54ContextCarbamoyl Phosphate Synthetase DeficiencyCCarbamoyl Phosphate Synthetase Deficiency, also called hyperammonemia due to carbamoyl phosphate synthetase 1 deficiency, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of the urea cycle caused by a defective CPS1 gene. The CPS1 gene codes for the protein carbamoyl phosphate synthetase I, which plays a role in the urea cycle. This disorder is characterized by a large accumulation of ammonia in the blood. Symptoms of the disorder include unusual movements, seizures, unusual sleeping or coma. Treatment with citrulline or arginine, which maintains a regular rate of protein creation. It is estimated that carbamoyl phosphate synthetase deficiency affects 1 in 800,000 individuals in Japan.
DiseasePW000191CenterPathwayVisualizationContext20641003800#000099PathwayVisualization10778Urea CycleUrea, also known as carbamide, is a waste product made by a large variety of living organisms and is the main component of urine. Urea is created in the liver, through a string of reactions that are called the Urea Cycle. This cycle is also
called the Ornithine Cycle, as well as the Krebs-Henseleit Cycle. There are some essential compounds required for the completion of this cycle, such as arginine, citrulline and ornithine. Arginine cleaves and creates urea and ornithine, and the reactions that follow see urea residue build up on ornithine, which recreates arginine and keeps the cycle going. Ornithine is transported to the mitochondrial matrix, and once there, ornithine carbamoyltransferase uses carbamoyl phosphate to create citrulline. After this, citrulline is transported to the cytosol. Once here, citrulline and aspartate team up to create argininosuccinic acid. After this, argininosuccinate lyase creates l-arginine. L-arginine finally uses arginase-1 to create ornithine again, which will be transported to the mitochondrial matrix and restart the urea cycle once more.
Metabolic167413SubPathway1117500Compound15675100SubPathway1118500Compound156767SubPathway1119500Compound156773SubPathway1120105Compound1567875SubPathway1121105Compound156791SubPathway1122105Compound1569860SubPathway1150134Compound46997SubPathway1151134Compound47003SubPathway1152134Compound470175SubPathway1153134Compound470277SubPathway1154134Compound470373SubPathway115595Compound470496SubPathway115695Compound47053SubPathway115795Compound470613SubPathway115895Compound47087SubPathway116095Compound470975SubPathway116195Compound471075SubPathway1162148Compound471113SubPathway116335Compound471291SubPathway116435Compound471375SubPathway116535Compound471428SubPathway1166140Compound871515SubPathway1167396Compound871628SubPathway1168396Compound87172SubPathway116988Compound87184SubPathway1170123Compound8135Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.78Pathway136Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.78Pathway1CellCL:00000006MyocyteCL:00001875HepatocyteCL:00001823NeuronCL:000054012AstrocyteCL:00001274CardiomyocyteCL:00007467Epithelial CellCL:00000662Platelet CL:00002338Beta cellCL:000063923T CellCL:00000841Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote24Solanum lycopersicum4081EukaryoteTomato4Arabidopsis thaliana3702EukaryoteThale cress18Saccharomyces cerevisiae4932EukaryoteYeast49Bathymodiolus platifrons220390EukaryoteDeep sea mussel23Pseudomonas aeruginosa287Prokaryote12Mus musculus10090EukaryoteMouse17Rattus norvegicus10116EukaryoteRat5Bos taurus9913EukaryoteCattle10Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm21Xenopus laevis8355EukaryoteAfrican clawed frog60Nitzschia sp.0001EukaryoteNitzschia42Bacteria2ProkaryoteBacteria19Schizosaccharomyces pombe4896Eukaryote25Escherichia coli (strain K12)83333Prokaryote29Saccharomyces cerevisiae (strain ATCC 204508 / S288c)559292EukaryoteBaker's yeast51Picea sitchensis3332EukaryoteSitka spruce5CytoplasmGO:00057373Mitochondrial MatrixGO:00057591CytosolGO:000582911Extracellular SpaceGO:000561531Periplasmic SpaceGO:00056202MitochondrionGO:000573935ChloroplastGO:000950732Inner MembraneGO:007025810Cell MembraneGO:000588613Endoplasmic ReticulumGO:000578324Mitochondrial Intermembrane SpaceGO:00057584PeroxisomeGO:00057776LysosomeGO:00057647Endoplasmic Reticulum MembraneGO:000578912Mitochondrial Inner MembraneGO:000574316Lysosomal LumenGO:004320218Melanosome MembraneGO:003316225Golgi ApparatusGO:000579414Mitochondrial Outer MembraneGO:000574120Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520215NucleusGO:000563436MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032540PeriplasmGO:004259727Peroxisome MembraneGO:000577819Sarcoplasmic ReticulumGO:00165299MuscleBTO:0000887141188Blood VesselBTO:000110274111LiverBTO:000075972924BrainBTO:000014289163Sympathetic Nervous SystemBTO:00018324Adrenal MedullaBTO:000004971825IntestineBTO:000064828StomachBTO:0001307155267Nervous SystemBTO:000148411HeartBTO:000056273106KidneyBTO:00006717182Endothelium 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W_BS000024372102PW_BS000028215114PW_BS0000213551914PW_BS0000357413PW_BS00007475251017PW_BS0000755311015PW_BS00005380231PW_BS000548393151PW_BS00017396212415PW_BS0005645511215PW_BS000055116481123PW_BS0005831197811223PW_BS000588500L-GlutamineHMDB0000641Glutamine (Gln) is one of the 20 amino acids encoded by the standard genetic code. Its side chain is an amide; it is formed by replacing a side-chain hydroxyl of glutamic acid with an amine functional group. glutamine is found in foods high in proteins, such as fish, red meat, beans, and dairy products. glutamine is a supplement that is used in weightlifting, bodybuilding, endurance and other sports, as well as by those who suffer from muscular cramps or pain particularly elderly people. The main use of glutamine within the diet of either group is as a means of replenishing the body's stores of amino acids that have been used during exercise or everyday activities. Studies which are looking into problems with excessive consumption of glutamine thus far have proved inconclusive. However, normal supplementation is healthy mainly because glutamine is supposed to be supplemented after prolonged periods of exercise (for example, a workout or exercise in which amino acids are required for use) and replenishes amino acid stores; this being the main reason glutamine is recommended during fasting or for people who suffer from physical trauma, immune deficiencies, or cancer. There is a significant body of evidence that links glutamine-enriched diets with intestinal effects; aiding maintenance of gut barrier function, intestinal cell proliferation and differentiation, as well as generally reducing septic morbidity and the symptoms of Irritable Bowel Syndrome. The reason for such "cleansing" properties is thought to stem from the fact that the intestinal extraction rate of glutamine is higher than that for other amino acids, and is therefore thought to be the most viable option when attempting to alleviate conditions relating to the gastrointestinal tract. These conditions were discovered after comparing plasma concentration within the gut between glutamine-enriched and non glutamine-enriched diets. However, even though glutamine is thought to have "cleansing" properties and effects, it is unknown to what extent glutamine has clinical benefits, due to the varied concentrations of glutamine in varieties of food. It is also known that glutamine has various effects in reducing healing time after operations. Hospital waiting times after abdominal surgery are reduced by providing parenteral nutrition regimens containing amounts of glutamine to patients. Clinical trials have revealed that patients on supplementation regimes containing glutamine have improved nitrogen balances, generation of cysteinyl-leukotrienes from polymorphonuclear neutrophil granulocytes and improved lymphocyte recovery and intestinal permeability (in postoperative patients) - in comparison to those who had no glutamine within their dietary regime; all without any side-effects. (http://en.wikipedia.org/wiki/glutamine).56-85-9C00064596118050GLN5746DB00130N[C@@H](CCC(N)=O)C(O)=OC5H10N2O3InChI=1S/C5H10N2O3/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H2,7,8)(H,9,10)/t3-/m0/s1ZDXPYRJPNDTMRX-VKHMYHEASA-N(2S)-2-amino-4-carbamoylbutanoic acid146.1445146.069142196-0.173L-glutamine00FDB012164(2s)-2,5-diamino-5-oxopentanoate;(2s)-2,5-diamino-5-oxopentanoic acid;(2s)-2-amino-4-carbamoylbutanoate;(2s)-2-amino-4-carbamoylbutanoic acid;(s)-2,5-diamino-5-oxopentanoate;(s)-2,5-diamino-5-oxopentanoic acid;2-aminoglutaramic acid;Cebrogen;Glavamin;Glumin;Glutamic acid 5-amide;Glutamic acid amide;Glutamine;L-(+)-glutamine;L-2-aminoglutaramic acid;L-2-aminoglutaramidic acid;L-glutamic acid 5-amide;L-glutamic acid gamma-amide;L-glutamid;L-glutamide;L-glutamin;L-glutamine;L-glutaminsaeure-5-amid;Levoglutamid;Levoglutamida;Levoglutamide;Levoglutamidum;Levoglutamina;Polyglutamine;Stimulina;Gamma-glutamine;Q;Glutamate 5-amide;Glutamate amidePW_C000500Gln6184434270123021155658107565910860681576846166684738390225117931981269229042352318423533157701625377296111773331337839513279164114792641168065213511320794116207109120038122120348406121219409121961124122998120123789137124514118125416297125668479126359299126812483126956205127163501127718388128411208105L-AlanineHMDB0000161Alanine is a non-essential amino acid made in the body from either the conversion of the carbohydrate pyruvate or the breakdown of DNA and the dipeptides carnosine and anserine. It is highly concentrated in muscle and is one of the most important amino acids released by muscle, functioning as a major energy source. Plasma alanine is often decreased when the BCAA (branched-chain amino acids) are deficient. This finding may relate to muscle metabolism. Alanine is highly concentrated in meat products and other high-protein foods like wheat germ and cottage cheese. Alanine is an important participant as well as a regulator of glucose metabolism. Alanine levels parallel blood sugar levels in both diabetes and hypoglycemia, and alanine reduces both severe hypoglycemia and the ketosis of diabetes. It is an important amino acid for lymphocyte reproduction and immunity. Alanine therapy has helped dissolve kidney stones in experimental animals. Normal alanine metabolism, like that of other amino acids, is highly dependent upon enzymes that contain vitamin B6. Alanine, like GABA, taurine, and glycine, is an inhibitory neurotransmitter in the brain (http://www.dcnutrition.com/AminoAcids/). L-Alanine has been found to be associated with glucagon deficiency, which is an inborn error of metabolism.56-41-7C00041595016977L-ALPHA-ALANINE5735DB00160C[C@H](N)C(O)=OC3H7NO2InChI=1S/C3H7NO2/c1-2(4)3(5)6/h2H,4H2,1H3,(H,5,6)/t2-/m0/s1QNAYBMKLOCPYGJ-REOHCLBHSA-N(2S)-2-aminopropanoic acid89.093289.0476784730.702L-alanine00FDB000556(2s)-2-aminopropanoate;(2s)-2-aminopropanoic acid;(s)-(+)-alanine;(s)-2-aminopropanoate;(s)-2-aminopropanoic acid;(s)-2-amino-propanoate;(s)-2-amino-propanoic acid;(s)-alanine;2-aminopropanoate;2-aminopropanoic acid;2-aminopropionate;2-aminopropionic acid;2-ammoniopropanoate;2-ammoniopropanoic acid;Ala;Alanine;L-(+)-alanine;L-2-aminopropanoate;L-2-aminopropanoic acid;L-2-aminopropionate;L-2-aminopropionic acid;L-a-alanine;L-a-aminopropionate;L-a-aminopropionic acid;L-alpha-alanine;L-alpha-aminopropionate;L-alpha-aminopropionic acid;A-alanine;A-aminopropionate;A-aminopropionic acid;Alpha-alanine;Alpha-aminopropanoate;Alpha-aminopropanoic acid;Alpha-aminopropionate;Alpha-aminopropionic acid;A;L-alanin;L-α-alaninePW_C000105Ala102294316814465014535114542630221534393540711754181035431118545212055571325578133563710756381085883105652985835022512271151126203112627181523022242452320424533184253431577969346779753277798832678008111780921127916511480693135119910122120015124120026406121145423121151424121164416121220409122139407123717458123723459123736452123790137124691119125300297125393299125404479126296481126850205126933388126944501127860206134Oxoglutaric acidHMDB0000208Oxoglutaric acid, also known as alpha-ketoglutarate, alpha-ketoglutaric acid, AKG, or 2-oxoglutaric acid, is classified as a gamma-keto acid or a gamma-keto acid derivative. gamma-Keto acids are organic compounds containing an aldehyde substituted with a keto group on the C4 carbon atom. alpha-Ketoglutarate is considered to be soluble (in water) and acidic. alpha-Ketoglutarate is a key molecule in the TCA cycle, playing a fundamental role in determining the overall rate of this important metabolic process (PMID: 26759695). In the TCA cycle, AKG is decarboxylated to succinyl-CoA and carbon dioxide by AKG dehydrogenase, which functions as a key control point of the TCA cycle. Additionally, AKG can be generated from isocitrate by oxidative decarboxylation catalyzed by the enzyme known as isocitrate dehydrogenase (IDH). In addition to these routes of production, AKG can be produced from glutamate by oxidative deamination via glutamate dehydrogenase, and as a product of pyridoxal phosphate-dependent transamination reactions (mediated by branched-chain amino acid transaminases) in which glutamate is a common amino donor. AKG is a nitrogen scavenger and a source of glutamate and glutamine that stimulates protein synthesis and inhibits protein degradation in muscles. In particular, AKG can decrease protein catabolism and increase protein synthesis to enhance bone tissue formation in skeletal muscles (PMID: 26759695). Interestingly, enteric feeding of AKG supplements can significantly increase circulating plasma levels of hormones such as insulin, growth hormone, and insulin-like growth factor-1 (PMID: 26759695). It has recently been shown that AKG can extend the lifespan of adult C. elegans by inhibiting ATP synthase and TOR (PMID: 24828042). In combination with molecular oxygen, alpha-ketoglutarate is required for the hydroxylation of proline to hydroxyproline in the production of type I collagen. A recent study has shown that alpha-ketoglutarate promotes TH1 differentiation along with the depletion of glutamine thereby favouring Treg (regulatory T-cell) differentiation (PMID: 26420908). alpha-Ketoglutarate has been found to be associated with fumarase deficiency, 2-ketoglutarate dehydrogenase complex deficiency, and D-2-hydroxyglutaric aciduria, which are all inborn errors of metabolism (PMID: 8338207).328-50-7C0002651309152-KETOGLUTARATE50DB02926OC(=O)CCC(=O)C(O)=OC5H6O5InChI=1S/C5H6O5/c6-3(5(9)10)1-2-4(7)8/h1-2H2,(H,7,8)(H,9,10)KPGXRSRHYNQIFN-UHFFFAOYSA-N2-oxopentanedioic acid146.0981146.021523302-0.442oxoglutarate0-2FDB0033612-ketoglutarate;2-ketoglutaric acid;2-oxo-1,5-pentanedioate;2-oxo-1,5-pentanedioic acid;2-oxoglutarate;2-oxoglutaric acid;2-oxopentanedioate;2-oxopentanedioic acid;Oxoglutarate;Alpha-ketoglutaric acid;Oxoglutaric acid;A-ketoglutarate;A-ketoglutaric acid;Alpha-ketoglutarate;α-ketoglutarate;α-ketoglutaric acidPW_C000134AKG152423141414684991867331110842126351447501455261467545375103541411754381185564132600814760361556069157609216164821786530857471222751522475191518209225837422011863198126812897705425377135133774811117752311277746129779673457797034677976327779843477842533480018368806941351131629411997240612002212412008440712017412212055241412081441812098940812114642312115242412116042512275712012283111912318645012339945412355437412371845812372445912373246012535747912540029912545548112553329712580048912592948212690050112694038812699320612706620512725550612738850295L-Glutamic acidHMDB0000148Glutamic acid (Glu), also referred to as glutamate (the anion), is one of the 20 proteinogenic amino acids. It is not among the essential amino acids. Glutamate is a key molecule in cellular metabolism. In humans, dietary proteins are broken down by digestion into amino acids, which serves as metabolic fuel or other functional roles in the body. Glutamate is the most abundant fast excitatory neurotransmitter in the mammalian nervous system. At chemical synapses, glutamate is stored in vesicles. Nerve impulses trigger release of glutamate from the pre-synaptic cell. In the opposing post-synaptic cell, glutamate receptors, such as the NMDA receptor, bind glutamate and are activated. Because of its role in synaptic plasticity, it is believed that glutamic acid is involved in cognitive functions like learning and memory in the brain. Glutamate transporters are found in neuronal and glial membranes. They rapidly remove glutamate from the extracellular space. In brain injury or disease, they can work in reverse and excess glutamate can accumulate outside cells. This process causes calcium ions to enter cells via NMDA receptor channels, leading to neuronal damage and eventual cell death, and is called excitotoxicity. The mechanisms of cell death include: * Damage to mitochondria from excessively high intracellular Ca2+. * Glu/Ca2+-mediated promotion of transcription factors for pro-apoptotic genes, or downregulation of transcription factors for anti-apoptotic genes. Excitotoxicity due to glutamate occurs as part of the ischemic cascade and is associated with stroke and diseases like amyotrophic lateral sclerosis, lathyrism, and Alzheimer's disease. glutamic acid has been implicated in epileptic seizures. Microinjection of glutamic acid into neurons produces spontaneous depolarization around one second apart, and this firing pattern is similar to what is known as paroxysmal depolarizing shift in epileptic attacks. This change in the resting membrane potential at seizure foci could cause spontaneous opening of voltage activated calcium channels, leading to glutamic acid release and further depolarization. (http://en.wikipedia.org/wiki/Glutamic_acid).56-86-0C000253303216015GLT30572DB00142N[C@@H](CCC(O)=O)C(O)=OC5H9NO4InChI=1S/C5H9NO4/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H,7,8)(H,9,10)/t3-/m0/s1WHUUTDBJXJRKMK-VKHMYHEASA-N(2S)-2-aminopentanedioic acid147.1293147.053157781-0.263L-glutamic acid0-1FDB012535(2s)-2-aminopentanedioate;(2s)-2-aminopentanedioic acid;(s)-(+)-glutamate;(s)-(+)-glutamic acid;(s)-2-aminopentanedioate;(s)-2-aminopentanedioic acid;(s)-glutamate;(s)-glutamic acid;1-amino-propane-1,3-dicarboxylate;1-amino-propane-1,3-dicarboxylic acid;1-aminopropane-1,3-dicarboxylate;1-aminopropane-1,3-dicarboxylic acid;2-aminoglutarate;2-aminoglutaric acid;2-aminopentanedioate;2-aminopentanedioic acid;Aciglut;Aminoglutarate;Aminoglutaric acid;E;Glt;Glu;Glusate;Glut;Glutacid;Glutamicol;Glutamidex;Glutaminate;Glutaminic acid;Glutaminol;Glutaton;L-(+)-glutamate;L-(+)-glutamic acid;L-glu;L-glutamate;L-glutaminate;L-glutaminic acid;L-a-aminoglutarate;L-a-aminoglutaric acid;L-alpha-aminoglutarate;L-alpha-aminoglutaric acid;A-aminoglutarate;A-aminoglutaric acid;A-glutamate;A-glutamic acid;Alpha-aminoglutarate;Alpha-aminoglutaric acid;Alpha-glutamate;Alpha-glutamic acid;Acide glutamique;Acido glutamico;Acidum glutamicum;Glutamate;Glutamic acid;L-glutaminsaeurePW_C000095Glu1624436581191138416414969911054214485014562614625453231115344113541511754391185565132563110756321085859105600614760711576191946531856838187684418870927270937171652057182207751422475181518208225837322011792198118551611200422212621311268328912697290423483154234931842845320770202537733213377525112779713467797732777981347782913458064913512002312412004012212008640712034740612069212612081641812114742312115342412115742512283311912299712012329944312340145412371945812372545912372946012540129912541829712545748112566747912576930112580248912694138812699520612716250112725750614073884140739597164Pyruvic acidHMDB0000243Pyruvic acid is an intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed.) Biological Source: Intermediate in primary metabolism including fermentation processes. Present in muscle in redox equilibrium with Lactic acid. A common constituent, as a chiral cyclic acetal linked to saccharide residues, of bacterial polysaccharides. Isolated from cane sugar fermentation broth and peppermint. Constituent of Bauhinia purpurea, Cicer arietinum (chickpea), Delonix regia, Pisum sativum (pea) and Trigonella caerulea (sweet trefoil) Use/Importance: Reagent for regeneration of carbonyl compdounds from semicarbazones, phenylhydrazones and oximes. Flavoring ingredient (Dictionary of Organic Compounds).127-17-3C00022106032816PYRUVATE1031DB00119CC(=O)C(O)=OC3H4O3InChI=1S/C3H4O3/c1-2(4)3(5)6/h1H3,(H,5,6)LCTONWCANYUPML-UHFFFAOYSA-N2-oxopropanoic acid88.062188.0160439940.181pyruvic acid0-1FDB0082932-oxopropanoate;2-oxopropanoic acid;2-oxopropionate;2-oxopropionic acid;Acetylformate;Acetylformic acid;Bts;Pyroracemate;Pyroracemic acid;Pyruvate;A-ketopropionate;A-ketopropionic acid;Alpha-ketopropionate;Alpha-ketopropionic acid;2-ketopropionic acid;2-oxopropansaeure;2-oxopropionsaeure;Acide pyruvique;Alpha-oxopropionsaeure;Brenztraubensaeure;Ch3cocooh;2-ketopropionate;α-ketopropionate;α-ketopropionic acid;A-oxopropionsaeure;α-oxopropionsaeurePW_C000164Pyr1722044228118131449501457265365103540511754401185444120556613255701335893955920147595115160221556067156607416161261606383164671786510177653285745722274952208200225126223115292249153491877310111779723467797832778090112800043688004236780695135112879941156831211199504061200111241201751221208784071211484231211544241234541191237204581237264591253404791253902991255342971258544811268835011269313881270672051278582061148Pyridoxal 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'P182324453518122140119696201110421450501458262120102150495325111541611754211035441118545512055671325581133653385701816071672057216212722221311858161121751511262331126281812684289126892907701725377037225770412937705222477526112777643417797334677979327782923457885533278862331806961359863071199121221200241241200294061200874071208174181211494231211554241220691231220763831228341191234024541237214581237274591246204471246273981253022971254022991254074791254584811258034891262242981262314951269423881269475011269962061272585061277865131277933901420WaterHMDB0002111Water 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_C001420H2O558949109513941513162144811352615624286521069120770338231883821094311377491465541590432018242532222678602727462778172805293143703164723634614598364727374941935030275156751959752141005227945236103529710553191115343113535511254021105470123548312554921265507127553413055371145541129559113556081185622108569165759140577810158411435853146587710758909559101475940151603215560591576087161612316361331596215162181666477178650718066001526713117684018868881607162205718120771932067211211722821372382147243215729519873502167388210740121274672227492224750019075881708201225823722684141629265261185027711922164120112811221328512250286122642871232724912520227126326512693290127052911271529213007298130193001302530113037302132612231332729415340308423273154269531843691322769142937701925377102132771311337721513477378331773973327747133377516115775363347762833677722337777593417781634377982347780713297823535278242353782703567911336080014368800393708059122880656119938303839479438411055739011063939111584439811987923211991512211996340612000840712004640812011312412036541212043040512043840912060641512079441412115842512124042912135112112138141912160743412211838212238443612275312012279737412280444312301244612306437612307213712313144712314213612316244812323145112338445012373046012381046412394045512416546912467039912493847112494547212530529712535347912538648112542448212548029912568248312570747812574548712605449012623849512627348412676448012689650112696350212701738812717720812719920912722750412750650712757651512783638912808239512817651314067479014067583414075518535AmmoniaHMDB0000051Ammonia 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_C000035NH39791125133814244382479135501414685425332225723533811160161477022160717720511786198118482771188521512708291127182927696622577046294773291337734313277469333774991137753933477597115779853477799311278072329792442938065013580657119116203109119921122120049408120053126120136407120343406120363412120462405121046124121161425122119382122800374122805443122993120123010446123096376123610118123733460124671399125311297125427482125431301125502481125663479125708478126102299126274484126966502126970207127039206127158501127200209127600388127837389721NADHMDB0000902NAD (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_C000721NAD140415033538651101114211344312735146654222949277917283529310794807184813184819284902649603151679552381035334111536011254691235482125559013556101185696100573810858271415912147594215160241556072157607616163851646917867721176890160701218870971637174205719720674051987459222824122683592259085224118192161232224913006298130183001325622342404322426193157710413277120133772091347737033177650336776673347770233277709130779151137798334778406356800063688069011993825124110552388112750166112853941199291221199524061201714071208344191209844081211594251212421261212594291218173831226143841227421201231304471231411361234194551235493741237314601238124431238294641243703981251871211253192971253424791255304811258062991258254901259244821265154951267654801268855011272785071273835021280893901283603911284283951407571851144NADHHMDB0001487NADH 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_C001144NADH14341533490864810111521275514695422304927811728362931099480618481218482128490464959315169955240103533211153581125466123547912555931355698100573710858291415915147594515160271556079161638716472178677111768931607011188709916371722057195206746222282442268360225908622411809198118212161232024913003298130153001325522342403322426183157710713277123133772081347737133177651336776683347770033277707130779171137798634780009368806911199382212411054938811285494115838118119955406120172407120378122120986408121162425121244126121693429121818383122616384122745120123127447123138136123551374123734460123814443124242464124371398125189121125345479125531481125762297125808299125926482126516495126767480126888501127385502128090390128362391128429395140759185414Adenosine triphosphateHMDB0000538Adenosine triphosphate (ATP) is a nucleotide consisting of a purine base (adenine) attached to the first carbon atom of ribose (a pentose sugar). Three phosphate groups are esterified at the fifth carbon atom of the ribose. ATP is incorporated into nucleic acids by polymerases in the processes of DNA replication and transcription. ATP contributes to cellular energy charge and participates in overall energy balance, maintaining cellular homeostasis. ATP can act as an extracellular signaling molecule via interactions with specific purinergic receptors to mediate a wide variety of processes as diverse as neurotransmission, inflammation, apoptosis, and bone remodelling. Extracellular ATP and its metabolite adenosine have also been shown to exert a variety of effects on nearly every cell type in human skin, and ATP seems to play a direct role in triggering skin inflammatory, regenerative, and fibrotic responses to mechanical injury, an indirect role in melanocyte proliferation and apoptosis, and a complex role in Langerhans cell-directed adaptive immunity. During exercise, intracellular homeostasis depends on the matching of adenosine triphosphate (ATP) supply and ATP demand. Metabolites play a useful role in communicating the extent of ATP demand to the metabolic supply pathways. Effects as different as proliferation or differentiation, chemotaxis, release of cytokines or lysosomal constituents, and generation of reactive oxygen or nitrogen species are elicited upon stimulation of blood cells with extracellular ATP. The increased concentration of adenosine triphosphate (ATP) in erythrocytes from patients with chronic renal failure (CRF) has been observed in many studies but the mechanism leading to these abnormalities still is controversial. (PMID: 15490415, 15129319, 14707763, 14696970, 11157473).56-65-5C00002595715422ATP5742DB00171NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1OC10H16N5O13P3InChI=1S/C10H16N5O13P3/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(26-10)1-25-30(21,22)28-31(23,24)27-29(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H,23,24)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1ZKHQWZAMYRWXGA-KQYNXXCUSA-N({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)phosphonic acid507.181506.995745159-2.057adenosine triphosphate0-3FDB0218135'-(tetrahydrogen triphosphate) adenosine;5'-atp;Atp;Adenosine 5'-triphosphate;Adenosine 5'-triphosphorate;Adenosine 5'-triphosphoric acid;Adenosine triphosphate;Adenylpyrophosphorate;Adenylpyrophosphoric acid;Adephos;Adetol;Adynol;Atipi;Atriphos;Cardenosine;Fosfobion;Glucobasin;Myotriphos;Phosphobion;Striadyne;Triadenyl;Triphosphaden;Triphosphoric acid adenosine ester;Adenosine-5'-triphosphate;H4atp;Adenosine triphosphoric acid;Adenosine-5'-triphosphoric acidPW_C000414ATP922146082661641422478137333279959343997632105182112102146492156142160582405592434272726462812293029663163723616613617514399234474314768914864545032895035265155752059752151005250104529110153131115346112539010354061175430118544312055421295556132556913356031355621108584614358541465876107589714759241516048155610916162301666493178683918868701606976199715720571842067209210722521372292117298198730221673902177408218743216374812227499190818622511847277119031701201028112039164121782851257822612691290132642231532730842326315426213224269431877028253772181347723332977468333776323367803733278041350781681287821435178240353784113357849411578850130788653317891933480028368800461848067411985629194826124113234941132823881162801091199141221199924061201544071202453821203624121212464291213921231213974331214714081219744101220651251220793831220834051224024221224444351229193991230094461238164641239514471239564681240293741245274441246161361246303981246343761249434721249723751250114701253042971253714791253922991255154811255954841261234851262203001262344951262404781265474911265964991269135011271233891277315161277813951277963901278012091281195081281675171407708911316Carbon 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_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905021407981851034Adenosine diphosphateHMDB0001341Adenosine diphosphate, abbreviated ADP, is a nucleotide. It is an ester of pyrophosphoric acid with the nucleotide adenine. ADP consists of the pyrophosphate group, the pentose sugar ribose, and the nucleobase adenine. ADP is the product of ATP dephosphorylation by ATPases. ADP is converted back to ATP by ATP synthases.58-64-0C00008602216761ADP5800NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1OC10H15N5O10P2InChI=1S/C10H15N5O10P2/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(24-10)1-23-27(21,22)25-26(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1XTWYTFMLZFPYCI-KQYNXXCUSA-N[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]phosphonic acid427.2011427.029414749-2.126adenosine-diphosphate0-2FDB021817Adp;Adenosindiphosphorsaeure;Adenosine 5'-pyrophosphate;Adenosine diphosphate;Adenosine pyrophosphate;Adenosine-5'-diphosphate;Adenosine-5-diphosphate;Adenosine-diphosphate;5'-adenylphosphoric acid;Adenosine 5'-diphosphate;H3adp;5'-adenylphosphate;Adenosine 5'-diphosphoric acid;Adenosine-5'-diphosphoric acidPW_C001034ADP2341348415224821380159631597831061141518219014921041821131021615824085924352727284727364628552931657236356144002344763147709150362651577520897521710053151115349112539210354461205544129557213356241085741117576410158491435856146587810758991475926151605015561111616231166649517867009468411886872160715920571872067208210722621372312117300198730321673912177410218743316374832228187225118512771190517012013281121802851326222315329308423283154239831342622322426963187702925377087132772161347730632977472333776633367803933278043350781701287821535178244353784143357849511578705331788491307892033480030368806221188065113580676119948271241132833881162041091199441221199944061201564071203183821203664121212484291213941231213994331214724081218993831219764101220641251220854051224054221224454351229733991230134461238184641239534471239584681240303741244523981245294441246151361246363761249474721249753751250124701253342971253734791254922991255174811256454841261254851262193001262354951262424781265504911265974991269155011277335161277803951277973901278032091281225081281685171283133891441Phosphoric acidHMDB0002142Phosphoric acid, also known as orthophosphoric acid or phosphoric(V) acid, is a mineral acid with the chemical formula H3PO4. Alternatively, orthophosphoric acid molecules can combine with themselves to form a variety of compounds referred to as phosphoric acids in a more general way. For a discussion of these, see Phosphoric acids and Phosphates. Appears to exist only as a food additive and produced synthetically. --Wikipedia.7664-38-2C00009100426078979OP(O)(O)=OH3O4PInChI=1S/H3O4P/c1-5(2,3)4/h(H3,1,2,3,4)NBIIXXVUZAFLBC-UHFFFAOYSA-Nphosphoric acid97.995297.9768950963phosphoric acid0-2FDB013380Acide phosphorique (french);Acido fosforico [italian];Acidum phosphoricum;Diphosphate tetrasodium;Fosforzuuroplossingen [dutch];Marphos;Nfb;Ortho- phosphoric acid;Orthophosphoric acid;Phosphoric acid (acd/name 4.0);Phosphorsaeure;Phosphorsaeureloesungen [german];Sodium pyrophosphate;Sodium pyrophosphate decahydrate;Sodium pyrophosphate decahydrate biochemica;Sonac;Tetra-sodium pyrophosphate;Tetrasodium pyrophosphate decahydrate;Tetrasodium pyrophosphate 10-hydrate;White phosphoric acid;[po(oh)3];Acide phosphorique;H3po4;Phosphate;Phosphorsaeureloesungen;OrthophosphatePW_C001441PhosfrA171844044831315924316782190249191054856359131475943151689116011782198118282131182921611852277126383177105132773301337747333377779111780001127827235678706331787073348067113580677119119918122120126124120157407120344406120367412121383419121900383121902408122858118122994120123014446123942455124453398124455374125308297125493299125518481125664479126056490126855205127030388127053206127159501127508507862Carbamoyl phosphateHMDB0001096Carbamoyl phosphate is a precursor of both arginine and pyrimidine biosynthesis. It is a labile and potentially toxic intermediate. Carbamoyl phosphate is a molecule that is involved in ridding the body of excess nitrogen in the urea cycle, and also in the synthesis of pyrimidines. It is produced from carbon dioxide, ammonia, and phosphate (from ATP) by the enzyme carbamoyl phosphate synthase. -- Wikipedia.590-55-6C0016927817672CARBAMOYL-P272NC(=O)OP(O)(O)=OCH4NO5PInChI=1S/CH4NO5P/c2-1(3)7-8(4,5)6/h(H2,2,3)(H2,4,5,6)FFQKYPRQEYGKAF-UHFFFAOYSA-N(carbamoyloxy)phosphonic acid141.0199140.982708755-0.963carbamoyl-phosphate0-2FDB022424Carbamic acid monoanhydride with phosphorate;Carbamic acid monoanhydride with phosphoric acid;Carbamoyl-p;Carbamoyl-phosphate;Carbamoylphosphate;Carbamyl phosphate;Carbamyl-phosphate;Aminocarbonyl dihydrogen phosphate;Carbamic phosphoric monoanhydride;Carbamoyl phosphate;Monocarbamoyl phosphate;Phosphoric acid mono(formamide)ester;Aminocarbonyl dihydrogen phosphoric acid;Carbamoyl phosphoric acid;Carbamoyl phosphoric acid;Carbamyl phosphoric acid;Monocarbamoyl phosphoric acid;Phosphate mono(formamide)esterPW_C000862CarbmP898441427022926813118531611273029077331133775471117872513280672135120092122120345406122199124122995120124751118125462297125665479126361299126999205127160501127923388123L-Aspartic acidHMDB0000191Aspartic acid (Asp, D), also known as aspartate, the name of its anion, is one of the 20 natural proteinogenic amino acids which are the building blocks of proteins. As its name indicates, aspartic acid is the carboxylic acid analog of asparagine. As a neurotransmitter, aspartic acid may provide resistance to fatigue and thus lead to endurance, although the evidence to support this idea is not strong (Wikipedia). Aspartic acid is a nonessential amino acid that is made from glutamic acid by enzymes using vitamin B6. The amino acid has important roles in the urea cycle and DNA metabolism. Aspartic acid is a major excitatory neurotransmitter, which is sometimes found to be increased in epileptic and stroke patients. It is decreased in depressed patients and in patients with brain atrophy. Aspartic acid supplements are being evaluated. Five grams can raise blood levels. Magnesium and zinc may be natural inhibitors of some of the actions of aspartic acid. Aspartic acid, with the amino acid phenylalanine, is a part of a new natural sweetener, aspartame. This sweetener is an advance in artificial sweeteners, and is probably safe in normal doses to all except phenylketonurics. The jury is still out on the long-term effects it has on many brain neurohormones. Aspartic acid may be a significant immunostimulant of the thymus and can protect against some of the damaging effects of radiation. Many claims have been made for the special value of administering aspartic acid in the form of potassium and magnesium salts. Since aspartic acid is relatively nontoxic, studies are now in progress to elucidate its pharmacological and therapeutic roles (http://www.dcnutrition.com/AminoAcids).56-84-8C000494436744517053L-ASPARTATE5745DB00128N[C@@H](CC(O)=O)C(O)=OC4H7NO4InChI=1S/C4H7NO4/c5-2(4(8)9)1-3(6)7/h2H,1,5H2,(H,6,7)(H,8,9)/t2-/m0/s1CKLJMWTZIZZHCS-REOHCLBHSA-N(2S)-2-aminobutanedioic acid133.1027133.0375077170.033L-aspartic acid0-1FDB012567Asp;(+)-aspartate;(+)-aspartic acid;(2s)-aspartate;(2s)-aspartic acid;(l)-aspartate;(l)-aspartic acid;(r)-2-aminosuccinate;(s)-(+)-aspartate;(s)-(+)-aspartic acid;(s)-2-aminosuccinate;(s)-2-aminosuccinic acid;(s)-aminobutanedioate;(s)-aminobutanedioic acid;(s)-aspartate;(s)-aspartic acid;(s)-amino-butanedioate;(s)-amino-butanedioic acid;2-amino-3-methylsuccinate;2-amino-3-methylsuccinic acid;2-aminosuccinate;2-aminosuccinic acid;Aminosuccinate;Asparagate;Asparagic acid;Asparaginate;Asparaginic acid;Asparatate;Aspartate;H-asp-oh;L-(+)-aspartate;L-(+)-aspartic acid;L-aminosuccinate;L-aminosuccinic acid;L-asparagate;L-asparagic acid;L-asparaginate;L-asparaginic acid;L-aspartate;Alpha-aminosuccinate;Alpha-aminosuccinic acid;(s)-2-aminobutanedioic acid;Aspartic acid;D;L-asparaginsaeure;(s)-2-aminobutanedioatePW_C000123Asp48893911013110341104421994256631075664108685118875701608185225118021981268629012704291423633184236431577323111775101327753211378288133782893457930711280699135119878232119881117120037122120421124120809406120813418123057118123394120123398454125415297125795479125799489126955205127250501127254506127555388148Oxalacetic acidHMDB0000223Oxaloacetic acid, also known as oxosuccinic acid or oxalacetic acid, is a four-carbon dicarboxylic acid appearing as an intermediate of the citric acid cycle. In vivo, oxaloacetate (the ionized form of oxaloacetic acid) is formed by the oxidation of L-malate, catalyzed by malate dehydrogenase, and reacts with Acetyl-CoA to form citrate, catalyzed by citrate synthase.(wikipedia) A class of ketodicarboxylic acids derived from oxalic acid. Oxaloacetic acid is an intermediate in the citric acid cycle and is converted to aspartic acidD by a transamination reaction.328-42-7C0003697030744OXALACETIC_ACID945OC(=O)CC(=O)C(O)=OC4H4O5InChI=1S/C4H4O5/c5-2(4(8)9)1-3(6)7/h1H2,(H,6,7)(H,8,9)KHPXUQMNIQBQEV-UHFFFAOYSA-N2-oxobutanedioic acid132.0716132.005873238-0.362oxalacetate0-2FDB0014792-ketosuccinate;2-ketosuccinic acid;2-oxobutanedioate;2-oxobutanedioic acid;2-oxosuccinate;2-oxosuccinic acid;Ketosuccinate;Ketosuccinic acid;Oaa;Oxalacetate;Oxaloacetate;Oxaloacetic acid;Oxaloethanoate;Oxaloethanoic acid;Oxosuccinate;Oxosuccinic acid;A-ketosuccinate;A-ketosuccinic acid;Alpha-ketosuccinate;Alpha-ketosuccinic acid;3-carboxy-3-oxopropanoic acid;Keto-succinic acid;Oxalacetic acid;Oxobutanedioic acid;3-carboxy-3-oxopropanoate;Keto-succinate;OxobutanedioatePW_C000148Oaa25496911151099311094211132168885371103544812055741336033155608816164781787468222751322475171518372220837822511744117118911601270729112717292437923227750813277533113775383347795811278009111782903458001536880700135119964406120048408120062126120180122120419124120815418121207407122799374122813443123055118123400454123777119125354479125426482125439301125537297125801489125807299126897501126965502126977207127070205127256506127261388140OrnithineHMDB0000214Ornithine is an amino acid produced in the urea cycle by the splitting off of urea from arginine. It is a central part of the urea cycle, which allows for the disposal of excess nitrogen. L-Ornithine is also a precursor of citrulline and arginine. In order for ornithine produced in the cytosol to be converted to citrulline, it must first cross the inner mitochondrial membrane into the mitochondrial matrix where it is carbamylated by ornithine transcarbamylase. This transfer is mediated by the mitochondrial ornithine transporter (SLC25A15; AF112968; ORNT1). Mutations in the mitochondrial ornithine transporter result in hyperammonemia, hyperornithinemia, homocitrullinuria (HHH) syndrome, a disorder of the urea cycle. (PMID 16256388) The pathophysiology of the disease may involve diminished ornithine transport into mitochondria, resulting in ornithine accumulation in the cytoplasm and reduced ability to clear carbamoyl phosphate and ammonia loads. (OMIM 838970).70-26-8C00077626215729L-ORNITHINE6026DB00129NCCC[C@H](N)C(O)=OC5H12N2O2InChI=1S/C5H12N2O2/c6-3-1-2-4(7)5(8)9/h4H,1-3,6-7H2,(H,8,9)/t4-/m0/s1AHLPHDHHMVZTML-BYPYZUCNSA-N(2S)-2,5-diaminopentanoic acid132.161132.0898776380.113ornithine01FDB003654(+)-s-ornithine;(s)-2,5-diaminopentanoate;(s)-2,5-diaminopentanoic acid;(s)-ornithine;(s)-a,d-diaminovalerate;(s)-a,d-diaminovaleric acid;5-amino-l-norvaline;L-(-)-ornithine;L-ornithine;(s)-2,5-diaminovaleric acid;(s)-alpha,delta-diaminovaleric acid;(s)-2,5-diaminovalerate;(s)-a,delta-diaminovalerate;(s)-a,delta-diaminovaleric acid;(s)-alpha,delta-diaminovalerate;(s)-α,δ-diaminovalerate;(s)-α,δ-diaminovaleric acid;(s)-a,δ-diaminovalerate;(s)-a,δ-diaminovaleric acidPW_C000140Ornithi459412128118561611187819877475133774921111203714061203991221230161201230421351258682971273292051104PhosphateHMDB0001429Phosphate is a salt of phosphoric acid. In organic chemistry, a phosphate, or organophosphate, is an ester of phosphoric acid. Organic phosphates are important in biochemistry, biogeochemistry and ecology. Phosphate (Pi) is an essential component of life. In biological systems, phosphorus is found as a free phosphate ion in solution and is called inorganic phosphate, to distinguish it from phosphates bound in various phosphate esters. Inorganic phosphate is generally denoted Pi and at physiological (neutral) pH primarily consists of a mixture of HPO<sup>2-</sup><sub>4</sub> and H<sub>2</sub>PO<sup>-</sup><sub>4</sub> ions. phosphates are most commonly found in the form of adenosine phosphates, (AMP, ADP and ATP) and in DNA and RNA and can be released by the hydrolysis of ATP or ADP. Similar reactions exist for the other nucleoside diphosphates and triphosphates. Phosphoanhydride bonds in ADP and ATP, or other nucleoside diphosphates and triphosphates, contain high amounts of energy which give them their vital role in all living organisms. Phosphate must be actively transported into cells against its electrochemical gradient. In vertebrates, two unrelated families of Na+-dependent Pi transporters carry out this task. Remarkably, the two families transport different Pi species: whereas type II Na+/Pi cotransporters (SCL34) prefer divalent HPO4(2), type III Na+/Pi cotransporters (SLC20) transport monovalent H2PO4. The SCL34 family comprises both electrogenic and electroneutral members that are expressed in various epithelia and other polarized cells. Through regulated activity in apical membranes of the gut and kidney, they maintain body Pi homeostasis, and in salivary and mammary glands, liver, and testes they play a role in modulating the Pi content of luminal fluids. Phosphate levels in the blood play an important role in hormone signaling and in bone homeostasis. In classical endocrine regulation, low serum phosphate induces the renal production of the seco-steroid hormone 1,25-dihydroxyvitamin D3 (1,25(OH)2D3).This active metabolite of vitamin D acts to restore circulating mineral (i.e. phosphate and calcium) levels by increasing absorption in the intestine, reabsorption in the kidney, and mobilization of calcium and phosphate from bone. Thus, chronic renal failure is associated with hyperparathyroidism, which in turn contributes to osteomalacia (softening of the bones). Another complication of chronic renal failure is hyperphosphatemia (low levels of phosphate in the blood). Hyperphosphatemia (excess levels of phosphate in the blood) is a prevalent condition in kidney dialysis patients and is associated with increased risk of mortality. Hypophosphatemia (hungry bone syndrome) has been associated to postoperative electrolyte aberrations and after parathyroidectomy. (PMID: 17581921, 11169009, 11039261, 9159312, 17625581)Fibroblast growth factor 23 (FGF-23) has recently been recognized as a key mediator of phosphate homeostasis, its most notable effect being promotion of phosphate excretion. FGF-23 was discovered to be involved in diseases such as autosomal dominant hypophosphatemic rickets, X-linked hypophosphatemia, and tumor-induced osteomalacia in which phosphate wasting was coupled to inappropriately low levels of 1,25(OH)2D3. FGF-23 is regulated by dietary phosphate in humans. In particular it was found that phosphate restriction decreased FGF-23, and phosphate loading increased FGF-23.14265-44-2C00009106118367CPD-85871032OP(O)(O)=OH3O4PInChI=1S/H3O4P/c1-5(2,3)4/h(H3,1,2,3,4)NBIIXXVUZAFLBC-UHFFFAOYSA-Nphosphoric acid97.995297.9768950963phosphoric acid0-2DBMET00532FDB022617Nfb orthophosphate;O-phosphoric acid;Ortho-phosphate;Orthophosphate (po43-);Orthophosphate(3-);Phosphate;Phosphate (po43-);Phosphate anion(3-);Phosphate ion (po43-);Phosphate ion(3-);Phosphate trianion;Phosphate(3-);Phosphoric acid ion(3-);Pi;[po4](3-);Orthophosphate;Phosphate ion;Po4(3-);Phosphoric acid;Orthophosphoric acid;Phosphoric acid ionPW_C001104Pi2448488145818188312980317631417674925001027294727374631292931667236366138512342492244753150312751587520797521610053171115351112538110354471205543129557313356051355625108569365848143585514659111475941151604015561001616294107648717866911016714117684218868891607161205718920672122117306198738921074022127436163747522281962258258227101182411013425711748132117611151177321311904170119271641201428112728290132632233481917422553044235031542435318436923227701825377194293772171347794033677966130780483327805732978245353786693318002236889279308938313839479638411055839011064039111323594115845398116206109119982406120069122120699407121057124121216125121268429121352121121409123121423382121852405123304119123621118123786136123838464123968447123981399124405376124948472125362479125446297125774481125954299126221478126594300126604298126723484126904501127413388127783209128166395128177513128315389723CitrullineHMDB0000904Citrulline is an amino acid made from ornithine and carbamoyl phosphate in one of the central reactions in the urea cycle. It is also produced from arginine as a by-product of the reaction catalyzed by NOS family (NOS). In this reaction, arginine is first oxidized into N(omega)-hydroxyarginine, which is then further oxidized to citrulline concomitant with the release of nitric oxide (EC 1.14.13.39). Citrulline's name is derived from citrullus, the Latin word for watermelon, from which it was first isolated.372-75-8C00327699209816349L-CITRULLINE9367DB00155N[C@@H](CCCNC(N)=O)C(O)=OC6H13N3O3InChI=1S/C6H13N3O3/c7-4(5(10)11)2-1-3-9-6(8)12/h4H,1-3,7H2,(H,10,11)(H3,8,9,12)/t4-/m0/s1RHGKLRLOHDJJDR-BYPYZUCNSA-N(2S)-2-amino-5-(carbamoylamino)pentanoic acid175.1857175.095691297-0.904L-citrulline00FDB011841(2s)-2-amino-5-(carbamoylamino)pentanoate;(2s)-2-amino-5-(carbamoylamino)pentanoic acid;(s)-2-amino-5-ureidopentanoate;(s)-2-amino-5-ureidopentanoic acid;(s)-2-amino-5-(aminocarbonyl)aminopentanoate;(s)-2-amino-5-(aminocarbonyl)aminopentanoic acid;2-amino-5-uredovalerate;2-amino-5-uredovaleric acid;2-amino-5-ureidovalerate;2-amino-5-ureidovaleric acid;A-amino-d-ureidovalerate;A-amino-d-ureidovaleric acid;Amino-ureidovalerate;Amino-ureidovaleric acid;Cir;Cit;Cytrulline;D-ureidonorvaline;Dl-citrulline;Gammaureidonorvaline;H-cit-oh;L(+)-2-amino-5-ureidovalerate;L(+)-2-amino-5-ureidovaleric acid;L(+)-citrulline;L-2-amino-5-ureido-valerate;L-2-amino-5-ureido-valeric acid;L-2-amino-5-ureidovalerate;L-2-amino-5-ureidovaleric acid;L-citrulline;L-cytrulline;L-n5-carbamoyl-ornithine;N()-carbamylornithine;N(5)-(aminocarbonyl)-dl-ornithine;N(delta)-carbamylornithine;N-carbamylornithine;N5-(aminocarbonyl)-l-ornithine;N5-(aminocarbonyl)ornithine;N5-(aminocarbonyl)-ornithine;N5-carbamoyl-l-ornithine;N5-carbamoylornithine;N5-carbamylornithine;N<sup>5</sup>-(aminocarbonyl)ornithine;Nd-carbamylornithine;Ndelta-carbamy-ornithine;Ndelta-carbamylornithine;Ngamma-carbamylornithine;Sitrulline;Ureidonorvaline;Ureidovalerate;Ureidovaleric acid;Alpha-amino-delta-ureidovalerate;Alpha-amino-delta-ureidovaleric acid;Alpha-amino-gamma-ureidovalerate;Alpha-amino-gamma-ureidovaleric acid;Delta-ureidonorvaline;Citrulline;N(5)-(aminocarbonyl)-l-ornithine;A-amino-delta-ureidovalerate;A-amino-delta-ureidovaleric acid;α-amino-δ-ureidovalerate;α-amino-δ-ureidovaleric acid;δ-ureidonorvaline;N(δ)-carbamylornithine;A-amino-δ-ureidovalerate;A-amino-δ-ureidovaleric acidPW_C000723Citruln728130441184419811881161127002907746411177495133120080122120403406122828135123046120125452297126991205396L-ArginineHMDB0000517Arginine is an essential amino acid that is physiologically active in the L-form. In mammals, arginine is formally classified as a semi-essential or conditionally essential amino acid, depending on the developmental stage and health status of the individual. Infants are unable to effectively synthesize arginine, making it nutritionally essential for infants. Adults, however, are able to synthesize arginine in the urea cycle. Arginine can be considered to be a basic amino acid as the part of the side chain nearest to the backbone is long, carbon-containing, and hydrophobic, whereas the end of the side chain is a complex guanidinium group. With a pKa of 12.48, the guanidinium group is positively charged in neutral, acidic, and even most basic environments. Because of the conjugation between the double bond and the nitrogen lone pairs, the positive charge is delocalized. This group is able to form multiple H-bonds. L-Arginine is an amino acid that has numerous functions in the body. It helps dispose of ammonia, is used to make compounds such as nitric oxide, creatine, L-glutamate, and L-proline, and it can be converted into glucose and glycogen if needed. In large doses, L-arginine also stimulates the release of the hormones growth hormone and prolactin. Arginine is a known inducer of mTOR (mammalian target of rapamycin) and is responsible for inducing protein synthesis through the mTOR pathway. mTOR inhibition by rapamycin partially reduces arginine-induced protein synthesis (PMID: 20841502). Catabolic disease states such as sepsis, injury, and cancer cause an increase in arginine utilization, which can exceed normal body production, leading to arginine depletion. Arginine also activates AMP kinase (AMPK) which then stimulates skeletal muscle fatty acid oxidation and muscle glucose uptake, thereby increasing insulin secretion by pancreatic beta-cells (PMID: 21311355). Arginine is found in plant and animal proteins, such as dairy products, meat, poultry, fish, and nuts. The ratio of L-arginine to lysine is also important: soy and other plant proteins have more L-arginine than animal sources of protein.74-79-3C000622878216467ARG6082DB00125N[C@@H](CCCNC(N)=N)C(O)=OC6H14N4O2InChI=1S/C6H14N4O2/c7-4(5(11)12)2-1-3-10-6(8)9/h4H,1-3,7H2,(H,11,12)(H4,8,9,10)/t4-/m0/s1ODKSFYDXXFIFQN-BYPYZUCNSA-N(2S)-2-amino-5-carbamimidamidopentanoic acid174.201174.111675712-1.885L-arginine01DBMET00502FDB002257(s)-2-amino-5-[(aminoiminomethyl)amino]pentanoate;(s)-2-amino-5-[(aminoiminomethyl)amino]pentanoic acid;(s)-2-amino-5-[(aminoiminomethyl)amino]-pentanoate;(s)-2-amino-5-[(aminoiminomethyl)amino]-pentanoic acid;2-amino-5-guanidinovalerate;2-amino-5-guanidinovaleric acid;5-[(aminoiminomethyl)amino]-l-norvaline;Arginine;L-(+)-arginine;L-a-amino-d-guanidinovalerate;L-a-amino-d-guanidinovaleric acid;L-alpha-amino-delta-guanidinovalerate;L-alpha-amino-delta-guanidinovaleric acid;N5-(aminoiminomethyl)-l-ornithine;(2s)-2-amino-5-(carbamimidamido)pentanoic acid;(2s)-2-amino-5-guanidinopentanoic acid;(s)-2-amino-5-guanidinopentanoic acid;(s)-2-amino-5-guanidinovaleric acid;Arg;L-arg;L-arginin;R;(2s)-2-amino-5-(carbamimidamido)pentanoate;(2s)-2-amino-5-guanidinopentanoate;(s)-2-amino-5-guanidinopentanoate;(s)-2-amino-5-guanidinovaleratePW_C000396Arg105834483562010756231171184619812732290425313224255431877467111780951127923929379240164120056122122142407122808135124694119125434297126299481126973205127863206200UreaHMDB0000294Urea is a highly soluble organic compound formed in the liver from ammonia produced by the deamination of amino acids. It is the principal end product of protein catabolism and constitutes about one half of the total urinary solids. Urea is formed in a cyclic pathway known simply as the urea cycle. In this cycle, amino groups donated by ammonia and L-aspartate are converted to urea. Urea is essentially a waste product; it has no physiological function. It is dissolved in blood (in humans in a concentration of 2.5 - 7.5 mmol/liter) and excreted by the kidney in the urine. In addition, a small amount of urea is excreted (along with sodium chloride and water) in human sweat.57-13-6C00086117616199UREA1143DB03904NC(N)=OCH4N2OInChI=1S/CH4N2O/c2-1(3)4/h(H4,2,3,4)XSQUKJJJFZCRTK-UHFFFAOYSA-Nurea60.055360.032362760.842urea00FDB012174Arf;Alphadrate;B-i-k;Bromisovalum;Bubber shet;Calmurid;Calmurid hc;Carbaderm;Carbamide;Carbamide resin;Carbonyl diamide;Carbonyl diamine;Carbonyldiamide;Carbonyldiamine;Carmol;Helicosol;Hyanit;Isourea;Keratinamin;Keratinamin kowa;Mocovina;Onychomal;Panafil;Ure;Urea;Ureaphil;Ureophil;Beta-i-k;E927b;H2nc(o)nh2;Harnstoff;Karbamid;Ur;UreePW_C000200Urea47287505260715260825756108118791984263031577493111778013341204001221205874081230431351232153741257274821272105021027ManganeseHMDB0001333Manganese is an essential trace nutrient in all forms of life. Physiologically, it. exists as an ion in the body. It is concentrated in cell mitochondria, mostly in the pituitary gland, liver, pancreas, kidney, and bone, influences the synthesis of mucopolysaccharides, stimulates hepatic synthesis of cholesterol and fatty acids, and is a cofactor in many enzymes, including arginase and alkaline phosphatase in the liver.16397-91-4C196102785429035MN%2b325916[Mn++]MnInChI=1S/Mn/q+2WAEMQWOKJMHJLA-UHFFFAOYSA-Nmanganese(2+) ion54.93854.9380496360manganese(2+) ion22FDB003636Manganese;Manganese (ii) ion;Manganese(ii);Manganese, ion (mn2+);Manganous ion;Mn(2+);Mn2+PW_C001027Mn2+274473814864915534322712239432513145394103545012055761336052155611316164971786926160748522211880198119392251195816412471249133601511522130677050294774941117783213277961112782673567849011578524331792472938003236811999640612040112212105812412121140712129538312137841912248840512304413512362211812378111912386539812393745512505437612537547912597649512605149012606029712615829912654348112664247812691750112742939012750350712751220512776538812811620612821820936Argininosuccinic acidHMDB0000052Arginosuccinic acid is a basic amino acid. Some cells synthesize it from citrulline, aspartic acid and use it as a precursor for arginine in the urea cycle or Citrulline-NO cycle. The enzyme that catalyzes the reaction is argininosuccinate synthetase. Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase. Defects in the arginosuccinate lyase enzyme can lead to arginosuccinate lyase deficiency. Argininosuccinate (ASA) lyase deficiency results in defective cleavage of ASA. This leads to an accumulation of ASA in cells and an excessive excretion of ASA in urine (arginosuccinic aciduria). In virtually all respects, this disorder shares the characteristics of other urea cycle defects. The most important characteristic of ASA lyase deficiency is its propensity to cause hyperammonemia in affected individuals. ASA in affected individuals is excreted by the kidney at a rate practically equivalent to the glomerular filtration rate (GFR). Whether ASA itself causes a degree of toxicity due to hepatocellular accumulation is unknown; such an effect could help explain hyperammonemia development in affected individuals. Regardless, the name of the disease is derived from the rapid clearance of ASA in urine, although elevated levels of ASA can be found in plasma. ASA lyase deficiency is associated with high mortality and morbidity rates. Symptoms of ASA lyase deficiency include anorexia, irritability rapid breathing, lethargy and vomiting. Extreme symptoms include coma and cerebral edema.2387-71-5C03406169501568216059N[C@@H](CCCNC(=N)N[C@@H](CC(O)=O)C(O)=O)C(O)=OC10H18N4O6InChI=1S/C10H18N4O6/c11-5(8(17)18)2-1-3-13-10(12)14-6(9(19)20)4-7(15)16/h5-6H,1-4,11H2,(H,15,16)(H,17,18)(H,19,20)(H3,12,13,14)/t5-,6-/m0/s1KDZOASGQNOPSCU-WDSKDSINSA-N(2S)-2-{N'-[(4S)-4-amino-4-carboxybutyl]carbamimidamido}butanedioic acid290.2731290.122634328-2.807argininosuccinic acid0-1FDB0218852-(n(omega)-l-arginine)succinate;2-(n(omega)-l-arginine)succinic acid;2-(n(omega)-l-arginino)succinate;2-(n(omega)-l-arginino)succinic acid;2-(nw-l-arginino)butanedioate;2-(nw-l-arginino)butanedioic acid;Asa;Argininosuccinate;Argininosuccinic acid;Arginosuccinate;Arginosuccinic acid;L-argininosuccinate;L-argininosuccinic acid;L-arginosuccinate;L-arginosuccinic acid;N(omega)-(l-arginino)succinate;N(omega)-(l-arginino)succinic acid;N-(((4-amino-4-carboxybutyl)amino)iminomethyl)-l-aspartate;N-(((4-amino-4-carboxybutyl)amino)iminomethyl)-l-aspartic acid;N-(l-arginino) succinate;N-(l-arginino) succinic acid;N-(l-arginino)succinate;N-(l-arginino)succinic acid;N-[(4-amino-4-carboxybutyl)amidino]-l-aspartate;N-[(4-amino-4-carboxybutyl)amidino]-l-aspartic acid;N-[[(4-amino-4-carboxybutyl)amino]iminomethyl]-l-aspartate;N-[[(4-amino-4-carboxybutyl)amino]iminomethyl]-l-aspartic acid;2-(nomega-l-arginino)succinate;2-(nomega-l-arginino)succinic acidPW_C000036ASA73811845198127012907746511112005512212280713512543329712697220588Fumaric acidHMDB0000134Fumaric acid is a precursor to L-malate in the Krebs tricarboxylic acid cycle. It is formed by the oxidation of succinate by succinate dehydrogenase. Fumarate is converted by fumarase to malate. A fumarate is a salt or ester of the organic compound fumaric acid, a dicarboxylic acid. (wikipedia).110-17-8C001222188378818012FUM10197150DB04299OC(=O)\C=C\C(O)=OC4H4O4InChI=1S/C4H4O4/c5-3(6)1-2-4(7)8/h1-2H,(H,5,6)(H,7,8)/b2-1+VZCYOOQTPOCHFL-OWOJBTEDSA-N(2E)-but-2-enedioic acid116.0722116.010958616-0.682fumaric acid0-2FDB003291(2e)-but-2-enedioate;(2e)-but-2-enedioic acid;(e)-2-butenedioate;(e)-2-butenedioic acid;2-(e)-butenedioate;2-(e)-butenedioic acid;Allomaleate;Allomaleic acid;Boletate;Boletic acid;Fc 33;Fumarate;Fumaric acid;Lichenate;Lichenic acid;Sodium fumarate;Trans-1,2-ethylenedicarboxylate;Trans-1,2-ethylenedicarboxylic acid;Trans-2-butenedioate;Trans-2-butenedioic acid;Trans-butenedioate;Trans-butenedioic acid;(2e)-2-butenedioic acid;E297;Fumarsaeure;Trans-but-2-enedioic acid;(2e)-2-butenedioate;Trans-but-2-enedioatePW_C000088Fumarat1028254172004250534538810260471566107162645810764591086492179676311768371667480223906515111804198127132904240032242496318424973157714813477466111791071328002736911780813311998938412004312212159912412266140612277212112279413512415711812523612012536948012542129712679347912691139112696020512756538812839150132Adenosine monophosphateHMDB0000045Adenosine monophosphate, also known as 5'-adenylic acid and abbreviated AMP, is a nucleotide that is found in RNA. It is an ester of phosphoric acid with the nucleoside adenosine. AMP consists of the phosphate group, the pentose sugar ribose, and the nucleobase adenine. AMP can be produced during ATP synthesis by the enzyme adenylate kinase. AMP has recently been approved as a 'Bitter Blocker' additive to foodstuffs. When AMP is added to bitter foods or foods with a bitter aftertaste it makes them seem 'sweeter'. This potentially makes lower calorie food products more palatable.61-19-8C00020608316027AMP5858DB00131NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1C10H14N5O7PInChI=1S/C10H14N5O7P/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(22-10)1-21-23(18,19)20/h2-4,6-7,10,16-17H,1H2,(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1UDMBCSSLTHHNCD-KQYNXXCUSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}phosphonic acid347.2212347.063084339-2.025adenylate0-2DBMET00485FDB0218065'-amp;5'-adenosine monophosphate;5'-adenylate;5'-adenylic acid;Amp;Adenosine 5'-monophosphate;Adenosine 5'-phosphate;Adenosine 5'-phosphorate;Adenosine 5'-phosphoric acid;Adenosine phosphate;Adenosine-5'-monophosphorate;Adenosine-5'-monophosphoric acid;Adenosine-5-monophosphorate;Adenosine-5-monophosphoric acid;Adenosine-monophosphate;Adenosine-phosphate;Adenovite;Adenylate;Adenylic acid;Cardiomone;Lycedan;Muscle adenylate;Muscle adenylic acid;My-b-den;My-beta-den;Phosaden;Phosphaden;Phosphentaside;5'-o-phosphonoadenosine;Adenosine 5'-(dihydrogen phosphate);Adenosine monophosphate;Adenosine-5'p;Adenosini phosphas;Ado5'p;Fosfato de adenosina;Pa;Pado;Phosphate d'adenosine;5'-adenosine monophosphoric acid;Adenosine phosphoric acid;Adenosine 5'-(dihydrogen phosphoric acid);Adenosine 5'-monophosphoric acid;Adenosine monophosphoric acid;Adenosine-5'-monophosphate;Phosphoric acid d'adenosinePW_C000032AMP112344628270167343288122118914457254867545033895251104540811754231035432118545712055581325583133577910157951086977199707218811789198118681611198815112003222125802261263631126942901333122542266342646315772343297732511178392334788091157932011280399180684135809007119916122120016124120031406120246382120888405121954408122920399123464376124507374125306297125394299125409479125596484126853205126934388126949501127124389127311209127711502140771891170PyrophosphateHMDB0000250The anion, the salts, and the esters of pyrophosphoric acid are called pyrophosphates. The pyrophosphate anion is abbreviated PPi and is formed by the hydrolysis of ATP into AMP in cells. This hydrolysis is called pyrophosphorolysis. The pyrophosphate anion has the structure P2O74-, and is an acid anhydride of phosphate. It is unstable in aqueous solution and rapidly hydrolyzes into inorganic phosphate. Pyrophosphate is an osteotoxin (arrests bone development) and an arthritogen (promotes arthritis). It is also a metabotoxin (an endogenously produced metabolite that causes adverse health affects at chronically high levels). Chronically high levels of pyrophosphate are associated with hypophosphatasia. Hypophosphatasia (also called deficiency of alkaline phosphatase or phosphoethanolaminuria) is a rare, and sometimes fatal, metabolic bone disease. Hypophosphatasia is associated with a molecular defect in the gene encoding tissue non-specific alkaline phosphatase (TNSALP). TNSALP is an enzyme that is tethered to the outer surface of osteoblasts and chondrocytes. TNSALP hydrolyzes several substances, including inorganic pyrophosphate (PPi) and pyridoxal 5'-phosphate (PLP), a major form of vitamin B6. When TSNALP is low, inorganic pyrophosphate (PPi) accumulates outside of cells and inhibits the formation of hydroxyapatite, one of the main components of bone, causing rickets in infants and children and osteomalacia (soft bones) in adults. Vitamin B6 must be dephosphorylated by TNSALP before it can cross the cell membrane. Vitamin B6 deficiency in the brain impairs synthesis of neurotransmitters which can cause seizures. In some cases, a build-up of calcium pyrophosphate dihydrate crystals in the joints can cause pseudogout.14000-31-8C0001364410218361PPI559142DB04160OP(O)(=O)OP(O)(O)=OH4O7P2InChI=1S/H4O7P2/c1-8(2,3)7-9(4,5)6/h(H2,1,2,3)(H2,4,5,6)XPPKVPWEQAFLFU-UHFFFAOYSA-N(phosphonooxy)phosphonic acid177.9751177.9432255064pyrophosphoric acid0-3FDB021918(4-)diphosphoric acid ion;(p2o74-)diphosphate;Diphosphate;Diphosphoric acid;Ppi;Pyrometaphosphate;Pyrophosphate;Pyrophosphate tetraanion;Pyrophosphate(4-) ion;[o3popo3](4-);Diphosphat;P2o7(4-);Pyrophosphat;Pyrophosphate ion;Phosphonato phosphoric acid;Pyrophosphoric acid;Pyrophosphoric acid ionPW_C000170Ppi122354638429237353288222121731620492410592815294175144868545034895252104529410154091175424103543311854581205548111555913255841335606135565510858791076239166697819970731887134163727216073121987318213827515182832101186916112002222120411641231522512323249125122881257922612695290152193061537518347601742561315426973187723532977317128776353367841633578928331791531127995013479958130800473728041717085630194786384948141259481938298678223110634391113270395113275389115527136115532399119934122120017124120032406120330410120936407121261429121341121121486383122407422122985444123502119123831464124044398124977375125324297125395299125410479125597484125656485125876481126552491126869205126935388126950501127337206128124508140772891353CalciumHMDB0000464Calcium is essential for the normal growth and maintenance of bones and teeth, and calcium requirements must be met throughout life. Requirements are greatest during periods of growth, such as childhood, during pregnancy and when breast-feeding. Long-term calcium deficiency can lead to osteoporosis, in which the bone deteriorates and there is an increased risk of fractures. Adults need between 1,000 and 1,300 mg of calcium in their daily diet. Calcium is essential for living organisms, particularly in cell physiology, and is the most common metal in many animals. Physiologically, it exists as an ion in the body. Calcium combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes. Calcium is an important component of a healthy diet. A deficit can affect bone and tooth formation, while overretention can cause kidney stones. Vitamin D is needed to absorb calcium. Dairy products, such as milk and cheese, are a well-known source of calcium. However, some individuals are allergic to dairy products and even more people, particularly those of non-European descent, are lactose-intolerant, leaving them unable to consume dairy products. Fortunately, many other good sources of calcium exist. These include: seaweeds such as kelp, wakame and hijiki; nuts and seeds (like almonds and sesame); beans; amaranth; collard greens; okra; rutabaga; broccoli; kale; and fortified products such as orange juice and soy milk. Calcium has also been found to assist in the production of lymphatic fluids.14127-61-8C0007627129108CA%2b2266DB01373[Ca++]CaInChI=1S/Ca/q+2BHPQYMZQTOCNFJ-UHFFFAOYSA-Ncalcium(2+) ion40.07839.9625911550calcium(2+) ion22FDB003513Ca;Calcium element;Ca(2+);Ca2+;Calcium ion;Calcium, doubly charged positive ionPW_C000353Ca2+276163038553146012941159932199735104631163461164471478491491421552432116582138172796182937931597131607239422941866647821048222853401115780101717920572322117258160728119011774213118371981184221012198164122152851528815115350308693361773893317760011578154132782663567852634578724130789081148041374805892288182651120220122120465405121049124121300418121377419121850383121923125122370409122895135123099376123613118123870454123936455124403398124476136124924137125571297125711478125981489126009299126050490126533495127203209127434506127460388127502507128105390140676790140677834140695501700Neutral amino acid transporter B(0)Q15758Has a broad substrate specificity, a preference for zwitterionic amino acids, and a sodium-dependence. It accepts as substrates all neutral amino acids, including glutamine, asparagine, and branched-chain and aromatic amino acids, and excludes methylated amino acids, anionic amino acids, and cationic amino acids. Act as a cell surface receptor for feline endogenous virus RD114, baboon M7 endogenous virus and type D simian retrovirusesHMDBP01955SLC1A519q13.3GQ91905811420847514316314143925532251Neutral amino acid transporter AP43007Transporter for alanine, serine, cysteine, and threonine. Exhibits sodium dependenceHMDBP03110SLC1A42p15-p13AK29568711431641414400553144244308795Alanine aminotransferase 1P24298Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate. Participates in cellular nitrogen metabolism and also in liver gluconeogenesis starting with precursors transported from skeletal muscles (By similarity).
HMDBP00850GPT8q24.3U7073212.6.1.2192145926183284110465348512624311404528021404537140509114343839314372632214409053639Glutaminase liver isoform, mitochondrialQ9UI32Plays an important role in the regulation of glutamine catabolism. Promotes mitochondrial respiration and increases ATP generation in cells by catalyzing the synthesis of glutamate and alpha-ketoglutarate. Increases cellular anti-oxidant function via NADH and glutathione production. May play a role in preventing tumor proliferation.
HMDBP00675GLS212q13CH47105413.5.1.2137844544132314391354658Glutamate dehydrogenase 1, mitochondrialP00367
Mitochondrial glutamate dehydrogenase that converts L-glutamate into alpha-ketoglutarate. Plays a key role in glutamine anaplerosis by producing alpha-ketoglutarate, an important intermediate in the tricarboxylic acid cycle. May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate (By similarity).
HMDBP00694GLUD110q23.3X6630611.4.1.3144414705448753352Carbamoyl-phosphate synthase [ammonia], mitochondrialP31327Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
HMDBP00359CPS12q35CH47106316.3.4.1644244841348573278Aspartate aminotransferase, mitochondrialP00505Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol. Facilitates cellular uptake of long-chain free fatty acids.
HMDBP00284GOT216q21BC00052512.6.1.1; 2.6.1.711023111142416944876814182996214213954870Ornithine carbamoyltransferase, mitochondrialP00480HMDBP00927OTCXp21.1AK29275712.1.3.346041439156143916541882Mitochondrial ornithine transporter 1Q9Y619Ornithine transport across inner mitochondrial membrane, from the cytoplasm to the matrix.
HMDBP02312SLC25A1513q14AL161614184421714404255292Arginase-1P05089HMDBP00298ARG16q23AY07448813.5.3.147481437001164143842231440391197661Argininosuccinate lyaseP04424HMDBP00697ASL7q11.21BC03314614.3.2.1106814314711437221164143918261440411197622Argininosuccinate synthaseP00966HMDBP00656ASS19q34.1L0008316.3.4.5748143146114372111641439172614404011972270Calcium-binding mitochondrial carrier protein Aralar1O75746Catalyzes the calcium-dependent exchange of cytoplasmic glutamate with mitochondrial aspartate across the mitochondrial inner membrane. May have a function in the urea cycle.
HMDBP03143SLC25A122q24AC0680391844517141827552362Alanine aminotransferase 2Q8TD30Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate.
HMDBP03670GPT216q12.1AC01884512.6.1.214515018334748Neutral amino acid transporter B(0)1PW_P00074884317001396Neutral amino acid transporter A1PW_P000396418225112Alanine aminotransferase 11PW_P000002379522114814236Glutaminase liver isoform, mitochondrial1PW_P0000363763911311332Glutamate dehydrogenase 1, mitochondrial1PW_P0000323365861254112Carbamoyl-phosphate synthase [ammonia], mitochondrial1PW_P000112127352143913304Aspartate aminotransferase, mitochondrial1PW_P000304323278213411481117Ornithine carbamoyltransferase, mitochondrial1PW_P00011713487034574365Mitochondrial ornithine transporter1PW_P00036538718821123Arginase-11PW_P00012314129235810272471817Argininosuccinate lyase1PW_P000017186614104111Argininosuccinate synthase1PW_P000011126224711305Calcium-binding mitochondrial carrier protein Aralar11PW_P000305324227011353531395Alanine aminotransferase 21PW_P00039541723622191114812falsePW_R000002Both71051Compoundfalse81341Compoundtrue9951Compoundtrue101641Compoundfalse222.6.1.24993952.6.1.243falsePW_R000043Right1625001Compoundfalse16314201Compoundtrue164951Compoundfalse165351Compoundtrue43363.5.1.238falsePW_R000038Right136951Compoundfalse13714201Compoundtrue1387211Compoundtrue1391341Compoundfalse140351Compoundtrue14111441Compoundtrue38321.4.1.3208falsePW_R000208Right9124142Compoundtrue913351Compoundfalse91413161Compoundtrue91514201Compoundtrue91610342Compoundtrue91714411Compoundtrue9188621Compoundfalse1331126.3.4.1678falsePW_R000078Both3031231Compoundfalse3041341Compoundfalse3051481Compoundtrue306951Compoundfalse372304423falsePW_R000423Right17398621Compoundfalse17401401Compoundfalse174111041Compoundtrue17427231Compoundfalse1361172.1.3.3164falsePW_R000164Right7363961Compoundfalse73714201Compoundtrue7381401Compoundfalse7392001Compoundtrue1371233.5.3.117falsePW_R000017Right80362Compoundfalse81881Compoundfalse823961Compoundfalse18174.3.2.111falsePW_R000011Right494141Compoundtrue507231Compoundfalse511231Compoundfalse52321Compoundtrue531701Compoundtrue54361Compoundfalse12116.3.4.5139PW_T0001391615001Compound158Right1017482013-09-05T02:17:20-06:002013-09-05T02:17:20-06:0014141PW_T0001411631051Compound158Right1033962013-09-05T02:21:55-06:002013-09-05T02:21:55-06:001439PW_T000039411401Compound84Right427230Compound84Left123652013-07-17T12:26:44-06:002013-07-17T12:26:44-06:001731PW_T000031311231Compound442Right32951Compound442Left73052013-07-09T11:27:41-06:002013-07-09T11:27:41-06:001760725001581false61025010regular2001906073500881false610138010regular20019060741051581false133025010regular2001906075105881false133086010regular2001908282134481false1125102510regular200190828395481false1330138010regular2001908284164481false1125123010regular2001908285114849false1387116519regular1003583211420349false845153510regular7878832235363false1181178810regular787883241420449false1290158510regular78788325721459false1480160510regular50308326134481false1560184710regular20019083271144460false1485180710regular50308329414442false1117195710regular503083301316452false1338184810regular787883311420449false1270193710regular787883321034443false1120212610regular503083331441446false1268211910regular44438334862481false1118218610regular2001908495123481false2140191210regular2001908496148481false2065138010regular2001908497114849false1830166319regular100358504140481false813241110regular20019085051104446false1546237210regular44438506723481false1828240810regular2001908524140881false813295110regular2001908525723881true488295610regular2001908526723481true488241110regular2001908531396881false813349110regular20019085321420849false773339610regular78788533200881false1018312110regular2001908534102789false863331119regular10025853736881false1828349110regular200190853888881false1218370110regular2001908544140881true2073291310regular2001908545140481true2073234810regular2001908546723881false1828295310regular2001908547414842false1818324310regular50308548123881false2153305810regular200190854932844false1815342610regular50308550170845false1988340910regular6343863195481false2275238210regular2001908632954281false2288342310regular20019086333539false2260269520regular100252490170076false6354958subunitregular150702492225176false13554958subunitregular15070418179546false135211758subunitregular16080422763932false95214408subunitregular150704232658417false134016738subunitregular18085423535242false114220248subunitregular15070447527846false179516688subunitregular160804481870416false133324618subunitregular17090449718821776false838271119subunitregular150704503292816false82833068subunitregular17090451066188false144835468subunitregular14085452118821776false1853271819subunitregular15070452462288false185833098subunitregular14085464922701776false2240271019subunitregular15070222874810724832490223039610724852492347421074415941811533828512483Cofactor352036107342054227352532107442104232352811210744213423536983041074445244751603849712952Cofactor370411710744458448137203651074474449737261231078448045031620853413023Cofactor3733171078448745103744365107449845213747111078450145243836305107462446491653863313295Cofactor9225M710 440 C710 470 710 465 710 495 83false18trueM 702.5 382.0096189432334 L 710 395 L 717.5 382.0096189432334false9226M710 1380 C710 1350 710 595 710 565 83false18trueM 42.5 307.0096189432334 L 50 320 L 57.5 307.0096189432334false9229M1430 440 C1430 470 1430 466 1430 496 83false18trueM 1422.5 383.0096189432334 L 1430 396 L 1437.5 383.0096189432334false9230M1430 860 C1430 830 1430 595 1430 565 83false18trueM 432.5 552.0096189432334 L 440 565 L 447.5 552.0096189432334false12479M1430 1050 C1430 1080 1432 1145 1432 1175 5false18falsefalse12480M1325 1120 C1367 1120 1431 1130 1432 1175 5false18falsefalse12481M1430 1380 C1430 1326 1432 1335 1432 1255 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false12482M1325 1325 C1367 1325 1430 1315 1432 1255 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false12483M1707.5 950 L1707.5 1000 L1757.5 950 z10true1812592M810 1475 C847 1475 922 1475 952 1475 5false1812593M884 1535 C884 1509 922 1475 952 1475 5false1812594M1330 1475 C1300 1475 1132 1475 1102 1475 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false12595M1220 1788 C1221 1630 1219 1472 1102 1475 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false12601M1430 1570 C1430 1600 1430 1643 1430 1673 5false1812602M1368 1624 C1410 1624 1430 1643 1430 1673 5false1812603M1480 1620 C1455 1621 1430 1643 1430 1673 5false1812604M1560 1942 C1517 1942 1458 1942 1431 1941 C1431 1894 1430 1858 1430 1758 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false12605M1259 1827 C1335 1827 1430 1805 1430 1758 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false12606M1485 1822 C1456 1825 1430 1788 1430 1758 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false12610M1167 1972 C1198 1972 1217 1994 1217 2024 5false1812611M1220 1866 C1220 1896 1217 1994 1217 2024 5false1812612M1338 1887 C1286 1887 1218 1928 1217 2024 5false1812613M1270 1976 C1234 1976 1217 1994 1217 2024 5false1812614M1170 2141 C1199 2141 1217 2124 1217 2094 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false12615M1268 2140.5 C1231 2140.5 1217 2124 1217 2094 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false12616M1218 2186 C1218 2156 1217 2124 1217 2094 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false12948M2140 2007 C2032 2004 1877 1878 1875 1748 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false12949M1760 1942 C1794 1942 1849 1941 1879 1941 C1878 1907 1876 1848 1875 1748 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 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Space190530201.61.620015295305314753516852722277640#FFEBEB423712091Carbamoyl Phosphate Synthetase DeficiencyCCarbamoyl Phosphate Synthetase Deficiency, also called hyperammonemia due to carbamoyl phosphate synthetase 1 deficiency, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of the urea cycle caused by a defective CPS1 gene. The CPS1 gene codes for the protein carbamoyl phosphate synthetase I, which plays a role in the urea cycle. This disorder is characterized by a large accumulation of ammonia in the blood. Symptoms of the disorder include unusual movements, seizures, unusual sleeping or coma. Treatment with citrulline or arginine, which maintains a regular rate of protein creation. It is estimated that carbamoyl phosphate synthetase deficiency affects 1 in 800,000 individuals in Japan.
DiseasePW_X000054Context54274352ProteinMutated27535CompoundIncreased71624TissueDamaged9[Uniprot: P31327](http://www.uniprot.org/uniprot/P31327)54Context10[OMIM: Entry 237300](http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=237300)54Context11[eMedicine: Article 942159-overview](http://emedicine.medscape.com/article/942159-overview)54Context128486760Hoshide R, Matsuura T, Haraguchi Y, Endo F, Yoshinaga M, Matsuda I: Carbamyl phosphate synthetase I deficiency. One base substitution in an exon of the CPS I gene causes a 9-basepair deletion due to aberrant splicing. J Clin Invest. 1993 May;91(5):1884-7. doi: 10.1172/JCI116405.54Context137246541McReynolds JW, Crowley B, Mahoney MJ, Rosenberg LE: Autosomal recessive inheritance of human mitochondrial carbamyl phosphate synthetase deficiency. Am J Hum Genet. 1981 May;33(3):345-53.54Context149711878Finckh U, Kohlschutter A, Schafer H, Sperhake K, Colombo JP, Gal A: Prenatal diagnosis of carbamoyl phosphate synthetase I deficiency by identification of a missense mutation in CPS1. Hum Mutat. 1998;12(3):206-11. doi: 10.1002/(SICI)1098-1004(1998)12:3<206::AID-HUMU8>3.0.CO;2-E.54Context27802715876373Yefimenko I, Fresquet V, Marco-Marin C, Rubio V, Cervera J: Understanding carbamoyl phosphate synthetase deficiency: impact of clinical mutations on enzyme functionality. J Mol Biol. 2005 May 27;349(1):127-41. doi: 10.1016/j.jmb.2005.03.078. Epub 2005 Apr 12.54Context279384Craigen WJ. Mouse Models of Human Genetic Disorders. In: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. eds. The Online Metabolic and Molecular Bases of Inherited Disease New York, NY: McGraw-Hill; 2014. http://ommbid.mhmedical.com/content.aspx?bookid=971§ionid=62642751.54Context