201ContextAdrenoleukodystrophy, X-LinkedAdrenoleukodystrophy (ALD) is an X-linked recessive transmission disease. Central nervous system signs and symptoms have been consistently more prominent than signs of adrenal involvement. Behavioral changes are the most common initial finding and range from aggressive outbursts to withdrawal. Such behavior is generally accompanied by a gradually failing memory and poor school performance. Loss of vision is an early finding in some patients and is a prominent feature at some stage in most affected individuals. The initial visual loss appears as homonomous hemianopsia in some individuals and is usually associated with intact pupillary reflexes. Optic atrophy is less common as an initial finding but eventually develops in almost all cases. Gait disturbance is also an early finding and as is stiff-legged, unsteady and accompanied by hyperreflexia. In almost all cases there is spastic quadraplegia and a variable degree of decorticate posturing. Hearing loss, dysarthria and dysphagia develop at about the same time as gait disturbance. Seizures are a typical symptom in many affected individuals in the the end stages of the disease progression.DiseasePW000492CenterPathwayVisualizationContext52634504900#000099PathwayVisualization106346Beta Oxidation of Very Long Chain Fatty AcidsThe degradation of fatty acids occurs is many ways, but for the most part in most species it occurs mainly through the beta-oxidation cycle. Take mammals for example, in this subset of species we find that beta-oxidation takes place not only in mitochondria, but in peroxisomes as well. In contrast, it tends to be the case that in plants and fungi beta-oxidation is only seen in peroxisomes. The reason the beta-oxidation cycle is found to occur in both mitochondria and peroxisomes in mammals is thought to be that extremely long chain fatty acids will in fact undergo oxidation in both locations, an initial or first oxidation in peroxisomes and second oxidation in the mitochondria. There is however a difference between the oxidation cycle which occurs in both these organelles. Namely, that the oxidation undergone in peroxisomes does not have any coupling to ATP synthesis, unlike the corresponding oxidation which occurs in the mitochondria. We find rather that electrons are passed to molecules of oxygen, which produces hydrogen peroxide. Moreover, there is an enzyme which is found only peroxisomes which ties into this process. It can turn hydrogen peroxide back into water and oxygen and is catalase. To expound further the differences between the oxidation cycle found in the peroxisomes and the mitchondria consider the following three key differences. One, in the peroxisome the beta-oxidation cycle takes as a necessary input a special enzyme called, peroxisomal carnitine acyltransferase, which is needed to move an activated acyl group from outside the peroxisome to inside it. In mitochondrial oxidation similar but different enzymes are used called carnitine acyltransferase I and II. Difference number two is that oxidation in the peroxisome commences with catalysis induced by an enzyme called acyl CoA oxidase. Also, it should be noted that another enzyme called beta-ketothiolase which aids in peroxisomal beta-oxidation has a substrate specificity which differs from that of the mitochondrial beta-ketothiolase. Turning now to how the oxidation cycle function in mitochondria, note that the mitochondrial beta-oxidation pathway is composed of four repeating reactions that take place with each fatty acid molecule. The oxidation of fatty acid chains is a process of progress through repetition. With each turn of the cycle two carbons are removed from the fatty acid chain and the energy of the chemical bonds once housed by the molecule is captured by the reduced energy carriers NADH and FADH2. Acetyl-CoA is created in this 4 step reaction beta-oxidation process and is sent to the TCA cycle. Once inside the TCA cycle, the process of oxidation continues until even the acetyl-CoA is oxidized to CO2. More NADH and FADH2 result. Metabolic1309Beta oxidationSubPathway4642794Compound54651171Compound466940Compound310Beta oxidationSubPathway4672751Compound54681928Compound469940Compound312Beta oxidationSubPathway4711928Compound3472940Compound117Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.346Pathway118Lodish, H. et al. Molecular cell biology. (2004) New York: W.H Freeman.346Pathway119Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.346Pathway120Vance, D.E., and Vance, J.E. Biochemistry of lipids, lipoproteins, and membranes (4th ed.) (2002) Amsterdam; Boston: Elsevier.346Pathway280511444549Lawson 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)90058-4.346Pathway28051215466478Yu 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 4.346Pathway28051310407780Wanders 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):442-87.346Pathway28051510051453Muoio 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):783-91.346Pathway28051711673457Hunt MC, Solaas K, Kase BF, Alexson SE: Characterization of an acyl-coA thioesterase that functions as a major regulator of peroxisomal lipid metabolism. J Biol Chem. 2002 Jan 11;277(2):1128-38. doi: 10.1074/jbc.M106458200. Epub 2001 Oct 22.346Pathway28051810527927Zammit VA: The malonyl-CoA-long-chain acyl-CoA axis in the maintenance of mammalian cell function. Biochem J. 1999 Nov 1;343 Pt 3:505-15.346Pathway1CellCL:00000002Platelet CL:00002335HepatocyteCL:00001823NeuronCL:00005404CardiomyocyteCL:00007468Beta cellCL:00006397Epithelial CellCL:000006611Colorectal Cancer CellCL:00010641Homo sapiens9606EukaryoteHuman12Mus musculus10090EukaryoteMouse17Rattus norvegicus10116EukaryoteRat5Bos taurus9913EukaryoteCattle10Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm2Bacteria2ProkaryoteBacteria3Escherichia coli562Prokaryote19Schizosaccharomyces pombe4896Eukaryote24Solanum lycopersicum4081EukaryoteTomato4Arabidopsis thaliana3702EukaryoteThale cress18Saccharomyces cerevisiae4932EukaryoteYeast21Xenopus laevis8355EukaryoteAfrican clawed frog25Escherichia coli (strain K12)83333Prokaryote49Bathymodiolus platifrons220390EukaryoteDeep sea mussel23Pseudomonas aeruginosa287Prokaryote60Nitzschia sp.0001EukaryoteNitzschia41CytosolGO: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:00057582Endothelium BTO:00003931LiverBTO:00007597297Nervous SystemBTO:000148418PancreasBTO:000098825IntestineBTO:00006488Blood VesselBTO:000110274115cardiocyteBTO:000153922BladderBTO:00001232111PW_BS0000021321121PW_BS0001321181171PW_BS000118124151PW_BS0001242991101PW_BS000024388161PW_BS0001124311PW_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_BS0001171203171PW_BS00012012915121PW_BS0001291333121PW_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_BS000001943PW_BS000094109323PW_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_BS0001154812101PW_BS00011548414101PW_BS00011548515101PW_BS00011530013101PW_BS0000244957101PW_BS0001154781010PW_BS00011549127101PW_BS0001154991510PW_BS000115501361PW_BS0001153891461PW_BS0001125161561PW_BS0001153951361PW_BS000113390761PW_BS000112209106PW_BS0000245082761PW_BS000115517156PW_BS0001158911421PW_BS000552171211PW_BS000017111811PW_BS0000116131PW_BS0000061021231PW_BS00010216212181PW_BS000162224241PW_BS0000241951318PW_BS0000242491341PW_BS00002429341PW_BS0000242881441PW_BS0000243841251PW_BS0001004141551PW_BS00011512112171PW_BS00012145015171PW_BS00011548012101PW_BS0001154824101PW_BS0001153911261PW_BS000112502461PW_BS0001151861221PW_BS000024185321PW_BS00002415111PW_BS000015471914PW_BS00004731323PW_BS000024215114PW_BS000021432511PW_BS000043562611PW_BS00005621425181PW_BS00002435625121PW_BS000028372102PW_BS0000284192551PW_BS00011545525171PW_BS00011549025101PW_BS0001155072561PW_BS000115422411PW_BS00004234524121PW_BS0000284182451PW_BS00011545424171PW_BS00011548924101PW_BS0001155062461PW_BS0001157132210111PW_BS00051280231PW_BS0005485311015PW_BS0000535511215PW_BS0000551589hexacosanoic acidHMDB0002356X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder biochemically characterized by the accumulation of very long chain fatty acids (VLCFA), particularly hexacosanoic acid (C(26:0)) and tetracosanoic acid (C(24:0)), in tissues and biological fluids. (PMID 16750542).506-46-710469310092-PG10037CCCCCCCCCCCCCCCCCCCCCCCCCC(O)=OC26H52O2InChI=1S/C26H52O2/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-16-17-18-19-20-21-22-23-24-25-26(27)28/h2-25H2,1H3,(H,27,28)XMHIUKTWLZUKEX-UHFFFAOYSA-Nhexacosanoic acid396.6899396.396730908-7.341hexacosanoic acid0-1FDB003045Cerate;Ceratinate;Ceratinic acid;Ceric acid;Cerinate;Cerinic acid;Cerotic acid;Cerylate;Cerylic acid;Hexacosanoate;Hexacosanoate (n-c26:0);Hexacosanoic acid;N-hexacosanoate;N-hexacosanoic acid;C26:0;Ch3-[ch2]24-cooh;Hexacosansaeure;Hexacosoic acid;Hexaeicosanoic acid;N-c26:0;Cerotate;Hexacosoate;Hexaeicosanoate;FA(26:0)PW_C001589C26:0261027754913280619118122514124126667299128247388414Adenosine triphosphateHMDB0000538Adenosine triphosphate (ATP) is a nucleotide consisting of a purine base (adenine) attached to the first carbon atom of ribose (a pentose sugar). Three phosphate groups are esterified at the fifth carbon atom of the ribose. ATP is incorporated into nucleic acids by polymerases in the processes of DNA replication and transcription. ATP contributes to cellular energy charge and participates in overall energy balance, maintaining cellular homeostasis. ATP can act as an extracellular signaling molecule via interactions with specific purinergic receptors to mediate a wide variety of processes as diverse as neurotransmission, inflammation, apoptosis, and bone remodelling. Extracellular ATP and its metabolite adenosine have also been shown to exert a variety of effects on nearly every cell type in human skin, and ATP seems to play a direct role in triggering skin inflammatory, regenerative, and fibrotic responses to mechanical injury, an indirect role in melanocyte proliferation and apoptosis, and a complex role in Langerhans cell-directed adaptive immunity. During exercise, intracellular homeostasis depends on the matching of adenosine triphosphate (ATP) supply and ATP demand. Metabolites play a useful role in communicating the extent of ATP demand to the metabolic supply pathways. Effects as different as proliferation or differentiation, chemotaxis, release of cytokines or lysosomal constituents, and generation of reactive oxygen or nitrogen species are elicited upon stimulation of blood cells with extracellular ATP. The increased concentration of adenosine triphosphate (ATP) in erythrocytes from patients with chronic renal failure (CRF) has been observed in many studies but the mechanism leading to these abnormalities still is controversial. (PMID: 15490415, 15129319, 14707763, 14696970, 11157473).56-65-5C00002595715422ATP5742DB00171NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1OC10H16N5O13P3InChI=1S/C10H16N5O13P3/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(26-10)1-25-30(21,22)28-31(23,24)27-29(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H,23,24)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1ZKHQWZAMYRWXGA-KQYNXXCUSA-N({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)phosphonic acid507.181506.995745159-2.057adenosine triphosphate0-3FDB0218135'-(tetrahydrogen triphosphate) adenosine;5'-atp;Atp;Adenosine 5'-triphosphate;Adenosine 5'-triphosphorate;Adenosine 5'-triphosphoric acid;Adenosine triphosphate;Adenylpyrophosphorate;Adenylpyrophosphoric acid;Adephos;Adetol;Adynol;Atipi;Atriphos;Cardenosine;Fosfobion;Glucobasin;Myotriphos;Phosphobion;Striadyne;Triadenyl;Triphosphaden;Triphosphoric acid adenosine ester;Adenosine-5'-triphosphate;H4atp;Adenosine triphosphoric acid;Adenosine-5'-triphosphoric acidPW_C000414ATP922146082661641422478137333279959343997632105182112102146492156142160582405592434272726462812293029663163723616613617514399234474314768914864545032895035265155752059752151005250104529110153131115346112539010354061175430118544312055421295556132556913356031355621108584614358541465876107589714759241516048155610916162301666493178683918868701606976199715720571842067209210722521372292117298198730221673902177408218743216374812227499190818622511847277119031701201028112039164121782851257822612691290132642231532730842326315426213224269431877028253772181347723332977468333776323367803733278041350781681287821435178240353784113357849411578850130788653317891933480028368800461848067411985629194826124113234941132823881162801091199141221199924061201544071202453821203624121212464291213921231213974331214714081219744101220651251220793831220834051224024221224444351229193991230094461238164641239514471239564681240293741245274441246161361246303981246343761249434721249723751250114701253042971253714791253922991255154811255954841261234851262203001262344951262404781265474911265964991269135011271233891277315161277813951277963901278012091281195081281675171407708911099Coenzyme AHMDB0001423Coenzyme A (CoA, CoASH, or HSCoA) is a coenzyme notable for its role in the synthesis and oxidization of fatty acids and the oxidation of pyruvate in the citric acid cycle. It is adapted from beta-mercaptoethylamine, panthothenate, and adenosine triphosphate. It is also a parent compound for other transformation products, including but not limited to, phenylglyoxylyl-CoA, tetracosanoyl-CoA, and 6-hydroxyhex-3-enoyl-CoA. Coenzyme A is synthesized in a five-step process from pantothenate and cysteine. In the first step pantothenate (vitamin B5) is phosphorylated to 4'-phosphopantothenate by the enzyme pantothenate kinase (PanK, CoaA, CoaX). In the second step, a cysteine is added to 4'-phosphopantothenate by the enzyme phosphopantothenoylcysteine synthetase (PPC-DC, CoaB) to form 4'-phospho-N-pantothenoylcysteine (PPC). In the third step, PPC is decarboxylated to 4'-phosphopantetheine by phosphopantothenoylcysteine decarboxylase (CoaC). In the fourth step, 4'-phosphopantetheine is adenylylated to form dephospho-CoA by the enzyme phosphopantetheine adenylyl transferase (CoaD). Finally, dephospho-CoA is phosphorylated using ATP to coenzyme A by the enzyme dephosphocoenzyme A kinase (CoaE). Since coenzyme A is, in chemical terms, a thiol, it can react with carboxylic acids to form thioesters, thus functioning as an acyl group carrier. CoA assists in transferring fatty acids from the cytoplasm to the mitochondria. A molecule of coenzyme A carrying an acetyl group is also referred to as acetyl-CoA. When it is not attached to an acyl group, it is usually referred to as 'CoASH' or 'HSCoA'. Coenzyme A is also the source of the phosphopantetheine group that is added as a prosthetic group to proteins such as acyl carrier proteins and formyltetrahydrofolate dehydrogenase. Acetyl-CoA is an important molecule itself. It is the precursor to HMG CoA which is a vital component in cholesterol and ketone synthesis. Furthermore, it contributes an acetyl group to choline to produce acetylcholine in a reaction catalysed by choline acetyltransferase. Its main task is conveying the carbon atoms within the acetyl group to the citric acid cycle to be oxidized for energy production (Wikipedia).85-61-0C0001068161146900CO-A6557CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)[C@@H](O)C(=O)NCCC(=O)NCCSC21H36N7O16P3SInChI=1S/C21H36N7O16P3S/c1-21(2,16(31)19(32)24-4-3-12(29)23-5-6-48)8-41-47(38,39)44-46(36,37)40-7-11-15(43-45(33,34)35)14(30)20(42-11)28-10-27-13-17(22)25-9-26-18(13)28/h9-11,14-16,20,30-31,48H,3-8H2,1-2H3,(H,23,29)(H,24,32)(H,36,37)(H,38,39)(H2,22,25,26)(H2,33,34,35)/t11-,14-,15-,16+,20-/m1/s1RGJOEKWQDUBAIZ-IBOSZNHHSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[(3R)-3-hydroxy-2,2-dimethyl-3-({2-[(2-sulfanylethyl)carbamoyl]ethyl}carbamoyl)propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid767.534767.115208365-2.2210coenzyme A0-4FDB022614Acetoacetyl coenzyme a sodium salt;Coa;Coa hydrate;Coa-sh;Coash;Coenzyme a;Coenzyme a hydrate;Coenzyme a-sh;Coenzyme ash;Coenzymes a;Depot-zeel;Propionyl coa;Propionyl coenzyme a;S-propanoate;S-propanoate coa;S-propanoate coenzyme a;S-propanoic acid;S-propionate coa;S-propionate coenzyme a;Zeel;[(2r,3s,4r,5r)-5-(6-amino-9h-purin-9-yl)-4-hydroxy-3-(phosphonooxy)tetrahydrofuran-2-yl]methyl 3-hydroxy-4-({3-oxo-3-[(2-sulfanylethyl)amino]propyl}amino)-2,2-dimethyl-4-oxobutyl dihydrogen diphosphatePW_C001099CoA21143868845387922892172407592414224595281329286231334211335118461810462958484214486554487965232102524710452801035477124573410857771016023155607516163841646817869301606961162697319970831887108163729319873472107458222822915190812269090224912417092151951301329915318249254884942616315769072937711913377222134772303297729211177550132775553347756311277633336776721297799611578047332780563507841333578567130792593337997433180005368806201188062737480635119806653769382838293834383986742881105553891105613901158423991158473981199514061201474051202313841203051221206344071207621171214061231214214331215211251216664291216824081217144141224044221227411201229041211229601351239654471239794681240791361242204641242654501249743751253414791255094781255794801255924841256342971260844811265494911265604821267463001268845011270462091271093911273012051275402061276673881281215081281335021283403951407511861407631851407678912751Hexacosanoyl-CoA HMDB0006459Hexacosanoyl-CoA, also known as 26:0 CoA(4-) or cerotoyl-CoA(4-), belongs to the class of organic compounds known as very long-chain fatty acyl coas. These are acyl CoAs where the group acylated to the coenzyme A moiety is a very long aliphatic chain of 22 carbon atoms or more. Hexacosanoyl-CoA is considered to be a practically insoluble (in water) and relatively neutral molecule. hexacosanoyl CoA is an intermediate in Biosynthesis of fatty acids. hexacosanoyl CoA (26:O CoA) oxidation was detected in peroxisomal and. mitochondrial fractions.252023356486828184701CCCCCCCCCCCCCCCCCCCCCCCCCC(=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=C2NC47H86N7O17P3SInChI=1S/C47H86N7O17P3S/c1-4-5-6-7-8-9-10-11-12-13-14-15-16-17-18-19-20-21-22-23-24-25-26-27-38(56)75-31-30-49-37(55)28-29-50-45(59)42(58)47(2,3)33-68-74(65,66)71-73(63,64)67-32-36-41(70-72(60,61)62)40(57)46(69-36)54-35-53-39-43(48)51-34-52-44(39)54/h34-36,40-42,46,57-58H,4-33H2,1-3H3,(H,49,55)(H,50,59)(H,63,64)(H,65,66)(H2,48,51,52)(H2,60,61,62)/t36-,40-,41-,42+,46-/m1/s1FHLYYFPJDVYWQH-CPIGOPAHSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-2-({[({[(3R)-3-[(2-{[2-(hexacosanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]-3-hydroxy-2,2-dimethylpropoxy](hydroxy)phosphoryl}oxy)(hydroxy)phosphoryl]oxy}methyl)-4-hydroxyoxolan-3-yl]oxy}phosphonic acid1146.2091145.501374587-4.679cerotoyl-coa0-4FDB023919C26:0 coa;C26:0 coenzyme a;26:0 coa(4-);C26:0-coa(4-);C26:0-coenzyme a(4-);Cerotoyl-coa(4-);Cerotoyl-coenzyme a(4-);Hexacosanoyl-coa;Hexacosanoyl-coenzyme a(4-)PW_C002751HexCoA2611226165775511327757033480621118806423741225151241225254081266682991266774821282483881282575021034Adenosine diphosphateHMDB0001341Adenosine diphosphate, abbreviated ADP, is a nucleotide. It is an ester of pyrophosphoric acid with the nucleotide adenine. ADP consists of the pyrophosphate group, the pentose sugar ribose, and the nucleobase adenine. ADP is the product of ATP dephosphorylation by ATPases. ADP is converted back to ATP by ATP synthases.58-64-0C00008602216761ADP5800NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1OC10H15N5O10P2InChI=1S/C10H15N5O10P2/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(24-10)1-23-27(21,22)25-26(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1XTWYTFMLZFPYCI-KQYNXXCUSA-N[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]phosphonic acid427.2011427.029414749-2.126adenosine-diphosphate0-2FDB021817Adp;Adenosindiphosphorsaeure;Adenosine 5'-pyrophosphate;Adenosine diphosphate;Adenosine pyrophosphate;Adenosine-5'-diphosphate;Adenosine-5-diphosphate;Adenosine-diphosphate;5'-adenylphosphoric acid;Adenosine 5'-diphosphate;H3adp;5'-adenylphosphate;Adenosine 5'-diphosphoric acid;Adenosine-5'-diphosphoric acidPW_C001034ADP234134841522482138015963159783106114151821901492104182113102161582408592435272728472736462855293165723635614400234476314770915036265157752089752171005315111534911253921035446120554412955721335624108574111757641015849143585614658781075899147592615160501556111161623116664951786700946841188687216071592057187206720821072262137231211730019873032167391217741021874331637483222818722511851277119051701201328112180285132622231532930842328315423983134262232242696318770292537708713277216134773063297747233377663336780393327804335078170128782153517824435378414335784951157870533178849130789203348003036880622118806511358067611994827124113283388116204109119944122119994406120156407120318382120366412121248429121394123121399433121472408121899383121976410122064125122085405122405422122445435122973399123013446123818464123953447123958468124030374124452398124529444124615136124636376124947472124975375125012470125334297125373479125492299125517481125645484126125485126219300126235495126242478126550491126597499126915501127733516127780395127797390127803209128122508128168517128313389423MagnesiumHMDB0000547Magnesium salts are essential in nutrition, being required for the activity of many enzymes, especially those concerned with oxidative phosphorylation. Physiologically, it exists as an ion in the body. It is a component of both intra- and extracellular fluids and is excreted in the urine and feces. Deficiency causes irritability of the nervous system with tetany, vasodilatation, convulsions, tremors, depression, and psychotic behavior. Magnesium ion in large amounts is an ionic laxative, and magnesium sulfate (Epsom salts) is sometimes used for this purpose. So-called "milk of magnesia" is a water suspension of one of the few insoluble magnesium compounds, magnesium hydroxide; the undissolved particles give rise to its appearance and name. Milk of magnesia is a mild base, and is commonly used as an antacid.22537-22-0C003058881842013-HYDROXY-MAGNESIUM-PROTOPORP865DB01378[Mg++]MgInChI=1S/Mg/q+2JLVVSXFLKOJNIY-UHFFFAOYSA-Nmagnesium(2+) ion24.30523.9850418980magnesium(2+) ion22FDB003518Magnesium;Magnesium ions;Magnesium ion;Magnesium, doubly charged positive ion;Magnesium, ion (mg(2+));Mg(2+);Mg2+PW_C000423Mg2+868227426816476272726811581918883229363998339922111674614834915294317641421241024115929422331262933737454031477491486954497456525310453291115356112537610359061475934151603815560941616250166648417865941646881160697919971702057194206722721372332117250214731021673131987473222117631321184321012312225123242491251328812581226127292901527528515337308771371337723632977937336783933347841733578489115785223317853635678574130800203688004518480048372806231188065413580865158096525381841519383238394900271085962231105593901156873981199744061200701221202473821207024071209814081211811241212654291213194191219241251220864051224084221227591201229213991233071191235463741238354641238894551244771361246373761249783751254472971255984841256694791257774811259214821259472991259734951260004901262434781265534911267533001271253891271645011273805021274073881274515071278042091281255081283473951407738911340Tetracosanoic acidHMDB0002003Lignoceric acid, also tetracosanoic acid, is a normal carboxylic acid, a very long chain fatty acid (VLCFA). Very long chain fatty acids (VLCFAs) are exclusively oxidized in peroxisomes and their levels are significantly increased in tissues of patients with peroxisomal disorders. (PMID 15565636).557-59-5C083201119728866TETRACOSANOATE10724CCCCCCCCCCCCCCCCCCCCCCCC(O)=OC24H48O2InChI=1S/C24H48O2/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-16-17-18-19-20-21-22-23-24(25)26/h2-23H2,1H3,(H,25,26)QZZGJDVWLFXDLK-UHFFFAOYSA-Ntetracosanoic acid368.6367368.36543078-7.211lignoceric acid0-1FDB021823Lignocerate;Lignoceric acid;N-tetracosanoate;N-tetracosanoic acid;Tetracosanoate;Tetracosanoic acid;Ch3-[ch2]22-cooh;Lignozerinsaeure;Tetracosanic acid;Tetracosansaeure;Tetracosoic acid;Tetraeicosanoic acid;Tetraicosanoic acid;Tetracosanate;Tetracosoate;Tetraeicosanoate;Tetraicosanoate;FA(24:0)PW_C001340cosan4a2614277553132806251181225171241266702991282503882794Tetracosanoyl-CoA HMDB0006526Tetracosanoyl-CoA, also known as S-tetracosanoate or lignoceroyl-CoA, belongs to the class of organic compounds known as very long-chain fatty acyl coas. These are acyl CoAs where the group acylated to the coenzyme A moiety is a very long aliphatic chain of 22 carbon atoms or more. Tetracosanoyl-CoA is considered to be a practically insoluble (in water) and relatively neutral molecule. Tetracosanoyl-CoA has been primarily detected in urine. Within the cell, tetracosanoyl-CoA is primarily located in the membrane (predicted from logP), peroxisome and cytoplasm. In humans, tetracosanoyl-CoA is involved in the adrenoleukodystrophy, X-linked pathway and the Beta oxidation OF very long chain fatty acids pathway. Tetracosanoyl-CoA is also involved in several metabolic disorders, some of which include de novo triacylglycerol biosynthesis TG(24:0/22:0/24:1(15Z)) pathway, de novo triacylglycerol biosynthesis TG(18:4(6Z,9Z,12Z,15Z)/24:0/20:4(8Z,11Z,14Z,17Z)) pathway, de novo triacylglycerol biosynthesis TG(24:0/22:4(7Z,10Z,13Z,16Z)/24:0) pathway, and de novo triacylglycerol biosynthesis TG(24:0/22:4(7Z,10Z,13Z,16Z)/20:4(5Z,8Z,11Z,14Z)) pathway. Tetracosanoyl-CoA is an intermediate in the biosynthesis of unsaturated fatty acids. Tetracosanoyl-CoA is converted from Palmitoyl-CoA in multiple steps. It is then converted to lignoceric acid via a thiol-ester hydrolase (E 3.1.2.-).24305-30-4C16529 308222752974[H][C@](O)(C(=O)NCCC(=O)NCCSC(=O)CCCCCCCCCCCCCCCCCCCCCCC)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@@H](O)C1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC45H82N7O17P3SInChI=1S/C45H82N7O17P3S/c1-4-5-6-7-8-9-10-11-12-13-14-15-16-17-18-19-20-21-22-23-24-25-36(54)73-29-28-47-35(53)26-27-48-43(57)40(56)45(2,3)31-66-72(63,64)69-71(61,62)65-30-34-39(68-70(58,59)60)38(55)44(67-34)52-33-51-37-41(46)49-32-50-42(37)52/h32-34,38-40,44,55-56H,4-31H2,1-3H3,(H,47,53)(H,48,57)(H,61,62)(H,63,64)(H2,46,49,50)(H2,58,59,60)/t34-,38+,39?,40+,44-/m1/s1MOYMQYZWIUKGGY-LIEWTTMESA-N{[(2R,4S,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[(3R)-3-hydroxy-2,2-dimethyl-3-[(2-{[2-(tetracosanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid1118.1561117.470074459-4.329[(2R,4S,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3R)-3-hydroxy-2,2-dimethyl-3-[(2-{[2-(tetracosanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxyphosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid0-4FDB023959Lignoceroyl-CoA;Lignoceroyl-coenzyme A;S-tetracosanoate;S-tetracosanoate CoA;S-tetracosanoate coenzyme A;S-tetracosanoic acid;Tetracosanoyl-coenzyme A;CoA(24:0)PW_C002794Tetraco261522619525964492997922775541327756933480626118806413748189733187432329957543831011953821225181241225244081266712991266764821282513881282565021288953981171Tetradecanoyl-CoA HMDB0001521Tetradecanoyl-CoA (or myristoyl-CoA) is an intermediate in fatty acid biosynthesis, fatty acid elongation and the beta oxidation of fatty acids. It is also used in the myristoylation of proteins. The first pass through the beta-oxidation process starts with the saturated fatty acid palmitoyl-CoA and produces myristoyl-CoA. A total of four enzymatic steps are required, starting with VLCAD CoA dehydrogenase (Very Long Chain) activity, followed by three enzymatic steps catalyzed by enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and ketoacyl-CoA thiolase, all present in the mitochondria. Myristoylation of proteins is also catalyzed by the presence of myristoyl-CoA along with Myristoyl-CoA:protein N-myristoyltransferase (NMT). Myristoylation is an irreversible, co-translational (during translation) protein modification found in animals, plants, fungi and viruses. In this protein modification a myristoyl group (derived from myristioyl CoA) is covalently attached via an amide bond to the alpha-amino group of an N-terminal amino acid of a nascent polypeptide. It is more common on glycine residues but also occurs on other amino acids. Myristoylation also occurs post-translationally, for example when previously internal glycine residues become exposed by caspase cleavage during apoptosis. Myristoylation plays a vital role in membrane targeting and signal transduction in plant responses to environmental stress. Compared to other species that possess a single functional myristoyl-CoA: protein N-myristoyltransferase (NMT) gene copy, human, mouse and cow possess 2 NMT genes, and more than 2 protein isoforms.3130-72-1C025936511315532TETRADECANOYL-COA58623CCCCCCCCCCCCCC(=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=C2NC35H62N7O17P3SInChI=1S/C35H62N7O17P3S/c1-4-5-6-7-8-9-10-11-12-13-14-15-26(44)63-19-18-37-25(43)16-17-38-33(47)30(46)35(2,3)21-56-62(53,54)59-61(51,52)55-20-24-29(58-60(48,49)50)28(45)34(57-24)42-23-41-27-31(36)39-22-40-32(27)42/h22-24,28-30,34,45-46H,4-21H2,1-3H3,(H,37,43)(H,38,47)(H,51,52)(H,53,54)(H2,36,39,40)(H2,48,49,50)/t24-,28-,29-,30+,34-/m1/s1DUAFKXOFBZQTQE-QSGBVPJFSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[(3R)-3-hydroxy-2,2-dimethyl-3-[(2-{[2-(tetradecanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid977.89977.313573819-2.659S-tetradecanoyl-coenzyme A0-4FDB022670Myristoyl-coa;Myristoyl-coenzyme a;N-c14:0coa;N-c14:0coenzyme a;S-tetradecanoyl-coenzyme a;Tetradecanoyl coa;Tetradecanoyl coenzyme a;Tetradecanoyl-coenzyme a;CoA(14:0)PW_C001171COA14:08713902426235528710369681617125163756821025685492595222772261337788911281816329818193319569538295698383110852389114199390120239406120660407122912120123274119125587479127117501128837399128840398140761185940Acetyl-CoAHMDB0001206The main function of coenzyme A is to carry acyl groups (such as the acetyl group) or thioesters. Acetyl-CoA is an important molecule itself. It is the precursor to HMG CoA, which is a vital component in cholesterol and ketone synthesis. (wikipedia). acetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.72-89-9C0002444449315351ACETYL-COA392413CC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC23H38N7O17P3SInChI=1S/C23H38N7O17P3S/c1-12(31)51-7-6-25-14(32)4-5-26-21(35)18(34)23(2,3)9-44-50(41,42)47-49(39,40)43-8-13-17(46-48(36,37)38)16(33)22(45-13)30-11-29-15-19(24)27-10-28-20(15)30/h10-11,13,16-18,22,33-34H,4-9H2,1-3H3,(H,25,32)(H,26,35)(H,39,40)(H,41,42)(H2,24,27,28)(H2,36,37,38)/t13-,16-,17-,18+,22-/m1/s1ZSLZBFCDCINBPY-ZSJPKINUSA-N{[(2R,3S,4R,5R)-2-({[({[(3R)-3-[(2-{[2-(acetylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]-3-hydroxy-2,2-dimethylpropoxy](hydroxy)phosphoryl}oxy)(hydroxy)phosphoryl]oxy}methyl)-5-(6-amino-9H-purin-9-yl)-4-hydroxyoxolan-3-yl]oxy}phosphonic acid809.571809.125773051-2.279acetyl-CoA0-4FDB022491Ac-coa;Ac-coenzyme a;Ac-s-coa;Ac-s-coenzyme a;Acetyl coenzyme-a;Acetyl-coa;Acetyl-coenzyme a;Acetyl-s-coa;Acetyl-s-coenzyme a;Acetylcoenzyme-a;S-acetate coa;S-acetate coenzyme a;S-acetyl coenzyme a;Accoa;Acetyl coenzyme a;S-acetyl-coa;S-acetyl-coenzyme a;Acetylcoenzyme aPW_C000940Ac-CoA21343858842324162244652896173340114840145278103547612457331086025155607716163861647017869231607106163729119874602228245151827721012582226130122994261531577121133772911117756211277706132779941157835513478433334800073688063411980663376901241701199534061201454051203041221206324071224174081226263841227431201229591351231371181249863741252001211253434791255074781256332971265644821265724811267784801268865011270442091273942051276653881281375021281452061283743911407621851928Lauroyl-CoAHMDB0003571Lauroyl-CoA, also known as dodecanoyl-CoA or C12:0-CoA, belongs to the class of organic compounds known as 2,3,4-saturated fatty acyl coas. These are acyl-CoAs carrying a 2,3,4-saturated fatty acyl chain. Thus, lauroyl-CoA is considered to be a fatty ester lipid molecule. Lauroyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). Lauroyl-CoA has been primarily detected in urine. Within the cell, lauroyl-CoA is primarily located in the cytoplasm and peroxisome. Lauroyl-CoA exists in all living organisms, ranging from bacteria to humans. Lauroyl-CoA participates in a number of enzymatic reactions. In particular, Acetyl-CoA and lauroyl-CoA can be biosynthesized from 3-oxodecanoyl-CoA; which is mediated by the enzyme acetyl-CoA C-acyltransferase. In addition, Lauroyl-CoA can be biosynthesized from dodecanoic acid through the action of the enzyme short chain acyl-CoA synthetase. In humans, lauroyl-CoA is involved in the Beta oxidation OF very long chain fatty acids pathway, the fatty acid metabolism pathway, and the adrenoleukodystrophy, X-linked pathway. Lauroyl-CoA is also involved in several metabolic disorders, some of which include de novo triacylglycerol biosynthesis TG(i-22:0/12:0/i-17:0) pathway, de novo triacylglycerol biosynthesis TG(i-24:0/i-24:0/12:0) pathway, de novo triacylglycerol biosynthesis TG(i-22:0/13:0/12:0) pathway, and de novo triacylglycerol biosynthesis TG(22:0/a-13:0/12:0) pathway. Lauroyl-CoA is a substrate for Protein FAM34A.6244-92-4C0183216543615521LAUROYLCOA-CPD145018CCCCCCCCCCCC(=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=C2NC33H58N7O17P3SInChI=1S/C33H58N7O17P3S/c1-4-5-6-7-8-9-10-11-12-13-24(42)61-17-16-35-23(41)14-15-36-31(45)28(44)33(2,3)19-54-60(51,52)57-59(49,50)53-18-22-27(56-58(46,47)48)26(43)32(55-22)40-21-39-25-29(34)37-20-38-30(25)40/h20-22,26-28,32,43-44H,4-19H2,1-3H3,(H,35,41)(H,36,45)(H,49,50)(H,51,52)(H2,34,37,38)(H2,46,47,48)/t22-,26-,27-,28+,32-/m1/s1YMCXGHLSVALICC-GMHMEAMDSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-2-({[({[(3R)-3-[(2-{[2-(dodecanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]-3-hydroxy-2,2-dimethylpropoxy](hydroxy)phosphoryl}oxy)(hydroxy)phosphoryl]oxy}methyl)-4-hydroxyoxolan-3-yl]oxy}phosphonic acid949.837949.282273691-2.569lauroyl-coa0-4FDB023198Dodecanoyl-coa;Dodecanoyl-coenzyme a;Lauroyl coenzyme a;C12:0-coa;Coenzyme a, s-dodecanoate;Lauroyl-coenzyme a;CoA(12:0)PW_C001928COA12:0867390842622526742528810369841617121163259212225924497724013377566132778851128063811890808210120252406120656407122521124122926120123270119125603479126674299127130501128254388140777185497Dodecanoic acidHMDB0000638Lauric acid, or dodecanoic acid is the main fatty acid in coconut oil and in palm kernel oil, and is believed to have antimicrobial properties. It is a white, powdery solid with a faint odor of bay oil. Lauric acid, although slightly irritating to mucous membranes, has a very low toxicity and so is used in many soaps and shampoos.143-07-7C026793893308053756DB03017CCCCCCCCCCCC(O)=OC12H24O2InChI=1S/C12H24O2/c1-2-3-4-5-6-7-8-9-10-11-12(13)14/h2-11H2,1H3,(H,13,14)POULHZVOKOAJMA-UHFFFAOYSA-Ndodecanoic acid200.3178200.177630012-4.301lauric acid0-1FDB0030101-undecanecarboxylate;1-undecanecarboxylic acid;Abl;Aliphat no. 4;Dodecanoate;Dodecanoic acid;Dodecylate;Dodecylic acid;Edenor c 1298-100;Emery 651;Hystrene 9512;Kortacid 1299;Laurate;Lauric acid;Laurostearate;Laurostearic acid;Lunac l 70;Lunac l 98;N-dodecanoate;N-dodecanoic acid;Neo-fat 12;Neo-fat 12-43;Nissan naa 122;Philacid 1200;Prifac 2920;Univol u 314;Vulvate;Vulvic acid;Dodecylcarboxylate;Dodecylcarboxylic acid;Laate;Laic acid;FA(12:0)PW_C00049712:02671526722267336949160829921077556334775651127757113280628374806371198064311812247612412251940812252040712662929912667248212667348112820438812825250212825320644L-CarnitineHMDB0000062Carnitine is not an essential amino acid; it can be synthesized in the body. However, it is so important in providing energy to muscles (including the heart) that some researchers are now recommending carnitine supplements in the diet, particularly for people who do not consume much red meat (the main food source for carnitine). Carnitine has been described as a vitamin, an amino acid, or a metabimin (i.e. an essential metabolite). Like the B vitamins, carnitine contains nitrogen and is very soluble in water, and to some researchers carnitine is a vitamin (Liebovitz 1984). It was found that an animal (yellow mealworm) could not grow without carnitine in its diet. However, as it turned out, almost all other animals, including humans, do make their own carnitine; thus, it is no longer considered a vitamin. Nevertheless, in certain circumstances, such as deficiencies of methionine, lysine, or vitamin C or kidney dialysis, carnitine shortages develop. Under these conditions, carnitine must be absorbed from food, and for this reason it is sometimes referred to as a "metabimin" or a conditionally essential metabolite. Like the other amino acids used or manufactured by the body, carnitine is an amine. But like choline, which is sometimes considered to be a B vitamin, carnitine is also an alcohol (specifically, a trimethylated carboxy-alcohol). Thus, carnitine is an unusual amino acid and has different functions than most other amino acids, which are usually employed by the body in the construction of protein. Carnitine is an essential factor in fatty acid metabolism in mammals. Its most important known metabolic function is to transport fat into the mitochondria of muscle cells, including those in the heart, for oxidation. This is how the heart gets most of its energy. In humans, about 25% of carnitine is synthesized in the liver, kidney, and brain from the amino acids lysine and methionine. Most of the carnitine in the body comes from dietary sources such as red meat and dairy products. Inborn errors of carnitine metabolism can lead to brain deterioration like that of Reye's syndrome, gradually worsening muscle weakness, Duchenne-like muscular dystrophy, and extreme muscle weakness with fat accumulation in muscles. Borum et al. (1979) describe carnitine as an essential nutrient for pre-term babies and individuals who are unable to eat a normal diet (e.g. non-ketotic hypoglycemics, kidney dialysis patients) (PMID: 115309). In conditions such as kwashiorkor, cirrhosis, and heart muscle disease (cardiomyopathy) as well as in inborn errors of metabolism such as type IV hyperlipidemia and propionic or organic aciduria (acid urine resulting from genetic or other anomalies), carnitine is essential to life and carnitine supplements are valuable. Carnitine therapy may also be useful in a wide variety of clinical conditions. Carnitine supplementation has improved some patients who have angina secondary to coronary artery disease. It may also be worth a trial for patients with any form of hyperlipidemia or muscle weakness. Carnitine supplements may also be useful in many forms of toxic or metabolic liver disease and in cases of heart muscle disease. Hearts undergoing severe arrhythmia quickly deplete their stores of carnitine. Athletes, particularly in Europe, have used carnitine supplements for improved endurance. Carnitine may improve muscle building by improving fat utilization and may even be useful in treating obesity. Carnitine joins a long list of nutrients which may be of value in treating pregnant women, hypothyroid individuals, and male infertility due to the low motility of sperm. Carnitine deficiency is noted in abnormal liver function, renal dialysis patients, and severe to moderate muscular weakness with associated anorexia (http://www.dcnutrition.com). Carnitine is a biomarker for the consumption of meat.541-15-1C003181091716347CARNITINE10455DB00583C[N+](C)(C)C[C@H](O)CC([O-])=OC7H15NO3InChI=1S/C7H15NO3/c1-8(2,3)5-6(9)4-7(10)11/h6,9H,4-5H2,1-3H3/t6-/m1/s1PHIQHXFUZVPYII-ZCFIWIBFSA-N(3R)-3-hydroxy-4-(trimethylazaniumyl)butanoate161.1989161.105193351-1.601L-carnitine00FDB000572(-)-(r)-3-hydroxy-4-(trimethylammonio)butyrate;(-)-carnitine;(r)-(3-carboxy-2-hydroxypropyl)trimethylammonium hydroxide;(r)-carnitine;(s)-carnitine;1-carnitine;3-carboxy-2-hydroxy-n,n,n-trimethyl-1-propanaminium;3-hydroxy-4-trimethylammoniobutanoate;3-hydroxy-4-trimethylammoniobutanoic acid;Bicarnesine;Carniking;Carniking 50;Carnilean;Carnipass;Carnipass 20;Carnitene;Carnitine;Carnitor;D-carnitine;Dl-carnitine;Karnitin;L-(-)-carnitine;L-carnitine;L-gamma-trimethyl-beta-hydroxybutyrobetaine;Levocarnitina;Levocarnitine;Levocarnitinum;R-(-)-3-hydroxy-4-trimethylaminobutyrate;Vitamin bt;Delta-carnitine;Gamma-trimethyl-ammonium-beta-hydroxybutirate;Gamma-trimethyl-beta-hydroxybutyrobetaine;Gamma-trimethyl-hydroxybutyrobetaine;(-)-l-carnitine;3-carboxy-2-hydroxy-n,n,n-trimethyl-1-propanaminium hydroxide, inner salt;CarnicorPW_C000044L-Carnt67531885228911724575251932675228858288745230102524510469591626971199772201347722832977558334775611127756713277748129783391117834513379258333806303748063311980639118120229384120241382120554414122412408122421407122522124122617122122621406122902121122915399123188450125191135125195120125577480125590484126558482126569481126675299126769297126773479127107391127119389128131502128142206128255388128364205128368501140749186140765891129L-AcetylcarnitineHMDB0000201L-Acetylcarnitine (ALCAR or ALC) is an acetic acid ester of carnitine that facilitates movement of acetyl-CoA into the matrices of mammalian mitochondria during the oxidation of fatty acids. In addition to his metabolic role, acetyl-L-carnitine posses unique neuroprotective, neuromodulatory, and neurotrophic properties this may play an important role in counteracting various disease processes (PMID ID: 15363640).3040-38-8C02571173024(-)o-acetylcarnitine21243783CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)CC9H17NO4InChI=1S/C9H17NO4/c1-7(11)14-8(5-9(12)13)6-10(2,3)4/h8H,5-6H2,1-4H3/t8-/m1/s1RDHQFKQIGNGIED-MRVPVSSYSA-N(3R)-3-(acetyloxy)-4-(trimethylazaniumyl)butanoate203.238203.115758031-2.860acetyl-L-carnitine00FDB021904(+-)-acetylcarnitine;(-)-acetylcarnitine;(r)-acetylcarnitine;Alcar;Acetyl-l-(-)-carnitine;Acetyl-l-carnitine;Acetyl-carnitine;Acetylcarnitine;L-acetylcarnitine;L-carnitine acetyl ester;L-o-acetylcarnitine;Levocarnitine acetyl;Nicetile;O-acetyl-l-carnitine;O-acetylcarnitine;3-(acetyloxy)-4-(trimethylammonio)butanoate;Acetyl-dl-carnitine;Dl-o-acetylcarnitine;3-(acetyloxy)-4-(trimethylammonio)butanoic acidPW_C000129L-Alcar24615247022506422515477559334775601337757313278441345806313748063212080645118122418408122424406122431418122527124124997454126565482126573479126579489126679299128138502128146501128152506128258388546Long-chain-fatty-acid--CoA ligase 1P33121Activation of long-chain fatty acids for both synthesis of cellular lipids, and degradation via beta-oxidation. Preferentially uses palmitoleate, oleate and linoleate.
HMDBP00577ACSL14q35CH47105616.2.1.388422241259261225254104132636166135628329137847713141136315714ATP-binding cassette sub-family D member 1P33897Z3100618448595715ATP-binding cassette sub-family D member 2Q9UBJ2BC1049011844959301Peroxisomal 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.
HMDBP00307CROT7q21.1CH23694912.3.1.13724665252235722Peroxisomal membrane protein 11CQ96HA9Peroxisomal biogenesis factor 11CAL833945184765928Carnitine 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.
HMDBP00028CRAT9q34.1X7982712.3.1.7246052520314105380214112845712Peroxisomal membrane protein PEX13Q92968AF04875518473595713Peroxisomal membrane protein PEX14O75381AF04518618474592100Mitochondrial 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, mitochondrialP23786HMDBP00311CPT21p32U0964312.3.1.21893172670326762523310213562213413785271314105261410638021411305314113155246Long-chain-fatty-acid--CoA ligase 11PW_P00024626554611134231636ATP-binding cassette sub-family D1PW_P0006366875714168857151629Peroxisomal carnitine O-octanoyltransferase1PW_P0006296753011673Peroxisomal membrane protein 11C1PW_P00067374757221628Carnitine O-acetyltransferase1PW_P000628674281620Peroxisomal membrane protein1PW_P0006206645712166557131644Mitochondrial carnitine/acylcarnitine carrier protein1PW_P00064470221001248Carnitine O-palmitoyltransferase 2, 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227 C127 177 177 127 227 127 C1575 127 3329 127 4677 127 C4727 127 4777 177 4777 227 C4777 1135 4777 2316 4777 3224 C4777 3274 4727 3324 4677 3324 C3329 3324 1575 3324 227 3324 C177 3324 127 3274 127 3224 C127 2316 127 1135 127 227 1true64650.03197.0323M819 1264 C819 1214 869 1164 919 1164 C1447 1164 2132 1164 2660 1164 C2710 1164 2760 1214 2760 1264 C2760 1670 2760 2197 2760 2603 C2760 2653 2710 2703 2660 2703 C2132 2703 1447 2703 919 2703 C869 2703 819 2653 819 2603 C819 2197 819 1670 819 1264 94true61941.01539.0324M3340 1474 C3340 1424 3390 1374 3440 1374 C3739 1374 4129 1374 4428 1374 C4478 1374 4528 1424 4528 1474 C4528 1933 4528 2531 4528 2990 C4528 3040 4478 3090 4428 3090 C4129 3090 3739 3090 3440 3090 C3390 3090 3340 3040 3340 2990 C3340 2531 3340 1933 3340 1474 84true61188.01716.0327M3212 1397 C3212 1347 3262 1297 3312 1297 C3680 1297 4157 1297 4525 1297 C4575 1297 4625 1347 4625 1397 C4625 1905 4625 2566 4625 3074 C4625 3124 4575 3174 4525 3174 C4157 3174 3680 3174 3312 3174 C3262 3174 3212 3124 3212 3074 C3212 2566 3212 1905 3212 1397 84true61413.01877.0308235Intracellular space395225201.61.620015309235Extracellular space3905201.61.620015310235Peroxisome3051425201.91.920015311235Mitochondria33751230201.61.62001532015Mitochondrial Inner Membrane32451415201.61.61601532115Mitochondrial Outer Membrane30701310201.61.616015890886235Mitochondrial Matrix39732460202.22.220015130580167883211812742268339#FBEBFC4191015021313881108432231319460931574#FFEBEB413861838Adrenoleukodystrophy, X-LinkedAdrenoleukodystrophy (ALD) is an X-linked recessive transmission disease. Central nervous system signs and symptoms have been consistently more prominent than signs of adrenal involvement. Behavioral changes are the most common initial finding and range from aggressive outbursts to withdrawal. Such behavior is generally accompanied by a gradually failing memory and poor school performance. Loss of vision is an early finding in some patients and is a prominent feature at some stage in most affected individuals. The initial visual loss appears as homonomous hemianopsia in some individuals and is usually associated with intact pupillary reflexes. Optic atrophy is less common as an initial finding but eventually develops in almost all cases. Gait disturbance is also an early finding and as is stiff-legged, unsteady and accompanied by hyperreflexia. In almost all cases there is spastic quadraplegia and a variable degree of decorticate posturing. Hearing loss, dysarthria and dysphagia develop at about the same time as gait disturbance. Seizures are a typical symptom in many affected individuals in the the end stages of the disease progression.DiseasePW_X000201Context20110215714ProteinMutated102249CompoundIncreased10231589CompoundIncreased137314TissueDamaged137424TissueDamaged1625[Metagen: ADRENOLEUKODYSTROPHY, X-LINKED](http://metagene.de/program/d.prg?id_d=199)201Context1626[OMIM: 300100](http://omim.org/entry/300100})201Context1819[NIH](http://ghr.nlm.nih.gov/condition/x-linked-adrenoleukodystrophy)201Context27829120301491Raymond GV, Moser AB, Fatemi A: X-Linked Adrenoleukodystrophy 201Context2793519256488Lu JF, Lawler AM, Watkins PA, Powers JM, Moser AB, Moser HW, Smith KD: A mouse model for X-linked adrenoleukodystrophy. Proc Natl Acad Sci U S A. 1997 Aug 19;94(17):9366-71. doi: 10.1073/pnas.94.17.9366.201Context