 1830 2627 5false187161M2290 2753 C2240 2678 2095 2626 1980 2627 5false6trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7162M2015 2695 C2015 2676 2010 2627 1980 2627 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7163M2094.5 2713 C2078.5 2671 2023 2627 1980 2627 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7164M250 450 L250 500 L300 450 z10true187261M3155 985 C3185 985 3230 985 3260 985 83false187262M3465 985 C3435 985 3440 985 3410 985 83false18trueM 3412.0096189432334 982.5 L 3425 975 L 3412.0096189432334 967.5false7263M3160 1370 C3190 1370 3235 1370 3265 1370 83false187264M3465 1370 C3435 1370 3445 1370 3415 1370 83false18trueM 3242.0096189432334 1187.5 L 3255 1180 L 3242.0096189432334 1172.5false7265M3160 1715 C3190 1715 3235 1715 3265 1715 83false18trueM 3017.9903810567666 2677.5 L 3005 2685 L 3017.9903810567666 2692.5false7266M3465 1715 C3435 1715 3445 1715 3415 1715 83false18trueM 3412.0096189432334 1392.5 L 3425 1385 L 3412.0096189432334 1377.5false7275M4215 985 C4185 985 4030 985 4000 985 5false18trueM 3887.0096189432334 982.5 L 3900 975 L 3887.0096189432334 967.5false7276M4090 1085 C4090 1057 4046 985 4000 985 5false18trueM 3822.5 1022.0096189432334 L 3830 1035 L 3837.5 1022.0096189432334false7277M3765 985 C3795 985 3820 985 3850 985 5false18falsefalse7278M3805 930 C3805 953 3820 985 3850 985 5false18falsefalse7283M4215 1370 C4185 1370 4042 1370 4012 1370 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7284M4090 1275 C4090 1301 4080 1366 4012 1370 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7285M3765 1370 C3795 1370 3832 1370 3862 1370 5false18falsefalse7286M3820 1320 C3820 1339 3832 1370 3862 1370 5false18falsefalse7303M4365 1855 C4365 1885 4365 1880 4365 1910 5false187304M4365 2040 C4365 2010 4365 2010 4365 1980 5false18trueM 4042.5 1762.0096189432334 L 4050 1775 L 4057.5 1762.0096189432334false7887M4215 1715 C4185 1715 4062 1715 4032 1715 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7888M4095 1785 C4095 1757 4062 1715 4032 1715 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7889M3765 1715 C3795 1715 3852 1715 3882 1715 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false7890M3830 1670 C3830 1691 3852 1715 3882 1715 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1475100135059518846036882Left518946046883Left519046056884Left519146066885Right519246076886Right519346086887Right141110661628147910013543520846246908Left520946256909Left521046226910Right14151070163214821008645521946336920Left522046246921Right522146346922Right1418433163514831008635522246376925Left522346386926Left522446396927Left522546336928Right522646406929Right522746416930Right14194321636152110013795534147307095Left534247517096Left534347527097Right534447537098Right145910991685152610013845535147327111Left535247607112Left535347617113Right535447627114Right146311041693153410013815536547317141Left536647517142Left536747567143Right536847727144Right146911011708153910086259537647797158Left537747807159Left537847817160Left537947787161Right538047827162Right538147837163Right147411141716156410013793544448487275Left544548497276Left544648387277Right544748507278Right149910991761156610013813545248547283Left545348497284Left545448397285Right545548557286Right150111011763165810013843578648567887Left578751927888Left578848407889Right578951937890Right1590110419151247110026946076904Left27046226905Right541649371287310027747527120Left27847667121Right551697381297410027947567122Left28047677123Right561698391307510028147617128Left28247707129Right571702401317610028346377156Left28447787157Right581715411337710028747667261Left28848387262Right601755421347810028947677263Left29048397264Right611756431357910029147707265Left29248407266Right62175744259213100217false128095016regular23846227057Left23947307058Right260214100217false1285134016regular24046227059Left24147317060Right261215100217false1285168516regular24246227061Left24347327062Right272236100217false4290191016regular25048567303Left25148657304Right2356721303351.01.00243993402361335004401.01.0021490240187221272524152.62.60314327267479711323325082.22.2-2523280360261M277 902 C277 852 327 802 377 802 C1073 802 1978 802 2674 802 C2724 802 2774 852 2774 902 C2774 1365 2774 1966 2774 2429 C2774 2479 2724 2529 2674 2529 C1978 2529 1073 2529 377 2529 C327 2529 277 2479 277 2429 C277 1966 277 1365 277 902 94true62497.01727.0263M3269 798 C3269 1093 3275 2099 3275 2395 84false66.01597.0264M3396 797 C3396 1088 3403 2094 3403 2394 84false67.01597.0266M127 276 C127 226 177 176 227 176 C1544 176 3255 176 4572 176 C4622 176 4672 226 4672 276 C4672 1124 4672 2228 4672 3076 C4672 3126 4622 3176 4572 3176 C3255 3176 1544 3176 227 3176 C177 3176 127 3126 127 3076 C127 2228 127 1124 127 276 1true64545.03000.0291235Peroxisome2190380201.31.320015292235Mitochondria3865795201.31.32001529315Mitochondrial Inner Membrane33051105201.31.31601529415Mitochondrial Outer Membrane31601170201.31.316015295235Intracellular Space435315201.31.320015296235Extracellular Space43570201.31.3200151032321523294819275325104#FBEBFC42459169110437055443201797455823964#FFEBEB413571599