48ContextAromatic L-Aminoacid Decarboxylase DeficiencyAromatic L-Aminoacid Decarboxylase Deficiency (DOPA decarboxylase; DDC) is an autosomal recessive disease caused by a mutation in the DDC gene which codes for aromatic-L-aminoacid decarboxylase. A deficiency in this enzyme results in accumulation of 3-methoxytyrosine, 5-hydroxy-L-tryptophan, and L-Dopa in plasma, spinal fluid, and urine; 3-methoxytyramine and dopamine in urine. It also results in decreased concentrations of homovanillic acid, S-adenosylmethionine, and 5-hydroxytryptophol in spinal fluid; and epinephrine, norepinephrine in plasma. Symptoms include temperature instability, hypotonia, mental and motor retardation, and cerebral atrophy.DiseasePW000090CenterPathwayVisualizationContext9026502900#000099PathwayVisualization2325Catecholamine BiosynthesisThe Catecholamine Biosynthesis pathway depicts the synthesis of catecholamine neurotransmitters. Catecholamines are chemical hormones released from the adrenal glands as a response to stress that activate the sympathetic nervous system. They are composed of a catechol group and are derived from amino acids. The commonly found catecholamines are epinephrine (adrenaline), norepinephrine (noradrenaline) and dopamine. They are synthesized in catecholaminergic neurons by four enzymes, beginning with tyrosine hydroxylase (TH), which generates L-DOPA from tyrosine. The L-DOPA is then converted to dopamine via aromatic L-amino acid decarboxylase (AADC), which becomes norepinephrine via dopamine beta-hydroxylase (DBH); and finally is converted to epinephrine via phenylethanolamine N-methyltransferase (PNMT).Metabolic1796Sympathetic Nervous SystemSubPathway131948Compound83797Parasympathetic Nervous SystemSubPathway132048Compound8741Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.25Pathway42Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.25Pathway27961624925409Starke K: History of catecholamine research. Chem Immunol Allergy. 2014;100:288-301. doi: 10.1159/000359962. Epub 2014 May 23.25Pathway3NeuronCL:00005401CellCL:00000002Platelet CL:00002335HepatocyteCL:00001824CardiomyocyteCL:00007467Epithelial CellCL:00000666MyocyteCL:00001871Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote4Arabidopsis thaliana3702EukaryoteThale cress23Pseudomonas aeruginosa287Prokaryote12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat6Caenorhabditis elegans6239EukaryoteRoundworm24Solanum lycopersicum4081EukaryoteTomato18Saccharomyces cerevisiae4932EukaryoteYeast49Bathymodiolus platifrons220390EukaryoteDeep sea mussel10Drosophila melanogaster7227EukaryoteFruit fly2Bacteria2ProkaryoteBacteria19Schizosaccharomyces pombe4896Eukaryote21Xenopus laevis8355EukaryoteAfrican clawed frog25Escherichia coli (strain K12)83333Prokaryote60Nitzschia sp.0001EukaryoteNitzschia456Andro6666Eukaryote5CytoplasmGO:00057371CytosolGO:000582915NucleusGO:000563431Periplasmic SpaceGO:000562011Extracellular SpaceGO:000561535ChloroplastGO:00095072MitochondrionGO:000573954Endocytic VesicleGO:003013955Exocytic VesicleGO:00703826LysosomeGO:00057644PeroxisomeGO:000577713Endoplasmic ReticulumGO:000578310Cell MembraneGO:000588616Lysosomal LumenGO:00432027Endoplasmic Reticulum MembraneGO:00057893Mitochondrial MatrixGO:000575918Melanosome MembraneGO:003316214Mitochondrial Outer MembraneGO:000574124Mitochondrial Intermembrane SpaceGO:000575836MembraneGO:001602012Mitochondrial Inner MembraneGO:000574325Golgi ApparatusGO:000579420Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520253Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032540PeriplasmGO:004259732Inner MembraneGO:007025817NucleoplasmGO:000565439Mitochondrial membraneGO:00319663Sympathetic Nervous SystemBTO:00018324Adrenal MedullaBTO:00000497182Endothelium BTO:00003931LiverBTO:000075972925IntestineBTO:000064828StomachBTO:0001307155267Nervous SystemBTO:00014848Blood 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000158Tyrosine is an essential amino acid that readily passes the blood-brain barrier. Once in the brain, it is a precursor for the neurotransmitters dopamine, norepinephrine and epinephrine, better known as adrenalin. These neurotransmitters are an important part of the body's sympathetic nervous system, and their concentrations in the body and brain are directly dependent upon dietary tyrosine. Tyrosine is not found in large concentrations throughout the body, probably because it is rapidly metabolized. Folic acid, copper and vitamin C are cofactor nutrients of these reactions. Tyrosine is also the precursor for hormones, thyroid, catecholestrogens and the major human pigment, melanin. Tyrosine is an important amino acid in many proteins, peptides and even enkephalins, the body's natural pain reliever. Valine and other branched amino acids, and possibly tryptophan and phenylalanine may reduce tyrosine absorption. A number of genetic errors of tyrosine metabolism occur. Most common is the increased amount of tyrosine in the blood of premature infants, which is marked by decreased motor activity, lethargy and poor feeding. Infection and intellectual deficits may occur. Vitamin C supplements reverse the disease. Some adults also develop elevated tyrosine in their blood. This indicates a need for more vitamin C. More tyrosine is needed under stress, and tyrosine supplements prevent the stress-induced depletion of norepinephrine and can cure biochemical depression. However, tyrosine may not be good for psychosis. Many antipsychotic medications apparently function by inhibiting tyrosine metabolism. L-dopa, which is directly used in Parkinson's, is made from tyrosine. Tyrosine, the nutrient, can be used as an adjunct in the treatment of Parkinson's. Peripheral metabolism of tyrosine necessitates large doses of tyrosine, however, compared to L-dopa. (http://www.dcnutrition.com).60-18-4C00082605717895TYR5833DB00135N[C@@H](CC1=CC=C(O)C=C1)C(O)=OC9H11NO3InChI=1S/C9H11NO3/c10-8(9(12)13)5-6-1-3-7(11)4-2-6/h1-4,8,11H,5,10H2,(H,12,13)/t8-/m0/s1OUYCCCASQSFEME-QMMMGPOBSA-N(2S)-2-amino-3-(4-hydroxyphenyl)propanoic acid181.1885181.073893223-1.373L-tyrosine00FDB000446(-)-a-amino-p-hydroxyhydrocinnamate;(-)-a-amino-p-hydroxyhydrocinnamic acid;(-)-alpha-amino-p-hydroxyhydrocinnamate;(-)-alpha-amino-p-hydroxyhydrocinnamic acid;(s)-(-)-tyrosine;(s)-2-amino-3-(p-hydroxyphenyl)propionate;(s)-2-amino-3-(p-hydroxyphenyl)propionic acid;(s)-3-(p-hydroxyphenyl)alanine;(s)-tyrosine;(s)-a-amino-4-hydroxybenzenepropanoate;(s)-a-amino-4-hydroxybenzenepropanoic acid;(s)-a-amino-4-hydroxy-benzenepropanoate;(s)-a-amino-4-hydroxy-benzenepropanoic acid;(s)-alpha-amino-4-hydroxybenzenepropanoate;(s)-alpha-amino-4-hydroxybenzenepropanoic acid;(s)-alpha-amino-4-hydroxy-benzenepropanoate;(s)-alpha-amino-4-hydroxy-benzenepropanoic acid;2-amino-3-(4-hydroxyphen yl)-2-amino-3-(4-hydroxyphenyl)-propanoate;2-amino-3-(4-hydroxyphen yl)-2-amino-3-(4-hydroxyphenyl)-propanoic acid;3-(4-hydroxyphenyl)-l-alanine;4-hydroxy-l-phenylalanine;Benzenepropanoate;Benzenepropanoic acid;L-tyrosine;L-p-tyrosine;Tyr;Tyrosine;P-tyrosine;(2s)-2-amino-3-(4-hydroxyphenyl)propanoic acid;L-tyrosin;Y;(-)-α-amino-p-hydroxyhydrocinnamate;(-)-α-amino-p-hydroxyhydrocinnamic acid;(2s)-2-amino-3-(4-hydroxyphenyl)propanoate;(s)-α-amino-4-hydroxybenzenepropanoate;(s)-α-amino-4-hydroxybenzenepropanoic acidPW_C000103Tyr3961968620128481979247072356651075666108588610583422251234815142412318424133157705122477754341784691117904612879102132792362538218937782473378117773402117774403121001122121589124122652441122653442122655410123566135124147118125226476125227477125229444127552388627SapropterinHMDB0000787Sapropterin, also known as THBP or 6R-BH4, belongs to the class of organic compounds known as biopterins and derivatives. These are coenzymes containing a 2-amino-pteridine-4-one derivative. They are mainly synthesized in several parts of the body, including the pineal gland. Sapropterin is slightly soluble (in water) and a very weakly acidic compound (based on its pKa). Sapropterin has been detected in multiple biofluids, such as blood and cerebrospinal fluid. Within the cell, sapropterin is primarily located in the cytoplasm. In humans, sapropterin is involved in catecholamine biosynthesis pathway. Sapropterin is also involved in a couple of metabolic disorders, which include aromatic L-aminoacid decarboxylase deficiency and tyrosine hydroxylase deficiency. Sapropterin is converted from 7,8-dihydroneopterin triphosphate by 6-pyruvoyl tetrahydropterin synthase and sepiapterin reductase. It is essential in the formation of neurotransmitters and for nitric oxide synthase (PMID 16946131).62989-33-7C002729816201644917991951[H][C@@]1(CNC2=C(N1)C(=O)N=C(N)N2)[C@@H](O)[C@H](C)OC9H15N5O3InChI=1S/C9H15N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h3-4,6,12,15-16H,2H2,1H3,(H4,10,11,13,14,17)/t3-,4+,6-/m0/s1FNKQXYHWGSIFBK-RPDRRWSUSA-N(6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-1,4,5,6,7,8-hexahydropteridin-4-one241.2471241.117489371-2.046tetrahydrobiopterin00FDB022243(6r)-5,6,7,8-tetrahydro-l-biopterin;(6r)-5,6,7,8-tetrahydrobiopterin;(6r)-l-erythro-5,6,7,8-tetrahydrobiopterin;(6r)-l-erythro-tetrahydrobiopterin;(6r)-tetrahydrobiopterin;6r-bh4;6r-tetrahydro-l-biopterin;6b-5,6,7,8-tetrahydro-l-biopterin;R-thbp;Sapropterin;[6r-[6r*(1r*,2s*)]]-2-amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4(1h)-pteridinone;L-erythro-tetrahydrobiopterinPW_C0006276R-BH468720777553411065OxygenHMDB0001377Oxygen is the third most abundant element in the universe after hydrogen and helium and the most abundant element by mass in the Earth's crust. Diatomic oxygen gas constitutes 20.9% of the volume of air. All major classes of structural molecules in living organisms, such as proteins, carbohydrates, and fats, contain oxygen, as do the major inorganic compounds that comprise animal shells, teeth, and bone. Oxygen in the form of O2 is produced from water by cyanobacteria, algae and plants during photosynthesis and is used in cellular respiration for all living organisms. Green algae and cyanobacteria in marine environments provide about 70% of the free oxygen produced on earth and the rest is produced by terrestrial plants. Oxygen is used in mitochondria to help generate adenosine triphosphate (ATP) during oxidative phosphorylation. For animals, a constant supply of oxygen is indispensable for cardiac viability and function. To meet this demand, an adult human, at rest, inhales 1.8 to 2.4 grams of oxygen per minute. This amounts to more than 6 billion tonnes of oxygen inhaled by humanity per year. At a resting pulse rate, the heart consumes approximately 8-15 ml O2/min/100 g tissue. This is significantly more than that consumed by the brain (approximately 3 ml O2/min/100 g tissue) and can increase to more than 70 ml O2/min/100 g myocardial tissue during vigorous exercise. As a general rule, mammalian heart muscle cannot produce enough energy under anaerobic conditions to maintain essential cellular processes; thus, a constant supply of oxygen is indispensable to sustain cardiac function and viability. However, the role of oxygen and oxygen-associated processes in living systems is complex, and they and can be either beneficial or contribute to cardiac dysfunction and death (through reactive oxygen species). Reactive oxygen species (ROS) are a family of oxygen-derived free radicals that are produced in mammalian cells under normal and pathologic conditions. Many ROS, such as the superoxide anion (O2-)and hydrogen peroxide (H2O2), act within blood vessels, altering mechanisms mediating mechanical signal transduction and autoregulation of cerebral blood flow. Reactive oxygen species are believed to be involved in cellular signaling in blood vessels in both normal and pathologic states. The major pathway for the production of ROS is by way of the one-electron reduction of molecular oxygen to form an oxygen radical, the superoxide anion (O2-). Within the vasculature there are several enzymatic sources of O2-, including xanthine oxidase, the mitochondrial electron transport chain, and nitric oxide (NO) synthases. Studies in recent years, however, suggest that the major contributor to O2- levels in vascular cells is the membrane-bound enzyme NADPH-oxidase. Produced O2- can react with other radicals, such as NO, or spontaneously dismutate to produce hydrogen peroxide (H2O2). In cells, the latter reaction is an important pathway for normal O2- breakdown and is usually catalyzed by the enzyme superoxide dismutase (SOD). Once formed, H2O2 can undergo various reactions, both enzymatic and nonenzymatic. The antioxidant enzymes catalase and glutathione peroxidase act to limit ROS accumulation within cells by breaking down H2O2 to H2O. Metabolism of H2O2 can also produce other, more damaging ROS. For example, the endogenous enzyme myeloperoxidase uses H2O2 as a substrate to form the highly reactive compound hypochlorous acid. Alternatively, H2O2 can undergo Fenton or Haber-Weiss chemistry, reacting with Fe2+/Fe3+ ions to form toxic hydroxyl radicals (-.OH). (PMID: 17027622, 15765131).7782-44-7C0000797715379CPD-6641952O=OO2InChI=1S/O2/c1-2MYMOFIZGZYHOMD-UHFFFAOYSA-Ndioxygen31.998831.9898292440singlet oxygen00FDB022589Dioxygen;Molecular oxygen;O2;Oxygen;Oxygen molecule;[oo];Dioxygene;Disauerstoff;E 948;E-948;E948PW_C001065O295911052451650018505854914625286383649106743168820754157634769338362137549201624253122280329426042474713546712354801255493126550812758091085973147612915970061887032163705016073192137533210756021283951511181621611864198118832151189421112057225120631641224728612279226123252491270629112716292130042981301630013026301130383021326022342276174265731576910293770442947721413477350111773631307737733177395332774971137751211577537334776263367772333777736112777471297775634177805114778121337807032978151132783813457880534379111360120047408120383122120426405120542407120553414120594409120601406120883415121045124121104383121605434121656429122117382122573418122689384122798374122822443123027135123060376123128447123139136123163448123176119123187450123219137123226120123459451123609118123669398124163469124214464124669399125145454125275121125425482125706478125731483125737297125740479125884481126100299126272484126522495126721489126825480126964502126986207127198209127214208127219205127222501127305504127345206127557388127574515127835389128081395128095390128312506128432391117L-DopaHMDB0000181L-Dopa is the naturally occurring form of dihydroxyphenylalanine and the immediate precursor of dopamine. Unlike dopamine itself, L-Dopa can be taken orally and crosses the blood-brain barrier. It is rapidly taken up by dopaminergic neurons and converted to dopamine. In particular, it is metabolized to dopamine by aromatic L-amino acid decarboxylase. Pyridoxal phosphate (vitamin B6) is a required cofactor for this decarboxylation, and may be administered along with levodopa, usually as pyridoxine. L-Dopa is used for the treatment of Parkinsonian disorders and Dopa-Responsive Dystonia and is usually given with agents that inhibit its conversion to dopamine outside of the central nervous system. Peripheral tissue conversion may be the mechanism of the adverse effects of levodopa. It is standard clinical practice to co-administer a peripheral DOPA decarboxylase inhibitor - carbidopa or benserazide - and often a catechol-O-methyl transferase (COMT) inhibitor, to prevent synthesis of dopamine in peripheral tissue.59-92-7C00355604715765L-DOPA5824DB01235N[C@@H](CC1=CC=C(O)C(O)=C1)C(O)=OC9H11NO4InChI=1S/C9H11NO4/c10-6(9(13)14)3-5-1-2-7(11)8(12)4-5/h1-2,4,6,11-12H,3,10H2,(H,13,14)/t6-/m0/s1WTDRDQBEARUVNC-LURJTMIESA-N(2S)-2-amino-3-(3,4-dihydroxyphenyl)propanoic acid197.1879197.0688078454levodopa00FDB000567(-)-3-(3,4-dihydroxyphenyl)-l-alanine;(-)-dopa;(2s)-2-amino-3-(3,4-dihydroxyphenyl)propanoate;(2s)-2-amino-3-(3,4-dihydroxyphenyl)propanoic acid;3,4-dihydroxy-l-phenylalanine;3,4-dihydroxyphenyl-l-alanine;3,4-dihydroxyphenylalanine;3-(3,4-dihydroxyphenyl)-l-alanine;3-hydroxy-l-tyrosine;Bendopa;Cidandopa;Dihydroxy-l-phenylalanine;Dihydroxyphenylalanine;Dopaflex;Dopaidan;Dopal;Dopalina;Dopar;Doparkine;Doparl;Dopasol;Dopaston;Dopastone;Dopastral;Dopicar;Doprin;Eldopal;Eldopar;Eldopatec;Eurodopa;Helfo-dopa;Insulamina;L-(-)-dopa;L-3-(3,4-dihydroxyphenyl)-alanine;L-4-5-dihydroxyphenylalanine;L-dihydroxyphenylalanine;L-dopa;L-b-(3,4-dihydroxyphenyl)-a-alanine;L-beta-(3,4-dihydroxyphenyl)-alpha-alanine;Laradopa;Larodopa;Ledopa;Levedopa;Levodopa;Levopa;Maipedopa;Parda;Pardopa;Prodopa;Syndopa;Veldopa;Weldopa;B-(3,4-dihydroxyphenyl)-l-alanine;B-(3,4-dihydroxyphenyl)-a-l-alanine;B-(3,4-dihydroxyphenyl)alanine;Beta-(3,4-dihydroxyphenyl)-l-alanine;Beta-(3,4-dihydroxyphenyl)-alpha-l-alanine;Beta-(3,4-dihydroxyphenyl)alanine;L-beta-(3,4-dihydroxyphenyl)alanine;Levodopum;β-(3,4-dihydroxyphenyl)-l-alanine;β-(3,4-dihydroxyphenyl)alanine;L-b-(3,4-dihydroxyphenyl)alanine;L-β-(3,4-dihydroxyphenyl)alaninePW_C000117L-Dopa39719689202025277042293777573417912013212161512412417311812758438827DihydrobiopterinHMDB0000038Dihydrobiopterin (BH2) is an oxidation product of tetrahydrobiopterin. Tetrahydrobiopterin is a natural occurring cofactor of the aromatic amino acid hydroxylase and is involved in the synthesis of tyrosine and the neurotransmitters dopamine and serotonin. Tetrahydrobiopterin is also essential for nitric oxide synthase catalyzed oxidation of L-arginine to L-citrulline and nitric oxide.6779-87-9C0295325264277BIOPTERIN247C[C@H](O)[C@H](O)C1=NC2=C(NC1)NC(N)=NC2=OC9H13N5O3InChI=1S/C9H13N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h3,6,15-16H,2H2,1H3,(H4,10,11,13,14,17)/t3-,6-/m0/s1FEMXZDUTFRTWPE-DZSWIPIPSA-N2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-1,4,7,8-tetrahydropteridin-4-one239.2312239.101839307-2.175dihydrobiopterin00FDB021884(6r)-6-(l-erythro-1,2-dihydroxypropyl)-7,8-dihydro-6h-pterin;(s-(r*,s*))-2-amino-6-(1,2-dihydroxypropyl)-7,8-dihydro-4(1h)-pteridinone;2-amino-6-((1r,2s)-1,2-dihydroxypropyl)-7,8-dihydro-4(1h)-pteridinone;2-amino-6-(1,2-dihydroxypropyl)-7,8-dihydro-4(1h)-pteridinone;6,7-dihydrobiopterin;7,8-dihydro-l-biopterin;7,8-dihydrobiopterin;Bh2;Dihydrobiopterin;L-erythro-1-(2-amino-7,8-dihydro-4-hydroxy-6-pteridinyl)-1,2-propanediol;L-erythro-7,8-dihydrobiopterin;L-erythro-dihydrobiopterin;L-erythro-q-dihydrobiopterin;Quinoid-dihydrobiopterin;Quinonoid dihydrobiopterin;Quinonoid dihydro-(6h)-biopterinPW_C000027BH3690201960277758341786651321223291241248801181264962991280673881420WaterHMDB0002111Water 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_C001420H2O5589491095139415131621448113526156242865210691207703382318838210943113774914655415904320182425322226786027274627781728052931437031647236346145983647273749419350302751567519597521410052279452361035297105531911153431135355112540211054701235483125549212655071275534130553711455411295591135560811856221085691657591405778101584114358531465877107589095591014759401516032155605915760871616123163613315962151621816664771786507180660015267131176840188688816071622057181207719320672112117228213723821472432157295198735021673882107401212746722274922247500190758817082012258237226841416292652611850277119221641201128112213285122502861226428712327249125202271263265126932901270529112715292130072981301930013025301130373021326122313327294153403084232731542695318436913227691429377019253771021327713113377215134773783317739733277471333775161157753633477628336777223377775934177816343779823477807132978235352782423537827035679113360800143688003937080591228806561199383038394794384110557390110639391115844398119879232119915122119963406120008407120046408120113124120365412120430405120438409120606415120794414121158425121240429121351121121381419121607434122118382122384436122753120122797374122804443123012446123064376123072137123131447123142136123162448123231451123384450123730460123810464123940455124165469124670399124938471124945472125305297125353479125386481125424482125480299125682483125707478125745487126054490126238495126273484126764480126896501126963502127017388127177208127199209127227504127506507127576515127836389128082395128176513140674790140675834140755185544Fe2+HMDB0000692Iron is a chemical element with the symbol Fe and atomic number 26. Iron makes up 5% of the Earth's crust and is second in abundance to aluminium among the metals and fourth in abundance among the elements. Physiologically, it. exists as an ion in the body. Iron (as Fe2+, ferrous ion) is a necessary trace element used by all known living organisms. Iron-containing enzymes, usually containing heme prosthetic groups, participate in catalysis of oxidation reactions in biology, and in transport of a number of soluble gases. Iron is an essential constituent of hemoglobin, cytochrome, and other components of respiratory enzyme systems. Its chief functions are in the transport of oxygen to tissue (hemoglobin) and in cellular oxidation mechanisms. Inorganic iron involved in redox reactions is also found in the iron-sulfur clusters of many enzymes, such as nitrogenase (involved in the synthesis of ammonia from nitrogen and hydrogen) and hydrogenase. A class of non-heme iron proteins is responsible for a wide range of functions such as ribonucleotide reductase (reduces ribose to deoxyribose; DNA biosynthesis) and purple acid phosphatase (hydrolysis of phosphate esters). When the body is fighting a bacterial infection, the body sequesters iron inside of cells (mostly stored in the storage molecule ferritin) so that it cannot be used by bacteria. Depletion of iron stores may result in iron-deficiency anemia. Iron is used to build up the blood in anemia. Humans experience iron toxicity above 20 milligrams of iron for every kilogram of weight, and 60 milligrams per kilogram is a lethal dose. Over-consumption of iron, often the result of children eating large quantities of ferrous sulfate tablets intended for adult consumption, is the most common toxicological cause of death in children under six. The DRI lists the Tolerable Upper Intake Level (UL) for adults as 45 mg/day. For children under fourteen years old the UL is 40 mg/day. Iron is a metal extracted from iron ore, and is almost never found in the free elemental state.15438-31-0C148182728429033Ferric-Hydroxamate-Complexes25394DB01592[Fe++]FeInChI=1S/Fe/q+2CWYNVVGOOAEACU-UHFFFAOYSA-Nlambda2-iron(2+) ion55.84555.9349421330lambda2-iron(2+) ion22FDB016251Armco iron;Carbonyl iron;Fe;Ferrovac e;Hematite;Infed;Loha;Limonite;Magnetite;Malleable iron;Metopirone;Metyrapone;Pzho;Pzh2m;Remko;Suy-b 2;Taconite;Venofer;Wrought iron;Fe (ii) ion;Fe(ii);Fe2+;Fe(2+);Ferrous ion;Iron ion(2+)PW_C000544Fe2+39819641367831692207098177727041163705216012060225121431517717913277740112777511297776034177782111120544407120557414120570122121765124123178119123191450123204135124316118126143299126185481127650388140710491407161852DopamineHMDB0000073Dopamine is a member of the catecholamine family of neurotransmitters in the brain and is a precursor to epinephrine (adrenaline) and norepinephrine (noradrenaline). Dopamine is synthesized in the body (mainly by nervous tissue and adrenal glands) first by the hydration of the amino acid tyrosine to DOPA by tyrosine hydroxylase and then by the decarboxylation of DOPA by aromatic-L-amino-acid decarboxylase. Dopamine is a major transmitter in the extrapyramidal system of the brain, and important in regulating movement. A family of receptors (dopamine receptors) mediates its action, which plays a major role in reward-motivated behaviour. Dopamine has many other functions outside the brain. In blood vessels, dopamine inhibits norepinephrine release and acts as a vasodilator (at normal concentrations); in the kidneys, it increases sodium excretion and urine output; in the pancreas, it reduces insulin production; in the digestive system, it reduces gastrointestinal motility and protects intestinal mucosa; and in the immune system, it reduces the activity of lymphocytes. Parkinson's disease, a degenerative condition causing tremor and motor impairment, is caused by a loss of dopamine-secreting neurons in an area of the midbrain called the substantia nigra. There is evidence that schizophrenia involves altered levels of dopamine activity, and most antipsychotic drugs used to treat this are dopamine antagonists, which reduce dopamine activity. Attention deficit hyperactivity disorder, bipolar disorder, and addiction are also characterized by defects in dopamine production or metabolism. It has been suggested that animals derived their dopamine-synthesizing machinery from bacteria via horizontal gene transfer that may have occurred relatively late in evolutionary time. This is perhaps a result of the symbiotic incorporation of bacteria into eukaryotic cells that gave rise to mitochondria. Dopamine is elevated in the urine of people who consume bananas. When present in sufficiently high levels, dopamine can be a neurotoxin and a metabotoxin. A neurotoxin is a compound that disrupts or attacks neural tissue. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of dopamine are associated with neuroblastoma, Costello syndrome, leukemia, phaeochromocytoma, aromatic L-amino acid decarboxylase deficiency, and Menkes disease (MNK). High levels of dopamine can lead to hyperactivity, insomnia, agitation and anxiety, depression, delusions, excessive salivation, nausea, and digestive problems.51-61-6C0375868118243DOPAMINE661DB00988NCCC1=CC(O)=C(O)C=C1C8H11NO2InChI=1S/C8H11NO2/c9-4-3-6-1-2-7(10)8(11)5-6/h1-2,5,10-11H,3-4,9H2VYFYYTLLBUKUHU-UHFFFAOYSA-N4-(2-aminoethyl)benzene-1,2-diol153.1784153.078978601-1.313dopamine01FDB0121632-(3,4-dihydroxyphenyl)ethylamine;3,4-dihydroxyphenethylamine;3,4-dihydroxyphenylethylamine;3-hydroxytyramine;4-(2-aminoethyl)-1,2-benzenediol;4-(2-aminoethyl)-pyrocatechol;4-(2-aminoethyl)catechol;4-(2-aminoethyl)pyrocatechol;Deoxyepinephrine;Dopamin;Dopamine;Dopaminum;Dopastat;Dophamine;Dynatra;Hydroxytyramin;Hydroxytyramine;Intropin;Oxytyramine;Revivan;A-(3,4-dihydroxyphenyl)-b-aminoethane;Alpha-(3,4-dihydroxyphenyl)-beta-aminoethane;4-(2-aminoethyl)benzene-1,2-diol;DopaminaPW_C000052LDP400196942020172420802318774318871416115770432937776234179112360791381328005098800511841216064341216371241241644691241951181275755151276113881316Carbon 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_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905021407981851148Pyridoxal 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'P1823244535181221401196962011104214505014582621201021504953251115416117542110354411185455120556713255811336533857018160716720572162127222213118581611217515112623311262818126842891268929077017253770372257704129377052224775261127776434177973346779793277829234578855332788623318069613598630711991212212002412412002940612008740712081741812114942312115542412206912312207638312283411912340245412372145812372745912462044712462739812530229712540229912540747912545848112580348912622429812623149512694238812694750112699620612725850612778651312779339031Ascorbic acidHMDB0000044Ascorbic acid is found naturally in citrus fruits and many vegetables and is an essential nutrient in human diets. It is necessary to maintain connective tissue and bone. The biologically active form of ascorbic acid is vitamin C. Vitamin C is a water soluble vitamin. Primates (including humans) and a few other species in all divisions of the animal kingdom, notably the guinea pig, have lost the ability to synthesize ascorbic acid and must obtain it in their food. Vitamin C functions as a reducing agent and coenzyme in several metabolic pathways. Vitamin C is considered an antioxidant. [PubChem] Ascorbic acid is an electron donor for enzymes involved in collagen hydroxylation, biosynthesis of carnitine and norepinephrine, tyrosine metabolism, and amidation of peptide hormones. Ascrobic acid (vitamin C) deficiency causes scurvy. The amount of vitamin C necessary to prevent scurvy may not be adequate to maintain optimal health. The ability of vitamin C to donate electrons also makes it a potent water-soluble antioxidant that readily scavenges free radicals such as molecular oxygen, superoxide, hydroxyl radical, and hypochlorous acid. In this setting, several mechanisms could account for a link between vitamin C and heart disease. One is the relation between LDL oxidation and vitamins C and E. Vitamin C in vitro can recycle vitamin E, which can donate electrons to prevent LDL oxidation in vitro. As the lipid-phase vitamin E is oxidized, it can be regenerated by aqueous vitamin C. Other possibilities are that vitamin C could decrease cholesterol by mechanisms not well characterized, or could improve vasodilatation and vascular reactivity, perhaps by decreasing the interactions of nitric oxide with oxidants. (PMID: 10799361).50-81-7C000725467006729073ASCORBATE10189562DB00126[H][C@@]1(OC(=O)C(O)=C1O)[C@@H](O)COC6H8O6InChI=1S/C6H8O6/c7-1-2(8)5-3(9)4(10)6(11)12-5/h2,5,7-10H,1H2/t2-,5+/m0/s1CIWBSHSKHKDKBQ-JLAZNSOCSA-N(5R)-5-[(1S)-1,2-dihydroxyethyl]-3,4-dihydroxy-2,5-dihydrofuran-2-one176.1241176.0320879880.144vitamin C0-1FDB001224(+)-sodium l-ascorbate;(+)-ascorbate;(+)-ascorbic acid;3-keto-l-gulofuranolactone;3-oxo-l-gulofuranolactone;Adenex;Allercorb;Antiscorbic vitamin;Antiscorbutic vitamin;Arco-cee;Ascoltin;Ascor-b.i.d.;Ascorb;Ascorbajen;Ascorbate;Ascorbic acid;Ascorbicab;Ascorbicap;Ascorbicin;Ascorbin;Ascorbutina;Ascorin;Ascorteal;Ascorvit;C-level;C-long;C-quin;C-span;C-vimin;Cantan;Cantaxin;Catavin c;Ce lent;Ce-mi-lin;Ce-vi-sol;Cebicure;Cebid;Cebion;Cebione;Cecon;Cee-caps td;Cee-vite;Cegiolan;Ceglion;Ceklin;Celaskon;Celin;Cell c;Cemagyl;Cemill;Cenetone;Cenolate;Cereon;Cergona;Cescorbat;Cetamid;Cetane;Cetane-caps tc;Cetane-caps td;Cetebe;Cetemican;Cevalin;Cevatine;Cevex;Cevi-bid;Cevimin;Cevital;Cevitamate;Cevitamic acid;Cevitamin;Cevitan;Cevitex;Cewin;Chewcee;Ciamin;Cipca;Citriscorb;Citrovit;Colascor;Concemin;Davitamon c;Dora-c-500;Duoscorb;Ferrous ascorbate;Hicee;Hybrin;Ido-c;Juvamine;Kangbingfeng;Kyselina askorbova;L(+)-ascorbate;L(+)-ascorbic acid;L-(+)-ascorbate;L-(+)-ascorbic acid;L-3-ketothreohexuronic acid lactone;L-ascorbate;L-ascorbic acid;L-lyxoascorbate;L-lyxoascorbic acid;L-threo-ascorbic acid;L-xyloascorbate;L-xyloascorbic acid;Laroscorbine;Lemascorb;Liqui-cee;Meri-c;Natrascorb;Natrascorb injectable;Planavit c;Proscorbin;Redoxon;Ribena;Ronotec 100;Rontex 100;Roscorbic;Rovimix c;Scorbacid;Scorbu c;Scorbu-c;Secorbate;Sodascorbate;Suncoat vc 40;Testascorbic;Vasc;Vicelat;Vicin;Vicomin c;Viforcit;Viscorin;Viscorin 100m;Vitace;Vitacee;Vitacimin;Vitacin;Vitamin c;Vitamisin;Vitascorbol;Xitix;Gamma-lactone l-threo-hex-2-enonate;Gamma-lactone l-threo-hex-2-enonic acid;Acide ascorbique;Acido ascorbico;Acidum ascorbicum;Acidum ascorbinicum;Ascorbinsaeure;E 300;E-300;E300PW_C000031VitC67931698201267520211462521071214615112297225134022224250631877752129777663417842933479116115115855336120558414120993408121610405123192450123558374124168376125933482127392502127579209142NorepinephrineHMDB0000216Precursor of epinephrine that is secreted by the adrenal medulla and is a widespread central and autonomic neurotransmitter. Norepinephrine is the principal transmitter of most postganglionic sympathetic fibers and of the diffuse projection system in the brain arising from the locus ceruleus. It is also found in plants and is used pharmacologically as a sympathomimetic.51-41-2C0054743926018357NOREPINEPHRINE388394DB00368NC[C@H](O)C1=CC(O)=C(O)C=C1C8H11NO3InChI=1S/C8H11NO3/c9-4-8(12)5-1-2-6(10)7(11)3-5/h1-3,8,10-12H,4,9H2/t8-/m0/s1SFLSHLFXELFNJZ-QMMMGPOBSA-N4-[(1R)-2-amino-1-hydroxyethyl]benzene-1,2-diol169.1778169.0738932234norepinephrine01FDB000895(-)-(r)-norepinephrine;(-)-arterenol;(-)-noradrenaline;(-)-norepinephrine;(-)-alpha-(aminomethyl)protocatechuyl alcohol;(r)-(-)-norepinephrine;(r)-4-(2-amino-1-hydroxyethyl)-1,2-benzenediol;(r)-noradrenaline;(r)-norepinephrine;4-(2-amino-1-hydroxyethyl)-1,2-benzenediol;4-[(1r)-2-amino-1-hydroxyethyl]-1,2-benzenediol;Adrenor;Aktamin;Arterenol;L-2-amino-1-(3,4-dihydroxyphenyl)ethanol;L-3,4-dihydroxyphenylethanolamine;L-arterenol;L-noradrenaline;L-norepinephrine;L-alpha-(aminomethyl)-3,4-dihydroxybenzyl alcohol;Levarterenol;Levoarterenol;Levonor;Levonoradrenaline;Levonorepinephrine;Levophed;Nor-epirenan;Noradrenalin;Noradrenaline;Norartrinal;Norepirenamine;Sympathin e;Norepinefrina;Norepinephrine;NorepinephrinumPW_C000142Norpp4061969920999141081371165481166153179743180717776734179117115121611405124169376127580209977Dehydroascorbic acidHMDB0001264Dehydroascorbic acid is the oxidized form of vitamin C. Reduced Vitamin C concentrations in the brain exceed those in blood by 10 fold. Dehydroascorbic acid readily enters the brain and is retained in the brain tissue in the form of ascorbic acid (ascorbic acid is not able to cross the blood-brain barrier). Therefore, transport of dehydroascorbic acid by the Glucose Transporter 1 (GLUT1, Glucose transporters are integral membrane glycoproteins involved in transporting glucose into most cells. GLUT1 is a major glucose transporter in the mammalian blood-brain barrier. It is present at high levels in primate erythrocytes and brain endothelial cells.) is a mechanism by which the brain acquires vitamin C. (OMIM 138140).490-83-5C0042521032817242L-DEHYDRO-ASCORBATE182283[H][C@@]1(OC(=O)C(=O)C1=O)[C@H](O)COC6H6O6InChI=1S/C6H6O6/c7-1-2(8)5-3(9)4(10)6(11)12-5/h2,5,7-8H,1H2/t2-,5-/m1/s1SBJKKFFYIZUCET-DUZGATOHSA-N(5R)-5-[(1R)-1,2-dihydroxyethyl]oxolane-2,3,4-trione174.1082174.0164379240.042(5R)-5-[(1R)-1,2-dihydroxyethyl]oxolane-2,3,4-trione0-1FDB0214591-dehydroascorbate;1-dehydroascorbic acid;Dhaa;Dehydro-l-ascorbate;Dehydro-l-ascorbic acid;Dehydroascorbate;L-dehydroascorbate;L-dehydroascorbic acid;L-threo-2,3-hexodiulosonic acid gamma-lactone;L-threo-hexo-2,3-diulosono-1,4-lactone;Oxidized ascorbate;Oxidized ascorbic acid;Oxidized vitamin cPW_C000977DHAA70020202214122982251340422277768341791181151216124051241703761275812098525Pyrroloquinoline quinoneHMDB0013636Enzymes containing PQQ are called quinoproteins. PQQ and quinoproteins play a role in the redox metabolism and structural integrity of cells and tissues [PMID:2558842]. It was reported that aminoadipate semialdehyde dehydrogenase (AASDH) might also use PQQ as a cofactor, suggesting a possibility that PQQ is a vitamin in mammals. [PMID:12712191].72909-34-3C00113102418315PQQ997DB03205OC(=O)C1=CC2=C(N1)C1=C(N=C(C=C1C(O)=O)C(O)=O)C(=O)C2=OC14H6N2O8InChI=1S/C14H6N2O8/c17-10-4-2-6(14(23)24)15-8(4)7-3(12(19)20)1-5(13(21)22)16-9(7)11(10)18/h1-2,15H,(H,19,20)(H,21,22)(H,23,24)MMXZSJMASHPLLR-UHFFFAOYSA-N4,5-dioxo-1H,4H,5H-pyrrolo[2,3-f]quinoline-2,7,9-tricarboxylic acid330.206330.012415178-3.534pyrroloquinoline quinone0-3C00113Methoxatin;Pqq;Pyrrolo-quinoline quinone;Pyrroloquinoline-quinone;2,4,6-tricarboxylic-pyrrolo[2,3-5,6]quinoline 8,9-quinone;2,7,9-tricarboxy-1h-pyrrolo(2,3-f)quinoline-4,5-dione;4,5-dihydro-4,5-dioxo-1h-pyrrolo[2,3-5,6]quinoline-2,7,9-tricarboxylic acid;4,5-dioxo-4,5-dihydro-1h-pyrrolo[2,3-f]quinoline-2,7,9-tricarboxylate;Coenzyme pqq;4,5-dihydro-4,5-dioxo-1h-pyrrolo[2,3-5,6]quinoline-2,7,9-tricarboxylate;4,5-dioxo-4,5-dihydro-1h-pyrrolo[2,3-f]quinoline-2,7,9-tricarboxylic acidPW_C008525Pqq40719701202023147776934179119115121613405124171376127582209514CopperHMDB0000657Copper is an essential nutrient to all higher plants and animals. Physiologically, it exists as an ion in the body. In animals, it is found primarily in the bloodstream, as a cofactor in various enzymes, and in copper-based pigments. In the body, copper shifts between the cuprous (Cu1+) and cupric (Cu2+) forms, though the majority of the body's copper is in the Cu2+ form. The ability of copper to easily accept and donate electrons explains its important role in oxidation-reduction (redox) reactions and in scavenging free radicals. Copper is a critical functional component of a number of essential enzymes known as cuproenzymes. For instance, the copper-dependent enzyme, cytochrome c oxidase, plays a critical role in cellular energy production. By catalyzing the reduction of molecular oxygen (O2) to water (H2O), cytochrome c oxidase generates an electrical gradient used by the mitochondria to create the vital energy-storing molecule, ATP. Another cuproenzyme, lysyl oxidase, is required for the cross-linking of collagen and elastin, which are essential for the formation of strong and flexible connective tissue. Another cuproeznyme, Monoamine oxidase (MAO), plays a role in the metabolism of the neurotransmitters norepinephrine, epinephrine, and dopamine. MAO also functions in the degradation of the neurotransmitter serotonin, which is the basis for the use of MAO inhibitors as antidepressants. One of the most important cuproenzymes is Superoxide dismutase (SOD). SOD functions as an antioxidant by catalyzing the conversion of superoxide radicals (free radicals or ROS) to hydrogen peroxide, which can subsequently be reduced to water by other antioxidant enzymes. Two forms of SOD contain copper: 1) copper/zinc SOD is found within most cells of the body, including red blood cells, and 2) extracellular SOD is a copper-containing enzyme found at high levels in the lungs and low levels in blood plasma. In sufficient amounts, copper can be poisonous or even fatal to organisms. Copper is normally bound to cuproenzymes (such as SOD, MOA) and is thus only toxic when unsequestered and unmediated. It is believed that zinc and copper compete for absorption in the digestive tract so that a diet that is excessive in one of these minerals may result in a deficiency in the other. An imbalance of zinc and copper status might be involved in human hypertension.15158-11-9C000702709929036CUCL225221[Cu++]CuInChI=1S/Cu/q+2JPVYNHNXODAKFH-UHFFFAOYSA-Ncopper(2+) ion63.54662.9296010790copper(2+) ion22FDB003582Copper;Cu;Copper (ii) ion;Copper(ii) cation;Copper, ion (cu2+);Cu(ii);Cu2+;Cu(2+);Cupric ionPW_C000514Cu40819552147022075615761877133201122019246587109700918942791317770492947759911577770341778071147781111177817343781531327911436079246293120464405120596409120599122120607415121048124121608434123098376123221137123224135123232451123612118124166469125710478125733483125738297125746487127202209127216208127220205127228504127577515127588388921S-AdenosylmethionineHMDB0001185S-Adenosylmethionine (CAS: 29908-03-0), also known as SAM or AdoMet, is a physiologic methyl radical donor involved in enzymatic transmethylation reactions and present in all living organisms. It possesses anti-inflammatory activity and has been used in the treatment of chronic liver disease (From Merck, 11th ed). S-Adenosylmethionine is a natural substance present in the cells of the body. It plays a crucial biochemical role by donating a one-carbon methyl group in a process called transmethylation. S-Adenosylmethionine, formed from the reaction of L-methionine and adenosine triphosphate catalyzed by the enzyme S-adenosylmethionine synthetase, is the methyl-group donor in the biosynthesis of both DNA and RNA nucleic acids, phospholipids, proteins, epinephrine, melatonin, creatine, and other molecules.485-80-3C000192476216515414S-ADENOSYLMETHIONINE31983DB00118C[S+](CC[C@H](N)C(O)=O)C[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2NC15H23N6O5SInChI=1S/C15H22N6O5S/c1-27(3-2-7(16)15(24)25)4-8-10(22)11(23)14(26-8)21-6-20-9-12(17)18-5-19-13(9)21/h5-8,10-11,14,22-23H,2-4,16H2,1H3,(H2-,17,18,19,24,25)/p+1/t7-,8+,10+,11+,14+,27?/m0/s1MEFKEPWMEQBLKI-AIRLBKTGSA-O[(3S)-3-amino-3-carboxypropyl]({[(2S,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl})methylsulfanium399.445399.145063566-2.565SAMe11FDB022473(3s)-5'-[(3-amino-3-carboxypropyl)methylsulfonio]-5'-deoxyadenosine;2-s-adenosyl-l-methionine;5'-deoxyadenosine-5'-l-methionine disulfate ditosylate;Active methionine;Ademetionine;Adenosylmethionine;Adomet;Donamet;L-s-adenosylmethionine;S-(5'-adenosyl)-l-methionine;S-(5'-deoxyadenosin-5'-yl)-l-methionine;S-adenosyl methionine;S-adenosyl-l-methionine disulfate tosylate;S-adenosyl-l-methionine;S-adenosyl-methionine;S-adenosylmethionine;5'-deoxyadenosine-5'-l-methionine disulphate ditosylate;S-adenosyl-l-methionine disulphate tosylate;(3s)-5'-[(3-amino-3-carboxypropyl)methylsulfonio]-5'-deoxyadenosine, inner salt;[1-(adenin-9-yl)-1,5-dideoxy-beta-d-ribofuranos-5-yl][(3s)-3-amino-3-carboxypropyl](methyl)sulfonium;Acylcarnitine;Sam;SamePW_C000921SAMe519863330704201220318802720662468110502350560413571361637540210754421376321608266151923519511874198120312221235822515293249153451815363309768972937689916476984224774881117773133877772341780991327830335178335346791551127996136180861229483038294833386113286389113288397115543399115546401120393122120537413120939407121052124122282435123171449123505119123616118124836470125859297125879481126304299126447499127321205127340206127595388128017517749S-AdenosylhomocysteineHMDB0000939S-Adenosyl-L-homocysteine (SAH) is formed by the demethylation of S-adenosyl-L-methionine. S-Adenosylhomocysteine (AdoHcy or SAH) is also the immediate precursor of all of the homocysteine produced in the body. The reaction is catalyzed by S-adenosylhomocysteine hydrolase and is reversible with the equilibrium favoring formation of SAH. In vivo, the reaction is driven in the direction of homocysteine formation by the action of the enzyme adenosine deaminase which converts the second product of the S-adenosylhomocysteine hydrolase reaction, adenosine, to inosine. Except for methyl transfer from betaine and from methylcobalamin in the methionine synthase reaction, SAH is the product of all methylation reactions that involve S-adenosylmethionine (SAM) as the methyl donor. Methylation is significant in epigenetic regulation of protein expression via DNA and histone methylation. The inhibition of these SAM-mediated processes by SAH is a proven mechanism for metabolic alteration. Because the conversion of SAH to homocysteine is reversible, with the equilibrium favoring the formation of SAH, increases in plasma homocysteine are accompanied by an elevation of SAH in most cases. Disturbances in the transmethylation pathway indicated by abnormal SAH, SAM, or their ratio have been reported in many neurodegenerative diseases, such as dementia, depression, and Parkinson's disease (PMID: 18065573, 17892439). Therefore, when present in sufficiently high levels, S-adenosylhomocysteine can act as an immunotoxin and a metabotoxin. An immunotoxin disrupts, limits the function, or destroys immune cells. A metabotoxin is an endogenous metabolite that causes adverse health effects at chronically high levels. Chronically high levels of S-adenosylhomocysteine are associated with S-adenosylhomocysteine (SAH) hydrolase deficiency and adenosine deaminase deficiency. S-Adenosylhomocysteine forms when there are elevated levels of homocysteine and adenosine. S-Adenosyl-L-homocysteine is a potent inhibitor of S-adenosyl-L-methionine-dependent methylation reactions. It is toxic to immature lymphocytes and can lead to immunosuppression (PMID: 221926).979-92-0C000212524622216680ADENOSYL-HOMO-CYS388301N[C@@H](CCSC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2N)C(O)=OC14H20N6O5SInChI=1S/C14H20N6O5S/c15-6(14(23)24)1-2-26-3-7-9(21)10(22)13(25-7)20-5-19-8-11(16)17-4-18-12(8)20/h4-7,9-10,13,21-22H,1-3,15H2,(H,23,24)(H2,16,17,18)/t6-,7+,9+,10+,13+/m0/s1ZJUKTBDSGOFHSH-WFMPWKQPSA-N(2S)-2-amino-4-({[(2S,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl}sulfanyl)butanoic acid384.411384.12158847-1.975S-adenosyl-L-homocysteine00DBMET00514FDB022327(s)-5'-(s)-(3-amino-3-carboxypropyl)-5'-thioadenosine;2-s-adenosyl-l-homocysteine;5'-deoxy-s-adenosyl-l-homocysteine;5'-s-(3-amino-3-carboxypropyl)-5'-thio-l-adenosine;Adenosyl-l-homocysteine;Adenosyl-homo-cys;Adenosylhomo-cys;Adenosylhomocysteine;Adohcy;Formycinylhomocysteine;L-5'-s-(3-amino-3-carboxypropyl)-5'-thior-adenosine;L-s-adenosyl-homocysteine;L-s-adenosylhomocysteine;S-(5'-adenosyl)-l-homocysteine;S-(5'-deoxyadenosin-5'-yl)-l-homocysteine;S-(5'-deoxyadenosine-5')-l-homocysteine;S-adenosyl-l-homocysteine;S-adenosyl-homocysteine;Sah;(2s)-2-amino-4-({[(2s,3s,4r,5r)-5-(6-amino-9h-purin-9-yl)-3,4-dihydroxytetrahydrofuran-2-yl]methyl}sulfanyl)butanoic acid;S-[1-(adenin-9-yl)-1,5-dideoxy-beta-d-ribofuranos-5-yl]-l-homocysteine;S-adenosylhomocysteinePW_C000749SAH520857518635307052012213188227206724683105025505607136713716375422107546213763416082681519237195118751981235922515294249153643097748911177611130777333387777334178098132783053517833734679156112799623618086322948313829483438611328738911328939711554439911554740112039412212048612512053941312094040712105312412228443512303713512317344912350611912361711812483847012588048112630329912644949912734120612759638812801951748EpinephrineHMDB0000068Epinephrine is a catecholamine, a sympathomimetic monoamine derived from the amino acids phenylalanine and tyrosine. It is the active sympathomimetic hormone secreted from the adrenal medulla in most species. It stimulates both the alpha- and beta- adrenergic systems, causes systemic vasoconstriction and gastrointestinal relaxation, stimulates the heart, and dilates bronchi and cerebral vessels. It is used in asthma and cardiac failure and to delay absorption of local anesthetics. Epinephrine also constricts arterioles in the skin and gut while dilating arterioles in leg muscles. It elevates the blood sugar level by increasing hydrolysis of glycogen to glucose in the liver, and at the same time begins the breakdown of lipids in adipocytes. Epinephrine has a suppressive effect on the immune system.51-43-4C00788581628918L-EPINEPHRINE5611DB00668CNC[C@H](O)C1=CC(O)=C(O)C=C1C9H13NO3InChI=1S/C9H13NO3/c1-10-5-9(13)6-2-3-7(11)8(12)4-6/h2-4,9-13H,5H2,1H3/t9-/m0/s1UCTWMZQNUQWSLP-VIFPVBQESA-N4-[(1R)-1-hydroxy-2-(methylamino)ethyl]benzene-1,2-diol183.2044183.089543287-0.994epinephrine01FDB021889(-)-(r)-epinephrine;(-)-3,4-dihydroxy-a-[2-(methylamino)ethyl]benzyl alcohol;(-)-3,4-dihydroxy-alpha-[2-(methylamino)ethyl]benzyl alcohol;(-)-3,4-dihydroxy-a-[(methylamino)methyl]-benzyl alcohol;(-)-3,4-dihydroxy-alpha-[(methylamino)methyl]-benzyl alcohol;(-)-adrenaline;(-)-epinephrine;(r)-4-[1-hydroxy-2-(methylamino)ethyl]-1,2-benzenediol;(r)-adrenaline;(r)-epinephrine;4-[(1r)-1-hydroxy-2-(methylamino)ethyl]-1,2-benzenediol;Adnephrine;Adrenal;Adrenalin;Adrenaline;Adrenine;Adrin;Ana-kit;Bosmin;Bronkaid mist;Chelafrin;Epifrin;Epiglaufrin;Epinefrina;Epinephran;Epinephrine;Epipen;Epirenan;Eppy;Exadrin;Glauposine;Hemisine;Hemostasin;Hemostatin;Hypernephrin;Isoptoepinal;L-1-(3,4-dihydroxyphenyl)-2-methylaminoethanol;L-adrenaline;L-epinephrine;L-epirenamine;L-methylaminoethanolcatechol;Levoepinephrine;Levorenen;Levorenin;Levorenine;Levoreninum;Lyodrin;Methylarterenol;Mucidrina;Nephridine;Nieraline;Paranephrin;Primatene mist;R-(-)-epinephrine;Renaglandin;Renaleptine;Renalina;Renoform;Renostypticin;Renostyptin;Scurenaline;Simplene;Styptirenal;Supracapsulin;Supranephrane;Suprarenaline;Suprarenin;Surrenine;Sus-phrine;Takamina;Vasoconstrictine;Vasotonin;(-)-3,4-dihydroxy-alpha-((methylamino)methyl)benzyl alcohol;(r)-(-)-adrenaline;(r)-(-)-adnephrine;(r)-(-)-epinephrine;(r)-(-)-epirenamine;Epinephrin;Epinephrinum;Epipen jr;Primatene;(-)-3,4-dihydroxy-a-((methylamino)methyl)benzyl alcohol;(-)-3,4-dihydroxy-α-((methylamino)methyl)benzyl alcoholPW_C000048Eppy7062074914108715108841116945206824339146356346368750846577774341777763427777734079129132828075182838381121626124124184118127597388267Tyrosine 3-monooxygenaseP07101Plays an important role in the physiology of adrenergic neurons.
HMDBP00273TH11p15.5M2478711.14.16.23991969320135585285139399331396947891405488714127048143224391441732272Aromatic-L-amino-acid decarboxylaseP20711Catalyzes the decarboxylation of L-3,4-dihydroxyphenylalanine (DOPA) to dopamine, L-5-hydroxytryptophan to serotonin and L-tryptophan to tryptamine.
HMDBP00278DDC7p12.2M8458814.1.1.2840219697202009213558659913939687139448481396957891405493314055086514130577143183391433632614396860247Dopamine beta-hydroxylaseP09172Conversion of dopamine to noradrenaline.
HMDBP00253DBH9q34X1325511.14.17.140919703202024141394008413940247613940337813969678914127186614262421426254814289439142897106014336626398Phenylethanolamine N-methyltransferaseP11086Converts noradrenaline to adrenaline.
HMDBP00406PNMT17qBC03724612.1.1.287072020692139401871433682693Tyrosine 3-monooxygenase1PW_P000093108267148544196Aromatic-L-amino-acid decarboxylase1PW_P000096111272491148198Dopamine beta-hydroxylase1PW_P0000981132475085251515142201Phenylethanolamine N-methyltransferase1PW_P0002012193981707falsePW_R000707Right28921031Compoundtrue28936271Compoundfalse289410651Compoundtrue28951171Compoundfalse2896271Compoundtrue289714201Compoundtrue213931.14.16.2224falsePW_R000224Right9721171Compoundfalse973521Compoundfalse97413161Compoundtrue214964.1.1.28634falsePW_R000634Right2625521Compoundfalse4347311Compoundtrue434810651Compoundtrue26261421Compoundfalse43499771Compoundtrue435014201Compoundtrue118981.14.17.1466falsePW_R000466Right19359211Compoundtrue19361421Compoundfalse19377491Compoundtrue1938481Compoundfalse2182012.1.1.281559PW_T0015591802481Compound51381Both14620PW_T014620Diffusion15064481Compound2051Right10041032081false860199510regular20019010056272081false1015221010regular200190100610652065false1086192110regular787810071172081false1520199710regular2001901008272081false1325221210regular200190100914202049false1370191710regular78781010544209false1220211010regular100251011522081false2175199510regular200190101213162052false2021194110regular787810131148209false1870212210regular100351014312081false2375185210regular200190101510652065false2121189810regular787810161422081false2175145910regular20019010179772081false2380159410regular200190101814202049false2120165410regular787810198525209false2230183219regular100351020514209false2230176719regular1002510219212081false2380133510regular20019010227492081false2380111410regular2001901023482081false217597910regular2001901611017485181false217052910regular20019016114714838181false56030910regular200190309267202false119520558subunitregular15070310272206false184020528subunitregular16080311247208false220517678subunitregular14085312398202false220012898subunitregular1507028493232030930915010101364Cofactor28596232031031015110131368Cofactor28698232031131115210191375Cofactor15310201376Cofactor28720123203123121358M1060 2090 C1090 2090 1165 2090 1195 2090 5false181359M1115 2210 C1115 2173 1165 2090 1195 2090 5false181360M1125 1999 C1125 2031 1165 2090 1195 2090 5false181361M1520 2092 C1490 2092 1375 2090 1345 2090 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1362M1425 2212 C1425 2162 1375 2090 1345 2090 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1363M1409 1995 C1409 2030 1375 2090 1345 2090 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1364M865 85 L865 135 L915 85 z10true181365M1720 2092 C1750 2092 1810 2092 1840 2092 5false181366M2175 2090 C2145 2090 2030 2092 2000 2092 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1367M2060 2019 C2060 2050 2030 2092 2000 2092 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1368M550 150 L550 200 L600 150 z10true181369M2275 1995 C2275 1960 2277 1882 2275 1852 5false181370M2375 1947 C2322 1944 2277 1887 2275 1852 5false181371M2199 1937 C2232 1940 2280 1900 2275 1852 5false181372M2275 1649 C2277 1698 2276 1741 2275 1767 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1373M2380 1689 C2342 1688 2275 1732 2275 1767 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1374M2198 1693 C2241 1695 2275 1738 2275 1767 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1375M550 150 L550 200 L600 150 z10true181376M550 150 L550 200 L600 150 z10true181377M2380 1430 C2327 1430 2276 1403 2275 1359 5false181378M2275 1459 C2275 1430 2276 1396 2275 1359 5false181379M2380 1209 C2337 1208 2274 1255 2275 1289 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false1380M2275 1169 C2274 1198 2275 1257 2275 1289 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2199150M2170 624 C2140 624 1845 613 1815 613 83true18falsefalse2199151M660 499 C659 535 660 565 659 610 C744 610 2092 624 2170 624 83false18trueM 362.94685504416486 162.26155629629605 L 348 161 L 354.38088772118584 174.57513432307834falsefalsefalse2251496M760 404 C825 381 1092 293 1165 269 149false18trueM 1155.0018499947635 280.181994297655 L 1165 269 L 1150.3170338706398 265.93234525344525false2251634M760 404 C790 404 1195 404 1225 404 148false18falsetrueM 1140 439 L 1140 424 L 1140 4094338540M2275 979 C2275 949 2270 879 2270 849 83false184338541M2270 719 C2270 749 2270 819 2270 849 83false18trueM 1191.9468550441647 372.261556296296 L 1177 371 L 1183.380887721186 384.5751343230783false3002370720108610041358Left108710051359Left108810061360Left108910071361Right109010081362Right109110091363Right2822132843012322420109210071365Left109310111366Right109410121367Right2832142853022363420109510111369Left109610141370Left109710151371Left109810161372Right109910171373Right110010181374Right2842172863032346620110110211377Left110210161378Left110310221379Right110410231380Right285218287242601559234912716110172199150Left4912816114712199151Right105807146202321286010234338540Left21286116110174338541Right6032679623217false116523416regular5732516114712251496Left6033379723217false122536916regular5733216114712251634Left59711559951.61.60282924007768990951.91.902165004007777418404401.61.6021158025018719711129871.81.8029048449753M726 1017 C726 967 776 917 826 917 C1387 917 2116 917 2677 917 C2727 917 2777 967 2777 1017 C2777 1443 2777 1997 2777 2423 C2777 2473 2727 2523 2677 2523 C2116 2523 1387 2523 826 2523 C776 2523 726 2473 726 2423 C726 1997 726 1443 726 1017 1true62051.01606.056235Adrenal Medulla Cell950865201.31.32001557235Kidney1901360201.31.32001558235Adrenal Medulla270960201.31.320015686235Activation890285201.01.020015687235Inhibition895360201.01.020015688235Nervous System445185201.31.320015694235Bloodstream2140420201.31.3200151644911176968932804255039421081657Aromatic L-Aminoacid Decarboxylase DeficiencyAromatic L-Aminoacid Decarboxylase Deficiency (DOPA decarboxylase; DDC) is an autosomal recessive disease caused by a mutation in the DDC gene which codes for aromatic-L-aminoacid decarboxylase. A deficiency in this enzyme results in accumulation of 3-methoxytyrosine, 5-hydroxy-L-tryptophan, and L-Dopa in plasma, spinal fluid, and urine; 3-methoxytyramine and dopamine in urine. It also results in decreased concentrations of homovanillic acid, S-adenosylmethionine, and 5-hydroxytryptophol in spinal fluid; and epinephrine, norepinephrine in plasma. Symptoms include temperature instability, hypotonia, mental and motor retardation, and cerebral atrophy.DiseasePW_X000048Context48249272ProteinMutated250117CompoundIncreased26052CompoundDecreased261142CompoundDecreased26248CompoundDecreased70124TissueDamaged70214TissueDamaged482Engelke, U., van der Graaf, M., Heerschap, A., Hoenderop, S., Moolenaar, S., Morava, E., Wevers, R. Handbook of 1H-NMR spectroscopy in inborn errors of metabolism: body fluid NMR spectroscopy and in vivo MR spectroscopy (2nd ed) (2007) p.22 Heilbronn: SPS Verlagsgesellschaft48Context483[Uniprot: P20711](http://www.uniprot.org/uniprot/P20711)48Context484[OMIM: Entry 608643](http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=608643)48Context48517240182Verbeek MM, Geurtz PB, Willemsen MA, Wevers RA: Aromatic L-amino acid decarboxylase enzyme activity in deficient patients and heterozygotes. Mol Genet Metab. 2007 Apr;90(4):363-9. doi: 10.1016/j.ymgme.2006.12.001. Epub 2007 Jan 19.48Context48612891654Swoboda KJ, Saul JP, McKenna CE, Speller NB, Hyland K: Aromatic L-amino acid decarboxylase deficiency: overview of clinical features and outcomes. Ann Neurol. 2003;54 Suppl 6:S49-55. doi: 10.1002/ana.10631.48Context48715079002Pons R, Ford B, Chiriboga CA, Clayton PT, Hinton V, Hyland K, Sharma R, De Vivo DC: Aromatic L-amino acid decarboxylase deficiency: clinical features, treatment, and prognosis. Neurology. 2004 Apr 13;62(7):1058-65.48Context27831720505134Brun L, Ngu LH, Keng WT, Ch'ng GS, Choy YS, Hwu WL, Lee WT, Willemsen MA, Verbeek MM, Wassenberg T, Regal L, Orcesi S, Tonduti D, Accorsi P, Testard H, Abdenur JE, Tay S, Allen GF, Heales S, Kern I, Kato M, Burlina A, Manegold C, Hoffmann GF, Blau N: Clinical and biochemical features of aromatic L-amino acid decarboxylase deficiency. Neurology. 2010 Jul 6;75(1):64-71. doi: 10.1212/WNL.0b013e3181e620ae. Epub 2010 May 26.48Context27937223275025Lee NC, Shieh YD, Chien YH, Tzen KY, Yu IS, Chen PW, Hu MH, Hu MK, Muramatsu S, Ichinose H, Hwu WL: Regulation of the dopaminergic system in a murine model of aromatic L-amino acid decarboxylase deficiency. Neurobiol Dis. 2013 Apr;52:177-90. doi: 10.1016/j.nbd.2012.12.005. Epub 2012 Dec 26.48Context