36ContextAcute Intermittent PorphyriaAcute intermittent porphyria (AIP), also called Swedish porphyria, is a rare inborn error of metabolism (IEM) and autosomal dominant disorder of heme biosynthesis caused by a defective HMBS gene. The HMBS gene codes for the protein hydroxymethylbilane synthase (porphobilinogen deaminase) which catalyzes the synthesis of porphobilinogen into hydroxymethylbilane. This disorder is characterized by a large accumulation of 5-aminolevulinic acid or porphobilinogen in both urine and serum. Most patients are asymptomatic between attacks. Symptoms of the disorder include abdominal pain, constipation, vomiting, hypertension, muscle weakness, seizures, delirium, coma, and depression. Treatment involves undertaking a high-carbohydrate diet and, during severe attacks, a glucose 10% infusion. It is estimated that AIP affects 5.9 per 1 000 000 people.DiseasePW000174CenterPathwayVisualizationContext18948504750#000099PathwayVisualization10396Porphyrin MetabolismThis pathway depicts the synthesis and breakdown of porphyrin. The porphyrin ring is the framework for the heme molecule, the pigment in hemoglobin and red blood cells. The first reaction in porphyrin ring biosynthesis takes place in the mitochondria and involves the condensation of glycine and succinyl-CoA by delta-aminolevulinic acid synthase (ALAS). Delta-aminolevulinic acid (ALA) is also called 5-aminolevulinic acid. Following its synthesis, ALA is transported into the cytosol, where ALA dehydratase (also called porphobilinogen synthase) dimerizes 2 molecules of ALA to produce porphobilinogen. The next step in the pathway involves the condensation of 4 molecules of porphobilinogen to produce hydroxymethylbilane (also known as HMB). The enzyme that catalyzes this condensation is known as porphobilinogen deaminase (PBG deaminase). This enzyme is also called hydroxymethylbilane synthase or uroporphyrinogen I synthase. Hydroxymethylbilane (HMB) has two main fates. Most frequently it is enzymatically converted into uroporphyrinogen III, the next intermediate on the path to heme. This step is mediated by two enzymes: uroporphyrinogen synthase and uroporphyrinogen III cosynthase. Hydroxymethylbilane can also be non-enzymatically cyclized to form uroporphyrinogen I. In the cytosol, the uroporphyrinogens (uroporphyrinogen III or uroporphyrinogen I) are decarboxylated by the enzyme uroporphyrinogen decarboxylase. These new products have methyl groups in place of the original acetate groups and are known as coproporphyrinogens. Coproporphyrinogen III is the most important intermediate in heme synthesis. Coproporphyrinogen III is transported back from the cytosol into the interior of the mitochondria, where two propionate residues are decarboxylated (via coproporphyrinogen-III oxidase), which results in vinyl substituents on the 2 pyrrole rings. The resulting product is called protoporphyrinogen IX. The protoporphyrinogen IX is then converted into protoporphyrin IX by another enzyme called protoporphyrinogen IX oxidase. The final reaction in heme synthesis also takes place within the mitochondria and involves the insertion of the iron atom into the ring system generating the molecule known heme b. The enzyme catalyzing this reaction is known as ferrochelatase. The largest repository of heme in the body is in red blood cells (RBCs). RBCs have a life span of about 120 days. When the RBCs have reached the end of their useful lifespan, the cells are engulfed by macrophages and their constituents recycled or disposed of. Heme is broken down when the heme ring is opened by the enzyme known as heme oxygenase, which is found in the endoplasmic reticulum of the macrophages. The oxidation process produces the linear tetrapyrrole biliverdin, ferric iron (Fe3+), and carbon monoxide (CO). The carbon monoxide (which is toxic) is eventually discharged through the lungs. In the next reaction, a second methylene group (located between rings III and IV of the porphyrin ring) is reduced by the enzyme known as biliverdin reductase, producing bilirubin. Bilirubin is significantly less extensively conjugated than biliverdin. This reduction causes a change in the colour of the molecule from blue-green (biliverdin) to yellow-red (bilirubin). In hepatocytes, bilirubin-UDP-glucuronyltransferase (bilirubin-UGT) adds two additional glucuronic acid molecules to bilirubin to produce the more water-soluble version of the molecule known as bilirubin diglucuronide. In most individuals, intestinal bilirubin is acted on by the gut bacteria to produce the final porphyrin products, urobilinogens and stercobilins. These are excreted in the feces. The stercobilins oxidize to form brownish pigments which lead to the characteristic brown colour found in normal feces. Some of the urobilinogen produced by the gut bacteria is reabsorbed and re-enters the circulation. These urobilinogens are converted into urobilins that are then excreted in the urine which cause the yellowish colour in urine.Metabolic163998SubPathway107178Compound264Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.96Pathway65Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.96Pathway1CellCL:00000003NeuronCL:00005406MyocyteCL:00001875HepatocyteCL:00001824CardiomyocyteCL:00007467Epithelial CellCL:00000662Platelet CL:00002331Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote17Rattus norvegicus10116EukaryoteRat12Mus musculus10090EukaryoteMouse24Solanum lycopersicum4081EukaryoteTomato18Saccharomyces cerevisiae4932EukaryoteYeast4Arabidopsis thaliana3702EukaryoteThale cress23Pseudomonas aeruginosa287Prokaryote5Bos taurus9913EukaryoteCattle10Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm21Xenopus laevis8355EukaryoteAfrican clawed frog60Nitzschia sp.0001EukaryoteNitzschia42Bacteria2ProkaryoteBacteria49Bathymodiolus platifrons220390EukaryoteDeep sea mussel19Schizosaccharomyces pombe4896Eukaryote25Escherichia coli (strain K12)83333Prokaryote3Mitochondrial MatrixGO:00057595CytoplasmGO:00057371CytosolGO:000582915NucleusGO:000563431Periplasmic SpaceGO:000562011Extracellular SpaceGO:00056152MitochondrionGO:000573913Endoplasmic ReticulumGO:00057834PeroxisomeGO:000577725Golgi ApparatusGO:00057947Endoplasmic Reticulum MembraneGO:000578912Mitochondrial Inner MembraneGO:000574335ChloroplastGO:000950724Mitochondrial Intermembrane SpaceGO:000575810Cell MembraneGO:00058866LysosomeGO:000576416Lysosomal LumenGO:004320218Melanosome MembraneGO:003316214Mitochondrial Outer MembraneGO:000574120Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520236MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032540PeriplasmGO:004259732Inner MembraneGO:007025839Mitochondrial membraneGO:00319668Smooth Endoplasmic Reticulum GO:00057901LiverBTO:00007597294Adrenal MedullaBTO:000004971828StomachBTO:0001307155263Sympathetic Nervous SystemBTO:00018329MuscleBTO:00008871411824BrainBTO:0000142891625IntestineBTO:00006487Nervous SystemBTO:00014848Blood VesselBTO:0001102741111HeartBTO:000056273106KidneyBTO:00006717182Endothelium 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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_C000078Gly314179818122188127282929542010354541205580133564010756411085863105600714770141607439374411667442151179419811872161124291511523322242419318424203157764433677742111780221327830435180708135120028406120097122120117124121687429122283435122850118124236464124837470125406479125466297125484299126448499126946501127003205127021388128018517808Succinyl-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 acid867.607867.131252359-2.19104-({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-Amnva3459336702701616078102112785771321220151241221484071245691181247001191261652991263074811278692061316Carbon 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'P18232445351812214011969620111042145050145826212010215049532511154161175421103544111854551205567132558113365338570181607167205721621272222131185816112175151126233112628181268428912689290770172537703722577041293770522247752611277764341779733467797932778292345788553327886233180696135986307119912122120024124120029406120087407120817418121149423121155424122069123122076383122834119123402454123721458123727459124620447124627398125302297125402299125407479125458481125803489126224298126231495126942388126947501126996206127258506127786513127793390166PorphobilinogenHMDB0000245Porphobilinogen (PBG) is a pyrrole-containing intermediate in the biosynthesis of porphyrins. It is generated from aminolevulinate (ALA) by the enzyme ALA dehydratase. Porphobilinogen is then converted into hydroxymethylbilane by the enzyme porphobilinogen deaminase (also known as hydroxymethylbilane synthase). Under certain conditions, porphobilinogen can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, and hereditary coproporphyria (HCP). There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).487-90-1C00931102117381PORPHOBILINOGEN995NCC1=C(CC(O)=O)C(CCC(O)=O)=CN1C10H14N2O4InChI=1S/C10H14N2O4/c11-4-8-7(3-10(15)16)6(5-12-8)1-2-9(13)14/h5,12H,1-4,11H2,(H,13,14)(H,15,16)QSHWIQZFGQKFMA-UHFFFAOYSA-N3-[5-(aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid226.2292226.095356946-1.924porphobilinogen0-1FDB0219165-(aminomethyl)-4-(carboxymethyl)-pyrrole-3-propionate;5-(aminomethyl)-4-(carboxymethyl)-pyrrole-3-propionic acid;Pbg;PorphobilinogenPW_C000166PBG367327019160785791321220171241245711181261672991420WaterHMDB0002111Water 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_C001420H2O55894910951394151316214481135261562428652106912077033823188382109431137749146554159043201824253222267860272746277817280529314370316472363461459836472737494193503027515675195975214100522794523610352971055319111534311353551125402110547012354831255492126550712755341305537114554112955911355608118562210856916575914057781015841143585314658771075890955910147594015160321556059157608716161231636133159621516218166647717865071806600152671311768401886888160716220571812077193206721121172282137238214724321572951987350216738821074012127467222749222475001907588170820122582372268414162926526118502771192216412011281122132851225028612264287123272491252022712632651269329012705291127152921300729813019300130253011303730213261223133272941534030842327315426953184369132276914293770192537710213277131133772151347737833177397332774713337751611577536334776283367772233777759341778163437798234778071329782353527824235378270356791133608001436880039370805912288065611993830383947943841105573901106393911158443981198792321199151221199634061200084071200464081201131241203654121204304051204384091206064151207944141211584251212404291213511211213814191216074341221183821223844361227531201227973741228044431230124461230643761230721371231314471231421361231624481232314511233844501237304601238104641239404551241654691246703991249384711249454721253052971253534791253864811254244821254802991256824831257074781257454871260544901262384951262734841267644801268965011269635021270173881271772081271992091272275041275065071275765151278363891280823951281765131406747901406758341407551859795ZincHMDB0015532Zinc is an essential element, necessary for sustaining all life. It is a trace element in the diet, forming an essential part of many enzymes, and playing an important role in protein synthesis and in cell division. Physiologically, it exists as an ion in the body. It is estimated that 3000 of the hundreds of thousands of proteins in the human body contain zinc prosthetic groups. In addition, there are over a dozen cell types in the human body that secrete zinc ions, and the roles of these secreted zinc signals in medicine and health are now being actively studied. Intriguingly, brain cells in the mammalian forebrain are one type of cell that secretes zinc, along with its other neuronal messenger substances. Cells in the salivary gland, prostate, immune system, and intestine are other types that secrete zinc. Obtaining a sufficient zinc intake during pregnancy and in young children is a problem, especially among those who cannot afford a good and varied diet. Zinc deficiency is associated with anemia, short stature, hypogonadism, impaired wound healing, and geophagia. Brain development is stunted by zinc deficiency in utero and in youth. Zinc is an activator of certain enzymes, such as carbonic anhydrase. Carbonic anhydrase is important in the transport of carbon dioxide in vertebrate blood. Even though zinc is an essential requirement for a healthy body, too much zinc can be harmful. Excessive absorption of zinc can also suppress copper and iron absorption. The free zinc ion in solution is highly toxic to plants, invertebrates, and even vertebrate fish. The Free Ion Activity Model (FIAM) is well-established in the literature and shows that just micromolar amounts of the free ion kill some organisms.7440-66-6239942736322430DB01593[Zn++]ZnInChI=1S/Zn/q+2PTFCDOFLOPIGGS-UHFFFAOYSA-Nzinc(2+) ion65.40963.9291465780zinc(2+) ion2230zn;Cinc;Zincum;Zink;Zn;Zn(ii);Zn2+PW_C009795Zinc578171121904321371721544936102940837446918454314499931668910766901016699108702016011758115122291511263365423973154239931877030253780231327832811278811111120119124120898122122308407122852118123469135124860119125486299126474481127023388127317205128043206887HydroxymethylbilaneHMDB0001137Hydroxymethylbilane is a molecule involved in the metabolism of porphyrin. In the third step, it is generated by the enzyme porphobilinogen deaminase , and in the next step the enzyme uroporphyrinogen III synthase converts it into uroporphyrinogen III. -- Wikipedia.73023-76-4C0102478816645HYDROXYMETHYLBILANE767OCC1=C(CC(O)=O)C(CCC(O)=O)=C(CC2=C(CC(O)=O)C(CCC(O)=O)=C(CC3=C(CC(O)=O)C(CCC(O)=O)=C(CC4=C(CC(O)=O)C(CCC(O)=O)=CN4)N3)N2)N1C40H46N4O17InChI=1S/C40H46N4O17/c45-17-32-25(12-40(60)61)21(4-8-36(52)53)29(44-32)15-31-24(11-39(58)59)20(3-7-35(50)51)28(43-31)14-30-23(10-38(56)57)19(2-6-34(48)49)27(42-30)13-26-22(9-37(54)55)18(16-41-26)1-5-33(46)47/h16,41-45H,1-15,17H2,(H,46,47)(H,48,49)(H,50,51)(H,52,53)(H,54,55)(H,56,57)(H,58,59)(H,60,61)WDFJYRZCZIUBPR-UHFFFAOYSA-N3-(5-{[3-(2-carboxyethyl)-4-(carboxymethyl)-5-(hydroxymethyl)-1H-pyrrol-2-yl]methyl}-2-{[4-(2-carboxyethyl)-5-{[4-(2-carboxyethyl)-3-(carboxymethyl)-1H-pyrrol-2-yl]methyl}-3-(carboxymethyl)-1H-pyrrol-2-yl]methyl}-4-(carboxymethyl)-1H-pyrrol-3-yl)propanoic acid854.8098854.285796066-4.42133-(5-{[3-(2-carboxyethyl)-4-(carboxymethyl)-5-(hydroxymethyl)-1H-pyrrol-2-yl]methyl}-2-{[4-(2-carboxyethyl)-5-{[4-(2-carboxyethyl)-3-(carboxymethyl)-1H-pyrrol-2-yl]methyl}-3-(carboxymethyl)-1H-pyrrol-2-yl]methyl}-4-(carboxymethyl)-1H-pyrrol-3-yl)propanoic acid0-8FDB0224463,8,13,18-tetrakis(carboxymethyl)-19-(hydroxymethyl)bilane-2,7,12,17-tetrapropanoate;3,8,13,18-tetrakis(carboxymethyl)-19-(hydroxymethyl)bilane-2,7,12,17-tetrapropanoic acid;3,8,13,18-tetrakis(carboxymethyl)-5,10,15,22,23,24-hexahydro-19-(hydroxymethyl)-21h-biline-2,7,12,17-tetrapropanoate;3,8,13,18-tetrakis(carboxymethyl)-5,10,15,22,23,24-hexahydro-19-(hydroxymethyl)-21h-biline-2,7,12,17-tetrapropanoic acid;3-[2-[[4-(2-carboxyethyl)-5-[[4-(2-carboxyethyl)-5-[[4-(2-carboxyethyl)-3-(carboxymethyl)-1h-pyrrol-2-yl]methyl]-3-(carboxymethyl)-1h-pyrrol-2-yl]methyl]-3-(carboxymethyl)-1h-pyrrol-2-yl]methyl]-4-(carboxymethyl)-5-(hydroxymethyl)-1h-pyrrol-3-yl]propanoate;3-[2-[[4-(2-carboxyethyl)-5-[[4-(2-carboxyethyl)-5-[[4-(2-carboxyethyl)-3-(carboxymethyl)-1h-pyrrol-2-yl]methyl]-3-(carboxymethyl)-1h-pyrrol-2-yl]methyl]-3-(carboxymethyl)-1h-pyrrol-2-yl]methyl]-4-(carboxymethyl)-5-(hydroxymethyl)-1h-pyrrol-3-yl]propanoic acid;Hydroxymethylbilane;Preuroporphyrinogen;(hydroxymethyl)bilanePW_C000887HMBilan3677270211607858113212201912412457311812616929935AmmoniaHMDB0000051Ammonia 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_C000035NH39791125133814244382479135501414685425332225723533811160161477022160717720511786198118482771188521512708291127182927696622577046294773291337734313277469333774991137753933477597115779853477799311278072329792442938065013580657119116203109119921122120049408120053126120136407120343406120363412120462405121046124121161425122119382122800374122805443122993120123010446123096376123610118123733460124671399125311297125427482125431301125502481125663479125708478126102299126274484126966502126970207127039206127158501127200209127600388127837389854Uroporphyrinogen IIIHMDB0001086Uroporphyrinogens are porphyrinogen variants in which each pyrrole ring has one acetate side chain and one propionate side chain; it is formed by condensation 4 four molecules of porphobilinogen. 4 isomers are possible but only 2 commoly are found, types I and III. Uroporphyrinogen III is a functional intermediate in heme biosynthesis while Uroporphyrinogen I is produced in an abortive side reaction.1976-85-8C01051117915437UROPORPHYRINOGEN-III1146OC(=O)CCC1=C2CC3=C(CCC(O)=O)C(CC(O)=O)=C(CC4=C(CC(O)=O)C(CCC(O)=O)=C(CC5=C(CC(O)=O)C(CCC(O)=O)=C(CC(N2)=C1CC(O)=O)N5)N4)N3C40H44N4O16InChI=1S/C40H44N4O16/c45-33(46)5-1-17-21(9-37(53)54)29-14-27-19(3-7-35(49)50)22(10-38(55)56)30(43-27)15-28-20(4-8-36(51)52)24(12-40(59)60)32(44-28)16-31-23(11-39(57)58)18(2-6-34(47)48)26(42-31)13-25(17)41-29/h41-44H,1-16H2,(H,45,46)(H,47,48)(H,49,50)(H,51,52)(H,53,54)(H,55,56)(H,57,58)(H,59,60)HUHWZXWWOFSFKF-UHFFFAOYSA-N3-[9,14,20-tris(2-carboxyethyl)-5,10,15,19-tetrakis(carboxymethyl)-21,22,23,24-tetraazapentacyclo[16.2.1.1³,⁶.1⁸,¹¹.1¹³,¹⁶]tetracosa-1(20),3,5,8,10,13,15,18-octaen-4-yl]propanoic acid836.7946836.27523138-4.2812uroporphyrinogen-III0-8FDB0224173,8,13,17-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydro-2,7,12,18-porphinetetrapropionate;3,8,13,17-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydro-2,7,12,18-porphinetetrapropionic acid;3,8,13,17-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydro-21h,23h-porphine-2,7,12,18-tetrapropanoate;3,8,13,17-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydro-21h,23h-porphine-2,7,12,18-tetrapropanoic acid;3,8,13,17-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropanoate;3,8,13,17-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropanoic acid;Urogen iii;Uroporphyrinogen iii;Uroporphyrinogen-iii;3,8,13,17-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropionic acid;Uro'gen iii;3,8,13,17-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropionatePW_C000854Urppr336792702316078583132122021124124575118126171299732Uroporphyrin IIIHMDB0000916Uroporphyrin is the porphyrin produced by oxidation of the methylene bridges in uroporphyrinogen. Uroporphyrins have four acetic acid and four propionic acid side chains attached to their pyrrole rings. The enzyme uroporphyrinogen I synthase catalyzes the formation of hydroxymethylbilane from four molecules of porphobilinogen. Uroporphyrinogen III cosynthase then catalyzes the conversion of hydroxymethylbilane into uroporphyrinogen III. Otherwise, hydroxymethylbilane cyclizes nonenzymatically to form uroporphyrinogen I. Uroporphyrinogen I and III yield their respective uroporphyrins via autooxidation or their respective coproporphyrinogens via decarboxylation. Excessive amounts of uroporphyrin I are excreted in congenital erythropoietic porphyria, and both uroporphyrin I and uroporphyrin III are excreted in porphyria cutanea tarda. Uroporphyrin I and III are the most common isomers. Under certain conditions, uroporphyrin III can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, porphyria cutanea tarda, and hereditary coproporphyria (HCP). There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).18273-06-8C0246915436UROPORPHYRIN_III16736727OC(=O)CCC1=C(CC(O)=O)/C2=C/C3=N/C(=C\C4=C(CCC(O)=O)C(CC(O)=O)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(CCC(O)=O)=C4CC(O)=O)/C(CC(O)=O)=C3CCC(O)=OC40H38N4O16InChI=1S/C40H38N4O16/c45-33(46)5-1-17-21(9-37(53)54)29-14-27-19(3-7-35(49)50)22(10-38(55)56)30(43-27)15-28-20(4-8-36(51)52)24(12-40(59)60)32(44-28)16-31-23(11-39(57)58)18(2-6-34(47)48)26(42-31)13-25(17)41-29/h13-16,41,44H,1-12H2,(H,45,46)(H,47,48)(H,49,50)(H,51,52)(H,53,54)(H,55,56)(H,57,58)(H,59,60)/b25-13-,26-13-,27-14-,28-15-,29-14-,30-15-,31-16-,32-16-VZVFNUAIRVUCEW-UJJXFSCMSA-N3-[9,14,20-tris(2-carboxyethyl)-5,10,15,19-tetrakis(carboxymethyl)-21,22,23,24-tetraazapentacyclo[16.2.1.1^{3,6}.1^{8,11}.1^{13,16}]tetracosa-1,3,5,7,9,11(23),12,14,16,18(21),19-undecaen-4-yl]propanoic acid830.7469830.228281188-4.35103-[9,14,20-tris(2-carboxyethyl)-5,10,15,19-tetrakis(carboxymethyl)-21,22,23,24-tetraazapentacyclo[16.2.1.1^{3,6}.1^{8,11}.1^{13,16}]tetracosa-1,3,5,7,9,11(23),12,14,16,18(21),19-undecaen-4-yl]propanoic acid0-8FDB0056643,8,13,17-tetrakis(carboxymethyl)porphyrin-2,7,12,18-tetrapropanoate;3,8,13,17-tetrakis(carboxymethyl)porphyrin-2,7,12,18-tetrapropanoic acid;3,8,13,17-tetramethyl-2,7,12,18-porphinetetrapropionate;3,8,13,17-tetramethyl-2,7,12,18-porphinetetrapropionic acid;Coproporphyrin iii;Uroporphyrin iii;3,8,13,17-tetrakis(carboxymethyl)porphyrin-2,7,12,18-tetrapropionic acid;3,8,13,17-tetrakis(carboxymethyl)porphyrin-2,7,12,18-tetrapropionatePW_C000732Urop3368127024160785851321220231241245771181261732991496Uroporphyrinogen IHMDB0002211Uroporphyrinogens are porphyrinogen variants in which each pyrrole ring has one acetate side chain and one propionate side chain; it is formed by condensation 4 four molecules of porphobilinogen. 4 isomers are possible but only 2 commoly are found, types I and III. Uroporphyrinogen III is a functional intermediate in heme biosynthesis while Uroporphyrinogen I is produced in an abortive side reaction.1867-62-5C0576644077528766UROPORPHYRINOGEN-III389644OC(=O)CCC1=C2CC3=C(CC(O)=O)C(CCC(O)=O)=C(CC4=C(CC(O)=O)C(CCC(O)=O)=C(CC5=C(CC(O)=O)C(CCC(O)=O)=C(CC(N2)=C1CC(O)=O)N5)N4)N3C40H44N4O16InChI=1S/C40H44N4O16/c45-33(46)5-1-17-21(9-37(53)54)29-14-26-19(3-7-35(49)50)23(11-39(57)58)31(43-26)16-28-20(4-8-36(51)52)24(12-40(59)60)32(44-28)15-27-18(2-6-34(47)48)22(10-38(55)56)30(42-27)13-25(17)41-29/h41-44H,1-16H2,(H,45,46)(H,47,48)(H,49,50)(H,51,52)(H,53,54)(H,55,56)(H,57,58)(H,59,60)QTTNOSKSLATGQB-UHFFFAOYSA-N3-[9,14,19-tris(2-carboxyethyl)-5,10,15,20-tetrakis(carboxymethyl)-21,22,23,24-tetraazapentacyclo[16.2.1.1³,⁶.1⁸,¹¹.1¹³,¹⁶]tetracosa-1(20),3,5,8,10,13,15,18-octaen-4-yl]propanoic acid836.7946836.27523138-4.2812uroporphyrinogen I0-8FDB0229103,8,13,18-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydro-(8ci)-2,7,12,17-porphinetetrapropionate;3,8,13,18-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydro-(8ci)-2,7,12,17-porphinetetrapropionic acid;3,8,13,18-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydro-21h,23h-porphine-2,7,12,17-tetrapropanoate;3,8,13,18-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydro-21h,23h-porphine-2,7,12,17-tetrapropanoic acid;3,8,13,18-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,17-tetrapropanoate;3,8,13,18-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,17-tetrapropanoic acid;3,8,13,18-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,17-tetrapropionic acid;Uro'gen i;Uroporphyrinogen-i;3,8,13,18-tetrakis(carboxymethyl)-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,17-tetrapropionatePW_C001496Uropg1368427025160785861321220241241245781181261742991451Coproporphyrinogen IHMDB0002158Coproporphyrinogen I is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrinogen I is a tetrapyrrole dead-end product resulting from the spontaneous oxidation of the methylene bridges of coproporphyrinogen arising from heme synthesis. It is secreted in feces and urine. Coproporphyrinogen I is biosynthesized from the tetrapyrrole hydroxymethylbilane, which is converted by the action of uroporphyrinogen synthase to uroporphyrinogen I. Uroporphyrinogen I is subsequently converted into coproporphyrinogen I through a series of four decarboxylations. Increased levels of coproporphyrinogens can indicate congenital erythropoietic porphyria or sideroblastic anemia, which are inherited disorders. Porphyria is a pathological state characterized by abnormalities of porphyrin metabolism and results in the excretion of large quantities of porphyrins in the urine and in extreme sensitivity to light. A large number of factors are capable of increasing porphyrin excretion, owing to different and multiple causes and etiologies: (1) the main site of the chronic hepatic porphyria disease process concentrates on the liver, (2) a functional and morphologic liver injury is almost regularly associated with this chronic porphyria, and (3) the toxic form due to occupational and environmental exposure takes mainly a subclinical course. Hepatic factors include disturbance in coproporphyrinogen metabolism, which results from inhibition of coproporphyrinogen oxidase as well as from the rapid loss and diminished utilization of coproporphyrinogen in the hepatocytes. This may also explain why coproporphyrin, its autoxidation product, predominates physiologically in the urine. Decreased biliary excretion of coproporphyrin leading to a compensatory urinary excretion. Therefore, the coproporphyrin ring isomer ratio becomes a sensitive index for impaired liver function, intrahepatic cholestasis, and disturbed activity of hepatic uroporphyrinogen decarboxylase. In itself, secondary coproporphyrinuria is not associated with porphyria symptoms of a hepatologic-gastroenterologic, neurologic, or dermatologic order, even though coproporphyrinuria can occur with such symptoms (PMID: 3327428). Under certain conditions, coproporphyrinogen I can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, hereditary coproporphyria (HCP), congenital erythropoietic porphyria, and sideroblastic anemia. There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503). Coproporphyrinogen I can be found in a number of food items, including cascade huckleberry, hyacinth bean, horseradish tree, and watercress.31110-56-2C0576844077628607COPROPORPHYRINOGEN_I389645CC1=C2CC3=C(CCC(O)=O)C(C)=C(CC4=C(CCC(O)=O)C(C)=C(CC5=C(CCC(O)=O)C(C)=C(CC(N2)=C1CCC(O)=O)N5)N4)N3C36H44N4O8InChI=1S/C36H44N4O8/c1-17-21(5-9-33(41)42)29-14-26-19(3)23(7-11-35(45)46)31(39-26)16-28-20(4)24(8-12-36(47)48)32(40-28)15-27-18(2)22(6-10-34(43)44)30(38-27)13-25(17)37-29/h37-40H,5-16H2,1-4H3,(H,41,42)(H,43,44)(H,45,46)(H,47,48)WIUGGJKHYQIGNH-UHFFFAOYSA-N3-[9,14,19-tris(2-carboxyethyl)-5,10,15,20-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1³,⁶.1⁸,¹¹.1¹³,¹⁶]tetracosa-1(20),3,5,8,10,13,15,18-octaen-4-yl]propanoic acid660.7566660.315914404-4.778coproporphyrinogen I0-4FDB0228743,8,13,18-tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,17-tetrapropanoate;3,8,13,18-tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,17-tetrapropanoic acid;3,8,13,18-tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,17-tetrapropionic acid;Coproporphyrin i;3,8,13,18-tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,17-tetrapropionatePW_C001451CPPgen36992702616078587132122025124124579118126175299502Coproporphyrin IHMDB0000643Coproporphyrin I is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrin I is a tetrapyrrole dead-end product from the spontaneous oxidation of the methylene bridges of coproporphynogen, arising from heme synthesis and secreted in feces and urine. Increased levels of coproporphyrins can indicate congenital erythropoietic porphyria or sideroblastic anaemia. Porphyria is a pathological state characterised by abnormalities of porphyrin metabolism and results in the excretion of large quantities of porphyrins in the urine and in extreme sensitivity to light. A large number of factors are capable of increasing porphyrin excretion, owing to different and multiple causes and etiologies: 1) the main site of the chronic hepatic porphyria disease process concentrates on the liver, 2) a functional and morphologic liver injury is almost regularly associated with this chronic porphyria, 3) the toxic form due to occupational and environmental exposure takes mainly a subclinical course. Hepatic factors includes disturbance in coproporphyrinogen metabolism, which results from inhibition of coproporphyrinogen oxidase as well as from the rapid loss from, and diminished utilization of coproporphyrinogen in the hepatocytes, which may also explain why coproporphyrin, its autoxidation product, predominates physiologically in the urine; decreased biliary excretion of coproporphyrin leading to a compensatory urinary excretion, so that the coproporphyrin ring isomer ratio (1:III) becomes a sensitive index for impaired liver function and intrahepatic cholestasis; and disturbed activity of hepatic uroporphyrinogen decarboxylase. In itself, secondary coproporphyrinuria is not associated with porphyria symptoms of a hepatologic-gastroenterologic, neurologic, or dermatologic order, even though coproporphyrinuria can occur with such symptoms. (PMID: 3327428).531-14-6C057692842116736701CC1=C(CCC(O)=O)/C2=C/C3=N/C(=C\C4=C(C)C(CCC(O)=O)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(CCC(O)=O)=C4C)/C(CCC(O)=O)=C3CC36H38N4O8InChI=1S/C36H38N4O8/c1-17-21(5-9-33(41)42)29-14-26-19(3)23(7-11-35(45)46)31(39-26)16-28-20(4)24(8-12-36(47)48)32(40-28)15-27-18(2)22(6-10-34(43)44)30(38-27)13-25(17)37-29/h13-16,37,40H,5-12H2,1-4H3,(H,41,42)(H,43,44)(H,45,46)(H,47,48)/b25-13-,26-14-,27-15-,28-16-,29-14-,30-13-,31-16-,32-15-VORBHEGMEBOMMB-JRHDEHKPSA-N3-[9,14,19-tris(2-carboxyethyl)-5,10,15,20-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1^{3,6}.1^{8,11}.1^{13,16}]tetracosa-1(20),2,4,6(24),7,9,11,13(22),14,16,18-undecaen-4-yl]propanoic acid654.7089654.268964212-4.3363-[9,14,19-tris(2-carboxyethyl)-5,10,15,20-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1^{3,6}.1^{8,11}.1^{13,16}]tetracosa-1(20),2,4,6(24),7,9,11,13(22),14,16,18-undecaen-4-yl]propanoic acid0-4FDB0221593,8,13,18-tetramethyl-2,7,12,17-porphinetetrapropionate;3,8,13,18-tetramethyl-2,7,12,17-porphinetetrapropionic acid;3,8,13,18-tetramethyl-21h,23h-porphine-2,7,12,17-tetrapropionate;3,8,13,18-tetramethyl-21h,23h-porphine-2,7,12,17-tetrapropionic acid;3,8,13,17-tetramethylporphyrin-2,7,12,18-tetrapropanoic acid;3,8,13,17-tetramethylporphyrin-2,7,12,18-tetrapropanoatePW_C000502CopropI37042702716078589132122027124124581118126177299747Uroporphyrin IHMDB0000936Uroporphyrin is the porphyrin produced by oxidation of the methylene bridges in uroporphyrinogen. Uroporphyrins have four acetic acid and four propionic acid side chains attached to their pyrrole rings. The enzyme uroporphyrinogen I synthase catalyzes the formation of hydroxymethylbilane from four molecules of porphobilinogen. Uroporphyrinogen III cosynthase then catalyzes the conversion of hydroxymethylbilane into uroporphyrinogen III. Otherwise, hydroxymethylbilane cyclizes nonenzymatically to form uroporphyrinogen I. Uroporphyrinogen I and III yield their respective uroporphyrins via autooxidation or their respective coproporphyrinogens via decarboxylation. Excessive amounts of uroporphyrin I are excreted in congenital erythropoietic porphyria, and both uroporphyrin I and uroporphyrin III are excreted in porphyria cutanea tarda. Uroporphyrin I and III are the most common isomers. Under certain conditions, uroporphyrin I can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, porphyria cutanea tarda, and hereditary coproporphyria (HCP). There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).607-14-7C0576727484UROPORPHYRIN_I16736725OC(=O)CCC1=C(CC(O)=O)/C2=C/C3=N/C(=C\C4=C(CCC(O)=O)C(CC(O)=O)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(CC(O)=O)=C4CCC(O)=O)/C(CC(O)=O)=C3CCC(O)=OC40H38N4O16InChI=1S/C40H38N4O16/c45-33(46)5-1-17-21(9-37(53)54)29-14-26-19(3-7-35(49)50)23(11-39(57)58)31(43-26)16-28-20(4-8-36(51)52)24(12-40(59)60)32(44-28)15-27-18(2-6-34(47)48)22(10-38(55)56)30(42-27)13-25(17)41-29/h13-16,41,44H,1-12H2,(H,45,46)(H,47,48)(H,49,50)(H,51,52)(H,53,54)(H,55,56)(H,57,58)(H,59,60)/b25-13-,26-14-,27-15-,28-16-,29-14-,30-13-,31-16-,32-15-DAFUFNRZWDWXJP-JRHDEHKPSA-N3-[9,14,19-tris(2-carboxyethyl)-5,10,15,20-tetrakis(carboxymethyl)-21,22,23,24-tetraazapentacyclo[16.2.1.1³,⁶.1⁸,¹¹.1¹³,¹⁶]tetracosa-1(21),2,4,6,8(23),9,11,13,15,17,19-undecaen-4-yl]propanoic acid830.7469830.228281188-4.3510uroporphyrin I0-8FDB0223262,7,12,17-porphinetetrapropionate;2,7,12,17-porphinetetrapropionic acid;3,3',3'',3'''-(3,8,13,18-tetrakis-carboxymethyl-21h,23h-porphine-2,7,12,17-tetrayl)-tetrakis-propionate;3,3',3'',3'''-(3,8,13,18-tetrakis-carboxymethyl-21h,23h-porphine-2,7,12,17-tetrayl)-tetrakis-propionic acid;3,3',3'',3'''-(3,8,13,18-tetrakis-carboxymethyl-porphyrin-2,7,12,17-tetrayl)-tetra-propionate;3,3',3'',3'''-(3,8,13,18-tetrakis-carboxymethyl-porphyrin-2,7,12,17-tetrayl)-tetra-propionic acid;3,8,13,18-tetrakis(carboxymethyl)porphyrin-2,7,12,17-tetrapropanoate;3,8,13,18-tetrakis(carboxymethyl)porphyrin-2,7,12,17-tetrapropanoic acid;3-[7,12,17-tris-(2-carboxy-ethyl)-3,8,13,18-tetrakis-carboxymethyl-22,24-dihydro-porphin-2-yl]-propionate;3-[7,12,17-tris-(2-carboxy-ethyl)-3,8,13,18-tetrakis-carboxymethyl-22,24-dihydro-porphin-2-yl]-propionic acid;Uroporphyrin i;3,8,13,18-tetrakis(carboxymethyl)porphyrin-2,7,12,17-tetrapropionic acid;3,8,13,18-tetrakis(carboxymethyl)porphyrin-2,7,12,17-tetrapropionatePW_C000747Urop137052702816078590132122028124124582118126178299974Coproporphyrinogen IIIHMDB0001261Coproporphyrinogen III is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrinogen III is a tetrapyrrole dead-end product resulting from the spontaneous oxidation of the methylene bridges of coproporphyrinogen arising from heme synthesis. It is secreted in feces and urine. Coproporphyrinogen III is biosynthesized from the tetrapyrrole hydroxymethylbilane, which is converted by the action of uroporphyrinogen III synthase to uroporphyrinogen III. Uroporphyrinogen III is subsequently converted into coproporphyrinogen III through a series of four decarboxylations. Increased levels of coproporphyrinogens can indicate congenital erythropoietic porphyria or sideroblastic anemia, which are inherited disorders. Porphyria is a pathological state characterized by abnormalities of porphyrin metabolism and results in the excretion of large quantities of porphyrins in the urine and in extreme sensitivity to light. A large number of factors are capable of increasing porphyrin excretion, owing to different and multiple causes and etiologies: (1) the main site of the chronic hepatic porphyria disease process concentrates on the liver, (2) a functional and morphologic liver injury is almost regularly associated with this chronic porphyria, and (3) the toxic form due to occupational and environmental exposure takes mainly a subclinical course. Hepatic factors include disturbance in coproporphyrinogen metabolism, which results from inhibition of coproporphyrinogen oxidase as well as from the rapid loss and diminished utilization of coproporphyrinogen in the hepatocytes. This may also explain why coproporphyrin, its autoxidation product, predominates physiologically in the urine. Decreased biliary excretion of coproporphyrin leading to a compensatory urinary excretion. Therefore, the coproporphyrin ring isomer ratio (1:III) becomes a sensitive index for impaired liver function, intrahepatic cholestasis, and disturbed activity of hepatic uroporphyrinogen decarboxylase. In itself, secondary coproporphyrinuria is not associated with porphyria symptoms of a hepatologic-gastroenterologic, neurologic, or dermatologic order, even though coproporphyrinuria can occur with such symptoms (PMID: 3327428). Under certain conditions, coproporphyrinogen III can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, hereditary coproporphyria (HCP), congenital erythropoietic porphyria, and sideroblastic anemia. In particular, coproporphyrinogen III is accumulated and excreted excessively in the feces in acute intermittent porphyria, protoporphyria, and variegate porphyria. There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).
2624-63-7C0326332115439COPROPORPHYRINOGEN_III315CC1=C2CC3=C(C)C(CCC(O)=O)=C(CC4=C(CCC(O)=O)C(C)=C(CC5=C(CCC(O)=O)C(C)=C(CC(N2)=C1CCC(O)=O)N5)N4)N3C36H44N4O8InChI=1S/C36H44N4O8/c1-17-21(5-9-33(41)42)29-14-27-19(3)22(6-10-34(43)44)30(39-27)15-28-20(4)24(8-12-36(47)48)32(40-28)16-31-23(7-11-35(45)46)18(2)26(38-31)13-25(17)37-29/h37-40H,5-16H2,1-4H3,(H,41,42)(H,43,44)(H,45,46)(H,47,48)NIUVHXTXUXOFEB-UHFFFAOYSA-N3-[9,14,20-tris(2-carboxyethyl)-5,10,15,19-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1^{3,6}.1^{8,11}.1^{13,16}]tetracosa-1(20),3,5,8,10,13,15,18-octaen-4-yl]propanoic acid660.7566660.315914404-4.7783-[9,14,20-tris(2-carboxyethyl)-5,10,15,19-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1^{3,6}.1^{8,11}.1^{13,16}]tetracosa-1(20),3,5,8,10,13,15,18-octaen-4-yl]propanoic acid0-4FDB0225183,8,13,17-tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropanoate;3,8,13,17-tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropanoic acid;5,10,15,20,22,24-hexahydro-3,8,13,17-tetramethyl-2,7,12,18-porphinetetrapropionate;5,10,15,20,22,24-hexahydro-3,8,13,17-tetramethyl-2,7,12,18-porphinetetrapropionic acid;5,10,15,20,22,24-hexahydro-3,8,13,17-tetramethyl-21h,23h-porphine-2,7,12,18-tetrapropanoate;5,10,15,20,22,24-hexahydro-3,8,13,17-tetramethyl-21h,23h-porphine-2,7,12,18-tetrapropanoic acid;Coproporphyrinogen;Coproporphyrinogen iii;Coproporphyrinogen-iii;3,8,13,17-tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropionic acid;Coproporphyrin iii;3,8,13,17-tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropionatePW_C000974Coprgn33706237123702916070311637859113278593112122029124122031407124583118124585119126179299126181481445Coproporphyrin IIIHMDB0000570Coproporphyrin III is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrin III is a tetrapyrrole dead-end product from the spontaneous oxidation of the methylene bridges of coproporphynogen, arising from heme synthesis and secreted in feces and urine. Increased levels of coproporphyrins can indicate congenital erythropoietic porphyria or sideroblastic anaemia. Porphyria is a pathological state characterised by abnormalities of porphyrin metabolism and results in the excretion of large quantities of porphyrins in the urine and in extreme sensitivity to light. A large number of factors are capable of increasing porphyrin excretion, owing to different and multiple causes and etiologies: 1) the main site of the chronic hepatic porphyria disease process concentrates on the liver, 2) a functional and morphologic liver injury is almost regularly associated with this chronic porphyria, 3) the toxic form due to occupational and environmental exposure takes mainly a subclinical course. Hepatic factors includes disturbance in coproporphyrinogen metabolism, which results from inhibition of coproporphyrinogen oxidase as well as from the rapid loss from, and diminished utilization of coproporphyrinogen in the hepatocytes, which may also explain why coproporphyrin, its autoxidation product, predominates physiologically in the urine; decreased biliary excretion of coproporphyrin leading to a compensatory urinary excretion, so that the coproporphyrin ring isomer ratio (1:III) becomes a sensitive index for impaired liver function and intrahepatic cholestasis; and disturbed activity of hepatic uroporphyrinogen decarboxylase. In itself, secondary coproporphyrinuria is not associated with porphyria symptoms of a hepatologic-gastroenterologic, neurologic, or dermatologic order, even though coproporphyrinuria can occur with such symptoms. (PMID: 3327428).14643-66-4C057702760916736509DB04461CC1=C(CCC(O)=O)/C2=C/C3=N/C(=C\C4=C(C)C(CCC(O)=O)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(C)=C4CCC(O)=O)/C(CCC(O)=O)=C3CC36H38N4O8InChI=1S/C36H38N4O8/c1-17-21(5-9-33(41)42)29-14-27-19(3)22(6-10-34(43)44)30(39-27)15-28-20(4)24(8-12-36(47)48)32(40-28)16-31-23(7-11-35(45)46)18(2)26(38-31)13-25(17)37-29/h13-16,37,40H,5-12H2,1-4H3,(H,41,42)(H,43,44)(H,45,46)(H,47,48)/b25-13-,26-13-,27-14-,28-15-,29-14-,30-15-,31-16-,32-16-JWFCYWSMNRLXLX-UJJXFSCMSA-N3-[9,15,19-tris(2-carboxyethyl)-5,10,14,20-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1^{3,6}.1^{8,11}.1^{13,16}]tetracosa-1(20),2,4,6(24),7,9,11,13(22),14,16,18-undecaen-4-yl]propanoic acid654.7089654.268964212-4.3363-[9,15,19-tris(2-carboxyethyl)-5,10,14,20-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1^{3,6}.1^{8,11}.1^{13,16}]tetracosa-1(20),2,4,6(24),7,9,11,13(22),14,16,18-undecaen-4-yl]propanoic acid0-4FDB0055573,8,13,17-tetramethylporphyrin-2,7,12,18-tetrapropanoic acid;3,8,13,17-tetramethylporphyrin-2,7,12,18-tetrapropanoatePW_C000445CoprIII370727030160785921321220301241245841181261802991065OxygenHMDB0001377Oxygen 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_C001065O2959110524516500185058549146252863836491067431688207541576347693383621375492016242531222803294260424747135467123548012554931265508127580910859731476129159700618870321637050160731921375332107560212839515111816216118641981188321511894211120572251206316412247286122792261232524912706291127162921300429813016300130263011303830213260223422761742657315769102937704429477214134773501117736313077377331773953327749711377512115775373347762633677723337777361127774712977756341778051147781213378070329781511327838134578805343791113601200474081203831221204264051205424071205534141205944091206014061208834151210451241211043831216054341216564291221173821225734181226893841227983741228224431230271351230603761231284471231391361231634481231761191231874501232191371232261201234594511236091181236693981241634691242144641246693991251454541252751211254254821257064781257314831257372971257404791258844811261002991262724841265224951267214891268254801269645021269862071271982091272142081272192051272225011273055041273452061275573881275745151278353891280813951280953901283125061284323911051HydrogenHMDB0001362Hydrogen is a colorless, odorless, nonmetallic, tasteless, highly flammable diatomic gas with the molecular formula H2. With an atomic weight of 1.00794, hydrogen is the lightest element. Besides the common H1 isotope, hydrogen exists as the stable isotope Deuterium and the unstable, radioactive isotope Tritium. Hydrogen is the most abundant of the chemical elements, constituting roughly 75% of the universe's elemental mass. Hydrogen can form compounds with most elements and is present in water and most organic compounds. It plays a particularly important role in acid-base chemistry, in which many reactions involve the exchange of protons between soluble molecules. Oxidation of hydrogen, in the sense of removing its electron, formally gives H+, containing no electrons and a nucleus which is usually composed of one proton. That is why H+ is often called a proton. This species is central to discussion of acids. Under the Bronsted-Lowry theory, acids are proton donors, while bases are proton acceptors. A bare proton H+ cannot exist in solution because of its strong tendency to attach itself to atoms or molecules with electrons. However, the term 'proton' is used loosely to refer to positively charged or cationic hydrogen, denoted H+. H2 is a product of some types of anaerobic metabolism and is produced by several microorganisms, usually via reactions catalyzed by iron- or nickel-containing enzymes called hydrogenases. These enzymes catalyze the reversible redox reaction between H2 and its component two protons and two electrons. Creation of hydrogen gas occurs in the transfer of reducing equivalents produced during pyruvate fermentation to water.1333-74-0C002825883867318276ALPHA-GLUCOSE-16-BISPHOSPHATE762[H][H]H2InChI=1S/H2/h1HUFHFLCQGNIYNRP-UHFFFAOYSA-Ndihydrogen2.01592.0156500640dihydrogen00FDB016247Dihydrogen;Hydrogen;Hydrogen cation;Hydrogen gas;Hydrogen ion;Hydronium;Proton;E 949;E-949;E949;H2;Molecular hydrogenPW_C001051H217568238822696331467049521070331637045160127741511327022578594112786031327877211111316394121448122122032407122037124124006135124586119124591118126073297126182481126188299127529205128005388128323206863Protoporphyrinogen IXHMDB0001097Protoporphyrinogen IX is an intermediate in heme biosynthesis. It is a porphyrinogen in which two pyrrole rings each have one methyl and one propionate side chain, and the other two pyrrole rings each have one methyl and one vinyl side chain. Fifteen isomers are possible but only one, type IX, occurs naturally. Protoporphyrinogen is produced by oxidative decarboxylation of coproporphyrinogen. Under certain conditions, protoporphyrinogen IX can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, and hereditary coproporphyria (HCP). In particular, protoporphyrinogen IX is accumulated and excreted excessively in the feces in acute intermittent porphyria, protoporphyria, and variegate porphyria. There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).7412-77-3C0107912189315435PROTOPORPHYRINOGEN108741CC1=C2CC3=C(C)C(C=C)=C(CC4=C(C)C(C=C)=C(CC5=C(C)C(CCC(O)=O)=C(CC(N2)=C1CCC(O)=O)N5)N4)N3C34H40N4O4InChI=1S/C34H40N4O4/c1-7-21-17(3)25-13-26-19(5)23(9-11-33(39)40)31(37-26)16-32-24(10-12-34(41)42)20(6)28(38-32)15-30-22(8-2)18(4)27(36-30)14-29(21)35-25/h7-8,35-38H,1-2,9-16H2,3-6H3,(H,39,40)(H,41,42)UHSGPDMIQQYNAX-UHFFFAOYSA-N3-[20-(2-carboxyethyl)-9,14-diethenyl-5,10,15,19-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1^{3,6}.1^{8,11}.1^{13,16}]tetracosa-1(20),3,5,8,10,13,15,18-octaen-4-yl]propanoic acid568.7058568.304955788-5.0163-[20-(2-carboxyethyl)-9,14-diethenyl-5,10,15,19-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1^{3,6}.1^{8,11}.1^{13,16}]tetracosa-1(20),3,5,8,10,13,15,18-octaen-4-yl]propanoic acid0-2FDB0224255,10,15,20,22,24-hexahydro protoporphyrin ix deriv.;7,12-diethenyl-3,8,13,17-tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,18-dipropanoate;7,12-diethenyl-3,8,13,17-tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,18-dipropanoic acid;7,12-diethenyl-5,10,15,20,22,24-hexahydro-3,8,13,17-tetramethyl-21h,23h-porphine-2,18-dipropanoate;7,12-diethenyl-5,10,15,20,22,24-hexahydro-3,8,13,17-tetramethyl-21h,23h-porphine-2,18-dipropanoic acid;Protoporphyrinogen;Protoporphyrinogen ix;Protoporphyrinogen-ixPW_C000863Protop9371437034163785951121220334071245871191261834811783Hydrogen 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_C001783H2O29891135188855114627287551512433169121749512534223818104749134752315495126550212355101275810108600514770381638396151118172161188621512461226127092911271929213028301130352981304030213405222426583157702222577047294770792937750011377540334775981157772033277725337778061147781011177819326780733297815213278598112120050408120102122120463405120595409120609416120954407121047124122120382122801374122814443122839135123097376123157447123165448123220137123234452123520119123611118124672399125428482125469297125709478125732483125748488125895481126103299126275484126967502126978207127006205127201209127215208127230505127356206127601388127838389964FADHMDB0001248FAD, also known as flavitan or adeflavin, belongs to the class of organic compounds known as flavin nucleotides. These are nucleotides containing a flavin moiety. Flavin is a compound that contains the tricyclic isoalloxazine ring system, which bears 2 oxo groups at the 2- and 4-positions. FAD is a drug which is used to treat eye diseases caused by vitamin b2 deficiency, such as keratitis and blepharitis. FAD is slightly soluble (in water) and a moderately acidic compound (based on its pKa). FAD has been found in human liver and muscle tissues, and has also been detected in multiple biofluids, such as feces and blood. Within the cell, FAD is primarily located in the cytoplasm, mitochondria, endoplasmic reticulum and peroxisome. FAD exists in all living organisms, ranging from bacteria to humans. In humans, FAD is involved in the risedronate action pathway, the ibandronate action pathway, the valine, leucine and isoleucine degradation pathway, and the pyrimidine metabolism pathway. FAD is also involved in several metabolic disorders, some of which include the oncogenic action OF L-2-hydroxyglutarate in hydroxygluaricaciduria pathway, gaba-transaminase deficiency, 4-hydroxybutyric aciduria/succinic semialdehyde dehydrogenase deficiency, and the saccharopinuria/hyperlysinemia II pathway. FAD is a condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972).146-14-5C0001664397516238FAD559059DB03147CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=NC(=O)NC(=O)C1=N2C27H33N9O15P2InChI=1S/C27H33N9O15P2/c1-10-3-12-13(4-11(10)2)35(24-18(32-12)25(42)34-27(43)33-24)5-14(37)19(39)15(38)6-48-52(44,45)51-53(46,47)49-7-16-20(40)21(41)26(50-16)36-9-31-17-22(28)29-8-30-23(17)36/h3-4,8-9,14-16,19-21,26,37-41H,5-7H2,1-2H3,(H,44,45)(H,46,47)(H2,28,29,30)(H,34,42,43)/t14-,15+,16+,19-,20+,21+,26+/m0/s1VWWQXMAJTJZDQX-UYBVJOGSSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}[({[(2R,3S,4S)-5-{7,8-dimethyl-2,4-dioxo-2H,3H,4H,10H-benzo[g]pteridin-10-yl}-2,3,4-trihydroxypentyl]oxy}(hydroxy)phosphoryl)oxy]phosphinic acid785.5497785.157134455-2.279flavine-adenine dinucleotide0-3FDB0225111h-purin-6-amine flavin dinucleotide;1h-purin-6-amine flavine dinucleotide;Adenine-flavin dinucleotide;Adenine-flavine dinucleotide;Adenine-riboflavin dinuceotide;Adenine-riboflavin dinucleotide;Adenine-riboflavine dinucleotide;Fad;Flamitajin b;Flanin f;Flavin adenine dinucleotide;Flavin adenine dinucleotide oxidized;Flavin-adenine dinucleotide;Flavine adenosine diphosphate;Flavine-adenine dinucleotide;Flavitan;Flaziren;Isoalloxazine-adenine dinucleotide;Riboflavin 5'-adenosine diphosphate;Riboflavin-adenine dinucleotide;Riboflavine-adenine dinucleotide;AdeflavinPW_C000964FAD9991145186819232164253176282882518840211881414894216122916224921335825362237232646023646883147411347581048816526810352851025335111549612655111275613118603015560541566082161611616263901647517864991796666107703916371752057321213746522274872239076224118182161188721511899211122962251232824912443151125192271259522612710291127202921302930113041302436233187708029377126133771521347750111377507112775181157754133477615132777263377805432978375345789303317922233679272358800123688003436980714119119958406119999384120051408120107407120432405120453122120490124121278429121298418121417382121489383122748120122776121122802374122823443123066376123087135123166448123849464123868454123976399124047398125348479125378480125429482125474481125697297125979489126107299126277484126891501126920391126968502126987207127011206127310209127432506127602388127840389140790185140799186163Protoporphyrin IXHMDB0000241Protoporphyrins are tetrapyrroles containing 4 methyl, 2 propionic, and 2 vinyl side chains. Protoporphyrin is produced by oxidation of the methylene bridge of protoporphyrinogen. Protoporphyrin IX is the only naturally occurring isomer; it is an intermediate in heme biosynthesis, combining with ferrous iron to form protoheme IX, the heme prosthetic group of hemoglobin. Protoporphyrin IX is created by the enzyme protoporphyrinogen oxidase. The enzyme ferrochelatase converts it into heme. Protoporphyrin IX naturally occurs in small amounts in feces. Protoporphyrin IX is also responsible for the brown pigment (ooporphyrin) of birds' eggs. Protoporphyrin IX is used as a branch point in the biosynthetic pathway leading to heme (by insertion of iron) and chlorophylls (by insertion of Mg and further side-chain transformation). Protoporphyrin IX can be used to treat liver disorders, mainly as the sodium salt. Under certain conditions, protoporphyrin IX can act as a neurotoxin, a phototoxin, and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. A phototoxin causes cell damage upon exposure to light. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, and hereditary coproporphyria (HCP). In particular, it is accumulated and excreted excessively in the feces in acute intermittent porphyria, protoporphyria, and variegate porphyria. There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).553-12-8C0219115430PROTOPORPHYRIN_IX10469486DB02285CC1=C(CCC(O)=O)/C2=C/C3=N/C(=C\C4=C(C)C(C=C)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(C=C)=C4C)/C(C)=C3CCC(O)=OC34H34N4O4InChI=1S/C34H34N4O4/c1-7-21-17(3)25-13-26-19(5)23(9-11-33(39)40)31(37-26)16-32-24(10-12-34(41)42)20(6)28(38-32)15-30-22(8-2)18(4)27(36-30)14-29(21)35-25/h7-8,13-16,35,38H,1-2,9-12H2,3-6H3,(H,39,40)(H,41,42)/b25-13-,26-13-,27-14-,28-15-,29-14-,30-15-,31-16-,32-16-KSFOVUSSGSKXFI-UJJXFSCMSA-N3-[20-(2-carboxyethyl)-9,14-diethenyl-5,10,15,19-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1^{3,6}.1^{8,11}.1^{13,16}]tetracosa-1(21),2,4,6,8(23),9,11,13,15,17,19-undecaen-4-yl]propanoic acid562.6582562.258005596-4.4143-[20-(2-carboxyethyl)-9,14-diethenyl-5,10,15,19-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1^{3,6}.1^{8,11}.1^{13,16}]tetracosa-1(21),2,4,6,8(23),9,11,13,15,17,19-undecaen-4-yl]propanoic acid0-2FDB0042773,3'-(3,7,12,17-tetramethyl-8,13-divinyl-21h,23h-porphine-2,18-diyl)-bis-propionate;3,3'-(3,7,12,17-tetramethyl-8,13-divinyl-21h,23h-porphine-2,18-diyl)-bis-propionic acid;3,3'-(3,7,12,17-tetramethyl-8,13-divinylporphine-2,18-diyl)di;Kammerer's prophyrin;Ooporphyrin;Porphyrinogen ix;Ppix;Protoporphyrin;Protoporphyrin ix;Protoporphyrin-"ix";Protoporphyrin-ix;3,7,12,17-tetramethyl-8,13-divinylporphyrin-2,18-dipropanoic acid;H2ppix;3,7,12,17-tetramethyl-8,13-divinylporphyrin-2,18-dipropanoatePW_C000163PrtopIX3718337261737274703716370431617059162785971127860113378620134122036406122054384124590120124608121126187479126206480544Fe2+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+3981964136783169220709817772704116370521601206022512143151771791327774011277751129777603417778211112054440712055741412057012212176512412317811912319145012320413512431611812614329912618548112765038814071049140716188133Fe3+HMDB0012943Fe3+, also known as ferric ion or fe(iii), belongs to the class of inorganic compounds known as homogeneous transition metal compounds. These are inorganic compounds containing only metal atoms,with the largest atom being a transition metal atom. Fe3+ exists in all living organisms, ranging from bacteria to humans. 2,3-Dihydroxybenzoylserine and fe3+ can be biosynthesized from ferric enterobactin through its interaction with the enzyme enterochelin esterase. Outside of the human body, fe3+ can be found in a number of food items such as bamboo shoots, catjang pea, chickpea, and orange bell pepper. This makes fe3+ a potential biomarker for the consumption of these food products. The major activity of supplemental iron is in the prevention and treatment of iron deficiency anemia. Iron has putative immune-enhancing, anticarcinogenic and cognition-enhancing activities.20074-52-6C148192993629034CPD-1013427815[Fe+3]FeInChI=1S/Fe/q+3VTLYFUHAOXGGBS-UHFFFAOYSA-Niron(3+) ion55.84555.9349421330iron(3+) ion33C14819Fe(iii);Ferric ion;Iron(3+);Fe (iii) ion;Fe(3+);Ferric iron;Iron, ion (fe(3+))PW_C008133Fe3+1230312968142049199727042163118411601267515177431111774503317859911278721132120949407121013122121127383121593124123515119123578135123696398124151118125890481127351206127559388140718181799HemeHMDB0003178Heme is the color-furnishing portion of hemoglobin. It is found free in tissues and as the prosthetic group in many hemeproteins. A heme or haem is a prosthetic group that consists of an iron atom contained in the center of a large heterocyclic organic ring called a porphyrin. Not all porphyrins contain iron, but a substantial fraction of porphyrin-containing metalloproteins have heme as their prosthetic subunit; these are known as hemoproteins.14875-96-8C0003217627HEME_A24604415DB02577CC1=C(CCC(O)=O)C2=CC3=[N+]4C(=CC5=C(C)C(C=C)=C6C=C7C(C)=C(C=C)C8=[N+]7[Fe--]4(N2C1=C8)N56)C(C)=C3CCC(O)=OC34H32FeN4O4InChI=1S/C34H34N4O4.Fe/c1-7-21-17(3)25-13-26-19(5)23(9-11-33(39)40)31(37-26)16-32-24(10-12-34(41)42)20(6)28(38-32)15-30-22(8-2)18(4)27(36-30)14-29(21)35-25;/h7-8,13-16H,1-2,9-12H2,3-6H3,(H4,35,36,37,38,39,40,41,42);/q;+2/p-2/b25-13-,26-13-,27-14-,28-15-,29-14-,30-15-,31-16-,32-16-;KABFMIBPWCXCRK-RGGAHWMASA-L4,20-bis(2-carboxyethyl)-10,15-diethenyl-5,9,14,19-tetramethyl-2lambda5,22,23lambda5,25-tetraaza-1-ferraoctacyclo[11.9.1.1^{1,8}.1^{3,21}.0^{2,6}.0^{16,23}.0^{18,22}.0^{11,25}]pentacosa-2,4,6,8,10,12,14,16(23),17,19,21(24)-undecaene-2,23-bis(ylium)-1,1-diuide616.487616.177297665-5.4824,20-bis(2-carboxyethyl)-10,15-diethenyl-5,9,14,19-tetramethyl-2lambda5,22,23lambda5,25-tetraaza-1-ferraoctacyclo[11.9.1.1^{1,8}.1^{3,21}.0^{2,6}.0^{16,23}.0^{18,22}.0^{11,25}]pentacosa-2,4,6,8,10,12,14,16(23),17,19,21(24)-undecaene-2,23-bis(ylium)-1,1-diuide0-2FDB016272(protoporphyrinato)iron;Ferroheme;Ferroheme b;Ferroprotoheme;Ferroprotoporphyrin;Ferroprotoporphyrin ix;Ferrous protoheme;Ferrous protoheme ix;Haem;Hem;Heme;Iron protoporphyrin;Iron protoporphyrin ix;Iron(ii) protoporphyrin ix;Protoferroheme;Protohaem;Protoheme;Protoheme ix;Reduced hematinPW_C001799Heme247163081032486082766512443135449141336196318280629293893238113367263421143734440433148232851709554721235485125551712958301416246786283165971517044160706016173262131183519811898211120651641300929813021300422781776915293769312497735111177364130773673317739833277517115776293367781333478380133786021327896311279932134120431405120603408120955407121085383121658429121746124121910122122570406122691384123065376123133447123144136123228374123521119123650398124216464124297118124463135125142120125277121125742482125896481126196299126499297126512495126718479126827480127224502127357206127632388128070205128083395128086390128309501128434391405582Fe-2SHMDB0061344Bis(λ²-iron(2+) ion) disulfane tetrasulfanide belongs to the class of inorganic compounds known as transition metal sulfides. These are inorganic compounds containing a sulfur atom of an oxidation state of -2, in which the heaviest atom bonded to the oxygen is a transition metal.S.S.[SH-].[SH-].[SH-].[SH-].[Fe++].[Fe++]Fe2H8S6InChI=1S/2Fe.6H2S/h;;6*1H2/q2*+2;;;;;;/p-4MZMMVZPHZTYDNI-UHFFFAOYSA-Jbis(lambda2-iron(2+) ion) disulfane tetrasulfanide312.11311.7648990bis(lambda2-iron(2+) ion) disulfane tetrasulfanide02PW_C0405582Fe2S37422401284257174389346265475431482994837285056454971265512127704616013030301130423027771511377727337783771347838613279207112117811133121586407121795124122567384122664406123152443123167448124144119124346118125140121125239120126189299126715480126796479127680388128306391128394501768Farnesyl pyrophosphateHMDB0000961Farnesyl pyrophosphate, also known as farnesyl diphosphoric acid or farnesyl-PP, belongs to the class of organic compounds known as sesquiterpenoids. These are terpenes with three consecutive isoprene units. Farnesyl pyrophosphate is considered to be a practically insoluble (in water) and relatively neutral molecule. Farnesyl pyrophosphate has been found in human testicle tissue, and has also been primarily detected in blood. Farnesyl pyrophosphate can be found anywhere throughout the human cell, such as in membrane (predicted from logP), peroxisome, mitochondria, and endoplasmic reticulum. Farnesyl pyrophosphate exists in all living organisms, ranging from bacteria to humans. Farnesyl pyrophosphate participates in a number of enzymatic reactions. In particular, Ferroheme b and farnesyl pyrophosphate can be converted into heme O; which is mediated by the enzyme heme O synthase. Furthermore, Farnesyl pyrophosphate and isopentenyl pyrophosphate can be converted into octaprenyl diphosphate through the action of the enzyme octaprenyl diphosphate synthase. Furthermore, Farnesyl pyrophosphate can be biosynthesized from geranyl-PP and isopentenyl pyrophosphate through its interaction with the enzyme geranyl diphosphate synthase / farnesyl diphosphate synthase. Finally, Farnesyl pyrophosphate and isopentenyl pyrophosphate can be converted into di-trans,octa-cis-undecaprenyl diphosphate; which is catalyzed by the enzyme undecaprenyl diphosphate synthase. In humans, farnesyl pyrophosphate is involved in the simvastatin action pathway, the risedronate action pathway, the alendronate action pathway, and the ibandronate action pathway. Farnesyl pyrophosphate is also involved in several metabolic disorders, some of which include the hyper-igd syndrome pathway, the chondrodysplasia punctata II, X linked dominant (CDPX2) pathway, the porphyria variegata (PV) pathway, and lysosomal acid lipase deficiency (wolman disease). Outside of the human body, farnesyl pyrophosphate can be found in a number of food items such as sweet basil, apple, spearmint, and nuts. This makes farnesyl pyrophosphate a potential biomarker for the consumption of these food products. Farnesyl pyrophosphate is an intermediate in the HMG-CoA reductase pathway used by organisms in the biosynthesis of terpenes and terpenoids. -- Wikipedia.372-97-4C0044844571317407FARNESYL-PP393270DB07780CC(C)=CCC\C(C)=C\CC\C(C)=C\COP(O)(=O)OP(O)(O)=OC15H28O7P2InChI=1S/C15H28O7P2/c1-13(2)7-5-8-14(3)9-6-10-15(4)11-12-21-24(19,20)22-23(16,17)18/h7,9,11H,5-6,8,10,12H2,1-4H3,(H,19,20)(H2,16,17,18)/b14-9+,15-11+VWFJDQUYCIWHTN-YFVJMOTDSA-N{[hydroxy({[(2E,6E)-3,7,11-trimethyldodeca-2,6,10-trien-1-yl]oxy})phosphoryl]oxy}phosphonic acid382.33382.131027238-3.683farnesyl diphosphate0-2FDB022339(2e,6e)-farnesyl diphosphate;(2e,6e)-farnesyl pyrophosphate;(e,e)-farnesyl diphosphate;(e,e)-farnesyl pyrophosphate;(all-e)-farnesyl diphosphate;2-trans,6-trans-farnesyl diphosphate;2-trans,6-trans-farnesyl pyrophosphate;All-trans-farnesyl pyrophosphate;Farnesyl diphosphate;Farnesyl pyrophosphate;Farnesyl-pp;Trans-farnesyl pyrophosphate;Trans-trans-farnesyl diphosphate;Trans-trans-farnesyl pyrophosphate;(2e,6e)-farnesol diphosphate;Trans,trans-farnesyl diphosphate;(2e,6e)-farnesol diphosphoric acid;2-trans,6-trans-farnesyl diphosphoric acid;(2e,6e)-farnesyl diphosphoric acid;(2e,6e)-farnesyl pyrophosphoric acid;(all-e)-farnesyl diphosphoric acid;(e,e)-farnesyl pyrophosphoric acid;2-trans,6-trans-farnesyl pyrophosphoric acid;All-trans-farnesyl pyrophosphoric acid;Farnesyl diphosphoric acid;Farnesyl pyrophosphoric acid;Trans,trans-farnesyl diphosphoric acid;Trans-trans-farnesyl diphosphoric acidPW_C000768FarPP8135161483744270471607314198828421012316225123191517860513278926111809683121482122122039407124040135124593119126191481900Heme OHMDB0001162Heme O belongs to the class of organic compounds known as metallotetrapyrroles. These are polycyclic compounds containing a tetrapyrrole skeleton combined with a metal atom. Heme O is considered to be a practically insoluble (in water) and relatively neutral molecule. Within the cell, heme O is primarily located in the membrane (predicted from logP). Heme O exists in all living organisms, ranging from bacteria to humans. In humans, heme O is involved in the porphyrin metabolism pathway. Heme O is also involved in several metabolic disorders, some of which include the porphyria variegata (PV) pathway, congenital erythropoietic porphyria (cep) or gunther disease pathway, the hereditary coproporphyria (HCP) pathway, and the acute intermittent porphyria pathway. Heme O differs from the closely related heme A by having a methyl group at ring position 8 instead of the formyl group, the isoprenoid chain at position 2 is the same (Wikipedia).137397-56-9C1567224480HEME_O3571849CC(C)=CCC\C(C)=C\CC\C(C)=C\CCC(O)C1=C(C)/C2=C/C3=N/C(=C\C4=C(CCC(O)=O)C(C)=C5/C=C6\N=C(\C=C\1/N\2[Fe]N45)C(C)=C6C=C)/C(CCC(O)=O)=C3CC49H58FeN4O5InChI=1S/C49H60N4O5.Fe/c1-10-35-31(6)40-26-45-49(46(54)19-13-18-30(5)17-12-16-29(4)15-11-14-28(2)3)34(9)41(53-45)24-38-32(7)36(20-22-47(55)56)43(51-38)27-44-37(21-23-48(57)58)33(8)39(52-44)25-42(35)50-40;/h10,14,16,18,24-27,46,54H,1,11-13,15,17,19-23H2,2-9H3,(H4,50,51,52,53,55,56,57,58);/q;+2/p-2/b29-16+,30-18+,38-24-,39-25-,40-26-,41-24-,42-25-,43-27-,44-27-,45-26-;FISPASSVCDRERW-KVGORYHISA-L3-[(11Z,16Z)-20-(2-carboxyethyl)-15-ethenyl-10-[(4E,8E)-1-hydroxy-5,9,13-trimethyltetradeca-4,8,12-trien-1-yl]-5,9,14,19-tetramethyl-21,23,24,25-tetraaza-22-ferrahexacyclo[9.9.3.1³,⁶.1¹³,¹⁶.0⁸,²³.0¹⁸,²¹]pentacosa-1(20),2,4,6(25),7,9,11,13(24),14,16,18-undecaen-4-yl]propanoic acid838.854838.375663119-5.1733-[(11Z,16Z)-20-(2-carboxyethyl)-15-ethenyl-10-[(4E,8E)-1-hydroxy-5,9,13-trimethyltetradeca-4,8,12-trien-1-yl]-5,9,14,19-tetramethyl-21,23,24,25-tetraaza-22-ferrahexacyclo[9.9.3.1³,⁶.1¹³,¹⁶.0⁸,²³.0¹⁸,²¹]pentacosa-1(20),2,4,6(25),7,9,11,13(24),14,16,18-undecaen-4-yl]propanoic acid0-2FDB022457Heme oPW_C000900Heme O3745265881097010189704816042792317786061328096731220404071245941191261924813003Heme AHMDB0006901Heme A differs from heme B in that a methyl side chain at ring position 8 is oxidized into a formyl group, and one of the vinyl side chains, at ring position 2, has been replaced by an isoprenoid chain. Like heme B, heme A is often attached to the apoprotein (cytochromes or globins) through a coordination bond between the heme iron and a conserved amino acid side-chain. An example of a metalloprotein that contains heme A is cytochrome c oxidase. Both the formyl group and the isoprenoid side chain are thought to play important roles in conservation of the energy of oxygen reduction by cytochrome c oxidase.(Wikipedia).57560-10-8C15670CC(C)=CCC\C(C)=C\CC\C(C)=C\CCC(O)C1=C2C=C3C(C)=C(C=C)C4=[N+]3[Fe]35N6C(=C4)C(C)=C(CCC(O)=O)C6=CC4=[N+]3C(=CC(N25)=C1C)C(C=O)=C4CCC(O)=OC49H56FeN4O6InChI=1S/C49H58N4O6.Fe/c1-9-34-31(6)39-25-45-49(46(55)18-12-17-30(5)16-11-15-29(4)14-10-13-28(2)3)33(8)40(52-45)24-44-37(27-54)36(20-22-48(58)59)43(53-44)26-42-35(19-21-47(56)57)32(7)38(51-42)23-41(34)50-39;/h9,13,15,17,23-27,46,55H,1,10-12,14,16,18-22H2,2-8H3,(H4,50,51,52,53,54,56,57,58,59);/q;+4/p-2/b29-15+,30-17+,38-23-,39-25-,40-24-,41-23-,42-26-,43-26-,44-24-,45-25-;RRJCKGQQRUXGQR-ONCSLILDSA-L4,20-bis(2-carboxyethyl)-15-ethenyl-5-formyl-10-[(4E,8E)-1-hydroxy-5,9,13-trimethyltetradeca-4,8,12-trien-1-yl]-9,14,19-trimethyl-2lambda5,22,23lambda5,25-tetraaza-1-ferraoctacyclo[11.9.1.1^{1,8}.1^{3,21}.0^{2,6}.0^{16,23}.0^{18,22}.0^{11,25}]pentacosa-2,4,6,8,10,12,14,16(23),17,19,21(24)-undecaene-2,23-bis(ylium)852.837852.354927677-6.6534,20-bis(2-carboxyethyl)-15-ethenyl-5-formyl-10-[(4E,8E)-1-hydroxy-5,9,13-trimethyltetradeca-4,8,12-trien-1-yl]-9,14,19-trimethyl-2lambda5,22,23lambda5,25-tetraaza-1-ferraoctacyclo[11.9.1.1^{1,8}.1^{3,21}.0^{2,6}.0^{16,23}.0^{18,22}.0^{11,25}]pentacosa-2,4,6,8,10,12,14,16(23),17,19,21(24)-undecaene-2,23-bis(ylium)20FDB024148(sp-4-2)[7-ethenyl-17-formyl-12-[(4e,8e)-1-hydroxy-5,9,13-trimethyl-4,8,12-tetradecatrienyl]-3,8,13-trimethyl-21h,23h-porphine-2,18-dipropanoato(4-)-kappan21,kappan22,kappan23,kappan24]-ferrate(2-);Heme a;[sp-4-2-(e,e)]-[7-ethenyl-17-formyl-12-(1-hydroxy-5,9,13-trimethyl-4,8,12-tetradecatrienyl)-3,8,13-trimethyl-21h,23h-porphine-2,18-dipropanoato(4-)-n21,n22,n23,n24]-ferrate(2-)PW_C003003Heme A37513704916378608112122042407124596119126194481797BiliverdinHMDB0001008Biliverdin is a green pigment formed as a byproduct of hemoglobin breakdown. It consists of four linearly connected pyrrole rings (a tetrapyrrole). Biliverdin is formed when the heme group in hemoglobin is cleaved at its alpha-methene bridge. The resulting biliverdin is then reduced to bilirubin, a yellow pigment, by the enzyme biliverdin reductase. The changing color of a bruise from deep purple to yellow over time is a graphical indicator of this reaction. Biliverdin occurs in the bile of amphibia and of birds, but not in normal human bile or serum.114-25-0C00500535343917033BILIVERDINE4510089CC1=C(C=C)\C(NC1=O)=C\C1=C(C)C(CCC(O)=O)=C(N1)\C=C1/N=C(/C=C2\NC(=O)C(C=C)=C2C)C(C)=C1CCC(O)=OC33H34N4O6InChI=1S/C33H34N4O6/c1-7-20-19(6)32(42)37-27(20)14-25-18(5)23(10-12-31(40)41)29(35-25)15-28-22(9-11-30(38)39)17(4)24(34-28)13-26-16(3)21(8-2)33(43)36-26/h7-8,13-15,35H,1-2,9-12H2,3-6H3,(H,36,43)(H,37,42)(H,38,39)(H,40,41)/b26-13-,27-14-,28-15-QBUVFDKTZJNUPP-BBROENKCSA-N3-(2-{[(2Z)-3-(2-carboxyethyl)-5-{[(2Z)-4-ethenyl-3-methyl-5-oxo-2,5-dihydro-1H-pyrrol-2-ylidene]methyl}-4-methyl-2H-pyrrol-2-ylidene]methyl}-5-{[(2Z)-3-ethenyl-4-methyl-5-oxo-2,5-dihydro-1H-pyrrol-2-ylidene]methyl}-4-methyl-1H-pyrrol-3-yl)propanoic acid582.657582.247834831-4.655biliverdine0-2FDB022366Biliverdin ix;1,3,6,7-tetramethyl-4,5-dicarboxyethyl-2,8-divinylbilenone;Biliverdin ix alpha;Biliverdine;Dehydrobilirubin;Protobiliverdin ix;UteroverdinePW_C000797Bilvdn37732705116078610132122044124124598118126197299721NADHMDB0000902NAD (or Nicotinamide adenine dinucleotide) is used extensively in glycolysis and the citric acid cycle of cellular respiration. The reducing potential stored in NADH can be converted to ATP through the electron transport chain or used for anabolic metabolism. ATP "energy" is necessary for an organism to live. Green plants obtain ATP through photosynthesis, while other organisms obtain it by cellular respiration. (wikipedia). Nicotinamide adenine dinucleotide is a A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed).53-84-9C00003589315846NAD5682NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP([O-])(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1OC21H27N7O14P2InChI=1S/C21H27N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1-4,7-8,10-11,13-16,20-21,29-32H,5-6H2,(H5-,22,23,24,25,33,34,35,36,37)/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BAWFJGJZGIEFAR-NNYOXOHSSA-N1-[(2R,3R,4S,5R)-5-({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl phosphono}oxy)(hydroxy)phosphoryl]oxy}methyl)-3,4-dihydroxyoxolan-2-yl]-3-(C-hydroxycarbonimidoyl)-1lambda5-pyridin-1-ylium663.4251663.109121631-2.5281-[(2R,3R,4S,5R)-5-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl phosphono}oxy(hydroxy)phosphoryl)oxy]methyl}-3,4-dihydroxyoxolan-2-yl]-3-(C-hydroxycarbonimidoyl)-1lambda5-pyridin-1-ylium0-1FDB0223093-carbamoyl-1-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;3-carbamoyl-1-beta-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-beta-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;Adenine-nicotinamide dinucleotide;Co-i;Codehydrase i;Codehydrogenase i;Coenzyme i;Cozymase;Cozymase i;Diphosphopyridine nucleotide;Diphosphopyridine nucleotide oxidized;Endopride;Nad trihydrate;Nad-oxidized;Nicotinamide adenine dinucleotide;Nicotinamide adenine dinucleotide oxidized;Nicotinamide dinucleotide;Nicotineamide adenine dinucleotide;Oxidized diphosphopyridine nucleotide;Pyridine nucleotide diphosphate;[(3s,2r,4r,5r)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl {[(3s,2r,4r,5r)-5-(3-carbamoylpyridyl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxyphosphoryl) hydrogen phosphate;[adenylate-32-p]-nad;Beta-diphosphopyridine nucleotide;Beta-nad;Beta-nicotinamide adenine dinucleotide;Beta-nicotinamide adenine dinucleotide trihydrate;Dpn;Nad;Nad+;Nadide;B-nad;β-nadPW_C000721NAD1404150335386511011142113443127351466542229492779172835293107948071848131848192849026496031516795523810353341115360112546912354821255590135561011856961005738108582714159121475942151602415560721576076161638516469178677211768901607012188709716371742057197206740519874592228241226835922590852241181921612322249130062981301830013256223424043224261931577104132771201337720913477370331776503367766733477702332777091307791511377983347784063568000636880690119938251241105523881127501661128539411992912211995240612017140712083441912098440812115942512124212612125942912181738312261438412274212012313044712314113612341945512354937412373146012381244312382946412437039812518712112531929712534247912553048112580629912582549012592448212651549512676548012688550112727850712738350212808939012836039112842839514075718538BilirubinHMDB0000054Bilirubin is a bile pigment that is a degradation product of heme. In particular, bilirubin is a yellow breakdown product of normal heme catabolism. Its levels are elevated in certain diseases and it is responsible for the yellow colour of bruises. Bilirubin is an excretion product and the body does not control its levels. Bilirubin levels reflect the balance between production and excretion. Thus, there is no "normal" level of bilirubin. Bilirubin consists of an open chain of four pyrroles (tetrapyrrole). In contrast, the heme molecule is a closed ring of four pyrroles, called porphyrin (Wikipedia). Bilirubin is found to be associated with Crigler-Najjar syndrome type I, which is an inborn error of metabolism.635-65-4C00486528035216990BILIRUBIN4444055CC1=C(C=C)\C(NC1=O)=C\C1=C(C)C(CCC(O)=O)=C(CC2=C(CCC(O)=O)C(C)=C(N2)\C=C2/NC(=O)C(C=C)=C2C)N1C33H36N4O6InChI=1S/C33H36N4O6/c1-7-20-19(6)32(42)37-27(20)14-25-18(5)23(10-12-31(40)41)29(35-25)15-28-22(9-11-30(38)39)17(4)24(34-28)13-26-16(3)21(8-2)33(43)36-26/h7-8,13-14,34-35H,1-2,9-12,15H2,3-6H3,(H,36,43)(H,37,42)(H,38,39)(H,40,41)/b26-13-,27-14-BPYKTIZUTYGOLE-IFADSCNNSA-N3-(2-{[3-(2-carboxyethyl)-5-{[(2Z)-4-ethenyl-3-methyl-5-oxo-2,5-dihydro-1H-pyrrol-2-ylidene]methyl}-4-methyl-1H-pyrrol-2-yl]methyl}-5-{[(2Z)-3-ethenyl-4-methyl-5-oxo-2,5-dihydro-1H-pyrrol-2-ylidene]methyl}-4-methyl-1H-pyrrol-3-yl)propanoic acid584.6621584.263484904-4.786bilirubin0-2FDB021886(4z,15z)-bilirubin ixa;(z,z)-bilirubin ixa;1,10,19,22,23,24-hexahydro-2,7,13,17-tetramethyl-1,19-dioxo-3,18-divinyl-biline-8,12-dipropionate;1,10,19,22,23,24-hexahydro-2,7,13,17-tetramethyl-1,19-dioxo-3,18-divinyl-biline-8,12-dipropionic acid;2,17-diethenyl-1,10,19,22,23,24-hexahydro-3,7,13,18-tetramethyl-1,19-dioxo-21h-biline-8,12-dipropanoate;2,17-diethenyl-1,10,19,22,23,24-hexahydro-3,7,13,18-tetramethyl-1,19-dioxo-21h-biline-8,12-dipropanoic acid;3-(2-((3-(2-carboxyethyl)-4-methyl-5-((3-methyl-5-oxo-4-vinyl-1,5-dihydro-2h-pyrrol-2-ylidene)methyl)-1h-pyrrol-2-yl)methyl)-4-methyl-5-((4-methyl-5-oxo-3-vinyl-1,5-dihydro-2h-pyrrol-2-ylidene)methyl)-1h-pyrrol-3-yl)propanoate;3-(2-((3-(2-carboxyethyl)-4-methyl-5-((3-methyl-5-oxo-4-vinyl-1,5-dihydro-2h-pyrrol-2-ylidene)methyl)-1h-pyrrol-2-yl)methyl)-4-methyl-5-((4-methyl-5-oxo-3-vinyl-1,5-dihydro-2h-pyrrol-2-ylidene)methyl)-1h-pyrrol-3-yl)propanoic acid;3-(2-((3-(2-carboxyethyl)-4-methyl-5-[(z)-(3-methyl-5-oxo-4-vinyl-1,5-dihydro-2h-pyrrol-2-ylidene)methyl]-1h-pyrrol-2-yl)methyl)-4-methyl-5-[(z)-(4-methyl-5-oxo-3-vinyl-1,5-dihydro-2h-pyrrol-2-ylidene;3-[2-[[3-(2-carboxyethyl)-5-[(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1h-pyrrol-2-yl]methyl]-5-[(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1h-pyrrol-3-yl]propanoate;3-[2-[[3-(2-carboxyethyl)-5-[(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1h-pyrrol-2-yl]methyl]-5-[(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1h-pyrrol-3-yl]propanoic acid;3-[2-[[3-(2-carboxyethyl)-5-[(z)-(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1h-pyrrol-2-yl]methyl]-5-[(z)-(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1h-pyrrol-3-yl]propanoate;3-[2-[[3-(2-carboxyethyl)-5-[(z)-(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1h-pyrrol-2-yl]methyl]-5-[(z)-(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1h-pyrrol-3-yl]propanoic acid;Bilirubin;Bilirubin ix-alpha;Cholerythrin;Hematoidin;1,10,19,22,23,24-hexahydro-2,7,13,17-tetramethyl-1,19-dioxo-3,18-divinylbiline-8,12-dipropionic acid;2,7,13,17-tetramethyl-1,19-dioxo-3,18-divinyl-1,10,19,22,23,24-hexahydro-21h-biline-8,12-dipropanoic acid;8,12-bis(2-carboxyethyl)-2,7,13,17-tetramethyl-3,18-divinylbiladiene-ac-1,19(21h,24h)-dione;Bilirubin ixalpha;1,10,19,22,23,24-hexahydro-2,7,13,17-tetramethyl-1,19-dioxo-3,18-divinylbiline-8,12-dipropionate;2,7,13,17-tetramethyl-1,19-dioxo-3,18-divinyl-1,10,19,22,23,24-hexahydro-21h-biline-8,12-dipropanoatePW_C000038BilRubn377827054160786131321220471241246011181262002991144NADHHMDB0001487NADH 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_C001144NADH14341533490864810111521275514695422304927811728362931099480618481218482128490464959315169955240103533211153581125466123547912555931355698100573710858291415915147594515160271556079161638716472178677111768931607011188709916371722057195206746222282442268360225908622411809198118212161232024913003298130153001325522342403322426183157710713277123133772081347737133177651336776683347770033277707130779171137798634780009368806911199382212411054938811285494115838118119955406120172407120378122120986408121162425121244126121693429121818383122616384122745120123127447123138136123551374123734460123814443124242464124371398125189121125345479125531481125762297125808299125926482126516495126767480126888501127385502128090390128362391128429395140759185746Uridine diphosphate glucuronic acidHMDB0000935Uridine diphosphate glucuronic acid is a nucleoside diphosphate sugar which serves as a source of glucuronic acid for polysaccharide biosynthesis. It may also be epimerized to UDP Iduronic acid, which donates Iduronic acid to polysaccharides. In animals, UDP glucuronic acid is used for formation of many glucosiduronides with various aglycones. The transfer of glucuronic acid from UDP-alpha-D-glucuronic acid onto a terminal galactose residue is done by beta1,3-glucuronosyltransferases, responsible for the completion of the protein-glycosaminoglycan linkage region of proteoglycans and of the HNK1 epitope of glycoproteins and glycolipids. In humans the enzyme galactose-beta-1,3-glucuronosyltransferase I completes the synthesis of the common linker region of glycosaminoglycans (GAGs) by transferring glucuronic acid (GlcA) onto the terminal galactose of the glycopeptide primer of proteoglycans. The GAG chains of proteoglycans regulate major biological processes such as cell proliferation and recognition, extracellular matrix deposition, and morphogenesis. (PMID: 16815917).2616-64-0C001671747317200UDP-GLUCURONATE16522O[C@@H]1[C@@H](COP(O)(=O)OP(O)(=O)O[C@H]2O[C@@H]([C@@H](O)[C@H](O)[C@H]2O)C(O)=O)O[C@H]([C@@H]1O)N1C=CC(=O)NC1=OC15H22N2O18P2InChI=1S/C15H22N2O18P2/c18-5-1-2-17(15(26)16-5)12-9(22)6(19)4(32-12)3-31-36(27,28)35-37(29,30)34-14-10(23)7(20)8(21)11(33-14)13(24)25/h1-2,4,6-12,14,19-23H,3H2,(H,24,25)(H,27,28)(H,29,30)(H,16,18,26)/t4-,6-,7+,8+,9-,10-,11+,12-,14-/m1/s1HDYANYHVCAPMJV-LXQIFKJMSA-N(2S,3S,4S,5R,6R)-6-({[({[(2R,3S,4R,5R)-5-(2,4-dioxo-1,2,3,4-tetrahydropyrimidin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)-3,4,5-trihydroxyoxane-2-carboxylic acid580.2853580.034284934-1.519udp-α-D-glucuronic acid0-3FDB022325Glucopyranuronic acid 1-ester with uridine 5'-pyrophosphate;Udp glucuronate;Udp glucuronic acid;Udp-d-glucuronate;Udp-d-glucuronic acid;Udp-glcua;Udp-glucuronate;Udp-glucuronic acid;Udp-alpha-d-glucuronate;Udp-alpha-delta-glucuronate;Udp-delta-glucuronate;Udp-delta-glucuronic acid;Udpga;Udpglucuronate;Uga;Uridine 5'-[3-(d-glucopyranosyloxyuronic acid) dihydrogen diphosphate];Uridine 5'-diphospho-a-d-glucuronate;Uridine 5'-diphospho-a-d-glucuronic acid;Uridine 5'-diphospho-alpha-delta-glucuronate;Uridine 5'-diphospho-alpha-delta-glucuronic acid;Uridine 5'-diphospho-glucuronic acid;Uridine 5'-diphosphoglucuronate;Uridine 5'-diphosphoglucuronic acid;Uridine diphosphate glucuronate;Uridine diphosphate glucuronic acid;Uridine diphosphate-glucuronate;Uridine diphospho-d-glucuronate;Uridine diphospho-d-glucuronic acid;Uridine diphospho-delta-glucuronate;Uridine diphospho-delta-glucuronic acid;Uridine diphosphoglucuronate;Uridine diphosphoglucuronic acid;Uridine pyrophosphoglucuronate;Uridine pyrophosphoglucuronic acid;Uridinediphosphoglucuronic acid;A-d-glucopyranuronic acid 1->5'-ester with uridine 5'-(trihydrogen pyrophosphate);A-d-glucopyranuronic acid ester with uridine 5'-pyrophosphate;Alpha-d-glucopyranuronic acid 1-p'-ester with uridine 5'-(trihydrogen diphosphate);Alpha-delta-glucopyranuronic acid 1->5'-ester with uridine 5'-(trihydrogen pyrophosphate);Alpha-delta-glucopyranuronic acid 1-p'-ester with uridine 5'-(trihydrogen diphosphate);Alpha-delta-glucopyranuronic acid ester with uridine 5'-pyrophosphate;Uridine-5'-diphosphate-glucuronic acid;Udp-a-d-glucuronate;Udp-a-d-glucuronic acid;Udp-α-d-glucuronate;Udp-α-d-glucuronic acid;Udpglucuronic acid;Uridine diphosphoric acid glucuronic acid;Uridine-5'-diphosphate-glucuronate;Uridine-5'-diphosphoric acid-glucuronic acidPW_C000746UDPGlcA1129822194923032235194479184516104844364944925176955525130583214170551607735833178403111786151321208301221220491241223383831234151351246031181248893981258212971262012991265064951272742051280783901838Bilirubin diglucuronideHMDB0003325Bilirubin diglucuronide is a water soluble version of bilirubin. Conversion of bilirubin IX-alpha to a water-soluble form by disruption of the hydrogen bonds is essential for elimination by the liver and kidney. This is achieved by glucuronic acid conjugation of the propionic acid side chains of bilirubin. Bilirubin glucuronides are water-soluble and are readily excreted in bile. Bilirubin is primarily excreted in normal human bile as diglucuronide; unconjugated bilirubin accounts for only 1-4% of pigments in normal bile.17459-92-6C05787545991118392BILIRUBIN-BISGLUCURONOSIDE4573640CC1=C(C=C)\C(NC1=O)=C\C1=C(C)C(CCC(=O)O[C@@H]2O[C@@H]([C@@H](O)[C@H](O)[C@H]2O)C(O)=O)=C(CC2=C(CCC(=O)O[C@@H]3O[C@@H]([C@@H](O)[C@H](O)[C@H]3O)C(O)=O)C(C)=C(N2)\C=C2/NC(=O)C(C=C)=C2C)N1C45H52N4O18InChI=1S/C45H52N4O18/c1-7-20-19(6)40(58)49-27(20)14-25-18(5)23(10-12-31(51)65-45-37(57)33(53)35(55)39(67-45)43(62)63)29(47-25)15-28-22(17(4)24(46-28)13-26-16(3)21(8-2)41(59)48-26)9-11-30(50)64-44-36(56)32(52)34(54)38(66-44)42(60)61/h7-8,13-14,32-39,44-47,52-57H,1-2,9-12,15H2,3-6H3,(H,48,59)(H,49,58)(H,60,61)(H,62,63)/b26-13-,27-14-/t32-,33-,34-,35-,36+,37+,38-,39-,44+,45+/m0/s1SCJLWMXOOYZBTH-BTVQFETGSA-N(2S,3S,4S,5R,6S)-6-{[3-(2-{[3-(3-{[(2S,3R,4S,5S,6S)-6-carboxy-3,4,5-trihydroxyoxan-2-yl]oxy}-3-oxopropyl)-5-{[(2E)-3-ethenyl-5-hydroxy-4-methyl-2H-pyrrol-2-ylidene]methyl}-4-methyl-1H-pyrrol-2-yl]methyl}-5-{[(2E)-4-ethenyl-5-hydroxy-3-methyl-2H-pyrrol-2-ylidene]methyl}-4-methyl-1H-pyrrol-3-yl)propanoyl]oxy}-3,4,5-trihydroxyoxane-2-carboxylic acid936.921936.32766085-3.8712(2S,3S,4S,5R,6S)-6-{[3-(2-{[3-(3-{[(2S,3R,4S,5S,6S)-6-carboxy-3,4,5-trihydroxyoxan-2-yl]oxy}-3-oxopropyl)-5-{[(2E)-3-ethenyl-5-hydroxy-4-methylpyrrol-2-ylidene]methyl}-4-methyl-1H-pyrrol-2-yl]methyl}-5-{[(2E)-4-ethenyl-5-hydroxy-3-methylpyrrol-2-ylidene]methyl}-4-methyl-1H-pyrrol-3-yl)propanoyl]oxy}-3,4,5-trihydroxyoxane-2-carboxylic acid0-2FDB023140(2s,3s,4s,5r,6s)-6-[3-[2-[[3-[2-[(2s,3r,4s,5s,6s)-6-carboxy-3,4,5-trihydroxy-oxan-2-yl]oxycarbonylethyl]-5-[(e)-(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1h-pyrrol-2-yl]methyl]-5-[(e)-(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1h-pyrrol-3-yl]propanoyloxy]-3,4,5-trihydroxy-oxane-2-carboxylate;(2s,3s,4s,5r,6s)-6-[3-[2-[[3-[2-[(2s,3r,4s,5s,6s)-6-carboxy-3,4,5-trihydroxy-oxan-2-yl]oxycarbonylethyl]-5-[(e)-(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1h-pyrrol-2-yl]methyl]-5-[(e)-(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1h-pyrrol-3-yl]propanoyloxy]-3,4,5-trihydroxy-oxane-2-carboxylic acid;Bilirubin beta-diglucuronide;Bilirubin-bisglucuronoside;Bis(glucosyluronic acid)bilirubin;Bilirubin b-diglucuronide;Bilirubin β-diglucuronidePW_C001838BRubnDG3780265811056582107658310870561604268231542685320426863187861613280384304803939880394310122050124124604118126202299201Uridine 5'-diphosphateHMDB0000295Uridine 5'-diphosphate is a uracil nucleotide containing a pyrophosphate group esterified to C5 of the sugar moiety. UDP is an important extracellular pyrimidine signaling molecule that mediates diverse biological effects via P1 and P2 purinergic receptors, such as the uptake of thymidine and proliferation of gliomas. (PMID: 14558596). UDP induces intracellular Ca(2+) responses and oscillations in HeLa cells, due to the activation of P2Ys (G-protein coupled ATP receptors). (PMID: 1257952).58-98-0C00015603117659UDP5809DB03435O[C@H]1[C@@H](O)[C@@H](O[C@@H]1COP(O)(=O)OP(O)(O)=O)N1C=CC(=O)NC1=OC9H14N2O12P2InChI=1S/C9H14N2O12P2/c12-5-1-2-11(9(15)10-5)8-7(14)6(13)4(22-8)3-21-25(19,20)23-24(16,17)18/h1-2,4,6-8,13-14H,3H2,(H,19,20)(H,10,12,15)(H2,16,17,18)/t4-,6-,7-,8-/m1/s1XCCTYIAWTASOJW-XVFCMESISA-N[({[(2R,3S,4R,5R)-5-(2,4-dioxo-1,2,3,4-tetrahydropyrimidin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]phosphonic acid404.1612404.002196946-1.666UDP0-2FDB0075095'-udp;Udp;Uridine 5'-diphosphate;Uridine 5'-pyrophosphate;Uridine 5'-pyrophosphorate;Uridine 5'-pyrophosphoric acid;Uridine diphosphate;Uridine pyrophosphate;Uridine 5'-diphosphoric acid;Uridine diphosphoric acidPW_C000201UDP410815172154543219749235293115294463184518104846364946925178955527130583414170571607246213724821472872108433151119131641261522577304111773603317792433678255132782643561203151221212524291213651241213754191221013831223781251229701351238224641239241181239344551246523981249321361256432971260382991260484901262574951274903881275005071278203902078UrobilinogenHMDB0004158Urobilinogen is a colourless product of bilirubin reduction. It is formed in the intestines by bacterial action. Some urobilinogen is reabsorbed, taken up by the hepatocytes into the circulation and excreted by the kidney. This constitutes the normal "intrahepatic urobilinogen cycle". Increased amounts of bilirubin are formed in haemolysis which generates increased urobilinogen in the gut. In liver disease (such as hepatitis) the intrahepatic urobilinogen cycle is inhibited also increasing urobilinogen levels. Urobilinogen is converted to the yellow pigmented urobilin apparent in urine. The urobilinogen remaining in the intestine (stercobilinogen) is oxidized to brown stercobilin which gives the feces their characteristic color. In biliary obstruction, below normal amounts of conjugated bilirubin reach the intestine for conversion to urobilinogen. With limited urobilinogen available for reabsorption and excretion, the amount of urobilin found in the urine is low.17208-65-0C05791440784389649CCC1=C(C)C(=O)NC1CC1=C(C)C(CCC(O)=O)=C(CC2=C(CCC(O)=O)C(C)=C(CC3NC(=O)C(C=C)=C3C)N2)N1C33H42N4O6InChI=1S/C33H42N4O6/c1-7-20-19(6)32(42)37-27(20)14-25-18(5)23(10-12-31(40)41)29(35-25)15-28-22(9-11-30(38)39)17(4)24(34-28)13-26-16(3)21(8-2)33(43)36-26/h8,26-27,34-35H,2,7,9-15H2,1,3-6H3,(H,36,43)(H,37,42)(H,38,39)(H,40,41)KSQFFJKKJAEKTB-UHFFFAOYSA-N3-(2-{[3-(2-carboxyethyl)-5-[(4-ethenyl-3-methyl-5-oxo-2,5-dihydro-1H-pyrrol-2-yl)methyl]-4-methyl-1H-pyrrol-2-yl]methyl}-5-[(3-ethyl-4-methyl-5-oxo-2,5-dihydro-1H-pyrrol-2-yl)methyl]-4-methyl-1H-pyrrol-3-yl)propanoic acid590.7098590.310435096-4.4663-(2-{[3-(2-carboxyethyl)-5-[(4-ethenyl-3-methyl-5-oxo-1,2-dihydropyrrol-2-yl)methyl]-4-methyl-1H-pyrrol-2-yl]methyl}-5-[(3-ethyl-4-methyl-5-oxo-1,2-dihydropyrrol-2-yl)methyl]-4-methyl-1H-pyrrol-3-yl)propanoic acid0-2FDB023323D-urobilinogen;UrobilinogenPW_C002078Urbilgn378127058160786181321220521241246061181262042991050Carbon monoxideHMDB0001361Carbon monoxide, with the chemical formula CO, is a colorless, odorless, and tasteless gas. It is the product of the incomplete combustion of carbon-containing compounds, notably in internal-combustion engines. It consists of one carbon atom covalently bonded to one oxygen atom. It is a gas at room temperature. Carbon monoxide is a significantly toxic gas and is the most common type of fatal poisoning in many countries. Exposures can lead to significant toxicity of the central nervous system and heart. Carbon monoxide has a higher diffusion coefficient compared to oxygen and the only enzyme in the human body that produces carbon monoxide is heme oxygenase which is located in all cells and breaks down heme. Because it has a higher diffusion coefficient than oxygen the body easily gets rid of any CO made. When CO is not ventilated it binds to hemoglobin, which is the principal oxygen-carrying compound in blood; this produces a compound known as carboxyhemoglobin. The traditional belief is that carbon monoxide toxicity arises from the formation of carboxyhemoglobin, which decreases the oxygen-carrying capacity of the blood and inhibits the transport, delivery, and utilization of oxygen by the body. The affinity between hemoglobin and carbon monoxide is approximately 230 times stronger than the affinity between hemoglobin and oxygen so hemoglobin binds to carbon monoxide in preference to oxygen. Following poisoning, long-term sequelae often occur. Carbon monoxide can also have severe effects on the fetus of a pregnant woman. Despite its serious toxicity, CO is extremely useful and underpins much modern technology, being a precursor to a myriad of useful - even life-saving - products. Carbon monoxide, though thought of as a pollutant today, has always been present in the atmosphere, chiefly as a product of volcanic activity. It occurs dissolved in molten volcanic rock at high pressures in the earth's mantle. Carbon monoxide contents of volcanic gases vary from less than 0.01% to as much as 2% depending on the volcano. It also occurs naturally in bushfires. Because natural sources of carbon monoxide are so variable from year to year, it is extremely difficult to accurately measure natural emissions of the gas. (wikipedia).630-08-0C0023728117245275[C-]#[O+]COInChI=1S/CO/c1-2UGFAIRIUMAVXCW-UHFFFAOYSA-N28.0127.99491462-1.1100FDB022578Co;Carbon monoxide;[co];C#oPW_C001050CO3774270531607861113212204512412459911812619829920AlcoholCompound42910CompoundPW_EC00002030879ChEBIAlcohol55AH2CompoundPW_EC00005517499ChEBIAH266A CompoundPW_EC00006613193ChEBIA 225-aminolevulinate synthase, nonspecific, mitochondrialP13196HMDBP00022ALAS13p21.1AB06332212.3.1.3734603367121428344997Delta-aminolevulinic acid dehydrataseP13716Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen.
HMDBP01060ALAD9q33.1M1392814.2.1.2436742351Porphobilinogen deaminaseP08397Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
HMDBP00358HMBS11q23.3X0480812.5.1.6136782350Uroporphyrinogen-III synthaseP10746Catalyzes cyclization of the linear tetrapyrrole, hydroxymethylbilane, to the macrocyclic uroporphyrinogen III, the branch point for the various sub-pathways leading to the wide diversity of porphyrins. Porphyrins act as cofactors for a multitude of enzymes that perform a variety of processes within the cell such as methionine synthesis (vitamin B12) or oxygen transport (heme).
HMDBP00357UROS10q25.2-q26.3AL36017614.2.1.7536802244Uroporphyrinogen decarboxylaseP06132Catalyzes the decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III.
HMDBP00250UROD1p34BT00673714.1.1.3737002245Coproporphyrinogen-III oxidase, mitochondrialP36551Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of rings A and B of coproporphyrinogen III.
HMDBP00251CPOX3q12Z3480711.3.3.33715314284042958Protoporphyrinogen oxidaseP50336Catalyzes the 6-electron oxidation of protoporphyrinogen-IX to form protoporphyrin-IX.
HMDBP01021PPOX1q22AL59071411.3.3.43719314284117453Ferritin, mitochondrialQ8N4E7Stores iron in a soluble, non-toxic, readily available form. Important for iron homeostasis. Has ferroxidase activity. Iron is taken up in the ferrous form and deposited as ferric hydroxides after oxidation.
HMDBP00470FTMT5q21.3BC03441911.16.3.137333995Ferrochelatase, mitochondrialP22830Catalyzes the ferrous insertion into protoporphyrin IX.
HMDBP01058FECH18q21.3D0072614.99.1.1373233743280969171512Protoheme IX farnesyltransferase, mitochondrialQ12887Converts protoheme IX and farnesyl diphosphate to heme O (By similarity).
HMDBP01631COX10AC00538912.5.1.-3746280970178097133850Cytochrome c oxidase assembly protein COX15 homologQ7KZN9May be involved in the biosynthesis of heme A.
HMDBP08634COX15AL133353137523193Heme oxygenase 1P09601Heme oxygenase cleaves the heme ring at the alpha methene bridge to form biliverdin. Biliverdin is subsequently converted to bilirubin by biliverdin reductase. Under physiological conditions, the activity of heme oxygenase is highest in the spleen, where senescent erythrocytes are sequestrated and destroyed.
HMDBP00198HMOX122q13.1CR45650511.14.99.337752134627814225723664Biliverdin reductase AP53004Reduces the gamma-methene bridge of the open tetrapyrrole, biliverdin IX alpha, to bilirubin with the concomitant oxidation of a NADH or NADPH cofactor.
HMDBP00700BLVRA7p13AC00498511.3.1.24377921346288473UDP-glucuronosyltransferase 2B11O75310UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds.
HMDBP00491UGT2B114q13.2BC06944112.4.1.17356101433222214923539365729446418144194973784Beta-glucuronidaseP08236Plays an important role in the degradation of dermatan and keratan sulfates.
HMDBP00839GUSB7q21.11S7246213.2.1.3123509378222909Feline leukemia virus subgroup C receptor-related protein 2Q9UPI3Transporter specific for a calcium-chelator complex, important for growth and calcium metabolismHMDBP07688FLVCR214q24.3AK29700218395-aminolevulinate synthase, nonspecific, mitochondrial1PW_P00083996422135411481905Delta-aminolevulinic acid dehydratase1PW_P0009051030997136697958906Porphobilinogen deaminase1PW_P00090610313511907Uroporphyrinogen-III synthase1PW_P00090710323501908Uroporphyrinogen decarboxylase1PW_P00090810332441909Coproporphyrinogen-III oxidase, mitochondrial1PW_P00090910342451911Protoporphyrinogen oxidase1PW_P000911103695813689641913Ferritin, mitochondrial1PW_P00091310384531372405581912Ferrochelatase, mitochondrial1PW_P00091210379951369405581916Protoheme IX farnesyltransferase, mitochondrial1PW_P000916104115121918Cytochrome c oxidase assembly protein COX15 homolog1PW_P000918104338501919Heme oxygenase 11PW_P00091910441931920Biliverdin reductase A1PW_P000920104566413739795142UDP-glucuronosyltransferase 2B111PW_P000042434731604Beta-glucuronidase1PW_P000604648784113603FLVCR21PW_P0136032347529091871falsePW_R001871Right6909781Compoundfalse69108081Compoundtrue69118941Compoundfalse691213161Compoundtrue16998392.3.1.371872falsePW_R001872Right69138941Compoundfalse69141661Compoundfalse691514202Compoundtrue17009054.2.1.241874falsePW_R001874Right69181664Compoundfalse691914201Compoundtrue69208871Compoundfalse6921354Compoundtrue17029062.5.1.61306falsePW_R000306Right12908871Compoundfalse12918541Compoundfalse129214201Compoundtrue17069074.2.1.751877PW_R001877Right69288541Compoundfalse69297321Compoundfalse1879PW_R001879Right69328871Compoundfalse693314961Compoundfalse1880falsePW_R001880Right693414961Compoundfalse693514511Compoundfalse693613164Compoundtrue17089084.1.1.371883PW_R001883Right694114511Compoundfalse69425021Compoundfalse1884PW_R001884Right694314961Compoundfalse69447471Compoundfalse1885falsePW_R001885Right69458541Compoundfalse69469741Compoundfalse694713164Compoundtrue17099084.1.1.371886PW_R001886Right69489741Compoundfalse69494451Compoundfalse1889falsePW_R001889Right69549741Compoundfalse695510651Compoundtrue695610512Compoundtrue69578631Compoundfalse695813162Compoundtrue695914202Compoundtrue17109091.3.3.31897falsePW_R001897Left69785444Compoundfalse697910514Compoundtrue698010651Compoundtrue698181334Compoundfalse698214202Compoundtrue17189131.16.3.11896falsePW_R001896Right69741631Compoundfalse69755441Compoundfalse697617991Compoundfalse697710512Compoundtrue17179124.99.1.11900falsePW_R001900Right698717991Compoundfalse69887681Compoundtrue69899001Compoundfalse17269162.5.1.-1911falsePW_R001911Right70109001Compoundfalse701130031Compoundfalse17419181924falsePW_R001924Right70427971Compoundfalse70437211Compoundtrue7044381Compoundfalse704511441Compoundtrue17669201.3.1.241925falsePW_R001925Right7046381Compoundfalse70477461Compoundtrue704818381Compoundfalse70492011Compoundtrue1767422.4.1.171932falsePW_R001932Right706218381Compoundfalse706314201Compoundtrue706420781Compoundfalse7065201ElementCollectiontrue17836043.2.1.311892falsePW_R001892Right69648631Compoundfalse696510653Compoundtrue69661631Compoundfalse696717833Compoundtrue17169111.3.3.41921falsePW_R001921Right703017991Compoundfalse7031553ElementCollectiontrue703210653Compoundtrue70337971Compoundfalse70345441Compoundtrue703510501Compoundtrue7036663ElementCollectiontrue703714203Compoundtrue17659191.14.99.3178PW_T0001782109741Compound23Right1220PW_T00122014631631Compound174Left10411PW_T0104111078317991Compound38Right1577136032019-08-08T11:35:51-06:002019-08-08T11:35:51-06:003728978282false48538510regular3002807290808281false79524010regular2001907291894282false123538510regular30028072921316252false111634910regular78787293114829false98547010regular100357296166282false185038510regular30028072971420249false176537510regular78787298979529false163548010regular1002573031420249false215537510regular78787304887282false249038310regular300280730535263false239136910regular78787306854282false249090310regular30028073071420249false271081310regular78787308732282false204090310regular30028073141496282false294038310regular30028073181451282false359538110regular30028073191316252false349139210regular78787333502282false404538110regular3002807334747282false294090310regular3002807350974282false2488144310regular30028073511316252false2719133910regular78787356445282false2943144310regular3002807361974382false1963144310regular30028073641065365false1963173910regular787873651051355false2176173910regular78787366863382false1964203810regular30028073671316352false1960194410regular787873681420349false2177194310regular787873761065365false1657231410regular787873781783356false1657252410regular7878737996439false1786242319regular100257401163482false1420261010regular3002807409544381false1190283510regular20019074131051355false1106277010regular787874141065365false1106300810regular787874158133381false655283310regular20019074161420349false875277310regular787874361799382false1423316010regular30028074371051255false1694304110regular787874384055829false1790296520regular100257443768381false1203339510regular2001907444900382false1433366010regular30028074563003382false1433413510regular3002807534797282false3330316410regular3002807542721259false3670320410regular5030754338282false3995316410regular30028075441144260false3900320410regular50307545979529false3760325910regular100257546746281false4300297910regular20020075471838282false3995245410regular3002807548201281false4300271910regular20019075491420249false3855244410regular787875502078282false3250245410regular30028015902801631782false1965261010regular30028026916811799882false2458316010regular30028031668781065265false2829313710regular78783166879544281false3205354610regular20019031668801050281false3050335110regular20019031668811420249false3121313610regular787817720372false3585242912regular100902500955372false2723305012regular100902501066372false3225304912regular1009034042226false9504858subunitregular16080340699727false15804858subunitregular19085341135122false22304908subunitregular15070341635022f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C2674 1379 2640 1373 2640 1343 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false11127M2788 1583 C2818 1583 2819 1671 2843 1688 5true1811128M2943 1583 C2913 1583 2821 1584 2788 1583 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false11133M2488 1583 C2458 1583 2369 1671 2393 1688 5true1811134M2263 1583 C2293 1583 2446 1582 2488 1583 83false18trueM 1625.9903810567666 1435.5 L 1613 1443 L 1625.9903810567666 1450.5false11137M2113 1723 C2113 1753 2112 1808 2112 1838 5false1811138M2041 1778 C2090 1778 2112 1808 2112 1838 5false1811139M2176 1778 C2138 1777 2112 1808 2112 1838 5false1811140M2114 2038 C2114 2008 2112 1948 2112 1918 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false11141M2038 1983 C2074 1982 2113 1968 2112 1918 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false11142M2177 1982 C2132 1981 2112 1948 2112 1918 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false11154M1781 2378 L1781 2428 L1831 2378 z10true1811190M1190 2930 C1160 2930 1130 2929 1100 2929 5false18falsefalse11191M1145 2848 C1145 2887 1130 2929 1100 2929 5false18falsefalse11192M1145 3008 C1140 2959 1130 2929 1100 2929 5false18falsefalse11193M955 2973 C985 2973 920 2929 950 2929 5false18trueM 302.9903810567666 2645.5 L 290 2653 L 302.9903810567666 2660.5false11194M914 2851 C915 2892 920 2929 950 2929 5false18trueM 296.8176919869723 2588.791455071237 L 284 2581 L 283.6612479693498 2595.9961744142206false11219M1570 2890 C1570 2902 1570 2915 1569 2928 C1621 2930 1771 2931 1841 2930 C1840 2949 1840 2955 1840 2970 5false1811220M1390 2930 C1460 2930 1809 2929 1840 2929 C1840 2966 1839 2945 1840 2970 5false1811221M1573 3160 C1573 3144 1574 3141 1573 3125 C1644 3125 1752 3122 1841 3124 C1841 3109 1841 3062 1840 3050 5false18trueM 25.946855044164835 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25.575134323078345false11358M3630 3304 C3660 3304 3710 3304 3740 3304 5false1811359M3695 3234 C3690 3267 3710 3304 3740 3304 5false1811360M3995 3304 C3965 3304 3920 3304 3890 3304 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false11361M3925 3234 C3924 3270 3920 3304 3890 3304 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false11362M800 400 L800 450 L850 400 z10true1811363M4145 3164 C4145 3134 4145 3014 4145 2984 5false1811364M4300 3079 C4242 3079 4146 3054 4145 2984 5false1811365M4145 2734 C4145 2764 4145 2884 4145 2914 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false11366M4300 2814 C4241 2815 4144 2850 4145 2914 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false11367M3995 2594 C3965 2594 3865 2594 3835 2594 5false1811368M3894 2522 C3888 2566 3865 2594 3835 2594 5false1811369M3550 2594 C3580 2594 3665 2594 3695 2594 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false11370M3635 2529 C3634 2557 3665 2594 3695 2594 5false1811393M485 525 C455 525 410 525 380 525 5false18trueM 387.0096189432334 392.5 L 400 385 L 387.0096189432334 377.5false2170047M1965 2750 C1935 2750 1750 2750 1720 2750 83true18falsefalse2170048M1720 2750 C1750 2750 1935 2750 1965 2750 83false18trueM 1567.9903810567666 2992.5 L 1555 3000 L 1567.9903810567666 3007.5false2170049M2114 2318 C2114 2333 2114 2350 2115 2367 C2024 2369 1933 2367 1831 2369 C1831 2387 1831 2411 1831 2428 5false182170050M1735 2353 C1779 2352 1832 2359 1831 2428 5false182170051M2115 2610 C2115 2591 2115 2576 2116 2565 C2089 2564 1903 2566 1829 2566 C1830 2548 1831 2530 1831 2508 5false18trueM 718.9468550441649 1772.261556296296 L 704 1771 L 710.3808877211858 1784.5751343230784false2170052M1735 2563 C1775 2564 1830 2566 1831 2508 5false18trueM 718.9468550441649 1772.261556296296 L 704 1771 L 710.3808877211858 1784.5751343230784false3681313M1723 3300 C1753 3300 1983 3300 2013 3300 83false183681314M2458 3300 C2428 3300 2193 3300 2163 3300 83false18trueM 612.9468550441649 2583.261556296296 L 598 2582 L 604.3808877211858 2595.575134323078false4333672M2758 3300 C2788 3300 2910 3302 2940 3302 5false184333673M2868 3215 C2869 3251 2888 3302 2940 3302 5false184333674M3330 3304 C3300 3304 3120 3302 3090 3302 5false18trueM 2332.9468550441647 3208.261556296296 L 2318 3207 L 2324.380887721186 3220.575134323078false4333675M3305 3546 C3302 3370 3279 3302 3090 3302 5false18trueM 2332.9468550441647 3208.261556296296 L 2318 3207 L 2324.380887721186 3220.575134323078false4333676M3150 3351 C3151 3313 3122 3304 3090 3302 5false18trueM 2332.9468550441647 3208.261556296296 L 2318 3207 L 2324.380887721186 3220.575134323078false4333677M3160 3214 C3160 3262 3120 3302 3090 3302 5false18trueM 2332.9468550441647 3208.261556296296 L 2318 3207 L 2324.380887721186 3220.575134323078false4333678M2773 3150 C2773 3219 2826 3301 2940 3302 5false184333679M3275 3149 C3276 3202 3196 3301 3090 3302 5false18trueM 2332.9468550441647 3208.261556296296 L 2318 3207 L 2324.380887721186 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1428 C475 1378 525 1328 575 1328 C1087 1328 1751 1328 2263 1328 C2313 1328 2363 1378 2363 1428 C2363 2367 2363 3589 2363 4528 C2363 4578 2313 4628 2263 4628 C1751 4628 1087 4628 575 4628 C525 4628 475 4578 475 4528 C475 3589 475 2367 475 1428 84true61888.03300.0527M578 1517 C578 1467 628 1417 678 1417 C996 1417 1410 1417 1728 1417 C1778 1417 1828 1467 1828 1517 C1828 2392 1828 3530 1828 4405 C1828 4455 1778 4505 1728 4505 C1410 4505 996 4505 678 4505 C628 4505 578 4455 578 4405 C578 3530 578 2392 578 1517 84true61250.03088.0532M2870 3250 C2870 3200 2920 3150 2970 3150 C3000 3150 3040 3150 3070 3150 C3120 3150 3170 3200 3170 3250 C3170 3280 3170 3320 3170 3350 C3170 3400 3120 3450 3070 3450 C3040 3450 3000 3450 2970 3450 C2920 3450 2870 3400 2870 3350 C2870 3320 2870 3280 2870 3250 92true6300.0300.0536M3985 2900 C3985 2850 4035 2800 4085 2800 C4115 2800 4155 2800 4185 2800 C4235 2800 4285 2850 4285 2900 C4285 2930 4285 2970 4285 3000 C4285 3050 4235 3100 4185 3100 C4155 3100 4115 3100 4085 3100 C4035 3100 3985 3050 3985 3000 C3985 2970 3985 2930 3985 2900 92true6300.0300.0537M126 225 C126 175 176 125 226 125 C1530 125 3225 125 4529 125 C4579 125 4629 175 4629 225 C4629 1560 4629 3295 4629 4630 C4629 4680 4579 4730 4529 4730 C3225 4730 1530 4730 226 4730 C176 4730 126 4680 126 4630 C126 3295 126 1560 126 225 1true64503.04605.0405235Intracellular Space1285160202.22.220015406235Mitochondria6301260201.61.62001540715Outer mitochondrial membrane18551245202.22.21601540815Inner mitochondrial membrane13351335202.22.216015409235Microsome38702785201.31.320015410235Microsome29103110201.01.020015411235Lysosome36052645201.41.420015254102910414961348235346134#FFEBEB418573265Acute Intermittent PorphyriaAcute intermittent porphyria (AIP), also called Swedish porphyria, is a rare inborn error of metabolism (IEM) and autosomal dominant disorder of heme biosynthesis caused by a defective HMBS gene. The HMBS gene codes for the protein hydroxymethylbilane synthase (porphobilinogen deaminase) which catalyzes the synthesis of porphobilinogen into hydroxymethylbilane. This disorder is characterized by a large accumulation of 5-aminolevulinic acid or porphobilinogen in both urine and serum. Most patients are asymptomatic between attacks. Symptoms of the disorder include abdominal pain, constipation, vomiting, hypertension, muscle weakness, seizures, delirium, coma, and depression. Treatment involves undertaking a high-carbohydrate diet and, during severe attacks, a glucose 10% infusion. It is estimated that AIP affects 5.9 per 1 000 000 people.DiseasePW_X000036Context3699894CompoundIncreased100166CompoundIncreased101351ProteinMutated6898TissueDamaged6909TissueDamaged69124TissueDamaged69211TissueDamaged1131[Uniprot: P08397](http://www.uniprot.org/uniprot/P08397)36Context1132[OMIM: Entry 176000](http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=176000)36Context113313658350GOLDBERG A: Acute intermittent porphyria: a study of 50 cases. Q J Med. 1959 Apr;28(110):183-209.36Context11347866402Astrin KH, Desnick RJ: Molecular basis of acute intermittent porphyria: mutations and polymorphisms in the human hydroxymethylbilane synthase gene. Hum Mutat. 1994;4(4):243-52. doi: 10.1002/humu.1380040403.36Context1135329053Becker DM, Kramer S: The neurological manifestations of porphyria: a review. Medicine (Baltimore). 1977 Sep;56(5):411-23.36Context11362246851Beukeveld GJ, Wolthers BG, Nordmann Y, Deybach JC, Grandchamp B, Wadman SK: A retrospective study of a patient with homozygous form of acute intermittent porphyria. J Inherit Metab Dis. 1990;13(5):673-83.36Context113712372055Floderus Y, Shoolingin-Jordan PM, Harper P: Acute intermittent porphyria in Sweden. Molecular, functional and clinical consequences of some new mutations found in the porphobilinogen deaminase gene. Clin Genet. 2002 Oct;62(4):288-97.36Context27827320301372Whatley SD, Badminton MN: Acute Intermittent Porphyria 36Context279336http://www.ashg.org/2008meeting/abstracts/fulltext/f22209.htm36Context