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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-11-09 23:12:16 UTC
HMDB IDHMDB0000130
Secondary Accession Numbers
  • HMDB00130
Metabolite Identification
Common NameHomogentisic acid
DescriptionHomogentisic 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.
Structure
Data?1592334513
Synonyms
ValueSource
2,5-Dihydroxyphenylacetic acidChEBI
2-(3,6-DIHYDROXYPHENYL)acetIC ACIDChEBI
2,5-DihydroxyphenylacetateKegg
2-(3,6-DIHYDROXYPHENYL)acetateGenerator
HomogentisateGenerator
(2,5-Dihydroxyphenyl)-acetateHMDB
(2,5-Dihydroxyphenyl)-acetic acidHMDB
2,5-Dihydroxy-a-toluateHMDB
2,5-Dihydroxy-a-toluic acidHMDB
2,5-Dihydroxy-alpha-toluateHMDB
2,5-Dihydroxy-alpha-toluic acidHMDB
2,5-Dihydroxy-benzeneacetateHMDB
2,5-Dihydroxy-benzeneacetic acidHMDB
AlcaptonHMDB
Homogentisate acidHMDB
HomogentisinateHMDB
Homogentisinic acidHMDB
Melanic acidHMDB
Acid, homogentisicHMDB
Chemical FormulaC8H8O4
Average Molecular Weight168.1467
Monoisotopic Molecular Weight168.042258744
IUPAC Name2-(2,5-dihydroxyphenyl)acetic acid
Traditional Namehomogentisic acid
CAS Registry Number451-13-8
SMILES
OC(=O)CC1=C(O)C=CC(O)=C1
InChI Identifier
InChI=1S/C8H8O4/c9-6-1-2-7(10)5(3-6)4-8(11)12/h1-3,9-10H,4H2,(H,11,12)
InChI KeyIGMNYECMUMZDDF-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as 2(hydroxyphenyl)acetic acids. These are phenylacetic acids that carry a hydroxyl group at the 2-position.
KingdomOrganic compounds
Super ClassBenzenoids
ClassBenzene and substituted derivatives
Sub ClassPhenylacetic acids
Direct Parent2(hydroxyphenyl)acetic acids
Alternative Parents
Substituents
  • 2(hydroxyphenyl)acetic acid
  • Hydroquinone
  • 1-hydroxy-2-unsubstituted benzenoid
  • Phenol
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aromatic homomonocyclic compound
Molecular FrameworkAromatic homomonocyclic compounds
External Descriptors
Ontology
Physiological effect

Adverse health effect

Disposition

Biological location

Source

Process

Naturally occurring process

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point153 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility850 mg/mL at 25 °CNot Available
LogP0.86SANGSTER (1994)
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Not Available133.1http://allccs.zhulab.cn/database/detail?ID=AllCCS00000526
Predicted Molecular Properties
PropertyValueSource
Water Solubility8.1 g/LALOGPS
logP0.81ALOGPS
logP1ChemAxon
logS-1.3ALOGPS
pKa (Strongest Acidic)3.57ChemAxon
pKa (Strongest Basic)-5.9ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area77.76 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity41.33 m³·mol⁻¹ChemAxon
Polarizability15.57 ųChemAxon
Number of Rings1ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+138.0331661259
DarkChem[M-H]-132.88831661259
AllCCS[M+H]+135.79532859911
AllCCS[M-H]-131.25632859911
DeepCCS[M+H]+135.36430932474
DeepCCS[M-H]-132.53430932474
DeepCCS[M-2H]-169.34330932474
DeepCCS[M+Na]+144.88130932474
AllCCS[M+H]+135.832859911
AllCCS[M+H-H2O]+131.432859911
AllCCS[M+NH4]+139.932859911
AllCCS[M+Na]+141.132859911
AllCCS[M-H]-131.332859911
AllCCS[M+Na-2H]-132.332859911
AllCCS[M+HCOO]-133.432859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
Homogentisic acidOC(=O)CC1=C(O)C=CC(O)=C13377.3Standard polar33892256
Homogentisic acidOC(=O)CC1=C(O)C=CC(O)=C11715.9Standard non polar33892256
Homogentisic acidOC(=O)CC1=C(O)C=CC(O)=C11791.2Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Homogentisic acid,1TMS,isomer #1C[Si](C)(C)OC(=O)CC1=CC(O)=CC=C1O1822.5Semi standard non polar33892256
Homogentisic acid,1TMS,isomer #2C[Si](C)(C)OC1=CC=C(O)C=C1CC(=O)O1815.4Semi standard non polar33892256
Homogentisic acid,1TMS,isomer #3C[Si](C)(C)OC1=CC=C(O)C(CC(=O)O)=C11819.5Semi standard non polar33892256
Homogentisic acid,2TMS,isomer #1C[Si](C)(C)OC(=O)CC1=CC(O[Si](C)(C)C)=CC=C1O1831.4Semi standard non polar33892256
Homogentisic acid,2TMS,isomer #2C[Si](C)(C)OC(=O)CC1=CC(O)=CC=C1O[Si](C)(C)C1862.8Semi standard non polar33892256
Homogentisic acid,2TMS,isomer #3C[Si](C)(C)OC1=CC=C(O[Si](C)(C)C)C(CC(=O)O)=C11857.6Semi standard non polar33892256
Homogentisic acid,3TMS,isomer #1C[Si](C)(C)OC(=O)CC1=CC(O[Si](C)(C)C)=CC=C1O[Si](C)(C)C1852.9Semi standard non polar33892256
Homogentisic acid,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CC1=CC(O)=CC=C1O2074.0Semi standard non polar33892256
Homogentisic acid,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC1=CC=C(O)C=C1CC(=O)O2076.0Semi standard non polar33892256
Homogentisic acid,1TBDMS,isomer #3CC(C)(C)[Si](C)(C)OC1=CC=C(O)C(CC(=O)O)=C12066.5Semi standard non polar33892256
Homogentisic acid,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CC1=CC(O[Si](C)(C)C(C)(C)C)=CC=C1O2317.5Semi standard non polar33892256
Homogentisic acid,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)CC1=CC(O)=CC=C1O[Si](C)(C)C(C)(C)C2333.6Semi standard non polar33892256
Homogentisic acid,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)OC1=CC=C(O[Si](C)(C)C(C)(C)C)C(CC(=O)O)=C12340.2Semi standard non polar33892256
Homogentisic acid,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CC1=CC(O[Si](C)(C)C(C)(C)C)=CC=C1O[Si](C)(C)C(C)(C)C2516.8Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - Homogentisic acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-000t-0945000000-2f4b9ef9c9ee6e7cd1ed2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Homogentisic acid GC-MS (3 TMS)splash10-0016-3955000000-1cdc64ab3032ff29df292014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Homogentisic acid EI-B (Non-derivatized)splash10-0a5c-0679000000-97890865f85a79efa2e62017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Homogentisic acid GC-EI-TOF (Non-derivatized)splash10-000t-0945000000-2f4b9ef9c9ee6e7cd1ed2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Homogentisic acid GC-MS (Non-derivatized)splash10-0016-3955000000-1cdc64ab3032ff29df292017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Homogentisic acid GC-EI-TOF (Non-derivatized)splash10-0002-0944000000-fd48cc27da661ce248332017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (Non-derivatized) - 70eV, Positivesplash10-00di-4900000000-ea40fc8d43156151710c2017-08-28Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (3 TMS) - 70eV, Positivesplash10-014i-5093000000-c342ba08a9bff049fc1c2017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TMS_1_3) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TMS_2_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TMS_2_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TMS_2_3) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TBDMS_1_3) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TBDMS_2_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TBDMS_2_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TBDMS_2_3) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homogentisic acid GC-MS (TBDMS_3_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-00di-0900000000-9c395aa569b4b54f86812012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-00xs-9500000000-ab92286f0d5bc843814b2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-014l-9000000000-35740a40f4fdf3e84c3c2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative-QTOFsplash10-014i-0900000000-f4eba60d63bd99e18b8b2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative-QTOFsplash10-00di-0900000000-12e330dc18f0b128b9612012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative-QTOFsplash10-00di-0900000000-4bd53b0472edd31e86092012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative-QTOFsplash10-00di-0900000000-7121173193a9ba3751a42012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative-QTOFsplash10-00di-1900000000-04eca21bf25c671b8b6e2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative-QTOFsplash10-00di-0900000000-780d3740762c2ff89d9a2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid LC-ESI-QQ , negative-QTOFsplash10-014i-0900000000-f4eba60d63bd99e18b8b2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid LC-ESI-QQ , negative-QTOFsplash10-00di-0900000000-12e330dc18f0b128b9612017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid LC-ESI-QQ , negative-QTOFsplash10-00di-0900000000-4bd53b0472edd31e86092017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid LC-ESI-QQ , negative-QTOFsplash10-00di-0900000000-7121173193a9ba3751a42017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid LC-ESI-QQ , negative-QTOFsplash10-00di-1900000000-04eca21bf25c671b8b6e2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid LC-ESI-QTOF , negative-QTOFsplash10-00di-0900000000-9c85164306514c6d4c4b2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid , negative-QTOFsplash10-00di-0900000000-867a4e32ba2d14fd0aad2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid 10V, Negative-QTOFsplash10-00di-0900000000-ac8e05a9300b51b24e772021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid 40V, Negative-QTOFsplash10-00di-1900000000-eeb664cef1197d6097352021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homogentisic acid 20V, Negative-QTOFsplash10-00di-0900000000-1d890267ec7f3a5843b32021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homogentisic acid 10V, Positive-QTOFsplash10-0gb9-0900000000-a650bbb95019f3305d032017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homogentisic acid 20V, Positive-QTOFsplash10-00di-1900000000-94f13b2c7b97263537012017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homogentisic acid 40V, Positive-QTOFsplash10-05tf-9300000000-968a6390a00dcd77e5662017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homogentisic acid 10V, Negative-QTOFsplash10-01b9-0900000000-0f9d5926d702af7d4d632017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homogentisic acid 20V, Negative-QTOFsplash10-01b9-1900000000-7f257033d3b1952b3c272017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homogentisic acid 40V, Negative-QTOFsplash10-0a4i-9800000000-bbcb0f0d7348cd1663b12017-07-26Wishart LabView Spectrum

NMR Spectra

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

IR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted IR SpectrumIR Ion Spectrum (Quadrupole Ion Trap, ESI-, Adduct: [M-H]-)2022-02-11FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Quadrupole Ion Trap, ESI+, Adduct: [M+Na]+)2022-02-11FELIX labView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue Locations
  • Cartilage
  • Kidney
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.043 (0.014 - 0.071) uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedNormal details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified0.04 +/- 0.17 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified14.333 +/- 9.278 umol/mmol creatinineInfant (0-1 year old)Not Specified
Normal
details
UrineDetected and Quantified1.70 (0.5 - 2.8) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified16.593 +/- 12.251 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
details
UrineDetected and Quantified16.771 +/- 13.262 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
details
UrineDetected and Quantified7.315 +/- 4.698 umol/mmol creatinineAdolescent (13-18 years old)Not Specified
Normal
details
UrineDetected and Quantified7.672 +/- 3.330 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
details
UrineDetected and Quantified0.0 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.917 +/- 1.061 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified33.33-46.5 uMAdult (>18 years old)Not SpecifiedAlkaptonuria details
BloodDetected and Quantified35.5 (33.0 - 38.0) uMAdult (>18 years old)BothAlkaptonuria 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 QuantifiedChildren (6 - 18 years old)Not SpecifiedUnclassified IBD details
UrineDetected and Quantified1746.67-2500.00 umol/mmol creatinineAdult (>18 years old)Not SpecifiedAlkaptonuria details
UrineDetected and Quantified666.085 umol/mmol creatinineInfant (0-1 year old)FemaleHomogentisic Acid Oxidase deficiency (Alkaptonuria) details
UrineDetected and Quantified2100 umol/mmol creatinineInfant (0-1 year old)FemaleHomogentisic Acid Oxidase deficiency (Alkaptonuria) details
UrineDetected and Quantified4690 umol/mmol creatinineChildren (1 - 13 years old)FemaleHomogentisic Acid Oxidase deficiency (Alkaptonuria) details
UrineDetected and Quantified5020 umol/mmol creatinineChildren (1 - 13 years old)FemaleHomogentisic Acid Oxidase deficiency (Alkaptonuria) details
UrineDetected and Quantified>1189.438 umol/mmol creatinineNewborn (0-30 days old)MaleHomogentisic Acid Oxidase deficiency (Alkaptonuria) details
UrineDetected and Quantified1570-2220 umol/mmol creatinineChildren (1 - 13 years old)FemaleHomogentisic Acid Oxidase deficiency (Alkaptonuria) details
UrineDetected and Quantified1980-2710 umol/mmol creatinineChildren (1 - 13 years old)FemaleHomogentisic Acid Oxidase deficiency (Alkaptonuria) details
UrineDetected and Quantified1819 (1134 - 2504) umol/mmol creatinineAdult (>18 years old)BothAlkaptonuria details
UrineDetected and Quantified> 2780 umol/mmol creatinineAdult (>18 years old)BothAlkaptonuria details
UrineDetected and Quantified>1189.438 umol/mmol creatinineNewborn (0-30 days old)Male
Alkaptonuria
details
UrineDetected and Quantified3.009 +/- 1.469 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
Associated Disorders and Diseases
Disease References
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 ]
Alkaptonuria
  1. Phornphutkul C, Introne WJ, Perry MB, Bernardini I, Murphey MD, Fitzpatrick DL, Anderson PD, Huizing M, Anikster Y, Gerber LH, Gahl WA: Natural history of alkaptonuria. N Engl J Med. 2002 Dec 26;347(26):2111-21. [PubMed:12501223 ]
  2. La Du BN Jr: Are we ready to try to cure alkaptonuria? Am J Hum Genet. 1998 Apr;62(4):765-7. [PubMed:9529368 ]
  3. Bory C, Boulieu R, Chantin C, Mathieu M: Diagnosis of alcaptonuria: rapid analysis of homogentisic acid by HPLC. Clin Chim Acta. 1990 Jul;189(1):7-11. [PubMed:2383921 ]
  4. Datta AK, Mandal S, Dasgupta A, Ghosh TK: Alkaptonuria diagnosed in a 4-month-old baby girl: a case report. Cases J. 2008 Nov 13;1(1):308. doi: 10.1186/1757-1626-1-308. [PubMed:19014543 ]
  5. Olah AV, Llyes I, Szoke A, Csizy I, Toth J, Varga J: Urinary homogentisic acid in alkaptonuric and healthy children. Clin Chem Lab Med. 2003 Mar;41(3):356-9. [PubMed:12705346 ]
  6. Thalagahage KN, Jayaweera JA, Kumbukgolla WW, Senavirathne I: Detection of alkaptonuria in a 1-week-old infant. BMJ Case Rep. 2015 May 8;2015. pii: bcr2014208505. doi: 10.1136/bcr-2014-208505. [PubMed:25956497 ]
  7. Bory C, Boulieu R, Chantin C, Mathieu M: Homogentisic acid determined in biological fluids by HPLC. Clin Chem. 1989 Feb;35(2):321-2. [PubMed:2914385 ]
  8. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Associated OMIM IDs
DrugBank IDDB08327
Phenol Explorer Compound IDNot Available
FooDB IDFDB030907
KNApSAcK IDC00007309
Chemspider ID759
KEGG Compound IDC00544
BioCyc IDHOMOGENTISATE
BiGG ID1485308
Wikipedia LinkHomogentisic_acid
METLIN ID331
PubChem Compound780
PDB IDNot Available
ChEBI ID44747
Food Biomarker OntologyNot Available
VMH IDHGENTIS
MarkerDB IDMDB00000062
Good Scents IDNot Available
References
Synthesis ReferenceNot Available
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. Phornphutkul C, Introne WJ, Perry MB, Bernardini I, Murphey MD, Fitzpatrick DL, Anderson PD, Huizing M, Anikster Y, Gerber LH, Gahl WA: Natural history of alkaptonuria. N Engl J Med. 2002 Dec 26;347(26):2111-21. [PubMed:12501223 ]
  3. La Du BN Jr: Are we ready to try to cure alkaptonuria? Am J Hum Genet. 1998 Apr;62(4):765-7. [PubMed:9529368 ]
  4. Concepcion Masip T, Banares Baudet F, Traba ML, Rodriguez de Minon Cifuentes JL: [Alkaptonuria, prostatic calculi, and ectopic ureter]. Actas Urol Esp. 1997 Feb;21(2):167-70. [PubMed:9214216 ]
  5. Ehongo A, Schrooyen M, Pereleux A: [Important bilateral corneal astigmatism in a case of ocular ochronosis]. Bull Soc Belge Ophtalmol. 2005;(295):17-21. [PubMed:15849984 ]
  6. Deutsch JC, Santhosh-Kumar CR: Quantitation of homogentisic acid in normal human plasma. J Chromatogr B Biomed Appl. 1996 Feb 23;677(1):147-51. [PubMed:8925087 ]
  7. Janocha S, Wolz W, Srsen S, Srsnova K, Montagutelli X, Guenet JL, Grimm T, Kress W, Muller CR: The human gene for alkaptonuria (AKU) maps to chromosome 3q. Genomics. 1994 Jan 1;19(1):5-8. [PubMed:8188241 ]
  8. de Haas V, Carbasius Weber EC, de Klerk JB, Bakker HD, Smit GP, Huijbers WA, Duran M, Poll-The BT: The success of dietary protein restriction in alkaptonuria patients is age-dependent. J Inherit Metab Dis. 1998 Dec;21(8):791-8. [PubMed:9870204 ]
  9. Hegedus ZL, Nayak U: Homogentisic acid and structurally related compounds as intermediates in plasma soluble melanin formation and in tissue toxicities. Arch Int Physiol Biochim Biophys. 1994 May-Jun;102(3):175-81. [PubMed:8000039 ]
  10. Venkataseshan VS, Chandra B, Graziano V, Steinlauf P, Marquet E, Irmiere V, Needle MA: Alkaptonuria and renal failure: a case report and review of the literature. Mod Pathol. 1992 Jul;5(4):464-71. [PubMed:1495952 ]
  11. Bory C, Boulieu R, Chantin C, Mathieu M: Diagnosis of alcaptonuria: rapid analysis of homogentisic acid by HPLC. Clin Chim Acta. 1990 Jul;189(1):7-11. [PubMed:2383921 ]

Enzymes

General function:
Involved in 4-hydroxyphenylpyruvate dioxygenase activity
Specific function:
Key enzyme in the degradation of tyrosine.
Gene Name:
HPD
Uniprot ID:
P32754
Molecular weight:
40497.105
Reactions
4-Hydroxyphenylpyruvic acid + Oxygen → Homogentisic acid + CO(2)details
4-Hydroxyphenylpyruvic acid + Oxygen → Homogentisic acid + Carbon dioxidedetails
General function:
Involved in homogentisate 1,2-dioxygenase activity
Specific function:
Not Available
Gene Name:
HGD
Uniprot ID:
Q93099
Molecular weight:
49963.41
Reactions
Homogentisic acid + Oxygen → Maleylacetoacetic aciddetails