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Record Information
Version4.0
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-09-27 06:56:14 UTC
HMDB IDHMDB0000130
Secondary Accession Numbers
  • HMDB00130
Metabolite Identification
Common NameHomogentisic acid
DescriptionHomogentisic acid is an intermediate of the metabolic breakdown of tyrosine and phenylalanine; it occurs in the urine in cases of alkaptonuria. (OMIN 203500) Homogentisic acid is the primary precursor of melanin synthesis in Vibrio cholerae.
Structure
Thumb
Synonyms
ValueSource
2,5-Dihydroxyphenylacetic acidChEBI
2-(3,6-DIHYDROXYPHENYL)acetIC ACIDChEBI
2,5-DihydroxyphenylacetateGenerator
HomogentisateGenerator
2-(3,6-DIHYDROXYPHENYL)acetateGenerator
(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, homogentisicMeSH
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
DescriptionThis compound belongs to the class of chemical entities known as 2(hydroxyphenyl)acetic acids. These are phenylacetic acids that carry a hydroxyl group at the 2-position.
KingdomChemical entities
Super ClassOrganic compounds
ClassBenzenoids
Sub ClassBenzene and substituted derivatives
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

Health effect:

  Health condition:

    Musculoskeletal and connective tissue disorders:

Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

  Tissue and substructures:

  Organ and components:

Source:

Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

Role

Industrial application:

  Pharmaceutical industry:

Indirect biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point153 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility850 mg/mL at 25 °CNot Available
LogP0.86SANGSTER (1994)
Predicted 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
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-000t-0945000000-2f4b9ef9c9ee6e7cd1edView in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-0016-3955000000-1cdc64ab3032ff29df29View in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0a5c-0679000000-97890865f85a79efa2e6View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000t-0945000000-2f4b9ef9c9ee6e7cd1edView in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0016-3955000000-1cdc64ab3032ff29df29View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0944000000-fd48cc27da661ce24833View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, PositiveNot AvailableView in JSpectraViewer
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, PositiveNot AvailableView in JSpectraViewer
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-00di-0900000000-9c395aa569b4b54f8681View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00xs-9500000000-ab92286f0d5bc843814bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-014l-9000000000-35740a40f4fdf3e84c3cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-014i-0900000000-f4eba60d63bd99e18b8bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-00di-0900000000-12e330dc18f0b128b961View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-00di-0900000000-4bd53b0472edd31e8609View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-00di-0900000000-7121173193a9ba3751a4View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-00di-1900000000-04eca21bf25c671b8b6eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-00di-0900000000-780d3740762c2ff89d9aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-014i-0900000000-f4eba60d63bd99e18b8bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-0900000000-12e330dc18f0b128b961View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-0900000000-4bd53b0472edd31e8609View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-0900000000-7121173193a9ba3751a4View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-1900000000-04eca21bf25c671b8b6eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , negativesplash10-00di-0900000000-9c85164306514c6d4c4bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - , negativesplash10-00di-0900000000-867a4e32ba2d14fd0aadView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0gb9-0900000000-a650bbb95019f3305d03View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00di-1900000000-94f13b2c7b9726353701View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-05tf-9300000000-968a6390a00dcd77e566View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-01b9-0900000000-0f9d5926d702af7d4d63View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-01b9-1900000000-7f257033d3b1952b3c27View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4i-9800000000-bbcb0f0d7348cd1663b1View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,1H] 2D NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm
Biofluid Locations
  • Blood
  • Feces
  • Urine
Tissue Location
  • Cartilage
  • Connective Tissue
  • Kidney
Pathways
NameSMPDB/PathwhizKEGG
AlkaptonuriaPw000180Pw000180 greyscalePw000180 simpleNot Available
Disulfiram PathwayPw000431Pw000431 greyscalePw000431 simpleNot Available
Dopamine beta-hydroxylase deficiencyPw000474Pw000474 greyscalePw000474 simpleNot Available
HawkinsinuriaPw000181Pw000181 greyscalePw000181 simpleNot Available
Monoamine oxidase-a deficiency (MAO-A)Pw000509Pw000509 greyscalePw000509 simpleNot Available
Displaying entries 1 - 5 of 12 in total
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.043 (0.014 - 0.071) uMAdult (>18 years old)BothNormal details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedNormal details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)FemaleNormal details
UrineDetected and Quantified0.04 +/- 0.17 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified1.70 (0.5 - 2.8) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.0 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified1.917 +/- 1.061 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified35.5 (33.0 - 38.0) uMAdult (>18 years old)BothAlkaptonuria details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedCrohns disease details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedUlcerative colitis details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedUnclassified IBD details
UrineDetected and Quantified1.0 (0.0-2.0) umol/mmol creatinineAdult (>18 years old)BothAlkaptonuria details
UrineDetected and Quantified3000.0 (1000.0-5000.0) umol/mmol creatinineChildren (1-13 years old)BothHomogentisic 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 Quantified3.009 +/- 1.469 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
Associated Disorders and Diseases
Disease References
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. MetaGene
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB021899
KNApSAcK IDNot Available
Chemspider ID759
KEGG Compound IDC00544
BioCyc IDNot Available
BiGG ID1485308
Wikipedia LinkHomogentisic acid
METLIN ID331
PubChem Compound780
PDB IDOMD
ChEBI ID44747
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