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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:02:37 UTC
HMDB IDHMDB00396
Secondary Accession Numbers
  • HMDB02321
Metabolite Identification
Common Name2-Ethylhydracrylic acid
DescriptionIsolated excretion of 2-Ethylhydracrylic acid (2-methylbutyrylglycine; 2-MBG) is the hallmark of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), a defect in the proximal pathway of L-isoleucine oxidation (PMID 15615815 ). Normal human urine contains small amounts (less than 4 mg/g of creatinine) of 2-ethylhydracrylic acid. Urinary excretion of 2-ethylhydracrylic acid is variably increased in defects of isoleucine oxidation at distal steps in the catabolic pathway (3-oxoacyl-CoA thiolase deficiency and methylmalonyl-CoA mutase deficiency) and is diminished when proximal steps of the oxidative pathway are blocked as in branched-chain oxo acid decarboxylase deficiency ('maple-syrup-urine' disease). (PMID: 1016232 ).
Structure
Thumb
Synonyms
ValueSource
2-(Hydroxymethyl)-butyric acidChEBI
3-Hydroxy-2-ethylpropanoic acidChEBI
beta-Hydroxy-alpha-ethylpropionic acidChEBI
2-(Hydroxymethyl)-butyrateGenerator
2-EthylhydracrylateGenerator
3-Hydroxy-2-ethylpropanoateGenerator
b-Hydroxy-a-ethylpropionateGenerator
b-Hydroxy-a-ethylpropionic acidGenerator
beta-Hydroxy-alpha-ethylpropionateGenerator
β-hydroxy-α-ethylpropionateGenerator
β-hydroxy-α-ethylpropionic acidGenerator
2-(Hydroxymethyl)butyrateHMDB
2-(Hydroxymethyl)butyric acidHMDB
2-Ethyl-3-hydroxypropionateHMDB
2-Ethyl-3-hydroxypropionic acidHMDB
3-Hydroxy-2-ethylpropionateHMDB
3-Hydroxy-2-ethylpropionic acidHMDB
3-Hydroxy-propionic acid ethyl esterHMDB
Chemical FormulaC5H10O3
Average Molecular Weight118.1311
Monoisotopic Molecular Weight118.062994186
IUPAC Name2-(hydroxymethyl)butanoic acid
Traditional Name2-ethylhydracrylic acid
CAS Registry Number4374-62-3
SMILES
CCC(CO)C(O)=O
InChI Identifier
InChI=1S/C5H10O3/c1-2-4(3-6)5(7)8/h4,6H,2-3H2,1H3,(H,7,8)
InChI KeyInChIKey=ZMZQVAUJTDKQGE-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as beta hydroxy acids and derivatives. These are compounds containing a carboxylic acid substituted with a hydroxyl group on the C3 carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassHydroxy acids and derivatives
Sub ClassBeta hydroxy acids and derivatives
Direct ParentBeta hydroxy acids and derivatives
Alternative Parents
Substituents
  • Branched fatty acid
  • Beta-hydroxy acid
  • Fatty acyl
  • Fatty acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Primary alcohol
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locationsNot Available
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 Solubility387.0 mg/mLALOGPS
logP0.03ALOGPS
logP0.18ChemAxon
logS0.52ALOGPS
pKa (Strongest Acidic)4.51ChemAxon
pKa (Strongest Basic)-2.7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area57.53 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity28.22 m3·mol-1ChemAxon
Polarizability11.9 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, PositiveNot Available
Biological Properties
Cellular LocationsNot Available
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 Quantified3.9 (0.1-14.5) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified4.3 (0.1-6.5) umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified4.0 (1.0-13.3) umol/mmol creatinineChildren (1-13 years old)Both
Normal
details
UrineDetected and Quantified4.6 (1.0-7.3) umol/mmol creatinineChildren (1-13 years old)Both
Normal
details
UrineDetected and Quantified2.0 (1.2-6.4) umol/mmol creatinineAdolescent (13-18 years old)Both
Normal
details
UrineDetected and Quantified2.1(1.3-2.9) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified<2 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022014
KNApSAcK IDNot Available
Chemspider ID164207
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00396
Metagene LinkHMDB00396
METLIN ID3785
PubChem Compound188979
PDB IDNot Available
ChEBI ID741550
References
Synthesis ReferencePrice, Charles C.; Cypher, George A.; Krishnamurti, I. V. Thermal reaction of formaldehyde with allyl cyanide. Journal of the American Chemical Society (1952), 74 2987-91.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Korman SH, Andresen BS, Zeharia A, Gutman A, Boneh A, Pitt JJ: 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation. Clin Chem. 2005 Mar;51(3):610-7. Epub 2004 Dec 22. [15615815 ]
  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. [8087979 ]
  3. Ohdoi C, Nyhan WL, Kuhara T: Chemical diagnosis of Lesch-Nyhan syndrome using gas chromatography-mass spectrometry detection. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 15;792(1):123-30. [12829005 ]
  4. Mamer OA, Tjoa SS, Scriver CR, Klassen GA: Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites. Biochem J. 1976 Dec 15;160(3):417-26. [1016232 ]