Human Metabolome Database Version 3.5

Showing metabocard for 2-Hydroxyadipic acid (HMDB00321)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-05-29 13:25:36 -0600
Secondary Accession Numbers None
Metabolite Identification
Common Name 2-Hydroxyadipic acid
Description 2-Hydroxyadipic acid is a hydroxy-dicarboxylic acid formed by the reduction of 2-ketoadipic acid. Deficiency of 2-ketoadipic dehydrogenase causes 2-ketoadipic acidemia (OMIM 245130 Link_out), a condition characterized by accumulation and excretion of 2-hydroxyadipic acid (with 2-ketoadipic and 2-aminoadipic) probably without adverse phenotypic effects.(OMMBID - The Metabolic and Molecular Bases of Inherited Disease, CH.95). A method involving derivatization and combined gas chromatography--mass spectrometry has been recently developed to separate the enantiomers of 3-hydroxyadipic acid (PMID: 3980660 Link_out). It has been shown that 3-hydroxyadipic acid excreted in urine consists of at least 95% of the L-enantiomer. This finding supports the hypothesis that dicarboxylic acids are degraded by ordinary beta-oxidation, and indicates that adipic acid may be converted into succinic acid. (PMID: 3980660 Link_out).
Structure Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
  1. 2,3,4-Trideoxyhexarate
  2. 2,3,4-Trideoxyhexaric acid
  3. 2-Hydroxy-adipate
  4. 2-Hydroxy-adipic acid
  5. 2-Hydroxy-hexanedioate
  6. 2-Hydroxy-hexanedioic acid
  7. 2-Hydroxyadipate
  8. 2-Hydroxyadipic acid
  9. 2-Hydroxyhexanedioate
  10. 2-Hydroxyhexanedioic acid
  11. a-Hydroxyadipate
  12. a-Hydroxyadipic acid
  13. alpha-Hydroxyadipate
  14. alpha-Hydroxyadipic acid
  15. DL-2-Hydroxyadipate
  16. DL-2-Hydroxyadipic acid
Chemical Formula C6H10O5
Average Molecular Weight 162.1406
Monoisotopic Molecular Weight 162.05282343
IUPAC Name 2-hydroxyhexanedioic acid
Traditional IUPAC Name 2-hydroxyadipic acid
CAS Registry Number 18294-85-4
InChI Identifier InChI=1S/C6H10O5/c7-4(6(10)11)2-1-3-5(8)9/h4,7H,1-3H2,(H,8,9)(H,10,11)
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Organic Acids and Derivatives
Class Hydroxy Acids and Derivatives
Sub Class Medium-chain Hydroxy Acids and Derivatives
Other Descriptors
  • 2-hydroxydicarboxylic acid(ChEBI)
  • Aliphatic Acyclic Compounds
  • Dicarboxylic acids(Lipidmaps)
  • Fatty Acids and Conjugates
  • Alpha Hydroxy Acid
  • Carboxylic Acid
  • Dicarboxylic Acid Derivative
  • Secondary Alcohol
Direct Parent Medium-chain Hydroxy Acids and Derivatives
Status Detected and Quantified
  • Endogenous
Biofunction Not Available
Application Not Available
Cellular locations
  • Cytoplasm (predicted from logP)
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point Not Available Not Available
Boiling Point Not Available Not Available
Water Solubility Not Available Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 70.3 g/L ALOGPS
LogP -0.79 ALOGPS
LogP -0.38 ChemAxon
LogS -0.36 ALOGPS
pKa (strongest acidic) 3.44 ChemAxon
pKa (strongest basic) -3.8 ChemAxon
Hydrogen Acceptor Count 5 ChemAxon
Hydrogen Donor Count 3 ChemAxon
Polar Surface Area 94.83 A2 ChemAxon
Rotatable Bond Count 5 ChemAxon
Refractivity 34.23 ChemAxon
Polarizability 14.88 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge -2 ChemAxon
Gas-MS Spectrum
1H NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
MS/MS Spectrum GC-MS
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Urine
Tissue Location Not Available
Pathways Not Available
Normal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Expected but not Quantified
Not Applicable Not Available Not Available Comment Normal
  • Not Applicable
Urine Detected and Quantified
7.7 (0.1-19.6) umol/mmol creatinine Newborn (0-30 days old) Both Normal
Urine Detected and Quantified
7.3 (0.1-15.4) umol/mmol creatinine Infant (0-1 year old) Both Normal
Urine Detected and Quantified
2.2 (0.1-6.8) umol/mmol creatinine Children (1-13 year old) Both Comment Normal
Urine Detected and Quantified
1.6 (0.1-5.1) umol/mmol creatinine Children (1-13 year old) Both Comment Normal
Urine Detected and Quantified
2.4 (0.1-5.8) umol/mmol creatinine Adolescent (13-18 years old) Both Comment Normal
Urine Detected and Quantified
1.0 (0.0-2.0) umol/mmol creatinine Adult (>18 years old) Both Normal
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Urine Detected and Quantified 135.0 (50.0-220.0) umol/mmol creatinine Children (1-13 year old) Both 2-Ketoadipic Acidemia
Associated Disorders and Diseases
Disease References
2-ketoadipic acidemia
      Associated OMIM IDs
      DrugBank ID Not Available
      DrugBank Metabolite ID Not Available
      Phenol Explorer Compound ID Not Available
      Phenol Explorer Metabolite ID Not Available
      FoodDB ID FDB021947
      KNApSAcK ID Not Available
      Chemspider ID 167943 Link_out
      KEGG Compound ID C02360 Link_out
      BioCyc ID Not Available
      BiGG ID Not Available
      Wikipedia Link Not Available
      NuGOwiki Link HMDB00321 Link_out
      Metagene Link HMDB00321 Link_out
      METLIN ID 5310 Link_out
      PubChem Compound 193530 Link_out
      PDB ID Not Available
      ChEBI ID 17023 Link_out
      Synthesis Reference Griot, Rudolf. a-Hydroxyadipic acid. Helvetica Chimica Acta (1958), 41 2236-41.
      Material Safety Data Sheet (MSDS) Not Available
      General References
      1. Sewell AC, Heil M, Podebrad F, Mosandl A: Chiral compounds in metabolism: a look in the molecular mirror. Eur J Pediatr. 1998 Mar;157(3):185-91. Pubmed: 9537483 Link_out
      2. Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. Pubmed: 8087979 Link_out
      3. Inoue Y, Shinka T, Ohse M, Kuhara T: Differential chemical diagnosis of primary hyperoxaluria type II. Highly sensitive analysis of optical isomers of glyceric acid by GC/MS as diastereoisomeric derivatives. J Chromatogr B Analyt Technol Biomed Life Sci. 2005 Aug 25;823(1):2-6. Epub 2005 Apr 25. Pubmed: 16055048 Link_out
      4. Svendsen JS, Whist JE, Sydnes LK: Absolute configuration of 3-hydroxyadipic acid in human urine. J Chromatogr. 1985 Jan 11;337(1):9-19. Pubmed: 3980660 Link_out