19PathwayBetaine MetabolismBetaine (or trimethylglycine) is similar to choline (trimethylaminoethanol) but differs in choline's terminal carboxylic acid group trimethylglycine is reduced to a hydroxyl group. Betaine is obtained from diet as betaine or compounds containing choline in foods such as whole grains, beets and spinach. Betaine can also be synthesized from choline in the liver and kidney. First, choline is oxidized to betaine aldehyde by mitochondrial choline oxidase (choline dehydrogenase). Then, betaine aldehyde dehydrogenase oxidizes betaine aldehyde to betaine in the mitochondria or cytoplasm. In the liver, betaine functions as a methyl donor similar to choline, folic acid, S-adenosyl methionine and vitamin B12. Methyl donors are important for liver function, cellular replication and detoxification reactions. Betaine is also involved in the production of carnitine to protect from kidney damage and functions as an osmoprotectant in the inner medulla. MetabolicPW000012CenterPathwayVisualizationContext1222502800#000099PathwayVisualization1719Betaine MetabolismBetaine (or trimethylglycine) is similar to choline (trimethylaminoethanol) but differs in choline's terminal carboxylic acid group trimethylglycine is reduced to a hydroxyl group. Betaine is obtained from diet as betaine or compounds containing choline in foods such as whole grains, beets and spinach. Betaine can also be synthesized from choline in the liver and kidney. First, choline is oxidized to betaine aldehyde by mitochondrial choline oxidase (choline dehydrogenase). Then, betaine aldehyde dehydrogenase oxidizes betaine aldehyde to betaine in the mitochondria or cytoplasm. In the liver, betaine functions as a methyl donor similar to choline, folic acid, S-adenosyl methionine and vitamin B12. Methyl donors are important for liver function, cellular replication and detoxification reactions. Betaine is also involved in the production of carnitine to protect from kidney damage and functions as an osmoprotectant in the inner medulla. Metabolic116One Carbon Pool by FolateSubPathway271221Compound8281079Compound8312Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.19Pathway313Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.19Pathway27975226850693Day CR, Kempson SA: Betaine chemistry, roles, and potential use in liver disease. Biochim Biophys Acta. 2016 Jun;1860(6):1098-106. doi: 10.1016/j.bbagen.2016.02.001. Epub 2016 Feb 2.19Pathway1CellCL:00000005HepatocyteCL:00001824CardiomyocyteCL:00007463NeuronCL:00005407Epithelial CellCL:00000662Platelet CL:00002338Beta cellCL:00006396MyocyteCL:000018718ErythrocyteCL:00002321Homo sapiens9606EukaryoteHuman17Rattus norvegicus10116EukaryoteRat4Arabidopsis thaliana3702EukaryoteThale cress12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle10Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm2Bacteria2ProkaryoteBacteria3Escherichia coli562Prokaryote19Schizosaccharomyces pombe4896Eukaryote24Solanum lycopersicum4081EukaryoteTomato18Saccharomyces cerevisiae4932EukaryoteYeast21Xenopus laevis8355EukaryoteAfrican clawed frog23Pseudomonas aeruginosa287Prokaryote60Nitzschia sp.0001EukaryoteNitzschia425Escherichia coli (strain K12)83333Prokaryote49Bathymodiolus platifrons220390EukaryoteDeep sea mussel29Saccharomyces cerevisiae (strain ATCC 204508 / S288c)559292EukaryoteBaker's yeast51Picea sitchensis3332EukaryoteSitka spruce196Homo1924EukaryoteHuman11Extracellular SpaceGO:00056155CytoplasmGO:00057372MitochondrionGO:00057391CytosolGO:00058294PeroxisomeGO:00057773Mitochondrial MatrixGO:00057597Endoplasmic Reticulum MembraneGO:000578925Golgi ApparatusGO:000579412Mitochondrial Inner MembraneGO:00057436LysosomeGO:000576413Endoplasmic ReticulumGO:000578316Lysosomal LumenGO:004320235ChloroplastGO:000950710Cell MembraneGO:000588618Melanosome MembraneGO:003316214Mitochondrial Outer MembraneGO:000574120Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520215NucleusGO:000563431Periplasmic SpaceGO:000562036MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032540PeriplasmGO:004259727Peroxisome MembraneGO:000577832Inner MembraneGO:007025817NucleoplasmGO:000565439Mitochondrial membraneGO:003196619Sarcoplasmic ReticulumGO:001652924Mitochondrial Intermembrane SpaceGO:000575826Golgi Apparatus MembraneGO:00001398Smooth Endoplasmic Reticulum GO:00057901LiverBTO:00007597294Adrenal MedullaBTO:000004971825IntestineBTO:000064828StomachBTO:0001307155267Nervous SystemBTO:00014848Blood VesselBTO:0001102741111HeartBTO:000056273106KidneyBTO:00006717182Endothelium BTO:000039318PancreasBTO:00009889MuscleBTO:0000887141185cardiocyteBTO:00015393Sympathetic Nervous 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(or N,N,N-trimethylglycine) was named after its discovery in sugar beet (Beta vulgaris) in the 19th century. It is a small N-trimethylated amino acid, existing in zwitterionic form at neutral pH. It is now often called glycine betaine to distinguish it from other betaines that are widely distributed in microorganisms, plants, and animals. Many naturally occurring betaines serve as organic osmolytes, substances synthesized or taken up from the environment by cells for protection against osmotic stress, drought, high salinity, or high temperature. Intracellular accumulation of betaines permits water retention in cells, thus protecting from the effects of dehydration (Wikipedia). Betaine functions as a methyl donor in that it carries and donates methyl functional groups to facilitate necessary chemical processes. In particular, it methylates homocysteine to methionine, also producing N,N-dimethylglycine. The donation of methyl groups is important to proper liver function, cellular replication, and detoxification reactions. Betaine also plays a role in the manufacture of carnitine and serves to protect the kidneys from damage. Betaine comes from either the diet or by the oxidation of choline. Betaine insufficiency is associated with metabolic syndrome, lipid disorders, and diabetes, and may have a role in vascular and other diseases (PMID: 20346934). Betaine is important in development, from the pre-implantation embryo to infancy. Betaine is also widely regarded as an anti-oxidant. Betaine has been shown to have an inhibitory effect on NO release in activated microglial cells and may be an effective therapeutic component to control neurological disorders (PMID: 22801281). As a drug, betaine hydrochloride has been used as a source of hydrochloric acid in the treatment of hypochlorhydria. Betaine has also been used in the treatment of liver disorders, for hyperkalemia, for homocystinuria, and for gastrointestinal disturbances (Martindale, The Extra Pharmacopoeia, 30th Ed, p1341).107-43-7C0071924717750BETAINE242C[N+](C)(C)CC([O-])=OC5H11NO2InChI=1S/C5H11NO2/c1-6(2,3)4-5(7)8/h4H2,1-3H3KWIUHFFTVRNATP-UHFFFAOYSA-N117.1463117.078978601FDB009020(carboxymethyl)trimethylammonium hydroxide inner salt;(trimethylammonio)acetate;1-carboxy-n,n,n-trimethyl-methanaminium;1-carboxy-n,n,n-trimethyl-methanaminium hydroxide;1-carboxy-n,n,n-trimethylmethanaminium inner salt;Abromine;Aminocoat;Betafin;Betafin bcr;Betafin bp;Betaine;Cystadane;Ektasolve ee;Finnstim;Glycine betaine;Glycocoll betaine;Glycylbetaine;Greenstim;Loramine amb 13;Loramine amb-13;Lycine;N,n,n-trimethylglycine;Oxyneurine;Rubrine c;Trimethylaminoacetate;Trimethylaminoacetic acid;Trimethylbetaine glycine;Trimethylglycine;Trimethylglycocoll;A-earleine;Alpha-earleine;(trimethylammoniumyl)acetate;2-n,n,n-trimethylammonio acetate;Acidol;Bet;N,n,n-trimethylammonioacetate;Trimethylammonioacetate;(trimethylammoniumyl)acetic acid;2-(trimethylazaniumyl)acetic acid;2-n,n,n-trimethylammonio acetic acid;N,n,n-trimethylammonioacetic acid;Trimethylammonioacetic acidPW_C000030βine558155598189933470255921355618137685071228222677605111776181147810413278315112120470122120496409122150124122294407124702118124847119126309299126461481127871388128031206967βine aldehydeHMDB0001252Betaine aldehyde is an intermediate in the metabolism of glycine, serine and threonine. The human aldehyde dehydrogenase (EC 1.2.1.3) facilitates the conversion of betaine aldehyde to glycine betaine. Betaine aldehyde is a substrate for Choline dehydrogenase (mitochondrial). (PMID: 12467448, 7646513).7418-61-3C0057624915710BETAINE_ALDEHYDE244DB04401C[N+](C)(C)CC=OC5H12NOInChI=1S/C5H12NO/c1-6(2,3)4-5-7/h5H,4H2,1-3H3/q+1SXKNCCSPZDCRFD-UHFFFAOYSA-N102.1549102.091889011FDB022513(formylmethyl)trimethylammonium;(formylmethyl)trimethyl-ammonium;Btl;Betaine aldehyde;Glycine betaine aldehyde;N,n,n-trimethyl-2-oxo ethanaminium;N,n,n-trimethyl-2-oxo-ethanaminium;Trimethyl(formylmethyl)ammoniumPW_C000967BinAld56081898355891351228122677604111120469122721NADHMDB0000902NAD (or Nicotinamide adenine dinucleotide) is used extensively in glycolysis and the citric acid cycle of cellular respiration. The reducing potential stored in NADH can be converted to ATP through the electron transport chain or used for anabolic metabolism. ATP "energy" is necessary for an organism to live. Green plants obtain ATP through photosynthesis, while other organisms obtain it by cellular respiration. (wikipedia). Nicotinamide adenine dinucleotide is a A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed).53-84-9C00003589315846NAD5682NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP([O-])(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1OC21H27N7O14P2InChI=1S/C21H27N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1-4,7-8,10-11,13-16,20-21,29-32H,5-6H2,(H5-,22,23,24,25,33,34,35,36,37)/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BAWFJGJZGIEFAR-NNYOXOHSSA-N663.4251663.109121631FDB0223093-carbamoyl-1-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;3-carbamoyl-1-beta-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-beta-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;Adenine-nicotinamide dinucleotide;Co-i;Codehydrase i;Codehydrogenase i;Coenzyme i;Cozymase;Cozymase i;Diphosphopyridine nucleotide;Diphosphopyridine nucleotide oxidized;Endopride;Nad trihydrate;Nad-oxidized;Nicotinamide adenine dinucleotide;Nicotinamide adenine dinucleotide oxidized;Nicotinamide dinucleotide;Nicotineamide adenine dinucleotide;Oxidized diphosphopyridine nucleotide;Pyridine nucleotide diphosphate;[(3s,2r,4r,5r)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl {[(3s,2r,4r,5r)-5-(3-carbamoylpyridyl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxyphosphoryl) hydrogen phosphate;[adenylate-32-p]-nad;Beta-diphosphopyridine nucleotide;Beta-nad;Beta-nicotinamide adenine dinucleotide;Beta-nicotinamide adenine dinucleotide trihydrate;Dpn;Nad;Nad+;Nadide;B-nad;β-nadPW_C000721NAD140415033538651101114211344312735146654222949277917283529310794807184813184819284902649603151679552381035334111536011254691235482125559013556101185696100573810858271415912147594215160241556072157607616163851646917867721176890160701218870971637174205719720674051987459222824122683592259085224118192161232224913006298130183001325622342404322426193157710413277120133772091347737033177650336776673347770233277709130779151137798334778406356800063688069011993825124110552388112750166112853941199291221199524061201714071208344191209844081211594251212421261212594291218173831226143841227421201231304471231411361234194551235493741237314601238124431238294641243703981251871211253192971253424791255304811258062991258254901259244821265154951267654801268855011272785071273835021280893901283603911284283951407571851420WaterHMDB0002111Water is a chemical substance that is essential to all known forms of life. It appears colorless to the naked eye in small quantities, though it is actually slightly blue in color. It covers 71% of Earth's surface. Current estimates suggest that there are 1.4 billion cubic kilometers (330 million m3) of it available on Earth, and it exists in many forms. It appears mostly in the oceans (saltwater) and polar ice caps, but it is also present as clouds, rain water, rivers, freshwater aquifers, lakes, and sea ice. Water in these bodies perpetually moves through a cycle of evaporation, precipitation, and runoff to the sea. Clean water is essential to human life. In many parts of the world, it is in short supply. From a biological standpoint, water has many distinct properties that are critical for the proliferation of life that set it apart from other substances. It carries out this role by allowing organic compounds to react in ways that ultimately allow replication. All known forms of life depend on water. Water is vital both as a solvent in which many of the body's solutes dissolve and as an essential part of many metabolic processes within the body. Metabolism is the sum total of anabolism and catabolism. In anabolism, water is removed from molecules (through energy requiring enzymatic chemical reactions) in order to grow larger molecules (e.g. starches, triglycerides and proteins for storage of fuels and information). In catabolism, water is used to break bonds in order to generate smaller molecules (e.g. glucose, fatty acids and amino acids to be used for fuels for energy use or other purposes). Water is thus essential and central to these metabolic processes. Water is also central to photosynthesis and respiration. Photosynthetic cells use the sun's energy to split off water's hydrogen from oxygen. Hydrogen is combined with CO2 (absorbed from air or water) to form glucose and release oxygen. All living cells use such fuels and oxidize the hydrogen and carbon to capture the sun's energy and reform water and CO2 in the process (cellular respiration). Water is also central to acid-base neutrality and enzyme function. An acid, a hydrogen ion (H+, that is, a proton) donor, can be neutralized by a base, a proton acceptor such as hydroxide ion (OH-) to form water. Water is considered to be neutral, with a pH (the negative log of the hydrogen ion concentration) of 7. Acids have pH values less than 7 while bases have values greater than 7. Stomach acid (HCl) is useful to digestion. However, its corrosive effect on the esophagus during reflux can temporarily be neutralized by ingestion of a base such as aluminum hydroxide to produce the neutral molecules water and the salt aluminum chloride. Human biochemistry that involves enzymes usually performs optimally around a biologically neutral pH of 7.4. (Wikipedia).7732-18-5C0000196215377937OH2OInChI=1S/H2O/h1H2XLYOFNOQVPJJNP-UHFFFAOYSA-N18.015318.010564686FDB013390Dihydrogen oxide;Steam;[oh2];Acqua;Agua;Aqua;Bound water;Dihydridooxygen;Eau;H2o;Hoh;Hydrogen hydroxide;WasserPW_C001420H2O55894910951394151316214481135261562428652106912077033823188382109431137749146554159043201824253222267860272746277817280529314370316472363461459836472737494193503027515675195975214100522794523610352971055319111534311353551125402110547012354831255492126550712755341305537114554112955911355608118562210856916575914057781015841143585314658771075890955910147594015160321556059157608716161231636133159621516218166647717865071806600152671311768401886888160716220571812077193206721121172282137238214724321572951987350216738821074012127467222749222475001907588170820122582372268414162926526118502771192216412011281122132851225028612264287123272491252022712632651269329012705291127152921300729813019300130253011303730213261223133272941534030842327315426953184369132276914293770192537710213277131133772151347737833177397332774713337751611577536334776283367772233777759341778163437798234778071329782353527824235378270356791133608001436880039370805912288065611993830383947943841105573901106393911158443981198792321199151221199634061200084071200464081201131241203654121204304051204384091206064151207944141211584251212404291213511211213814191216074341221183821223844361227531201227973741228044431230124461230643761230721371231314471231421361231624481232314511233844501237304601238104641239404551241654691246703991249384711249454721253052971253534791253864811254244821254802991256824831257074781257454871260544901262384951262734841267644801268965011269635021270173881271772081271992091272275041275065071275765151278363891280823951281765131406747901406758341407551851144NADHHMDB0001487NADH is the reduced form of NAD+, and NAD+ is the oxidized form of NADH, A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). It forms NADP with the addition of a phosphate group to the 2' position of the adenosyl nucleotide through an ester linkage.(Dorland, 27th ed).58-68-4C0000443915316908NADH388299DB00157NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1OC21H29N7O14P2InChI=1S/C21H29N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1,3-4,7-8,10-11,13-16,20-21,29-32H,2,5-6H2,(H2,23,33)(H,34,35)(H,36,37)(H2,22,24,25)/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BOPGDPNILDQYTO-NNYOXOHSSA-N665.441665.124771695FDB0226491,4-dihydronicotinamide adenine dinucleotide;Dpnh;Dihydrocodehydrogenase i;Dihydrocozymase;Dihydronicotinamide adenine dinucleotide;Dihydronicotinamide mononucleotide;Enada;Nadh;Nadh2;Reduced codehydrogenase i;Reduced diphosphopyridine nucleotide;Reduced nicotinamide adenine diphosphate;Reduced nicotinamide-adenine dinucleotide;B-dpnh;B-nadh;Beta-dpnh;Beta-nadh;Nicotinamide adenine dinucleotide (reduced);Reduced nicotinamide adenine dinucleotidePW_C001144NADH1434153349086481011152127551469542230492781172836293109948061848121848212849046495931516995524010353321115358112546612354791255593135569810057371085829141591514759451516027155607916163871647217867711176893160701118870991637172205719520674622228244226836022590862241180919811821216123202491300329813015300132552234240332242618315771071327712313377208134773713317765133677668334777003327770713077917113779863478000936880691119938221241105493881128549411583811811995540612017240712037812212098640812116242512124412612169342912181838312261638412274512012312744712313813612355137412373446012381444312424246412437139812518912112534547912553148112576229712580829912592648212651649512676748012688850112738550212809039012836239112842939514075918565CholineHMDB0000097Choline is a basic constituent of lecithin that is found in many plants and animal organs. It is important as a precursor of acetylcholine, as a methyl donor in various metabolic processes, and in lipid metabolism. Choline is now considered to be an essential vitamin. While humans can synthesize small amounts (by converting phosphatidylethanolamine to phosphatidylcholine), it must be consumed in the diet to maintain health. Required levels are between 425 mg/day (female) and 550 mg/day (male). Milk, eggs, liver, and peanuts are especially rich in choline. Most choline is found in phospholipids, namely phosphatidylcholine or lecithin. Choline can be oxidized to form betaine, which is a methyl source for many reactions (i.e. conversion of homocysteine into methionine). Lack of sufficient amounts of choline in the diet can lead to a fatty liver condition and general liver damage. This arises from the lack of VLDL, which is necessary to transport fats away from the liver. Choline deficiency also leads to elevated serum levels of alanine amino transferase and is associated with increased incidence of liver cancer.62-49-7C0011430515354CPD-563299DB00122C[N+](C)(C)CCOC5H14NOInChI=1S/C5H14NO/c1-6(2,3)4-5-7/h7H,4-5H2,1-3H3/q+1OEYIOHPDSNJKLS-UHFFFAOYSA-N104.1708104.107539075FDB000710(2-hydroxyethyl)trimethyl ammonium;(2-hydroxyethyl)trimethylammonium;(beta-hydroxyethyl)trimethylammonium;2-hydroxy-n,n,n-trimethyl-ethanaminium;2-hydroxy-n,n,n-trimethylethanaminium;Bilineurine;Biocolina;Biocoline;Choline;Choline cation;Choline ion;Cholinum;Hepacholine;Hormocline;Lipotril;N,n,n-trimethylethanol-ammonium;N,n,n-trimethylethanolammonium;Neocolina;Paresan;N-trimethylethanolamine;TrimethylethanolaminePW_C000065Choline56235641556581497145612119561913768497121851511219716412278226153392153804977614112776191147853011579972132799803319482912494859383113285388115541118115753398120489407120497409121306405123876376125987478126471481127440209128040206590HomocysteineHMDB0000742Homocysteine is a sulfur-containing amino acid that arises during methionine metabolism. Although its concentration in plasma is only about 10 micromolar (uM), even moderate hyperhomocysteinemia is associated with increased incidence of cardiovascular disease and Alzheimer's disease. Elevations in plasma homocysteine are commonly found as a result of vitamin deficiencies, polymorphisms of enzymes of methionine metabolism, and renal disease. Pyridoxal, folic acid, riboflavin, and Vitamin B(12) are all required for methionine metabolism, and deficiency of each of these vitamins result in elevated plasma homocysteine. A polymorphism of methylenetetrahydrofolate reductase (C677T), which is quite common in most populations with a homozygosity rate of 10-15 %, is associated with moderate hyperhomocysteinemia, especially in the context of marginal folate intake. Plasma homocysteine is inversely related to plasma creatinine in patients with renal disease. This is due to an impairment in homocysteine removal in renal disease. The role of these factors, and of modifiable lifestyle factors, in affecting methionine metabolism and in determining plasma homocysteine levels is discussed. Homocysteine is an independent cardiovascular disease (CVD) risk factor modifiable by nutrition and possibly exercise. Homocysteine was first identified as an important biological compound in 1932 and linked with human disease in 1962 when elevated urinary homocysteine levels were found in children with mental retardation. This condition, called homocysteinuria, was later associated with premature occlusive CVD, even in children. These observations led to research investigating the relationship of elevated homocysteine levels and CVD in a wide variety of populations including middle age and elderly men and women with and without traditional risk factors for CVD. (PMID 17136938, 15630149).454-29-5C053304979197817230HOMO-CYS757N[C@@H](CCS)C(O)=OC4H9NO2SInChI=1S/C4H9NO2S/c5-3(1-2-8)4(6)7/h3,8H,1-2,5H2,(H,6,7)/t3-/m0/s1FFFHZYDWPBMWHY-VKHMYHEASA-N135.185135.035399227DBMET00508FDB001491(+-)-homocysteine;(s)-2-amino-4-mercapto-butanoate;(s)-2-amino-4-mercapto-butanoic acid;2-amino-4-mercapto-butanoate;2-amino-4-mercapto-butanoic acid;2-amino-4-mercapto-butyric acid;2-amino-4-mercapto-dl-butyrate;2-amino-4-mercapto-dl-butyric acid;2-amino-4-mercaptobutyric acid;2-amino-4-sulfanylbutanoate;2-amino-4-sulfanylbutanoic acid;D,l-homocysteine;Dl-2-amino-4-mercaptobutyric acid;Dl-2-amino-4-mercapto-butyric acid;Dl-homocysteine;Dl-homocysteine (free base);Hcy;Homo-cys;Homocysteine;L-2-amino-4-mercapto-butyric acid;L-homocysteine;Usaf b-12;2-amino-4-mercaptobutyratePW_C000590Hcys56681824255951358264225776071117810513212047612212215112412470311812579329712631029912724820512787238862DimethylglycineHMDB0000092Dimethylglycine (DMG) is an amino acid derivative found in the cells of all plants and animals and can be obtained in the diet in small amounts from grains and meat. The human body produces DMG when metabolizing choline into Glycine. Dimethylglycine that is not metabolized in the liver is transported by the circulatory system to body tissue. Dimethylglycine was popular with Russian athletes and cosmonauts owing to its reputed ability to increase endurance and reduce fatigue. DMG is also a byproduct of homocysteine metabolism. Homocysteine and betaine are converted to methionine and N, N-dimethylglycine by betaine-homocysteine methyltransferase.1118-68-9C0102667317724DIMETHYL-GLYCINE653DB02083CN(C)CC(O)=OC4H9NO2InChI=1S/C4H9NO2/c1-5(2)3-4(6)7/h3H2,1-2H3,(H,6,7)FFDGPVCHZBVARC-UHFFFAOYSA-N103.1198103.063328537FDB021893(dimethylamino)acetate;(dimethylamino)acetic acid;2-(dimethylamino)acetate;2-(dimethylamino)acetic acid;Dimethylglycine;N,n-dimethylaminoacetate;N,n-dimethylaminoacetic acid;N,n-dimethylglycine;N-methylsarcosine n,n-dimethyl-glycinePW_C000062DMglyc567819002255435596135776081117807911278316132120477122122125407122295124124677119124848118126282481126462299127845206128032388548L-MethionineHMDB0000696Methionine is an essential amino acid (there are 9 essential amino acids) required for normal growth and development of humans, other mammals, and avian species. In addition to being a substrate for protein synthesis, it is an intermediate in transmethylation reactions, serving as the major methyl group donor in vivo, including the methyl groups for DNA and RNA intermediates. Methionine is a methyl acceptor for 5-methyltetrahydrofolate-homocysteine methyltransferase (methionine synthase), the only reaction that allows for the recycling of this form of folate, and is also a methyl acceptor for the catabolism of betaine. Methionine is the metabolic precursor for cysteine. Only the sulfur atom from methionine is transferred to cysteine; the carbon skeleton of cysteine is donated by serine (PMID: 16702340). There is a general consensus concerning normal sulfur amino acid (SAA) requirements. WHO recommendations amount to 13 mg/kg per 24 h in healthy adults. This amount is roughly doubled in artificial nutrition regimens. In disease or after trauma, requirements may be altered for methionine, cysteine, and taurine. Although in specific cases of congenital enzyme deficiency, prematurity, or diminished liver function, hypermethioninemia or hyperhomocysteinemia may occur, SAA supplementation can be considered safe in amounts exceeding 2-3 times the minimum recommended daily intake. Apart from some very specific indications (e.g. acetaminophen poisoning) the usefulness of SAA supplementation is not yet established (PMID: 16702341). Methionine is known to exacerbate psychopathological symptoms in schizophrenic patients, but there is no evidence of similar effects in healthy subjects. The role of methionine as a precursor of homocysteine is the most notable cause for concern. Acute doses of methionine can lead to acute increases in plasma homocysteine, which can be used as an index of the susceptibility to cardiovascular disease. Sufficiently high doses of methionine can actually result in death. Longer-term studies in adults have indicated no adverse consequences of moderate fluctuations in dietary methionine intake, but intakes higher than 5 times the normal amount resulted in elevated homocysteine levels. These effects of methionine on homocysteine and vascular function are moderated by supplements of vitamins B-6, B-12, C, and folic acid (PMID: 16702346). When present in sufficiently high levels, methionine can act as an atherogen and a metabotoxin. An atherogen is a compound that when present at chronically high levels causes atherosclerosis and cardiovascular disease. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of methionine are associated with at least ten inborn errors of metabolism, including cystathionine beta-synthase deficiency, glycine N-methyltransferase deficiency, homocystinuria, tyrosinemia, galactosemia, homocystinuria-megaloblastic anemia due to defects in cobalamin metabolism, methionine adenosyltransferase deficiency, methylenetetrahydrofolate reductase deficiency, and S-adenosylhomocysteine (SAH) hydrolase deficiency. Chronically elevated levels of methionine in infants can lead to intellectual disability and other neurological problems, delays in motor skills, sluggishness, muscle weakness, and liver problems. Many individuals with these metabolic disorders tend to develop cardiovascular disease later in life. Studies on feeding rodents high levels of methionine have shown that methionine promotes atherosclerotic plaques independently of homocysteine levels (PMID: 26647293). A similar study in Finnish men showed the same effect (PMID: 16487911).63-68-3C00073613716643MET5907DB00134CSCC[C@H](N)C(O)=OC5H11NO2SInChI=1S/C5H11NO2S/c1-9-3-2-4(6)5(7)8/h4H,2-3,6H2,1H3,(H,7,8)/t4-/m0/s1FFEARJCKVFRZRR-BYPYZUCNSA-N149.211149.051049291DBMET00506FDB012683(2s)-2-amino-4-(methylsulfanyl)butanoate;(2s)-2-amino-4-(methylsulfanyl)butanoic acid;(l)-methionine;(s)-(+)-methionine;(s)-2-amino-4-(methylthio)butanoate;(s)-2-amino-4-(methylthio)butanoic acid;(s)-2-amino-4-(methylthio)-butanoate;(s)-2-amino-4-(methylthio)-butanoic acid;(s)-2-amino-4-(methylthio)butyric acid;(s)-methionine;2-amino-4-(methylthio)butyrate;2-amino-4-(methylthio)butyric acid;2-amino-4-methylthiobutanoate;2-amino-4-methylthiobutanoic acid;A-amino-g-methylmercaptobutyrate;A-amino-g-methylmercaptobutyric acid;Acimethin;Cymethion;G-methylthio-a-aminobutyrate;G-methylthio-a-aminobutyric acid;H-met-h;H-met-oh;L(-)-amino-alpha-amino-alpha-aminobutyric acid;L(-)-amino-gamma-methylthiobutyric acid;L-(-)-methionine;L-2-amino-4-(methylthio)butyric acid;L-2-amino-4-methylthiobutyric acid;L-methionin;L-methionine;L-methioninum;L-a-amino-g-methylthiobutyrate;L-a-amino-g-methylthiobutyric acid;L-alpha-amino-gamma-methylmercaptobutyric acid;L-alpha-amino-gamma-methylthiobutyrate;L-alpha-amino-gamma-methylthiobutyric acid;L-gamma-methylthio-alpha-aminobutyric acid;Liquimeth;Met;Mepron;Methilanin;Methionine;Methioninum;Metionina;Neo-methidin;Poly-l-methionine;Polymethionine;S-methionine;S-methyl-l-homocysteine;Toxin war;Alpha-amino-alpha-aminobutyric acid;Alpha-amino-gamma-methylmercaptobutyrate;Alpha-amino-gamma-methylmercaptobutyric acid;Gamma-methylthio-alpha-aminobutyrate;Gamma-methylthio-alpha-aminobutyric acid;M;(2s)-2-amino-4-(methylsulphanyl)butanoate;(2s)-2-amino-4-(methylsulphanyl)butanoic acid;(s)-2-amino-4-(methylthio)butyrate;L-a-amino-g-methylmercaptobutyrate;L-a-amino-g-methylmercaptobutyric acid;L-alpha-amino-gamma-methylmercaptobutyrate;L-α-amino-γ-methylmercaptobutyrate;L-α-amino-γ-methylmercaptobutyric acidPW_C000548Met56881825255971355680107568110858751058267151120332224255031542565318426933207698522477609111781061321204781221221521241247041181258582971263112991273202051278733881005Zinc (II) ionHMDB0001303Zinc is an essential element, necessary for sustaining all life.Physiologically, it exists as an ion in the body. It is estimated that 3000 of the hundreds of thousands of proteins in the human body contain zinc prosthetic groups. In addition, there are over a dozen types of cells in the human body that secrete zinc ions, and the roles of these secreted zinc signals in medicine and health are now being actively studied. Intriguingly, brain cells in the mammalian forebrain are one type of cell that secretes zinc, along with its other neuronal messenger substances. Cells in the salivary gland, prostate, immune system and intestine are other types that secrete zinc. Obtaining a sufficient zinc intake during pregnancy and in young children is a problem, especially among those who cannot afford a good and varied diet. Brain development is stunted by zinc deficiency in utero and in youth. Zinc is an activator of certain enzymes, such as carbonic anhydrase. Carbonic anhydrase is important in the transport of carbon dioxide in vertebrate blood. Even though zinc is an essential requirement for a healthy body, too much zinc can be harmful. Excessive absorption of zinc can also suppress copper and iron absorption. The free zinc ion in solution is highly toxic to plants, invertebrates, and even vertebrate fish. The Free Ion Activity Model (FIAM) is well-established in the literature, and shows that just micromolar amounts of the free ion kills some organisms.23713-49-7C000383205129105ZN%2b229723DB01593[Zn++]ZnInChI=1S/Zn/q+2PTFCDOFLOPIGGS-UHFFFAOYSA-N65.40963.929146578FDB003729Zinc;Zinc ion;Dietary zinc;Zinc cation;Zinc, ion (zn2+);Zn(ii);Zn(2+);Zn2+PW_C001005Zinc1323841188271165291529575130446831202931477054101175425103543411854591205560132558513355981357449166117871981246622612724290133211517696722577401111775801147792933680400112002012412003540612006012212044140912125742912307513712382746412539829912541347912543829712568548312693838812695350112697620512718020810795-Methyltetrahydrofolic acidHMDB00013965 methyltetrahydrofolic acid (5-MTHF) is the most biologically active form of the B-vitamin known as folic acid, also known generically as folate. 5-MTHF functions, in concert with vitamin B12, as a methyl-group donor involved in the conversion of the amino acid homocysteine to methionine. Methyl (CH3) group donation is vital to many bodily processes, including serotonin, melatonin, and DNA synthesis. Therapeutically, 5-MTHF is instrumental in reducing homocysteine levels, preventing neural tube defects, and improving vascular endothelial function. Research on folate supplementation suggests it plays a key role in preventing cervical dysplasia and protecting against neoplasia in ulcerative colitis. Folic acid also shows promise as part of a nutritional protocol to treat vitiligo, and may reduce inflammation of the gingiva. Furthermore, certain neurological, cognitive, and psychiatric presentations may be secondary to folate deficiency. Such presentations include depression, peripheral neuropathy, myelopathy, restless legs syndrome, insomnia, dementia, forgetfulness, irritability, endogenous depression, organic psychosis, and schizophrenia-like syndromes. After ingestion, the process of conversion of folic acid to the metabolically active coenzyme forms is relatively complex. Synthesis of the active forms of folic acid requires several enzymes, adequate liver and intestinal function, and adequate supplies of riboflavin (B2), niacin (B3), pyridoxine (B6), zinc, vitamin C, and serine. After formation of the coenzyme forms of the vitamin in the liver, these metabolically active compounds are secreted into the small intestine with bile (the folate enterohepatic cycle), where they are reabsorbed and distributed to tissues throughout the body. Human pharmacokinetic studies indicate folic acid has high bioavailability, with large oral doses of folic acid substantially raising plasma levels in healthy subjects in a time and dose dependent manner. Red blood cells (RBCs) appear to be the storage depot for folic acid, as RBC levels remain elevated for periods in excess of 40 days following discontinuation of supplementation. Folic acid is poorly transported to the brain and rapidly cleared from the central nervous system. The primary methods of elimination of absorbed folic acid are fecal (through bile) and urinary. Despite the biochemical complexity of this process, evidence suggests oral supplementation with folic acid increases the body's pool of 5-MTHF in healthy individuals. However, enzyme defects, mal-absorption, digestive system pathology, and liver disease can result in impaired ability to activate folic acid. In fact, some individuals have a severe congenital deficiency of the enzyme Methyl tetrahydrofolate reductase (5-MTHFR), which is needed to convert folic acid to 5-MTHF. Milder forms of this enzyme defect likely interact with dietary folate status to determine risk for some disease conditions. In individuals with a genetic defect of this enzyme (whether mild or severe), supplementation with 5- MTHF might be preferable to folic acid supplementation. (PMID: 17176169).134-35-0C00440439234156415-METHYL-THF388371CN1C(CNC2=CC=C(C=C2)C(=O)N[C@H](CCC(O)=O)C(O)=O)CNC2=C1C(=O)NC(N)=N2C20H25N7O6InChI=1S/C20H25N7O6/c1-27-12(9-23-16-15(27)18(31)26-20(21)25-16)8-22-11-4-2-10(3-5-11)17(30)24-13(19(32)33)6-7-14(28)29/h2-5,12-13,22H,6-9H2,1H3,(H,24,30)(H,28,29)(H,32,33)(H4,21,23,25,26,31)/t12?,13-/m1/s1ZNOVTXRBGFNYRX-ZGTCLIOFSA-N459.4558459.186631567DBMET00528FDB0226005-methyl tetrahydrofolate;5-methyl-5,6,7,8-tetrahydrofolate;5-methyl-tetrahydrofolate;5-methyltetrahydrofolate;5-methyltetrahydropteroylglutamate;Methyl folate;Methyl-tetrahydrofolate;N( 5)-methyltetrahydrofolate;N-(4-(((2-amino-1,4,5,6,7,8-hexahydro-5-methyl-4-oxo-6-pteridinyl)methyl)amino)benzoyl)-l-glutamate;N-(4-(((2-amino-1,4,5,6,7,8-hexahydro-5-methyl-4-oxo-6-pteridinyl)methyl)amino)benzoyl)-l-glutamic acid;N-(5-methyl-5,6,7,8-tetrahydropteroyl)-l-glutamate;N-(5-methyl-5,6,7,8-tetrahydropteroyl)-l-glutamic acid;N5-methyl-tetrahydrofolate;N5-methyl-tetrahydrofolic acid;N5-methyltetrahydrofolate;N5-methyltetrahydropteroyl mono-l-glutamate;[(6s)-5-methyl-5,6,7,8-tetrahydropteroyl]glutamatePW_C0010795-MTHFa570818212533311156001357173205782961321204811221222781241248321181257632971264442991280133881221Tetrahydrofolic acidHMDB0001846Tetrahydrofolate is a soluble coenzyme (vitamin B9) that is synthesized de novo by plants and microorganisms, and absorbed from the diet by animals. It is composed of three distinct parts: a pterin ring, a p-ABA (p-aminobenzoic acid) and a polyglutamate chain with a number of residues varying between 1 and 8. Only the tetra-reduced form of the molecule serves as a coenzyme for C1 transfer reactions. In biological systems, the C1-units exist under various oxidation states and the different tetrahydrofolate derivatives constitute a family of related molecules named indistinctly under the generic term folate. (PMID 16042593). Folate is important for cells and tissues that rapidly divide. Cancer cells divide rapidly, and drugs that interfere with folate metabolism are used to treat cancer. Methotrexate is a drug often used to treat cancer because it inhibits the production of the active form, tetrahydrofolate. Unfortunately, methotrexate can be toxic, producing side effects such as inflammation in the digestive tract that make it difficult to eat normally. -- Wikipedia; Signs of folic acid deficiency are often subtle. Diarrhea, loss of appetite, and weight loss can occur. Additional signs are weakness, sore tongue, headaches, heart palpitations, irritability, and behavioral disorders. Women with folate deficiency who become pregnant are more likely to give birth to low birth weight and premature infants, and infants with neural tube defects. In adults, anemia is a sign of advanced folate deficiency. In infants and children, folate deficiency can slow growth rate. Some of these symptoms can also result from a variety of medical conditions other than folate deficiency. It is important to have a physician evaluate these symptoms so that appropriate medical care can be given. -- Wikipedia; Folinic acid is a form of folate that can help 'rescue' or reverse the toxic effects of methotrexate. Folinic acid is not the same as folic acid. Folic acid supplements have little established role in cancer chemotherapy. There have been cases of severe adverse effects of accidental substitution of folic acid for folinic acid in patients receiving methotrexate cancer chemotherapy. It is important for anyone receiving methotrexate to follow medical advice on the use of folic or folinic acid supplements. -- Wikipedia. Low concentrations of folate, vitamin B12, or vitamin B6 may increase the level of homocysteine, an amino acid normally found in blood. There is evidence that an elevated homocysteine level is an independent risk factor for heart disease and stroke. The evidence suggests that high levels of homocysteine may damage coronary arteries or make it easier for blood clotting cells called platelets to clump together and form a clot. However, there is currently no evidence available to suggest that lowering homocysteine with vitamins will reduce your risk of heart disease. Clinical intervention trials are needed to determine whether supplementation with folic acid, vitamin B12 or vitamin B6 can lower your risk of developing coronary heart disease. -- Wikipedia.135-16-0C001011378185720506THF18714427DB00116NC1=NC(=O)C2=C(NC[C@H](CNC3=CC=C(C=C3)C(=O)NC(CCC(O)=O)C(O)=O)N2)N1C19H23N7O6InChI=1S/C19H23N7O6/c20-19-25-15-14(17(30)26-19)23-11(8-22-15)7-21-10-3-1-9(2-4-10)16(29)24-12(18(31)32)5-6-13(27)28/h1-4,11-12,21,23H,5-8H2,(H,24,29)(H,27,28)(H,31,32)(H4,20,22,25,26,30)/t11-,12?/m0/s1MSTNYGQPCMXVAQ-PXYINDEMSA-N445.4292445.170981503FDB022705(6s)-tetrahydrofolate;(6s)-tetrahydrofolic acid;5,6,7,8-tetrahydrofolate;5,6,7,8-tetrahydrofolic acid;Tetra-h-folate;Tetrahydrafolate;Tetrahydrofolate;Tetrahydrofolic acid;Tetrahydropteroyl mono-l-glutamate;TetrahydropteroylglutamatePW_C001221THFA4484571897531809253071115347112560113557861086009147706618871512057185206758316311797198426403157733613378118132120352406120482122120696407122166124123001120123301119124718118125673479125749297125771481126324299127168501127886388414Adenosine 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_C000414ATP92214608266164142247813733327995934399763210518211210214649215614216058240559243427272646281229302966316372361661361751439923447431476891486454503289503526515575205975215100525010452911015313111534611253901035406117543011854431205542129555613255691335603135562110858461435854146587610758971475924151604815561091616230166649317868391886870160697619971572057184206720921072252137229211729819873022167390217740821874321637481222749919081862251184727711903170120102811203916412178285125782261269129013264223153273084232631542621322426943187702825377218134772333297746833377632336780373327804135078168128782143517824035378411335784941157885013078865331789193348002836880046184806741198562919482612411323494113282388116280109119914122119992406120154407120245382120362412121246429121392123121397433121471408121974410122065125122079383122083405122402422122444435122919399123009446123816464123951447123956468124029374124527444124616136124630398124634376124943472124972375125011470125304297125371479125392299125515481125595484126123485126220300126234495126240478126547491126596499126913501127123389127731516127781395127796390127801209128119508128167517140770891921S-AdenosylmethionineHMDB0001185S-Adenosylmethionine (CAS: 29908-03-0), also known as SAM or AdoMet, is a physiologic methyl radical donor involved in enzymatic transmethylation reactions and present in all living organisms. It possesses anti-inflammatory activity and has been used in the treatment of chronic liver disease (From Merck, 11th ed). S-Adenosylmethionine is a natural substance present in the cells of the body. It plays a crucial biochemical role by donating a one-carbon methyl group in a process called transmethylation. S-Adenosylmethionine, formed from the reaction of L-methionine and adenosine triphosphate catalyzed by the enzyme S-adenosylmethionine synthetase, is the methyl-group donor in the biosynthesis of both DNA and RNA nucleic acids, phospholipids, proteins, epinephrine, melatonin, creatine, and other molecules.485-80-3C000192476216515414S-ADENOSYLMETHIONINE31983DB00118C[S+](CC[C@H](N)C(O)=O)C[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2NC15H23N6O5SInChI=1S/C15H22N6O5S/c1-27(3-2-7(16)15(24)25)4-8-10(22)11(23)14(26-8)21-6-20-9-12(17)18-5-19-13(9)21/h5-8,10-11,14,22-23H,2-4,16H2,1H3,(H2-,17,18,19,24,25)/p+1/t7-,8+,10+,11+,14+,27?/m0/s1MEFKEPWMEQBLKI-AIRLBKTGSA-O399.445399.145063566FDB022473(3s)-5'-[(3-amino-3-carboxypropyl)methylsulfonio]-5'-deoxyadenosine;2-s-adenosyl-l-methionine;5'-deoxyadenosine-5'-l-methionine disulfate ditosylate;Active methionine;Ademetionine;Adenosylmethionine;Adomet;Donamet;L-s-adenosylmethionine;S-(5'-adenosyl)-l-methionine;S-(5'-deoxyadenosin-5'-yl)-l-methionine;S-adenosyl methionine;S-adenosyl-l-methionine disulfate tosylate;S-adenosyl-l-methionine;S-adenosyl-methionine;S-adenosylmethionine;5'-deoxyadenosine-5'-l-methionine disulphate ditosylate;S-adenosyl-l-methionine disulphate tosylate;(3s)-5'-[(3-amino-3-carboxypropyl)methylsulfonio]-5'-deoxyadenosine, inner salt;[1-(adenin-9-yl)-1,5-dideoxy-beta-d-ribofuranos-5-yl][(3s)-3-amino-3-carboxypropyl](methyl)sulfonium;Acylcarnitine;Sam;SamePW_C000921SAMe5198633307042012203188027206624681105023505604135713616375402107544213763216082661519235195118741981203122212358225152932491534518153633097689729376899164769842247748811177731338777723417809913278303351783353467915511279961361808612294830382948333861132863891132883971155433991155464011203931221205374131209394071210521241222824351231714491235051191236161181248364701258592971258794811263042991264474991273212051273402061275953881280175171104PhosphateHMDB0001429Phosphate is a salt of phosphoric acid. In organic chemistry, a phosphate, or organophosphate, is an ester of phosphoric acid. Organic phosphates are important in biochemistry, biogeochemistry and ecology. Phosphate (Pi) is an essential component of life. In biological systems, phosphorus is found as a free phosphate ion in solution and is called inorganic phosphate, to distinguish it from phosphates bound in various phosphate esters. Inorganic phosphate is generally denoted Pi and at physiological (neutral) pH primarily consists of a mixture of HPO<sup>2-</sup><sub>4</sub> and H<sub>2</sub>PO<sup>-</sup><sub>4</sub> ions. phosphates are most commonly found in the form of adenosine phosphates, (AMP, ADP and ATP) and in DNA and RNA and can be released by the hydrolysis of ATP or ADP. Similar reactions exist for the other nucleoside diphosphates and triphosphates. Phosphoanhydride bonds in ADP and ATP, or other nucleoside diphosphates and triphosphates, contain high amounts of energy which give them their vital role in all living organisms. Phosphate must be actively transported into cells against its electrochemical gradient. In vertebrates, two unrelated families of Na+-dependent Pi transporters carry out this task. Remarkably, the two families transport different Pi species: whereas type II Na+/Pi cotransporters (SCL34) prefer divalent HPO4(2), type III Na+/Pi cotransporters (SLC20) transport monovalent H2PO4. The SCL34 family comprises both electrogenic and electroneutral members that are expressed in various epithelia and other polarized cells. Through regulated activity in apical membranes of the gut and kidney, they maintain body Pi homeostasis, and in salivary and mammary glands, liver, and testes they play a role in modulating the Pi content of luminal fluids. Phosphate levels in the blood play an important role in hormone signaling and in bone homeostasis. In classical endocrine regulation, low serum phosphate induces the renal production of the seco-steroid hormone 1,25-dihydroxyvitamin D3 (1,25(OH)2D3).This active metabolite of vitamin D acts to restore circulating mineral (i.e. phosphate and calcium) levels by increasing absorption in the intestine, reabsorption in the kidney, and mobilization of calcium and phosphate from bone. Thus, chronic renal failure is associated with hyperparathyroidism, which in turn contributes to osteomalacia (softening of the bones). Another complication of chronic renal failure is hyperphosphatemia (low levels of phosphate in the blood). Hyperphosphatemia (excess levels of phosphate in the blood) is a prevalent condition in kidney dialysis patients and is associated with increased risk of mortality. Hypophosphatemia (hungry bone syndrome) has been associated to postoperative electrolyte aberrations and after parathyroidectomy. (PMID: 17581921, 11169009, 11039261, 9159312, 17625581)Fibroblast growth factor 23 (FGF-23) has recently been recognized as a key mediator of phosphate homeostasis, its most notable effect being promotion of phosphate excretion. FGF-23 was discovered to be involved in diseases such as autosomal dominant hypophosphatemic rickets, X-linked hypophosphatemia, and tumor-induced osteomalacia in which phosphate wasting was coupled to inappropriately low levels of 1,25(OH)2D3. FGF-23 is regulated by dietary phosphate in humans. In particular it was found that phosphate restriction decreased FGF-23, and phosphate loading increased FGF-23.14265-44-2C00009106118367CPD-85871032OP(O)(O)=OH3O4PInChI=1S/H3O4P/c1-5(2,3)4/h(H3,1,2,3,4)NBIIXXVUZAFLBC-UHFFFAOYSA-N97.995297.976895096DBMET00532FDB022617Nfb orthophosphate;O-phosphoric acid;Ortho-phosphate;Orthophosphate (po43-);Orthophosphate(3-);Phosphate;Phosphate (po43-);Phosphate anion(3-);Phosphate ion (po43-);Phosphate ion(3-);Phosphate trianion;Phosphate(3-);Phosphoric acid ion(3-);Pi;[po4](3-);Orthophosphate;Phosphate ion;Po4(3-);Phosphoric acid;Orthophosphoric acid;Phosphoric acid ionPW_C001104Pi2448488145818188312980317631417674925001027294727374631292931667236366138512342492244753150312751587520797521610053171115351112538110354471205543129557313356051355625108569365848143585514659111475941151604015561001616294107648717866911016714117684218868891607161205718920672122117306198738921074022127436163747522281962258258227101182411013425711748132117611151177321311904170119271641201428112728290132632233481917422553044235031542435318436923227701825377194293772171347794033677966130780483327805732978245353786693318002236889279308938313839479638411055839011064039111323594115845398116206109119982406120069122120699407121057124121216125121268429121352121121409123121423382121852405123304119123621118123786136123838464123968447123981399124405376124948472125362479125446297125774481125954299126221478126594300126604298126723484126904501127413388127783209128166395128177513128315389170PyrophosphateHMDB0000250The 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_C000170Ppi122354638429237353288222121731620492410592815294175144868545034895252104529410154091175424103543311854581205548111555913255841335606135565510858791076239166697819970731887134163727216073121987318213827515182832101186916112002222120411641231522512323249125122881257922612695290152193061537518347601742561315426973187723532977317128776353367841633578928331791531127995013479958130800473728041717085630194786384948141259481938298678223110634391113270395113275389115527136115532399119934122120017124120032406120330410120936407121261429121341121121486383122407422122985444123502119123831464124044398124977375125324297125395299125410479125597484125656485125876481126552491126869205126935388126950501127337206128124508140772891749S-AdenosylhomocysteineHMDB0000939S-Adenosyl-L-homocysteine (SAH) is formed by the demethylation of S-adenosyl-L-methionine. S-Adenosylhomocysteine (AdoHcy or SAH) is also the immediate precursor of all of the homocysteine produced in the body. The reaction is catalyzed by S-adenosylhomocysteine hydrolase and is reversible with the equilibrium favoring formation of SAH. In vivo, the reaction is driven in the direction of homocysteine formation by the action of the enzyme adenosine deaminase which converts the second product of the S-adenosylhomocysteine hydrolase reaction, adenosine, to inosine. Except for methyl transfer from betaine and from methylcobalamin in the methionine synthase reaction, SAH is the product of all methylation reactions that involve S-adenosylmethionine (SAM) as the methyl donor. Methylation is significant in epigenetic regulation of protein expression via DNA and histone methylation. The inhibition of these SAM-mediated processes by SAH is a proven mechanism for metabolic alteration. Because the conversion of SAH to homocysteine is reversible, with the equilibrium favoring the formation of SAH, increases in plasma homocysteine are accompanied by an elevation of SAH in most cases. Disturbances in the transmethylation pathway indicated by abnormal SAH, SAM, or their ratio have been reported in many neurodegenerative diseases, such as dementia, depression, and Parkinson's disease (PMID: 18065573, 17892439). Therefore, when present in sufficiently high levels, S-adenosylhomocysteine can act as an immunotoxin and a metabotoxin. An immunotoxin disrupts, limits the function, or destroys immune cells. A metabotoxin is an endogenous metabolite that causes adverse health effects at chronically high levels. Chronically high levels of S-adenosylhomocysteine are associated with S-adenosylhomocysteine (SAH) hydrolase deficiency and adenosine deaminase deficiency. S-Adenosylhomocysteine forms when there are elevated levels of homocysteine and adenosine. S-Adenosyl-L-homocysteine is a potent inhibitor of S-adenosyl-L-methionine-dependent methylation reactions. It is toxic to immature lymphocytes and can lead to immunosuppression (PMID: 221926).979-92-0C000212524622216680ADENOSYL-HOMO-CYS388301N[C@@H](CCSC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2N)C(O)=OC14H20N6O5SInChI=1S/C14H20N6O5S/c15-6(14(23)24)1-2-26-3-7-9(21)10(22)13(25-7)20-5-19-8-11(16)17-4-18-12(8)20/h4-7,9-10,13,21-22H,1-3,15H2,(H,23,24)(H2,16,17,18)/t6-,7+,9+,10+,13+/m0/s1ZJUKTBDSGOFHSH-WFMPWKQPSA-N384.411384.12158847DBMET00514FDB022327(s)-5'-(s)-(3-amino-3-carboxypropyl)-5'-thioadenosine;2-s-adenosyl-l-homocysteine;5'-deoxy-s-adenosyl-l-homocysteine;5'-s-(3-amino-3-carboxypropyl)-5'-thio-l-adenosine;Adenosyl-l-homocysteine;Adenosyl-homo-cys;Adenosylhomo-cys;Adenosylhomocysteine;Adohcy;Formycinylhomocysteine;L-5'-s-(3-amino-3-carboxypropyl)-5'-thior-adenosine;L-s-adenosyl-homocysteine;L-s-adenosylhomocysteine;S-(5'-adenosyl)-l-homocysteine;S-(5'-deoxyadenosin-5'-yl)-l-homocysteine;S-(5'-deoxyadenosine-5')-l-homocysteine;S-adenosyl-l-homocysteine;S-adenosyl-homocysteine;Sah;(2s)-2-amino-4-({[(2s,3s,4r,5r)-5-(6-amino-9h-purin-9-yl)-3,4-dihydroxytetrahydrofuran-2-yl]methyl}sulfanyl)butanoic acid;S-[1-(adenin-9-yl)-1,5-dideoxy-beta-d-ribofuranos-5-yl]-l-homocysteine;S-adenosylhomocysteinePW_C000749SAH520857518635307052012213188227206724683105025505607136713716375422107546213763416082681519237195118751981235922515294249153643097748911177611130777333387777334178098132783053517833734679156112799623618086322948313829483438611328738911328939711554439911554740112039412212048612512053941312094040712105312412228443512303713512317344912350611912361711812483847012588048112630329912644949912734120612759638812801951734AdenosineHMDB0000050Adenosine is a nucleoside that is composed of adenine and D-ribose. Adenosine or adenosine derivatives play many important biological roles in addition to being components of DNA and RNA. For instance, adenosine plays an important role in energy transfer as adenosine triphosphate (ATP) and adenosine diphosphate (ADP). It also plays a role in signal transduction as cyclic adenosine monophosphate (cAMP). Adenosine itself is both a neurotransmitter and potent vasodilator. When administered intravenously adenosine causes transient heart block in the AV node. Due to the effects of adenosine on AV node-dependent supraventricular tachycardia, adenosine is considered a class V antiarrhythmic agent. Overdoses of adenosine intake (as a drug) can lead to several side effects including chest pain, feeling faint, shortness of breath, and tingling of the senses. Serious side effects include a worsening dysrhythmia and low blood pressure. When present in sufficiently high levels, adenosine can act as an immunotoxin and a metabotoxin. An immunotoxin disrupts, limits the function, or destroys immune cells. A metabotoxin is an endogenous metabolite that causes adverse health effects at chronically high levels. Chronically high levels of adenosine are associated with adenosine deaminase deficiency. Adenosine is a precursor to deoxyadenosine, which is a precursor to dATP. A buildup of dATP in cells inhibits ribonucleotide reductase and prevents DNA synthesis, so cells are unable to divide. Since developing T cells and B cells are some of the most mitotically active cells, they are unable to divide and propagate to respond to immune challenges. High levels of deoxyadenosine also lead to an increase in S-adenosylhomocysteine, which is toxic to immature lymphocytes.
58-61-7C002126096116335ADENOSINE54923DB00640NC1=C2N=CN([C@@H]3O[C@H](CO)[C@@H](O)[C@H]3O)C2=NC=N1C10H13N5O4InChI=1S/C10H13N5O4/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(18)6(17)4(1-16)19-10/h2-4,6-7,10,16-18H,1H2,(H2,11,12,13)/t4-,6-,7-,10-/m1/s1OIRDTQYFTABQOQ-KQYNXXCUSA-N267.2413267.096753929FDB0035541-(6-amino-9h-purin-9-yl)-1-deoxy-beta-d-ribofuranose;1-(6-amino-9h-purin-9-yl)-1-deoxy-beta-delta-ribofuranose;6-amino-9beta-d-ribofuranosyl-9h-purine;6-amino-9beta-delta-ribofuranosyl-9h-purine;9-beta-d-arabinofuranosyladenine;9-beta-d-ribofuranosidoadenine;9-beta-d-ribofuranosyl-9h-purin-6-amine;9-beta-d-ribofuranosyladenine;9-beta-delta-arabinofuranosyladenine;9-beta-delta-ribofuranosidoadenine;9-beta-delta-ribofuranosyl-9h-purin-6-amine;9-beta-delta-ribofuranosyladenine;9beta-d-ribofuranosyladenine;9beta-d-ribofuranosyl-9h-purin-6-amine;9beta-delta-ribofuranosyladenine;9beta-delta-ribofuranosyl-9h-purin-6-amine;Adenine nucleoside;Adenine riboside;Adenine-9beta-d-ribofuranoside;Adenine-9beta-delta-ribofuranoside;Adenocard;Adenocor;Adenoscan;Adenosin;Boniton;Myocol;Nucleocardyl;Sandesin;B-d-adenosine;Beta-adenosine;Beta-d-adenosine;Beta-delta-adenosine;(2r,3r,4s,5r)-2-(6-aminopurin-9-yl)-5-(hydroxymethyl)oxolane-3,4-diol;6-amino-9-beta-d-ribofuranosyl-9h-purine;Ade-rib;Adenine deoxyribonucleoside;Adenyldeoxyriboside;Ado;Deoxyadenosine;Desoxyadenosine;6-amino-9-b-d-ribofuranosyl-9h-purine;6-amino-9-β-d-ribofuranosyl-9h-purine;9-b-d-ribofuranosidoadenine;9-β-d-ribofuranosidoadenine;9-b-d-ribofuranosyl-9h-purin-6-amine;9-β-d-ribofuranosyl-9h-purin-6-amine;β-d-adenosinePW_C000034Adenosi352818962401315560911867691076770108118251981252624943737315437383187761213278702111119928122120487124122722135125318297126459299126864205128029388964FADHMDB0001248FAD, also known as flavitan or adeflavin, belongs to the class of organic compounds known as flavin nucleotides. These are nucleotides containing a flavin moiety. Flavin is a compound that contains the tricyclic isoalloxazine ring system, which bears 2 oxo groups at the 2- and 4-positions. FAD is a drug which is used to treat eye diseases caused by vitamin b2 deficiency, such as keratitis and blepharitis. FAD is slightly soluble (in water) and a moderately acidic compound (based on its pKa). FAD has been found in human liver and muscle tissues, and has also been detected in multiple biofluids, such as feces and blood. Within the cell, FAD is primarily located in the cytoplasm, mitochondria, endoplasmic reticulum and peroxisome. FAD exists in all living organisms, ranging from bacteria to humans. In humans, FAD is involved in the risedronate action pathway, the ibandronate action pathway, the valine, leucine and isoleucine degradation pathway, and the pyrimidine metabolism pathway. FAD is also involved in several metabolic disorders, some of which include the oncogenic action OF L-2-hydroxyglutarate in hydroxygluaricaciduria pathway, gaba-transaminase deficiency, 4-hydroxybutyric aciduria/succinic semialdehyde dehydrogenase deficiency, and the saccharopinuria/hyperlysinemia II pathway. FAD is a condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972).146-14-5C0001664397516238FAD559059DB03147CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=NC(=O)NC(=O)C1=N2C27H33N9O15P2InChI=1S/C27H33N9O15P2/c1-10-3-12-13(4-11(10)2)35(24-18(32-12)25(42)34-27(43)33-24)5-14(37)19(39)15(38)6-48-52(44,45)51-53(46,47)49-7-16-20(40)21(41)26(50-16)36-9-31-17-22(28)29-8-30-23(17)36/h3-4,8-9,14-16,19-21,26,37-41H,5-7H2,1-2H3,(H,44,45)(H,46,47)(H2,28,29,30)(H,34,42,43)/t14-,15+,16+,19-,20+,21+,26+/m0/s1VWWQXMAJTJZDQX-UYBVJOGSSA-N785.5497785.157134455FDB0225111h-purin-6-amine flavin dinucleotide;1h-purin-6-amine flavine dinucleotide;Adenine-flavin dinucleotide;Adenine-flavine dinucleotide;Adenine-riboflavin dinuceotide;Adenine-riboflavin dinucleotide;Adenine-riboflavine dinucleotide;Fad;Flamitajin b;Flanin f;Flavin adenine dinucleotide;Flavin adenine dinucleotide oxidized;Flavin-adenine dinucleotide;Flavine adenosine diphosphate;Flavine-adenine dinucleotide;Flavitan;Flaziren;Isoalloxazine-adenine dinucleotide;Riboflavin 5'-adenosine diphosphate;Riboflavin-adenine dinucleotide;Riboflavine-adenine dinucleotide;AdeflavinPW_C000964FAD999114518681923216425317628288251884021188141489421612291622492133582536223723264602364688314741134758104881652681035285102533511154961265511127561311860301556054156608216161161626390164751786499179666610770391637175205732121374652227487223907622411818216118872151189921112296225123282491244315112519227125952261271029112720292130293011304130243623318770802937712613377152134775011137750711277518115775413347761513277726337780543297837534578930331792223367927235880012368800343698071411911995840611999938412005140812010740712043240512045312212049012412127842912129841812141738212148938312274812012277612112280237412282344312306637612308713512316644812384946412386845412397639912404739812534847912537848012542948212547448112569729712597948912610729912627748412689150112692039112696850212698720712701120612731020912743250612760238812784038914079018514079918640696Phosphatidyl-ethanolamideHMDB0061371Pe-nme2(14:1(9Z)/14:1(9Z)) belongs to the class of organic compounds known as dimethylphosphatidylethanolamines. These are lipids with a structure containing a glycerol moiety linked at its terminal C3 atom to a N,N-dimethylphosphoethanolamine group, and at its C1 and C2 terminal atoms by an acyl group. Pe-nme2(14:1(9Z)/14:1(9Z)) is considered to be a practically insoluble (in water) and relatively neutral molecule. Pe-nme2(14:1(9Z)/14:1(9Z)) has been found throughout all human tissues, and has also been primarily detected in blood. Within the cell, pe-nme2(14:1(9Z)/14:1(9Z)) is primarily located in the membrane (predicted from logP) and intracellular membrane.[H]C(COC(=O)CCCCCCC\C=C/CCCC)(COP(O)(=O)OCCN(C)C)OC(=O)CCCCCCC\C=C/CCCCC35H66NO8PInChI=1S/C35H66NO8P/c1-5-7-9-11-13-15-17-19-21-23-25-27-34(37)41-31-33(32-43-45(39,40)42-30-29-36(3)4)44-35(38)28-26-24-22-20-18-16-14-12-10-8-6-2/h11-14,33H,5-10,15-32H2,1-4H3,(H,39,40)/b13-11-,14-12-SFPONTYWZIVRNN-XSYHWHKQSA-N659.8742659.452604605PW_C040696Phet4664256151187761613212049312440695Phosphatidyl-N-methylethanolamineHMDB0061372{2-[({2,3-bis[(9Z)-tetradec-9-enoyloxy]propoxy}(hydroxy)phosphoryl)oxy]ethyl}trimethylaminyl belongs to the class of organic compounds known as glycerophospholipids. These are derivatives of glycerophosphoric acid that contains at least one O-acyl, or O-alkyl, or O-(1-alkenyl) group attached to the glycerol residue.446215958CCCC\C=C/CCCCCCCC(=O)OCC(COP(O)(=O)OCC[N](C)(C)C)OC(=O)CCCCCCC\C=C/CCCCC36H69NO8PInChI=1S/C36H69NO8P/c1-6-8-10-12-14-16-18-20-22-24-26-28-35(38)42-32-34(33-44-46(40,41)43-31-30-37(3,4)5)45-36(39)29-27-25-23-21-19-17-15-13-11-9-7-2/h12-15,34H,6-11,16-33H2,1-5H3,(H,40,41)/b14-12-,15-13-CGNAOEKVHJOZMM-DZDAAMPGSA-N674.9087674.476079701PW_C040695PhNmeth46652561611877617132120494124423MagnesiumHMDB0000547Magnesium 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+86822742681647627272681158191888322936399833992211167461483491529431764142124102411592942233126293373745403147749148695449745652531045329111535611253761035906147593415160381556094161625016664841786594164688116069791997170205719420672272137233211725021473102167313198747322211763132118432101231222512324249125132881258122612729290152752851533730877137133772363297793733678393334784173357848911578522331785363567857413080020368800451848004837280623118806541358086515809652538184151938323839490027108596223110559390115687398119974406120070122120247382120702407120981408121181124121265429121319419121924125122086405122408422122759120122921399123307119123546374123835464123889455124477136124637376124978375125447297125598484125669479125777481125921482125947299125973495126000490126243478126553491126753300127125389127164501127380502127407388127451507127804209128125508128347395140773891457PotassiumHMDB0000586Potassium is an essential electrolyte. Potassium balance is crucial for regulating the excitability of nerves and muscles and so critical for regulating contractility of cardiac muscle. Although the most important changes seen in the presence of deranged potassium are cardiac, smooth muscle is also affected with increasing muscle weakness, a feature of both hyperkalaemia and hypokalaemia. Physiologically, it exists as an ion in the body. Potassium (K+) is a positively charged electrolyte, cation, which is present throughout the body in both intracellular and extracellular fluids. The majority of body potassium, >90%, are intracellular. It moves freely from intracellular fluid (ICF) to extracellular fluid (ECF) and vice versa when adenosine triphosphate increases the permeability of the cell membrane. It is mainly replaced inside or outside the cells by another cation, sodium (Na+). The movement of potassium into or out of the cells is linked to certain body hormones and also to certain physiological states. Standard laboratory tests measure ECF potassium. Potassium enters the body rapidly during food ingestion. Insulin is produced when a meal is eaten; this causes the temporary movement of potassium from ECF to ICF. Over the ensuing hours, the kidneys excrete the ingested potassium and homeostasis is returned. In the critically ill patient, suffering from hyperkalaemia, this mechanism can be manipulated beneficially by administering high concentration (50%) intravenous glucose. Insulin can be added to the glucose, but glucose alone will stimulate insulin production and cause movement of potassium from ECF to ICF. The stimulation of alpha receptors causes increased movement of potassium from ICF to ECF. A noradrenaline infusion can elevate serum potassium levels. An adrenaline infusion, or elevated adrenaline levels, can lower serum potassium levels. Metabolic acidosis causes a rise in extracellular potassium levels. In this situation, excess of hydrogen ions (H+) are exchanged for intracellular potassium ions, probably as a result of the cellular response to a falling blood pH. Metabolic alkalosis causes the opposite effect, with potassium moving into the cells. (PMID: 17883675).24203-36-9C0023881329103K%2b791DB01345[K+]KInChI=1S/K/q+1NPYPAHLBTDXSSS-UHFFFAOYSA-N39.098338.963706861FDB003521K+;Kalium;Potassium;Potassium (k+);Potassium (ion);Potassium cation;Potassium ion;Potassium ion (k+);Potassium ion (k1+);Potassium ion(+);Potassium ion(1+);Potassium monocation;Potassium(+);Potassium(1+);Potassium(1+) ion;Potassium(i) cation;K(+)PW_C000457K+573893119192622095153033663161723162713613513614615921147595215169021601181019815222306770232257711513277610111782413267824635312048412212119812412310513512376811812494445212494947212586029712596529912732220512742138814068083414068179014068778141AcceptorCompoundPW_EC00004115339ChEBIAccepto40Reduced acceptorCompoundPW_EC00004015022ChEBIRA287Alpha-aminoadipic semialdehyde dehydrogenaseP49419Multifunctional enzyme mediating important protective effects. Metabolizes betaine aldehyde to betaine, an important cellular osmolyte and methyl donor. Protects cells from oxidative stress by metabolizing a number of lipid peroxidation-derived aldehydes. Involved in lysine catabolism.
HMDBP00293ALDH7A15q31AC09951311.2.1.31; 1.2.1.3; 1.2.1.856181070314210726530Betaine--homocysteine S-methyltransferase 1Q93088Involved in the regulation of homocysteine metabolism. Converts betaine and homocysteine to dimethylglycine and methionine, respectively. This reaction is also required for the irreversible oxidation of choline.
HMDBP00559BHMT5q14.1AF11837312.1.1.556981878214211026145849118155Methionine synthaseQ99707Catalyzes the transfer of a methyl group from methyl-cobalamin to homocysteine, yielding enzyme-bound cob(I)alamin and methionine. Subsequently, remethylates the cofactor using methyltetrahydrofolate (By similarity).
HMDBP00160MTR1q43AL35918512.1.1.1357281826214284910081430121076627AdenosylhomocysteinaseP23526Adenosylhomocysteine is a competitive inhibitor of S-adenosyl-L-methionine-dependent methyl transferase reactions; therefore adenosylhomocysteinase may play a key role in the control of methylations via regulation of the intracellular concentration of adenosylhomocysteine.
HMDBP00662AHCY20q11.22AK29042213.3.1.13548189721430111076196Choline dehydrogenase, mitochondrialQ8NE62HMDBP00201CHDH3p21.1AC01246711.1.99.15633466328976171421043491421065514210896178Phosphatidylethanolamine N-methyltransferaseQ9UBM1Catalyzes three sequential methylation reactions of phosphatidylethanolamine (PE) by AdoMet, thereby producing phosphatidylcholine (PC).
HMDBP00081PEMT17p11.2AF29446612.1.1.17; 2.1.1.715761815571146662153653098086222138437757397S-adenosylmethionine synthase isoform type-2P31153Catalyzes the formation of S-adenosylmethionine from methionine and ATP.
HMDBP00405MAT2A2p11.2BC00168612.5.1.63618192721430061076146030262387Methionine adenosyltransferase 2 subunit betaQ9NZL9Non-catalytic regulatory subunit of S-adenosylmethionine synthetase 2 (MAT2A), an enzyme that catalyzes the formation of S-adenosylmethionine from methionine and ATP. Regulates the activity of S-adenosylmethionine synthetase 2 by changing its kinetic properties, rendering the enzyme more susceptible to S-adenosylmethionine inhibition.
HMDBP03965MAT2BAK3123651362819282143007107614603126167Alpha-aminoadipic semialdehyde dehydrogenase1PW_P0001671852874169Betaine--homocysteine S-methyltransferase 11PW_P00016918753047610054170Methionine synthase1PW_P0001701881551771005184Adenosylhomocysteinase1PW_P0000849862744772143508168Choline dehydrogenase, mitochondrial1PW_P0001681861961759641172Phosphatidylethanolamine N-methyltransferase1PW_P00017219078186S-adenosylmethionine synthase1PW_P00008610039721012387115042361514573667falsePW_R000667Right27419671Compoundfalse27427211Compoundtrue274314201Compoundtrue2744301Compoundfalse274511441Compoundtrue164167669falsePW_R000669Right2748301Compoundfalse27495901Compoundfalse2750621Compoundfalse27515481Compoundfalse1661692.1.1.5670falsePW_R000670Right27525901Compoundfalse275310791Compoundfalse27545481Compoundfalse275512211Compoundfalse1671702.1.1.13671falsePW_R000671Right27564141Compoundtrue27575481Compoundfalse275814201Compoundtrue27599211Compoundfalse276011041Compoundtrue27611701Compoundtrue168862.5.1.6673falsePW_R000673Right27667491Compoundfalse276714201Compoundtrue27685901Compoundfalse2769341Compoundtrue170843.3.1.1668falsePW_R000668Right2746651Compoundfalse8327411ElementCollectiontrue27479671Compoundfalse8328401ElementCollectiontrue1651681.1.99.1672falsePW_R000672Right27629211Compoundfalse2763406961Compoundfalse27647491Compoundfalse2765406951Compoundfalse1691729PW_T0000099301Compound153Right10PW_T00001010651Compound153Right769301581false120536010regular20019077030881false120578510regular200190771967881false177078510regular200190772721859false170578510regular50307731420849false167092010regular78787741144860false142579010regular503077565381false236578010regular200190788590881false1140103010regular20019078962881false48578510regular200190790548881false570103010regular200190791100589false89082510regular100257921079881false1045126010regular2001907931221881false675127010regular200190794100589false907107510regular10025795414842false340120510regular50307961420849false415107510regular7878797921881false350160010regular2001907981104846false510142010regular4443799170845false325142010regular6343812651581false236539010regular2001908137491881false1330160510regular20019099421420249false1505141010regular7878994334281false1520103010regular200190994472129false1385128720regular10025994596429false211282510regular10025994840696281false620181010regular200190994940695281false1110180010regular2002001453742389false315128010regular100251453845789false455133510regular1002522241372false226096012regular1009022340372false198096012regular1009024628788false15258408subunitregular1408525053088false8708408subunitregular1408525115582false88210908subunitregular15070543962728false135512878subunitregular14085544019622false20878408subunitregular15070544278182false91216658subunitregular15070749239786false29012858subunitregular160807493238782false42012808subunitregular150702211671782462462251691782502501057911061Cofactor2261701782512511067941066Cofactor445484172541154391956994415675Cofactor4455168172541254401957994515678Cofactor445717217254145442604686178739574927396749327811453722345Cofactor27821453822346Cofactor1033M1305 550 C1305 580 1306 747 1305 785 83false18trueM 42.84432942951901 236.81681454705455 L 50 250 L 57.839138219811716 237.21141477837804false1034M1305 785 C1305 755 363 293 387 310 5true181035M1770 880 C1740 880 1695 880 1665 880 5false181036M1730 815 C1727 848 1695 880 1665 880 5false181037M1709 920 C1712 884 1695 880 1665 880 5false181038M1405 880 C1435 880 1495 880 1525 880 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1039M1450 820 C1450 855 1495 880 1525 880 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1057M1205 880 C1168 881 1040 880 1010 880 5false181058M1240 1030 C1240 1006 1120 887 1010 880 5false181059M670 1030 C674 946 814 879 870 880 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1060M685 880 C717 880 840 880 870 880 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1061M400 400 L400 450 L450 400 z10true181062M1140 1125 C1110 1124 1062 1125 1032 1125 5false181063M1145 1260 C1145 1235 1108 1118 1032 1125 5false181064M770 1125 C810 1125 852 1125 882 1125 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1065M775 1270 C775 1247 793 1124 882 1125 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1066M400 400 L400 450 L450 400 z10true181067M775 1460 C775 1491 834 1534 887 1535 5false18trueM 624.0096189432334 1467.5 L 637 1460 L 624.0096189432334 1452.5false1068M1145 1450 C1146 1493 1083 1535 1037 1535 5false18trueM 857.7999929238226 1357.8124982102756 L 850 1345 L 842.8040475258699 1358.161241126507false1069M390 1220 C432 1219 450 1295 450 1325 5false61070M570 1125 C497 1133 455 1192 450 1325 5false61071M454 1153 C454 1183 450 1295 450 1325 5false61072M450 1600 C450 1570 450 1355 450 1325 5false6trueM 25.946855044164835 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