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Record Information
Version3.6
Creation Date2006-05-22 14:17:30 UTC
Update Date2016-02-11 01:05:11 UTC
HMDB IDHMDB01987
Secondary Accession NumbersNone
Metabolite Identification
Common Name2-Hydroxy-2-methylbutyric acid
Description2-Hydroxy-2-methylbutyric acid is an unusual metabolite found in the urine of patients with 2-hydroxyglutaric aciduria and maple syrup urine disease. 2-Hydroxy-2-methylbutyric acid is also a secondary metabolite found in human urine after exposure to the gasoline additive tert-amyl-Me-ether (TAME). (PMID 11504147 , 10828258 , 9260660 , 11482739 ).
Structure
Thumb
Synonyms
ValueSource
(+/-)-2-hydroxy-2-methylbutanoateHMDB
(+/-)-2-hydroxy-2-methylbutanoic acidHMDB
(+/-)-2-hydroxy-2-methylbutyric acidHMDB
(+/-)-a-hydroxy-a-methylbutyric acidHMDB
(+/-)-alpha-hydroxy-alpha-methylbutyric acidHMDB
2-Hydroxy-2-methyl butyrateHMDB
2-Hydroxy-2-methyl butyric acidHMDB
2-Hydroxy-2-methyl-butanoateHMDB
2-Hydroxy-2-methyl-butanoic acidHMDB
2-Hydroxy-2-methyl-butyrateHMDB
2-Hydroxy-2-methyl-butyric acidHMDB
2-Hydroxy-2-methyl-N-butyrateHMDB
2-Hydroxy-2-methyl-N-butyric acidHMDB
2-Hydroxy-2-methylbutanoateHMDB
2-Hydroxy-2-methylbutanoic acidHMDB
2-Hydroxy-2-methylbutyrateHMDB
2-Methyl-2-hydroxybutyric acidHMDB
a-Hydroxy-a-methyl-butyric acidHMDB
a-Hydroxy-a-methylbutyric acidHMDB
alpha-Hydroxy-alpha-methyl-butyric acidHMDB
alpha-Hydroxy-alpha-methylbutyric acidHMDB
Chemical FormulaC5H10O3
Average Molecular Weight118.1311
Monoisotopic Molecular Weight118.062994186
IUPAC Name2-hydroxy-2-methylbutanoic acid
Traditional Name2-hydroxy-2-methylbutyric acid
CAS Registry Number3739-30-8
SMILES
CCC(C)(O)C(O)=O
InChI Identifier
InChI=1S/C5H10O3/c1-3-5(2,8)4(6)7/h8H,3H2,1-2H3,(H,6,7)
InChI KeyInChIKey=MBIQENSCDNJOIY-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentHydroxy fatty acids
Alternative Parents
Substituents
  • Hydroxy fatty acid
  • Methyl-branched fatty acid
  • Branched fatty acid
  • Hydroxy acid
  • Alpha-hydroxy acid
  • Tertiary alcohol
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point74 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility349.0 mg/mLALOGPS
logP0.13ALOGPS
logP0.48ChemAxon
logS0.47ALOGPS
pKa (Strongest Acidic)4.1ChemAxon
pKa (Strongest Basic)-3.8ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area57.53 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity28.08 m3·mol-1ChemAxon
Polarizability11.73 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, N/A (Annotated)splash10-0kml-9100000000-e255071f7864eb24d52bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, N/A (Annotated)splash10-0a4m-9000000000-182db94e75153cc5fe7eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, N/A (Annotated)splash10-0a4l-9000000000-0b6fff2f0bd2398fd3daView in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot ApplicableNot AvailableNot Available
Normal
  • Not Applicable
details
UrineDetected and Quantified2.8 +/- 1.5 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.998 +/- 0.926 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified0.8(0.4-1.5) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified14.4 +/- 3.9 umol/mmol creatinineAdult (>18 years old)Both
tert-Amyl-methyl ether exposed
details
UrineDetected and Quantified2.384 +/- 3.843 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified3.961 +/- 6.507 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
details
Associated Disorders and Diseases
Disease References
tert-Amyl-methyl ether exposed
  1. Amberg A, Rosner E, Dekant W: Biotransformation and kinetics of excretion of tert-amyl-methyl ether in humans and rats after inhalation exposure. Toxicol Sci. 2000 Jun;55(2):274-83. [10828258 ]
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022784
KNApSAcK IDNot Available
Chemspider ID86129
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB01987
Metagene LinkHMDB01987
METLIN ID6416
PubChem Compound95433
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceYoung, Wm. G.; Dillon, Robert T.; Lucas, Howard J. Synthesis of the isomeric 2-butenes. Journal of the American Chemical Society (1929), 51 2528-34.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Holmes E, Foxall PJ, Spraul M, Farrant RD, Nicholson JK, Lindon JC: 750 MHz 1H NMR spectroscopy characterisation of the complex metabolic pattern of urine from patients with inborn errors of metabolism: 2-hydroxyglutaric aciduria and maple syrup urine disease. J Pharm Biomed Anal. 1997 Jul;15(11):1647-59. [9260660 ]
  2. Amberg A, Rosner E, Dekant W: Biotransformation and kinetics of excretion of tert-amyl-methyl ether in humans and rats after inhalation exposure. Toxicol Sci. 2000 Jun;55(2):274-83. [10828258 ]
  3. Fu X, Kimura M, Iga M, Yamaguchi S: Gas chromatographic-mass spectrometric screening for organic acidemias using dried urine filter paper: determination of alpha-ketoacids. J Chromatogr B Biomed Sci Appl. 2001 Jul 5;758(1):87-94. [11482739 ]
  4. Dekant W, Bernauer U, Rosner E, Amberg A: Biotransformation of MTBE, ETBE, and TAME after inhalation or ingestion in rats and humans. Res Rep Health Eff Inst. 2001 May;(102):29-71; discussion 95-109. [11504147 ]