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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-05-30 20:55:57 UTC
HMDB IDHMDB0000226
Secondary Accession Numbers
  • HMDB00226
Metabolite Identification
Common NameOrotic acid
DescriptionOrotic acid is classified as a pyrimidinemonocarboxylic acid. That is it is a uracil bearing a carboxy substituent at position C-6. It is also classified as a pyrimidinedione and a carboxylic acid. Orotic acid is a minor dietary constituent. Indeed, until it was realized that it could be synthesized by humans, orotic acid was known as vitamin B-13. The richest dietary sources of orotic acid are cow's milk and other dairy products as well as root vegetables such as carrots and beets. Dietary intake probably contributes to a basal rate of orotic acid excretion in urine because fasting decreases excretion by ~50%. However, it is now apparent that most urinary orotic acid is synthesized in the body, where it arises as an intermediate in the pathway for the synthesis of pyrimidine nucleotides. Orotic acid is converted to UMP by UMP synthase, a multifunctional protein with both orotate phosphoribosyltransferase and orotidylate decarboxylase activity. The most frequently observed inborn error of pyrimidine nucleotide synthesis is a mutation of the multifunctional protein UMP synthase (UMP synthase deficiency or orotic aciduria). This disorder prevents the conversion of orotic acid to UMP, and thus to other pyrimidines. As a result, plasma orotic acid accumulates to high concentrations, and increased quantities appear in the urine. Indeed, urinary orotic acid is so markedly increased in individuals harboring a mutation in UMP synthase that orotic acid crystals can form in the urine. The urinary concentration of orotic acid in individuals suffering from orotic aciduria can be of the order of millimoles of orotic acid per millimole creatinine. By comparison, the urinary level in unaffected individuals is ~ 1 ¬umol/mmol creatinine (PMID: 17513443 ). Orotic aciduria is characterized by megaloblastic anemia and orotic acid crystalluria that is frequently associated with some degree of physical and mental retardation. These features respond to appropriate pyrimidine replacement therapy and most cases appear to have a good prognosis. When present in sufficiently high levels, orotic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of orotic acid are associated with at least seven inborn errors of metabolism, including argininemia, LPI syndrome (lysinuric protein intolerance), hyperornithinemia-hyperammonemia-homocitrullinuria (HHH), OTC deficiency, citrullinemia type I, purine nucleoside phosphorylase deficiency, and orotic aciduria. Orotic acid is broadly classified as an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.
Structure
Data?1676999678
Synonyms
ValueSource
OrotsaeureChEBI
Uracil-6-carboxylic acidChEBI
Uracil-6-carboxylateGenerator
OrotateGenerator
1,2,3,6-Tetrahydro-2,6-dioxo-4-pyrimidecarboxylic acidHMDB
1,2,3,6-Tetrahydro-2,6-dioxo-4-pyrimidinecarboxylic acidHMDB
1,2,3,6-Tetrahydro-2,6-dioxopyrimidin-4-carbonsaeureHMDB
2,6-Dihydroxy-4-pyrimidinecarboxylic acidHMDB
2,6-Dihydroxypyrimidine-4-carboxylic acidHMDB
2,6-Dioxo-1,2,3,6-tetrahydro-pyrimidine-4-carboxylic acidHMDB
2,6-Dioxo-1,2,3,6-tetrahydropyrimidine-4-carboxylic acidHMDB
6-Carboxy-2,4-dihydroxypyrimidineHMDB
6-CarboxyuracilHMDB
6-Uracilcarboxylic acidHMDB
Acide orotiqueHMDB
Acido oroticoHMDB
Acidum oroticumHMDB
Animal galactose factorHMDB
LactiniumHMDB
MolkensaeureHMDB
OROHMDB
OrodinHMDB
OropurHMDB
OrotoninHMDB
OrotonsanHMDB
OrotsaureHMDB
OroturicHMDB
OrotylHMDB
Uracil-6-carbosaeureHMDB
Vitamin b13HMDB
Whey factorHMDB
Zinc orotateHMDB
Orotate, zincHMDB
Acid, oroticHMDB
Orotate, potassiumHMDB
Potassium orotateHMDB
Orotate, sodiumHMDB
Sodium orotateHMDB
Chemical FormulaC5H4N2O4
Average Molecular Weight156.0963
Monoisotopic Molecular Weight156.017106626
IUPAC Name2,6-dioxo-1,2,3,6-tetrahydropyrimidine-4-carboxylic acid
Traditional Nameorotic acid
CAS Registry Number65-86-1
SMILES
OC(=O)C1=CC(=O)NC(=O)N1
InChI Identifier
InChI=1S/C5H4N2O4/c8-3-1-2(4(9)10)6-5(11)7-3/h1H,(H,9,10)(H2,6,7,8,11)
InChI KeyPXQPEWDEAKTCGB-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as pyrimidinecarboxylic acids. These are pyrimidines with a structure containing a carboxyl group attached to the pyrimidine ring.
KingdomOrganic compounds
Super ClassOrganoheterocyclic compounds
ClassDiazines
Sub ClassPyrimidines and pyrimidine derivatives
Direct ParentPyrimidinecarboxylic acids
Alternative Parents
Substituents
  • Pyrimidine-6-carboxylic acid
  • Hydropyrimidine carboxylic acid derivative
  • Pyrimidone
  • Hydropyrimidine
  • Heteroaromatic compound
  • Vinylogous amide
  • Urea
  • Lactam
  • Azacycle
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic nitrogen compound
  • Organic oxygen compound
  • Organopnictogen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Aromatic heteromonocyclic compound
Molecular FrameworkAromatic heteromonocyclic compounds
External Descriptors
Ontology
Physiological effect
Disposition
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point345.5 °CNot Available
Boiling Point656.00 to 657.00 °C. @ 760.00 mm Hg (est)The Good Scents Company Information System
Water Solubility1.82 mg/mL at 18 °CNot Available
LogP-0.83SANGSTER (1994)
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Astarita_neg122.030932474
[M-H]-Baker125.230932474
[M+H]+Baker138.8530932474
[M-H]-Not Available123.0http://allccs.zhulab.cn/database/detail?ID=AllCCS00000366
[M+H]+Not Available138.85http://allccs.zhulab.cn/database/detail?ID=AllCCS00000366
Predicted Molecular Properties
PropertyValueSource
Water Solubility4.51 g/LALOGPS
logP-0.89ALOGPS
logP-1.2ChemAxon
logS-1.5ALOGPS
pKa (Strongest Acidic)2.83ChemAxon
pKa (Strongest Basic)-6ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area95.5 ŲChemAxon
Rotatable Bond Count1ChemAxon
Refractivity33.27 m³·mol⁻¹ChemAxon
Polarizability12.46 ųChemAxon
Number of Rings1ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+133.44131661259
DarkChem[M-H]-129.4431661259
AllCCS[M+H]+133.0732859911
AllCCS[M-H]-124.50732859911
DeepCCS[M+H]+130.01330932474
DeepCCS[M-H]-126.84830932474
DeepCCS[M-2H]-163.83330932474
DeepCCS[M+Na]+138.95730932474
AllCCS[M+H]+133.132859911
AllCCS[M+H-H2O]+128.632859911
AllCCS[M+NH4]+137.332859911
AllCCS[M+Na]+138.532859911
AllCCS[M-H]-124.532859911
AllCCS[M+Na-2H]-125.832859911
AllCCS[M+HCOO]-127.232859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
Orotic acidOC(=O)C1=CC(=O)NC(=O)N12650.6Standard polar33892256
Orotic acidOC(=O)C1=CC(=O)NC(=O)N11553.4Standard non polar33892256
Orotic acidOC(=O)C1=CC(=O)NC(=O)N11989.9Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Orotic acid,1TMS,isomer #1C[Si](C)(C)OC(=O)C1=CC(=O)[NH]C(=O)[NH]11660.4Semi standard non polar33892256
Orotic acid,1TMS,isomer #2C[Si](C)(C)N1C(=O)C=C(C(=O)O)[NH]C1=O1759.8Semi standard non polar33892256
Orotic acid,1TMS,isomer #3C[Si](C)(C)N1C(C(=O)O)=CC(=O)[NH]C1=O1718.4Semi standard non polar33892256
Orotic acid,2TMS,isomer #1C[Si](C)(C)OC(=O)C1=CC(=O)N([Si](C)(C)C)C(=O)[NH]11776.9Semi standard non polar33892256
Orotic acid,2TMS,isomer #1C[Si](C)(C)OC(=O)C1=CC(=O)N([Si](C)(C)C)C(=O)[NH]11882.7Standard non polar33892256
Orotic acid,2TMS,isomer #1C[Si](C)(C)OC(=O)C1=CC(=O)N([Si](C)(C)C)C(=O)[NH]12153.2Standard polar33892256
Orotic acid,2TMS,isomer #2C[Si](C)(C)OC(=O)C1=CC(=O)[NH]C(=O)N1[Si](C)(C)C1776.5Semi standard non polar33892256
Orotic acid,2TMS,isomer #2C[Si](C)(C)OC(=O)C1=CC(=O)[NH]C(=O)N1[Si](C)(C)C1869.5Standard non polar33892256
Orotic acid,2TMS,isomer #2C[Si](C)(C)OC(=O)C1=CC(=O)[NH]C(=O)N1[Si](C)(C)C2114.8Standard polar33892256
Orotic acid,2TMS,isomer #3C[Si](C)(C)N1C(C(=O)O)=CC(=O)N([Si](C)(C)C)C1=O1849.0Semi standard non polar33892256
Orotic acid,2TMS,isomer #3C[Si](C)(C)N1C(C(=O)O)=CC(=O)N([Si](C)(C)C)C1=O1925.6Standard non polar33892256
Orotic acid,2TMS,isomer #3C[Si](C)(C)N1C(C(=O)O)=CC(=O)N([Si](C)(C)C)C1=O2119.8Standard polar33892256
Orotic acid,3TMS,isomer #1C[Si](C)(C)OC(=O)C1=CC(=O)N([Si](C)(C)C)C(=O)N1[Si](C)(C)C1933.3Semi standard non polar33892256
Orotic acid,3TMS,isomer #1C[Si](C)(C)OC(=O)C1=CC(=O)N([Si](C)(C)C)C(=O)N1[Si](C)(C)C1962.0Standard non polar33892256
Orotic acid,3TMS,isomer #1C[Si](C)(C)OC(=O)C1=CC(=O)N([Si](C)(C)C)C(=O)N1[Si](C)(C)C1920.7Standard polar33892256
Orotic acid,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)C1=CC(=O)[NH]C(=O)[NH]11891.3Semi standard non polar33892256
Orotic acid,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)N1C(=O)C=C(C(=O)O)[NH]C1=O1975.4Semi standard non polar33892256
Orotic acid,1TBDMS,isomer #3CC(C)(C)[Si](C)(C)N1C(C(=O)O)=CC(=O)[NH]C1=O1969.6Semi standard non polar33892256
Orotic acid,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)C1=CC(=O)N([Si](C)(C)C(C)(C)C)C(=O)[NH]12222.3Semi standard non polar33892256
Orotic acid,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)C1=CC(=O)N([Si](C)(C)C(C)(C)C)C(=O)[NH]12294.6Standard non polar33892256
Orotic acid,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)C1=CC(=O)N([Si](C)(C)C(C)(C)C)C(=O)[NH]12322.0Standard polar33892256
Orotic acid,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)C1=CC(=O)[NH]C(=O)N1[Si](C)(C)C(C)(C)C2188.6Semi standard non polar33892256
Orotic acid,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)C1=CC(=O)[NH]C(=O)N1[Si](C)(C)C(C)(C)C2300.7Standard non polar33892256
Orotic acid,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)C1=CC(=O)[NH]C(=O)N1[Si](C)(C)C(C)(C)C2300.5Standard polar33892256
Orotic acid,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)N1C(C(=O)O)=CC(=O)N([Si](C)(C)C(C)(C)C)C1=O2305.5Semi standard non polar33892256
Orotic acid,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)N1C(C(=O)O)=CC(=O)N([Si](C)(C)C(C)(C)C)C1=O2360.9Standard non polar33892256
Orotic acid,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)N1C(C(=O)O)=CC(=O)N([Si](C)(C)C(C)(C)C)C1=O2288.1Standard polar33892256
Orotic acid,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)C1=CC(=O)N([Si](C)(C)C(C)(C)C)C(=O)N1[Si](C)(C)C(C)(C)C2477.5Semi standard non polar33892256
Orotic acid,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)C1=CC(=O)N([Si](C)(C)C(C)(C)C)C(=O)N1[Si](C)(C)C(C)(C)C2575.2Standard non polar33892256
Orotic acid,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)C1=CC(=O)N([Si](C)(C)C(C)(C)C)C(=O)N1[Si](C)(C)C(C)(C)C2324.8Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - Orotic acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0udi-1982000000-b20f2ec9a2f1acc84ae12014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Orotic acid GC-MS (3 TMS)splash10-0zfr-2693000000-07bf11b5177412647d9b2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Orotic acid GC-EI-TOF (Non-derivatized)splash10-0udi-1982000000-b20f2ec9a2f1acc84ae12017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Orotic acid GC-MS (Non-derivatized)splash10-0zfr-2693000000-07bf11b5177412647d9b2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Orotic acid GC-EI-TOF (Non-derivatized)splash10-0udi-1972000000-c04fb7381d3ca07c9b0a2017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Orotic acid GC-MS (Non-derivatized) - 70eV, Positivesplash10-06ri-6900000000-4ba5fde1f8f62c4a2ba22016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Orotic acid GC-MS (1 TMS) - 70eV, Positivesplash10-0229-9850000000-8164142820eb5162e7532017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Orotic acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Orotic acid GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Orotic acid GC-MS (TMS_1_3) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Orotic acid GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Orotic acid GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Orotic acid GC-MS (TBDMS_1_3) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
MSMass Spectrum (Electron Ionization)splash10-066r-9300000000-e5c3ef3304f93a38628c2014-09-20Not AvailableView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid Quattro_QQQ 10V, Negative-QTOF (Annotated)splash10-03di-0900000000-81d73aed73c250fff3ae2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid Quattro_QQQ 25V, Negative-QTOF (Annotated)splash10-0006-9300000000-ecb08d1854a2789480d12012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid Quattro_QQQ 40V, Negative-QTOF (Annotated)splash10-0006-9100000000-15748a4c13636c42f4f92012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positive-QTOFsplash10-02ti-4900000000-6fd6312d81f94faaaf112012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative-QTOFsplash10-03di-0900000000-1b7abf52e4a01cb855412012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid LC-ESI-QTOF , negative-QTOFsplash10-03di-0900000000-1b7abf52e4a01cb855412017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid , negative-QTOFsplash10-03di-0900000000-3dff3caa5c10aff8ce462017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid LC-ESI-QTOF , positive-QTOFsplash10-02ti-4900000000-6fd6312d81f94faaaf112017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid 10V, Positive-QTOFsplash10-004i-9000000000-351f585d58b38f6132de2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid 10V, Positive-QTOFsplash10-0a4r-0900000000-4e46209ca236b470bf7f2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid 20V, Positive-QTOFsplash10-014i-9300000000-aa9cd31d715d134ee8dd2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid 40V, Positive-QTOFsplash10-014i-9000000000-cdd01e740b6f9d1a909a2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Orotic acid 10V, Negative-QTOFsplash10-03di-1900000000-baf39ad8ab6f08fb1a7b2021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Orotic acid 10V, Positive-QTOFsplash10-0bt9-0900000000-1ad59feda583f2247b212015-04-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Orotic acid 20V, Positive-QTOFsplash10-03di-2900000000-0d0d27215e9f39a2a15b2015-04-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Orotic acid 40V, Positive-QTOFsplash10-0006-9100000000-918a38777f5d4e0496972015-04-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Orotic acid 10V, Negative-QTOFsplash10-0a4i-0900000000-d7bbee7f2dc7bb0c6a832015-04-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Orotic acid 20V, Negative-QTOFsplash10-03dl-7900000000-303fe3e06e98f518a2ee2015-04-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Orotic acid 40V, Negative-QTOFsplash10-0006-9000000000-b2c02975bbba65c2f1c62015-04-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Orotic acid 10V, Positive-QTOFsplash10-0bt9-0900000000-1ad59feda583f2247b212015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Orotic acid 20V, Positive-QTOFsplash10-03di-2900000000-0d0d27215e9f39a2a15b2015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Orotic acid 40V, Positive-QTOFsplash10-0006-9100000000-918a38777f5d4e0496972015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Orotic acid 10V, Negative-QTOFsplash10-0a4i-0900000000-d7bbee7f2dc7bb0c6a832015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Orotic acid 20V, Negative-QTOFsplash10-03dl-7900000000-303fe3e06e98f518a2ee2015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Orotic acid 40V, Negative-QTOFsplash10-0006-9000000000-b2c02975bbba65c2f1c62015-05-27Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 600 MHz, H2O, experimental)2012-12-04Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum

IR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)2023-02-03FELIX labView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Amniotic Fluid
  • Blood
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Liver
  • Pancreas
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
Amniotic FluidDetected and Quantified0.27 +/- 0.25 uMAdult (>18 years old)FemaleNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.89 +/- 0.63 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified2.874 +/- 8.0460 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified5.0 +/- 0.4 uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedNormal details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified<20 umol/mmol creatinineNewborn (0-30 days old)MaleNormal details
UrineDetected and Quantified9.9 +/- 7.1 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified0.26-3.20 umol/mmol creatinineAdult (>18 years old)Both
Normal
    • Hereditary Orotic...
details
UrineDetected and Quantified0.5-3.3 umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Hereditary Orotic...
details
UrineDetected and Quantified 0.49 - 3.44 umol/mmol creatinineInfant (0-1 year old)Both
Normal
details
UrineDetected and Quantified1.0-3.2 umol/mmol creatinineInfant (0-1 year old)BothNormal
    • Hereditary Orotic...
details
UrineDetected and Quantified0.63 +/- 0.21 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified1.3-5.3 umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • Hereditary Orotic...
details
UrineDetected and Quantified2.07 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified0-5 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified0-5 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0.23 +/- 0.13 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified0.16 +/- 0.12 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<4.33 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified2.05 +/- 0.15 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • Analysis of 40 NI...
details
UrineDetected and Quantified0.4 umol/mmol creatinineInfant (0-1 year old)Both
Normal
details
UrineDetected and Quantified0.061 +/- 0.003 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified1.05 +/- 1.22 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.0800-0.440 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified1.05 +/- 1.22 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified<3 umol/mmol creatinineNewborn (0-30 days old)Not SpecifiedNormal details
UrineDetected and Quantified0.0350-0.260 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Physician's Guide...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified38.506 +/- 91.954 uMAdult (>18 years old)Both
Uremia
details
BloodDetected and Quantified0.94 +/- 0.78 uMAdult (>18 years old)BothCanavan disease details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)BothCrohns disease details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)BothUlcerative colitis details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUnclassified IBD details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedCrohns disease details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUlcerative colitis details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
UrineDetected and Quantified427.09-2989.61 umol/mmol creatinineAdult (>18 years old)Not Specified
Orotic Aciduria hereditary
    • Hereditary Orotic...
details
UrineDetected and Quantified1.4-3.2 umol/mmol creatinineAdult (>18 years old)Male
Orotic Aciduria hereditary
    • Hereditary Orotic...
details
UrineDetected and Quantified1000 umol/mmol creatinineChildren (1-13 years old)Not Specified
Orotic Aciduria hereditary
    • Hereditary Orotic...
details
UrineDetected and Quantified293.333-573.333 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedOrotic Aciduria hereditary
    • Hereditary Orotic...
details
UrineDetected and Quantified5.6 umol/mmol creatinineChildren (1-13 years old)Not Specified
Orotic Aciduria hereditary
    • Hereditary Orotic...
details
UrineDetected and Quantified8.24 umol/mmol creatinineAdult (>18 years old)Female
Orotic aciduria I
details
UrineDetected and Quantified66.667-640 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedOrotic Aciduria hereditary
    • Hereditary Orotic...
details
UrineDetected and Quantified59-282 umol/mmol creatinineAdult (>18 years old)BothHyperornithinemia–Hyperammonemia-Homocitrullinuria syndrome details
UrineDetected and Quantified44.16(0.72-642.79) umol/mmol creatinineChildren (1-13 years old)Both
Lysinuric protein intolerance (LPI) syndrome
details
UrineDetected and Quantified2-2800 umol/mmol creatinineChildren (1-13 years old)MaleOTC-deficiency details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer details
UrineDetected and Quantified253.2 +/- 540.0 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedOTC-deficiency details
UrineDetected and Quantified6.11 +/- 5.48 umol/mmol creatinineAdult (>18 years old)BothCanavan disease details
UrineDetected and Quantified0.720-2339 umol/mmol creatinineChildren (1-13 years old)Male
N-acetylglutamate synthetase deficiency
details
UrineDetected and Quantified5.4 umol/mmol creatinineNewborn (0-30 days old)MalePhosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic details
UrineDetected and Quantified>20 umol/mmol creatinineNewborn (0-30 days old)MaleOrnithine transcarbamylase deficiency details
Associated Disorders and Diseases
Disease References
Canavan disease
  1. Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed:16139832 ]
Uremia
  1. Vanholder R, De Smet R, Glorieux G, Argiles A, Baurmeister U, Brunet P, Clark W, Cohen G, De Deyn PP, Deppisch R, Descamps-Latscha B, Henle T, Jorres A, Lemke HD, Massy ZA, Passlick-Deetjen J, Rodriguez M, Stegmayr B, Stenvinkel P, Tetta C, Wanner C, Zidek W: Review on uremic toxins: classification, concentration, and interindividual variability. Kidney Int. 2003 May;63(5):1934-43. doi: 10.1046/j.1523-1755.2003.00924.x. [PubMed:12675874 ]
Colorectal cancer
  1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  2. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  3. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Crohn's disease
  1. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Ulcerative colitis
  1. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Ornithine transcarbamylase deficiency
  1. Arranz JA, Riudor E, Rodes M, Roig M, Climent C, Rubio V, Sentis M, Burlina A: Optimization of allopurinol challenge: sample purification, protein intake control, and the use of orotidine response as a discriminative variable improve performance of the test for diagnosing ornithine carbamoyltransferase deficiency. Clin Chem. 1999 Jul;45(7):995-1001. [PubMed:10388475 ]
  2. Kopin AS, McBride EW, Chen C, Freidinger RM, Chen D, Zhao CM, Beinborn M: Identification of a series of CCK-2 receptor nonpeptide agonists: sensitivity to stereochemistry and a receptor point mutation. Proc Natl Acad Sci U S A. 2003 Apr 29;100(9):5525-30. doi: 10.1073/pnas.0831223100. Epub 2003 Apr 15. [PubMed:12697901 ]
  3. Drogari E, Leonard JV: Late onset ornithine carbamoyl transferase deficiency in males. Arch Dis Child. 1988 Nov;63(11):1363-7. [PubMed:3202644 ]
Orotic aciduria I
  1. Grohmann K, Lauffer H, Lauenstein P, Hoffmann GF, Seidlitz G: Hereditary orotic aciduria with epilepsy and without megaloblastic anemia. Neuropediatrics. 2015 Apr;46(2):123-5. doi: 10.1055/s-0035-1547341. Epub 2015 Mar 10. [PubMed:25757096 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
  3. Webster DR, Becroft DO, van Gennip AH, Van Kuilenburg AP. (2014). Hereditary Orotic Aciduria and Other Disorders of Pyrimidine Metabolism. In: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. eds. The Online Metabolic and Molecular Bases of Inherited Disease New York, NY. http://ommbid.mhmedical.com/content.aspx?bookid=971&sectionid=62636333. McGraw-Hill.
N-acetylglutamate synthetase deficiency
  1. Schubiger G, Bachmann C, Barben P, Colombo JP, Tonz O, Schupbach D: N-acetylglutamate synthetase deficiency: diagnosis, management and follow-up of a rare disorder of ammonia detoxication. Eur J Pediatr. 1991 Mar;150(5):353-6. [PubMed:2044610 ]
Hyperornithinemia-hyperammonemia-homocitrullinuria
  1. Rodes M, Ribes A, Pineda M, Alvarez L, Fabregas I, Fernandez Alvarez E, Coude FX, Grimber G: A new family affected by the syndrome of hyperornithinaemia, hyperammonaemia and homocitrullinuria. J Inherit Metab Dis. 1987;10(1):73-81. [PubMed:3106719 ]
Lysinuric protein intolerance
  1. Rajantie J: Orotic aciduria in lysinuric protein intolerance: dependence on the urea cycle intermediates. Pediatr Res. 1981 Feb;15(2):115-9. doi: 10.1203/00006450-198102000-00006. [PubMed:7254935 ]
Phosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic
  1. Santra S, Cameron JM, Shyr C, Zhang L, Drogemoller B, Ross CJ, Wasserman WW, Wevers RA, Rodenburg RJ, Gupte G, Preece MA, van Karnebeek CD: Cytosolic phosphoenolpyruvate carboxykinase deficiency presenting with acute liver failure following gastroenteritis. Mol Genet Metab. 2016 May;118(1):21-7. doi: 10.1016/j.ymgme.2016.03.001. Epub 2016 Mar 4. [PubMed:26971250 ]
Associated OMIM IDs
  • 271900 (Canavan disease)
  • 114500 (Colorectal cancer)
  • 266600 (Crohn's disease)
  • 311250 (Ornithine transcarbamylase deficiency)
  • 258900 (Orotic aciduria I)
  • 608300 (N-acetylglutamate synthetase deficiency)
  • 238970 (Hyperornithinemia-hyperammonemia-homocitrullinuria)
  • 222700 (Lysinuric protein intolerance)
  • 261680 (Phosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic)
DrugBank IDDB02262
Phenol Explorer Compound IDNot Available
FooDB IDFDB031072
KNApSAcK IDC00019689
Chemspider ID942
KEGG Compound IDC00295
BioCyc IDOROTATE
BiGG ID34527
Wikipedia LinkOrotic_acid
METLIN ID318
PubChem Compound967
PDB IDNot Available
ChEBI ID16742
Food Biomarker OntologyNot Available
VMH IDOROT
MarkerDB IDMDB00000108
Good Scents IDrw1099361
References
Synthesis ReferenceMitchell, Herschel K.; Nyc, Joseph F. Intermediates in the synthesis of orotic acid from oxalacetic ester and urea. Journal of the American Chemical Society (1947), 69 674-7.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed:2026685 ]
  2. Zieve L: Conditional deficiencies of ornithine or arginine. J Am Coll Nutr. 1986;5(2):167-76. [PubMed:3088083 ]
  3. Visek WJ: Ammonia: its effects on biological systems, metabolic hormones, and reproduction. J Dairy Sci. 1984 Mar;67(3):481-98. [PubMed:6371080 ]
  4. Arranz JA, Riudor E, Rodes M, Roig M, Climent C, Rubio V, Sentis M, Burlina A: Optimization of allopurinol challenge: sample purification, protein intake control, and the use of orotidine response as a discriminative variable improve performance of the test for diagnosing ornithine carbamoyltransferase deficiency. Clin Chem. 1999 Jul;45(7):995-1001. [PubMed:10388475 ]
  5. Sugio K, Gazdar AF, Albores-Saavedra J, Kokkinakis DM: High yields of K-ras mutations in intraductal papillary mucinous tumors and invasive adenocarcinomas induced by N-nitroso(2-hydroxypropyl)(2-oxopropyl)amine in the pancreas of female Syrian hamsters. Carcinogenesis. 1996 Feb;17(2):303-9. [PubMed:8625455 ]
  6. Finkelstein JE, Hauser ER, Leonard CO, Brusilow SW: Late-onset ornithine transcarbamylase deficiency in male patients. J Pediatr. 1990 Dec;117(6):897-902. [PubMed:2246687 ]
  7. Harris ML, Oberholzer VG: Conditions affecting the colorimetry of orotic acid and orotidine in urine. Clin Chem. 1980 Mar;26(3):473-9. [PubMed:7363468 ]
  8. Jakobs C, Sweetman L, Nyhan WL, Gruenke L, Craig JC, Wadman SK: Stable isotope dilution analysis of orotic acid and uracil in amniotic fluid. Clin Chim Acta. 1984 Nov 15;143(2):123-33. [PubMed:6391739 ]
  9. Visek WJ: Arginine and disease states. J Nutr. 1985 Apr;115(4):532-41. [PubMed:3884753 ]
  10. Mizutani Y, Wada H, Fukushima M, Yoshida O, Nakanishi H, Li YN, Miki T: Prognostic significance of orotate phosphoribosyltransferase activity in bladder carcinoma. Cancer. 2004 Feb 15;100(4):723-31. [PubMed:14770427 ]
  11. Valik D, Sedova Z, Starha J, Zeman J, Hruba E, Dvorakova L: Acute hyperammonaemic encephalopathy in a female newborn caused by a novel, de novo mutation in the ornithine transcarbamylase gene. Acta Paediatr. 2004 May;93(5):710-1. [PubMed:15174800 ]
  12. Mills GC, Schmalstieg FC, Newkirk KE, Goldblum RM: Cytosine and orotic acid in urine of immunodeficient children. Clin Chem. 1979 Mar;25(3):419-24. [PubMed:262183 ]
  13. Paradis D, Giguere R, Auray-Blais C, Draper P, Lemieux B: An automated method for the determination of orotic acid in the urine of children being screened for metabolic disorders. Clin Biochem. 1980 Aug;13(4):160-3. [PubMed:7449082 ]
  14. Potter M, Hammond JW, Sim KG, Green AK, Wilcken B: Ornithine carbamoyltransferase deficiency: improved sensitivity of testing for protein tolerance in the diagnosis of heterozygotes. J Inherit Metab Dis. 2001 Feb;24(1):5-14. [PubMed:11286382 ]
  15. van Kuilenburg AB, van Lenthe H, Loffler M, van Gennip AH: Analysis of pyrimidine synthesis "de novo" intermediates in urine and dried urine filter- paper strips with HPLC-electrospray tandem mass spectrometry. Clin Chem. 2004 Nov;50(11):2117-24. Epub 2004 Sep 16. [PubMed:15375016 ]
  16. Brosnan ME, Brosnan JT: Orotic acid excretion and arginine metabolism. J Nutr. 2007 Jun;137(6 Suppl 2):1656S-1661S. [PubMed:17513443 ]
  17. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Enzymes

General function:
Involved in catalytic activity
Specific function:
Catalyzes the conversion of dihydroorotate to orotate with quinone as electron acceptor.
Gene Name:
DHODH
Uniprot ID:
Q02127
Molecular weight:
42866.93
Reactions
L-Dihydroorotic acid + a quinone → Orotic acid + a quinoldetails
L-Dihydroorotic acid + Quinone → Orotic acid + Hydroquinonedetails
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
UMPS
Uniprot ID:
P11172
Molecular weight:
52221.075
Reactions
Orotidylic acid + Pyrophosphate → Orotic acid + Phosphoribosyl pyrophosphatedetails