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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-04-16 16:59:19 UTC
HMDB IDHMDB0000321
Secondary Accession Numbers
  • HMDB00321
Metabolite Identification
Common Name2-Hydroxyadipic acid
Description2-Hydroxyadipic acid is a hydroxy-dicarboxylic acid formed by the reduction of 2-ketoadipic acid. A deficiency of 2-ketoadipic dehydrogenase causes 2-ketoadipic acidemia or 2-oxoadipic acidemia (OMIM: 245130 ), a genetic disorder characterized by accumulation and excretion of 2-hydroxyadipic acid (with 2-ketoadipic and 2-aminoadipic) (OMMBID - The Online Metabolic and Molecular Bases of Inherited Disease, CH.95). When present in sufficiently high levels, 2-hydroxyadipic 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 2-hydroxyadipic acid are associated with at least three inborn errors of metabolism including 2-oxoadipic acidemia, 2-aminoadipic aciduria, and 2-oxoadipic aciduria. 2-Hydroxyadipic acid is 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, kidney abnormalities, liver damage, 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
Thumb
Synonyms
ValueSource
2-HydroxyadipateChEBI
2-Hydroxyhexanedioic acidChEBI
2-HydroxyhexanedioateGenerator
2,3,4-TrideoxyhexarateHMDB
2,3,4-Trideoxyhexaric acidHMDB
2-Hydroxy-adipateHMDB
2-Hydroxy-adipic acidHMDB
2-Hydroxy-hexanedioateHMDB
2-Hydroxy-hexanedioic acidHMDB
a-HydroxyadipateHMDB
a-Hydroxyadipic acidHMDB
alpha-HydroxyadipateHMDB
alpha-Hydroxyadipic acidHMDB
DL-2-HydroxyadipateHMDB
DL-2-Hydroxyadipic acidHMDB
Chemical FormulaC6H10O5
Average Molecular Weight162.1406
Monoisotopic Molecular Weight162.05282343
IUPAC Name2-hydroxyhexanedioic acid
Traditional Nameα-hydroxyadipic acid
CAS Registry Number18294-85-4
SMILES
OC(CCCC(O)=O)C(O)=O
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)
InChI KeyOTTXIFWBPRRYOG-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as medium-chain fatty acids. These are fatty acids with an aliphatic tail that contains between 4 and 12 carbon atoms.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentMedium-chain fatty acids
Alternative Parents
Substituents
  • Medium-chain fatty acid
  • Hydroxy fatty acid
  • Monosaccharide
  • Hydroxy acid
  • Dicarboxylic acid or derivatives
  • Alpha-hydroxy acid
  • Secondary alcohol
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Organic oxygen compound
  • Organooxygen compound
  • Alcohol
  • Carbonyl group
  • Organic oxide
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Indirect biological role:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility70.3 g/LALOGPS
logP-0.79ALOGPS
logP-0.38ChemAxon
logS-0.36ALOGPS
pKa (Strongest Acidic)3.44ChemAxon
pKa (Strongest Basic)-3.8ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area94.83 ŲChemAxon
Rotatable Bond Count5ChemAxon
Refractivity34.23 m³·mol⁻¹ChemAxon
Polarizability14.88 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-00c0-2920000000-14e371ab410997076c08View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00c0-2920000000-14e371ab410997076c08View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-00xu-9200000000-a5e63603281ceddefa05View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, Positivesplash10-01w0-9273000000-da104d83dee59e6410a4View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated)splash10-03di-2900000000-00b8bf461ba454c702f9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated)splash10-0ab9-9000000000-cbfcbe2f1e74c7fa8544View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated)splash10-052f-9000000000-1a3f1223d56568a37ca5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0002-1900000000-c97aa5c57fc34910b084View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00kb-9800000000-8b422e7bd31b7715f936View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0596-9000000000-145898d967788e6a4370View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-03dl-0900000000-b8d680487ea735b08660View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-014m-5900000000-2748f3664e3329b9721bView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0aba-9000000000-7bd0454dd4e607bf327dView in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue LocationNot Available
PathwaysNot Available
NameSMPDB/PathwhizKEGG
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodExpected but not Quantified Not AvailableNot Available
Normal
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Normal
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Normal
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified7.7 (0.1-19.6) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
    UrineDetected and Quantified7.3 (0.1-15.4) umol/mmol creatinineInfant (0-1 year old)BothNormal details
    UrineDetected and Quantified2.2 (0.1-6.8) umol/mmol creatinineChildren (1-13 years old)Both
    Normal
    details
    UrineDetected and Quantified1.6 (0.1-5.1) umol/mmol creatinineChildren (1-13 years old)Both
    Normal
    details
    UrineDetected and Quantified2.4 (0.1-5.8) umol/mmol creatinineAdolescent (13-18 years old)Both
    Normal
    details
    Abnormal Concentrations
    BiospecimenStatusValueAgeSexConditionReferenceDetails
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Colorectal cancer
    details
    UrineDetected and Quantified1.0 (0.0-2.0) umol/mmol creatinineAdult (>18 years old)Both2-ketoadipic acidemia
      • MetaGene: Metabol...
    details
    UrineDetected and Quantified135.0 (50.0-220.0) umol/mmol creatinineChildren (1-13 years old)Both2-Ketoadipic Acidemia
      • MetaGene: Metabol...
    details
    UrineDetected and Quantified80-170 umol/mmol creatinineChildren (1-13 years old)Not Specifiedalpha-Aminoadipic aciduria details
    UrineDetected and Quantified20 umol/mmol creatinineInfant (0-1 year old)Not Specified
    alpha-ketoadipic aciduria
    details
    UrineDetected and Quantified12.9 umol/mmol creatinineNewborn (0-30 days old)Not Specified
    alpha-ketoadipic aciduria
    details
    Associated Disorders and Diseases
    Disease References
    Alpha-aminoadipic aciduria
    1. Gray RG, O'Neill EM, Pollitt RJ: Alpha-aminoadipic aciduria: chemical and enzymatic studies. J Inherit Metab Dis. 1980;2(4):89-92. [PubMed:6796766 ]
    2-Ketoadipic acidemia
    1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
    Associated OMIM IDs
    DrugBank IDNot Available
    Phenol Explorer Compound IDNot Available
    FoodDB IDFDB021947
    KNApSAcK IDNot Available
    Chemspider ID167943
    KEGG Compound IDC02360
    BioCyc IDNot Available
    BiGG IDNot Available
    Wikipedia LinkNot Available
    METLIN ID5310
    PubChem Compound193530
    PDB IDNot Available
    ChEBI ID17023
    References
    Synthesis ReferenceGriot, Rudolf. a-Hydroxyadipic acid. Helvetica Chimica Acta (1958), 41 2236-41.
    Material Safety Data Sheet (MSDS)Not Available
    General References
    1. 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 ]
    2. 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 ]
    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 ]
    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 ]