100ContextHypermethioninemiaHypermethioninemia is a rare error of metabolism (IEM) which arises when there is a disfunction in the gene called AHCY. This gene is responsible for Adenosylhomocysteinase, an enzyme which takes S-adenosyl homocysteine as input, and produces homocysteine as its output. This outputted compound through the its respective pathway may be turned back into cysteine methionine. A dysfunctional defect Adenosylhomocysteinase can lead to the build of of these two compounds in the blood. Of particular interest is that individuals who are affected by hypermethioninemia present a wide spectrum of symptoms. This ranges anywhere from the complete absence of symptoms, to mental retardation, muscle weakness, liver problems, and unusual facial features.DiseasePW000101CenterPathwayVisualizationContext10132004100#000099PathwayVisualization66102Methionine MetabolismMethionine metabolism is a process that is necessary for humans. Methionine metabolism in mammals happens within two pathways, a methionine cycle and a transsulfuration sequence. These pathways have three common reactions with both pathways including the transformation of methionine to S-adenosylmethionine (SAM), the use of SAM in many different transmethylation reactions resulting in a methylated product plus S-adenosylhomocysteine, and the conversion of S-adenosylhomocysteine to produce the compounds homocysteine and adenosine. The reactions mentioned above not only produce cysteine, they also create a-ketobutyrate. This compound is then converted to succinyl-CoA through a three step process after being converted to propionyl-CoA. If the amino acids cysteine and methionine are available in enough quantity, the pathway will accumulate SAM and this will in turn encourage the production of cysteine and a-ketobutyrate, which are both glucogenic, through cystathionine synthase. When there is a lack of methionine, there is a decrease in the production of SAM, which limits cystathionine synthase activity.
Metabolic113398SubPathway206570Compound213527SubPathway208448Compound213670SubPathway209590Compound281Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.102Pathway82Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.102Pathway1CellCL:000000010Glial cellCL:00001253NeuronCL:00005404CardiomyocyteCL:00007465HepatocyteCL:00001827Epithelial CellCL:00000666MyocyteCL:00001872Platelet CL:00002338Beta cellCL:000063918ErythrocyteCL:00002321Homo sapiens9606EukaryoteHuman12Mus musculus10090EukaryoteMouse17Rattus norvegicus10116EukaryoteRat3Escherichia coli562Prokaryote24Solanum lycopersicum4081EukaryoteTomato18Saccharomyces cerevisiae4932EukaryoteYeast6Caenorhabditis elegans6239EukaryoteRoundworm23Pseudomonas aeruginosa287Prokaryote5Bos taurus9913EukaryoteCattle10Drosophila melanogaster7227EukaryoteFruit fly4Arabidopsis thaliana3702EukaryoteThale cress2Bacteria2ProkaryoteBacteria19Schizosaccharomyces pombe4896Eukaryote21Xenopus laevis8355EukaryoteAfrican clawed frog25Escherichia coli (strain K12)83333Prokaryote49Bathymodiolus platifrons220390EukaryoteDeep sea mussel60Nitzschia sp.0001EukaryoteNitzschia429Saccharomyces cerevisiae (strain ATCC 204508 / S288c)559292EukaryoteBaker's yeast51Picea sitchensis3332EukaryoteSitka spruce196Homo1924EukaryoteHuman3Mitochondrial MatrixGO:00057595CytoplasmGO:00057372MitochondrionGO:00057391CytosolGO:000582931Periplasmic SpaceGO:000562011Extracellular SpaceGO:000561535ChloroplastGO:000950713Endoplasmic ReticulumGO:00057837Endoplasmic Reticulum MembraneGO:000578919Sarcoplasmic ReticulumGO:001652915NucleusGO:00056346LysosomeGO:00057644PeroxisomeGO:000577710Cell MembraneGO:000588616Lysosomal LumenGO:004320218Melanosome MembraneGO:003316225Golgi ApparatusGO:000579414Mitochondrial Outer MembraneGO:000574112Mitochondrial Inner MembraneGO:000574320Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520236MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032540PeriplasmGO:004259724Mitochondrial Intermembrane SpaceGO:000575839Mitochondrial membraneGO:003196617NucleoplasmGO:000565432Inner MembraneGO:007025827Peroxisome MembraneGO:000577826Golgi Apparatus MembraneGO:00001397Nervous SystemBTO:00014845cardiocyteBTO:00015391LiverBTO:00007597294Adrenal MedullaBTO:000004971825IntestineBTO:000064828StomachBTO:0001307155268Blood VesselBTO:0001102741111HeartBTO:000056273106KidneyBTO:00006717183Sympathetic Nervous SystemBTO:00018329MuscleBTO:00008871411824BrainBTO:000014289162Endothelium 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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-N2-{[4-({[(6S)-4-hydroxy-2-imino-1,2,5,6,7,8-hexahydropteridin-6-yl]methyl}amino)phenyl]formamido}pentanedioic acid445.4292445.170981503-3.2292-{[4-({[(6S)-4-hydroxy-2-imino-5,6,7,8-tetrahydro-1H-pteridin-6-yl]methyl}amino)phenyl]formamido}pentanedioic acid0-1FDB022705(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_C001221THFA4484571897531809253071115347112560113557861086009147706618871512057185206758316311797198426403157733613378118132120352406120482122120696407122166124123001120123301119124718118125673479125749297125771481126324299127168501127886388120L-SerineHMDB0000187Serine is a nonessential amino acid derived from glycine. Like all the amino acid building blocks of protein and peptides, serine can become essential under certain conditions, and is thus important in maintaining health and preventing disease. Low-average concentration of serine compared to other amino acids is found in muscle. Serine is highly concentrated in all cell membranes. (http://www.dcnutrition.com/AminoAcids/) L-Serine may be derived from four possible sources: dietary intake; biosynthesis from the glycolytic intermediate 3-phosphoglycerate; from glycine ; and by protein and phospholipid degradation. Little data is available on the relative contributions of each of these four sources of l-serine to serine homoeostasis. It is very likely that the predominant source of l-serine will be very different in different tissues and during different stages of human development. In the biosynthetic pathway, the glycolytic intermediate 3-phosphoglycerate is converted into phosphohydroxypyruvate, in a reaction catalyzed by 3-phosphoglycerate dehydrogenase (3- PGDH; EC 1.1.1.95). Phosphohydroxypyruvate is metabolized to phosphoserine by phosphohydroxypyruvate aminotransferase (EC 2.6.1.52) and, finally, phosphoserine is converted into l-serine by phosphoserine phosphatase (PSP; EC 3.1.3.3). In liver tissue, the serine biosynthetic pathway is regulated in response to dietary and hormonal changes. Of the three synthetic enzymes, the properties of 3-PGDH and PSP are the best documented. Hormonal factors such as glucagon and corticosteroids also influence 3-PGDH and PSP activities in interactions dependent upon the diet. L-serine plays a central role in cellular proliferation. L-Serine is the predominant source of one-carbon groups for the de novo synthesis of purine nucleotides and deoxythymidine monophosphate. It has long been recognized that, in cell cultures, L-serine is a conditional essential amino acid, because it cannot be synthesized in sufficient quantities to meet the cellular demands for its utilization. In recent years, L-serine and the products of its metabolism have been recognized not only to be essential for cell proliferation, but also to be necessary for specific functions in the central nervous system. The findings of altered levels of serine and glycine in patients with psychiatric disorders and the severe neurological abnormalities in patients with defects of L-serine synthesis underscore the importance of L-serine in brain development and function. (PMID 12534373).56-45-1C00065595117115SER5736DB00133N[C@@H](CO)C(O)=OC3H7NO3InChI=1S/C3H7NO3/c4-2(1-5)3(6)7/h2,5H,1,4H2,(H,6,7)/t2-/m0/s1MTCFGRXMJLQNBG-REOHCLBHSA-N(2S)-2-amino-3-hydroxypropanoic acid105.0926105.0425930950.663L-serine00FDB012739(-)-serine;(s)-2-amino-3-hydroxypropanoate;(s)-2-amino-3-hydroxypropanoic acid;(s)-2-amino-3-hydroxy-propanoate;(s)-2-amino-3-hydroxy-propanoic acid;(s)-serine;(s)-a-amino-b-hydroxypropionate;(s)-a-amino-b-hydroxypropionic acid;(s)-alpha-amino-beta-hydroxypropionate;(s)-alpha-amino-beta-hydroxypropionic acid;(s)-b-amino-3-hydroxypropionate;(s)-b-amino-3-hydroxypropionic acid;(s)-beta-amino-3-hydroxypropionate;(s)-beta-amino-3-hydroxypropionic acid;2-amino-3-hydroxypropanoate;2-amino-3-hydroxypropanoic acid;3-hydroxy-l-alanine;L-(-)-serine;L-3-hydroxy-2-aminopropionate;L-3-hydroxy-2-aminopropionic acid;L-3-hydroxy-alanine;L-ser;Serine;B-hydroxy-l-alanine;Beta-hydroxy-l-alanine;Beta-hydroxyalanine;(2s)-2-amino-3-hydroxypropanoic acid;(s)-(-)-serine;L-2-amino-3-hydroxypropionic acid;L-serin;S;Ser;(2s)-2-amino-3-hydroxypropanoate;(s)-α-amino-β-hydroxypropionate;(s)-α-amino-β-hydroxypropionic acid;β-hydroxy-l-alanine;B-hydroxyalanine;β-hydroxyalanine;L-2-amino-3-hydroxypropionatePW_C000120Ser344818102261745642107564310858841056011147690716370862017087202709071709172720216074383744315744416675222248357225915424912173151126251815379494233531842336315773201117808813378112132799793319485838311575239811992412212205612412213640612271813512466711812468812012531429712620929912629347912686020512777138812785650111785,10-Methylene-THFHMDB00015335,10-Methylene-THF is an intermediate in glycine, serine and threonine metabolism and one carbon metabolism. 5,10-CH2-THF can also be used as a coenzyme in the biosynthesis of thymidine. More specifically it is the C1-donor in the reactions catalyzed by thymidylate synthase and thymidylate synthase (FAD). It also acts as a coenzyme in the synthesis of serine from glycine via the enzyme serine hydroxymethyl transferase. 5,10-Methylene-THF is a substrate for Methylenetetrahydrofolate reductase. This enzyme converts 5,10-methylenetetrahydrofolate to 5-methyltetrahydrofolate. This reaction is required for the multistep process that converts the amino acid homocysteine to methionine. The body uses methionine to make proteins and other important compounds. 5,10-CH2-THF is a substrate for many enzymes including Bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase (mitochondrial), Aminomethyltransferase (mitochondrial), Serine hydroxymethyltransferase (mitochondrial), Methylenetetrahydrofolate reductase, C-1-tetrahydrofolate synthase (cytoplasmic), Serine hydroxymethyltransferase (cytosolic) and Thymidylate synthase.3432-99-3C0014343917515636METHYLENE-THF388320[H][C@@]12CN(CN1C1=C(NC2)N=C(N)NC1=O)C1=CC=C(C=C1)C(=O)NC(CCC(O)=O)C(O)=OC20H23N7O6InChI=1S/C20H23N7O6/c21-20-24-16-15(18(31)25-20)27-9-26(8-12(27)7-22-16)11-3-1-10(2-4-11)17(30)23-13(19(32)33)5-6-14(28)29/h1-4,12-13H,5-9H2,(H,23,30)(H,28,29)(H,32,33)(H4,21,22,24,25,31)/t12-,13?/m1/s1QYNUQALWYRSVHF-PZORYLMUSA-N2-({4-[(6aR)-1-hydroxy-3-imino-3H,4H,5H,6H,6aH,7H,8H,9H-imidazo[1,5-f]pteridin-8-yl]phenyl}formamido)pentanedioic acid457.4399457.170981503-2.7572-({4-[(6aR)-1-hydroxy-3-imino-4H,5H,6H,6aH,7H,9H-imidazo[1,5-f]pteridin-8-yl]phenyl}formamido)pentanedioic acid0-1FDB022675(6r)-5,10-methylenetetrahydrofolate;5,10-methenyltetrahydropteroylglutamate;5,10-methylene-6-hydrofolate;5,10-methylene-6-hydrofolic acid;5,10-methylene-thf;5,10-methylenetetrahydrofolate;5,10-methylenetetrahydrofolic acid;N5>,n10-methylenetetrahydrofolate;(6r)-5,10-methylenetetrahydrofolic acidPW_C0011785XM-THF449495689853181125331111535911257851086010147627235706518871712057196206758216342639315773391337811913212035540612068312212070440712216712412300412012329313512330911912471911812567647912576129712577948112632529912717150112788738878GlycineHMDB0000123Glycine is a simple, nonessential amino acid, although experimental animals show reduced growth on low-glycine diets. The average adult ingests 3 to 5 grams of glycine daily. Glycine is involved in the body's production of DNA, phospholipids and collagen, and in release of energy. Glycine levels are effectively measured in plasma in both normal patients and those with inborn errors of glycine metabolism. (http://www.dcnutrition.com/AminoAcids/) Nonketotic hyperglycinaemia (OMIM 606899) is an autosomal recessive condition caused by deficient enzyme activity of the glycine cleavage enzyme system (EC 2.1.1.10). The glycine cleavage enzyme system comprises four proteins: P-, T-, H- and L-proteins (EC 1.4.4.2, EC 2.1.2.10 and EC 1.8.1.4 for P-, T- and L-proteins). Mutations have been described in the GLDC (OMIM 238300), AMT (OMIM 238310), and GCSH (OMIM 238330) genes encoding the P-, T-, and H-proteins respectively. The glycine cleavage system catalyses the oxidative conversion of glycine into carbon dioxide and ammonia, with the remaining one-carbon unit transferred to folate as methylenetetrahydrofolate. It is the main catabolic pathway for glycine and it also contributes to one-carbon metabolism. Patients with a deficiency of this enzyme system have increased glycine in plasma, urine and cerebrospinal fluid (CSF) with an increased CSF: plasma glycine ratio. (PMID 16151895).56-40-6C00037525712715428GLY730DB00145NCC(O)=OC2H5NO2InChI=1S/C2H5NO2/c3-1-2(4)5/h1,3H2,(H,4,5)DHMQDGOQFOQNFH-UHFFFAOYSA-N2-aminoacetic acid75.066675.0320284090.872glycine00FDB0004842-aminoacetate;2-aminoacetic acid;Aciport;Amino-acetate;Amino-acetic acid;Aminoacetate;Aminoacetic acid;Aminoethanoate;Aminoethanoic acid;Glicoamin;Glycocoll;Glycolixir;Glycosthene;Gyn-hydralin;Padil;Aminoessigsaeure;G;Gly;Glycin;Glykokoll;Glyzin;H2n-ch2-cooh;Hgly;LeimzuckerPW_C000078Gly3141798181221881272829295420103545412055801335640107564110858631056007147701416074393744116674421511794198118721611242915115233222424193184242031577644336777421117802213278304351807081351200284061200971221201171241216874291222834351228501181242364641248374701254064791254662971254842991264484991269465011270032051270213881280185171420WaterHMDB0002111Water is a chemical substance that is essential to all known forms of life. It appears colorless to the naked eye in small quantities, though it is actually slightly blue in color. It covers 71% of Earth's surface. Current estimates suggest that there are 1.4 billion cubic kilometers (330 million m3) of it available on Earth, and it exists in many forms. It appears mostly in the oceans (saltwater) and polar ice caps, but it is also present as clouds, rain water, rivers, freshwater aquifers, lakes, and sea ice. Water in these bodies perpetually moves through a cycle of evaporation, precipitation, and runoff to the sea. Clean water is essential to human life. In many parts of the world, it is in short supply. From a biological standpoint, water has many distinct properties that are critical for the proliferation of life that set it apart from other substances. It carries out this role by allowing organic compounds to react in ways that ultimately allow replication. All known forms of life depend on water. Water is vital both as a solvent in which many of the body's solutes dissolve and as an essential part of many metabolic processes within the body. Metabolism is the sum total of anabolism and catabolism. In anabolism, water is removed from molecules (through energy requiring enzymatic chemical reactions) in order to grow larger molecules (e.g. starches, triglycerides and proteins for storage of fuels and information). In catabolism, water is used to break bonds in order to generate smaller molecules (e.g. glucose, fatty acids and amino acids to be used for fuels for energy use or other purposes). Water is thus essential and central to these metabolic processes. Water is also central to photosynthesis and respiration. Photosynthetic cells use the sun's energy to split off water's hydrogen from oxygen. Hydrogen is combined with CO2 (absorbed from air or water) to form glucose and release oxygen. All living cells use such fuels and oxidize the hydrogen and carbon to capture the sun's energy and reform water and CO2 in the process (cellular respiration). Water is also central to acid-base neutrality and enzyme function. An acid, a hydrogen ion (H+, that is, a proton) donor, can be neutralized by a base, a proton acceptor such as hydroxide ion (OH-) to form water. Water is considered to be neutral, with a pH (the negative log of the hydrogen ion concentration) of 7. Acids have pH values less than 7 while bases have values greater than 7. Stomach acid (HCl) is useful to digestion. However, its corrosive effect on the esophagus during reflux can temporarily be neutralized by ingestion of a base such as aluminum hydroxide to produce the neutral molecules water and the salt aluminum chloride. Human biochemistry that involves enzymes usually performs optimally around a biologically neutral pH of 7.4. (Wikipedia).7732-18-5C0000196215377937OH2OInChI=1S/H2O/h1H2XLYOFNOQVPJJNP-UHFFFAOYSA-Nwater18.015318.0105646861water00FDB013390Dihydrogen oxide;Steam;[oh2];Acqua;Agua;Aqua;Bound water;Dihydridooxygen;Eau;H2o;Hoh;Hydrogen hydroxide;WasserPW_C001420H2O55894910951394151316214481135261562428652106912077033823188382109431137749146554159043201824253222267860272746277817280529314370316472363461459836472737494193503027515675195975214100522794523610352971055319111534311353551125402110547012354831255492126550712755341305537114554112955911355608118562210856916575914057781015841143585314658771075890955910147594015160321556059157608716161231636133159621516218166647717865071806600152671311768401886888160716220571812077193206721121172282137238214724321572951987350216738821074012127467222749222475001907588170820122582372268414162926526118502771192216412011281122132851225028612264287123272491252022712632651269329012705291127152921300729813019300130253011303730213261223133272941534030842327315426953184369132276914293770192537710213277131133772151347737833177397332774713337751611577536334776283367772233777759341778163437798234778071329782353527824235378270356791133608001436880039370805912288065611993830383947943841105573901106393911158443981198792321199151221199634061200084071200464081201131241203654121204304051204384091206064151207944141211584251212404291213511211213814191216074341221183821223844361227531201227973741228044431230124461230643761230721371231314471231421361231624481232314511233844501237304601238104641239404551241654691246703991249384711249454721253052971253534791253864811254244821254802991256824831257074781257454871260544901262384951262734841267644801268965011269635021270173881271772081271992091272275041275065071275765151278363891280823951281765131406747901406758341407551851148Pyridoxal 5'-phosphateHMDB0001491This is the active form of vitamin B6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (pyridoxamine). -- Pubchem; Pyridoxal-phosphate (PLP, pyridoxal-5'-phosphate) is a cofactor of many enzymatic reactions. It is the active form of vitamin B6 which comprises three natural organic compounds, pyridoxal, pyridoxamine and pyridoxine. -- Wikipedia.54-47-7C00018105118405PYRIDOXAL_PHOSPHATE1022DB00114CC1=NC=C(COP(O)(O)=O)C(C=O)=C1OC8H10NO6PInChI=1S/C8H10NO6P/c1-5-8(11)7(3-10)6(2-9-5)4-15-16(12,13)14/h2-3,11H,4H2,1H3,(H2,12,13,14)NGVDGCNFYWLIFO-UHFFFAOYSA-N[(4-formyl-5-hydroxy-6-methylpyridin-3-yl)methoxy]phosphonic acid247.1419247.024573569-1.643pyridoxal phosphate0-2FDB021820Apolon b6;Biosechs;Codecarboxylase;Coenzyme b6;Hairoxal;Hexermin-p;Hi-pyridoxin;Hiadelon;Himitan;Pal-p;Plp;Phosphopyridoxal;Phosphopyridoxal coenzyme;Pidopidon;Piodel;Pydoxal;Pyridoxal 5'-phosphate;Pyridoxal 5-phosphate;Pyridoxal p;Pyridoxal phosphate;Pyridoxal-p;Pyridoxyl phosphate;Pyromijin;Sechvitan;Vitahexin-p;Vitazechs;3-hydroxy-2-methyl-5-[(phosphonooxy)methyl]-4-pyridinecarboxaldehyde;3-hydroxy-5-(hydroxymethyl)-2-methylisonicotinaldehyde 5-phosphate;Phosphoric acid mono-(4-formyl-5-hydroxy-6-methyl-pyridin-3-ylmethyl) ester;Pyridoxal 5-monophosphoric acid ester;Pyridoxal 5'-(dihydrogen phosphate);Pyridoxal-5'-phosphate;Pyridoxal 5'-phosphoric acid;3-hydroxy-5-(hydroxymethyl)-2-methylisonicotinaldehyde 5-phosphoric acid;Phosphate mono-(4-formyl-5-hydroxy-6-methyl-pyridin-3-ylmethyl) ester;Pyridoxal 5-monophosphate ester;Pyridoxal 5'-(dihydrogen phosphoric acid);Pyridoxal 5-phosphoric acid;Pyridoxal phosphoric acid;Pyridoxal-5'-phosphoric acidPW_C001148Pyr-5'P182324453518122140119696201110421450501458262120102150495325111541611754211035441118545512055671325581133653385701816071672057216212722221311858161121751511262331126281812684289126892907701725377037225770412937705222477526112777643417797334677979327782923457885533278862331806961359863071199121221200241241200294061200874071208174181211494231211554241220691231220763831228341191234024541237214581237274591246204471246273981253022971254022991254074791254584811258034891262242981262314951269423881269475011269962061272585061277865131277933901144NADHHMDB0001487NADH is the reduced form of NAD+, and NAD+ is the oxidized form of NADH, A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). It forms NADP with the addition of a phosphate group to the 2' position of the adenosyl nucleotide through an ester linkage.(Dorland, 27th ed).58-68-4C0000443915316908NADH388299DB00157NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1OC21H29N7O14P2InChI=1S/C21H29N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1,3-4,7-8,10-11,13-16,20-21,29-32H,2,5-6H2,(H2,23,33)(H,34,35)(H,36,37)(H2,22,24,25)/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BOPGDPNILDQYTO-NNYOXOHSSA-N[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]({[(2R,3S,4R,5R)-5-(3-carbamoyl-1,4-dihydropyridin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy})phosphinic acid665.441665.124771695-2.358NADH0-2FDB0226491,4-dihydronicotinamide adenine dinucleotide;Dpnh;Dihydrocodehydrogenase i;Dihydrocozymase;Dihydronicotinamide adenine dinucleotide;Dihydronicotinamide mononucleotide;Enada;Nadh;Nadh2;Reduced codehydrogenase i;Reduced diphosphopyridine nucleotide;Reduced nicotinamide adenine diphosphate;Reduced nicotinamide-adenine dinucleotide;B-dpnh;B-nadh;Beta-dpnh;Beta-nadh;Nicotinamide adenine dinucleotide (reduced);Reduced nicotinamide adenine dinucleotidePW_C001144NADH1434153349086481011152127551469542230492781172836293109948061848121848212849046495931516995524010353321115358112546612354791255593135569810057371085829141591514759451516027155607916163871647217867711176893160701118870991637172205719520674622228244226836022590862241180919811821216123202491300329813015300132552234240332242618315771071327712313377208134773713317765133677668334777003327770713077917113779863478000936880691119938221241105493881128549411583811811995540612017240712037812212098640812116242512124412612169342912181838312261638412274512012312744712313813612355137412373446012381444312424246412437139812518912112534547912553148112576229712580829912592648212651649512676748012688850112738550212809039012836239112842939514075918510795-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-N(2R)-2-[(4-{[(2-amino-5-methyl-4-oxo-3,4,5,6,7,8-hexahydropteridin-6-yl)methyl]amino}phenyl)formamido]pentanedioic acid459.4558459.186631567-3.127(2R)-2-[(4-{[(2-amino-5-methyl-4-oxo-3,6,7,8-tetrahydropteridin-6-yl)methyl]amino}phenyl)formamido]pentanedioic acid0-2DBMET00528FDB0226005-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-MTHFa57081821253331115600135717320578296132120481122122278124124832118125763297126444299128013388721NADHMDB0000902NAD (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-N1-[(2R,3R,4S,5R)-5-({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl phosphono}oxy)(hydroxy)phosphoryl]oxy}methyl)-3,4-dihydroxyoxolan-2-yl]-3-(C-hydroxycarbonimidoyl)-1lambda5-pyridin-1-ylium663.4251663.109121631-2.5281-[(2R,3R,4S,5R)-5-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl phosphono}oxy(hydroxy)phosphoryl)oxy]methyl}-3,4-dihydroxyoxolan-2-yl]-3-(C-hydroxycarbonimidoyl)-1lambda5-pyridin-1-ylium0-1FDB0223093-carbamoyl-1-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;3-carbamoyl-1-beta-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-beta-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;Adenine-nicotinamide dinucleotide;Co-i;Codehydrase i;Codehydrogenase i;Coenzyme i;Cozymase;Cozymase i;Diphosphopyridine nucleotide;Diphosphopyridine nucleotide oxidized;Endopride;Nad trihydrate;Nad-oxidized;Nicotinamide adenine dinucleotide;Nicotinamide adenine dinucleotide oxidized;Nicotinamide dinucleotide;Nicotineamide adenine dinucleotide;Oxidized diphosphopyridine nucleotide;Pyridine nucleotide diphosphate;[(3s,2r,4r,5r)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl {[(3s,2r,4r,5r)-5-(3-carbamoylpyridyl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxyphosphoryl) hydrogen phosphate;[adenylate-32-p]-nad;Beta-diphosphopyridine nucleotide;Beta-nad;Beta-nicotinamide adenine dinucleotide;Beta-nicotinamide adenine dinucleotide trihydrate;Dpn;Nad;Nad+;Nadide;B-nad;β-nadPW_C000721NAD14041503353865110111421134431273514665422294927791728352931079480718481318481928490264960315167955238103533411153601125469123548212555901355610118569610057381085827141591214759421516024155607215760761616385164691786772117689016070121887097163717420571972067405198745922282412268359225908522411819216123222491300629813018300132562234240432242619315771041327712013377209134773703317765033677667334777023327770913077915113779833477840635680006368806901199382512411055238811275016611285394119929122119952406120171407120834419120984408121159425121242126121259429121817383122614384122742120123130447123141136123419455123549374123731460123812443123829464124370398125187121125319297125342479125530481125806299125825490125924482126515495126765480126885501127278507127383502128089390128360391128428395140757185964FADHMDB0001248FAD, 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-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}[({[(2R,3S,4S)-5-{7,8-dimethyl-2,4-dioxo-2H,3H,4H,10H-benzo[g]pteridin-10-yl}-2,3,4-trihydroxypentyl]oxy}(hydroxy)phosphoryl)oxy]phosphinic acid785.5497785.157134455-2.279flavine-adenine dinucleotide0-3FDB0225111h-purin-6-amine flavin dinucleotide;1h-purin-6-amine flavine dinucleotide;Adenine-flavin dinucleotide;Adenine-flavine dinucleotide;Adenine-riboflavin dinuceotide;Adenine-riboflavin dinucleotide;Adenine-riboflavine dinucleotide;Fad;Flamitajin b;Flanin f;Flavin adenine dinucleotide;Flavin adenine dinucleotide oxidized;Flavin-adenine dinucleotide;Flavine adenosine diphosphate;Flavine-adenine dinucleotide;Flavitan;Flaziren;Isoalloxazine-adenine dinucleotide;Riboflavin 5'-adenosine diphosphate;Riboflavin-adenine dinucleotide;Riboflavine-adenine dinucleotide;AdeflavinPW_C000964FAD9991145186819232164253176282882518840211881414894216122916224921335825362237232646023646883147411347581048816526810352851025335111549612655111275613118603015560541566082161611616263901647517864991796666107703916371752057321213746522274872239076224118182161188721511899211122962251232824912443151125192271259522612710291127202921302930113041302436233187708029377126133771521347750111377507112775181157754133477615132777263377805432978375345789303317922233679272358800123688003436980714119119958406119999384120051408120107407120432405120453122120490124121278429121298418121417382121489383122748120122776121122802374122823443123066376123087135123166448123849464123868454123976399124047398125348479125378480125429482125474481125697297125979489126107299126277484126891501126920391126968502126987207127011206127310209127432506127602388127840389140790185140799186590HomocysteineHMDB0000742Homocysteine 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-N(2S)-2-amino-4-sulfanylbutanoic acid135.185135.035399227-0.963L-homocysteine00DBMET00508FDB001491(+-)-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_C000590Hcys566818242559513582642257760711178105132120476122122151124124703118125793297126310299127248205127872388548L-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-N(2S)-2-amino-4-(methylsulfanyl)butanoic acid149.211149.051049291-0.802L-methionine00DBMET00506FDB012683(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-Nzinc(2+) ion65.40963.9291465780zinc(2+) ion22FDB003729Zinc;Zinc ion;Dietary zinc;Zinc cation;Zinc, ion (zn2+);Zn(ii);Zn(2+);Zn2+PW_C001005Zinc13238411882711652915295751304468312029314770541011754251035434118545912055601325585133559813574491661178719812466226127242901332115176967225774011117758011477929336804001120020124120035406120060122120441409121257429123075137123827464125398299125413479125438297125685483126938388126953501126976205127180208570L-HomoserineHMDB0000719Homoserine is a more reactive variant of the amino acid serine. In this variant, the hydroxyl side chain contains an additional CH2 group which brings the hydroxyl group closer to its own carboxyl group, allowing it to chemically react to form a five-membered ring. This occurs at the point that amino acids normally join to their neighbours in a peptide bond. Homoserine is therefore unsuitable for forming proteins and has been eliminated from the repertoire of amino acids used by living things. Homoserine is the final product on the C-terminal end of the N-terminal fragment following a cyanogen bromide cleavage. (wikipedia).672-15-1C002631264715699HOMO-SER12126DB04193N[C@@H](CCO)C(O)=OC4H9NO3InChI=1S/C4H9NO3/c5-3(1-2-6)4(7)8/h3,6H,1-2,5H2,(H,7,8)/t3-/m0/s1UKAUYVFTDYCKQA-VKHMYHEASA-N(2S)-2-amino-4-hydroxybutanoic acid119.1192119.0582431590.553L-homoserine00FDB000522(s)-2-amino-4-hydroxybutanoate;(s)-2-amino-4-hydroxybutanoic acid;(s)-2-amino-4-hydroxy-butanoate;(s)-2-amino-4-hydroxy-butanoic acid;(s)-homoserine;2-amino-4-hydroxy-butyrate;2-amino-4-hydroxy-butyric acid;2-amino-4-hydroxy-l-butyrate;2-amino-4-hydroxy-l-butyric acid;2-amino-4-hydroxybutanoate;2-amino-4-hydroxybutanoic acid;2-amino-4-hydroxybutyrate;2-amino-4-hydroxybutyric acid;Homoserine;L-homoserine;(2s)-2-amino-4-hydroxybutanoatePW_C000570Homoser18272757516082532257830013212228112412483511812644629912801638867L-CystathionineHMDB0000099Cystathionine is a dipeptide formed by serine and homocysteine. Cystathioninuria is a prominent manifestation of vitamin-B6 deficiency. The transsulfuration of methionine yields homocysteine, which combines with serine to form cystathionine, the proximate precursor of cysteine through the enzymatic activity of cystathionase. In conditions in which cystathionine gamma-synthase or cystathionase is deficient, for example, there is cystathioninuria. Although cystathionine has not been detected in normal human serum or plasma by most conventional methods, gas chromatographic/mass spectrometric methodology detected a mean concentration of cystathionine in normal human serum of 140 nM, with a range of 65 to 301 nM.567 Cystathionine concentrations in CSF have been 10, 1, and 0.5 uM, and "not detected." Only traces (i.e., <1 uM) of cystathionine are present in normal CSF.587. gamma-Cystathionase deficiency provided the first instance in which, in a human, the major biochemical abnormality due to a defined enzyme defect was clearly shown to be alleviated by administration of large doses of pyridoxine. The response in gamma-cystathionase-deficient patients is not attributable to correction of a preexisting deficiency of this vitamin. (OMMBID, Chap. 88).56-88-2C022912524399717482L-CYSTATHIONINE388392N[C@@H](CCSC[C@H](N)C(O)=O)C(O)=OC7H14N2O4SInChI=1S/C7H14N2O4S/c8-4(6(10)11)1-2-14-3-5(9)7(12)13/h4-5H,1-3,8-9H2,(H,10,11)(H,12,13)/t4-,5-/m0/s1ILRYLPWNYFXEMH-WHFBIAKZSA-N(2S)-2-amino-4-{[(2R)-2-amino-2-carboxyethyl]sulfanyl}butanoic acid222.262222.067427636-1.114L-cystathionine00DBMET00486FDB001976(r)-s-(2-amino-2-carboxyethyl)-l-homocysteine;Cystathionine;L-(+)-cystathionine;S-[(2r)-2-amino-2-carboxyethyl]-l-homocysteine;[r-(r*,s*)]-2-amino-4-[(2-amino-2-carboxyethyl)thio]-butanoate;[r-(r*,s*)]-2-amino-4-[(2-amino-2-carboxyethyl)thio]-butanoic acid;S-(beta-amino-beta-carboxyethyl)homocysteinePW_C000067L-Cystt1048818282825722782612257812513278162111120761122122174124123358135124726118125794297126331299127249205127894388448L-CysteineHMDB0000574Cysteine is a naturally occurring, sulfur-containing amino acid that is found in most proteins, although only in small quantities. Cysteine is unique amongst the twenty natural amino acids as it contains a thiol group. Thiol groups can undergo oxidation/reduction (redox) reactions; when cysteine is oxidized it can form cystine, which is two cysteine residues joined by a disulfide bond. This reaction is reversible since the reduction of this disulphide bond regenerates two cysteine molecules. The disulphide bonds of cystine are crucial to defining the structures of many proteins. Cysteine is often involved in electron-transfer reactions, and help the enzyme catalyze its reaction. Cysteine is also part of the antioxidant glutathione. N-Acetyl-L-cysteine (NAC) is a form of cysteine where an acetyl group is attached to cysteine's nitrogen atom and is sold as a dietary supplement. Cysteine is named after cystine, which comes from the Greek word kustis meaning bladder (cystine was first isolated from kidney stones). Oxidation of cysteine can produce a disulfide bond with another thiol and further oxidation can produce sulphfinic or sulfonic acids. The cysteine thiol group is also a nucleophile and can undergo addition and substitution reactions. Thiol groups become much more reactive when they are ionized, and cysteine residues in proteins have pKa values close to neutrality, so they are often in their reactive thiolate form in the cell. The thiol group also has a high affinity for heavy metals and proteins containing cysteine will bind metals such as mercury, lead, and cadmium tightly. Due to this ability to undergo redox reactions, cysteine has antioxidant properties. Cysteine is an important source of sulfur in human metabolism, and although it is classified as a non-essential amino acid, cysteine may be essential for infants, the elderly, and individuals with certain metabolic disease or who suffer from malabsorption syndromes. Cysteine may at some point be recognized as an essential or conditionally essential amino acid (Wikipedia). Cysteine is important in energy metabolism. As cystine, it is a structural component of many tissues and hormones. Cysteine has clinical uses ranging from baldness to psoriasis to preventing smoker's hack. In some cases, oral cysteine therapy has proved excellent for treatment of asthmatics, enabling them to stop theophylline and other medications. Cysteine also enhances the effect of topically applied silver, tin, and zinc salts in preventing dental cavities. In the future, cysteine may play a role in the treatment of cobalt toxicity, diabetes, psychosis, cancer, and seizures (http://www.dcnutrition.com/AminoAcids/).52-90-4C00097586217561CYS5653DB00151N[C@@H](CS)C(O)=OC3H7NO2SInChI=1S/C3H7NO2S/c4-2(1-7)3(5)6/h2,7H,1,4H2,(H,5,6)/t2-/m0/s1XUJNEKJLAYXESH-REOHCLBHSA-N(2R)-2-amino-3-sulfanylpropanoic acid121.158121.019749163-0.723L-cysteine00DBMET00503FDB012678(+)-2-amino-3-mercaptopropionic acid;(2r)-2-amino-3-mercaptopropanoate;(2r)-2-amino-3-mercaptopropanoic acid;(2r)-2-amino-3-sulfanylpropanoate;(2r)-2-amino-3-sulfanylpropanoic acid;(r)-(+)-cysteine;(r)-2-amino-3-mercaptopropanoate;(r)-2-amino-3-mercaptopropanoic acid;(r)-2-amino-3-mercapto-propanoate;(r)-2-amino-3-mercapto-propanoic acid;(r)-cysteine;2-amino-3-mercaptopropanoate;2-amino-3-mercaptopropanoic acid;2-amino-3-mercaptopropionate;2-amino-3-mercaptopropionic acid;3-mercapto-l-alanine;Acetylcysteine;B-mercaptoalanine;Carbocysteine;Cisteina;Cisteinum;Cystein;Cysteine;Cysteinum;Free cysteine;Half-cystine;L cysteine;L-(+)-cysteine;L-2-amino-3-mercaptopropanoate;L-2-amino-3-mercaptopropanoic acid;L-2-amino-3-mercaptopropionic acid;L-cystein;L-cysteine;Polycysteine;Thioserine;Alpha-amino-beta-thiolpropionic acid;Beta-mercaptoalanine;C;Cys;E920;L-zystein;(2r)-2-amino-3-sulphanylpropanoate;(2r)-2-amino-3-sulphanylpropanoic acid;L-2-amino-3-mercaptopropionatePW_C000448Cys17481867228649287015576710158011086756117675910770781887496224759416082562278260225120122811226915142651315437303227777811177795113777961328070413512012512212013112412058012612286311812321044312549129712549829912702920512703538832-Ketobutyric acidHMDB00000052-Ketobutyric acid is a substance that is involved in the metabolism of many amino acids (glycine, methionine, valine, leucine, serine, threonine, isoleucine) as well as propanoate metabolism and C-5 branched dibasic acid metabolism. More specifically, alpha-ketobutyric acid is a product of the lysis of cystathionine. It is also one of the degradation products of threonine. It can be converted into propionyl-CoA (and subsequently methylmalonyl CoA, which can be converted into succinyl CoA, a citric acid cycle intermediate), and thus enter the citric acid cycle.600-18-0C0010958308312-OXOBUTANOATE57DB04553CCC(=O)C(O)=OC4H6O3InChI=1S/C4H6O3/c1-2-3(5)4(6)7/h2H2,1H3,(H,6,7)TYEYBOSBBBHJIV-UHFFFAOYSA-N2-oxobutanoic acid102.0886102.031694058-0.1112-oxobutanoic acid0-1FDB0033592-ketobutanoate;2-ketobutanoic acid;2-ketobutyrate;2-oxo-butanoate;2-oxo-butanoic acid;2-oxo-butyrate;2-oxo-butyric acid;2-oxo-n-butyrate;2-oxo-n-butyric acid;2-oxobutanoate;2-oxobutanoic acid;2-oxobutyrate;2-oxobutyric acid;3-methylpyruvate;3-methylpyruvic acid;Methyl-pyruvate;Methyl-pyruvic acid;Propionyl-formate;Propionyl-formic acid;A-keto-n-butyrate;A-keto-n-butyric acid;A-ketobutyrate;A-ketobutyric acid;A-oxo-n-butyrate;A-oxo-n-butyric acid;A-oxobutyrate;A-oxobutyric acid;Alpha-keto-n-butyrate;Alpha-keto-n-butyric acid;Alpha-ketobutric acid;Alpha-ketobutyrate;Alpha-ketobutyric acid;Alpha-oxo-n-butyrate;Alpha-oxo-n-butyric acid;Alpha-oxobutyrate;Alpha-oxobutyric acid;2-ketobutyric acid;3-methyl pyruvic acid;3-methyl pyruvate;α-ketobutyrate;α-ketobutyric acid;α-oxo-n-butyrate;α-oxo-n-butyric acidPW_C0000032KBA337818682269238274151838322542272478126132781631117864313379027112119922122122176124122274406122577407122716135124728118124829120125149119125312297126333299126438479126726481126858205127896388128007501128319206921S-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-O[(3S)-3-amino-3-carboxypropyl]({[(2S,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl})methylsulfanium399.445399.145063566-2.565SAMe11FDB022473(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_C000921SAMe519863330704201220318802720662468110502350560413571361637540210754421376321608266151923519511874198120312221235822515293249153451815363309768972937689916476984224774881117773133877772341780991327830335178335346791551127996136180861229483038294833386113286389113288397115543399115546401120393122120537413120939407121052124122282435123171449123505119123616118124836470125859297125879481126304299126447499127321205127340206127595388128017517749S-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-N(2S)-2-amino-4-({[(2S,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl}sulfanyl)butanoic acid384.411384.12158847-1.975S-adenosyl-L-homocysteine00DBMET00514FDB022327(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_C000749SAH5208575186353070520122131882272067246831050255056071367137163754221075462137634160826815192371951187519812359225152942491536430977489111776111307773333877773341780981327830535178337346791561127996236180863229483138294834386113287389113289397115544399115547401120394122120486125120539413120940407121053124122284435123037135123173449123506119123617118124838470125880481126303299126449499127341206127596388128019517783S-AdenosylmethioninamineHMDB0000988S-Adenosylmethioninamine is a biological sulfonium compound known as the major biological methyl donor. It is also a donor of methylene groups, amino groups, ribosyl groups and aminopropyl groups (PMID 15130560). S-Adenosylmethioninamine is a prodcut of enzyme adenosylmethionine decarboxylase [EC 4.1.1.50] in methionine metabolism pathway (KEGG).22365-13-5C0113743941515625S-ADENOSYLMETHIONINAMINE388529C[S+](CCCN)C[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2NC14H23N6O3SInChI=1S/C14H23N6O3S/c1-24(4-2-3-15)5-8-10(21)11(22)14(23-8)20-7-19-9-12(16)17-6-18-13(9)20/h6-8,10-11,14,21-22H,2-5,15H2,1H3,(H2,16,17,18)/q+1/t8-,10-,11-,14-,24?/m1/s1ZUNBITIXDCPNSD-LSRJEVITSA-N{[(2S,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl}(3-aminopropyl)methylsulfanium355.436355.155234322-2.494decarboxylated sam12FDB022353(5-deoxy-5-adenosyl)(3-aminopropyl) methylsulfonium salt;Dadomet;Decarboxylated adomet;Decarboxylated s-adenosylmethionine;Decarboxylated sam;S-adenosyl-l-methioninamine;S-adenosylmethioninamine;(5-deoxy-5-adenosyl)(3-aminopropyl)methylsulfonium;(5-deoxy-5-adenosyl)(3-aminopropyl)methylsulfonium cation;(5-deoxy-5-adenosyl)(3-aminopropyl)methylsulfonium salt;[1-(adenin-9-yl)-1,5-dideoxy-beta-d-ribofuranos-5-yl](3-aminopropyl)(methyl)sulfonium;S--adenosylmethioninamine;S-adenosyl-(5')-3-methylthiopropylamine;S-adenosyl-3-methylthiopropylamine;(5-deoxy-5-adenosyl)(3-aminopropyl)methylsulphoniumPW_C000783Dadomet12048188526787108678810778308132788361111209141221222871241234811351248411181258612971264522991273232051280223881316Carbon dioxideHMDB0001967Carbon dioxide is a colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. Carbon dioxide is produced during respiration by all animals, fungi and microorganisms that depend on living and decaying plants for food, either directly or indirectly. It is, therefore, a major component of the carbon cycle. Additionally, carbon dioxide is used by plants during photosynthesis to make sugars which may either be consumed again in respiration or used as the raw material to produce polysaccharides such as starch and cellulose, proteins and the wide variety of other organic compounds required for plant growth and development. When inhaled at concentrations much higher than usual atmospheric levels, it can produce a sour taste in the mouth and a stinging sensation in the nose and throat. These effects result from the gas dissolving in the mucous membranes and saliva, forming a weak solution of carbonic acid. Carbon dioxide is used by the food industry, the oil industry, and the chemical industry. Carbon dioxide is used to produce carbonated soft drinks and soda water. Traditionally, the carbonation in beer and sparkling wine comes about through natural fermentation, but some manufacturers carbonate these drinks artificially.124-38-9C0001128016526274O=C=OCO2InChI=1S/CO2/c2-1-3CURLTUGMZLYLDI-UHFFFAOYSA-Nmethanedione44.009543.9898292440.630carbon dioxide00DBMET00423FDB014084Carbon oxide;Carbon-12 dioxide;Carbonic acid anhydride;Carbonic acid gas;Carbonic anhydride;[co2];Co2;E 290;E-290;E290;R-744PW_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905021407981851092PutrescineHMDB0001414Putrescine is a polyamine. Putrescine is related to cadaverine (another polyamine). Both are produced by the breakdown of amino acids in living and dead organisms and both are toxic in large doses. Putrescine and cadaverine are largely responsible for the foul odor of putrefying flesh, but also contribute to the odor of such processes as bad breath and bacterial vaginosis. Putrescine is also found in semen. Putrescine attacks s-adenosyl methionine and converts it to spermidine. Spermidine in turn attacks another s-adenosyl methionine and converts it to spermine. Putrescine is synthesized in small quantities by healthy living cells by the action of ornithine decarboxylase. The polyamines, of which putrescine is one of the simplest, appear to be growth factors necessary for cell division. Putrescine apparently has specific role in skin physiology and neuroprotection. (PMID: 15009201, 16364196). Pharmacological interventions have demonstrated convincingly that a steady supply of polyamines is a prerequisite for cell proliferation to occur. Genetic engineering of polyamine metabolism in transgenic rodents has shown that polyamines play a role in spermatogenesis, skin physiology, promotion of tumorigenesis and organ hypertrophy as well as neuronal protection. Transgenic activation of polyamine catabolism not only profoundly disturbs polyamine homeostasis in most tissues, but also creates a complex phenotype affecting skin, female fertility, fat depots, pancreatic integrity and regenerative growth. Transgenic expression of ornithine decarboxylase antizyme has suggested that this unique protein may act as a general tumor suppressor. Homozygous deficiency of the key biosynthetic enzymes of the polyamines, ornithine and S-adenosylmethionine decarboxylase is not compatible with murine embryogenesis.110-60-1C00134104517148PUTRESCINE13837702DB01917NCCCCNC4H12N2InChI=1S/C4H12N2/c5-3-1-2-4-6/h1-6H2KIDHWZJUCRJVML-UHFFFAOYSA-Nbutane-1,4-diamine88.151588.1000483940.432putrescine02FDB0014941,4-butanediamine;1,4-butylenediamine;1,4-diaminobutane;1,4-tetramethylenediamine;Butylenediamine;Putrescin;Tetramethyldiamine;Tetramethylenediamine;1,4-butanediammoniumPW_C001092Putrsce120681887267111174369632278310132788371111209151221222881241234821351248421181258622971264532991273242051280233889105'-MethylthioadenosineHMDB00011735'-Methylthioadenosine (MTA) is a naturally occurring sulfur-containing nucleoside present in all mammalian tissues. It is produced from S-adenosylmethionine mainly through the polyamine biosynthetic pathway, where it behaves as a powerful inhibitory product. MTA is metabolized solely by MTA-phosphorylase, to yield 5-methylthioribose-1-phosphate and adenine, a crucial step in the methionine and purine salvage pathways, respectively. Evidence suggests that MTA can affect cellular processes in many ways. For instance, MTA has been shown to influence the regulation of gene expression, proliferation, differentiation, and apoptosis (PMID: 15313459). 5-Methylthioadenosine can be found in human urine. Elevated excretion appears in children with severe combined immunodeficiency syndrome (SCID) (PMID: 3987052).2457-80-9C00170439176175095-METHYLTHIOADENOSINE388321CSC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2NC11H15N5O3SInChI=1S/C11H15N5O3S/c1-20-2-5-7(17)8(18)11(19-5)16-4-15-6-9(12)13-3-14-10(6)16/h3-5,7-8,11,17-18H,2H2,1H3,(H2,12,13,14)/t5-,7-,8-,11-/m1/s1WUUGFSXJNOTRMR-IOSLPCCCSA-N(2R,3R,4S,5S)-2-(6-amino-9H-purin-9-yl)-5-[(methylsulfanyl)methyl]oxolane-3,4-diol297.334297.089560061-1.663methylthioadenosine00FDB0224651-(6-amino-9h-purin-9-yl)-1-deoxy-5-s-methyl-5-thio-beta-d-ribofuranose;1-(6-amino-9h-purin-9-yl)-1-deoxy-5-s-methyl-5-thio-beta-delta-ribofuranose;5'-(methylthio)-5'-deoxyadenosine;5'-(methylthio)adenosine;5'-deoxy-5'-(methylthio)adenosine;5'-methylthioadenosine;5'-s-methyl-5'-thio-adenosine;5'-s-methyl-5'-thioadenosine;5-methylthioadenosine;Mta;Methylthioadenosine;S-methyl-5'-thioadenosine;S-methyl-5-thioadenosine;Thiomethyladenosine;9-(5-s-methyl-5-thio-beta-d-ribofuranosyl)-9h-purin-6-amine;(2r,3r,4s,5s)-2-(6-aminopurin-9-yl)-5-(methylsulphanylmethyl)oxolane-3,4-diol;9-(5-s-methyl-5-thio-b-d-ribofuranosyl)-9h-purin-6-amine;9-(5-s-methyl-5-thio-β-d-ribofuranosyl)-9h-purin-6-aminePW_C000910MTA12078188827831113278838111120916122122289124123483135124843118125863297126454299127325205128024388971SpermidineHMDB0001257Spermidine is a polyamine formed from putrescine. It is found in almost all tissues in association with nucleic acids. It is found as a cation at all pH values, and is thought to help stabilize some membranes and nucleic acid structures. It is a precursor of spermine.124-20-9C00315110216610SPERMIDINE1071DB03566NCCCCNCCCNC7H19N3InChI=1S/C7H19N3/c8-4-1-2-6-10-7-3-5-9/h10H,1-9H2ATHGHQPFGPMSJY-UHFFFAOYSA-N(4-aminobutyl)(3-aminopropyl)amine145.2459145.157897623-0.653spermidine03FDB0120391,5,10-triazadecane;1,8-diamino-4-azaoctane;4-azaoctamethylenediamine;4-azaoctane-1,8-diamine;Aminopropylbutandiamine;N-(3-aminopropyl)-1,4-butane-diamine;N-(3-aminopropyl)-1,4-butanediamine;N-(3-aminopropyl)-1,4-diamino-butane;N-(3-aminopropyl)-1,4-diaminobutane;N-(3-aminopropyl)-4-aminobutylamine;N-(4-aminobutyl)-1,3-diaminopropane;N-(gamma-aminopropyl)tetramethylenediamine;Spd;Spermidin;SpermidinePW_C000971Spermd120881889267121174369332278312132788391111209171221222901241234841351248441181258642971264552991273262051280253881104PhosphateHMDB0001429Phosphate 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-Nphosphoric acid97.995297.9768950963phosphoric acid0-2DBMET00532FDB022617Nfb orthophosphate;O-phosphoric acid;Ortho-phosphate;Orthophosphate (po43-);Orthophosphate(3-);Phosphate;Phosphate (po43-);Phosphate anion(3-);Phosphate ion (po43-);Phosphate ion(3-);Phosphate trianion;Phosphate(3-);Phosphoric acid ion(3-);Pi;[po4](3-);Orthophosphate;Phosphate ion;Po4(3-);Phosphoric acid;Orthophosphoric acid;Phosphoric acid ionPW_C001104Pi24484881458181883129803176314176749250010272947273746312929316672363661385123424922447531503127515875207975216100531711153511125381103544712055431295573133560513556251085693658481435855146591114759411516040155610016162941076487178669110167141176842188688916071612057189206721221173061987389210740221274361637475222819622582582271011824110134257117481321176111511773213119041701192716412014281127282901326322334819174225530442350315424353184369232277018253771942937721713477940336779661307804833278057329782453537866933180022368892793089383138394796384110558390110640391113235941158453981162061091199824061200691221206994071210571241212161251212684291213521211214091231214233821218524051233041191236211181237861361238384641239684471239813991244053761249484721253624791254462971257744811259542991262214781265943001266042981267234841269045011274133881277832091281663951281775131283153897705-Methylthioribose 1-phosphateHMDB00009635-Methylthioribose 1-phosphate is an intermediate in methionine biosynthesis. It is converted from 5'-Deoxy-5'-methylthioadenosine by 5'-Deoxy-5'-methylthioadenosine phosphorylase. Then it is converted to methionine (PMID 2153115). In the methionine salvage pathway 5-methylthioribose 1-phosphate isomerase (M1Pi) catalyzes the conversion of 5-methylthioribose 1-phosphate (MTR-1-P) to 5-methylthioribulose 1-phosphate (MTRu-1-P).72843-83-5C041885347772027859CPD-444CSCC1OC(OP(O)(O)=O)[C@H](O)[C@@H]1OC6H13O7PSInChI=1S/C6H13O7PS/c1-15-2-3-4(7)5(8)6(12-3)13-14(9,10)11/h3-8H,2H2,1H3,(H2,9,10,11)/t3?,4-,5-,6?/m1/s1JTFITTQBRJDSTL-WATOWXBHSA-N{[(3R,4S)-3,4-dihydroxy-5-[(methylsulfanyl)methyl]oxolan-2-yl]oxy}phosphonic acid260.202260.011959972-0.964[(3R,4S)-3,4-dihydroxy-5-[(methylsulfanyl)methyl]oxolan-2-yl]oxyphosphonic acid0-2FDB0223411-phospho-5-s-methylthioribose;1-phosphomethylthioribose;5-methylthio-5-deoxy-d-ribose 1-phosphate;5-methylthio-5-deoxy-d-ribose-1-phosphate;5-methylthio-d-ribose-1-phosphate;5-methylthioribose 1-phosphate;5-methylthioribose-1-phosphate;D-ribofuranoside;S-methyl-5-thio-alpha-d-ribose 1-phosphate;S5-methyl-5-thio-d-ribose-1-phosphatePW_C0007705MTR1P189327831413212229212412484611812645729912802838834AdenosineHMDB0000050Adenosine 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-N(2R,3R,4S,5R)-2-(6-amino-9H-purin-9-yl)-5-(hydroxymethyl)oxolane-3,4-diol267.2413267.096753929-1.284adenosine00FDB0035541-(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_C000034Adenosi35281896240131556091186769107677010811825198125262494373731543738318776121327870211111992812212048712412272213512531829712645929912686420512802938865CholineHMDB0000097Choline 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-N(2-hydroxyethyl)trimethylazanium104.1708104.107539075-1.591choline11FDB000710(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_C000065Choline5623564155658149714561211956191376849712185151121971641227822615339215380497761411277619114785301157997213279980331948291249485938311328538811554111811575339812048940712049740912130640512387637612598747812647148112744020912804020630βineHMDB0000043Betaine (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-N2-(trimethylazaniumyl)acetate117.1463117.078978601-1.960(trimethylammonio)acetate00FDB009020(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_C000030βine55815559818993347025592135561813768507122822267760511177618114781041327831511212047012212049640912215012412229440712470211812484711912630929912646148112787138812803120662DimethylglycineHMDB0000092Dimethylglycine (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-N2-(dimethylamino)acetic acid103.1198103.0633285370.961dimethylglycine00FDB021893(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_C000062DMglyc5678190022554355961357760811178079112783161321204771221221254071222951241246771191248481181262824811264622991278452061280323881065OxygenHMDB0001377Oxygen is the third most abundant element in the universe after hydrogen and helium and the most abundant element by mass in the Earth's crust. Diatomic oxygen gas constitutes 20.9% of the volume of air. All major classes of structural molecules in living organisms, such as proteins, carbohydrates, and fats, contain oxygen, as do the major inorganic compounds that comprise animal shells, teeth, and bone. Oxygen in the form of O2 is produced from water by cyanobacteria, algae and plants during photosynthesis and is used in cellular respiration for all living organisms. Green algae and cyanobacteria in marine environments provide about 70% of the free oxygen produced on earth and the rest is produced by terrestrial plants. Oxygen is used in mitochondria to help generate adenosine triphosphate (ATP) during oxidative phosphorylation. For animals, a constant supply of oxygen is indispensable for cardiac viability and function. To meet this demand, an adult human, at rest, inhales 1.8 to 2.4 grams of oxygen per minute. This amounts to more than 6 billion tonnes of oxygen inhaled by humanity per year. At a resting pulse rate, the heart consumes approximately 8-15 ml O2/min/100 g tissue. This is significantly more than that consumed by the brain (approximately 3 ml O2/min/100 g tissue) and can increase to more than 70 ml O2/min/100 g myocardial tissue during vigorous exercise. As a general rule, mammalian heart muscle cannot produce enough energy under anaerobic conditions to maintain essential cellular processes; thus, a constant supply of oxygen is indispensable to sustain cardiac function and viability. However, the role of oxygen and oxygen-associated processes in living systems is complex, and they and can be either beneficial or contribute to cardiac dysfunction and death (through reactive oxygen species). Reactive oxygen species (ROS) are a family of oxygen-derived free radicals that are produced in mammalian cells under normal and pathologic conditions. Many ROS, such as the superoxide anion (O2-)and hydrogen peroxide (H2O2), act within blood vessels, altering mechanisms mediating mechanical signal transduction and autoregulation of cerebral blood flow. Reactive oxygen species are believed to be involved in cellular signaling in blood vessels in both normal and pathologic states. The major pathway for the production of ROS is by way of the one-electron reduction of molecular oxygen to form an oxygen radical, the superoxide anion (O2-). Within the vasculature there are several enzymatic sources of O2-, including xanthine oxidase, the mitochondrial electron transport chain, and nitric oxide (NO) synthases. Studies in recent years, however, suggest that the major contributor to O2- levels in vascular cells is the membrane-bound enzyme NADPH-oxidase. Produced O2- can react with other radicals, such as NO, or spontaneously dismutate to produce hydrogen peroxide (H2O2). In cells, the latter reaction is an important pathway for normal O2- breakdown and is usually catalyzed by the enzyme superoxide dismutase (SOD). Once formed, H2O2 can undergo various reactions, both enzymatic and nonenzymatic. The antioxidant enzymes catalase and glutathione peroxidase act to limit ROS accumulation within cells by breaking down H2O2 to H2O. Metabolism of H2O2 can also produce other, more damaging ROS. For example, the endogenous enzyme myeloperoxidase uses H2O2 as a substrate to form the highly reactive compound hypochlorous acid. Alternatively, H2O2 can undergo Fenton or Haber-Weiss chemistry, reacting with Fe2+/Fe3+ ions to form toxic hydroxyl radicals (-.OH). (PMID: 17027622, 15765131).7782-44-7C0000797715379CPD-6641952O=OO2InChI=1S/O2/c1-2MYMOFIZGZYHOMD-UHFFFAOYSA-Ndioxygen31.998831.9898292440singlet oxygen00FDB022589Dioxygen;Molecular oxygen;O2;Oxygen;Oxygen molecule;[oo];Dioxygene;Disauerstoff;E 948;E-948;E948PW_C001065O29591105245165001850585491462528638364910674316882075415763476933836213754920162425312228032942604247471354671235480125549312655081275809108597314761291597006188703216370501607319213753321075602128395151118162161186419811883215118942111205722512063164122472861227922612325249127062911271629213004298130163001302630113038302132602234227617426573157691029377044294772141347735011177363130773773317739533277497113775121157753733477626336777233377773611277747129777563417780511477812133780703297815113278381345788053437911136012004740812038312212042640512054240712055341412059440912060140612088341512104512412110438312160543412165642912211738212257341812268938412279837412282244312302713512306037612312844712313913612316344812317611912318745012321913712322612012345945112360911812366939812416346912421446412466939912514545412527512112542548212570647812573148312573729712574047912588448112610029912627248412652249512672148912682548012696450212698620712719820912721420812721920512722250112730550412734520612755738812757451512783538912808139512809539012831250612843239111952-Oxo-4-methylthiobutanoic acidHMDB00015532-oxo-4-Methylthiobutanoic acid, also known as 2-keto-4-methylthiobutyrate or 4-methylthio-2-oxobutanoate, belongs to the class of organic compounds known as thia fatty acids. These are fatty acid derivatives obtained by insertion of a sulfur atom at specific positions in the chain. 2-oxo-4-Methylthiobutanoic acid is slightly soluble (in water) and a weakly acidic compound (based on its pKa). 2-oxo-4-Methylthiobutanoic acid has been primarily detected in urine. Within the cell, 2-oxo-4-methylthiobutanoic acid is primarily located in the cytoplasm and adiposome. 2-oxo-4-Methylthiobutanoic acid exists in all living organisms, ranging from bacteria to humans. In humans, 2-oxo-4-methylthiobutanoic acid is involved in the methionine metabolism pathway. 2-oxo-4-Methylthiobutanoic acid is also involved in several metabolic disorders, some of which include methylenetetrahydrofolate reductase deficiency (MTHFRD), the homocystinuria-megaloblastic anemia due to defect in cobalamin metabolism, CBLG complementation type pathway, methionine adenosyltransferase deficiency, and the hypermethioninemia pathway. Outside of the human body, 2-oxo-4-methylthiobutanoic acid can be found in a number of food items such as lotus, wasabi, watercress, and chickpea. This makes 2-oxo-4-methylthiobutanoic acid a potential biomarker for the consumption of these food products. 4-Methylthio-2-oxobutanoic acid is the direct precursor of methional, which is a potent inducer of apoptosis in a BAF3 murine lymphoid cell line which is interleukin-3 (IL3)-dependent. (PMID 7848263).583-92-6C0118047333574CPD-479460DB02238CSCCC(=O)C(O)=OC5H8O3SInChI=1S/C5H8O3S/c1-9-3-2-4(6)5(7)8/h2-3H2,1H3,(H,7,8)SXFSQZDSUWACKX-UHFFFAOYSA-N4-(methylsulfanyl)-2-oxobutanoic acid148.18148.019414812-1.3012-oxo-4-thiomethylbutyric acid0-1FDB0116262-keto-4-methylthiobutyrate;2-keto-4-methylthiobutyric acid;2-ketomethiobutyrate;2-oxo-4-methylthiobutanoate;2-oxo-4-methylthiobutanoic acid;4-methylthio-2-ketobutanoate;4-methylthio-2-ketobutanoic acid;4-methylthio-2-ketobutyrate;4-methylthio-2-oxobutanoate;4-methylthio-2-oxobutanoic acid;4-methylthio-2-oxobutyrate;Kmtb;Keto-4-methylthiobutyrate;Ketomethiobutyrate;Ketomethiobutyric acid;Methylthiobutyrate;Methylthiobutyric acid;Alpha-keto-methiolbutyric acidPW_C001195KMTB190827831713212229612412484911812646329912803338835AmmoniaHMDB0000051Ammonia is a colourless alkaline gas and is one of the most abundant nitrogen-containing compounds in the atmosphere. It is an irritant with a characteristic pungent odor that is widely used in industry. Inasmuch as ammonia is highly soluble in water and, upon inhalation, is deposited in the upper airways, occupational exposures to ammonia have commonly been associated with sinusitis, upper airway irritation, and eye irritation. Acute exposures to high levels of ammonia have also been associated with diseases of the lower airways and interstitial lung. Small amounts of ammonia are naturally formed in nearly all tissues and organs of the vertebrate organism. Ammonia is both a neurotoxin and a metabotoxin. In fact, it is the most common endogenous neurotoxin. A neurotoxin is a compound that causes damage to neural tissue and neural cells. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Ammonia is recognized to be central in the pathogenesis of a brain condition known as hepatic encephalopathy, which arises from various liver diseases and leads to a build up ammonia in the blood (hyperammonemia). More than 40% of people with cirrhosis develop hepatic encephalopathy. Part of the neurotoxicity of ammonia arises from the fact that it easily crosses the blood-brain barrier and is absorbed and metabolized by the astrocytes, a population of cells in the brain that constitutes 30% of the cerebral cortex. Astrocytes use ammonia when synthesizing glutamine from glutamate. The increased levels of glutamine lead to an increase in osmotic pressure in the astrocytes, which become swollen. There is increased activity of the inhibitory gamma-aminobutyric acid (GABA) system, and the energy supply to other brain cells is decreased. This can be thought of as an example of brain edema. The source of the ammonia leading to hepatic encephalopathy is not entirely clear. The gut produces ammonia, which is metabolized in the liver, and almost all organ systems are involved in ammonia metabolism. Colonic bacteria produce ammonia by splitting urea and other amino acids, however this does not fully explain hyperammonemia and hepatic encephalopathy. The alternative explanation is that hyperammonemia is the result of the intestinal breakdown of amino acids, especially glutamine. The intestines have significant glutaminase activity, predominantly located in the enterocytes. On the other hand, intestinal tissues only have a little glutamine synthetase activity, making it a major glutamine-consuming organ. In addition to the intestine, the kidney is an important source of blood ammonia in patients with liver disease. Ammonia is also taken up by the muscle and brain in hepatic coma, and there is confirmation that ammonia is metabolized in muscle. Excessive formation of ammonia in the brains of Alzheimer's disease patients has also been demonstrated, and it has been shown that some Alzheimer's disease patients exhibit elevated blood ammonia concentrations. Ammonia is the most important natural modulator of lysosomal protein processing. Indeed, there is strong evidence for the involvement of aberrant lysosomal processing of beta-amyloid precursor protein (beta-APP) in the formation of amyloid deposits. Inflammatory processes and activation of microglia are widely believed to be implicated in the pathology of Alzheimer's disease. Ammonia is able to affect the characteristic functions of microglia, such as endocytosis, and cytokine production. Based on these facts, an ammonia-based hypothesis for Alzheimer's disease has been suggested (PMID: 17006913, 16167195, 15377862, 15369278). Chronically high levels of ammonia in the blood are associated with nearly twenty different inborn errors of metabolism including: 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, 3-methyl-crotonylglycinuria, argininemia, argininosuccinic aciduria, beta-ketothiolase deficiency, biotinidase deficiency, carbamoyl phosphate synthetase deficiency, carnitine-acylcarnitine translocase deficiency, citrullinemia type I, hyperinsulinism-hyperammonemia syndrome, hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, isovaleric aciduria, lysinuric protein intolerance, malonic aciduria, methylmalonic aciduria, methylmalonic aciduria due to cobalamin-related disorders, propionic acidemia, pyruvate carboxylase deficiency, and short chain acyl CoA dehydrogenase deficiency (SCAD deficiency). Many of these inborn errors of metabolism are associated with urea cycle disorders or impairment of amino acid metabolism. High levels of ammonia in the blood (hyperammonemia) lead to the activation of NMDA receptors in the brain. This results in the depletion of brain ATP, which in turn leads to the release of glutamate. Ammonia also leads to the impairment of mitochondrial function and calcium homeostasis, thereby decreasing ATP synthesis. Excess ammonia also increases the formation of nitric oxide (NO), which in turn reduces the activity of glutamine synthetase, and thereby decreases the elimination of ammonia in the brain (PMID: 12020609). As a neurotoxin, ammonia predominantly affects astrocytes. Disturbed mitochondrial function and oxidative stress, factors implicated in the induction of the mitochondrial permeability transition, appear to be involved in the mechanism of ammonia neurotoxicity. Ammonia can also affect the glutamatergic and GABAergic neuronal systems, the two prevailing neuronal systems of the cortical structures. All of these effects can lead to irreversible brain damage, coma, and/or death. Infants with urea cycle disorders and hyperammonemia initially exhibit vomiting and increasing lethargy. If untreated, seizures, hypotonia (poor muscle tone, floppiness), respiratory distress (respiratory alkalosis), and coma can occur. Adults with urea cycle disorders and hyperammonemia will exhibit episodes of disorientation, confusion, slurred speech, unusual and extreme combativeness or agitation, stroke-like symptoms, lethargy, and delirium. Ammonia also has toxic effects when an individual is exposed to ammonia solutions. Acute exposure to high levels of ammonia in air may be irritating to skin, eyes, throat, and lungs and cause coughing and burns. Lung damage and death may occur after exposure to very high concentrations of ammonia. Swallowing concentrated solutions of ammonia can cause burns in the mouth, throat, and stomach. Splashing ammonia into eyes can cause burns and even blindness.7664-41-7C0001422216134AMMONIA217NH3NInChI=1S/H3N/h1H3QGZKDVFQNNGYKY-UHFFFAOYSA-Nammonia17.030517.0265491011ammonia01FDB003908Ammonia anhydrous;Ammonia inhalant;Ammonia solution strong [usan];Ammonia water;Ammoniak;Liquid ammonia;Am-fol;Ammonia;Ammonia (conc 20% or greater);Ammonia gas;Ammonia solution;Ammonia solution strong (nf);Ammonia water (jp15);Ammoniac [french];Ammoniaca [italian];Ammoniacum gummi;Ammoniak [german];Ammoniak kconzentrierter;Ammoniakgas;Ammonium ion;Amoniak [polish];Anhydrous ammonia;Aromatic ammonia vaporole;Azane;Nh(3);Nh3;Nitro-sil;Primaeres amin;Sekundaeres amin;Spirit of hartshorn;Tertiaeres amin;[nh3];Ammoniac;Amoniaco;R-717;Ammonia solution strongPW_C000035NH397911251338142443824791355014146854253322257235338111601614770221607177205117861981184827711885215127082911271829276966225770462947732913377343132774693337749911377539334775971157798534777993112780723297924429380650135806571191162031091199211221200494081200531261201364071203434061203634121204624051210461241211614251221193821228003741228054431229931201230104461230963761236101181237334601246713991253112971254274821254313011255024811256634791257084781261022991262744841269665021269702071270392061271585011272002091276003881278373891783Hydrogen peroxideHMDB0003125Hydrogen peroxide (H2O2) is a very pale blue liquid which appears colourless in a dilute solution, slightly more viscous than water. It is a weak acid. It has strong oxidizing properties and is therefore a powerful bleaching agent that is mostly used for bleaching paper, but has also found use as a disinfectant and as an oxidizer. Hydrogen peroxide in the form of carbamide peroxide is widely used for tooth whitening (bleaching), both in professionally- and in self-administered products. Hydrogen peroxide (H2O2) is a well-documented component of living cells. It plays important roles in host defense and oxidative biosynthetic reactions. In addition there is growing evidence that at low levels, H2O2 also functions as a signaling agent, particularly in higher organisms. H2O2 has increasingly been viewed as an important cellular signaling agent in its own right, capable of modulating both contractile and growth-promoting pathways with more far-reaching effects. Due to the accumulation of hydrogen peroxide in the skin of patients with the depigmentation disorder vitiligo, the human epidermis cannot have the normal capacity for autocrine synthesis, transport and degradation of acetylcholine as well as the muscarinic (m1-m5) and nicotinic signal transduction in keratinocytes and melanocytes. Accumulating evidence suggests that hydrogen peroxide (H(2)O(2)) plays an important role in cancer development. Experimental data have shown that cancer cells produce high amounts of H(2)O(2). An increase in the cellular levels of H(2)O(2) has been linked to several key alterations in cancer, including DNA alterations, cell proliferation, apoptosis resistance, metastasis, angiogenesis and hypoxia-inducible factor 1 (HIF-1) activation. (PMID: 17150302, 17335854, 16677071, 16607324, 16514169).7722-84-1C0002778416240HYDROGEN-PEROXIDE763OOH2O2InChI=1S/H2O2/c1-2/h1-2HMHAJPDPJQMAIIY-UHFFFAOYSA-Nperoxol34.014734.0054793082hydrogen peroxide00FDB014562Adeka super el;Albone;Albone 35;Albone ds;Anti-keim 50;Asepticper;Baquashock;Cix;Clarigel gold;Crestal whitestrips;Crystacide;Dentasept;Deslime lp;Hioxyl;Hipox;Hybrite;Hydrogen dioxide;Hydrogen peroxide;Inhibine;Lase peroxide;Lensan a;Magic bleaching;Metrokur;Mirasept;Nite white excel 2;Odosat d;Opalescence xtra;Oxigenal;Oxydol;Oxyfull;Oxysept;Oxysept i;Pegasyl;Perhydrol;Perone;Peroxaan;Peroxclean;Quasar brite;Select bleach;Superoxol;T-stuff;Whiteness hp;Whitespeed;Xtra white;[oh(oh)];Dihydrogen dioxide;H2o2;HoohPW_C001783H2O29891135188855114627287551512433169121749512534223818104749134752315495126550212355101275810108600514770381638396151118172161188621512461226127092911271929213028301130352981304030213405222426583157702222577047294770792937750011377540334775981157772033277725337778061147781011177819326780733297815213278598112120050408120102122120463405120595409120609416120954407121047124122120382122801374122814443122839135123097376123157447123165448123220137123234452123520119123611118124672399125428482125469297125709478125732483125748488125895481126103299126275484126967502126978207127006205127201209127215208127230505127356206127601388127838389414Adenosine 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_C000414ATP9221460826616414224781373332799593439976321051821121021464921561421605824055924342727264628122930296631637236166136175143992344743147689148645450328950352651557520597521510052501045291101531311153461125390103540611754301185443120554212955561325569133560313556211085846143585414658761075897147592415160481556109161623016664931786839188687016069761997157205718420672092107225213722921172981987302216739021774082187432163748122274991908186225118472771190317012010281120391641217828512578226126912901326422315327308423263154262132242694318770282537721813477233329774683337763233678037332780413507816812878214351782403537841133578494115788501307886533178919334800283688004618480674119856291948261241132349411328238811628010911991412211999240612015440712024538212036241212124642912139212312139743312147140812197441012206512512207938312208340512240242212244443512291939912300944612381646412395144712395646812402937412452744412461613612463039812463437612494347212497237512501147012530429712537147912539229912551548112559548412612348512622030012623449512624047812654749112659649912691350112712338912773151612778139512779639012780120912811950812816751714077089132Adenosine monophosphateHMDB0000045Adenosine monophosphate, also known as 5'-adenylic acid and abbreviated AMP, is a nucleotide that is found in RNA. It is an ester of phosphoric acid with the nucleoside adenosine. AMP consists of the phosphate group, the pentose sugar ribose, and the nucleobase adenine. AMP can be produced during ATP synthesis by the enzyme adenylate kinase. AMP has recently been approved as a 'Bitter Blocker' additive to foodstuffs. When AMP is added to bitter foods or foods with a bitter aftertaste it makes them seem 'sweeter'. This potentially makes lower calorie food products more palatable.61-19-8C00020608316027AMP5858DB00131NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1C10H14N5O7PInChI=1S/C10H14N5O7P/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(22-10)1-21-23(18,19)20/h2-4,6-7,10,16-17H,1H2,(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1UDMBCSSLTHHNCD-KQYNXXCUSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}phosphonic acid347.2212347.063084339-2.025adenylate0-2DBMET00485FDB0218065'-amp;5'-adenosine monophosphate;5'-adenylate;5'-adenylic acid;Amp;Adenosine 5'-monophosphate;Adenosine 5'-phosphate;Adenosine 5'-phosphorate;Adenosine 5'-phosphoric acid;Adenosine phosphate;Adenosine-5'-monophosphorate;Adenosine-5'-monophosphoric acid;Adenosine-5-monophosphorate;Adenosine-5-monophosphoric acid;Adenosine-monophosphate;Adenosine-phosphate;Adenovite;Adenylate;Adenylic acid;Cardiomone;Lycedan;Muscle adenylate;Muscle adenylic acid;My-b-den;My-beta-den;Phosaden;Phosphaden;Phosphentaside;5'-o-phosphonoadenosine;Adenosine 5'-(dihydrogen phosphate);Adenosine monophosphate;Adenosine-5'p;Adenosini phosphas;Ado5'p;Fosfato de adenosina;Pa;Pado;Phosphate d'adenosine;5'-adenosine monophosphoric acid;Adenosine phosphoric acid;Adenosine 5'-(dihydrogen phosphoric acid);Adenosine 5'-monophosphoric acid;Adenosine monophosphoric acid;Adenosine-5'-monophosphate;Phosphoric acid d'adenosinePW_C000032AMP112344628270167343288122118914457254867545033895251104540811754231035432118545712055581325583133577910157951086977199707218811789198118681611198815112003222125802261263631126942901333122542266342646315772343297732511178392334788091157932011280399180684135809007119916122120016124120031406120246382120888405121954408122920399123464376124507374125306297125394299125409479125596484126853205126934388126949501127124389127311209127711502140771891170PyrophosphateHMDB0000250The 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-N(phosphonooxy)phosphonic acid177.9751177.9432255064pyrophosphoric acid0-3FDB021918(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_C000170Ppi12235463842923735328822212173162049241059281529417514486854503489525210452941015409117542410354331185458120554811155591325584133560613556551085879107623916669781997073188713416372721607312198731821382751518283210118691611200222212041164123152251232324912512288125792261269529015219306153751834760174256131542697318772353297731712877635336784163357892833179153112799501347995813080047372804171708563019478638494814125948193829867822311063439111327039511327538911552713611553239911993412212001712412003240612033041012093640712126142912134112112148638312240742212298544412350211912383146412404439812497737512532429712539529912541047912559748412565648512587648112655249112686920512693538812695050112733720612812450814077289177410-FormyltetrahydrofolateHMDB000097210-Formyltetrahydrofolate (10-CHO-THF) is form of tetrahydrofolate that acts as a donor of formyl groups in anabolism. In particular, 10-CHO-THF is used as a substrate in a number of formyltransferase reactions. It plays an important role in purine biosynthesis, where 10-CHO-THF is a substrate for phosphoribosylaminoimidazolecarboxamide formyltransferase, as well as in the formylation of the methionyl initiator tRNA (fMet-tRNA), when 10-CHO-THF is a substrate for methionyl-tRNA formyltransferase. 10-Formyltetrahydrofolate is a substrate for Trifunctional purine biosynthetic protein adenosine-3, Bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase (mitochondrial), 10-formyltetrahydrofolate dehydrogenase, Folylpolyglutamate synthase (mitochondrial), Bifunctional purine biosynthesis protein PURH and C-1-tetrahydrofolate synthase (cytoplasmic).2800-34-2C002341223471563710-FORMYL-THF109092NC1=NC(=O)C2=C(NCC(CN(C=O)C3=CC=C(C=C3)C(=O)N[C@@H](CCC(O)=O)C(O)=O)N2)N1C20H23N7O7InChI=1S/C20H23N7O7/c21-20-25-16-15(18(32)26-20)23-11(7-22-16)8-27(9-28)12-3-1-10(2-4-12)17(31)24-13(19(33)34)5-6-14(29)30/h1-4,9,11,13,23H,5-8H2,(H,24,31)(H,29,30)(H,33,34)(H4,21,22,25,26,32)/t11?,13-/m0/s1AUFGTPPARQZWDO-YUZLPWPTSA-N(2S)-2-[(4-{N-[(4-hydroxy-2-imino-1,2,5,6,7,8-hexahydropteridin-6-yl)methyl]formamido}phenyl)formamido]pentanedioic acid473.4393473.165896125-3.158(2S)-2-[(4-{N-[(4-hydroxy-2-imino-5,6,7,8-tetrahydro-1H-pteridin-6-yl)methyl]formamido}phenyl)formamido]pentanedioic acid0-1FDB02234510-formyl-(6rs)-tetrahydrofolic acid;10-formyl-h4pteglu1;10-formyl-thf;10-formyl-tetrahydrofolate;10-formyltetrahydrofolate;10-formyltetrahydrofolic acid;10-formyltetrahydropteroylglutamate;10-formyltetrahydropteroylglutamic acid;10-fthf;N-[p-[n-[(2-amino-5,6,7,8-tetrahydro-4-hydroxy-6-pteridinyl)methyl]formamido]benzoyl]-glutamate;N-[p-[n-[(2-amino-5,6,7,8-tetrahydro-4-hydroxy-6-pteridinyl)methyl]formamido]benzoyl]-glutamic acid;N-[p-[n-[(2-amino-5,6,7,8-tetrahydro-4-hydroxy-6-pteridinyl)methyl]formamido]benzoyl]-l-glutamate;N-[p-[n-[(2-amino-5,6,7,8-tetrahydro-4-hydroxy-6-pteridinyl)methyl]formamido]benzoyl]-l-glutamic acid;N10-formyl-5,6,7,8-tetrahydrofolate;N10-formyl-5,6,7,8-tetrahydrofolic acid;N10-formyltetrahydrofolate;N10-formyltetrahydrofolic acid;N10-formyltetrahydropteroylglutamate;N10-formyl-h4f;N10-formyl-thfPW_C00077410-FTHF94789793191825316111535011271602057188206744016611796198783221321206711221206984071223011241232851351233031191248531181257542971257734811264672991280373881342Methionine sulfoxideHMDB0002005Methionine sulfoxide is an oxidation product of methionine with reactive oxygen species via 2-electron-dependent mechanism. Such oxidants can be generated from activated neutrophils; therefore, methionine sulfoxide can be regarded as a biomarker of oxidative stress in vivo. (PMID 12576054).62697-73-884749033L-METHIONINE_SULFOXIDE824CS(=O)CC[C@H](N)C(O)=OC5H11NO3SInChI=1S/C5H11NO3S/c1-10(9)3-2-4(6)5(7)8/h4H,2-3,6H2,1H3,(H,7,8)/t4-,10?/m0/s1QEFRNWWLZKMPFJ-YGVKFDHGSA-N(2S)-2-amino-4-methanesulfinylbutanoic acid165.21165.045964392-0.492L-methionine sulfoxide00FDB0227892-amino-4-(methylsulfinyl)-butanoate;2-amino-4-(methylsulfinyl)-butanoic acid;Dl-methionine sulfoxide;L-methionine (s)-s-oxide;L-methionine r-oxide;L-methionine sulfoxide;Met-so;S-oxide-methionine;Alpha-amino-gamma-(methylsulfinyl)-butyric acid;Methionine sulfoxide;Methionine sulphoxide;2-amino-4-methylsulfinylbutanoate;2-amino-4-methylsulphinylbutanoate;2-amino-4-methylsulphinylbutanoic acidPW_C001342Met-Sof190331921278327132122307124124859118126473299128042388423MagnesiumHMDB0000547Magnesium 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+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-Npotassium(1+) ion39.098338.9637068610potassium(1+) ion11FDB003521K+;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+57389311919262209515303366316172316271361351361461592114759521516902160118101981522230677023225771151327761011178241326782463531204841221211981241231051351237681181249444521249494721258602971259652991273222051274213881406808341406817901406877819795ZincHMDB0015532Zinc is an essential element, necessary for sustaining all life. It is a trace element in the diet, forming an essential part of many enzymes, and playing an important role in protein synthesis and in cell division. 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 cell types 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. Zinc deficiency is associated with anemia, short stature, hypogonadism, impaired wound healing, and geophagia. 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 kill some organisms.7440-66-6239942736322430DB01593[Zn++]ZnInChI=1S/Zn/q+2PTFCDOFLOPIGGS-UHFFFAOYSA-Nzinc(2+) ion65.40963.9291465780zinc(2+) ion2230zn;Cinc;Zincum;Zink;Zn;Zn(ii);Zn2+PW_C009795Zinc578171121904321371721544936102940837446918454314499931668910766901016699108702016011758115122291511263365423973154239931877030253780231327832811278811111120119124120898122122308407122852118123469135124860119125486299126474481127023388127317205128043206185SarcosineHMDB0000271Sarcosine is the N-methyl derivative of glycine. Sarcosine is metabolized to glycine by the enzyme sarcosine dehydrogenase, while glycine-N-methyl transferase generates sarcosine from glycine. Sarcosine is a natural amino acid found in muscles and other body tissues. In the laboratory it may be synthesized from chloroacetic acid and methylamine. Sarcosine is naturally found in the metabolism of choline to glycine. Sarcosine is sweet to the taste and dissolves in water. It is used in manufacturing biodegradable surfactants and toothpastes as well as in other applications. Sarcosine is ubiquitous in biological materials and is present in such foods as egg yolks, turkey, ham, vegetables, legumes, etc. Sarcosine is formed from dietary intake of choline and from the metabolism of methionine, and is rapidly degraded to glycine. Sarcosine has no known toxicity, as evidenced by the lack of phenotypic manifestations of sarcosinemia, an inborn error of sarcosine metabolism. Sarcosinemia can result from severe folate deficiency because of the folate requirement for the conversion of sarcosine to glycine (Wikipedia). Sarcosine has recently been identified as a biomarker for invasive prostate cancer. It was found to be greatly increased during prostate cancer progression to metastasis and could be detected in urine. Sarcosine levels were also increased in invasive prostate cancer cell lines relative to benign prostate epithelial cells.(PMID: 19212411).107-97-1C00213108815611SARCOSINE1057CNCC(O)=OC3H7NO2InChI=1S/C3H7NO2/c1-4-2-3(5)6/h4H,2H2,1H3,(H,5,6)FSYKKLYZXJSNPZ-UHFFFAOYSA-N2-(methylamino)acetic acid89.093289.0476784730.542sarcosine00FDB021925(methylamino)acetate;(methylamino)acetic acid;(methylamino)ethanoate;(methylamino)ethanoic acid;(methylamino)-acetate;(methylamino)-acetic acid;Methylglycine;N-methyl-glycine;N-methylaminoacetate;N-methylaminoacetic acid;N-methylglycine;Sarcosin;Sarcosinate;Sarcosine;Sarcosinic acid;Megly;Methylaminoacetic acid;Sar;2-(methylamino)acetate;MethylaminoacetatePW_C000185Sar18832725563780811127830635112212740712228543512467911912483947012628448112645049912784720612802051740676Pyruvoyl groupHMDB0061359Pyruvaldehyde, also known as 2-oxopropanal or 1,2-propanedione, belongs to the class of organic compounds known as alpha ketoaldehydes. These are organic compounds containing an aldehyde substituted with a keto group on the adjacent carbon. Pyruvaldehyde exists as a solid, soluble (in water), and an extremely weak acidic (essentially neutral) compound (based on its pKa). Pyruvaldehyde has been found in human liver and kidney tissues, and has also been detected in multiple biofluids, such as urine and blood. Pyruvaldehyde exists in all living organisms, ranging from bacteria to humans. In humans, pyruvaldehyde is involved in the glycine and serine metabolism pathway, the pyruvate metabolism pathway, spermidine and spermine biosynthesis pathway, and the pyruvaldehyde degradation pathway. Pyruvaldehyde is also involved in several metabolic disorders, some of which include pyruvate dehydrogenase complex deficiency, pyruvate kinase deficiency, pyruvate decarboxylase E1 component deficiency (pdhe1 deficiency), and the NON ketotic hyperglycinemia pathway. Outside of the human body, pyruvaldehyde can be found in a number of food items such as horseradish, grass pea, ginseng, and bamboo shoots. This makes pyruvaldehyde a potential biomarker for the consumption of these food products.78-98-8C0054688017158857DB03587CC(=O)C=OC3H4O2InChI=1S/C3H4O2/c1-3(5)2-4/h2H,1H3AIJULSRZWUXGPQ-UHFFFAOYSA-N2-oxopropanal72.062772.0211293720.400methylglyoxal00C005461,2-propanedione;2-ketopropionaldehyde;2-oxopropanal;2-oxopropionaldehyde;Acetylformaldehyde;Acetylformyl;Alpha-ketopropionaldehyde;Ch3cocho;Pyruvaldehyde;Pyruvic aldehyde;A-ketopropionaldehyde;α-ketopropionaldehyde;1-ketopropionaldehyde;2-keto propionaldehyde;2-oxo-propionaldehyde;Ketopropionaldehyde;Propanedione;Propanolone;Pyroracemic aldehydePW_C040676pyruv435127884113212092312412349011812587129912733238841AcceptorCompoundPW_EC00004115339ChEBIAccepto40Reduced acceptorCompoundPW_EC00004015022ChEBIRA72oxidized thioredoxin CompoundPW_EC00007215033ChEBIOT73reduced thioredoxinCompoundPW_EC00007315033ChEBIRT9tRNA(Met)RNAPW_NA00000929173TRNAMET363819132783191327882611111993712212229812412272913512485011812532729712646429912687120512803438813935222517L-Methionyl-tRNA(Met)RNAPW_NA00001716635LMTM9863191425362112719820678320132120705407122299124123310119124851118125780481126465299128035388133687813380511113384712213388913518N-formylmethionyl-tRNA(fMet)RNAPW_NA00001817119NFTF9873191925363112719920678323132120706407122302124123311119124854118125781481126468299128038388739Serine hydroxymethyltransferase, cytosolicP34896Interconversion of serine and glycine.
HMDBP00794SHMT117p11.2L2392812.1.2.1644818132142856104414300326183Methylenetetrahydrofolate reductaseP42898Catalyzes the conversion of 5,10-methylenetetrahydrofolate to 5-methyltetrahydrofolate, a co-substrate for homocysteine remethylation to methionine.
HMDBP00188MTHFR1p36.3AF10597711.5.1.20957818222143005107614301426155Methionine 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).
HMDBP00160MTR1q43AL35918512.1.1.1357281826214284910081430121076511Cystathionine gamma-lyaseP32929Catalyzes the last step in the trans-sulfuration pathway from methionine to cysteine. Has broad substrate specificity. Converts cystathionine to cysteine, ammonia and 2-oxobutanoate. Converts two cysteine molecules to lanthionine and hydrogen sulfide. Can also accept homocysteine as substrate. Specificity depends on the levels of the endogenous substrates. Generates the endogenous signaling molecule hydrogen sulfide (H2S), and so contributes to the regulation of blood pressure. Acts as a cysteine-protein sulfhydrase by mediating sulfhydration of target proteins: sulfhydration consists of converting -SH groups into -SSH on specific cysteine residues of target proteins such as GAPDH, PTPN1 and NF-kappa-B subunit RELA, thereby regulating their function.
HMDBP00538CTH1p31.1BC01580714.4.1.1338818692986317724Cystathionine beta-synthaseP35520Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury (By similarity).
HMDBP00779CBS21q22.3BC01138114.2.1.22346818292530Betaine--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.
HMDBP00559BHMT5q14.1AF11837312.1.1.556981878214211026411S-adenosylmethionine decarboxylase proenzymeP17707HMDBP00420AMD16q21CH47105114.1.1.501205818862212Spermidine synthaseP19623Catalyzes the production of spermidine from putrescine and decarboxylated S-adenosylmethionine (dcSAM). Has a strong preference for putrescine as substrate, and has very low activity towards 1,3-diaminopropane. Has extremely low activity towards spermidine.
HMDBP00218SRM1p36-p22M6423112.5.1.161209818902389S-methyl-5'-thioadenosine phosphorylaseQ13126Catalyzes the reversible phosphorylation of S-methyl-5'-thioadenosine (MTA) to adenine and 5-methylthioribose-1-phosphate. Involved in the breakdown of MTA, a major by-product of polyamine biosynthesis. Responsible for the first step in the methionine salvage pathway after MTA has been generated from S-adenosylmethionine. Has broad substrate specificity with 6-aminopurine nucleosides as preferred substrates.
HMDBP00397MTAP9p21HE65477712.4.2.2818942627AdenosylhomocysteinaseP23526Adenosylhomocysteine 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.
HMDBP00662AHCY20q11.22AK29042213.3.1.135481897214301110763501L-amino-acid oxidaseQ96RQ9Lysosomal L-amino-acid oxidase with highest specific activity with phenylalanine. May play a role in lysosomal antigen processing and presentation (By similarity).
HMDBP08281IL4I119q13.3-q13.4AF29346311.4.3.2100912825190921442061125590Methionine--tRNA ligase, cytoplasmicP56192HMDBP00623MARS12q13X9475416.1.1.10366819123191522611Methionyl-tRNA formyltransferase, mitochondrialQ96DP5Formylates methionyl-tRNA in mitochondria. A single tRNA(Met) gene gives rise to both an initiator and an elongator species via an unknown mechanism (By similarity).
HMDBP07377MTFMT15q22.31BC03368712.1.2.9988319202397S-adenosylmethionine synthase isoform type-2P31153Catalyzes the formation of S-adenosylmethionine from methionine and ATP.
HMDBP00405MAT2A2p11.2BC00168612.5.1.636181927214300610762387Methionine 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.
HMDBP03965MAT2BAK31236513628192821430071076196Choline dehydrogenase, mitochondrialQ8NE62HMDBP00201CHDH3p21.1AC01246711.1.99.1563346632897617142104349142106551421089614379Methionine-R-sulfoxide reductase B3Q8IXL7Catalyzes the reduction of free and protein-bound methionine sulfoxide to methionine. Isoform 2 is essential for hearing.
HMDBP09183MSRB312q14.3AK29906511.8.4.-19053192224378Methionine-R-sulfoxide reductase B2, mitochondrialQ9Y3D2Catalyzes the reduction of free and protein-bound methionine sulfoxide to methionine (By similarity). Upon oxidative stress, may play a role in the preservation of mitochondrial integrity by decreasing the intracellular reactive oxygen species build-up through its scavenging role, hence contributing to cell survival and protein maintenance.
HMDBP09182MSRB210p12AF15188911.8.4.-1906319242395Glycine N-methyltransferaseQ14749Catalyzes the methylation of glycine by using S-adenosylmethionine (AdoMet) to form N-methylglycine (sarcosine) with the concomitant production of S-adenosylhomocysteine (AdoHcy). Possible crucial role in the regulation of tissue concentration of AdoMet and of metabolism of methionine.
HMDBP00403GNMT6p12BC03262712.1.1.203472289778144240234435Methionine--tRNA ligase, mitochondrialQ96GW9HMDBP09251MARS22q33.1AB10701316.1.1.10193Serine hydroxymethyltransferase, cytosolic1PW_P00019321173948811481268Methylenetetrahydrofolate reductase1PW_P00026828718321219641170Methionine synthase1PW_P0001701881551771005182Cystathionine gamma-lyase1PW_P0000829651144511481335883Cystathionine beta-synthase1PW_P00008397724446114813438169Betaine--homocysteine S-methyltransferase 11PW_P00016918753047610054335S-adenosylmethionine decarboxylase proenzyme1PW_P0003353564114428406761336Spermidine synthase1PW_P0003363572122517S-methyl-5'-thioadenosine phosphorylase1PW_P000517541389384Adenosylhomocysteinase1PW_P000084986274477214350815L-amino-acid oxidase1PW_P000015163501113964192987Methionine--tRNA ligase, cytoplasmic1PW_P00008710259013658274Methionyl-tRNA formyltransferase, mitochondrial1PW_P0002742932611186S-adenosylmethionine synthase1PW_P00008610039721012387115042361514573168Choline dehydrogenase, mitochondrial1PW_P0001681861961759641518Methionine-R-sulfoxide reductase B31PW_P0005185424379125097951519Methionine-R-sulfoxide reductase B2, mitochondrial1PW_P0005195434378125197951844Glycine N-methyltransferase1PW_P000844969395111498Methionine--tRNA ligase, mitochondrial1PW_P01149820493443513377131059falsePW_R001059Right408512211Compoundfalse40861201Compoundtrue408711781Compoundfalse4088781Compoundtrue409314201Compoundtrue7171932.1.2.1788falsePW_R000788Right319911781Compoundfalse320011441Compoundtrue320110791Compoundfalse32027211Compoundtrue3212681.5.1.20670falsePW_R000670Right27525901Compoundfalse275310791Compoundfalse27545481Compoundfalse275512211Compoundfalse1671702.1.1.131073falsePW_R001073Right4145671Compoundfalse414614201Compoundtrue41474481Compoundfalse414831Compoundtrue750824.4.1.11063falsePW_R001063Right41025701Compoundfalse41031201Compoundtrue4104671Compoundfalse410514201Compoundtrue724834.2.1.22815falsePW_R000815Right33035901Compoundfalse33041201Compoundfalse330514201Compoundtrue3306671Compoundfalse356834.2.1.221078falsePW_R001078Right4165671Compoundfalse41665901Compoundfalse7591692.1.1.51079falsePW_R001079Right41679211Compoundfalse41687831Compoundfalse416913161Compoundtrue7623354.1.1.50516falsePW_R000516Right21557831Compoundfalse215610921Compoundfalse21579101Compoundfalse21589711Compoundfalse4083362.5.1.161080falsePW_R001080Right41709101Compoundfalse417111041Compoundtrue41727701Compoundfalse41735481Compoundtrue7645172.4.2.28673falsePW_R000673Right27667491Compoundfalse276714201Compoundtrue27685901Compoundfalse2769341Compoundtrue170843.3.1.1669falsePW_R000669Right2748301Compoundfalse27495901Compoundfalse2750621Compoundfalse27515481Compoundfalse1661692.1.1.5429falsePW_R000429Right17715481Compoundfalse177214201Compoundtrue177310651Compoundtrue177411951Compoundfalse1775351Compoundtrue177617831Compoundtrue774151.4.3.21086falsePW_R001086Right41905481Compoundfalse41914141Compoundtrue419291NucleicAcidtrue4193171NucleicAcidfalse4194321Compoundtrue41951701Compoundtrue776876.1.1.10168817114986.1.1.10795falsePW_R000795Right32297741Compoundfalse3230171NucleicAcidfalse323112211Compoundfalse3232181NucleicAcidfalse3302742.1.2.9671falsePW_R000671Right27564141Compoundtrue27575481Compoundfalse275814201Compoundtrue27599211Compoundfalse276011041Compoundtrue27611701Compoundtrue168862.5.1.61084falsePW_R001084Right4186651Compoundfalse8384411ElementCollectiontrue4187301Compoundfalse8385401ElementCollectiontrue7691681.1.99.11085falsePW_R001085Right41885481Compoundfalse8386721ElementCollectiontrue418913421Compoundfalse8387731ElementCollectiontrue7715181.8.4.-2284falsePW_R002284Right83885481Compoundfalse8389721ElementCollectiontrue839013421Compoundfalse8391731ElementCollectiontrue21645191.8.4.-1781falsePW_R001781Both66701851Compoundfalse66717491Compoundtrue66729211Compoundtrue6673781Compoundfalse15208442.1.1.2031421221281false264764010regular2001903143120281false253230010regular20019031441178281false198245510regular200190314578281false221230010regular2001903146142023true84761510regular1001003147114829false241749510regular100353157114423true136263010regular10010031581079281false198285010regular200190315972123true149299010regular100100316096429false202770019regular100253163590281false1922120510regular2001903164548281false2802120510regular2001903165100529false244289010regular100253184570281false131735010regular200190318567281false131785010regular20019033091420249false1222102010regular78783310448281false77766010regular20019033113281false777107510regular2001903312114829false109789510regular100353333120281false152250010regular20019033341420249false152276510regular78783335114829false137265019regular100353346120281false1627135010regular20019033471420249false1377135510regular78783348114829false1527124510regular100353351100529false174989519regular1002533539212781false602155010regular20019033557492781false602204010regular2001903357783281false1732154810regular20019033581316252false1388169410regular787833591092281false1527170310regular2001903360910281false1732205810regular2001903361971281false1527191310regular20019033691104246false1692224810regular44433370770281false1162205810regular2002003371548281false1292225310regular2001903375142023true507203010regular100100337634281false1612262510regular200190337772129false1407247019regular10025338865381false2237188510regular200190338930381false2237140510regular200190339162281false2587136510regular2001903392100529false2477125010regular1002534051420249false3052117010regular787834061065265false2977110010regular787834071195281false3492120010regular200200340835263false3352117010regular787834091783256false3427110510regular7878341096429false3197126010regular100253419414242false3272158010regular5030342032244false3277175010regular50303421170245false3082174510regular63433425774281false3372195710regular20019034261221281false3372222010regular20019034271342281false2802229510regular2001903437414242false193235510regular50303438142023true2507128010regular10010034391104246false163234510regular44433440170245false154740010regular6343344142329false176722519regular10025344245729false176720519regular10025999596439false2292169520regular100259996979539false2752206219regular100259997979539false2947206019regular1002531772071852381false807192010regular2001903177208782381false8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envelope7221450201.61.620015200235Mitochondria33721780201.31.320015201235Mitochondria23421590201.31.3200157531642782261018543785260641#FFEBEB41175752HypermethioninemiaHypermethioninemia is a rare error of metabolism (IEM) which arises when there is a disfunction in the gene called AHCY. This gene is responsible for Adenosylhomocysteinase, an enzyme which takes S-adenosyl homocysteine as input, and produces homocysteine as its output. This outputted compound through the its respective pathway may be turned back into cysteine methionine. A dysfunctional defect Adenosylhomocysteinase can lead to the build of of these two compounds in the blood. Of particular interest is that individuals who are affected by hypermethioninemia present a wide spectrum of symptoms. This ranges anywhere from the complete absence of symptoms, to mental retardation, muscle weakness, liver problems, and unusual facial features.DiseasePW_X000100Context100447627ProteinMutated448548CompoundIncreased449448CompoundIncreased1117[Uniprot: Q00266](http://www.uniprot.org/uniprot/Q00266)100Context1118[OMIM: Entry 250850](http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=250850)100Context11191527987Blom HJ, Davidson AJ, Finkelstein JD, Luder AS, Bernardini I, Martin JJ, Tangerman A, Trijbels JM, Mudd SH, Goodman SI, et al.: Persistent hypermethioninaemia with dominant inheritance. J Inherit Metab Dis. 1992;15(2):188-97.100Context11209042912Chamberlin ME, Ubagai T, Mudd SH, Levy HL, Chou JY: Dominant inheritance of isolated hypermethioninemia is associated with a mutation in the human methionine adenosyltransferase 1A gene. Am J Hum Genet. 1997 Mar;60(3):540-6.100Context11211191305Finkelstein JD, Kyle WE, Martin JJ: Abnormal methionine adenosyltransferase in hypermethioninemia. Biochem Biophys Res Commun. 1975 Oct 27;66(4):1491-7.100Context11227229751Gaull GE, Tallan HH, Lonsdale D, Przyrembel H, Schaffner F, von Bassewitz DB: Hypermethioninemia associated with methionine adenosyltransferase deficiency: clinical, morphologic, and biochemical observations on four patients. J Pediatr. 1981 May;98(5):734-41.100Context27840927465642Schweinberger BM, Wyse AT: Mechanistic basis of hypermethioninemia. Amino Acids. 2016 Nov;48(11):2479-2489. doi: 10.1007/s00726-016-2302-4. Epub 2016 Jul 27.100Context