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Record Information
Version4.0
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-09-27 07:39:36 UTC
HMDB IDHMDB0000396
Secondary Accession Numbers
  • HMDB00396
  • 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 KeyZMZQVAUJTDKQGE-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group.
KingdomChemical entities
Super ClassOrganic compounds
ClassLipids and lipid-like molecules
Sub ClassFatty Acyls
Direct ParentHydroxy fatty acids
Alternative Parents
Substituents
  • Hydroxy fatty acid
  • Branched fatty acid
  • Beta-hydroxy acid
  • Hydroxy acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Primary alcohol
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Disposition

Biological Location:

  Biofluid and excreta:

Source:

  Biological:

    Animal:

Route of exposure:

  Enteral:

Role

Indirect biological role:

Biological role:

Process

Naturally occurring process:

  Biological process:

    Chemical reaction:

    Biochemical process:

    Biochemical pathway:

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 g/LALOGPS
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 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity28.22 m³·mol⁻¹ChemAxon
Polarizability11.9 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, PositiveNot AvailableView in JSpectraViewer
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, PositiveNot AvailableView in JSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0uxr-3900000000-f4f29b39d6efac6dea03View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0zfr-9500000000-033bb7e1b24d83c3260eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4i-9000000000-f9809158ef0031b0966eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-014i-7900000000-2b7594fdf2745541476dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00rl-9000000000-15a1a560605b3560b3c5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0006-9000000000-e23602be1497f191e28fView in MoNA
Biological Properties
Cellular LocationsNot Available
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
PathwaysNot Available
NameSMPDB/PathwhizKEGG
No entries found
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not Quantified Not AvailableNot Available
Normal
    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
    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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed:1016232 ]

    Enzymes

    General function:
    Involved in sulfotransferase activity
    Specific function:
    Sulfotransferase that utilizes 3'-phospho-5'-adenylyl sulfate (PAPS) as sulfonate donor to catalyze the sulfate conjugation of many hormones, neurotransmitters, drugs and xenobiotic compounds. Sulfonation increases the water solubility of most compounds, and therefore their renal excretion, but it can also result in bioactivation to form active metabolites. Sulfates hydroxysteroids like DHEA. Isoform 1 preferentially sulfonates cholesterol, and isoform 2 avidly sulfonates pregnenolone but not cholesterol.
    Gene Name:
    SULT2B1
    Uniprot ID:
    O00204
    Molecular weight:
    39598.595
    Reactions
    2-Ethylhydracrylic acid → 2-[(sulfooxy)methyl]butanoic aciddetails
    General function:
    Involved in transferase activity, transferring hexosyl groups
    Specific function:
    UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform glucuronidates bilirubin IX-alpha to form both the IX-alpha-C8 and IX-alpha-C12 monoconjugates and diconjugate. Is also able to catalyze the glucuronidation of 17beta-estradiol, 17alpha-ethinylestradiol, 1-hydroxypyrene, 4-methylumbelliferone, 1-naphthol, paranitrophenol, scopoletin, and umbelliferone.
    Gene Name:
    UGT1A1
    Uniprot ID:
    P22309
    Molecular weight:
    59590.91
    Reactions
    2-Ethylhydracrylic acid → 6-(2-carboxy-2-ethylethoxy)-3,4,5-trihydroxyoxane-2-carboxylic aciddetails
    2-Ethylhydracrylic acid → 3,4,5-trihydroxy-6-{[2-(hydroxymethyl)butanoyl]oxy}oxane-2-carboxylic aciddetails