8997ContextNon-Ketotic HyperglycinemiaNon Ketotic Hyperglycinemeia (Glycine encephalopathy; Glycine cleavage system deficiency; NKH) is caused by mutations in several genes in the mitochondrial glycine cleavage system. These include the genes encoding P protein (GLDC), T protein (GCST), and, in one case, the H protein (GCSH). Most patients with GCE (Glycine Encephalopathy, or NKH) have a defect in the GLDC gene.The enzyme system for cleavage of glycine (glycine cleavage system), which is confined to the mitochondria, is composed of 4 protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). NKH is characterized by accumulation of glycine in plasma, spinal fluid and urine. Symptoms include seizures, respiratory distress, mental retardation, chorea, visual impairment and hydrocephalus.DiseasePW121790CenterPathwayVisualizationContext12206630503500#000099PathwayVisualization6435064466Glycine and Serine MetabolismThis pathway describes the synthesis and breakdown of several small amino acids, including glycine, serine, and cysteine. All of these compounds share common intermediates and almost all can be biosynthesized from one another. Serine and glycine are not essential amino acids and can be synthesized from several routes. On the other hand, cysteine is a conditionally essential amino acid, meaning that it can be endogenously synthesized but insufficient quantities may be produced due to certain diseases or conditions. Serine is central to the synthesis and breakdown of the other two amino acids. Serine can be synthesized via glycerate, which can be converted into glycerate 3-phosphate (via glycerate kinase), which in turn is converted into phosphohydroxypyruvate by phosphoglycerate dehydrogenase and then phosphoserine (via phosphoserine transaminase) and finally to serine (via phosphoserine phosphatase). The serine synthesized via this route can be used to create cysteine and glycine through the homocysteine cycle. In the homocysteine cycle, cystathionine beta-synthase catalyzes the condensation of homocysteine and serine to give cystathionine. Cystathionine beta-lyase then converts this double amino acid to cysteine, ammonia, and alpha-ketoglutarate. Glycine is biosynthesized in the body from the amino acid serine. In most organisms, the enzyme serine hydroxymethyltransferase (SHMT) catalyzes this transformation using tetrahydrofolate (THF), leading to methylene THF and glycine. Glycine can be degraded via three pathways. The predominant pathway in animals involves the glycine cleavage system, also known as the glycine decarboxylase complex or GDC. This system is usually triggered in response to high concentrations of glycine. The system is sometimes referred to as glycine synthase when it runs in the reverse direction to produce glycine. The glycine cleavage system consists of four weakly interacting proteins: T, P, L and H-proteins. The glycine cleavage system leads to the degradation of glycine into ammonia and CO2. In the second pathway, glycine is degraded in two steps. The first step in this degradation pathway is the reverse of glycine biosynthesis from serine with serine hydroxymethyltransferase (SHMT). The serine generated via glycine is then converted into pyruvate by the enzyme known as serine dehydratase. In the third route to glycine degradation, glycine is converted into glyoxylate by D-amino acid oxidase. Glyoxylate is then oxidized by hepatic lactate dehydrogenase into oxalate in an NAD+-dependent reaction.Metabolic1210802727SubPathway107076448Compound13210802884SubPathway107077164Compound13210802977SubPathway107078644Compound13210803076SubPathway107079644Compound13210803197SubPathway107080120Compound132229720Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.64466Pathway229721Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.64466Pathway1CellCL:00000002Platelet CL:00002335HepatocyteCL:00001824Cardiomyocyte CL:00007463NeuronCL:00005407Epithelial CellCL:00000666MyocyteCL:000018710Glial cellCL:00001258Beta cellCL:00006391Homo sapiens9606EukaryoteHuman12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat10Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm3Escherichia coli562Prokaryote24Solanum lycopersicum4081EukaryoteTomato18Saccharomyces cerevisiae4932EukaryoteYeast4Arabidopsis thaliana3702EukaryoteThale cress49Bathymodiolus platifrons220390EukaryoteDeep sea mussel23Pseudomonas aeruginosa287Prokaryote2Bacteria2ProkaryoteBacteria19Schizosaccharomyces pombe4896Eukaryote21Xenopus laevis8355EukaryoteAfrican clawed frog25Escherichia coli (strain K12)83333Prokaryote60Nitzschia sp.0001EukaryoteNitzschia429Saccharomyces cerevisiae (strain ATCC 204508 / S288c)559292EukaryoteBaker's yeast51Picea sitchensis3332EukaryoteSitka spruce14Mitochondrial Outer MembraneGO:00057416LysosomeGO:00057644PeroxisomeGO:000577713Endoplasmic ReticulumGO:00057835CytoplasmGO:000573710Cell MembraneGO:000588616Lysosomal LumenGO:00432022MitochondrionGO:00057397Endoplasmic Reticulum MembraneGO:00057891CytosolGO:000582911Extracellular SpaceGO:00056153Mitochondrial MatrixGO:000575918Melanosome MembraneGO:003316224Mitochondrial Intermembrane SpaceGO:000575835ChloroplastGO:000950736MembraneGO:001602012Mitochondrial Inner MembraneGO:000574325Golgi apparatusGO:000579420Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520215NucleusGO:000563431Periplasmic SpaceGO:000562053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032540PeriplasmGO:004259732Inner MembraneGO:007025839Mitochondrial membraneGO:00319668Smooth Endoplasmic Reticulum GO:000579027Peroxisome MembraneGO:000577819sarcoplasmic reticulumGO:001652917NucleoplasmGO:000565426Golgi apparatus membraneGO:00001392Endothelium BTO:00003931LiverBTO:00007597294Adrenal MedullaBTO:000004971825IntestineBTO:000064828StomachBTO:0001307155267Nervous SystemBTO:00014848Blood VesselBTO:0001102741111HeartBTO:000056273109MuscleBTO:00008871411824BrainBTO:000014289163Sympathetic Nervous 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0128184121PW_BS0000244101551PW_BS00011544415171PW_BS00011548515101PW_BS0001155161561PW_BS000115471914PW_BS00004731323PW_BS0000241873118PW_BS000024241529PW_BS00002425715291PW_BS00002430412PW_BS000024215114PW_BS000021562611PW_BS000056372102PW_BS00002848113PW_BS000048219314PW_BS00002430635511PW_BS0000241435AminoacetoneHMDB0002134Threonine dehydrogenase catalyzes the oxidation of threonine by NAD+ to glycine and acetyl-CoA, but when the ratio acetyl-CoA/CoA increases in nutritional deprivation (e.g., in diabetes) the enzyme produces aminoacetone (Chem. Res. Toxicol., 14 (9), 1323 -1329, 2001). Aminoacetone is thought to be a substrate for SSAO (semicarbazide-sensitive amine oxidase), leading to the production of the toxic product methylglyoxal (Journal of Chromatography B. Volume 824, Issues 1-2 , 25 September 2005, Pages 116-122 ).298-08-8C0188821517906AMINO-ACETONE210CC(=O)CNC3H7NOInChI=1S/C3H7NO/c1-3(5)2-4/h2,4H2,1H3BCDGQXUMWHRQCB-UHFFFAOYSA-N1-aminopropan-2-one73.093873.0527638510.861α-aminoacetone01FDB0228601-amino-(8ci,9ci)-2-propanone;1-amino-2-propanone;1-aminopropan-2-one;Amino-(6ci)-2-propanone;Amino-2-propanone;Alpha-aminoacetone;1-amino-propan-2-one;AmmonioacetonePW_C001435Amnactn253022780693291221163821246683991262714841278343891065OxygenHMDB0001377Oxygen 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-Noxidanone31.998831.9898292440singlet oxygen00FDB022589Dioxygen;Molecular oxygen;O2;Oxygen;Oxygen molecule;[oo];Dioxygene;Disauerstoff;E 948;E-948;E948PW_C001065O2959110524516500185058549146252863836491067431688207541576347693383621375492016242531222803294260424747135467123548012554931265508127580910859731476129159700618870321637050160731921375332107560212839515111816216118641981188321511894211120572251206316412247286122792261232524912706291127162921300429813016300130263011303830213260223422761742657315769102937704429477214134773501117736313077377331773953327749711377512115775373347762633677723337777361127774712977756341778051147781213378070329781511327838134578805343791113601200474081203831221204264051205424071205534141205944091206014061208834151210451241211043831216054341216564291221173821225734181226893841227983741228224431230271351230603761231284471231391361231634481231761191231874501232191371232261201234594511236091181236693981241634691242144641246693991251454541252751211254254821257064781257314831257372971257404791258844811261002991262724841265224951267214891268254801269645021269862071271982091272142081272192051272225011273055041273452061275573881275745151278353891280813951280953901283125061284323911420WaterHMDB0002111Water 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_C001420H2O558949109513941513162144811352615624286521069120770338231883821094311377491465541590432018242532222678602727462778172805293143703164723634614598364727374941935030275156751959752141005227945236103529710553191115343113535511254021105470123548312554921265507127553413055371145541129559113556081185622108569165759140577810158411435853146587710758909559101475940151603215560591576087161612316361331596215162181666477178650718066001526713117684018868881607162205718120771932067211211722821372382147243215729519873502167388210740121274672227492224750019075881708201225823722684141629265261185027711922164120112811221328512250286122642871232724912520227126326512693290127052911271529213007298130193001302530113037302132612231332729415340308423273154269531843691322769142937701925377102132771311337721513477378331773973327747133377516115775363347762833677722337777593417781634377982347780713297823535278242353782703567911336080014368800393708059122880656119938303839479438411055739011063939111584439811987923211991512211996340612000840712004640812011312412036541212043040512043840912060641512079441412115842512124042912135112112138141912160743412211838212238443612275312012279737412280444312301244612306437612307213712313144712314213612316244812323145112338445012373046012381046412394045512416546912467039912493847112494547212530529712535347912538648112542448212548029912568248312570747812574548712605449012623849512627348412676448012689650112696350212701738812717720812719920912722750412750650712757651512783638912808239512817651335AmmoniaHMDB0000051Ammonia 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_C001783H2O29891135188855114627287551512433169121749512534223818104749134752315495126550212355101275810108600514770381638396151118172161188621512461226127092911271929213028301130352981304030213405222426583157702222577047294770792937750011377540334775981157772033277725337778061147781011177819326780733297815213278598112120050408120102122120463405120595409120609416120954407121047124122120382122801374122814443122839135123097376123157447123165448123220137123234452123520119123611118124672399125428482125469297125709478125732483125748488125895481126103299126275484126967502126978207127006205127201209127215208127230505127356206127601388127838389905PyruvaldehydeHMDB0001167Pyruvaldehyde is an organic compound used often as a reagent in organic synthesis, as a flavoring agent, and in tanning. It has been demonstrated as an intermediate in the metabolism of acetone and its derivatives in isolated cell preparations, in various culture media, and in vivo in certain animals.78-98-8C0054688017158METHYL-GLYOXAL857DB03587CC(=O)C=OC3H4O2InChI=1S/C3H4O2/c1-3(5)2-4/h2H,1H3AIJULSRZWUXGPQ-UHFFFAOYSA-N2-oxopropanal72.062772.0211293720.400methylglyoxal00FDB0082951,2-propanedione;1-ketopropionaldehyde;2-keto propionaldehyde;2-ketopropionaldehyde;2-oxo-propionaldehyde;2-oxopropanal;Acetylformaldehyde;Acetylformyl;Ketopropionaldehyde;Methylglyoxal;Propanedione;Propanolone;Pyroracemic aldehyde;Pyruvaldehyde;Pyruvic aldehyde;Alpha-ketopropionaldehyde;2-oxopropionaldehyde;Ch3cocho;A-ketopropionaldehyde;α-ketopropionaldehydePW_C000905Pyruval117782535227807432978759111120875122122121382123451135124673399125851297126276484127525205127839389964FADHMDB0001248FAD, 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)CO[P@](O)(=O)O[P@@](O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=NC(=O)NC(=O)C1=N2C27H33N9O15P2InChI=1S/C27H33N9O15P2/c1-10-3-12-13(4-11(10)2)35(24-18(32-12)25(42)34-27(43)33-24)5-14(37)19(39)15(38)6-48-52(44,45)51-53(46,47)49-7-16-20(40)21(41)26(50-16)36-9-31-17-22(28)29-8-30-23(17)36/h3-4,8-9,14-16,19-21,26,37-41H,5-7H2,1-2H3,(H,44,45)(H,46,47)(H2,28,29,30)(H,34,42,43)/t14-,15+,16+,19-,20+,21+,26+/m0/s1VWWQXMAJTJZDQX-UYBVJOGSSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}[({[(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_C000964FAD9991145186819232164253176282882518840211881414894216122916224921335825362237232646023646883147411347581048816526810352851025335111549612655111275613118603015560541566082161611616263901647517864991796666107703916371752057321213746522274872239076224118182161188721511899211122962251232824912443151125192271259522612710291127202921302930113041302436233187708029377126133771521347750111377507112775181157754133477615132777263377805432978375345789303317922233679272358800123688003436980714119119958406119999384120051408120107407120432405120453122120490124121278429121298418121417382121489383122748120122776121122802374122823443123066376123087135123166448123849464123868454123976399124047398125348479125378480125429482125474481125697297125979489126107299126277484126891501126920391126968502126987207127011206127310209127432506127602388127840389721NADHMDB0000902NAD (or Nicotinamide adenine dinucleotide) is used extensively in glycolysis and the citric acid cycle of cellular respiration. The reducing potential stored in NADH can be converted to ATP through the electron transport chain or used for anabolic metabolism. ATP "energy" is necessary for an organism to live. Green plants obtain ATP through photosynthesis, while other organisms obtain it by cellular respiration. (wikipedia). Nicotinamide adenine dinucleotide is a A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed).53-84-9C00003589315846NAD5682NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1OC21H28N7O14P2InChI=1S/C21H27N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1-4,7-8,10-11,13-16,20-21,29-32H,5-6H2,(H5-,22,23,24,25,33,34,35,36,37)/p+1/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BAWFJGJZGIEFAR-NNYOXOHSSA-O1-[(2R,3R,4S,5R)-5-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium664.433664.116946663-2.5981-[(2R,3R,4S,5R)-5-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium1-1FDB0223093-carbamoyl-1-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;3-carbamoyl-1-beta-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-beta-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;Adenine-nicotinamide dinucleotide;Co-i;Codehydrase i;Codehydrogenase i;Coenzyme i;Cozymase;Cozymase i;Diphosphopyridine nucleotide;Diphosphopyridine nucleotide oxidized;Endopride;Nad trihydrate;Nad-oxidized;Nicotinamide adenine dinucleotide;Nicotinamide adenine dinucleotide oxidized;Nicotinamide dinucleotide;Nicotineamide adenine dinucleotide;Oxidized diphosphopyridine nucleotide;Pyridine nucleotide diphosphate;[(3s,2r,4r,5r)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl {[(3s,2r,4r,5r)-5-(3-carbamoylpyridyl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxyphosphoryl) hydrogen phosphate;[adenylate-32-p]-nad;Beta-diphosphopyridine nucleotide;Beta-nad;Beta-nicotinamide adenine dinucleotide;Beta-nicotinamide adenine dinucleotide trihydrate;Dpn;Nad;Nad+;Nadide;B-nad;β-nadPW_C000721NAD14041503353865110111421134431273514665422294927791728352931079480718481318481928490264960315167955238103533411153601125469123548212555901355610118569610057381085827141591214759421516024155607215760761616385164691786772117689016070121887097163717420571972067405198745922282412268359225908522411819216123222491300629813018300132562234240432242619315771041327712013377209134773703317765033677667334777023327770913077915113779833477840635680006368806901199382512411055238811275016611285394119929122119952406120171407120834419120984408121159425121242126121259429121817383122614384122742120123130447123141136123419455123549374123731460123812443123829464124370398125187121125319297125342479125530481125806299125825490125924482126515495126765480126885501127278507127383502128089390128360391128428395164Pyruvic acidHMDB0000243Pyruvic acid is an intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed.) Biological Source: Intermediate in primary metabolism including fermentation processes. Present in muscle in redox equilibrium with Lactic acid. A common constituent, as a chiral cyclic acetal linked to saccharide residues, of bacterial polysaccharides. Isolated from cane sugar fermentation broth and peppermint. Constituent of Bauhinia purpurea, Cicer arietinum (chickpea), Delonix regia, Pisum sativum (pea) and Trigonella caerulea (sweet trefoil) Use/Importance: Reagent for regeneration of carbonyl compdounds from semicarbazones, phenylhydrazones and oximes. Flavoring ingredient (Dictionary of Organic Compounds).127-17-3C00022106032816PYRUVATE1031DB00119CC(=O)C(O)=OC3H4O3InChI=1S/C3H4O3/c1-2(4)3(5)6/h1H3,(H,5,6)LCTONWCANYUPML-UHFFFAOYSA-N2-oxopropanoic acid88.062188.0160439940.181pyruvic acid0-1FDB0082932-oxopropanoate;2-oxopropanoic acid;2-oxopropionate;2-oxopropionic acid;Acetylformate;Acetylformic acid;Bts;Pyroracemate;Pyroracemic acid;Pyruvate;A-ketopropionate;A-ketopropionic acid;Alpha-ketopropionate;Alpha-ketopropionic acid;2-ketopropionic acid;2-oxopropansaeure;2-oxopropionsaeure;Acide pyruvique;Alpha-oxopropionsaeure;Brenztraubensaeure;Ch3cocooh;2-ketopropionate;α-ketopropionate;α-ketopropionic acid;A-oxopropionsaeure;α-oxopropionsaeurePW_C000164Pyr1722044228118131449501457265365103540511754401185444120556613255701335893955920147595115160221556067156607416161261606383164671786510177653285745722274952208200225126223115292249153491877310111779723467797832778090112800043688004236780695135112879941156831211199504061200111241201751221208784071211484231211544241234541191237204581237264591253404791253902991255342971258544811268835011269313881270672051278582061144NADHHMDB0001487NADH 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_C001144NADH143415334908648101115212755146954223049278117283629310994806184812184821284904649593151699552401035332111535811254661235479125559313556981005737108582914159151475945151602715560791616387164721786771117689316070111887099163717220571952067462222824422683602259086224118091981182121612320249130032981301530013255223424033224261831577107132771231337720813477371331776513367766833477700332777071307791711377986347800093688069111993822124110549388112854941158381181199554061201724071203781221209864081211624251212441261216934291218183831226163841227451201231274471231381361235513741237344601238144431242424641243713981251891211253454791255314811257622971258082991259264821265164951267674801268885011273855021280903901283623911284293952747L-2-Amino-3-oxobutanoic acidHMDB0006454L-2-Amino-3-oxobutanoic acid or L-2-amino acetic acid is involved in glycine/serine metabolism and is a breakdown product from glycine. It spontaneously decomposes to aminoacetone. Delta-aminolevuliinate synthase is the enzyme that catalyzes the interconversion between glycine and L-2-amino-3-oxobutanoic acid. Glycine C-acetyltransferase is also capable of catalyzing this reaction.C0350844003340673AMINO-OXOBUT389046DB03915CC(=O)[C@H](N)C(O)=OC4H7NO3InChI=1S/C4H7NO3/c1-2(6)3(5)4(7)8/h3H,5H2,1H3,(H,7,8)/t3-/m0/s1SAUCHDKDCUROAO-VKHMYHEASA-N(2S)-2-amino-3-oxobutanoic acid117.1033117.0425930950.2522-amino-3-ketobutyric acid00FDB023915(2s)-2-amino-3-oxobutanoate;(2s)-2-amino-3-oxobutanoic acid;(s)-2-amino-3-oxobutanoate;(s)-2-amino-3-oxobutanoic acid;2-amino-3-oxobutanoate;2-amino-3-oxobutanoic acid;L-2-amino acetic acid;L-2-amino-3-oxobutanoate;L-2-amino-3-oxobutanoic acid;L-2-amino-acetoacetate;2-amino-3-ketobutyric acid;2-amino-3-ketobutyrate;L-2-amino-acetoacetic acidPW_C002747L2AAA255017780761341221233841246751211262794801278423911316Carbon 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_C001316CO25081211204448013503186403677316952080651133431638491745225511731447052831035320111575010857711015968100602615560781616471178663710769221907017160703516370611887163205730819873332137461222753021082152258223151915824911849277119081701246422612688290426263154352331876994293771221337717013277470333777391127775012977763341780771347840535678427334789413317922713080008368806751198071713594836384113291391115549121119954406120089122120155407120364412120556414120833419120922124120991408121284125121505383122744120123011446123190450123418455123489118123556374123855136124063398125344479125460297125516481125824490125870299125931482126280480126887501127052206127277507127331388127390502940Acetyl-CoAHMDB0001206The main function of coenzyme A is to carry acyl groups (such as the acetyl group) or thioesters. Acetyl-CoA is an important molecule itself. It is the precursor to HMG CoA, which is a vital component in cholesterol and ketone synthesis. (wikipedia). acetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.72-89-9C0002444449315351ACETYL-COA392413CC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NC23H38N7O17P3SInChI=1S/C23H38N7O17P3S/c1-12(31)51-7-6-25-14(32)4-5-26-21(35)18(34)23(2,3)9-44-50(41,42)47-49(39,40)43-8-13-17(46-48(36,37)38)16(33)22(45-13)30-11-29-15-19(24)27-10-28-20(15)30/h10-11,13,16-18,22,33-34H,4-9H2,1-3H3,(H,25,32)(H,26,35)(H,39,40)(H,41,42)(H2,24,27,28)(H2,36,37,38)/t13-,16-,17-,18+,22-/m1/s1ZSLZBFCDCINBPY-ZSJPKINUSA-N{[(2R,3S,4R,5R)-2-({[({[(3R)-3-[(2-{[2-(acetylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]-3-hydroxy-2,2-dimethylpropoxy](hydroxy)phosphoryl}oxy)(hydroxy)phosphoryl]oxy}methyl)-5-(6-amino-9H-purin-9-yl)-4-hydroxyoxolan-3-yl]oxy}phosphonic acid809.571809.125773051-2.279acetyl-CoA0-4FDB022491Ac-coa;Ac-coenzyme a;Ac-s-coa;Ac-s-coenzyme a;Acetyl coenzyme-a;Acetyl-coa;Acetyl-coenzyme a;Acetyl-s-coa;Acetyl-s-coenzyme a;Acetylcoenzyme-a;S-acetate coa;S-acetate coenzyme a;S-acetyl coenzyme a;Accoa;Acetyl coenzyme a;S-acetyl-coa;S-acetyl-coenzyme a;Acetylcoenzyme aPW_C000940Ac-CoA213438588423241622446528961733401148401452781035476124573310860251556077161638616470178692316071061637291198746022282451518277210125822261301229942615315771211337729111177562112777061327799411578355134784333348000736880634119806633769012417011995340612014540512030412212063240712241740812262638412274312012295913512313711812498637412520012112534347912550747812563329712656448212657248112677848012688650112704420912739420512766538812813750212814520612837439178GlycineHMDB0000123Glycine 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_C000078Gly3141798181221881272829295420103545412055801335640107564110858631056007147701416074393744116674421511794198118721611242915115233222424193184242031577644336777421117802213278304351807081351200284061200971221201171241216874291222834351228501181242364641248374701254064791254662971254842991264484991269465011270032051270213881280185171099Coenzyme AHMDB0001423Coenzyme A (CoA, CoASH, or HSCoA) is a coenzyme notable for its role in the synthesis and oxidization of fatty acids and the oxidation of pyruvate in the citric acid cycle. It is adapted from beta-mercaptoethylamine, panthothenate, and adenosine triphosphate. It is also a parent compound for other transformation products, including but not limited to, phenylglyoxylyl-CoA, tetracosanoyl-CoA, and 6-hydroxyhex-3-enoyl-CoA. Coenzyme A is synthesized in a five-step process from pantothenate and cysteine. In the first step pantothenate (vitamin B5) is phosphorylated to 4'-phosphopantothenate by the enzyme pantothenate kinase (PanK, CoaA, CoaX). In the second step, a cysteine is added to 4'-phosphopantothenate by the enzyme phosphopantothenoylcysteine synthetase (PPC-DC, CoaB) to form 4'-phospho-N-pantothenoylcysteine (PPC). In the third step, PPC is decarboxylated to 4'-phosphopantetheine by phosphopantothenoylcysteine decarboxylase (CoaC). In the fourth step, 4'-phosphopantetheine is adenylylated to form dephospho-CoA by the enzyme phosphopantetheine adenylyl transferase (CoaD). Finally, dephospho-CoA is phosphorylated using ATP to coenzyme A by the enzyme dephosphocoenzyme A kinase (CoaE). Since coenzyme A is, in chemical terms, a thiol, it can react with carboxylic acids to form thioesters, thus functioning as an acyl group carrier. CoA assists in transferring fatty acids from the cytoplasm to the mitochondria. A molecule of coenzyme A carrying an acetyl group is also referred to as acetyl-CoA. When it is not attached to an acyl group, it is usually referred to as 'CoASH' or 'HSCoA'. Coenzyme A is also the source of the phosphopantetheine group that is added as a prosthetic group to proteins such as acyl carrier proteins and formyltetrahydrofolate dehydrogenase. Acetyl-CoA is an important molecule itself. It is the precursor to HMG CoA which is a vital component in cholesterol and ketone synthesis. Furthermore, it contributes an acetyl group to choline to produce acetylcholine in a reaction catalysed by choline acetyltransferase. Its main task is conveying the carbon atoms within the acetyl group to the citric acid cycle to be oxidized for energy production (Wikipedia).85-61-0C0001068161146900CO-A6557CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)[C@@H](O)C(=O)NCCC(=O)NCCSC21H36N7O16P3SInChI=1S/C21H36N7O16P3S/c1-21(2,16(31)19(32)24-4-3-12(29)23-5-6-48)8-41-47(38,39)44-46(36,37)40-7-11-15(43-45(33,34)35)14(30)20(42-11)28-10-27-13-17(22)25-9-26-18(13)28/h9-11,14-16,20,30-31,48H,3-8H2,1-2H3,(H,23,29)(H,24,32)(H,36,37)(H,38,39)(H2,22,25,26)(H2,33,34,35)/t11-,14-,15-,16+,20-/m1/s1RGJOEKWQDUBAIZ-IBOSZNHHSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[(3R)-3-hydroxy-2,2-dimethyl-3-({2-[(2-sulfanylethyl)carbamoyl]ethyl}carbamoyl)propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid767.534767.115208365-2.2210coenzyme A0-4FDB022614Acetoacetyl coenzyme a sodium salt;Coa;Coa hydrate;Coa-sh;Coash;Coenzyme a;Coenzyme a hydrate;Coenzyme a-sh;Coenzyme ash;Coenzymes a;Depot-zeel;Propionyl coa;Propionyl coenzyme a;S-propanoate;S-propanoate coa;S-propanoate coenzyme a;S-propanoic acid;S-propionate coa;S-propionate coenzyme a;Zeel;[(2r,3s,4r,5r)-5-(6-amino-9h-purin-9-yl)-4-hydroxy-3-(phosphonooxy)tetrahydrofuran-2-yl]methyl 3-hydroxy-4-({3-oxo-3-[(2-sulfanylethyl)amino]propyl}amino)-2,2-dimethyl-4-oxobutyl dihydrogen diphosphatePW_C001099CoA21143868845387922892172407592414224595281329286231334211335118461810462958484214486554487965232102524710452801035477124573410857771016023155607516163841646817869301606961162697319970831887108163729319873472107458222822915190812269090224912417092151951301329915318249254884942616315769072937711913377222134772303297729211177550132775553347756311277633336776721297799611578047332780563507841333578567130792593337997433180005368806201188062737480635119806653769382838293834383986742881105553891105613901158423991158473981199514061201474051202313841203051221206344071207621171214061231214214331215211251216664291216824081217144141224044221227411201229041211229601351239654471239794681240791361242204641242654501249743751253414791255094781255794801255924841256342971260844811265494911265604821267463001268845011270462091271093911273012051275402061276673881281215081281335021283403951148Pyridoxal 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'P1823244535181221401196962011104214505014582621201021504953251115416117542110354411185455120556713255811336533857018160716720572162127222213118581611217515112623311262818126842891268929077017253770372257704129377052224775261127776434177973346779793277829234578855332788623318069613598630711991212212002412412002940612008740712081741812114942312115542412206912312207638312283411912340245412372145812372745912462044712462739812530229712540229912540747912545848112580348912622429812623149512694238812694750112699620612725850612778651312779339062DimethylglycineHMDB0000092Dimethylglycine (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_C000062DMglyc5678190022554355961357760811178079112783161321204771221221254071222951241246771191248481181262824811264622991278452061280323881102FormaldehydeHMDB0001426Formaldehyde is a highly reactive aldehyde gas formed by oxidation or incomplete combustion of hydrocarbons. In solution, it has a wide range of uses: in the manufacture of resins and textiles, as a disinfectant, and as a laboratory fixative or preservative. Formaldehyde solution (formalin) is considered a hazardous compound, and its vapor toxic. (From Reynolds, Martindale The Extra Pharmacopoeia, 30th ed, p717) -- Pubchem; The chemical compound formaldehyde (also known as methanal), is a gas with a pungent smell. It is the simplest aldehyde. Its chemical formula is H2CO. Formaldehyde was first synthesized by the Russian chemist Aleksandr Butlerov in 1859 but was conclusively identified by August Wilhelm van Hofmann in 1867. Although formaldehyde is a gas at room temperature, it is readily soluble in water, and it is most commonly sold as a 37% solution in water called by trade names such as formalin or formol. In water, formaldehyde polymerizes, and formalin actually contains very little formaldehyde in the form of H2CO monomer. Usually, these solutions contain a few percent methanol to limit the extent of polymerization. Formaldehyde exhibits most of the general chemical properties of the aldehydes, except that is generally more reactive than other aldehydes. Formaldehyde is a potent electrophile. It can participate in electrophilic aromatic substitution reactions with aromatic compounds and can undergo electrophilic addition reactions with alkenes. In the presence of basic catalysts, formaldehyde undergoes a Cannizaro reaction to produce formic acid and methanol. Because formaldehyde resins are used in many construction materials, including plywood, carpet, and spray-on insulating foams, and because these resins slowly give off formaldehyde over time, formaldehyde is one of the more common indoor air pollutants. At concentrations above 0.1 mg/kg in air, inhaled formaldehyde can irritate the eyes and mucous membranes, resulting in watery eyes, headache, a burning sensation in the throat, and difficulty breathing. -- Wikipedia.50-00-0C0006771216842FORMALDEHYDE692DB03843C=OCH2OInChI=1S/CH2O/c1-2/h1H2WSFSSNUMVMOOMR-UHFFFAOYSA-Nformaldehyde30.02630.0105646860.820formaldehyde00DBMET00531FDB009445Methaldehyde;Methylene glycol;Aldeide formica;Chlodithan;Chlodithane;Fannoform;Formaldehyde;Formalina;Formaline;Formalith;Formic aldehyde;Formol;Methanal;Methylene oxide;Oxomethylene;Paraform;Formaldehyd;Formalin;OxomethanePW_C001102Formol65310255532562445891854711235484125130082981302030077703332777101307808011278083133122126407122129406123132447123143136124678119124681120126283481126286479127846206127849501185SarcosineHMDB0000271Sarcosine 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_C000185Sar1883272556378081112783063511221274071222854351246791191248394701262844811264504991278472061280205171120(R)-lipoic acidHMDB0001451Lipoic acid is a vitamin-like antioxidant that acts as a free-radical scavenger. Alpha-lipoic acid is also known as thioctic acid. It is a naturally occurring compound that is synthesized by both plants and animals. Lipoic acid contains two thiol groups which may be either oxidized or reduced. The reduced form is known as dihydrolipoic acid (DHLA). Lipoic acid (Delta E= -0.288) is therefore capable of thiol-disulfide exchange, giving it antioxidant activity. Lipoate is a critical cofactor for aerobic metabolism, participating in the transfer of acyl or methylamine groups via the 2-Oxoacid dehydrogenase (2-OADH) or alpha-ketoglutarate dehydrogenase complex. This enzyme catalyzes the conversion of alpha-ketoglutarate to succinyl CoA. This activity results in the catabolism of the branched chain amino acids (leucine, isoleucine and valine). Lipoic acid also participates in the glycine cleavage system(GCV). The glycine cleavage system is a multi-enzyme complex that catalyzes the oxidation of glycine to form 5,10 methylene tetrahydrofolate, an important cofactor in nucleic acid synthesis. Since Lipoic acid is an essential cofactor for many enzyme complexes, it is essential for aerobic life as we know it. This system is used by many organisms and plays a crucial role in the photosynthetic carbon cycle. Lipoic acid was first postulated to be an effective antioxidant when it was found it prevented vitamin C and vitamin E deficiency. It is able to scavenge reactive oxygen species and reduce other metabolites, such as glutathione or vitamins, maintaining a healthy cellular redox state. Lipoic acid has been shown in cell culture experiments to increase cellular uptake of glucose by recruiting the glucose transporter GLUT4 to the cell membrane, suggesting its use in diabetes. Studies of rat aging have suggested that the use of L-carnitine and lipoic acid results in improved memory performance and delayed structural mitochondrial decay. As a result, it may be helpful for people with Alzheimer's disease or Parkinson's disease. -- Wikipedia.62-46-4C16241611230314LIPOIC-ACID5886OC(=O)CCCC[C@@H]1CCSS1C8H14O2S2InChI=1S/C8H14O2S2/c9-8(10)4-2-1-3-7-5-6-11-12-7/h7H,1-6H2,(H,9,10)/t7-/m1/s1AGBQKNBQESQNJD-SSDOTTSWSA-N5-[(3R)-1,2-dithiolan-3-yl]pentanoic acid206.326206.043521072-2.961lipoic acid0-1FDB022631(+)-alpha-lipoic acid;(r)-(+)-lipoic acid;(r)-1,2-dithiolane-3-pentanoic acid;(r)-1,2-dithiolane-3-valeric acid;(r)-6,8-thioctic acid;Alpha-lipoic acid;Lipoic acid;R-la;Rla;Thioctic acid;Thioctic acid d-form;(r)-(+)-lipoate;R-(+)-lipoic acidPW_C001120Lipoate2565346724646010764611084235631542359318773411337808511212035740612213140712300612012468311912567847912628848112717350112785120685268-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acidHMDB00136398-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid belongs to the class of organic compounds known as medium-chain fatty acids. These are fatty acids with an aliphatic tail that contains between 4 and 12 carbon atoms. 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid is considered to be a practically insoluble (in water) and relatively neutral molecule. 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid has been detected in multiple biofluids, such as feces and urine. Within the cell, 8-[(aminomethyl)sulfanyl]-6-sulfanyloctanoic acid is primarily located in the membrane (predicted from logP), mitochondria, cytoplasm and adiposome. In humans, 8-[(aminomethyl)sulfanyl]-6-sulfanyloctanoic acid is involved in the glycine and serine metabolism pathway. 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid is also involved in several metabolic disorders, some of which include dihydropyrimidine dehydrogenase deficiency (DHPD), dimethylglycine dehydrogenase deficiency, the hyperglycinemia, non-ketotic pathway, and the ammonia recycling pathway. 8-[(aminomethyl)sulfanyl]-6-sulfanyloctanoic acid is a intermediate of the glycine cleavage system. It can be found covalently attached to the H-protein of the glycine cleavage system.53481912NCSCCC(S)CCCCC(O)=OC9H19NO2S2InChI=1S/C9H19NO2S2/c10-7-14-6-5-8(13)3-1-2-4-9(11)12/h8,13H,1-7,10H2,(H,11,12)YNZQCBXDUUGFIX-UHFFFAOYSA-N8-[(aminomethyl)sulfanyl]-6-sulfanyloctanoic acid237.383237.085720237-3.3138-[(aminomethyl)sulfanyl]-6-sulfanyloctanoic acid00PW_C0085268As6SA44742566377337133780861121203534061221324071230021201246841191256744791262894811271695011278522061221Tetrahydrofolic 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)-2-amino-4-oxo-1,4,5,6,7,8-hexahydropteridin-6-yl]methyl}amino)phenyl]formamido}pentanedioic acid445.4292445.170981503-3.2282-{[4-({[(6S)-2-amino-4-oxo-5,6,7,8-tetrahydro-1H-pteridin-6-yl]methyl}amino)phenyl]formamido}pentanedioic acid0-2FDB022705(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_C001221THFA44845718975318092530711153471125601135578610860091477066188715120571852067583163117971984264031577336133781181321203524061204821221206964071221661241230011201233011191247181181256734791257492971257714811263242991271685011278863887757DihydrolipoateHMDB0012210Dihydrolipoic acid is an organic compound that is the reduced form of lipoic acid. This carboxylic acid features a pair of thiol groups. It is optically active but only the R-enantiomer is biochemically significant. The lipoic acid/dihydrolipoic acid pair participate in a variety of biochemical transformations.( from Wiki). Inside the cell, alpha lipoic acid is readily reduced or broken down to dihydrolipoic acid. Dihydrolipoic acid is even more potent than alpha lipoic acid, neutralizing free radicals, preventing them from causing harm. It directly destroys damaging superoxide radicals, hydroperoxy radicals and hydroxyl radicals. It has been shown in vitro that dihydrolipoate (DL-6,8-dithioloctanoic acid) has antioxidant activity against microsomal lipid peroxidation.Dihydrolipoate is tested for its neuroprotective activity using models of hypoxic and excitotoxic neuronal damage in vitro and rodent models of cerebral ischemia in vivo. Dihydrolipoate, similarly to dimethylthiourea, is able to protect neurons against ischemic damage by diminishing the accumulation of reactive oxygen species within the cerebral tissue.(PMID: 1345759).462-20-4C02147421180476-S-ACETYL-DIHYDROLIPOATE408OC(=O)CCCCC(S)CCSC8H16O2S2InChI=1S/C8H16O2S2/c9-8(10)4-2-1-3-7(12)5-6-11/h7,11-12H,1-6H2,(H,9,10)IZFHEQBZOYJLPK-UHFFFAOYSA-N6,8-disulfanyloctanoic acid208.341208.059171136-3.303dihydrolipoic acid0-1C021476,8-bis-sulfanyloctanoate;6,8-bis-sulfanyloctanoic acid;6,8-dihydrothioctic acid;6,8-dimercapto-octanoate;6,8-dimercapto-octanoic acid;6,8-dimercaptooctanoate;6,8-dimercaptooctanoic acid;6,8-disulfanyloctanoate;6,8-disulfanyloctanoic acid;D,l-dihydrolipoate;D,l-dihydrolipoic acid;Dhla;Dihydro-dl-alpha-lipoate;Dihydro-dl-alpha-lipoic acid;Dihydro-lipoate;Dihydro-lipoic acid;Dihydro-thioctic acid;Dihydro-thiocytic acid;Dihydro-a-lipoate;Dihydro-a-lipoic acid;Dihydro-alpha-lipoate;Dihydro-alpha-lipoic acid;Dihydrolipoate;Dihydrolipoic acid;Dihydrothioctic acid;Dl-dihydro-a-6-thioctic acid;Dl-dihydro-alpha-6-thioctic acid;Reduced dl-6,8-thioctic acid;Reduced lipoate;Reduced lipoic acid;Reduced thioctic acid;6,8-bis-sulphanyloctanoate;6,8-bis-sulphanyloctanoic acid;6,8-dihydrothioctate;Dihydro-α-lipoate;Dihydro-α-lipoic acid;DihydrothioctatePW_C007757Dhlip2571346714773381337808711212035440612213440712300312012468611912567547912629148112717050112785420611785,10-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)-3-amino-1-oxo-1H,2H,5H,6H,6aH,7H,8H,9H-imidazolidino[1,5-f]pteridin-8-yl]phenyl}formamido)pentanedioic acid457.4399457.170981503-2.7562-({4-[(6aR)-3-amino-1-oxo-2H,5H,6H,6aH,7H,9H-imidazolidino[1,5-f]pteridin-8-yl]phenyl}formamido)pentanedioic acid0-2FDB022675(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-THF4494956898531811253311115359112578510860101476272357065188717120571962067582163426393157733913378119132120355406120683122120704407122167124123004120123293135123309119124719118125676479125761297125779481126325299127171501127887388120L-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_C000120Ser34481810226174564210756431085884105601114769071637086201708720270907170917272021607438374431574441667522224835722591542491217315112625181537949423353184233631577320111780881337811213279979331948583831157523981199241221220561241221364061227181351246671181246881201253142971262092991262934791268602051277713881278565011043Hydroxypyruvic acidHMDB0001352Hydroxypyruvic acid, also known as 3-hydroxypyruvate or OH-pyr, belongs to the class of organic compounds known as beta hydroxy acids and derivatives. Beta hydroxy acids and derivatives are compounds containing a carboxylic acid substituted with a hydroxyl group on the C3 carbon atom. Hydroxypyruvic acid is soluble (in water) and a moderately acidic compound (based on its pKa). Hydroxypyruvic acid has been detected in multiple biofluids, such as urine and blood. Within the cell, hydroxypyruvic acid is primarily located in the mitochondria, peroxisome and cytoplasm. Hydroxypyruvic acid exists in all living organisms, ranging from bacteria to humans. In humans, hydroxypyruvic acid is involved in the glycine and serine metabolism pathway. Hydroxypyruvic acid is also involved in several metabolic disorders, some of which include dimethylglycine dehydrogenase deficiency, the NON ketotic hyperglycinemia pathway, the sarcosinemia pathway, and 3-phosphoglycerate dehydrogenase deficiency. Outside of the human body, hydroxypyruvic acid can be found in a number of food items such as cloudberry, babassu palm, blackcurrant, and japanese pumpkin. This makes hydroxypyruvic acid a potential biomarker for the consumption of these food products. Hydroxypyruvic acid is an intermediate in the metabolism of Glycine, serine and threonine. It is a substrate for Serine--pyruvate aminotransferase and Glyoxylate reductase/hydroxypyruvate reductase.1113-60-6C0016896430841OH-PYR939DB02951OCC(=O)C(O)=OC3H4O4InChI=1S/C3H4O4/c4-1-2(5)3(6)7/h4H,1H2,(H,6,7)HHDDCCUIIUWNGJ-UHFFFAOYSA-N3-hydroxy-2-oxopropanoic acid104.0615104.0109586160.302hydroxypyruvic acid0-1FDB0081123-hydroxy-2-oxopropanoate;3-hydroxy-2-oxopropanoic acid;3-hydroxypyruvate;3-hydroxypyruvic acid;Hydroxypyruvate;Oh-pyr;Oh-pyruvate;Beta-hydroxypyruvatePW_C001043OH-Pyr343836013147126261878091112122138407124690119126295481127859206105L-AlanineHMDB0000161Alanine is a non-essential amino acid made in the body from either the conversion of the carbohydrate pyruvate or the breakdown of DNA and the dipeptides carnosine and anserine. It is highly concentrated in muscle and is one of the most important amino acids released by muscle, functioning as a major energy source. Plasma alanine is often decreased when the BCAA (branched-chain amino acids) are deficient. This finding may relate to muscle metabolism. Alanine is highly concentrated in meat products and other high-protein foods like wheat germ and cottage cheese. Alanine is an important participant as well as a regulator of glucose metabolism. Alanine levels parallel blood sugar levels in both diabetes and hypoglycemia, and alanine reduces both severe hypoglycemia and the ketosis of diabetes. It is an important amino acid for lymphocyte reproduction and immunity. Alanine therapy has helped dissolve kidney stones in experimental animals. Normal alanine metabolism, like that of other amino acids, is highly dependent upon enzymes that contain vitamin B6. Alanine, like GABA, taurine, and glycine, is an inhibitory neurotransmitter in the brain (http://www.dcnutrition.com/AminoAcids/). L-Alanine has been found to be associated with glucagon deficiency, which is an inborn error of metabolism.56-41-7C00041595016977L-ALPHA-ALANINE5735DB00160C[C@H](N)C(O)=OC3H7NO2InChI=1S/C3H7NO2/c1-2(4)3(5)6/h2H,4H2,1H3,(H,5,6)/t2-/m0/s1QNAYBMKLOCPYGJ-REOHCLBHSA-N(2S)-2-aminopropanoic acid89.093289.0476784730.702L-alanine00FDB000556(2s)-2-aminopropanoate;(2s)-2-aminopropanoic acid;(s)-(+)-alanine;(s)-2-aminopropanoate;(s)-2-aminopropanoic acid;(s)-2-amino-propanoate;(s)-2-amino-propanoic acid;(s)-alanine;2-aminopropanoate;2-aminopropanoic acid;2-aminopropionate;2-aminopropionic acid;2-ammoniopropanoate;2-ammoniopropanoic acid;Ala;Alanine;L-(+)-alanine;L-2-aminopropanoate;L-2-aminopropanoic acid;L-2-aminopropionate;L-2-aminopropionic acid;L-a-alanine;L-a-aminopropionate;L-a-aminopropionic acid;L-alpha-alanine;L-alpha-aminopropionate;L-alpha-aminopropionic acid;A-alanine;A-aminopropionate;A-aminopropionic acid;Alpha-alanine;Alpha-aminopropanoate;Alpha-aminopropanoic acid;Alpha-aminopropionate;Alpha-aminopropionic acid;A;L-alanin;L-α-alaninePW_C000105Ala10229431681446501453511454263022153439354071175418103543111854521205557132557813356371075638108588310565298583502251227115112620311262718152302224245232042453318425343157796934677975327779883267800811178092112791651148069313511991012212001512412002640612114542312115142412116441612122040912213940712371745812372345912373645212379013712469111912530029712539329912540447912629648112685020512693338812694450112786020675Glyoxylic acidHMDB0000119Glyoxylic acid or oxoacetic acid is an organic compound that is both an aldehyde and a carboxylic acid. Glyoxylic acid is a liquid with a melting point of -93°C and a boiling point of 111°C. It is an intermediate of the glyoxylate cycle, which enables certain organisms to convert fatty acids into carbohydrates. The conjugate base of glyoxylic acid is known as glyoxylate (PMID: 16396466). In humans, glyoxylate is produced via two pathways: (1) through the oxidation of glycolate in peroxisomes and (2) through the catabolism of hydroxyproline in mitochondria. In the peroxisomes, glyoxylate is converted into glycine by glyoxylate aminotransferase (AGT1) or into oxalate by glycolate oxidase. In the mitochondria, glyoxylate is converted into glycine by mitochondrial glyoxylate aminotransferase AGT2 or into glycolate by glycolate reductase. A small amount of glyoxylate is converted into oxalate by cytoplasmic lactate dehydrogenase. Glyoxylic acid is found to be associated with primary hyperoxaluria I, which is an inborn error of metabolism. Under certain circumstances, glyoxylate can be a nephrotoxin and a metabotoxin. A nephrotoxin is a compound that causes damage to the kidney and kidney tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. High levels of glyoxylate are involved in the development of hyperoxaluria, a key cause of nephrolithiasis (commonly known as kidney stones). Glyoxylate is both a substrate and inductor of sulfate anion transporter-1 (SAT-1), a gene responsible for oxalate transportation, allowing it to increase SAT-1 mRNA expression, and as a result oxalate efflux from the cell. The increased oxalate release allows the buildup of calcium oxalate in the urine, and thus the eventual formation of kidney stones. As an aldehyde, glyoxylate is also highly reactive and will modify proteins to form advanced glycation products (AGEs).298-12-4C0004876016891GLYOX740DB04343OC(=O)C=OC2H2O3InChI=1S/C2H2O3/c3-1-2(4)5/h1H,(H,4,5)HHLFWLYXYJOTON-UHFFFAOYSA-N2-oxoacetic acid74.035574.000393930.481glyoxylic acid0-1FDB001478Formylformate;Formylformic acid;Glyoxalate;Glyoxalic acid;Glyoxylate;Glyoxylic acid;Oxalaldehydate;Oxalaldehydic acid;Oxoacetate;Oxoacetic acid;Oxoethanoate;Oxoethanoic acid;A-ketoacetate;A-ketoacetic acid;Alpha-ketoacetate;Alpha-ketoacetic acid;Glyoxalsaeure;Glyoxylsaeure;α-ketoacetate;α-ketoacetic acid;OxaldehydatePW_C000075Glyoxal304344135419103545312055791335729108600414764561071247524915232222426143154263231878094112120027406122141407124693119125405479126298481126945501127862206396L-ArginineHMDB0000517Arginine is an essential amino acid that is physiologically active in the L-form. In mammals, arginine is formally classified as a semi-essential or conditionally essential amino acid, depending on the developmental stage and health status of the individual. Infants are unable to effectively synthesize arginine, making it nutritionally essential for infants. Adults, however, are able to synthesize arginine in the urea cycle. Arginine can be considered to be a basic amino acid as the part of the side chain nearest to the backbone is long, carbon-containing, and hydrophobic, whereas the end of the side chain is a complex guanidinium group. With a pKa of 12.48, the guanidinium group is positively charged in neutral, acidic, and even most basic environments. Because of the conjugation between the double bond and the nitrogen lone pairs, the positive charge is delocalized. This group is able to form multiple H-bonds. L-Arginine is an amino acid that has numerous functions in the body. It helps dispose of ammonia, is used to make compounds such as nitric oxide, creatine, L-glutamate, and L-proline, and it can be converted into glucose and glycogen if needed. In large doses, L-arginine also stimulates the release of the hormones growth hormone and prolactin. Arginine is a known inducer of mTOR (mammalian target of rapamycin) and is responsible for inducing protein synthesis through the mTOR pathway. mTOR inhibition by rapamycin partially reduces arginine-induced protein synthesis (PMID: 20841502). Catabolic disease states such as sepsis, injury, and cancer cause an increase in arginine utilization, which can exceed normal body production, leading to arginine depletion. Arginine also activates AMP kinase (AMPK) which then stimulates skeletal muscle fatty acid oxidation and muscle glucose uptake, thereby increasing insulin secretion by pancreatic beta-cells (PMID: 21311355). Arginine is found in plant and animal proteins, such as dairy products, meat, poultry, fish, and nuts. The ratio of L-arginine to lysine is also important: soy and other plant proteins have more L-arginine than animal sources of protein.74-79-3C000622878216467ARG6082DB00125N[C@@H](CCCNC(N)=N)C(O)=OC6H14N4O2InChI=1S/C6H14N4O2/c7-4(5(11)12)2-1-3-10-6(8)9/h4H,1-3,7H2,(H,11,12)(H4,8,9,10)/t4-/m0/s1ODKSFYDXXFIFQN-BYPYZUCNSA-N(2S)-2-amino-5-carbamimidamidopentanoic acid174.201174.111675712-1.885L-arginine01DBMET00502FDB002257(s)-2-amino-5-[(aminoiminomethyl)amino]pentanoate;(s)-2-amino-5-[(aminoiminomethyl)amino]pentanoic acid;(s)-2-amino-5-[(aminoiminomethyl)amino]-pentanoate;(s)-2-amino-5-[(aminoiminomethyl)amino]-pentanoic acid;2-amino-5-guanidinovalerate;2-amino-5-guanidinovaleric acid;5-[(aminoiminomethyl)amino]-l-norvaline;Arginine;L-(+)-arginine;L-a-amino-d-guanidinovalerate;L-a-amino-d-guanidinovaleric acid;L-alpha-amino-delta-guanidinovalerate;L-alpha-amino-delta-guanidinovaleric acid;N5-(aminoiminomethyl)-l-ornithine;(2s)-2-amino-5-(carbamimidamido)pentanoic acid;(2s)-2-amino-5-guanidinopentanoic acid;(s)-2-amino-5-guanidinopentanoic acid;(s)-2-amino-5-guanidinovaleric acid;Arg;L-arg;L-arginin;R;(2s)-2-amino-5-(carbamimidamido)pentanoate;(2s)-2-amino-5-guanidinopentanoate;(s)-2-amino-5-guanidinopentanoate;(s)-2-amino-5-guanidinovaleratePW_C000396Arg10583448356201075623117118461981273229042531322425543187746711178095112792392937924016412005612212214240712280813512469411912543429712629948112697320512786320683Guanidoacetic acidHMDB0000128Guanidoacetic acid is a metabolite in the Urea cycle and metabolism of amino groups, and in the metabolic pathways of several amino acids. This includes glycine, serine, threonine, arginine and proline metabolism. Guanidinoacetic acid is also a precursor of creatine, an essential substrate for muscle energy metabolism.352-97-6C00581394684816344GUANIDOACETIC_ACID743DB02751NC(=N)NCC(O)=OC3H7N3O2InChI=1S/C3H7N3O2/c4-3(5)6-1-2(7)8/h1H2,(H,7,8)(H4,4,5,6)BPMFZUMJYQTVII-UHFFFAOYSA-N2-carbamimidamidoacetic acid117.1066117.053826483-1.454glycocyamine00FDB021898(carboxymethyl)-guanidine;2-[[amino(imino)methyl]amino]acetate;2-[[amino(imino)methyl]amino]acetic acid;Betacyamine;Betasyamine;Glycocyamine;Guanidineacetate;Guanidineacetic acid;Guanidinoacetate;Guanidinoacetic acid;Guanidoacetate;Guanidoacetic acid;Guanidylacetate;Guanidylacetic acid;Guanyl glycine;N-amidino-glycine;N-amidinoglycine;[(aminoiminomethyl)amino]-acetate;[(aminoiminomethyl)amino]-acetic acid;A-guanidinoacetate;A-guanidinoacetic acid;Alpha-guanidinoacetate;Alpha-guanidinoacetic acid;B-guanidinoacetate;B-guanidinoacetic acid;Beta-guanidinoacetate;Beta-guanidinoacetic acidPW_C000083GIAA517434493118731617748713378096112120391406122143407123035120124695119126300481127864206144Orotidylic acidHMDB0000218Orotidylic acid (OMP), is a pyrimidine nucleotide which is the last intermediate in the biosynthesis of uridine monophosphate. Decarboxylation by Orotidylate decarboxylase affords Uridine 5'-phosphate which is the route to Uridine and its derivatives de novo and consequently one of the most important processes in nucleic acid synthesis (Dictionary of Organic Compounds). In humans, the enzyme UMP synthase converts OMP into uridine 5'- monophosphate. If UMP synthase is defective, orotic aciduria can result. (Wikipedia).2149-82-8C0110316061715842OROTIDINE-5-PHOSPHATE141140DB02957O[C@H]1[C@@H](O)[C@@H](O[C@@H]1COP(O)(O)=O)N1C(=O)NC(=O)C=C1C(O)=OC10H13N2O11PInChI=1S/C10H13N2O11P/c13-5-1-3(9(16)17)12(10(18)11-5)8-7(15)6(14)4(23-8)2-22-24(19,20)21/h1,4,6-8,14-15H,2H2,(H,16,17)(H,11,13,18)(H2,19,20,21)/t4-,6-,7-,8-/m1/s1KYOBSHFOBAOFBF-XVFCMESISA-N3-[(2R,3R,4S,5R)-3,4-dihydroxy-5-[(phosphonooxy)methyl]oxolan-2-yl]-2,6-dioxo-1,2,3,6-tetrahydropyrimidine-4-carboxylic acid368.1908368.02569578-1.6366-carboxy-5'-uridylic acid0-3FDB0123211,2,3,6-tetrahydro-2,6-dioxo-3-(5-o-phosphono-b-d-ribofuranosyl)-4-pyrimidinecarboxylate;1,2,3,6-tetrahydro-2,6-dioxo-3-(5-o-phosphono-b-d-ribofuranosyl)-4-pyrimidinecarboxylic acid;1,2,3,6-tetrahydro-2,6-dioxo-3-(5-o-phosphono-beta-delta-ribofuranosyl)-4-pyrimidinecarboxylate;1,2,3,6-tetrahydro-2,6-dioxo-3-(5-o-phosphono-beta-delta-ribofuranosyl)-4-pyrimidinecarboxylic acid;2,6-dioxo-3-(5-o-phosphono-beta-d-ribofuranosyl)-1,2,3,6-tetrahydropyrimidine-4-carboxylic acid;2,6-dioxo-3-(5-o-phosphono-beta-delta-ribofuranosyl)-1,2,3,6-tetrahydropyrimidine-4-carboxylic acid;5'-(dihydrogen phosphate) 6-carboxy-uridine;5'-(dihydrogen phosphate) orotidine;5'-omp;5'-phosphate orotidine;5-(dihydrogen phosphate)orotidine;6-carboxy-5'-uridylate;6-carboxy-5'-uridylic acid;Omp;Ometoprim;Omp (nucleotide);Orotidine 5'-(dihydrogen phosphate);Orotidine 5'-monophosphate;Orotidine 5'-phosphate;Orotidine monophosphate;Orotidine-5'-phosphate;Orotidylate;Orotidylic acid;Orotidine 5'-phosphoric acid;Orotidine 5'-(dihydrogen phosphoric acid)PW_C000144OMP27102345037809711278731132122144407122206124124696119124758118126301481126368299127865206127931388749S-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_C000749SAH5208575186353070520122131882272067246831050255056071367137163754221075462137634160826815192371951187519812359225152942491536430977489111776111307773333877773341780981327830535178337346791561127996236180863229483138294834386113287389113289397115544399115547401120394122120486125120539413120940407121053124122284435123037135123173449123506119123617118124838470125880481126303299126449499127341206127596388128019517921S-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_C000921SAMe51986333070420122031880272066246811050235056041357136163754021075442137632160826615192351951187419812031222123582251529324915345181536330976897293768991647698422477488111777313387777234178099132783033517833534679155112799613618086122948303829483338611328638911328839711554339911554640112039312212053741312093940712105212412228243512317144912350511912361611812483647012585929712587948112630429912644749912732120512734020612759538812801751746CreatineHMDB0000064Creatine is an amino acid that occurs in vertebrate tissues and in urine. In muscle tissue, creatine generally occurs as phosphocreatine. Creatine is excreted as creatinine in the urine. Creatine functions as part of the cell's energy shuttle. The high energy phosphate group of ATP is transferred to creatine to form phosphocreatine in the following reaction: Cr + ATP <-> PCr + ADP. This reaction is reversibly catalyzed by creatine kinase. In the human body, creatine is synthesized mainly in the liver by the use of parts from three different amino acids: arginine, glycine, and methionine. 95% of it is later stored in the skeletal muscles and the rest is stored in the brain, heart, and testes.57-00-1C0030058616919CREATINE566DB00148CN(CC(O)=O)C(N)=NC4H9N3O2InChI=1S/C4H9N3O2/c1-7(4(5)6)2-3(8)9/h2H2,1H3,(H3,5,6)(H,8,9)CVSVTCORWBXHQV-UHFFFAOYSA-N2-(N-methylcarbamimidamido)acetic acid131.1332131.069476547-1.503creatine00FDB005403((amino(imino)methyl)(methyl)amino)acetate;((amino(imino)methyl)(methyl)amino)acetic acid;(alpha-methylguanido)acetate;(alpha-methylguanido)acetic acid;Cosmocair c 100;Creatin;Creatine;Creatine hydrate;Kreatin;Krebiozon;Methylguanidoacetate;Methylguanidoacetic acid;N-(aminoiminomethyl)-n-methyl-glycine;N-methyl-n-guanylglycine;Phosphagen;[[amino(imino)methyl](methyl)amino]acetate;[[amino(imino)methyl](methyl)amino]acetic acid;(n-methylcarbamimidamido)acetic acid;Alpha-methylguanidino acetic acid;Methylglycocyamine;N-(aminoiminomethyl)-n-methylglycine;N-[(e)-amino(imino)methyl]-n-methylglycine;N-amidinosarcosine;N-carbamimidoyl-n-methylglycine;(a-methylguanido)acetate;(a-methylguanido)acetic acid;(α-methylguanido)acetate;(α-methylguanido)acetic acid;(n-methylcarbamimidamido)acetate;A-methylguanidino acetate;A-methylguanidino acetic acid;Alpha-methylguanidino acetate;α-methylguanidino acetate;α-methylguanidino acetic acidPW_C000046Creatin521834522118761987749011178100132120395122122146124123038135124698118126305299127867388808Succinyl-CoAHMDB0001022Succinyl-CoA is an important intermediate in the citric acid cycle, where it is synthesized from α-Ketoglutarate by α-ketoglutarate dehydrogenase (EC 1.2.4.2) through decarboxylation, and is converted into succinate through the hydrolytic release of coenzyme A by succinyl-CoA synthetase (EC 6.2.1.5). Succinyl-CoA may be an end product of peroxisomal beta-oxidation of dicarboxylic fatty acids; the identification of an apparently specific succinyl-CoA thioesterase (ACOT4, EC 3.1.2.3, hydrolyzes succinyl-CoA) in peroxisomes strongly suggests that succinyl-CoA is formed in peroxisomes. Acyl-CoA thioesterases (ACOTs) are a family of enzymes that catalyze the hydrolysis of the CoA esters of various lipids to the free acids and coenzyme A, thereby regulating levels of these compounds. (PMID: 16141203).604-98-8C00091439161153803-METHYLBENZYLSUCCINYL-COA388307CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)C(O)C(=O)NCCC(=O)NCCSC(=O)CCC(O)=OC25H40N7O19P3SInChI=1S/C25H40N7O19P3S/c1-25(2,20(38)23(39)28-6-5-14(33)27-7-8-55-16(36)4-3-15(34)35)10-48-54(45,46)51-53(43,44)47-9-13-19(50-52(40,41)42)18(37)24(49-13)32-12-31-17-21(26)29-11-30-22(17)32/h11-13,18-20,24,37-38H,3-10H2,1-2H3,(H,27,33)(H,28,39)(H,34,35)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t13-,18-,19-,20?,24-/m1/s1VNOYUJKHFWYWIR-FZEDXVDRSA-N4-{[2-(3-{3-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido}propanamido)ethyl]sulfanyl}-4-oxobutanoic acid867.607867.131252359-2.35104-({2-[3-(3-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-2-hydroxy-3-methylbutanamido)propanamido]ethyl}sulfanyl)-4-oxobutanoic acid0-5FDB022375Coa s-(hydrogen succinate);Coa s-succinate;Coenzyme a s-(hydrogen succinate);Coenzyme a s-succinate;S-(hydrogen butanedioate;S-(hydrogen butanedioate) coa;S-(hydrogen butanedioate) coenzyme a;S-(hydrogen butanedioic acid;S-succinoylcoenzyme a;Suc-co-a;Suc-coa;Succ-coa;Succ-coenzyme a;Succ-s-coa;Succ-s-coenzyme a;Succ-s-coenzyme-a;Succ-coenzyme-a;Succino-1-yl-coenzyme a;Succinyl coa;Succinyl coenzyme a;Succinyl-s-coa;Succinyl-s-coenzyme a;Succinyl-s-coenzyme-a;Succinylcoenzyme-a;Succinylcoenzyme aPW_C000808Suc-CoA233410553366925378103603915560971616485178701516073611637474222771401337810111278576132800213681199784061207694071220141241227631201233651191245681181253584791261642991263064811269015011278682068945-Aminolevulinic acidHMDB00011495-Aminolevulinic acid is an intermediate in heme synthesis. This is the first compound in the porphyrin synthesis pathway. It is produced by the enzyme ALA synthase, from glycine and succinyl CoA. This reaction is known as the Shemin pathway. Aminolevulinic acid plus blue light illumination using a blue light photodynamic therapy illuminator is indicated for the treatment of minimally to moderately thick actinic keratoses of the face or scalp.106-60-5C00430137175495-AMINO-LEVULINATE134DB00855NCC(=O)CCC(O)=OC5H9NO3InChI=1S/C5H9NO3/c6-3-4(7)1-2-5(8)9/h1-3,6H2,(H,8,9)ZGXJTSGNIOSYLO-UHFFFAOYSA-N5-amino-4-oxopentanoic acid131.1299131.0582431590.122aminolevulinic acid00FDB0224525-amino-4-oxo-pentanoate;5-amino-4-oxo-pentanoic acid;5-amino-4-oxopentanoate;5-amino-4-oxopentanoic acid;5-amino-4-oxovalerate;5-amino-4-oxovaleric acid;5-amino-levulinate;5-amino-levulinic acid;5-aminolaevulinate;5-aminolaevulinic acid;5-aminolevulinate;Aladerm;Amino-levulinic acid;Aminolevulinate;Aminolevulinic acid;Kerastick;Delta-aminolevulinate;Delta-aminolevulinic acid;5-ala;Dala;Delta-ala;5-aminolevulinic acid;δ-ala;δ-aminolevulinate;δ-aminolevulinic acidPW_C0008945-Amnva34593367027016160781021127857713212201512412214840712456911812470011912616529912630748112786920630βineHMDB0000043Betaine (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_C000030βine558155598189933470255921355618137685071228222677605111776181147810413278315112120470122120496409122150124122294407124702118124847119126309299126461481127871388128031206590HomocysteineHMDB0000742Homocysteine 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-CYS757NC(CCS)C(O)=OC4H9NO2SInChI=1S/C4H9NO2S/c5-3(1-2-8)4(6)7/h3,8H,1-2,5H2,(H,6,7)FFFHZYDWPBMWHY-UHFFFAOYSA-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_C001005Zinc1323841188271165291529575130446831202931477054101175425103543411854591205560132558513355981357449166117871981246622612724290133211517696722577401111775801147792933680400112002012412003540612006012212044140912125742912307513712382746412539829912541347912543829712568548312693838812695350112697620512718020890Glyceric acidHMDB0000139Glyceric acid is a colourless syrupy acid, obtained from oxidation of glycerol. It is a compound that is secreted excessively in the urine by patients suffering from D-glyceric aciduria, an inborn error of metabolism, and D-glycerate anemia. Deficiency of human glycerate kinase leads to D-glycerate acidemia/D-glyceric aciduria. Symptoms of the disease include progressive neurological impairment, hypotonia, seizures, failure to thrive, and metabolic acidosis. At sufficiently high levels, glyceric acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Glyceric acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated glyceric aciduria. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.473-81-4C00258439194323982-PG388334OC[C@@H](O)C(O)=OC3H6O4InChI=1S/C3H6O4/c4-1-2(5)3(6)7/h2,4-5H,1H2,(H,6,7)/t2-/m1/s1RBNPOMFGQQGHHO-UWTATZPHSA-N(2R)-2,3-dihydroxypropanoic acid106.0773106.026608680.783glycerate0-1FDB012242(r)-glycerate;D-glycerate;D-glyceric acid;Glycerate;Glyceric acid;A,b-hydroxypropionate;A,b-hydroxypropionic acid;Alpha,beta-hydroxypropionic acid;R-glyceric acid;Alpha,beta-hydroxypropionate;α,β-hydroxypropionate;α,β-hydroxypropionic acid;R-glyceratePW_C000090Glycera205283476257391086014147426203157803411178107132121387122122155124123946135124707118126313299127874388414Adenosine 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_C000414ATP922146082661641422478137333279959343997632105182112102146492156142160582405592434272726462812293029663163723616613617514399234474314768914864545032895035265155752059752151005250104529110153131115346112539010354061175430118544312055421295556132556913356031355621108584614358541465876107589714759241516048155610916162301666493178683918868701606976199715720571842067209210722521372292117298198730221673902177408218743216374812227499190818622511847277119031701201028112039164121782851257822612691290132642231532730842326315426213224269431877028253772181347723332977468333776323367803733278041350781681287821435178240353784113357849411578850130788653317891933480028368800461848067411985629194826124113234941132823881162801091199141221199924061201544071202453821203624121212464291213921231213974331214714081219744101220651251220793831220834051224024221224444351229193991230094461238164641239514471239564681240293741245274441246161361246303981246343761249434721249723751250114701253042971253714791253922991255154811255954841261234851262203001262344951262404781265474911265964991269135011271233891277315161277813951277963901278012091281195081281675176443-Phosphoglyceric acidHMDB00008073-phosphoglyceric acid (3PG) is a 3-carbon molecule that is a metabolic intermediate in both glycolysis and the Calvin cycle. This chemical is often termed PGA when referring to the Calvin cycle. In the Calvin cycle, two glycerate 3-phosphate molecules are reduced to form two molecules of glyceraldehyde 3-phosphate (GALP). (wikipedia).820-11-1C0059772417050G3P704OC(COP(O)(O)=O)C(O)=OC3H7O7PInChI=1S/C3H7O7P/c4-2(3(5)6)1-10-11(7,8)9/h2,4H,1H2,(H,5,6)(H2,7,8,9)OSJPPGNTCRNQQC-UHFFFAOYSA-N2-hydroxy-3-(phosphonooxy)propanoic acid186.0572185.99293909-0.954phosphoglycerate0-3FDB0222553-(dihydrogen phosphate)glycerate;3-(dihydrogen phosphate)glyceric acid;3-glycerophosphorate;3-glycerophosphoric acid;3-p-d-glycerate;3-p-glycerate;3-pga;3-pg;3-phospho-(r)-glycerate;3-phospho-d-glycerate;3-phospho-glycerate;3-phospho-glyceric acid;3-phosphoglycerate;3-phosphoglyceric acid;D-(-)-3-phosphoglyceric acid;D-glycerate 3-phosphate;G3p;Glycerate 3-phosphate;Glycerate-3-p;Glyceric acid 3-phosphate;Phosphoglycerate;Dl-glycerate 3-phosphate;Glycerate 3-phosphates;3-(dihydrogen phosphoric acid)glyceric acid;2-hydroxy-3-phosphonooxypropanoate;Dl-glyceric acid 3-phosphoric acid;Glyceric acid 3-phosphoric acid;Glyceric acid 3-phosphatesPW_C000644G3P2051822572574311759171475948151689716083582254262332277111132780331111211921241213861221237631181239451351259602991274173881034Adenosine diphosphateHMDB0001341Adenosine diphosphate, abbreviated ADP, is a nucleotide. It is an ester of pyrophosphoric acid with the nucleotide adenine. ADP consists of the pyrophosphate group, the pentose sugar ribose, and the nucleobase adenine. ADP is the product of ATP dephosphorylation by ATPases. ADP is converted back to ATP by ATP synthases.58-64-0C00008602216761ADP5800NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1OC10H15N5O10P2InChI=1S/C10H15N5O10P2/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(24-10)1-23-27(21,22)25-26(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1XTWYTFMLZFPYCI-KQYNXXCUSA-N[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]phosphonic acid427.2011427.029414749-2.126adenosine-diphosphate0-2FDB021817Adp;Adenosindiphosphorsaeure;Adenosine 5'-pyrophosphate;Adenosine diphosphate;Adenosine pyrophosphate;Adenosine-5'-diphosphate;Adenosine-5-diphosphate;Adenosine-diphosphate;5'-adenylphosphoric acid;Adenosine 5'-diphosphate;H3adp;5'-adenylphosphate;Adenosine 5'-diphosphoric acid;Adenosine-5'-diphosphoric acidPW_C001034ADP234134841522482138015963159783106114151821901492104182113102161582408592435272728472736462855293165723635614400234476314770915036265157752089752171005315111534911253921035446120554412955721335624108574111757641015849143585614658781075899147592615160501556111161623116664951786700946841188687216071592057187206720821072262137231211730019873032167391217741021874331637483222818722511851277119051701201328112180285132622231532930842328315423983134262232242696318770292537708713277216134773063297747233377663336780393327804335078170128782153517824435378414335784951157870533178849130789203348003036880622118806511358067611994827124113283388116204109119944122119994406120156407120318382120366412121248429121394123121399433121472408121899383121976410122064125122085405122405422122445435122973399123013446123818464123953447123958468124030374124452398124529444124615136124636376124947472124975375125012470125334297125373479125492299125517481125645484126125485126219300126235495126242478126550491126597499126915501127733516127780395127797390127803209128122508128168517128313389810Phosphohydroxypyruvic acidHMDB0001024Phosphohydroxypyruvic acid, also known as 3-phosphonooxypyruvate or hydroxypyruvic acid phosphate, belongs to the class of organic compounds known as glycerone phosphates. These are organic compounds containing a glycerone moiety that carries a phosphate group at the O-1 or O-2 position. Phosphohydroxypyruvic acid is soluble (in water) and a moderately acidic compound (based on its pKa). Within the cell, phosphohydroxypyruvic acid is primarily located in the cytoplasm. Phosphohydroxypyruvic acid exists in all living organisms, ranging from bacteria to humans. Phosphohydroxypyruvic acid participates in a number of enzymatic reactions. In particular, Phosphohydroxypyruvic acid can be biosynthesized from 3-phosphoglyceric acid; which is mediated by the enzyme D-3-phosphoglycerate dehydrogenase / α-ketoglutarate reductase. Furthermore, Phosphohydroxypyruvic acid and L-glutamic acid can be converted into oxoglutaric acid and DL-O-phosphoserine; which is mediated by the enzyme 3-phosphoserine aminotransferase / phosphohydroxythreonine aminotransferase. Furthermore, Phosphohydroxypyruvic acid can be biosynthesized from 3-phosphoglyceric acid through its interaction with the enzyme D-3-phosphoglycerate dehydrogenase / α-ketoglutarate reductase. Furthermore, Phosphohydroxypyruvic acid and L-glutamic acid can be converted into oxoglutaric acid and DL-O-phosphoserine; which is catalyzed by the enzyme 3-phosphoserine aminotransferase / phosphohydroxythreonine aminotransferase. Furthermore, Phosphohydroxypyruvic acid can be biosynthesized from 3-phosphoglyceric acid through its interaction with the enzyme D-3-phosphoglycerate dehydrogenase / α-ketoglutarate reductase. Finally, Phosphohydroxypyruvic acid and L-glutamic acid can be converted into oxoglutaric acid and DL-O-phosphoserine through its interaction with the enzyme 3-phosphoserine aminotransferase / phosphohydroxythreonine aminotransferase. In humans, phosphohydroxypyruvic acid is involved in the glycine and serine metabolism pathway. Phosphohydroxypyruvic acid is also involved in several metabolic disorders, some of which include the sarcosinemia pathway, the hyperglycinemia, non-ketotic pathway, dihydropyrimidine dehydrogenase deficiency (DHPD), and the NON ketotic hyperglycinemia pathway. Phosphohydroxypyruvic acid is a prduct of both enzyme phosphoglycerate dehydrogenase [EC 1.1.1.95] and phosphoserine transaminase [EC 2.6.1.52] in glycine, serine and threonine metabolism pathway (KEGG).3913-50-6C03232105309333-P-HYDROXYPYRUVATE103OC(=O)C(=O)COP(O)(O)=OC3H5O7PInChI=1S/C3H5O7P/c4-2(3(5)6)1-10-11(7,8)9/h1H2,(H,5,6)(H2,7,8,9)LFLUCDOSQPJJBE-UHFFFAOYSA-N2-oxo-3-(phosphonooxy)propanoic acid184.0414183.977289026-1.143phosphohydroxypyruvate0-3FDB0223772-oxo-3-(phosphonooxy)-propanoate;2-oxo-3-(phosphonooxy)-propanoic acid;3-phosphohydroxypyruvate;3-phosphohydroxypyruvic acid;3-phosphonooxypyruvate;3-phosphonooxypyruvic acid;Phosphohydroxypyruvate;Phosphohydroxypyruvic acid;Hydroxypyruvic acid phosphate;2-oxo-3-phosphonooxypropanoate;Hydroxypyruvate phosphate;Hydroxypyruvic acid phosphoric acid;3-phosphonatooxypyruvatePW_C0008103POHPyr347927810813212215712412470911812631529912787638895L-Glutamic acidHMDB0000148Glutamic acid (Glu), also referred to as glutamate (the anion), is one of the 20 proteinogenic amino acids. It is not among the essential amino acids. Glutamate is a key molecule in cellular metabolism. In humans, dietary proteins are broken down by digestion into amino acids, which serves as metabolic fuel or other functional roles in the body. Glutamate is the most abundant fast excitatory neurotransmitter in the mammalian nervous system. At chemical synapses, glutamate is stored in vesicles. Nerve impulses trigger release of glutamate from the pre-synaptic cell. In the opposing post-synaptic cell, glutamate receptors, such as the NMDA receptor, bind glutamate and are activated. Because of its role in synaptic plasticity, it is believed that glutamic acid is involved in cognitive functions like learning and memory in the brain. Glutamate transporters are found in neuronal and glial membranes. They rapidly remove glutamate from the extracellular space. In brain injury or disease, they can work in reverse and excess glutamate can accumulate outside cells. This process causes calcium ions to enter cells via NMDA receptor channels, leading to neuronal damage and eventual cell death, and is called excitotoxicity. The mechanisms of cell death include: * Damage to mitochondria from excessively high intracellular Ca2+. * Glu/Ca2+-mediated promotion of transcription factors for pro-apoptotic genes, or downregulation of transcription factors for anti-apoptotic genes. Excitotoxicity due to glutamate occurs as part of the ischemic cascade and is associated with stroke and diseases like amyotrophic lateral sclerosis, lathyrism, and Alzheimer's disease. glutamic acid has been implicated in epileptic seizures. Microinjection of glutamic acid into neurons produces spontaneous depolarization around one second apart, and this firing pattern is similar to what is known as paroxysmal depolarizing shift in epileptic attacks. This change in the resting membrane potential at seizure foci could cause spontaneous opening of voltage activated calcium channels, leading to glutamic acid release and further depolarization. (http://en.wikipedia.org/wiki/Glutamic_acid).56-86-0C000253303216015GLT30572DB00142N[C@@H](CCC(O)=O)C(O)=OC5H9NO4InChI=1S/C5H9NO4/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H,7,8)(H,9,10)/t3-/m0/s1WHUUTDBJXJRKMK-VKHMYHEASA-N(2S)-2-aminopentanedioic acid147.1293147.053157781-0.263L-glutamic acid0-1FDB012535(2s)-2-aminopentanedioate;(2s)-2-aminopentanedioic acid;(s)-(+)-glutamate;(s)-(+)-glutamic acid;(s)-2-aminopentanedioate;(s)-2-aminopentanedioic acid;(s)-glutamate;(s)-glutamic acid;1-amino-propane-1,3-dicarboxylate;1-amino-propane-1,3-dicarboxylic acid;1-aminopropane-1,3-dicarboxylate;1-aminopropane-1,3-dicarboxylic acid;2-aminoglutarate;2-aminoglutaric acid;2-aminopentanedioate;2-aminopentanedioic acid;Aciglut;Aminoglutarate;Aminoglutaric acid;E;Glt;Glu;Glusate;Glut;Glutacid;Glutamicol;Glutamidex;Glutaminate;Glutaminic acid;Glutaminol;Glutaton;L-(+)-glutamate;L-(+)-glutamic acid;L-glu;L-glutamate;L-glutaminate;L-glutaminic acid;L-a-aminoglutarate;L-a-aminoglutaric acid;L-alpha-aminoglutarate;L-alpha-aminoglutaric acid;A-aminoglutarate;A-aminoglutaric acid;A-glutamate;A-glutamic acid;Alpha-aminoglutarate;Alpha-aminoglutaric acid;Alpha-glutamate;Alpha-glutamic acid;Acide glutamique;Acido glutamico;Acidum glutamicum;Glutamate;Glutamic acid;L-glutaminsaeurePW_C000095Glu16244365811911384164149699110542144850145626146254532311153441135415117543911855651325631107563210858591056006147607115761919465318568381876844188709272709371716520571822077514224751815182082258373220117921981185516112004222126213112683289126972904234831542349318428453207702025377332133775251127797134677977327779813477829134580649135120023124120040122120086407120347406120692126120816418121147423121153424121157425122833119122997120123299443123401454123719458123725459123729460125401299125418297125457481125667479125769301125802489126941388126995206127162501127257506186PhosphoserineHMDB0000272The phosphoric acid ester of serine. As a constituent (residue) of proteins, its side chain can undergo O-linked glycosylation. This might be important in explaining some of the devastating consequences of diabetes. It is one of three amino acid residues that are commonly phosphorylated by kinases during cell signalling in eukaryotes. Phosphorylated serine residues are often referred to as phosphoserine. Serine proteases are a common type of protease. Serine, organic compound, one of the 20 amino acids commonly found in animal proteins. Only the L-stereoisomer appears in mammalian protein. It is not essential to the human diet, since it can be synthesized in the body from other metabolites, including glycine. Serine was first obtained from silk protein, a particularly rich source, in 1865. Its name is derived from the Latin for silk, sericum. Serine's structure was established in 1902.407-41-0C0100557689797158113-P-SERINE62074DB04522N[C@@H](COP(O)(O)=O)C(O)=OC3H8NO6PInChI=1S/C3H8NO6P/c4-2(3(5)6)1-10-11(7,8)9/h2H,1,4H2,(H,5,6)(H2,7,8,9)/t2-/m0/s1BZQFBWGGLXLEPQ-REOHCLBHSA-N(2S)-2-amino-3-(phosphonooxy)propanoic acid185.0725185.008923505-0.974phosphoserine0-2FDB0219263-o-phosphoserine;Dexfosfoserine;Fosforina;L-3-phosphoserine;L-o-phosphoserine;L-o-serine phosphate;L-phosphoserine;L-serine dihydrogen phosphate (ester);L-serine phosphate;L-serinephosphorate;L-serinephosphoric acid;L-seryl phosphate;(+)-l-serine dihydrogen phosphate (ester);(2s)-2-amino-3-(phosphonooxy)propanoic acid;(s)-2-amino-3-hydroxypropanoic acid 3-phosphate;3-phosphoserine;O-phosphoserine;PhosphoserinePW_C000186SEP3489278110132122159124124711118126317299127878388134Oxoglutaric acidHMDB0000208Oxoglutaric acid, also known as alpha-ketoglutarate, alpha-ketoglutaric acid, AKG, or 2-oxoglutaric acid, is classified as a gamma-keto acid or a gamma-keto acid derivative. gamma-Keto acids are organic compounds containing an aldehyde substituted with a keto group on the C4 carbon atom. alpha-Ketoglutarate is considered to be soluble (in water) and acidic. alpha-Ketoglutarate is a key molecule in the TCA cycle, playing a fundamental role in determining the overall rate of this important metabolic process (PMID: 26759695). In the TCA cycle, AKG is decarboxylated to succinyl-CoA and carbon dioxide by AKG dehydrogenase, which functions as a key control point of the TCA cycle. Additionally, AKG can be generated from isocitrate by oxidative decarboxylation catalyzed by the enzyme known as isocitrate dehydrogenase (IDH). In addition to these routes of production, AKG can be produced from glutamate by oxidative deamination via glutamate dehydrogenase, and as a product of pyridoxal phosphate-dependent transamination reactions (mediated by branched-chain amino acid transaminases) in which glutamate is a common amino donor. AKG is a nitrogen scavenger and a source of glutamate and glutamine that stimulates protein synthesis and inhibits protein degradation in muscles. In particular, AKG can decrease protein catabolism and increase protein synthesis to enhance bone tissue formation in skeletal muscles (PMID: 26759695). Interestingly, enteric feeding of AKG supplements can significantly increase circulating plasma levels of hormones such as insulin, growth hormone, and insulin-like growth factor-1 (PMID: 26759695). It has recently been shown that AKG can extend the lifespan of adult C. elegans by inhibiting ATP synthase and TOR (PMID: 24828042). In combination with molecular oxygen, alpha-ketoglutarate is required for the hydroxylation of proline to hydroxyproline in the production of type I collagen. A recent study has shown that alpha-ketoglutarate promotes TH1 differentiation along with the depletion of glutamine thereby favouring Treg (regulatory T-cell) differentiation (PMID: 26420908). alpha-Ketoglutarate has been found to be associated with fumarase deficiency, 2-ketoglutarate dehydrogenase complex deficiency, and D-2-hydroxyglutaric aciduria, which are all inborn errors of metabolism (PMID: 8338207).328-50-7C0002651309152-KETOGLUTARATE50DB02926OC(=O)CCC(=O)C(O)=OC5H6O5InChI=1S/C5H6O5/c6-3(5(9)10)1-2-4(7)8/h1-2H2,(H,7,8)(H,9,10)KPGXRSRHYNQIFN-UHFFFAOYSA-N2-oxopentanedioic acid146.0981146.021523302-0.442oxoglutarate0-2FDB0033612-ketoglutarate;2-ketoglutaric acid;2-oxo-1,5-pentanedioate;2-oxo-1,5-pentanedioic acid;2-oxoglutarate;2-oxoglutaric acid;2-oxopentanedioate;2-oxopentanedioic acid;Oxoglutarate;Alpha-ketoglutaric acid;Oxoglutaric acid;A-ketoglutarate;A-ketoglutaric acid;Alpha-ketoglutarate;α-ketoglutarate;α-ketoglutaric acidPW_C000134AKG15242314141468499186733111084212635144750145526146754537510354141175438118556413260081476036155606915760921616482178653085747122275152247519151820922583742201186319812681289770542537713513377481111775231127774612977967345779703467797632777984347784253348001836880694135113162941199724061200221241200844071201741221205524141208144181209894081211464231211524241211604251227571201228311191231864501233994541235543741237184581237244591237324601253574791254002991254554811255332971258004891259294821269005011269403881269932061270662051272555061273885021104PhosphateHMDB0001429Phosphate is a salt of phosphoric acid. In organic chemistry, a phosphate, or organophosphate, is an ester of phosphoric acid. Organic phosphates are important in biochemistry, biogeochemistry and ecology. Phosphate (Pi) is an essential component of life. In biological systems, phosphorus is found as a free phosphate ion in solution and is called inorganic phosphate, to distinguish it from phosphates bound in various phosphate esters. Inorganic phosphate is generally denoted Pi and at physiological (neutral) pH primarily consists of a mixture of HPO<sup>2-</sup><sub>4</sub> and H<sub>2</sub>PO<sup>-</sup><sub>4</sub> ions. phosphates are most commonly found in the form of adenosine phosphates, (AMP, ADP and ATP) and in DNA and RNA and can be released by the hydrolysis of ATP or ADP. Similar reactions exist for the other nucleoside diphosphates and triphosphates. Phosphoanhydride bonds in ADP and ATP, or other nucleoside diphosphates and triphosphates, contain high amounts of energy which give them their vital role in all living organisms. Phosphate must be actively transported into cells against its electrochemical gradient. In vertebrates, two unrelated families of Na+-dependent Pi transporters carry out this task. Remarkably, the two families transport different Pi species: whereas type II Na+/Pi cotransporters (SCL34) prefer divalent HPO4(2), type III Na+/Pi cotransporters (SLC20) transport monovalent H2PO4. The SCL34 family comprises both electrogenic and electroneutral members that are expressed in various epithelia and other polarized cells. Through regulated activity in apical membranes of the gut and kidney, they maintain body Pi homeostasis, and in salivary and mammary glands, liver, and testes they play a role in modulating the Pi content of luminal fluids. Phosphate levels in the blood play an important role in hormone signaling and in bone homeostasis. In classical endocrine regulation, low serum phosphate induces the renal production of the seco-steroid hormone 1,25-dihydroxyvitamin D3 (1,25(OH)2D3).This active metabolite of vitamin D acts to restore circulating mineral (i.e. phosphate and calcium) levels by increasing absorption in the intestine, reabsorption in the kidney, and mobilization of calcium and phosphate from bone. Thus, chronic renal failure is associated with hyperparathyroidism, which in turn contributes to osteomalacia (softening of the bones). Another complication of chronic renal failure is hyperphosphatemia (low levels of phosphate in the blood). Hyperphosphatemia (excess levels of phosphate in the blood) is a prevalent condition in kidney dialysis patients and is associated with increased risk of mortality. Hypophosphatemia (hungry bone syndrome) has been associated to postoperative electrolyte aberrations and after parathyroidectomy. (PMID: 17581921, 11169009, 11039261, 9159312, 17625581)Fibroblast growth factor 23 (FGF-23) has recently been recognized as a key mediator of phosphate homeostasis, its most notable effect being promotion of phosphate excretion. FGF-23 was discovered to be involved in diseases such as autosomal dominant hypophosphatemic rickets, X-linked hypophosphatemia, and tumor-induced osteomalacia in which phosphate wasting was coupled to inappropriately low levels of 1,25(OH)2D3. FGF-23 is regulated by dietary phosphate in humans. In particular it was found that phosphate restriction decreased FGF-23, and phosphate loading increased FGF-23.14265-44-2C00009106118367CPD-85871032[O-]P([O-])([O-])=OO4PInChI=1S/H3O4P/c1-5(2,3)4/h(H3,1,2,3,4)/p-3NBIIXXVUZAFLBC-UHFFFAOYSA-Kphosphoric acid94.971494.953423phosphoric 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_C001104Pi2448488145818188312980317631417674925001027294727374631292931667236366138512342492244753150312751587520797521610053171115351112538110354471205543129557313356051355625108569365848143585514659111475941151604015561001616294107648717866911016714117684218868891607161205718920672122117306198738921074022127436163747522281962258258227101182411013425711748132117611151177321311904170119271641201428112728290132632233481917422553044235031542435318436923227701825377194293772171347794033677966130780483327805732978245353786693318002236889279308938313839479638411055839011064039111323594115845398116206109119982406120069122120699407121057124121216125121268429121352121121409123121423382121852405123304119123621118123786136123838464123968447123981399124405376124948472125362479125446297125774481125954299126221478126594300126604298126723484126904501127413388127783209128166395128177513128315389423MagnesiumHMDB0000547Magnesium 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+868227426816476272726811581918883229363998339922111674614834915294317641421241024115929422331262933737454031477491486954497456525310453291115356112537610359061475934151603815560941616250166648417865941646881160697919971702057194206722721372332117250214731021673131987473222117631321184321012312225123242491251328812581226127292901527528515337308771371337723632977937336783933347841733578489115785223317853635678574130800203688004518480048372806231188065413580865158096525381841519383238394900271085962231105593901156873981199744061200701221202473821207024071209814081211811241212654291213194191219241251220864051224084221227591201229213991233071191235463741238354641238894551244771361246373761249783751254472971255984841256694791257774811259214821259472991259734951260004901262434781265534911267533001271253891271645011273805021274073881274515071278042091281255081283473951875D-SerineHMDB0003406D-Serine is a non-essential amino acid occurring in natural form as the L-isomer. It is synthesized from glycine or threonine. It is involved in the biosynthesis of purines, pyrimidines, and other amino acids. As a constituent (residue) of proteins, its side chain can undergo O-linked glycosylation. This might be important in explaining some of the devastating consequences of diabetes. It is one of three amino acid residues that are commonly phosphorylated by kinases during cell signalling in eukaryotes. Phosphorylated serine residues are often referred to as phosphoserine. Serine proteases are a common type of protease. Serine (IPA [sejin]), organic compound, one of the 20 amino acids commonly found in animal proteins. Only the L-stereoisomer appears in mammalian protein. It is not essential to the human diet, since it can be synthesized in the body from other metabolites, including glycine. Serine was first obtained from silk protein, a particularly rich source, in 1865. Its name is derived from the Latin for silk, sericum. Serine's structure was established in 1902.312-84-5C00740685754916523Serines64231DB03929N[C@H](CO)C(O)=OC3H7NO3InChI=1S/C3H7NO3/c4-2(1-5)3(6)7/h2,5H,1,4H2,(H,6,7)/t2-/m1/s1MTCFGRXMJLQNBG-UWTATZPHSA-N(2R)-2-amino-3-hydroxypropanoic acid105.0926105.0425930950.663D-serine00FDB023164(2r)-2-amino-3-hydroxypropanoate;(2r)-2-amino-3-hydroxypropanoic acid;(r)-2-amino-3-hydroxypropanoate;(r)-2-amino-3-hydroxypropanoic acid;D-serin;Dl-serine;Dsn;Serine d-form;(r)-2-amino-3-hydroxy-propionic acid;(r)-2-amino-3-hydroxy-propionatePW_C001875D-Ser35192681310768141087088202708948709471709520183612198362220836315143767318437683157812313212217212412472411812633029912789238867L-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_C0000032KBA33781868226923827415183832254227247812613278163111786431337902711211992212212217612412227440612257740712271613512472811812482912012514911912531229712633329912643847912672648112685820512789638812800750112831920632Adenosine 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_C000032AMP112344628270167343288122118914457254867545033895251104540811754231035432118545712055581325583133577910157951086977199707218811789198118681611198815112003222125802261263631126942901333122542266342646315772343297732511178392334788091157932011280399180684135809007119916122120016124120031406120246382120888405121954408122920399123464376124507374125306297125394299125409479125596484126853205126934388126949501127124389127311209127711502170PyrophosphateHMDB0000250The 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.14000-31-8C0001364410218361PPI559142DB04160[O-]P([O-])(=O)OP([O-])([O-])=OO7P2InChI=1S/H4O7P2/c1-8(2,3)7-9(4,5)6/h(H2,1,2,3)(H2,4,5,6)/p-4XPPKVPWEQAFLFU-UHFFFAOYSA-J(phosphonooxy)phosphonic acid173.9433173.9119253784pyrophosphoric 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_C000170Ppi12235463842923735328822212173162049241059281529417514486854503489525210452941015409117542410354331185458120554811155591325584133560613556551085879107623916669781997073188713416372721607312198731821382751518283210118691611200222212041164123152251232324912512288125792261269529015219306153751834760174256131542697318772353297731712877635336784163357892833179153112799501347995813080047372804171708563019478638494814125948193829867822311063439111327039511327538911552713611553239911993412212001712412003240612033041012093640712126142912134112112148638312240742212298544412350211912383146412404439812497737512532429712539529912541047912559748412565648512587648112655249112686920512693538812695050112733720612812450827L-Seryl-tRNARNAPW_NA00002729162LST3508278115132122163124124715118126321299127882388133693813380711113385012213389013526L-Seryl-tRNA(Ser)RNAPW_NA00002629162LSTS3509278116132122164124124716118126322299127883388133694813380811113385112213389113528Glycyl-tRNARNAPW_NA00002829156GT3516278120132122169124124721118126327299127889388133671813372711113383212213387713529Glycyl-tRNA(Gly)RNAPW_NA00002929156GTG3517278121132122170124124722118126328299127890388133672813372611113383112213387613515998Amine oxidase [flavin-containing] AQ8K0Z8
Catalyzes the oxidative deamination of biogenic and xenobiotic amines and has important functions in the metabolism of neuroactive and vasoactive amines in the central nervous system and peripheral tissues. MAOA preferentially oxidizes biogenic amines such as 5-hydroxytryptamine (5-HT), norepinephrine and epinephrine (By similarity).
Maoa121.4.3.413229032913249111113339313215361Aldehyde dehydrogenase, mitochondrialP47738
Is capable of converting retinaldehyde to retinoic acid.
Aldh2121.2.1.3117739112132291133132493111133403132138072-amino-3-ketobutyrate coenzyme A ligase, mitochondrialO88986Gcat122.3.1.29785781327958811213229213317087Dimethylglycine dehydrogenase, mitochondrialQ8R1S7
Catalyzes the demethylation of N,N-dimethylglycine to sarcosine. Also has activity with sarcosine in vitro.
Dmgdh121.5.8.413224411217091Sarcosine dehydrogenase, mitochondrialQ99LB7Sardh121.5.8.313224513313657Glycine dehydrogenase (decarboxylating), mitochondrialQ91W43
The glycine cleavage system catalyzes the degradation of glycine. The P protein (GLDC) binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO(2) is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein (GCSH) (By similarity).
Gldc121.4.4.2773421337958411213656Aminomethyltransferase, mitochondrialQ8CFA2
The glycine cleavage system catalyzes the degradation of glycine.
Amt122.1.2.10773401337958511217096Serine hydroxymethyltransferase, mitochondrialQ99K87
Catalyzes the cleavage of serine to glycine accompanied with the production of 5,10-methylenetetrahydrofolate, an essential intermediate for purine biosynthesis (By similarity). Serine provides the major source of folate one-carbon in cells by catalyzing the transfer of one carbon from serine to tetrahydrofolate (By similarity). Contributes to the de novo mitochondrial thymidylate biosynthesis pathway via its role in glycine and tetrahydrofolate metabolism: thymidylate biosynthesis is required to prevent uracil accumulation in mtDNA (By similarity). Also required for mitochondrial translation by producing 5,10-methylenetetrahydrofolate; 5,10-methylenetetrahydrofolate providing methyl donors to produce the taurinomethyluridine base at the wobble position of some mitochondrial tRNAs (PubMed:29452640). Associates with mitochondrial DNA (By similarity). In addition to its role in mitochondia, also plays a role in the deubiquitination of target proteins as component of the BRISC complex: required for IFNAR1 deubiquitination by the BRISC complex (By similarity).
Shmt2122.1.2.113229313314857Dihydrolipoyl dehydrogenase, mitochondrialQ99LD3
Lipoamide dehydrogenase is a component of the glycine cleavage system as well as an E3 component of three alpha-ketoacid dehydrogenase complexes (pyruvate-, alpha-ketoglutarate-, and branched-chain amino acid-dehydrogenase complex) (By similarity). The 2-oxoglutarate dehydrogenase complex is mainly active in the mitochondrion (By similarity). A fraction of the 2-oxoglutarate dehydrogenase complex also localizes in the nucleus and is required for lysine succinylation of histones: associates with KAT2A on chromatin and provides succinyl-CoA to histone succinyltransferase KAT2A (By similarity). In monomeric form may have additional moonlighting function as serine protease (PubMed:17404228). Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction (By similarity).
Dld121.8.1.411779313313229411214948Serine--pyruvate aminotransferase, mitochondrialQ8R128
Dual metabolic roles of gluconeogenesis (in the mitochondria) and glyoxylate detoxification (in the peroxisomes).
Agxt122.6.1.51; 2.6.1.4413229511215302Glycine amidinotransferase, mitochondrialQ6IU01
Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development.
Gatm122.1.4.113229611215308Guanidinoacetate N-methyltransferaseQ3US90
Converts guanidinoacetate to creatine, using S-adenosylmethionine as the methyl donor. Important in nervous system development.
Gamt122.1.1.2132297132169415-aminolevulinate synthase, nonspecific, mitochondrialQ64453Alas1122.3.1.3713229811213345213215373Betaine--homocysteine S-methyltransferase 1Q561N0
Involved 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.
Bhmt122.1.1.513224011113229913217099Glycine N-methyltransferaseQ91WN7
Catalyzes 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 (By similarity).
Gnmt122.1.1.2013224211113230013216326Glycerate kinaseQ8K0R7Glyctk122.7.1.3113230113217102D-3-phosphoglycerate dehydrogenaseQ8C603
Catalyzes the reversible oxidation of 3-phospho-D-glycerate to 3-phosphonooxypyruvate, the first step of the phosphorylated L-serine biosynthesis pathway. Does not catalyze the reversible oxidation of 2-hydroxyglutarate to 2-oxoglutarate and the reversible oxidation of (S)-malate to oxaloacetate.
Phgdh121.1.1.9513230213213721Phosphoserine aminotransferaseQ99K85
Catalyzes the reversible conversion of 3-phosphohydroxypyruvate to phosphoserine and of 3-hydroxy-2-oxo-4-phosphonooxybutanoate to phosphohydroxythreonine.
Psat1122.6.1.527811113217111Phosphoserine phosphataseQ5XHW3
Catalyzes the last step in the biosynthesis of serine from carbohydrates. The reaction mechanism proceeds via the formation of a phosphoryl-enzyme intermediates (By similarity).
Psph123.1.3.313230313213725L-serine dehydratase/L-threonine deaminaseQ8VBT2Sds124.3.1.17; 4.3.1.197812413213332411115458Serine hydroxymethyltransferase, cytosolicQ8R0X9
Interconversion of serine and glycine.
Shmt1122.1.2.113224311113230513217125Serine racemaseQ8CD11
Catalyzes the synthesis of D-serine from L-serine. D-serine is a key coagonist with glutamate at NMDA receptors. Has dehydratase activity towards both L-serine and D-serine.
Srr125.1.1.18; 4.3.1.18; 4.3.1.1713230713214810Cystathionine beta-synthaseQ91WU3
Hydro-lyase catalyzing the first step of the transsulfuration pathway, where the hydroxyl group of L-serine is displaced by L-homocysteine in a beta-replacement reaction to form L-cystathionine, the precursor of L-cysteine. This catabolic route allows the elimination of L-methionine and the toxic metabolite L-homocysteine (By similarity). Also involved in the production of hydrogen sulfide, a gasotransmitter with signaling and cytoprotective effects on neurons (By similarity).
Cbs124.2.1.2213230813213245611114805Cystathionine gamma-lyaseQ6H324
Catalyzes 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.
Cth124.4.1.113230913213245711113723Serine--tRNA ligase, cytoplasmicP26638
Catalyzes the attachment of serine to tRNA(Ser) in a two-step reaction: serine is first activated by ATP to form Ser-AMP and then transferred to the acceptor end of tRNA(Ser). Is probably also able to aminoacylate tRNA(Sec) with serine, to form the misacylated tRNA L-seryl-tRNA(Sec), which will be further converted into selenocysteinyl-tRNA(Sec). In the nucleus, binds to the VEGFA core promoter and prevents MYC binding and transcriptional activation by MYC. Recruits SIRT2 to the VEGFA promoter, promoting deacetylation of histone H4 at 'Lys-16' (H4K16). Thereby, inhibits the production of VEGFA and sprouting angiogenesis mediated by VEGFA.
Sars126.1.1.117811713213380911113724Glycine--tRNA ligaseQ9CZD3
Catalyzes the ligation of glycine to the 3'-end of its cognate tRNA. Is also able produce diadenosine tetraphosphate (Ap4A), a universal pleiotropic signaling molecule needed for cell regulation pathways, by direct condensation of 2 ATPs (By similarity).
Gars123.6.1.17; 6.1.1.14781221321337281117725UnknownZ00Z0012531111179261333792621127926511679266134792751337934111379342329793973317942513079496332795343367954613279604352796133547983411413680Aldehyde dehydrogenase family 16 member A1Q571I9Aldh16a112775951337814211113717Serine palmitoyltransferase 3Q8BG54
Serine palmitoyltransferase (SPT). The heterodimer formed with LCB1/SPTLC1 constitutes the catalytic core. The composition of the serine palmitoyltransferase (SPT) complex determines the substrate preference. The SPTLC1-SPTLC3-SPTSSA isozyme uses both C14-CoA and C16-CoA as substrates, while the SPTLC1-SPTLC3-SPTSSB has the ability to use a broader range of acyl-CoAs without apparent preference (By similarity).
Sptlc3122.3.1.50780781337810311213718Pyruvate dehydrogenase phosphatase regulatory subunit, mitochondrialQ7TSQ8
Decreases the sensitivity of PDP1 to magnesium ions, and this inhibition is reversed by the polyamine spermine.
Pdpr12780821127808413313608Glutathione reductase, mitochondrialP47791
Maintains high levels of reduced glutathione in the cytosol.
Gsr121.8.1.777130133790291127798Serine--pyruvate aminotransferase, mitochondrialO35423
Dual metabolic roles of gluconeogenesis (in the mitochondria) and glyoxylate detoxification (in the peroxisomes).
Agxt122.6.1.51; 2.6.1.4455821337809311214144Glycine amidinotransferase, mitochondrialQ9D964
Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development.
Gatm122.1.4.1794371337958611214189Guanidinoacetate N-methyltransferaseO35969
Converts guanidinoacetate to creatine, using S-adenosylmethionine as the methyl donor. Important in nervous system development.
Gamt122.1.1.2794381117958713213768S-methylmethionine--homocysteine S-methyltransferase BHMT2Q91WS4
Involved in the regulation of homocysteine metabolism. Converts homocysteine to methionine using S-methylmethionine (SMM) as a methyl donor.
Bhmt2122.1.1.10783021327946211113720D-3-phosphoglycerate dehydrogenaseQ61753
Catalyzes the reversible oxidation of 3-phospho-D-glycerate to 3-phosphonooxypyruvate, the first step of the phosphorylated L-serine biosynthesis pathway. Does not catalyze the reversible oxidation of 2-hydroxyglutarate to 2-oxoglutarate and the reversible oxidation of (S)-malate to oxaloacetate.
Phgdh121.1.1.95781091327877011113722Phosphoserine phosphataseQ99LS3
Catalyzes the last step in the biosynthesis of serine from carbohydrates. The reaction mechanism proceeds via the formation of a phosphoryl-enzyme intermediates (By similarity).
Psph123.1.3.37811313213651Serine racemaseQ9QZX7
Catalyzes the synthesis of D-serine from L-serine. D-serine is a key coagonist with glutamate at NMDA receptors. Has dehydratase activity towards both L-serine and D-serine.
Srr125.1.1.18; 4.3.1.18; 4.3.1.17773211117811413213763Serine hydroxymethyltransferase, cytosolicP50431
Interconversion of serine and glycine.
Shmt1122.1.2.1782951327949711113767Cystathionine beta-synthaseQ91WT9
Hydro-lyase catalyzing the first step of the transsulfuration pathway, where the hydroxyl group of L-serine is displaced by L-homocysteine in a beta-replacement reaction to form L-cystathionine, the precursor of L-cysteine. This catabolic route allows the elimination of L-methionine and the toxic metabolite L-homocysteine (By similarity). Also involved in the production of hydrogen sulfide, a gasotransmitter with signaling and cytoprotective effects on neurons (By similarity).
Cbs124.2.1.22783011327882011113766Cystathionine gamma-lyaseQ8VCN5
Catalyzes 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.
Cth124.4.1.1782991327881811120202Serine--tRNA ligase, mitochondrialQ9JJL8
Catalyzes the attachment of serine to tRNA(Ser). Is also probably able to aminoacylate tRNA(Sec) with serine, to form the misacylated tRNA L-seryl-tRNA(Sec), which will be further converted into selenocysteinyl-tRNA(Sec).
Sars2126.1.1.116975Amine oxidase [flavin-containing] A12PW_P00697514789772516851Aldehyde dehydrogenase, mitochondrial12PW_P0068511466313680469762-amino-3-ketobutyrate coenzyme A ligase, mitochondrial12PW_P00697614790137176977Dimethylglycine dehydrogenase, mitochondrial12PW_P00697714791137186978Sarcosine dehydrogenase, mitochondrial12PW_P00697814792137186771Glycine dehydrogenase [decarboxylating], mitochondrial12PW_P006771145821365726769Aminomethyltransferase, mitochondrial12PW_P006769145801365616979Serine hydroxymethyltransferase, mitochondrial12PW_P0069791479377256770Dihydrolipoyl dehydrogenase, mitochondrial12PW_P006770145811360811340Serine--pyruvate aminotransferase12PW_P0013401563779827445Glycine amidinotransferase, mitochondrial12PW_P007445153101414427446Guanidinoacetate N-methyltransferase12PW_P0074461531114189169805-aminolevulinate synthase, nonspecific, mitochondrial12PW_P006980147941371717463Betaine--homocysteine S-methyltransferase 112PW_P007463153281376846981Glycine N-methyltransferase12PW_P00698114795772516965Glycerate kinase12PW_P0069651477977256982D-3-phosphoglycerate dehydrogenase12PW_P006982147961372016983Phosphoserine aminotransferase12PW_P006983147971372116984Phosphoserine phosphatase12PW_P006984147981372216761L-serine dehydratase/L-threonine deaminase12PW_P006761145721365127490Serine hydroxymethyltransferase, cytosolic12PW_P007490153551376346987Serine racemase12PW_P006987148011372517397Cystathionine beta-synthase12PW_P007397152601376747396Cystathionine gamma-lyase12PW_P007396152591376646985Serine--tRNA ligase, cytoplasmic12PW_P006985147991372321337831116986Glycine--tRNA ligase12PW_P0069861480013724213372511111508Serine--tRNA ligase, mitochondrial12PW_P01150820503202022110016falsePW_R110016Right39990814351Compoundfalse39990910651Compoundtrue39991014201Compoundtrue399911351Compoundtrue39991217831Compoundtrue3999139051Compoundfalse10031769751.4.3.4110017falsePW_R110017Right3999149051Compoundfalse3999157211Compoundtrue3999161641Compoundfalse39991711441Compoundtrue39991814201Compoundtrue10031868511.2.1.31429PW_R001429Right540527471Compoundfalse540613161Compoundtrue540714351Compoundfalse110018falsePW_R110018Right3999199401Compoundtrue399920781Compoundfalse39992127471Compoundfalse39992210991Compoundtrue10031969762.3.1.29110019falsePW_R110019Right399923621Compoundfalse39992414201Compoundtrue39992511021Compoundtrue3999261851Compoundfalse10032069771.5.8.4110020falsePW_R110020Right3999271851Compoundfalse39992814201Compoundtrue39992911021Compoundtrue399930781Compoundfalse10032169781.5.8.3108575falsePW_R108575Right393981781Compoundfalse39398211201Compoundfalse39398385261Compoundfalse39398413161Compoundtrue9873267711.4.4.2108573falsePW_R108573Right39397212211Compoundfalse39397385261Compoundfalse39397477571Compoundfalse393975351Compoundfalse39397611781Compoundfalse9873067692.1.2.10110021falsePW_R110021Both39993111781Compoundfalse399932781Compoundfalse39993314201Compoundtrue39993412211Compoundfalse3999351201Compoundfalse10032269792.1.2.1108574falsePW_R108574Right39397777571Compoundfalse3939787211Compoundtrue39397911201Compoundfalse39398011441Compoundtrue9873167701.8.1.4110022falsePW_R110022Both3999361641Compoundfalse3999371201Compoundfalse39993810431Compoundfalse3999391051Compoundtrue1003231340110023falsePW_R110023Both399940781Compoundfalse3999411641Compoundfalse3999421051Compoundtrue399943751Compoundfalse1003241340110024falsePW_R110024Both399944781Compoundfalse3999453961Compoundtrue399946831Compoundfalse3999471441Compoundtrue10032574452.1.4.1110025falsePW_R110025Right399948831Compoundfalse3999497491Compoundtrue3999509211Compoundtrue399951461Compoundfalse10032674462.1.1.2110026falsePW_R110026Right399952781Compoundfalse3999538081Compoundtrue3999548941Compoundfalse39995510991Compoundtrue10032769802.3.1.37109812falsePW_R109812Right399099301Compoundfalse3991005901Compoundfalse399101621Compoundfalse3991025481Compoundfalse10009274632.1.1.5110027falsePW_R110027Both3999561851Compoundfalse3999577491Compoundtrue3999589211Compoundtrue399959781Compoundfalse10032869812.1.1.20110028falsePW_R110028Both399960901Compoundfalse3999614141Compoundtrue3999626441Compoundfalse39996310341Compoundtrue10032969652.7.1.31110029falsePW_R110029Both3999646441Compoundfalse3999657211Compoundtrue3999668101Compoundfalse39996711441Compoundtrue10033069821.1.1.95110030falsePW_R110030Both3999688101Compoundfalse399969951Compoundtrue3999701861Compoundfalse3999711341Compoundtrue10033169832.6.1.52110031falsePW_R110031Right3999721861Compoundfalse39997314201Compoundtrue3999741201Compoundfalse39997511041Compoundtrue10033269843.1.3.3110032falsePW_R110032Both3999761201Compoundfalse3999771641Compoundfalse399978351Compoundtrue1003336761110034falsePW_R110034Both3999851201Compoundfalse39998612211Compoundtrue399987781Compoundfalse39998811781Compoundtrue39998914201Compoundtrue10033574902.1.2.1110036falsePW_R110036Right3999961201Compoundfalse39999718751Compoundfalse1003376987110037falsePW_R110037Right3999985901Compoundfalse3999991201Compoundfalse40000014201Compoundtrue400001671Compoundfalse10033873974.2.1.22110038falsePW_R110038Right400002671Compoundfalse40000314201Compoundtrue4000044481Compoundfalse40000531Compoundfalse400006351Compoundtrue10033973964.4.1.1178739falsePW_R178739Right6745811201Compoundfalse6745824141Compoundtrue674583271NucleicAcidtrue674584321Compoundtrue674585261NucleicAcidfalse6745861701Compoundtrue16882969856.1.1.11168830115086.1.1.11110035falsePW_R110035Right399990781Compoundfalse3999914141Compoundtrue399992281NucleicAcidtrue399993321Compoundtrue3999941701Compoundtrue399995291NucleicAcidfalse10033669866.1.1.14265218614353293false69555010regular1001002652187106532965false59020010regular78782652188142032949false69519510regular787826521893532963false30520510regular78782652190178332956false19520510regular787826521919053293false31554510regular10010026521929643299false45537510regular10025265219372113359false28569010regular503026521941641333false31593010regular1001002652195114413360false28585010regular50302652196142013349false40083510regular7878265219727471343false89553910regular1001202652198131613452false79667010regular7878265219994013381false130550410regular2001902652200781333false135072710regular1001002652201109913385false103052410regular5030265220211481339false112553410regular100352652203621123false50089010regular1001002652204142011249false60078010regular7878265220511021123false84577510regular10010026522061851123false92089010regular10010026522079641129false70089010regular100252652208142013349false100579510regular7878265220911021333false134089410regular10010026522109641339false112590010regular100252652211112011281false154072710regular2001902652212852611281false193251010regular2002002652213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600 C600 602 610 350 580 350 5false183610835M629 278 C637 316 610 350 580 350 5false183610836M734 273 C736 340 610 350 580 350 5false183610837M344 283 C345 332 399 336 430 350 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610838M234 283 C240 349 400 350 430 350 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610839M365 545 C362 430 400 350 430 350 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610840M325 195 L325 245 L375 195 z10true183610841M365 645 C365 675 365 715 365 745 5false183610842M335 705 C351 713 365 715 365 745 5false183610843M365 930 C365 900 365 860 365 830 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610844M335 865 C358 852 365 860 365 830 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610845M400 874 C377 870 365 860 365 830 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610846M895 599 C865 599 791 57 815 74 5true183610847M835 670 C837 624 856 623 895 599 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610848M795 600 C825 600 866 600 895 599 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610849M1305 599 C1275 599 1280 599 1250 599 5false183610850M1350 777 C1303 791 1288 614 1250 599 5false183610851M995 599 C1025 599 1070 599 1100 599 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610852M1055 554 C1057 588 1070 599 1100 599 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610853M1150 394 L1150 444 L1200 394 z10true183610854M600 940 C630 940 650 940 680 940 5false183610855M639 858 C641 893 650 940 680 940 5false183610856M895 875 C892 901 860 940 830 940 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610857M920 940 C890 940 860 940 830 940 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610858M150 150 L150 200 L200 150 z10true183610859M1020 940 C1044 942 1083 942 1105 945 5false183610860M1044 873 C1062 914 1075 945 1105 945 5false183610861M1340 944 C1307 946 1285 945 1255 945 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610862M1400 827 C1400 843 1285 945 1255 945 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610863M150 150 L150 200 L200 150 z10true183610864M1450 777 C1480 777 1556 610 1690 612 5false183610865M1640 727 C1647 666 1660 612 1690 612 5false183610866M1932 610 C1907 611 1871 613 1850 612 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610867M1880 681 C1874 631 1880 612 1850 612 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610868M1785 307 L1785 357 L1835 307 z10true183610869M3105 514 C3094 452 2060 417 2202 611 5false183610870M2132 610 C2162 610 2185 610 2202 611 5false183610871M2395 724 C2398 684 2375 610 2352 611 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610872M2400 573 C2397 593 2374 611 2352 611 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610873M2455 609 C2362 610 2382 608 2352 611 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610874M2655 609 C2685 609 2745 609 2775 609 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610875M2725 724 C2719 680 2746 609 2775 609 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610876M2699 573 C2705 598 2745 609 2775 609 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610877M3005 609 C2963 607 2945 609 2915 609 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610878M2975 719 C2978 679 2945 609 2915 609 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610879M2525 414 L2525 464 L2575 414 z10true183610880M2395 914 C2339 980 2097 964 2067 964 5false183610881M2120 1004 C2112 973 2097 964 2067 964 5false183610882M1640 917 C1631 971 1877 964 1907 964 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610883M1845 1002 C1845 968 1877 964 1907 964 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610884M2042.5 889 L2042.5 939 L2092.5 889 z10true183610885M2905 954 C2940 974 2980 981 2980 1011 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610886M2975 819 C2975 849 2980 981 2980 1011 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610887M2980 1169 C2980 1139 2980 1121 2980 1091 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610888M2905 1159 C2933 1154 2980 1121 2980 1091 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610889M2970 1011.5 L2970 1061.5 L3020 1011.5 z10true183610890M2725 824 C2725 854 2725 991 2725 1021 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3610891M2805 954 C2748 964 2725 991 2725 1021 5false18trueM 25.946855044164835 13.26155629629604 L 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matrix665455201.01.0160151797201120213136315340215674#FFEBEB432661252Non-Ketotic HyperglycinemiaNon Ketotic Hyperglycinemeia (Glycine encephalopathy; Glycine cleavage system deficiency; NKH) is caused by mutations in several genes in the mitochondrial glycine cleavage system. These include the genes encoding P protein (GLDC), T protein (GCST), and, in one case, the H protein (GCSH). Most patients with GCE (Glycine Encephalopathy, or NKH) have a defect in the GLDC gene.The enzyme system for cleavage of glycine (glycine cleavage system), which is confined to the mitochondria, is composed of 4 protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). NKH is characterized by accumulation of glycine in plasma, spinal fluid and urine. Symptoms include seizures, respiratory distress, mental retardation, chorea, visual impairment and hydrocephalus.DiseasePW_X008997CompleteContext89974873113656ProteinMutated4873214857ProteinMutated4873313657ProteinMutated4873478CompoundIncreased4873524TissueDamaged4873614TissueDamaged2779291943691Hiraga K, Koyata H, Sakakibara T, Ishiguro Y, Matsui C: Non-ketotic hyperglycinemia: an aim of the second generation of studies on pathogenesis. Mol Biol Med. 1991 Feb;8(1):65-79.8997Context