8974ContextTyrosinemia Type 2 (or Richner-Hanhart Syndrome)Tyrosinemia II also known as Richner-Hanhart syndrome is an autosomal recessive disorder caused by a mutation in the TAT gene the encodes for tyrosine aminotransferase. A defect in this enzyme causes excess tyrosine to accumulate in the blood and urine, tyrosine crystals to form in the cornea, and increased excretion in the urine of 4-hydroxyphenylpyruvic acid, hydroxyphenyllactic acid, and p-hydroxyphenylacetic acid. Symptoms commonly appear in early childhood and include: mental retardation, photophobia (increased sensitivity to light), excessive tearing, eye redness and pain and skin lesions of the palms and soles. The patient is treated with restriction of dietary phenylalanine and tyrosine. Sometimes a tyrosine degradation inhibitor is also used to prevents the formation of fumarylacetoacetate from tyrosine. Trosinemia II is commonly misdiagnosed as herpes simplex keratitis. DiseasePW121767CenterPathwayVisualizationContext12204323003000#000099PathwayVisualization6437564491Phenylalanine and Tyrosine MetabolismIn man, phenylalanine is an essential amino acid which must be supplied in the dietary proteins. Once in the body, phenylalanine may follow any of three paths. It may be (1) incorporated into cellular proteins, (2) converted to phenylpyruvic acid, or (3) converted to tyrosine. Tyrosine is found in many high protein food products such as soy products, chicken, turkey, fish, peanuts, almonds, avocados, bananas, milk, cheese, yogurt, cottage cheese, lima beans, pumpkin seeds, and sesame seeds. Tyrosine can be converted into L-DOPA, which is further converted into dopamine, norepinephrine (noradrenaline), and epinephrine (adrenaline). Depicted in this pathway is the conversion of phenylalanine to phenylpyruvate (via amino acid oxidase or tyrosine amino transferase acting on phenylalanine), the incorporation of phenylalanine and/or tyrosine into polypeptides (via tyrosyl tRNA synthetase and phenylalyl tRNA synthetase) and the conversion of phenylalanine to tyrosine via phenylalanine hydroxylase. This reaction functions both as the first step in tyrosine/phenylalanine catabolism by which the body disposes of excess phenylalanine, and as a source of the amino acid tyrosine. The decomposition of L-tyrosine begins with an α-ketoglutarate dependent transamination through the tyrosine transaminase to para-hydroxyphenylpyruvate. The next oxidation step catalyzed by p-hydroxylphenylpyruvate-dioxygenase creates homogentisate. In order to split the aromatic ring of homogentisate, a further dioxygenase, homogentistate-oxygenase, is required to create maleylacetoacetate. Fumarylacetate is created by the action maleylacetoacetate-cis-trans-isomerase through rotation of the carboxyl group created from the hydroxyl group via oxidation. This cis-trans-isomerase contains glutathione as a coenzyme. Fumarylacetoacetate is finally split via fumarylacetoacetate-hydrolase into fumarate (also a metabolite of the citric acid cycle) and acetoacetate (3-ketobutyroate). Metabolic125761625SubPathway55579103Compound1115761787SubPathway55580103Compound111576182SubPathway5558188Compound1115558242Compound111229766Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.64491Pathway229767Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.64491Pathway1CellCL:00000006MyocyteCL:00001875HepatocyteCL:00001823NeuronCL:00005404Cardiomyocyte CL:00007467Epithelial CellCL:00000662Platelet CL:00002338Beta cellCL:00006391Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote4Arabidopsis thaliana3702EukaryoteThale cress23Pseudomonas aeruginosa287Prokaryote12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat24Solanum lycopersicum4081EukaryoteTomato18Saccharomyces cerevisiae4932EukaryoteYeast21Xenopus laevis8355EukaryoteAfrican clawed frog49Bathymodiolus platifrons220390EukaryoteDeep sea mussel60Nitzschia sp.0001EukaryoteNitzschia410Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm2Bacteria2ProkaryoteBacteria19Schizosaccharomyces pombe4896Eukaryote25Escherichia coli (strain K12)83333Prokaryote51Picea sitchensis3332EukaryoteSitka spruce5CytoplasmGO:000573731Periplasmic SpaceGO:00056201CytosolGO:000582911Extracellular SpaceGO:000561535ChloroplastGO:00095072MitochondrionGO:00057393Mitochondrial MatrixGO:000575913Endoplasmic ReticulumGO:000578324Mitochondrial Intermembrane SpaceGO:00057584PeroxisomeGO:000577710Cell MembraneGO:00058866LysosomeGO:00057647Endoplasmic Reticulum MembraneGO:000578916Lysosomal LumenGO:004320218Melanosome MembraneGO:003316225Golgi apparatusGO:000579414Mitochondrial Outer MembraneGO:000574112Mitochondrial Inner MembraneGO:000574320Endoplasmic Reticulum LumenGO:000578821SynapseGO:004520215NucleusGO:000563436MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032540PeriplasmGO:004259732Inner MembraneGO:007025839Mitochondrial membraneGO:003196654Endocytic VesicleGO:003013955Exocytic VesicleGO:007038227Peroxisome MembraneGO:000577819sarcoplasmic reticulumGO:001652926Golgi apparatus membraneGO:00001391LiverBTO:00007597299MuscleBTO:00008871411824BrainBTO:000014289163Sympathetic Nervous SystemBTO:00018324Adrenal MedullaBTO:000004971825IntestineBTO:000064828StomachBTO:0001307155267Nervous SystemBTO:00014848Blood VesselBTO:0001102741111HeartBTO:000056273102Endothelium BTO:000039318PancreasBTO:00009885cardiocyteBTO:00015398511PW_BS000008107313PW_BS00010710813PW_BS000108105113PW_BS0001052253541PW_BS000024151141PW_BS000151315123PW_BS0000243183123PW_BS000024253541PW_BS0000241115121PW_BS000111122551PW_BS0001221355171PW_BS0001352111PW_BS0000023211PW_BS0000034311PW_BS000004181311PW_BS000018311511PW_BS000031422411PW_BS0000425411PW_BS000005509516PW_BS000050261115PW_BS000026541315PW_BS000054103331PW_BS000103117131PW_BS0001171181171PW_BS0001181321121PW_BS0001321471241PW_BS0001471553241PW_BS0001551572241PW_BS0001571613181PW_BS0001611783211PW_BS00017885241011PW_BS000085222341PW_BS000024224241PW_BS00002422014PW_BS0000241985181PW_BS0000242892491PW_BS0000241333121PW_BS0001331122121PW_BS00011212915121PW_BS00012934524121PW_BS00002834695126PW_BS00002832711125PW_BS00002834713125PW_BS0000283344121PW_BS0000283683601PW_BS000028943PW_BS000094406351PW_BS000115124151PW_BS000124407251PW_BS0001154141551PW_BS0001154182451PW_BS000115408451PW_BS0001154239556PW_BS0001154241155PW_BS0001154251355PW_BS0001151203171PW_BS0001201192171PW_BS00011945015171PW_BS00011545424171PW_BS0001153744171PW_BS00005345895176PW_BS00011545911175PW_BS00011546013175PW_BS0001154793101PW_BS0001152991101PW_BS0000244812101PW_BS0001152975101PW_BS00002448924101PW_BS0001154824101PW_BS000115501361PW_BS000115388161PW_BS000112206261PW_BS000024205561PW_BS0000245062461PW_BS000115502461PW_BS00011511PW_BS00000114101PW_BS0000149611PW_BS0000091136121PW_BS0001131873118PW_BS000024188118PW_BS00002472513PW_BS000072711113PW_BS000071207661PW_BS0000242905491PW_BS0000243201123PW_BS000024126651PW_BS0001264436171PW_BS0001153016101PW_BS000024193513PW_BS000019204111PW_BS000020101711PW_BS00001049711PW_BS0000491601181PW_BS00016021217181PW_BS0000242137181PW_BS00002429341PW_BS00002434141121PW_BS00002833217121PW_BS0000283317121PW_BS000028711PW_BS0000071231751PW_BS000123383751PW_BS00010044717171PW_BS0001153987171PW_BS00011329817101PW_BS0000244957101PW_BS0001155131761PW_BS000115390761PW_BS00011213121PW_BS00001315111PW_BS0000152811611PW_BS000028331811PW_BS000033432511PW_BS0000432441011PW_BS000024221411PW_BS00002260251PW_BS00006046114PW_BS000046171211PW_BS00001729111PW_BS0000297028511PW_BS000070612517PW_BS0000613612011PW_BS0000363772113PW_BS00003793252011PW_BS00009327151PW_BS000027971521PW_BS000097100521PW_BS000100110231PW_BS0001101251351PW_BS00012512711651PW_BS00012713013121PW_BS0001301141112PW_BS0001146131PW_BS000006140103PW_BS000140101531PW_BS00010114315191PW_BS0001431465191PW_BS000146951721PW_BS0000951632181PW_BS00016315924PW_BS00015916611PW_BS0001661802211PW_BS00018015284PW_BS0001522111018PW_BS00002421425181PW_BS0000242156181PW_BS0000242164181PW_BS00002421013181PW_BS0000241901118PW_BS00002417018PW_BS000170226441PW_BS00002416212181PW_BS0001622771218PW_BS0000241644PW_BS0001642811251PW_BS0000242851041PW_BS0000242863641PW_BS0000242875341PW_BS0000242491341PW_BS0000242273441PW_BS00002465111PW_BS0000652916491PW_BS0000242924491PW_BS00002430013101PW_BS000024302116101PW_BS0000242231241PW_BS0000242941141PW_BS0000243081011PW_BS0000243221231PW_BS00002413412121PW_BS0001343331212PW_BS0000281151012PW_BS0001153361121PW_BS000028337116121PW_BS00002834318121PW_BS00002832914121PW_BS0000283522512PW_BS00002835325127PW_BS00002835625121PW_BS000028360410121PW_BS0000283702601PW_BS000028228361PW_BS0000243841251PW_BS0001003911261PW_BS000112232403PW_BS000024412125PW_BS000115405105PW_BS000115409115PW_BS0001154151851PW_BS000115429151PW_BS00011512112171PW_BS0001214192551PW_BS00011543441051PW_BS0001153821451PW_BS000100436255PW_BS0001154461217PW_BS0001153761017PW_BS0000531371117PW_BS00013713613171PW_BS000136448116171PW_BS00011545118171PW_BS0001154641171PW_BS00011545525171PW_BS000115469410171PW_BS00011539914171PW_BS0001134712517PW_BS00011547225177PW_BS0001154831110PW_BS0001154781010PW_BS00011548718101PW_BS00011549025101PW_BS00011548414101PW_BS00011548012101PW_BS000115208116PW_BS000024209106PW_BS0000245041861PW_BS0001155072561PW_BS00011551541061PW_BS0001153891461PW_BS0001123951361PW_BS00011316212PW_BS000016109323PW_BS0001095181PW_BS000051326812PW_BS00002841685PW_BS000115452817PW_BS000115488810PW_BS00011550586PW_BS0001155811411PW_BS0000581021231PW_BS00010215612241PW_BS00015617912211PW_BS0001793583912PW_BS00002836912601PW_BS000028231511PW_BS00002312815121PW_BS0001283775411PW_BS0000693785511PW_BS00006940254121PW_BS00011540355121PW_BS0001154415451PW_BS0001154425551PW_BS0001154101551PW_BS00011547654171PW_BS00011547755171PW_BS00011544415171PW_BS0001153211515PW_BS000032397113PW_BS000039592711PW_BS0000596618518PW_BS000066918511PW_BS000091892PW_BS0000891041431PW_BS00010419914181PW_BS0000242171518PW_BS00002421815181PW_BS000024350114121PW_BS0000283511512PW_BS00002833527121PW_BS000028184121PW_BS00002443311451PW_BS0001154222751PW_BS000115435155PW_BS000115468114171PW_BS00011537527171PW_BS0000534701517PW_BS00011548515101PW_BS00011549127101PW_BS0001154991510PW_BS0001155161561PW_BS0001155082761PW_BS000115517156PW_BS0001152881441PW_BS00002430635511PW_BS000024372102PW_BS000028215114PW_BS0000213551914PW_BS000035471914PW_BS0000477413PW_BS000074562611PW_BS000056104L-PhenylalanineHMDB0000159Phenylalanine is an essential amino acid and the precursor of the amino acid tyrosine. Like tyrosine, phenylalanine is also a precursor for catecholamines including tyramine, dopamine, epinephrine, and norepinephrine. Catecholamines are neurotransmitters that act as adrenalin-like substances. Interestingly, several psychotropic drugs (mescaline, morphine, codeine, and papaverine) also have phenylalanine as a constituent. Phenylalanine is highly concentrated in the human brain and plasma. Normal metabolism of phenylalanine requires biopterin, iron, niacin, vitamin B6, copper, and vitamin C. An average adult ingests 5 g of phenylalanine per day and may optimally need up to 8 g daily. Phenylalanine is highly concentrated in a number of high protein foods, such as meat, cottage cheese, and wheat germ. An additional dietary source of phenylalanine is artificial sweeteners containing aspartame. As a general rule, aspartame should be avoided by phenylketonurics and pregnant women. When present in sufficiently high levels, phenylalanine can act as a neurotoxin and a metabotoxin. A neurotoxin is a compound that disrupts or attacks neural cells and neural tissue. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of phenylalanine are associated with at least five inborn errors of metabolism, including Hartnup disorder, hyperphenylalaninemia due to guanosine triphosphate cyclohydrolase deficiency, phenylketonuria (PKU), tyrosinemia type 2 (or Richner-Hanhart syndrome), and tyrosinemia type III (TYRO3). Phenylketonurics have elevated serum plasma levels of phenylalanine up to 400 times normal. High plasma concentrations of phenylalanine influence the blood-brain barrier transport of large neutral amino acids. The high plasma phenylalanine concentrations increase phenylalanine entry into the brain and restrict the entry of other large neutral amino acids (PMID: 19191004). Phenylalanine has been found to interfere with different cerebral enzyme systems. Untreated phenylketonuria (PKU) can lead to intellectual disability, seizures, behavioural problems, and mental disorders. It may also result in a musty smell and lighter skin. Classic PKU dramatically affects myelination and white matter tracts in untreated infants; this may be one major cause of neurological disorders associated with phenylketonuria. Mild phenylketonuria can act as an unsuspected cause of hyperactivity, learning problems, and other developmental problems in children. It has been recently suggested that PKU may resemble amyloid diseases, such as Alzheimer's disease and Parkinson's disease, due to the formation of toxic amyloid-like assemblies of phenylalanine (PMID: 22706200). Phenylalanine also has some potential benefits. Phenylalanine can act as an effective pain reliever. Its use in premenstrual syndrome and Parkinson's may enhance the effects of acupuncture and electric transcutaneous nerve stimulation (TENS). Phenylalanine and tyrosine, like L-DOPA, produce a catecholamine-like effect. Phenylalanine is better absorbed than tyrosine and may cause fewer headaches. Low phenylalanine diets have been prescribed for certain cancers with mixed results. For instance, some tumours use more phenylalanine than others (particularly melatonin-producing tumours called melanomas).63-91-2C00079614017295PHE5910DB00120N[C@@H](CC1=CC=CC=C1)C(O)=OC9H11NO2InChI=1S/C9H11NO2/c10-8(9(11)12)6-7-4-2-1-3-5-7/h1-5,8H,6,10H2,(H,11,12)/t8-/m0/s1COLNVLDHVKWLRT-QMMMGPOBSA-N(2S)-2-amino-3-phenylpropanoic acid165.1891165.078978601-1.602L-phenylalanine00FDB014705(-)-beta-phenylalanine;(l)-phenylalanine;(s)-(-)-phenylalanine;(s)-2-amino-3-phenylpropionate;(s)-2-amino-3-phenylpropionic acid;(s)-2-amino-3-phenylpropanoate;(s)-2-amino-3-phenylpropanoic acid;(s)-phenylalanine;(s)-alpha-amino-benzenepropanoate;(s)-alpha-amino-benzenepropanoic acid;(s)-alpha-amino-beta-phenylpropionate;(s)-alpha-amino-beta-phenylpropionic acid;(s)-alpha-aminobenzenepropanoate;(s)-alpha-aminobenzenepropanoic acid;(s)-alpha-aminohydrocinnamate;(s)-alpha-aminohydrocinnamic acid;3-phenyl-l-alanine;L-2-amino-3-phenylpropionate;L-2-amino-3-phenylpropionic acid;Phe;Phenyl-alanine;Phenylalamine;Phenylalanine;Alpha-aminohydrocinnamate;Alpha-aminohydrocinnamic acid;Beta-phenyl-l-alanine;Beta-phenyl-alpha-alanine;Beta-phenylalanine;F;(s)-a-amino-b-phenylpropionate;(s)-a-amino-b-phenylpropionic acid;(s)-α-amino-β-phenylpropionate;(s)-α-amino-β-phenylpropionic acid;B-phenyl-l-alanine;β-phenyl-l-alaninePW_C000104Phe127985669107567010858881058340225834115142458315424593187706125378463111120997122123562135134Oxoglutaric 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_C000134AKG1524231414146849918673311108421263514475014552614675453751035414117543811855641326008147603615560691576092161648217865308574712227515224751915182092258374220118631981268128977054253771351337748111177523112777461297796734577970346779763277798434778425334800183688069413511316294119972406120022124120084407120174122120552414120814418120989408121146423121152424121160425122757120122831119123186450123399454123554374123718458123724459123732460125357479125400299125455481125533297125800489125929482126900501126940388126993206127066205127255506127388502131Phenylpyruvic acidHMDB0000205Phenylpyruvic acid is a keto-acid that is an intermediate or catabolic byproduct of phenylalanine metabolism. It has a slight honey-like odor. Levels of phenylpyruvate are normally very low in blood or urine. High levels of phenylpyruvic acid can be found in the urine of individuals with phenylketonuria (PKU). PKU is due to lack of the enzyme phenylalanine hydroxylase (PAH), so that phenylalanine is converted not to tyrosine but to phenylpyruvic acid. In particular, excessive phenylalanine can be metabolized into phenylketones through, a transaminase pathway route involving glutamate. Metabolites of this transamination reaction include phenylacetate, phenylpyruvate and phenethylamine. In persons with PKU, dietary phenylalanine either accumulates in the body or some of it is converted to phenylpyruvic acid. Individuals with PKU tend to excrete large quantities of phenylpyruvate, phenylacetate and phenyllactate, along with phenylalanine, in their urine. If untreated, mental retardation effects and microcephaly are evident by the first year along with other symptoms which include: unusual irritability, epileptic seizures and skin lesions. Hyperactivity, EEG abnormalities and seizures, and severe learning disabilities are major clinical problems later in life. A "musty or mousy" odor of skin, hair, sweat and urine (due to phenylacetate accumulation); and a tendency to hypopigmentation and eczema are also observed. The neural-development effects of PKU are primarily due to the disruption of neurotransmitter synthesis. In particular, phenylalanine is a large, neutral amino acid which moves across the blood-brain barrier (BBB) via the large neutral amino acid transporter (LNAAT). Excessive phenylalanine in the blood saturates the transporter. Thus, excessive levels of phenylalanine significantly decrease the levels of other LNAAs in the brain. But since these amino acids are required for protein and neurotransmitter synthesis, phenylalanine accumulation disrupts brain development, leading to mental retardation.156-06-9C0016699730851PHENYL-PYRUVATE972DB03884OC(=O)C(=O)CC1=CC=CC=C1C9H8O3InChI=1S/C9H8O3/c10-8(9(11)12)6-7-4-2-1-3-5-7/h1-5H,6H2,(H,11,12)BTNMPGBKDVTSJY-UHFFFAOYSA-N2-oxo-3-phenylpropanoic acid164.158164.047344122-2.251phenylpyruvic acid0-1FDB0201972-oxo-3-phenylpropanoate;2-oxo-3-phenylpropanoic acid;3-phenyl-2-oxopropanoate;3-phenyl-2-oxopropanoic acid;3-phenylpyruvate;3-phenylpyruvic acid;Keto-phenylpyruvate;Phenylpyroracemate;Phenylpyroracemic acid;Phenylpyruvate;Alpha-ketohydrocinnamate;Alpha-ketohydrocinnamic acid;B-phenylpyruvate;B-phenylpyruvic acid;Beta-phenylpyruvate;Beta-phenylpyruvic acid;Alpha-oxo-benzenepropanoic acid;Phenylbrenztraubensaeure;Phenylpyruvic acid;A-ketohydrocinnamate;A-ketohydrocinnamic acid;α-ketohydrocinnamate;α-ketohydrocinnamic acid;A-oxo-benzenepropanoate;A-oxo-benzenepropanoic acid;Alpha-oxo-benzenepropanoate;α-oxo-benzenepropanoate;α-oxo-benzenepropanoic acid;β-phenylpyruvate;β-phenylpyruvic acid;Keto-phenylpyruvic acid;2-hydroxy-3-phenyl-2-propenoic acid, 9ci;2-oxo-3-phenylpropanoic acid (mixture oxo and keto);A-hydroxycinnamic acid;A-oxobenzenepropanoic acid, 9ci;Fema 3892PW_C000131Ppyr128087846411112099812212356313595L-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_C000095Glu162443658119113841641496991105421448501456261462545323111534411354151175439118556513256311075632108585910560061476071157619194653185683818768441887092727093717165205718220775142247518151820822583732201179219811855161120042221262131126832891269729042348315423493184284532077020253773321337752511277971346779773277798134778291345806491351200231241200401221200864071203474061206921261208164181211474231211534241211574251228331191229971201232994431234014541237194581237254591237294601254012991254182971254574811256674791257693011258024891269413881269952061271625011272575061148Pyridoxal 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_C001420H2O55894910951394151316214481135261562428652106912077033823188382109431137749146554159043201824253222267860272746277817280529314370316472363461459836472737494193503027515675195975214100522794523610352971055319111534311353551125402110547012354831255492126550712755341305537114554112955911355608118562210856916575914057781015841143585314658771075890955910147594015160321556059157608716161231636133159621516218166647717865071806600152671311768401886888160716220571812077193206721121172282137238214724321572951987350216738821074012127467222749222475001907588170820122582372268414162926526118502771192216412011281122132851225028612264287123272491252022712632651269329012705291127152921300729813019300130253011303730213261223133272941534030842327315426953184369132276914293770192537710213277131133772151347737833177397332774713337751611577536334776283367772233777759341778163437798234778071329782353527824235378270356791133608001436880039370805912288065611993830383947943841105573901106393911158443981198792321199151221199634061200084071200464081201131241203654121204304051204384091206064151207944141211584251212404291213511211213814191216074341221183821223844361227531201227973741228044431230124461230643761230721371231314471231421361231624481232314511233844501237304601238104641239404551241654691246703991249384711249454721253052971253534791253864811254244821254802991256824831257074781257454871260544901262384951262734841267644801268965011269635021270173881271772081271992091272275041275065071275765151278363891280823951281765131065OxygenHMDB0001377Oxygen is the third most abundant element in the universe after hydrogen and helium and the most abundant element by mass in the Earth's crust. Diatomic oxygen gas constitutes 20.9% of the volume of air. All major classes of structural molecules in living organisms, such as proteins, carbohydrates, and fats, contain oxygen, as do the major inorganic compounds that comprise animal shells, teeth, and bone. Oxygen in the form of O2 is produced from water by cyanobacteria, algae and plants during photosynthesis and is used in cellular respiration for all living organisms. Green algae and cyanobacteria in marine environments provide about 70% of the free oxygen produced on earth and the rest is produced by terrestrial plants. Oxygen is used in mitochondria to help generate adenosine triphosphate (ATP) during oxidative phosphorylation. For animals, a constant supply of oxygen is indispensable for cardiac viability and function. To meet this demand, an adult human, at rest, inhales 1.8 to 2.4 grams of oxygen per minute. This amounts to more than 6 billion tonnes of oxygen inhaled by humanity per year. At a resting pulse rate, the heart consumes approximately 8-15 ml O2/min/100 g tissue. This is significantly more than that consumed by the brain (approximately 3 ml O2/min/100 g tissue) and can increase to more than 70 ml O2/min/100 g myocardial tissue during vigorous exercise. As a general rule, mammalian heart muscle cannot produce enough energy under anaerobic conditions to maintain essential cellular processes; thus, a constant supply of oxygen is indispensable to sustain cardiac function and viability. However, the role of oxygen and oxygen-associated processes in living systems is complex, and they and can be either beneficial or contribute to cardiac dysfunction and death (through reactive oxygen species). Reactive oxygen species (ROS) are a family of oxygen-derived free radicals that are produced in mammalian cells under normal and pathologic conditions. Many ROS, such as the superoxide anion (O2-)and hydrogen peroxide (H2O2), act within blood vessels, altering mechanisms mediating mechanical signal transduction and autoregulation of cerebral blood flow. Reactive oxygen species are believed to be involved in cellular signaling in blood vessels in both normal and pathologic states. The major pathway for the production of ROS is by way of the one-electron reduction of molecular oxygen to form an oxygen radical, the superoxide anion (O2-). Within the vasculature there are several enzymatic sources of O2-, including xanthine oxidase, the mitochondrial electron transport chain, and nitric oxide (NO) synthases. Studies in recent years, however, suggest that the major contributor to O2- levels in vascular cells is the membrane-bound enzyme NADPH-oxidase. Produced O2- can react with other radicals, such as NO, or spontaneously dismutate to produce hydrogen peroxide (H2O2). In cells, the latter reaction is an important pathway for normal O2- breakdown and is usually catalyzed by the enzyme superoxide dismutase (SOD). Once formed, H2O2 can undergo various reactions, both enzymatic and nonenzymatic. The antioxidant enzymes catalase and glutathione peroxidase act to limit ROS accumulation within cells by breaking down H2O2 to H2O. Metabolism of H2O2 can also produce other, more damaging ROS. For example, the endogenous enzyme myeloperoxidase uses H2O2 as a substrate to form the highly reactive compound hypochlorous acid. Alternatively, H2O2 can undergo Fenton or Haber-Weiss chemistry, reacting with Fe2+/Fe3+ ions to form toxic hydroxyl radicals (-.OH). (PMID: 17027622, 15765131).7782-44-7C0000797715379CPD-6641952O=OO2InChI=1S/O2/c1-2MYMOFIZGZYHOMD-UHFFFAOYSA-Noxidanone31.998831.9898292440singlet oxygen00FDB022589Dioxygen;Molecular oxygen;O2;Oxygen;Oxygen molecule;[oo];Dioxygene;Disauerstoff;E 948;E-948;E948PW_C001065O29591105245165001850585491462528638364910674316882075415763476933836213754920162425312228032942604247471354671235480125549312655081275809108597314761291597006188703216370501607319213753321075602128395151118162161186419811883215118942111205722512063164122472861227922612325249127062911271629213004298130163001302630113038302132602234227617426573157691029377044294772141347735011177363130773773317739533277497113775121157753733477626336777233377773611277747129777563417780511477812133780703297815113278381345788053437911136012004740812038312212042640512054240712055341412059440912060140612088341512104512412110438312160543412165642912211738212257341812268938412279837412282244312302713512306037612312844712313913612316344812317611912318745012321913712322612012345945112360911812366939812416346912421446412466939912514545412527512112542548212570647812573148312573729712574047912588448112610029912627248412652249512672148912682548012696450212698620712719820912721420812721920512722250112730550412734520612755738812757451512783538912808139512809539012831250612843239135AmmoniaHMDB0000051Ammonia is a colourless alkaline gas and is one of the most abundant nitrogen-containing compounds in the atmosphere. It is an irritant with a characteristic pungent odor that is widely used in industry. Inasmuch as ammonia is highly soluble in water and, upon inhalation, is deposited in the upper airways, occupational exposures to ammonia have commonly been associated with sinusitis, upper airway irritation, and eye irritation. Acute exposures to high levels of ammonia have also been associated with diseases of the lower airways and interstitial lung. Small amounts of ammonia are naturally formed in nearly all tissues and organs of the vertebrate organism. Ammonia is both a neurotoxin and a metabotoxin. In fact, it is the most common endogenous neurotoxin. A neurotoxin is a compound that causes damage to neural tissue and neural cells. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Ammonia is recognized to be central in the pathogenesis of a brain condition known as hepatic encephalopathy, which arises from various liver diseases and leads to a build up ammonia in the blood (hyperammonemia). More than 40% of people with cirrhosis develop hepatic encephalopathy. Part of the neurotoxicity of ammonia arises from the fact that it easily crosses the blood-brain barrier and is absorbed and metabolized by the astrocytes, a population of cells in the brain that constitutes 30% of the cerebral cortex. Astrocytes use ammonia when synthesizing glutamine from glutamate. The increased levels of glutamine lead to an increase in osmotic pressure in the astrocytes, which become swollen. There is increased activity of the inhibitory gamma-aminobutyric acid (GABA) system, and the energy supply to other brain cells is decreased. This can be thought of as an example of brain edema. The source of the ammonia leading to hepatic encephalopathy is not entirely clear. The gut produces ammonia, which is metabolized in the liver, and almost all organ systems are involved in ammonia metabolism. Colonic bacteria produce ammonia by splitting urea and other amino acids, however this does not fully explain hyperammonemia and hepatic encephalopathy. The alternative explanation is that hyperammonemia is the result of the intestinal breakdown of amino acids, especially glutamine. The intestines have significant glutaminase activity, predominantly located in the enterocytes. On the other hand, intestinal tissues only have a little glutamine synthetase activity, making it a major glutamine-consuming organ. In addition to the intestine, the kidney is an important source of blood ammonia in patients with liver disease. Ammonia is also taken up by the muscle and brain in hepatic coma, and there is confirmation that ammonia is metabolized in muscle. Excessive formation of ammonia in the brains of Alzheimer's disease patients has also been demonstrated, and it has been shown that some Alzheimer's disease patients exhibit elevated blood ammonia concentrations. Ammonia is the most important natural modulator of lysosomal protein processing. Indeed, there is strong evidence for the involvement of aberrant lysosomal processing of beta-amyloid precursor protein (beta-APP) in the formation of amyloid deposits. Inflammatory processes and activation of microglia are widely believed to be implicated in the pathology of Alzheimer's disease. Ammonia is able to affect the characteristic functions of microglia, such as endocytosis, and cytokine production. Based on these facts, an ammonia-based hypothesis for Alzheimer's disease has been suggested (PMID: 17006913, 16167195, 15377862, 15369278). Chronically high levels of ammonia in the blood are associated with nearly twenty different inborn errors of metabolism including: 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, 3-methyl-crotonylglycinuria, argininemia, argininosuccinic aciduria, beta-ketothiolase deficiency, biotinidase deficiency, carbamoyl phosphate synthetase deficiency, carnitine-acylcarnitine translocase deficiency, citrullinemia type I, hyperinsulinism-hyperammonemia syndrome, hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, isovaleric aciduria, lysinuric protein intolerance, malonic aciduria, methylmalonic aciduria, methylmalonic aciduria due to cobalamin-related disorders, propionic acidemia, pyruvate carboxylase deficiency, and short chain acyl CoA dehydrogenase deficiency (SCAD deficiency). Many of these inborn errors of metabolism are associated with urea cycle disorders or impairment of amino acid metabolism. High levels of ammonia in the blood (hyperammonemia) lead to the activation of NMDA receptors in the brain. This results in the depletion of brain ATP, which in turn leads to the release of glutamate. Ammonia also leads to the impairment of mitochondrial function and calcium homeostasis, thereby decreasing ATP synthesis. Excess ammonia also increases the formation of nitric oxide (NO), which in turn reduces the activity of glutamine synthetase, and thereby decreases the elimination of ammonia in the brain (PMID: 12020609). As a neurotoxin, ammonia predominantly affects astrocytes. Disturbed mitochondrial function and oxidative stress, factors implicated in the induction of the mitochondrial permeability transition, appear to be involved in the mechanism of ammonia neurotoxicity. Ammonia can also affect the glutamatergic and GABAergic neuronal systems, the two prevailing neuronal systems of the cortical structures. All of these effects can lead to irreversible brain damage, coma, and/or death. Infants with urea cycle disorders and hyperammonemia initially exhibit vomiting and increasing lethargy. If untreated, seizures, hypotonia (poor muscle tone, floppiness), respiratory distress (respiratory alkalosis), and coma can occur. Adults with urea cycle disorders and hyperammonemia will exhibit episodes of disorientation, confusion, slurred speech, unusual and extreme combativeness or agitation, stroke-like symptoms, lethargy, and delirium. Ammonia also has toxic effects when an individual is exposed to ammonia solutions. Acute exposure to high levels of ammonia in air may be irritating to skin, eyes, throat, and lungs and cause coughing and burns. Lung damage and death may occur after exposure to very high concentrations of ammonia. Swallowing concentrated solutions of ammonia can cause burns in the mouth, throat, and stomach. Splashing ammonia into eyes can cause burns and even blindness.7664-41-7C0001422216134AMMONIA217NH3NInChI=1S/H3N/h1H3QGZKDVFQNNGYKY-UHFFFAOYSA-Nammonia17.030517.0265491011ammonia01FDB003908Ammonia anhydrous;Ammonia inhalant;Ammonia solution strong [usan];Ammonia water;Ammoniak;Liquid ammonia;Am-fol;Ammonia;Ammonia (conc 20% or greater);Ammonia gas;Ammonia solution;Ammonia solution strong (nf);Ammonia water (jp15);Ammoniac [french];Ammoniaca [italian];Ammoniacum gummi;Ammoniak [german];Ammoniak kconzentrierter;Ammoniakgas;Ammonium ion;Amoniak [polish];Anhydrous ammonia;Aromatic ammonia vaporole;Azane;Nh(3);Nh3;Nitro-sil;Primaeres amin;Sekundaeres amin;Spirit of hartshorn;Tertiaeres amin;[nh3];Ammoniac;Amoniaco;R-717;Ammonia solution strongPW_C000035NH397911251338142443824791355014146854253322257235338111601614770221607177205117861981184827711885215127082911271829276966225770462947732913377343132774693337749911377539334775971157798534777993112780723297924429380650135806571191162031091199211221200494081200531261201364071203434061203634121204624051210461241211614251221193821228003741228054431229931201230104461230963761236101181237334601246713991253112971254274821254313011255024811256634791257084781261022991262744841269665021269702071270392061271585011272002091276003881278373891783Hydrogen peroxideHMDB0003125Hydrogen peroxide (H2O2) is a very pale blue liquid which appears colourless in a dilute solution, slightly more viscous than water. It is a weak acid. It has strong oxidizing properties and is therefore a powerful bleaching agent that is mostly used for bleaching paper, but has also found use as a disinfectant and as an oxidizer. Hydrogen peroxide in the form of carbamide peroxide is widely used for tooth whitening (bleaching), both in professionally- and in self-administered products. Hydrogen peroxide (H2O2) is a well-documented component of living cells. It plays important roles in host defense and oxidative biosynthetic reactions. In addition there is growing evidence that at low levels, H2O2 also functions as a signaling agent, particularly in higher organisms. H2O2 has increasingly been viewed as an important cellular signaling agent in its own right, capable of modulating both contractile and growth-promoting pathways with more far-reaching effects. Due to the accumulation of hydrogen peroxide in the skin of patients with the depigmentation disorder vitiligo, the human epidermis cannot have the normal capacity for autocrine synthesis, transport and degradation of acetylcholine as well as the muscarinic (m1-m5) and nicotinic signal transduction in keratinocytes and melanocytes. Accumulating evidence suggests that hydrogen peroxide (H(2)O(2)) plays an important role in cancer development. Experimental data have shown that cancer cells produce high amounts of H(2)O(2). An increase in the cellular levels of H(2)O(2) has been linked to several key alterations in cancer, including DNA alterations, cell proliferation, apoptosis resistance, metastasis, angiogenesis and hypoxia-inducible factor 1 (HIF-1) activation. (PMID: 17150302, 17335854, 16677071, 16607324, 16514169).7722-84-1C0002778416240HYDROGEN-PEROXIDE763OOH2O2InChI=1S/H2O2/c1-2/h1-2HMHAJPDPJQMAIIY-UHFFFAOYSA-Nperoxol34.014734.0054793082hydrogen peroxide00FDB014562Adeka super el;Albone;Albone 35;Albone ds;Anti-keim 50;Asepticper;Baquashock;Cix;Clarigel gold;Crestal whitestrips;Crystacide;Dentasept;Deslime lp;Hioxyl;Hipox;Hybrite;Hydrogen dioxide;Hydrogen peroxide;Inhibine;Lase peroxide;Lensan a;Magic bleaching;Metrokur;Mirasept;Nite white excel 2;Odosat d;Opalescence xtra;Oxigenal;Oxydol;Oxyfull;Oxysept;Oxysept i;Pegasyl;Perhydrol;Perone;Peroxaan;Peroxclean;Quasar brite;Select bleach;Superoxol;T-stuff;Whiteness hp;Whitespeed;Xtra white;[oh(oh)];Dihydrogen dioxide;H2o2;HoohPW_C001783H2O29891135188855114627287551512433169121749512534223818104749134752315495126550212355101275810108600514770381638396151118172161188621512461226127092911271929213028301130352981304030213405222426583157702222577047294770792937750011377540334775981157772033277725337778061147781011177819326780733297815213278598112120050408120102122120463405120595409120609416120954407121047124122120382122801374122814443122839135123097376123157447123165448123220137123234452123520119123611118124672399125428482125469297125709478125732483125748488125895481126103299126275484126967502126978207127006205127201209127215208127230505127356206127601388127838389964FADHMDB0001248FAD, also known as flavitan or adeflavin, belongs to the class of organic compounds known as flavin nucleotides. These are nucleotides containing a flavin moiety. Flavin is a compound that contains the tricyclic isoalloxazine ring system, which bears 2 oxo groups at the 2- and 4-positions. FAD is a drug which is used to treat eye diseases caused by vitamin b2 deficiency, such as keratitis and blepharitis. FAD is slightly soluble (in water) and a moderately acidic compound (based on its pKa). FAD has been found in human liver and muscle tissues, and has also been detected in multiple biofluids, such as feces and blood. Within the cell, FAD is primarily located in the cytoplasm, mitochondria, endoplasmic reticulum and peroxisome. FAD exists in all living organisms, ranging from bacteria to humans. In humans, FAD is involved in the risedronate action pathway, the ibandronate action pathway, the valine, leucine and isoleucine degradation pathway, and the pyrimidine metabolism pathway. FAD is also involved in several metabolic disorders, some of which include the oncogenic action OF L-2-hydroxyglutarate in hydroxygluaricaciduria pathway, gaba-transaminase deficiency, 4-hydroxybutyric aciduria/succinic semialdehyde dehydrogenase deficiency, and the saccharopinuria/hyperlysinemia II pathway. FAD is a condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972).146-14-5C0001664397516238FAD559059DB03147CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)CO[P@](O)(=O)O[P@@](O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=NC(=O)NC(=O)C1=N2C27H33N9O15P2InChI=1S/C27H33N9O15P2/c1-10-3-12-13(4-11(10)2)35(24-18(32-12)25(42)34-27(43)33-24)5-14(37)19(39)15(38)6-48-52(44,45)51-53(46,47)49-7-16-20(40)21(41)26(50-16)36-9-31-17-22(28)29-8-30-23(17)36/h3-4,8-9,14-16,19-21,26,37-41H,5-7H2,1-2H3,(H,44,45)(H,46,47)(H2,28,29,30)(H,34,42,43)/t14-,15+,16+,19-,20+,21+,26+/m0/s1VWWQXMAJTJZDQX-UYBVJOGSSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}[({[(2R,3S,4S)-5-{7,8-dimethyl-2,4-dioxo-2H,3H,4H,10H-benzo[g]pteridin-10-yl}-2,3,4-trihydroxypentyl]oxy}(hydroxy)phosphoryl)oxy]phosphinic acid785.5497785.157134455-2.279flavine-adenine dinucleotide0-3FDB0225111h-purin-6-amine flavin dinucleotide;1h-purin-6-amine flavine dinucleotide;Adenine-flavin dinucleotide;Adenine-flavine dinucleotide;Adenine-riboflavin dinuceotide;Adenine-riboflavin dinucleotide;Adenine-riboflavine dinucleotide;Fad;Flamitajin b;Flanin f;Flavin adenine dinucleotide;Flavin adenine dinucleotide oxidized;Flavin-adenine dinucleotide;Flavine adenosine diphosphate;Flavine-adenine dinucleotide;Flavitan;Flaziren;Isoalloxazine-adenine dinucleotide;Riboflavin 5'-adenosine diphosphate;Riboflavin-adenine dinucleotide;Riboflavine-adenine dinucleotide;AdeflavinPW_C000964FAD999114518681923216425317628288251884021188141489421612291622492133582536223723264602364688314741134758104881652681035285102533511154961265511127561311860301556054156608216161161626390164751786499179666610770391637175205732121374652227487223907622411818216118872151189921112296225123282491244315112519227125952261271029112720292130293011304130243623318770802937712613377152134775011137750711277518115775413347761513277726337780543297837534578930331792223367927235880012368800343698071411911995840611999938412005140812010740712043240512045312212049012412127842912129841812141738212148938312274812012277612112280237412282344312306637612308713512316644812384946412386845412397639912404739812534847912537848012542948212547448112569729712597948912610729912627748412689150112692039112696850212698720712701120612731020912743250612760238812784038940095L-erythro-tetrahydrobiopterinHMDB0059658L-Erythro-tetrahydrobiopterin, also known as R-THBP or 6R-BH4, belongs to the class of organic compounds known as biopterins and derivatives. These are coenzymes containing a 2-amino-pteridine-4-one derivative. They are mainly synthesized in several parts of the body, including the pineal gland. L-Erythro-tetrahydrobiopterin is a drug which is used for the treatment of tetrahydrobiopterin (bh4) deficiency. L-Erythro-tetrahydrobiopterin exists as a solid, slightly soluble (in water), and a very weakly acidic compound (based on its pKa). In humans, L-erythro-tetrahydrobiopterin is involved in the phenylalanine and tyrosine metabolism pathway. L-Erythro-tetrahydrobiopterin is also involved in a few metabolic disorders, which include the tyrosinemia type 2 (or richner-hanhart syndrome) pathway, the phenylketonuria pathway, and the tyrosinemia type 3 (tyro3) pathway. L-erythro-tetrahydrobiopterin is part of the Folate biosynthesis pathway. It is a substrate for: Sepiapterin reductase.62989-33-7C00272442575956040270[H][C@@]1(CNC2=C(N1)C(=O)NC(N)=N2)[C@@H](O)[C@H](C)OC9H15N5O3InChI=1S/C9H15N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h3-4,6,12,15-16H,2H2,1H3,(H4,10,11,13,14,17)/t3-,4+,6-/m0/s1FNKQXYHWGSIFBK-RPDRRWSUSA-N(6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-3,4,5,6,7,8-hexahydropteridin-4-one241.2471241.117489371-2.0766R-5,6,7,8-tetrahydrobiopterin00(-)-(6r)-2-amino-6-((1r,2s)-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4(3h)-pteridinone;(6r)-l-erythro-tetrahydrobiopterin;2-amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydoro-4(1h)-pteridinone;5,6,7,8-tetrahydrobiopterin;6r-5,6,7,8-tetrahydrobiopterin;6r-bh4;6r-l-5,6,7,8-tetrahydrobiopterin;L-erythro-tetrahydrobiopterin;R-thbp;Sapropterina;Sapropterinum;TetrahydrobiopterinPW_C040095EryTbpn1283878468111121000122123565135103L-TyrosineHMDB0000158Tyrosine is an essential amino acid that readily passes the blood-brain barrier. Once in the brain, it is a precursor for the neurotransmitters dopamine, norepinephrine and epinephrine, better known as adrenalin. These neurotransmitters are an important part of the body's sympathetic nervous system, and their concentrations in the body and brain are directly dependent upon dietary tyrosine. Tyrosine is not found in large concentrations throughout the body, probably because it is rapidly metabolized. Folic acid, copper and vitamin C are cofactor nutrients of these reactions. Tyrosine is also the precursor for hormones, thyroid, catecholestrogens and the major human pigment, melanin. Tyrosine is an important amino acid in many proteins, peptides and even enkephalins, the body's natural pain reliever. Valine and other branched amino acids, and possibly tryptophan and phenylalanine may reduce tyrosine absorption. A number of genetic errors of tyrosine metabolism occur. Most common is the increased amount of tyrosine in the blood of premature infants, which is marked by decreased motor activity, lethargy and poor feeding. Infection and intellectual deficits may occur. Vitamin C supplements reverse the disease. Some adults also develop elevated tyrosine in their blood. This indicates a need for more vitamin C. More tyrosine is needed under stress, and tyrosine supplements prevent the stress-induced depletion of norepinephrine and can cure biochemical depression. However, tyrosine may not be good for psychosis. Many antipsychotic medications apparently function by inhibiting tyrosine metabolism. L-dopa, which is directly used in Parkinson's, is made from tyrosine. Tyrosine, the nutrient, can be used as an adjunct in the treatment of Parkinson's. Peripheral metabolism of tyrosine necessitates large doses of tyrosine, however, compared to L-dopa. (http://www.dcnutrition.com).60-18-4C00082605717895TYR5833DB00135N[C@@H](CC1=CC=C(O)C=C1)C(O)=OC9H11NO3InChI=1S/C9H11NO3/c10-8(9(12)13)5-6-1-3-7(11)4-2-6/h1-4,8,11H,5,10H2,(H,12,13)/t8-/m0/s1OUYCCCASQSFEME-QMMMGPOBSA-N(2S)-2-amino-3-(4-hydroxyphenyl)propanoic acid181.1885181.073893223-1.373L-tyrosine00FDB000446(-)-a-amino-p-hydroxyhydrocinnamate;(-)-a-amino-p-hydroxyhydrocinnamic acid;(-)-alpha-amino-p-hydroxyhydrocinnamate;(-)-alpha-amino-p-hydroxyhydrocinnamic acid;(s)-(-)-tyrosine;(s)-2-amino-3-(p-hydroxyphenyl)propionate;(s)-2-amino-3-(p-hydroxyphenyl)propionic acid;(s)-3-(p-hydroxyphenyl)alanine;(s)-tyrosine;(s)-a-amino-4-hydroxybenzenepropanoate;(s)-a-amino-4-hydroxybenzenepropanoic acid;(s)-a-amino-4-hydroxy-benzenepropanoate;(s)-a-amino-4-hydroxy-benzenepropanoic acid;(s)-alpha-amino-4-hydroxybenzenepropanoate;(s)-alpha-amino-4-hydroxybenzenepropanoic acid;(s)-alpha-amino-4-hydroxy-benzenepropanoate;(s)-alpha-amino-4-hydroxy-benzenepropanoic acid;2-amino-3-(4-hydroxyphen yl)-2-amino-3-(4-hydroxyphenyl)-propanoate;2-amino-3-(4-hydroxyphen yl)-2-amino-3-(4-hydroxyphenyl)-propanoic acid;3-(4-hydroxyphenyl)-l-alanine;4-hydroxy-l-phenylalanine;Benzenepropanoate;Benzenepropanoic acid;L-tyrosine;L-p-tyrosine;Tyr;Tyrosine;P-tyrosine;(2s)-2-amino-3-(4-hydroxyphenyl)propanoic acid;L-tyrosin;Y;(-)-α-amino-p-hydroxyhydrocinnamate;(-)-α-amino-p-hydroxyhydrocinnamic acid;(2s)-2-amino-3-(4-hydroxyphenyl)propanoate;(s)-α-amino-4-hydroxybenzenepropanoate;(s)-α-amino-4-hydroxybenzenepropanoic acidPW_C000103Tyr396196862012848197924707235665107566610858861058342225123481514241231842413315770512247775434178469111790461287910213279236253821893778247337811777340211777440312100112212158912412265244112265344212265541012356613512414711812522647612522747712522944412755238815414a-HydroxytetrahydrobiopterinHMDB0002281Tetrahydrobiopterin (BH4) is essential to catalyze the conversion of phenylalanine to tyrosine by phenylalanine hydroxylase. During this physiological reaction, the oxidation of BH4 creates 4a-hydroxytetrahydropterin intermediates and hydrogen peroxide is formed. The hydrogen peroxide and the hydroxytetrahydropterin can both derive from alternate routes of breakdown of a common precursor, the corresponding 4a-hydroperoxytetrahydropterin. (PMID 8323303).70110-58-6C1552212980315374114908CC(O)C(O)C1CNC2=NC(N)=NC(=O)C2(O)N1C9H15N5O4InChI=1S/C9H15N5O4/c1-3(15)5(16)4-2-11-6-9(18,14-4)7(17)13-8(10)12-6/h3-5,14-16,18H,2H2,1H3,(H3,10,11,12,13,17)KJKIEFUPAPPGBC-UHFFFAOYSA-N(4aS,6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-4a-hydroxy-4,4a,5,6,7,8-hexahydropteridin-4-one257.2465257.112403993-1.736(4aS,6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-4a-hydroxy-5,6,7,8-tetrahydropteridin-4-one00FDB022944(6r)-6-(l-erythro-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4a-hydroxypterin;2-amino-6-(1,2-dihydroxypropyl)-4a-hydroxy-5,6,7,8-tetrahydropteridin-4(4ah)-one;2-amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4a-hydroxy-4(4ah)-pteridinone;2-amino-6-[(1r,2s)-1,2-dihydroxypropyl]-4a-hydroxy-1,5,6,7-tetrahydropteridin-4-one;4a-hydroxy-5,6,7,8-tetrahydrobiopterin;4a-hydroxytetrahydrobiopterin;4alpha-hydroxytetrahydrobiopterin;4a-hydroxy-5,6,4,8-tetrahydrobiopterinPW_C0015414aHtHbp12858202627847011179082132121002122121617124123567135124175118127586388544Fe2+HMDB0000692Iron is a chemical element with the symbol Fe and atomic number 26. Iron makes up 5% of the Earth's crust and is second in abundance to aluminium among the metals and fourth in abundance among the elements. Physiologically, it. exists as an ion in the body. Iron (as Fe2+, ferrous ion) is a necessary trace element used by all known living organisms. Iron-containing enzymes, usually containing heme prosthetic groups, participate in catalysis of oxidation reactions in biology, and in transport of a number of soluble gases. Iron is an essential constituent of hemoglobin, cytochrome, and other components of respiratory enzyme systems. Its chief functions are in the transport of oxygen to tissue (hemoglobin) and in cellular oxidation mechanisms. Inorganic iron involved in redox reactions is also found in the iron-sulfur clusters of many enzymes, such as nitrogenase (involved in the synthesis of ammonia from nitrogen and hydrogen) and hydrogenase. A class of non-heme iron proteins is responsible for a wide range of functions such as ribonucleotide reductase (reduces ribose to deoxyribose; DNA biosynthesis) and purple acid phosphatase (hydrolysis of phosphate esters). When the body is fighting a bacterial infection, the body sequesters iron inside of cells (mostly stored in the storage molecule ferritin) so that it cannot be used by bacteria. Depletion of iron stores may result in iron-deficiency anemia. Iron is used to build up the blood in anemia. Humans experience iron toxicity above 20 milligrams of iron for every kilogram of weight, and 60 milligrams per kilogram is a lethal dose. Over-consumption of iron, often the result of children eating large quantities of ferrous sulfate tablets intended for adult consumption, is the most common toxicological cause of death in children under six. The DRI lists the Tolerable Upper Intake Level (UL) for adults as 45 mg/day. For children under fourteen years old the UL is 40 mg/day. Iron is a metal extracted from iron ore, and is almost never found in the free elemental state.15438-31-0C148182728429033Ferric-Hydroxamate-Complexes25394DB01592[Fe++]FeInChI=1S/Fe/q+2CWYNVVGOOAEACU-UHFFFAOYSA-Nlambda2-iron(2+) ion55.84555.9349421330lambda2-iron(2+) ion22FDB016251Armco iron;Carbonyl iron;Fe;Ferrovac e;Hematite;Infed;Loha;Limonite;Magnetite;Malleable iron;Metopirone;Metyrapone;Pzho;Pzh2m;Remko;Suy-b 2;Taconite;Venofer;Wrought iron;Fe (ii) ion;Fe(ii);Fe2+;Fe(2+);Ferrous ion;Iron ion(2+)PW_C000544Fe2+398196413678316922070981777270411637052160120602251214315177179132777401127775112977760341777821111205444071205574141205701221217651241231781191231914501232041351243161181261432991261854811276503885584-Hydroxyphenylpyruvic acidHMDB00007074-Hydroxyphenylpyruvic acid (4-HPPA) is a keto acid that is involved in the tyrosine catabolism pathway. It is a product of the enzyme (R)-4-hydroxyphenyllactate dehydrogenase [EC 1.1.1.222] and is formed during tyrosine metabolism. The conversion from tyrosine to 4-HPPA is catalyzed by tyrosine aminotransferase. Additionally, 4-HPPA can be converted to homogentisic acid which is one of the precursors to ochronotic pigment. The enzyme 4-hydroxyphenylpyruvic acid dioxygenase (HPD) catalyzes the reaction that converts 4-hydroxyphenylpyruvic acid to homogentisic acid. A deficiency in the catalytic activity of HPD is known to lead to tyrosinemia type III, an autosomal recessive disorder characterized by elevated levels of blood tyrosine and massive excretion of tyrosine derivatives into urine. It has been shown that hawkinsinuria, an autosomal dominant disorder characterized by the excretion of 'hawkinsin,' may also be a result of HPD deficiency (PMID: 11073718). There are two isomers of HPPA, specifically 4HPPA and 3HPPA, of which 4HPPA is the most common.156-39-8C0117997915999P-HYDROXY-PHENYLPYRUVATE954DB07718OC(=O)C(=O)CC1=CC=C(O)C=C1C9H8O4InChI=1S/C9H8O4/c10-7-3-1-6(2-4-7)5-8(11)9(12)13/h1-4,10H,5H2,(H,12,13)KKADPXVIOXHVKN-UHFFFAOYSA-N3-(4-hydroxyphenyl)-2-oxopropanoic acid180.1574180.042258744-2.0824-hydroxyphenylpyruvic acid0-1FDB022193(p-hydroxyphenyl)pyruvate;(p-hydroxyphenyl)pyruvic acid;(p-hydroxyphenyl)-pyruvate;(p-hydroxyphenyl)-pyruvic acid;3-(4-hydroxyphenyl)-2-oxo-propanoate;3-(4-hydroxyphenyl)-2-oxo-propanoic acid;3-(4-hydroxyphenyl)-2-oxopropionate;3-(4-hydroxyphenyl)-2-oxopropionic acid;3-(4-hydroxyphenyl)pyruvate;3-(4-hydroxyphenyl)pyruvic acid;3-(p-hydroxyphenyl)-2-oxopropionate;3-(p-hydroxyphenyl)-2-oxopropionic acid;3-(p-hydroxyphenyl)pyruvate;3-(p-hydroxyphenyl)pyruvic acid;4-hydroxy-a-oxobenzenepropanoate;4-hydroxy-a-oxobenzenepropanoic acid;4-hydroxy-alpha-oxobenzenepropanoate;4-hydroxy-alpha-oxobenzenepropanoic acid;4-hydroxyphenylpyruvate;4hppa;Hppa;Hydroxyphenylpyruvate;Hydroxyphenylpyruvic acid;Testacid;P-hydroxyphenylpyruvic;3-(4-hydroxy-phenyl)pyruvic acid;3-(p-hydroxyphenyl)-2-oxopropanoic acid;4-hydroxy alpha-oxobenzenepropanoic acid;4-hydroxyphenylpyruvic acid;P-hydroxyphenylpyruvic acid;(4-hydroxyphenyl)pyruvate;3-(4-hydroxy-phenyl)pyruvate;3-(p-hydroxyphenyl)-2-oxopropanoate;4-hydroxy a-oxobenzenepropanoate;4-hydroxy a-oxobenzenepropanoic acid;4-hydroxy alpha-oxobenzenepropanoate;4-hydroxy α-oxobenzenepropanoate;4-hydroxy α-oxobenzenepropanoic acid;P-hydroxyphenylpyruvatePW_C0005584HPPA128781992283432251234915177039224770552537847111179103132121004122121590124123569135124148118127553388414Adenosine 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_C000414ATP9221460826616414224781373332799593439976321051821121021464921561421605824055924342727264628122930296631637236166136175143992344743147689148645450328950352651557520597521510052501045291101531311153461125390103540611754301185443120554212955561325569133560313556211085846143585414658761075897147592415160481556109161623016664931786839188687016069761997157205718420672092107225213722921172981987302216739021774082187432163748122274991908186225118472771190317012010281120391641217828512578226126912901326422315327308423263154262132242694318770282537721813477233329774683337763233678037332780413507816812878214351782403537841133578494115788501307886533178919334800283688004618480674119856291948261241132349411328238811628010911991412211999240612015440712024538212036241212124642912139212312139743312147140812197441012206512512207938312208340512240242212244443512291939912300944612381646412395144712395646812402937412452744412461613612463039812463437612494347212497237512501147012530429712537147912539229912551548112559548412612348512622030012623449512624047812654749112659649912691350112712338912773151612778139512779639012780120912811950812816751732Adenosine monophosphateHMDB0000045Adenosine monophosphate, also known as 5'-adenylic acid and abbreviated AMP, is a nucleotide that is found in RNA. It is an ester of phosphoric acid with the nucleoside adenosine. AMP consists of the phosphate group, the pentose sugar ribose, and the nucleobase adenine. AMP can be produced during ATP synthesis by the enzyme adenylate kinase. AMP has recently been approved as a 'Bitter Blocker' additive to foodstuffs. When AMP is added to bitter foods or foods with a bitter aftertaste it makes them seem 'sweeter'. This potentially makes lower calorie food products more palatable.61-19-8C00020608316027AMP5858DB00131NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1C10H14N5O7PInChI=1S/C10H14N5O7P/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(22-10)1-21-23(18,19)20/h2-4,6-7,10,16-17H,1H2,(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1UDMBCSSLTHHNCD-KQYNXXCUSA-N{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}phosphonic acid347.2212347.063084339-2.025adenylate0-2DBMET00485FDB0218065'-amp;5'-adenosine monophosphate;5'-adenylate;5'-adenylic acid;Amp;Adenosine 5'-monophosphate;Adenosine 5'-phosphate;Adenosine 5'-phosphorate;Adenosine 5'-phosphoric acid;Adenosine phosphate;Adenosine-5'-monophosphorate;Adenosine-5'-monophosphoric acid;Adenosine-5-monophosphorate;Adenosine-5-monophosphoric acid;Adenosine-monophosphate;Adenosine-phosphate;Adenovite;Adenylate;Adenylic acid;Cardiomone;Lycedan;Muscle adenylate;Muscle adenylic acid;My-b-den;My-beta-den;Phosaden;Phosphaden;Phosphentaside;5'-o-phosphonoadenosine;Adenosine 5'-(dihydrogen phosphate);Adenosine monophosphate;Adenosine-5'p;Adenosini phosphas;Ado5'p;Fosfato de adenosina;Pa;Pado;Phosphate d'adenosine;5'-adenosine monophosphoric acid;Adenosine phosphoric acid;Adenosine 5'-(dihydrogen phosphoric acid);Adenosine 5'-monophosphoric acid;Adenosine monophosphoric acid;Adenosine-5'-monophosphate;Phosphoric acid d'adenosinePW_C000032AMP112344628270167343288122118914457254867545033895251104540811754231035432118545712055581325583133577910157951086977199707218811789198118681611198815112003222125802261263631126942901333122542266342646315772343297732511178392334788091157932011280399180684135809007119916122120016124120031406120246382120888405121954408122920399123464376124507374125306297125394299125409479125596484126853205126934388126949501127124389127311209127711502170PyrophosphateHMDB0000250The anion, the salts, and the esters of pyrophosphoric acid are called pyrophosphates. The pyrophosphate anion is abbreviated PPi and is formed by the hydrolysis of ATP into AMP in cells. This hydrolysis is called pyrophosphorolysis. The pyrophosphate anion has the structure P2O74-, and is an acid anhydride of phosphate. It is unstable in aqueous solution and rapidly hydrolyzes into inorganic phosphate. Pyrophosphate is an osteotoxin (arrests bone development) and an arthritogen (promotes arthritis). It is also a metabotoxin (an endogenously produced metabolite that causes adverse health affects at chronically high levels). Chronically high levels of pyrophosphate are associated with hypophosphatasia. Hypophosphatasia (also called deficiency of alkaline phosphatase or phosphoethanolaminuria) is a rare, and sometimes fatal, metabolic bone disease. Hypophosphatasia is associated with a molecular defect in the gene encoding tissue non-specific alkaline phosphatase (TNSALP). TNSALP is an enzyme that is tethered to the outer surface of osteoblasts and chondrocytes. TNSALP hydrolyzes several substances, including inorganic pyrophosphate (PPi) and pyridoxal 5'-phosphate (PLP), a major form of vitamin B6. When TSNALP is low, inorganic pyrophosphate (PPi) accumulates outside of cells and inhibits the formation of hydroxyapatite, one of the main components of bone, causing rickets in infants and children and osteomalacia (soft bones) in adults. Vitamin B6 must be dephosphorylated by TNSALP before it can cross the cell membrane. Vitamin B6 deficiency in the brain impairs synthesis of neurotransmitters which can cause seizures. In some cases, a build-up of calcium pyrophosphate dihydrate crystals in the joints can cause pseudogout.14000-31-8C0001364410218361PPI559142DB04160[O-]P([O-])(=O)OP([O-])([O-])=OO7P2InChI=1S/H4O7P2/c1-8(2,3)7-9(4,5)6/h(H2,1,2,3)(H2,4,5,6)/p-4XPPKVPWEQAFLFU-UHFFFAOYSA-J(phosphonooxy)phosphonic acid173.9433173.9119253784pyrophosphoric acid0-3FDB021918(4-)diphosphoric acid ion;(p2o74-)diphosphate;Diphosphate;Diphosphoric acid;Ppi;Pyrometaphosphate;Pyrophosphate;Pyrophosphate tetraanion;Pyrophosphate(4-) ion;[o3popo3](4-);Diphosphat;P2o7(4-);Pyrophosphat;Pyrophosphate ion;Phosphonato phosphoric acid;Pyrophosphoric acid;Pyrophosphoric acid ionPW_C000170Ppi12235463842923735328822212173162049241059281529417514486854503489525210452941015409117542410354331185458120554811155591325584133560613556551085879107623916669781997073188713416372721607312198731821382751518283210118691611200222212041164123152251232324912512288125792261269529015219306153751834760174256131542697318772353297731712877635336784163357892833179153112799501347995813080047372804171708563019478638494814125948193829867822311063439111327039511327538911552713611553239911993412212001712412003240612033041012093640712126142912134112112148638312240742212298544412350211912383146412404439812497737512532429712539529912541047912559748412565648512587648112655249112686920512693538812695050112733720612812450884Homogentisic acidHMDB0000130Homogentisic acid, also known as melanic acid, is an intermediate in the breakdown or catabolism of tyrosine and phenylalanine. It is generated from the compound p-hydroxyphenylpyruvate through the enzyme p-hydroxyphenylpyruvate dehydrogenase. The resulting homogentisic acid is then broken down into 4-maleylacetoacetate via the enzyme homogentisate 1,2-dioxygenase. Homogentisic acid is also found in other organisms. For instance, it can found in Arbutus unedo (strawberry-tree) honey, in the bacterial plant pathogen Xanthomonas campestris as well as in the yeast Yarrowia lipolytica where it is associated with the production of brown pigments. Homogentisic acid can be oxidatively dimerized to form hipposudoric acid, one of the main constituents of the 'blood sweat' of hippopotamuses. When present in sufficiently high levels, homogentisic acid can function as an osteotoxin and a renal toxin. An osteotoxin is a substance that causes damage to bones and/or joints. A renal toxin causes damage to the kidneys. Chronically high levels of homogentisic acid are associated with alkaptonuria (OMIM: 203500), an inborn error of metabolism. Alkaptonuria is a rare inherited genetic disorder in which the body cannot process the amino acids phenylalanine and tyrosine. It is caused by a mutation in the enzyme homogentisate 1,2-dioxygenase (EC 1.13.11.5), which leads to an accumulation of homogentisic acid in the blood and tissues. Homogentisic acid and its oxidized form benzoquinone acetic acid are excreted in the urine, giving it an unusually dark color. The accumulating homogentisic acid (and benzoquinone acetic acid) causes damage to cartilage (ochronosis, leading to osteoarthritis) and heart valves as well as precipitating as kidney stones and stones in other organs. More specifically, homogentisic acid can be converted to benzoquinone acetic acid (BQA), and the resulting BQA can be readily converted to polymers that resemble the dark skin pigment melanin. These polymers are deposited in the collagen, a connective tissue protein, of particular tissues such as cartilage. This process is called ochronosis (as the tissue looks ochre); ochronotic tissue is stiffened and unusually brittle, impairing its normal function and causing damage. Homogentisic acid is the primary precursor of melanin synthesis in Vibrio cholerae.451-13-8C0054478044747759DB08327OC(=O)CC1=C(O)C=CC(O)=C1C8H8O4InChI=1S/C8H8O4/c9-6-1-2-7(10)5(3-6)4-8(11)12/h1-3,9-10H,4H2,(H,11,12)IGMNYECMUMZDDF-UHFFFAOYSA-N2-(2,5-dihydroxyphenyl)acetic acid168.1467168.042258744-1.323homogentisic acid0-1FDB021899(2,5-dihydroxyphenyl)-acetate;(2,5-dihydroxyphenyl)-acetic acid;2,5-dihydroxy-a-toluate;2,5-dihydroxy-a-toluic acid;2,5-dihydroxy-alpha-toluate;2,5-dihydroxy-alpha-toluic acid;2,5-dihydroxy-benzeneacetate;2,5-dihydroxy-benzeneacetic acid;2,5-dihydroxyphenylacetate;2,5-dihydroxyphenylacetic acid;Alcapton;Homogentisate;Homogentisate acid;Homogentisinate;Homogentisinic acid;Melanic acid;2-(3,6-dihydroxyphenyl)acetic acid;2-(3,6-dihydroxyphenyl)acetatePW_C000084HMGNCA1295819962905915178478111791041321210121221215921241235771351241501181275583881316Carbon 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_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905028133Fe3+HMDB0012943Fe3+, also known as ferric ion or fe(iii), belongs to the class of inorganic compounds known as homogeneous transition metal compounds. These are inorganic compounds containing only metal atoms,with the largest atom being a transition metal atom. Fe3+ exists in all living organisms, ranging from bacteria to humans. 2,3-Dihydroxybenzoylserine and fe3+ can be biosynthesized from ferric enterobactin through its interaction with the enzyme enterochelin esterase. Outside of the human body, fe3+ can be found in a number of food items such as bamboo shoots, catjang pea, chickpea, and orange bell pepper. This makes fe3+ a potential biomarker for the consumption of these food products. The major activity of supplemental iron is in the prevention and treatment of iron deficiency anemia. Iron has putative immune-enhancing, anticarcinogenic and cognition-enhancing activities.20074-52-6C148192993629034CPD-1013427815[Fe+3]FeInChI=1S/Fe/q+3VTLYFUHAOXGGBS-UHFFFAOYSA-Niron(3+) ion55.84555.9349421330iron(3+) ion33C14819Fe(iii);Ferric ion;Iron(3+);Fe (iii) ion;Fe(3+);Ferric iron;Iron, ion (fe(3+))PW_C008133Fe3+12303129681420491997270421631184116012675151774311117745033178599112787211321209494071210131221211273831215931241235151191235781351236963981241511181258904811273512061275593881376Maleylacetoacetic acidHMDB00020524-Maleylacetoacetate is an intermediate in the metabolism of tyrosine. Homogentisate 1,2-dioxygenase is the enzyme, which catalyzes the conversion of homogentisate to 4-maleylacetoacetate. Homogentisate 1,2-dioxygenase or HGD is involved in the catabolism of aromatic rings, more specifically in the break down of the amino acids tyrosine and phenylalanine.5698-52-2C010365280393479044-MALEYL-ACETOACETATE4444078OC(=O)CC(=O)CC(=O)\C=C/C(O)=OC8H8O6InChI=1S/C8H8O6/c9-5(1-2-7(11)12)3-6(10)4-8(13)14/h1-2H,3-4H2,(H,11,12)(H,13,14)/b2-1-GACSIVHAIFQKTC-UPHRSURJSA-N(2Z)-4,6-dioxooct-2-enedioic acid200.1455200.032087988-2.082maleylacetoacetic acid0-2FDB0228174-maleylacetoacetate;4-maleylacetoacetic acid;MaleylacetoacetatePW_C001376MalaceA1298819992906115178480111791051321210151221215951241235801351241531181275613889794IronHMDB0015531Iron is a metallic element found in certain minerals, in nearly all soils, and in mineral waters. Iron is required for life. It exists in all living species, ranging from bacteria to humans. It can be found primarily in blood and it is an essential constituent of hemoglobin, cytochrome, and other components of respiratory enzyme systems. Its chief functions are in the transport of oxygen to tissue (hemoglobin) and in cellular oxidation mechanisms. Depletion of iron stores may result in iron-deficiency anemia. Iron is used to build up the blood in anemia. In humans, iron is involved in several metabolic pathways, some of which include the rofecoxib pathway, magnesium salicylate action pathway, etodolac pathway, and diclofenac pathway. Iron is also involved in several metabolic disorders, some of which include adenine phosphoribosyltransferase deficiency (APRT), porphyria variegata (PV), adenylosuccinate lyase deficiency, and AICA-ribosiduria. The major activity of supplemental iron is in the prevention and treatment of iron-deficiency anemia. Iron has putative immune-enhancing, anticarcinogenic, and cognition-enhancing activities. Iron can be found in a number of food items such as chinese water chestnut, hyssop, daikon radish, and peppermint, which makes it a potential biomarker for the consumption of these food products.7439-89-6C00023239251824822368DB01592[Fe++]FeInChI=1S/Fe/q+2CWYNVVGOOAEACU-UHFFFAOYSA-Nlambda2-iron(2+) ion55.84555.9349421330lambda2-iron(2+) ion22C0002326fe;Eisen;Fe;Fer;Ferrum;HierroPW_C009794Iron1138812665153321635493119294341187330213120562257768313077928336782611327840911178428334789383311208381221209924081212564291213711241215023831217271251234231351235573741238264641239301181240603981242781361258292971259324821260442991272822051273915021274963889814-Fumarylacetoacetic acidHMDB0001268Fumarylacetoacetate is an intermediate in the metabolism of tyrosine. Fumarylacetoacetate hydrolase (FAH) is an enzyme which catalyzes the hydrolysis of 4-fumarylacetoacetate into fumarate and acetoacetate. FAH is the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia.28613-33-4C010615280398309074-FUMARYL-ACETOACETATE4444081OC(=O)CC(=O)CC(=O)\C=C/C(O)=OC8H8O6InChI=1S/C8H8O6/c9-5(1-2-7(11)12)3-6(10)4-8(13)14/h1-2H,3-4H2,(H,11,12)(H,13,14)/b2-1-GACSIVHAIFQKTC-UPHRSURJSA-N(2E)-4,6-dioxooct-2-enedioic acid200.1455200.032087988-2.0824-fumarylacetoacetic acid0-2FDB022522(e)-4,6-dioxo-2-octenedioate;(e)-4,6-dioxo-2-octenedioic acid;4-fumaryl-acetoacetate;4-fumarylacetoacetate;Fumarylacetoacetate;FumarylacetoacetonePW_C000981Fumaryl13008200129063151784821117910613212101712212159612412358213512415411812756238842Acetoacetic acidHMDB0000060Acetoacetic acid (AcAc) is a weak organic acid that can be produced in the human liver under certain conditions of poor metabolism leading to excessive fatty acid breakdown (diabetes mellitus leading to diabetic ketoacidosis). It is then partially converted into acetone by decarboxylation and excreted either in urine or through respiration. Persistent mild hyperketonemia is a common finding in newborns. Ketone bodies serve as an indispensable source of energy for extrahepatic tissues, especially the brain and lung of developing rats. Another important function of ketone bodies is to provide acetoacetyl-CoA and acetyl-CoA for synthesis of cholesterol, fatty acids, and complex lipids. During the early postnatal period, acetoacetate and beta-hydroxybutyrate are preferred over glucose as substrates for synthesis of phospholipids and sphingolipids in accord with requirements for brain growth and myelination. Thus, during the first two weeks of postnatal development, when the accumulation of cholesterol and phospholipids accelerates, the proportion of ketone bodies incorporated into these lipids increases. On the other hand, an increased proportion of ketone bodies are utilized for cerebroside synthesis during the period of active myelination. In the lung, AcAc serves better than glucose as a precursor for the synthesis of lung phospholipids. The synthesized lipids, particularly dipalmityl phosphatidylcholine, are incorporated into surfactant, and thus have a potential role in supplying adequate surfactant lipids to maintain lung function during the early days of life (PMID: 3884391). The acid is also present in the metabolism of those undergoing starvation or prolonged physical exertion as part of gluconeogenesis. When ketone bodies are measured by way of urine concentration, acetoacetic acid, along with beta-hydroxybutyric acid or acetone, is what is detected.541-50-4C0016496153443-KETOBUTYRATE94DB01762CC(=O)CC(O)=OC4H6O3InChI=1S/C4H6O3/c1-3(5)2-4(6)7/h2H2,1H3,(H,6,7)WDJHALXBUFZDSR-UHFFFAOYSA-N3-oxobutanoic acid102.0886102.0316940580.371acetoacetic acid0-1FDB0218013-ketobutyrate;3-ketobutyric acid;3-oxo-butanoate;3-oxo-butanoic acid;3-oxobutyrate;3-oxobutyric acid;Acetoacetate;Diacetate;Diacetic acid;3-oxobutanoic acid;Beta-ketobutyric acid;3-oxobutanoate;B-ketobutyrate;B-ketobutyric acid;Beta-ketobutyrate;β-ketobutyrate;β-ketobutyric acidPW_C000042LIN5974105331302820032692916073601639066151152422227769413377835132782271127848311112051140612076740712101912212159812412311712012336311912358413512415611812660929912756438888Fumaric 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_C000088Fumarat10282541720042505345388102604715661071626458107645910864921796763117683716674802239065151118041981271329042400322424963184249731577148134774661117910713280027369117808133119989384120043122121599124122661406122772121122794135124157118125236120125369480125421297126793479126911391126960205127565388128391501353CalciumHMDB0000464Calcium 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+276163038553146012941159932199735104631163461164471478491491421552432116582138172796182937931597131607239422941866647821048222853401115780101717920572322117258160728119011774213118371981184221012198164122152851528815115350308693361773893317760011578154132782663567852634578724130789081148041374805892288182651120220122120465405121049124121300418121377419121850383121923125122370409122895135123099376123613118123870454123936455124403398124476136124924137125571297125711478125981489126009299126050490126533495127203209127434506127460388127502507128105390423MagnesiumHMDB0000547Magnesium salts are essential in nutrition, being required for the activity of many enzymes, especially those concerned with oxidative phosphorylation. Physiologically, it exists as an ion in the body. It is a component of both intra- and extracellular fluids and is excreted in the urine and feces. Deficiency causes irritability of the nervous system with tetany, vasodilatation, convulsions, tremors, depression, and psychotic behavior. Magnesium ion in large amounts is an ionic laxative, and magnesium sulfate (Epsom salts) is sometimes used for this purpose. So-called "milk of magnesia" is a water suspension of one of the few insoluble magnesium compounds, magnesium hydroxide; the undissolved particles give rise to its appearance and name. Milk of magnesia is a mild base, and is commonly used as an antacid.22537-22-0C003058881842013-HYDROXY-MAGNESIUM-PROTOPORP865DB01378[Mg++]MgInChI=1S/Mg/q+2JLVVSXFLKOJNIY-UHFFFAOYSA-Nmagnesium(2+) ion24.30523.9850418980magnesium(2+) ion22FDB003518Magnesium;Magnesium ions;Magnesium ion;Magnesium, doubly charged positive ion;Magnesium, ion (mg(2+));Mg(2+);Mg2+PW_C000423Mg2+868227426816476272726811581918883229363998339922111674614834915294317641421241024115929422331262933737454031477491486954497456525310453291115356112537610359061475934151603815560941616250166648417865941646881160697919971702057194206722721372332117250214731021673131987473222117631321184321012312225123242491251328812581226127292901527528515337308771371337723632977937336783933347841733578489115785223317853635678574130800203688004518480048372806231188065413580865158096525381841519383238394900271085962231105593901156873981199744061200701221202473821207024071209814081211811241212654291213194191219241251220864051224084221227591201229213991233071191235463741238354641238894551244771361246373761249783751254472971255984841256694791257774811259214821259472991259734951260004901262434781265534911267533001271253891271645011273805021274073881274515071278042091281255081283473951005Zinc (II) ionHMDB0001303Zinc is an essential element, necessary for sustaining all life.Physiologically, it exists as an ion in the body. It is estimated that 3000 of the hundreds of thousands of proteins in the human body contain zinc prosthetic groups. In addition, there are over a dozen types of cells in the human body that secrete zinc ions, and the roles of these secreted zinc signals in medicine and health are now being actively studied. Intriguingly, brain cells in the mammalian forebrain are one type of cell that secretes zinc, along with its other neuronal messenger substances. Cells in the salivary gland, prostate, immune system and intestine are other types that secrete zinc. Obtaining a sufficient zinc intake during pregnancy and in young children is a problem, especially among those who cannot afford a good and varied diet. Brain development is stunted by zinc deficiency in utero and in youth. Zinc is an activator of certain enzymes, such as carbonic anhydrase. Carbonic anhydrase is important in the transport of carbon dioxide in vertebrate blood. Even though zinc is an essential requirement for a healthy body, too much zinc can be harmful. Excessive absorption of zinc can also suppress copper and iron absorption. The free zinc ion in solution is highly toxic to plants, invertebrates, and even vertebrate fish. The Free Ion Activity Model (FIAM) is well-established in the literature, and shows that just micromolar amounts of the free ion kills some organisms.23713-49-7C000383205129105ZN%2b229723DB01593[Zn++]ZnInChI=1S/Zn/q+2PTFCDOFLOPIGGS-UHFFFAOYSA-Nzinc(2+) ion65.40963.9291465780zinc(2+) ion22FDB003729Zinc;Zinc ion;Dietary zinc;Zinc cation;Zinc, ion (zn2+);Zn(ii);Zn(2+);Zn2+PW_C001005Zinc1323841188271165291529575130446831202931477054101175425103543411854591205560132558513355981357449166117871981246622612724290133211517696722577401111775801147792933680400112002012412003540612006012212044140912125742912307513712382746412539829912541347912543829712568548312693838812695350112697620512718020821tRNA(Tyr)RNAPW_NA00002129182TRNATYR1292829812784721117906713212100512212174312412357013512429411812762938822L-Tyrosyl-tRNA(Tyr)RNAPW_NA00002229161LTTT129387847311112100612212357113519tRNA(Phe)RNAPW_NA00001929184TRNAPHE128887847511112100812212357313520L-Phenylalanyl-tRNA(Phe)RNAPW_NA00002029153LPTP128987847611112100912212357413514059Aspartate aminotransferase, cytoplasmicP05201
Biosynthesis of L-glutamate from L-aspartate or L-cysteine. Important regulator of levels of glutamate, the major excitatory neurotransmitter of the vertebrate central nervous system. Acts as a scavenger of glutamate in brain neuroprotection. The aspartate aminotransferase activity is involved in hepatic glucose synthesis during development and in adipocyte glyceroneogenesis. Using L-cysteine as substrate, regulates levels of mercaptopyruvate, an important source of hydrogen sulfide. Mercaptopyruvate is converted into H(2)S via the action of 3-mercaptopyruvate sulfurtransferase (3MST). Hydrogen sulfide is an important synaptic modulator and neuroprotectant in the brain (By similarity).
Got1122.6.1.1; 2.6.1.3794431327950311113729Amine oxidase [flavin-containing] BQ8BW75
Catalyzes the oxidative deamination of biogenic and xenobiotic amines and has important functions in the metabolism of neuroactive and vasoactive amines in the central nervous system and peripheral tissues. MAOB preferentially degrades benzylamine and phenylethylamine (By similarity).
Maob121.4.3.478137111783181327846611313638Tyrosine aminotransferaseQ8QZR1
Transaminase involved in tyrosine breakdown. Converts tyrosine to p-hydroxyphenylpyruvate. Can catalyze the reverse reaction, using glutamic acid, with 2-oxoglutarate as cosubstrate (in vitro). Has much lower affinity and transaminase activity for phenylalanine.
Tat122.6.1.577260132779803277967811113987Phenylalanine-4-hydroxylaseP16331Pah121.14.16.17967911113790Aminoacyl tRNA synthase complex-interacting multifunctional protein 1P31230
Non-catalytic component of the multisynthase complex (PubMed:12060739). Stimulates the catalytic activity of cytoplasmic arginyl-tRNA synthase. Binds tRNA. Possesses inflammatory cytokine activity. Negatively regulates TGF-beta signaling through stabilization of SMURF2 by binding to SMURF2 and inhibiting its SMAD7-mediated degradation. Involved in glucose homeostasis through induction of glucagon secretion at low glucose levels. Promotes dermal fibroblast proliferation and wound repair. Regulates KDELR1-mediated retention of HSP90B1/gp96 in the endoplasmic reticulum. Plays a role in angiogenesis by inducing endothelial cell migration at low concentrations and endothelian cell apoptosis at high concentrations. Induces maturation of dendritic cells and monocyte cell adhesion. Modulates endothelial cell responses by degrading HIF-1A through interaction with PSMA7 (By similarity).
Aimp1127847411114305Phenylalanine--tRNA ligase alpha subunitQ8C0C7Farsa126.1.1.207968011113791Leucine-rich repeat-containing protein 47Q505F5Lrrc471278477111141084-hydroxyphenylpyruvate dioxygenaseP49429
Key enzyme in the degradation of tyrosine.
Hpd121.13.11.27796811117989613213793Homogentisate 1,2-dioxygenaseO09173Hgd121.13.11.5784811117990113213959Maleylacetoacetate isomeraseQ9WVL0
Probable bifunctional enzyme showing minimal glutathione-conjugating activity with ethacrynic acid and 7-chloro-4-nitrobenz-2-oxa-1, 3-diazole and maleylacetoacetate isomerase activity. Has also low glutathione peroxidase activity with t-butyl and cumene hydroperoxides. Is able to catalyze the glutathione dependent oxygenation of dichloroacetic acid to glyoxylic acid (By similarity).
Gstz1125.2.1.2; 2.5.1.18796821117989713213794FumarylacetoacetaseP35505Fah123.7.1.278484111798981327796Probable inactive 1-aminocyclopropane-1-carboxylate synthase-like protein 2Q3UX83Accsl12556813277974346784671117725UnknownZ00Z00125311111792613337926211279265116792661347927513379341113793423297939733179425130794963327953433679546132796043527961335479834114592Alanine--tRNA ligase, cytoplasmicP49588
Catalyzes the attachment of alanine to tRNA(Ala) in a two-step reaction: alanine is first activated by ATP to form Ala-AMP and then transferred to the acceptor end of tRNA(Ala). Also edits incorrectly charged tRNA(Ala) via its editing domain.
HMDBP00625AARS16q22AC01218416.1.1.714218368137924-hydroxyphenylpyruvate dioxygenase-like proteinQ8K248
May have dioxygenase activity.
Hpdl121.13.-.-7847911120205Tyrosine--tRNA ligase, cytoplasmicQ91WQ3
Catalyzes the attachment of tyrosine to tRNA(Tyr) in a two-step reaction: tyrosine is first activated by ATP to form Tyr-AMP and then transferred to the acceptor end of tRNA(Tyr).
Yars126.1.1.113381711120199Phenylalanine--tRNA ligase beta subunitQ9WUA2Farsb126.1.1.201338061117451Aspartate aminotransferase, cytoplasmic12PW_P007451153161405927456L-amino-acid oxidase12PW_P007456153211372917083Tyrosine aminotransferase12PW_P00708314914779627084Phenylalanine-4-hydroxylase12PW_P00708414915772521Alanine--tRNA ligase, cytoplasmic1PW_P000001159211100513270874-hydroxyphenylpyruvate dioxygenase12PW_P007087149191379227088Homogentisate 1,2-dioxygenase12PW_P007088149201379317089Maleylacetoacetate isomerase12PW_P00708914921772527090Fumarylacetoacetase12PW_P007090149221379427085Tyrosine--tRNA ligase, cytoplasmic12PW_P007085149162020521337931117086Phenylalanine--tRNA ligase12PW_P0070861491714305214918201992133778111110174falsePW_R110174Both4005771041Compoundfalse4005781341Compoundtrue4005791311Compoundfalse400580951Compoundtrue100484745110048570832.6.1.5110175falsePW_R110175Right4005811041Compoundfalse40058214201Compoundtrue40058310651Compoundtrue4005841311Compoundfalse400585351Compoundtrue40058617831Compoundtrue10048674561.4.3.2110176falsePW_R110176Right4005871041Compoundfalse400588400951Compoundfalse40058910651Compoundtrue4005901031Compoundfalse40059115411Compoundfalse10048770841.14.16.1110177falsePW_R110177Both4005921031Compoundfalse4005931341Compoundtrue4005945581Compoundfalse400595951Compoundtrue10048870832.6.1.5110178falsePW_R110178Right4005961031Compoundfalse40059714201Compoundtrue40059810651Compoundtrue4005995581Compoundfalse400600351Compoundtrue40060117831Compoundtrue10048974561.4.3.2110179falsePW_R110179Right4006024141Compoundtrue4006031031Compoundfalse400604211NucleicAcidtrue400605321Compoundtrue4006061701Compoundtrue400607221NucleicAcidfalse10049070856.1.1.1110180falsePW_R110180Right4006084141Compoundtrue4006091041Compoundfalse400610191NucleicAcidtrue400611321Compoundtrue4006121701Compoundtrue400613201NucleicAcidfalse10049170866.1.1.20110181falsePW_R110181Right4006145581Compoundfalse40061510651Compoundtrue400616841Compoundfalse40061713161Compoundtrue10049270871.13.11.27110182falsePW_R110182Right400618841Compoundfalse40061910651Compoundtrue40062013761Compoundfalse10049370881.13.11.5110183falsePW_R110183Right40062113761Compoundfalse4006229811Compoundfalse1004947089110184falsePW_R110184Right4006239811Compoundfalse40062414201Compoundtrue400625421Compoundfalse400626881Compoundfalse10049570903.7.1.2158417810411181false75089010regular20019015841791341113false605127010regular100110158418013111181false760182010regular2001901584181951113false605150010regular100110158418211481119false685145010regular100351584183142011349false1147128010regular78781584184106511365false1148118110regular787815841853511363false1145153110regular78781584186178311356false1143143110regular787815841879641139false1232135510regular1002515841881341113false1005118510regular1001101584189951113false1005143010regular100110158419011481119false910138010regular1003515841914009511181false117069210regular2001901584192106511165false1236105810regular7878158419310311181false190589410regular2001901584194154111181false151068910regular20019015841955441119false138593710regular1002515841961341113false1775126410regular100110158419755811181false1910177410regular2001901584198951113false1775149910regular10011015841991341113false2135119410regular1001101584200951113false2135142410regular100110158420111481119false2060138110regular100351584202142011349false1630127910regular78781584203106511365false1630117910regular787815842043511363false1635152910regular78781584205178311356false1635142910regular787815842069641139false1525135610regular10025158420741411142false188082410regular503015842083211144false187865410regular5030158420917011145false197155210regular6343158421041411142false73081510regular503015842113211144false72864010regular5030158421217011145false82156310regular63431584213106511165false2131195210regular787815842148411181false2515177610regular2001901584215131611152false2411195210regular7878158421681331119false2260182610regular100251584217106511165false2438170210regular78781584218137611181false2519135610regular200190158421997941119false2507160110regular10025158422098111181false251996610regular2001901584221142011149false243989110regular78781584222421113false241968110regular1001101584223881113false271968610regular10010015842243531119false250480110regular1002515842254231119false263980110regular1002524142196111false209581414120nucleic_acidregular12011524152296111false208661914120nucleic_acidregular12011524161996111false92581514120nucleic_acidregular12011524172096111false94661514120nucleic_acidregular120115592700140591116false73013958subunitregular16080592701137291132false126713608subunitregular15070592702136381116false82013208subunitregular16080592703139871116false13609428subunitregular16080592704136381116false197013218subunitregular16080592705137291132false144013618subunitregular15070592706137901116false19257118subunitregular1608059270714305111113false8057028noneregular16080592708137911116false7707028proteinregular16080592709141081116false223518318subunitregular16080592710137931112false254216118subunitregular15070592711139591116false253912118subunitregular16080592712137941116false25398018subunitregular16080481372745164375111591519592700749615841822158645Cofactor481373745664375113591520592701749715841872158652Cofactor481374708364375111591521592702749815841902158657Cofactor481375708464375111591522592703749915841952158663Cofactor481376708364375111591523592704750015842012158673Cofactor481377745664375113591524592705750115842062158680Cofactor481378164375111591525592706481379164375111591526592707591527592708481380708764375111591528592709750215842162158697Cofactor481381708864375111591529592710750315842192158701Cofactor481382708964375111591530592711481383709064375111591531592712750415842242158708Cofactor750515842252158709Cofactor2158641M810 1084 C810 1125 810 1365 810 1395 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158642M705 1325 C753 1325 810 1365 810 1395 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158643M810 1818 C810 1781 810 1505 810 1475 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158644M705 1555 C757 1555 810 1505 810 1475 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158645M150 150 L150 200 L200 150 z10true182158646M951 1043 C1059 1043 1342 1152 1342 1360 5false182158647M1225 1319 C1259 1319 1342 1330 1342 1360 5false182158648M1226 1220 C1253 1220 1342 1330 1342 1360 5false182158649M960 1915 C1071 1915 1342 1639 1342 1430 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158650M1223 1570 C1258 1548 1342 1460 1342 1430 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158651M1221 1470 C1251 1470 1342 1460 1342 1430 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158652M837 1305 L837 1355 L887 1305 z10true182158653M900 1085 C900 1115 900 1290 900 1320 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158654M1005 1240 C961 1240 900 1276 900 1320 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158655M900 1815 C900 1785 900 1430 900 1400 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158656M1005 1485 C961 1485 900 1459 900 1400 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158657M150 150 L150 200 L200 150 z10true182158658M950 985 C1001 985 1330 982 1360 982 5false182158659M1270 882 C1270 917 1330 982 1360 982 5false182158660M1275 1058 C1275 1027 1330 982 1360 982 5false182158661M1905 989 C1875 989 1550 982 1520 982 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158662M1610 879 C1601 923 1547 982 1517 982 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158663M150 150 L150 200 L200 150 z10true182158664M1960 1089 C1960 1119 1960 1369 1960 1399 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158665M1875 1319 C1915 1319 1960 1369 1960 1399 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158666M1962 1772 C1962 1742 1960 1509 1960 1479 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158667M1875 1554 C1908 1554 1960 1509 1960 1479 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158668M150 150 L150 200 L200 150 z10true182158669M2050 1087 C2050 1117 2050 1291 2050 1321 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158670M2135 1249 C2107 1249 2050 1291 2050 1321 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158671M2050 1773 C2050 1743 2050 1431 2050 1401 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158672M2135 1479 C2103 1479 2050 1431 2050 1401 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158673M150 150 L150 200 L200 150 z10true182158674M1904 1056 C1788 1056 1515 1143 1515 1361 5false182158675M1630 1318 C1584 1318 1515 1331 1515 1361 5false182158676M1630 1218 C1600 1218 1515 1331 1515 1361 5false182158677M1910 1869 C1801 1869 1515 1642 1515 1431 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158678M1635 1568 C1597 1545 1515 1461 1515 1431 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158679M1635 1468 C1598 1468 1515 1461 1515 1431 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158680M2065 1306.5 L2065 1356.5 L2115 1306.5 z10true182158681M1930 839 C1962 839 2005 821 2005 791 5false182158682M2005 894 C2005 864 2005 821 2005 791 5false182158683M1928 669 C1962 669 2005 681 2005 711 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158684M2002.5 595 C2002.5 625 2005 681 2005 711 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158685M2100 844 C2070 844 2005 821 2005 791 5false182158686M2091 649 C2062 649 2005 681 2005 711 5false182158687M780 830 C813 830 850 812 850 782 5false182158688M850 890 C850 860 850 812 850 782 5false182158689M778 655 C807 655 850 672 850 702 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158690M852.5 606 C852.5 636 850 672 850 702 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158691M930 845 C898 845 850 812 850 782 5false182158692M951 645 C915 645 850 672 850 702 5false18trueM 983.5 669.9903810567666 L 976 657 L 968.5 669.9903810567666false2158693M2110 1869 C2140 1869 2205 1871 2235 1871 5false182158694M2170 1952 C2170 1914 2205 1871 2235 1871 5false182158695M2515 1871 C2485 1871 2425 1871 2395 1871 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158696M2450 1952 C2450 1915 2425 1871 2395 1871 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158697M150 150 L150 200 L200 150 z10true182158698M2615 1776 C2615 1746 2617 1711 2617 1681 5false182158699M2516 1741 C2546 1741 2617 1711 2617 1681 5false182158700M2619 1546 C2619 1576 2617 1581 2617 1611 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158701M150 150 L150 200 L200 150 z10true182158702M2619 1356 C2619 1326 2619 1321 2619 1291 5false182158703M2619 1156 C2619 1186 2619 1181 2619 1211 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158704M2619 966 C2619 936 2619 911 2619 881 5false182158705M2517 930 C2549 930 2619 911 2619 881 5false182158706M2519 736 C2555 736 2619 771 2619 801 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158707M2719 736 C2682 736 2619 771 2619 801 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false2158708M150 150 L150 200 L200 150 z10true182158709M150 150 L150 200 L200 150 z10true182158710M2106 949 C2136 949 2160 949 2230 949 5false18trueM 2217.0096189432334 956.5 L 2230 949 L 2217.0096189432334 941.5false2158711M2106 1039 C2136 1039 2160 1039 2230 1039 5false18trueM 2217.0096189432334 1071.5 L 2230 1064 L 2217.0096189432334 1056.5false2158712M2769 686 C2766 654 2753 606 2699 596 5false18trueM 2701.9903810567666 513.5 L 2689 521 L 2701.9903810567666 528.5false2158713M2469 681 C2465 651 2491 607 2549 596 5false18trueM 2526.0096189432334 528.5 L 2539 521 L 2526.0096189432334 513.5false48751364375110174111195430215841782158641Left195430315841792158642Left195430415841802158643Right195430515841812158644Right44606410048448137248751464375110175113195430615841782158646Left195430715841832158647Left195430815841842158648Left195430915841802158649Right195431015841852158650Right195431115841862158651Right44606510048648137348751564375110174111195431215841782158653Left195431315841882158654Left195431415841802158655Right195431515841892158656Right44606610048548137448751664375110176111195431615841782158658Left195431715841912158659Left195431815841922158660Left195431915841932158661Right195432015841942158662Right44606710048748137548751764375110177111195432115841932158664Left195432215841962158665Left195432315841972158666Right195432415841982158667Right48751864375110177111195432515841932158669Left195432615841992158670Left195432715841972158671Right195432815842002158672Right44606810048848137648751964375110178113195432915841932158674Left195433015842022158675Left195433115842032158676Left195433215841972158677Right195433315842042158678Right195433415842052158679Right44606910048948137748752064375110179111195433515842072158681Left195433615841932158682Left195433715842082158683Right195433815842092158684Right65924142158685Left66024152158686Right44607010049048137848752164375110180111195433915842102158687Left195434015841782158688Left195434115842112158689Right195434215842122158690Right66124162158691Left66224172158692Right44607110049148137948752264375110181111195434315841972158693Left195434415842132158694Left195434515842142158695Right195434615842152158696Right44607210049248138048752364375110182111195434715842142158698Left195434815842172158699Left195434915842182158700Right44607310049348138148752464375110183111195435015842182158702Left195435115842202158703Right44607410049448138248752564375110184111195435215842202158704Left195435315842212158705Left195435415842222158706Right195435515842232158707Right44607510049548138357030576166437514false223091416regular5408415841932158710Left57031576176437514false2230100416regular5408515841932158711Left57032576186437514false254956116regular5408615842232158712Left5408715842222158713Left10357368107512151.71.7-252528532010357472470102.32.3029200145220163M369 597 C369 547 419 497 469 497 C1178 497 2099 497 2808 497 C2858 497 2908 547 2908 597 C2908 1041 2908 1619 2908 2063 C2908 2113 2858 2163 2808 2163 C2099 2163 1178 2163 469 2163 C419 2163 369 2113 369 2063 C369 1619 369 1041 369 597 1true62539.01666.024491715Intracellular Space445795201.01.01601524491815Extracellular Space65800201.01.01601524491915Liver53080201.01.016015102217628207309440296322284426541788Tyrosinemia Type 2 (or Richner-Hanhart Syndrome)Tyrosinemia II also known as Richner-Hanhart syndrome is an autosomal recessive disorder caused by a mutation in the TAT gene the encodes for tyrosine aminotransferase. A defect in this enzyme causes excess tyrosine to accumulate in the blood and urine, tyrosine crystals to form in the cornea, and increased excretion in the urine of 4-hydroxyphenylpyruvic acid, hydroxyphenyllactic acid, and p-hydroxyphenylacetic acid. Symptoms commonly appear in early childhood and include: mental retardation, photophobia (increased sensitivity to light), excessive tearing, eye redness and pain and skin lesions of the palms and soles. The patient is treated with restriction of dietary phenylalanine and tyrosine. Sometimes a tyrosine degradation inhibitor is also used to prevents the formation of fumarylacetoacetate from tyrosine. Trosinemia II is commonly misdiagnosed as herpes simplex keratitis. DiseasePW_X008974CompleteContext897448615104CompoundIncreased48616103CompoundIncreased4861715941ProteinMutated4861814TissueDamaged4861924TissueDamaged4862016TissueDamaged2780107648039al-Hemidan AI, al-Hazzaa SA: Richner-Hanhart syndrome (tyrosinemia type II). Case report and literature review. Ophthalmic Genet. 1995 Mar;16(1):21-6.8974Context